Cerebral Palsy [2nd ed. 2020] 3319745573, 9783319745572

Table of contents :
Foreword
Preface
Acknowledgments
Acknowledgments from the First Edition
Contents
About the Editors
Contributors
Part I: Diagnosis and Pathology
1 The Child, the Parent, and the Goal in Treating Cerebral Palsy
Introduction
How Different Is the Child with CP?
Family Impacts of the Child with CP
Care-Providing Community
Cerebral Palsy Clinic
Family Care Provider and Professional Care Provider Relationship
Family Response Patterns
Dealing with Blame
Giving and Dealing with Prognosis
Giving the Diagnosis
Medical Therapeutic Relationship to Child and Family
The Physical Therapist Relationship
When the Doctor-Family Relationship Is Not Working
When the Family Chooses Medical Treatment Against the Physician´s Advice
Recommending Surgery
A Plan for Managing Complications
When Complications Occur
The Final Goal
Cases
References
Part II: Etiology of Cerebral Palsy
2 Cerebral Palsy and the Relationship to Prematurity
Introduction
Natural History
Prevalence of Cerebral Palsy in Premature Infant
Etiologies of Cerebral Palsy Related to Prematurity
Intraventricular Hemorrhage (IVH)
Periventricular Leukomalacia (PVL)
Chorioamnionitis
Postnatal Glucocorticoid Therapy
Bronchopulmonary Dysplasia
Apnea of Prematurity
Neonatal Sepsis
Patent Ductus Arteriosus
Hypoxic-Ischemic Encephalopathy
Necrotizing Enterocolitis
Hypocarbia
Hyperbilirubinemia
Treatment
Perinatal and Postnatal Interventions to Reduce the Risk of Cerebral Palsy in Preterm Infants
Antenatal Glucocorticoids
Magnesium for Neuroprotection
Caffeine for Apnea of Prematurity
Delayed Cord Clamping
Follow-Up of the Premature Infant
Conclusion
References
3 Genetic Abnormalities and Congenital Malformations as a Cause of Cerebral Palsy
Introduction
Evolving Evidence in the Genetics of Cerebral Palsy
Natural History
Congenital Anomalies and Coexisting Conditions
Other Contributing Causes of Cerebral Palsy
Intrauterine Growth Restriction
Multiple Pregnancy
Intrauterine Infection
Thrombophilia
Hypoxia-Ischemia
Prematurity
Single-Gene Causes of Cerebral Palsy
Copy Number Variants
Recommendation for Treatment/Assessments
Cross-References
References
4 Infectious Etiologies of Cerebral Palsy
Introduction
Natural History
Epidemiology and Pathophysiology
Etiologies
Cytomegalovirus (CMV)
Testing, Treatment, and Outcomes
Herpes Simplex Virus (HSV) and Other Human Herpes Viruses
Testing, Treatment, and Outcomes
Enteroviruses and Parechoviruses
Testing, Treatment, and Outcomes
Emerging Viruses: Chikungunya Virus and Zika Virus
Testing, Treatment, and Outcomes
Neonatal Bacterial Pathogens
Testing, Treatment, and Outcomes
Other Pathogens to Consider
Testing, Treatment, and Outcomes
References
5 Perinatal Stroke as an Etiology of Cerebral Palsy
Introduction
Natural History
Epidemiology
Risk Factors
Pathophysiology
Diagnosis
Treatment
Acute Treatment
Chronic Treatment
Prevention
Complications of Stroke and Treatment
Conclusions
Cross-References
References
6 Problems During Delivery as an Etiology of Cerebral Palsy in Full-Term Infants
Introduction
Natural History
Problems During Birth as an Etiology of CP
Prematurity
Hypoxic-Ischemic Injury
Low Apgar Scores
Abnormal FHR Tracing
Meconium-Stained Amniotic Fluid
Intracranial Hemorrhage
Perinatal Stroke
Abnormal Labor
Umbilical Cord Complications
Placental Complications
Placental Abruption
Placental Infarction
Uterine Rupture
Instrumentation at Delivery
Fetal Presentation
Multiple Births
Chorioamnionitis
Treatment
Perinatal and Postnatal Interventions to Reduce the Risk of Cerebral Palsy
Complications
Cross-References
References
7 Postnatal Causes of Cerebral Palsy
Introduction
Natural History
Infectious Causes
Neonatal Sepsis
Viral Infections
Trauma
Congenital Heart Defects
Stroke
Neoplasms
Summary
Cross-References
References
Further Reading
8 Animal Models of Cerebral Palsy: What Can We Learn About Cerebral Palsy in Humans
Introduction
Studies in Mice
Infection/Inflammation model
Hypoxic/Ischemic Model
Studies in Rats
Hypoxic/Ischemic Model
Infection/Inflammation Model
Studies on the Effectiveness of Treatment Methods
Studies in Rabbits
Hypoxic/Ischemic Model
Infection/Inflammation Model
Studies in Sheep
Hypoxic/Ischemic Model
Infection/Inflammation Model
Studies on the Effectiveness of Treatment Methods
Studies in Nonhuman Primates
Hypoxic/Ischemic Model
Infection/Inflammation Model
Studies on the Effectiveness of Treatment Methods
Studies in Other Animals
Conclusion
Cross-References
References
9 The Effects of Umbilical Cord Blood and Cord Tissue Cell Therapies in Animal and Human Models of Cerebral Palsy
Introduction
Natural History
Treatment
Animal Studies of Cell Therapy in Brain Injuries
Animal Studies in Stroke
Animal Studies in Hypoxic/Ischemic Brain Injury
Animal Studies in Intraventricular Hemorrhage (IVH) and Periventricular Leukomalacia (PVL)
Summary of Animal Studies
Human Studies of Cell Therapy in Brain Injuries
Clinical Trials of Autologous Umbilical Cord Blood in CP
Clinical Trials of Allogeneic Umbilical Cord Blood in CP
Clinical Trials of MSCs in CP
Clinical Trials of CB in Babies with Hypoxic/Ischemic Encephalopathy (HIE)
Complications
Summary
Cross-References
References
10 Risk Factors for Developing Cerebral Palsy
Introduction
Gestational Age and Birth Weight
Prematurity as a Risk Factor for Cerebral Palsy
Prevention of CP in Preterm Infants
Birth Weight as Related to Gestational Age
Prevention of CP in Infants with Deviations from Optimal Intrauterine Growth
Twin or Multiple Birth
Assisted Reproductive Technology and the Risk for CP
Prevention of CP in Twins or Multiple Births
Maternal Risk Factors
Prevention of CP Related to Maternal Factors
Congenital Infections
Prevention of CP Related to Congenital Infections
Congenital Malformations
Prevention of CP Related to Congenital Malformations
Coagulopathies
Prevention of CP Related to Coagulopathies
Genetic Variants and CP
Prevention of CP Related to Genetic Causes
Perinatal Risk Factors
Prevention of CP Related to Perinatal Causes
Neonatal Risk Factors
Prevention of CP Related to Neonatal Causes
Post-neonatally Acquired CP
Prevention of CP Related to Postnatal Causes
Epilogue
Cross-References
References
Part III: Epidemiology
11 Epidemiology of Cerebral Palsy
Introduction
Definition and Classification of CP
Frequency and Patterns of Occurrence
Birth Prevalence: Overall Trends
Trends by Birth Weight and Gestational Age
Trends in Motor Severity and CP Subtypes
Accompanying Impairments
Survival in CP
Major Risk Factors
Multiple Birth
Congenital Anomalies
Congenital Cytomegalovirus
Prevention of CP
References
12 Health and Healthcare Disparities in Children with Cerebral Palsy
Introduction
The Disabled Population as a Population with Health and Healthcare Disparities
Health and Healthcare Disparities in Children with Special Healthcare Needs
Health and Healthcare Disparities in Children with Special Healthcare Needs With and Without Disability and/or Medical Complex...
Health and Healthcare Disparities in Children with Cerebral Palsy
Racial Variance Within Cerebral Palsy Prevalence
Socioeconomic Impact on Cerebral Palsy Prevalence
Identifying Vulnerability Causing Health and Healthcare Disparities in the Cerebral Palsy Population
The Measurement of Healthcare Disparities in Cerebral Palsy
Strategies to Resolve Healthcare Disparities in the Cerebral Palsy Population
Predisposing and Enabling Factor Disparity Interventions
Need Factor Interventions Within the CP Population
The Medical Home and Care Coordination
Disparities in the Transition into Adult Healthcare
Transition of Orthopedic Services in the CP Population
Quality, Cost, and Value: Their Impact and Importance on Disparities in Children with Disabilities
Value for CSHCN, CMC, and Children with CP: The Patient and Family Perspective
Value of CSHCN, CMC, and Children with CP: The Provider and Payor Perspectives
Value and Healthcare Delivery Models in CSHCN, CMC, and Children with CP
Value and Alternative Payment Models in CSHCN, CMC, and Children with Cerebral Palsy
A High-Value Musculoskeletal Model of Care Delivery for the CP Child
Health Policy to Prevent Health and Healthcare Disparities in CSHCN
Cases
Case 1 (Pre-care Coordination) (Fig. 7)
Case 2 (Fig. 8)
References
Part IV: Pathology
13 Neuroimaging Pathology in Cerebral Palsy
Introduction
Fetal Neuroimaging Techniques
Hypoxic-Ischemic Brain Injury
Preterm
White Matter Injury of Prematurity or Periventricular Leukomalacia (Mild to Moderate Hypoperfusion)
Profound Hypotension in Preterm Infants
Germinal Matrix and Intraventricular Hemorrhage
Porencephalic Cyst
Term Infants
Watershed Predominant Pattern of Injury
Basal Ganglia/Thalamus Pattern
Perinatal Stroke
Congenital Infections of the Central Nervous System
Cytomegalovirus
Toxoplasmosis
Lymphocytic Choriomeningitis Virus
Congenital Malformations
Lissencephaly (The Agyria-Pachygyria Complex)
Microcephaly with Simplified Gyral Pattern (MSG)
Schizencephaly
Megalencephaly-Postaxial Polydactyly-Polymicrogyria-Hydrocephalus Syndrome (MPPH)
Septo-Optic Dysplasia
18q-Syndrome
Syntelencephaly
Joubert Syndrome and Related Disorders (Molar Tooth Malformations)
Rhombencephalosynapsis
Aicardi Syndrome
Hydranencephaly
Miscellaneous
Kernicterus
Conclusion
Cross-References
References
14 Current Imaging: PET Scan Use in Cerebral Palsy
Introduction
Cranial Ultrasonogram
Magnetic Resonance Imaging
Positron Emission Tomography
Diffusion Tensor Imaging
Conclusion
Cross-References
References
15 Neuromuscular Junction Changes in Spastic Cerebral Palsy
Introduction
Structure and Action of the NMJ
NMJ Formation during Development
Postsynaptic Maturation
Presynaptic Maturation
NMJ Microanatomic Organization in CP
NMJ Ultrastructure in CP
NMJ Gene Expression in CP
Medications That Target NMJs
Conclusion
Cross-References
References
16 Muscle Changes at the Cellular-Fiber Level in Cerebral Palsy
Introduction
Muscle Growth
Longitudinal Growth and Sarcomere Addition
Postnatal Development
Sarcomere Adaptation
Sarcomeres in Children with CP
Extracellular Matrix
Changes in ECM Content
Passive Mechanical Properties of Muscle Fibers and Bundles
Muscle Stem Cells, Postnatal Development, and Contractures
Satellite Cells (Muscle Stem Cells)
Function of Satellite Cells
Satellite Cells in Children with Cerebral Palsy
Summary
References
17 Muscle Size, Composition, and Architecture in Cerebral Palsy
Introduction
Natural History
Muscle Anatomy and Typical Muscle Growth and Development
Skeletal Muscle Size and Architecture in Typically Developing Children
Skeletal Muscle Size, Composition, and Architecture in Children with CP
Assessing Muscle in Children with CP
Factors Contributing to Atypical Muscle Growth and Development in Children with CP
Physical Activity
Muscle Spasticity
Medications
Surgery
Treatment
Summary
Cross-References
References
18 Bone Size, Architecture, and Strength Deficits in Cerebral Palsy
Introduction
Natural History
Bone Anatomy and Typical Bone Growth and Development
Bone Growth and Development in Children with CP
High Rate of Fragility Fractures in Children with CP
Assessing Bone in Children with CP
Factors Contributing to Atypical Bone Growth and Development in Children with CP
Gross Motor Function and Physical Activity
Muscle
Nutrition
Medications
Bone Health in Adults with CP
Treatment
Summary
Cross-References
References
Part V: Diagnosis
19 When and How to Evaluate the Child with Possible Cerebral Palsy
Introduction
Etiology and Pathology
Congenital (Antenatal) Etiologies
Neonatal and Perinatal Etiologies
Postnatal Etiologies
Classification of Cerebral Palsy
Diagnosis of Cerebral Palsy
Clinical Diagnosis
Neuroimaging
Metabolic and Genetic Testing
Coagulopathies
Diagnostic Evaluations for Associated Conditions
Conclusion
Cross-References
References
20 Cerebral Palsy Prognosis Based on the Physical and Neurologic Examination
Introduction
Making the Diagnosis
Early Diagnostic Uncertainty Complicates Early Prognosis
Developing a Prognosis
Prognosis: Comorbidities and Life Expectancy
The Challenge of Masqueraders
Early Neurologic Examination Predicting Specific CP Syndromes
Specific Syndromes
Conclusion
Cross-References
References
21 Classification Terminology in Cerebral Palsy
Introduction
Goals and Environment
Technique
Type and Topography of Neuromotor Impairment
Spasticity
Dyskinesia
Ataxia
Hypotonia
Neuroanatomical Classifications
Gait Pattern Classifications
Functional Classification Systems
Gross Motor Function
Manual Ability
Communication
Evidence of Effectiveness
Cross-Reference
References
22 Measuring Outcomes in Children with Cerebral Palsy
Introduction
Generic Versus Disease-Specific Measures
Self-Report Versus Parent Proxy Reporting
International Classification of Functioning
Functional Outcome Measures
Pediatric Outcomes Data Collection Instrument (PODCI)
Gillette Functional Assessment Questionnaire (FAQ)
Shriner´s Hospital Upper Extremity Evaluation (SHUEE)
Pediatric Evaluation of Disability Inventory (PEDI) and Pediatric Evaluation of Disability Inventory Computer-Adaptive Test (P...
Quality of Life/Health-Related Quality of Life Measures
Cerebral Palsy Quality of Life Questionnaire (CP-QOL-Child)
Cerebral Palsy Quality of Life Questionnaire (CP-QOL-Teen)
Caregivers Priorities and Child Health Index of Life with Disabilities (CPCHILD)
Pediatric Quality of Life Inventory (PedsQL) 3.0 Cerebral Palsy (CP) Module
DISABKIDS-CP Module (CPM)
Conclusion
Cross-References
References
23 Biomarker Blood Tests for Cerebral Palsy
Introduction
Types and Classes of Biomarkers
Basics of Diagnostic Biomarker Test Performance
Circulating Biomarkers in the Blood
Epigenetic Biomarkers and DNA Methylation in Blood Cells
Conclusion
Cross-References
References
Part VI: General Medical Concerns
24 General Nutrition for Children with Cerebral Palsy
Introduction
Natural History
Etiology of Impaired Growth in Children with Cerebral Palsy
Evaluating Growth in the Child with Non-ambulatory Cerebral Palsy
Evaluating for Nutritional Deficiencies in the Child with Cerebral Palsy
Role of Diet in Bone Health
Treatment
Nutritional Requirements
Nutrition Interventions
Oral Nutrition
Enteral Nutrition
Bone Health
Follow-Up
Complications of the Treatment and Disease Process
Complications of Calorie Boosting
Complications of Enteral Tube Feedings
Complications of Mineral Supplementation for Bone Health
Cross-References
References
25 Managing the Child with Cerebral Palsy Who Has Medical Complexity
Introduction
Role of Primary Care
Care Coordination
Practice Transformation
Models of Care
Medical Care
Transition
Payments
Cases
Case 1: Agitation
Case 2: Fever
Cross-References
References
26 Managing Bone Fragility in the Child with Cerebral Palsy
Introduction
Natural History
Cerebral Palsy
Medical Effects of CP
Primary Effects
Secondary Medical Problems
Bone Basics
Gross Motor Function Classification System
Relationship of BMD and Fracture
Pathophysiology/Etiology of Compromised Bone Health
Malnutrition/Suboptimal Nutrition
Puberty
Weight Bearing
Medications
Treatment and Complications: Identification and Prevention
Identification of Risk Factors and Prevention
Review Medical Risk Factors
Medication Selection/Consideration
Nutrition Assessment
Laboratory Evaluation
Weight Bearing
Standing
Vibration
Assessment of Bone Density
Handling/Mechanics
Education of Care Providers (School, Nurses/Aides, Families)
Pharmacologic Treatment
Bisphosphonates
Other
Summary/Wrap Up
Case Studies
Cross-References
References
27 Managing Irritability and Nonoperative Pain in the Noncommunicative Child with Cerebral Palsy
Introduction
Natural History
Assessment of Pain
Treatment and Complications
Gastrointestinal Etiologies of Pain
Constipation
Gastroesophageal Reflux
Feeding Intolerance
Visceral Hyperalgesia
Musculoskeletal
Spasticity
Treatment and Complications
Dystonia
Treatment and Complications
CNS Shunt Malfunction
Complications
Surgical Complications
Muscle Overuse Injuries
Occult Fractures
Treatment and Complications
Kidney Stones
Treatment and Complications
Paroxysmal Sympathetic Hyperactivity
Treatment and Complications
Conclusion
Cases
Cross-References
References
28 Palliative Care for Individuals with Cerebral Palsy
Introduction
Natural History
Advance Care Planning
Decision-Making Support
Assessing Medical Understanding
Goals of Care
End of Life Care
Case Discussion
Case Discussion
Pain and Symptom Management
Care Coordination
Anticipatory Grief/Bereavement
Family Support
Treatment
Case Discussion
Complications
Cross-References
References
29 Aging with Cerebral Palsy: Adult Musculoskeletal Issues
Introduction
Spine
Hip
Lower Extremity, Knee, and Foot
Rehabilitation Issues
Health Care System Issues for the Adult
Conclusion
Cross-References
References
30 Life Care Planning for the Child with Cerebral Palsy
Introduction
Definition of a Life Care Plan
Purpose of a Life Care Plan
Goals and Environment
Healthcare Providers
Treatment Interventions
Common Nonsurgical Interventions
Common Orthopedic Surgical Interventions
Diagnostics
Medications
Medication for Spasticity
Antiepileptic Medication
Cognitive and Behavioral Medication
Medication for Incontinence
Medication for Acid Reflux
Medication for Depression/Anxiety
Laboratory
Therapy
Traditional Therapy
Nontraditional Therapy
Mental Health Therapy
Education
School
Educational Advocate
Educational Therapist/Tutor
Vocational Rehabilitation Program
Day Program
Assistive Technology
Activities of Daily Living Equipment
Mobility Equipment
Assistive Technology for Cognition
Orthotic Devices
Nursing/Attendant Care
Care Attendant
Respite Care
Life Skills Coach
Care Facility
Professional Services
Fiduciary/Trustee
Attorney
Conservatorship/Guardianship
Benefits/Resources
Federal and State Benefit Programs
Social Security Benefits (All States) and Supplemental Security Income (SSI)
Social Security Disability Insurance (SSDI)
Medi-Cal (California)/Medicaid (All Other States)
Developmental Disability Services
Department of Social Services
Department of Rehabilitation
Recreation
Camps
Organizations
Home Modifications
Transportation
Technique
Case History
Cross-References
References
Resources
Part VII: Central Neurologic Problems
31 Epilepsy in the Child with Cerebral Palsy
Introduction
Natural History
Seizures and Epileptogenesis
Clinical Features of Seizures and Epilepsy in a Child with Cerebral Palsy
Treatment Considerations
The Diagnosis of Epilepsy in the Cerebral Palsies and the Role of EEG
Treatment of Epilepsy Syndromes and Seizure Categories in Cerebral Palsy
Specific Epilepsy Considerations and Syndromes in Cerebral Palsy
Epilepsy Remission in Cerebral Palsy
Complications of Epilepsy and Its Treatment in Cerebral Palsy
Summary
Cross-References
References
32 Epilepsy Surgery for the Child with Cerebral Palsy
Introduction
Impact
When Should Surgery Be Considered?
Evaluation for Epilepsy Surgery
Studies
Invasive or Intracranial EEG Monitoring
Epilepsy Surgery Conference
Types of Epilepsy Surgery
Resective Surgeries
Hemispherectomy
Selection
Infancy
Outcome
Complications
Other Resective Surgical Techniques Including Lesionectomy and Multilobar and Lobar Resection
Corpus Callosotomy
Indication
Adverse Effect
Neurostimulation
VNS
Minimally Invasive Epilepsy Surgery
Conclusion
Cross-References
Bibliography
33 Hydrocephalus in the Child with Cerebral Palsy
Hydrocephalus in Cerebral Palsy
Introduction
Natural History
Anatomy and Physiology
Physical Findings
Causes
Treatment
Complications
Cross-References
References
Part VIII: Psychologic and Psychiatric Problems
34 Psychiatric Disorders in Children with Cerebral Palsy
Introduction
Natural History
Identification
Incidence/Prevalence
Evaluation
Treatment/Remediation
Illustrative Case Study
Recognition by IEP Regarding Motor Issues/Condition
Conclusion/Areas for Future Studies
Cross-References
References
35 Autism Spectrum Disorder in the Child with Cerebral Palsy
Introduction
Definition of CP and ASD
Epidemiology
Diagnosis
Challenges in ASD Diagnosis for Children with CP
Natural History
Comorbidities and Common Risk Factors
Preterm Birth/Low Birth Weight
Maternal Infection and Inflammation
Perinatal Hypoxia/Ischemia
Genetic Factors
Epilepsy
Intellectual Disability
Motor Coordination Abnormalities
Treatment
Medical Testing
Medical Treatments
Pharmacotherapy
Rehabilitative Therapies
Early Intensive Behavioral Interventions (ABA, Developmental Models)
Complementary and Alternative Medicine
Conclusion
Cross-References
References
36 Family Stress Associated with Cerebral Palsy
Introduction
Parent Stress
Psychological Well-Being
Physical Health Outcomes
Disability Severity
Family Adaptation
Parent Personal Resources: Social Support and Self-Efficacy
Assessment Tools and Interventions
The Role of Respite Care Services
Financial Resources and Socioeconomic Status
Psychosocial Interventions and Parent Stress
Conclusions
Cross-References
References
37 The Impact of Cerebral Palsy on Siblings
Introduction
Children: Playmates, Mentors, and Friends
Challenges
Benefits
Adults: Caregivers, Supports, and Friends
Caregiving
Benefits
Challenges
Advice to Healthcare Professionals
Conclusion
Cross-References
References
Part IX: Neuromotor Function
38 Motor Control and Muscle Tone Problems in Cerebral Palsy
Introduction
Pathophysiology
Anatomic Motor Control Structure
Central Motor System
Peripheral Motor Control
Development of the Anatomic Structure
Central Nervous System
Peripheral Motor System
Controller Mechanisms and Theory
Sensory System Feedback Versus Feed-Forward Control
Controller Options: Maturation Theory
Controller Options: Dynamic Systems Theory
The Cause of Chaotic Attractors
A Unified Theory of Motor Control
Pathology Treatments
Disorders of Muscle Tone
Motor Tone
Measuring Muscle Tone
Spasticity
Effects of Spasticity on Nerves
Effects of Spasticity on Muscles and Tendons
Effects of Spasticity on Bones
Functional Effects of Spasticity on Sitting, Gait, and Activities of Daily Living
Hypotonia
The Effects of Hypotonia
Functional Problems
Treatments of Tone
Movement Disorders
Motor Control: Movement Disorders
Dystonia
Secondary Effects of Dystonia
Athetosis
Sensory Motor Effects of Athetosis
Treatment
Treatment: Therapy
Chorea and Ballismus
Summary of Motor Control Treatments
Disorders of Balance (Ataxia)
Treatment of Ataxia
Orthotics
Summary of Treatment: Ataxia
Cases
Cross-References
References
39 Spasticity Assessment in Cerebral Palsy
Introduction
Joint-Level Assessments to Infer About Muscle Function
Definitions
Measurement Methods
Measurement Errors
Qualitative Assessment Methods
Quantitative Assessment Methods
Passive Muscle Assessments
Active Muscle Assessments
Clinical Interpretation of Instrumented Assessments
Conclusion
Cross-References
References
40 Medical Management of Spasticity in Children with Cerebral Palsy
Introduction
Pathophysiology
Assessment
Treating Spasticity
Therapy Services
Bracing and Positioning
Chemodenervation
Oral Medications
Intrathecal Baclofen
Neurosurgery
Orthopedic Surgery
Summary Discussion
Conclusion
Cross-References
References
41 Focal Management of Spasticity in Cerebral Palsy
Introduction
Natural History
Effects of Spasticity on Nerves
Effects of Spasticity on Muscles and Tendons
Effects of Spasticity on Bones
Functional Effects of Spasticity on Sitting, Gait, and Activities of Daily Living
Treatments
Peripheral Nervous System
Neuromotor Junction and the Muscle
Botulinum Toxin (Botox)
Complications of Botulinum Toxin
Alcohol and Phenol
Direct Surgical Treatment of the Musculotendinous Unit
Orthotics
Therapy
A Global Approach to Managing Spasticity
Cases
Cross-References
References
42 Intrathecal Baclofen Therapy: Assessment and Medical Management
Introduction
History
Pharmacology of Baclofen
Criteria
Screening Trial
Pump Implantation
Pump Management
Complications
Outcomes
Summary
References
43 Intrathecal Medication Administration in Cerebral Palsy
Introduction
Natural History
Treatment
Complications
Cross-References
References
44 Dorsal Rhizotomy for Spasticity Management in Cerebral Palsy
Introduction
Some History
Treatment
Patient Selection
Decision Procedure
General Selection Criteria for SDR
Individual Selection Criteria for SDR
Timing of SDR Surgery
Surgical Procedure
Neurophysiologic Intraoperative Monitoring
Postoperative Rehabilitation Program
Expected Outcome
Complications
Conclusions
References
45 Dystonia and Movement Disorders in Children with Cerebral Palsy
Introduction
Pathophysiology
Dystonia
Athetosis, Chorea, and Choreoathetosis
Natural History
Treatment
Dystonia
Medications
Global Treatment
Focal Dystonia Treatment
Secondary Effects of Dystonia
Athetosis and Choreoathetosis
Treatment
Treating Secondary Effects of Athetosis
Chorea and Ballismus
Conclusion
Cases
Cross-References
References
46 Deep Brain Stimulation for Pediatric Dystonia
Introduction
Epidemiology
Preliminary Management
History of Surgical Interventions for Dystonia
Theoretical Mechanism of DBS
Preoperative Planning and Lead Implantation
Implantable Pulse Generators
Complications
Functional Outcome
Future Directions
Cross-References
References
47 Ataxia and Disorders of Balance in Children with Cerebral Palsy
Introduction
Balance Components
Ataxia
Vestibular System
Common Vestibular Disorders
Conclusion
Cross-References
References
48 Assessing Dynamic Balance in Children with Cerebral Palsy
Introduction
Natural History
Treatment
Testing
Task-Oriented Assessment of Dynamic Balance
Timed or Distance-Based Walking Tests of Dynamic Balance
Marker-Based Assessment of Dynamic Balance
Other Instrumented Assessment of Dynamic Balance
Treatment
Exercise/Therapy
Vestibular Stimulation
Taijiquan
Body Weight-Supported Treadmill Training
Virtual Reality and Interactive Gaming
Hippotherapy
Vibrational Therapies
Electrical Stimulation Therapies
Surgical Interventions
Complications
Cross-References
References
Part X: Gastrointestinal
49 Overview of Feeding and Growth in the Child with Cerebral Palsy
Introduction
Natural History
Prevalence and Pathophysiology
Etiology of Feeding and Growth Problems
Oral-Motor Dysfunction
Inappropriate Dietary Intake
Caregiver Dependency
Abnormal Energy Expenditure
Evaluations/Assessments
Ability to Take in Adequate Nutrition
Growth and Anthropometric Measurements
Physical Examination
Swallow Study
Adequacy of Intake and Energy Needs
Laboratory Evaluation
GI Concerns
Social History
Treatment/Management of Problems
Intake of Nutrition/Feeding Interventions
Nutritional Adequacy
Other Medical Management
Conclusion
Cross-References
References
50 Gastrostomy and Jejunostomy Feedings in Children with Cerebral Palsy
Introduction
Oropharyngeal Dysphagia
Evaluation
Gastroesophageal Reflux
Evaluation
Management
Gastrostomy Tube
Gastrojejunostomy
Complications of Gastrostomy and Gastrojejunostomy Tubes
During Placement
Post-placement Complications
Conclusion
Cases
Cross-References
References
51 Gastroesophageal Reflux in the Child with Cerebral Palsy
Introduction
Natural History
Pathophysiology and Etiology
Clinical Presentation and Symptoms
Treatment
Diagnosis and Testing
Esophagogastroduodenoscopy (EGD) and Biopsy
Esophageal pH Monitoring (EpHM)
Combined Multiple Intraluminal Impedance and pH Monitoring (CMII)
Barium Contrast Radiography/Upper GI (UGI) Series
Nuclear Scintigraphy/Gastric Emptying Scan (GES)
Management
Conservative Management
Pharmacologic Management
Surgical Management
Complications
Reflux Esophagitis and Peptic Strictures
Barrett Esophagus and Adenocarcinoma
Conclusion
Cross-References
References
52 Medical and Surgical Therapy for Constipation in Patients with Cerebral Palsy
Introduction
Natural History
Physiopathology
Treatment
History and Physical Exam
Abdominal Radiography
Sitz Marker Study
Motility Studies
Rectal Biopsies
Medical Therapy
Retrograde Enemas and Fecal Disimpaction
Antegrade Enteral Cleanout at Home
Admission for Bowel Cleanout
Maintenance Therapy
Diet
Medications that Induce Constipation
Complications
Surgical Therapy and Interventions
Medically Refractory Constipation
Volvulus
Perforation
Summary
Case Example
References
Part XI: Ear, Nose, and Throat
53 Medical Management of Sialorrhea in the Child with Cerebral Palsy
Introduction
Natural History
Physiology
Pathophysiology
Clinical Assessment of Drooling
Treatment
Non-pharmacologic Management of Drooling
Pharmacologic Interventions for Drooling
Botulinum Toxin Injections
Outcomes
Technique
Adverse Effects
Surgery
Future Directions
Cross-Reference
References
54 Surgical Options for Sialorrhea Management in Children with Cerebral Palsy
Introduction
Noninvasive Therapies
Pharmaceutical Therapies
Botulinum Toxin Injections
Surgical Management
Conclusion
Cross-References
References
55 Auditory Rehabilitation in Children with Cerebral Palsy
Introduction
Diagnosis of Hearing Loss
Treatment
Conventional Amplification
Cochlear Implantation
Candidacy Assessment with Audiology
Candidacy Assessment with Speech and Language Pathology
Candidacy Assessment with Otolaryngology
Candidacy Assessment with Social Work
Preoperative Counseling
Intraoperative Considerations
Complications
Postoperative Rehabilitation
Conclusion
Cross-References
References
56 Upper Airway Obstruction in the Child with Cerebral Palsy: Indication for Adenotonsillectomy
Introduction
Prevalence
Etiology
Impact on Quality of Life
Diagnostic Considerations
Treatment of OSA in CP
Tonsillectomy
Additional Surgical Interventions
The Role of Tracheostomy
Postoperative Management and Complications
Cross-References
References
57 Surgical Management of Tracheostomies and Tracheal Diversion in Children with Cerebral Palsy
Introduction
History
Natural History and Pathophysiology
Indications
Treatment
Tracheostomy Technique
Diversion Techniques
Complications
Complications After Tracheostomy
Immediate Complications
Early Complications
Late Complications
Complications of Tracheal Diversion
Conclusions
Cross-References
References
Part XII: Genitourinary
58 Toilet Training and Bladder Control in Children with Cerebral Palsy
Introduction
Natural History
Normal Toilet Training and Voiding Review
Voiding Issues in Upper Motor Neuron Versus Lower Motor Neuron Lesions
Factors Influencing Toilet Training in Children with CP
Effect of Constipation on Voiding
Summary of Toilet Training
Treatment
Urologic Evaluation
Noninvasive Testing
Invasive Testing: Urodynamic Studies
Treatment Options
Environmental Modification and Communication
Bowel Management
Medications
Anticholinergic Medications
Desmopressin
Bladder Catheterization
Selective Dorsal Rhizotomy
Complications
Upper Urinary Tract Deterioration
Changes in Adulthood
Cross-References
References
59 Neurogenic Bladder in Cerebral Palsy: Upper Motor Neuron
Introduction
Natural History
Normal Bladder Function
Lower Urinary Tract Dysfunction in Cerebral Palsy
Pathogenesis of Urinary Tract Symptoms in Cerebral Palsy
Diagnostic Work Up and Treatment
Work Up of Urological Symptoms in Children with Cerebral Palsy
Treatment of Lower Urinary Tract Symptoms in Children with Cerebral Palsy
Complications
Cross-References
References
60 Kidney Stones: Risks, Prevention, and Management in Cerebral Palsy
Introduction
Immobilization and Hypercalciuria
The Ketogenic Diet and Kidney Stone Risk
Antiepileptic Medications
Topiramate
Zonisamide
Treatment
Medical Management of Acute Renal Colic
Surgical Management of Kidney Stones
Imaging
Surgical Modalities for the Treatment of Kidney Stones
Extracorporeal Shock Wave Lithotripsy (ESWL)
Endoscopic Lithotripsy
Percutaneous Nephrolithotomy (PCNL)
Prevention of Recurrent Kidney Stones
Adequate Fluid Intake
Sodium
Calcium
Protein
Alkali Therapy/Potassium Citrate
Diuretics
Cross-References
References
61 Undescended Testis in Boys with Cerebral Palsy
Introduction
Natural History
Prevalence
Associated Risk Factors
Treatment and Complications
References
62 Gynecological Issues in Girls and Young Women with Cerebral Palsy
Introduction
Natural History
Puberty and Menstruation
Sexuality in Young Women
The Office Visit
History
Physical Examination
Treatment
Menstrual Suppression
Nonhormonal Treatment
Hormonal Treatment
Surgical Treatment
Contraception
Preventative Health and Screening
Complications
Pregnancy
Cross-References
References
Part XIII: Pulmonary
63 Bronchopulmonary Dysplasia and Cerebral Palsy
Introduction
Natural History
Definition
Incidence
Pathology of BPD
Radiology
Treatment
Prevention of BPD
Antenatal
Postnatal
Surfactant Therapy
Caffeine
Vitamin A
Oxygen Therapy
Corticosteroids
Treatment of Established BPD
Diuretics
Bronchodilators
Nutrition
Immunizations and RSV Prophylaxis
Outcome/Prognosis
Respiratory Outcomes
Neurodevelopmental Outcomes
Conclusion
Cross-References
References
64 Asthma in a Child with Cerebral Palsy
Introduction
Natural History
Pathophysiology for Asthma
Natural History of Risk Factors for Respiratory Illness
Diagnostic Observations and Dynamic Imaging Studies
Role of Historical Information to Treat Asthma
Treatment for Asthma
Complications of Treatment for Asthma
Measurable Parameters for Asthma Symptoms
Validated Surveys as a Diagnostic Tool for Asthma
Laboratory Studies
Limitations of Functional Respiratory Studies
Summary
Cross-References
References
65 Aspiration in the Child with Cerebral Palsy
Introduction
Definitions
Natural History
Aspiration from Above
Aspiration from Below
Screening and Diagnostic Testing
Complications
Aspiration Syndromes
Tracheobronchitis
Aspiration Pneumonitis
Aspiration Pneumonia
Treatment
Summary
Cross-References
References
66 Medical Management of Tracheostomy in the Child with Cerebral Palsy
Introduction: What Is a Tracheostomy?
Natural History of Respiratory Issues in Patients with Cerebral Palsy
Treatment: Indications for Tracheostomy Tube Placement in Patients with CP
Evaluation for Tracheostomy Placement
Complications: Potential Risks of Trach Placement
Care Following Trach Placement
Decannulation of the Tracheostomy Tube
Summary
Cross-References
References
67 Obstructive Sleep Apnea in Children with Cerebral Palsy
Introduction
Prevalence
Normal Breathing During Sleep
Sleep-Disordered Breathing and OSA in Children with Cerebral Palsy
Clinical History
Diagnosis
Treatment Options
Cross-References
References
Part XIV: Endocrine
68 Short Stature in Children with Cerebral Palsy
Introduction
Natural History
Overview of Normal Growth
Measurements of Growth
Growth Charts
Diagnosis and Treatment
Maturational Assessments of Growth
Bone Age
Body Composition: Overview
Body Composition: Skinfold Thickness
Body Composition: DXA Technique
Complications (Non-nutritive Factors Affecting Growth)
Growth Hormone-IGF-1 Axis: Normal
Growth Hormone Axis: Assessment in Cerebral Palsy
Growth Hormone Deficiency Treatment in Cerebral Palsy
Other Non-nutritive Factors in Growth Disorders
Functional Changes with Growth
Approach to Growth Problems in Children with Cerebral Palsy
Cross-References
References
69 Growth Attenuation for the Child with Cerebral Palsy
Introduction
Natural History
Treatment
Complications
Ethical Considerations
Autonomy and Family Preferences
Beneficence, Nonmaleficence, and the Best Interest Principle
Justice and Contextual Features
Perspective of Stakeholders
Conclusion
Cross-References
References
70 Premature and Delayed Sexual Maturation in Children with Cerebral Palsy
Introduction
Normal Pubertal Maturation
Phase of Fetus and Infant Puberty Development
Phase of Childhood Hormonal Suppression
Phase of Adolescent Puberty
Physical Assessment of Puberty
Adrenal Role in Puberty
Natural History
Precocious Puberty
Incomplete Precocious Puberty
Benign Variants
Pathological Variants
Complete Precocious Puberty
Delayed Puberty
Diagnosis and Treatment
Diagnosis of Central Precocious Puberty
Treatment of Central Precocious Puberty
Diagnosis of Delayed Puberty
Treatment of Delayed Puberty
Complications
Studies of Pubertal Milestones in Children with Cerebral Palsy
Studies of Pubertal Hormonal and Metabolic Changes in Children with Cerebral Palsy
Studies of Mechanisms of Pubertal Disruption in Cerebral Palsy
Conclusion
Cross-References
References
71 Endocrine Dysfunction in Children with Cerebral Palsy
Introduction
Natural History
Pituitary Gland
Thyroid Gland
Adrenal Gland
Treatment
Pituitary Gland
Thyroid Gland
Adrenal Gland
Complications
Cross-References
References
Part XV: Eyes
72 Testing Visual Function and Visual Evaluation Outcomes in the Child with Cerebral Palsy
Introduction
Natural History
Vision Disorders Commonly Associated with Cerebral Palsy
Evaluation and Treatment of Ocular and Vision Disorders Associated with Cerebral Palsy
Preparation for the Vision Evaluation
Preliminary Information from Caregiver
Information from Educators and Rehabilitation Therapists
Clinical History
Ocular Health Examination
Visual Skills Evaluation
What Are the Primary Visual Skills that Can Be Helpful for Professionals Working with Children with CP to Be Aware of?
Color Vision
Glare Sensitivity and Dark Adaptation
Vision Evaluation Outcomes
Complications of the Disease Process and Treatment
Treatment and Management of Identified Vision Disorders
Integration of Recommendations into Education and Rehabilitation Plans
Cross-References
References
Further Readings
73 Strabismus Management in the Child with Cerebral Palsy
Introduction
Natural History
Treatment
Nonsurgical Treatment
Surgical Treatment
Complications
Binocular Vision
Amblyopia and Visual Acuity
Strabismus Surgery Failure
Psychosocial Complications
Cross-References
References
74 Cortical Visual Impairment in the Child with Cerebral Palsy
Introduction
Natural History
Case History
Pathophysiology
Etiology
Characteristics
Visual Acuity
Visual Field
Higher Order Deficits
Treatment
Diagnosis
Interventions
Prognosis
Multidisciplinary Team
Complications
Cross-References
References
Part XVI: Dental
75 Dental Hygiene for Children with Cerebral Palsy
Introduction
Goals and Environment
Body Function and Structure
Impact on Oral Hygiene
Environmental Factors
Personal (Family) Factors
Early Intervention and Counseling by Pediatric Services
Technique
Early Establishment of Dental Home
Oral Hygiene
Non-cariogenic Diet
Anticipatory Guidance
Conditioning
Regular Dental Care Management
New Strategies
Evidence of Effectiveness
Conclusion
Cross-References
References
76 General Dentistry for Children with Cerebral Palsy
Introduction
Common Issues
Similarities and Differences to Other Mental and Physical Challenges
Risk Factors
Quality of Life
Dental Awareness and Oral Hygiene Education for Parents and Caregivers
Clinical Concerns
Lip Biting
Bruxism, Clenching, and Grinding
Drooling
Incompetent Lip Seal
Dental Trauma
Caries
Periodontal Disease (Gingivitis, Periodontitis)
GERD, Erosion, and Wear
Malocclusion
Temporomandibular Joint (TMJ) Dysfunction
Dental Treatment for Children with CP
Before Treatment
Oral Drugs
Caregiver in Room
Special Operatory Chairs
Safety Restraint
Nitrous Oxide
Opening the Mouth
Keeping the Mouth Open
Treatment: Oral Exam and Cleaning
Illuminating the Oral Field
Illuminating Inside the Oral Cavity
Intraoral Photos and Video
X-Ray Imaging
Magnification
Isolation
Treatment: Complex Dental Procedures
When Office Efforts Don´t Succeed
IV Sedation
General Anesthesia
OR (Operating Room) Follow-Up in the Office
Oral Hygiene at Home or Institution
Training for Dentist and Dental Staff
Access to Care
Conclusion
Case Studies
Cross-References
References
77 Management of Skeletal Facial Deformation and Malocclusion in Cerebral Palsy
Introduction
Etiology
Functional Deficits and Impact
Assessment
Treatment
Summary
Cross-References
Glossary of Terms
References
Part XVII: Anesthesia Management
78 Medical Evaluation for Preoperative Surgical Planning in the Child with Cerebral Palsy
Introduction
Medical Evaluation/Optimization
General
Musculoskeletal
Neuro/Developmental
Respiratory
Gastroenterology/Nutrition
Cardiovascular
Renal/Urologic/Genitourinary
Endocrinology
Hematology
Psychiatric
Medications
Laboratory Evaluation
Miscellaneous
Summary
Case Histories
Cross-References
References
79 Anesthesia in the Child with Cerebral Palsy
Introduction
Surgical Epidemiology
Perioperative Concerns
Neurologic
Respiratory
Cardiovascular
Gastrointestinal
Fluids and Electrolytes
Musculoskeletal
Thermoregulatory
Pharmacologic
Positioning
Management
Preoperative
Intraoperative
Postoperative
Conclusion
Cross-References
References
80 Postoperative Pain and Spasticity Management in the Child with Cerebral Palsy
Introduction
Epidemiology of Pain
Pathophysiology of Pain
Special Challenges Relating to Pain Management
Chronic Systemic Sources of Pain
Pain Assessment
Multimodal Approach to Pain
Approaches to Pain Management
Opioids
Nonopioid Drugs
Antispasmodics
Regional Approaches
Postoperative Spasticity Management
Monitoring
Pain Management for Specific Procedures
Summary
Cross-References
References
81 Regional Anesthesia in Patients with Cerebral Palsy
Introduction
Caudal Anatomy and Analgesia
Epidural Anatomy/Analgesia
Epidural Placement in Patients with a Baclofen Catheter
Neuraxial Blockade in Patients after Spinal Instrumentation
Peripheral Nerve Blocks
Surgical Site and Type of Regional Blocks
Hip and Thigh Surgeries
Lumbar Plexus Block
Fascia Iliaca Compartment Block
Thigh Surgery and Femur Fracture
Femoral Nerve Block
Lateral Femoral Cutaneous Nerve Block
Knee Surgery
Sciatic Nerve Block
Adductor Canal Block
Foot and Ankle Surgeries
Regional Blocks for the Upper Extremity
Shoulder Surgery
Interscalene Block
Upper Arm, Elbow, Forearm, Wrist, and Hand Surgery
Supraclavicular Block
Infraclavicular Block
Axillary Block
Cross-References
References
82 Anesthetic Management of Spine Fusion
Introduction
Anesthetic Management
Preoperative Care and Workup
Intraoperative Care
Total Intravenous Anesthesia (TIVA)
Procoagulants and Management of Blood Loss
Neuromonitoring and Its Influence on the Anesthetic Management
Anesthetic Choices to Facilitate Neuromonitoring
Postoperative Management
Conclusion
References
83 Postoperative Care of the Cerebral Palsy Patient
Introduction
Respiratory Considerations
Patients at Respiratory Baseline
The Intubated Patient
Upper Airway Obstruction and Respiratory Effort
Impaired Mucociliary Function and Secretion Clearance
Postoperative Respiratory Complications
Liberation from the ICU or Stepdown Unit
Cardiovascular Considerations
Background
Modalities of Monitoring Hemodynamics
Fluid Resuscitation
Vasopressor Support
Norepinephrine
Dopamine
Hydrocortisone
Gastrointestinal Considerations
Nutrition
Nausea and Vomiting
Constipation
Pancreatitis
Genitourinary and Renal
Fluid Management
Electrolyte Abnormalities
Urinary Retention
Hematology
Bleeding/Anemia
Venous Thromboembolism
VTE Prevention
VTE Treatment
Fever and Infectious Disease
Introduction
Fever Timing
Noninfectious Etiologies of Postoperative Fever
Diagnostic Testing
Antibiotics
Neurology
Spasticity
Seizures
Sleep
Mood
Early Mobilization/Postoperative Rehabilitation
Cross-References
References
Part XVIII: Complementary Medical Treatments
84 Complementary and Alternative Medicine in Cerebral Palsy
Introduction
Selected CAM Therapies Utilized in Cerebral Palsy
Acupuncture
Description
Evidence
Myofascial Structural Integration/Rolfing
Description
Evidence
Adeli Suit (TheraSuit) Treatment
Description
Evidence
Craniosacral Therapy
Description
Evidence
Hyperbaric Oxygen Therapy
Description
Evidence
Electrical Stimulation (e-Stim)
Description
Evidence
Cannabinoids
Description
Evidence
Stem-Cell Therapy
Description
Evidence
Summary
References
85 Hyperbaric Oxygen Therapy for Cerebral Palsy: Definition and Principles
Introduction
History of the Development of HBOT
Adverse Effects of HBOT
Current Policy and Regulation of HBOT
Cost
Rationale for Use of HBOT in Cerebral Palsy
Goals and Environment
Technique
Evidence of Effectiveness
Summary and Conclusions
References
86 Acupuncture and Traditional Chinese Medicine Used to Treat Cerebral Palsy
Introduction
Treatment
Acupuncture and Moxibustion
Scalp Acupuncture Therapy
Electrical Acupuncture Therapy
Acupuncture Point Injection Therapy
Tui Na Therapy (Massage Therapy)
Supine Position
Prone Position
Alternative Treatment
Triceps Surae Spasticity
Knee Hyperextension
Adductor Muscle Spasticity
Traditional Chinese Medication for Topical Use
Comprehensive Chinese Medicine Therapy and Intervention
Complications
Pain
Infection
Acupuncture Syncope
Allergy
Curved or Fixed Needles
Case 1
Conclusion
Cross-References
References
87 Osteopathic Manipulative Treatment and Acupuncture in Cerebral Palsy
Introduction
Natural History
Treatment
Outlook
Cross-References
References
Part XIX: Gait in Cerebral Palsy
88 Musculoskeletal Physiology Impacting Cerebral Palsy Gait
Introduction
Natural History and Pathophysiology
Central Nervous System
Biomechanics
Muscle Mechanics
Force Production
Muscle Fiber Types
Muscle Anatomy
Muscle Length-Tension Relationship (Blix Curve)
Muscle Control
Muscle Force-Generating Capacity
Muscle Excursion
Increasing Muscle Excursion
Connective Tissue Mechanics
Growth of the Muscle-Tendon Unit
Bone Mechanics
Joint Mechanics
Joint Motor Mechanics
Single-Joint Muscles
Multiple-Joint Muscles
Treatment
Cross-References
References
89 Normal Human Gait
Introduction
Natural History and Pathophysiology
Gait Cycle
Stance Phase
Swing Phase
Body Segments Important in the Gait Cycle
Ankle
Foot Segment
Knee
Hip
Pelvis
HAT Segment
Treatment
Simplified Joint Functions
Simplified Cycle Functions
Global Body Mechanics of Human Gait
Cognitive Subsystem
Balance Subsystem (Chap. 47, ``Ataxia and Disorders of Balance in Children with Cerebral Palsy´´)
Energy Production
Motor Control
Structural Stability
Cases
Cross-References
References
90 Cerebral Palsy Gait Pathology
Introduction
Natural History and Pathology
Balance
The Impact of Growth and Development
Interventions
Motor Control
The Impact of Growth and Development
Interventions
Motor Power
Impact of Growth and Development
Interventions
Musculoskeletal Subsystem
Cross-References
References
91 History and Physical Examination Components of Gait Analysis
Introduction
History Related to Current Disability
Physical Examination
Global Function Measures
Interrater and Intrarater Reliability
Motor Control
Muscle Strength
Muscle Tone
Passive Range-of-Motion Assessment
Reliability
Cross-References
References
92 Diagnostic Gait Analysis Technique for Cerebral Palsy
Introduction
Components of Gait Analysis Assessment
History
Physical Examination
Global Function Measures
Motor Control (Chap. 91, ``History and Physical Examination Components of Gait Analysis´´)
Muscle Strength (Chap. 91, ``History and Physical Examination Components of Gait Analysis´´)
Muscle Tone
Passive Range-of-Motion Assessment
Video Recording
Kinematics (Chap. 93, ``Kinematics and Kinetics: Technique and Mechanical Models´´)
Measurement System
Data Reduction Algorithms (Chap. 93, ``Kinematics and Kinetics: Technique and Mechanical Models´´)
Measurement Accuracy
Kinetics (Chap. 93, ``Kinematics and Kinetics: Technique and Mechanical Models´´)
Measurement Accuracy
Electromyography
Pedobarograph (Chap. 95, ``Pedobarograph Foot Evaluations in Children with Cerebral Palsy´´)
Oxygen Consumption (Chap. 97, ``Aerobic Conditioning and Walking Activity Assessment in Cerebral Palsy´´)
Activity Monitoring (Chap. 97, ``Aerobic Conditioning and Walking Activity Assessment in Cerebral Palsy´´)
Summary of Gait Analysis
Cross-References
References
93 Kinematics and Kinetics: Technique and Mechanical Models
Introduction
Goals and Environment
Technique
Gait Evaluation
Marker Sets and Models
Calculating Kinematics
Clinical Implications of Kinematics
Calculating Kinetics
Clinical Implications of Kinetics
Evidence of Effectiveness
References
94 Foot Kinematics: Models Used to Study Feet in Children with Cerebral Palsy
Introduction
Measurement Other than Motion Analysis
Motion Analysis: Single-Segment Foot Model
Motion Analysis: Multisegment Foot Model
Normal Multisegment Foot Model Kinematics
Clinical Examples
Summary
Cross-References
References
95 Pedobarograph Foot Evaluations in Children with Cerebral Palsy
Introduction
Natural History
Therapeutic Use of the Pedobarograph
Evaluations Complimentary to Pedobarograph
Complications
Cross-References
References
96 Measuring Femoral and Tibial Torsion in Children with Cerebral Palsy
Introduction
Natural History and Methods
Natural History
Methods
Natural Pathophysiology: Measuring Tibial Torsion
Physical Examination
Trans-Malleolar Axis (TMA)
Thigh-Foot Angle (TFA)
Imaging Technology
Radiography
Plane Radiograph (X-ray)
Fluoroscopy
Computed Tomography, Magnetic Resonance Imaging, and EOS System
CT and MRI
EOS System
Ultrasonography
Three-Dimensional Motion Analysis
Natural Pathophysiology: Measuring Femoral Torsion
Physical Examination
Imaging Technology
Radiography
Computed Tomography, Magnetic Resonance Imaging, and EOS System
Ultrasonography
Three-Dimensional Motion Analysis
Conclusion
References
97 Aerobic Conditioning and Walking Activity Assessment in Cerebral Palsy
Introduction
Pathophysiology and Measurement Techniques
Oxygen Consumption and Cost
Cardiovascular Conditioning
Activity Monitors
Treatment Implications
Data Interpretation
Treatment Recommendations
Complications
Cross-References
References
98 Gait Analysis Interpretation in Cerebral Palsy Gait: Developing a Treatment Plan
Introduction
History and Pathophysiology
Outpatient Clinical Assessment
When Is Gait Analysis Needed to Develop Treatment Plan?
How Should Gait Analysis Be Applied?
Treatment Plan Development
Foot Contact-Weight Acceptance
Midstance
Late Stance Phase
Early Swing Phase
Late Swing Phase
Complications
Cross-References
References
99 Gait Treatment Outcome Assessments in Cerebral Palsy
Introduction
Evaluating Individual Domains
Body Function and Structure
Health Condition-Related Quality of Life
Personal Factors
Participation
Environment Factors
Conclusions
Cross-References
References
100 Hemiplegic or Unilateral Cerebral Palsy Gait
Introduction
Natural History and Pathophysiology
Etiology
Treatment
Hemiplegia Type 0
Hemiplegia Type 1
Hemiplegia Type 2
Outcome of Plantar Flexor Tendon Lengthening
Rotational Deformities
Hemiplegia Type 3
Stiff Knee Gait
Rotational Deformities
Hemiplegia Type 4
Rotational Deformities
Limb Length Discrepancy
Complications
Cases
Cross-References
References
101 Diplegic Gait Pattern in Children with Cerebral Palsy
Introduction
Natural History and Treatment
Diplegia in Young Children (The Prancing Toe Walker) (True Equinus)
Mild Involvement
Moderate Degree of Involvement
Severe Involvement
Surgical Treatment of the Prancing Toe Walker (True Equinus)
Middle Childhood, Early Crouch, and Recurvatum of the Knee
Adolescent Severe Crouch
Knee Recurvatum (Back-Kneeing)
Complications
Cases
Cross-References
References
102 Hip and Pelvic Kinematic Pathology in Cerebral Palsy Gait
Introduction
Natural History and Pathophysiology
Hip Joint
Sagittal Plane
Coronal Plane Hip Pathology
Transverse Plane Deformity
Pelvis
Pelvic Rotation
Pelvic Tilt
Pelvic Obliquity
HAT Segment
Treatment and Outcome Summary
Cases
Cross-References
References
103 Crouch Gait in Cerebral Palsy
Introduction
Natural History and Pathophysiology
Pathophysiology
Treatment
Performing the Crouched Gait Surgery
Spasticity Reduction in Adolescents and Young Adults
Complications
Cases
Cross-References
References
104 Knee Deformities Impact on Cerebral Palsy Gait
Introduction
Natural History and Pathophysiology
Knee Position at Weight Acceptance
Midstance Knee
Terminal Stance Knee Position
Early Swing Phase
Terminal Swing Phase
Treatment Summary
Cases
Cross-References
References
105 Foot Deformities Impact on Cerebral Palsy Gait
Introduction
Natural History and Pathophysiology
Secondary Adaptations
Treatment
The Foot as a Functional Moment Arm in Contact with the Ground Reaction Force
Secondary Adaptations
Treatment
The Ankle as a Power Output Joint
Ankle Dorsiflexion in Swing Phase
Treatment Summary and Outcome Expectations
Cases
Cross-References
References
106 Complications from Gait Treatment in Children with Cerebral Palsy
Introduction
Natural History and Pathophysiology
Treatment
Complications of Gait Analysis
Complications of Surgery Planning
Interrelated Effect of Multiple Procedures
Complications of Surgical Execution
Complications of Rehabilitation
Monitoring the Outcome of Gait Development and Treatment
Energy Use Measurement
Cases
Cross-References
References
107 The Evolution of Knee Flexion During Gait in Patients with Cerebral Palsy
Introduction
Current Knowledge
Assumptions
The Concept of the Development of Knee Flexion Gait
Starting with the Foot
Starting with the Knee
Starting with the Hip
Conclusions
Limitations
Cross-References
References
Part XX: Upper Extremity
108 The Upper Extremity in Cerebral Palsy: An Overview
Introduction
Natural History
Normal Development of Function of Children´s Upper Extremities
Classifying Upper Extremity Function
Treatment
Specific Treatments
Complications
Conclusion
Cross-References
References
109 Upper Extremity Assessment and Outcome Evaluation in Cerebral Palsy
Introduction
Mode of Administration
Classifications Versus Tests
Norm-Referenced Versus Criterion-Referenced Tests
Assessing Hand Use in Infants
The ICF Framework
Capacity Versus Performance
Psychometric Properties
Clinical Utility
Final Points
Upper Extremity Evaluation: Examples of Commonly Used Tools
Body Function Assessments
Classifications
Body Function Assessments
Activity and Participation Assessments
Classifications
Questionnaires
Observation Based
Speed and Dexterity
Quality of Movements
Bimanual Performance
Functional Activity Performance
Development of Hand Skills
Individualized Measures
Cross-References
References
110 Physical Examination and Kinematic Assessment of the Upper Extremity in Cerebral Palsy
Introduction
Natural History: Evaluation of Patients
Guidelines for Setting Goals
Early Childhood: Ages 0-6 Years
Middle Childhood: Ages 6-12 Years
Adolescence: Ages 12 Years and Older
Treatment: Assessment to Develop a Plan
Pretreatment Evaluation
Classification of Upper Extremity Involvement
Assessment of Specific Impairment
Physical Examination
Sensation
Selective Motor Control
Muscle Strength
Kinematic Assessment
Electromyography (EMG)
Developing a Treatment Plan
Cross-References
References
111 Spasticity, Dystonia, and Athetosis Management in the Upper Extremity in Cerebral Palsy
Introduction
Natural History of Spasticity and Movement Disorder
Measuring Spasticity and Movement Disorder
Treatment
Spasticity
Movement Disorder: Dystonia, Athetosis, and Chorea
Conclusion
Cross-References
References
112 Single-Event Multilevel Surgery for the Upper Extremity in Cerebral Palsy
Introduction
Natural History: Pathophysiology
Treatment
Patient Age
Neurologic Type
Voluntary Control
Sensibility
Intelligence
Patient Motivation
Developing the SEMLS Plan
Outcome and Complications of SEMLS
Cross-References
References
113 Shoulder and Elbow Problems in Cerebral Palsy
Introduction
Shoulder
Shoulder Contractures
Natural History
Treatment
Outcome of Treatment
Other Treatment
Complications of Treatment
Shoulder Instability
Natural History
Treatment
Elbow
Elbow Flexion Contracture
Natural History
Treatment
Complications of Treatment
Radial Head Dislocation
Natural History
Treatment
Complications of Treatment
Cases
Cross-References
References
114 Forearm, Thumb, and Finger Deformities in Cerebral Palsy
Introduction
Natural History (Pathophysiology and Symptoms)
Regional Treatment
Forearm Pronation
Natural History
Treatment
Outcome of Treatment
Complications of Treatment
Wrist Flexion Deformity
Natural History
Diagnostic Evaluations
Mild Wrist Flexion Deformity
Moderate Wrist Flexion Deformity
Severe Wrist Flexion Deformity
Very Severe Wrist Flexion Deformity
Wrist Extension Contracture
Treatment
Outcome of Treatment
Other Treatment
Complications of Treatment
Thumb
Natural History
Diagnostic Evaluations
Treatment
House Classification
Type 1
Type 2
Type 3
Type 4
Outcome of Treatment
Other Treatment
Complications of Treatment
Finger Flexion
Treatment
Outcome of Treatment
Other Treatment
Complications of Treatment
Finger Swan Neck
Treatment
Outcome of Treatment
Conclusion
Cases
Cross-References
References
115 Upper Extremity Operative Procedures in Cerebral Palsy
Introduction
Individual Procedures
Shoulder Adductor, Extension, and External Rotator Lengthening
Indication
Procedure
Postoperative Care
Humeral Derotation Osteotomy
Indication
Procedure
Postoperative Care
Elbow Flexion Contracture Release
Indication
Procedure
Postoperative Care
Pronator Release or Transfer
Indication
Procedure
Postoperative Care
Flexor Carpi Ulnaris (FCU) Transfer for Wrist Flexion Deformity
Indication
Procedure
Postoperative Care
Carpectomy and Wrist Fusion
Indication
Procedure
Postoperative Care
Thumb Adductor Lengthening
Indication
Procedure
Postoperative Care
Webspace Lengthening and Z-Plasty
Indication
Procedure
Postoperative Care
Metacarpal Phalangeal Joint Fusion of the Thumb
Indication
Procedure
Postoperative Care
Extensor Pollicis Longus Rerouting
Indication
Procedure
Postoperative Care
Palmaris Longus or Brachioradialis Transfer to the Abductor Pollicis
Indication
Procedure
Postoperative Care
Volar Plate Advancement and Sublimis Slip Reinforcement for Swan Neck Deformity
Indication
Procedure
Postoperative Care
Central Extensor Slip Release for Swan Neck Deformity
Indication
Procedure
Cross-References
References
Part XXI: Spine
116 Spinal Deformity in Children with Cerebral Palsy: An Overview
Introduction
Natural History and Treatment
Scoliosis
Kyphosis and Lordosis
Early Onset Scoliosis
Complications
Problems Related to Spinal Deformity
Conclusion
Cross-References
References
117 Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management
Introduction
Natural History, Incidence, and Etiology
Natural History
Treatment: Nonoperative
Conservative Treatment
Orthotics
Seating
Therapy
Electrical Stimulation
Botulinum Toxin
Conclusion
Cases
Cross-References
References
118 Surgical Treatment of Scoliosis Due to Cerebral Palsy
Introduction
Etiology
Incidence and Natural History
Indications for Scoliosis Surgery
Preoperative Orthopedic Evaluation
Preoperative Management and Preparation
Preparing for Intraoperative Bleeding
Soft Bone
Maintaining Spinal Cord Integrity
Prophylaxis to Prevent Deep Wound Infection
Operative Treatment
Evolution of Instrumentation for Neuromuscular Scoliosis Correction
Unit Rod
Current Methods of Pelvic Fixation, Pre-contoured Rods with Pelvic Screw, and Segmental Spine Fixation
Sublaminar Wires vs. Pedicle Screws
Rigid Scoliosis in the Cerebral Palsy Child
Surgical Outcomes
Functional Outcomes and Quality of Life
Cross-References
References
119 Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy
Introduction
Etiology/Pathogenesis/Natural History
Patient Assessment and Preoperative Considerations
Nonoperative Treatment
Surgical Treatment
Medical/Anesthesia Considerations (Anesthesia for Cerebral Palsy Spine Fusion Surgery)
Operative Principles
Preoperative Planning
Current Preferred Surgical Treatment Methods (Spinal Procedure Atlas for Cerebral Palsy Deformities)
Intraoperative Positioning
Instrumentation
Fusion to the Pelvis
Kyphosis Correction
Lumbar and Thoracolumbar Kyphosis
Thoracic Kyphosis
Hyperlordosis
Rigid Kyphotic and Hyperlordotic Deformities
Evidence-Based Outcomes
Summary
Cross-References
References
120 Early-Onset Scoliosis in Cerebral Palsy
Introduction
Natural History and Etiology
Treatment Options for Early-Onset Scoliosis
Early Short Fusion as an Option
Growing Rod Constructs
Spinal Deformity in Very Small Children Who Are Older
Recommendation for Early-Onset Spine Fusion in Children with CP
Long-Term Outcome of Early Spine Fusion
Conclusion
Cases
Cross-References
References
121 Complications of Spine Surgery in Cerebral Palsy
Introduction
Natural History
Overall Risk Factors
Death: Mortality
Transition Time
Immediate Postoperative Period
Preoperative Problems
Poor Nutrition
Intraoperative Complications
Respiratory Problems
Bleeding Problems (Chapter ``Anesthetic Management of Spine Fusion´´)
Epidural Bleeding
Bone Bleeding
Dural Leak
Perforation of the Pelvis with Unit Rod
Medial Pelvic Perforation
Lateral Pelvic Perforation
Acetabular Perforation
Wires Pulling Through Laminae
Rod Either Too Long or Too Short
Spinal Cord Monitoring: Loss of Motor Evoked Potentials
Postoperative Complications
Hypotension
Thrombophlebitis and Pulmonary Embolism
Coagulopathy
Respiratory Failure
Pneumothorax or Hemothorax and Pleural Effusion
Reflux and Aspiration
Pancreatitis
Colicystitis
Duodenal Obstruction
Constipation
Poor Feeding
Seating Adjustments
Hair Loss
Doing Posterior Spinal Fusion When Families Refuse Blood Transfusions
Dealing with Families Who Refuse Spinal Fusion
Handling Families and Children When a No Resuscitation Status Is Requested
Cases
Cross-References
References
122 Neuromonitoring and Anesthesia for Spinal Fusion in Cerebral Palsy
Introduction
Personnel and Practical Aspects of Neuromonitoring
Technical Aspects and Interpretation of IONM
Somatosensory Evoked Potentials (SSEP)
Motor Evoked Potentials (MEP)
Electromyography (EMG)
Stagnara Wake Up Test
Physiological Application and Risk of Neuromonioring
Blood Supply to the Spinal Cord
Risk of Neuromonitoring
Anesthesia
Inhaled Anesthetics
Intravenous Anesthetics
Nonanesthetic Intraoperative Influences
Clinical Application
References
123 Cervical Spine in Children with Cerebral Palsy
Introduction
Treatment: Cervical Spine Problems
Extensor Posturing
Occipital Subluxation, Posturing
Atlantoaxial Instability and Subluxation with or without Os Odontoideum
Congenital Atlantoaxial Displacement with Os Odontiodeum
Cervical Spine Spondylosis
Inability to Hold up the Head
Severe Upper Thoracic Kyphosis with Lower Cervical Lordosis
Complications of Cervical Spinal Deformity
Cervicothoracic Junction Kyphosis
Cross-References
References
124 Pelvic Alignment and Spondylolisthesis in Children with Cerebral Palsy
Introduction
Natural History and Pathophysiology
Pelvic Malalignment
Pelvic Obliquity
Etiology
Natural History
Treatment
Seating Adjustment
Surgical Correction of Pelvic Obliquity
Anterior Pelvic Tilt
Etiology
Natural History
Treatment
Pelvis Rotational Malalignment
Spondylolisthesis
Natural History
Treatment
Conclusion
Cases
Cross-References
References
125 Infections and Late Complications of Spine Surgery in Cerebral Palsy
Introduction
Pathology of Long-Term Complication
Postoperative Infections
Persistent Fevers
Superficial Wound Infections
Acute Deep Wound Infection
Treatment
Mechanical Problems
Pain in the Spine
Proximal End Prominence or Wire Prominence
Nonunion-Pelvic Leg Halos-Rod Fracture
Crankshaft
Neck Stiffness
Decreased Floor or Bed Mobility
Special Problems with Spinal Surgery
Doing Revision Spinal Surgery in Children with Cerebral Palsy
Fall-Off from a Short Fusion
Torsional Collapse
Pseudarthrosis
Hardware Failure
Correcting Deformity Posterior Dorsal Rhizotomy
Correcting Spinal Deformity in Ambulatory Children
Mortality
Conclusion
Cases
Cross-References
References
126 Spinal Procedure Atlas for Cerebral Palsy Deformities
Introduction
Posterior Spinal Fusion with Single Unit Rod or Modular Unit Rod Using Cantilever Correction
Indication
Procedure
Postoperative Care
Special Consideration for Correction of Kyphosis and Lordosis
Anterior Spinal Release
Indication
Procedure
Postoperative Care
Cross-References
References
Part XXII: Hip
127 Hip Problems in Children with Cerebral Palsy: An Overview
Introduction
Natural History, Etiology, and Pathophysiology
Treatment
Typical Posterior Lateral Dislocations in Children with Spasticity
Other Hip Deformities
Femoral Torsional
Complications in CP Hip Management
Surgical Procedures
Conclusion
Cross-References
References
128 Etiology of Hip Displacement in Children with Cerebral Palsy
Introduction
Natural History and Pathophysiology
Posterior-Superior Hip Subluxation
Etiology
Secondary Pathology
Tertiary Changes
Treatment and Outcomes
Conclusion
References
129 Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy
Introduction
Natural History
Childhood
Adolescence
Adult
Diagnostic Evaluations
Hip Radiograph
Computed Tomography Scans
Ultrasound
Bone Scan
Arthrography
Treatment
Surveillance Algorithm
Surveillance Results
Conclusion
References
130 Prophylactic Treatment of Hip Subluxation in Children with Cerebral Palsy
Introduction
Treatment
Specific Prophylactic Treatment
Operative Procedures
The Outcome of Preventative Treatment
Other Treatment
Iliopsoas Transfer
Adductor Transfer
Botulinum Toxin Injection
Intrathecal Baclofen
Dorsal Rhizotomy
Abduction Orthosis
Complications of Preventative Treatment
Pain
Infections
Hyperabduction
Cases
References
131 Hip Reconstruction in Children with Cerebral Palsy
Introduction
Natural History
Treatment
Indications for Reconstructive Treatment
Recommended Surgical Reconstruction Approach
Outcome of Reconstruction Treatment
Other Reconstructive Treatment Options: Varus Osteotomy Without Acetabular Osteotomy
Other Pelvic Osteotomies
Indication for Doing Bilateral Femoral Varus Osteotomy
Complications of Reconstruction
Loss of Fixation
Repeat Early Dislocation
Heterotopic Ossification
Sleep Problems
Prolonged Hip Pain
Avascular Necrosis
Intraarticular Extension of Pelvic Osteotomy
Other
References
132 Palliative or Salvage Hip Management in Children with Cerebral Palsy
Introduction
Natural History
Treatment
Recommended Treatment
Total Hip Replacement
Interposition Arthroplasty
Resection Arthroplasty (Castle Procedure)
Proximal Femoral Valgus Osteotomy (McHale Procedure)
Subtrochanteric Valgus Osteotomy Without Femoral Head Resection (Schanz Osteotomy)
Femoral Head Resection (Girdlestone Procedure)
Hip Fusion
Complications of Palliative Treatment
Chronic Pain Syndrome
Persistent Pain
Conclusion
Cases
References
133 Anterior Dislocation of the Hip in Cerebral Palsy
Introduction
Natural History, Pathophysiology, and Etiology
Treatment
Type I Anterior Hip Dislocation
Treatment
Type II Anterior Hip Dislocation
Treatment
Other Treatment Recommendations
Complications
Type III Anterior Hip Dislocation: Present in Hypotonic and Hypermobile Hips
Treatment
Complications
Inferior Hip Dislocation
Conclusion
References
134 Hypotonic and Special Hip Problems in Cerebral Palsy
Introduction
Natural History and Etiology
Hypotonic Hip Subluxation
The Natural History of Hypotonic Hip Disease
Treatment: Hypotonic Hip
Complications
Hip Dislocation in Children with Down Syndrome and Cerebral Palsy
Developmental Hip Dysplasia (DDH) in Children with Cerebral Palsy
Established Developmental Dislocation
Slipped Capital Femoral Epiphysis
Perthes Disease in Children with Spasticity
Cases
References
135 Femoral Anteversion in Children with Cerebral Palsy
Introduction
Natural History and Pathophysiology
Internal Rotation Posture
Anteversion or Internal Femoral Torsion
Measuring Anteversion and Coxa Valga
Physical Examination
Radiographic Measurement
CT Scan
Ultrasound
Fluoroscopy
Magnetic Resonance Imaging Scan
Femoral Torsion Measurement Summary
Measuring Coxa Valga
Etiology of Femoral Anteversion and Coxa Valga
Muscle Contractures
Etiology of Coxa Valga
Natural History
Treatment
Femoral Anteversion
Methods for Correcting Anteversion
Proximal Femoral Derotation
Midshaft and Distal Femoral Osteotomy
Orthotic Treatment
Muscle Lengthening
Conclusion
Cases
Cross-References
References
136 Windblown Hip Deformity and Hip Contractures in Cerebral Palsy
Introduction
Natural History and Pathophysiology
Treatment
Hyperabducted Hip Deformity
Postoperative Management
Hip Flexion Contracture
Assessment and Measurement of Hip Flexion Contracture
Etiology
Natural History
Treatment
Osteotomy
Windblown Hips
Etiology
Asymmetric Neurologic Involvement
Symmetric Neurologic Involvement
Natural History
Treatment (Table 1)
Indications for Specific Treatment
Middle Childhood
Adolescence
Adult
Other Treatment Options for Windblown Hips
Orthotic Management
Tone Reduction
Hip Pain After Correction of Windblown Hip
Windblown Hip Deformity and Pelvic Obliquity (Hip Problems in Children with Cerebral Palsy: An Overview)
Anteversion, Coxa Valga, and Internal Rotation Contracture
Anteversion and Coxa Valga Relationship
Coxa Valga
Cases
References
137 Complications of Hip Treatment in Children with Cerebral Palsy
Introduction
Natural History and Symptoms
Delayed Treatment
Recurrent Contracture and Dislocation
Hip Wound Infections
Adductor Wound Infections
Femoral Osteotomy Infections
Femoral Osteotomy Nonunions
Fractures of the Femur
Fixation Failure
Trochanteric Fracture
Distal End of Plate Fractures
Leg Length Discrepancy
Caused by Adductor Contracture
Secondary to Varus Osteotomy
Dislocated Hip
Pelvic Obliquity
Heterotopic Ossification
After Adductor Lengthening
Prophylactic Treatment of Heterotopic Ossification
Treatment
Postoperative Hip Pain
Plate Bursitis
Medial Plate Protrusion
Degenerative Arthritis
Sudden Pain in Therapy
Avascular Necrosis
Thrombophlebitis and Pulmonary Embolism
Hip Joint Stiffness
Cases
Cross-References
References
138 Surgical Atlas of Cerebral Palsy Hip Procedures
Introduction
Procedures
Adductor and Iliopsoas Lengthening with Proximal Hamstring Lengthening
Indication
Procedure
Postoperative Care
Iliopsoas Lengthening: Over the Pelvic Brim Approach
Indication
Procedure
Postoperative Care
Proximal Femoral Osteotomy
Indication
Procedure
Postoperative Care
Peri-Ilial Pelvic Osteotomy (Dega Osteotomy)
Indication
Procedure
Postoperative Care
Pemberton-Type Pelvic Osteotomy for Anterior Dislocation
Indication
Procedure
Postoperative Care
Abductor Lengthening
Indication
Procedure
Postoperative Care
Resection Arthroplasty (Castle Procedure)
Indication
Procedure
Postoperative Care
Interposition Arthroplasty
Indication
Procedure
Postoperative Care
Femoral Derotation with an Intramedullary Nail
Indication
Procedure
Postoperative Care
Revision Adductor Lengthening
Indication
Procedure
Postoperative Care
Conclusion
References
Part XXIII: Knee
139 Overview of Knee Problems in Cerebral Palsy
Introduction
Natural History and Pathophysiology
Treatment
Knee Flexion Deformity
Patellar Femoral Instability
Knee Extension Pathology (Chap. 148, ``Ankle Equinus in Cerebral Palsy´´)
Knee Varus-Valgus and Tibial Torsion (Chap. 149, ``Equinovarus Foot Deformity in Cerebral Palsy´´)
Complications
Cross-References
References
140 Anterior Knee Pain and Patellar Subluxation in Cerebral Palsy
Introduction
Natural History
Treatment (Chap. 103, ``Crouch Gait in Cerebral Palsy´´)
Anterior Knee Pain and Extensor Mechanism Insufficiency
Extensor Mechanism Stress
Outcome of Treatment: Anterior Knee Pain
Patellar Subluxation and Dislocation
Outcome of Patellar Dislocation Treatment
Complications
Cases
Cross-References
References
141 Knee Flexion Deformity in Cerebral Palsy
Introduction
Natural History and Etiology
Natural History
Etiology
Secondary Pathology
Tertiary Changes
Treatment
Hamstring Contractures
Indications
Specific Treatments (Atlas of Knee Operative Procedures In Cerebral Palsy)
Outcome of Treatment
Other Treatment
Moderate Knee Flexion Contracture
Indications and Treatments
Outcome of Moderate Knee Flexion Contracture
Severe Knee Flexion Contracture
Indications and Treatments
Outcome of Treatment
Other Treatment
Complications
Cases
Cross-References
References
142 Stiff Knee and Knee Extension Deformities in Cerebral Palsy
Introduction
Natural History and Pathology
Pathology
Inadequate Knee Flexion
Stiff Knee Gait
Knee Extension Contractures
Secondary Pathology
Tertiary Changes
Treatment and Outcome
Diagnostic Studies
Treatment of Dynamic Knee Stiffness
Other Treatment
Outcome of Treatment
Treatment of Fixed Knee Extension Contractures
Outcome of Treatment
Other Treatment
Complications
Cases
Cross-Reference
References
143 Tibial Torsion and Knee Instability in Cerebral Palsy
Introduction
Natural History and Etiology
Knee Mechanical Instability
Intraarticular Pathology
Varus and Valgus Deformity
Tibial Torsion
Natural History
Etiology
Diagnostic Evaluations
Treatment
Ligament Instability
Varus and Valgus Deformity
Tibial Torsion
Outcome of Tibial Osteotomy
Other Treatments
Complications
Cases
Cross-References
References
144 Atlas of Knee Operative Procedures in Cerebral Palsy
Introduction
Treatment
Hamstring Lengthening
Indication
Procedure
Postoperative Care
Rectus Transfer
Indication
Procedure
Postoperative Care
Posterior Knee Capsulotomy
Indication
Procedure
Postoperative Care
Repair of Dislocation of the Patella
Indication
Procedure
Postoperative Care
Tibial Osteotomy with Cast
Indication
Procedure
Postoperative Care
Tibial Osteotomy with Intramuscular Nail
Indication
Procedure
Postoperative Care
Patellar Advancement
Distal Femoral Osteotomy
Indication
Procedure
Postoperative Care
Cross-References
References
Part XXIV: Ankle, Foot, and Toes
145 Foot Deformities in Children with Cerebral Palsy: An Overview
Introduction
Natural History and Pathophysiology (Chap. 146, ``Natural History of Foot Deformities in Children with Cerebral Palsy´´)
Equinus
Equinovarus
Planovalgus
Hallux Valgus with Bunion
Treatment
Equinus Treatment (Chap. 148, ``Ankle Equinus in Cerebral Palsy´´)
Equinovarus (Chap. 149, ``Equinovarus Foot Deformity in Cerebral Palsy´´)
Planovalgus (Chap. 150, ``Planovalgus Foot Deformity in Cerebral Palsy´´)
Hallux Valgus with Bunion (Chap. 151, ``Forefoot and Toe Deformities in Cerebral Palsy´´)
Conclusion
Cross-References
References
146 Natural History of Foot Deformities in Children with Cerebral Palsy
Introduction
Natural History and Pathophysiology
Treatment
Complications
Cases
Cross-References
References
147 Ankle Valgus in Cerebral Palsy
Introduction
Natural History
Treatment
Diagnostic Evaluations
Indications for Intervention
Outcome of Treatment
Complications of Treatment
Conclusion
Cases
Cross-References
References
148 Ankle Equinus in Cerebral Palsy
Introduction
Natural History and Pathophysiology
Etiology
Secondary Pathology
Tertiary Changes
Treatment
Diagnostic Evaluations
Treatment
Cavus and Equinus
Outcome of Treatment
Other Treatment
Complications of Treatment
Cases
Cross-References
References
149 Equinovarus Foot Deformity in Cerebral Palsy
Introduction
Natural History and Pathophysiology
Secondary Pathology
Tertiary Changes
Treatment
Indications and Treatment
Adolescents
Fixed Heel Varus
Severe Fixed Spastic Clubfeet
Outcome of Treatment
Other Treatments
Complications of Treatment
Conclusion
Cases
Cross-References
References
150 Planovalgus Foot Deformity in Cerebral Palsy
Introduction
Natural History (Chap. 146, ``Natural History of Foot Deformities in Children with Cerebral Palsy´´)
Etiology
Pathologic Deformity in Ambulators
Primary Pathology
Secondary Pathology
Tertiary Pathology
Midfoot Break with Rocker Bottom Foot
Pathologic Deformity in Nonambulators
Treatment
Diagnostic Evaluations
Indications
Reconstruction
Lateral Column Lengthening
Subtalar Fusion
Calcaneocuboid Lengthening Fusion
Isolated Talonavicular fusion
Multiple Arthrodesis
Outcome of Treatment
Other Treatments
Complications of Treatment
Surgical Procedures (Chap. 152, ``Atlas of Foot and Ankle Procedures in Cerebral Palsy´´)
Midfoot Supination and Dorsal Bunion
Indications and Treatment
Complications
Cases
Cross-References
References
151 Forefoot and Toe Deformities in Cerebral Palsy
Introduction
Natural History
Dorsal Bunion
Hallux Valgus with Bunion
Pathology
Natural History
Diagnostic Evaluations
Extended Hallux
Minor Toes
Ingrown Toenails
Treatment and Outcome
Dorsal Bunion
Hallux Valgus with Bunion
Outcome of Treatment Hallux Valgus
Extended Hallux Treatment
Minor Toes Treatment
Ingrown Toe Nails (Onychocryptosis) Treatment
Blue Feet: Sympathetic Vascular Dysfunction
Treatment
Complications
Complications of Dorsal Bunion
Complications of Hallux Valgus
Cases
Cross-References
References
152 Atlas of Foot and Ankle Procedures in Cerebral Palsy
Introduction
Ankle Epiphysiodesis Screw
Indication
Procedure
Postoperative Care
Subtalar Fusion
Indication
Procedure
Postoperative Care
Lateral Column Lengthening Through the Calcaneus
Indication
Procedure
Postoperative Care
Lateral Column Lengthening Through the Calcaneocuboid Joint
Indication
Procedure
Postoperative Care
Medial Column Correction: Forefoot Supination and First Ray Elevation
Indication
Procedure
Postoperative Care
Triple Arthrodesis
Indication
Procedure
Postoperative Care
Gastrocnemius Lengthening
Indication
Procedure
Postoperative Care
Achilles Tendon Lengthening
Indication
Procedure
Postoperative Care
Tibialis Posterior Lengthening or Split Transfer
Indication
Procedure
Postoperative Care
Split Tibialis Anterior Transfer
Indication
Procedure
Postoperative Care
Lengthening of the Tibialis Anterior
Indication
Procedure
Postoperative Care
Bunion Correction
Indication
Procedure
Fusion of the First Metatarsal Phalangeal Joint
Indication
Procedure
Postoperative Care
Correction of Clawed Toes
Indication
Procedure
Postoperative Care
Medial Border Great Toenail Resection
Indication
Procedure
Postoperative Care
References
Part XXV: Therapy Management in Cerebral Palsy: Introduction
153 Therapy Management of the Child with Cerebral Palsy: An Overview
Introduction
The Second Edition of Dr. Freeman Miller´s Medical Text: Cerebral Palsy
Therapy Management in the Child with Cerebral Palsy
Introduction: Expert Opinion
Life Span Approaches and Environmental Settings
Body Structure and Functions
Activity and Participation
Adaptive Technology and Supports
Complementary Therapy Approaches
Cross-References
References
154 Physical Therapy Elements in the Management of the Child with Cerebral Palsy
Introduction
Goals and Environment
Technique
Key Points
Adaptations in the Movement System Require Repetition and Repeated Exposure to Stimuli to Induce the Desired Change
PT Interventions Must Address Goals and Outcomes That Are Meaningful to the Child and Family
Motor Learning Is an Active Process: Practice with Variable, Graded Sensory and Task Constraints Should Be Balanced for Engage...
PT Interventions Should Not Only Emphasize the Learning of the Skill but Also the ``Retrieval´´ and Actual Use of the Skill in...
The Timing of Interventions Affects Intervention Outcomes
The Sum of Impairments Does Not Equal the Limits on Activities or Restrictions in Participation
Address the Need for Adherence and Behavior Change Directly with Evidence-Based Approaches
Evidence of Effectiveness
Conclusion
Cross-References
References
155 Occupational Therapy Elements in the Management of the Child with Cerebral Palsy
Introduction
Goals and Environment
Techniques
Body Function and Structure Impact on Activity
Occupational Therapy Intervention Strategies Body Function and Structure Impact on Activity
Upper Limb Strengthening and Orthotics
Tactile and Sensory Integration Interventions for Children with Unilateral Cerebral Palsy
Constraint-Induced Movement Therapy and Hand-Arm Bimanual Training
Occupational Therapy Interventions to Improve Participation in Activity
Specific Occupational Therapy Strategies
Interventions for Feeding, Eating, and Drinking
Approaches to Self-Care Activities
Family Considerations in Occupational Therapy
Evidence of Effectiveness
Cross-References
References
156 Speech, Language, and Hearing Practice Elements in the Management of the Child with Cerebral Palsy
Introduction
Goals and Environment
Technique
Communication Activity and Participation
The Role of Communication in a Child´s Participation in Life Situations
Speech Practice Elements
Language Practice Elements
Hearing Practice Elements
Elements of Environmental and Personal Factors
Evidence of Effectiveness
References
Part XXVI: Lifespan Approaches and Environmental Settings
157 Therapies in Newborn and Pediatric Intensive Care Units for the Neurologic At-Risk Infants
Introduction
Goals and Environment
Technique
Examination
NICU
Positioning
Range of Motion/Active Movement
Developmental Skills
PICU
Positioning
Range of Motion/Splinting and Casting
Mobility
Evidence of Effectiveness
Case Studies
Case 1: NICU
Case 2: PICU
Cross-References
References
158 Early Intervention Services for Young Children with Cerebral Palsy
Introduction
Goals and Environment
Body Function and Structure, Impact on Activity, and Participation in Activity
Personal Factors
Environment Factors
Technique
Evaluation
Interventions
Teaming Models
Evidence of Effectiveness
Cases
Cross-References
References
159 Innovative Approaches to Promote Mobility in Children with Cerebral Palsy in the Community
Introduction
Introduction: Community Mobility Is a Human Right
Mobility Goals and Environments
Community Mobility Approaches Are Built upon a Comprehensive Scientific Foundation
Technique
Assistive and Rehabilitative Tech Approaches to Community Mobility
Evidence of Effectiveness
Modified Ride-On Cars (ROCs): Hybrid Technology for Clinic and Community
General Features of ROCs
Real-World Body Weight Support Systems: Hybrid Technology for Impairments, Functional Activities, and Participation Within EE
Conclusion: Creating the High-Impact Community Mobility Professional
Cross-References
References
160 Outpatient-Based Therapy Services for Children and Youth with Cerebral Palsy
Introduction
The Setting
Accessing Services
Episodic Care
Frequency of Outpatient Therapy Services
Evolving Care
Cross-References
Appendix A
References
161 School-Based Therapy Services for Youth with Cerebral Palsy
Introduction
Educationally Relevant Services Provided by Medical Professionals
Related Service Provision Under IDEA
Section 504 and IDEA
Goals and Environment
IEP as the Structure for School-Based Practice
Educational Environment as a Practice Setting
Technique
Evidence of Effectiveness
Conclusion
Case Study: Christian
Cross-References
References
162 Community Resources: Sports and Active Recreation for Individuals with Cerebral Palsy
Introduction: Promoting Participation in Sports and Active Recreation
Goals and Environment
Techniques
Case Example
Evidence of Effectiveness
Cross-References
References
163 Clinical Therapy Services for Adults with Cerebral Palsy
Introduction
Health Needs
Rehabilitation Needs and Environment
Assessing Rehabilitation Needs
Goals
Techniques
Conclusion
Cross-References
References
164 Community Engagement for Adults with Cerebral Palsy
Introduction
Goals and Environment
Techniques and Evidence of Effectiveness
Transportation
Home Health and Support Services
Assistive Technology and Home and Workplace Accessibility Accommodations
Assistive Technology
Home Modifications and Accessibility Adaptations
Other Assistive Technology Devices
Workplace Modifications
Postsecondary Education, Work, and Vocational Services
Postsecondary Education
Work and Vocational Services
Clinical Case Example
References
Part XXVII: Body Structure and Functions
165 Postural Control in Children and Youth with Cerebral Palsy
Introduction
Systems Underlying Posture
Development and Theory
Categorizing and Testing Posture
Deficits in Posture in Cerebral Palsy
Goals and Environment
Technique
Assessments
Principles of Motor Learning
Interventions
Biomechanical
Strength or Muscle Facilitation
Massed Practice
Enhanced Feedback
Perturbation Training
Evidence of Effectiveness
Evaluation of Research
Improvement
Adaptability
Retention
Consistency
Theoretical Concepts
Professional Practice Reflections with Respect to Device Modifications
Cross-References
References
166 Selective Voluntary Motor Control in Children and Youth with Spastic Cerebral Palsy
Introduction
Goals and Environment
Technique
Evaluation of SVMC
Treatment Approaches
Evidence of Effectiveness
Cross-References
References
167 Using Hippotherapy Strategies for Children and Youth with Cerebral Palsy
Introduction
Goals and the Environment
Environment
Technique
Examination/Evaluation
Plan of Care
Hippotherapy Principles
Evidence of Effectiveness
Cross-References
References
168 Muscle Performance in Children and Youth with Cerebral Palsy: Implications for Resistance Training
Introduction
Central and Peripheral Contributions to Impairments in Muscle Performance
Goals and Environment
Technique: Dosing Guidelines for Resistance Training
Application: What Is the Goal of the Intervention?
Evidence of Effectiveness
Cross-References
References
169 Aquatic Therapy for Individuals with Cerebral Palsy Across the Lifespan
Introduction
Goals and Environment
Body Function and Structure
Impact on Activity
Impact on Participation
Environmental Factors Personal (Family) Factors
Aquatic Properties
Refraction
Hydrostatic Pressure
Density/Specific Gravity
Buoyancy (Archimedes´ Principle)
Drag/Turbulence
Physiological Effects
Assessment and Evaluation
Specialized Aquatic Techniques
Halliwick Concept
Bad Ragaz Ring Method
WATSU/Water Shiatsu
Transitioning to Land-Based Therapy
Evidence of Effectiveness
Case Studies
Case 1 (Longitudinal)
Birth History
7 Years of Age
12 Years Old
14-22 Years of Age
Case 2
Birth History
Cross-References
Bibliography
170 Functional Electrical Stimulation Interventions for Children and Youth with Cerebral Palsy
Introduction
History of Electrical Stimulation
Types of Electrical Stimulation
Goals and Environment
Technique
FES Background
Neuroprosthetic and Neurotherapeutic Mechanisms of FES
Rationale for FES Use in CP
FES Precautions
Risks Associated with External Electrical Stimulation of Muscles
Risks Associated with Exercise
Commercially Available FES Devices
FES for Hand Function
FES-Assisted Walking Devices
FES Cycling Devices
Evidence of Effectiveness
FES for the Upper Extremities and Trunk
FES-Assisted Walking
FES Application to the Ankle Dorsiflexor Muscles
Ankle Dorsiflexor Neuroprosthetic Effects
Ankle Dorsiflexor Neurotherapeutic Effects
FES as an Alternative to Ankle-Foot Orthoses
FES Application to the Ankle Plantar Flexors (APFs)
FES Cycling
FES Tolerance and Acceptability
Conclusion
Limitations and Future Direction
References
171 Aerobic and Anaerobic Fitness in Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
International Classification of Functioning, Disability, and Health (ICF-CY)
Energy Demands
Physical Strain
Physical Fitness
Aerobic Capacity
Aerobic Performance
Anaerobic Performance
Technique
Physical Fitness Testing
Testing Aerobic Fitness
Testing Energy Demands of Walking and Physical Strain
Testing Anaerobic Fitness
Evidence of Effectiveness
Fitness Training
Effect on the Aerobic Capacity
VO2peak
Anaerobic Threshold
Effect on Aerobic Performance
Effect on Anaerobic Performance
The Role of Exercise Testing and Advices on an Individual Basis
References
172 Flexibility in Children and Youth with Cerebral Palsy
Introduction
Neural Contributions to Reduced Flexibility
Nonneural Contributions to Reduced Flexibility
Goals and Environment
Body Function and Structure
Activity
Participation
Environmental Factors
Personal Factors
Measurement Techniques
Measuring Flexibility
Measuring Flexibility: Spasticity
Measuring Flexibility: Increased Passive Muscle Stiffness
Measuring Flexibility: Contracture
Interventions
Interventions to Improve Flexibility in Children and Youth with CP
Interventions to Address Spasticity
Interventions to Address Increased Passive Muscle Stiffness
Interventions to Address Lower Extremity Contracture
Interventions to Address Upper Extremity Contracture
Evidence of Effectiveness
Measuring Flexibility: Spasticity
Measuring Flexibility: Increased Passive Muscle Stiffness
Measuring Flexibility: Contracture
Interventions to Address Spasticity and Their Impact on Spasticity
Interventions to Address Spasticity and Their Impact on Impairments, Activity, and Participation
Interventions to Address Increased Passive Muscle Stiffness and Their Impact on Passive Muscle Stiffness
Interventions to Address Contracture and Their Impact on Contracture
Interventions to Address Contracture and Their Impact on Impairments, Activity, and Participation
Summary of Evidence Effectiveness
References
173 Postsurgical Therapy for the Individual with Cerebral Palsy
Introduction
Goals and Environment: Service Delivery
Preoperative Evaluation and Planning
Postoperative Inpatient Acute Hospital Therapy Model
Home-Based Therapy Model
Rehabilitation Program Therapy Service Model
Outpatient Therapy Service Model
School-Based Therapy
Recovery Expectations
Single Event Multilevel Surgery (SEMLS) for the Individual with Cerebral Palsy
Special Considerations for the Progression of Weight Bearing after Orthopedic Surgeries
Technique
Hip
Knee
Lower Leg & Foot
Spine
Intrathecal Baclofen Pump
Upper Extremity
Summary/Evidence of Effectiveness
Cross-References
References
Part XXVIII: Activity and Participation
174 Fine Motor Skill Development in Children and Youth with Unilateral Cerebral Palsy
Introduction
Goals and Environment
Neural Integrity
Classification of Grip/Pinch, Prehension, and Upper Extremity Function
Atypical Prehension and Upper Extremity Function
Muscle Activation
Prehension Patterns
Sensibility
Anticipatory Control
Selective Motor Control
Technique: Strengthening of Prehension and Upper Extremity Function
Assessment/Outcome Measures
Classification of Functional Hand Use
Effective Intervention Strategies
Evidence of Effectiveness
References
175 Functional Mobility and Gait in Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Technique
Classification
The International Classification of Functioning, Disability, and Health ICF
Gross Motor Function Classification System
Sagittal Gait Patterns: Spastic Hemiplegic Cerebral Palsy
Sagittal Gait Patterns: Spastic Diplegic Cerebral Palsy
Assessment
Instrumented Gait Analysis (IGA)
Video Gait Analysis
Functional Mobility Scale
Functional Assessment Questionnaire
Gross Motor Function Measure
The Pediatric Evaluation of Disability Inventory (PEDI)
Timed Walking Measures
Timed Up and Go Test (TUG)
The Functional Independence Measure for Children (WeeFIM)
Goal Attainment Scale (GAS)
Activity Monitoring
The Canadian Occupational Performance Measure
The Children´s Assessment of Participation and Enjoyment
Gait Outcomes Assessment List
Evidence of Effectiveness
Intramuscular Injections of Botulinum Toxin A
Selective Dorsal Rhizotomy
Single Event Multilevel Surgery
Muscle Strength Interventions
Functional Electrical Stimulation
Robotic-Assisted Gait Training
Summary
Cross-References
References
176 Robot-Assisted Gait Training for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Positioning RAGT Within the ICF
Goals of Robotic Assisted Gait Training
Technique
Equipment
Control Modes
Exergames
Indications, Contra-Indications, Possible Complications
Dosage of RAGT
Evidence of Effectiveness
Cases
References
177 Treadmill Training for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Body Functions and Structures
Activity
Participation
Contextual Factors
Technique
Treadmill Training for Children with Hemiplegic CP or Unilateral Impairment
Treadmill Training for Children with Diplegic (Bilateral) CP and GMFCS Levels I-II
Treadmill Training for Children with Diplegic or Quadriplegic CP (Bilateral) and GMFCS Levels III and IV
Evidence of Effectiveness
Cross-References
References
178 Functional ADL Training for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Technique
Mental Functions as They Relate to Functional Skill Acquisition Within the ICF Framework
Sensory Functions as They Relate to Functional Skill Acquisition Within the ICF Framework
Neuromusculoskeletal and Movement-Related Functions as They Relate to Functional Skill Acquisition Within the ICF Framework
Evidence of Effectiveness
Cross-References
References
179 Constraint-Induced Movement Therapy for Children and Youth with Hemiplegic/Unilateral Cerebral Palsy
Introduction
The Problem to Be Addressed
What Is CIMT: The Theoretical Concepts
Principles for Training
Goals and ICF
Technique and Environment
Intensive Group Models/Day-Camps
Distributed Individual-Based Models Home/Preschool Models
Combined Models CIMT/Bimanual Training/GOAL
Distributed Internet/Phone Models
Evidence of Efficacy
Natural History of the Development of Hand Function
Early Plasticity and Early Training
Strength, Limitation, and Cost Effectiveness
In Summary: CIMT in a Life Perspective
Cross-References
References
180 Assessment and Treatment of Feeding in Children and Youth with Cerebral Palsy
Introduction
Natural History
Assessment
Examples of How the Health Condition (CP) May Impact Feeding
Diet History and Bowel/Bladder Elimination
Feeding History/Caregiver Interview
Clinical Assessment
Oral Motor Assessment
Feeding Observation
Self-Feeding
Trial Therapy
Cervical Auscultation
Instrumental Assessments
Intervention
Caregivers and the Child
Physicians, Dentists, and Physician Assistants
Psychologist
Dietitian
Speech and Language Pathologist (SLP)
Occupational Therapist (OT)
Physical Therapist (PT)
Social Worker
Educational/Support Staff
Elements of Treatment
Tube Feeding
Goals of Treatment
Conclusion
References
181 Communication in Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Speech Intelligibility
Language
Literacy
Technique
Assessment
Assessment of Speech
Connection Between Speech and Language
Assessment of Language
Assessment of Early Language
Assessment of Conventional Language
Assessment of Emergent and Conventional Literacy
Intervention
Speech Intervention
Language Intervention
Language Modeling
Partner-Focused Intervention Approaches
Intervention for Emergent and Conventional Literacy
Modifications
Evidence of Effectiveness
Cross-References
References
182 Mobility Supports in Educational Curriculum for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Techniques to Determine Services, Dosing, and Transition/Discontinuation
Interventions and Evidence of Effectiveness
Positioning
MOVE Program
Mobility Training
Fitness Interventions (Strengthening, Stretching, and Aerobic Conditioning)
CMT
Consultation
Case Studies
Cross-References
References
183 Gaming Technologies for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Body Function and Structure Goals
Activity Goals
Participation Goals
Technique
Technology and Equipment
Fine Motor Function and Activity Protocol Parameters
Gross Motor Function and Activity Protocol Parameters
Postural Control and Balance Protocol Parameters
Fitness Protocol Parameters
Participation
Summary of Evidence of Effectiveness and Future Applications
VR/AVG as a Supplement to Rehabilitation Interventions
VR/AVG Systems in Development
VR/AVGs in Clinical Practice
Case Examples
Conclusion
Cross-References
References
Part XXIX: Adaptive Technology and Supports
184 Wheeled Mobility Options and Indications for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Overview of Current Issues
Technique
Wheeled Mobility Examination and Evaluation
Wheeled Mobility Options
Wheeled Mobility Training
Transfer of Behavior
Practice
Feedback
Evidence of Effectiveness
Case Scenarios
Cross-References
References
185 Ambulatory Assistive Devices for Children and Youth with Cerebral Palsy
Introduction
Standing Devices
Ambulatory Devices
Types of Ambulatory Devices
Gait Trainer
Walkers
Crutches
Canes
Therapeutic Ambulation Devices
Cross-References
References
186 Seating and Positioning Approaches for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Technique: Seating and Positioning Components - Definition of Terms (Fig 1)
Evidence of Effectiveness
Cross-References
References
187 Activities of Daily Living Supports for Persons with Cerebral Palsy
Introduction
Technique: ADLs
Evidence of Effectiveness
Cross-References
References
188 Lower Extremity Orthoses for Children and Youth with Cerebral Palsy
Introduction
History and Terminology
Treatment and Expected Outcome
Hip Frontal Plane
Hip Transverse Plane
Twister Cables
Derotation Elastic Wraps
Hip Multiplane
SWASH Brace
Knee Orthoses
Knee Sagittal Plane
Recurvatum: Back-Kneeing
Knee Flexion Contracture
Ankle-Foot Orthoses
Equinus in Stance and Swing Phase
Ankle Hyper-Dorsiflexion with Stance Knee Flexion (Crouch)
Swing Phase Ankle Equinus with Normal Stance Ankle Posture
Foot Deformities
Specific Orthotic Design Considerations by Age and Special Conditions
Preambulatory Stage
Articulated Ground Reaction AFO
Wraparound AFO Design
Anterior Ankle Strap
Foot Plate
Off-The-Shelf (Ready-To-Fit) Shoe Inlays and Arch Supports
Complications, Risks, and Benefits of Lower Extremity Orthotic
Cross-References
References
189 Upper Extremity Orthotics for Children and Youth with Cerebral Palsy
Introduction
Goals and Environment
Technique
General Principles of Splinting
Postoperative Orthotic Intervention
Contracture Management
Functional
Motor Reeducation and Exercise Orthoses
Evidence of Effectiveness
References
190 Augmentative and Alternative Communication for Cerebral Palsy
Introduction
Goals and Environment
Technique
AAC Evaluation
Identifying Communication Needs to Increase Participation
Determining AAC System Including Symbols, Aids, Techniques, and Strategies
AAC Intervention
Being Able to Carry Out One´s Communication Rights
Interventions Using the WHO ICF Framework
Communication Competencies
For Beginning Communicators
Evidence of Effectiveness
Conclusion
Cross-References
References
Part XXX: Complementary Therapy Approaches
191 Neurodevelopmental Treatment Clinical Practice Model´s Role in the Management of Children with Cerebral Palsy
Introduction
Goal and Environment
Body Function and Structure
Activity and Participation
Contextual Factors in the ICF
Technique
Information Gathering
Examination
Evaluation
Intervention
Pretest
Set Up
Handling
Preparation
Simulation
Posttest
Home Program and Carryover
Important Factors
Frequency
Indications, Contraindications, and Possible Complications
Evidence of Effectiveness
Cross-References
References
192 Complementary Therapy Approaches for Children and Youth with Cerebral Palsy
Introduction
Therapeutic Suits and Garments
Therapeutic Suits: Goals and Environment
Therapeutic Suits: Technique
Therapeutic Suits: Evidence of Effectiveness
Therapeutic Garments: Goals and Environment
Therapeutic Garments: Techniques
Therapeutic Garments: Evidence of Effectiveness
Therapeutic Taping
Goals and Environment
Techniques
Evidence of Effectiveness
Yoga
Goals and Environment
Technique
Evidence of Effectiveness
Pilates
Goals and Environment
Technique
Evidence of Effectiveness
Myofascial Release
Goals and Environment
Technique
Evidence of Effectiveness
Craniosacral Therapy
Goals and Environment
Technique
Evidence of Effectiveness
Conductive Education
Goals and Environment
Technique
Evidence of Effectiveness
Patterning
Goals and Environment
Technique
Evidence of Effectiveness
Feldenkrais
Goals and Environment
Technique
Evidence of Effectiveness
Vojta Therapy
Goals and Environment
Technique
Evidence of Effectiveness
Medical Marijuana
Goals and Environment
Technique
Evidence of Effectiveness
Cross-References
References
Index

Citation preview

Freeman Miller Steven Bachrach Nancy Lennon Margaret E. O’Neil Editors

Cerebral Palsy Second Edition

Cerebral Palsy

Freeman Miller • Steven Bachrach Nancy Lennon • Margaret E. O’Neil Editors

Cerebral Palsy Second Edition

With 1503 Figures and 175 Tables

Editors Freeman Miller Department of Orthopaedics Nemours/Alfred I. duPont Hospital for Children Wilmington, DE, USA Nancy Lennon Nemours/Alfred I. duPont Hospital for Children Wilmington, DE, USA

Steven Bachrach Department of Pediatrics Nemours/Alfred I. duPont Hospital for Children Wilmington, USA Margaret E. O’Neil Columbia University Irving Medical Center Programs in Physical Therapy New York, NY, USA

ISBN 978-3-319-74557-2 ISBN 978-3-319-74558-9 (eBook) ISBN 978-3-319-74559-6 (print and electronic bundle) https://doi.org/10.1007/978-3-319-74558-9 1st edition: © Springer Science+Business Media, Inc. 2005 2nd edition: © Springer Nature Switzerland AG 2020 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG. The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

We dedicate this work to the many children, youth, and adults with cerebral palsy and like conditions, as well as their families, who have occupied our practice for the past 30 years. We thank them for entrusting us with their care and for all they have taught us. It is our goal to transmit this knowledge to a broad range of health professionals so other individuals affected by this condition may benefit from the collaborative, comprehensive, coordinated, and interprofessional approach we use in our clinical care model.

Foreword

In 2005, Springer published a landmark textbook with the title “Cerebral Palsy” by Dr. Freeman Miller, Director of the Cerebral Palsy Program at the world-renowned Alfred I duPont Hospital for Children, Nemours Foundation, Wilmington, Delaware. This comprehensive text has become a classic in the field and is highly regarded by physicians from all specialities who care for children with cerebral palsy. Surgeons have turned to this text when confronted with perplexing issues in the management of children with cerebral palsy. They have invariably found authoritative, practical answers to the most difficult management problems. There seems to be no scenario which Dr. Miller and his colleagues have not encountered and consequently they are able to give practical help, when it is most needed. I have turned to this text throughout the past 14 years on many occasions for advice on how to approach surgery for a specific child. I have also used it to prepare teaching material for our Residents and Fellows in Orthopedic Surgery at the Royal Children’s Hospital, Melbourne. With such a success story, why a second edition? The field has moved quickly during the past 14 years and a wealth of new research has been published in fields from epidemiology, early diagnosis, early intervention, stem cell therapies, hip surveillance, movement disorder management, medical care, and surgical techniques. Dr. Miller and his colleagues have contributed mightily to that worldwide research effort, with numerous publications in the peer-reviewed literature. The second edition is in three manageable volumes and has combined e-publication, shorter chapters, easier access, and a wealth of new material from the duPont team and from colleagues from the USA and around the world. There is no more extensive, up-to-date, and complete repository of practical information addressing care of the child with cerebral palsy than this second edition. Given that orthopedic surgeons should be “physicians who operate,” the new material on the medical care and perioperative management of children with cerebral palsy will be invaluable. The emphasis is on care for children with cerebral palsy, but there is invaluable content dealing with the needs of adolescents, young adults, and the sometimes perplexing problem of transition to adult services. Dr. Miller and I were both heavily influenced by the late Dr. Mercer Rang, when we were fellows at the Hospital for Sick Children in Toronto. The Mercer Rang influence flows as a current underpinning this text, in terms of clarity, authority, and humanity. vii

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Who should read this book and who will find it helpful? The short answer is all who are involved in contemporary care of children with cerebral palsy. This includes orthopedic surgeons, neurosurgeons, neurologists, developmental pediatricians, physiatrists, physiotherapists, and occupational therapists. I congratulate Dr. Miller and his co-contributors on this mammoth effort. This book will have a worldwide reach. It will influence a generation of clinicians and researchers and it will improve the standards of care for children with cerebral palsy around the globe. Director, Hugh Williamson Kerr Graham, M.D., FRCS(Ed), FRACS Gait Laboratory Orthopaedic Surgeon Royal Children’s Hospital, Melbourne Professor of Orthopaedic Surgery University of Melbourne

Foreword

Preface

Cerebral palsy is a condition that is life long, affecting the individual, family, and immediate community. The goal of supporting the individual with cerebral palsy to live life with the least impact of their disability requires a focus on the individual’s and the family’s needs and goals. Furthermore, it is important for society to be sensitive to and accommodate individuals with disabilities by limiting architectural impediments, having accessible public transportation and accessible communication available. The educational system provides the key means for helping the individual function in society to his or her maximum ability by providing academic and vocational skill building. In many ways, the medical care system probably has the least significant role in preparing the child with cerebral palsy to function at his or her best in society. However, the medical care system is the place where parents receive a diagnosis or an explanation about their child’s developmental problem. It is almost universally the place where parents also expect their child to receive services in order to achieve the same milestones that children with “typical development” achieve. Parents want their children to “be normal” like any other child in our modern society, but this hope is often challenged by the reality of the emerging disability. The goal of this text is to provide a resource with updated evidence to support health professionals in their role as managers of children with disabilities, so that these children can optimize their function and grow into adults who are as healthy, mobile, and functional as possible, despite their diagnosis. This is with the knowledge that ideal medical management during growth will only improve but not cure the condition or remove the impairments that contribute to the disability. Probably the most significant aspect of good medical management through childhood is helping the child and family to maintain realistic goals and hopes during the growth and development years of the child. This requires the medical practitioner to get to know the child and family and have honest, realistic expectations of the child’s function, which are communicated in a compassionate manner. For many reasons, by far the largest difficulty in providing this kind of care is the limited time spent with patients in many medical practices. There is also the sense, especially among some physicians, that cerebral palsy cannot be cured (meaning make the child normal) and, therefore, it is a frustrating condition to treat. This is often seen in the statement “don’t torment the poor child with surgery, just let him be.” The physician who makes this statement communicates hopelessness to the child ix

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and to the family. On the other hand, it is also crucial not to fall into the trap of telling frustrated parents a small surgery or some other invasive treatment can be done, because they are frustrated that the child is not progressing. All medical decisions, including “to do and not to do interventions,” should always consider both the short-term impact and the long-term impact and outcome. With every decision the medical practitioner should ask “what will be the impact of this recommendation by the time the child is a mature adult?” The ability to “prognosticate” what will happen to the child as he or she matures to adulthood and when to conduct or implement an intervention is the most difficult component of care, especially for young practitioners who have little experience to draw upon. It is the goal of this text to provide this insight and, as much as possible, to guide the reader in the trajectory of the condition so that interventions are timely and delivered at the most optimal time for best outcomes. This brings up the issue of the very poor scientific documentation of the natural history of cerebral palsy, although there has been increased interest and publications related to natural history since the first edition of this text 15 years ago. However, there is still limited scientifically based natural history and few long-term studies reporting the impact of childhood conditions and intervention outcomes in adults. Due to this deficit, much of what is written in this text is still expert-based observation. The goal of writing this is not to document what is currently absolute fact but to provide at least the starting point of information with the hope that others will be stimulated to ask questions and pursue research to prove or disprove the concepts presented here on long-term trajectories and outcomes. Research to improve treatment and outcomes for children with cerebral palsy needs to be planned and evaluated, taking into account its long-term impact on the child’s growth and development. All treatment should also consider the negative impact on a child’s experience. As an example, the impact of wearing ankle orthotics is often minimal for the young child, with an immediate benefit to improvement in the child’s gait. There probably is no long-term benefit. This cost–benefit ratio is documented with a number of moderately good studies in which the child is tested with and without the brace. On the other hand, if the child develops a strong sense of opposition around brace wear at 10 years of age because of peer pressure, the brace wear cannot be justified on a cost–benefit analysis. It is also important to consider the quality of the scientific evidence, ranging from double-blinded protocols to case reports. However, it is equally important not to get hung up on this being the final answer. As an example, there are excellent double-blinded studies to show that botulinum toxin decreases spasticity and improves gait, but only for a few months. This knowledge has to be kept in the context of our goal, which is the child’s maximum possible function at full maturity. There is currently increased evidence to suggest that botulinum toxin has a negative long-term effect by causing permanent muscle fibrosis. Therefore, the family and physician should be deciding together whether the short-term gain outweighs the long-term negative impact of botulinum toxin. A major problem is that the data to make these choices often have a high subjective element.

Preface

Preface

xi

You will note that this book is divided into four comprehensive parts: (1) Etiology of CP, (2) General Medical Concerns, (3) Orthopedic Concerns, and (4) Therapy Management. It is the goal of this book to stimulate interest among a wide range of interdisciplinary providers in doing research to improve the knowledge base that is focused on the long-term outcome of our treatments. However, just because the scientific knowledge base is poor, does not mean that we should not be making an effort to apply the best knowledge available in our clinical management and care. In the last 15–20 years there has been a significant increase in interest by specialists in the primary and secondary conditions and body systems that are impacted by cerebral palsy and cerebral palsy–like conditions. The current expansion of this text has followed this interest and we have tried to find experts in each of the body systems to present the current evidence and state of the art relative to their expertise. Many of these experts have developed the interest due to seeing an increasing number of children with cerebral palsy, and a major way the knowledge base of an individual professional is expanded is by personal experience. This means the child and family should be followed over time by the same practitioner with good documentation. One of the best sources of information has been the children that were followed during growth period for 10–20 years with videotapes every year or two. The review of such video history can be very educational. Careful ongoing follow-up is also crucial to providing patient- and family-centered care to address the hopes, needs, and goals of the families and individuals with cerebral palsy. Freeman Miller Steven Bachrach Nancy Lennon Margaret E. O’Neil

Acknowledgments

The production of this second edition of the textbook on Cerebral Palsy was made easier by the work of the many people acknowledged in the first edition. The major expansion of this second edition required recruitment of many specialists in other disciplines. We thank the many authors and coauthors of all the chapters for their participation. In addition, we are very appreciative of the contributions by artists for illustrations that greatly enhance the submitted chapters. Erin Brown provided many illustrations for the first edition of the book. Most of these illustrations are also present in this volume, some of the illustrations have been updated. Steven Cook has submitted illustrations in the ear, nose, and throat chapters. The illustrations of Karlee Diane Rogers are very helpful to understand the anatomy of the bones and muscles in the respective chapters. The completion of this volume would not have been possible without the support of the staff at Springer. We especially thank Barbara Wolf and Veronika Mang for their steadfast guidance and assistance.

Acknowledgments from the First Edition The support of the administration of the Nemours Foundation, especially the support of Roy Proujansky and J. Richard Bowen in giving me time to work on this project was crucial. It was only through the generous support in caring for my patients by my partners and staff, Kirk Dabney, Suken Shah, Peter Gabos, Linda Duffy, and Marilyn Boos, that I was able to dedicate time to writing. I am very grateful for the generous material provided by all the contributors and for the extensive and extremely important role of the feedback given to me by the consultants. In spite of having an extremely busy practice, Kirk Dabney still found time to read all of the first section, making very valuable improvements, and writing major sections of the upper extremity chapter. With his wide experience, Michael Alexander made an excellent contribution in the editorial support of the section on rehabilitation. The task of writing and editing would have been impossible without the dedicated work of Kim Eissmann, Linda Donahue, and Lois Miller. To add a personal touch to the cases, a unique name was assigned by Lois Miller. The CD, which accompanied the first edition only required a great effort of technical programming to make it work intuitively on all computer formats. Tim Niiler patiently persisted with this frustrating task until it all worked. Videos were masked and formatted by Robert DiLorio. Production of the graphics was a major effort in xiii

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understanding the complex material in which Erin Browne excelled. This production would have been impossible without her dedication to understanding the concepts and bringing them to visual clarity. I would also like to thank the staff of Chernow Editorial Services, especially Barbara Chernow. Without the long support through the evolution of this book by Robert Albano and his staff at Springer, this project would also have been much more difficult. And finally, I am most grateful for the many families and children who have allowed me to learn from them what it is like to live with the many different levels of motor impairments. It is to the families and children that I dedicate this work in the hope that it will lead to improved care and understanding by medical professionals.

Acknowledgments

Contents

Volume 1 Section 1: Diagnosis and Pathology Part I 1

Diagnosis and Pathology

............................

1

The Child, the Parent, and the Goal in Treating Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Freeman Miller

3

Part II

Etiology of Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . .

21

2

Cerebral Palsy and the Relationship to Prematurity Michael Favara, Jay Greenspan, and Zubair H. Aghai

......

23

3

Genetic Abnormalities and Congenital Malformations as a Cause of Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . Kristen Ferriero and Pamela Arn

37

4

Infectious Etiologies of Cerebral Palsy . . . . . . . . . . . . . . . . . . Neil Rellosa

45

5

Perinatal Stroke as an Etiology of Cerebral Palsy . . . . . . . . . Nidhi Shah and Gregory C. Griffin

55

6

Problems During Delivery as an Etiology of Cerebral Palsy in Full-Term Infants . . . . . . . . . . . . . . . . . . . . . . . . . . . Patrick Philpot, Jay Greenspan, and Zubair H. Aghai

7

Postnatal Causes of Cerebral Palsy . . . . . . . . . . . . . . . . . . . . Laura Owens, Eileen Shieh, and Abigail Case

8

Animal Models of Cerebral Palsy: What Can We Learn About Cerebral Palsy in Humans . . . . . . . . . . . . . . . . . . . . . . Asher Ornoy

9

The Effects of Umbilical Cord Blood and Cord Tissue Cell Therapies in Animal and Human Models of Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Jessica M. Sun and Joanne Kurtzberg

67 77

85

97 xv

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10

Contents

Risk Factors for Developing Cerebral Palsy . . . . . . . . . . . . . 111 Antigone Papavasileiou and Marianna Petra

Part III Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

129

11

Epidemiology of Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . 131 Kate Himmelmann, Sarah McIntyre, Shona Goldsmith, Hayley Smithers-Sheedy, and Linda Watson

12

Health and Healthcare Disparities in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147 Kirk W. Dabney, Ruth Ziegler, and Laurens Holmes

Part IV

Pathology

.........................................

175

13

Neuroimaging Pathology in Cerebral Palsy . . . . . . . . . . . . . . 177 Rahul M. Nikam, Arabinda K. Choudhary, Vinay Kandula, and Lauren Averill

14

Current Imaging: PET Scan Use in Cerebral Palsy . . . . . . . 217 Sreenath Thati Ganganna and Harry T. Chugani

15

Neuromuscular Junction Changes in Spastic Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 227 Karyn G. Robinson and Robert E. Akins

16

Muscle Changes at the Cellular-Fiber Level in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241 Sudarshan Dayanidhi and Richard L. Lieber

17

Muscle Size, Composition, and Architecture in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253 Christopher M. Modlesky and Chuan Zhang

18

Bone Size, Architecture, and Strength Deficits in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269 Christopher M. Modlesky and Chuan Zhang

Part V

Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

285

19

When and How to Evaluate the Child with Possible Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287 Sonika Agarwal

20

Cerebral Palsy Prognosis Based on the Physical and Neurologic Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 297 S. Charles Bean

21

Classification Terminology in Cerebral Palsy . . . . . . . . . . . . 309 Katherine B. Bevans and Carole A. Tucker

Contents

xvii

22

Measuring Outcomes in Children with Cerebral Palsy . . . . . 325 Colyn J. Watkins, Rachel L. DiFazio, and Benjamin J. Shore

23

Biomarker Blood Tests for Cerebral Palsy Robert E. Akins and Karyn G. Robinson

. . . . . . . . . . . . . . 339

Section 2: General Medical Part VI

General Medical Concerns . . . . . . . . . . . . . . . . . . . . . . . . . .

347

24

General Nutrition for Children with Cerebral Palsy . . . . . . . 349 Nicole Fragale, Natalie Navarre, and Jaclyn Rogers

25

Managing the Child with Cerebral Palsy Who Has Medical Complexity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Dennis Z. Kuo, Sara R. Slovin, and Amy E. Renwick

26

Managing Bone Fragility in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371 Heidi H. Kecskemethy and Steven Bachrach

27

Managing Irritability and Nonoperative Pain in the Noncommunicative Child with Cerebral Palsy . . . . . . . . . . . 395 Tracy Hills and Steven Bachrach

28

Palliative Care for Individuals with Cerebral Palsy . . . . . . . 413 Elissa Miller, Carly Levy, and Lindsay Ragsdale

29

Aging with Cerebral Palsy: Adult Musculoskeletal Issues . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423 M. Wade Shrader

30

Life Care Planning for the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 433 Doreen Casuto, Andrea Nebel, and Haydee Piña

Part VII

Central Neurologic Problems . . . . . . . . . . . . . . . . . . . . . . .

455

31

Epilepsy in the Child with Cerebral Palsy . . . . . . . . . . . . . . . 457 Stephen Falchek

32

Epilepsy Surgery for the Child with Cerebral Palsy . . . . . . . 469 Badal G. Jain and Harry T. Chugani

33

Hydrocephalus in the Child with Cerebral Palsy Jeffrey Campbell

Part VIII 34

. . . . . . . . . 483

Psychologic and Psychiatric Problems . . . . . . . . . . . . . .

Psychiatric Disorders in Children with Cerebral Palsy Rhonda S. Walter and Richard S. Kingsley

495

. . . . 497

xviii

Contents

35

Autism Spectrum Disorder in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505 Meghan Harrison and Persephone Jones

36

Family Stress Associated with Cerebral Palsy . . . . . . . . . . . . 515 Heidi Fritz and Carrie Sewell-Roberts

37

The Impact of Cerebral Palsy on Siblings . . . . . . . . . . . . . . . 547 Claire Shrader and Stephanie Chopko

Part IX

Neuromotor Function

..............................

557

38

Motor Control and Muscle Tone Problems in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 559 Freeman Miller

39

Spasticity Assessment in Cerebral Palsy . . . . . . . . . . . . . . . . 585 Lynn Bar-On, Jaap Harlaar, and Kaat Desloovere

40

Medical Management of Spasticity in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 601 Maura McManus

41

Focal Management of Spasticity in Cerebral Palsy . . . . . . . . 611 Freeman Miller

42

Intrathecal Baclofen Therapy: Assessment and Medical Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 629 Maura McManus

43

Intrathecal Medication Administration in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 639 Julieanne P. Sees and Freeman Miller

44

Dorsal Rhizotomy for Spasticity Management in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 651 P. Nilsson, N. Wesslén, H. Axelsson, G. Ahlsten, and L. Westbom

45

Dystonia and Movement Disorders in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 661 Freeman Miller and Stephen Falchek

46

Deep Brain Stimulation for Pediatric Dystonia . . . . . . . . . . . 679 Michelle A. Wedemeyer and Mark A. Liker

47

Ataxia and Disorders of Balance in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 689 Robert O’Reilly and Erin Field

48

Assessing Dynamic Balance in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 695 Timothy A. Niiler

Contents

xix

Part X

Gastrointestinal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

727

49

Overview of Feeding and Growth in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 729 Devendra I. Mehta, Nneka Ricketts-Cameron, and Heidi H. Kecskemethy

50

Gastrostomy and Jejunostomy Feedings in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 741 J. Fernando del Rosario

51

Gastroesophageal Reflux in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 751 Arieda Gjikopulli, Erika Kutsch, Loren Berman, and Sky Prestowitz

52

Medical and Surgical Therapy for Constipation in Patients with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . 767 Erin A. Teeple, Roberto A. Gomez Suarez, and Charles D. Vinocur

Part XI

Ear, Nose, and Throat . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

781

53

Medical Management of Sialorrhea in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 783 Jeremiah Sabado and Laura Owens

54

Surgical Options for Sialorrhea Management in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . 799 Christopher Tsang, Steven Cook, and Udayan Shah

55

Auditory Rehabilitation in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 811 Kiley Trott, Amy Powell, Yell Inverso, and William J. Parkes

56

Upper Airway Obstruction in the Child with Cerebral Palsy: Indication for Adenotonsillectomy . . . . . . . . . . . . . . . 819 Brian Swendseid and Heather C. Nardone

57

Surgical Management of Tracheostomies and Tracheal Diversion in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 829 Richard Schmidt, Christopher Tsang, and Patrick Barth

Part XII 58

Genitourinary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

841

Toilet Training and Bladder Control in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 843 Puneeta Ramachandra and T. Ernesto Figueroa

xx

Contents

59

Neurogenic Bladder in Cerebral Palsy: Upper Motor Neuron . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 853 Hong Truong and Ahmad H. Bani Hani

60

Kidney Stones: Risks, Prevention, and Management in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 871 Carlos E. Araya and Ahmad H. Bani Hani

61

Undescended Testis in Boys with Cerebral Palsy Julia Spencer Barthold and Jennifer A. Hagerty

62

Gynecological Issues in Girls and Young Women with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 891 Beth I. Schwartz and Chelsea Kebodeaux

Part XIII

. . . . . . . . . 885

Pulmonary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

905

63

Bronchopulmonary Dysplasia and Cerebral Palsy . . . . . . . . 907 Frances Flanagan and Anita Bhandari

64

Asthma in a Child with Cerebral Palsy . . . . . . . . . . . . . . . . . 917 Katherine A. King and Dawn Selhorst

65

Aspiration in the Child with Cerebral Palsy . . . . . . . . . . . . . 925 Aaron Chidekel and Lauren Greenawald

66

Medical Management of Tracheostomy in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 937 Jodi Gustave and Ambika Shenoy

67

Obstructive Sleep Apnea in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 949 Abigail Strang and Aaron Chidekel

Part XIV

Endocrine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

957

68

Short Stature in Children with Cerebral Palsy . . . . . . . . . . . 959 Kevin J. Sheridan

69

Growth Attenuation for the Child with Cerebral Palsy . . . . 979 Jonathan M. Miller and Evan Graber

70

Premature and Delayed Sexual Maturation in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 985 Kevin J. Sheridan

71

Endocrine Dysfunction in Children with Cerebral Palsy . . . 1003 Hussein Elmufti and Robert C. Olney

Part XV 72

Eyes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1013

Testing Visual Function and Visual Evaluation Outcomes in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . 1015 Elise Ciner, Sarah Appel, Marcy Graboyes, and Erin Kenny

Contents

xxi

73

Strabismus Management in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1041 Jonathan H. Salvin and Dorothy Hendricks

74

Cortical Visual Impairment in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1049 Sharon S. Lehman

Part XVI Dental . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1057 75

Dental Hygiene for Children with Cerebral Palsy . . . . . . . . . 1059 Nadarajah Ganeshkumar

76

General Dentistry for Children with Cerebral Palsy . . . . . . . 1067 Harvey Levy

77

Management of Skeletal Facial Deformation and Malocclusion in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . 1105 Joseph A. Napoli, Stephanie Drew, and Tim C. Jaeger

Part XVII

Anesthesia Management

. . . . . . . . . . . . . . . . . . . . . . . . . 1121

78

Medical Evaluation for Preoperative Surgical Planning in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . 1123 Emily Fingado and David Pressel

79

Anesthesia in the Child with Cerebral Palsy . . . . . . . . . . . . . 1131 Dinesh K. Choudhry and Mary C. Theroux

80

Postoperative Pain and Spasticity Management in the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1145 B. Randall Brenn and Dinesh K. Choudhry

81

Regional Anesthesia in Patients with Cerebral Palsy . . . . . . 1159 Kesavan Sadacharam, Robert P. Brislin, and R. Scott Lang

82

Anesthetic Management of Spine Fusion . . . . . . . . . . . . . . . . 1185 Mary C. Theroux and Sabina Dicindio

83

Postoperative Care of the Cerebral Palsy Patient . . . . . . . . . 1193 Hussam Alharash, Maxine Ames, Smitha Mathew, David Rappaport, and Nicholas Slamon

Part XVIII

Complementary Medical Treatments

. . . . . . . . . . . . . 1215

84

Complementary and Alternative Medicine in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1217 Rachel M. Thompson and William Lawrence Oppenheim

85

Hyperbaric Oxygen Therapy for Cerebral Palsy: Definition and Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1227 Jenny L. Wilson and Barry Russman

xxii

Contents

86

Acupuncture and Traditional Chinese Medicine Used to Treat Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1237 Shuyun Jiang

87

Osteopathic Manipulative Treatment and Acupuncture in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1251 Julieanne P. Sees

Volume 2 Section 3: Orthopedic Part XIX

Gait in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1255

88

Musculoskeletal Physiology Impacting Cerebral Palsy Gait . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1257 Freeman Miller

89

Normal Human Gait . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1277 Freeman Miller

90

Cerebral Palsy Gait Pathology . . . . . . . . . . . . . . . . . . . . . . . . 1299 Freeman Miller

91

History and Physical Examination Components of Gait Analysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1309 Daveda Taylor

92

Diagnostic Gait Analysis Technique for Cerebral Palsy . . . . 1323 Freeman Miller

93

Kinematics and Kinetics: Technique and Mechanical Models . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1339 Kristen Nicholson

94

Foot Kinematics: Models Used to Study Feet in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . 1355 John Henley

95

Pedobarograph Foot Evaluations in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1373 Freeman Miller

96

Measuring Femoral and Tibial Torsion in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1381 Brian Po-Jung Chen

97

Aerobic Conditioning and Walking Activity Assessment in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1401 Nancy Lennon and Freeman Miller

98

Gait Analysis Interpretation in Cerebral Palsy Gait: Developing a Treatment Plan . . . . . . . . . . . . . . . . . . . . . . . . . 1413 Freeman Miller

Contents

xxiii

99

Gait Treatment Outcome Assessments in Cerebral Palsy . . . 1429 Freeman Miller

100

Hemiplegic or Unilateral Cerebral Palsy Gait . . . . . . . . . . . . 1437 Freeman Miller

101

Diplegic Gait Pattern in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1457 Freeman Miller

102

Hip and Pelvic Kinematic Pathology in Cerebral Palsy Gait . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1471 Freeman Miller

103

Crouch Gait in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . 1489 Freeman Miller

104

Knee Deformities Impact on Cerebral Palsy Gait . . . . . . . . . 1505 Freeman Miller

105

Foot Deformities Impact on Cerebral Palsy Gait Freeman Miller

106

Complications from Gait Treatment in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1533 Freeman Miller

107

The Evolution of Knee Flexion During Gait in Patients with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1543 Reinald Brunner

Part XX

. . . . . . . . . 1517

Upper Extremity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1557 . . . . 1559

108

The Upper Extremity in Cerebral Palsy: An Overview Freeman Miller

109

Upper Extremity Assessment and Outcome Evaluation in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1569 Lena Krumlinde-Sundholm and Lisa V. Wagner

110

Physical Examination and Kinematic Assessment of the Upper Extremity in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . 1599 Freeman Miller

111

Spasticity, Dystonia, and Athetosis Management in the Upper Extremity in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . 1609 Freeman Miller

112

Single-Event Multilevel Surgery for the Upper Extremity in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1619 Freeman Miller

113

Shoulder and Elbow Problems in Cerebral Palsy . . . . . . . . . 1629 Freeman Miller

xxiv

Contents

114

Forearm, Thumb, and Finger Deformities in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1643 Jennifer Ty and Freeman Miller

115

Upper Extremity Operative Procedures in Cerebral Palsy . . . 1669 Freeman Miller

Part XXI

Spine . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1699

116

Spinal Deformity in Children with Cerebral Palsy: An Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1701 Freeman Miller

117

Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management . . . . . . . . . . . . . . . . 1711 Freeman Miller

118

Surgical Treatment of Scoliosis Due to Cerebral Palsy . . . . . 1723 Kirk W. Dabney and M. Wade Shrader

119

Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . 1743 Kirk W. Dabney

120

Early-Onset Scoliosis in Cerebral Palsy . . . . . . . . . . . . . . . . . 1763 Freeman Miller

121

Complications of Spine Surgery in Cerebral Palsy . . . . . . . . 1777 Freeman Miller

122

Neuromonitoring and Anesthesia for Spinal Fusion in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1801 Sabina Dicindio, Anthony DiNardo, and Mary C. Theroux

123

Cervical Spine in Children with Cerebral Palsy . . . . . . . . . . 1813 Freeman Miller

124

Pelvic Alignment and Spondylolisthesis in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1823 Freeman Miller

125

Infections and Late Complications of Spine Surgery in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1833 Freeman Miller

126

Spinal Procedure Atlas for Cerebral Palsy Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1851 Freeman Miller and Kirk W. Dabney

Part XXII Hip . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1871 127

Hip Problems in Children with Cerebral Palsy: An Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1873 Freeman Miller

Contents

xxv

128

Etiology of Hip Displacement in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1881 Freeman Miller

129

Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1893 Freeman Miller

130

Prophylactic Treatment of Hip Subluxation in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1907 Freeman Miller

131

Hip Reconstruction in Children with Cerebral Palsy . . . . . . 1923 Freeman Miller

132

Palliative or Salvage Hip Management in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1951 Freeman Miller

133

Anterior Dislocation of the Hip in Cerebral Palsy . . . . . . . . . 1973 Freeman Miller

134

Hypotonic and Special Hip Problems in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1989 Freeman Miller

135

Femoral Anteversion in Children with Cerebral Palsy . . . . . 2009 Freeman Miller

136

Windblown Hip Deformity and Hip Contractures in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2027 Freeman Miller

137

Complications of Hip Treatment in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2049 Freeman Miller

138

Surgical Atlas of Cerebral Palsy Hip Procedures . . . . . . . . . 2079 Freeman Miller

Part XXIII

Knee . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2117

139

Overview of Knee Problems in Cerebral Palsy . . . . . . . . . . . 2119 Freeman Miller

140

Anterior Knee Pain and Patellar Subluxation in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2127 Freeman Miller

141

Knee Flexion Deformity in Cerebral Palsy Freeman Miller

142

Stiff Knee and Knee Extension Deformities in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2159 Freeman Miller

. . . . . . . . . . . . . . 2137

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Contents

143

Tibial Torsion and Knee Instability in Cerebral Palsy . . . . . 2171 Freeman Miller

144

Atlas of Knee Operative Procedures in Cerebral Palsy Freeman Miller

Part XXIV

. . . . 2185

Ankle, Foot, and Toes . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2209

145

Foot Deformities in Children with Cerebral Palsy: An Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2211 Freeman Miller

146

Natural History of Foot Deformities in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2223 Freeman Miller and Chris Church

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Ankle Valgus in Cerebral Palsy Freeman Miller

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Ankle Equinus in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . 2243 Freeman Miller

149

Equinovarus Foot Deformity in Cerebral Palsy Freeman Miller

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Planovalgus Foot Deformity in Cerebral Palsy . . . . . . . . . . . 2289 Freeman Miller

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Forefoot and Toe Deformities in Cerebral Palsy . . . . . . . . . . 2329 Freeman Miller

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Atlas of Foot and Ankle Procedures in Cerebral Palsy . . . . . 2351 Freeman Miller

. . . . . . . . . . . . . . . . . . . . . . . 2233

. . . . . . . . . . 2267

Volume 3 Section 4: Therapy Management in Cerebral Palsy Part XXV Therapy Management in Cerebral Palsy: Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2393 153

Therapy Management of the Child with Cerebral Palsy: An Overview . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2395 Margaret E. O’Neil and Nancy Lennon

154

Physical Therapy Elements in the Management of the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2405 Carole A. Tucker and Katherine B. Bevans

155

Occupational Therapy Elements in the Management of the Child with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . 2417 Laura K. Vogtle

Contents

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156

Speech, Language, and Hearing Practice Elements in the Management of the Child with Cerebral Palsy . . . . . . . . . . . 2431 Mary Jo Cooley Hidecker

Part XXVI Lifespan Approaches and Environmental Settings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2443 157

Therapies in Newborn and Pediatric Intensive Care Units for the Neurologic At-Risk Infants . . . . . . . . . . . . . . . . 2445 Rachel Unanue Rose

158

Early Intervention Services for Young Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2455 Alyssa LaForme Fiss and Lynn Jeffries

159

Innovative Approaches to Promote Mobility in Children with Cerebral Palsy in the Community . . . . . . . . . . . . . . . . . 2473 James C. (Cole) Galloway

160

Outpatient-Based Therapy Services for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . 2483 Karen Josefyk and Allyson Menard

161

School-Based Therapy Services for Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2491 Laurie Ray and Susan K. Effgen

162

Community Resources: Sports and Active Recreation for Individuals with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . 2507 Maria A. Fragala-Pinkham and Jennifer Miros

163

Clinical Therapy Services for Adults with Cerebral Palsy . . . 2519 Mary Gannotti and David Frumberg

164

Community Engagement for Adults with Cerebral Palsy . . . 2543 Margo N. Orlin and Susan Tachau

Part XXVII Body Structure and Functions . . . . . . . . . . . . . . . . . . . 2563 165

Postural Control in Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2565 Sandra L. Saavedra and Adam D. Goodworth

166

Selective Voluntary Motor Control in Children and Youth with Spastic Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . 2587 Theresa Sukal-Moulton and Eileen Fowler

167

Using Hippotherapy Strategies for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2611 Annette M. Willgens and Ellen A. Erdman

168

Muscle Performance in Children and Youth with Cerebral Palsy: Implications for Resistance Training . . . . . . 2629 Noelle G. Moreau

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Contents

169

Aquatic Therapy for Individuals with Cerebral Palsy Across the Lifespan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2641 Deborah E. Thorpe and Emily E. Paul

170

Functional Electrical Stimulation Interventions for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . 2661 Samuel C. K. Lee, Ahad Behboodi, James F. Alesi, and Henry Wright

171

Aerobic and Anaerobic Fitness in Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . 2687 Astrid C. J. Balemans and Eline A. M. Bolster

172

Flexibility in Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2709 Catie Christensen

173

Postsurgical Therapy for the Individual with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2733 Karen R. Turner, Betsy Mullan, Nicole Needles, and Danielle Stapleton

Part XXVIII

Activity and Participation . . . . . . . . . . . . . . . . . . . . . . . 2751

174

Fine Motor Skill Development in Children and Youth with Unilateral Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . 2753 Susan V. Duff and Aviva L. Wolff

175

Functional Mobility and Gait in Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2767 Pam Thomason

176

Robot-Assisted Gait Training for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2797 Hubertus J. A. van Hedel and Andreas Meyer-Heim

177

Treadmill Training for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2817 Ann Tokay Harrington

178

Functional ADL Training for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2829 Faithe R. Kalisperis, Kathleen Miller-Skomorucha, and Jason Beaman

179

Constraint-Induced Movement Therapy for Children and Youth with Hemiplegic/Unilateral Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2845 Ann-Christin Eliasson and Andrew M. Gordon

Contents

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180

Assessment and Treatment of Feeding in Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . 2857 Marianne E. Gellert-Jones

181

Communication in Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2883 Beth A. Mineo

182

Mobility Supports in Educational Curriculum for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . 2903 Kathleen Benson, Kristin Capone, Kimberly Duch, and Christine Palmer-Casey

183

Gaming Technologies for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2917 Torey Gilbertson, Lin-Ya Hsu, Sarah Westcott McCoy, and Margaret E. O’Neil

Part XXIX

Adaptive Technology and Supports

. . . . . . . . . . . . . . 2947

184

Wheeled Mobility Options and Indications for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . 2949 Maria A. Jones

185

Ambulatory Assistive Devices for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2963 Mary Bolton and Maureen Donohoe

186

Seating and Positioning Approaches for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . 2977 Elizabeth Koczur, Denise Peischl, and Carrie Strine

187

Activities of Daily Living Supports for Persons with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2987 Maureen Donohoe and Patricia Hove

188

Lower Extremity Orthoses for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2999 David Hudson, Heather Michalowski, and Freeman Miller

189

Upper Extremity Orthotics for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3023 Tracy M. Shank and Charles Cericola

190

Augmentative and Alternative Communication for Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3041 Mary Jo Cooley Hidecker

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Contents

Part XXX

Complementary Therapy Approaches . . . . . . . . . . . . . 3051

191

Neurodevelopmental Treatment Clinical Practice Model’s Role in the Management of Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3053 Faithe R. Kalisperis, Jeanne-Marie Shanline, and Jane Styer-Acevedo

192

Complementary Therapy Approaches for Children and Youth with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . 3069 Roberta O’Shea and Gina Siconolfi-Morris

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3083

About the Editors

Dr. Freeman Miller was Co-director of the Cerebral Palsy Program and the Clinical Director of the Gait Analysis Laboratory at the Alfred I. duPont Hospital for Children for 30 years. He continues as an emeritus staff member at Alfred I. duPont Hospital for Children providing consultative services and is active in the research program. His clinical practice of pediatric orthopedics is limited to children with cerebral palsy. For the past 25 years, Dr. Miller has held Adjunct Professor appointments in the Departments of Mechanical Engineering and Physical Education at the University of Delaware. He is also a member of the University BIOMS program, which is an interdisciplinary graduate program in biomedical engineering. Current and past research interests include investigation of surgical outcomes of CP surgery through gait analysis, mathematical modeling of the hip joint in children with CP, hip monitoring and management for children with CP, and management of spinal deformity in CP. Dr. Miller has published approximately 200 articles in peer-reviewed journals. He has been invited to give many lectures in over 35 different countries. As a coauthor with Dr. Bachrach, he published a book Cerebral Palsy: A Guide for Caregiving directed at families and nonmedical care providers, which was published in 1995, second edition in 2006, and in 2017 was revised and released as the third edition. A medical textbook, Cerebral Palsy, with 1080 pages outlining musculoskeletal care of the child with cerebral palsy, was written by Dr. Miller and published in 2005 by Springer-Verlag.

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About the Editors

Dr. Steven Bachrach was Co-director of the Cerebral Palsy Program and Chief of the Division of General Pediatrics at the Nemours/Alfred I. duPont Hospital for Children in Wilmington, DE, for nearly 30 years. He is Board Certified in Pediatrics and Neuro-developmental Disabilities, and his clinical practice encompassed both general pediatrics and the care of children with developmental disabilities. He continues as an emeritus staff member at Alfred I. duPont Hospital for Children, primarily involved in educational and research efforts. Dr. Bachrach also served for 20 years as Medical Director of the HMS School for Children with Cerebral Palsy in Philadelphia, PA, and currently serves on their Board of Directors. He is also a consultant to the Alyn Hospital in Jerusalem, Israel. Dr. Bachrach has a faculty appointment as Professor of Pediatrics, Clinical and Educational Scholarship Track, at the Sidney Kimmel Medical College of Thomas Jefferson University. He has been a member of the American Academy of Cerebral Palsy and Developmental Medicine for over 35 years and has often been a presenter at their annual meetings. His research focus has been on children with cerebral palsy, and especially the evaluation and treatment of osteoporosis in children who are non-ambulatory. Dr. Bachrach has published over 30 articles in peer-reviewed journals, as well as a number of book chapters and abstracts. As a coauthor with Dr. Miller, he published the book Cerebral Palsy: A Guide for Caregiving, which was aimed at a lay audience and published by Johns Hopkins Press in 1995, second edition in 2006, and the third edition in 2017. Dr. Bachrach has been married for 46 years and has 4 children and 14 grandchildren, all of whom he enjoys spending time with.

About the Editors

xxxiii

Ms. Nancy Lennon a physical therapist, is the manager of the Cerebral Palsy (CP) Program at the Nemours/Alfred I. duPont Hospital for Children in Wilmington, Delaware, where she works with Dr. Miller and Dr. Bachrach. She has many years of experience in the Gait Analysis Laboratory and in Nemours Biomedical Research, where she collaborates with Dr. O’Neil. Ms. Lennon has a Bachelor of Science degree in physical therapy from the University of Delaware and a Master of Science degree from Hahnemann University. She has 30 years of clinical experience in working with children and families affected by cerebral palsy, 15 years of experience conducting clinical research, and 5 years of experience coordinating family engagement and advisory activities at the hospital. Margaret E. O’Neil is a Professor in the Programs in Physical Therapy, Department of Rehabilitation and Regenerative Medicine at Columbia University, Vagelos College of Physicians and Surgeons. She conducts research, advises graduate students, and teaches content in pediatric physical therapy and research. She has received grant funding from several federal and professional organizations. In her research, she examines objective measures of physical activity for youth with cerebral palsy and effectiveness of activity-based interventions (including active video games and virtual reality) to promote fitness and activity. She collaborates with an interprofessional team to conduct her research and they have published widely on these topics. She co-teaches clinical workshops on measurement and intervention strategies to promote strength and fitness in children with disabilities. She serves as a grant reviewer for multiple agencies and is a member of the APTA and AACPDM where she serves on multiple committees.

Contributors

Sonika Agarwal Division of Neurology, Perelman School of Medicine at University of Pennsylvania/Children’s Hospital of Philadelphia, Philadelphia, PA, USA Zubair H. Aghai Nemours/Thomas Jefferson University, Philadelphia, PA, USA G. Ahlsten Department of Pediatrics, University Hospital, Uppsala, Sweden Robert E. Akins Nemours Biomedical Research, Nemours – Alfred I. duPont Hospital for Children, Wilmington, DE, USA James F. Alesi Department of Physical Therapy, University of Delaware, Newark, DE, USA Hussam Alharash Critical Care Division, Department of Pediatrics, Nemours, AI DuPont Hospital for Children, Wilmington, DE, USA Maxine Ames Division of General Pediatrics, Nemours, AI DuPont Hospital for Children, Wilmington, DE, USA Sarah Appel William Feinbloom Vision Rehabilitation Center, The Eye Institute of Salus University, Philadelphia, PA, USA Carlos E. Araya Division of Pediatric Nephrology, Nemours Children’s Hospital, Orlando, FL, USA Pamela Arn Department of Pediatrics, Nemours Children’s Specialty Care, Jacksonville, FL, USA Department of Clinical Genomics, Mayo Clinic, Jacksonville, FL, USA Lauren Averill Nemours A I duPont Hospital for Children, Wilmington, DE, USA H. Axelsson Department of Neuroscience, Neurophysiology, University Hospital, Uppsala, Sweden Steven Bachrach Department of Pediatrics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, USA

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Astrid C. J. Balemans Department of Rehabilitation Medicine, Amsterdam Movement Sciences, Amsterdam Public Health, VU University Medical Center, Amsterdam, The Netherlands Center of Excellence for Rehabilitation Medicine, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht University and De Hoogstraat Rehabilitation, Utrecht, The Netherlands Ahmad H. Bani Hani Division of Pediatric Urology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Department of Urology and Pediatrics, Sidney Kimmel Medical collegeThomas Jefferson University, Philadelphia, PA, USA Lynn Bar-On Department of Rehabilitation Sciences, KU Leuven, Leuven, Belgium Department of Rehabilitation Medicine, Laboratory for Clinical Movement Analysis, MOVE Research Institute Amsterdam, VU University Medical Center, Amsterdam, The Netherlands Patrick Barth Division of Pediatric Otolaryngology, Nemours duPont Hospital for Children, Wilmington, DE, USA Department of Otolaryngology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA Julia Spencer Barthold Nemours Biomedical Research and Department of Surgery, Division of Urology, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Jason Beaman Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA S. Charles Bean Emeritus Professor of Pediatric Neurology, Nemours AI duPont Hospital, Wilmington, USA Ahad Behboodi Department of Physical Therapy, University of Delaware, Newark, DE, USA Kathleen Benson Physical Therapy, John G. Leach School, New Castle, DE, USA Loren Berman Division of Pediatric Surgery, Department of Pediatrics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Katherine B. Bevans Department of Health and Rehabilitation Sciences, College of Public Health, Temple University, Philadelphia, PA, USA Anita Bhandari Department of Pediatrics, Division of Pulmonary Medicine, Children’s Hospital of Philadelphia, Philadelphia, PA, USA Eline A. M. Bolster Department of Rehabilitation Medicine, Amsterdam Movement Sciences, Amsterdam Public Health, VU University Medical Center, Amsterdam, The Netherlands

Contributors

Contributors

xxxvii

Mary Bolton Nemours/Alfred I DuPont Hospital for Children, Wilmington, DE, USA Robert P. Brislin Division of Surgical Anesthesiology, Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Reinald Brunner Children’s University Hospital Basel, Basel, Switzerland Basel University, Basel, Switzerland Jeffrey Campbell Nemours/A.I. duPont Hospital for Children, Wilmington, DE, USA Kristin Capone Physical Therapy, John G. Leach School, New Castle, DE, USA Abigail Case Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Thomas Jefferson University, Philadelphia, PA, USA Doreen Casuto Rehabilitation Care Coordination, American Association of Nurse Life Care Planners, San Diego, CA, USA Charles Cericola Nemours A I duPont Hospital for Children, Wilmington, DE, USA Brian Po-Jung Chen Department of Pediatric Orthopedics and Traumatology, Poznań University of Medical Sciences, Poznań, Poland Aaron Chidekel Division of Pediatric Pulmonology, Nemours A.I. duPont Hospital for Children, Wilmington, DE, USA Stephanie Chopko Nemours /Alfred I. duPont Hospital for Children, Wilmington, DE, USA Sidney Kimmel Medical College/Thomas Jefferson University, Philadelphia, PA, USA Arabinda K. Choudhary Nemours A I duPont Hospital for Children, Wilmington, DE, USA Dinesh K. Choudhry Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Department of Anesthesiology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA Catie Christensen Nationwide Children’s Hospital, Westerville, OH, USA Harry T. Chugani NYU Comprehensive Epilepsy Center, Department of Neurology, NYU School of Medicine, New York, NY, USA Chris Church Nemours Alfred I. duPont Hospital for Children, Wilmington, DE, USA Elise Ciner Pediatric and Binocular Vision Service, The Eye Institute of Salus University, Philadelphia, PA, USA

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Steven Cook Division of Pediatric Otolaryngology, Department of Surgery, Nemours Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA Mary Jo Cooley Hidecker Division of Communication Disorders, University of Wyoming, Laramie, WY, USA Kirk W. Dabney Department of Orthopedics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA Sudarshan Dayanidhi Shirley Ryan AbilityLab, Chicago, IL, USA J. Fernando del Rosario Pediatric Gastroenterology, Hepatology and Nutrition, Nemours/A.I. DuPont Hospital for Children, Wilmington, DE, USA Kaat Desloovere Department of Rehabilitation Sciences, KU Leuven, Leuven, Belgium Sabina Dicindio Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Department of Pediatrics, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA Rachel L. DiFazio Orthopedic Center, Boston Children’s Hospital, Boston, MA, USA Anthony DiNardo SpecialtyCare, Nashville, TN, USA Maureen Donohoe Nemours/Alfred I DuPont Hospital for Children, Wilmington, DE, USA Stephanie Drew Department of Surgery, Division of Oral and Maxillofacial Surgery, Emory University School of Medicine, Atlanta, GA, USA Kimberly Duch Physical Therapy, John G. Leach School, New Castle, DE, USA Susan V. Duff Department of Physical Therapy, Crean College of Health and Behavioral Sciences, Chapman University, Irvine, CA, USA Susan K. Effgen Department of Rehabilitation Sciences, College of Health Sciences, University of Kentucky, Lexington, KY, USA Ann-Christin Eliasson Department of Woman and Child Health, Astrid Lindgren Children’s Hospital, Karolinska Institutet, Stockholm, Sweden Hussein Elmufti Division of Endocrinology, Diabetes, and Metabolism, Nemours Children’s Specialty Care, Jacksonville, FL, USA Department of Pediatrics, University of Florida, Jacksonville, FL, USA Ellen A. Erdman Widener University, Chester, PA, USA Stephen Falchek Division of Pediatric Neurology, Department of Pediatrics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

Contributors

Contributors

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Michael Favara Nemours/Thomas Jefferson University, Philadelphia, PA, USA Kristen Ferriero Alfred I. duPont Hospital for Children, Wilmington, DE, USA Erin Field Otolaryngology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA T. Ernesto Figueroa Division of Pediatric Urology, Department of Surgery, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Department of Urology and Pediatrics, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA Emily Fingado Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Alyssa LaForme Fiss Department of Physical Therapy, Mercer University, Atlanta, GA, USA Frances Flanagan Division of Pulmonary and Respiratory Diseases, Boston Children’s Hospital, Boston, MA, USA Eileen Fowler David Geffen School of Medicine University of California at Los Angeles, Los Angeles, CA, USA Maria A. Fragala-Pinkham Adaptive Sports and Medical Rehabilitation Research Center, Franciscan Children’s Hospital, Boston, MA, USA Nicole Fragale Clinical Nutrition, Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Heidi Fritz Department of Psychology, Salisbury University, Salisbury, MD, USA David Frumberg Department of Orthopedics and Rehabilitation, Yale School of Medicine, New Haven, CT, USA James C. (Cole) Galloway Department of Physical Therapy, University of Delaware, Newark, DE, USA Department of Psychology, University of Delaware, Newark, DE, USA Department of Linguistics and Cognitive Science, University of Delaware, Newark, DE, USA Biomechanics and Movement Science Graduate Program, University of Delaware, Newark, DE, USA Nadarajah Ganeshkumar Dover Pediatric Dentistry, Dover, NH, USA Sreenath Thati Ganganna Nemours/Alfred I. duPont Hospital for Children, Nemours Neuroscience Center, Wilmington, DE, USA Mary Gannotti Department of Rehabilitation Sciences, University of Hartford, West Hartford, CT, USA

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Marianne E. Gellert-Jones Clinical Feeding Specialist/Speech Language Pathologist, HMS School for Children with Cerebral Palsy, Philadelphia, PA, USA Torey Gilbertson Department of Rehabilitation Medicine, Division of Physical Therapy, University of Washington, Seattle, WA, USA Arieda Gjikopulli Division of Pediatric Gastroenterology and Nutrition, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Shona Goldsmith Cerebral Palsy Alliance, University of Sydney, Sydney, NSW, Australia Roberto A. Gomez Suarez Department of Gastroenterology, Nemours Children’s Hospital, Orlando, FL, USA Adam D. Goodworth Department of Rehabilitation Sciences, University of Hartford, West Hartford, CT, USA Andrew M. Gordon Department of Biobehavioral Sciences, Teachers College, Columbia University, New York, NY, USA Evan Graber Nemours A.I. duPont Hospital for Children and Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA Marcy Graboyes William Feinbloom Vision Rehabilitation Center, The Eye Institute of Salus University, Philadelphia, PA, USA Lauren Greenawald Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Jay Greenspan Nemours/Thomas Jefferson University, Philadelphia, PA, USA Department of Pediatrics, Nemours A.I. duPont Hospital for Children, Thomas Jefferson University, Philadelphia, PA, USA Gregory C. Griffin Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Jodi Gustave Division of Pediatric Pulmonology, Department of Pediatrics, Nemours/A.I. Dupont Hospital for Children, Wilmington, DE, USA Jennifer A. Hagerty Nemours Biomedical Research and Department of Surgery, Division of Urology, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Jaap Harlaar Department of Rehabilitation Medicine, Laboratory for Clinical Movement Analysis, MOVE Research Institute Amsterdam, VU University Medical Center, Amsterdam, The Netherlands Department of Biomechanical Engineering, Delft University of Technology, Delft, The Netherlands Ann Tokay Harrington Physical Therapy, Arcadia University, Glenside, PA, USA

Contributors

Contributors

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Meghan Harrison Department of Pediatrics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA Division of Developmental Medicine, Department of Pediatrics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA Dorothy Hendricks Division of Ophthalmology, Nemours/A.I. DuPont Hospital for Children, Wilmington, DE, USA Departments of Ophthalmology and Pediatrics, Sydney Kimmel Medical College/Wills Eye Hospital, Philadelphia, PA, USA John Henley Alfred I. duPont Hospital for Children, Wilmington, DE, USA Mary Jo Cooley Hidecker Division of Communication Disorders, University of Wyoming, Laramie, WY, USA Tracy Hills Department of Pediatrics, Monroe Carell Junior Children’s Hospital, Vanderbilt University, Nashville, TN, USA Kate Himmelmann Department of Pediatrics at Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden Laurens Holmes Department of Orthopedics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA Patricia Hove Tower Health Medical Group, Pennsylvania Hand Center, Paoli, PA, USA Lin-Ya Hsu Department of Rehabilitation Medicine, Division of Physical Therapy, University of Washington, Seattle, WA, USA David Hudson Western Carolina University, Cullowhee, NC, USA Yell Inverso Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Tim C. Jaeger Department of Surgery, Division of Oral and Maxillofacial Surgery, Emory University School of Medicine, Atlanta, GA, USA Badal G. Jain Division of Neurology, Department of Pediatrics, Nemours/ Alfred. I. duPont Hospital for Children, Wilmington, DE, USA Lynn Jeffries Department of Rehabilitation Sciences, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA Shuyun Jiang Yueyang Hospital of Integrated Traditional Chinese and Western Medicine, Shanghai University of Traditional Chinese Medicine, Shanghai, China Maria A. Jones Physical Therapy Program, Academic Affairs, Oklahoma City University, Oklahoma City, OK, USA Persephone Jones Department of Pediatrics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA Division of Developmental Medicine, Department of Pediatrics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA

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Karen Josefyk Nemours/A.I. duPont Hospital for Children, Wilmington, DE, USA Faithe R. Kalisperis Gait Lab, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Vinay Kandula Nemours A I duPont Hospital for Children, Wilmington, DE, USA Chelsea Kebodeaux Department of Obstetrics and Gynecology, Christiana Care Health System, Newark, DE, USA Heidi H. Kecskemethy Departments of Biomedical Research and Medical Imaging, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Erin Kenny William Feinbloom Vision Rehabilitation Center, The Eye Institute of Salus University, Philadelphia, PA, USA Katherine A. King Sidney Kimmel School of Medicine, Thomas Jefferson University, Philadelphia, PA, USA Pulmonary Division, Nemours Alfred I. duPont Hospital for Children, Wilmington, DE, USA Richard S. Kingsley Emeritus Staff, Division of Behavioral Health, Nemours, Alfred I. duPont Hospital for Children, Wilmington, DE, USA Elizabeth Koczur Alfred I. duPont Hospital For Children, Wilmington, DE, USA Lena Krumlinde-Sundholm Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden Dennis Z. Kuo Department of Pediatrics, University at Buffalo, Buffalo, NY, USA Joanne Kurtzberg Marcus Center for Cellular Cures, Robertson Clinical and Translational Cell Therapy Program, Duke University Medical Center, Durham, NC, USA Erika Kutsch Division of Pediatric Gastroenterology and Nutrition, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA R. Scott Lang Division of Surgical Anesthesiology, Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Samuel C. K. Lee Department of Physical Therapy, University of Delaware, Newark, DE, USA Sharon S. Lehman Nemours Children’s Health System, AI duPont Hospital for Children, Wilmington, DE, USA Sidney Kimmel Medical College, Philadelphia, PA, USA

Contributors

Contributors

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Nancy Lennon Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Carly Levy Department of Pediatrics, Division of Palliative Medicine, Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Sidney Kimmel Medical College Thomas Jefferson University, Philadelphia, PA, USA Harvey Levy Department of Surgery, Frederick Memorial Hospital, Frederick, MD, USA University of Maryland School of Dentistry, Baltimore, MD, USA Richard L. Lieber Shirley Ryan AbilityLab, Chicago, IL, USA Mark A. Liker Division of Neurosurgery, Children’s Hospital of Los Angeles, Los Angeles, CA, USA Department of Neurosurgery, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA Smitha Mathew Critical Care Division, Department of Pediatrics, Nemours, AI DuPont Hospital for Children, Wilmington, DE, USA Sarah Westcott McCoy Department of Rehabilitation Medicine, Division of Physical Therapy, University of Washington, Seattle, WA, USA Sarah McIntyre Cerebral Palsy Alliance, University of Sydney, Sydney, NSW, Australia Maura McManus Nemours Alfred I Dupont Hospital for Children, Wilmington, DE, USA Devendra I. Mehta Center for Digestive Health and Nutrition, Arnold Palmer Hospital for Children, Orlando, FL, USA The Florida State University, Tallahassee, FL, USA Allyson Menard Nemours/A.I. duPont Hospital for Children, Wilmington, DE, USA Andreas Meyer-Heim Rehabilitation Center for Children and Adolescents, University Children’s Hospital Zurich, Affoltern am Albis, Switzerland Heather Michalowski Lawall Prosthetics and Orthotics, Wilmington, DE, USA Elissa Miller Department of Pediatrics, Division of Palliative Medicine, Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Sidney Kimmel Medical College Thomas Jefferson University, Philadelphia, PA, USA Freeman Miller Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

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Jonathan M. Miller Nemours A.I. duPont Hospital for Children and Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA Kathleen Miller-Skomorucha Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Beth A. Mineo College of Education and Human Development, University of Delaware, Newark, DE, USA Jennifer Miros The Carol and Paul Hatfield Cerebral Palsy Sports Program and The Cerebral Palsy Center, St. Louis Children’s Hospital, St. Louis, MO, USA Christopher M. Modlesky Department of Kinesiology, University of Georgia, Athens, GA, USA Noelle G. Moreau Department of Physical Therapy, School of Allied Health Professions, Louisiana State University, Health Sciences Center, New Orleans, LA, USA Betsy Mullan Physical Therapy, OPT Therapy Services, Ltd., Wilmington, DE, USA Joseph A. Napoli Division of Pediatric Plastic and Maxillofacial Surgery, Department of Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Sydney Kimmel Medical College, Thomas Jefferson University, Wilmington, DE, USA Heather C. Nardone Division of Pediatric Otolaryngology, Nemours/Alfred I duPont Hospital for Children, Wilmington, DE, USA Otolaryngology and Pediatrics, Thomas Jefferson University, Philadelphia, PA, USA Natalie Navarre Clinical Nutrition, Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Andrea Nebel Rehabilitation Care Coordination, American Association of Nurse Life Care Planners, San Diego, CA, USA Nicole Needles DPT, Nemours: A.I. duPont Hospital for Children, Wilmington, DE, USA Kristen Nicholson Nemours/A. I. duPont Hospital for Children, Wilmington, DE, USA Timothy A. Niiler Gait Laboratory, Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Rahul M. Nikam Nemours A I duPont Hospital for Children, Wilmington, DE, USA P. Nilsson Department of Neuroscience, Neurosurgery, University Hospital, Uppsala, Sweden

Contributors

Contributors

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Margaret E. O’Neil Columbia University, Irving Medical Center, Programs in Physical Therapy, New York, NY, USA Robert O’Reilly Children’s Hospital of Philadelphia, Philadelphia, PA, USA Roberta O’Shea Physical Therapy, Governors State University, University Park, IL, USA Robert C. Olney Division of Endocrinology, Diabetes, and Metabolism, Nemours Children’s Specialty Care, Jacksonville, FL, USA William Lawrence Oppenheim Ronald Reagan UCLA Medical Center, Santa Monica, CA, USA Margo N. Orlin Department of Physical Therapy and Rehabilitation Sciences, Drexel University, Philadelphia, PA, USA Asher Ornoy Laboratory of Teratology, Department of Medical Neurobiology, Israel Canada Research Institute, Hebrew University Hadassah Medical School, Jerusalem, Israel Laura Owens Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Thomas Jefferson University, Philadelphia, PA, USA Christine Palmer-Casey Physical Therapy, John G. Leach School, New Castle, DE, USA Antigone Papavasileiou Iaso Children’s Hospital, Athens, Greece William J. Parkes Division of Otolaryngology, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Emily E. Paul UNC Therapy Services, Meadowmont Wellness Center, Chapel Hill, NC, USA Denise Peischl Alfred I. duPont Hospital For Children, Wilmington, DE, USA Marianna Petra Department of Orthopaedics, Pendeli Children’s Hospital, Athens, Greece Patrick Philpot Thomas Jefferson University, Philadelphia, PA, USA Haydee Piña Rehabilitation Care Coordination, San Diego, CA, USA Amy Powell Outpatient Therapy Services, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA David Pressel Capital Health, Trenton, NJ, USA Sky Prestowitz Philadelphia College of Osteopathic Medicine, Philadelphia, PA, USA Lindsay Ragsdale Department of Pediatrics, Pediatric Palliative Care, Kentucky Children’s Hospital, University of Kentucky, Lexington, KY, USA

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Puneeta Ramachandra Division of Pediatric Urology, Department of Surgery, Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Department of Urology and Pediatrics, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA B. Randall Brenn Monroe Carrell Children’s Hospital at Vanderbilt, Vanderbilt University Medical Center, Nashville, TN, USA David Rappaport Division of General Pediatrics, Nemours, AI DuPont Hospital for Children, Wilmington, DE, USA Laurie Ray Allied Health Sciences, Division of Physical Therapy, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA Neil Rellosa Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Amy E. Renwick Nemours Alfred I. duPont Hospital for Children, Wilmington, DE, USA Nneka Ricketts-Cameron Center for Digestive Health and Nutrition, Arnold Palmer Hospital for Children, Orlando, FL, USA Karyn G. Robinson Nemours Biomedical Research, Nemours – Alfred I. duPont Hospital for Children, Wilmington, DE, USA Jaclyn Rogers Clinical Nutrition, Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Rachel Unanue Rose Maryville University, Physical Therapy Program, St. Louis, MO, USA Barry Russman Shriners Hospitals for Children, Portland, OE, USA Pediatric Neurology, Oregon Health and Science University, Portland, OR, USA Sandra L. Saavedra Department of Rehabilitation Sciences, University of Hartford, West Hartford, CT, USA Jeremiah Sabado Division of Pediatric Radiology, Department of Medical Imaging, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Kesavan Sadacharam Division of Surgical Anesthesiology, Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Jonathan H. Salvin Division of Ophthalmology, Nemours/A.I. DuPont Hospital for Children, Wilmington, DE, USA Departments of Ophthalmology and Pediatrics, Sydney Kimmel Medical College/Wills Eye Hospital, Philadelphia, PA, USA

Contributors

Contributors

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Richard Schmidt Division of Pediatric Otolaryngology, Nemours duPont Hospital for Children, Wilmington, DE, USA Department of Otolaryngology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA Beth I. Schwartz Department of Obstetrics and Gynecology, Thomas Jefferson University, Philadelphia, PA, USA Division of Adolescent Medicine and Pediatric Gynecology, Department of Pediatrics, Nemours/A.I. duPont Hospital for Children, Wilmington, DE, USA Julieanne P. Sees Nemours Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Dawn Selhorst Respiratory Care Department, Nemours Alfred I. duPont Hospital for Children, Wilmington, DE, USA Carrie Sewell-Roberts Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Nidhi Shah Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Udayan Shah Division of Pediatric Otolaryngology, Department of Surgery, Nemours Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Department of Otolaryngology - Head and Neck Surgery and Pediatrics, Wilmington, DE, USA Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA Tracy M. Shank Nemours A I duPont Hospital for Children, Wilmington, DE, USA Jeanne-Marie Shanline Therapy Services, Nemours Alfred I. duPont Hospital for Children, Wilmington, DE, USA Ambika Shenoy Division of Pediatric Pulmonology, Department of Pediatrics, Nemours/A.I. Dupont Hospital for Children, Wilmington, DE, USA Kevin J. Sheridan Endocrinology, Gillette Children’s Specialty Hospital, Saint Paul, MN, USA Eileen Shieh Nemours/AI duPont Hospital for Children, Wilmington, DE, USA Thomas Jefferson University, Philadelphia, PA, USA Benjamin J. Shore Orthopedic Center, Boston Children’s Hospital, Boston, MA, USA Claire Shrader Mississippi College, Clinton, MS, USA M. Wade Shrader Cerebral Palsy, Nemours A.I. duPont Hospital for Children, Wilmington, DE, USA

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Gina Siconolfi-Morris Nemours Children’s Health System, Wilmington, DE, USA Nicholas Slamon Critical Care Division, Department of Pediatrics, Nemours, AI DuPont Hospital for Children, Wilmington, DE, USA Sara R. Slovin Nemours Alfred I. duPont Hospital for Children, Wilmington, DE, USA Hayley Smithers-Sheedy Cerebral Palsy Alliance, University of Sydney, Sydney, NSW, Australia Danielle Stapleton Nemours: A.I. duPont Hospital for Children, Wilmington, DE, USA Abigail Strang Division of Pediatric Pulmonology, Nemours A.I. duPont Hospital for Children, Wilmington, DE, USA Carrie Strine Alfred I. duPont Hospital For Children, Wilmington, DE, USA Jane Styer-Acevedo NDT and Aquatic Therapy, Upper Darby, PA, USA Physical Therapy Department Arcadia University, Glenside, PA, USA Neuro-Developmental Treatment Association (NDTA), Laguna Beach, CA, USA Theresa Sukal-Moulton Feinberg School of Medicine, Northwestern University, Chicago, IL, USA Jessica M. Sun Marcus Center for Cellular Cures, Robertson Clinical and Translational Cell Therapy Program, Duke University Medical Center, Durham, NC, USA Brian Swendseid Department of Otolaryngology, Thomas Jefferson University, Philadelphia, PA, USA Susan Tachau Pennsylvania Assistive Technology Foundation (PATF), King of Prussia, PA, USA Daveda Taylor The Gait and Motion Analysis Laboratory, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Erin A. Teeple Department of Surgery, Nemours/A.I. Dupont Hospital for Children, Wilmington, DE, USA Mary C. Theroux Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Department of Pediatrics, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA Department of Anesthesiology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA Pam Thomason Hugh Williamson Gait Analysis Laboratory, Royal Children’s Hospital, Melbourne, VIC, Australia

Contributors

Contributors

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Rachel M. Thompson Department of Orthopaedics, Orthopaedic Institute for Children/UCLA, Los Angeles, CA, USA Ronald Reagan UCLA Medical Center, Santa Monica, CA, USA Deborah E. Thorpe Division of Physical Therapy, School of Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA Kiley Trott Department of Otolaryngology – Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA Nemours/Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Hong Truong Department of Urology, Thomas Jefferson University Hospital, Philadelphia, PA, USA Christopher Tsang Division of Pediatric Otolaryngology, Department of Surgery, Nemours Alfred I. DuPont Hospital for Children, Wilmington, DE, USA Carole A. Tucker Department of Health and Rehabilitation Sciences, College of Public Health, Temple University, Philadelphia, PA, USA Karen R. Turner Nemours: A.I. duPont Hospital for Children, Wilmington, DE, USA Jennifer Ty AI DuPont Hospital for Children, Wilmington, DE, USA Hubertus J. A. van Hedel Rehabilitation Center for Children and Adolescents, University Children’s Hospital Zurich, Affoltern am Albis, Switzerland Charles D. Vinocur Department of Surgery, Nemours/A.I. Dupont Hospital for Children, Wilmington, DE, USA Laura K. Vogtle Department of Occupational Therapy, University of Alabama School of Health Professions, Birmingham, AL, USA Lisa V. Wagner Shriners Hospitals for Children, Greenvill, SC, USA Rhonda S. Walter Retired, Nemours, Alfred I. duPont Hospital for Children, Wilmington, DE, USA Colyn J. Watkins Orthopedic Center, Boston Children’s Hospital, Boston, MA, USA Linda Watson Western Australian Register of Developmental Anomalies (WARDA), Western Australian Department of Health, Perth, Australia Michelle A. Wedemeyer Department of Neurosurgery, University of Southern California, Keck School of Medicine, Los Angeles, CA, USA N. Wesslén Department of Neuroscience, Neurosurgery, University Hospital, Uppsala, Sweden L. Westbom Department of Pediatrics, University Hospital, Lund, Sweden Annette M. Willgens Temple University, Philadelphia, PA, USA

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Jenny L. Wilson Shriners Hospitals for Children, Portland, OE, USA Pediatric Neurology, Oregon Health and Science University, Portland, OR, USA Aviva L. Wolff Department of Rehabilitation, Hospital for Special Surgery, New York, NY, USA Henry Wright Department of Physical Therapy, University of Delaware, Newark, DE, USA Chuan Zhang Department of Kinesiology, University of Georgia, Athens, GA, USA Ruth Ziegler Department of Orthopedics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA

Contributors

Part I Diagnosis and Pathology

1

The Child, the Parent, and the Goal in Treating Cerebral Palsy Freeman Miller

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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How Different Is the Child with CP? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Family Impacts of the Child with CP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Care-Providing Community . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Cerebral Palsy Clinic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Family Care Provider and Professional Care Provider Relationship . . . . . . . . . . . . . . .

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Family Response Patterns . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Dealing with Blame . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Giving and Dealing with Prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 Giving the Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 Medical Therapeutic Relationship to Child and Family . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 The Physical Therapist Relationship . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 When the Doctor–Family Relationship Is Not Working . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 When the Family Chooses Medical Treatment Against the Physician’s Advice . . . 12 Recommending Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 A Plan for Managing Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14 When Complications Occur . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 The Final Goal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19

Keywords F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_194

Cerebral palsy · Physician · Parent · Child · Complication · Diagnosis · Prognosis

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Introduction Cerebral palsy (CP) is a childhood condition in which there is a motor disability (palsy) caused by a static, nonprogressive lesion in the brain (cerebral). The definition from a scientific perspective was last updated in 2017. Cerebral palsy describes a group of permanent disorders of the development of movement and posture causing activity limitation that are attributed to nonprogressive disturbances that occur in the developing fetal and infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, behavior, by epilepsy, and by secondary musculoskeletal problems (Rosenbaum et al. 2007). The causative event has to occur in early childhood, usually defined as less than 2 years of age. Children with CP have a condition that is stable and nonprogressive; therefore, they are in most ways normal children with special needs. Understanding the medical and anatomic problems in individuals with CP is important; however, it is also important to always keep in mind the greater long-term goal, which is similar to that for all normal children. The goal for these children, their families, medical care, education, and society at large is for them to grow and develop to their maximum personal capabilities so that they may succeed as contributing members of society. This goal is especially important to keep in perspective during the more medical and anatomically detailed concerns discussed in the remainder of this text. The goal of this chapter is to consider some aspects of the complex interaction between the family, child, and medical care provider in the context of long-term medical treatment requirements.

How Different Is the Child with CP? When addressing each of the specific anatomic concerns, the significance of these anatomic problems relative to the whole child’s success needs to be kept in the proper context. The problems of children with CP should be evaluated in the

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perspective of normal growth and development similar to any normal children with an illness, such as an ear infection, who need medical treatment. However, keeping the specific problems of children with CP in the proper context is not always easy. The significance of this proper context is somewhat similar to the significance of having a child doing spelling homework on Wednesday evening to pass a spelling examination on Thursday. Likewise, practicing the piano is necessary to succeed in the piano recital. Even though each of these acts is important toward the final goal of having a confident, educated, and self-directed young adult who is making a contribution in society, the exact outcome of each event may not be all that important in the overall goal. Often, the success of a minor goal such as doing well on a specific test is less important than a major failure, but the measure of failure or success may be hard to recognize until years later. As with many childhood events, the long-term effect may be determined more by how the event was handled than by the specific outcome of the event. Children with CP experience their unique CP treatment, in addition to all the typical childhood experiences. Different children may experience events, such as surgery and ongoing physical and occupational therapy, very differently. The long-term impact of these events from the children’s perspectives is often either negative or positive, depending on their relationship with both therapists and physicians. These children may have cognitive, behavior, communication, and physical problems, which are the major focus of this text; however, CP affects the whole family and community. The process of growing and developing involves many factors. One of the most important factors in children’s long-term success is a family caretaker. Likewise, for children with CP, families may be impacted by the CP as much as the children with the physical problems. It is very important for medical care providers to see the problems related to CP as not only involving the children but also involving the families. Society is understanding that the education of normal children works best when the family care providers

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The Child, the Parent, and the Goal in Treating Cerebral Palsy

actively participate. Likewise, providing medical care for children with CP must consider their whole families. The outcome for these children will be determined largely by their families, just as the success of normal children’s education is impacted by their families. The importance of family does not provide an excuse for medical care providers or educators to become pessimistic if they do not perceive the family is doing its part. In this circumstance, professional care providers still must give as much as possible to each child but recognize their place and limits in the care of these children. Medical care providers who fail to recognize their own limits in the ability to provide care often will become overwhelmed by their sense of failure and will burn out quickly.

Family Impacts of the Child with CP A healthy liaison should be developed between children with CP, the family unit, and the medical care providers. Cerebral palsy is a condition that varies extremely from very mild motor effects to very severe motor disabilities with many comorbidities. In addition, there are great variations among families. To provide proper care for children with CP, physicians need to have some understanding of the family structure in which the children are living. Because of time pressures, this insight is often difficult to develop. Families vary from young, teenage mothers who may have the support of their families to single-parent families and to families with two wage earners and other children. All the pressures of caring for a child with a disability are added onto the other pressures that families of normal children have. Because most children with CP develop problems in infancy and early childhood, families grow and develop within the context of these disabilities. Often, the father and mother will react differently or come to different levels of acceptance. It is our impression that these different reactions may cause marital stress leading to high levels of divorce, most frequently when the children are 1–4 years old. Although this is our impression, there is no clear objective evidence that the

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divorce rate for these families is higher than in the normal population. Another high time of family stress is during the teenage or young adult years for those individuals with severe motor disabilities. Often, as these individuals are growing to full adult size and the parents are aging, it becomes very apparent to the parents that this is not a problem that is going away nor are these young individuals capable of going off to college and making a life of their own. The response of an individual family varies greatly with the wide variability of severity of CP. Many families develop a stable and very supportive structure for their disabled child. Physicians and other medical care providers may be amazed at how well these families deal with very complex medical problems. For many of these families, however, the medical complexities have accumulated slowly and are themselves a part of the growth and development phenomena. With multiple medical treatments often provided by many different medical specialists, a high level of stress develops in almost every family. It is helpful for the family to have some caregiver support and help in dealing with the stigma of having a child with CP (Ansari et al. 2016). For the medical professional, continuing to be aware of this stress and listening for it during contact with families are important. Families with less education and limited financial resources may do remarkably well, whereas a family with more education and more financial resources may not be able to cope with the stresses of a child with a severe disability. It is extremely difficult to judge which family can manage and which family will develop difficulty, so it is important not to become prejudiced either for or against specific families. Medical care providers should continue to be sensitive to how the family unit is managing to deal with their stresses (Bartlett et al. 2017). Some families will be seen to be doing well and then suddenly will become overwhelmed in the face of other family stress. This stress may be illness in other family members, financial pressures, job changes, marital stress, and, most commonly, the effects of aging on the parents, siblings, and individuals with CP (Bemister et al. 2014).

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Care-Providing Community Children with CP develop in supporting communities, which vary with each individual child. There are four general segments of these caring communities, with the family or direct caregivers being the primary relationship. This primary relationship is surrounded by community support services, the medical care system, and the educational system (Fig. 1). The community support includes many options such as church, scouting, camping, respite services, and recreational programs. The educational system includes both educational professionals and therapeutic professionals, especially physical and occupational therapists. The focus of this larger text is to address

Fig. 1 A large and extensive care team surrounds the family with a child who has cerebral palsy. These care providers are roughly organized around the educational system, primary medical care provider, the cerebral palsy

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primarily the medical issues, so there will be no specific in-depth discussion of these support services, except to remind medical professionals that other services provide crucial roles in the lives of children and their families. The organization of the medical care system tends to organize around the general medical care and the specialty care for the problems specific to CP. It is very important for families to have an established general medical care provider, either a pediatrician or family practice physician. Families must be encouraged to maintain regular follow-up with a primary care physician because very few orthopedists or other specialists have the training or time to provide the full general medical care needs of these children. Standard

specialized medical team, and community support services. Significant overlap and good communication provide the best resources to the child and the family

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The Child, the Parent, and the Goal in Treating Cerebral Palsy

immunizations and well child care examinations especially may be overlooked. However, most families see their child’s most apparent problem as the visible motor disability and will focus more medical attention on this disability at the risk of overlooking routine well child care. The physician managing the motor disability should remind parents of the importance of well child care by inquiring if the child has had a routine physical examination and up-to-date immunizations. A physical or occupational therapist will provide most of the medical professional special care needs related to the CP. The specialty medical care needs are provided in a specialty clinic, usually associated with a children’s hospital.

Cerebral Palsy Clinic Another way to organize the management of these well child care needs is with a multidisciplinary clinic in which a primary care pediatrician is present. The administrative structure for setting up a clinic to care for children with CP is not as well defined as it is for diseases such as spina bifida. Spina bifida, meningomyelocele, or spinal dysfunction clinics are all well-established concepts and are present in most major pediatric hospitals. These clinics, which are set up to manage children with spinal cord dysfunction, have a well-defined multidisciplinary team. This team works very well for these children because they all have similar multidisciplinary needs ranging from neurosurgery to orthopedics, urology, and rehabilitation (Brochard et al. 2017). However, this model does not work as well for children with CP because their needs vary greatly. These needs range from a child with hemiplegia who is being monitored for a mild gastrocnemius contracture only to a child who is ventilator dependent with severe osteoporosis, spasticity, seizures, and gastrointestinal problems. It is impossible to have all medical specialists available in a clinic setting, especially in today’s environment where everyone has to account for their time by doing productive work, described mainly as billable time. There are two models currently being used in most pediatric centers for the care of children with

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CP. One model has a core group of clinicians who see the children, often including an orthopedist, pediatrician, physiatrist, social worker, physical therapist, and orthotist. The second model consists of families making separate appointments for each required specialist. The advantage of the first model is that it helps families coordinate their child’s needs. The major disadvantage is that it is costly and not reimbursed by the fragmented American healthcare system. Many families find this model to not be time efficient as they often have duplicated services. The advantage of the second system is its efficiency to families and healthcare providers; however, there is often less communication between healthcare providers, and the responsibility of coordinating care from many different specialists tends to fall more to families. From a practical perspective, considering the cost restriction of the healthcare environment, the best system is some blending of the two clinic models. We use this blended model, and it works for many patients with CP and their families. We schedule outpatient clinics where an orthopedist and pediatrician share the same physical office space; however, each child is given an individual appointment with each physician. If there are only musculoskeletal concerns, only the orthopedist is scheduled to see the child. However, if a child also has additional medical needs, the pediatrician is seen before or after the orthopedic appointment. Orthotics, rehabilitation engineering for wheelchair services, nutritionists, social workers, and physical and occupational therapy are available in very close proximity to this outpatient clinic. If a child had a recognized problem before the clinic visit, appointments are coordinated to see other specialists. However, if the problem is found at the current visit, such as an orthosis that is too small, this child can be sent to the orthotist and be molded on the same day for a new orthotic. This clinic also has a special coordinator to help parents schedule appointments with other specialists such as dentistry, gastroenterology, or neurology. This structure is most efficient for medical care providers; avoids duplication of services, such as having a physical therapist evaluate a child who is getting ongoing community-based therapy; and

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can potentially provide maximal efficient use of the parents’ time. The main problem arising with this system is that it requires cooperation between many areas in the hospital. This model only works if the needed specialists are all working on the same day and are willing to work around each other’s schedules. For example, holding the CP clinic on a day that the dental clinic is closed or the orthotist is not available does not work. Although individual appointments are made with specialists, schedules often are not maintained perfectly, so if the orthopedic appointment is for 10 a.m. but the child is not seen until 11 a.m., the time of the next appointment with a neurologist, all the schedules are affected. Making this system work requires flexibility by all involved. One area of efficiency that the medical care system pays little attention to is the parents or caretaker’s time. Most caretakers have to schedule a whole day to take a child to a physician appointment because it means taking the child out of the school, usually driving some distance, seeing the physician, and then returning home. This system of actively trying to schedule a number of appointments on the same day allows parents to make use of the whole day, avoiding more days out of work for the parent and out of school days for the child. Coordination between team members is accomplished by weekly team meetings where outpatient children with specific needs, along with pending and present in-hospital patients, are discussed. No matter what administrative structure is used for the outpatient management of children with CP, because of the diverse population and needs, there are always individuals who will not fit the structure. Therefore, an important aspect of providing medical care to this patient population is to have flexibility in the delivery system.

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relationship may be somewhat different for educational professionals than for medical care professionals. This discussion focuses primarily on the medical care professional relationship, specifically on the care of the motor disabilities provided by a physician. The first aspect of treating children with CP is ensuring that the families have heard and come to some level of acceptance that their child has a problem called CP, which is permanent and will not go away. Hearing and acknowledging a diagnosis is a process that requires families first to come to terms with hearing the words and, second, to internalize these words. This process may take many years, with families initially acknowledging that there is a problem, but still expecting a cure soon. In the initial session with families to discuss this diagnosis, it is important that physicians allow plenty of time to answer all their questions, do not demand that they immediately accept the physicians’ words, and avoid definitive words that bring a sense of hopelessness to families. During this discussion with families, there is little role for the use of absolutist terms like “never,” “will not,” “cannot,” “will die,” or “will never amount to anything.” These terms often strike families as extremely cruel and threaten to remove all their hope, which they desperately need. Having time to answer all the family’s questions and allowing them to have their own doubts is important. As the physician relationship develops with a family, especially in the context of a clinic for CP, the families will slowly come to their own realization. However, this process of coming to terms with the diagnosis may be impacted by the circumstances and situations surrounding the etiology.

Family Response Patterns Family Care Provider and Professional Care Provider Relationship The specific organizational model for providing care is not as important as the fact that the medical care provided to the child with CP must always be provided to the family–child unit. This

All families come to terms with their children’s problems in their own way; however, there are several problems that are based on mechanisms surrounding the inciting event or the time of the diagnosis. In general, most families struggle to understand why this happened to their children and who is at fault.

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Obstetric difficulties surrounding delivery can be the clear cause of CP. However, many of these birthing problems are probably due to a fetus that was already sick. Nevertheless, the birthing problems often focus the parents on looking for someone to blame, frequently the obstetrician. Some families can come to the point where they can release this need to blame; for others, it may lead to finding a legal solution by way of bringing a legal suit against the individual or organization perceived to be at fault. These legal pursuits are often encouraged by lawyers, and for many families, this only leads to more disappointment when some of the legal efforts are unsuccessful. For families who win legal judgments, there may be some sense of justice; however, the difficulty of caring for a child with a disability continues, and the need to come to terms with why this happened does not disappear by receiving money from a successful lawsuit. Some parents, who have difficulty dealing with why this happened to their child, will be very suspicious of the medical system and will be perceived as being very difficult. There is a tendency for medical care providers, doctors, nurses, and therapists to avoid contact with these families, which often leads to more stress because the families feel that they are being avoided. This kind of very suspicious family, especially with underlying unresolved anger related to the initial diagnosis, needs to be kept exceptionally well informed and have frequent contact with the senior attending physician. When a child is hospitalized, it is important to have the attending physician meet with the family frequently and always keep them appraised of changes and expected treatment. This level of communication with families sounds very simple; however, we have seen many families who endured a series of terrible events in hospitals, such as oversights or staff failure to recognize an evolving event that the family already pointed out. When these situations are brought up with staff, such as nurses and residents, there is a tendency for the response to be “they brought it on themselves.” This kind of thinking is unacceptable because lack of contact with the senior responsible medical staff is usually the main cause.

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It is important for medical staff to recognize this pattern of behavior in families and respond very consciously by increasing communication and frequent contact. Again, the primary responsibility for this contact rests with the senior treating physician, who must display confidence, knowledge, and control of the situation to comfort the family. These families are very perceptive of physicians and care providers who do not have experience and confidence in dealing with their children’s problems. Often, these families have considerable experience in hospitals and notice when things are overlooked or symptoms are not addressed in an appropriate time (Case 1).

Dealing with Blame Medical care providers must not get into situations where they inadvertently inflame this need to blame someone for the cause of these children’s CP. When parents give their perception of the history of the inciting event, it should be accepted as such without comment. Medical care providers should not tell parents how terrible the person they blame was or anything else that gives the impression that the CP could have been avoided if only this or that were done. This kind of postmortem evaluation of past medical events helps medical practitioners to learn; however, a detailed dissection of long-gone medical events to look for a person to blame seldom helps the families to come to terms with their children’s disabilities. By far, most of these families’ “need to find someone to blame” is a stable enduring part of their lives, and if the treating physician acknowledges this need and focuses their concerns on the children’s current care and situation, the blame issue tends to fall to the background. There is no need for the orthopedic physician caring for these children’s motor disabilities to get an extensive history of the birth and delivery directed at understanding the etiology of the CP from the families, so long as the diagnosis of CP is appropriate. Instead, the families’ mental energies should be directed at the goal, which is to help their children be all they can be, given the current circumstances. However, trying to convince the

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parents that they have to give up looking for a cause or a person to blame is also futile. If the parents are totally immobilized and cannot move forward, arranging psychotherapy may be worthwhile; however, most parents will perceive this as another attempt to sweep away the problem of who is responsible. Another common scenario for the diagnosis of CP is when a parent or grandparent recognizes some slow development in a child. This child was then taken to see the family doctor or pediatrician who reassured the family that they were overreacting. Often, these families end up going to their primary care provider two, three, or four times to hear the same response, that is, that they are just overreacting. The child is a little slow, but there is nothing to worry about. These families often want to lay the blame for the CP upon the physician, believing that this delayed diagnosis is why the child currently is so severe. There is almost no circumstance where a delayed diagnosis will be of any significance. It is important for these parents to have their concerns about the delayed diagnosis acknowledged, but then they must be reassured that this delay did not, in any way, cause their child to have a greater severity of CP. Some of these families will have difficulty developing other trusting relationships with physicians and may call, especially initially, for many minor concerns until confidence in their physician is developed. Sometimes CP is the result of an accident or event in childhood, such as a toddler with a near drowning, or a child with a closed head injury from a motor vehicle accident in which the parent was the driver. In these situations, the parents often feel a substantial amount of blame for causing their child’s disability. This self-blame and guilt may be even more difficult for a parent to come to terms with than blame focused outward. One response to the inwardly focused blame is to search for extraordinary cures, demand more therapy, or get more devices. This behavior seems to be one of “making it up to the child.” It is helpful to reassure the family that things besides more therapy or more devices, such as maximizing the child’s educational ability, will help the child.

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Giving and Dealing with Prognosis Another experience frequently reported by parents whose children were in neonatal nurseries is the comment that the children probably will not survive and, if they do, will be vegetables. This comment has been reported to us by parents of children who end up with hemiplegia as well as children with quadriplegia. We believe this comment stems from the great difficulty of making a specific prognosis of outcome in the neonatal period. Also, some physicians tell families the worst possible outcome, believing that when the children do better, the families will be grateful for their good luck. However, this explanation almost never has the intended outcome, and much more commonly the families perceive these comments as the physician being incompetent or deceitful. Often, these families will interpret attempts by later physicians to discuss prognosis or expected results of surgery as being too pessimistic. For these families, it is important to be as realistic as possible; however, their optimism may cause some disappointment as their expectations of greater outcomes are not realized. Generally, these families do come to appropriate expectations but continue to have some negative feelings about their neonatal experience. An important aspect of giving prognosis or information that is requested by families is to always acknowledge that it is imperfect. Requests to know if a child will walk or sit should be answered as honestly as possible, always avoiding absolutist terms such as “never,” “cannot,” or “will not.”

Giving the Diagnosis Another common problem surrounding diagnosis of children with CP is failure to give the parents a diagnosis. A common example of this is a mother of a 5-year-old who is unable to sit and brings the child to see the orthopedist to find out why the child cannot walk. The history reveals a normal pregnancy and delivery; however, by age 12 months, the child was not sitting,

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The Child, the Parent, and the Goal in Treating Cerebral Palsy

so the mother started going to doctors to find out what was wrong with the child. She has seen three neurologists and a geneticist, has had skin biopsies, muscle biopsies, computed tomography (CT) scan, magnetic resonance imaging (MRI) scan, and many blood tests, but everything is normal. The mother hears from these doctors that they can find nothing wrong with her child; however, what the doctors probably told the mother is that the medical tests are normal and they do not know what caused the child’s current disability. Families need to be told what is wrong with their child. This type of family is easily helped by explaining that the child has CP. Physicians should clearly explain that even though they do not understand why the child has CP, it is the diagnosis, which they know exactly how to treat. Taking time and providing information to these families will stop the endless and futile search for “why” and allow them to focus on caring for and treating their children. This situation is caused almost entirely by physicians not being clear in communication with parents and the particular aversion by some physicians to giving a diagnosis of CP. This aversion is very similar to wanting to avoid telling a patient that she has cancer and therefore telling her that she has a non-benign growth whose cause cannot be explained. In this way, CP is like cancer in that a physician often cannot determine the etiology; however, the treatment options are well defined and should be started immediately.

Medical Therapeutic Relationship to Child and Family There are many different types of therapeutic relationships that work for families and their children; however, there are some patterns that work better than others. These patterns each have their risks and benefits as well. The major therapeutic relationships in the treatment of motor problems of children with CP include the parents, the physical therapists, and the physicians. The parents will spend the most time with their children and will

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know them best. Often, the parents recognize developmental gains and day-to-day variability in their child’s function first. Physical therapists will spend the most therapeutic time during treatment with children and will bring the experience of similar children. This in-depth experience with similar children allows therapists to help parents understand the expected changes as well as teach parents and children how to maximize their function. The orthopedist treating the motor disability will have the least experience with an individual child but will have the broadest experience with many children to understand the expectations of what will occur. The physician’s experience with each child, however, will be much more superficial, and the physician depends on the parents’ and therapists’ observations of the children’s function over time and the variability of function during the day. Recognizing these individual strengths will allow the parents’, therapists’, and orthopedists’ perception of individual children to be combined to make the best therapeutic judgment.

The Physical Therapist Relationship The role of the primary treating physical therapist, especially for the young child between the ages of 1 and 5 years, will incorporate the typical role that the grandmother and the general pediatrician play for normal children. In addition, the therapist fulfilling this role must have knowledge and experience in dealing with children with CP. This role model involves time spent teaching the parents how to handle and do exercises with their child. This role also involves helping the parents sort out different physician recommendations, encouraging the parents, and showing and reminding parents of the positive signs of progress in the child’s development. When this role works well, it is the best therapeutic relationship a family has. The positive aspects of this role are providing the parents with insight and expectations of their child, reassuring the family that they are providing excellent care and being readily available to answer the family’s questions.

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The “grandmothering” role of the therapist has associated risks. One of the greatest risks in our current, very unstable medical environment is that a change in funding or insurance coverage may abruptly end the relationship. An abrupt change can be very traumatic to a family. The therapist must be careful not to be overly demanding of the family but to help the family find what works for them. Occasionally, a therapist may be fixated on a specific treatment program and believe that it is best for the child; however, the parents may not be in a situation to follow through with all this treatment. The parents feel guilty, and the therapist may try to use this guilt to get them to do more. The physical therapist in this role as a therapeutic “grandmother” can help parents sort out what medical care and choices are available. The therapist can help parents by attending physician appointments and making the parent ask the right questions, which is often not possible because of funding restrictions. The physical therapist must not give specific medical advice beyond helping parents get the correct information. Therapists with extensive experience should recognize that they have great, detailed, and deep experience with a few children and that generalizing from the experience of one child is dangerous. We have heard therapists tell parents on many occasions that their child should never have a certain operation because the therapist once saw a child who did poorly with that surgery. This type of advice is inappropriate because one child’s experience may have been a rare complication of the operation. Also, there are many different ways of doing surgery. This would be like telling someone to never get in a car again after seeing a car accident. A more appropriate response to the family would be giving them questions to ask the doctor specifically about the circumstance with which the therapist is concerned and has experience. Another physical therapist therapeutic relationship pattern is the purely clinical relationship in which the therapist thinks the family is incompetent, unreliable, or irresponsible and only wants to deal with the child. Almost

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invariably, this same therapist next will complain that the family and child never do the home exercise program or that the child is not brought to therapy regularly. This relationship may work for a school-based therapist or a therapist doing inpatient therapy, but it leads to great frustration for both the therapist and family when it is applied to an outpatient-based, ongoing developmental therapy. In this environment, the therapist must try to understand and work within the family’s available resources.

When the Doctor–Family Relationship Is Not Working Medical care providers need to understand that personalities are such that one individual can never meet everyone’s needs. This does not mean that as soon as the doctor–family relationship becomes difficult, it is not working. At this time, the relationship needs to be discussed, and the physician should be open about giving the family permission to go to another doctor. Some families will just leave without saying anything and others will feel guilty about wanting to leave. Physicians must be honest with themselves because this situation tends to make a physician feel like a failure. There may be a combined sense of relief that the family left and a sense of failure and anger that the family does not trust their physician. These are normal feelings that the physician should acknowledge and not place blame on themselves or the family.

When the Family Chooses Medical Treatment Against the Physician’s Advice Families may seek a second opinion for a specific treatment recommendation. This desire to get a second opinion should not be seen by the primary treating physician as a lack of faith or confidence. The family may require a second opinion for insurance purposes or, for many families, they just want to make sure they are

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The Child, the Parent, and the Goal in Treating Cerebral Palsy

getting the correct treatment. Usually, getting a second opinion should be viewed as a very prudent move on the family’s part and should be encouraged. Families should be given all the records and support that are needed for them to get a meaningful second opinion. If this second opinion is similar to that given by the primary physician, the family is often greatly comforted in moving ahead. However, there is still variability in medical treatment for children with CP, so depending on the family’s choice of opinions, the recommendations may be slightly to diametrically opposed. In a circumstance where the recommendation of another physician differs significantly, the primary physician must be clear with the family and place the second opinion in the perspective of their recommendation. Sometimes the words used may sound very different, but the recommendations are very similar. In other circumstances, the recommendation may be diametrically opposed, and the primary physician must recognize this and explain to the family the reasons for their recommendation. When recommendations are diametrically opposed, clear documentation, including the discussions concerning the other opinion, is especially important. This situation has a high risk for disappointment. Often, families have great difficulty in choosing between divergent opinions, even when one opinion is based on published scientific data and the other opinion is completely lacking in any scientific basis. Therefore, a family may base their decision on other family contacts, a therapist’s recommendations, or the personality of the physician. Physicians must understand that it is the family’s responsibility and power to make these choices; therefore, with rare exception, no matter how medically wrong the physician believes these decisions are, the family must be given the right to choose. Only in rare, directly life-threatening circumstances will a child protective service agency even consider getting involved, and then this involvement is usually very temporary. With a long and chronic condition such as CP, temporary intervention by a child protective agency generally is of no use in interacting with families. With

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clear documentation of the recommendations, the physician must let the family proceed as they choose; however, we always tell them that we would be happy to see them back at any time. When they undergo treatment against their primary physician’s advice and return, usually after several years, the physician should not make the previous situation a conflict. The family usually feels guilty and may not want to discuss past events. Occasionally, they will come back and blame the physician for the problems because they have transferred the blame for the recommendation. Nothing will be gained by bringing up these past problems with the family, and the focus should be to move on with the problems at hand as they present themselves.

Recommending Surgery For children who have had regular appropriate medical care, the need for specific orthopedic procedures is usually anticipated over 1–2 years and as a consequence is not a surprising recommendation. We prefer to have these discussions in the presence of the child. For young children, there is no sense that something is being hidden from them. Children in middle childhood and young adulthood can take in as much as possible, allowing us, as their physicians, to directly address their concerns as well. For younger children, those under age 8 years, their main concern is that they will be left alone. We reassure them that we make a major effort to allow the parents to stay with them during preinduction in the surgical suite and again in the recovery room. We also reassure children that their parents will be with them throughout the whole hospitalization. As children get older, especially at adolescence, there is often an adult type of concern about not waking up from anesthesia or having other severe complications leading to death. These individuals may have great anxiety but have few of the adult coping skills that allow the rationality to say that this surgery is done every day and people do wake up. Some of these adolescents need a great deal of reassurance, most of which should be directed at

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trying to get them to use adult rational coping skills. If adolescents are having problems with sleeping or anxiety attacks as the surgery date approaches, treating them with an antianxiety or sedative agent is very helpful. Some adolescents and young adults with cognitive limitations develop substantial agitation over surgery. Parents of such children are usually very aware of this tendency and may wish to not tell them about having surgery until the day before or the day of surgery. Although this is a reasonable practice for individuals with severe mental disability who are not able to cognitively process the planned surgery, approaching children who are cognitively able to process the event in this way is only going to make them distrustful of their parents and doctors. In preparing children and families for surgery, it is important to discuss the expected outcome of the surgery with them. Part of this discussion must focus on what will not happen, specifically that their child will still have CP after the surgery. If the goal is to prevent or treat hip dislocation, showing radiographs to the families helps them understand the plan. They also need to be told what to expect of the procedure from a functional perspective, such as “Will the child still be able to stand? Will the child be able to roll? Will the child’s sitting be affected? Will the child’s walking ability be affected?” For children in whom the surgery is expected to improve walking, showing families videotapes of similar children before and after surgery helps them get a perception of what level of improvement is anticipated.

A Plan for Managing Complications Discussion of possible complications is also important; however, the expected outcome should be honestly approached. Some surgeons tend to have very pessimistic expectations with regard to expected outcome and complications. Surgeons with this approach soon overwhelm themselves and their families with their assessment of the poor balance between the expected outcome and the possible complications. Most surgeons who

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have a large CP practice tend more toward the overly optimistic approach in which the outcomes clearly will be worth the risk of the complications. The risk of an overly optimistic approach to families occurs when there are complications. These families may be surprised and angry and find it difficult to deal with the unexpected. It is difficult for physicians to have the perfect balance, but each physician should be aware of their own tendency. Usually, an honest assessment and feedback from partners will identify which personality trait, either optimistic or pessimistic, a physician tends to use when approaching families. By recognizing this tendency, surgeons can be more sensitive to what families are hearing and make suggestions to moderate this perception. There are families who for some reason or another have not been obtaining appropriate medical care for their children. Then, when these children are adolescents, they may come to see a CP surgeon with a painful hip dislocation, severe scoliosis, or other deformities that are in a severely neglected state. Some of these families are surprised to hear that only a surgical procedure will be the appropriate treatment. Some families may be very resistant to surgery and will want to try everything else. These families must understand that only surgery will correct the problem, but the surgery seldom has to occur on an emergency basis. If a surgeon perceives a family’s hesitancy and attempts to mollify them by suggesting that a brace, injections, or some other modality be tried even though it will provide no long-term benefit, the family will likely hear uncertainty in the physician’s approach. Families may miss the message completely that only surgery will address the problem when they are appeased by nonsurgical treatment. Giving children temporizing measures to provide relief of pain is appropriate; however, doctors must be clear to families that these measures are only providing temporary pain relief and are not treatments. By giving families a little time with the use of these temporary measures, physicians can develop a relationship with the families. There are situations where medical and psychiatric treatment may be required before the surgical treatment can occur. For all these reasons, it is

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important to be clear about the required treatment, its expected outcomes, and then to outline the full treatment plan. As this treatment plan is undertaken, the relationship a physician has developed with children and families will allow them to be confident that the recommended treatment can occur in a safe and effective way.

When Complications Occur When treatment of a child does not go well, the physician must first recognize this as a complication. The judgment of recognizing a complication is one of the most difficult to develop, and some physicians may never do it well. Many complications, especially in orthopedics, do not present with the drama of a cardiac arrest. In orthopedics, a more typical example is the presentation of a deep wound infection. Every wound with a little erythema and a mild superficial drainage is not a deep wound infection. However, when a deep wound infection is present, it should be acknowledged as such. These families should be told of the complication, and a definitive treatment plan should be described. For this process to work, physicians first have to acknowledge the complication to themselves. We have seen many physicians who cannot bring themselves to acknowledge the magnitude of the complication. Likewise, we have seen physicians who overreact to relatively minor problems that will resolve if left alone. Finding a balance requires physicians to be honest with themselves and be aware of their own tendency toward optimistic or pessimistic ends of the spectrum. The optimist tends to see the complication as minor variance of normal, whereas the pessimist tends to be overly concerned that any wound change may be a deep wound infection. By being aware of one’s own tendency, as experience is gained, an approach to diagnosing and acknowledging complications and then making specific treatment plans will be developed. Complications tend to make physicians feel like failures, and a good retrospective evaluation of the treatment course may demonstrate errors of judgment or execution. These errors

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should be viewed as learning experiences and opportunities to teach oneself as well as others. A significant number of the case histories in this book are careful analyses of complications that have occurred in our practice. It is important that the approach to analyzing a complication is to determine the exact cause of the complication when possible so that it may be avoided in the future. Saying that “I will never do that operation again” is an inappropriate response to complications. This response comes very close to that of people who say they will never get in a car again after they have had a car accident. Our goal is to always have a complication-free treatment and recovery for every patient; however, we learn the most from careful analysis of our complications and poor outcomes. Once physicians acknowledge the complications to themselves, the families then need to be told. Families may react with quiet acceptance, frustration, or anger. These feelings are often the same feelings that physicians have about the same complication. If physicians are willing to share some of their frustration and concern about the complications, it often helps families to put the problem in perspective. It is very important to explain to families what to expect from a complication. This explanation should include a detailed outline of the expected treatment plan. If a complication arises that physicians are not comfortable treating, getting a second opinion from, or seeking the help of, another physician is very important. This step should be explained carefully to families. Frequent contact with families is very important, especially if they develop considerable anger and anxiety, because if they feel that the doctor is trying to avoid them, these feelings often increase. Complications should be managed very much like the initial decision to have an operation. First, specific problems should be carefully defined to families. Next, the range of options and expected outcomes, with respect to the short- and long-term implications, should be placed forward as specifically as possible. As much as possible, families should be told the detailed expected timeline and exact treatments. For instance, if repeat or additional surgery is expected in the future as a

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consequence of a complication, this should be laid out for families. If antibiotics are to be used, families should be told for how long and what factors will be monitored to determine a good outcome. This kind of detail gives families a sense that there is someone in charge with experience in dealing with these complications and helps them deal with the fear of the unknown, which the complications often bring to the foreground. Complications need to be recorded in detail in the medical record and should reflect all the objective observations and alternatives that were considered. This record is not the place where blame should be directed. What is observed to have occurred should be documented objectively without rewriting history. For example, if the toes are found to be insensate and without blood flow in a child who has had a cast on a foot following surgery, this should be reflected in the medical record, followed by a recording of the immediate action taken, such as removing or opening the cast, and the outcome of that action, such as the improved and returned blood flow to the toes. There is no reason to speculate that the cast was applied too tightly, or that the nursing staff failed to elevate the cast, and so forth. This kind of analysis is important but should be done after the patient is treated appropriately, and there has been time to reflect on the whole situation. Often, these initial assessments are incomplete and wrong and most frequently are written to protect the writer. Later, during a more thorough investigation or legal action, these assessments only make it appear as if the writer was trying to cover up or shift blame to someone else. During stressful treatment periods, especially when dealing with difficult complications, it is very important to ask partners and other colleagues to evaluate the patients and give unbiased opinions. A treating physician can develop a biased view, especially in the face of complications where one would not like to acknowledge personal culpability. Involving other colleagues also gives families the sense that their physician really is trying to keep all options open. If these consultants do have different opinions, these opinions should be discussed between the physicians

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first, and then the options should be outlined for families with a unified recommendation wherever possible. Giving families different treatment recommendations and expected treatment outcomes from several different consultants should be avoided.

The Final Goal The goal in treating children with CP is for them to grow and develop within the context of a normal family. Their medical treatment and medical condition should be an experience just as a normal part of who they are. For example, a 6-year-old child who fractures her femur will have a 6-month treatment course until most of the rehabilitation is completed. This occurrence will remain a definite event in the child and family’s growth and development; however, when she is graduating from high school and going off to college, this medical event probably will have faded into many other growing-up experiences. This is the pattern that we want to try to mimic in children with CP. In the past, children might have spent 30–50% of their growing-up years in hospitals having and recovering from surgeries trying to make them walk better or to make them straighter, which was very detrimental. Mercer Rang termed this the “birthday syndrome,” in which children were in the hospital for most of their birthdays and nurses were baking their birthday cakes and having birthday parties for them rather than their families at home (Rang 1990). Many of these children came to see the hospital staff as a second family (Fig. 2). This seldom happens currently because of greatly shortened hospital stays and improved diagnostic studies. For most children with CP, all orthopedic management should ideally be done with only two major surgical events during their growth and development. This ideal is not possible to achieve in all children but should continue to be the goal. Striving for decreasing the number of orthopedic operative events in children’s lives and moderating the amount of other medical treatments to only those that will have definite and lasting benefit should be continued. For example, an ambulatory child with normal

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Fig. 2 The typical approach to the surgical treatment of children with CP was to perform a surgery almost every year. This concept often led to children spending a great deal of time in the hospital, to the point where the nursing

staff would become “pseudoparents,” more often celebrating birthdays with the children than the children’s own families

cognitive function should not be having physical or occupational therapy at any time that interferes with their education. Therapeutic goals should be planned during summer months or in ways that do not interfere with education. Twenty years ago, the use of inhibition casting was popular. It was believed that this technique decreased contractures and managed spasticity. These children were in leg casts for 8 weeks, often requiring trips to the clinic to change the cast every 2 weeks. After 2 or 3 months, the whole process would have to be repeated. If families could tolerate the stress, although few did, these children would be in a cast for 30–50% of their growing years. The time and behavioral stress placed on these families meant that a large part of their lives revolved around their children’s medical treatments. When these children graduated from high school, they tended to see all these casting events as a major focus of their growingup experience instead of the more normal

childhood growing experiences, such as going to the beach, going to Disney World, or other parties and events. In young adulthood, the success of the whole individual with CP is determined much more by the family and the individual’s educational experience than by the activities of the medical treatment. The medical care system can help children and families cope with the disability and allow individuals with CP to function at their maximum ability. However, the medical care system also must recognize that too much focus on perfection of function may cause damage to the growth and development of the children and family unit, especially in the social, psychologic, and educational domains. Achieving this balance varies with each child and family. For example, many successful young adults without disabilities do not have the ideal maximization of their physical function because the focus of their interests is sedentary activities. Just as with

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these nondisabled young adults, there is great variation in how important maximizing physical function and appearance is to each individual with CP. When young adults are truly able to make informed and well-articulated decisions, then they have arrived at a level of success in young adulthood. Just as with nondisabled adolescents and young adults, the medical care providers should stress the importance of good physical conditioning; however, trying to enforce a specific level of physical activity against the person’s wishes tends not to be very productive. Individuals with disabilities should be allowed to make these decisions in the same way that individuals without disabilities are allowed to decide, even if their physician thinks it is not in their best interest. Therefore, the final goal is to encourage the development of individual adults who are as competent as possible to make their own decisions, who develop the confidence to make those decisions, and are then willing to make decisions and live with the consequences. Always in the context of this final goal, we as orthopedic physicians want the individual’s physical impairments minimized as much as technically possible.

Cases

Case 1 Susan

Susan was born after a normal pregnancy and delivery at term and was discharged home from the hospital as a normal newborn. At 3 weeks of age, her grandmother thought that her head looked abnormal, and Susan was taken to a pediatrician where a workup revealed hydrocephalus. A shunt was placed at 4 weeks of age, followed by some complications. After this time, she was noted by her parents and grandmother to be less strong and less interactive. However, she did well, and by age 3 years was crawling, rolling, and talking. At age 3 years, she developed severe seizures and

was hospitalized. During this hospitalization, she had a rather severe overdose of antiseizure medication along with other subsequent complications and lost the ability to crawl, roll, and talk. Her parents started patterning therapy when she did not rapidly regain these functions. She also started to develop increased spasticity and had more trouble with her trunk control. By age 6 years, Susan had an adductor lengthening and was developing scoliosis. She was started in a body jacket to help control her scoliosis, and by age 8 years, she had a painful dislocated hip. After the family searched for several different opinions, they elected to go ahead and have the hip reconstructed. Because Susan had substantial complications with loss of neurologic function on several previous admissions, her parents were perceived as being extremely anxious during the hospitalization. The operative procedure and the recovery phase of the hip reconstruction went very well, and the family was very gracious. By age 9 years, she needed to have additional soft-tissue lengthenings of her right shoulder for a painful dislocation as well as for progressive varus deformity of the feet. The family was less anxious during this procedure than they had been with the prior procedure because they were more comfortable with the staff. By age 12, the scoliosis had progressed substantially, requiring a posterior spinal fusion. The family was very anxious about this very large procedure. Their anxiety was perceived by some staff as being over reactive; however, considering the history of their experience with past medical treatment, we felt it was appropriate. At the time of the posterior spinal fusion, the shunt tubing was noted to be broken; however, she was no longer dependent on her shunt so shunt repair was not performed. (continued)

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The Child, the Parent, and the Goal in Treating Cerebral Palsy

By age 13 years, she developed more lethargy and a shunt revision was recommended. During this shunt revision, she had severe complications including an infection that required the shunt to be externalized. The external drainage was not controlled carefully enough, and, as a consequence, the ventricles collapsed, causing intracranial bleeding. This episode caused substantial neurologic functional loss, so she was now less able to interact socially with her parents on top of her very severe spastic quadriplegic pattern motor disability. In addition, her seizures increased substantially. This episode made her parents extremely anxious about medical treatment, especially about the fear of developing complications and having functional loss. Shortly after the shunt problems, she was noted on routine medical examination to have a retinal detachment requiring surgery. This surgery occurred without any complications. She continued to have problems with her seizures, and her parents were anxious to have control of the seizures while at the same time to allow her to regain some of her alertness and contact with her parents, which they much enjoyed. This family was often perceived by nurses and house staff as being exceedingly difficult to deal with because they were so anxious and always wanted to observe and understand specific treatments and know exactly which medications were being administered. This family was extremely dedicated to the care of their daughter, and the anxieties that they expressed were very understandable considering their history. Often, medical care providers, especially

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physicians and nurses, were not aware of this history and therefore did not understand the parents’ anxieties. This anxiety tends to make nursing staff and medical staff try to avoid the parents, which just greatly increases their anxiety level. These parents had more than one hospitalization per year on average with their daughter and were very aware of what her proper medical management should be. They were very astute in picking up inexperience in both the nursing and medical staff and would become much more anxious when they sensed this inexperience or discomfort in dealing with their daughter.

References Ansari NJ, Dhongade RK, Lad PS, Borade A, Yg S, Yadav V, Mehetre A, Kulkarni R (2016) Study of parental perceptions on health & social needs of children with neuro-developmental disability and it’s impact on the family. J Clin Diagn Res 10(12): SC16–SC20 Bartlett D, Chiarello LA, Hjorngaard T, Sieck Taylor B (2017) Moving from parent “consultant” to parent “collaborator”: one pediatric research team’s experience. Disabil Rehabil 39(21):2228–2235 Bemister TB, Brooks BL, Dyck RH, Kirton A (2014) Parent and family impact of raising a child with perinatal stroke. BMC Pediatr 14:182 Brochard C, Peyronnet B, Dariel A, Menard H, Manunta A, Ropert A, Neunlist M, Bouguen G, Siproudhis L (2017) Bowel dysfunction related to spina bifida: keep it simple. Dis Colon Rectum 60(11):1209–1214 Rang M (1990) Cerebral palsy. In: Morrissy R (ed) Lovell and Winter’s pediatric orthopedics, vol 1. Lippincott, Philadelphia, pp 465–506 Rosenbaum P, Paneth N, Leviton A, Goldstein M, Bax M, Damiano D, Dan B, Jacobsson B (2007) A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl 109:8–14

Part II Etiology of Cerebral Palsy

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Cerebral Palsy and the Relationship to Prematurity Michael Favara, Jay Greenspan, and Zubair H. Aghai

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 Prevalence of Cerebral Palsy in Premature Infant . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 Etiologies of Cerebral Palsy Related to Prematurity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 Perinatal and Postnatal Interventions to Reduce the Risk of Cerebral Palsy in Preterm Infants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34

Abstract

Cerebral palsy (CP) is the most common cause of childhood-onset, lifelong physical disability in most countries around the world. Prematurity is the leading identifiable risk factor of cerebral palsy and is defined as birth occurring prior to 37 weeks’ gestation. Premature infants are at a much higher risk for developing CP than full-term infants, and the risk increases as gestational age and birthweight decreases. Despite technological advances in neonatal care over the past several decades, cerebral palsy remains a major neurologic sequela

M. Favara (*) · J. Greenspan · Z. H. Aghai Nemours/Thomas Jefferson University, Philadelphia, PA, USA e-mail: [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_1

among extremely low birth weight survivors, affecting 9–17% of survivors. Antenatal and perinatal factors that influence the risk of CP include gestational age at delivery, birth weight, presence of multiple gestation, and chorioamnionitis. Following delivery, there are a number of risk factors that contribute to a premature infant’s development of CP. These include intraventricular hemorrhage, periventricular leukomalacia, bronchopulmonary dysplasia, presence of a patent ductus arteriosus, necrotizing enterocolitis, hyperbilirubinemia, hypocarbia, neonatal sepsis, hypoxia, and apnea. Interventions to reduce the risk of CP include antenatal glucocorticoid administration, magnesium sulfate for neuroprotection, and delayed cord clamping at delivery.

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Keywords

Natural History

Cerebral palsy · Preterm infants · Etiology · Periventricular leukomalacia · Intraventricular hemorrhage

Prevalence of Cerebral Palsy in Premature Infant

Introduction Cerebral palsy (CP) is the most common cause of childhood-onset, lifelong physical disability in most countries around the world. It is defined as a disorder of motor behavior attributable to disturbances in the developing fetal or infant brain. It has a reported prevalence of 1.5–4 per 1000 live births. Prematurity is the leading identifiable risk factor for development of cerebral palsy. Premature delivery is a result of either premature labor, premature rupture of amniotic membranes, or maternal or fetal conditions that necessitate delivery. The incidence of premature birth in the United States is approximately 10% of all births, and this number increases around the developing world. Infants born at extreme preterm gestation are at risk for both death and disability. Premature birth exposes the fetus to extrauterine life before it is ready, and this exposure may be particularly harmful to the developing brain. While the survival rates have improved for this population over the past few decades, a significant improvement in overall neurodevelopmental outcome has not yet been realized. Although preterm infants have a higher incidence of all neurodevelopmental disabilities than full-term infants, most preterm infants are free of major disability. CP remains a major neurologic sequela among extremely low birth weight (birth weight < 1000 grams) survivors, affecting 9–17% of survivors (Vohr et al. 2000). Overall, approximately one fifth to one quarter of extremely premature infants who survive have at least one major disability – impaired mental development, CP, blindness, or deafness. Impaired mental development is the most prevalent disability with 17–21% of survivors affected, followed by cerebral palsy, with 12–15% of survivors being affected (Lorenz 2001).

Prematurity is a major contributor to perinatal morbidity in the United States and around the world. A normal human pregnancy is estimated to be 38–42 weeks from the first day of the mother’s last menstrual period, with 40 weeks being the average gestation. Premature birth is defined as birth occurring prior to 37 completed weeks from the first day of the last menstrual period. Late preterm births, occurring between 34 and 36 weeks of gestation, are the fastest growing subset of neonates, accounting for approximately 74% of all preterm births and about 8% of total births. The most common risk factor for premature birth is a prior history of premature birth, with an estimated 20–40% recurrence rate. Multiple gestation pregnancies are also associated with an increased risk of premature birth, with an estimated 30–50% of multiple gestations delivering prior to 37 weeks, with the higher order of multiple gestations delivering earliest. There are significant ethnic and socioeconomic differences in the United States that contribute to premature delivery, with African-Americans having twice the incidence of preterm births than Caucasians. Over the past several decades, advances in neonatal and perinatal medicine have shifted the limits of viability for prematurity from 28 weeks down to 22 weeks of gestation. As a result of progressive changes in both perinatal and neonatal management over the last six decades, there has been an increase in survival in the smallest and most fragile infants, leading to a change in the rates of neonatal morbidity, brain injury, chronic lung disease, and sepsis. Survival rates for infants born premature, both with and without significant disability, increases with each week of completed gestation. Survival for infants born at 22 weeks of gestation remains very low, with reported survival without long-term disability between 0% and 21% in the few studies performed. This survival rate improves with each increasing week of completed

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gestation, with an estimated survival of 5–46% in infants born between 23 and 24 weeks’ gestation and 40–59% in infants born between 25 and 26 weeks of gestation. Premature infants are at a much higher risk for developing CP than full-term infants, and the risk increases as gestational age and birth weight decreases. The incidence of premature birth is only 10%, but 40% of children with cerebral palsy are born prematurely. In a large cohort of children with CP, 25% of children were born before 32 weeks’ gestation, 10–20% were born between 32 and 36 weeks’ gestation, and 60% were born after 36 weeks’ gestation (Hirvonen et al. 2014). In data obtained from Europe and Australia, the prevalence of CP was between 35.0 and 79.5 per 1000 live births for children born at 28–31 weeks gestation and 6.1 per 1000 live births among children born at 32–36 weeks gestation. This is drastically different than infants born at 37 weeks of gestation or greater, where the risk of CP is 1.1 per 1000 live births. The prevalence of CP is much higher in preterm infants born at 200% of the federal poverty level positively predicted medical home transition within the Hispanic population of CSHCN. No characteristics were positively predictive of medical home transition within the non-Hispanic Black population. The authors recommended a more culturally tailored intervention which incorporates quality improvement approaches to progress medical home transition, especially for the Black population. In addition, policy measures which improve transition service reimbursements and improve health literacy and communication style may further increase transition rates. Transition of Orthopedic Services in the CP Population Over 90% of children with CP will survive beyond their 18th birthday (Strauss et al. 2008). After the transition of the CP adolescent to adult care, it may be very difficult to find access to an adult orthopedic surgeon experienced in neuromuscular surgical care. It is important to anticipate this and to recommend most major surgical care (e.g., spinal fusion, multilevel surgeries, hip reconstructions, etc.) while the child with CP still has access to an experienced pediatric CP surgeon. This may also be true for other pediatric specialists. In the future, it will be important for adult specialty societies to provide more expertise in the care of cerebral palsy adults during residency training so that adult specialty providers will be adequately prepared to provide care for this population, therefore becoming less of an access issue. The orthopedic needs upon transition will differ depending on the motor involvement and

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current functional status and deformities of the young person with CP. Parents and young adults with CP often report an increase in burden of care, a decrease in provider communication for what is often a very different subculture between pediatric and adult-oriented medical and orthopedic care. As an example, the adult CP patient may find herself seeing several orthopedists, given the fields’ many narrow areas of sub-specialization (spine, hip, upper extremity, foot/ankle) that may be required. Burns et al. recommend clear communication and documentation from the pediatric to the adult orthopedic CP provider which they term “the minimum clinical data set,” a transition document that accurately summarizes some of the medical record, corrects inconsistent information, and focuses on the musculoskeletal history and active/ongoing problems (Stevenson et al. 1997). The document may include patient information regarding spasticity management, muscle contractures, bone health/fracture management, pain management, hip dysplasia/degenerative arthritis, spinal deformity, torsional malalignments, need for orthotics and other mobility aides, etc. (Burns et al. 2014). This document should be reviewed in detail with the patient and family. The motor classification of the patient should also be clearly documented. Spine and hip radiographs should be performed prior to transition. In addition, health-related quality of life metrics are helpful to document outcome measures in order to manage specific treatable issues which may impact need factors causing vulnerability to quality of life, therefore becoming a health disparity to the CP adult compared to his or her peers.

industrialized nations, but especially true through the health equity lens (Davis et al. 2014; Schneider et al. 2017). Coupling this paradox with the fact that the USA leads the world in healthcare expenditures, with healthcare spending reaching approximately 17% of our gross domestic product, has caused us to face the harsh reality of how we can deliver a higher quality of healthcare for equal or lower cost (Adverse health conditions and health risk behaviors associated with intimate partner violence—United States, 2005 2008). The value proposition, defined by the value equation, is simply put as quality in the numerator and cost in the denominator, but may prove to be difficult to define, depending on whose prospective quality and cost comes from (Bachman et al. 2017; Moriates et al. 2015). From the patient and family’s prospective, quality equates to what benefits the patient and family, while the denominator cost equates to the financial impact on the family (e.g., out-ofpocket costs, family financial burden, days missed from work/school, cost of family member providing care, etc.). The definition varies when the perspective moves to the payor or the provider with the cost side equating to healthcare expenditures or cost to the payor or provider, while quality relates more directly to medical outcome measures and the patient experience within the healthcare system.

Quality, Cost, and Value: Their Impact and Importance on Disparities in Children with Disabilities

The financial impact on families of CSHCN is substantial. Children from low-income families have higher financial barriers to healthcare and pay a higher proportion of out-of-pocket income (Wong et al. 2005). Newacheck reported that CSHCN pay two times the out-of-pocket healthcare needs compared to those without special healthcare needs (Newacheck and Kim 2005). According to the 2001 National Survey of CSHCN, greater than 80% of families reported

A global comparison of healthcare shows that while the USA is one of the most technologically advanced, it is ironically has one of the worse delivery systems among technologically advanced countries. This is the case when compared using several quality measures with other

Value for CSHCN, CMC, and Children with CP: The Patient and Family Perspective

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having annual out-of-pocket healthcare expenditures averaging $752 (Porterfield and DeRigne 2011). The 2009–2010 National Survey of CSHCN has shown annual out-of-pocket payments for greater than 50% of children to be greater than $250, with 22% of families spending greater than $1000 of out-of-pocket costs (U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau 2013b). Outof-pocket costs for low-income families had a higher likelihood of being covered compared to the 2000 survey. This is likely due to the current coverage by Medicaid and SCHIP, which limit copays to families. Non-Hispanic White families are most likely to pay > $1000 out-of-pocket expenses which are likely due to the lower public insurance status in this population. The survey, however, did not address relative out-of-pocket expenses (out-of-pocket cost as a proportion of family income) as previously reported by Wong et al. (2005) or financial burden reported by Lindley and Mark (2010), both of whom reported higher levels of relative out-of-pocket income and financial burden, respectively, for lower-income children. This was validated in the 2009–2010 survey, which did ask families whether the child’s condition or need caused a financial problem for the family (U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau 2013c). While children from lower-income families with CSHCN had lower out-of-pocket costs, they reported higher financial problems caused by the child’s condition compared to higher-income families (U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau 2013c). The highest percentage of families reporting financial problems due to the child’s condition were those who were uninsured (47.6%). Public insurance appears to partially mitigate this burden (21%) compared to those with private insurance (19%). Those families with children who have conditions affecting daily activities the most (i.e., severe physical disabilities) have the highest reported financial problems (38.5%) (U.S. Department of Health and Human

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Services, Health Resources and Services Administration, Maternal and Child Health Bureau 2013c). Time burden spent providing care to the CSHCN is another potential burden to the family. This includes time giving medications and therapies, arranging or coordinating care providers, providing transportation to medical appointments, etc. This time burden was reported higher in low-income families with 20% of poor families reporting spending 11 h or more per week providing care compared to only 6% in families with incomes of 400% above the poverty level (U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau 2013d). Similar to financial burden, time burden was more common in families of children with greater functional disability (29.3%) (U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau 2013d). Romley et al. did a cost analysis on family-provided care from the 2009–2010 CSHCN survey finding that 5.6 million CSHCN received 1.5 billion hours of care annually (Romley et al. 2017). This equates to an estimated annual home health aide cost of $35.7 billion, or $6500 per child per year. Alternatively, it equates to an annual minimum wage of $11.6 billion or $2100 per child per year. Additionally, annual forgone earnings were estimated at $17.6 billion. The greatest amount of familyprovided care lived below the poverty level were Hispanic, were parents/guardians without a high school diploma, and had CSHCN with severe conditions. Of CSHCN listed, families of children with CP had the highest number of familyprovided care hours per week with an estimated 14.4 care hours per week per child given (Romley et al. 2017). Socioeconomic disparities also exist according to the impact of CSHCN on parent employment with the percentage of parents of CSHCN cutting back or stopping their employment which increased according to the level of poverty (33% in those below the poverty line versus only 18% in those families greater than 400% above the poverty line). This effect on employment was found to be highest among

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children with greater disability and medical complexity.

Value of CSHCN, CMC, and Children with CP: The Provider and Payor Perspectives Delivering high-value healthcare to vulnerable populations such as CSHCN with disabilities, CMC, and children with CP is also important from the payor and provider perspective since they carry such a heavy cost burden. Healthcare expenditures related to the entire disabled population (children and adults) have been estimated at 400 billion dollars annually, with a majority spent on public programs (Anderson et al. 2010). While CSHCN represent a small percentage (15–18%) of the pediatric population, they account for approximately 42% of total pediatric medical costs, 33.6% of total healthcare costs of pediatric healthcare spending, and 52% of children’s hospital days according to 2000 Medical Expenditures Panel Survey (Strauss et al. 2008). CSHCN with medical complexity represent a smaller subgroup of CSHCN of only 0.4–0.7% of US children, but with higher expenditures per child and account for 15–33% of all children’s healthcare spending, approximately $50–$110 billion annually. These patients often have high inhospital, emergency department visits, pharmacy charges, and homecare costs and represented 71% of unplanned 30-day readmissions (Berry et al. 2014). These authors proposed a business case of how cost savings in each of these areas could provide a greater investment into outpatient and community care of these children. The same study showed children with neurologic or neuromuscular conditions represented approximately 25% of CSHCN with medical complexity. Many children with cerebral palsy fall within this subgroup, especially those who are GMFCS levels 3, 4, and 5. In the case of children with CP, the CDC estimated that the lifetime costs of children born with cerebral palsy in the year 2000 would total approximately 11.5 billion dollars (Autism and Development Disabilities Monitoring (ADDM) Cerebral Palsy Network 2013). Medicaidenrolled children with CP had a cost that was 10 times higher ($15,047 per Medicaid-enrolled

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child, per year) than children without CP ($1674 per Medicaid-enrolled child, per year), while those children with both CP and an intellectual disability had a cost that was 26 times higher (per Medicaid-enrolled child, per year $41,664) (Autism and Development Disabilities Monitoring (ADDM) Cerebral Palsy Network 2013). Considering the high medical cost and family burden per year of CSHCN, CSHCN with medical complexity, and children with CP coupled with the many poor-quality measures already stated, the value equation from the payors, healthcare organizations, and the family perspective leaves much room for improvement. In order to improve value for these vulnerable pediatric populations, including children with CP, innovative healthcare delivery and payment models will be crucial. In addition, these models must create equitable outcomes within their respective subpopulations (e.g., by socioeconomic status, race, ethnicity, language, etc.). Ultimately, improvement in value for CSHCN, CMC, and children with CP will depend on improving or creating healthcare delivery models that decrease expenditures and decrease the time and cost burden to families while improving both the quality of medical care and quality of life for these pediatric populations and their caretakers. On the payor/provider side, alternative payment models will need to incentivize and hold providers accountable for meeting cost and quality targets. Finally, state and federal policies will need to protect patient’s interest by regulating a balance between payors and providers to keep the patient’s and family’s needs at the center. Since children with CP are a subgroup of CSHCN, CMC, and children with disabilities, the examples and principles that follow are largely applicable to the CP population as well.

Value and Healthcare Delivery Models in CSHCN, CMC, and Children with CP Most models of healthcare delivery for CMC and CSHCN heavily emphasize enhanced care coordination as well as the importance of integrating primary, subspecialty, and community care, each of which is critical to address the need, predisposing, and enabling factors within vulnerable

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Health and Healthcare Disparities in Children with Cerebral Palsy

populations described earlier and which are necessary to eliminate the health outcome disparities within the CP, CMC, and CSHCN subpopulations. In their review of care for CMC, Pordes and colleagues describe three categories of care delivery models for children with medical complexity: primary care-centered, co-managementcentered, and episode-based models (Pordes et al. 2018). Each category has separate implications for quality and cost for CMC. The primary care-centered model is defined by the patient-centered medical home, described earlier in this chapter. Its advantage is that it is typically geographically closer in proximity to the family’s home and therefore results in easier travel, access, and less time and financial burden to the family. In addition, their knowledge and access to community resources is often better than the other models of care. On the other hand, resources and infrastructure may be lacking for care coordination, and adequate medical record sharing across systems and/or an adequate knowledge/skill level to take care of condition-specific problems such as CP may be minimal. The latter may negatively impact cost, quality, and clinical decision-making. In addition, the evidence for the cost-effectiveness of the medical home for CSHCN is mixed with one study supporting decreased inpatient expenditures, especially in those with physical limitations (Romaire et al. 2012). There is also some evidence that a hybrid community-based primary care-centered model linked to a tertiary care center for CMC may decrease healthcare system costs as well as parent out-of-pocket costs while improving quality of life domains like comfort, emotions, and social within the child’s quality of life (Cohen et al. 2012). Co-management models are usually connected with a tertiary care center such as children’s hospitals which often have a higher service delivery cost, but may also have dedicated resources for the start-up cost of care coordination and electronic medical record systems compared to a primary care-centered model, leading to better care coordination and integration. This model is often consultative and also provides greater expertise, but also stands the risk of the specialist not

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becoming involved in the care of the patient until the problem has become too advanced (e.g., hip dislocation and advanced scoliosis in the CP child) if the primary care physician is the sole gatekeeper to specialty care. There is also a risk for diffusion of responsibility of coordination and integration of care with other specialties and community-based services (i.e., “no captain of the ship”), which can result in lack of care accountability to the patient and family. Also, enrollment criteria within many collaborative care models may leave some subsets of children with unmet medical and technology needs. Episode-based models are most effective when acute medical management is required such as the need for around-the-clock bedside management and primarily occurs within an inpatient facility. The greatest advantage is the ability to treat the child during their most vulnerable clinical status with caretakers who have a high degree of familiarity with the specific condition of the child. In the more involved CP child, this is often the case post-surgery or for an acute medical episode. While there is also the advantage that the burden of care provision is removed from the family during this acute episode, time and financial burden is often highest for the family due to missed time from work and time with other children during the acute medical episode of care. In addition, episode-based care has a high-cost tag, despite often being associated with strict discharge standardizations. Finally, this model of care is most associated with poor care continuity and coordination with the child’s primary medical provider and educational and community care teams. CSHCN and CMC are at especially high risk for hospitalization and may require weeks to months for the recovery of overall health and function. In addition to the comprehensive care models described above, home healthcare and/or post-acute facility care may help to reduce the overall cost of care to the healthcare system and out-of-pocket cost and burden to the parents of prolonged acute care hospitalizations in the case of home healthcare. In addition, lengthy stays in an acute care facility increase the risk in CMC for inhospital nosocomial infections. In a national study by Berry et al., discharge of children to

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home healthcare and post-acute facilities was much less common than for adults and was more common in children with four or more chronic conditions (CMC) and with neuromuscular complex chronic conditions (Berry et al. 2016). Racial/ethnic disparities were found with a lower percentage of Hispanic children discharged to both home healthcare and to post-acute care facilities. On the other hand, a higher percentage of non-Hispanic Black children were discharged to post-acute care facilities. It is unclear why these racial/demographic disparities exist. Further study is necessary to ascertain the role and outcomes of post-acute care and home healthcare post-hospital admission in CMC and their success in different racial, ethnic, and socioeconomic groups.

Value and Alternative Payment Models in CSHCN, CMC, and Children with Cerebral Palsy Currently, an increasing interest in improving overall value in healthcare is evolving by improving cost-effectiveness through the use of alternative payment models or so-called value-based purchasing strategies. This shift from fee-for-service to value has primarily been used in adult healthcare systems, with value-based purchasing slowly being incorporated into pediatric healthcare and will likely have a significant impact on the care of all CSHCN (Bachman et al. 2017). Alternative payment models vary from pay-for-performance features to full-risk models that pay providers a capitated fee for the care of a specified population (Langer et al. 2018). Those which have pay-for-performance features pay bonuses for improved high-quality performance through incentivizing payments for value and health outcomes such as patient-centered care, care coordination, improving social determinants of health, integrating behavioral health and community-based organizations, and clinic system integration. In addition to rewarding high quality, other types of alternative payment models, such as accountable care organizations (ACOs), are penalized for poor outcomes. ACOs are formed by groups of physicians and typically receive a risk-adjusted global budget for a population (capitation) and must manage total costs.

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Accordingly, ACOs are also focused on coordinated care in order to avoid unnecessary duplication of services while encouraging quality outcomes. Finally, bundled or episodic payments create a single payment for a single service or an episode of care such as a surgical episode. For example, in the case of the child with cerebral palsy, a bundled payment may include a preoperative gait analysis and a single-event multilevel surgery, plus the postoperative rehabilitation/therapy services as one episodic payment. The provider therefore assumes the full financial risk for the entire episode, including any preventable surgical complications, and various bonus payments for achieving performance measures. Whether the impact of alternative payment models will be positive or negative is currently largely unknown. Incentives under alternative payment models allow investment into care coordination, community health workers, and care management, typically not reimbursed in most fee-for-service models. In the child with cerebral palsy, value-based purchasing strategies will require knowledge of the lifetime cost per child with cerebral palsy according to their motor and intellectual involvement, as well as the lifetime cost per CP child according to coexisting medical morbidities. Further complicating this is the added cost of social complexity found in the overlying socioeconomic and racial disparities that frequently occur in the CP population. In addition, while the pay-for-performance features of valuebased purchasing strategies theoretically incentivize and improve the quality side of the value equation, there currently are no consistently agreed upon quality outcome metrics that impact CSHCN or children with cerebral palsy. Also, financial penalties in alternative payment models may bias providers into the selection of caring for less medically and socially complex patients (Joynt Maddox 2018) which may further increase socioeconomic and/or racial and ethnic disparities within the CSHCN, CMC, and cerebral palsy populations. Adequate risk adjustment will therefore be necessary to incentivize providers to care for complex pediatric patients. Predictive modeling of high-cost patients using parent-reported measures which include sociodemographic

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characteristics has been shown to be helpful in predicting the risk of high health costs in CSHCN (Leininger et al. 2015). Regardless of the delivery or payment model, care management for the delivery of care to CSHCN, especially within integrated systems such as state Medicaid systems, is critical for controlling cost through the reduction of duplicative services, internal incentives for cost reductions, and improvements for the coordination of care and decreasing administrative costs in the processing of claims (Marcu et al. 2016). Through the combination of innovative care delivery systems, alternative payment models, and care management, high-value care for all CSHCN, especially those CMC, and children with CP can hopefully be achieved.

A High-Value Musculoskeletal Model of Care Delivery for the CP Child Within our own healthcare organization, a large cerebral palsy center, an integrated primary/specialty musculoskeletal care collaborative model exists with medical care coordination occurring in tandem with orthopedic care coordination. A team of primary care physicians rotate, caring for the more medically complex surgical CP patients during their preoperative work-up and their inpatient surgical episode. Patients who are close geographic proximity are also a part of the medical home. In this model, there is also an integrated team of neuromuscular orthopedic surgeons and rehabilitation medicine specialists who see the child as an outpatient every 6 months. A team of nurse practitioners and physician assistants share the role as neuromuscular care coordinator and communicate with the family prior to their first visit to ascertain the family’s needs and then develop a preliminary plan of care which includes the possible need for orthotic services, therapy services, wheelchair or other assistive devices, augmentative communication, bone health, gait analysis, primary care, mental/behavioral health services, and/or specialty care (including orthopedic surgery). Next, a coordinated visit is set up between the necessary specialty care providers and the family and child. Ongoing communication between the musculoskeletal care coordinator, primary care provider, and the family takes

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place to assist with the musculoskeletal needs of the child, including assistance with therapy, equipment needs, and community resources. Care coordinators rotate between their care coordination role, seeing patients in the outpatient clinic and being a part of the inpatient team during episodes of inpatient care. The need for orthopedic surgery is common for the child with CP. In our model upon anticipation of a surgical episode, the musculoskeletal care coordinator begins to prepare the child and family for surgery. This first step involves educating the family and child (age appropriate) about the surgical procedure and the postoperative course, including assistance with any medical and community resources that will be needed to support the family and child postoperatively. The goal is to minimize inhospital acute care and time/financial burden to the family, expedite the rehabilitation process, and assimilate the child and family back into their home and community. Families with a diverse race/ethnic background, a more rural geography, a community of lower socioeconomic background, a limited English proficiency, and an immigrant status often have increased barriers to the necessary resources to provide the proper postoperative equipment, support, and rehabilitation services (Parish et al. 2012; Eneriz-Wiemer et al. 2014; Kan et al. 2016). In such cases, the child may benefit from inpatient or intensive outpatient rehabilitation in a hospital or post-acute care setting in order to mitigate these barriers and achieve optimal and equitable surgical outcomes. Using this care delivery model allows each member of the team to familiarize themselves with each child and their family. In addition, this model allows the patient to move between primary carecentered, collaborative care-centered, and episodebased care models when necessary while preserving patient-/family-centered care coordinators who have developed a relationship with the patient.

Health Policy to Prevent Health and Healthcare Disparities in CSHCN In addition to the creation of innovative healthcare delivery and alternate payment models aimed to mitigate disparities in health need factors in

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pediatric vulnerable populations such as children with CP, health programs and policies at both the state and federal levels are put in place to help regulate and protect CSHCN with disabilities, CMC, and children with CP at a population health level. For example, the Rehabilitation Act of 1973, Section 504, protects the rights of individuals with disabilities who are involved in programs or activities that receive federal financial assistance from the Department of Education (The Rehabilitation Act 2017). Additionally, the Americans with Disabilities Act of 1990, Title II (ADA), prohibits discrimination based on disability and guarantees equal opportunity for those with disabilities in employment, public accommodations, telecommunications, and state and local governments (Americans with Disabilities Act (ADA) 2015). Furthermore, there are available programs and policies that are aimed to help children with healthcare needs and disabilities to smoothly transition into adulthood. Some examples of health-related public programs that are being implemented are Title V of the Social Security Act and the Individuals with Disabilities Education Act (IDEA) (Field and Jette 2007). The former allows the Maternal and Child Health Bureau (MCHB) to direct a major program of state block grants that offer support services for mothers, children, and infants. Additionally, it offers specialized national programs that are catered toward children with special healthcare needs or disabilities. IDEA mandates that children with disabilities who require special education and associated services receive free appropriate public education. Federal policies also require that school districts pay for certain services and assistive technologies for children who require special education and associated services. IDEA also mandates that transition planning into adulthood should begin no later than age 16. This transition is intended to be results-oriented and dedicated to helping the child reach their highest functional and academic potential and facilitates fluid movement between the different aspects of their life, from school to post-school activities, community involvement, employment,

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independent living, etc. (Field and Jette 2007). An early focus on long-term planning for a child’s transition into adult life can help families identify and ameliorate potential concerns. There is increasing pressure on our national healthcare and public health sectors to sufficiently address the existing and future health needs of children with disabilities across their lifespan (Krahn et al. 2015). Even with better access to insurance, the American health system is not well designed to meet the needs of people with serious long-term health conditions or disabilities. CSHCN and disabilities are more likely than other children to have health insurance and are somewhat more likely to have public insurance compared to other children (Field and Jette 2007). As mentioned previously, this population also utilizes more healthcare services than other children (Bachman et al. 2017). Medicaid, along with other public insurance options, covers 44% of CSHCN, serving as the primary source of coverage for more than one-third of this population (Schubel and House 2017). Medicaid is considered the gold standard for insurance coverage of CSHCN and disabilities because states are required to provide the Early and Periodic Screening, Diagnostic and Treatment (EPSDT) benefit, which guarantees individuals under the age of 21 access to comprehensive and preventive health services (Schubel and House 2017). These services include regular wellchild visits; hearing, vision, and dental screenings; and treatments for physical, mental, and developmental diseases and disabilities, as well as longterm services and supports. Even with the availability of public insurance, however, there is instability across Medicare, Medicaid, and State Child Health Insurance Program (SCHIP). For example, children are often disenrolled and reenrolled in different program options due to strict program requirements, changes in family income, or characteristics of the child that determine whether they are eligible for Medicaid, other public coverage, or no coverage (Field and Jette 2007). This is especially concerning for CMC. In 2015, Advancing Care for Kids Act was introduced as a bill to congress, which would give states the option of providing services to CMC under the Medicaid and SCHIP

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programs through a Medicaid Children’s Care Coordination Program (Langer et al. 2018). The Act helps to account for the inability of typical policy measures which are focused on access, cost, and quality in the general population and adult chronic conditions and are unable to take into account the nuances of delivery systems for CMC (Langer et al. 2018). In the future, policy makers will need to address a number of policy issues to achieve optimum care in CMC (Bachman et al. 2017; Langer et al. 2018). First, providers, payors, and policy makers will need to reach a definitive consensus definition of CMC in order to better understand the true number of patients within the CMC population to ascertain better accuracy of the number of CMC for resource allocation, quality, cost, and reimbursement for this medically complex population. There also needs to be consensus on broadly measured quality measures for CSHCN and CMC in order to establish viable quality metrics for alternative payment models. This will help enable adequate risk complexity adjustment to prevent providers who care for increased numbers of CSHCN, especially CMC, from being disadvantaged by alternative payment models with pay-for-

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performance features. Additionally, financial incentives should be explicitly guarded not to bias providers from selecting complex cases. In addition, increased funding and reimbursements are needed for both home- and communitybased services that are currently underfunded. Further measures are also necessary to mitigate family hardship burdens. CSHCN, with and without disabilities, and CMC are particularly at risk for marginalization and health inequity. Risk adjustments for poor functional status should be considered in pay-for-performance alternative payment models along with medical complexity. Finally, vulnerable pediatric populations also have an increase in both racial and ethnic and socioeconomic disparities, all of which will require consideration in value-based purchasing and alternative payment models.

Cases Case 1 (Pre-care Coordination) (Fig. 7) This is a 14-year-old young lady with diagnoses that include quadriplegic CP with microcephaly, seizure disorder, a need for gastrostomy tube

Fig. 7 (Left) The patient’s AP radiograph immediately postoperatively after her spinal fusion. (Right) A decubitus ulcer that the patient developed due to poor nutrition 2 months postop

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feedings with dysmotility and FTT, global developmental delay, and severe intellectual disability (GMFCS V, nonverbal). The child was initially seen at age 3 (2006) by orthopedics and rehabilitation medicine. She is seen by multiple providers including developmental pediatrics, gastroenterology, general surgery nephrology, neurology, ophthalmology, and urology. Socially, the child lives with parents and four siblings. They live approximately 2 h, drive from hospital, and attend special needs school. The child attends a special needs school 1 h. away. The family has no home nursing. Most recently, the child was noted to have a progressive scoliosis (see figure below), and a posterior spinal fusion with instrumentation was recommended; however, the family was reluctant to have surgery until 1 year later. Preoperatively, the child was seen by pulmonology, neurology, and pediatrics for clearance. The child was below the tenth percentile weight for height ratio with intermittent nutritional problems due to social issues. The surgery was without complications; however, postoperatively after discharge, the patient developed a fever, wound breakdown, and

multiple decubitus ulcers (medial malleolus, over prominent pelvic fixation, and ischium (see figures below)). The patient required a 1-month hospitalization for wound care, rotational skin flap, and antibiotic treatment. Opportunities for improvement included care coordination to improve communication between specialty providers and work on improving the child’s nutrition status prior to surgery as well as improve education about the surgery with the family.

Fig. 8 (Left) An AP radiograph of this 13-year-old male with CP, quadriplegia who had previous left femoral head resection and valgus osteotomy to treat left hip pain. (Right) Development of a right hip dislocation and also right hip pain with recurrent left hip pain. This shows the final postop radiograph of this patient after treatment with a

left hip replacement to treat the failed left femoral head resection, and a right femoral varus derotational osteotomy with and right peri-acetabular pelvic osteotomy. Now with improved care coordination, the patient had improved outcomes and is pain free

Case 2 (Fig. 8) This child is a 13-year-old male with a history of quadriplegic pattern CP (GMFCS 5), severe spasticity, communication disorder requiring communication device, poor oral intake due to swallowing disorder, gastroesophageal reflux, mild reactive airway disease, mild sleep apnea, and chronic nutritional deprivation with BMI < fifth percentile. The child had previous hip procedures, a femoral head resection, and valgus osteotomy for hip pain and continues to have chronic pain requiring chronic opioids. The family called to request a second opinion due to the child’s continued chronic hip pain.

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Socially, the child lives with his grandmother and aunt in a rural community with poor access to services and is greater than 2 h away from the hospital. Our care coordinator called the family prior to their visit and did a telephone intake history and set up coordinated visits with orthopedics, rehabilitation medicine, our pediatrics complex medicine team, pain management, and nutritional and therapy services. Outside studies and all records were requested prior to the visit. A presurgical and surgical plan was coordinated and discussed with the family, the school, the home pediatrician, and the subspecialty teams which would include initiating oral dietary supplements and increasing caloric intake. Approximately 4 months later, right hip reconstruction and a left hip replacement were performed after the child’s nutritional status improved. A home nutritional and pain management plan was developed for the family and coordinated with the home pediatrician. A therapy plan was coordinated with the local private and school therapists. The child has since successfully had a G-tube placement to achieve further nutritional improvement.

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174 Romley JA, Shah AK, Chung PJ, Elliott MN, Vestal KD, Schuster MA (2017) Family-provided health care for children with special health care needs. Pediatrics [Internet] 139(1):e20161287. Available from: http://pediatrics. aappublications.org/lookup/doi/10.1542/peds.2016-1287 Rosen-Reynoso M, Porche MV, Kwan N, Bethell C, Thomas V, Robertson J et al (2016) Disparities in access to easy-to-use services for children with special health care needs. Matern Child Health J 20(5):1041–1053 Schneider EC, Sarnak DO, Squires D, Shah A, Doty MM (2017) Mirror mirror 2017: international comparison reflects flaws and opportunities for better U.S. Health Care [Internet]. Available from: http://www. commonwealthfund.org/interactives/2017/july/mirrormirror/ Schubel J. House ACA (2017) Repeal bill puts children with disabilities and special health care needs at severe risk Shi L, Stevens GD (2010a) A general framework to study vulnerable populations. In: Vulnerable populations in the United States, 2nd edn. Jossey-Bass, SanFrancisco, Ca, p 3 Shi L, Stevens GD (2010b) A general framework to study vulnerable populations. In: Vulnerable populations in the United States, 2nd edn. Jossey-Bass, SanFrancisco, Ca, pp 18–28 Solaski M, Majnemer A, Oskoui M (2014) Contribution of socio-economic status on the prevalence of cerebral palsy: a systematic search and review. Dev Med Child Neurol 56(11):1043–1051 Spencer NJ, Blackburn CM, Read JM (2015) Disabling chronic conditions in childhood and socioeconomic disadvantage: a systematic review and meta-analyses of observational studies. BMJ Open 5(9):e007062 Stevenson CJ, Pharoah PO, Stevenson R (1997) Cerebral palsy–the transition from youth to adulthood. Dev Med Child Neurol 39(5):336–342 Strauss D, Brooks J, Rosenbloom L, Shavelle R (2008) Life expectancy in cerebral palsy: an update. Dev Med Child Neurol 50:487–493 Strickland BB, Singh GK, Kogan MD, Mann MY, van Dyck PC, Newacheck PW (2009) Access to the medical home: new findings from the 2005-2006 national survey of children with special health care needs. Pediatrics 123(6):e996-1004 Strickland BB, Jones JR, Newacheck PW, Bethell CD, Blumberg SJ, Kogan MD (2015) Assessing systems quality in a changing health care environment: the 2009-10 national survey of children with special health care needs. Matern Child Health J 19(2):353–361 Tan-McGrory A, Bennett-AbuAyyash C, Gee S, Dabney K, Cowden JD, Williams L et al (2018) A patient and family data domain collection framework for identifying disparities in pediatrics: results from the pediatric health equity collaborative. BMC Pediatr 18(1) The Rehabilitation Act. 2017 Feb 27 [cited 21 Jan 2018]; Available from: https://www2.ed.gov/policy/speced/reg/ narrative.html

K. W. Dabney et al. U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau (2013a) The national survey of children with special health care needs chartbook 2009–2010. U.S. Department of Health and Human Services, Rockville, p 19 U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau (2013b) The national survey of children with special health care needs chartbook 2009–2010. U.S. Department of Health and Human Services, Rockville, pp 51–52 U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau (2013c) The national survey of children with special health care needs chartbook 2009–2010. U.S. Department of Health and Human Services, Rockville, p 53 U.S. Department of Health and Human Services, Health Resources and Services Administration, Maternal and Child Health Bureau (2013d) The national survey of children with special health care needs chartbook 2009–2010. U.S. Department of Health and Human Services, Rockville, p 54 Van Naarden Braun K, Doernberg N, Schieve L, Christensen D, Goodman A, Yeargin-Allsopp M (2016) Birth prevalence of cerebral palsy: a population-based study. Pediatrics 137(1): e20152872 Wilber N, Mitra M, Klein Walker D, Allen D, Meyers AR, Tupper P (2002) Disability as a public health issue: findings and reflections from the Massachusetts survey of secondary conditions. Milbank Q 80 (2):393–421 Winter S, Autry A, Boyle C, Yeargin-Allsopp M (2002) Trends in the prevalence of cerebral palsy in a population-based study. Pediatrics 110(6):1220–1225 Wong ST, Galbraith A, Kim S, Newacheck PW (2005) Disparities in the financial burden of children’s healthcare expenditures. Arch Pediatr Adolesc Med 159(11):1008–1013 World Health Organization (2011) World report on disability. World Report on Disability 2011 Wu YW, Xing G, Fuentes-Afflick E, Danielson B, Smith LH, Gilbert WM (2011) Racial, ethnic, and socioeconomic disparities in the prevalence of cerebral palsy. Pediatrics 127(3):e674–e681 Yeargin-Allsopp M, Van Naarden Braun K, Doernberg NS, Benedict RE, Kirby RS, Durkin MS (2008) Prevalence of cerebral palsy in 8-year-old children in three areas of the United States in 2002: a multisite collaboration. Pediatrics 121(3):547–554 Young NL (2007) The transition to adulthood for children with cerebral palsy: what do we know about their health care needs? J Pediatr Orthop 27(4):476–479 Zan H, Scharff RL (2015) The heterogeneity in financial and time burden of caregiving to children with chronic conditions. Matern Child Health J 19(3):615–625

Part IV Pathology

Neuroimaging Pathology in Cerebral Palsy

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Rahul M. Nikam, Arabinda K. Choudhary, Vinay Kandula, and Lauren Averill

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178 Fetal Neuroimaging Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179 Hypoxic-Ischemic Brain Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 180 Preterm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 Term Infants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 186 Congenital Infections of the Central Nervous System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Cytomegalovirus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Toxoplasmosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Lymphocytic Choriomeningitis Virus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

189 191 193 193

Congenital Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Lissencephaly (The Agyria-Pachygyria Complex) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Microcephaly with Simplified Gyral Pattern (MSG) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Schizencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Megalencephaly-Postaxial Polydactyly-Polymicrogyria-Hydrocephalus Syndrome (MPPH) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Septo-Optic Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18q-Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Syntelencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Joubert Syndrome and Related Disorders (Molar Tooth Malformations) . . . . . . . . . . . . . . Rhombencephalosynapsis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Aicardi Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hydranencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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R. M. Nikam (*) · A. K. Choudhary (*) · V. Kandula · L. Averill Nemours A I duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_10

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R. M. Nikam et al. Miscellaneous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Kernicterus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211

Abstract

Neuroimaging in cerebral palsy, although not a prerequisite for diagnosis, provides vital insights in elucidating the etiopathogenesis and has widespread implications in treatment, prognosis, and planning early interventions to curtail complications. Neuroimaging is abnormal in a majority of children with cerebral palsy. Magnetic resonance imaging, due to its superior soft tissue differentiation, multiplanar capabilities, and prospect for functional imaging, is uniquely qualified for assessment of fetal and postnatal brain. Cranial ultrasound and computed tomography, although inferior in soft tissue resolution, have a potential role in emergent situations. Hypoxic-ischemic encephalopathy is one of the commonly identified causes of cerebral palsy with other etiologies being congenital infections of central nervous system such as Cytomegalovirus (CMV) and toxoplasmosis and congenital malformations. Keywords

Cerebral palsy · Magnetic resonance imaging · Hypoxic-ischemic brain injury · Congenital infections of CNS

Introduction Cerebral palsy (CP) is a clinical diagnosis and is defined as “a group of disorders of development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in developing fetal or infant brain” (Bax et al. 2005). According to the systematic review and meta-analysis of 49 population-based studies on the prevalence of CP conducted by Oskoui et al. 2013, the pooled overall prevalence of CP was 2.11 per 1000 live births (95% confidence

interval (CI) 1.98–2.25). Although neuroimaging is not a prerequisite for the diagnosis of CP, neuroimaging findings are abnormal in more than 80% of children with CP (Himmelmann et al. 2017; Korzeniewski et al. 2008; Krägeloh-Mann and Horber 2007). Also, neuroimaging provides vital insights into etiology, pathogenesis, and prognosis and has implications for prevention and interventional strategies. Accurate determination of the etiology of CP has specific implications regarding treatment, prognosis, and ongoing medical management of associated conditions (Ashwal et al. 2004). Neuroimaging is frequently obtained if there is history of perinatal complications, prematurity, or when neonatal examination is positive for neurologic symptoms or signs. The array of imaging techniques available for evaluation of neonatal brain includes ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI). At times, the risks of obtaining a neuroimaging study in an affected neonate may potentially outweigh the benefits of further defining the etiology, which is predominately related to transportation of the neonate out of the intensive care unit and possible need for sedation. Cranial ultrasound (US) is the preferred initial investigation, particularly in high-risk and unstable premature infants due to its portability, widespread availability, low cost, speed, and lack of ionizing radiation. Cranial US is most useful for detection and follow-up of hydrocephalus, intracranial hemorrhage, and periventricular leukomalacia (PVL). The major limitations met with cranial US include operator dependence, image quality reliant on acoustic window, and suboptimal evaluation of the posterior fossa and myelination. Also, diffuse white matter injury, as can be seen in preterm infants, is not well evaluated by cranial US. Computed tomography (CT), based on the principle of X-ray attenuation, produces high-

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resolution images of the brain and provides exquisite osseous details. It is the least sensitive modality for evaluation of neonatal hypoxic-ischemic encephalopathy (HIE), because high water content of the neonatal brain results in poor parenchymal contrast resolution. However, CT offers a comparatively rapid investigative tool in emergent situations and has a role in evaluation of intracranial hemorrhage, hydrocephalus, and parenchymal calcifications. The superior soft tissue resolution and multiplanar capabilities of magnetic resonance imaging (MRI) make it uniquely qualified for the assessment of the brain. MRI utilizes a strong static magnetic field and radiofrequency waves to generate images and has no known potential harmful effects in biologic tissues within clinically prescribed parameters. Compared to CT, MRI does not use ionizing radiation. MRI, however, poses several limitations such as longer scan times and need for sedation and is precluded in patients with certain implanted ferrous instrumentation, cardiac pacemakers, and neurostimulators. The various imaging sequences acquired for evaluation of the brain include sagittal 3D T1 weighted, sagittal 3D T2 weighted, axial and coronal fluid-attenuated inversion recovery (FLAIR), diffusion weighted (DWI), and susceptibility weighted (SWI). Additional imaging sequences such as MR spectroscopy (MRS), MR perfusion, and MR angiography (MRA) can be acquired as per the clinical concern. Sagittal T1-weighted images are vital for assessment of midline structures including the corpus callosum, pituitary gland, hypothalamus, and cerebellar vermis and hemispheric convexities such as perisylvian regions. Also, myelination is best evaluated by T1-weighted images from birth to 6 months of age. Axial or sagittal T2-weighted images are important for evaluation of parenchymal pathologies including structural abnormalities, hypoxic-ischemic insult, leukoencephalopathies, and neoplasms. From 6 to 8 months of age until approximately 24 months, myelination is better assessed with T2-weighted images. FLAIR-weighted sequence suppresses free fluid within the ventricular and subarachnoid space, increasing conspicuity of parenchymal pathologies. Diffusion-weighted

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imaging (DWI) exploits the random Brownian motion of protons within the voxel of tissue and is useful in evaluation of acute infarction, hypoxic-ischemic insult, grading of gliomas, differentiation of epidermoid from arachnoid cyst, encephalitides, demyelinating diseases, and leukoencephalopathies such as adrenoleukodystrophy. Susceptibility-weighted imaging (SWI) is exquisitely sensitive for blood products and calcium and is of paramount importance in evaluation of hemorrhagic transformation of infarction, HIE, and perinatal infections. Proton magnetic resonance spectroscopy (HMRS) is a noninvasive diagnostic method of analyzing tissue metabolism, yields chemical data reflecting tissue composition, and plays a vital role as an adjunct to conventional imaging in diagnosis of mitochondrial and metabolic disorders, leukoencephalopathies, and grading of gliomas.

Fetal Neuroimaging Techniques Evaluation of the fetal brain is performed as part of routine second trimester fetal ultrasound, providing information about fetal growth parameters, brain morphology, and ventricular size. When a brain abnormality is suspected with ultrasound, fetal MRI is often performed to confirm the suspected abnormality, detect additional abnormalities, or elucidate inconclusive sonographic findings. A recent meta-analysis of 27 studies including 1184 fetuses showed that in 23% of cases, fetal MRI demonstrated additional or different pathology than detected with ultrasound (van Doorn et al. 2016). In an additional 8% of sonographically suspected brain abnormalities, MRI was normal. On follow-up, fetal MRI had 80% complete agreement with the postnatal assessment, while neurosonography agreed in 54% of cases. In another meta-analysis, fetal brain MRI changed the clinical management of the pregnancy in 30% of cases (Rossi and Prefumo 2014). Although providing detailed anatomic information, fetal MRI is considered a problem-solving tool supplementary to ultrasound due to higher cost, longer scan time, and lower accessibility.

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Fetal MRI is typically performed in the second and third trimesters at 1.5 T, although recently reports of highly detailed brain MRI at 3 T are also emerging (Victoria et al. 2014). There are theoretical risks to the fetus from radiofrequency energy deposition, magnetic field exposure, and acoustic noise. Nonetheless, fetal MRI is a highly useful tool with no adverse outcomes reported (Bouyssi-Kobar et al. 2015; Strizek et al. 2015). Fetuses as young as 18 weeks’ gestation can be evaluated with MRI, although greater information can be obtained later in gestation as the sulcation pattern of the brain matures. The most commonly employed fetal MRI techniques are routine motion-insensitive sequences available from all manufacturers, performed at 3–5 mm slice thickness. Gyral anatomy, cortical development, and white matter signal intensity are best assessed with T2-weighted single-shot fast spin echo and steady-state free precession sequences, while the presence of hemorrhage, calcification, or fat is evaluated with gradient echo T1-weighted and T2-weighted echoplanar sequences (Lyons et al. 2015; Manganaro et al. 2017). Advanced imaging techniques including diffusion-weighted imaging and diffusion tensor imaging can also be employed for evaluation of ischemia and fiber tracking, respectively. Table 1 enumerates the most common known etiologies associated with cerebral palsy. The harmonized classification of magnetic resonance imaging based on pathogenic patterns (MRI classification system) was proposed by the Surveillance of Cerebral Palsy in Europe (SCPE) network and is described in Table 2 (Himmelmann 2016).

Hypoxic-Ischemic Brain Injury The incidence of hypoxic-ischemic encephalopathy (HIE) is 2–9 per 1000 live births and is one of the most common identified causes of cerebral palsy (Chao et al. 2006). The underlying pathophysiology of HIE is complex and not completely understood. Perinatal asphyxia is the most important cause of HIE (Chao et al. 2006) and results in hypoxemia, hypercarbia, acidosis,

R. M. Nikam et al. Table 1 Etiology of cerebral palsy Hypoxic-ischemic insult White matter injury of immaturity Basal ganglia/thalamic damage Focal infarct Cortical/subcortical damage Infections CMV Toxoplasmosis LCMV Rubella (German measles) Varicella (chicken pox) Herpes Syphilis Zika Congenital malformations Bilirubin encephalopathy CNS injury in multiple pregnancies Birth trauma Neonatal hypoglycemia Genetic abnormalities Coagulopathies causing stroke

Table 2 The harmonized classification of magnetic resonance imaging based on pathogenic pattern A. Maldevelopments A1. Disorder of cortical formation (proliferation and/or migration and/or organization) A2. Miscellaneous (e.g., holoprosencephaly, DandyWalker malformation, agenesis of corpus callosum, cerebellar hypoplasia) B. Predominant white matter injury B1. PVL: mild, severe B2. Sequelae of IVH or periventricular hemorrhagic infarction B3. Combination of PVL and IVH sequelae C. Predominant gray matter injury C1. Basal ganglia/thalamus (mild/moderate/severe) C2. Cortico-subcortical lesions only (watershed lesions in parasagittal distribution/multicystic encephalomalacia) C3. Arterial infarctions D. Miscellaneous: Hypomyelination, cerebellar atrophy, cerebral atrophy E. Normal

and decreased systemic blood pressure (Barkovich 2012). There is resultant loss of normal vascular autoregulation of the term neonate, described as “passive pressure flow” (Barkovich

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2012; Del Toro et al. 1991; Boylan et al. 2000). Furthermore, preterm infants and infants with neurologic impairment and seizures demonstrate an absent autoregulatory response (Boylan et al. 2000). Thus, the decreased systemic blood pressure and “passive pressure flow” contribute to the majority of hypoxic-ischemic brain injury. Intrauterine asphyxia occurs when placental blood flow and gas exchange is interrupted and can be caused by fetal factors (fetomaternal hemorrhage, fetal thrombosis, and fetal bradycardia), inadequate placental perfusion (maternal hypotension, preeclampsia, chronic vascular disease, abruptio placenta), impaired maternal oxygenation (asthma, pulmonary embolism, pneumonia, carbon monoxide poisoning, severe anemia), or disrupted umbilical circulation (tight nuchal cord, cord prolapse). Postnatal asphyxia results from underlying severe hyaline membrane disease, pneumonia, meconium aspiration, or congenital heart anomalies that cause neonatal pulmonary failure or hypotension (Chao et al. 2006). The various patterns of brain injury are a result of three primary factors: maturity of the brain, severity of hypotension, and duration of the hypoperfusion event (Chao et al. 2006; Barkovich 1992, 2012; Okereafor et al. 2008). A premature infant with mild to moderate hypoperfusion will sustain insult to the periventricular and deep white matter (“white matter injury of prematurity” or “periventricular leukomalacia”), which are the regions at highest risk due to their state of immaturity and vascular supply, with sparing of subcortical white matter and cerebral cortex (Barkovich 2012). With maturation of the brain and its vascular system, the pattern of injury begins to change between 34th and 38th post-conceptional weeks. In term infants, a similar degree of mild to moderate hypoperfusion leads to injury of watershed portions of the cerebral cortex and underlying subcortical and periventricular white matter (Li et al. 2009). The thalami and brainstem are most metabolically active in early third trimester. From mid-third trimester to 40 weeks of gestation, the brainstem, thalami, basal ganglia, and peri-Rolandic regions have the highest

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metabolic activity. The visual cortex becomes increasing more metabolically active by 44 weeks post-conceptional age. After third/ fourth postnatal months, the remainder of the cerebral cortex and basal ganglia become increasingly metabolically active and most vulnerable to hypoxic-ischemic insult (Barkovich 1992, 2012).

Preterm White Matter Injury of Prematurity or Periventricular Leukomalacia (Mild to Moderate Hypoperfusion) The most common pattern of injury of the premature brain is periventricular leukomalacia. Screening cranial US may demonstrate hyperechoic periventricular flare adjacent to trigones and frontal horns in focal and more generalized increased echogenicity in diffuse white matter injury. Neonatal cranial US has significant limitations in demonstrating non-cystic white matter injury with low sensitivity (26%) and low positive predictive value (36%). However, US demonstrates high reliability in the detection of cystic white matter injury (Inder et al. 2003). Three different types of white matter injury have been described and can be categorized with magnetic resonance imaging (MRI). These include diffuse white matter injury, with mildest clinical manifestations; focal/multifocal non-cavitary white matter injury, with intermediate severity; and focal/multifocal cavitary white matter injury, with severe clinical manifestations (Volpe 2008a). Early MRI findings include small T1 hyperintense foci in periventricular white matter and diffuse T2 hyperintense white matter signal abnormality. Diffusion-weighted imaging may demonstrate foci of restricted diffusion in periventricular white matter and may be negative before 24 h or after 5 days. Subacute findings include cavitary changes in periventricular white matter, and chronic findings include loss of periventricular white matter volume, angular ventricular morphology with “squared off” trigones, cortical ribbon extending to the ventricular margins, and focal thinning of body of corpus callosum (Figs. 1 and 2).

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Fig. 1 14-month-old female, born at 32 weeks of gestation, with spastic diplegic cerebral palsy. Axial FLAIRweighted images through the centrum semiovale (a) and basal ganglia (b) demonstrate abnormal hyperintense signal within the periventricular and subcortical white matter (black stars and white arrows, respectively), with associated enlargement of the lateral ventricles secondary to white matter volume loss. Coronal T2-weighted image (c) at the level of the atria of lateral ventricles further depicts abnormal hyperintense signal within the

periventricular and subcortical white matter. Of note, there is enlargement of both lateral ventricles with cortical sulci reaching up to the ventricular margins with resultant undulating ventricular contour (white arrow), related to white matter volume loss. Midline sagittal T1-weighted image (d) demonstrates thinning of the corpus callosum (white arrowhead), secondary to transcallosal axonal degeneration. Findings are characteristic of periventricular leukomalacia

Profound Hypotension in Preterm Infants In preterm infants, the thalami, brainstem, and cerebellum are most susceptible to severe hypotension. There may be coexisting white matter injury and germinal matrix hemorrhage (Barkovich and Sargent 1995). The imaging

findings in preterm infants with profound hypotension include hyperechogenic parenchyma on US and restricted diffusion, T1 hyperintense, and variable T2 signal abnormality on MRI, in the aforementioned distribution (Fig. 3).

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Fig. 2 16-year-old female, born at 31 weeks of gestation, with spastic quadriplegic cerebral palsy. Axial FLAIRweighted image (a) through the superior aspects of lateral ventricles demonstrates confluent abnormal hyperintense signal involving bilateral periventricular white matter (white arrows) with associated severe white matter volume

loss. The white matter volume loss is best appreciated on axial (b) and coronal (c) T2-weighted images as evidenced by enlargement of lateral ventricles, cortical sulci reaching up to the ventricular margins, and undulating ventricular contour (white arrows). Findings are characteristic of periventricular leukomalacia

Germinal Matrix and Intraventricular Hemorrhage The incidence of germinal matrix and intraventricular hemorrhage (GMH/IVH) among preterm neonates is 45%, and it is inversely related to gestational age and weight at birth (Kadri et al. 2006). GMH is most common in infants less than

32 weeks of gestational age and below 1500 gm weight and is unusual after 34 weeks of gestation (Volpe 2008b; Greisen 1992). Hemodynamic instability associated with birth is presumably related to these germinal matrix hemorrhages, as 40% have their onset within 5 h of birth and 90% within the first 4 days (Volpe 2008b). The most frequent

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Fig. 3 1-day-old baby, born at 35 weeks of gestation with unresponsiveness and in utero trauma. Sagittal T1-weighted image (a) shows hyperintensity in the dorsal brainstem (white arrow). Axial T1-weighted images (b and c) show hyperintensity in the bilateral thalami (black stars), Globi pallidi (white arrow), lateral

putamina (black open arrow), amygdalae (black arrowhead), and midbrain (black arrow). Axial diffusionweighted image (d) shows reduced diffusivity in the bilateral ventrolateral thalami (white arrows). Features are in keeping with profound hypotensive injury

area of GMH is near the posterior aspect of caudate head, the ganglionic eminence portion of germinal matrix. Within the cerebellum, the external granular layer represents the germinal zone (Barkovich 2012; Rakic and Sidman 1970), and hemorrhages within this area are not uncommon with prevalence of

approximately 19% in premature neonates (Steggerda et al. 2009). Risk factors for cerebellar hemorrhage include birth weight less than 750 gm, emergent Cesarean section, patent ductus arteriosus, and low 5 days’ minimum pH. GMH has been divided into four grades – grade I, GMH at the caudothalamic groove;

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Fig. 4 2 1/2-year-old male with spastic quadriplegic cerebral palsy, born preterm at 25 weeks of gestation with obstetric history significant for premature rupture of membranes. Axial T2-weighted image (a) through the lateral ventricles demonstrates severe white matter volume loss involving the bilateral cerebral hemispheres, more pronounced on the right, with undulation of ventricular margins (white arrow). Axial (b) and sagittal (c) T2-weighted images depict severe atrophy of the

bilateral cerebellar hemispheres and vermis (black arrows) and corpus callosum (white arrowhead). Susceptibility-weighted images (d and e) demonstrate old blood products within the lateral ventricles, left caudothalamic groove, and residual cerebellum (white arrows). These findings are compatible with sequela of prior grade IV intraventricular hemorrhage and cerebellar germinal matrix hemorrhage

grade II, GMH with IVH; grade III, GMH with IVH and ventriculomegaly; and grade IV: hemorrhagic periventricular venous infarction (Fig. 4).

represents the underlying etiology for congenital porencephalic cysts. The acquired variety may result following trauma, surgery, vascular insult, or infection. Familial porencephaly is a rare autosomal dominant condition characterized by mutation of the gene encoding procollagen type IV A1 (chromosome 13q, COL4A1), essential for basement membrane stability (Breedveld et al. 2006), with resultant increase in risk of intracerebral hemorrhage. Inherited thrombophilia, most often secondary to heterozygosity for factor V Leiden mutation (gene F5), also predisposes to porencephalic cysts (Fig. 5).

Porencephalic Cyst Porencephaly is defined as a congenital or acquired CSF-filled parenchymal cavity that usually communicates with the ventricular system and/or subarachnoid space and is lined by reactive gliosis/astrocytic proliferation. In utero encephaloclastic process caused by cerebral vascular insult or infectious injury (CMV)

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Fig. 5 10-year-old female, born preterm at 24 weeks of gestation with obstetric history significant for placental abruption. Axial (a) and coronal (b) T2-weighted and axial FLAIR-weighted (c) images demonstrate a large fluid-filled cyst in the left cerebral hemisphere, in the frontoparietal region corresponding to the left middle cerebral artery territory. This cyst is lined by white matter

(white arrows) (distinct from gray matter-lined open-lip schizencephaly). Postcontrast sagittal T1-weighted image (d) demonstrates gracile corpus callosum. Findings are most consistent with a porencephalic cyst, typically due to encephaloclastic insult (e.g., intrauterine infections or ischemia)

Term Infants

ischemic insult in term infants with prolonged partial hypoxia, and injury manifests as restricted diffusion in watershed territories, involving both the cortex and subcortical white matter. Children with the watershed predominant pattern of injury may develop symptomatic parieto-occipital epilepsy in late childhood, low

Watershed Predominant Pattern of Injury The watershed zones between anterior and middle cerebral arteries and middle and posterior cerebral arteries are most predisposed to

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Fig. 6 9-year-old male with diplegic cerebral palsy. History of hypoxic-ischemic insult at 3 years of age secondary to atypical refractory Kawasaki’s disease. Axial T2 (a and b) and axial FLAIR (c and d) images through the lateral ventricles and posterior fossa demonstrate generalized cerebral and cerebellar atrophy. Abnormal T2/FLAIR

hyperintense signal is visualized in the periventricular white matter of both occipital lobes, extending to the left occipital pole (white arrow). Additionally, there is signal abnormality involving the cerebellar cortex (arrowhead). Findings are most consistent with sequela of profound hypoxic-ischemic insult

IQ, and visuospatial cognitive dysfunction (Oguni et al. 2008) (Figs. 6 and 7).

prolapsed cord (Okereafor et al. 2008; Oguni et al. 2008). Children with the basal ganglia/ thalamic pattern of injury tend to be severely disabled due to dyskinetic cerebral palsy (de Vries and Groenendaal 2010). In extremely severe profound hypoxic-ischemic injury, all of the supratentorial structures may be affected (Fig. 8).

Basal Ganglia/Thalamus Pattern This pattern is also referred to as a pattern following acute near total asphyxia and is most often seen ensuing an acute sentinel event such as uterine rupture, placental abruption, or a

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Fig. 7 10-year-old male with incontinentia pigmenti and spastic hemiplegic cerebral palsy. Coronal T2 (a) and T1 (b) weighted images demonstrate right-sided cerebral volume loss as demonstrated by prominence of cerebral convexity sulci and lateral ventricle (black star). Also, there is diffuse abnormal T2/FLAIR hyperintense signal involving

the right cerebral white matter (white arrow), thalamus and posterior limb of internal capsule (axial FLAIR-weighted images, c and d). There are additional areas of abnormal hyperintense signal in the left frontal and parietal white matter (white arrowhead). These findings are most compatible with sequela of remote hypoxic-ischemic insult

Perinatal Stroke Ischemic perinatal stroke is defined as “a group of heterogeneous conditions in which there is a focal disruption of cerebral blood flow secondary to arterial or cerebral venous thrombosis, or embolization between 20 weeks of fetal life through 28th postnatal day, confirmed by neuroimaging or neuropathologic studies” (Machado

et al. 2015). In the newborn, unlike the older child or adult, there are no clinical signs that permit even a presumptive diagnosis of perinatal stroke; diagnosis rests on neuroimaging (Nelson 2007). Neonates and young infants usually present with encephalopathy and/or seizures. Perinatal stroke is the commonest cause of hemiplegic cerebral palsy (Basu 2014) and has a prevalence

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Fig. 8 7-year-old female, born at 32 weeks of gestation with mixed spastic and dystonia cerebral palsy, secondary to perinatal hypoxic-ischemic insult related to fetal hydrops. Obstetric history was significant for preeclampsia. Axial T2-weighted (a), axial FLAIR-weighted (b), and coronal T2-weighted (c) images demonstrate abnormal

of 1 in 2300 to 5000 live births (Barkovich 2012), often leading to long-term neurological disability, including congenital hemiplegia, seizure, and cognitive disorders, and has an extremely variable presentation depending upon the age, cause, and involved vascular territory (Lanni et al. 2011) (Figs. 9 and 10).

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hyperintense signal in the bilateral posterior putamina and thalami (white arrows), with associated volume loss in keeping with sequela of profound hypoxic-ischemic insult. Of note is subtle hyperintense signal in the periventricular white matter with mild white matter volume loss

Congenital Infections of the Central Nervous System Transplacental infection of the fetus during development can lead to a wide spectrum of insults to the brain. The most important set of

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Fig. 9 11-month-old female, born by Cesarean section at 38 weeks of gestation for failure of progression of labor and preeclampsia, with left-sided hemiplegic cerebral palsy. Axial FLAIR-weighted images (a and b) demonstrate abnormal hyperintense signal compatible with gliosis with a small area of encephalomalacia (white arrows) within the right corona radiata extending along the posterior limb of internal capsule. Axial T1-weighted

image (c) shows corresponding T1 hypointense signal (white arrow). Additional signal abnormality is visualized in the right posterior putamen and lateral thalamus (d, coronal T2-weighted image, white arrow). Findings are compatible with remote infarction in right middle cerebral artery territory. Of note is mild ex vacuo enlargement of right lateral ventricle

these infections are represented by the TORCH acronym, denoting Toxoplasma gondii, rubella, Cytomegalovirus, herpesviruses, and syphilis (Hedlund et al. 2012). This list has been expanded to also include lymphocytic choriomeningitis virus, varicella zoster,

parvovirus B19, and recently Zika virus (Ribeiro et al. 2017). Although the different infectious agents can produce specific clinical and imaging features, the most important factor in the type and degree of brain injury is the fetal gestational age at the time of infection. Infections during the

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first and second trimester typically cause developmental brain abnormalities due to disruption of neuronal migration, while infections in the third trimester may cause focal destructive lesions or only minor problems such as hearing impairment. Fetal ultrasound is the first line of imaging in congenital CNS infections and may provide the first evidence of infection. Fetal and

Fig. 10 (continued)

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postnatal MRI and postnatal CT offer greater detail of brain involvement.

Cytomegalovirus Cytomegalovirus (CMV) is an endemic herpesvirus spread by close human-to-human contact with

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Fig. 10 5-year-old male with spastic quadriplegic cerebral palsy, hemophagocytic lymphohistiocytosis, and juvenile xanthogranuloma. Postnatal history was significant for respiratory distress, anemia, thrombocytopenia, and direct hyperbilirubinemia. Serial imaging demonstrates evolution of hypoxic-ischemic insult. Initial imaging at 6 days of age: Axial diffusion-weighted (a, b) and apparent diffusion coefficient (c, d) images through the centrum semiovale and basal ganglia depict extensive restricted diffusion in the bilateral cerebral hemispheres, basal ganglia, and thalami. Axial T2-weighted image (e) demonstrates corresponding abnormal hyperintense signal in the regions of restricted diffusion. There is gyriform T1 hyperintense signal in the bilateral frontoparietal lobes (f, axial T1-weighted image, white arrows). Findings are most consistent with severe prolonged hypoxic-ischemic insult. Follow-up imaging at 5 weeks of age: The regions

demonstrating restricted diffusion have evolved into extensive areas of cystic encephalomalacia in the bilateral cerebral hemispheres (g and h, axial FLAIR-weighted images, i, parasagittal T2-weighted image). There are multiple foci of hyperintense signal on T1-weighted (j) and hypointense signal on susceptibility-weighted (k) imaging, compatible with remote blood products. The regions of encephalomalacia appear T1 hypointense. Imaging at 18 months of age: Further evolution of regions of cystic encephalomalacia, including progressive confluence of encephalomalacia in the right frontal lobe, with volume loss of the bilateral cerebral hemispheres (l, axial FLAIRweighted and m, axial T2-weighted images). Again, visualized are multiple foci of hypointense signal on susceptibility-weighted imaging (n) in the bilateral parieto-occipital regions in keeping with remote blood products

bodily fluids and can be identified in 0.6 to 0.7% of all live births (Colugnati et al. 2007). CMV is spread to the fetus transplacentally and poses the greatest risk to a developing fetus in the first or second trimester, especially in mothers

who acquire a primary infection during pregnancy. There is a wide range of outcomes with congenital CMV infection, with most children asymptomatic at birth. However, congenital CMV infection can cause sensorineural hearing

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loss, visual impairment, developmental delay, cognitive impairment, seizures, and cerebral palsy (Malm and Engman 2007). Neuroimaging, both pre- and postnatally, provides important diagnostic and prognostic information in congenital CMV infection. Ventriculomegaly, microcephaly, or periventricular calcifications detected on prenatal ultrasound may be the first indication of infection. Fetal MRI provides greater detail of brain involvement, allowing increased sensitivity and positive predictive value of long-term complications (Doneda et al. 2010). Anterior temporal polar cysts, intraventricular septa, cerebellar hypoplasia or dysplasia, cortical migration anomalies, and white matter abnormalities are features well depicted with fetal MRI (Averill et al. 2015). In fact, the characteristic but nonspecific anterior temporal lesions and intraventricular septations are often more pronounced in the fetus compared to older children. These features should be specifically evaluated with MRI in a child with unexplained developmental delay and hearing loss. Likewise, these areas should be given special scrutiny in an MRI scan of a child showing polymicrogyria, as the diagnosis of congenital CMV infection may curtail unnecessary lengthy and expensive genetic testing. Ultrasound and CT more easily depict the classic periventricular calcifications seen in approximately half of the cases (Fink et al. 2010) (Fig. 11).

Toxoplasmosis Toxoplasmosis, caused by the parasite Toxoplasma gondii, is the second most common congenital infection after CMV (Hedlund et al. 2012). T. gondii infects birds and mammals, with domestic cats implicated as the major source of human infection. Although most infections are asymptomatic, transplacental infection, especially before 20 weeks’ gestation, can cause severe neurological complications including microcephaly, hydrocephalus, quadriplegia or diplegia, cognitive impairment, and seizures (Diebler et al.

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1985; Jeong et al. 2015). Chorioretinitis is the most consistent feature of symptomatic congenital toxoplasmosis and can manifest at birth or years later (Safadi et al. 2003). The hallmark calcifications of toxoplasmosis are depicted well with pre- and postnatal ultrasound as well as CT and susceptibility-weighted MRI, usually involving the basal ganglia, periventricular and subcortical white matter, and cortex, occasionally taking on a tram track appearance (Surendrababu et al. 2006). These calcifications can decrease or resolve with treatment, corresponding to clinical improvement (Patel et al. 1996). Prenatal ultrasound may show macrocephaly due to hydrocephalus, although microcephaly is also seen. The combined sonographic features of hydrocephalus and parenchymal calcifications are a poor prognostic sign (Malinger et al. 2011). Extensive brain parenchymal destruction with porencephaly or hydranencephaly can be seen in severe cases of congenital toxoplasmosis, but abnormalities of cortical migration are atypical, in contrast to congenital CMV (Hedlund et al. 2012) (Fig. 12).

Lymphocytic Choriomeningitis Virus Lymphocytic choriomeningitis virus (LCMV) is an arenavirus endemic in wild mice and can also be harbored in pet mice, guinea pigs, and hamsters (Bonthius 2012). The virus can be passed to humans coming in contact with rodent secretions, urine, feces, and their bedding causing a flu-like illness and transmitted through the placenta to the developing fetus. The gestational age at infection dictates the severity of brain abnormalities, with earlier infection causing more profound injury (Bonthius et al. 2007a). Long-term morbidity including cerebral palsy, cognitive impairment, seizures, and visual impairment is common, although the true spectrum of disease is not known (Bonthius et al. 2007b). Congenital LCMV infection is thought to be uncommon but also under recognized (Delaine et al. 2017; Schulte et al. 2006). It should be suspected in infants with chorioretinitis in

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Fig. 11 Fetus at 37 weeks’ gestation with congenital cytomegalovirus (CMV) infection. Fetal brain MRI was performed for possible arachnoid cyst seen on prenatal ultrasound. Parasagittal T2 MR image (a) shows cystic dilation of the occipital and temporal horns (white arrows) of the lateral ventricles with a thin septation (curved white arrow). Axial T2-weighted image (b) shows bilateral anterior temporal lobe cysts with T2 hyperintensity in the surrounding white matter (dashed white arrow), near completely resolved by 7 months of age (image d). Postnatal axial T2-weighted image (c) performed at 7 months

of age demonstrates polymicrogyria in the bilateral frontal (black arrows) and perisylvian regions more easily seen than on the fetal MRI. Also note white matter hyperintensity involving the bilateral frontal lobes (dashed black arrows). An axial T2-weighted image (d) through the posterior fossa additionally shows mild cerebellar dysplasia (open arrows), well depicted postnatally but only subtly visible prenatally (b). The constellation of above features is highly suggestive of early in utero timing of CMV infection

association with hydrocephalus or microcephaly, who test negative for the more commonly encountered CMV and toxoplasmosis infections (Hedlund et al. 2012, Anderson et al. 2014).

LCMV has strong tropism for neuroblasts, leading to periventricular calcifications, periventricular cysts, and neuronal migration abnormalities similar to congenital CMV infection (Wright 1997;

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Fig. 12 Premature infant born at 33 weeks gestation with increasing head circumference, hyperbilirubinemia, and metabolic acidosis. Coronal (a) and sagittal (b) ultrasound, sagittal fast imaging employing steady-state acquisition (FIESTA) MRI (c and d) performed in the newborn period, and axial T2-weighted MRI (e) performed at 6 months of age. Imaging shows moderate hydrocephalus including lateral ventricles and the third ventricle (*), but no significant enlargement of the fourth ventricle. FIESTA images demonstrate obstruction at the level of the aqueduct of Sylvius (white arrow) likely secondary to ependymitis.

Bonthius 2012; Anderson et al. 2014). Cerebellar hypoplasia and porencephalic cysts can also be seen (Bonthius et al. 2007b). Communicating hydrocephalus, similar to toxoplasmosis, is seen in more than 50% of affected neonates (Hedlund et al. 2012; Bonthius et al. 2007b). Those without hydrocephalus often have microcephaly due to poor brain development (Delaine et al. 2017). Different from other TORCH infections, though, LCMV typically has few systemic effects in the rest of the body (Fig. 13).

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Subcortical cysts are noted on ultrasound, a few of them hyperechoic (curved white arrows), and MR shows hypointensity within the cysts (black arrow) likely representing calcification/hemorrhage. Diffuse hyperintense T2 signal within the cerebral white matter, likely representing cerebral edema and delayed myelination (dashed arrow). Abnormally shaped globes bilaterally with retinal detachment, secondary to chorioretinitis (open arrow). Significant progression of white matter volume loss and atrophy is seen on MRI performed at 6 months of age (e). Serology testing was positive for toxoplasma

Congenital Malformations The prevalence of congenital anomalies in children with CP is higher than in the general population, with majority being malformations of cerebrum such as microcephaly, hydrocephaly, and schizencephaly (Garne et al. 2008; MacLennan et al. 2015). Also, there is increased prevalence of noncerebral malformations such as cardiac, musculoskeletal, and renal (MacLennan

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Fig. 13 4-day-old infant with microcephaly and sensorineural hearing loss. Coronal ultrasound (a), axial CT (b), axial (c), and coronal (d) T2-weighted MR images demonstrate moderate hydrocephalus (*) secondary to aqueductal obstruction likely from necrotizing ependymitis. Periventricular calcifications are shown on CT and ultrasound (straight arrows). MRI demonstrates abnormal sulcation of the posterior frontal and perisylvian

regions (curved arrows) with underlying polymicrogyria. There is also diffuse white matter volume loss (black arrows). Imaging studies of neonates and infants with lymphocytic choriomeningitis virus (LCMV) are nearly identical to those of congenital CMV and toxoplasmosis. Diagnosis of LCMV was made by serologic studies in this child

et al. 2015). According to the data from 11 CP registries contributing to the European Cerebral Palsy Database, 11.9% of children with CP were reported to have a congenital malformation, with majority (8.6%) were diagnosed with a cerebral malformation (Garne et al. 2008).

Lissencephaly (The Agyria-Pachygyria Complex) Lissencephaly encompasses malformations caused by arrested neuronal migration, resulting in thick four-layer cortex and smooth brain

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surface. Both agyria and pachygyria are likely caused by abnormal regulation of microtubule activities. A variety of gene alterations are implicated in the pathogenesis such as the LIS1 (17p13.3) responsible for regulating microtubule motor protein cytoplasmic dynein (Toba et al. 2012); DCX (Xq22.3–q23) encoding for doublecortin, a neuronal microtubule-associated protein involved in cell division and/or cell migration (Bahi-Buisson et al. 2013); RELN (7q22) encoding for reelin, an extracellular matrix protein that regulates neuronal migration and synaptic plasticity; ARX (Xp21.1, homeobox-containing gene); and TUBA1A (12q12–q14.3) encoding for microtubule constituent protein (Okumura et al. 2013). Microcephaly is seen in lissencephaly associated with ARX, RELN, and TUBA1A mutations. Miller-Dieker syndrome results from large LIS1 deletions and includes, in addition to classic lissencephaly, craniofacial, cardiac, and renal anomalies and omphalocele (Chih-Ping Chen and Shu-Chin Chien 2010). Characteristic imaging findings of lissencephaly include absence or diminished number of cortical sulci throughout the cerebral hemisphere with thick cortex, hourglass or figure of 8 shape of cerebral hemispheres, and truncated arborization of white matter. T2-weighted imaging in neonates may demonstrate three discrete layers of the cortex, including relatively thin and smooth, outer cellular layer, deeper thick layer of arrested neurons mimicking band heterotopia, and intervening cell-sparse layer (Barkovich et al. 1991) (Fig. 14).

Microcephaly with Simplified Gyral Pattern (MSG) Microcephaly with simplified gyral pattern (MSG) describes malformations associated with microcephaly (head circumference of 3 or more standard deviations below the mean) and too few and abnormally shallow sulci ( 0.80 Adolescent: 4/5 domains with internal reliability coefficient > 0.80 (Daltroy et al. 1998) Good test/retest reliability in parents Good inter-rater reliability coefficients between parents and other caregivers (>0.80) (Novacheck et al. 2000) Excellent intra and inter observer reliability (Davids et al. 2006)

Validated in CP, good to excellent validity across four domains (Dumas and Fragala-Pinkham 2012; Haley et al. 2010; Shore et al. 2017).

Validated for CP (McCarthy et al. 2002)

Validated for CP (Davids et al. 2006)

Validated for CP (Novacheck et al. 2000)

Validity Validated for CP (Daltroy et al. 1998; Wren et al. 2007; McCarthy et al. 2002)

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(McCarthy et al. 2002; Wren et al. 2007). Importantly, it is also used as an instrument to evaluate the effectiveness of surgical interventions (McCarthy et al. 2002). Compared to other similar instruments, the PODCI assesses more advanced functions such as sports and outdoor play (Damiano et al. 2005). It is, therefore, a good instrument for orthopedic interventions, as these interventions typically seek to improve higherlevel motor function but demonstrates floor and ceiling effects in children with CP who demonstrate greater functional deficits (Vitale et al. 2001). However, because it does include satisfaction and expectations as part of the assessment, the PODCI is not a purely functional outcome assessment. The instrument, therefore, seeks to assess a child and parent’s overall satisfaction with their treatment, not simply motor functional assessment.

Gillette Functional Assessment Questionnaire (FAQ) The Gillette Functional Assessment Questionnaire (FAQ) is a child or parent reported questionnaire that assesses functional ability in ambulatory children with CP (GMFCS I-III). The instrument includes a ten-level classification of ambulatory ability coupled with 22 other higher level functional activities rated on a five-point Likert scale (Gorton et al. 2011). The FAQ is validated for patients with CP, describes ambulatory function, and can monitor change after intervention (Oeffinger et al. 2007; Stout et al. 2008). Importantly, the FAQ only assesses the domain of functional ability, without evaluation of happiness, pain, or expectations (Novacheck et al. 2000). The FAQ seeks to assess the child’s community ambulatory status, which is often difficult to assess in the clinical setting. Clinic and gait lab environments often have a simple, even terrain, allowing providers to see the child at their “best.” This may not be reflective of the child’s function in their community. The FAQ provides insight into the child’s function in the community, with all of its challenges of uneven ground, crowds, tight quarters, and obstacles.

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Shriner’s Hospital Upper Extremity Evaluation (SHUEE) The SHUEE is a validated instrument for evaluation of upper extremity function in children with CP (Davids et al. 2006; Smitherman et al. 2011). The SHUEE differs from other functional measures in that it is a video-based instrument. It is performed by an occupational therapist with a standardized set of tasks and standardized camera position. The study has duration of 15 min and requires administration and scoring by a qualified occupational therapist. Unlike many other functional outcome measures, the SHUEE does not rely solely on patient or parent recall but rather real-time functional performance. While there is a history-based assessment of the child’s ability to perform activities of daily living, a significant component of the instrument is direct observation of how the child uses the involved extremity. The instrument measures the active and passive range of motion from the shoulder to the fingers. It also evaluates the spontaneous use of the extremity while performing specific tasks on demand. Finally, the instrument measures the patient’s ability to grasp and release the digits with the wrist held in flexion, neutral, and extension (Davids et al. 2006). The video recorded component of the assessment can be a useful part of the medical record, particularly when comparing preand postintervention function.

Pediatric Evaluation of Disability Inventory (PEDI) and Pediatric Evaluation of Disability Inventory Computer-Adaptive Test (PEDI-CAT) The PEDI functional outcome instrument evaluates three functional domains including Self-care (Daily Activities), Mobility, and Social Function. It is meant for children with disabilities between the ages of 6 months to 7 years. With more than 200 items, it requires 30–60 min to administer. It is meant to be completed by a proxy, such as a parent or therapist (Feldman et al. 1990; McCarthy et al. 2002). Items on the PEDI are generally easier in content, and while good for evaluation of children

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with moderate or severe disability, the instrument demonstrates a ceiling effect with children who demonstrate higher function (Shore et al. 2017). Released in 2012, the PEDI-CAT is an update of the PEDI instrument for children from birth to 21 years of age. The PEDI-CAT is a computeradaptive test (CAT) that requires no special equipment other than software installed on a computer or tablet. It requires no physical testing. The PEDI-CAT evaluates ability in the same three functional areas as the PEDI (Daily Activities, Mobility, and Social/Cognitive) but also has a fourth Responsibility domain that reports the child’s participation and amount of responsibility assumed for activities of daily living. The CAT platform of the PEDI-CAT was built with a set of 218 coordinated items (item banks) based on functional ability of children with CP and seeks to describe the unique ability of the individual child. Parents are first asked a question, which represents a task from the middle of an ability range, and then further questions are directed according to how the parents answer this first question according to their child’s ability level. Parents are asked to answer questions about their child’s functional ability on a four-point rating scale (unable, hard, a little hard, and easy) (Haley et al. 2010). Both the PEDI and PEDI-CAT are validated in children with CP. The PEDI has demonstrated good concurrent validity when compared to other functional instruments (McCarthy et al. 2002; Han et al. 2011). Similarly, the PEDI-CAT correlates well with previously validated instruments in patients with CP and is able to differentiate across both fine and gross motor functional levels (Dumas and Fragala-Pinkham 2012; Dumas et al. 2015, 2017; Shore et al. 2017). The PEDI-CAT is a useful outcome instrument that can be used for any school age child with a wide spectrum of disability.

Quality of Life/Health-Related Quality of Life Measures Health related quality of life (HRQOL) is a multidimensional theoretical construct consisting of three broad domains: physical, psychological,

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and social well-being. The physical domain includes the impact of illness on functioning, the psychological domain includes coping and adaptation, and the social domain incorporates the individual’s relationship with family and friends (Berzon 1998; Cooley 1998; Taylor et al. 2008). HRQOL and quality of life (QOL) are terms that are sometimes used interchangeably. However, QOL is a much broader construct that includes aspects of life that are not easily changed by health care services, such as social interactions with peers and family relationships (Varni and Limbers 2009). In children with CP, HRQOL measurements can be used to track changes over time and/or determine the effects of specific interventions on a child’s HRQOL. Improvements in HRQOL should be considered one of the primary goals in the management and treatment of children with CP. Measuring HRQOL in children with CP is particularly challenging since these children demonstrate a wide range of functional and cognitive abilities (Vitale et al. 2005). A limited number of HRQOL instruments exist to measure outcomes in children with CP and a gold standard does not exist. The Pediatric outcomes data collection instrument (PODCI) (Daltroy et al. 1998) and the Child Health Questionnaire (McCullough and Parkes 2008) were designed to measure functional status which does not capture the multidimensionality of the HRQOL. Early researchers believed that physical functioning played a major role in the child’s HRQOL and therefore this became the primary focus of early outcomes studies in children with CP. This idea has since proven to be inaccurate in that adolescents perception of their life is not solely based on their physical functioning; therefore, studies using the PODCI and CHQ are not good indicators of HRQOL (Rosenbaum et al. 2007; Shelly et al. 2008). HRQOL has remained largely unmeasured due to limitations in measurement. Four diseasespecific, psychometrically sound, and validated instruments have been identified to measure the impact of having CP on HRQOL in children and adolescents: Cerebral Palsy Quality of Life Questionnaire (CP-QOL-Child and -Teen) (Waters et al. 2007), Caregivers Priorities and Child

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Health Index of Life with Disabilities (CPCHILD) (Narayanan et al. 2006), Pediatric Quality of Life Inventory 3.0 Cerebral Palsy Module (PedsQLCP) (Varni et al. 2006), and the DISABKIDS (Mueller-Godeffroy et al. 2016). Please refer to Table 2 for a summary of these instruments.

Cerebral Palsy Quality of Life Questionnaire (CP-QOL-Child) The Cerebral Palsy Quality of Life Questionnaire (CP-QOL-Child) (Waters et al. 2007) was designed to assess quality of life changes in children with CP with a focus on well-being rather than illness. The CP-QOL-Child has child selfreport for children ages 9–12 years and parent proxy-report for children ages 4–12 years. The self-report measure has 55 items while the parent proxy report has 66 items. Items related to service access and primary caregiver health are only included in the parent proxy measure. This measure assesses seven aspects of quality of life: (1) Social Well-Being and acceptance (11 items); (2) Functioning (12 items); (3) Participation and Well-being (6 items); (4) Emotional Well-Being (6 items); (5) Access to Services (5 items); (6) Pain and Impact of Disability (8 items); and (7) Family Health (4 items). The measure was developed based on the International Classification of Function (ICF) (WHO 2001) and the definition of quality of life. The questions ask how the child feels about his/her life and how the parent proxy feels about their child’s life in terms of family, friends, health, and school. The vast majority of the questions begin with: “How do you think your child feels about. . ..” or “How do you feel about. . ..” Therefore, the focus of the questions is on how the child feels and not what they can do. Reponses are on a 9-point rating scale ranging from 1 = very happy to 9 = very unhappy. Total scores can range from 0 to 100 with 100 representing a higher HRQOL (Waters et al. 2007). The CP-QOL-Child has been used to measure quality of life in children with cerebral palsy after single-event multilevel surgery. Both self-report and parent proxy CP-QOL-Child measures were

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administered pre- and postoperatively. High quality of life scores were reported by the children following SEMLS, which were significantly higher than their parent’s scores ( p < 0.05). Significant differences ( p < 0.05) between GMFCS level III and levels IV–V were identified in the functional-related domains. No significant changes were noted in the socioemotional domains (Himpens et al. 2013).

Cerebral Palsy Quality of Life Questionnaire (CP-QOL-Teen) The CP-QOL-Teen is a measure of QOL designed for teens with CP. It consists of adolescent selfreport and parent proxy measures. The self-report measure has 72 items and the parent proxy measure has 17 additional items related to access to services and caregiver health for a total of 89 items. The questionnaire has seven domains: (1) General Well-Being and Participation (21 items); (2) Feelings about Functioning (5 items); (3) Communication and Physical Health (16 items); (4) School Well-being (7 items); (5) Access to Services (9 items); (6) Social Well-being (7 items); (7) Family Health and Well-being (4 items). Each of the items is worded the same as the CP-QOL-Child questionnaire. Reponses are on a 9-point rating scale ranging from 1 = very happy to 9 = very unhappy. Total scores can range from 0 to 100 with 100 representing a higher HRQOL (Davis et al. 2013). At the time of publication of this book, no published outcomes studies utilizing the CP-QOL-Teen measure could be identified.

Caregivers Priorities and Child Health Index of Life with Disabilities (CPCHILD) The CPCHILD was designed to measure HRQOL in children with severe nonambulatory CP (Narayanan et al. 2006). It was developed specifically to measure the effectiveness of interventions in children with GMFCS level IV–V CP. The CPCHILD has parent proxy and child

Pediatric QOL Inventory (PedsQL) CP Module (Varni et al. 2006)

Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) (Narayanan et al. 2006)

DISABKIDS (Baars et al. 2005)

Measure Cerebral Palsy Quality of Life Questionnaire (CP QOL-Child) (Waters et al. 2007)

Australia

International Australia, France, Germany, Greece, Holland, Sweden Canada

Country of origin United Kingdom

5–18 2–18

2–18

4–16 4–16

Age range 9–12 4–12

Self-report Parent proxy

Parent proxy Questionnaire used only in children with severe CP (GMFCS IV-V)

Self-report Parent proxy

Respondent Self-report Parent proxy

Table 2 Disease-specific quality of life/health-related quality of life measures

7: daily activities, school activities, movement and balance, pain and hurt, fatigue, eating activities, speech and communication

6: personal care, positioning, transfers and mobility, communication and social interaction, comfort, emotions and behavior, health and overall QOL

6: Medication, limitation, emotion, independence, social inclusion, social exclusion

Domains 4: physical well-being, social well-being, emotional wellbeing, acceptance by others

35

36

37

# Items 52

Parent proxy: internal consistency (0.7 or > in all domains) Test-retest (0.88–0.96) Self-report: internal consistency (0.63–0.93) Parentproxy: internal consistency (0.88–0.96)

Reliability Self-report: Not completed Parent Proxy: Cronbach Alpha 0.74–0.89 Test-Re-test (0.76–0.89) Internal consistency with acceptable results

Parent-proxy: correlations between the CP module and generic core scale were varied ranging from weak (0.14–0.23) to moderate (0.40–0.43) and significant (90.52–0.84)

Parent proxy: correlated with related instruments

Validity Self-report: not completed Parent proxy: moderately correlated with the KIDSCREEN (r = 0.30–0.57) Moderately correlated with the Global QOL (r = 0.18–0.58) and global health (r = 0.21–0.56)

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self-report measures for children and youths ages 3–18 years. The measure consists of 37 items, which are categorized into seven different domains: (1) Activities of daily living (9 items); (2) Positioning, transferring, and mobility (8 items); (3) Comfort and emotions (9 items); (4) Communication and social interaction (7 items); (5) Health (3 items); and (6) Overall quality of life (1 item). The last domain, #7, examines the importance of each item to the child’s quality of life (36 items). The caregivers are asked to rate how difficult each of the listed activities were in the past 2 weeks. Activities included such things as toileting, getting in and out of bed, and sitting in a wheelchair. In addition, caregivers are asked to choose the level of assistance that was required to help their child perform these activities. Items are rated on a 6-point ordinal scale. For items related to activities, a “level of assistance” modifier is included, a 4-point ordinal scale from independent (0) to total assistance (3). For items related to symptoms, a 3-point ordinal “level of intensity” scale was added with (0) being severe and (3) being none. Scores are reported for each domain and in total with 0 being the worst and 100 being the best (Narayanan et al. 2006). The CPCHILD has demonstrated the ability to detect changes in HRQOL in children with severe cerebral palsy who received oral modafinil for spasticity reduction (Murphy et al. 2008) and following a spinal fusion (Narayanan et al. 2011). The English version has been translated into: Bahasa Malaysian, Brazilian Portuguese, Canadian French, Danish, Dutch, Farsi, German, Hebrew, Korean, Norwegian, Spanish, and Swedish. The CPCHILD has been used to demonstrate change after spinal and hip surgery in nonambulant children with CP. In a prospective longitudinal study, the CPCHILD was found to relate to the preoperative migration percentage. A negative correlation was detected between the preoperative migration percentage and the preoperative CPCHILD score (r = 0.50; p = 0.002). The preoperative CPCHILD total scores differed between the migration-percentile groups (mean difference = 13 points; 95% confidence interval = 3.3 to 22.8; p = 0.01). However, after hip surgery, the CPCHILD score improved similarly

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for both groups (DiFazio et al. 2016). This relationship continued throughout the follow-up period. Difazio et al. (2017) demonstrated that following spinal fusion, there was a significant improvement in CPCHILD scores 1 year after surgical intervention; however, at 2 years, those scores returned to the baseline.

Pediatric Quality of Life Inventory (PedsQL) 3.0 Cerebral Palsy (CP) Module The PedsQL is an instrument designed to measure HRQOL in children and adolescents with CP (Varni et al. 2006). It has child self-report for children ages 5–18 years and parent proxy-report for children ages 2–18 years. The PedsQL is selfadministered for children ages 8–18 years and is interviewer-administered for children ages 5–7 years. It has 35 items encompassing 7 scales: (1) Daily Activities (9 items); (2) School Activities (4 items); (3) Movement and Balance (5 items); (4) Pain and Hurt (4 items); (5) Fatigue (4 items); (6) Eating Activities (5 items); and (7) Speech and Communication (4 items). The instructions ask the parent or child to report how much of a problem each of the items has been during the past month. A 5-point Likert scale is used with responses ranging from 0 = never a problem to 4 = almost always a problem. The response options are simplified for the young child self-report (ages 5–7 years) and include only three options. Scores can range from 0 to 100 with a higher score indicating a better HRQOL (Varni et al. 2006). There is no data published on responsiveness to change following an intervention. In addition, there is an overemphasis on the functional domains, which may limit its ability to measure all aspects of quality of life. The instrument has been translated into numerous languages.

DISABKIDS-CP Module (CPM) The DISABKIDS CP module was designed to measure HRQOL in children and youths who

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suffer from chronic health conditions such as CP (Baars et al. 2005; Mueller-Godeffroy et al. 2016). The instruments are available as self- or proxy report in children between the ages of 4–16 years. The CP module was intended to be used in conjunction with the 37-item DISABKIDS chronic generic module (DCGM37), which assesses 6 domains: (1) Independence; (2) Physical limitations; (3) Emotion; (4) Social inclusion; (5) Social exclusion; and (6) Treatment (Simeoni et al. 2007). The DISABKIDS CP module has two specific domains: (1) Impact of the condition (8 Items) and (2) Communication (2 items). The impact domain asks questions about difficulty with walking or other physical activities such as swimming, getting dressed, sports, and climbing stairs. The communication domain asks questions about the impact of the condition on the child’s ability to communicate with others. Responses are in a Likert-scale format. Scores can range from 0 to 100 with higher scores indicating better adjustment to CP (Mueller-Godeffroy et al. 2016, translated into Dutch, English, French, German, Greek, Swedish, and Norwegian). All of these measures have both self-report and parent proxy versions. Each measure focuses on different aspects of the child’s physical and psychological well-being. Evaluation of these instruments demonstrates sound psychometric properties with acceptable reliability and validity and responsiveness to change.

Conclusion An increasing demand for evidence-based decision-making has challenged the medical community to clearly demonstrate which treatments translate to functional and clinical improvements in a patient’s environment. Outcome tools are designed to measure functional performance, as a baseline descriptive assessment, select treatment goals, and to evaluate treatment. Selecting the appropriate outcome instrument depends on many factors including: the specific research question, type of intervention to be evaluated, the instrument’s psychometric properties, the

C. J. Watkins et al.

construct/theoretical underpinnings that the instrument measures, age of children, and patient versus parent proxy versions to name a few. No single instrument can be used to measure all of the components of the ICF. Therefore, multiple measures may be the most appropriate. The goal moving forward is to demonstrate responsiveness of the outcome measures we use so that we accurately assess change after interventions in children with CP. Outcomes measurement is complex in children with CP. Ideally, therapists, researchers, and orthopedic surgeons hoping to assess HRQOL in children with CP should have a choice of valid, reliable, easy to administer, low-cost instruments, suited to the cultural and societal background of the children involved. Outcome measurement allows providers to measure a child’s abilities before and after treatments and track changes over time. When applied to clinical practice, the collection of outcome instruments can be thought of as a toolbox that contains the tools (the measures that reflect the core set items) that the clinician can use to evaluate the effect of the task at hand.

Cross-References ▶ Epidemiology of Cerebral Palsy ▶ Family Stress Associated with Cerebral Palsy ▶ Foot Deformities in Children with Cerebral Palsy: An Overview ▶ Functional ADL Training for Children and Youth with Cerebral Palsy ▶ Health and Healthcare Disparities in Children with Cerebral Palsy ▶ Hip Problems in Children with Cerebral Palsy: An Overview ▶ Muscle Performance in Children and Youth with Cerebral Palsy: Implications for Resistance Training ▶ Musculoskeletal Physiology Impacting Cerebral Palsy Gait ▶ Overview of Knee Problems in Cerebral Palsy ▶ Spinal Deformity in Children with Cerebral Palsy: An Overview

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▶ Therapy Management of the Child with Cerebral Palsy: an Overview ▶ The Upper Extremity in Cerebral Palsy: An Overview

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Biomarker Blood Tests for Cerebral Palsy

23

Robert E. Akins and Karyn G. Robinson

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339 Types and Classes of Biomarkers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 340 Basics of Diagnostic Biomarker Test Performance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 340 Circulating Biomarkers in the Blood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 341 Epigenetic Biomarkers and DNA Methylation in Blood Cells . . . . . . . . . . . . . . . . . . . . . 342 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345

Abstract

Together, the multiple types of cerebral palsy (CP) represent the most common physical disability in childhood with the spastic type accounting for ~77% of cases. CP arises with a disturbance in the brain, which in most cases occurs between 24 weeks gestation and birth. Early intervention is desirable, but early diagnosis is very challenging, and diagnosis is often delayed for several months or even years. No current molecular biomarker platform readily identifies individuals with CP, but screening assays that could measure blood biomarkers,

preferably collected at the time of birth, may allow for earlier diagnosis, intervention, and the development of novel therapeutics. Biomarkers that identify CP patients are needed, and multiple efforts to identify useful biomarkers in blood samples are underway. It is important to understand the nature of diagnostic biomarkers and the types of blood biomarkers being investigated in individuals with CP. Keywords

Cerebral palsy · Biomarker · Blood test

Introduction R. E. Akins (*) · K. G. Robinson Nemours Biomedical Research, Nemours – Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_211

Cerebral palsy (CP) arises from a disruption of brain development. In most cases, this disruption occurs during fetal life, and many children are 339

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born with CP (Hadders-Algra 2014; Nelson and Ellenberg 1986). Clinical intervention at an early stage is highly desirable in CP; (Hadders-Algra 2014; Spittle et al. 2015) unfortunately, it can take several years for a child to be diagnosed (Hubermann et al. 2016). Even with babies who are highly affected at birth, the diagnosis of CP doesn’t occur until months or sometimes years later (Hubermann et al. 2016; Maitre et al. 2013). Thus, although most individuals with CP are born with it, diagnostic challenges can delay therapy and services until after important therapeutic opportunities may have already been missed. Improving the ability to detect CP, especially at an early age so that interventions may begin earlier, would be a tremendous benefit to the personal health of individuals with CP as well as to their families and to the healthcare systems that provide their care. Among several strategies to help identify individuals with CP, significant effort has been exerted to find molecules in the blood that can serve as biomarkers. In this chapter, we discuss work toward the development of screening assays for CP based on blood biomarkers.

Types and Classes of Biomarkers Broadly defined, biomarkers are personal characteristics that can be measured and evaluated. Biomarkers can indicate physiological or pathological processes or a response to a therapeutic intervention. They may indicate things that are happening currently in an individual or may indicate things that happened in the past. Gene expression patterns, levels of a protein or metabolite in the blood or urine, blood pressure, and changes in brain activity seen by imaging are all examples of biomarkers. A working group of the US National Institutes of Health and Food and Drug Administration recognized seven classes of biomarkers based on usage (Group F-NBW 2016): 1. Diagnostic: Detects or confirms a disease or condition or a subtype of a disease. 2. Prognostic: Indicates the likelihood that a disease-related event or condition will occur.

R. E. Akins and K. G. Robinson

3. Monitoring: Used over time to assess the presence, status, or extent of a disease, medical condition, or the effects of a treatment. 4. Pharmacodynamic/response: Shows that a biological response has occurred after exposure to a specific agent. 5. Predictive: Identifies individuals who are likely to experience a known effect. 6. Safety: Indicates the likelihood, presence, or extent of toxicity after an exposure. 7. Susceptibility/risk: Indicates the potential for developing a disease or condition. In the context of CP, there are several types of biomarkers that are used or that may be useful, including brain imaging and movement assessments. Given the challenges associated with identifying individuals with CP early in life, potential diagnostic biomarkers have received significant attention.

Basics of Diagnostic Biomarker Test Performance The utility of a diagnostic test depends on how well it identifies individuals with a condition from others who do not have the condition. The performance of a diagnostic test can be quantified using some basic characteristics, including: 1. Accuracy: How often the diagnostic test gives the correct answer; often given as a percent. 2. Sensitivity: How often the diagnostic test gives a positive result when the individual has the condition. 3. Specificity: How often the diagnostic test gives a negative result when the individual does not have the condition. 4. Positive Predictive Value (PPV): The proportion of individuals with a positive test result who have the condition. 5. Negative Predictive Value (NPV): The proportion of individuals with a negative test result who do not have the condition. If a perfect diagnostic biomarker test were available, all patients with a condition would be

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Table 1 Example of results for a mock diagnostic biomarker study

Number of participants Positive biomarker test Negative biomarker test

Individual Individual does not has condition have condition Total 100 200 300 99 True positives (TP) 1 False negative (FN)

4 False positives (FP)

103

196 True negatives 197 (TN)

detected and no patients without the disease would be diagnosed. In practice, however, perfect biomarker tests are difficult to achieve, and understanding a test’s performance characteristics is critical. To evaluate a biomarker test, a reference diagnosis is needed for comparison. As shown in Table 1, comparison to a reference diagnosis allows the calculation of the true and false positive as well as true and false negative determinations by the biomarker test. The information from Table 1 allows calculation of performance characteristics for the mock validation study involving 300 participants. These characteristics also allow an estimation of how the test might perform if it were used in a clinical setting. For example, if the imaginary test were a diagnostic for a condition that occurs with the birth prevalence of CP (about 1 in 500 children) (Data and Statistics 2017; Key Findings 2017) and was applied to a group of 10,000, the expected results would be as shown in Table 2. Importantly, this example shows how the prevalence of the condition being assessed has a dramatic effect on the relative number of false positive results and on the predictive value of the diagnostic test. Also of importance, the specific conditions under which the diagnostic test can be employed are extremely crucial and can dramatically affect test performance. In addition, the cost, complexity, and reliability of the test are all important. Clear conditions for when the test is used, low cost, straightforward interpretation, and minimal variance when the test is performed at different times or clinical sites would all be expected to increase the usefulness of any diagnostic biomarker test.

Table 2 Illustrative comparisons between study and clinical performance data for a fictitious diagnostic test Study characteristics 300 99 1 96 4 98.3% 99.0% 98.0% 0.961

# Tests performed # True positive # False negative # True negative # False positive Accuracy Sensitivity Specificity Positive predictive value (PPV) Negative predictive 0.995 value (NPV)

Expected clinical characteristics 10,000 19.8 0.2 9780.4 199.6 98.0% 99.0% 98.0% 0.09025 0.99998

Circulating Biomarkers in the Blood The idea that factors circulating in umbilical cord blood may be associated with CP is an intriguing possibility that has been investigated by several groups (Costantine et al. 2011; Palatnik et al. 2015; Varner et al. 2015; Sorokin et al. 2014). In short, molecules that become elevated in the blood as a result of inflammation, infection, brain or neural injury, hypoxia, asphyxia, or ischemia, which are associated with the onset of CP, may be elevated in the cord blood of newborns at high risk of developing CP. Researchers have investigated the relationships between conditions like these and elevated blood levels of specific proteins. For example, one study looked at levels of S100 calcium binding protein B (S100B), neuron-specific enolase (NSE), and the soluble receptor for advanced glycation end-products (sRAGE) (Costantine et al. 2011); a study measured serum magnesium (Palatnik et al. 2015); another study examined the inflammatory cytokines interleukin-8 (IL-8), interleukin-1 beta (IL-1 β), and tumor necrosis factor alpha (TNF-α) (Varner et al. 2015); another looked at interleukin-6 (IL-6), C-reactive protein (CRP), and myeloperoxidase (MPO) (Sorokin et al. 2014). Unfortunately, despite the strong suspected relationships between the markers measured in these studies and CP, the levels of serum analytes measured did not yield significant

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associations with CP or with poor neurodevelopmental outcomes. Despite the lack of strong correlation with CP in the studies involving circulating biomarkers from cord blood so far, the possibility that such biomarkers can be developed is still being researched. The timing, levels, and patterns of circulating biomarker levels are very complex, and additional analytic work to resolve the possibility of using such levels in cord blood to identify newborns at high risk for developing CP is needed.

Epigenetic Biomarkers and DNA Methylation in Blood Cells Recent studies have examined the possibility that epigenetic biomarkers may be present in CP. Epigenetics refers to mechanisms that alter gene expression without affecting the primary sequence of the coding DNA. The three primary epigenetic alterations include DNA methylation, post-translational modification of histones, and effects mediated by small or microRNAs (miRNA) (Epigenetics and Public Health 2014). Both DNA methylation in blood cells (Crowgey et al. 2018; Mohandas et al. 2018; Yuan 2017) and circulating, cell-free miRNA (Chapman et al. 2018) have been investigated as potential biomarkers in CP. MicroRNAs are small (about 22 nucleotides), nearly ubiquitous, and under tight biosynthetic control (Treiber et al. 2018; Gebert and MacRae 2018). They perform critical regulatory functions under a variety of physiologic and pathologic conditions and can be found circulating in the blood either within exosomes or as free nucleic acids (Gebert and MacRae 2018). Changes in circulating miRNA have been associated with a variety of pathologies, and miRNAs may be useful diagnostic biomarkers in conditions ranging from breast cancer (Bahrami et al. 2018) to traumatic brain injury (Toffolo et al. 2018). A recent report discusses the possibility that miRNA regulation of gene expression subsequent to preterm brain injury may be important in the development of motor dysfunction in CP (Chapman et al. 2018). Additional work is needed

R. E. Akins and K. G. Robinson

to verify whether miRNA is a useful diagnostic biomarker for CP. DNA methylation describes the addition of a methyl group (-CH3) to a specific carbon on cytosine bases. DNA methylation is essential for normal growth and development, and the process of DNA methylation has been extensively studied (Ziller et al. 2013; Smith and Meissner 2013). Studies have shown that significant stresses in fetal or early life can alter DNA methylation profiles in a variety of tissues, including blood cells. Importantly, the cascades that are suspected of leading to CP involve hypoxia, infection, inflammation, and growth restriction, (Adams Waldorf and McAdams 2013; McIntyre et al. 2013; MacLennan et al. 2015) and studies show that DNA methylation is altered by hypoxia, bacterial infection, inflammation, intra-uterine growth restriction, and trauma or stress (Blaze and Roth 2015; Hartley et al. 2013; Pacis et al. 2015; Xiao et al. 2016; Houtepen et al. 2016; Labonte et al. 2012; Rask-Andersen et al. 2016; Stirzaker et al. 2015; Joubert et al. 2016). Alterations in the methylation of DNA in blood cells of individuals with CP have been assessed in studies from our group (Crowgey et al. 2018) and others (Mohandas et al. 2018; Jiao et al. 2017). In a small study examining blood from four pairs of monozygotic twins in which one developed CP while the other did not, Jiao and co-workers found differences in DNA methylation in 190 genes including genes that were hypo-methylated (153) and others that were hyper-methylated (37) (Jiao et al. 2017). Mohandas, et al. also looked at monozygotic twins that were discordant for CP, but they evaluated DNA methylation in dried blood spots from 15 twin pairs obtained during newborn screening. Their group identified 33 sites with altered methylation and two larger regions that were differentially methylated in CP; a significant number of the sites they found were indicative of inflammatory signaling, cytokine secretions, and cellular immunity (Mohandas et al. 2018). Our team at the Alfred I. duPont Hospital has also investigated DNA methylation in blood cells in the hope that CP arising gestationally might be diagnosed using epigenetic biomarkers

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Biomarker Blood Tests for Cerebral Palsy

in blood cells (Crowgey et al. 2018). Since methylation patterns that have been altered in utero can be sustained long term, (Joubert et al. 2016) we hypothesized that children with discrete types of CP have specific methylation patterns that are sustained later in life. Using a whole genome approach that took advantage of methylationsensitive-restriction-enzyme digestion, nextgeneration DNA sequencing, a computational pipeline to reassemble and analyze methylation patterns, and machine learning algorithms to identify sets of potentially diagnostic methylation sites, our group was able to develop an approach that identified a group of spastic di- and quadriplegic CP patients from controls. In brief, the AIDHC study involved analysis of methylation patterns in 32 genomic DNA samples from peripheral blood mononuclear cells, 16 from subjects with spastic CP with an average age of 14.7  3.3 years and 16 from control subjects with an average age of 15.0  2.2 years. All study participants were surgical patients scheduled for spinal fusion surgery at the hospital and all provided informed consent for the IRB-approved study. A total of 1.47 million potential methylation sites were identified across all 32 study participants; of these, 61,278 individual sites had at least 10% variability between the CP and control cohorts. To assess the ability to discriminate between the CP and control groups based on DNA methylation patterns, a clustering analysis was performed on the 61,278 sites. The clustering analysis collapses complex, multidimensional datasets into a small number of components that cluster the differential relationships within the original data, effectively isolating the patterns that are conserved within each group that were also divergent between the two groups (CP vs. control). There was a strong separation of the two groups (see Fig. 1). The results of the clustering analysis strongly indicated that there were substantial differences in the methylation patterns of the study participants with CP and the controls. However, 62 k is a large number of methylation sites to evaluate for a potential diagnostic test. To determine whether there were significantly smaller sets of methylation sites that would also discriminate the two groups, a guided, machine-learning approach

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was used to find sets that had 40 or fewer sites. In brief, this approach involved the following steps. 1. Filter the 61,278 potentially informative sites based on a false-discovery-rate (FDR) adjusted p value 98% and specificity >90% across all 20 replicates, the set of methylation sites was scored as a “good.” 5. Repeat all of step 3 again with any sites considered “good” in step 4 given a slightly higher chance of being included in subsequent models. 6. After 6.8 million LDAs, 252 models based on alfentanil (Scheufler and Zentner 2002; Lotto et al. 2004). Neuromuscular blocking agents (NMBA) have no effect on SSEP but, depending on the degree of motor block, can prevent recording of MEPs (Pajewski et al. 2007). Rocuronium, 0.5 mg/kg, may be given at the beginning of surgery as a single dose for exposure if baseline evoked potentials are recordable.

Postoperative Management Postoperative management of CP patients may frequently require mechanical ventilation and ongoing fluid management including use of vasopressors (see also ▶ Chap. 79, “Anesthesia in the Child with Cerebral Palsy”). In our experience, earlier extubation of trachea is beneficial resulting in a significant decrease in the need for sedation and thus better hemodynamic stability and far less use of vasopressors. Duration of stay in the ICU varies between 3 and 10 days, and the major risk factor for longer stay is infection (Sponseller et al. 2009). An experienced PICU team is very helpful in managing the hemodynamic status of the patients with the use of vasopressors in addition

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Anesthetic Management of Spine Fusion

to judicious use of fluid boluses with a goal of extubating the trachea early (if patient was not extubated in the operating room). Pain management should include antispasm medications (benzodiazepines still the most commonly used agent) as well as use of multimodal analgesics. Intravenous acetaminophen, low-dose ketamine infusion (see also ▶ Chap. 79, “Anesthesia in the Child with Cerebral Palsy”), dexmedetomidine, and ketorolac when coagulation is not compromised (also in limited doses) are used with very good results. Infusions of more potent opioids such as fentanyl are often poorly tolerated resulting in greater decrease in blood pressure measurements and escalating doses of vasopressors and need for increasing fluid boluses to maintain adequate blood pressures. As in the care of many complex medical patients, experience in caring for CP patients is valuable in the postoperative period as it is during intraoperative period.

Conclusion The common difficulty encountered when faced with a spastic quadriplegic CP patient is that the baseline physiological state of the patient, which is different from a non-CP patient, may pose concerns and may even result in cancelation of the surgery. The following points are important to consider: 1. CP patients are often chronically underhydrated as evidenced by dark-colored and scanty urine when Foley is placed and higher than normally expected hemoglobin/ hematocrit. Such patients would benefit from hydration shortly after induction of anesthesia until clear urine is evident, and repeat the laboratory measurements such as hemoglobin/ hematocrit and clotting studies, accepting these new values as the baseline. 2. Higher than average blood loss should be the expectation, and earlier use of fresh frozen plasma, platelets, and packed red cells will allow patients to stay hemodynamically stable with less “total body edema” than if massive amounts of crystalloids were to be administered. Extubating the trachea at the end of surgery is a

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reasonable goal in majority of CP patients following spine fusion especially if the facial and oropharyngeal edema is minimized. 3. Hypothermia is so common in CP patients that pre-warming patients in the holding room prior to arrival to the operating room may avoid the severe hypothermia that these patients often experience during anesthesia. 4. Use of procoagulant, namely, TXA, has been successful in decreasing blood loss and improving the overall outcome following spine fusion in CP patients. 5. Postoperative pain management should include multimodal analgesic agents including agents to treat spasticity.

References Anderson PR, Puno MR, Lovell SL, Swayze CR (1985) Postoperative respiratory complications in non-idiopathic scoliosis. Acta Anaesthesiol Scand 29:186–192 Brenn BR, Theroux MC, Dabney KW, Miller F (2004) Clotting parameters and thromboelastography in children with neuromuscular and idiopathic scoliosis undergoing posterior spinal fusion. Spine (Phila Pa 1976) 29:E310–E314 Dehmer JJ, Adamson WT (2010) Massive transfusion and blood product use in the pediatric trauma patient. Semin Pediatr Surg 19:286–291 Devlin VJ, Schwartz DM (2007) Intraoperative neurophysiologic monitoring during spinal surgery. J Am Acad Orthop Surg 15:549–560 Dias RC, Miller F, Dabney K, Lipton G, Temple T (1996) Surgical correction of spinal deformity using a unit rod in children with cerebral palsy. J Pediatr Orthop 16:734–740 Dicindio S, Arai L, Mcculloch M, Sadacharam K, Shah SA, Gabos P, Dabney K, Theroux MC (2015) Clinical relevance of echocardiogram in patients with cerebral palsy undergoing posterior spinal fusion. Paediatr Anaesth 25:840–845 Dicindio S, Theroux M, Shah S, Miller F, Dabney K, Brislin RP, Schwartz D (2003) Multimodality monitoring of transcranial electric motor and somatosensoryevoked potentials during surgical correction of spinal deformity in patients with cerebral palsy and other neuromuscular disorders. Spine (Phila Pa 1976) 28:1851–1855. discussion 1855–6 Goobie SM, Meier PM, Sethna NF, Soriano SG, Zurakowski D, Samant S, Pereira LM (2013) Population pharmacokinetics of tranexamic acid in paediatric patients undergoing craniosynostosis surgery. Clin Pharmacokinet 52:267–276

1192 Ho AM, Dion PW, Yeung JH, Ng CS, Karmakar MK, Critchley LA, Rainer TH, Cheung CW, Tay BA (2010) Fresh-frozen plasma transfusion strategy in trauma with massive and ongoing bleeding. Common (sense) and sensibility. Resuscitation 81:1079–1081 Kakinohana M, Fuchigami T, Nakamura S, Kawabata T, Sugahara K (2002) Propofol reduces spinal motor neuron excitability in humans. Anesth Analg 94:1586–1588. table of contents Kalkman CJ, Drummond JC, Ribberink AA (1991) Low concentrations of isoflurane abolish motor evoked responses to transcranial electrical stimulation during nitrous oxide/opioid anesthesia in humans. Anesth Analg 73:410–415 Kawaguchi M, Sakamoto T, Inoue S, Kakimoto M, Furuya H, Morimoto T, Sakaki T (2000) Low dose propofol as a supplement to ketamine-based anesthesia during intraoperative monitoring of motor-evoked potentials. Spine (Phila Pa 1976) 25:974–979 Lotto ML, Banoub M, Schubert A (2004) Effects of anesthetic agents and physiologic changes on intraoperative motor evoked potentials. J Neurosurg Anesthesiol 16:32–42 Macdonald DB, Skinner S, Shils J, Yingling C, American Society of Neurophysiological M (2013) Intraoperative motor evoked potential monitoring - a position statement by the American Society of Neurophysiological Monitoring. Clin Neurophysiol 124:2291–2316 Nathan N, Tabaraud F, Lacroix F, Moulies D, Viviand X, Lansade A, Terrier G, Feiss P (2003) Influence of propofol concentrations on multipulse transcranial motor evoked potentials. Br J Anaesth 91:493–497 Olivant Fisher A, Husain K, Wolfson MR, Hubert TL, Rodriguez E, Shaffer TH, Theroux MC (2012) Hyperoxia during one lung ventilation: inflammatory and oxidative responses. Pediatr Pulmonol 47:979–986 Pajewski TN, Arlet V, Phillips LH (2007) Current approach on spinal cord monitoring: the point of view of the neurologist, the anesthesiologist and the spine surgeon. Eur Spine J 16(Suppl 2):S115–S129 Pechstein U, Nadstawek J, Zentner J, Schramm J (1998) Isoflurane plus nitrous oxide versus propofol for recording of motor evoked potentials after high frequency repetitive electrical stimulation. Electroencephalogr Clin Neurophysiol 108:175–181 Phan HH, Wisner DH (2010) Should we increase the ratio of plasma/platelets to red blood cells in massive transfusion: what is the evidence? Vox Sang 98:395–402 Pidcoke HF, Aden JK, Mora AG, Borgman MA, Spinella PC, Dubick MA, Blackbourne LH, Cap AP (2012) Ten-year analysis of transfusion in operation Iraqi freedom and operation enduring freedom: increased plasma and platelet use correlates with improved survival. J Trauma Acute Care Surg 73:S445–S452 Reames DL, Smith JS, Fu KM, Polly DW Jr, Ames CP, Berven SH, Perra JH, Glassman SD, Mccarthy RE, Knapp RD Jr, Heary R, Shaffrey CI, Scoliosis Research Society M and Mortality C (2011) Complications in the surgical treatment of 19,360 cases of pediatric

M. C. Theroux and S. Dicindio scoliosis: a review of the Scoliosis Research Society morbidity and mortality database. Spine (Phila Pa 1976) 36:1484–1491 Scheufler KM, Zentner J (2002) Motor-evoked potential facilitation during progressive cortical suppression by propofol. Anesth Analg 94:907–912. table of contents Schwartz DM, Auerbach JD, Dormans JP, Flynn J, Drummond DS, Bowe JA, Laufer S, Shah SA, Bowen JR, Pizzutillo PD, Jones KJ, Drummond DS (2007) Neurophysiological detection of impending spinal cord injury during scoliosis surgery. J Bone Joint Surg Am 89:2440–2449 Sethna NF, Zurakowski D, Brustowicz RM, Bacsik J, Sullivan LJ, Shapiro F (2005) Tranexamic acid reduces intraoperative blood loss in pediatric patients undergoing scoliosis surgery. Anesthesiology 102:727–732 Shapiro F, Sethna N (2004) Blood loss in pediatric spine surgery. Eur Spine J 13(Suppl 1):S6–17 Shapiro F, Zurakowski D, Sethna NF (2007) Tranexamic acid diminishes intraoperative blood loss and transfusion in spinal fusions for duchenne muscular dystrophy scoliosis. Spine (Phila Pa 1976) 32:2278–2283 Sloan T, Sloan H, Rogers J (2010) Nitrous oxide and isoflurane are synergistic with respect to amplitude and latency effects on sensory evoked potentials. J Clin Monit Comput 24:113–123 Sponseller PD, Shah SA, Abel MF, Sucato D, Newton PO, Shufflebarger H, Lenke LG, Letko L, Betz R, Marks M, Bastrom T (2009) Scoliosis surgery in cerebral palsy: differences between unit rod and custom rods. Spine (Phila Pa 1976) 34:840–844 Theroux MC, Corddry DH, Tietz AE, Miller F, Peoples JD, Kettrick RG (1997) A study of desmopressin and blood loss during spinal fusion for neuromuscular scoliosis: a randomized, controlled, double-blinded study. Anesthesiology 87:260–267 Theroux MC, Fisher AO, Horner LM, Rodriguez ME, Costarino AT, Miller TL, Shaffer TH (2010) Protective ventilation to reduce inflammatory injury from one lung ventilation in a piglet model. Paediatr Anaesth 20:356–364 Theroux MC, Olivant A, Lim D, Bernardi JP, Costarino AT, Shaffer TH, Miller TL (2008) Low dose methylprednisolone prophylaxis to reduce inflammation during one-lung ventilation. Paediatr Anaesth 18:857–864 Thompson GH, Florentino-Pineda I, Poe-Kochert C, Armstrong DG, Son-Hing J (2008) Role of Amicar in surgery for neuromuscular scoliosis. Spine (Phila Pa 1976) 33:2623–2629 Tsirikos AI, Chang WN, Dabney KW, Miller F (2003) Comparison of one-stage versus two-stage anteroposterior spinal fusion in pediatric patients with cerebral palsy and neuromuscular scoliosis. Spine (Phila Pa 1976) 28:1300–1305 Verma K, Errico TJ, Vaz KM, Lonner BS (2010) A prospective, randomized, double-blinded single-site control study comparing blood loss prevention of tranexamic acid (TXA) to epsilon aminocaproic acid (EACA) for corrective spinal surgery. BMC Surg 10:13

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Hussam Alharash, Maxine Ames, Smitha Mathew, David Rappaport, and Nicholas Slamon

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1194 Respiratory Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Patients at Respiratory Baseline . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The Intubated Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Upper Airway Obstruction and Respiratory Effort . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Impaired Mucociliary Function and Secretion Clearance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Postoperative Respiratory Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Liberation from the ICU or Stepdown Unit . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Cardiovascular Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Background . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Modalities of Monitoring Hemodynamics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Fluid Resuscitation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Vasopressor Support . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Gastrointestinal Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Nutrition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Nausea and Vomiting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Constipation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pancreatitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Genitourinary and Renal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Fluid Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Electrolyte Abnormalities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Urinary Retention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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H. Alharash · S. Mathew · N. Slamon (*) Critical Care Division, Department of Pediatrics, Nemours, AI DuPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected]; [email protected]; [email protected] M. Ames · D. Rappaport Division of General Pediatrics, Nemours, AI DuPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_87

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H. Alharash et al. Hematology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Bleeding/Anemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Venous Thromboembolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . VTE Prevention . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . VTE Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Fever and Infectious Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Fever Timing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Noninfectious Etiologies of Postoperative Fever . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Diagnostic Testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Antibiotics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Neurology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Spasticity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Seizures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Sleep . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Early Mobilization/Postoperative Rehabilitation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1209 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1210 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1210

Abstract

Many patients with cerebral palsy (CP) will undergo surgery at some point, often orthopedic procedures. Patients with more severe involvement (GMFCS IV and V) are most likely to need surgery and are also the group most likely to have other medical conditions that can complicate their surgery and post-op recovery. This chapter addresses the most common of these complications, including how to assess and treat them. Keywords

Cerebral Palsy · Postoperative · Respiratory complications · Thromboembolism · Fever

Introduction Patients with cerebral palsy may have a variety of medical issues that can complicate their surgical and postoperative management. These complications may include issues such as respiratory failure, upper airway obstruction, or acute respiratory distress syndrome, all of which may result in prolonged intubation and ventilation in the postop period. Longer orthopedic procedures such as spinal fusion and osteotomies carry a higher risk

of blood loss due to the length of the procedure, which can result in the need for both transfusion and volume resuscitation. These needs can continue into the postoperative period. A systemic inflammatory response (SIRS) can develop, manifested by capillary leak, intravascular volume depletion and subsequent tachycardia and hypotension. Electrolyte abnormalities and renal insufficiency can also develop as a result of the volume depletion, and urinary retention by the bladder may make it hard to evaluate fluid balance. Pancreatitis is a common complication after spinal surgery and can lead to pain, nausea, and vomiting, resulting in inadequate nutritional intake. Fever is common, and assessing whether this is due to infection or inflammation is crucial in how it is managed. These and other potential postoperative management issues are discussed, as is the goal of having the patient up and moving as quickly as possible, so as to achieve rehabilitation goals.

Respiratory Considerations Patients with cerebral palsy (CP) often have respiratory dysfunction which is a significant cause of morbidity and mortality (Reddihough et al. 2001; Himmelmann and Sundh 2015). While there may

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be compensated respiratory compromise at baseline, there can be acute decompensation in the postoperative period (Cashman and Dolin 2004; Sun et al. 2015). There is impairment of secretion control with a poor cough, respiratory muscle weakness, risk of aspiration, thoracic dystrophy with resultant poor pulmonary compliance, obstructive apnea, bronchial reactivity, and recurrent pulmonary infections (Seddon and Khan 2003). Each of these risks must be considered during postoperative management of patients with CP. Not every postoperative patient will require intensive care monitoring in the postoperative period. Disposition of a postoperative patient to an intensive care unit is often a multidisciplinary decision involving the surgeon and anesthesiologist or intensivist. Reasons for critical care involvement include failure to extubate following surgical anesthetic, impending respiratory failure, which may be due to extrathoracic, intrathoracic or neurological reasons, or the need for frequent pulmonary clearance interventions that would be impractical in the regular inpatient care area. This will depend on the unique capabilities of each center’s general pediatric unit and whether there is the availability for a stepdown unit. Patients with CP will often have baseline home respiratory support prior to the planned surgical intervention. It is important to perform a careful review of the support type, frequency, and settings the patient requires when well. In some cases, the baseline respiratory care can be continued in the postoperative period. However, due to the effects of anesthesia and pain control on respiratory drive, worsening of upper airway obstruction, and postoperative splinting, patients may require additional support in the form of more frequent baseline therapies or different respiratory modalities as they recover. Some patients remain intubated postoperatively, and thus management and ventilator liberation will also be discussed in this chapter.

Patients at Respiratory Baseline Patients not requiring additional support, or support which is achievable in the general medical unit, can safely be dispositioned as such for

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postoperative recovery. These patients will benefit from initial postoperative observation in the PACU (Post Anesthesia Care Unit) to ensure stability and safety for general pediatric disposition after extubation. This monitoring is to ensure that the patient recovers well from anesthesia and is able to oxygenate and ventilate with a level of support appropriate for the capabilities of the eventual unit of disposition. Care should be taken to ensure that adequate pain control is achievable at this point without blunting the respiratory drive. The clinician should be familiar with the specific capabilities of the general pediatric floor with regards to the ability to provide care such as frequent suctioning, soft collar, nasopharyngeal airway, oral airway, non-invasive positive pressure ventilation, and continuous pulse oximetry if there is a concern these interventions may be required.

The Intubated Patient Some patients return from surgery mechanically ventilated via an endotracheal tube and instead of being extubated in the PACU are dispositioned to the ICU for continued care. The need for continuous sedation and analgesia to allow tolerance of invasive mechanical ventilation may lead to significant impact on hemodynamics and subsequent morbidity. Risk of ventilator-associated infection is also an important consideration. Thus, extubation should be the goal as soon as the indications for mechanical ventilation have resolved. While mechanically ventilated, the patient should be supported with a lung protective strategy using a pressure regulated volume control mode of 6 cc/kg per breath and an end-expiratory pressure that recruits alveoli with the minimal required inspired oxygen. An age-appropriate rate coupled with continuous end-tidal CO2 monitoring assures adequate ventilation without unnecessary shear force injury. Certain patients with pulmonary hypoplasia due to longstanding thoracic dystrophy may require lower tidal volumes and should be monitored by the clinician for high peak inspiratory breaths and excessive tidal volumes. Patients with intrinsic lung disease or

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significant postoperative inflammation or acute respiratory distress syndrome may require higher positive end-expiratory pressures to support their oxygenation. Ventilator liberation should be attempted when the patient is able to demonstrate adequate respiratory drive, stable respiratory mechanics, appropriate oxygenation and mental status that will assure proper secretion control via a strong gag and cough. Children with severe developmental delays may be difficult to assess for extubation readiness. The effect of sedatives and muscle relaxants may be difficult to distinguish from baseline neurologic impairment. A spontaneous breathing trial with Mapleson bag at the bedside is an excellent opportunity to assess both patient respiratory drive and ability to oxygenate prior to an attempt at extubation. Extubation can be to baseline respiratory support, but the patient will often require a bridge to baseline support. Bridging support can vary depending on the clinical situation and support availability. It can range from supplemental oxygen provided by simple nasal cannula or facemask for mild hypoxia, to high flow nasal cannula for increased dead space washout as well as some minimal positive end-expiratory pressure (Kubicka et al. 2008; Mikalsen et al. 2016), to modalities that provide noninvasive pressure ventilatory support such as CPAP or BiPAP (continuous positive airway pressure, bilevel positive airway pressure).

Upper Airway Obstruction and Respiratory Effort Neuromuscular compromise in the musculature of the oropharynx, as well as structural abnormalities in the airway due to neck rotation and asymmetry, may cause upper airway obstruction in patients with cerebral palsy at baseline (Wilkinson et al. 2006). These patients may also have decreased respiratory drive and respiratory muscle strength with associated hypoventilation at baseline. Often this obstruction and hypoventilation may be exacerbated or present as a new concern in the postoperative period due to muscle tone and sensorium depressing effects of anesthesia and

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analgesia. Patients with CP and scoliosis may also have difficulty mounting effective respiratory breaths while supine and recumbent, which may exacerbate the problem (Noble-Jamieson et al. 1986). These patients will require extra support for oxygenation and ventilation and may require close monitoring, especially if they demonstrate hypoxia or depressed mental status. Patients with CP admitted for postoperative monitoring may already have a home regimen for dealing with upper airway obstruction and neuromuscular hypoventilation which may include BiPAP or CPAP, either during sleep or at intervals throughout the day. Patients with upper airway obstruction in the postoperative period may benefit from repositioning into a modified sniffing position using a shoulder role. The use of a nasopharyngeal airway or cervical soft collar is also adjunctive measures to bypass or alleviate upper airway obstructive symptoms. Patients may also require an increase in frequency or level of noninvasive positive airway pressure support. Other interventions to support upper airway patency such as a nasopharyngeal airway or a soft collar may be of benefit to alleviate obstruction in the upper airway. Special emphasis should be placed on screening for atelectasis due to hypoventilation – repeated lung exams should be conducted, and the institution of noninvasive positive pressure ventilation should be trialed if atelectasis is suspected. Titration of the postoperative pain control regimen may be required to prevent any iatrogenic hypoventilation. The extra support can be weaned off back to baseline support as tolerated in the postoperative period. Due to increased risk of aspiration and reflux as well as a slower swallow reflex in patients with CP, special care should be taken with those requiring increased respiratory support, and they should be kept without nutrition by mouth or gastric tube if there is an absence of fundoplication (Casas et al. 1994; Arvedson et al. 1994). Postpyloric feeds through a nasoduodenal tube, or gastrojejunal tube may be used during this period for continued nutrition (Moriichi et al. 2016). Patients with the above concerns may be more suitably monitored in an ICU setting depending on the degree of support required and

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the capabilities of the general pediatric floor or stepdown unit.

Impaired Mucociliary Function and Secretion Clearance It is ideal in the postoperative period to have a functioning mucociliary escalator to mobilize secretions from the lower airway, a sufficiently responsive and strong cough to clear the secretions out of the middle to the upper airway, and the muscle coordination to swallow and divert the secretions away from the airway and towards the gastrointestinal tract. Patients with CP have deficits in some or all of these key mechanisms at baseline, and these deficits may be exacerbated in the postoperative period. Secretion clearance out of the lower airway can be aided with the use of chest physiotherapy and includes scheduled inhaled nebulization using saline to thin secretions. These nebulized therapies are often given in conjunction with albuterol to mitigate the risk of bronchospasm and achieve bronchodilation. The secretions in the lower airway can also be mobilized using intermittent percussive ventilation or systems such as the MetaNeb™ which combines percussive ventilation with aerosolized medications. Patients with a weak cough may benefit from cough assist therapies which both trigger a cough and bring secretions to the pharyngeal space for oral suctioning. Clearance of the nasopharyngeal and oropharyngeal spaces can be accomplished by direct catheter suctioning with frequency adjusted as needed. In patients with copious secretions, a nasopharyngeal airway can be utilized to aid in the suctioning of the nasopharynx. Frequency of suctioning requirements need to be considered when determining the patient’s disposition and patients with a frequency of suctioning that is practically unsustainable in the general pediatric unit may benefit from disposition or transfer to a stepdown or ICU unit. The use of anticholinergic medications such as glycopyrrolate for sialorrhea can be maintained at the preoperative schedule and dosage. Care should be taken when considering an increase in

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the use of anticholinergics as there is a risk of acutely thickening secretions and increasing the risk of acute pulmonary mucous plugging.

Postoperative Respiratory Complications Patients with CP may have postoperative respiratory complications which are further compounded by a lower respiratory functional baseline. The postoperative course may be complicated by systemic inflammation, which can cause ARDS (acute respiratory distress syndrome). This complication is often compounded by the need for fluid resuscitation due to concurrent hemodynamic instability and can result in hypoxic and hypercarbic respiratory failure. This patient population may require aggressive respiratory interventions including positive pressure ventilation, possibly with endotracheal intubation. Lung protective strategies should be employed as they have been shown to ameliorate ongoing pulmonary injury as the patient recovers from the systemic inflammation and the ARDS. Patients with chronic obstructive airway diseases which are resolved surgically should be monitored for development of post-obstructive pulmonary edema (POPE), which is also known as negative pressure pulmonary edema, a serious condition which can present in the first 24 h after surgery. The presentation can be similar to that of ARDS with the separating feature being the faster resolution of POPE within 24 h of the onset of symptoms of respiratory distress. Similarly to ARDS, the patient may require respiratory support in the form of positive pressure ventilation and possible endotracheal intubation. Acute mucous plugging is a concern in CP patients with altered pulmonary clearance and limited mobility. It often presents as acute hypoxemia that occurs after pulmonary clearance regimens or repositioning. If reoccurring, it may be an indication that the patient requires more frequent pulmonary clearance treatments. It may also indicate an overuse of sialorrhea treatments, which may cause secretions to become more viscous and difficult to mobilize.

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Liberation from the ICU or Stepdown Unit As patients who require ICU or stepdown level of care continue to recover, they should be evaluated for whether any additional support is needed. As support is weaned back to baseline settings, patients can be considered for transfer out of the ICU. In certain circumstances, extra support may be initiated which was not required preoperatively. Sometimes these therapies cannot be weaned and reflect a new baseline for the patient. Discussion regarding the details of new baseline therapies should be discussed with the physician who will responsible for care in the regular inpatient care area prior to transfer.

Cardiovascular Considerations Background Hemodynamics of all patients should be monitored regularly during any hospitalization. However, this is especially true for those patients that have undergone a surgical procedure. The type of orthopedic procedure, length of the procedure and the specific orthopedic manipulation involved can lead to variable hemodynamic changes. For this reason some patients are admitted to the intensive care unit for continuous monitoring of their hemodynamics. Certain patients may be stable for the regular inpatient care area.

Modalities of Monitoring Hemodynamics During the first 48 h, a key focus should be on continuous heart rate and blood pressure monitoring, serial physical examinations of perfusion and strict attention to urine output. Blood pressure monitoring can be completed intermittently via cuff measurements on the upper extremity. However, due to the length of the case, many patients with cerebral palsy have an invasive arterial line placed prior to the start of the case. This line remains useful in the intensive care environment

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as it provides access to continuous measurement of arterial pressure as well as a device that allows for laboratory sampling without painful serial blood draws. A Foley catheter is also helpful in assessing urine output and renal function as a marker of oxygen delivery to the tissues. Although these tools are helpful in assessing hemodynamics, the necessity of these devices should be routinely considered and they should be removed when no longer needed to reduce the risk of infection and aid in mobilizing the patient.

Fluid Resuscitation Longer orthopedic procedures such as spinal fusions and osteotomies carry a higher risk of blood loss due to the length of the procedure. (Edler et al. 2003) Interoperatively these procedures require both transfusion and volume resuscitation. These needs can continue into the postoperative period. Many surgeons will place a central venous line at the beginning of the operation to establish stable access for these expected resuscitation needs. A systemic inflammatory response (SIRS) is also often manifested by capillary leak, intravascular volume depletion, subsequent tachycardia, and hypotension.

Vasopressor Support Due to this SIRS response, fluid resuscitation may be required in the initial postoperative period. Either colloid or crystalloid can be used, dependent on the needs of the patient. Due to the nature of the surgical procedure, it is important to consider that signs of shock may be secondary to blood loss. In this case, the administration of colloid in the form of packed red blood cells may be more helpful than crystalloid alone. Goals of therapy should be titration to normal systolic blood pressure for age and fulfillment of end organ oxygen delivery needs. Vasopressor support should be started in those cases where a significant amount of volume was required to maintain adequate hemodynamics. 60 ml/kg of isotonic fluid given in rapid fashion over the first

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15 min of resuscitation prior to pressor initiation is a concept applied from the sepsis literature.

Norepinephrine The choice of vasopressor support depends on the goal of therapy, which could include vasoconstriction, improved cardiac contractility, improved diastolic filling, or increased heart rate. Many clinicians initiate a norepinephrine infusion with a pediatric dose starting at 0.05 mg/kg/min. Norepinephrine through beta 1 and alpha adrenergic and moderate stimulation of beta2 receptors leads to increased heart rate, increased inotropy and elevated systemic vascular resistance, thus raising blood pressure. The onset of action ranges from 1–2 min and the infusion can be titrated to a maximum dosage of 2 mg/kg/min to maintain an appropriate age based blood pressure with appropriate adequate end organ function. If central concentration of pressors are used a central line should be placed due to risk of extravasation ischemia with the use of peripheral line for access. (Dellinger et al. 2013). Dopamine Although not as readily used as norepinephrine, dopamine can still be used as an agent in cases of hypotension and low cardiac output. Dopamine has dose specific effects shown in Table 1. Hydrocortisone Important consideration should be paid to children and adolescent patients with a history of adrenal insufficiency or panhypopituitarism. Table 1 Dose-dependent effects of Dopamine with receptors involved and clinical effects Dose 1–5mcg/ kg/min 5–15mcg/ kg/min

20–50 mcg/kg/min

Receptor effect Dopamine, beta1 effects Beta1 effects Alpha effects

Alpha effects

Clinical effects Increase renal blood flow Increase renal blood flow Increase cardiac output Increase heart rate Increase contractility Increase systemic venous resistance

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These patients will likely need stress dose steroid pre- or intraoperatively with continued stress dosing for 3–5 days postoperatively or as long as they remain under stressful conditions. In cases of escalating pressor support and fluid and pressor refractory shock, hydrocortisone at shock dosing should be initiated. (Schulman et al. 2007) (Table 2).

Gastrointestinal Considerations Nutrition Issues related to the GI tract represent a pivotal part of postoperative management. Patients with cerebral palsy often undergo procedures that are long, involved, and bloody, necessitating fluid replacement and even blood transfusions. These patients may have relatively poor nutritional status at baseline (Soylu et al. 2008; Caselli et al. 2017). Postoperatively, these patients are vulnerable to electrolyte derangements and fluid overload (please see the section regarding fluid management for further details). Good nutrition improves wound healing, fluid status, and general well-being. Aggressive nutrition also helps reduce risks associated with intravenous nutrition – such as infection and thromboembolism – and length of stay. The question of how and when to restart feeds after surgery is a common one, and there are little data to guide this decision. Children with cerebral palsy vary greatly in how they ingest nutrition at baseline, ranging from entirely gastrostomy-tube fed to eating solely by mouth – or some combination thereof. This large range in feeding regimens necessitates an individualized postoperative approach. Table 2 Hydrocortisone dosing Level of stress Mild to moderate stress Major stress or surgery Physiologic replacement Septic shock

Dose 20–50 mg/m2/day divided into 3 or 4 doses IV 100 mg/m2/day in divided doses every 6 h 8–10 mg/m2/day PO 50–100 mg/m2/day

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Adequate nutrition in the postoperative period is vital for healing. Immediately postoperatively, while the patient remains under the lingering effects of anesthesia, it is reasonable to withhold feeds. Once the patient has returned to baseline, as long as there is little concern that the patient will need to return to surgery or that the patient is an aspiration risk, providing enteral nutrition to the patient is strongly encouraged. Prompt initiation of feeds helps avoid unwanted electrolyte derangements or fluid overload related to intravenous hydration. Patients who are fed via an enteric tube and had previously been tolerating their feeds will likely tolerate a fairly rapid return to their home feeding regimen. There are multiple methods for incrementally increasing their feeds. Often the patient’s parents or caregivers are the best guide for this process. Inquire about how the family alters the patient’s feeds when the patient has been sick or undergone procedures in the past. Patients with gastrostomy tubes often receive bolus feeds during the day, and many receive continuous feeds overnight. Patients with duodenostomy or jejunostomy tubes receive feeds continuously, often for 12–24 h per day. A frequent first step in identifying a postoperative feeding plan is to determine the baseline volume of feeds provided over a 24-h period. This volume is then divided by 24 to determine an hourly feeding rate to be provided continuously (do not forget to include water flushes). As feeds are increased enterally, the rate of IV fluids, which post operatively will typically be at a maintenance rate, should be titrated down so that the total amount combined of IV fluids and feeds remains constant. Often feeds are first provided at half of the 24-h rate and then, if tolerated, are increased to three-quarters of the maximum rate 4 h later, and, if tolerated, to the full rate 4 h thereafter. Alternately, feeds may be diluted with electrolyte solutions such as Pedialyte. Some families and practitioners prefer to start with half-strength feeds (formula diluted 1:1 with Pedialyte). The diluted feeds will then be started at half of the maximum hourly rate and increased slowly as tolerated. Once the maximum rate is reached, the

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feeds may then be diluted to three-quarters strength and then full strength. Patients who eat by mouth may start on a clear liquid diet after they have returned to their baseline mental status. If they tolerate a clear diet, they may receive a regular diet, usually the following morning. Recognition of the thickness of the oral feeds is critical; if the patient previously took feeds of a honey-thick or nectar-thick consistency, he/she should receive that same consistency in each phase as their diet advances. After lengthy and involved surgeries like spinal fusions, patients with cerebral palsy, especially those who were picky eaters at baseline, may demonstrate oral aversion for a relatively prolonged period of time. A prolonged period of oral aversion may necessitate alternative approaches such as nasogastric tube feeding. Generally we suggest this decision should be made after no more than 4–5 days of limited nutrition.

Nausea and Vomiting Nausea is a frequent side effect of certain surgical procedures and of anesthesia. Mitigating this side effect is critical, as it will hinder the patient’s ability to tolerate feeds or participate in postoperative rehabilitation activities. The mainstay of nausea treatment is antiemetic medications such as ondansetron (Zofran). Ondansetron is available as an oral dissolving tablet (ODT), liquid or IV formulation. For severe nausea, the IV formulation is recommended; for more mild nausea in patients able to eat by mouth, the ODT is the preferred route, with the liquid formulation able to be provided via an enteric tube. Ondansetron may prolong the QTc so this should be monitored, especially in patients on many medications, as polypharmacy increases patients’ risk of QTc prolongation. Other medications frequently used for the treatment of nausea include metoclopramide (Reglan) and prochlorperazine (Compazine). These medications are dopamine agonists that work as promotility agents. A careful review of the patient’s other medications is important as

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these medications more frequently interact with antipsychotic or antiepileptic medications than does ondansetron. These medications also have QTc prolonging properties, so their use also often necessitates monitoring of the QTc. Benzodiazepines also have antiemetic properties and so may be particularly useful in the treatment of nausea in patients with cerebral palsy, as they can treat spasticity and anxiety as well. Lorazepam (Ativan) is a short-acting benzodiazepine often used for this purpose. Patients receiving benzodiazepines for spasticity or opioids postoperatively should be monitored closely for sleepiness or respiratory depression. Risks and benefits of adding additional benzodiazepines such as diazepam in patients who receive benzodiazepines at baseline should be weighed.

Constipation Children with CP often suffer from constipation at baseline (Veugelers et al. 2010); accordingly, postoperative constipation is frequently an issue. Motility issues preoperatively can be exacerbated by opioid-related constipation in addition to postoperative ileus. Constipation is an uncomfortable and even painful process that can hinder the patient’s ability to tolerate feeds and participate in rehabilitation-related activities. One of the best tools to combat postoperative constipation is to aggressively increase stooling preoperatively. The home bowel regimen should be started immediately postoperatively. If opioid medications are used, increasing the dose or initiating the use of a promotility agent such as senna may be helpful. Newer medications aimed at reducing specifically opioid-induced constipation have not yet been studied in children. Polyethylene glycol (Miralax) and docusate (Colace) are two osmotic agents usually well tolerated in pediatric patients and are very safe. Miralax should be titrated to effect, and so dosing twice or even three times daily may be necessary. Suppositories or enemas may be used in cases of more significant constipation, especially when stool seems to be more distal in the GI tract.

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Pancreatitis Postoperative pancreatitis is associated with significant morbidity and mortality. Mortality associated with acute pancreatitis is most often due to a systemic inflammatory response and can lead to organ failure and sepsis (Gloor et al. 2001). In a study examining pancreatitis following posterior spinal fusion in children with CP, 55% of patients developed pancreatitis postoperatively as defined by amylase or lipase increasing to above three times the normal limit. This study identified the presence of a gastrostomy tube as a risk factor for developing pancreatitis after spinal fusion (Abousamra et al. 2016). Initial management of acute pancreatitis involves supportive care consisting of fluid resuscitation, pain management and nutrition. Patients may require a minimum of 5–10 ml/kg/hr. isotonic crystalloid solution above their maintenance requirements unless cardiovascular or renal compromise requires more stringent fluid restriction. Clinical improvement in addition to electrolyte balance should be monitored frequently (at least every 6 h) during aggressive fluid resuscitation (Wu and Banks 2013). Any vital sign instability suggests worsening of the patient’s condition and should prompt consideration of transfer to an intensive care unit. In patients with mild to moderate pancreatitis, oral feeding may be initiated as soon as pain is tolerable. A low-fat, low-residue diet is often recommended to begin, with feeds being advanced slowly as tolerated. In patients unable to tolerate oral feeding, a nasoduodenal or nasojejunal feeding tube should be considered and continuous feeds of a high-protein, low-fat, semi-elemental feeding formula (such as Peptamen AF) should be initiated at a slow rate and increased as tolerated (Working Group IAP/APA Acute Pancreatitis Guidelines 2013). In patients who receive feeds via gastrostomy, duodenostomy or jejunostomy tubes, resumption of home feeding regimen may be attempted as tolerated. If patients with gastrostomy tubes are unable to tolerate their feeding regimen, advancement to a jejunostomy tube may be required,

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along with switching their home formula to highprotein, low-fat, semi-elemental formula. If the patient is unable to tolerate enteral feeds entirely, total parental nutrition (TPN) should be considered, especially once approaching 7 days after surgery. Pancreatitis may be associated with a variety of systemic problems, including pleural effusions and serious bacterial infections. In adults, these infections may include bloodstream infections, pneumonia or urinary tract infections (Besselink et al. 2009). If an infection is suspected, consideration of antibiotics while the source of infection is being determined may be reasonable, although if cultures are negative and no source of infection is identified, antibiotics may be discontinued. Prophylactic antibiotics are not recommended in acute pancreatitis regardless of severity (Tenner et al. 2013).

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assess ongoing losses via drains, diarrhea, or vomiting. Postoperative patients may need replacement of up to three times the estimated fluid lost, although for small volumes, 1:1 replacement is usually adequate. Normal saline or lactated Ringers are the optimal replacement fluid, although dextrose-containing fluids should be used for maintenance. Colloid fluids, most commonly albumin, may also be used for volume expansion. The effects of albumin are shortlived, as it remains in the intravascular space only for a matter of hours, and studies in critically ill adults have not demonstrated benefit in using colloid solutions versus crystalloid (Strunden et al. 2011). Response to fluid resuscitation should be monitored carefully by frequent vital sign measurements and frequent assessment of the patient’s electrolyte status.

Electrolyte Abnormalities

Genitourinary and Renal Fluid Management Management of fluid and electrolyte balance represents a pivotal part of postoperative management of patients with cerebral palsy (CP). Patients with CP often undergo procedures that are long, involved, and bloody, necessitating fluid replacement and even blood transfusions. Postoperatively, these patients are vulnerable to electrolyte derangements and fluid overload. The goal of postoperative fluid management is to ensure adequate organ perfusion, maintain appropriate electrolyte and pH levels, and prevent catabolism. The stress response caused by surgery is largely mediated by cytokines and hormones such as vasopressin, aldosterone, cortisol, and catecholamines. This stress response is important intraoperatively to help offset potential hypovolemia, though these hormones may cause their own complications. Postoperative hypovolemia may result from third spacing of fluid, fluid loss in the OR, bleeding, and/or insensible losses. A first step in the postoperative management of fluids is to ascertain the estimated blood loss during the procedure, and the volume and type of fluids provided intraoperatively. Next, one should

The body’s stress reaction after surgery and iatrogenic interventions may contribute to postoperative electrolyte abnormalities. Classically, release of aldosterone will lead to potassium wasting and resultant hypokalemia. Cortisol and catecholamine release may lead to hyperglycemia, which is associated with a host of postoperative complications. Poor nutrition at baseline may exacerbate these factors. Use of intravenous fluids may also contribute to electrolyte abnormalities. Large volumes of intravenous crystalloids may lead to a hyperchloremic metabolic acidosis, hyponatremia, hypomagnesemia, or hypokalemia. Hyponatremia may result from physiologic release of antidiuretic hormone (ADH) in response to intraoperative and postoperative stress. ADH acts on the kidney to cause retention of free water, leading to a relative hyponatremia. Mild hyponatremia will usually resolve on its own, whereas moderate to severe hyponatremia is more worrisome. Moderate to severe hyponatremia tends to occur in patients with brain injury (Cuesta and Thompson 2015) and will respond to the use of isotonic or hypertonic fluids. Aldosterone release in response to hypovolemia or hypotension may cause hypokalemia and

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sodium retention as well. The degree of hypokalemia is usually mild and volume repletion should lead to an increase in serum potassium concentrations.

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in any patient who is experiencing agitation, discomfort or vital sign changes such as tachycardia, which cannot otherwise be explained. Patients who underwent CIC prior to surgery should have their home catheterization regimen resumed postoperatively.

Urinary Retention Postoperative urinary retention (POUR) occurs commonly following anesthesia. Patients with CP are at an increased risk as they may have disordered voiding at baseline (though often undiagnosed). Data regarding the incidence of POUR in children are scarce; however, the adult literature estimates an incidence between 5% and 70% (Baldini et al. 2009). In patients without baseline voiding dysfunction, POUR is usually transient, though patients with CP are at an increased risk of developing POUR regardless of whether they had a history of dysfunction prior to surgery. Prolonged anesthesia is an additional risk factor for POUR, which is often the case in patients with CP undergoing surgery. There are no strict guidelines for children regarding when to assess for urinary retention; however, the adult literature recommends a bladder ultrasound in patients who have not voided for >4 h after surgery, and, if >600 ml are identified, the patient should undergo bladder catheterization (Baldini et al. 2009). The Koff Formula, the gold standard for estimating the urinary bladder capacity in children greater than 1 year of age (Koff 1983), is as follows: Bladder capacity (ounces) = age (years) + 2 If ultrasound of the patient’s bladder demonstrates a volume approaching their predicted bladder capacity, bladder catheterization may be required. Clean intermittent catheterization (CIC) is the recommended approach, as it incurs a lower complication risk than an indwelling urinary catheter (Patel et al. 2001). There is no clear recommendation about the frequency required for CIC; however, in a patient without previous voiding dysfunction, we recommend they may be monitored clinically and bladder scanned every 6–8 h, if they are not spontaneously voiding. Discomfort related to bladder distension should be suspected

Hematology Bleeding/Anemia Although patients’ risk of bleeding is typically associated more with the surgical procedure performed than the diagnosis of cerebral palsy, bleeding after surgical procedures may require a somewhat different approach in CP patients. For example, patients who undergo spinal fusion surgery for neuromuscular scoliosis tend to have higher blood losses than those who do so for idiopathic scoliosis. This discrepancy may be due to differences in coagulation factors despite normal prothrombin time (PT) and partial thromboplastin time (PTT) (Brenn et al. 2004). Other medical factors may also play a role; for example, patients who receive valproic acid for seizures likely have an increased risk of bruising and bleeding as a medication effect or due to thrombocytopenia. Patients undergoing orthopedic procedures such as osteotomies and spinal fusions are likely to have more bleeding than those undergoing other procedures. In these patients, we recommend checking hemoglobin daily for 2 or 3 days postoperatively. Trauma patients may be at high risk for hemorrhage depending on the severity and location of their injuries. For surgical patients generally, postoperative transfusion is typically reserved for patients with underlying cardiac or pulmonary disease or those who demonstrate significant anemia (Hemoglobin 5 days along with concomitant renal insufficiency due to other drugs like diuretics and ACE (angiotension converting enzyme) inhibitors significantly increases the risk of renal injury. (Misurac et al. 2013) Most critical care teams will discuss the use of these drugs with the surgeon in the immediate postoperative period. In addition to the above medications, many critical care physicians will prescribe both an antispasmodic drug such as diazepam and a narcotic or narcotic analogue like morphine, or nalbuphine. Following long operations with wide exposure like a spinal fusion, large muscles in the back may become more spastic than usually found in children with cerebral palsy. While benzodiazepines like diazepam do not have analgesic effect in and of themselves, the prevention of severe cramping and spasm of back musculature can in effect reduce pain. Nalbuphine as a semisynthetic narcotic is thought to mimic many of the mu receptor effects seen with drugs like morphine or hydromorphone, but without as great a sedative effect. It is especially useful in children with cerebral palsy who can have altered levels of consciousness in the postoperative period and are at risk for worsening upper airway tone with subsequent obstruction. In addition to pain management, adequate caloric intake is key to postoperative wound healing. Following large procedures such as orthopedic procedures, there is a physiologic requirement for not only maintenance calories but also additional calories for wound healing and continued growth. Adjusting caloric goals with a dietician in the postoperative period is paramount to adequate recovery and healing. One of the goals of early mobilization following orthopedic surgery in patients with cerebral palsy is moving them from the supine position in the bed to a sitting position in a chair. It is important for many reasons including reduction of pressure on new surgical wounds, improvement of respiratory mechanics and pulmonary hygiene, and often it will help with feeding tolerance and improved bowel function. Coordination of this move from bed to chair requires teamwork. Many children with cerebral palsy use a wheelchair and will need extensive adjustments to the

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chair to accommodate changes due to surgery. The children with spinal fusion are often significantly taller with a straighter back than in the preoperative period. Occupational therapy and materials management technicians will usually be responsible for wheel chair modifications. Nursing and physical therapists then often are responsible for moving the child from bed to chair. Empiric administration of pain medication often aids in this transition and allows for a more productive therapy session. Goals of care for physical therapy not only include passive and active ranges of motion but also parental education. Educating the families on how to care for their child following surgery is key in enabling them to succeed postoperatively. Independent functioning at home with caretaker assistance is as important as in hospital therapies. Finally, children with continued respiratory requirements such as high flow nasal cannula or BiPAP may require assistance from respiratory therapists during transitions in and out of bed.

Cross-References ▶ Anesthesia in the Child with Cerebral Palsy ▶ General Nutrition for Children with Cerebral Palsy ▶ Medical and Surgical Therapy for Constipation in Patients with Cerebral Palsy ▶ Medical Evaluation for Preoperative Surgical Planning in the Child with Cerebral Palsy ▶ Neurogenic Bladder in Cerebral Palsy: Upper Motor Neuron ▶ Postoperative Pain and Spasticity Management in the Child with Cerebral Palsy

References Abousamra O, Nishnianidze T, Rogers KJ, Er MS, Sees JP, Dabney KW et al (2016) Risk factors for pancreatitis after posterior spinal fusion in children with cerebral palsy. J Pediatr Orthop Part B 27(2):163–167 Arvedson J, Rogers B, Buck G, Smart P, Msall M (1994) Silent aspiration prominent in children with dysphagia. Int J Pediatr Otorhinolaryngol 28(2–3):173–181 Badawy SM, Rychlik K, Sharathkumar AA (2016) Current practice of pharmacological thromboprophylaxis for

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prevention of venous thromboembolism in hospitalized children: a survey of pediatric hemostasis and thrombosis experts in North America. J Pediatr Hematol Oncol 38(4):301–307 Baldini G, Bagry H, Aprikian A, Carli F (2009) Postoperative urinary retention: anesthetic and perioperative considerations. Anesthesiology 110(5):1139–1157 Besselink MG, van Santvoort HC, Boermeester MA, Nieuwenhuijs VB, van Goor H, Dejong CHC et al (2009) Timing and impact of infections in acute pancreatitis. Br J Surg 96(3):267–273 Blackmer AB, Feinstein JA (2016) Management of sleep disorders in children with neurodevelopmental disorders: a review. Pharmacotherapy 36(1):84–98 Bonadio WA (1993) Defining fever and other aspects of body temperature in infants and children. Pediatr Ann 22(8):467–473 Brenn BR, Theroux MC, Dabney KW, Miller F (2004) Clotting parameters and thromboelastography in children with neuromuscular and idiopathic scoliosis undergoing posterior spinal fusion. Spine 29(15): E310–E314 Cameron S et al (2015) Early mobilization in the critical care unit: a review of adult and pediatric literature. J Crit Care 30(4):664–672. https://doi.org/10.1016/j. jcrc.2015.03.032 Casas M, Kenny D, McPherson K (1994) Swallowing/ ventilation interactions during oral swallow in normal children and children with cerebral palsy. Dysphagia 9(1):40–46 Caselli TB, Lomazi EA, Montenegro MAS, BellomoBrandão MA (2017) Assessment of nutritional status of children and adolescents with spastic quadriplegic cerebral palsy. Arq Gastroenterol 54(3):201–205 Cashman JN, Dolin SJ (2004) Respiratory and haemodynamic effects of acute postoperative pain management: evidence from published data. Br J Anaesth 93(2):212–223 Cincinnati Children’s Hospital. Venous thromboembolism (VTE) prophylaxis in children and adolescents [Internet]. Available from: https://www.google.com/url?sa= t&rct=j&q=&esrc=s&source=web&cd=1&cad=rja& uact=8&ved=0ahUKEwjNjqOk2sXVAhXB4yYKHc DCBpcQFggmMAA&url=https%3A%2F%2Fwww.c incinnatichildrens.org%2F-%2Fmedia%2Fcincinnati% 2520childrens%2Fhome%2Fservice%2Fj%2Fanderson -center%2Fevidence-based-care%2Frecommendation s%2Ftype%2Fvenous%2520thromboembolism%2520 best%2520181&usg=AFQjCNGqvfwYqCVKIwIPB MTwtmWz3%2D%2DFQg Colver A (2010) Why are children with cerebral palsy more likely to have emotional and behavioural difficulties? Dev Med Child Neurol 52(11):986 Cuesta M, Thompson C (2015) The relevance of hyponatraemia to perioperative care of surgical patients. Surgeon 13(3):163–169 de Jonge JC, Melis GI, Gebbink TA, de Kort GAP, Leijten FSS (2014) Safety of a dedicated brain MRI protocol in patients with a vagus nerve stimulator. Epilepsia 55(11):e112–e115

1211 Dellinger RP et al (2013) Surviving sepsis campaign: international guidelines for management of severe sepsis and septic shock: 2012. Critical Care Medicine 41:580–637. https://doi.org/10.1097/CCM.0b013e 31827e83af Edler A, Murray DJ, Forbes RB (2003) Blood loss during posterior spinal fusion surgery in patients with neuromuscular disease: is there an increased risk? Pediatric Anesthesia 13:818–822. https://doi.org/10.1046/ j.1460-9592.2003.01171.X Epstein NE (2014) A review article on the benefits of early mobilization following spinal surgery and other medical/ surgical procedures. Surg Neurol Int 5(Suppl 3): S66–S73. https://doi.org/10.4103/2152-7806.130674 Foster T, Rai AIK, Weller RA, Dixon TA, Weller EB (2010) Psychiatric complications in cerebral palsy. Curr Psychiatry Rep 12(2):116–121 Garibaldi RA, Brodine S, Matsumiya S, Coleman M (1985) Evidence for the non-infectious etiology of early postoperative fever. Infect Control 6(07):273–277 Gloor B, Müller CA, Worni M, Martignoni ME, Uhl W, Büchler MW (2001) Late mortality in patients with severe acute pancreatitis. Br J Surg 88(7):975–979 Gregersen M, Damsgaard EM, Borris LC (2015) Blood transfusion and risk of infection in frail elderly after hip fracture surgery: the TRIFE randomized controlled trial. Eur J Orthop Surg Traumatol Orthop Traumatol 25(6):1031–1038 Himmelmann K, Sundh V (2015) Survival with cerebral palsy over five decades in western Sweden. Dev Med Child Neurol 57(8):762–767. https://doi.org/10.1111/ dmcn.12718 Hollway JA, Aman MG (2011) Pharmacological treatment of sleep disturbance in developmental disabilities: a review of the literature. Res Dev Disabil 32(3):939–962 Humes DJ, Nordenskjöld A, Walker AJ, West J, Ludvigsson JF (2015) Risk of venous thromboembolism in children after general surgery. J Pediatr Surg 50(11):1870–1873 Kim S-J, Sabharwal S (2014) Risk factors for venous thromboembolism in hospitalized children and adolescents: a systemic review and pooled analysis. J Pediatr Orthop Part B 23(4):389–393 Koff SA (1983) Estimating bladder capacity in children. Urology 21(3):248 Kubicka ZJ, Limauro J, Darnall RA (2008) Heated, humidified high-flow nasal cannula therapy: yet another way to deliver continuous positive airway pressure? Pediatrics 121(1):82–88 Laupland KB, Zygun DA, Davies HD, Church DL, Louie TJ, Doig CJ (2002) Incidence and risk factors for acquiring nosocomial urinary tract infection in the critically ill. J Crit Care 17(1):50–57 Lélis ALPA, Cardoso MVLM, Hall WA (2016) Sleep disorders in children with cerebral palsy: an integrative review. Sleep Med Rev 30:63–71 Mandelli M, Tognoni G, Garattini S (1978) Clinical pharmacokinetics of diazepam. Clin Pharmacokinet 3(1):72–91

1212 McKenzie SG (1983) Introduction to the pharmacokinetics and pharmacodynamics of benzodiazepines. Prog Neuro-Psychopharmacol Biol Psychiatry 7(4–6):623–627 Mikalsen IB, Davis P, Øymar K (2016) High flow nasal cannula in children: a literature review. Scand J Trauma Resusc Emerg Med 24(1):93 Misurac J et al (2013) Nonsteroidal anti-inflammatory drugs are an important cause of acute kidney injury in children. J Pediatr 162(6):1153–1159. https://doi.org/ 10.1016/j.jpeds.2012.11.069 Monagle P, Chan AKC, Goldenberg NA, Ichord RN, Journeycake JM, Nowak-Göttl U et al (2012) Antithrombotic therapy in neonates and children: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest 141(2 Suppl):e737S–e801S Moriichi A, Kawaguchi A, Kobayashi Y, Yoneoka D, Ota E (2016) The effectiveness and safety of various methods of post-pyloric feeding tube placement and verification in infants and children. Kawaguchi A ed. Cochrane Database Syst Rev Munin MC, Rudy TE, Glynn NW, Crossett LS, Rubash HE (1998) Early inpatient rehabilitation after elective hip and knee arthroplasty. JAMA 279(11):847–852. https://doi.org/10.1001/jama.279.11.847 Munro H et al (2002) Low-dose ketorolac improves analgesia and reduces morphine requirements following posterior spinal fusion in adolescents. Can J Anesth 49(5):461–466 Mustard RA (1987) C-reactive protein levels predict postoperative septic complications. Arch Surg 122(1):69 Newman CJ, O’Regan M, Hensey O (2006) Sleep disorders in children with cerebral palsy. Dev Med Child Neurol 48(7):564–568 Nguyen M, Tharani S, Rahmani M, Shapiro M (2014) A review of the use of clonidine as a sleep aid in the child and adolescent population. Clin Pediatr (Phila) 53(3):211–216 Noble-Jamieson CM, Heckmatt JZ, Dubowitz V, Silverman M (1986) Effects of posture and spinal bracing on respiratory function in neuromuscular disease. Arch Dis Child 61(2):178–181 Panella JJ (2016) Preoperative Care of Children: strategies from a child life perspective. AORN J 104(1):11–22 Patel MI, Watts W, Grant A (2001) The optimal form of urinary drainage after acute retention of urine. BJU Int 88(1):26–29 Peltola V, Mertsola J, Ruuskanen O (2006) Comparison of total white blood cell count and serum C-reactive protein levels in confirmed bacterial and viral infections. J Pediatr 149(5):721–724 Pugin J, Auckenthaler R, Mili N, Janssens J-P, Lew PD, Suter PM (1991) Diagnosis of ventilator-associated pneumonia by bacteriologic analysis of bronchoscopic and nonbronchoscopic “blind” bronchoalveolar lavage fluid. American Review of Respiratory Disease 143(5_pt_1):1121–1129

H. Alharash et al. Raffini L, Huang Y-S, Witmer C, Feudtner C (2009) Dramatic increase in venous thromboembolism in children’s hospitals in the United States from 2001 to 2007. Pediatrics 124(4):1001–1008 Reddihough D, Baikie G, Walstab J (2001) Cerebral palsy in Victoria, Australia: mortality and causes of death. J Paediatr Child Health 37(2):183–186. https://doi.org/ 10.1046/j.1440-1754.2001.00644.x Robinson AA, Malow BA (2013) Gabapentin shows promise in treating refractory insomnia in children. J Child Neurol 28(12):1618–1621 Schlefman A, Rappaport DI, Adams-Gerdts W, Stubblefield SC (2016) Brief report: healing touch consults at a tertiary care Children’s hospital. Hosp Pediatr 6(2):114–118 Schulman D, Palmert MR, Kemp SF (2007) Adrenal insufficiency: still a cause of morbidity and death in childhood. Pediatrics 119(2):484–494 Retrieved 10 Oct 2017 Seddon PC, Khan Y (2003) Respiratory problems in children with neurological impairment. Arch Dis Child 88(1):75–78. https://doi.org/10.1136/ADC.88.1.75 Singhi P, Jagirdar S, Khandelwal N, Malhi P (2003) Epilepsy in children with cerebral palsy. J Child Neurol 18(3):174–179 Soylu OB, Unalp A, Uran N, Dizdarer G, Ozgonul FO, Conku A et al (2008) Effect of nutritional support in children with spastic quadriplegia. Pediatr Neurol 39(5):330–334 Strunden MS, Heckel K, Goetz AE, Reuter DA (2011) Perioperative fluid and volume management: physiological basis, tools and strategies. Ann Intensive Care 1:2 Sun Z, Sessler DI, Dalton JE, Devereaux PJ, Shahinyan A, Naylor AJ et al (2015) Postoperative hypoxemia is common and persistent: a prospective blinded observational study. Anesth Analg 121(3):709–715 Tenner S, Baillie J, De Witt J, Vege SS, American College of Gastroenterology (2013) American College of Gastroenterology guideline: management of acute pancreatitis. Am J Gastroenterol 108(9):1400–1415, 1416 Tsao GJ, Messner AH, Seybold J, Sayyid ZN, Cheng AG, Golianu B (2015) Intraoperative acupuncture for posttonsillectomy pain: a randomized, doubleblind, placebo-controlled trial. Laryngoscope 125(8):1972–1978 Veugelers R, Benninga MA, Calis EAC, Willemsen SP, Evenhuis H, Tibboel D et al (2010) Prevalence and clinical presentation of constipation in children with severe generalized cerebral palsy. Dev Med Child Neurol 52(9):e216–e221 Wilkinson DJ, Baikie G, Berkowitz RG, Reddihough DS (2006) Awake upper airway obstruction in children with spastic quadriplegic cerebral palsy. J Paediatr Child Health 42(1–2):44–48. https://doi.org/10.1111/ j.1440-1754.2006.00787.x Woods BI, Rosario BL, Chen A, Waters JH, Donaldson W, Kang J et al (2013) The association between perioperative allogeneic transfusion volume and postoperative

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infection in patients following lumbar spine surgery. J Bone Joint Surg Am 95(23):2105–2110 Working Group IAP/APA Acute Pancreatitis Guidelines (2013) IAP/APA evidence-based guidelines for the management of acute pancreatitis. Pancreatol Off J Int Assoc Pancreatol IAP Al 13(4 Suppl 2):e1–e15

1213 Wu BU, Banks PA (2013) Clinical management of patients with acute pancreatitis. Gastroenterology 144(6):1272–1281 Yeung RSW, Buck JR, Filler RM (1982) The significance of fever following operations in children. J Pediatr Surg 17(4):347–349

Part XVIII Complementary Medical Treatments

Complementary and Alternative Medicine in Cerebral Palsy

84

Rachel M. Thompson and William Lawrence Oppenheim

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1218 Selected CAM Therapies Utilized in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Acupuncture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Myofascial Structural Integration/Rolfing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Adeli Suit (TheraSuit) Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Craniosacral Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hyperbaric Oxygen Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Electrical Stimulation (e-Stim) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Cannabinoids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Stem-Cell Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1219 1219 1220 1220 1221 1222 1222 1223 1224

Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1224 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1225

Abstract

Many parents of children with cerebral palsy turn to complementary and alternative medicine (CAM) when traditional medical care does not “deliver” either for symptom management or in providing a cure. For cerebral palsy, CAM practitioners frequently utilize

R. M. Thompson Department of Orthopaedics, Orthopaedic Institute for Children/UCLA, Los Angeles, CA, USA Ronald Reagan UCLA Medical Center, Santa Monica, CA, USA e-mail: [email protected] W. L. Oppenheim (*) Ronald Reagan UCLA Medical Center, Santa Monica, CA, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_88

acupuncture, spinal manipulation, Reiki, reflexology, healing touch, yoga, craniosacral therapy, and hyperbaric oxygen. In North America, the use of CAM has been reported to be between 35–56% for children with CP and 27% for adolescents with CP. A typical course of alternative treatment, regardless of modality, costs between $3500 and $4500 in the United States, and most spending on CAM is out-of-pocket. Despite cost, many patients and their families are willing to pay regardless of the lack of scientific evidence supporting the treatment’s efficacy. Currently there is limited evidence to support some narrow benefits associated with the use of acupuncture, myofascial structural integration, and the Adeli/TheraSuit. There is no evidence to support the use of hyperbaric 1217

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oxygen or threshold electrical stimulation. And while there is theoretical evidence supporting the use of cannabinoids and stem-cell therapy, there is currently no evidence to support usage specifically in cerebral palsy. Keywords

Complementary medicine · Alternative medicine · Alternative treatments · Eastern medicine · Osteopathic medicine

Introduction Cerebral palsy is a complex, heterogeneous, chronic neurologic disorder without a known cure. As a result, families of children with CP utilize traditional medical care in many forms – physical and occupational therapy, orthopedic surgery, neurosurgery, neurology, physiatry, and prosthetics and orthotics. Many families also turn to complementary and alternative treatments (CAM) when traditional medical care does not “deliver” either for symptom management or in providing meaningful, significant relief from a chronic neurologic disease process with no known cure. Many families believe that if conventional medicine cannot offer a meaningful chance for a cure, alternative medicine may at least offer hope for a cure or significant symptom improvement. As would be expected, patients who are more involved (higher GMFCS) – those with more limited manual ability and/or with greater activity limitations – are more likely to seek out CAM as more traditional methods are less likely to provide effective treatment for their limitations (Majnemer et al. 2013). However, CAM treatments are not necessarily evidenced-based. Rather, they typically rely on highly constituted belief systems usually based on a scientifically plausible theory, which often unfortunately prove inadequate when subjected to rigorous scientific testing. They typically boast a noted key practitioner along with trained/certified disciples who dispense therapies. Some modalities tout non-for-profit foundations, professional societies, newsletters, and websites, all of which seek to reinforce their

R. M. Thompson and W. L. Oppenheim

legitimacy regardless of scientific evidence supporting their efficacy. Despite the lack of scientific evidence or objective benefits gained, parents and caregivers are likely to believe that their family members benefitted from the treatment(s) provided. Simply put, after investing one’s time, energy, and money, the patient and/or the patient’s family members want to believe in the benefit obtained from such participation. Further, there is a well-documented participation effect experienced with CAM and traditional methods, whereby those who participate in a treatment may even objectively “improve” just for having participated. Finally, there is the placebo effect, whereby the participant gains benefits produced by an inert treatment that is not attributable to the properties of the treatment itself, and is thus only attributable to the patient’s belief in the treatment. This may be mediated by endorphins and other modalities as part of the mind-body connection. The National Center for Complementary and Integrative Health (NCCIH) of the US National Institutes of Health (NIH) (formerly the National Center for Complementary and Alternative Medicine) defines CAM as “a group of diverse medical and healthcare systems, practices and products that are not considered to be part of conventional or allopathic medicine” (NCCIH 2016). The NCCIH is clear to point out that the majority of CAM is practiced in conjunction with, rather than as a true alternative to, conventional therapies. The NCCIH reports that in 2012, 33.2% of adults age 18 and over and 11.6% of children age 4 to 17 used some form of CAM. This translates to over $30 billion in out-of-pocket healthcare dollars spent. Given the complexity and chronicity of cerebral palsy, it is not surprising that use of CAM in children and adults with CP is higher than in the general population. Moreover, in North American children with CP, the use of CAM has been reported to be between 35% and 56% (Hurvitz et al. 2003; Samdup et al. 2006) and 27% in adolescents with CP (Majnemer et al. 2013). Oppenheim reported that the number of adults with CP utilizing complementary or alternative treatment is unknown (Oppenheim 2009). However, in the absence of a definitive treatment

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or cure for CP, the use of CAM in adults with CP likely mimics the trend for its use in children. While an estimate of dollars spent on CAM in patients with CP is not readily available in the current literature, Weisleder reported that typical alternative treatments, regardless of modality, cost between $3500 and $4500 for a complete course in the United States (Weisleder 2010). The majority of spending on CAM is out-of-pocket, and many patients and their families are willing to pay regardless of a stated lack of scientific evidence supporting their efficacy (Novak et al. 2013). Interestingly, a Taiwanese study did show a reduction in dollars spent in emergency department visits, outpatient consultations, and hospitalizations in patients with CP who regularly utilized complementary medicine as compared to those who did not (Liao et al. 2017). In sum, CAM presently encompasses a broad and lengthy list of treatment modalities and lifestyle choices. For our purposes here, we will review the evidence, however limited, behind the most popular and/or most controversial complementary modalities. This includes myofascial structural integration, Adeli suit treatment, threshold electrical stimulation, craniosacral therapy, acupuncture, hyperbaric oxygen, cannabinoids, and stem-cell therapy. Some treatment modalities once considered alternative have gained popularity and are now considered mainstream with scientific evidence supporting their efficacy and will not be included in this chapter. For example, hippotherapy (therapeutic horseback riding) promotes exercise, social participation, and balance. Its methods have been scientifically tested and are now routinely utilized and no longer considered a CAM therapy.

Selected CAM Therapies Utilized in Cerebral Palsy Acupuncture Description Acupuncture is a classic example of complementary medicine and has been used in children with CP for over 30 years (Sanner and Sundequist

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1981). According to traditional Chinese theory, health is achieved by maintaining an uninterrupted flow of Qi (energy) along 14 meridians, and disease is a result of an interruption of this flow or an imbalance between yin and yang – a concept in Chinese medicine whereby there are two opposing forces within the body – masculine and feminine, and hot and cold. When these forces are equal, the body is said to be harmonious, balanced, and healthy. Acupuncture restores this flow and balance (Kaptchuk 2002). Fine needles are inserted into specific points on the body to correct any disruption of flow. Each session lasts 15–20 min and is typically performed a couple of times per week. Electrical current may be applied through the needles. In addition, some practitioners are employing “laser acupuncture,” which utilizes low-level lasers concentrated onto acupuncture points (Dabbous et al. 2016).

Evidence Acupuncture has been used broadly for many of the symptoms associated with cerebral palsy typically in conjunction with traditional medicine. In fact, a recent review of the literature resulted in 35 randomized controlled trials of acupuncture that included 3286 children (Zhang et al. 2010). Although the majority of included studies had poor methodological quality, a meta-analysis demonstrated that acupuncture combined with conventional treatment improved activity of daily living ( p < 0.00001) compared with conventional treatment alone. Further, with the addition of either herbal medicine or tu’ina (traditional Chinese hands-on therapy), patients demonstrated improvements in independence and verbal function compared to conventional therapy alone. No adverse events were reported in association with these forms of complementary treatment in this review. Specifically, some researchers have demonstrated acupuncture’s efficacy in improving extremity circulation (Svedberg et al. 2001), decreasing drooling (Wong et al. 2001), decreasing painful spasms in dystonic CP (Sanner and Sundequist 1981), and improving GMFM (Sun et al. 2004). However, the evidence provided is based on smaller, uncontrolled case series. As

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such, Novak et al. gave acupuncture a “yellow light,” indicating that there is weak/insufficient evidence in support of the use of acupuncture in CP (Novak et al. 2013). It is a safe adjuvant to traditional medicine, but more robust randomized trials are required to determine true efficacy.

Myofascial Structural Integration/ Rolfing Description Rolfing – myofascial structural integration – was first developed and described by Dr. Ida Rolf in 1973. It “is a technique which aims to align the body – head, thorax, pelvis, legs – in an optimal position with respect to the gravitational field” (Rolf 1977). The goal in Rolfing is to bring the body into a more vertical alignment, allowing for improved function and decreased energy consumption with standing and walking. There is a standard, reproducible protocol utilized by Rolf practitioners. Therapy consists of ten 1 hour sessions performed on a patient for 10 consecutive weeks. Trained Rolf practitioners perform manual manipulation – stretching, lengthening, and “repositioning” of the fascia and muscles through deep and gentle pressure on a passive patient. Essentially, the manipulations attempt to release adhesions that may be present within or between myofascial layers. Practitioners’ efforts are concentrated on areas of asymmetry that prevent proper alignment (Perry et al. 1981). The loosening and repositioning of muscles and fascia theoretically allows for improved alignment. Evidence Rolfing was first independently evaluated on ten patients at Rancho Los Amigos Hospital in 1981 with mixed results (Perry et al. 1981). Prior to and after completing therapy, all included patients were evaluated for range of motion, manual muscle testing, and gait parameters. These investigators showed inconsistent changes in range of motion and strength with improvements in gait velocity, stride length, and cadence only in mildly

R. M. Thompson and W. L. Oppenheim

affected patients. They hypothesized that the gains in tissue mobility can only be capitalized on by those patients that have good baseline neurologic capability. More contemporary researchers were unable to verify quantitative improvements after Rolfing in a randomized control trial of patients 9 years old Do gait analysis and if decreased dorsiflexion or increased early stance phase plantarflexion moment or toe strike AND

NO

< 7 years old

Dorsiflexion present but plantarflexion due to spasticity Bululinum injection repeat 3-4 times

Knee flexed dorsiflexion less then 0 degrees Do open Z-lengthening of the Achilles tendon (TAL)

Type 4

Can the child tolerate AFO with ankle at neutral with the knee extended?

YES

< 9 years old

Type 3

Knee flexed dorsiflexion greater then 0 degrees Do gastrocnemus ONLY lengthening

Botulimum not effective? Tendon Achilles or gastrocnemus lengthening

No passive dorsiflexion and has fixed flexion contracture of the gastrocnemus Tendon Achilles or gastrocnemus lengthening

> 7 years old Gait analysis to confirm type 2 hemiplegia What is the ankle dorsiflexion with knee flexed?

Greater than 0 degrees

Less than 0 degrees

Do gastrocnemus lengthening

Do open TAL

(continued)

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Gait Analysis Interpretation in Cerebral Palsy Gait: Developing a Treatment Plan

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Table 1 (continued) Gait Treatment

Type 4

Type 3 A. Hemiplegia (continued)

Treat ankle with Type 2 protocol Does the child need ankle muscle surgery?

Foot contact knee flexion of 15 degrees greater than normal side

NO

Do indicated ankle surgery

Is there >10 degree fixed knee flexion contracture?

Do distal hamstring lengthening

Knee flexion in swing phase of less than 50 degrees or late peak knee flexion & rectus EMG active & toe drag

Torsional malalignment with foot progression greater than 10 degrees internal or greater than 20 degrees external foot progression angle

Treat ankle with Type 2 protocol Does the child need ankle muscle surgery?

YES

Does the child also have...?

[Does the child need treatment for SHD? (Use hip protocol)]

YES

NO

Do distal hamstring lengthening

Continue therapy & orthotics

Do rectus transfer Correct at femur or tibia or both if needed

YES Correct all deformities as indicated on full analysis Use same indications as when no other surgery needed

NO

Is there more than 1 cm leg shortening? Do femoral epiphysiodesis at appropriate age

Has increased stance phase hip flexion and greater than 20 degrees hip flex contracture? Do a psoas myofascial lengthening

Has more than 10 degrees of stance phase internal hip rotation and less than 15 degrees external hip rotation on PE?

If child is still making function gains just continue therapy

Do a femoral derotation

(continued)

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Table 1 (continued) Gait Treatment B. Diplegia What is the child’s age?

50, >25 degrees knee flexion at foot contact, >40 degrees knee flexion midstance, or >10 degrees knee flexion contracture Do distal hamstring lengthening followed with knee extension splinting

Symptoms of in or out toeing & foot progression greater than 0 degrees internal or foot progression greater than 10 degrees external

Severe toe drag, greater than 100 cm/sec walking velocity, peak knee flexion 10 degrees; on physical examination external hip rotation 20 degrees, and >60 degrees popliteal angle

YES

NO

Do analysis to determine if impairment is correctable

Continue with therapy & devices until functional plateau

Severe internal hip rotation in stance of >20 degrees

Ankle equinus 15 degrees Do knee capsulotomy

Severe hip adduction with scissoring in stance and 60 cm/sec walking velocity with independent gait & decreased knee flexion in swing Do rectus transfer

Planovalgus feet with or without external tibial torsion Do planovalgus correction &, if needed, tibial osteotomy

The primary cause of midstance knee flexion may also originate in the knee joint. The most typical is a patient with a fixed knee flexion contracture, who is unable to get full knee extension

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even when supine. Contracture of the hamstring muscles is another sequela or etiology of midstance knee flexion. The role of the hamstring in midstance knee flexion has generated extensive research and considerable controversy. Modeling has demonstrated that often the origin to insertion length of the hamstring muscle is longer than normal or normal length. This however does not consider the fiber length of the muscle and the fact that when the knee is in severe flexion, the mechanical advantage of the muscle to create further knee flexion is much greater than its ability to function as a hip extender (Arnold et al. 2006). There are also a number of papers which have shown that lengthening the hamstring will improve knee extension in midstance and foot contact regardless of the preoperative length (Park et al. 2009; Laracca et al. 2014; Carney et al. 2006). Although hamstring contractures and fix knee flexion contractures may be secondary deformities due to poor control at the level of the foot, they are still deformities which usually require treatment to correct the midstance knee flexion. Another relatively common problem in adolescence for patients with severe midstance knee flexion is anterior knee pain due to stress fractures of the patella, apophysitis, or tendinitis of the patellar tendon. This anterior knee pain is always a secondary response to high stress placed on that knee extension mechanism due to incompetence at the ground reaction force level of the plantar flexion-knee extension couple. The primary treatment for this should be determining the primary cause of the incompetence of the plantar flexion-knee extension couple. In some patients the incompetence and stretching out of the knee extensor mechanism have become severe enough that it also requires treatment as it is now a fixed secondary deformity. In some patients the primary deformity driving increased knee flexion in midstance may be a hip flexion contracture. It is most common that the hip flexion contracture is secondary and that often the amount of hip extension lag is considerably less than the physical examination of the hip flexion contracture. Severe femoral anteversion may also be either a primary or a secondary factor related to

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increased knee flexion in midstance. There is considerable uncertainty whether the position of the hip on the kinematic evaluation or the structural position of the femur by physical examination is more important in determining the functional impact of the hip extensors in midstance phase. It is my opinion that the primary judgment should be based upon the kinematic position of the hip during midstance and not the physical examination. There are some adolescents and children who by physical examination have quite significant increased femoral anteversion but who are able to control it very well and function without any apparent impact on their gait. Increased anterior pelvic tilt and lumbar lordosis are also associated with increased knee flexion in midstance. It is very difficult to ascertain if this is a primary deformity or if it is a compensatory secondary deformity. There is another group of children and adolescents who have posterior pelvic tilt with high knee flexion in midstance. It is these individuals who typically have shortened hamstrings from origin to insertion and who have the pelvic position most likely as a secondary effect of the hamstring contracture. In summary midstance positional problems are most often related to problems emanating from the foot and ankle complex. This may be due to true equinus in which there is the inability to get the foot flat causing knee flexion, or it may be the incompetence of the foot through collapse such as planovalgus or equinovarus or torsional malalignment such as internal or external tibial torsion. Other primary causes of increased knee flexion in midstance include knee flexion contractures, hamstring contractures, hip flexion contractures, and femoral torsional malalignment. Secondary effects include failure of the knee extensor mechanism or increased anterior pelvic tilt or lack of hip extension (Table 1).

Late Stance Phase The function of late stance phase includes having the center of mass anterior to the weight-bearing limb with pre-positioning of the ankle in

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dorsiflexion to initiate third rocker for propulsion to move the body forward. This should occur with the ankle in maximum dorsiflexion, the knee in maximum extension, and the hip in maximum extension. A strong concentric contraction of the gastrocsoleus occurs to initiate forceful plantar flexion; at the same time, there is increasing hip flexion activation. For this propulsion mechanism to be effective, there needs to be a neutral rotation alignment of the foot with the knee axis and the hip axis. Pathology which occurs and negatively affects third rocker starting at the ankle includes plantar flexion contracture. If the ankle is in plantar flexion at the initiation of third rocker, there is no opportunity for meaningful increase plantar flexion for propulsion. This propulsion against the floor is also a major driver for increasing knee flexion and hip flexion in late stance phase in preparation for swing. Poor foot position such as planovalgus and torsional malalignment also make the function of third rocker plantar flexion ineffective. Since the second most important power input aspect of late stance is hip flexors, weakness of the hip flexor and hip abductor may also cause decreased initiation to propel the thigh forward toward swing phase. Internal rotation of the hip may also be significant either as primary or secondary deformity impacting hip flexion. Another hip flexor is the rectus femoris muscle which may activate early to assist with hip flexion; however it then reduces knee flexion and becomes a problem for transitioning into swing phase. In summary late stance phase problems tend to mostly originate from position of the ankle and the stability of the foot to provide a good propulsion in third rocker. Additional primary ideologies include lack of hip flexor power, hip adductor power, or early and overactive rectus femoris (Table 1).

Early Swing Phase The primary role of early swing phase is to shorten the swing limb, and so it can clear the

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floor and swing forward without toe drag. In early swing phase, it should also be developing momentum so there is a pendulum swing bringing the foot forward in preparation for late swing and foot contact. The parameters of early swing phase should be increasing velocity of hip flexion and knee flexion with dorsiflexion at the ankle. Peak knee flexion should occur just after the foot passes the weight-bearing contralateral limb. The hip flexion moment continues until peak knee flexion with the modulation between hip flexion and knee flexion being controlled by the rectus femoris muscle. The most common pathology occurring in early swing in children with CP is the lack of clearing the foot expressed by toe drag and complaints from parents of rapid shoe wear and tripping. A common cause of toe drag is related to rectus spasticity or early initiation of the rectus muscle in late stance phase and continuing with significant activity through early swing. The abnormal rectus activity is identified by increased EMG activity of the rectus muscle compared to no activity in the vastus medialis. Vastus muscles should have very little activity going into swing phase even when they are used secondarily to support high knee flexion in late stance (Rha et al. 2015). Associated causes of toe drag maybe poor push-off due to foot positioning, poor pre-positioning in late stance, and weakness of the hip flexors. In rare cases fixed knee extension contractors may be a contributing etiology. Pure ankle equinus is seldom an etiology of toe drag. This is best demonstrated by the fact that controlling the equinus with the use of a plantar flexion limiting ankle foot orthosis almost always makes toe drag worse not better usually due to removing the push-off power from the ankle. In summary the primary pathology related to early swing phase is inability for the foot to clear the floor when swinging forward. This results in toe dragging and tripping. A common primary cause is a spastic or overactive rectus femoris muscle; however other causes may be inadequate push-off power from the plantar flexion-knee extension couple and weak hip flexor power (Table 1).

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Late Swing Phase The main function of late swing phase is to advance the foot forward to gait step length and for pre-positioning for foot contact. The role requires maximum hip flexion and knee extension to gain adequate step length. To prepare the foot for floor contact, pre-positioning of the ankle should be at neutral, the knee is in near-full extension, and the hip reaches maximum flexion. The hip starts into few degrees toward extension from maximum flexion in preparation for a smooth landing of the foot on the floor. The hip and knee are controlled by the hamstring muscles in late swing phase. The foot should be at neutral or a slight dorsiflexion and is controlled by the tibialis anterior. The most common pathology in children with CP occurs with overactive hamstrings in which there is too much knee flexion and not enough knee extension in terminal swing phase, which limits step lengthen. Other primary causes of lack of terminal knee extension include fixed knee flexion contractures, hip flexion contractures, or inadequate power generation in late stance and early swing to generate the momentum to swing the limb forward. The lack of knee extension in late swing may also be a secondary adaptation for severe equinus with the patient planning a forefoot strike. Higher knee flexion allows better absorption of the energy from the forward-falling center of mass. In summary the primary problems in late swing phase are lack of full terminal knee extension and hip flexion. A primary etiology is spastic or overactive hamstrings; however other primary etiologies may include fixed knee flexion contracture and poor power input to gain swing momentum in late stance and early swing (Table 1).

Complications Complications involved in interpreting gait analysis fall into several categories. The first is not having an adequate history in with an assumption that the neurologic system is static when in fact there is an evolving neurologic lesion. At times it

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may be difficult to be clear whether the neurologic changes identified by gait analysis are static, especially when there is no medical record or gait data demonstrating previous function. The expected functional changes due to musculoskeletal deformity development may mask an underlying progressive neurologic condition. Whenever there is a concern about a possible neurologic progression, a full neurologic evaluation is strongly recommended. I have personally seen the gait analysis of a child with diplegia pattern CP who was having progressive loss of function, which I felt was beyond the expected for the child’s neurologic involvement. Based on this assessment, we recommended an MRI evaluation of her spinal cord, and an astrocytoma of the spinal cord was identified. It is also crucial to be able to determine the difference between spasticity and movement disorder especially when considerations for musculoskeletal surgical corrections are considered. This may be especially difficult during a single clinical evaluation of a patient in whom spasticity and dystonia may have quite similar appearances. Having video evaluations of the patient’s whole body and having multiple cycles of kinematic and kinetic evaluation are crucial when there is a possibility of movement disorder. Another important element is having a caregiver or parent present who can report if the gait that they are seeing in the gait laboratory is actually reflective of what they see at home and in the community. If there is a major divergence, then one should make interpretations with caution and hopefully see the child back after 4–6 months and repeat the evaluation. Other elements which should always be considered during interpretation of gait analysis are the desires of the patient especially adolescents who may have strong opinions about what they are concerned about and what they would like to have corrected. The recommendations and interpretations of the gait analysis should be done with some consideration toward the desires of the family and the patient. This does not mean that one ignores clear mechanical problems identified by gait analysis; however, one should add to the interpretation the relative severity and functional impact which one perceives the individual issues

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currently are creating and how they might develop in the future. The impacts of the mechanical factors identified are also strongly influenced by the functional level of the patient. By this I mean GMFCS level I patient will have much greater impact from a severe torsional malalignment than a GMFCS level IV patient who is basically doing transfer and exercise ambulation.

Cross-References ▶ Foot Deformities Impact on Cerebral Palsy Gait ▶ Hip and Pelvic Kinematic Pathology in Cerebral Palsy Gait ▶ Knee Deformities Impact on Cerebral Palsy Gait ▶ Normal Human Gait

References Arnold AS, Liu MQ, Schwartz MH, Ounpuu S, Dias LS, Delp SL (2006) Do the hamstrings operate at increased muscle-tendon lengths and velocities after surgical lengthening? J Biomech 39:1498–1506 Baddar A, Granata K, Damiano DL, Carmines DV, Blanco JS, Abel MF (2002) Ankle and knee coupling in patients with spastic diplegia: effects of gastrocnemius-soleus lengthening. J Bone Joint Surg Am 84-A:736–744 Carney BT, Oeffinger D, Meo AM (2006) Sagittal knee kinematics following hamstring lengthening. Iowa Orthop J 26:41–44 Ferrari A, Brunner R, Faccioli S, Reverberi S, Benedetti MG (2015) Gait analysis contribution to problems identification and surgical planning in CP patients: an agreement study. Eur J Phys Rehabil Med 51:39–48 Filho MC, Yoshida R, Carvalho Wda S, Stein HE, Novo NF (2008) Are the recommendations from threedimensional gait analysis associated with better postoperative outcomes in patients with cerebral palsy? Gait Posture 28:316–322 Gough M, Shortland AP (2008) Can clinical gait analysis guide the management of ambulant children with bilateral spastic cerebral palsy? J Pediatr Orthop 28:879–883 Gough M, Schneider P, Shortland AP (2008) The outcome of surgical intervention for early deformity in young ambulant children with bilateral spastic cerebral palsy. J Bone Joint Surg (Br) 90:946–951

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Kadhim M, Miller F (2014) Crouch gait changes after planovalgus foot deformity correction in ambulatory children with cerebral palsy. Gait Posture 39:793–798 Laracca E, Stewart C, Postans N, Roberts A (2014) The effects of surgical lengthening of hamstring muscles in children with cerebral palsy–the consequences of pre-operative muscle length measurement. Gait Posture 39:847–851 Lofterod B, Terjesen T (2008) Results of treatment when orthopaedic surgeons follow gait-analysis recommendations in children with CP. Dev Med Child Neurol 50:503–509 Niklasch M, Doderlein L, Klotz MC, Braatz F, Wolf SI, Dreher T (2015) Asymmetric pelvic and hip rotation in children with bilateral cerebral palsy: uni- or bilateral femoral derotation osteotomy? Gait Posture 41:670–675 Park MS, Chung CY, Lee SH, Choi IH, Cho TJ, Yoo WJ, Park BS, Lee KM (2009) Effects of distal hamstring lengthening on sagittal motion in patients with diplegia: hamstring length and its clinical use. Gait Posture 30:487–491 Perry J, Burnfield JM (2010) Gait analysis: normal and pathological function. SLACK, Thorofare Perry J, Thorofare NJ (1992) Gait analysis: normal and pathologic function. Slack, Thorofare Rasmussen HM, Pedersen NW, Overgaard S, Hansen LK, Dunkhase-Heinl U, Petkov Y, Engell V, Baker R, Holsgaard-Larsen A (2015) The use of instrumented gait analysis for individually tailored interdisciplinary interventions in children with cerebral palsy: a randomised controlled trial protocol. BMC Pediatr 15:202 Rha DW, Cahill-Rowley K, Young J, Torburn L, Stephenson K, Rose J (2015) Biomechanical and clinical correlates of swing-phase knee flexion in individuals with spastic cerebral palsy who walk with flexedknee gait. Arch Phys Med Rehabil 96:511–517 Rha DW, Cahill-Rowley K, Young J, Torburn L, Stephenson K, Rose J (2016) Biomechanical and clinical correlates of stance-phase knee flexion in persons with spastic cerebral palsy. PM R 8:11–18. quiz 18 Rodda JM, Graham HK, Carson L, Galea MP, Wolfe R (2004) Sagittal gait patterns in spastic diplegia. J Bone Joint Surg (Br) 86:251–258 Salazar-Torres JJ, McDowell BC, Kerr C, Cosgrove AP (2011) Pelvic kinematics and their relationship to gait type in hemiplegic cerebral palsy. Gait Posture 33:620–624 Wren TA, Otsuka NY, Bowen RE, Scaduto AA, Chan LS, Dennis SW, Rethlefsen SA, Healy BS, Hara R, Sheng M, Kay RM (2013) Outcomes of lower extremity orthopedic surgery in ambulatory children with cerebral palsy with and without gait analysis: results of a randomized controlled trial. Gait Posture 38:236–241

Gait Treatment Outcome Assessments in Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1430 Evaluating Individual Domains . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Body Function and Structure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Health Condition–Related Quality of Life . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Personal Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Participation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Environment Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1434 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1435 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1435

Abstract

Children with cerebral palsy (CP) who are able to ambulate almost all require treatment to improve their ambulatory abilities throughout growth and development. The treatment includes many modalities such as physical therapy, botulinum toxin, dorsal rhizotomy, orthopedic surgery, and the use of orthotics and assistive devices. In addition to all of these treatment modalities, the child’s gait is also strongly influenced by their normal motor development and the environment in which the child is living. To understand the individual impact of each of these influences requires

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_100

good long-term outcome studies. And additional impact is the wide variety of motor ability for children with CP who are able to walk. The gross motor function classification system (GMFCS) is our current state-of-the-art method for classifying individual motor abilities. In addition to considering the individual’s level of impairment, it is important to consider the other domains as defined by the International Classification of Functioning, Disability and Health (ICF) which impact the individual’s function in society. This requires that we consider the person’s overall health condition as well as the local environmental factors which influence the person’s ability to be active. It is also important to consider the opportunity and personal interest in participation of specific activities and other personal factors which may influence their level of activity. In this 1429

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context of multiple treatment modalities overlaying with multiple individual personal factors to assess makes outcome assessment a complicated and challenging problem. The goal of this chapter is to assess the individual areas which need to be evaluated. Keywords

Cerebral palsy · GDI · GPS · PODCI · PEDICAT · FAQ · Gait outcome

Introduction Children with cerebral palsy (CP) who are able to ambulate almost all require treatment to improve their ambulatory abilities throughout growth and development. The treatment includes many modalities such as physical therapy, botulinum toxin, dorsal rhizotomy, orthopedic surgery, and the use of orthotics and assistive devices. In addition to all of these treatment modalities, the child’s gait is also strongly influenced by their normal motor development and the environment in which the child is living. To understand the individual impact of each of these influences requires good long-term outcome studies. And additional impact is the wide variety of motor ability for children with CP who are able to walk. The gross motor function classification system (GMFCS) is our current state-of-the-art method for classifying individuals motor abilities. The individual with GMFCS level I who has a gait that is functionally almost normal may respond very differently compared to individuals at GMFCS level III who require the use of an Fig. 1 International classification of functioning, disability and health defines the interactions between the person’s health condition, body function impairment, environment, personal factors, and participation level in their overall activities

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assistive device to walk. In addition to considering the individual’s level of impairment, it is important to consider the other domains as defined by the International Classification of Functioning, Disability and Health (ICF) which impact the individual’s function in society (Fig. 1). This requires that we consider the person’s overall health condition as well as the local environmental factors which influence the person’s ability to be active. It is also important to consider the opportunity and personal interest in participation of specific activities and other personal factors which may influence their level of activity. In this context of multiple treatment modalities overlaying with multiple individual personal factors to assess makes outcome assessment a complicated and challenging problem (Franki et al. 2014). The goal of this chapter is to assess the individual areas which we need to investigate. To accomplish this goal, we need to determine what outcome assessment tools are available and are useful to understand the impact of gait treatment in children with CP and their final functional outcome.

Evaluating Individual Domains Body Function and Structure The treatment of the child with the gait impairment is relied heavily on impacting specific body function areas such as stretching muscles, working on orthopedic realignments, and muscle lengthening as well as reducing spasticity. The most significant major advancement in the assessment of the gait abnormalities in cerebral palsy

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has occurred with the development of threedimensional diagnostic data analysis (Gage 1983). Gait analysis has allowed very detailed definition of the pathologic abnormalities with the goal of formulating treatment plans to address the specific impairment. As a consequence, most of the outcome assessment focus has also been in utilizing very detailed postoperative and longer-term three-dimensional gait analysis. This is an excellent tool for assessing the change in gait parameters such as the specific change in joint range of motion or joint position in different phases of gait. This is also an excellent tool for assessing gait symmetry, gait velocity, and cadence. An example of this approach is the investigation of a child who is having problems with toe dragging and tripping. On further investigation the problem was defined on three-dimensional gait analysis as having a delayed or low peak knee flexion in swing phase. EMG assessment often shows an early or overactive rectus femoris muscle as one of the etiologies. Based on this determination, prescription for a rectus femoris transfer or resection may be made. The outcome assessment of this procedure requires a follow-up three-dimensional gait analysis to assess if the knee flexion velocity or peak knee flexion has increased (Miller et al. 1997; Lee et al. 2014; Dreher et al. 2012). It is also important to follow up 10 or 20 years after surgery to see if the improvement is maintained long term (Saw et al. 2003; Haumont et al. 2013). It should also be important to ascertain if the degree of tripping and toe drag which was the patient’s complaint has also improved. As three-dimensional gait analysis developed, the initial outcome studies focused heavily on comparing self-selected gait velocity, step length, and various joint ranges of motion. The specific outcomes often focused on specific areas targeted for treatment as an example above when rectus transfer was performed and the primary outcome measures would be peak knee flexion in swing, timing of knee flexion in swing, and total knee range of motion during the gait cycle (Niiler et al. 2007). These are important outcome measures because the treatment was specifically targeted to impact these impairments. However, these are not the only impairments of interest.

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The gait of the child with CP is complex and often individual impairments at one joint do not necessarily translate to improvement in the gait overall. Recognition of this more global impact requires a more global assessment of the overall gait function. There has been an overall general agreement that the goal of treating the child with cerebral palsy is to move the gait pattern toward the function of an unimpaired individual. Therefore, improvement is considered to have been made when the child’s gait moves toward a normal gait pattern of the unimpaired age-matched child. Gait velocity, cadence, and step length are considered more global measures. The outcome of these global measures is complicated by the fact that as the child’s stance stability improves, their self-selected gait speed may actually decrease so it is difficult to ascertain if this is improvement or deterioration. Other individuals especially at GMFCS level IV may have very slow gait speeds in which the individual joint impairments are largely a byproduct of the slow gait velocity. Global assessment of an impairment which is been reported is the changing need for different assistive devices (Gough et al. 2004). A child who moves from the use of a posterior walker, to forearm crutches, to independent ambulation is considered to be improving toward the normal ambulatory ability of reduced need for assistive device. These changing needs are often a heavy focus of parents as they are desiring to see improvement in the child’s ambulatory ability. This also gets confusing when there is a difference in what a child can do and what the child is most functional using. The problem with using assistive device level as a definitive outcome measure is that it is very dependent upon the child’s motivation, the environment in which the child lives, and the opportunity for exploring the use of different assistive devices. Functional motor score (FMS) is a validated instrument that has been largely dependent on assessing different levels of assistive device use (Graham et al. 2004; Harvey et al. 2007). The advantage of the FMS scale is that it assesses how active the child currently is in different environments. There are six levels of function that are to be assessed using three different environments. The first environment rates

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going short distances of approximately 5 m such as the child ambulating in their own home. The second distance evaluates function at the 50 m level, more typically how the child would ambulate in their school environment. The third level determines how the child would ambulate if they require to go a distance of 500 m such as a large shopping mall or an amusement park. Another global functional validated tool is the Gillette Functional Activity Questionnaire (FAQ) which has 10 levels and provides an assessment of the functional mobility in the home and community (Novacheck et al. 2000). Both of these tools provide good assessments of the individual child’s current functioning especially related to which specific assistive devices they require. These are excellent tools to monitor for change overtime. Children with cerebral palsy have an increased energy cost of walking due to the impairment of the abnormal gait. And early optimistic goal of outcome assessment was that by improving the child’s gait pattern, there would be a decrease in their energy cost of walking by making their gait more energy-efficient. However multiple studies have found that this is a very complex measure and to realistically be able to improve oxygen consumption by doing correction of specific gait pathologies is generally not realistic. One of the problems with this measure when it is used in childhood is that as the child grows and increases body mass, energy cost of walking measured by ml(O2)/kg (body weight)/meter(walked) will decrease (Kamp et al. 2014). This is true of normal individuals, and it is true of individuals with CP. Therefore, when one assesses the oxygen cost of walking in an 8-yearold and then assesses it again 1, 5, and 10 years later, it has to be normalized to the expected change of improvement just from gaining body mass. When this approach is utilized, there are seldom major changes in the oxygen cost of walking except for children who have very severe gait impairments corrected. As the interest increased for better composite assessments, new global calculation calculated measures were developed from the threedimensional gait analysis data. Initial attempt at this was the Gillette Gait Index (GGI) which attempted to reduce all of the gait data to a single

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number by comparing it to its deviation from normal gait data. This index provided a single number which was nondimensional and was not standardized to understand how far from normal the individual was but did show reliable trends of improvement (Wren et al. 2007). The measure was nonlinear, so it would have very large increases for reasons that were hard to understand. A further refinement of this measure, now called the Gait Deviation Index (GDI), was developed and uses normal gait data to assess how far the individual’s gait deviations are from normal (Schwartz and Rozumalski 2008; Molloy et al. 2010). GDI reduces the normal number to 100 each one standard deviation from the normal mean is 10 points. This calculation has now greatly reduced the nonlinearity of the assessment and makes it more objectively evident about how abnormal and individual’s gait pattern is. A problem with the GDI, however, is that it is still difficult to understand exactly which component of the impairment is creating the assessment of the abnormality. One can also calculate a gait deviation index for each individual joint and typically the GDI is calculated for one side of the body and the mean of the two sides is used for the whole body. The Gait Profile Score (GPS) was developed to be similar to the GDI; however, it directly reports the deviation of abnormal motion in degrees of joint motion through the gait cycle (Baker et al. 2009). This is a simpler measure in that it summates and then means amount of motion beyond difference of the normal one standard deviation from normal mean of the individual changes at each joint level. The measures of the GDI and GPS have been shown to correlate (Baker et al. 2009; Rasmussen et al. 2015) with minimally clinically significant differences for GDI being 10 and for GPS being 1.6 degrees (Baker et al. 2012). A kinetic GDI is also available to assist in defining the kinetic profile of an individual (Rozumalski and Schwartz 2011). The use of Gross Motor Function Measures (GMFM) as an outcome measure of body function is also widely reported. Although this is not a specific measure related to gait, it does assess motor function of many activities which are directly related to gait and increasing scores

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clearly correlate also with improvement in gait function. This is not a very specific measure of improvement and is more of a general body and motor coordination measure of change.

Health Condition–Related Quality of Life There aren’t currently many validated instruments to assess health condition related to quality of life. It is a challenge to determine the best instrument for an individual situation. These tools are largely patient or caretaker reported outcomes and that such are very excellent tools to evaluate change over time as they are reported by the patient and/or their caretakers. The most commonly available instruments currently reported for use to assess outcome of gait treatment include Child Health Questionnaire (CHQ), Pediatric Outcomes Data Collection Instrument (PODCI), and Pediatric Evaluation and Disability Inventory (PEDI) and the Pediatric Evaluation and Disability Inventory – Computer Assisted Testing (PEDI-CAT). One of the problems with these patient reported outcome instruments is that none of them are specifically targeted at evaluating the outcome of gait treatment in children with CP. They are targeted at understanding the child’s general function, and this is a very important aspect for ongoing follow-up. Each of these tools has its positives and negatives, and selecting the correct one requires both understanding the strengths and weaknesses and choosing one that is available. The CHQ is the most general evaluation tool of the child’s health status and their function and well-being. This questionnaire is not directed at a child with physical disability but does ask questions related to physical function. It is really directed at any child health problems and their impact on the child’s function and quality of life (Akerstedt et al. 2010; Thomason et al. 2011; Wren et al. 2013). As such CHQ has less direct relevance when the goal is specifically measuring the outcome of gait treatment in children with cerebral palsy. The PODCI was specifically developed to evaluate children with pediatric orthopedic disabilities. It evaluates the child in five

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domains, extremity and physical functioning, transfer and basic mobility, sports and physical functioning, pain, happiness, and global functioning. Either caretakers or the patient can selfreport. For children over age 8, it is useful to have both parents and child report separately, since we have found that there may be significant differences reported. Since the scale is developed specifically for children with pediatric orthopedic disabilities, it tends to be very relevant for a group of children, GMFCS levels II and III (Cuomo et al. 2007). The problem with this instrument is that children functioning at GMFCS level I often are so close to normal that there is a strong ceiling effect and there is no ability to measure any change over time although this may not always be the case (McMulkin et al. 2007). GMFCS levels IV and V have the opposite problem with a floor effect due to the score being so low that is very difficult to make a positive or negative impact that can be measured (Sullivan et al. 2007). The PEDI is another instrument which was developed to assess primarily physical disability and has had many of the same benefits and problems as the PODCI, with floor and ceiling effects. There has been a lot of effort placed into developing the computer-assisted PEDI (Pedi-CAT), and as a consequence, the PEDI-CAT is now a very robust tool. There has also been a lot of effort at working to improve the function of the upper and lower functional levels. Currently the ceiling effect aspect of the PEDI-CAT seems to be greatly improved; however, there is still a need for further work on the floor effect for GMFCS IVand V. The real benefit of the PEDI-CAT is that it is simpler with fewer questions to gain the same scoring because of the computer-assisted design techniques. This makes it an excellent option for use in a clinical gait analysis laboratory (Haley et al. 2011; Dumas and Fragala-Pinkham 2012; Fragala-Pinkham et al. 2016; Kramer et al. 2016; Dumas et al. 2017; Shore et al. 2017).

Personal Factors In an attempt to evaluate underlying personal factors and goals of individuals, Goal Attainment

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Scale (GAS) has become more commonplace in the assessment of treatment for children with gait disability from CP. The mechanism for using Goal Attainment Scales varies between having the professional investigator assist the patient in developing goals, and in other situations, it is entirely the patient or the family who develops the goals. After the individual goals are defined, the outcome is then scored by how many of these goals are met at the defined end point. This has a theoretically very positive advantage for children with gait disabilities, in that it gives the individual a sense of being able to define their own goals and should motivate them to work toward these goals as part of the rehabilitation program. The Goal Attainment Scale maybe the most important aspect to the family or to the individual; however, it does not correlate with specific impairment correction of kinematic or kinetic deficits as measured by GPS (McMorran et al. 2016). There is still a significant spread between the outcome based on GAS and other measures GDI, PODCI, and FAQ (Gordon et al. 2011). These outcome differences suggest that there is a need to improve methods for defining attainable goals.

Environment Factors

Participation

Conclusions

Children with CP tend to have participation through their connection with the educational system. And at this time, there is no specific instrument to evaluate participation for children. Participation becomes a much bigger issue after individuals leave the educational system and then either become independent functioning adults with jobs and families or continue to be dependent at some level. Some of this participation is based on their physical mobility function, but a lot is based upon their cognitive and psychosocial function. There are excellent wellvalidated modules in the Patient-Reported Outcomes Measurement Information System (PROMIS) outcomes evaluation tool to assess participation. The Canadian Occupational Performance Measure (COPM) is another tool to assess participation in adults with physical disabilities (Gannotti et al. 2013).

Our recommendation for outcome evaluation following treatments of the impairments for children’s CP gait abnormality has to include a direct technical assessment of the impairment be addressed. This means if the goal is to improve knee flexion in swing phase, then this parameter has to be measured preoperatively and postoperatively in a consistent and accurate way. If the goal is to decrease toe walking or equinus gait, ankle motion during gait has to be accurately assessed preoperatively and at follow-up. After assessing the focal directed impairment treatment, there should be a more global measure of gait impairment in the individual based on measure joint position. Our preferred method for this is using GDI although GPS has equal validity. To assess overall functional ability and assistive device use, we prefer to assess the patient using FMS, although the FAQ is another equal alternative.

Environmental factors such as the weather, the living environment, and the availability to be outside and participating in sport activities are all a components impacting the opportunity for individuals with gait disabilities to exercise and improve their function. Although not directly tied to strictly environmental factors, the daily amount of walking an individual does is influenced by the environment in which they are living. This is also complicated by the fact that the amount of steps an individual takes in a day is also strongly influenced by their level of impairment as assessed by the GMFCS level (Ishikawa et al. 2013; Wilson et al. 2015). We feel the use of a step count monitor is an excellent mechanism to gauge the level of recovery during the rehabilitation following major treatment interventions such as orthopedic surgery. Because of the environmental impact, it is very important to have a baseline assessment preferably in the same season of the year and with the same combination of weekend and weekdays because of the strong environmental impact.

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Assessment of general health and patient reported outcome can be done with the PODCI for GMFCS level II and III. Another robust option is to use the PEDI-CAT which seems to function better for GMFCS I and likely functions better for GMFCS level IV. To monitor rehabilitation recovery and environmental impact, we prefer to use step count monitoring with a good baseline prior to treatment. The development of a reliable method to use a Goal Attainment system seems like it would have additional promise of helping to motivate the patient and family during rehabilitation, although at this time we are not using this in the clinical environment due its questionable reliability. Another issue which needs to be considered is when to do an outcome assessment. This is entirely dependent upon the intervention being evaluating. If one is evaluating the outcome of botulinum toxin into the gastrocnemius, an evaluation at 3 months is optimal since most of the effect will have left by 6 months. Based on prior studies, there would be little merit in doing an evaluation 1 year after injection. Likewise, if one is evaluating the impact of orthotics on gait, then it is not likely that there will be a need to do a longterm evaluation, since there is no data currently that orthotics have an impact after they have been discontinued. If, on the other hand, one is doing a simple soft tissue surgery such as hamstring lengthening and gastrocnemius recession, the ideal time for a postop follow-up would be 1 year. However, if there is a high burden orthopedic surgery involving multiple osteotomies at the knee and feet, full recovery would not be expected until 2 years postoperatively, and the follow-up evaluation would best be done closer to 2 years after surgery. Therefore, the timing for an outcome evaluation following treatment has to be tailored to the specific treatment and treatment effects which are expected.

Cross-References ▶ Cerebral Palsy Gait Pathology ▶ Gait Analysis Interpretation in Cerebral Palsy Gait: Developing a Treatment Plan

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References Akerstedt A, Risto O, Odman P, Oberg B (2010) Evaluation of single event multilevel surgery and rehabilitation in children and youth with cerebral palsy–a 2-year follow-up study. Disabil Rehabil 32(7):530–539 Baker R, McGinley JL, Schwartz MH, Beynon S, Rozumalski A, Graham HK, Tirosh O (2009) The gait profile score and movement analysis profile. Gait Posture 30(3):265–269 Baker R, McGinley JL, Schwartz M, Thomason P, Rodda J, Graham HK (2012) The minimal clinically important difference for the gait profile score. Gait Posture 35(4):612–615 Cuomo AV, Gamradt SC, Kim CO, Pirpiris M, Gates PE, McCarthy JJ, Otsuka NY (2007) Health-related quality of life outcomes improve after multilevel surgery in ambulatory children with cerebral palsy. J Pediatr Orthop 27(6):653–657 Dreher T, Gotze M, Wolf SI, Hagmann S, Heitzmann D, Gantz S, Braatz F (2012) Distal rectus femoris transfer as part of multilevel surgery in children with spastic diplegia–a randomized clinical trial. Gait Posture 36 (2):212–218 Dumas HM, Fragala-Pinkham MA (2012) Concurrent validity and reliability of the pediatric evaluation of disability inventory-computer adaptive test mobility domain. Pediatr Phys Ther 24(2):171–176 discussion 176 Dumas HM, Fragala-Pinkham MA, Rosen EL, O'Brien JE (2017) Construct validity of the pediatric evaluation of disability inventory computer adaptive test (PEDICAT) in children with medical complexity. Disabil Rehabil 39(23):2446–2451 Fragala-Pinkham MA, Dumas HM, Lombard KA, O'Brien JE (2016) Responsiveness of the pediatric evaluation of disability inventory-computer adaptive test in measuring functional outcomes for inpatient pediatric rehabilitation. J Pediatr Rehabil Med 9(3):215–222 Franki I, De Cat J, Deschepper E, Molenaers G, Desloovere K, Himpens E, Vanderstraeten G, Van den Broeck C (2014) A clinical decision framework for the identification of main problems and treatment goals for ambulant children with bilateral spastic cerebral palsy. Res Dev Disabil 35(5):1160–1176 Gage JR (1983) Gait analysis for decision-making in cerebral palsy. Bull Hosp Jt Dis Orthop Inst 43(2):147–163 Gannotti ME, Gorton GE 3rd, Nahorniak MT, Masso PD (2013) Gait and participation outcomes in adults with cerebral palsy: a series of case studies using mixed methods. Disabil Health J 6(3):244–252 Gordon AB, McMulkin ML, Baird GO (2011) Modified goal attainment scale outcomes for ambulatory children: with and without orthopedic surgery. Gait Posture 33(1):77–82 Gough M, Eve LC, Robinson RO, Shortland AP (2004) Short-term outcome of multilevel surgical intervention in spastic diplegic cerebral palsy compared with the natural history. Dev Med Child Neurol 46(2):91–97

1436 Graham HK, Harvey A, Rodda J, Nattrass GR, Pirpiris M (2004) The functional mobility scale (FMS). J Pediatr Orthop 24(5):514–520 Haley SM, Coster WJ, Dumas HM, Fragala-Pinkham MA, Kramer J, Ni P, Tian F, Kao YC, Moed R, Ludlow LH (2011) Accuracy and precision of the pediatric evaluation of disability inventory computer-adaptive tests (PEDI-CAT). Dev Med Child Neurol 53(12):1100–1106 Harvey A, Graham HK, Morris ME, Baker R, Wolfe R (2007) The functional mobility scale: ability to detect change following single event multilevel surgery. Dev Med Child Neurol 49(8):603–607 Haumont T, Church C, Hager S, Cornes MJ, Poljak D, Lennon N, Henley J, Taylor D, Niiler T, Miller F (2013) Flexed-knee gait in children with cerebral palsy: a 10-year follow-up study. J Child Orthop 7(5):435–443 Ishikawa S, Kang M, Bjornson KF, Song K (2013) Reliably measuring ambulatory activity levels of children and adolescents with cerebral palsy. Arch Phys Med Rehabil 94(1):132–137 Kamp FA, Lennon N, Holmes L, Dallmeijer AJ, Henley J, Miller F (2014) Energy cost of walking in children with spastic cerebral palsy: relationship with age, body composition and mobility capacity. Gait Posture 40(1):209–214 Kramer JM, Liljenquist K, Coster WJ (2016) Validity, reliability, and usability of the pediatric evaluation of disability inventory-computer adaptive test for autism spectrum disorders. Dev Med Child Neurol 58(3):255–261 Lee SY, Kwon SS, Chung CY, Lee KM, Choi Y, Kim TG, Shin WC, Choi IH, Cho TJ, Yoo WJ, Park MS (2014) Rectus femoris transfer in cerebral palsy patients with stiff knee gait. Gait Posture 40(1):76–81 McMorran D, Robinson LW, Henderson G, Herman J, Robb JE, Gaston MS (2016) Using a goal attainment scale in the evaluation of outcomes in patients with diplegic cerebral palsy. Gait Posture 44:168–171 McMulkin ML, Baird GO, Gordon AB, Caskey PM, Ferguson RL (2007) The pediatric outcomes data collection instrument detects improvements for children with ambulatory cerebral palsy after orthopaedic intervention. J Pediatr Orthop 27(1):1–6 Miller F, Cardoso Dias R, Lipton GE, Albarracin JP, Dabney KW, Castagno P (1997) The effect of rectus EMG patterns on the outcome of rectus femoris transfers. J Pediatr Orthop 17(5):603–607 Molloy M, McDowell BC, Kerr C, Cosgrove AP (2010) Further evidence of validity of the gait deviation index. Gait Posture 31(4):479–482 Niiler TA, Richards JG, Miller F (2007) Concurrent surgeries are a factor in predicting success of rectus transfer outcomes. Gait Posture 26(1):76–81

F. Miller Novacheck TF, Stout JL, Tervo R (2000) Reliability and validity of the Gillette functional assessment questionnaire as an outcome measure in children with walking disabilities. J Pediatr Orthop 20(1):75–81 Rasmussen HM, Nielsen DB, Pedersen NW, Overgaard S, Holsgaard-Larsen A (2015) Gait deviation index, gait profile score and gait variable score in children with spastic cerebral palsy: intra-rater reliability and agreement across two repeated sessions. Gait Posture 42(2):133–137 Rozumalski A, Schwartz MH (2011) The GDI-kinetic: a new index for quantifying kinetic deviations from normal gait. Gait Posture 33(4):730–732 Saw A, Smith PA, Sirirungruangsarn Y, Chen S, Hassani S, Harris G, Kuo KN (2003) Rectus femoris transfer for children with cerebral palsy: long-term outcome. J Pediatr Orthop 23(5):672–678 Schwartz MH, Rozumalski A (2008) The gait deviation index: a new comprehensive index of gait pathology. Gait Posture 28(3):351–357 Shore BJ, Allar BG, Miller PE, Matheney TH, Snyder BD, Fragala-Pinkham MA (2017) Evaluating the discriminant validity of the pediatric evaluation of disability inventory: computer adaptive test in children with cerebral palsy. Phys Ther 97(6):669–676 Sullivan E, Barnes D, Linton JL, Calmes J, Damiano D, Oeffinger D, Abel M, Bagley A, Gorton G, Nicholson D, Rogers S, Tylkowski C (2007) Relationships among functional outcome measures used for assessing children with ambulatory CP. Dev Med Child Neurol 49(5):338–344 Thomason P, Baker R, Dodd K, Taylor N, Selber P, Wolfe R, Graham HK (2011) Single-event multilevel surgery in children with spastic diplegia: a pilot randomized controlled trial. J Bone Joint Surg Am 93(5):451–460 Wilson NC, Signal N, Naude Y, Taylor D, Stott NS (2015) Gait deviation index correlates with daily step activity in children with cerebral palsy. Arch Phys Med Rehabil 96(10):1924–1927 Wren TA, Do KP, Hara R, Dorey FJ, Kay RM, Otsuka NY (2007) Gillette gait index as a gait analysis summary measure: comparison with qualitative visual assessments of overall gait. J Pediatr Orthop 27(7):765–768 Wren TA, Otsuka NY, Bowen RE, Scaduto AA, Chan LS, Dennis SW, Rethlefsen SA, Healy BS, Hara R, Sheng M, Kay RM (2013) Outcomes of lower extremity orthopedic surgery in ambulatory children with cerebral palsy with and without gait analysis: results of a randomized controlled trial. Gait Posture 38(2):236–241

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1438 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1438 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1438 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hemiplegia Type 0 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hemiplegia Type 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hemiplegia Type 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Outcome of Plantar Flexor Tendon Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Rotational Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hemiplegia Type 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Stiff Knee Gait . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Rotational Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hemiplegia Type 4 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Rotational Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Limb Length Discrepancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1439 1440 1441 1441 1442 1443 1443 1444 1444 1444 1445 1446

Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1447 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1447 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1454 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1454

Abstract

Hemiplegic pattern cerebral palsy (CP) means the motor lesion is primarily located on one side of the body usually involving both the arm and a leg. Another synonymous term is unilateral CP. There are many children who have

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_101

primary involvement on one side of the body; however, they may have also some contralateral abnormalities. There are no clear definitions of when unilateral or hemiplegic pattern CP becomes bilateral or diplegia or quadriplegic pattern CP. Hemiplegic pattern CP makes up approximately one third of all children with the diagnosis. The vast majority of children with hemiplegic pattern CP tend to be highfunctioning community ambulators with Gross Motor Function Classification System 1437

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(GMFCS) I or II. Large majority of individuals with hemiplegic pattern CP become full and normal independent functioning members in the society. The functional ability of individuals with unilateral CP tends to be much more influenced by concurrent cognitive disability or epilepsy and then motor impairment. Many children with unilateral CP do develop musculoskeletal deformities impairing their gait pattern and upper extremity function, which are amendable to surgical correction. The Winter’s classification divides hemiplegic gait into four patterns. Type 1 has ankle plantar flexion in swing phase with an inactive or very weak tibialis anterior, which is the cause of the plantar flexion. Type 2 has an equinus gait pattern but with spastic or contracted plantar flexors, which overpower an active dorsiflexor. Type 3 includes the ankle position of type 2, further adding abnormal function of the knee joint. Type 4 includes all problems of type 3 with the addition of abnormal function of the hip joint muscles. The separation of these types is usually easy through a combination of physical examination, EMG, kinematic evaluation, and kinetic data. As with all biological groups, however, there are intermediate patients. The goal of this chapter is to review the natural history and treatment plan for the individual with hemiplegic pattern CP. Keywords

Cerebral palsy · Hemiplegia · Unilateral · Winter’s classification

Introduction Hemiplegic pattern cerebral palsy (CP) means the motor lesion is primarily located on one side of the body usually involving both the arm and a leg. Another synonymous term is unilateral CP a. There are many children who have primary involvement on one side of the body; however, they may have also some contralateral abnormalities. There are no clear definitions of when unilateral or hemiplegic pattern CP becomes bilateral or diplegia or quadriplegic pattern CP. Although most publications present these groupings as

F. Miller

absolute distinct, there is not a clear separation between the groups, and as a consequence, a significant number of children can be categorized either way. Hemiplegic pattern CP makes up approximately one third of all children with the diagnosis (Andersen et al. 2008). The vast majority of children with hemiplegic pattern CP tend to be high-functioning community ambulators with Gross Motor Function Classification System (GMFCS) I or II. Large majority of individuals with hemiplegic pattern CP become full independent normal functioning member in the society. From the perspective of the International Classification of Functioning, Disability, and Health (ICF), these individuals will often have high participation rates. The functional ability of individuals with unilateral CP tends to be much more influenced by concurrent cognitive disability or epilepsy and then motor impairment (Beckung et al. 2008). The goal of this chapter is to review the natural history and treatment plan for the individual with hemiplegic pattern CP.

Natural History and Pathophysiology Etiology The central nervous system pathology which causes hemiplegic pattern CP tends to be primarily localized to one hemisphere of the brain. There are multiple etiologies, which can create this unilateral lesion, but a common cause is third trimester anterior cerebral artery infarction (DarmencyStamboul et al. 2012). A risk factor for this last trimester infarction may be a familial hypercoagulable state. There are questions about how aggressive the work-up should be for children with hemiplegia to determine the presence of a familial hypercoagulable state especially looking for issues such as factor V Leiden deficiency, protein S, and protein C. One study has reported that screening for these in a population of children with hemiplegic CP compared with a normal cohort of children found a similar incidence (Turedi Yildirim et al. 2015). Based on current evidence, the required work-up for a child who presents with a typical hemiplegic pattern CP is

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still not clear. There are no clear benefits to doing any hematologic work-up if the family history does not suggest a hypercoagulable state. The most common presentation of hemiplegic pattern CP is parents noticing that the child is preferentially using one hand for upper extremity activities. This may be observed as early as 6–9 months of age, when typical hand dominance should not be presenting until 18–24 months. There may also be a delay in independent walking especially with asymmetric toe walking. It is common for the initial toe walking to be more symmetric then suggested by the physical examination of mild increased muscle tone and likely reduced range of motion in the affected ankle. The hand is often in a fisted position with the thumb held in the palm. When the child is crawling, they may be crawling on the fisted hand or occasionally even utilizing the dorsum of the right wrist as a support. In this early stage between 9 and 18 months of age, the wrist is usually in a neutral position at the elbow held in flexion. As the child develops good independent ambulation, the unaffected limb tends to come down to the side and by 3–4 years of age develops reciprocal swing with the contralateral leg. The involved upper extremity tends to be held with slight shoulder flexion and internal rotation and elbow flexion, and then gradually the wrist will become slightly flexed, and the fingers start to open up from the flexed finger position. By 5–6 years of age, the child holds the plegic arm in the typical position that is usually recognized as hemiplegia with slight shoulder flexion and internal rotation, elbow flexion, and forearm pronation and wrist in mild flexion with fingers extended and thumb adducted. During late childhood and into adolescence, the shoulder flexion and internal rotation and elbow-flexed posture of the upper extremity during gait tend to relax, and the arm comes down to the side developing more normal posture. It is also during this time that the forearm pronates more; the wrist may become more flexed with the thumb adducted with the fingers having more difficulty with grasping. This change is usually seen as cosmetic improvement but not as functional improvement (Riad et al. 2007a, 2011)

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(▶ Chap. 108, “The Upper Extremity in Cerebral Palsy: An Overview”). The ability to clearly classify a child with hemiplegia as opposed to diplegia or quadriplegia may be ambiguous. Many children by 18 months of age with classic presentation of asymmetric early hand use and toe walking with classic asymmetric physical examination can be given with the diagnosis of hemiplegic CP. There is a subgroup of children who will make very large gains and by 4 or 5 years of age may essentially look normal. Another group of children with similar findings may develop much more apparent skeletal manifestations and even some abnormality on the contralateral side making the pattern identification an asymmetric diplegia. Another group of early ambulators appeared to be very symmetric and appeared to have a diagnosis of bilateral CP but, as the child grows especially by age 5–6 years, may present as unilateral CP. Therefore, it is wise not to be overly confident in explaining to families with young children under age 3 as to the exact long-term outcome. However, it is clear that if the child is independently ambulating, one can have great confidence that the child will be GMFCS I or II as an adult.

Treatment Many children with unilateral CP do develop musculoskeletal deformities impairing their gait pattern and upper extremity function, which are amendable to surgical correction. A few children, usually with severe mental retardation, do not become functional ambulators (GMFCS III) (Beckung et al. 2008). Often, inability to have functional ambulation (GMFCS IVor V) is related to poor function in the upper extremity, which makes the use of an assistive device difficult. There have been several attempts to classify patterns of hemiplegic gait (Hullin et al. 1996; Winters et al. 1987), but the classification of Winters et al. (1987) is easy to remember and has the most direct implications for treatment (Fig. 1). This classification divides hemiplegic gait into four patterns. Type 1 has ankle plantar flexion in swing phase with an inactive or very weak tibialis

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Fig. 1 The best classification of hemiplegia is that of Winters et al. (1987) in which type 1 is due to a weak or paralyzed ankle dorsiflexor causing a drop foot. Type 2 has equinus foot position due to a contracture of the gastrocnemius or gastrocsoleus preventing dorsiflexion. Type 3 has spastic or contracted hamstrings or quadriceps muscles in

addition to type 2 ankle. Type 4 has spastic or weak hip muscles in addition to type 3 deformity. Almost all patients are relatively easy to classify into one or the other type, which is then helpful for planning treatment. Transverse rotational plane malalignments do not fit into this classification and should be seen as an additional problem

anterior as the etiology of the plantar flexion. The pattern is most common in adult stroke or peroneal nerve palsy. Type 2 has an equinus gait pattern but with spastic or contracted plantar flexors, which overpower an active dorsiflexor. Type 3 includes the ankle position of type 2, further adding abnormal function of the knee joint. The knee has increased spasticity in the hamstring or rectus with increased forefoot contact and stance phase knee flexion with or without decreased swing phase knee flexion. Type 4 includes all problems of type 3 with the addition of abnormal function of the hip joint muscles. Type 4 manifests with increased tone in the adductors and hip flexors and with internal femoral torsion. The separation of these types is usually easy through a combination of physical examination, EMG, kinematic evaluation, and kinetic data. As with all biological groups, however, there are intermediate patients. A modification of the system was the addition of type 0 added by Riad et al. (2007b). This is a group of patients who are not able to clearly be placed in either type 1 or

2 because they have relatively neutral ankle position. These tend to be very mild patients who have decreased ankle dorsiflexion but have a foot flat strike and come to some dorsiflexion during the second rocker. Patients who have been treated for type 2 with plantar flexor lengthenings often fall into this pattern. The Winter’s classification system does not consider transverse plane deformities; however, most children with significant residual internal femoral torsion are classified as type 4. Tibial torsion is uncommon but may occur with types 2, 3, and 4.

Hemiplegia Type 0 Patients with hemiplegic type 0 pattern CP are the second most common group. Many of these patients were originally classified as type 2 but have been treated with plantar flexor lengthenings, so they have an ankle that is in a neutral position typically with some limited dorsiflexion and also some reduced active plantarflexion (Riad

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et al. 2007b). The moments generated at the ankle are usually reduced in magnitude compared to the unaffected side, but they have relatively normal timing of the moment. The primary treatment for this group is ongoing monitoring and focusing on activity especially muscle strength training (Lee et al. 2008). If these children are still in middle childhood under the age of 10, there is a reasonably high risk that during the adolescent growth, spurt equinus will reoccur and they’ll fall back into the type 2 pattern. The use of orthotics to limit plantarflexion is somewhat controversial. It is likely possible with the diligent use of orthotics to probably prevent recurrent equinus contracture; however, the risk is increasing muscle weakness, which is always present. All attempts should be made to avoid the weakness making it worse. Children with hemiplegia tend to migrate their power generation to the proximal muscles, so even the uninvolved side has a tendency to do less power generation and be somewhat weaker. Strength training should focus on the whole lower extremity bilaterally and not only on a single involved calf muscle (Riad et al. 2008).

Hemiplegia Type 1 In CP hemiplegia type 1 is the least common pattern of involvement. Type 1 occurs more with adult stroke or with a peripheral nerve injury. If this type is identified in a child with CP, the physical examination will demonstrate full passive dorsiflexion; however, no active dorsiflexion can be elicited. The kinematic examination will show plantar flexion at initial contact and no dorsiflexion in swing phase. The EMG will demonstrate a tibialis anterior that is silent or nearly silent. The primary treatment for type 1 hemiplegia is a relatively flexible leaf spring AFO (Case 1). In very rare situations where the tibialis posterior has normal tone and normal phasic firing, the tibialis posterior can be transferred through the interosseous membrane to the dorsum of the foot. However, this transfer is mainly used with peripheral nerve palsy. With central lesions, relearning is difficult as this is an out-of-phase transfer, and transfer of the spastic tibialis

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posterior through the interosseous membrane leads to very severe foot deformities over time and should be avoided.

Hemiplegia Type 2 The most common subtype of hemiplegia is type 2, making up approximately 75% of all children with hemiplegia. Typically, children learn to walk independently between 15 and 20 months of age, either with toe walking or foot flat with a planovalgus. The early treatment is to provide the children support through the use of an orthotic, usually starting with a solid ankle AFO and then with an articulated AFO for the second orthotic. If a child has a very spastic gastrocsoleus, botulinum toxin injection for two or three cycles can help parents apply the AFO and make AFO wear more comfortable for the child. Usually, by 4–7 years of age, the gastrocsoleus contracture has become so severe that brace wear is no longer possible. On physical examination, children often demonstrate a contracture of both the gastrocnemius and soleus. The kinematic examination will show equinus throughout the gait cycle, and knee flexion at foot contact may be increased as children preposition the knee to avoid high external extension moments from the ground reaction force during weight acceptance. Often, these children will be toe walking on the unaffected side as well, and a careful assessment is required to make sure that this is compensatory toe walking and not mild spastic response in a limb that was erroneously thought to be normal. The physical examination and kinematic evaluation are most useful for this assessment. The unaffected ankle should have adequate dorsiflexion measuring 5–10 with knee extension. The ankle moment should show normal late stance phase plantar flexion moment or a variable moment, one or two of which look almost normal. The affected ankle will also be more consistently abnormal with high early plantar flexion moments. If children have been allowed to walk on the toes until late middle childhood, their unaffected ankles will often develop plantar flexion contractures from persistent toe walking. The physical examination will

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show a reduced ankle range of motion, and the ankle moment will still show the same variability with much better power generation than the affected ankle. The step length of the affected side is usually longer, and the stance phase time of the normal limb is longer. These changes occur because the affected leg has a normal swing phase, due to normal knee and hip motor control, but is more unstable in stance phase. If the normal ankle has a fixed contracture, it will need a gastrocnemius lengthening, or this normal ankle will become a driving force toward toe walking after correction of the contracture on the primarily involved side (Case 2). For patients with severe equinus contractures, typically, those who are unable to come within 20 to plantigrade will have significantly overlengthened tibialis anterior muscle and tendon. After appropriate plantar flexor lengthening usually with an open tendon Achilles lengthening, the tibialis anterior is not efficient to provide adequate dorsiflexion because of its redundancy. The tendon will slowly readjust its length; however, a significant period of time will be required during which time the individual has to wear an articulated or leaf spring AFO to prevent foot drop in swing phase. For a child under age 10, this period of time may be a number of months; however, for an older individual past skeletal maturity, the time frame is often a couple of years. This long-term need for orthotic use further magnifies the weakness that is already present in the plantar flexor muscles. Based on this, for an individual past the age of 10, we would recommend considering a plication of the tibialis anterior tendon to preclude this long-term need for orthotic management. Results of this procedure have documented good postoperative active ankle dorsiflexion (Rutz et al. 2011; Tsang et al. 2016). We have found that it is wise to make sure there is at least 10 plantarflexion after the tendon has been shortened, because too much shortening can create a calcaneus gait pattern if the child loses too much plantarflexion.

Outcome of Plantar Flexor Tendon Lengthening The need for postoperative orthotic use varies, but braces are not routinely needed. If children do not

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gain foot flat at initial contact by 3–6 months after surgery, an AFO should be used, usually an AFO that allows dorsiflexion to encourage the tibialis anterior to gain function. This AFO can be either an articulated AFO or a half-height wraparound AFO with an anterior ankle strap. With appropriate early treatment, most children with type 2 hemiplegic pattern CP can be free of an orthosis by early grade school. Some children will develop an equinus contracture again in late childhood or adolescence. If an adolescent is willing to tolerate the orthosis, another round of Botox injections and orthotic wear can delay surgery until he/she is near the completion of growth. Approximately 25% of type 2 hemiplegics will need a second gastrocnemius or tendon Achilles lengthening in adolescence (Joo et al. 2011). Younger age at the first lengthening and severe equinus deformity are related to recurrence. It is also important to recognize that having a recurrent equinus deformity is much preferred over having an overlengthened plantar flexor that is incompetent. The risk of overlengthening is less in individuals with hemiplegia and then with bilateral involvement because they have at least one good limb to stand on. Adolescents or young adults with type 2 hemiplegia should seldom need to wear an orthosis after their last lengthening. Routine long-term use of an AFO should be avoided as this will weaken the muscle and make the person permanently brace dependent. Long toe flexor spasticity may also be present, but this seldom needs surgical treatment. In early childhood, the feet are often in a planovalgus position; however, as children gain increased tone, gastrocnemius and soleus equinus develop. Often, this equinus causes the planovalgus to correct and sometimes even overcorrect. Children with type 2 hemiplegia develop planovalgus that needs treatment less frequently and then those with bilateral involvement. Surgical treatment should not be considered until 8–10 years of age because this planovalgus may continue to slowly improve unless it is very severe. The predominant problem for children with type 2 hemiplegia is equinovarus, usually due to a spastic or overactive tibialis posterior. In occasional children, equinovarus is due to a spastic tibialis anterior. The diagnosis of the specific

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etiology of the varus between these two tendons requires a combination of physical examination and EMG data. The physical examination will often demonstrate increased tone in the muscle most responsible. The EMG should show a normal tibialis anterior that is active during preswing and initial swing phase and again in terminal swing at initial contact. Significant activity during midstance is abnormal. The tibialis posterior may be active throughout stance phase, more so in terminal stance, and should be silent in swing phase (Renders et al. 1997). Most commonly, the tibialis posterior is constantly active on EMG and spastic on physical examination, although there are cases where it is only active in swing phase. If the subtalar motion is supple, allowing full correction of the varus, a split transfer of the tibialis posterior to the peroneus brevis on the lateral side is performed. If the tibialis anterior is most affected, it is split transferred to the cuboid or to a slip of the peroneus longus. If both tendons are abnormal, both can have a split transfer performed at the same time. If the subtalar joint is not allowing overcorrection into some valgus, a calcaneal osteotomy may be required, although this is rare in type 2 hemiplegia.

Rotational Deformities Transverse plane torsional deformities are not common in type 2 hemiplegia and are usually mild, similar to torsional deformities in normal children. Because the torsional deformities are mild, surgical treatment should not be considered until late middle childhood or adolescence. Limb length discrepancy is usually approximately 1 cm shorter on the involved side, which is anatomically perfect. Shoe lifts should not be given, as they will only require children to make an adaptation, which increases the difficulty of swinging the leg through. This degree of shortness causes no short-term or long-term problems. Treatment of the spasticity, which is limited to the plantar flexors in type 2 hemiplegia, requires only local measures such as tendon lengthening, Botox, and/or bracing. There is no role for dorsal rhizotomy or intrathecal baclofen because the local treatments are effective and much simpler.

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Because both the gastrocnemius and soleus seem to contract together in many of these children, it is reasonable to consider nighttime orthotic wear to try to stretch the soleus and perhaps the gastrocnemius. A nighttime orthosis is usually attempted when contractures are present; however, most children object to this orthosis because they are unable to fall asleep, and therefore, in practice, this seldom works. The nighttime orthotic use has to include keeping the knee fully extended, so the gastrocnemius gets stretched as well as the soleus.

Hemiplegia Type 3 Children with type 3 involvement have all the concerns and problems of the children with type 2 involvement. Children with type 3 hemiplegia tend to start walking slightly later than with type 2, usually at 18–24 months of age. They almost all start with toe walking on both feet and usually will not need assistive devices to start walking. The diagnosis of type 3 hemiplegia requires establishing evidence that the knee is involved in the pathology. On physical examination, there may be increased tone in the hamstrings or rectus muscles and increased hamstring contracture, usually at least 20 and often 30–40 more than the unaffected side. Knee flexion at initial contact will be high, more than 25 . In midstance, the knee flexion continues to be increased. Almost all type 3 patterns have abnormal hamstring activity. On the EMG, this activity is usually premature onset in swing phase and prolonged activity in stance phase. The presence of a fixed knee flexion contracture of more than 5 is also evidence of hamstring involvement. The step length is usually shorter than the normal side due to the overactivity of the hamstrings, and the stance time is variable, sometimes longer and sometimes shorter depending on the stability of stance phase (Case 3). Treatment of the hamstring contractures and overactivity may use botulinum toxin injections for several cycles in young children, along with gastrocnemius injections. When the hamstring contracture is causing progressive knee flexion contracture, surgical lengthening should be performed. If the gastrocsoleus contractures need to be addressed, the hamstrings should also

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be lengthened at the same time, or knee flexion in midstance will draw these children to either toe walk again or stand with a crouched gait on the affected side, which also draws the unaffected side into a crouched gait pattern with increased knee flexion in stance. Those with more severe fixed knee flexion contractures, especially those with more than 20 lacking full knee extension, will need to have this addressed as well. The options for addressing your fix knee flexion contracture may include knee extension osteotomy, posterior knee capsulotomy, or anterior femoral epiphysiodesis. For a young child under age 10, the knee flexion contracture can often be stretched with aggressive therapy and orthotic use following hamstring lengthening. If there is limited therapy availability or the contracture is more stiff, a posterior knee capsulotomy is a good solution in this age group (Taylor et al. 2016). The use of guided growth with anterior epiphysiodesis has been reported (Macwilliams et al. 2011); however, in our experience, the deformity correction is too slow, and it adds further to the length discrepancy which is already a problem in some children. For the adolescent and young adult in whom the flexion contracture is stiff and more than 20 , a knee extension osteotomy is the preferred approach (Stout et al. 2008).

Stiff Knee Gait Some children with type 3 hemiplegia have involvement of the rectus. This involvement will be noted by the parents as a complaint of toe dragging, frequent tripping, and rapid shoe wear, especially on the anterior aspect of the shoe box. The physical examination may or may not demonstrate increased rectus tone and a positive Ely test. The kinematic evaluation will show swing phase peak knee flexion to be less than the normal, usually less than 50 , and the peak is often late, close to midswing. For children with late or low knee flexion in swing, when the EMG activity of the rectus muscle in swing phase is increased and evidence of complaints of toe dragging is present, then a distal transfer of the rectus is indicated. This transfer is almost always performed with hamstring lengthening and gastrocnemius or tendon

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Achilles lengthening. Similar to type 2 hemiplegia, approximately 25% of the children will need two plantar flexor tendon lengthenings, one at age 4–7 years and the other at adolescence. A few children will need three lengthenings (Joo et al. 2011). These tend to be children who needed the first lengthening very early due to more severe contracture, sometimes as early as the third year of life. The goal of delaying the first tendon lengthening is to try to avoid the second or third tendon lengthening, although there is no physical documentation that this strategy is effective.

Rotational Deformities Transverse plane deformities are more common with type 3 hemiplegic involvement and then type 2. If tibial torsion and femoral anteversion are causing increased tripping or are very cosmetically objectionable by 5–7 years of age, surgical correction can be considered. Although rare in type 3 hemiplegia, if children have a very asymmetric pelvic rotation as an adaptation for unilateral torsion usually tibial, correction should be considered as early as age 5–7 years. Because the functional impairment is greater, the limb length discrepancy tends to be slightly greater than for type 2 hemiplegia, often between 1 and 2 cm at maturity. For most children, this limb length discrepancy works perfectly well to help with foot clearance during swing phase in a limb that does not have a good ability to shorten during preswing and initial swing phase. A shoe lift should not be used, and radiographic monitoring of limb length is needed only with a discrepancy of over 1.5 cm. If the knee flexion contracture is more than 10 , additional shortening will occur. To prevent further leg shortening, knee flexion contracture prevention is important. Like type 2 hemiplegia, there is no role for the global treatment of spasticity in type 3 hemiplegia.

Hemiplegia Type 4 Type 4 hemiplegia is the third most common pattern, however, making up less than 5–10% of all children with hemiplegia. It is relatively

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common to find type 4 hemiplegia that overlaps with asymmetric diplegia or mild quadriplegia, and it is relatively uncommon to find a child with type 4 hemiplegia who is completely normal on the contralateral side. Most children with type 4 hemiplegia are either GMFCS II or GMFCS III level ambulators. The children who are GMFCS III usually are using a single lofstrand crunch in the uninvolved limb. Children with type 4 involvement walk later, between the ages of 2 and 3 years. Many children will use a walker during the learning period of walking. The walker may need to be fitted with an arm platform on the involved side. The diagnosis of type 4 hemiplegia is made by the presence of increased tone in the adductor or hip flexor muscles and by evidence on the kinematic examination of decreased hip extension in midstance. Increased internal rotation of the hip is very common. Both the stance time and the step length will be shortened as the involved limb. The limb can neither swing normally nor is very stable in stance phase. All the problems and considerations of type 2 and type 3 have to now be added into the treatment of type 4. In addition, concern for overactivity and contracture of the adductors and hip flexors has to be considered as well. Increased internal rotation of the hip is also common secondary to increased femoral anteversion. It is important to recognize that children with type 4 hemiplegia can develop spastic hip disease, so they have to be monitored by physical examination and radiographs for hip dysplasia. This is the highest-risk group of GMFCS I or GMFCS II functional ambulators who develop hip subluxation during the adolescent growth (Abousamra et al. 2016; Rutz et al. 2012) (▶ Chap. 129, “Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy”). From the perspective of children’s gait, the decisions about surgery are usually based mostly on the function at the level of the ankle and knee. Based on the evaluation of these joints, surgery of the hip has to be considered as an additional procedure. Adductor lengthening is only needed occasionally. If the abduction is greater than 20 on physical examination and abduction is present at foot contact, surgery is seldom indicated. Iliopsoas lengthening is indicated if hamstring lengthening is to be done, a hip flexion contracture of more than 20 is

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present, anterior pelvic tilt is more than 25 , and there is less than 10 of hip flexion at maximum extension in mid- or terminal stance. Usually, these hip lengthenings are needed only once; however, additional lengthenings, especially hamstring and gastrocnemius lengthenings, are very commonly needed. Probably 75–90% of children with type 4 hemiplegia need at least two lengthening procedures, and approximately 25% may need a third lengthening procedure. Treatment of the distal problems follows the pattern of type 2 and type 3; however, the muscle tone and contractures tend to be worse. Soft tissue balancing procedures may improve the gait of type 4 hemiplegia, but it will not address the hip dysplasia (Rutz et al. 2012).

Rotational Deformities Transverse plane deformities, especially increased femoral anteversion, are common in type 4 hemiplegia. Usually, this is added to the neurologic tendency for pelvic rotation with the affected side rotated posteriorly. In occasional children, this pelvic rotation may be so severe that they present with almost sideways walking. This sideways walking pattern can also be described as crab walking. This gait pattern is very ineffective and should be addressed at the young age of 5–7 years. Femoral derotation is required to allow the pelvis to rotate anteriorly on the affected side creating a more symmetric gait pattern. Femoral derotation should be considered if the pelvic rotation is more than 15–20 on the involved side and the physical examination shows an asymmetric femoral rotation with more internal rotation on the affected side. Femoral derotation can be combined with all the other soft tissue lengthenings that may be needed. Children with type 4 hemiplegia may develop foot deformities similar to diplegia in which the planovalgus improves into middle childhood but then gets worse again in adolescence. It is important for children with type 3 and 4 hemiplegia to have a full detailed analysis of their gait and then to plan to do a single-event multilevel surgical (SEMLS) correction. The outcome of SEMLS in hemiplegia is similar to the outcome in bilateral CP (Schranz et al. 2016).

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Limb Length Discrepancy Limb length discrepancy is an active concern in type 4 hemiplegia, because many children have 2–2.5 cm of shortness on the affected side. The functional impact of the limb shortness is increased with the tendency for knee and hip flexion deformities to add more functional shortening to the real shortening. Also, this leg length discrepancy may be further complicated by adductor contractures that may limit hip abduction allowing the pelvis to drop on the affected side, which further magnifies the limb length inequality. If the limb length cannot be functionally accommodated, the use of a shoe lift is recommended for type 4 hemiplegia. This group

Fig. 2 In hemiplegic types 1–3, it is better to have a mild shortness of the affected limb. Naturally, this ends up being between 1 and 2 cm, which helps limb clearance in swing. However, in type 4, there is a tendency to have increased hip adduction and flexion contractures that greatly magnify any other leg shortness. Also, hip extension and abduction are major mechanisms for accommodating leg length

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also merits close radiographic monitoring of limb length with the goal in some children of doing a distal femoral epiphysiodesis to arrest growth or to use epiphysiodesis plates for temporary growth arrest on the noninvolved side. The goal in type 4 hemiplegia is to have the affected limb length equal to 1 cm longer than the noninvolved side because of the functional impact of the inability to accommodate for joint positions during stance phase, which take precedence over swing phase dysfunction (Case 4). There is benefit to having a longer affected limb only in definite type 4 hemiplegia. In all other types, which make up more than 90% of hemiplegia, the affected limb should be approximately 1 cm shorter for maximum function (Fig. 2).

shortness, and when this is deficient in type 4 hemiplegia, the limb shortness becomes an impairment in its own right. Therefore, careful attention should be paid to limb length in type 4 with a goal usually of having symmetric limb lengths. An occasional patient may even function better with a longer limb on the affected side

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the presence of one normal arm, a double-rim onearm-drive chair should be considered, or depending on the environment and personal need, a power wheelchair may be more functional.

Complications

Fig. 3 A 13-year-old boy first presented with severe fixed equinus with maximum dorsiflexion being minus 60 . He had many episodes of casting and botulinum toxin which were clearly insufficient for the severity of the deformity. He now has developed secondary deformities, fixed knee flexion contracture, and rigid cavus. He is currently in his mid-30s, and these secondary deformities continue to cause him significant disability. This could all have been avoided with appropriate surgical management at the appropriate age before secondary deformities developed

In some children with type 4 hemiplegia, the use of intrathecal baclofen can be considered for treating severe spasticity even though it is unilateral. We have not used intrathecal baclofen in this population except for those with severe hemiplegic dystonia, and there are no reports specifically addressing its use. The local treatment of the degree of spasticity present in many children with type 4 hemiplegia is not very effective. In severe type 4 hemiplegia GMFCS III and GMFCS IV, an assistive device is needed long term for ambulation. These children with GMFCS IV require a platform walker and those with GMFCS III who usually walk with one crutch or cane. The most functional device is found by trial and error in physical therapy. In the children who are GMFCS IV with limited ambulation, wheelchairs are needed. Because of

Complications related to the management of children and young adults with hemiplegic CP tend to be most related to either poor timing of treatment or lack of treatment. Allowing severe equinus contractures to develop only incurs significant secondary osseous deformity which should be avoided (Fig. 3). Appropriate timing of correction of equinus contractures may incur the risk of needing repeat lengthening; however, this is less deforming in the long term than the secondary bone deformities which occur with neglected contractures. Attempts should also be made to avoid long-term orthotic use because this will create an orthotic dependence due to weakness of the muscle. Care should be taken to avoid overlengthening muscles and to creating external femoral torsion as these deformities become impairments in their own right. However, it is less functionally devastating in the person with hemiplegia to have overlengthening than it is in children with diplegia. Children with hemiplegia still have one good leg they can stand on and depend on for function.

Cases

Case 1 Tania

Tania, an 18-year-old girl, had hemiplegia as a result of a traumatic brain injury sustained at age 8 years. Her main complaint was that she could not lift her foot. Physical examination of her right ankle demonstrated an active toe extensor and some apparent activity of the tibialis anterior on withdrawal stimulus of a pin stick on (continued)

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Fig. C.1

the sole. Ankle dorsiflexion was 10 with knee flexion and 20 with knee extension. Ankle kinematics showed no active dorsiflexion in swing phase and no EMG activity of the tibialis anterior (Fig. C.1). Observation of her gait demonstrated an extended hallux in swing phase, but no apparent dorsiflexion was in swing phase. Knee and hip motion appeared to be normal. She was ordered a leaf spring AFO that worked well when it was worn.

Case 2 Christian

Christian, a boy with hemiplegia, started walking at 17 months of age. He used a solid ankle AFO until he was 2.5 years old. He then used articulating AFOs until he was 4 years old, when he complained

that the orthotics caused him pain. After multiple attempts to make the orthotics comfortable, he was allowed to walk without orthotics for 1 year until age 5 years, when he had a full analysis. The physical examination demonstrated that he had popliteal angles of 35 bilaterally, and ankle dorsiflexion on the right was only 25 with both knee flexion and extension. On the left, he had ankle dorsiflexion to 20 with knee flexion but only 5 with knee extension. The observation of his gait showed that he was toe walking bilaterally, although it is higher on the right than the left. It was recommended that he have an open Z-lengthening of the tendon Achilles. Postoperatively, he used an articulated AFO for 1 year, and following this, he developed good active dorsiflexion with a plantigrade foot position (Fig. C2.1).

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Fig. C2.1

Case 3 Kwame

Kwame, an 18-month-old boy, was initially seen with a complaint that he was late in

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learning to walk. He was reported to have been premature by 8 weeks but had been healthy since discharge from the hospital. On physical examination, he had increased tone through the lower and upper extremities, but it seemed worse on the left side. He was placed in an AFO, and over the next 6 months, he started walking. By age 5 years, he was developing significant internal rotation of the femur and having a stiff knee gait as well as significant toe walking bilaterally. At this time, the physical examination showed that he had hip abduction of 25 on the left and 45 on the right and internal rotation on the left of 75 and on the right of 60 . The popliteal angle on the left was 68 compared with 50 on the right. The left ankle dorsiflexion with the knee extended was 20 , while on the right it was 4 . The knee flexed ankle dorsiflexion on the left was 8 , while on the right it was 11 . The kinematics demonstrated low normal knee flexion in swing phase, increased knee flexion at foot contact, and bilateral early ankle dorsiflexion in stance phase, with less total dorsiflexion on the left side. Internal rotation of the left femur was also noted (Fig. C3.1). The EMG showed much less clear activity patterns on the left with the rectus having high variability and the hamstring having very early initiation on the left. The right side looked normal (Fig. C3.2). Except for the internal rotation of the hip, the primary pathology seemed to be in the left knee and ankle; therefore, this is a type 3 hemiplegia. Based on this, the femur was derotated, hamstring lengthened, distal rectus transferred to the sartorius, and (continued)

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Fig. C3.1

a tendon Achilles lengthening performed (Fig. C3.3). He did well for 4 years, but then he again developed a significant ankle equinus requiring a second tendon Achilles

and distal hamstring lengthening. As he entered puberty, he was doing well with a nearly symmetric gait pattern.

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Fig. C3.2

Case 4 Jeremy

When Jeremy was 9 years old, his parents complained that he tripped over his right leg and could not run. Jeremy had moderate mental retardation and no other history of medical problems. The left side was normal on physical examination, but on the right side, he had weakness, especially at the hip abductors and extensors. He had no

spasticity of the gastrocnemius but increased tone in the hamstrings with a popliteal angle of 50 on the right and 30 on the left. Ankle dorsiflexion on the right was 15 with knee flexion and 5 with knee extension. Hip abduction was limited to 10 on the right, full flexion was present, and a 2.5 cm shortness was noted on the (continued)

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Fig. C3.3

right side (Fig. C4.1). Jeremy was put in an AFO and given a 1.5 cm shoe lift, which improved the tripping symptoms. An adductor and hamstring lengthening was performed, and the leg length was

monitored with annual scanograms. Because this was believed to represent a type 4 hemiplegia without much compensation attempted by toe walking, a femoral (continued)

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Fig. C4.1

Fig. C4.2

epiphysiodesis was planned when his remaining growth would leave the right leg approximately equal to 1 cm long. At age 12.5 years, the epiphysiodesis was

performed (Fig. C4.2), and by age 16 years, he was left with several millimeters of increased lengthening on the right side (Fig. C4.3). He was weaned off of the shoe lift and out of the AFO. At the completion of growth, he walked without assistance. This is the typical limb length problem of type 4 hemiplegia, which should be managed to gain equal limb lengthening to slightly overlengthening on the involved side. With the other types of hemiplegia, the goal is to leave the child with a 1 to 2 cm shortness on the involved side, which will help with limb clearance and accommodate for the tendency for premature heel rise from gastrocnemius spasticity or contracture.

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Fig. C4.3

Cross-References ▶ Foot Deformities Impact on Cerebral Palsy Gait ▶ Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy ▶ The Upper Extremity in Cerebral Palsy: An Overview

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deformity in children with cerebral palsy: assessment of predisposing factors for recurrence in a long-term follow-up study. J Child Orthop 5:289–296 Lee JH, Sung IY, Yoo JY (2008) Therapeutic effects of strengthening exercise on gait function of cerebral palsy. Disabil Rehabil 30:1439–1444 Macwilliams BA, Harjinder B, Stevens PM (2011) Guided growth for correction of knee flexion deformity: a series of four cases. Strategies Trauma Limb Reconstr 6:83–90 Renders A, Detrembleur C, Rossillon R, Lejeune T, Rombouts JJ (1997) Contribution of electromyographic analysis of the walking habits of children with spastic foot in cerebral palsy: a preliminary study. Rev Chir Orthop Reparatrice Appar Mot 83:259–264. SRC – GoogleScholar Riad J, Coleman S, Lundh D, Brostrom E (2011) Arm posture score and arm movement during walking: a comprehensive assessment in spastic hemiplegic cerebral palsy. Gait Posture 33:48–53 Riad J, Coleman S, Miller F (2007a) Arm posturing during walking in children with spastic hemiplegic cerebral palsy. J Pediatr Orthop 27:137–141 Riad J, Haglund-Akerlind Y, Miller F (2007b) Classification of spastic hemiplegic cerebral palsy in children. J Pediatr Orthop 27:758–764 Riad J, Haglund-Akerlind Y, Miller F (2008) Power generation in children with spastic hemiplegic cerebral palsy. Gait Posture 27:641–647 Rutz E, Baker R, Tirosh O, Romkes J, Haase C, Brunner R (2011) Tibialis anterior tendon shortening in combination with Achilles tendon lengthening in spastic equinus in cerebral palsy. Gait Posture 33:152–157 Rutz E, Passmore E, Baker R, Graham HK (2012) Multilevel surgery improves gait in spastic hemiplegia but

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does not resolve hip dysplasia. Clin Orthop Relat Res 470:1294–1302 Schranz C, Kruse A, Kraus T, Steinwender G, Svehlik M (2016) Does unilateral single-event multilevel surgery improve gait in children with spastic hemiplegia? A retrospective analysis of a long-term follow-up. Gait Posture 52:135–139 Stout JL, Gage JR, Schwartz MH, Novacheck TF (2008) Distal femoral extension osteotomy and patellar tendon advancement to treat persistent crouch gait in cerebral palsy. J Bone Joint Surg Am 90:2470–2484 Taylor D, Connor J, Church C, Lennon N, Henley J, Niiler T, Miller F (2016) The effectiveness of posterior knee capsulotomies and knee extension osteotomies in

1455 crouched gait in children with cerebral palsy. J Pediatr Orthop B 25:543–550 Tsang ST, McMorran D, Robinson L, Herman J, Robb JE, Gaston MS (2016) A cohort study of tibialis anterior tendon shortening in combination with calf muscle lengthening in spastic equinus in cerebral palsy. Gait Posture 50:23–27 Turedi Yildirim A, Sutcu R, Koroglu M, Delibas N, Kisioglu N, Akar N, Ergurhan Ilha I (2015) The role of prothrombotic factors in children with hemiplegic cerebral palsy. Minerva Pediatr 67:279–284 Winters TF Jr, Gage JR, Hicks R (1987) Gait patterns in spastic hemiplegia in children and young adults. J Bone Joint Surg Am 69:437–441

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1458 Natural History and Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1459 Diplegia in Young Children (The Prancing Toe Walker) (True Equinus) . . . . . . . . . . . . 1459 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1465 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1465 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1468 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1468

Abstract

Diplegic pattern (bilateral) involvement in children with cerebral palsy (CP) has a wide spectrum, blending with the quadriplegic pattern at the more neurologically severe end of bilateral involvement and blending with the hemiplegic pattern on the more severely asymmetric end of the spectrum. It is not as easy to make severity grouping such as is defined for hemiplegia in large part because of variability and blending in children with diplegia. There are definitely children with mild diplegia and children with severe diplegia, but these groups seem to be opposite ends of a standard distribution curve with a mean being moderate involvement. Severity of involvement tends to increase from distal to

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_102

proximal similar to hemiplegia; however, there are few children with diplegia with only ankle involvement. Most children with diplegia have some hip, knee, and ankle involvement. The method for planning treatment that is easy to remember and relates directly to the treatment plan is based on the age of children rather than on the individual severity. Young children under age 5 are primarily managed with physical therapy and orthotics occasionally using botulinum toxin injections for severe spasticity. It is during this stage that consideration of general spasticity reduction with the use of dorsal rhizotomy or intrathecal baclofen can also be entertained. Treatment in middle childhood between the ages of 5 and 10 should focus on correcting impairments such as toe walking, flexed knee gait, and severe torsional deformities of the lower extremities. At this age corrections usually are done at a single event multilevel surgery (SEMLS) with muscle lengthening 1457

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and occasionally correction of torsional malalignments. Adolescence to growth maturity between ages 10 and 16 years is the time when final deformity corrections are preformed, often with foot stabilization, further muscle lengthening, and bone alignment correction.

F. Miller

Diplegic pattern (bilateral CP) involvement in children with cerebral palsy (CP) has a wide spectrum, blending with the quadriplegic pattern at the more neurologically severe end of bilateral involvement and blending with the hemiplegic

pattern on the more severely asymmetric end of the spectrum. Attempts to classify diplegic gait patterns usually end with parameters directly related to age, such as limb length, or indirectly related to age, such as jump position versus crouch (Lin et al. 2000) (Fig. 1). Another attempt was published using sagittal plane only using pattern recognition and kinematic variables. The system proposed that Rodda et al. (2004) has five categories with group 1 being true equinus with ankle in equinus and knee extended, group 2 jump gait with ankle equinus and knee flexed, group 3 apparent equinus which has a neutral ankle but a flexed knee, group 4 crouch gait which is defined as ankle dorsiflexion and knee flexion, and group 5 asymmetric with a combination of patterns (Fig. 2). The concept of this classification is excellent for planning treatment; however, the drawback of the system is that there is a lot of blending

Fig. 1 The patterns of diplegia are more difficult to define than those of hemiplegia. It is better to divide the stages or ages rather than pattern of involvement. Children younger than 5 years old tend to toe walk with equinus knee stiffness and often internal rotation of the hips. By middle childhood, they often develop a crouched gait pattern which, if left untreated, gets rapidly worse during the

adolescent growth period. This problem may drive a child into a wheelchair if it is untreated and severe. Some children in middle childhood start to back-knee, and this may become worse in adolescence to the point where it causes severe knee pain if it is not addressed again, causing the child to end up in a wheelchair. Many children though will reach a stable plateau at milder level

Keywords

Cerebral palsy · Crouch gait · Jump gait · Equinus · Equinovarus

Introduction

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Fig. 2 There is also an attempt to classify diploid gait pattern based on stance phase ankle, knee, and hip position as defined by Rodda (Rodda et al. 2004). These positions

can be helpful to define the current posture and may be helpful in considering treatment options, however they are not a gradation of severity

between groups. These patterns sometimes define the natural history as children mature. Over the course of the child’s growth and development from beginning walking some children with follow this progression, however there is also high variability in this natural development. With short-term follow-up (2.5 years), Rodda reported no change in pattern when using gait analysis (Rodda et al. 2004). There is no easy and relatively separable severity grouping such as is defined for hemiplegia in large part because of variability and blending over in children with diplegia. There are definitely children with mild diplegia and children with severe diplegia, but these groups seem to be opposite ends of a standard distribution curve with a mean being moderate involvement. Severity of involvement tends to increase from distal to proximal similar to hemiplegia; however, there are few children with diplegia with only ankle involvement. Most children with diplegia have some hip, knee, and ankle involvement. The method for planning treatment that is easy to remember and relates directly to the treatment plan is based on the age of children rather than on the individual severity. Therefore, young children, middle childhood-aged children,

and adolescents to young adults will be the age groups, and within each age group, mild, moderate, and severe involvement is considered, where children with gross motor function classification system (GMFCS) I or high-functioning II are considered mild, children with GMFCS level II and high-functioning level III are moderate, and children with marginal functional rated ambulation GMFCS III and high-functioning GMFCS IV level who do functional standing for transfers belong to severe involvement.

Natural History and Treatment Diplegia in Young Children (The Prancing Toe Walker) (True Equinus) Mild Involvement Children with mild diplegia may start walking between 18 and 24 months of age. Usually, these children initiate independent ambulation by toe walking with extended hips and knees. Typically, the spasticity in the gastrocnemius and hamstrings is mild, and there may even be a question of these children being idiopathic toe walkers or mild

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diplegic pattern GMFCS I level CP. The toe walking is easy to control with an AFO, and as children gain motor control and balance, some will start to walk foot flat without an AFO. However, other children will become more spastic, occasionally with severe spasticity requiring Botox injections just to tolerate brace wear. If these children are still toe walking without an AFO by 5–7 years of age, surgical tendon lengthening should be considered. If the ankle dorsiflexion with knee extension is less than 5 , and the maximum dorsiflexion in stance phase is occurring during weight acceptance instead of terminal stance, gastrocnemius lengthening is indicated. If there is a high early plantar flexion moment with a big power absorption and poor push-off power generation, gastrocnemius lengthening is also indicated. If the initial contact knee flexion is increased above 20 and the popliteal angle is increased, then hamstring lengthening should also be considered. It is expected that children with mild diplegia will need only one surgical procedure to maximize gait. Most children with mild diplegia do not have transverse plane deformities; however, if they do, the correction can be made at the same time, between the ages of 5 and 10 years (Case 1).

Moderate Degree of Involvement Most children with diplegia would be defined as moderate. If balance is adequate, most moderate children (GMFCS II) walk independently between the ages of 24 and 36 months. If balance is a problem, walker use will continue to be required, starting with crutch training around 4–5 years of age. For children GMFCS III functional community ambulation with crutches should not be expected until age 5 years and sometimes will not occur until children are 8–10 years old. In the first year of independent ambulation, these children will walk with the arms in the high guard position, walk fast up on the toes, and when they want to stop, they will run to find fixed objects like a wall or fall to the floor. Most children walk with knee stiffness, extended hips and knees, and with increased rotation of the pelvis. Some children have transverse plane deformities with increased femoral anteversion being most common, but they also may have tibial torsion. Many children at this age with

F. Miller

moderate diplegia walk with ankle equinus and varus. Surgical treatment is planned for between 5 and 10 years of age after children have had 6–12 months of no improvement in ambulatory speed, walking endurance, or improvement in balance. The primary treatment at this age is aggressive physical therapy using the teaching modalities and repetitive practice to improve balance and motor control. Passive stretching may be taught to caretakers as well as performed by therapists. Localized treatment with Botox may be beneficial if there are specific focal problems such as gastrocnemius, spasticity, or hamstring spasticity that are causing impediments to progress in gait learning (Case 2).

Severe Involvement The most severe end of the diplegic pattern GMFCS III and IV is children who have very significant asymmetry and who start walking with a walker at 2–3 years of age and, if they come to independent ambulation, do so only after 4 or 5 years of age, usually following surgery. These children are high, early toe walkers in their bare feet. They may be able to get feet flat, often with significant planovalgus. Many of the toe walkers have varus foot position associated with equinus. Transverse plane deformities are common, with both tibial torsion and femoral anteversion. Spasticity tends to include the hip, knee, and ankle almost equally. These children have to be closely monitored for spastic hip disease, which will occur in a significant number and requires early adductor lengthening. Often, these children are best treated with solid AFOs until they are 4 or 5 years of age. Physical therapy is the mainstay of treatment, with the focus being the same as with children with moderate involvement. These children seldom have significant benefit from Botox because of the diffuse widespread involvement of the increased spasticity. Surgical Treatment of the Prancing Toe Walker (True Equinus) Surgical treatment planning is usually focused at the interface between early childhood and middle childhood. By 5 or 7 years of age, children are reaching a plateau in neurologic development, and the rate of learning motor and balance skills

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is plateauing as well. Socially, children are preparing to enter kindergarten or first grade if they have adequate cognitive skills. For cognitively high-functioning children, the goal should be to have the gait impairment surgically corrected and rehabilitation completed before entering first or second grade. Entering first grade is a significant transition point for many children as they change from primary gross motor skills orientation to primary fine motor skills and cognitive skills learning. This transition period should include decreasing physical therapy and transitioning to normal age-appropriate athletic activities that individual children’s functional levels and community ambulatory abilities allow. For example, having a child play soccer 2 days a week with a team would be better than spending that time in physical therapy doing medically oriented therapy, especially for a child who is GMFCS I or II level ambulator. As children reach a gait functional plateau, usually between 5 and 7 years of age, a full analysis and evaluation of their gait function is performed. A surgical plan based on the single event multilevel surgery (SEMLS) is made and the actual surgery planned to least disturb families’ normal activities. The SEMLS approach means defining all aspects of the gait pathology and correcting those that are amendable to surgical correction at one surgical setting. First, a decision has to be made if a tone reduction procedure is indicated or if the treatment is to be all musculoskeletal based. If children are independent ambulators and the physical examination demonstrates increased tone throughout the lower extremities and minimal fixed muscle contractures, the kinematics demonstrate decreased range of motion at the hip, knee, and ankle, and there are no transverse plane deformities, have no movement disorders and good cognitive function, are considered excellent candidates for a tone reduction procedure. Children who meet all these criteria are very rarely seen, so there are almost always relative contraindications. Current reported data from rhizotomy in this age group suggests that ambulatory ability is not improved much over physical therapy alone (McLaughlin et al. 1998). Dorsal rhizotomy decreases spasticity, and the joint range of motion increases, especially at the hip and

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knee (Boscarino et al. 1993; Peacock et al. 1991; Vaughan et al. 1991). Muscle contractures do not resolve, and there is no impact on transverse plane deformities (Thomas et al. 1997). There are very few data comparing direct musculoskeletal surgery with dorsal rhizotomy, with the only report suggesting a better chance of independent ambulation following muscle surgery than dorsal rhizotomy (Marty et al. 1995). Based on these reports and our own experience, we no longer recommend dorsal rhizotomy to any child; however, it is still used in some centers. The use of intrathecal baclofen for this population has been reported in small cases series (Shilt et al. 2008). As noted in this previous report, there are reduced Ashworth scores indicating reduced spasticity. Kinematic changes in our series had clinically insignificant improvement, but families generally felt children were doing better. We had three boys who as they became adolescents wanted to have the spasticity return because it made them feel stronger. The large size of the pump and the need for frequent refills have made families hesitant to have these pumps implanted. The baclofen pump is ideal for controlling spasticity and allowing children to be as functional as possible. Sometimes this means having more spasticity as we have learned when the patient has the choice. This is the major problem with dorsal rhizotomy, once it is done there is no recovery the spasticity if that is desired. Part of the problem with dorsal rhizotomy is that too much tone may be removed and children are left weak. With the pump, this can be modulated and allows the patient and family to come to agreement of what works best. Clearly, the mainstay of surgical treatment of children with diplegia is direct correction of the deformities that are causing the functional impairment to gait. The goal should be to correct all the impairments that can be corrected with one surgery using the SEMLS approach. The analysis starts distally and works proximally. If there is a varus foot deformity with equinus that seems to be causing toe walking, there is a temptation to suggest that this should be corrected. In early and middle childhood diplegia, unless the varus foot deformity is fixed, no surgery should be done on the tibialis anterior or tibialis posterior. Almost all these children will convert to planovalgus later,

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and any surgery on the foot at this age will only speed up that process. If children have a planovalgus deformity that is supple and are tolerating an orthotic, continuation of the orthotic is in order. If the deformity is severe, causing problems with orthotic wear, correction of the planovalgus is indicated, usually with a lateral column lengthening at this age. For severe fixed deformities, subtalar fusion is indicated. Ankle dorsiflexion on physical examination will almost always demonstrate a discrepancy between gastrocnemius and soleus muscle contractures. Usually, the ankle is in plantar flexion at initial contact and comes to early dorsiflexion, but still lacks normal dorsiflexion. The ankle moment shows increased plantar flexion moment early in stance with a high power absorption in middle stance and low push-off power generation. These parameters indicate the need for gastrocnemius lengthening. The whole Achilles tendon should never be cut with a percutaneous tenotomy in children with diplegia. Although one study reportedly found no difference between open Z-lengthening of the Achilles tendon and doing a gastrocnemius-only lengthening (Yngve et al. 1996), there is no known or theoretical benefit to lengthening the soleus tendon if the muscle is not contracted. Any excessive weakening or lengthening of the plantar flexor complex in diplegia is a very high risk of developing crouch gait, and if the child already has a stance-phase knee flexion gait pattern, rapid progression occurs. Almost all children with diplegia have increased popliteal angles on physical examination and increased knee flexion at initial contact and during weight acceptance. Most will continue with increased knee flexion in midstance as well, which indicates hamstring lengthening is needed. If the knee goes into extension in midstance phase but has a very high popliteal angle of greater than 60 and knee flexion at initial contact of more than 40 , hamstring lengthening is still indicated but usually only medial semitendinosus and semimembranosus lengthenings. However, if children have external tibial torsion, a biceps lengthening should also be added. If children’s popliteal angles and knee flexion are intermediate between the two and they have full knee extension in

F. Miller

midstance phase, only the semitendinosus should be lengthened. Hamstring lengthening in children with full knee extension should be done with caution because over-lengthening will likely lead to back-kneeing. As the knee flexes into swing phase, rectus dysfunction may be diagnosed by prolonged swing phase rectus activity on EMG, low peak knee flexion, and late peak knee flexion in swing phase. If the walking velocity is greater than 80 cm/s and families complain of toe drag, a rectus transfer or resection is indicated. At the hip, a flexion contracture of more than 20 with anterior pelvic tilt of greater than 20 and decreased hip extension in early stance phase are indications for iliopsoas lengthening. If the indication is borderline, the procedure should be done if children are independent ambulators, but not if they are using walking aids or walk slower than 80 cm/s. Transverse plane deformities need to be assessed and should be addressed if the foot progression angle is more than 10 internal or 30 external. At this age, children almost never have an external progression foot angle; however, internal foot progression angle, which may be due to the internal tibial torsion or femoral anteversion or a combination of both, is common. On physical examination, significantly greater internal hip rotation compared with external rotation suggests increased femoral anteversion, and if this is combined with 20 or more of internal rotation of the hip on the kinematic evaluation, and especially if this occurs in early stance phase, it should be corrected with a proximal femoral derotation osteotomy. If the transmalleolar axis-to-thigh angle is internal, or the internal torsion measures more than 20 internal on the kinematic evaluation, a tibial derotation osteotomy is indicated. In some children, both will be present, and both should be corrected. Do not overcorrect at one level to compensate for the other level. This compensatory overcorrection will lead to the knee joint axis being out of line with the forward line of progression and will likely deteriorate or increase as children grow, requiring later correction. Do not overcorrect tibial torsion from internal to external because the natural history is for the tibia to go into external torsion with growth in children with diplegia.

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After a full gait assessment, children can have the specific surgical plan made. Each limb should be assessed separately, as many children with diplegia have some asymmetry and require different surgical procedures on each limb. In general, most children with diplegia need gastrocnemius lengthening with some hamstring lengthening. Very rarely is only a gastrocnemius lengthening indicated. The surgical procedure should be done so that children can be rapidly mobilized and returned to physical therapy for rehabilitation. Postoperatively, most children will continue to need some level of foot support, often with an AFO, to assist with dorsiflexion until the tibialis anterior develops muscle tone and correct length.

Middle Childhood, Early Crouch, and Recurvatum of the Knee After the surgical correction and postoperative rehabilitation, which should be expected to last 1 year as an outpatient with gradually decreasing physical therapy, children with diplegia should be in a stable motor pattern for middle childhood and into adolescence. Often, these GMFCS II children will be more stable; however, they will also walk slower because they are now standing foot flat and do not have the falling gait that was present with the high prancing toe walking posture. Parents may see this slower gait as regression, but they have to be informed to expect this change, which will now allow the children to focus on developing a more stable gait. Children with diplegia in middle childhood tend to be drawn to several postural attractors.

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This is the age when prominent back-kneeing or crouched gait pattern will start to be seen consistently. This is the time when there may be sudden shifts in ankle position as the posture is being drawn to back-kneeing (recurvatum) or crouch positions (Fig. 3). With the correct soft-tissue balance, almost all children who are GMFCS I or II ambulators will tend to fall into a mild crouched position, which is the goal of treatment. This position is most functional when the crouch is mild, meaning midstance-phase knee flexion is less than 20–25 , and the children have an ankle dorsiflexion maximum of less than 20 . In middle childhood, this tends to be stable with children gaining confidence in walking ability with less falling and having longer community endurance. If the ankle dorsiflexion is increasing above 20 , a dorsiflexion resisting AFO or ground reaction AFO should be applied. If the midstance knee flexion goes above 30 and children develop increasing knee flexion contracture and progressive hamstring contracture, repeat muscle lengthening has to be considered. These contractures seldom become a problem until approximately 5–7 years after the initial surgery, when the children are in early adolescence. During middle childhood, there is little need for routine physical therapy for children who are GMFCS I or II ambulators. These children should be encouraged to get involved in sports activities, such as martial arts or swimming. For children who are GMFCS III or IV, walking aids, therapy directed at learning to use forearm crutches (Lofstrand) before the age of 10 years, and weaning Left

Right Dorsiflexion

Ankle Motion

Plantarflexion

30.0

30.0

20.0

20.0

10.0

10.0

0.0

0.0

–10.0

–10.0

–20.0

–20.0 Age 6

Fig. 3 As growth occurs and muscle length changes along with changes in the muscle strength to body mass ratio changes, children often make sudden significant shifts in posture; this shows the concept of a shift from one strong

Age 8

attractor to another strong attractor. In this example, a child changed from a flat foot premature heel rise gait pattern to toe walking with ankle equinus. These relatively quick shifts are difficult to predict

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off the walker are recommended. Learning to use crutches may require a period of training by physical therapy during the summer, or during a time when it does not interfere with school work. Passive range of motion should not be routinely done by physical therapists, and children should be encouraged to do it themselves under the direction of the parents or caretakers. Activities that build cardiovascular endurance such as soccer and stretching and balance activities such as the martial arts are strongly encouraged for children to explore.

Adolescent Severe Crouch During adolescent growth, the flexible childhood crouch may progress rapidly in severity of the stance-phase knee flexion with some individuals having 90 of knee flexion in stance. If left untreated this increasing crouch precludes ambulation, and the individual will require the use of a wheelchair. During the time the stance-phase knee flexion is increasing, the fixed knee flexion contracture is also increasing. The primary intervention for this severe progressive crouch is correction of the knee with an extension shortening osteotomy of the femur, plication of the patellar tendon, and correction of all other deformities creating lever arm dysfunction as planovalgus feet, tibial torsion, or femoral torsion. When the fixed knee flexion contracture is very severe, more than 40 or 45 , a period of cast stretching may be indicated before the extension osteotomy is performed. It is also advised to address the problem of the severe hamstring contracture by shortening the femur, which will also protect the sciatic nerve from a stretch injury. Correction of severe crouch should be performed before the individual is using a wheelchair full time for all walking. A strong motivation by the patient to continue walking is also important. My success in getting a patient back to fulltime ambulation, if they have been using a wheelchair for most their mobility for 1 year or more, has been poor. It is very important to intervene before the crouch becomes so severe that a wheelchair is required if the goal is to maintain long-term ambulation. It is also important to have aggressive rehabilitation available immediately after surgery, which can continue for 6–12 months (▶ Chap. 103, “Crouch Gait in Cerebral Palsy”).

F. Miller

Knee Recurvatum (Back-Kneeing) Some GMFCS I or II children who fall into the back-knee attractor have a gastrocnemius that is a little too tight for the hamstrings and can be easily controlled with an AFO that limits plantar flexion. These children need full calf-length articulated AFOs that block plantar flexion at 5 of dorsiflexion. Often, with full-time brace wear, the hamstrings will gain strength over time, and the back-kneeing will slowly resolve as children grow. The second pattern of back-kneeing is children who go into the jump position with ankle equinus and knee flexion, where the body is anterior to the hip and the knee joint axis. This pattern may be due to a missed iliopsoas contracture that was not lengthened or may result from a weak gastrocsoleus. The use of a solid AFO in 5 of dorsiflexion should provide a trial. Also, if there is increased lordosis and more than 30 of hip flexion contracture, the hip flexor should be suspected as the primary cause. If the problem is a contracted hip flexor that was missed in the original operation, this may need to be lengthened to get children to stand upright. The third posture creating back-kneeing is taken by GMFCS I or II ambulators who back-knee in stance with hyperlordosis. In this posture, the HAT center of mass is behind the hip joint but in front of the knee joint. A trial with a solid AFO is the best treatment of this pattern. Most children who are GMFCS I or II ambulators in middle childhood respond well to AFO treatment for back-kneeing. Back-kneeing in children who use walking aids GMFCS III or IV, such as walkers or forearm crutches, is a major problem (Case 3). This backkneeing may be due to a motor control problem in which individuals lean forward on the crutches, usually with hyperlordosis. With the center of mass of the HAT segment far forward of the hip and knee joint, there is a large knee external extension moment on the knee. If individuals have any shortness of the gastrocsoleus, the knee will hyperextend and go into back-kneeing. Also, if individuals have weakness in the gastrocsoleus, they will back-knee. The primary treatment is to use AFOs that prevent plantar flexion; however, with the use of walking aids, AFOs often do not work well as individuals will simply allow the forefoot to rise

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from the floor. If the knee flexion moment is very high in midstance and individuals complain of knee pain or passive range of knee hyperextension demonstrates an increase of more than 10–15 , the only option is the use of a KAFO with an extension stop knee hinge. It is important to make sure that there is no contracture of the gastrocsoleus. Ankle dorsiflexion has to be 5–10 in knee extension, or the gastrocnemius should be lengthened. Many individuals will continue to have back-kneeing but will remain stable and pain-free over many years. Often, the back-kneeing will include a valgus extension thrust in midstance; however, the knee flexion moment is not large, probably because weight bearing on the upper extremity through the walking aid helps to reduce the magnitude of the ground reaction force. Another way of understanding this is that as individuals move the center of mass of the HAT segment further forward, more weight is shifted to the arms. Although the extension moment at the knee is getting longer, there is a decreased amount of weight from the HAT segment carried by the feet, which decreases the magnitude of the knee extension moment. Tone management in middle childhood is a more difficult issue. If spasticity or a mixed movement disorder is felt to be a significant impairment to the gait pathology, this is the ideal age to consider a trial with an intrathecal baclofen and see if the underlying strength is present to allow continued walking. The role for dorsal rhizotomy in the middle childhood period is very small, although there are some centers persisting with doing rhizotomy at this age.

Complications Complications of treatment of young children with diplegia generally fall into two groups, one is allowing deformities to become severe and persistent. An example is the child who develops severe equinus, so he is unable to bring his ankle to more than 20 of dorsiflexion. Another common deformity is the development of fixed knee flexion contracture at a young age. Initially these flexion contractures tend to be soft and easily managed with splinting; if there is severe hamstring contracture, they need surgical lengthening.

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The second group is overtreatment in which contractures are lengthened too much or too early. A common example is a mild equinus contracture that is managed with Achilles tendon lengthening when only the gastrocnemius even has a small contracture. When Achilles tendon is lengthened in a child who also has hamstring contracture but the hamstrings are not addressed, the force is toward driving the child into a crouch gait pattern with hyper-dorsiflexion and increased knee flexion. The second frequently missed and overtreated combination is the child who has equinovarus foot positioning with some internal tibial torsion in early childhood with severe intoeing. There may some spasticity of the tibialis posterior. If the treatment for this young child includes lengthening or transfer of the tibialis posterior with Achilles tendon lengthening and especially if external tibial rotation osteotomy is added, the child will with 100% certainty develop external foot progression angle with a severe planovalgus foot (Liggio and Kruse 2001). This usually takes between 2 and 3 years to develop a severe deformity which will again require surgical correction.

Cases

Case 1 Cherisse

Cherisse, an 18-month-old girl with increased stiffness in the legs, was seen for slow walking development. Although there was no history of birth problems, she had a workup with a brain MRI that was normal, and a diagnosis of diplegic CP was made. She was placed in an AFO, and her mother was encouraged to have her move using heavy push toys. By age 2 years, she was walking independently, and by age 3 years, she was walking on her toes, going faster but falling a lot. She was wearing an articulated AFO and was in physical therapy where she had good continued improvement up to age 4 years. Therefore, she was continued for another year in the same (continued)

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program. By age 5 years, both her mother and therapist who were working with her felt that there had been little additional progress in the past 6 months. At this time, her physical examination demonstrated a popliteal angle of 50 , knee extended ankle dorsiflexion of 5 , and bilateral and kneeflexed ankle dorsiflexion of 15 . Internal rotation of the hips was 70 with external rotation of 20 . Other ranges of motion were normal. Kinematics demonstrated increased knee flexion at foot contact, premature ankle dorsiflexion, and internal rotation of the hips (Fig. C1.1). The gastrocnemius had 2+ spasticity, and the hamstrings and hip adductors had 1+ spasticity. Her mother was given the option to have either a dorsal rhizotomy or orthopedic surgery, and she chose to do the orthopedic procedures. Cherisse had bilateral hamstring lengthening, gastrocnemius lengthening, and femoral derotation osteotomy. One year after surgery, her gait had improved with better knee motion and correction of the internal rotation. This improvement was maintained 4 years later. It is expected that this girl will likely not need more surgery and that she will be an excellent ambulator as an adult.

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walker, and in therapy, he was working on balance development with the use of quad canes, which were nonfunctional for ambulation outside the therapy environment. By age 6 years, he was practicing with Lofstrand crutches, and by age 8 years, he was starting to practice walking independently. He was finding more stability and walking more with back-kneeing and ankle dorsiflexion even though he did not have equinus contractures (Fig. C2.1). It was clear at this time, however, that he would be a permanent crutch user as age 8 years is a common plateau point, and he had been receiving intensive therapy, which means significant additional improvement cannot be expected. He had no significant structural limitations that could be corrected, and most of his ambulation problems were related to poor balance with the arms in the high guard position. Over the next 4 years, he continued to work on his balance, but as he entered puberty, it was clear that he would never be able to walk independent of the crutches except for very short times in home areas.

Case 3 Frederico

Case 2 Daymond

Daymond, a 2-year-old boy, presented with a history of prematurity and slow motor development. At that time, he was just starting to hold on to and push some toys. He was placed in solid ankle AFOs, and after 1 year of physical therapy, he was able to walk slowly in the posterior walker but could not get into the walker by himself. By age 4 years, through continued therapy, he learned to get up into a standing position and increased his walking speed. He was also switched to articulating AFOs. By age 5 years, he was walking well with the

Frederico, a 7-year-old boy, presented with his mother who complained that he had severe back-kneeing when he walked with his walker or used his crutches. He had AFOs, which he complained did not help him and he did not want to wear them. On physical examination he had normal hip motion and knee popliteal angles of 40 bilaterally. Knee-extended ankle dorsiflexion was 10 , and knee-flexed ankle dorsiflexion was +15 . Frederico had poor balance and could not stand without holding on. Kinematic evaluation showed increased knee flexion at foot contact, knee hyperextension with almost every step in (continued)

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1467 Right

Fig. C1.1 Flexion

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Fig. C2.1

midstance, early and decreased ankle dorsiflexion with significant early plantar flexion, and very little additional plantar flexion at toe-off. He had gastrocnemius lengthenings, which were the main cause of the back-kneeing; this was also the reason he could not tolerate the AFOs. He was then gradually weaned out of the AFOs and gained knee control, although he still had a tendency to be either in crouch or convert into knee hyperextension in midstance.

Cross-References ▶ Cerebral Palsy Gait Pathology ▶ Crouch Gait in Cerebral Palsy ▶ Knee Deformities Impact on Cerebral Palsy Gait ▶ Overview of Knee Problems in Cerebral Palsy

References Boscarino LF, Ounpuu S, Davis RB, Gage JR, DeLuca PA (1993) III, Effects of selective dorsal rhizotomy on gait in children with cerebral palsy. J Pediatr Orthop 13:174–179 Liggio FJ, Kruse R (2001) Split tibialis posterior tendon transfer with concomitant distal tibial derotational osteotomy in children with cerebral palsy. J Pediatr Orthop 21:95–101 Lin CJ, Guo LY, Su FC, Chou YL, Cherng RJ (2000) Common abnormal kinetic patterns of the knee in gait in spastic diplegia of cerebral palsy. Gait Posture 11:224–232. SRC – GoogleScholar Marty GR, Dias LS, Gaebler-Spira D, J. (1995) Selective posterior rhizotomy and soft-tissue procedures for the treatment of cerebral diplegia. Joint Surg Am 77:713–718. SRC – GoogleScholar McLaughlin JF, Bjornson KF, Astley SJ (1998) Selective dorsal rhizotomy: efficacy and safety in an investigator-masked randomized clinical trial [see comments]. Dev Med Child Neurol 40:220–232. SRC – GoogleScholar Peacock WJ, Staudt LA (1991) Functional outcomes following selective posterior rhizotomy in children with

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cerebral palsy. J Neurosurg 74:380–385. SRC – GoogleScholar Rodda JM, Graham HK, Carson L, Galea MP, Wolfe R (2004) Sagittal gait patterns in spastic diplegia. J Bone Joint Surg (Br) 86:251–258 Shilt JS, Reeves S, Lai LP, Wetter J, Cabrera MN, Kolaski K, Smith BP (2008) The outcome of intrathecal baclofen treatment on spastic diplegia: preliminary results with a minimum of two year follow-up. J Pediatr Rehabil Med 1:255–261

1469 Thomas SS, Aiona MD, Buckon CE, Piatt JH (1997) Does gait continue to improve 2 years after selective dorsal rhizotomy? J Pediatr Orthop 17:387–391 Vaughan CL, Berman B, Peacock WJ (1991) Cerebral palsy and rhizotomy, a 3-year follow-up evaluation with gait analysis. J Neurosurg 74:178–184. SRC – GoogleScholar Yngve DA, Chambers C (1996) Vulpius and Z-lengthening. J Pediatr Orthop 16:759–764. SRC – GoogleScholar

Hip and Pelvic Kinematic Pathology in Cerebral Palsy Gait

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1472 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hip Joint . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pelvis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . HAT Segment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Treatment and Outcome Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1481 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1482 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1486 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1486

Abstract

The gait of children with cerebral palsy (CP) is heavily influenced by motor control, balance, musculoskeletal alignment, and forcegenerating ability. The force input and motor aspect of gait resides primarily in the musculoskeletal system of the pelvis and the two lower extremities. The body above the pelvis including the head, arms, and trunk (HAT) is often considered as the transport cargo. This is an overly simplistic view in that motion of the arms contributed to balance and momentum as well as energy conservation. Trunk motion also is important in maintaining balance as well as

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] # Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_103

adding power input through motion in the thoracolumbar spine. There is little understanding or published data on the exact importance of movements of the HAT segment in pathologic gait of children with cerebral palsy. The most common abnormality at the level of the hip is increased internal rotation in children who are GMFCS level I–III functional ambulators. The most common treatment is femoral derotation osteotomy. The ideal age for doing this is late childhood to adolescence (8–14 years old), and the level in the femur where the derotation occurs is not important. Hip flexion contractures or limited hip extension in stance phase is the next most common problem. Lengthening of hip flexors provides minimal improvement and has risk of creating hip flexor weakness. Hip adductor contractures are also relatively common in 1471

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GMFCS IV and V but less common in highly functional GMFCS I–III ambulators. Pelvic motion is often a compensation for problems related to hip motion. Increased hip flexion contractures causes increased anterior pelvic tilt and may cause increased motion of the pelvis in the anterior tilt direction. The pelvis is also impacted by the lumbar spine in which fixed lumbar lordosis creates anterior pelvic tilt, and lumbar scoliosis may cause a fixed pelvic obliquity. The goal of this chapter will be to consider the pathologic movements and the compensation, which often develop as secondary problems in the gait of children with CP. Keywords

Cerebral palsy · Pelvic obliquity · Pelvic rotation · Pelvic obliquity · Hip flexion · Hip rotation · Hip adduction

Introduction The gait of children with cerebral palsy (CP) is heavily influenced by motor control, balance, musculoskeletal alignment, and force-generating ability. The force input and motor aspect of gait resides primarily in the musculoskeletal system of the pelvis and the two lower extremities. The body above the pelvis including the head, arms, and trunk (HAT) are often considered as the transport cargo. This is an overly simplistic view in that motion of the arms contributed to balance and momentum as well as energy conservation. Trunk motion also is important in maintaining balance as well as adding power input through motion in the thoracolumbar spine. There is little understanding or published data on the exact importance of movements of the HAT segment in pathologic gait of children with cerebral palsy. Understanding the impact of power and position at the level of the hip and pelvis is considerably better. Hip extension power is important to maintain the relationship of the pelvis and the thigh through the hip joint. Hip abductor power is also important to maintain a level pelvis. The interaction of rotation of the thigh relative to the pelvis often causes compensatory movements at the

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level of the pelvis. The goal of this chapter will be to consider the pathologic movements and the compensation, which often develop as secondary issues in the gait of children with CP.

Natural History and Pathophysiology Hip Joint Sagittal Plane The major role of the hip joint is to allow progression of the limb under the body and provide three degrees of motion between the lower limb and the body. The hip joint is also an important power output joint to propel the body forward. In the sagittal plane, the hip is typically flexed at initial contact, which is seldom a problem even if the flexion is slightly exaggerated. At weight acceptance the hip is starting to extend as the body is moving forward over the fixed limb. The ankle and knee should be acting as shock absorbers. If the ankle and knee are held stiff, the hip extension may be slowed. The hip extenders are very active in weight acceptance as the body falls forward with momentum. The main hip extenders are the gluteus maximus and the gluteus medius along with the hamstrings, which forcefully contract and output power, effectively lifting the body up again. If the hip extensors are weak, some compensation may occur by shifting power input even more proximally and using the spine extensors or the paraspinal muscles to create increased lumbar lordosis. This works to keep the body up right but may feed into increased anterior pelvic tilt and hip flexion. Weak hip extensors are assessed by physical examination and by the weight acceptance hip extension moment and power generation in early stance phase. Another sign of hip extensor weakness is an early crossover of the hip moment from extension in early stance to flexion in terminal stance. This crossover should occur between mid- and late stance and not during weight acceptance. Treatment of weak hip extensors should include a strengthening program. For severe weakness, an ambulatory aid, either a crutch or a walker that allows the arms to assist the hip extensors in lifting the forward fall of the body during weight acceptance, should be prescribed.

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Normal

Preswing

Midswing

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Weak Hip Flexor

Preswing

Midswing

Fig. 1 The primary hip flexors assist with increasing hip flexion acceleration in preswing and into early swing phase. If these muscles are not functioning because of

weakness or contracture, the abdominal muscles can provide an adaptive mechanism by increasing pelvic tilt motion to augment inadequate hip flexion

In midstance phase, the hip continues to extend as the weight-bearing limb moves behind the body. Hip flexion contractures, mainly of the hip flexors, primarily the psoas, may cause the extension to be limited. This limitation requires secondary adaptation of increasing anterior pelvic tilt and may prevent full knee extension (Fig. 1). Hip flexion contracture may be measured by several different physical examination methods, but it is most important to have a sense of what the normal range is for the method used. Hip extension in the kinematic measurement in midstance should come nearly to neutral or up to 20 of extension; however, the normal range for the specific marker placement should be considered. Treatment of hip extension deficiency includes stretching exercises of the hip flexors or lengthening the psoas through a myofascial lengthening of the common iliopsoas tendon. Lengthening of the psoas has not been shown to consistently decrease anterior pelvic tilt (DeLuca et al. 1998); however, one report found that it did better in younger children (Sutherland et al. 1997). Modeling studies suggest that the iliopsoas may be shortened more relative to normal in crouched gait than the hamstrings (Delp et al. 1996). Occasionally, a contracture of the rectus femoris or the fascia latae can contribute to the hip flexion contracture. Contractures of the rectus femoris and the fascia latae should be evident on physical examination. In terminal stance phase, the hip again starts to flex, and much of the power for this hip flexion in normal gait comes from the gastrocsoleus push-

off burst. However, in most children with CP, this gastrocsoleus burst is deficient, and the direct hip flexors are the primary power output source to move the limb forward. This burst is also the main source of power that causes knee flexion. The primary hip flexor muscles are first the iliopsoas, followed slightly later in the cycle by the adductors, primarily the adductor brevis and the gracilis. Inactivity or weakness of the hip flexors is demonstrated by delayed hip flexion on the kinematic measurement and by absent hip flexion moment or late crossover from the extension to flexion moment in late stance phase. The compensations for a weak hip flexor are increased pelvic movement, usually a posterior pelvic tilt in terminal stance and a slow velocity of walking, especially caused by decreased cadence. Treatment of hip flexor weakness is first to avoid excessive surgical lengthening of the psoas and adductors. Strengthening exercises are the only option for adding strength to these muscles if weakness is the major problem. The use of assistive devices, such as walkers or crutches, will not help with the problem of hip flexor weakness and often makes it worse. The weakness of the hip flexors in terminal stance is magnified by crutch use because crutch users generally lean forward, increasing their hip flexion and causing the need for even more hip flexion in swing phase. The forward lean also tends to put less prestretch on the hip flexors, making them even less effective as power generators. Having the muscle at the optimum position on the length–tension curve is an

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flexion and knee extension at a time when momentum of the swinging limb is needed going into terminal swing. In terminal swing, the excessive activity of the hamstrings is again the most common problem. The effects of this activity are most dramatic at the knee, but the hamstrings contraction, if it is very excessive, may also limit hip flexion in terminal swing. Compensation occurs at the pelvis, where a posterior pelvic tilt may occur as a compensation for excessive hamstrings force in terminal swing. If the hamstrings or vastus muscles are very weak, the gluteus maximus and medius may substitute by a forceful contraction in terminal swing, which causes the knee to fully extend. This contraction places the knee in a fully extended preposition for initial contact (Fig. 2). This is a position of maximum inherent stability for initial contact and weight acceptance, but it allows poor shock absorption at the knee.

Fig. 2 The hip extensors also provide important function in controlling knee position. During stance, this is provided in coordination with the gastrocsoleus in which knee extension is produced as a result of hip extension. Momentum is moving the body forward over the fixed stance phase foot, allowing the hip and gastrocsoleus to control knee stability. In swing phase, the deceleration of the hip flexion by the hip extensors can allow the knee to swing into full extension if the hamstrings are not activated

important way to increase the muscle’s functional strength, but crutch use tends to do the opposite with hip flexors. Another common disability from weak hip flexors is the inability to step up on a curb or a stair step. Difficulty with stepping into vehicles or bathtubs is also common complaint. In initial swing, the hip flexor continues to be active as the force for initiating the forward swing of the swing-phase limb. The hip flexor is also the force that produces the knee flexion. Problems of terminal stance are continued with the same implications in initial swing. In midswing, there is seldom much direct impact, except for the common problem in CP of premature initiation of hamstring contractions, which tends to limit hip

Coronal Plane Hip Pathology The coronal plane motion of the hip is used to keep the center of mass of the body in midline and allow the feet to be under the body close to the midline. At initial contact, the hip is abducted slightly, which decreases in midstance and then increases again at toe-off. During swing, the process is repeated. If there is a contracture of the adductor at initial contact, there will be less hip flexion, and the foot will be positioned across the midline, where it tends to impede the forward line of progress during swing phase of the contralateral limb. This pattern, in which the foot is positioned across the midline, causes the scissoring gait pattern. In the scissoring gait pattern, the swing-phase foot gets trapped behind a foot that has been placed too medially. If the adductor contracture or overactivity is unilateral, the uncontracted hip can abduct, compensating along with pelvic obliquity. This pelvic obliquity will then cause a limb-length discrepancy, which has to be compensated for. The primary assessment of coronal plane hip pathology is based on physical examination measurement of hip abduction with the hip extended and the measurement of hip abduction on the kinematic evaluation. The hip should abduct slightly at initial contact. Then,

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there may be several degrees of abduction in midstance phase and swing phase. The main treatment for overactive or contracted adductors usually requires surgical lengthening. A contracted adductor is not a common problem in children who are gross motor function classification system (GMFCS) I or II functional ambulators. Some children who are marginal ambulators GMFCS III or IV and require walkers or gait trainers have increased adduction such that the feet are always crossed and they cannot step. Some of these children have adduction because of poor motor control, in which a total flexor response is used to initiate stepping (Case 1). This flexor response includes hip flexion, adduction, knee flexion, and ankle plantar flexion. Even if the adductor is lengthened, for some children, the motion continues unless all the adductors are removed, which will only cause a new problem. Unilateral increased hip adduction can also be a secondary response to limb-length inequality. In children with CP, this inequality can be a physically short limb but is more commonly a functional limb shortening due to asymmetric hip, knee, or ankle flexion. Treatment of the limb-length inequality will treat the hip adduction. Asymmetric adduction on one hip and abduction on the opposite hip may also be caused by fixed pelvic obliquity emanating from spinal deformities. Increased hip abduction leads to a wide-based gait, which is cosmetically unappealing and is very functionally disabling if the children are functional ambulators. The wide-based position forces excessive side-to-side movement of the body to keep the center of mass over the weightbearing limb. If children have increased abduction with a wide-based gait but have no abduction contracture on physical examination, the cause of the wide-based gait is weakness of the adductor muscles. Usually, the cause is incompetent adductors secondary to excessive adductor lengthening or the addition of an obturator neurectomy to an adductor lengthening (Case 2). The best treatment of this problem is to prevent it from happening by not doing excessive adductor lengthening or neuroectomies on functional ambulators at GMFCS I–III level. However, if presented with the problem, working on strengthening the

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remaining adductor strength and allowing the children to grow often slowly correct the problem. There are no other treatments available. The widebased gait may also be due to an abduction contracture, usually of the gluteus medius or fascia latae. The etiology of wide-based gait due to a contracture requires identifying the source of the contracture, and the kinematic measure should show increased abduction, especially in midstance phase. Once the specific source of the abduction contracture is identified, the treatment is surgical lengthening of the contracted muscle. Fixed contractures of the hip joint may also cause the same effect as muscle contractures. Sometimes, this contracture requires a radiographic evaluation of the joint to determine if the source is the muscle only or a combination of the muscle and subluxation or dysplasia of the hip joint.

Transverse Plane Deformity Transverse plane deformity in children is common and is often confused with coronal plane deformity. The difference between scissoring, which is excessive hip adduction, and hip internal rotation gait is often missed. Scissoring is a completely different motion requiring a different treatment (Fig. 3). Hip rotation is defined as a rotation of the knee joint axis relative to the center of hip motion in the pelvis. In normal gait, this rotation around the mechanical axis of the femur allows the feet to stay in the midline and allows the pelvis to rotate on top of the femur, which are both motions that work to decrease movement of the HAT segment and therefore conserve energy. At initial contact, the normal hip has slight external rotation of approximately 10 , and then it slowly internally rotates, reaching a maximum at terminal stance or initial swing phase. If the hip is positioned in internal rotation at initial contact, then during stance phase as the knee flexes, there is an obligatory hip adduction and the knee may impact the opposite limb (Case 1). If the internal rotation is present during midstance, such as in a crouched gait pattern, the knees often rub during swing phase of the contralateral limb. Internal rotation positioning in terminal swing also causes the knee to cross the midline, a problem that continues into initial swing. Another primary effect of this

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Fig. 3 Crossing over of the knees is often called scissoring gait. However, it is better to use the term scissoring gait only when it is caused by true hip hyperadduction. Most of the time, crossing over of the knees is due to internal rotation of the hips, often secondary to increased femoral anteversion and not caused by primary increased hip adduction

internal rotation is placing the knee axis out of line with the forward line of motion. This position causes significant alteration in mechanical efficiency of the push-off power generated at the ankles. Secondary adaptation to the internal rotation of the hip includes decreased knee flexion at weight acceptance in swing phase and decreased ankle push-off power burst and requires the use of more hip power. If the internal rotation is unilateral, the pelvis may rotate posteriorly on the side of the internal hip rotation, and then the contralateral hip compensates with external rotation. The amount of internal rotation is assessed by physical examination with children prone and the hips extended (Case 3). The kinematic measure should show external rotation through almost all of the gait cycle. There

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are two problems with the kinematic measure of which clinicians must always be aware. First, the measure is very dependent on defining the axis of the knee joint by the person placing the marker. An error of 5–10 in defining the knee joint axis is not uncommon. The second major issue is all clinical gait software programs currently use rotation as the last Euler angle to decompose the motion. This means that often the measured degree of rotation is less than clinicians perceive, probably because they are mentally derotating the hip first. This is not an error in the kinematics or the clinicians’ assessments but is related only to the method of expressing the position. Clinically, the hip rotation may be more significant than the kinematic measure suggests. The principal cause of the increased internal rotation is increased femoral anteversion. A secondary cause may be a contracture of the internal rotators. A third cause may be motor control problems as mentioned with increased scissoring, which are often seen in GMFCS III and IV marginal ambulators. For children who previously had surgery on the hip and in whom there is a question as to the specific cause of the internal rotation, measurement of the femoral anteversion with ultrasound or CT scan should be considered. Children in middle childhood or older who are GMFCS I–III functional ambulators tend to do poorly with internal rotation that is greater than 10 during terminal stance phase (Akalan et al. 2013). From middle childhood on, there is little apparent spontaneous correction of the internal rotation. Children who are GMFCS I or II and have any internal rotation during stance phase are easily cosmetically observed as having internal rotation. Some children with 0–15 of internal rotation of the hip in stance phase seem to have very few measurable mechanical problems; however, parents often notice that they trip more frequently, which may be due to decreased knee flexion to avoid knees crossing over the midline. These increased problems that require sophisticated motor control probably cause children with CP to be clumsier. Also, during running when there is increased knee flexion, a heel whip will appear if children have persistent internal rotation. This heel whip clearly adds to children’s poor

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coordination during running. Treatment of increased internal rotation is a derotation femoral osteotomy, which will improve the foot progression angle (Tylkowski, Rosenthal, and Simon) (Kwon et al. 2013; Niklasch et al. 2015). If the source of the internal rotation is felt to be a contracture of the internal rotators of the hip, the most usual cause is the anterior fibers of the gluteus medius and the gluteus minimus (Joseph 1998). Excessive external rotation of the hip during gait is rarely a primary problem of gait in children with CP. Usually, this external rotation is associated with hypotonia and may be part of a progressive anterior hip subluxation syndrome (Case 4). Typically, these children start losing functional ambulatory ability as the hip increases its external rotation at the same time the anterior subluxation is increasing. The treatment is to correct the hip joint pathology. The second situation where external rotation may be seen is secondary to excessive external rotation of the femur following femoral derotation for treatment of femoral anteversion (Niklasch et al. 2015). The rule of thumb should be that a little external rotation is better than a little internal rotation, with the goal being 0–20 of external rotation. However, too much external rotation, meaning greater than 20 , is worse than a little internal rotation of 0–10 . The goal should be to have 0–10 of femoral anteversion, and the kinematic measure should show 5–20 of external rotation of the femur during stance. Femurs with excessive external rotation may need to be turned back into internal rotation again. Imaging studies should be obtained to fully assess the deformity before undertaking repeat surgery because external rotation contractures can occasionally occur. These external rotation contractures usually involve the posterior half of the gluteus medius and the short external rotators of the hip joint.

Pelvis Pelvic motion is viewed as motion of the pelvis in the room coordinate system. Observational gait analysis of pelvic motion is difficult because this body segment does not have clear borders, and it is socially difficult to have children undressed at

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the pelvic level. Therefore, trying to see the pelvis move is somewhat like watching the neighbor’s television through a window covered with a curtain. Pathologic motion of the pelvis occurs either with excessive motion or asymmetric motion. Excessive pelvic motion is defined as more than 10 on the kinematic measure in any of the three directions and is usually due to increased tone, which has stiffened the hip joint and limits hip motion (Table 1). Often, treatment is not needed as this is a functional way of increasing mobility that has only a slightly increased energy cost. This increased pelvic rotation may cause heel whip during running, therefore making running more difficult. The only available treatment is to decrease muscle tone by rhizotomy or intrathecal baclofen, both of which cause or bring out muscle weakness. Often, the weakness is more impairing to the gait function than the stiffness.

Pelvic Rotation Asymmetric pelvic rotation may be primarily caused by motor control or as a secondary adaptation for asymmetric hip rotation. Children with very asymmetric neurologic involvement, especially severe hemiplegic patterns, often lead with the most functional side of the body. Leading with the functional side of the body seems to be a motor control attractor, probably because it is easier to control the impaired limb in the trailing position. If the asymmetry is only 10–20 , trailing of the involved side is not very cosmetically apparent and usually needs no treatment. Most rotations greater than 20 are cosmetically apparent and cause functional problems, such as increased tripping and poor coordination, especially in GMFCS I or II functional ambulators. If the rotation is severe, sometimes reaching 45–60 , children are walking sideways, which is ineffective and very cosmetically noticeable (Case 5). Severe rotation is often a combination of asymmetric hip motion and motor control, which should be addressed by making all efforts to correct hip asymmetries and even slightly overcorrecting these asymmetries. Many children have pelvic rotation asymmetry due to asymmetric hip rotation or adduction. Physical examination should focus on hip rotation with hips extended and with hip abduction. The

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Table 1 Segment and joint compensations Problem Pelvis Increased anterior tilt

As the primary etiology

Compensatory effect for

Component of lumbar lordosis that is compensated by increased hip flexion

Compensating for a hip flexion contracture or hip extensor weakness

Increased pelvic tilt motion Asymmetric pelvic rotation Increased pelvic rotation motion Asymmetric pelvic obliquity Increased drop on swing side Hip Decreased flexion in swing Decreased flexion Decreased extension stance Increased abduction Increased adduction (scissoring) Increased internal rotation Increased external rotation

Hemiplegia type motor control

Lumbar scoliosis

Hip stiffness or hip weakness Or high spasticity Asymmetric femoral rotation – pelvis rotates posterior on the hip internally rotated side Decreased push-off from gastrocsoleus, hip stiffness, or hip flexor weakness Hip abduction or adduction contracture, limb-length discrepancy, ankle plantar flexion contracture, asymmetric knee flexion contracture Abductor muscle weakness

Hip joint stiffness or extension muscle contractures (hamstrings or gluteus) Hip flexor weakness

Weak push-off power burst from the ankle plantar flexors

Hip flexor contracture, joint stiffness

Lack of knee extension

Weak adductor muscle, hip joint, or abductor contractures Adductor contracture

Adduction contracture of the opposite hip, ataxia

Increased femoral anteversion, contracture of internal rotators External rotation contracture, retroversion of femur

Asymmetric pelvic rotation, external tibial torsion, severe planovalgus Asymmetric pelvic rotation often due to opposite hip internal rotation Internal tibial torsion

hip on the side of the pelvis that is rotated posteriorly should have more internal rotation or have less passive external rotation. Typically, this hip has increased adduction and flexion contracture as well. The treatment is to do a unilateral hip derotation and adductor lengthening if the adductor is contracted, meaning there is less than 20 of hip abduction with the knee extended. Excessive adductor lengthening should not be done; a percutaneous adductor longus tenotomy only is often sufficient.

Pelvic Tilt Anterior pelvic tilt may have increased magnitude, be asymmetric, or be increased in either

Hip extension contracture

Poor motor control Component of internal hip rotation gait

direction. Increased magnitude of pelvic motion is very common and is related to increased tone in the lower extremities. Also, the increased magnitude serves as another proximal power input joint and is used to propel the swing limb forward. Increased pelvic tilt is also present with hip flexion contractures, specifically with iliopsoas contracture, and has been called the double bump pelvic motion. This term is somewhat misleading because it suggests a new pathologic movement pattern of the pelvis, which is not true. This pelvic motion is only a magnification of the normal movement. In many patients, this pelvic motion serves a useful secondary adaptation to help with swing phase in a lower limb with increased

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stiffness or decreased power output. If children are very functional at GMFCS I or II with good ankle push-off power generation, it is possible to decrease this pelvic motion through lengthening the hamstrings and the psoas, which increases the hip joint range of motion (Westwell et al. 2009). If the hip is the main source of power output, these lengthenings run the risk of shifting the length–tension curve such that the weakness of the hip muscles will be magnified and the pelvic tilt range may increase even more to compensate (DeLuca et al. 1998). Increase in anterior pelvic tilt primarily occurs due to increased hip flexion contractures or secondarily occurs due to increased lumbar lordosis. The normal upper range for anterior pelvic tilt is 15–20 , although this varies somewhat with different marker placement algorithms. An increase to 25 is common in children with CP. Weakness of the hip extensors and increased force in the hip flexors are the primary causes of increased anterior pelvic tilt. Primary lumbar lordosis is another cause, and it may be difficult to separate primary lumbar lordosis from lumbar lordosis as a secondary response to increased anterior pelvic tilt due to increased hip flexion forces. Increased pelvic tilt and lumbar lordosis are strong attractors in motor control, possibly because they increase stability and lock the lumbar spine, thereby producing more mechanical stiffening. Iliopsoas lengthening should be performed if lumbar lordosis is flexible, hip flexor contracture is present, hamstring lengthening is needed to improve knee kinematics, and these individuals are GMFCS I or II ambulators. If a child does not meet all these criteria, iliopsoas lengthening may have more side effects than benefits. If the lordosis is stiff, muscle surgery will not affect anterior pelvic tilt. If the iliopsoas is not contracted, psoas lengthening will only weaken effective hip flexion. However, if hamstring lengthening is performed and the contracted hip flexor is not lengthened, the anterior pelvic tilt will usually get worse; however this can be reduced if only the medial hamstrings are lengthened DeLuca et al. (1998). These individuals often develop the jump position with forward lean of the trunk on the anterior tilted pelvis. Over time, the compensation is obtained by

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having increased lordosis (Fig. 4). For individuals who use walkers or crutches, hip flexor lengthening will increase apparent weakness due to increased anterior pelvic tilt from always leaning forward. Increased posterior tilt is usually defined as abnormal if there is any posterior tilt past neutral. The principle cause of posterior pelvic tilt is a contracture of the hamstrings. The posterior pelvic tilt has to be correlated by physical examination. The posterior tilt may be due to gluteus contractures; however, we have never seen this in children with CP. Treatment is lengthening of the hamstrings if they are contracted. A secondary cause of posterior pelvic tilt is lumbar kyphosis or,

Fig. 4 Some children who are GMFCS I or II ambulators have significant hamstring contractures requiring lengthening of the hamstrings. They should be carefully examined to be sure that there are not significant hip flexor contractures. This boy, 1 year after hamstring lengthening, has developed severe hyperlordosis primarily because the hip flexors were not lengthened. There are children, however, who naturally take on this posture and, when they are examined, may not have a hip flexion contracture

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Pelvic Obliquity Most causes of abnormal pelvic obliquity are due to asymmetric contractures of the hip adductors or abductors or weakness of one of the muscle groups. This pelvic obliquity may be secondary to apparent or real limb-length discrepancy, or it may be secondary to fixed scoliosis. Pelvic obliquity may be asymmetric when one side has strong muscles and hip hiking on the swing side is used to help with clearance. The Trendelenburg gait, often discussed by writers concerned with hip pathology, is really only a magnification of normal movement pattern, much like the double bump anterior pelvic motion. This gait is a response to mild weakness in the abductors as the hip on the swing side drops more to pretension the abductor muscle until it finds the strength to resist. Increased movement of

the center of mass of the HAT segment over the weight-bearing limb is usually combined with this, thereby decreasing the force needed to resist the drop of the pelvis. This pattern may also suggest mechanical instability of the hip joint, such as hip subluxation (Metaxiotis et al. 2000), and hip radiographs should be obtained. With severe weakness of the abductor muscles, the center of mass of the HAT segment will move completely over the weight-bearing limb, usually with elevation of the pelvis on the swing side. This movement is called a hip lurch, in which the trunk muscles can also be used to control the drop of the pelvis on the swing limb side (Fig. 5). Treatment of Trendelenburg gait is by strengthening of the abductor muscles when possible. Treatment of the lurch gait pattern is by strongly encouraging patients to use forearm crutches, which will decrease both the energy of walking and the force on the joints in the lower extremities, especially the knee joint. Some of these movement patterns may also occur secondary to pain in the

Fig. 5 Obliquity of the pelvis can change in different ways to accommodate muscle weakness, hip pain, or motor coordination problems. In normal gait, the abductors are used to maintain the pelvis with only minimal motion. As the muscles develop mild weakness, the pelvis may drop on the swing limb side in a motion commonly called Trendelenburg gait. There is little movement of the center

of mass in this gait pattern. As the weakness or pain becomes more severe, the pelvis raises on the swing limb side as the center of mass moves laterally over the stance phase limb, causing a gait pattern commonly called a lurch. As the muscle weakness becomes more severe, the pelvis may drop on the swing limb side as the center of mass moves laterally over the stance limb

more commonly, total spinal kyphosis. Correction of the kyphosis will correct the posterior pelvic tilt.

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hip or knee joints. Therefore, a good medical history should be available with the gait analysis.

HAT Segment The real function of gait is to move the HAT segment in space. This segment, however, is not only passive cargo. By the use of trunk muscles, neck muscles, and arm movements, the HAT segment can position its center of mass to assist in gait. In normal gait, the HAT segment primarily involves passive motion, which will cause the center of mass to have the least movement away from the line of progression. Through the motor control system, the center of mass can be positioned in front of the hip joint to allow the hip extensors to be more effective as power generators, or it can be positioned behind the hip joint so the weak hip extensors are not stressed and the anterior hip capsule or hip flexors are the primary supports of the mass (Fig. 6). As was discussed with lurching, the trunk muscles can output force and provide power for movement in children (Fig. 5). The contribution of active power generation of the HAT segment is not well understood. Typically, the trunk is rotated posteriorly on the

Fig. 6 The motor control system can adjust the trunk alignment and the position of the center of mass or center of gravity (COG), so either the ground reaction force goes directly through the hip joint, therefore requiring little hip muscle power posterior to the hip in which the hip flexors

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involved side of individuals with hemiplegia. Often, the arms are in the high to medium guard positions with elbow and shoulder flexion in individuals with poor balance. Treatment specific for asymmetries of trunk motion or increased magnitude is primarily directed at determining the need for assistive devices. Individuals with 20–30 of trunk motion side to side usually do better with walking aids such as crutches, especially for longdistance walking.

Treatment and Outcome Summary Abnormal movements at the level of the hip and pelvis and children with CP are common. The most common abnormality at the level of the hip is increased internal rotation in children who are GMFCS level I–III functional ambulators. The most common treatment is femoral derotation osteotomy. The ideal age for doing this is late childhood to adolescence (8–14 years old) Tylkowski et al. (1980), but there is a risk for recurrence until adulthood (Dreher et al. 2012; Church et al. 2017). The level in the femur where the derotation occurs is not important (Pirpiris et al. 2003). Hip flexion contractures or

are the main active muscles or anterior to the hip joint, requiring mainly hip extensor use. It is often hard to understand why the motor control system chooses one pattern over the other in children with CP

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limited hip extension in stance phase is the next most common problem. Lengthening of hip flexors provides minimal improvement and has risk of creating hip flexor weakness (Bialik et al. 2009). Hip adductor contractures are also relatively common in GMFCS IV and V but not in highly functional GMFCS I–III ambulators. Transfer of the adductor muscles to the posterior ischium has been proposed but has not been found to be beneficial to gait function as it increased pelvic obliquity (Scott et al. 1996). Pelvic motion is often a compensation for problems related to hip motion. Increased hip flexion contractures causes increased anterior pelvic tilt and may cause increased motion of the pelvis in the anterior tilt direction. The pelvis is also impacted by the lumbar spine in which fixed lumbar lordosis creates anterior pelvic tilt, and lumbar scoliosis may cause a fixed pelvic obliquity. Other causes of increased pelvic obliquity include asymmetric adduction contractures, asymmetric leg lengths, and hip abductor weakness. Treatment requires attempting to bring symmetry through correction of the asymmetric component driving the deformity. This may include lengthening of the adductors or addressing asymmetric leg length and working on abductor muscle strengthening. Developing treatment plans for both pelvic and hip position requires assessment of hip extensors and knee extensors as well as the plantar flexors at the ankle, all of which have multiple impacts (Arnold et al. 2005) (Table 1).

Cases

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physical examination, Jacob was not able to sit unsupported. He could self-feed with a spoon (if the food was sticky like mashed potatoes), had no speech, and was in a special education classroom for children with severe cognitive limitations. The physical examination demonstrated Ashworth grade 1 and 2 spasticity throughout most muscles in the lower extremity and the upper extremity. He had no ability to do individually isolated joint movement in the lower extremity. The hip demonstrated a symmetric 30 of abduction, popliteal angles were 40 , hip internal rotation was 50 , and external rotation was 30 . Jacob was cooperative in trying to stand and take steps when being held from the back. He had a gait trainer, which he enjoyed. Based on this assessment, Jacob was believed to have significant spasticity; however, this was not felt to be the main cause of the scissoring. The scissoring was due to poor motor control and poor motor planning. It was not thought that he would benefit from further surgical lengthening of the adductor because these were not contracted, and part of the cause of the scissoring was his poor coordination in the use of hip flexors to advance the limb. A baclofen trial was given, but he could not stand with the decreased spasticity after the baclofen injection, and his parents felt the benefit of the decreased spasticity during custodial care would not make up for his functional loss of not being able to stand.

Case 1 Jacob

Jacob, a 10-year-old boy, was brought in by his father with the main complaint that he could not walk because his feet crossed over each other when he stood and tried to walk. His father was most concerned about the boy’s spasticity, which he felt was limiting his ability to walk and was making bathing, dressing, and transferring more difficult. On

Case 2 Sean

Sean, a 5-year-old boy with quadriplegia, had an adductor lengthening and distal hamstring lengthening to treat spastic hip disease at age 3 years. By age 5 years, he walked efficiently with a walker; however, his parents were concerned about his wide(continued)

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based gait and foot drag. On physical examination, he was not able to get into the walker without assistance but had functional gait once he was in the walker. His hip abduction was 50 on each side, full hip flexion and extension was present, the popliteal angle was 40 , and he had grade 2 spasticity in the rectus, with a positive Ely test at 40 . Kinematic evaluation showed increased hip abduction and decreased knee flexion in swing phase with EMGs of the rectus, which were very active in swing phase. His hip radiographs were completely normal. His gait was characterized by a wide-based gait with foot drag and knee stiffness in swing phase. Based on these data, Sean had bilateral rectus transfers because the knee stiffness was believed to be adding to the tendency to have a wide-based gait. He was initiating a circumduction maneuver because of adductor weakness to assist with foot clearance. After the rectus transfers, his base of support narrowed and knee flexion increased nicely. His foot drag also decreased.

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problem had become much more symptomatic over the past year. Tonya had normal cognitive function and no other medical problems. On physical examination, she had 70 of hip internal rotation and 10 external hip rotation. Hip abduction was 20 , popliteal angles were 60 , and the feet were normal. Her gait demonstrated a foot flat gait pattern with mild knee flexion in stance, decreased knee flexion in swing, severe internally rotated knees with heel whip, and mild increased lumbar lordosis. Kinematics showed hip internal rotation of 20 in stance phase. The EMG of the rectus showed mild increased activity in swing phase and that hamstring activity was normal (Fig. C3.1). Based on the EMG activity, the main problem was believed to result from femoral anteversion, and she had femoral derotation osteotomies bilaterally. This procedure resolved all her complaints and substantially improved her knee motion and hip extension.

Case 4 Hameen Case 3 Tonya

Tonya, an 11-year-old girl with a diagnosis of spastic diplegia, complained of increased difficulty in walking due to clumsiness and pain from her knees knocking together. This

Hameen, a 10-year-old boy with hypotonia and mental retardation, had increased difficulty in ambulation. He used to walk everywhere using a posterior walker, but now his mother stated that he refused to walk except for very short distances. She did not (continued)

Fig. C3.1

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perceive that he had any pain. Nine months before this presentation, he had a femoral osteotomy for a subluxating hip at another hospital. Following this osteotomy, his gait had not improved, although he was walking almost as well as he was before that surgery. His health had otherwise not changed, except his mother felt his external rotation of the feet, especially on the left side, was getting worse. On physical examination he was noted to have generalized hypotonia, hip abduction was 60 , full flexion and extension, hip external rotation to more than 90 , and an internal rotation to 60 . The left hip had a click with rotation. Anterior palpation suggested that the femoral head was subluxating anteriorly. A radiograph was obtained that showed a mild lateral displacement of the femoral head with a healed femoral osteotomy (Fig. C4.1), and the CT scan showed that it was slightly anterior (Fig. C4.2). He was observed

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walking with a posterior walker and severe external rotation of the left hip. The cause of his decreased walking tolerance was thought to be the anterior hip subluxation, and he had a Pemberton pelvic osteotomy without a varus femoral osteotomy because the soft tissue was believed to have enough laxity (Fig. C4.3). By 1 year after the surgery, he had returned to his usual walking tolerance, and by 6 years after surgery, he was a fully independent community ambulator with a stable hip (Fig. C4.4). Although he continued to have external foot progression on the left and bilateral back knee, he was without symptoms (Fig. C4.5).

Case 5 Christopher

Christopher, a 6-year-old boy, presented with a diagnosis of CP and a peculiar gait pattern. His parents were concerned that he tripped a (continued)

Fig. C4.1

Fig. C4.2

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Fig. C4.3

Fig. C4.4

lot, and they wanted to improve the appearance of his walking. He had normal speech and was cognitively age appropriate. He had no other medical problems, and his parents felt that he had had very little change in his

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gait in the past year. On physical examination he had significant spasticity in his left upper extremity, with internal rotation at the shoulder, elbow flexion, and wrist flexion. He could use gross grasp of the fingers. He was using the hand as a helper hand without prompting. He had full hip flexion and extension, and abduction was 15 on the left and 28 on the right. Internal rotation of the hip was 80 on the left and 50 on the right. External rotation was 5 on the left and 30 on the right. Knee popliteal angles were 55 on the left and 40 on the right. Ankle dorsiflexion with extended knee was 7 on the left and 0 on the right. Dorsiflexion with the knee flexed was 0 on the left and 8 on the right. His gait demonstrated severe pelvic rotation with the left side being posterior 45–65 throughout the whole cycle. The left knee appeared to be internally rotated relative to the pelvis. The right foot was internally rotated and the left foot was neutral. Both knees were in hyperextension in midstance, with increased knee flexion at foot contact. The upper extremity was held in elbow flexion and internal rotation of the shoulder. Christopher’s pelvic rotation seemed mostly caused by asymmetric hip rotation with the left hip being internally rotated; therefore, a left femoral derotation osteotomy was performed to correct this. The deformity was probably being exaggerated because of his hemiplegic motor control problems. Lengthening of the adductor on the left also helped to allow the limb to externally rotate and abduct. Lengthening the tendon Achilles on the left and the gastrocnemius on the right helped the knee extension in midstance. Following these procedures, the pelvic rotation improved significantly; however, he developed a planovalgus foot, partly due to a split transfer of the tibialis posterior tendon, which should not have been done. Several other operative (continued)

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Fig. C4.5 Flexion

80.0 60.0

Knee Motion

40.0 20.0 0.0

Extension

–20.0

Dorsiflexion

30.0 20.0

Ankle Motion

10.0 0.0 –10.0

Plantarflexion Toes In

–20.0 30.0 0.0

FPA –25.0

Toes Out

procedures for other problems were required during his growth period; however, the pelvic rotation remained corrected until he reached full maturity.

Cross-References ▶ Femoral Anteversion in Children with Cerebral Palsy ▶ Foot Deformities Impact on Cerebral Palsy Gait ▶ Knee Deformities Impact on Cerebral Palsy Gait

References Akalan NE, Temelli Y, Kuchimov S (2013) Discrimination of abnormal gait parameters due to increased femoral anteversion from other effects in cerebral palsy. Hip Int 23:492–499

–60.0

Arnold AS, Anderson FC, Pandy MG, Delp SL (2005) Muscular contributions to hip and knee extension during the single limb stance phase of normal gait: a framework for investigating the causes of crouch gait. J Biomech 38:2181–2189 Bialik GM, Pierce R, Dorociak R, Lee TS, Aiona MD, Sussman MD (2009) Iliopsoas tenotomy at the lesser trochanter versus at the pelvic brim in ambulatory children with cerebral palsy. J Pediatr Orthop 29:251–255 Church C, Lennon N, Pineault K, Abousamra O, Niiler T, Henley J, Dabney K, Miller F (2017) Persistence and recurrence following femoral derotational osteotomy in ambulatory children with cerebral palsy. J Pediatr Orthop 37(7):447–453 Delp SL, Arnold AS, Speers RA, Moore CA (1996) Hamstrings and psoas lengths during normal and crouch gait: implications for muscle-tendon surgery. J Orthop Res 14:144–151, SRC – GoogleScholar DeLuca PA, Ounpuu S, Davis RB, Walsh JH (1998) Effect of hamstring and psoas lengthening on pelvic tilt in patients with spastic diplegic cerebral palsy. J Pediatr Orthop 18:712–718 Dreher T, Wolf SI, Heitzmann D, Swartman B, Schuster W, Gantz S, Hagmann S, Doderlein L, Braatz F (2012) Long-term outcome of femoral derotation osteotomy in children with spastic diplegia. Gait Posture 36:467–470

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Joseph B (1998) Treatment of internal rotation gait due to gluteus medius and minimus overactivity in cerebral palsy: anatomical rationale of a new surgical procedure and preliminary results in twelve hips. Clin Anat (New York, NY) 11:22–28 Kwon DG, Lee SY, Kim TW, Chung CY, Lee KM, Sung KH, Akhmedov B, Choi IH, Cho TJ, Yoo WJ, Park MS (2013) Short-term effects of proximal femoral derotation osteotomy on kinematics in ambulatory patients with spastic diplegia. J Pediatr Orthop B 22:189–194 Metaxiotis D, Accles W, Siebel A, Doederlein L (2000) Hip deformities in walking patients with cerebral palsy. Gait Posture 11:86–91, SRC – GoogleScholar Niklasch M, Dreher T, Doderlein L, Wolf SI, Ziegler K, Brunner R, Rutz E (2015) Superior functional outcome after femoral derotation osteotomy according to gait analysis in cerebral palsy. Gait Posture 41:52–56

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Pirpiris M, Trivett A, Baker R, Rodda J, Nattrass GR, Graham HK (2003) Femoral derotation osteotomy in spastic diplegia. Proximal or distal? J Bone Joint Surg Br 85:265–272 Scott AC, Chambers C, Cain TE (1996) Adductor transfers in cerebral palsy: long-term results studied by gait analysis. J Pediatr Orthop 16:741–746 Sutherland DH, Zilberfarb JL, Kaufman KR, Wyatt MP, Chambers HG (1997) Psoas release at the pelvic brim in ambulatory patients with cerebral palsy: operative technique and functional outcome [see comments]. J Pediatr Orthop 17:563–570, SRC – GoogleScholar Tylkowski CM, Rosenthal RK, Simon SR (1980) Proximal femoral osteotomy in cerebral palsy. Clin Orthop Relat Res 151:183–92 Westwell M, Ounpuu S, DeLuca P (2009) Effects of orthopedic intervention in adolescents and young adults with cerebral palsy. Gait Posture 30:201–206

Crouch Gait in Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1490 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1490 Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1491 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1493 Performing the Crouched Gait Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1495 Spasticity Reduction in Adolescents and Young Adults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1495 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1496 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1497 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1503 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1503

Abstract

Children with cerebral palsy (CP) start to walk usually with an assistive device typically a walker. Children who start to walk independently by age 3 are usually more mild and at lower risk for late severe deformities which impair their gait. As children develop and mature in their gait pattern, they very frequently develop abnormal knee kinematic patterns especially increased knee flexion in stance phase. This is much more common in children with diplegia or bilateral CP compared to those compared with hemiplegia or

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_104

unilateral involvement. A very common problem is increased knee flexion in stance phase which is typically called crouch gait. Crouch gait (flexed knee gait) is a very complex multidimensional deformity in children whose natural history is extremely variable. The primary focus of crouch gait tends to be knee flexion in stance phase; however, this syndrome often involves torsional malalignment of the femur or tibia, ankle positional problems either equinus or hyper-dorsiflexion, as well as foot postural problems typically planovalgus. Treatment of crouch gait requires very careful assessment with three-dimensional gait analysis and identification of all the pathologic features which require correction. Surgical correction is usually carried out with single-

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event multilevel surgery (SEMLS). This chapter defines the current understanding of the etiology of crouch gait, the correct full evaluation, and the surgical planning requirements. Keywords

Cerebral palsy · Crouch gait · Knee flexion · Knee extension osteotomy · Single-event multilevel surgery · Jump gait · True equinus · Apparent equinus

Introduction Children with cerebral palsy (CP) start to walk usually with an assistive device typically a walker. Children who start to walk independently by age 3 are usually more mild and at lower risk for late severe deformities which impair their gait. As children develop and mature in their gait pattern, they very frequently develop abnormal knee kinematic patterns especially increased knee flexion in stance phase. This is much more common in children with diplegia or bilateral CP compared to those compared with hemiplegia or unilateral involvement. A very common problem is increased knee flexion in stance phase which is typically called crouch gait. The term “crouch gait” has also been used in classification systems with much more specific meaning that includes the definition of ankle position as well. A common classification published by Rhoda uses crouch gait to mean only those patients who have increased knee flexion in midstance phase and hyperdorsiflexion of the ankle (Rodda et al. 2006). In this classification scheme, patients with increased knee flexion in midstance with ankle equinus defined as having “jump gait” and those with neutral ankle motion are defined as having “apparent equinus gait.” This classification system has been demonstrated to be internally consistent and valid for classification of an individual patient at one time in their assessment (Rodda et al. 2006). The problem with this definition and classification system is that this is a continuum along which many patients move during their development

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and does not classify patients over time into a specific group which is stable. More specifically this classification system does not function the way the Winter classification for hemiplegia functions in which a child once reaching a stable gait pattern in middle childhood will remain in that group throughout their maturity (Winters et al. 1987). Because crouch gait occurs primarily in individuals with bilateral CP, there are many patients who also have very asymmetrical patterns which further make classification difficult. Because of the evolutionary difficulty with defining bilateral gait patterns, we will use the term “crouch gait” as we proceed in this discussion to mean increased stance-phase knee flexion as measured by maximum stance knee extension in stance and then will further clarify other parameters such as ankle position as they are significant. The goal of this chapter will be to define the etiology of couch gait, discuss important parameters affecting its natural history, and discuss treatment options.

Natural History and Pathophysiology Most young children with diplegic CP before the age of 3 will start walking utilizing a walker and will typically be in the equinus often with some knee flexion. For many of the children, the primary driver is equinus with some compensation at the knee to provide for stability. A few children in the young age between 3 and 5 will start to develop some knee flexion contracture because the knee flexion in stance is becoming obligatory often in spite of ankle position. When the ankle is controlled with an orthotic, the knee will often still be flexion causing the child to go up on the toe of the shoe. In general, we believe this group of children is at the highest risk for having later progressive severe crouch gait as they continue to grow and mature; however there is no published data to document this. This lack of data is due to the fact that these children tend to be treated at a young age because of the fear of later progression, and those children who do not

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receive early treatment usually have had no quantitative monitoring or documentation. During adolescence with the rapid onset of weight and height growth, the classic crouch gait develops, gets worse, and may prevent some children from functional ambulation if it is not treated appropriately. The crouch pattern may be seen in all levels of severity; however, it is primarily encountered in moderate and severe diplegia. This adolescent crouched gait typically has increased knee flexion in midstance with increased ankle dorsiflexion and usually increased hip flexion. The toe walking knee flexion pattern is seldom seen in full adolescence or nearly adultsized individuals. The muscles and joints are not strong enough to support the body weight for chronic ambulation with the typical early childhood toe walking pattern. If young children are left untreated, the natural history during late middle childhood, when knee flexion in stance increases and the foot starts to dorsiflex, causes collapsing through the midfoot and hindfoot as severe planovalgus foot deformities develop. During the time when children are growing rapidly and increasing weight quickly, midstance phase knee flexion will increase, and ankle dorsiflexion and hip flexion will also increase by a compensatory amount. Individuals who use walking aids tend to increase weight bearing on the walking aids during this time by increasing anterior lean (Case 1). Many adolescents with mild crouch gait, defined as knee flexion in midstance between 10 and 25 , will not progress or need any treatment except occasional single joint level treatment, such as correction of planovalgus feet. The high risk for progression and need for surgical should be done on individuals with moderate crouch, meaning midstance phase knee flexion of 25–45 . Only rarely, and usually in medically neglected patients, is surgery done in severe crouched gait with knee flexion in midstance greater than 45 . As with many other conditions, allowing the crouch to become severe means the treatment is less effective. The symptoms of increasing crouch include the complaint of knee

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pain as the stress rises on the knee extensor muscles to support weight bearing. Distal pole of the patella and tibial tubercle apophysitis may occur, especially during rapid growth (Elhassan et al. 2013a, b; Rethlefsen et al. 2015). Walking endurance will decrease, and feet collapsing will start causing more pain with long-distance walking as the planovalgus develops higher pressure areas. The orthotics are no longer able to support the collapsing feet. All these progressive additive impairments combine to frustrate adolescents, and parents typically complain that the individual is losing motivation to walk.

Pathophysiology The exact etiology of the development of crouch gait in an individual child is extremely complex and almost never able to be completely be defined. At a specific time in the child’s life, the observed posture of hip, knee, and ankle position is a complex combination of the child’s motor control, balance, bodyweight, muscle lengths, bony configuration, overall muscle endurance, and cardiovascular conditioning. This is only a partial list, and there is poor understanding of the relative importance of each that adds to this complexity. I believe the best way to understand the evolution of crouch gait in a growing child is to conceptualize this problem the same way that weather forecasts are produced. Weather forecasting technology uses many inputs from many different locations and across time to predict the weather in a specific location. Weather forecasting is most accurate when one is looking at short-term forecasts such as 24–72 h forecast. There is moderate predictability for forecasts from 10 to 14 days. As the forecast time into the future increases, its accuracy drops quickly. A forecast of 30 days into the future may not be much better than looking at the historical record of weather in this area. Weather forecasting uses complex computer modeling with multiple known inputs. They also recognize that each input may have somewhat unpredictable affect over time. How does the

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understanding of weather forecasting help in understanding crouch gait? The factors that have an impact on the development of crouch gait include many different parameters which change over time. Each of these parameters will have a changing impact over time as the child grows and the gait pattern continues to develop. The other important lesson from weather forecasting is the time element. When a child is seen at age 3 walking with a walker usually with ankle equinus and knee flexion, it is almost impossible to predict what that child will look like when they are 10 or 12 except with very great generalization. However, when that same child is walking independent with a mild crouch gait with midstance knee flexion approximately 20 , and is a full community functional ambulator at GMFCS II, one can predict with relative high degree of certainty that the risk for a long-term collapse into severe crouch gait will be very minimal. On the other hand, if a 10-year-old child has midstance phase knee flexion 40 , increasing planovalgus feet with external tibial torsion, and is complaining of decreased community walking endurance at GMFCS II, the prognosis is also relatively clear. Without aggressive treatment this 10-year-old may progressively lose ambulatory function as the crouch increases, knee pain develops from high stress, and he will develop the need for an assistive device or a wheelchair. Children with these two outcomes may look very similar when they are between 2 and 3 years of age. How can we understand that one develops a relatively functional and benign compensation for their CP and the other develops a rather malignant maladaptive response in the musculoskeletal system leading to loss of ambulatory ability? Some of the differences likely reside in how the central nervous system lesion has compensated. Also the impact of balance in addition to motor control also likely impacted by the child’s personality in their motivation and drive for early and significant amount of motor activity. The impact of early motor training and physical therapy is further undefined elements. As the child grows, muscle lengths relative to bone length may become more unequal. We do not

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understand why in some children with apparently the same amount of spasticity progressive contractures develop. Another important element is the increasing body size primarily weight which has a direct impact on the force and stress upon muscles and bones. The increasing force of weight is greater than the increasing strength of the muscle and bone. This discrepancy has a tendency to cause further deformation of bones, feet, and muscles. This interaction between weight and strength is especially important as a child who has mild to moderate planovalgus feet with mild gastrocsoleus contractures and hamstring contractures will have a rapid growth spurt which tends to increase the contractures at the gastrocsoleus and the hamstrings and places a great increase force across the planovalgus feet often with an external rotation moment driving increased external tibial torsion. As we consider the pathophysiology in an individual child, at an individual age, with a specific set of complaints, we need to consider and think about all of the multiple factors impacting the child at that time in their life. Because this is an extremely complex system, some conceptual simplification can be useful. One simplification approach is to consider what are primary deformities that can be corrected and separately identify compensatory deformities which may improve without correction or may have become fixed and also need to be corrected (Davids and Bagley 2014). This conceptualization approach is useful for addressing various correctable problems which may be identified in the child with crouch gait. Examples include muscle contractures, tendons that are too long, torsional malalignment, and foot postures. There is a risk with the above approach in overlooking the many issues which are not easily remedial, especially if there are no surgical options. These include the individual’s motor control, personal motivation, availability of rehabilitation services, balance, GMFCS level, walking velocity, and cardiovascular conditioning. Because of the wide variation in individual etiologies and interactions, it is very important to have a full quantitative analysis of crouch gait. The goal of this evaluation should

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focus on identifying all of the factors which seem to be important in this individual’s current gait posture and which may have an impact on any planned surgical corrections.

Treatment Appropriate treatment for crouched gait should focus on early detection and intervention before the problem becomes severe. Early detection means children should be followed closely, every 6 months during middle childhood. A full gait study is done preoperatively to assess the full deformity and should be obtained 1 year after the first surgery, which occurred between the ages of 5 and 7 years. Children’s weight should be monitored on every clinic visit, and as they start gaining weight fast and complaining of high stress pain at the knees or the feet, another gait study is indicated. Also, the physical examination should be monitored, especially the passive knee extension and popliteal angle, to monitor progressive hamstring contractures and fixed knee flexion contractures. If there is a significant increase in either of these, a gait study should be made as well. Any significant change in community ambulatory endurance should prompt a full evaluation. Ambulatory children should not be allowed to become dependent on wheelchairs for community ambulation (Case 2). This level of deterioration makes the recovery and rehabilitation exceedingly more difficult. The full evaluation of children with a significant increase in crouch or symptomatic loss of function from crouch should be carefully assessed to make sure all components of the crouched gait are found. All elements that are identified and are correctable should be corrected at the same time. The foot must be a stiff segment and be aligned within 20 of the forward line of movement and within 20 of right angle to the knee joint axis. This means if the foot has a significant planovalgus or a midfoot break, it must be corrected. A stable and correctly aligned foot is mandatory in the correction of crouch because the ground reaction

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force has to be controlled through the foot as a functional moment arm. Poor moment arm function of the foot causing the ground reaction force to be ineffective in producing knee extension is often one of the primary pathologies of a crouched gait pattern. The foot has to come to within neutral dorsiflexion in midstance so it can be placed in an orthosis, or the gastrocsoleus must provide the force needed to control the ground reaction force. If the gastrocnemius or soleus is contracted, it must be lengthened but only to neutral dorsiflexion at the end range. Never do uncontrolled, percutaneous tendon Achilles lengthenings in adolescent crouching individuals. These individuals will likely never be able to stand again without using a fixed AFO. Tibial torsion must be assessed next, and if it is contributing to the malalignment of the foot causing the foot to be out of line with the knee joint axis, a tibial derotation is required. Physical examination of passive range of motion of the knee should allow extension to within 10 of full extension. If the fixed knee flexion contracture is between 10 and 25 , a posterior knee capsulotomy is required (Taylor et al. 2016). Some have advocated using guided growth of the distal femur (Klatt and Stevens 2008); however, we have not found this to be reliable during rapid growth when the knee flexion contracture is increasing. If the fixed knee flexion contracture is greater than 20–30 , a distal femoral extension osteotomy is required (Stout et al. 2008; Das et al. 2012; Taylor et al. 2016). Distal hamstring lengthening is always indicated with crouched gait unless the procedure has been done in the preceding year or when an extension osteotomy is preformed (Healy et al. 2011). During the extension osteotomy, usually additional bone shortening is added which is another way to length the hamstring, and this also protects the sciatic nerve from stretch lesion (Taylor et al. 2016). The indication to do a hamstring lengthening is a popliteal angle of more than 50 with an initial contact knee flexion of more than 25 and knee flexion in midstance phase of more than 25 . Using muscle length assessment (origin to insertion length) has

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been advocated by some; however, the complexity of the system suggests that this approach is not very useful in predicting outcome such as improved knee flexion in stance (Laracca et al. 2014). There are many publications discussing the hamstring muscle origin to insertion length and lengthening or shortening velocity during gait (Schutte et al. 1997; Agarwal-Harding et al. 2010; Thompson et al. 2001); however, none of these consider modeling of the individual muscle fiber lengths and tendon lengths of the muscle. Therefore, we have no data where on the length tension curve an individual muscle is working and is the likely reason these whole muscle tendon lengths do not relate well to kinematic outcomes (Arnold et al. 2006). If individuals have decreased knee flexion in swing phase or late knee flexion in swing phase with toe drag, a rectus transfer or resection should be performed. Many clinicians are hesitant about doing rectus transfers in individuals with crouched posture; however, remember that the rectus is only 15% of the strength of the quadriceps and the muscle is not even active, except in pathologic cases in midstance phase. If children are very slow walkers, GMFCS III or IV, rectus transfer has less benefit. This discussion presumes GMFCS I–II–III, independent ambulators, or ambulators who use walking aids with good walking speed of over 80 cm/s, but do not use wheelchairs for community ambulation. This type of ambulator will gain much more from the rectus transfer than the risk of weakness (Gage 1991). If children require a distal femoral osteotomy to correct fixed knee flexion contracture, a shortening of the patellar ligament is usually required as well (Case 3). The next concern is the axis of the knee joint, which should be between 0 internal and 20 external at initial contact. If there is significant internal rotation, meaning more than 5–10 of internal rotation at initial contact, and the physical examination shows significantly more internal than external rotation of the hip, the femoral internal rotation should be corrected. Usually, this correction is made by doing a femoral derotational osteotomy, but if

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there is a question of the source of the internal rotation, a CT scan of the femur should be obtained to evaluate the source of the internal rotation. Last, the hip flexor may need lengthening if the hip flexion contracture is more than 20 and midstance phase hip extension is less than 30 . If there is more than 30 of anterior pelvic tilt but less hip flexion, hip flexor lengthening is also indicated, usually doing an intramuscular lengthening of the iliopsoas. The impact of psoas lengthening to improve hip flexion contractures or anterior pelvic tilt has demonstrated relatively modest impacts (Mallet et al. 2016; Schwartz et al. 2013; Morais Filho et al. 2006). In our experience those children who walk without aids GMFCS I and II have potentially the most benefit without problem of related hip flexor weakness (Morais Filho et al. 2006). Although it is reported that release of the muscle from the lessor trochanter is safe and does not lead to weakness (Bialik et al. 2009), we have patients who complain of difficulty with high steps or stepping into a bathtub even after intramuscular releases. While assessing the crouched pattern, it is important to assess each lower extremity independently, as the surgery will often need to be asymmetric. Correction of the torsional malalignments is extremely important for the correct mechanical function of the lower extremity, especially when there is decreased motor control. In summary of doing the gait assessment and developing a treatment plan, it is important to also consider the child and family goal, postoperative rehabilitation plan, the child’s motivation, the family’s ability to follow through, as well as the specific gait pathology. The quantitative assessment should then start by combining the physical examination, kinematic, kinetic, and pedobarograph data. It is important to assess the right and left side separately. Consideration should include in the evaluation of the long-term goal which will need to consider is the child’s current function and walking velocity as well as their current community function. Although the most apparent aspect of the crouch gait is the high knee flexion in stance phase, very careful attention has

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to be paid to the posture of the feet, the torsional alignment of the tibia and femur, muscle lengths, especially hamstring, gastrocnemius length, and position of the patella relative to patellar tendon length. Any areas of pain such as the distal pole of the patella, the tibial tubercle, or the feet need to be carefully evaluated with physical examination an x-ray.

Performing the Crouched Gait Surgery The surgery for crouched gait often involves many procedures at different joints. This is the classic example of the child who most benefits from single-event multilevel surgery (SEMLS). After a thorough diagnostic evaluation and making a specific surgical plan, one has to develop an operation plan. Since this often involves bilateral surgery at multiple joints, it is an ideal situation to have two surgical teams, one working on either side. This reduces the anesthesia time and decreases the fatigue time of the surgical team. The preferred order is to start at the hip and correct the hip rotation, with iliopsoas lengthening if needed. Then the knee is addressed by hamstring lengthening followed by knee capsulotomy or femoral extension if indicated. The foot deformity is corrected next, followed by a careful intraoperative assessment of the torsional alignment to make the final determination of the need for a tibial osteotomy. After the tibial osteotomy, another intraoperative assessment should be made to show that the hip fully extends and the knee can be fully extended and lies in approximately 10 of external rotation. The footto-thigh alignment should be 20 external to neutral with neutral dorsiflexion. Postoperative rehabilitation should start in the hospital with the goal of having children at least standing before discharge and plan for immediate home rehabilitation. For some children when it is available, a course of inpatient rehabilitation may greatly facilitate the acute rehabilitation process. Parents need to expect that the acute rehabilitation will take 3 months until these individuals are close to

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their preoperative function, and then it will take at least 1 year of rehabilitation to reach maximum function, and almost all children continue to improve in the second year postoperative. If there is weakness or a tendency for the gastrocsoleus not to have good strength, a ground reaction AFO has to be used postoperatively. This is the ideal time to use the anterior articulated ground reaction AFO, which will allow the gastrocsoleus to gain strength, and over 1–2 years, the orthotic can be weaned away and the correction will be maintained. The outcome of surgery for crouched gait is excellent if there is a complete diagnosis, correction of all deformities, and follow-through with good rehabilitation. If the surgery is done at adolescence near the end of growth or when individuals are well into adolescence, the correction will be permanent, and no additional procedures will be needed.

Spasticity Reduction in Adolescents and Young Adults Some adolescents have very limited motion because of severe spasticity but are nevertheless good ambulators. Many of these teenagers also have crouch gait pattern. The use of intrathecal baclofen is a reasonable option; however, it often unmasks weakness when the spasticity is reduced. There have been no objective reports on the effects of intrathecal baclofen on gait in this age and especially in the face of crouch gait. In our personal experience with intrathecal baclofen for this age, individuals will have a mild increased crouch and may slow their gait slightly. The patients, however, report feeling more comfortable and find dressing and other activities of daily living easier. We have however had three patients in whom the weakness was so disabling that they wish to have the intrathecal baclofen discontinued. This highlights one of the major benefits of intrathecal baclofen in situations where it is not clear whether it will be beneficial, it is completely reversible with no long-term consequence. Dorsal

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rhizotomy is not indicated in this group, as the risks far outweigh any benefits that could be expected, and since it is not reversible, there is a real risk of making individuals worse.

Complications Complications of crouch gait include those which are due to errors in judgment related to diagnostic decisions and to surgical execution. A common problem in individuals with crouch gait is medical neglect in which the crouch gait is allowed to become so severe that the child no longer can or wants to walk because of discomfort. Once an adolescent enters a wheelchair and uses it for most of their daily ambulation, it is extremely difficult and seldom successful to do aggressive treatment with the goal of getting them back into full-time ambulation. Part of this is related to the motivation of the individual, but probably, more is related to the development of cardiovascular deconditioning and progressive muscle weakness which is very difficult to overcome. Therefore, it is very important to have careful monitoring by individuals with expertise in understanding progressive loss of function and the importance of reacting in an appropriate timely manner. Any major changes in assistive devices such as going from crutches to walker or walker to wheelchair should clearly be a sign that a diligent work up for mechanical problems of gait should be undertaken. Complications related to the diagnostic process include not doing an adequate full objective evaluation. For complex children with crouch gait, this should almost always include a full three-dimensional diagnostic gait analysis except in rare circumstances when it’s absolutely not available. The most common tendency is to miss important aspects of the gait pathology. Examples of this under diagnosis is failing to recognize the importance of planovalgus feet in a crouch gait pattern or failing to recognize external tibial torsion as a significant component of the pathology. This same tendency extends

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into the operating room where the surgeon may choose to not do a procedure because under anesthesia the deformity does seem to be so severe. It is important to utilize judgment intraoperatively under anesthesia; however, the surgeon needs to be aware of the tendency to fail to do a procedure, which was clearly indicated by gait analysis, which will almost always still be present postoperatively. This is especially true of planovalgus feet and external tibial torsion. The most common major postoperative complication is related to not having adequate physical therapy especially discontinuing the physical therapy too early because the expected recovery after a large surgery of this magnitude should be 1 year not 30 days or 90 days as the insurance company may wish. Other complications which may occur as a consequence of surgical correction of crouch gait especially the fixed knee flexion deformity component is stretch of the sciatic nerve. The sciatic nerve palsy seems to be most common when there has not been significant enough shortening of the bone, and the knee is held in full extension immediately postoperatively without motion. Our current standard is to place the knee in 30 of flexion postoperatively and then start on the second day with passive motion from minus 30 to full extension when the patient is awake. Our goal is to have the knee in full extension for nighttime sleeping by 2 weeks postoperatively if the patient is comfortable. The same protocol is followed for posterior knee capsulotomy and for release of severe hamstring contractures. If sciatic nerve palsy does develop, it is important to maintain the knee in minus 30–40 of extension until, and with very slow passive stretching that’s pain-free, the knee can be brought to full extension. In rare cases following knee extension osteotomy, it may be beneficial to return the child to the operating room to perform further bone shortening. The goal with these patients is not to do further injury to the sciatic nerve by stretching it but also not to lose full knee

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Fig. C1.1

extension which was the goal of the initial procedure.

Cases

Case 1 Elizabeth

Elizabeth, a 14-year-old girl, presented with the concern that her walking had become so difficult that she could no longer walk around her junior high school. According to her parents, she did not even own a wheelchair when she was in grade school, as she was able to walk

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everywhere using a walker. They were concerned that she would completely lose her ability to walk. She had no previous surgeries and currently received no physical therapy. She had grown rapidly in the past 2 years, and in the past year, as she had spent more time in the wheelchair, she had gained a lot of weight. A physical examination demonstrated hip abduction to 20 , almost symmetric hip rotation with 40 internal and 30 external rotation; popliteal angles were 70 , the knees had 10 fixed knee flexion contractures, and the feet had severely fixed planovalgus deformities. The kinematics showed high knee flexion at foot contact and decreased knee flexion in swing phase, with a severely reduced knee range of motion (Fig. C1.1). The pedobarograph showed severe planovalgus with external foot progression of 34 on the right and 19 on the left (Fig. C1.2). Most weight bearing was in the medial midfoot (Fig. C1.3). The main cause of the loss of ambulation appeared to be the crouch gait caused primarily by severe and progressive planovalgus foot deformities, which prevented the foot from functioning as a rigid moment arm, with the majority of the weight bearing on the medial midfoot (Fig. C1.3). This lever arm disease needed to be corrected by stabilizing the foot so it was a stiff and stable structure, and it had to be aligned with the axis of the knee joint. Correction of the planovalgus with a triple arthrodesis both stabilized the foot and corrected the malalignment. Hamstrings were lengthened, and after a 1-year rehabilitation period, she was again doing most of her ambulation as a community ambulator using crutches. The foot pressure showed a dramatic improvement although there was still more weight (continued)

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Fig. C1.3

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Fig. C1.4

Fig. C1.5

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Fig. C1.6

Fig. C3.1

bearing on the medial forefoot than the lateral forefoot, indicating some mild residual valgus (Fig. C1.4). There is also

increased weight bearing on the heel, indicating continued weakness in the (continued)

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Fig. C3.3

Fig. C3.2

gastrocsoleus (Figs. C1.4 and C1.5). The kinematics demonstrate a good improvement in knee extension and ankle plantar flexion (Fig. C1.6). Elizabeth would have become a permanent wheelchair user if her feet had not been corrected.

Case 2

Case 7.7 brought forward.

Case 3 Brandon

Brandon, a 3-year-old boy, started to walk using a walker while in physical therapy. He did well walking in his school environment; however, his mother reported that he refused to use the walker at home. During the next several years, his grandmother cared for him; then, at age 7 years, he

again returned to his mother and his initial school. He had developed significant knee flexion deformities that made walking difficult; however, he moved freely on the floor in reciprocating quadruped crawl. A popliteal angle of 90 and 30 knee flexion contractures were found on physical examination. He had knee capsulotomies and hamstring lengthening bilaterally; however, the stress of the surgery and a breakdown in the social service system led to very little physical therapy. By the time he returned to school 4 months later, and the school got him back to the clinic, his knee flexion contractures were slightly worse than preoperatively. Over the next several years, he was in school but received only sporadic therapy. At age 10 years, his mother was very concerned because he crawled everywhere, but he was getting bigger and he refused to stand on his feet. In the classroom and at home he did a lot of knee walking and had several episodes of severe knee bursitis, which required his mother to try to keep him off his knees. His mother’s main concern was that soon she could not care for him if she had to carry him everywhere. At this time, he was in a (continued)

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F. Miller Fig. C3.6

Fig. C3.4

Fig. C3.5

self-contained special needs classroom with a teacher’s aide. He had moderate mental retardation, functioning at the 3-year-old level. On physical examination he had a

popliteal angle of 100 and fixed knee flexion contractures of 60 bilaterally (Fig. C3.1) but excellent knee flexion (Fig. C3.2). He had a large callus on the anterior knee, demonstrating that he did a lot of knee walking (Fig. C3.3). His hip motion and hip radiographs were normal, and his feet were in plantigrade and without deformity. Knee radiographs showed no abnormalities (Fig. C3.4). Observation of his movement on the floor showed that he was very proficient as a reciprocal quadruped crawler and a very functional independent knee walker. Based on the assessment that he had excellent balance with good motor control and motor planning skills, he had hamstring lengthening, distal femoral extension osteotomy, patellar tendon plication, and transfer of the rectus to the sartorius (Figs. C3.5 and C3.6). After the osteotomy healed (Fig. C3.7) and after a 1-year rehabilitation period, he was able to walk in the school and home using a posterior walker with full knee extension. (continued)

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Fig. C3.7

Limited knee flexion prevented proficient crawling or knee walking, which drove him toward walking with the walker. In the second year after this procedure, Brandon developed scoliosis, which required a spinal fusion, and that required another year of rehabilitation. It is expected that he will continue to make more gains in his walking ability over the next several years as his motivation to walk improves. The mental retardation is a significant factor in the speed of the rehabilitation but probably not in the final outcome.

Cross-References ▶ Diplegic Gait Pattern in Children with Cerebral Palsy ▶ Gait Analysis Interpretation in Cerebral Palsy Gait: Developing a Treatment Plan ▶ Knee Deformities Impact on Cerebral Palsy Gait

References Agarwal-Harding KJ, Schwartz MH, Delp SL (2010) Variation of hamstrings lengths and velocities with walking speed. J Biomech 43:1522–1526

Arnold AS, Liu MQ, Schwartz MH, Ounpuu S, Dias LS, Delp SL (2006) Do the hamstrings operate at increased muscle-tendon lengths and velocities after surgical lengthening? J Biomech 39:1498–1506 Bialik GM, Pierce R, Dorociak R, Lee TS, Aiona MD, Sussman MD (2009) Iliopsoas tenotomy at the lesser trochanter versus at the pelvic brim in ambulatory children with cerebral palsy. J Pediatr Orthop 29:251–255 Das SP, Pradhan S, Ganesh S, Sahu PK, Mohanty RN, Das SK (2012) Supracondylar femoral extension osteotomy and patellar tendon advancement in the management of persistent crouch gait in cerebral palsy. Indian J Orthop 46:221–228 Davids JR, Bagley AM (2014) Identification of common gait disruption patterns in children with cerebral palsy. J Am Acad Orthop Surg 22:782–790 Elhassan Y, Mahon J, Kiernan D, Brien TO (2013a) A greenstick fracture of the patella: a unique fracture in CP crouch gait. BMJ Case Rep. https://doi.org/ 10.1136/bcr-2013-009717 Elhassan Y, O’Sullivan R, Walsh M, Brien TO (2013b) Knee extensor disruption in mild diplegic cerebral palsy: a risk for adolescent athletes. BMJ Case Rep 2013 Gage J (1991) Gait analysis in cerebral palsy. Mac Keith Press, London Healy MT, Schwartz MH, Stout JL, Gage JR, Novacheck TF (2011) Is simultaneous hamstring lengthening necessary when performing distal femoral extension osteotomy and patellar tendon advancement? Gait Posture 33:1–5 Klatt J, Stevens PM (2008) Guided growth for fixed knee flexion deformity. J Pediatr Orthop 28:626–631 Laracca E, Stewart C, Postans N, Roberts A (2014) The effects of surgical lengthening of hamstring muscles in children with cerebral palsy – the consequences of pre-operative muscle length measurement. Gait Posture 39:847–851

1504 Mallet C, Simon AL, Ilharreborde B, Presedo A, Mazda K, Pennecot GF (2016) Intramuscular psoas lengthening during single-event multi-level surgery fails to improve hip dynamics in children with spastic diplegia. Clinical and kinematic outcomes in the short- and mediumterms. Orthop Traumatol Surg Res 102:501–506 Morais Filho MC, de Godoy W, Santos CA (2006) Effects of intramuscular psoas lengthening on pelvic and hip motion in patients with spastic diparetic cerebral palsy. J Pediatr Orthop 26:260–264 Rethlefsen SA, Nguyen DT, Wren TA, Milewski MD, Kay RM (2015) Knee pain and patellofemoral symptoms in patients with cerebral palsy. J Pediatr Orthop 35:519–522 Rodda JM, Graham HK, Nattrass GR, Galea MP, Baker R, Wolfe R (2006) Correction of severe crouch gait in patients with spastic diplegia with use of multilevel orthopaedic surgery. J Bone Joint Surg Am 88:2653–2664 Schutte LM, Hayden SW, Gage JR (1997) Lengths of hamstrings and psoas muscles during crouch gait: effects of femoral anteversion. J Orthop Res 15:615–621

F. Miller Schwartz MH, Rozumalski A, Truong W, Novacheck TF (2013) Predicting the outcome of intramuscular psoas lengthening in children with cerebral palsy using preoperative gait data and the random forest algorithm. Gait Posture 37:473–479 Stout JL, Gage JR, Schwartz MH, Novacheck TF (2008) Distal femoral extension osteotomy and patellar tendon advancement to treat persistent crouch gait in cerebral palsy. J Bone Joint Surg Am 90:2470–2484 Taylor D, Connor J, Church C, Lennon N, Henley J, Niiler T, Miller F (2016) The effectiveness of posterior knee capsulotomies and knee extension osteotomies in crouched gait in children with cerebral palsy. J Pediatr Orthop B 25:543–550 Thompson NS, Baker RJ, Cosgrove AP, Saunders JL, Taylor TC (2001) Relevance of the popliteal angle to hamstring length in cerebral palsy crouch gait. J Pediatr Orthop 21:383–387 Winters TF Jr, Gage JR, Hicks R (1987) Gait patterns in spastic hemiplegia in children and young adults. J Bone Joint Surg Am 69:437–441

Knee Deformities Impact on Cerebral Palsy Gait

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1505 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Knee Position at Weight Acceptance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Midstance Knee . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Terminal Stance Knee Position . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Early Swing Phase . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Terminal Swing Phase . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Treatment Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1506 1506 1506 1508 1509 1509 1511

Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1512 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1514 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1514

Keywords

Cerebral palsy · Knee flexion · Toe drag · Knee flexion contracture · Back-kneeing · Stiff knee

Introduction The knee is the largest joint in the lower extremity; however, in children with cerebral palsy (CP), it generally has fewer problems then either the foot or the hip. The primary function of the knee is to allow limb length adjustment so the foot can clear the floor during swing phase and to provide

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_105

stability in stance phase. At initial contact, the knee should have slight flexion, so it can participate with the ankle in absorbing the shock of weight transfer. If the knee is completely extended, it is difficult to develop smooth movement into flexion and, therefore, will not provide good shock absorption. These three main functions of the knee joint are controlled by muscles primarily the hamstrings for knee flexion, the quadriceps for knee extension, and the gastrocsoleus to modulate knee flexion in stance phase. Most of these muscles cross at least two joints and therefore have very complex control requirements. Due to these complex control requirements, there is often a problem in children with CP who have motor control and balance issues to properly control the time and magnitude of the muscles controlling knee motion. The goal 1505

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of this chapter is to review in detail the periods in the gait cycle where these control issues are especially problematic relative to the knee motion.

Natural History and Pathophysiology Knee Position at Weight Acceptance At the time of foot contact, the knee should be in mild flexion and then continue into flexion as the weight is transferred from the contralateral limb. Abnormally increased knee flexion at foot contact is common. This increased flexion helps shock absorption; however, this is often associated with plantar flexion and toe strike, which places an immediate strong external extension moment on the knee that the hamstrings have to resist. During weight acceptance, there tend to be two patterns of knee motion; one is immediate extension from initial contact position and the other is increased knee flexion, which may occur because of eccentric gastrocsoleus contraction, weak gastrocnemius, or a poor moment arm of the foot. The amount of knee flexion during weight acceptance should be 10–20 if it is normally controlled by the gastrocnemius and soleus eccentric contraction. If the degree of knee flexion is more than 20 , it is likely due to weakness of the gastrocsoleus or an insufficient moment arm at the foot. Gastrocnemius recession will decrease forefoot strike and as a consequence will reduce knee flexion at foot contact during loading phase (Baddar et al. 2002). Since the degree of knee flexion in swing is modulated mainly by the hamstrings, and in children with CP, full knee extension at initial contact usually is the result of overlengthening of the hamstrings. Full knee extension at initial contact is also seen in children with hypotonia and ataxia.

Midstance Knee As the gait cycle proceeds to midstance, if there was knee flexion during weight acceptance, knee extension should now begin. If the knee flexion

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continues into midstance, then a crouched gait pattern is present (Case 1). The primary causes of increased knee flexion in midstance are knee flexion contractures, hamstring contractures, a deficient foot moment arm, and gastrocsoleus weakness (Fig. 1). A secondary etiology may be a significant hip flexion contracture, which can limit knee extension in midstance. Often, there are several causes of increased knee flexion in midstance, and all primary and secondary causes should be identified. This identification involves considering the actual magnitude of the flexion by evaluating the knee extension in midstance on the kinematic evaluation, the ankle moment in midstance, and the knee moment in midstance. If the ankle moment is normal or below normal, and the knee flexion is not increased, then the ankle weakness and foot moment arm are the most likely causes. If the kinematics show the knee extending to the limits of the fixed knee flexion contracture measured on physical examination, then the knee joint contracture is a likely cause. If the ankle has a high plantar flexion moment and the knee has a high flexion moment, it is likely a combination of contracture of the gastrocnemius and the hamstrings. If the hip extension peak occurs early, is decreased, and the physical examination shows a significant hip flexion contracture, then hip flexion contracture may also be contributing to the midstance phase knee flexion deformity. If children use ambulatory aids such as crutches and the hamstring muscles are not really contracted, there is a tendency for them to fall into back-kneeing, both when the gastrocsoleus is overactive and when it is too weak. If children are independent ambulators or have overactive hamstrings, they will be strongly drawn to a crouched gait pattern. If children are very strong and have high tone, they will be drawn to keep the knees stiff and vault in midstance phase. This vault action raises the body and increases the energy cost of walking; however, it has the benefit of allowing the contralateral leg to clear the floor during swing. Also, by raising the body in midstance, the body can then fall forward in terminal stance, so forward momentum can be used at initial contact, and the

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Fig. 1 The hamstrings effect on knee flexion in stance or crouched gait results from the hamstrings muscle ability to generate the same magnitude of force at three different points on the length-tension curve based on the level of contracture. At normal fiber length, the muscle still has the ability to generate more force with increased contraction. With a moderate contracture, there is decreased force generation as the muscle further lengthens, and, with a severe contracture, there is rapid increase in force due to passive increase in tension from the connective tissue (a). In addition to the impact of the contracture on the hamstrings force-generating ability, the ability to generate joint moment depends on the position of the hip and knee

joint. The hamstrings may be at the same length and generate the same force; however, if the hip and knee are flexed, as in a crouched gait, there is a large moment arm at the knee generating much more knee flexion force than when the knee is near full extension (b). Therefore, the end-to-end length of the muscle in crouch may be the same as in upright stance, but this does not mean the hamstring contracture is not a problem. One must consider the contracture effect on the length-tension relationship, and as this drives the knee into flexion, the crouch is a selfpropagating position because more knee flexion increases the hamstrings mechanical advantage through an increasing knee moment arm

contralateral limb can use the gluteus to lift the body back up again (Fig. 2). The back-kneeing position (genu recurvatum) in midstance phase is an especially difficult problem to address. This position has been shown to follow three patterns, with one pattern having predominantly overactive gastrocsoleus muscles (Klotz et al. 2016; Svehlik et al. 2010), the second having the HAT (Head Arms Trunk) segment center of gravity move anterior to the knee often in the face of a weak gastrocnemius, and the third pattern having the HAT center of gravity moving posterior to the hip but anterior to the knee (Simon et al. 1978). Overlengthening of the hamstrings especially in the face of a spastic or short gastrocnemius is another cause of back-kneeing (Zwick et al. 2010). Treatment for all back-kneeing is to

make sure the gastrocnemius has enough length to allow dorsiflexion with knee extension and to be careful not to over lengthen the hamstrings. If dorsiflexion with knee extension is possible, children should be placed in an orthosis that allows 3–5 of dorsiflexion while limiting plantar flexion with a stop at 5 dorsiflexion. This orthosis can usually be an articulated AFO. If there is a pattern in which the ground reaction force is moving either significantly in front or behind the knee in the face of a weak gastrocsoleus, a solid ankle AFO should be used to assist the gastrocsoleus in ankle control. Back-kneeing that is especially difficult to control is present in children who use walkers or crutches, because the center of mass of the HAT segment can be so far forward that even in the AFOs, the toes of the shoes and AFOs will

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Fig. 2 The gluteus maximus, primarily and along with the other hip extensors, are the secondary muscles generating forward motion. This function is accomplished by the muscle having a strong contraction at foot contact and early stance, in which the forward falling HAT segment and center of mass are decelerated and lifted. The strong contraction between momentum of the

forward falling body and the fixed foot uses the lifting of the body by a concentric contraction. When the gastrocsoleus is inactivated by an equinus contracture or by the use of very high-heeled shoes, the hip extensors become the primary power output muscles generating power for walking

just rise with all the weight being borne on the heel. This persistent back-kneeing in spite of appropriate orthotics in children with assistive devices may cause progressive back-kneeing because of increasing knee hyperextension and the development of pain. The only treatment for this kind of progressive back-kneeing is through the use of a knee-ankle-foot-orthosis (KAFO) with extension blocking hinges at the knee.

for swing through. If flexion is delayed or decreased, it may be due to a lack of push-off power burst from the ankle, a lack of hip flexor power, too much contraction of the rectus, or co-contraction between the hamstrings and the vastus muscles. This is also the phase in the gait cycle; if the knee is in high flexion more than 30 or 40 , there is very little room for increasing knee flexion velocity. This crouch gait posture is often a primary cause of the lack of flexion in swing phase. This is also the phase in the gait cycle where high flexion of more than 30 or 40 leaves very little room for increasing knee flexion velocity at toe off. The main approach to improve function in late stance phase is to maximize function at the ankle so it can provide maximum power at push-off. It is

Terminal Stance Knee Position As the gait cycle progresses to terminal stance, the knee should start to flex as part of the process to accommodate the plantar flexion from the ankle joint and to start the process of shortening the limb

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also important to have adequate hip flexor power and have the hip a position where the flexor power can be applied. The risk is to have the hip hyper flexed at this stage where it is limited in providing additional flexor power input.

Early Swing Phase As the knee joint moves to early swing phase, the peak flexion should be occurring in initial swing in the first 20–30% of swing phase. The stiff knee gait syndrome may be present if there is a decreased magnitude of knee flexion, meaning less than 55–65 of peak flexion, or the flexion occurs late in midswing phase. This syndrome is the principal cause of toe drag. An important element in the diagnoses of the etiology of reduced knee flexion is due to the late stance phase conditions, which were noted above. These initial conditions include inadequate power input, excessive rectus activity, and inadequate knee extension in stance (Goldberg et al. 2003). The most common primary cause of stiff knee gait syndrome in children with CP is a rectus muscle that is contracting out of phase, too early, or with too much force. Secondary causes of decreased knee flexion in swing phase are the low push-off power bursts from the gastrocsoleus, decreased hip flexor power, and a knee joint axis that is rotated out of line with the forward line of progression. A fixed knee extension contracture may also cause limited knee flexion. In individual patients, any of the secondary etiologies may be the primary cause. To diagnose the overactive rectus as the primary cause requires an EMG of the rectus, which is active for a prolonged period in late stance and swing phase, the time of maximum swing phase knee flexion is late, and the magnitude of maximum swing phase knee flexion is decreased. Additional data to reinforce the rectus muscle as the cause of the stiff knee are provided by the physical examination showing a contracted rectus muscle with a positive Ely test and a rectus that is spastic. A poor push-off power burst at the ankle and little or no hip flexion power

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generation at toe off suggest that some of the problem is coming from these sources. When the stiff knee syndrome is due to an overactive rectus, the required treatment is to remove the rectus from its insertion on the patella (Case 2). This removal requires transferring the rectus to some other muscle, with the sartorius and the gracilis being the most common sites. The specific site of the transfer does not matter (Ounpuu et al. 1993); however, it is better to be transferred and not only released from the quadriceps tendon (Ounpuu et al. 1993). More recent reports suggest a significant resection of the tendon may be equal to a transfer (Presedo et al. 2012). If the tendon is released only, it will probably reattach to the underlying tendon and go back to doing its old job again. The primary goal of this transfer is to remove the action of the rectus from knee extension but preserve its function as a hip flexor. Usually, the contraction pattern is appropriate for hip flexion, and if it is to have an effect on the knee, it should work as a knee flexor. Good results with increased knee flexion in swing phase and an earlier peak knee flexion have been well documented by several studies (Ounpuu et al. 1993). The distal transfer is better than the proximal release (McCarthy et al. 1988) and works best when there is good walking velocity and swing phase EMG activity of the rectus but not constantly on rectus EMG activity (Miller et al. 1997).

Terminal Swing Phase During terminal swing phase, the knee should be extending in preparation for initial contact. This extension is controlled by eccentric contraction of the hamstring muscles. The impact of the hamstring insufficiency to allow the knee to fully extend has already been noted. In cerebral palsy gait, a much more common problem is overactivity of the hamstrings with early initiation on the EMG. Often, the primary problem is a contracture of the hamstrings and overactivity of the hamstrings muscle; however, the secondary cause is

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decreased momentum from slow hip flexion. This increased knee flexion at the end of swing phase causes short step lengths (Fig. 3). Treatment of diminished knee extension in terminal swing phase is primarily directed at the hamstrings, where surgical lengthening is the main treatment option. The function of the hamstrings is extremely complex, and the benefit of hamstring lengthening to improving knee extension at initial contact is less consistent (Thometz et al. 1989). Most reports showing positive results of hamstring lengthening come from the pre-gait analysis literature and have no dynamic data; however, they suggest that the popliteal angle remains improved after 2–4 years (Atar et al. 1993; Damron et al. 1991). There are reports showing improvement in stance knee extension, loss of knee flexion in swing phase, and mild increased lumbar lordosis after hamstring lengthening (Hsu and Li 1990). There have been many modeling studies showing that the hamstring length is often not significantly shortened when measured from origin to insertion in the crouched gait midstance posture (Delp et al. 1996; Hoffinger et al. 1993). These findings fail to consider that these patients also have greatly decreased muscle fiber length as demonstrated

Fig. 3 An important function of the knee is to develop extension at foot contact. Lack of knee extension at foot contact can be a significant a cause of short step lengths

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by high popliteal angles. These modeling originto-insertion measurements miss the significant impact of the change of muscle power based on where the muscle length falls on the lengthtension curve and the impact of the change of the moment arm based on joint position (see Fig. 1). With the knee flexed 60 , the moment arm for knee flexion by the hamstrings is much greater than when the knee is extended. This same change in moment arm also occurs at the hip; however, the length the moment arm change is less significant at the hip. There are also three separate muscles, the semimembranosus, semitendinosus, and long head of the biceps, which make up the primary hamstrings, and each of these muscles has a different fiber length but very similar origin and insertion sites. As all the variables involved with hamstring contraction are added to the force generated, which depends on the velocity of the contraction, the complexity of the control of the force impact on the hip and knee from the hamstrings is demonstrated. These variables include three muscles, each with different fiber lengths, approximately 1500 motor units in each muscle, and variable moment arms at two points for each muscle. With this great level of complexity, it is easy to see why

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hamstring muscles are often poorly controlled in children with motor control problems. This complexity can also explain why the outcome of lengthening is not very predictable. However, based on clinical experience, severely short hamstrings do not work well even if the simplistic modeling suggests that the origin-to-insertion length of the hamstrings in the midstance part of the gait cycle is long enough.

Treatment Summary The major problems related to the knee in gait problems of the child with cerebral palsy are very dependent upon the phase of the gait cycle (Table 1). At foot contact and weight acceptance, the primary problem is lack of full knee extension with only occasionally a child who has too much extension at foot contact. This may be related to forefoot striking and ankle equinus or insufficient hamstring strength or control. Treatment is primarily focused on addressing foot position and evaluating hamstrings muscle lengthen and motor control. In midstance the problem is often related to lever arm dysfunction due to poor foot function or fixed knee flexion contractures. It may also be related to overactivity of hamstrings or contracture of hamstrings. Treatment for midstance problems requires attention to correction of lever arm dysfunction, making sure there is adequate passive knee extension and addressing over activity of the hamstrings. Terminal stance problems are related to an adequate knee extension and inadequate power input from the ankle and hip flexors. This does not allow knee flexion velocity to develop at toe off for adequate knee swing. Peak knee flexion should occur in early swing phase. The causes of lack of peak knee flexion or delayed peak knee flexion include the initial conditions occurring in late stance phase which are lack of hip flexor power, lack of power input from the ankle, and overactivity of the rectus femoris muscle. Treatment should include identifying the most likely etiology and then

1511 Table 1 Segment and joint compensations Problem Knee Increased flexion at foot contact

Decreased knee flexion at foot contact Lack of weight acceptance knee flexion Genu recurvatum decreased midstance flexion (back-knee) Increased midstance flexion (crouch)

Lack knee flexion swing (stiff knee gait)

As the primary etiology

Compensatory effect for

Knee flexion contracture Premature hamstring activity Hamstring contracture Weak hamstrings

Toe strike due to ankle equinus Weak ankle pushoff Hip flexor weakness

Knee stiffness

Ankle plantar flexor contractures

Contracture or overactivity of gastrocsoleus Hamstring weakness

Poor motor control Hamstrings that are too weak compared with the gastrocsoleus Weak gastrocsoleus Lack of plantar flexion (hyper dorsiflexion) Balance problems Severe abnormal foot progression angle Hip flexion contracture Ankle equinus Poor push-off power from the gastrocsoleus Poor hip flexor power Low gait velocity

Knee joint contracture Hamstring contracture Lever arm disease (planovalgus feet)

Overactivity of the rectus muscle Knee stiffness or contracture Quadriceps contracture

Quadriceps weakness Hypotonia

addressing this concern. Late swing phase is primarily controlled at the knee by the hamstrings if there is adequate momentum causing the forward swing of the shank. Over activity of the hamstring muscle will cause an increased knee flexion at foot contact and weakness or inactivity of the hamstring will cause hyperextension of the knee at foot contact at foot contact. Treatment in this phase is directed at having

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adequate length of the hamstrings and being careful not to over lengthen or weaken the hamstrings (Fig. 1).

Cases

Case 1 Michael

Michael, a 5-year-old boy, was evaluated 1 year after he walked independently without the use of his walker. His parents complained that he fell a lot and had trouble stopping without falling at the end of a walk. Michael appeared to be ageappropriate cognitively and had significant spasticity in the lower extremities. He also had some increased tone in the upper extremities and poor hand coordination. His gait demonstrated toe walking with mild knee flexion in stance phase and significant internal rotation of the hips. After a full evaluation, he underwent a reconstruction with bilateral femoral derotation osteotomies, distal hamstring lengthening, and gastrocnemius lengthening. In his rehabilitation, gait training focused on ambulation with crutches, which he learned to manage well. By age 10 years, he was in a regular school and walked with Lofstrand crutches (Fig. C1.1). He then fell and sustained a femur fracture, which was treated in his community hospital by placing him in a hip spica cast for 3 months. Following this, he could barely walk short, in-home distances with a walker (Fig. C1.1). Shortly before the fracture accident, his parents went through an acrimonious divorce. Following removal from the cast, he was placed in a wheelchair, and there was little or no effort to try to rehabilitate him. Over the next 3 years, his father, who was very enthused about the boy’s ambulatory ability, successfully petitioned the court to get custody from the mother,

Fig. C1.1

Fig. C1.2

who felt ambulation was hopeless. This change in homes greatly lifted the boy’s spirits, and in spite of not being able to stand to transfer himself by age 14 years, (continued)

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Fig. C1.4 Fig. C1.3

he was enthused about trying to get back to walking. By this time he had severe crouch stance posture, severe planovalgus feet, knee flexion contractures, and hamstring contractures (Figs. C1.2 and C1.3). At this time, Michael was doing well academically in a regular school. He underwent bilateral planovalgus correction with triple arthrodesis (Fig. C1.4), gastrocnemius lengthening, posterior knee capsulotomies, and hamstring lengthening. By 6 months postoperatively, he could again walk in the house for short distances using a walker and ground reaction AFOs. By 9 months postoperatively, he made further progress with increased walking endurance, and by 2 years after surgery, he was again doing community ambulation and had worked back toward crutch use. The problems that caused Michael to stop walking were all reversible, including social home environment, his depression and lack of motivation, and the physical deformities. The key to having clinical confidence in getting him out of the wheelchair was having

documentation in the videos or other gait analysis of his prior walking ability, and then making sure that all the factors were addressed before the physical deformities were corrected. The success of getting Michael walking again was probably as much a result of the change in home environment as it was the medical care.

Case 2 Josie

Josie, a 16-year-old girl, presented with the complaint of frequent tripping and wearing out the front of her shoes very quickly. She has never had surgery, attends high school where she is an average student, and desires treatment for her complaints. On physical examination she had good hip motion and full knee range of motion with popliteal angles of 45 bilaterally. An Ely test was positive at 60 ; the rectus had 1+ spasticity on the Ashworth scale. Ankle dorsiflexion with the knee extended was 5 . Kinematics showed knee extension in stance to the (continued)

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Right Flexion

Left

80.0

80.0

60.0 40.0

60.0 40.0

20.0

20.0

0.0 Extension –20.0

–20.0

Flex/Extension

0.0

Preoperative

Postoperative 1 year

Fig. C2.1

normal range but only 35 peak flexion in swing phase. The ankle kinematic showed early ankle plantar flexion. The ankle moment had a significant early plantar flexion moment. The ankle power showed a midstance generation burst indicating a significant vault. An EMG of the rectus showed constant swing phase rectus activity, but no significant stance activity. Bilateral rectus transfers were performed, and she had significant increase in swing phase knee flexion immediately after surgery (Fig. C2.1). This improvement was maintained 3 years later, along with excellent improvement in symptoms. She now reports much less tripping and never wears out the toes of her shoes. Although patients with isolated stiff knee gait are rare, this demonstrates the excellent benefit of rectus transfer when the indications are correct. Often, the cause of swing phase knee stiffness is not so isolated but also includes poor hip flexor power and poor ankle push-off.

Cross-References ▶ Foot Deformities Impact on Cerebral Palsy Gait ▶ Hip and Pelvic Kinematic Pathology in Cerebral Palsy Gait

References Atar D, Zilberberg L, Votemberg M, Norsy M, Galil A (1993) Effect of distal hamstring release on cerebral palsy patients. Bull Hosp Jt Dis 53:34–36 Baddar A, Granata K, Damiano DL, Carmines DV, Blanco JS, Abel MF (2002) Ankle and knee coupling in patients with spastic diplegia: effects of gastrocnemius-soleus lengthening. J Bone Joint Surg Am 84-A:736–744 Damron T, Breed AL, Roecker E (1991) Hamstring tenotomies in cerebral palsy: longterm retrospective analysis. J Pediatr Orthop 11:514–519. SRC – GoogleScholar Delp SL, Arnold AS, Speers RA, Moore CA (1996) Hamstrings and psoas lengths during normal and crouch gait: implications for muscle-tendon surgery. J Orthop Res 14:144–151. SRC – GoogleScholar Goldberg SR, Ounpuu S, Delp SL (2003) The importance of swing-phase initial conditions in stiff-knee gait. J Biomech 36:1111–1116 Hoffinger SA, Rab GT, Abou-Ghaida H (1993) Hamstrings in cerebral palsy crouch gait. J Pediatr Orthop 13:722–726. SRC – GoogleScholar Hsu LC, Li HS (1990) Distal hamstring elongation in the management of spastic cerebral palsy. J Pediatr Orthop 10:378–381. SRC – GoogleScholar Klotz MC, Heitzmann DW, Wolf SI, Niklasch M, Maier MW, Dreher T (2016) The influence of timing of knee recurvatum on surgical outcome in cerebral palsy. Res Dev Disabil 48:186–192 McCarthy RE, Simon S, Douglas B, Zawacki R, Reese N (1988) Proximal femoral resection to allow adults who have severe cerebral palsy to sit. J Bone Joint Surg Am 70:1011–1016. SRC – GoogleScholar Miller F, Dias R, Lipton GE, Albarracin JP, Dabney KW, Castagno P (1997) Cardoso the effect of rectus EMG patterns on the outcome of rectus femoris transfers. J Pediatr Orthop 17:603–607. SRC – GoogleScholar

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Ounpuu S, Muik E, Davis RB, Gage JR, DeLuca PA (1993) III, Rectus femoris surgery in children with cerebral palsy. A comparison between the effect of transfer and release of the distal rectus femoris on knee motion. Part, II. J Pediatr Orthop 13:331–335. SRC – GoogleScholar Presedo A, Megrot F, Ilharreborde B, Mazda K, Pennecot GF (2012) Rectus femoris distal tendon resection improves knee motion in patients with spastic diplegia. Clin Orthop Relat Res 470:1312–1319 Simon SR, Deutsch SD, Nuzzo RM (1978) Genu recurvatum in spastic cerebral palsy. Report on findings by gait analysis. J Bone Joint Surg 60:882–894. SRC – GoogleScholar

1515 Svehlik M, Zwick EB, Steinwender G, Saraph V, Linhart WE (2010) Genu recurvatum in cerebral palsy – part a: influence of dynamic and fixed equinus deformity on the timing of knee recurvatum in children with cerebral palsy. J Pediatr Orthop B 19:366–372 Thometz J, Simon S, Rosenthal R (1989) The effect on gait of lengthening of the medial hamstrings in cerebral palsy. J Bone Joint Surg 71:345–353. SRC – GoogleScholar Zwick EB, Svehlik M, Steinwender G, Saraph V, Linhart WE (2010) Genu recurvatum in cerebral palsy – part B: hamstrings are abnormally long in children with cerebral palsy showing knee recurvatum. J Pediatr Orthop B 19:373–378

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1518 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Secondary Adaptations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The Foot as a Functional Moment Arm in Contact with the Ground Reaction Force . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Secondary Adaptations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The Ankle as a Power Output Joint . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Ankle Dorsiflexion in Swing Phase . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Treatment Summary and Outcome Expectations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1525 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1526 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1532 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1532

Abstract

Based on our understanding of normal gait, we know the musculoskeletal subsystems function as a series of mechanical components linked by joints. Each of these segment components and the connecting joints has a specific role in gait. As the demands of an abnormal gait occur due to abnormalities in motor control, energy production, and balance problems, the mechanical aspects of the musculoskeletal system require adjustments to occur. There can be adaptive adjustments that accommodate for the problem

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_201

at a different location, or the problem may be primary and the source of the problem requiring the adaptation elsewhere. Sorting out this impact is very important when planning treatment because secondary adaptations need no treatment, as they will resolve when the primary problem is addressed. The foot is an important element in this mechanical system because it makes contact with the floor and requires stability. The foot can be compromised by a primary deformity in the foot itself, such as planovalgus, equinovarus, or mid foot break. In these situations, the foot becomes flexible and is not a stable base upon which to support the body. Some of these deformities also cause abnormal rotation,

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causing the foot not line up with the knee axis, further making stability difficult. There may also be contractures that prevent the ankle from allowing the foot to dorsiflex which is a major cause of toe walking. This creates a difficult environment for the muscles to generate power for pushoff. The goal of this chapter is to further explain the impacts of these foot deformities on the gait of children with cerebral palsy. Keywords

Cerebral palsy · Planovalgus · Equinus · Lever arm · Moment arm · Foot progression angle

Introduction Based on our understanding of normal gait, we know the musculoskeletal subsystems function as a series of mechanical components linked by joints. Each of these segment components and the connecting joints has a specific role in gait. As the demands of an abnormal gait occur due to abnormalities in motor control, energy production, and balance problems, the mechanical aspects of the musculoskeletal system require adjustments to occur. There can be adaptive adjustments that accommodate for the problem at a different location, or the problem may be primary and the source of the problem requiring the adaptation elsewhere. Sorting out this impact is very important when planning treatment because secondary adaptations need no treatment, as they will resolve when the primary problem is addressed. However, there are situations where an adaptive secondary change over time can become part of the primary problem. An example of such a problem is the combination of toe walking with hemiplegia in young children. The mechanical system prefers to be symmetric, and in young children who have great strength for their body weight, if forced to toe walk on one side, will usually prefer to toe walk on both sides. If children have a pure hemiplegic pattern and the unaffected ankle has full range of motion, an orthotic

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is needed only on the affected side. This orthotic will stop the toe walking on the opposite side as well. If the toe walking has been ignored in older children and they have been walking on their toes for 4–6 years, the unaffected side, even if there is no neurologic pathology, will have become contracted; therefore, they cannot walk feet flat. The adaptive deformity has now become a primary impairment in its own right and if surgical treatment is planned, the unaffected leg must be addressed as well. As we consider the individual segments of the musculoskeletal system, we will pay special attention to this primary versus secondary aspect of the deformity. This chapter will primarily address the impact of deformities at the level of the foot and ankle as it impacts the gait of children with cerebral palsy (CP).

Natural History and Pathophysiology The foot has the role of being a stable segment aligned with the forward line of progression and providing a moment arm in contact with the floor. The ankle provides the primary energy output for mobility by the force it applies to the floor and provides motor output for postural control, as well as being part of the shock absorption function during weight acceptance. The primary role of the foot segment is to provide a stable, stiff connection to the ground during stance phase. The primary problems occurring at the foot are foot deformities that preclude a stable base of support. These deformities are mainly planovalgus, and less commonly, varus deformity. Another problem is the loss of stiffness of the foot segment, which occurs because of increased range of motion in the midfoot allowing for midfoot dorsiflexion, also called midfoot break (Maurer et al. 2013). This combination of foot pathology leads to less stability of the foot as a stiff segment and further leads to less stable support with the ground by focusing the pressure into a smaller contact area (Case 1). The primary cause of foot deformities is poor motor control, which is added to by the mechanical force driving

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this as a progressive deformity. Foot deformities are discussed fully in ▶ Chaps. 148, “Ankle Equinus in Cerebral Palsy” and ▶ “Equinovarus Foot Deformity in Cerebral Palsy.” The degree of dysfunction caused by the foot deformity is best assessed with a pedobarograph, where only pressure on the medial midfoot would suggest a very severe foot deformity with poor mechanical function. Also, an assessment of the ankle moment often demonstrates low plantar flexion moment in late stance, but a high or normal plantar flexion moment in early stance. A foot that has lost its stiffness also cannot provide support against which the gastrocnemius muscle can work to provide push-off power.

Secondary Adaptations When a foot is unstable, balancing and motor control subsystems are stressed and one response is to increase the stiffness at the proximal joint through increased tone and increased motor co-contraction, especially at the knee. The vastus muscles, as primary knee extenders, are usually activated to assist with maintaining upright posture with the knee in flexion as part of the crouched gait pattern. These secondary changes, especially in adolescents with greatly increased body mass, add to the pathomechanics causing a foot deformity to become more severe. Alternatively, there are patients where the foot is the initial primary cause of the crouched gait pattern (Case 1).

Treatment In young children, the primary treatment of the unstable foot is the use of custom-molded foot orthotics, usually starting with solid ankle AFOs; then, if the deformities are not too severe, the AFOs can be articulated. However, if the foot deformity is severe, articulated orthotics do not work well because motion tends to occur in the subtalar joint. At some point, many of these children need surgical stabilization of the foot. There are many surgical options that are discussed fully

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in the chapter on the foot and ankle (▶ Chaps. 149, “Equinovarus Foot Deformity in Cerebral Palsy” and ▶ 150, “Planovalgus Foot Deformity in Cerebral Palsy”).

The Foot as a Functional Moment Arm in Contact with the Ground Reaction Force The other major function of the foot, in addition to being a stable, stiff segment, is to be a moment arm upon which the ground reaction force can act; this means the foot has to have an alignment that is in line with the forward line of progression and at right angles to the ankle and knee joint axes. Torsional malalignment of the foot does not allow the power output at the ankle to have a moment arm on which to work. This torsional malalignment may have its primary etiology as part of the foot deformity. The planovalgus deformity may cause an external rotation of the foot relative to the ankle joint and knee axis and the equinovarus causes internal rotation of the foot relative to the ankle joint axis. The torsional malalignment may also be due to tibial torsion, femoral anteversion, or pelvic rotation (Case 7.6). The alignment of the foot is best assessed by the foot progression angle on the kinematic evaluation. The source of the rotational malalignment is best determined by tibial torsion and femoral rotation measures on the kinematic evaluation compared with the physical examination. On the physical examination, femoral rotation with hip extension is assessed. Tibial torsion is measured with a transmalleolar axis-to-thigh angle. In general, a normal foot progression angle is 0–20 external. Most individuals with CP do well until the angle is more than 10 internal or 30 external. The foot progression angle, which is more than 30 external, will rapidly start to have a negative effect on the moment arm, as an effective length of the moment arm rapidly shortens. This number is due to the length of the moment arm being the length of the foot times the cosine of the rotation angle (Fig. 1). Therefore, changes of the first 20–30 cause minimal change in the affected moment arm.

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Fig. 1 The torsional alignment of the foot, knee, and the forward line of progression of the body is very important. If the foot is not stable or lined up with the knee axis, the plantar flexion–knee extension couple cannot function, and the child drops into a crouched gait pattern. As the foot rotates relative to the knee axis, the moment arm of the foot decreases. The length of the moment arm is determined by the cosine of the angle of rotation times the length of the foot. This means that there is very little effect on the first 20–30 of external or internal rotation; however, over 30 , the moment arms rapidly lose length, and the moment arm falls very fast when there is more than 45 of external rotation. With out-toeing rotation of the foot the external knee extension moment decreases and valgus knee moment increases. With in-toeing the external knee extension moment also decreases but the knee varus moment increases.

Secondary Adaptations As the moment arm becomes less effective, the plantar flexion moment generated by the ankle decreases. As with foot deformities, the same secondary effects of increased stiffness and increased co-contractions occur. There may also be a residual moment, which tends to cause the deformity to get worse. In a foot with severe external rotation, the moment arm in the direction of forward motion has decreased greatly. However, the moment arm generating an external rotation moment has increased and now may be a mechanical factor to increase the deformity, either by increasing the foot deformity, or by causing increased external tibial torsion as children grow. This external rotation moment arm may also cause

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external rotation subluxation by rotating the tibia through the knee joint. There is an increase in the varus-valgus moment arm as well, but this seldom seems to cause mechanical or growth problems, probably because the force is somewhat reduced with the increased co-contraction required for walking, which is common in this combination of deformities. Many children have a combination of external rotation and planovalgus foot deformity, which makes a double-dose insult to the moment arm function of the foot. This insult is a principal cause of severe crouched gait and has been termed lever arm disease by Gage (Gage 1991) (see Fig. 1). The lever arm is another name for a moment arm, and the importance of this concept to the etiology of crouched gait is often missed. Failing to understand the importance of the moment arm in the crouched gait pattern is like spending time sewing a skin wound on the leg of a child with an injury while failing to see the underlying fracture. All orthopedists know that the open fracture is really much more significant than the skin wound, and likewise, the lever arm dysfunction at the foot is much more significant as a contribution to crouched gait in most children than the knee flexion, which is readily apparent (Case 2).

Treatment Malrotation of a foot progression angle can be treated with a foot orthotic if a major portion of the malrotation comes from the foot deformity. If the malrotation is secondary to torsional deformity more proximally, the only treatment option is surgical correction of the malrotation (Aiona et al. 2012). In some children, the rotation is present in two or three locations and a decision has to be made if all or several need to be corrected (Gaston et al. 2011). A relatively common example is severe planovalgus feet with external tibial torsion and increased femoral anteversion. In this situation, based on the physical examination and kinematic measurements, a judgment of how many of the deformities need to be corrected has to be made. These data have to be combined with an intraoperative assessment. For example, after the planovalgus foot deformity has been surgically corrected, the foot-to-thigh angle should be

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1521 Table 1 Segment and joint compensations

Fig. 2 As the foot develops more equinus, it also tends to go into internal rotation of the foot relative to the tibia. When the severe equinus is corrected, as the foot goes into dorsiflexion it also goes into external rotation relative to the tibia. When correcting severe equinus, this secondary rotational change always has to be considered, so one should not be surprised that the individual now has severe external tibial torsion after tendon Achilles lengthening

checked. If the foot-to-thigh angle is more than 25–30 externally, tibial osteotomy is definitely needed, but if the foot-to-thigh angle is between 10 internal and 10 external, no tibial osteotomy is needed. The midpoint ranges have to include consideration of children’s level of function with more accurate correction attempted in children with better functional ability. In situations in which there is internal tibial torsion and femoral anteversion, the decision about doing one or both levels may be especially difficult. Correcting significant equinus also causes the foot to go from internal rotation to external rotation. Therefore, when making the decision on the need for rotational correction, the final determination should be made after surgical correction of the equinus (Fig. 2). One rule that should almost always be applied is do not create compensatory deformities or, in other words, do NOT externally rotate the tibia past neutral to compensate for femoral anteversion. This compensation often leads to

As the primary Problem etiology Foot and ankle Equinus at Gastrocnemius foot contact and/or soleus contracture, Weak dorsiflexors Lack of first Gastrocnemius rocker and/or soleus contracture or Muscle overactivity (spasticity), Ankle stiffness, Premature Lack of first second rocker rocker, Spastic gastrocnemius or soleus Contracted gastrocnemius or soleus High early Spastic or plantar flexion contracted moment gastrocnemius or soleus Decreased late Contracture of stance plantar gastrocsoleus, or flexion weak moment gastrocsoleus Decreased Lack of plantar push-off flexion in third power rocker

Internal or external foot progression

Tibial torsion, Femoral torsion Planovalgus, or Equinovarus or varus feet

Compensatory effect for Severe knee flexion contracture

Lack of knee extension in midstance

Unstable foot (planovalgus, or midfoot break) Lever arm disease, Planovalgus, Severe torsional malalignment Severe muscle weakness or poor balance and is used to stabilize posture Motor control problem

progressive deformity of external tibial torsion (Table 1).

The Ankle as a Power Output Joint The ankle is the principal power output joint and is an important part of being a shock absorber

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along with the knee. Ankle position at initial contact is very important in the shock absorption function. If initial foot contact is with toe strike, the foot and gastrocnemius may absorb some energy; however, if the position is foot flat, there is often a very hard strike, with the floor having to absorb the energy of initial contact. Children walking with this pattern can often be heard walking down hallways because of the loud sound and vibrations set up in the floor. The lack of shock absorption is measured on the vertical vector of the ground reaction force. The loading response may show a magnitude of 1.5 to 2 times body weight when normal children’s loading force should be between 1.1 to 1.2 times body weight (Fig. 3a). The loss of shock absorption also occurs in children in whom there is an incompetent gastrocsoleus, a situation where they strike only on the heel but have little ability to absorb the load except through the heel pad. This situation is primarily seen in children whose Achilles tendon has been transected by tenotomy or in paralytic conditions with active dorsiflexion but no plantarflexor function. During weight acceptance, the position of the ankle joint is determined by the gastrocsoleus muscle. If the muscle is contracted and unable to allow 15–20 of dorsiflexion by eccentric contraction, a premature heel rise will occur. If the eccentric contraction initiates a concentric contraction, a premature plantar flexion will occur in midstance phase, causing a midstance phase rise in the center of gravity, called a vault. A major burst of power generation will be associated with the vault (see Fig. 3d). The premature gastrocnemius and soleus contraction may also cause the heel to rise, but with increased knee flexion. The center of gravity does not rise; however, the child’s crouch increases. The second possible response to increased plantar flexion in midstance is knee extension, producing backkneeing. The reasons for these three attractors for knee response to overactivity of the gastrocnemius in midstance are discussed in the knee chapter (▶ Chap. 93, “Kinematics and Kinetics: Technique and Mechanical Models”). The primary reason for the gastrocnemius and soleus having a premature contraction in midstance phase may be a contracture of the

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gastrocnemius, which most commonly does not allow the muscle sufficient excursion for the required 20 of dorsiflexion as the knee is coming to near full extension. The treatment of this contracture is lengthening of the muscle–tendon unit, usually by gastrocnemius lengthening only. Appropriate gastrocnemius lengthening can restore some push-off power and normalize the ankle moment (Rose et al. 1993) (Saraph et al. 2000). Another primary cause of premature gastrocnemius contraction may be related to decreased motor control, making independent control of eccentric contractions difficult. These difficulties may be correlated with increased tone and increased sensitivity in the tendon stretch reflex, which together initiate a concentric contraction at the foot contact. This concentric contraction continues through weight acceptance and midstance and is best treated with an AFO that blocks plantar flexion but allows dorsiflexion. As the gait cycle moves to late stance, the time for the power burst of the gastrocnemius occurs. If the transition from midstance to terminal stance has the ankle in plantar flexion, the mechanical advantage of the moment arm of the foot will be compromised. If the ankle is in 0–10 of plantar flexion, this may not be a significant compromise; however, if the ankle is in 20–45 of plantar flexion as terminal stance is entered, there is very little ability to generate a push-off power burst. The amount of the power burst also depends on the amount of stretch and muscle fiber length relative to the rest length or, in other words, it depends upon the muscle’s position on the length–tension curve. If the muscle is already almost completely shortened through a contraction, little additional power can be generated. Power output that is required for the push-off power burst can be generated only with a concentric contraction, in which the muscle actually shortens. The poor prepositioning of the ankle joint in terminal stance often precludes significant push-off power generation (see Fig. 3d). The secondary adaptations for the decreased ankle pushoff power generation require that the hip extensors become the primary power generators for forward motion of gait. This proximal migration of power generation is often combined with increased

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Fig. 3 At initial contact and loading phase, the stance limb functions as a shock absorber. When the limb is not shortening through the knee, there is a very high impact force as the weight is shifted on the loading limb; this is seen best

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on the vertical vector of the ground reaction force (a). If the ankle then also develops a premature plantar flexion in midstance called a vault, power that lifts the center of mass vertically is generated (b). From the peak of the

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pelvic rotation. This change increases the total energy of walking, but is a good trade-off when motor control is not sufficient to manage the more distal ankle power generation. This same process is invoked in the role of fashion by the use of highheeled shoes. The high-heeled shoes prevent the prepositioning of the ankle in slight dorsiflexion during terminal stance, therefore precluding the push-off power from the gastrocsoleus. This forces power generation to the hip extensors, which also increases the amount of pelvic rotation. Treatment of the plantar flexion prepositioning of the ankle at the start of terminal stance can include the use of orthotics. Although the orthotic can block the midstance problems of vault, backkneeing, or increased crouch, it will not preposition the foot to allow push-off power burst because it prevents active plantar flexion. An articulated AFO may preserve some push off power; however, it is greatly reduced from normal (Romkes and Brunner 2002). The use of a leaf-spring orthosis is another option; however, the stiffness required to prevent the midstance phase plantar flexion almost always prevents the terminal stance phase plantar flexion burst as well. In many patients, the gastrocnemius is much more of a problem than the soleus. The gastrocnemius covers three joints and tends to develop a more severe contracture more quickly. Based on the physical examination, the degree of contracture between the gastrocnemius and the soleus can be separated based on the degree of dorsiflexion of the ankle with the knee flexed versus extended. This examination records the excursion of the soleus compared with dorsiflexion of the ankle with the knee extended, which reflects the excursion of the gastrocnemius. Usually, lengthening only the

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gastrocnemius will greatly improve the premature contraction problem in middle stance, and in some situations, allows improved push-off power development by improved prepositioning of the ankle. It is very important to avoid overlengthening because the ankle generally functions better in mild equinus than hyperdorsiflexion, a position where it can generate no plantar flexion. Many children who had their Achilles tendons transected require lifelong use of AFOs to stabilize their ankle joints.

Ankle Dorsiflexion in Swing Phase Dorsiflexion in swing phase has two roles. First, in early swing phase, dorsiflexion helps to shorten the limb and allows swing through. Second, in terminal swing phase, dorsiflexion is part of prepositioning the limb for initial contact. Most children with CP have active dorsiflexor power produced by the tibialis anterior. If the EMG of the tibialis anterior is phasic in its activity, but very little dorsiflexion is produced, the cause is usually co-contraction with the gastrocnemius and soleus, or the tibialis anterior is attempting to contract against a contracted gastrocnemius muscle. In the presence of a phasic contracting tibialis anterior muscle, the ability for it to produce dorsiflexion will be enhanced with gastrocnemius lengthening. If the plantar flexion contracture was severe, the tibialis anterior may be overstretched and will require using an orthotic for some time to contract and function in its proper length (Fig. 4) or alternatively requires shortening of the tibialis anterior tendon (Rutz et al. 2011; Tsang et al. 2016). Some children with incompetent Achilles tendons develop dorsiflexion contractures because there

ä Fig. 3 (continued) vault, the body falls forward into terminal stance (c); however, the ankle is usually still in equinus, thereby decreasing the ability for the ankle to generate power from additional push-off. This occurs

because the muscle tends to be positioned on the wrong side of the length–tension curve to have maximum ability to generate power, and the remaining range of motion is limited (d)

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Fig. 4 A problem of equinus contracture that gets most of the attention is the shortened gastrocsoleus which limits the active range of motion. This active range of motion can be changed by lengthening the tendon Achilles or the gastrocnemius; however, the second problem is that the tibialis anterior has developed an overlengthened tendon since it is

functioning in the equinus position. After lengthening the tendon Achilles, the tibialis anterior is now much too long; therefore, it is not functioning as an antagonist muscle in the same range in which the gastrocsoleus is working. Time is required for the tibialis anterior to shorten or it has to be surgically shortened

is no gastrocnemius strength to overcome the tibialis anterior power. Some children with inadequate dorsiflexion combined with a stiff knee have severe toe drag in early swing phase. The dorsiflexion is a secondary cause of toe drag with the stiff knee being the primary cause. Often, this order is confused and the equinus gets the primary blame. For example, an individual with complete paralysis of the tibialis anterior causing a drop foot but otherwise a normal functioning extremity will never drag his toes. He will instead develop hyperflexion of the hip and knee to allow clearing of the foot. The only time an equinus foot position will cause toe drag is when it is associated with a knee that has decreased knee flexion in early swing phase. Many children with toe drag have dorsiflexion of the ankle and still drag their toes. This dorsiflexion also explains why children wearing orthotics that prevent plantar flexion still have toe drag. This again shows that the toe drag actually was due to the knee and not the plantar flexion. The treatment of decreased dorsiflexion power preventing active dorsiflexion is a very light, flexible leaf-spring AFO. These AFOs will control dorsiflexion and still allow some plantar flexion to occur. These AFOs are useful only when the gastrocnemius

and the soleus have relatively normal tone and muscle length.

Treatment Summary and Outcome Expectations The pathologic leading to the development of planovalgus deformity or equinovarus deformity or a midfoot break is caused by poor motor control and propagated by pathologic mechanical forces during growth. The primary treatment in the growing child is to provide orthotic support as long as this is tolerated. As the child develops near normal adult size, deformity is usually becoming more severe and requires surgical stabilization. Correction of the equinovarus, midfoot break, or planovalgus needs to focus on creating an anatomically aligned foot which is stable for weight bearing. This correction should also focus on appropriate rotational alignment of the foot with the knee axis (Theologis 2013). This may require surgical correction of the rotation above the level of the foot if foot deformity correction is not sufficient. If the surgical corrections are performed at or near completion of growth, the outcome expectations should be for permanent correction of the foot deformity. Corrections in

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Fig. C1.1

middle childhood have a significant risk for recurrence, and surgical corrections in early childhood have a higher risk of recurrence or over correction. Correcting the force generating capacity of the foot requires stability as defined above. Furthermore, it requires the correct length of the muscles in their proper preposition. Muscles with contractures such as the gastrocnemius contracture will require lengthening to place them in the proper position. The goal is to normalize foot contact to the floor by the end of growth with the goal of having heel contact, normal second rocker, and push off with normal equinus. Although this goal cannot always be accomplished because of poor motor control, it is still the ideal position. Having a foot that is stable and in normal sequential contact with the floor with normal rotation alignment will greatly improve the mechanics at the hip and knee (Kadhim and Miller 2014; Brunner 2010). The accomplishment of this at the end of growth should be maintained into adulthood (Fig. 1).

Cases

Case 1 Joshua

Joshua, a boy with asymmetric diplegia, walked with a posterior walker. By age 6 years, he was walking independently, although very asymmetrically, with extreme knee stiffness on the left. At that time he had a rectus transfer on the left, and he continued to do well until age 15 years. As he was going though his adolescent growth, he

gradually developed more right foot planovalgus and external rotation, and complained of having increased knee pain with ambulation. He was placed in a ground reaction AFO but, because of poor moment arm due to the external rotation, this was of little help. The knee pain was believed to be due to high joint reaction force external valgus moment at the knee and high shear stress in the knee. The foot pressure demonstrated a moderate right planovalgus foot deformity with an external foot progression angle of 35 , although a weightbearing radiograph of the foot was nearly normal (Fig. C1.1). He also had 45 of external thigh–foot angle on physical examination. Based on these data, the crouch and knee pain were thought to result from a combination of planovalgus and external tibial torsion. Also, a radiograph of his knee demonstrated mild increased knee valgus measuring 12 . The planovalgus was corrected with a lateral column lengthening and the tibial torsion with an osteotomy of the tibia (Fig. C1.2). It was elected to leave the knee valgus because this was on the border of normal and due to secondary forces from the leg below. One year after the surgery, he was walking without knee pain and no orthotics; however, he still had a mild degree of knee valgus but with improved crouch (Fig. C1.3). The right foot demonstrates a mild residual valgus deformity; however, the left foot is slightly overcorrected into varus (Figs. C1.4 and C1.5). (continued)

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Fig. C1.2

Fig. C1.3

The right gastrocsoleus is still somewhat incompetent based on the prolonged heel contact or late heel rise on the right

(Fig. C7.5.4). To completely correct this deformity, a high tibial varus osteotomy (continued)

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Fig. C1.4

Fig. C1.5

would have been required. This demonstrates the typical occurrence of these deformities as an adolescent goes through the final growth, often with problems occurring at several levels, which combine to cause a severe problem.

Case 2 Lakesia

Lakesia, a 15-year-old girl with a diagnosis of spastic diplegia, was in a regular high school and was a varsity swimmer on the high school swim team. She had also been playing lacrosse as a recreational sport. (continued)

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Fig. C2.1

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Fig. C2.2

Over the past 2 years, she had grown rapidly and gained weight. During that time she gradually started to develop more knee pain, worse on the left than the right, to the point that she had trouble walking around

her school and she could not run to play lacrosse. Her family doctor told her to buy and use a wheelchair. Her gait involved a significant amount of trunk lurching with (continued)

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Fig. C2.3

mild crouching, stiff knee gait, and internal rotation of the knees. On physical examination, both knees had mild diffuse tenderness, with no effusion, mechanical instability, click, or joint line tenderness. Hip motion demonstrated 80 of internal rotation, 10 of external rotation, full knee flexion and extension with popliteal angles of 70 , and transmalleolar-to-thigh axis of 30 external on the left and 20 on the right. Both feet demonstrated a planovalgus deformity, and both feet had significant bunions. Radiographs of the knees were normal. She was initially evaluated in the sports clinic where a diagnosis of intraarticular pathology was made, and she was scheduled for knee arthroscopy, where an inflamed plica was found and excised. Following a 6-month rehabilitation program, she still continued with the same pain, and she was now using the wheelchair for all ambulation except for household ambulation. An evaluation in the gait

laboratory found significant internal rotation of the hips, external tibial torsion on the right, and internal tibial torsion on the left with the planovalgus feet, increased knee flexion at foot contact, and decreased knee flexion in swing phase (Figs. C2.1 and C2.2). Because there was minimal EMG activity in the rectus in swing phase (Fig. C2.3), a trial of Botox to the left rectus also demonstrated no change in the motion of the left knee in swing phase. It was thought that the decreased knee flexion in swing was due to the poor push-off and poor mechanical advantage on the hip flexors at push-off. She was immediately referred to physical therapy and taught crutch walking to try to get her out of the wheelchair. She was then reconstructed with bilateral femoral derotation osteotomies, left tibial rotation, bilateral lateral column lengthenings, bunion corrections, and hamstring lengthenings. One year following surgery, she was (continued)

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pain free, was again swimming on the varsity swim team, and was no longer using the wheelchair for any community mobility, except for very long walks such as at airports or amusement parks. In all community ambulation, she used the Lofstrand crutches, which she preferred over the wheelchair.

Cross-References ▶ Ankle Equinus in Cerebral Palsy ▶ Equinovarus Foot Deformity in Cerebral Palsy ▶ Hip and Pelvic Kinematic Pathology in Cerebral Palsy Gait ▶ Kinematics and Kinetics: Technique and Mechanical Models ▶ Knee Deformities Impact on Cerebral Palsy Gait ▶ Planovalgus Foot Deformity in Cerebral Palsy

References Aiona M, Calligeros K, Pierce R (2012) Coronal plane knee moments improve after correcting external tibial torsion in patients with cerebral palsy. Clin Orthop Relat Res 470:1327–1333 Brunner R (2010) Measures to improve gait in patients with cerebral palsy. Orthopade 39:15–22

F. Miller Gage J (1991) Gait analysis in cerebral palsy. Mac Keith Press, London Gaston MS, Rutz E, Dreher T, Brunner R (2011) Transverse plane rotation of the foot and transverse hip and pelvic kinematics in diplegic cerebral palsy. Gait Posture 34:218–221 Kadhim M, Miller F (2014) Crouch gait changes after planovalgus foot deformity correction in ambulatory children with cerebral palsy. Gait Posture 39:793–798 Maurer JD, Ward V, Mayson TA, Davies KR, Alvarez CM, Beauchamp RD, Black AH (2013) A kinematic description of dynamic midfoot break in children using a multi-segment foot model. Gait Posture 38:287–292 Romkes J, Brunner R (2002) Comparison of a dynamic and a hinged ankle-foot orthosis by gait analysis in patients with hemiplegic cerebral palsy. Gait Posture 15:18–24 Rose SA, DeLuca PA, Davis RB 3rd, Ounpuu S, Gage JR (1993) Kinematic and kinetic evaluation of the ankle after lengthening of the gastrocnemius fascia in children with cerebral palsy. J Pediatr Orthop 13:727–732 Rutz E, Baker R, Tirosh O, Romkes J, Haase C, Brunner R (2011) Tibialis anterior tendon shortening in combination with Achilles tendon lengthening in spastic equinus in cerebral palsy. Gait Posture 33:152–157 Saraph V, Zwick EB, Uitz C, Linhart W, Steinwender G (2000) The Baumann procedure for fixed contracture of the gastrocsoleus in cerebral palsy, Evaluation of function of the ankle after multilevel surgery. J Bone Joint Surg Br 82:535–540 Theologis T (2013) Lever arm dysfunction in cerebral palsy gait. J Child Orthop 7:379–382 Tsang ST, McMorran D, Robinson L, Herman J, Robb JE, Gaston MS (2016) A cohort study of tibialis anterior tendon shortening in combination with calf muscle lengthening in spastic equinus in cerebral palsy. Gait Posture 50:23–27

Complications from Gait Treatment in Children with Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1534 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1534 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Complications of Gait Analysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Complications of Surgery Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Interrelated Effect of Multiple Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Complications of Surgical Execution . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Complications of Rehabilitation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Monitoring the Outcome of Gait Development and Treatment . . . . . . . . . . . . . . . . . . . . . . . Energy Use Measurement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1540 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1541 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1541

Abstract

Gait abnormalities are one of the most common problems which occur in children with cerebral palsy (CP) who are able to walk. The primary classification for children with CP includes the Gross Motor Function Classification System (GMFCS) which is really based on the child’s ability to be mobile. For children at GMFCS levels I–III, the primary problem which most affects them is abnormalities in their gait. As the child develops improved

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_202

skills and starts to ambulate often using an assistive device such as a posterior walker, the parents want to know what the child will be like walking when they are teenagers. Making these long-term predictions is very difficult especially when the child is only 2 or 3 years old. As the child ages and especially by middle childhood between 5 and 7 years of age, gait pattern becomes more clearly defined, and better long-term expectations can be defined. Because of the highly varied nature of the natural history of CP, a common mistake that is made especially by clinicians with limited experience is having too much confidence in the long-term prediction for an individual child. There are many real and potential 1533

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complications in the treatment of gait problems in children with CP. Often, there is the presumption that nonoperative treatment has no complications; however, not addressing evolving deformities can lead to loss of ambulatory ability. This chapter addresses the common complications related to failure to treat, surgical decision-making, common surgical procedures, and postoperative rehabilitation.

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is careful clinical assessment to prevent any functional regression during this time. A full analysis of the gait problems with timely correction is the key to optimal outcome. In previous chapters we have discussed the expected natural history and treatment requirements for different patterns and gait pathologies. The goal of this chapter is to review the pitfalls that occur in managing and treating gait pathology in children with CP.

Keywords

Cerebral palsy · Complications · Gait analysis · Surgery planning · Rehabilitation

Introduction Gait abnormalities are one of the most common problems which occur in children with cerebral palsy (CP) who are able to walk. The primary classification for children with CP includes the Gross Motor Function Classification System (GMFCS) which is really based on the child’s ability to be mobile. For children at GMFCS levels I–III, the primary problem which most affects them are abnormalities in their gait. Treatment of the child’s gait problems typically starts with physical therapy in the first years of life. To this we add orthotics mostly around the foot and ankle and assistive devices to help the child walk. A major focus for many young parents of children with CP is their goal to have them walk. For some parents the focus tends to be that if the child could only walk, then all the problems would disappear. As the child develops improved skills and starts to ambulate often using an assistive device such as a posterior walker, the parents want to know what the child will be like walking when they are teenagers. Making these long-term predictions is very difficult especially when the child is only 2 or 3 years old. As the child ages and especially by middle childhood between 5 and 7 years of age, the gait pattern becomes more clearly defined, and better long-term expectations can be defined. It is during this middle childhood period and going in to adolescences that significant gait abnormalities develop which are amendable to surgical management. An important aspect of proper management

Natural History and Pathophysiology Because of the highly varied nature of the natural history of CP, a common mistake that is made especially by clinicians with limited experience is having too much confidence in the long-term expectation for an individual child. GMFCS grading system is advocated by some individuals as being stable throughout a lifetime, and a grade that can be assessed as early as age 2 will remain stable. Based on reported studies of populations, the use of age and GMFCS does have long-term prognostic value (Palisano et al. 2000). Children assigned a GMFCS level between 2 and 4 years old will have a different GMFCS level 42% of the time by adolescence (Gorter et al. 2009). This means that almost ½ of the assignments will change; therefore giving even gross estimates based on GMFCS level in young children must be done with caution. The neuromotor development is just too variable to give prognostic information with great confidence to families. Clinicians need to understand that families often will hold on to these prognostications and when the child fails to meet expectations, they will want to blame themselves or someone else. It is more common that GMFCS reassignments are made to a lower level than to a higher level (Gorter et al. 2009), which further adds to the frustration of families. There are many real and potential complications in the treatment of gait problems in children with CP. Often, there is the presumption that nonoperative treatment has no complications; however, this is false. The most severe complication of nonoperative treatment is to continue to treat a deformity that is clearly getting worse but the progression is ignored. A typical example is a

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child who is increasing in crouch with increasing knee flexion contracture, but there is no decision to address the problem. When the knee flexion contracture finally gets to the point that the child can no longer walk, a decision has to be made to put him in a wheelchair or try surgery. This delayed and poor clinical judgment will be the direct cause of the child being in a wheelchair for the remainder of his life, or it may be the direct cause of the complications, which are incurred much more commonly in correcting severe knee flexion contractures than in correcting milder deformities. Individuals who are good community ambulators (GMFCS levels I–III) at age 7 or 8 do not go into wheelchairs at age 15 years unless there is some complication or supervening medical problem unrelated to CP. This means a child at age 7–8 who does not own a wheelchair should not need a wheelchair when he is 15 or 25 years old.

Treatment Treatment-related complications are very extensive. Most people think of surgical complications when we discuss complications related to treatment; however there are also complications related to nonoperative treatment. The use of ankle foot orthoses (AFO) is almost ubiquitous in children with CP. Complications related to orthotic use may occur at several levels. The use of inappropriate orthotics can lead to severe skin breakdown or permanent scars on the calf from breakdown of the subcutaneous fat layer. The use of restrictive ankle orthotics such as the fixed ankle AFOs or even articulated AFOs will immobilize the plantar flexor. Immobilization is a very potent form of producing muscle atrophy or muscle weakness. Therefore, the use of AFOs should always seriously consider both the negative affect, i.e., causing muscle weakness, as well as the positive affect by improving stable stance stability for the child. Children who use AFOs throughout their childhood and adolescence without any opportunity for activity without their orthotics will almost certainly become completely dependent upon their orthotics for the remainder of their lives. The severity of the muscle atrophy and the

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loss of sensory feedback will preclude the young adult from shedding the orthotic during physical activity. To prevent this AFO dependence, it is important to start in early childhood and during middle childhood with the child having times of activity when they are barefoot or use only shoes. This may mean that the child is walking on their forefoot or that they have some planovalgus collapse; however this experience is important to develop adequate sensory feedback and improve muscle strength as much as possible. Very specific clinical indications should be adhered to in ordering and recommending use of orthotics to assist maximizing the benefit and decreasing the complication risk (Davids et al. 2007). Another complication of nonoperative management is to have children in walking aids that are inappropriate. This means that children should have the correct training before being allowed to use crutches or walkers. Parents have to be informed of the risks of walking aids, such as being aware of wet floors with the use of crutches or open stair doors for individuals with poor judgment. Transition to less restrictive walking aids should be accomplished when it is functional for the child. A complication which occurs frequently is that a child is pushed into using a less restrictive walking aid such as crutches or single point canes under the goal that it will stress his balance and motor control to improve and thereby improve walking ability. This is the therapeutic approach that clearly has merit in the environment of therapy. However, in the child’s day-to-day life, it is very important that the child has functional mobility and if they are not comfortable with crutches, then they should be using walkers or the device in which they are comfortable being independent and mobile. Pushing a child to use the device which they are not comfortable often adds to the child’s frustration and may become a long-term impediment to them accepting changing to less restrictive aids.

Complications of Gait Analysis Complications that arise in the analysis of gait for preoperative planning are usually recognized by the analysis team. Parents or caretakers should be

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asked if the current gait is representative of the child’s home and community ambulation. Children spend enough time during the analysis that experienced therapists will also see how constant and representative their gait is during the whole evaluation. Children may be able to walk for doctors or therapists in a 10-min clinic examination, but this walk is almost impossible for them to maintain for a 2-h laboratory evaluation. Also, the current standard is to evaluate multiple gait cycles, with at least 10–15 cycles usually being evaluated. Evaluating multiple gait cycles also removes the concern about a representative specific cycle. Some children, especially those with behavior problems, have trouble with the level of cooperation that is required to get a full gait analysis. Also, it is difficult to get a full evaluation in children before age 3 years because of the cooperation required. Another complication evaluating gait data is to recognize the sensitivity of the rotational measures to proper marker placement on the extremities. Therefore, hip rotation and tibial torsion have to always be compared with the physical examination and with the knee varus-valgus measures on the kinematics as an assurance of accuracy. If the knee joint axis is incorrect, the knee will demonstrate increased varus-valgus movement as the knee flexes. There also needs to always have a careful evaluation of EMG patterns with the thought that leads may have gotten switched. If the pattern is really confusing, consider lead mix-up as a possibility and have the EMG repeated. Assessing and recognizing spasticity is an important aspect of gait analysis. It is extremely important to not presume the increase resistance to movement and posturing is always spasticity because movement disorders often present with a pattern that is very similar to typical spastic patterns. The recommendations for treatment when movement disorder is recognized are very different from spasticity management. Spasticity tends to have a very predictable response to surgical management and orthotics; however movement disorder tends to be very unpredictable. The first and most important thing to address in individuals with dystonia is to diagnose the dystonia and make sure it is not misinterpreted as spasticity

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(Lebiedowska et al. 2004). Diagnosing dystonia was addressed fully in the motor control and dystonia chapters (▶ Chap. 168, “Muscle Performance in Children and Youth with Cerebral Palsy: Implications for Resistance Training” and ▶ 45, “Dystonia and Movement Disorders in Children with Cerebral Palsy”). Often, a foot will look like it has severe varus deformity; then on another day, the foot will be in valgus. If surgeons do not have a video record and are not very attentive, a presumption of a spastic equinovarus foot deformity may easily be made. These feet may look like ideal feet for tendon transfers because they are supple; however, tendon transfers tend to cause severe overreaction in the opposite direction. There is no role for tendon transfer in dystonia. We had one patient in whom we did a rectus transfer, not recognizing that it was dystonia and not spasticity. This individual spent 9 months with a flexed knee every time she tried to walk. With persistent therapy and bracing, and under the threat of reversing the transfer, the muscle suddenly went silent and knee flexion in stance stopped. Botulinum toxin is an extremely effective agent to block the muscle effects of dystonia, with its major side effect being that it typically only works for three to four injection cycles and then the body becomes immune. If the individual has a foot deformity that is symptomatic, the correct treatment is fusion, usually a triple arthrodesis with transection of the offending muscles. Very little other surgery except for fusion is of benefit in ambulatory individuals with dystonia. Derotation osteotomies may have very un-intended responses as shown in a case example of a boy who went from internal to severe external rotation with a perfect amount of bone correction (Fig. 1). Individuals often have spasticity associated with the athetosis, which works as a shock absorber on the pathologic movement. Individuals with athetosis may develop significant deformities that make ambulation more difficult, and there is merit in addressing these problems. Therapy to improve athetoid gait is limited, but sometimes adding resistance through the use of ankle weights or a weighted vest can be helpful. Procedures that will provide stability have the most

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Fig. 1 A 15-year-old boy presented with increasing difficulty walking, and based on gait analysis, he was recommended to have a femoral derotation. (a) Postoperative findings show severe external rotation of the hip, (b) although the physical examination documented symmetric internal and external rotation of the hip in a prone position. The cause for this poor outcome was initially unrecognized dystonia. This was a gait analysis interpretation error

reliable outcome. For example, correction of planovalgus feet with a fusion is a reliable procedure. There is no benefit of trying muscle balancing or joint preservation treatment in the face of athetosis. Ambulatory problems related to pure chorea and ballismus are rare and very unpredictable. We have never had occasion in which surgery was required. Again, if there is foot instability, a fusion would be a reasonable option.

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planovalgus deformity that needs to be corrected (Case 1). Some common misinterpreted secondary problems are the midstance phase equinus on the normal side of a child with hemiplegia, hip flexor weakness in children with increased hip flexion and anterior pelvic tilt but high lordosis as they rest on the anterior hip capsule, weakness of the quadriceps as a cause of crouch, and intraarticular knee pathology as a cause of knee pain in adolescents with crouched gait. Many decisions on specific data are somewhat arbitrary, but having the data is an excellent way to develop an understanding of what the data mean. As a clinical decision is made, the result is then evaluated after the rehabilitation period, and understanding of the significance of the data is developed. Also, some of the errors in interpretation are related to not taking natural history into account. An example is the response of the common equinovarus foot position seen in early childhood. If these children are diplegic, the common natural history is for this deformity to completely reverse and become a planovalgus foot, so aggressive treatment should seldom be considered for the early childhood equinovarus posture. Another error is in not considering the energy cost of walking (Bowen et al. 1999). Children who use 2 ml oxygen per kilogram per meter walking are not going to be community ambulators, and judgment has to be directed as to their real function, which will primarily be sitting in a wheelchair (Piccinini et al. 2007). Also, children’s general condition should be considered as the complaints related to walking may in part result from very poor cardiovascular conditioning and not specific deformities.

Complications of Surgery Planning Complications of surgery planning are mostly related to not identifying all the problems or misinterpreting a compensatory problem for a primary problem (Wren et al. 2013). A common example of missing problems is not identifying the spastic rectus in the crouched gait pattern, missing internally rotated hips in children with an ipsilateral posterior rotation of the pelvis, and missing internal tibial torsion when there is severe

Interrelated Effect of Multiple Procedures When interpreting gait data, there should be an awareness of the impact of adding procedures together. Most procedures are relatively independent of each other; however, there are some interactions. Understanding the impact of multiple concurrent procedures is somewhat like understanding drug interactions. Some specific

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combinations to watch out for include tibial derotation for internal tibial torsion in the ipsilateral side of a foot that is having posterior tibial tendon surgery for equinovarus. In a small series of 10 limbs, 8 failed and required repeat surgery, all with overcorrection (Liggio and Kruse 2001). Based on this, we recommend choosing the deformity that seems to be the worst, or primary, deformity. Another procedure interaction is planovalgus foot correction so that the heel is in neutral through the use of a subtalar fusion and then doing a supramalleolar osteotomy to correct ankle valgus. This combination of procedures will leave the heel with a residual varus deformity, which is highly undesirable. Another interaction of procedures is that patients who have external tibial torsion that is not being corrected should not have only medial hamstring lengthening, as this will further imbalance the external rotation torque by allowing the biceps femoris muscle to create additional external torque through the knee joint.

Complications of Surgical Execution The most common complication of surgical execution is overcorrection of a deformity, especially in correction of femoral anteversion. Undercorrection may also occur in femoral rotation. The reason undercorrection occurs is that the femur is somewhat square, and often the plate used for fixation wants to set on the corner, but as the screws are tightened, it may rotate 10 or 15 in one direction or the other. Careful intraoperative evaluation after the fixation is important, and if the rotation is not corrected, it can be corrected immediately. Other intraoperative problems are specific to the procedure, such as recognizing that the foot will never look better than it does immediately after the surgery has been performed in the operating room; therefore, if the foot is still in valgus, it will be so when the cast is removed. Three and twelve months after surgery, this valgus will only get worse, not better. Correcting residual problems in the operating room is much easier than deciding to come back and correct them with a

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separate surgical procedure or a revision procedure.

Complications of Rehabilitation The major problem with rehabilitation is the lack of follow-through by families, or failure of families to be able to pay or get their insurance companies to pay for the therapy that is required. Most children can be rehabilitated as outpatients; however, there are a few especially complicated cases that really benefit from inpatient rehabilitation. The need for postoperative rehabilitation should be discussed with families, and an understanding of how and who will provide this is important even before undertaking the surgery. It is important to have therapists who clearly understand the goals for these children’s function, as it is of little benefit to have therapists spend a great deal of time working on sitting transfers when the goal of the surgery was to get the children walking. Postoperatively, the physical therapy has to be directed at the goal that was preoperatively defined through communication with the surgeon, who should be able to clearly articulate what the goals of the surgery were. Other issues in the postoperative period that may cause problems are postoperative pain and subsequent depression. Postoperative pain and depression need to be treated aggressively if they are interfering with the ability of patients to cooperate with the rehabilitation program. Often, using the correct pain medication and adding an antidepressant can be very helpful. A problem with chronic pain that often is overlooked is a stretch of the sciatic nerve. This may occur after therapy sessions or may be developed following stretching during surgical lengthening. The first important factor is that it has to be recognized. Once it is recognized as a sciatic palsy, the treatment requires great care to avoid recurrent stretching. This means ongoing therapy to maintain the knee in full extension must always be done with the child lying supine. The child should be prevented from long sitting with hips flexed and the knees extended. The family must be aware that all sitting should be done in the chair with

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knees fully flexed. The best pain control is to start with a full therapeutic dose of gabapentin and to maintain this dose until the child has been asymptomatic for at least 1 month. The gabapentin should then be slowly weaned leaving the night time dose as the final dose to wean away from. The typical course for resolution of a sciatic palsy requires 1 year to be fully pain-free and have recovery of motor function. For some severe sciatic palsies, this may require 2 or 3 years (Woratanarat et al. 2009). Almost all sciatic nerve palsies will resolve overtime in children with CP (Karol et al. 2008).

Monitoring the Outcome of Gait Development and Treatment Monitoring the outcome of gait treatment is an area where a clear consensus of a goal has been developed. In general, the goal is to make the different patterns of gait impairments move toward the normal means. Therefore, children who walk at 60 cm/s are considered improved if, following the treatment, they walk 90 cm/s. Likewise, children who go from 90 cm/s to 60 cm/s would be considered worse. This goal can be applied to joint motions, such as midstance phase knee flexion, maximum knee flexion in swing, or terminal stance power generation at the ankle. However, there are situations where this might not be exactly true, as in the example of a 5 years old with a high toe walking prancing gait pattern who can only move fast or fall over. He may have a walking speed of 90 cm/s; however, after soft-tissue lengthening, the foot is flat, and he can stand in one place and start and stop without falling, although the velocity has dropped to 60 cm/s. This child has clearly improved in the sense of stability, and even though the change in speed seems to be demonstrating the opposite, it is not a reflection of the goal of the initial treatment. The change in perspective of a specific child, the child’s age, the functional ability, and the goal of the surgery have to be considered. It is not very effective to measure the volume of a fluid with a thermometer, and in this same way, the measurement tool must reflect the treatment goal. Often,

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parents complain that the children do not walk better after an adductor lengthening performed as part of the preventive treatment of spastic hip disease. The parents need to be initially told that the goal was to prevent hip subluxation and not make their child walk better. At the same time, children are not expected to walk worse after the adductor lengthening, but the surgery was not directed at improving gait, and therefore gait improvement should not be expected.

Energy Use Measurement Another measure that has been advocated for assessing outcome of gait treatment is the energy efficiency measured by oxygen consumption. There have been suggestions of using physiologic cost index to measure energy efficiency in children with CP (Nene et al. 1993); however, this has so much variability that it is of no use in these children (Bowen et al. 1998; Boyd et al. 1999). If children have a high oxygen cost of walking, and improvement is desired, however, this is also a relative measure because a very energy-efficient gait can at the same time be completely nonfunctional. This nonfunctional but energyefficient gait is commonly seen in children with primary muscle disease (Bowen et al. 1999). There have been oral reports that rhizotomy is effective in decreasing the energy cost; however, it makes children act like muscle disease patients rather than spastic patients. The improvement in oxygen cost of walking has to be confirmed with an increased physical functionality, meaning children can do more in their environment. There has been increased interest in developing tools to assess children’s function as related to their environment. The pediatric MODEMS also called the PODCI questionnaire has been developed for use with children with physical disabilities. There has not been much reported use of this instrument in children with CP. Another scale, developed at the Gillette Hospital, the Gillette Functional Assessment Questionnaire, asks parents to grade children’s ambulatory ability on a 10 functional level scale (Novacheck et al. 2000). This same group has developed a scale or normality in the

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gait motion data, using principal component analysis of 16 gait variables (Schutte et al. 2000) which has now been updated to the Gait Deviation Index (GDI) (Schwartz and Rozumalski 2008). The GMFM and the Pediatric Evaluation of Disability Inventory (PEDI) are two other measures that can be used to give some measure of functional ability. At this time, a parent-reporting questionnaire with technical data from the gait analysis has to be combined as a measure of outcome. The outcome should also be considered over the child’s whole growth and development, not only for a 1-year follow-up period. This measure of outcome has to include obtaining as much information as possible about the natural history of the condition. Measuring outcome is an area that will require much work in the future, but it is crucial if the treatment algorithm for the gait impairment secondary to CP is to improve in a way that is documented.

Cases

Case 1 Nikkole

Nikkole, a 4-year-old girl, was evaluated with the concern that she was having trouble controlling her feet. According to her mother, she had made good progress in her walking ability in the past 3 months. Her hip radiographs were normal. She was continued in her physical therapy program to work on balance and motor control issues. Her mother was taught how to use walking sticks to help Nikkole with motor control and balance development. She continued to make good progress until age 6 years, when she plateaued in her motor skills development. At that time she had a full evaluation. On physical examination she was noted to have hip abduction of 25 and hip internal rotation of 70 on the right and 78 on the left. Hip external rotation was 5 on the right and 12 on the left. Popliteal angles were 65 on the right and 73 on the left. An

F. Miller Flexion 115.1

Knee Motion

50.0

Extension –16.9 Dorsiflexion

23.4

Ankle Motion

0.0 Right

Plantarflexion –26.5

Left

Fig. C1.1

Ely test was positive at 60 . Extended knee ankle dorsiflexion was 8 on the right and 10 on the left. Flexed knee ankle dorsiflexion was 5 on the right and 3 on the left. Observation of her gait demonstrated that she was efficient in ambulating with a posterior walker. However, she had severe internal rotation of the hips, with knee flexion at foot contact and in midstance, and a toe strike without getting flat foot at any time. The kinematics confirmed the same, and the EMG showed significant activity in swing phase of the rectus muscles. There was minimal motion at the knee with ankle equinus and lack of hip extension and internal rotation of the hip (Fig. C1.1). She had femoral derotation osteotomies, distal hamstring lengthenings, and gastrocnemius lengthenings. A rectus transfer was also recommended, but because of the fear of causing further crouch, she did not receive this procedure. Following the rehabilitation, she was taught to use Lofstrand crutches, with which she became proficient. Her main problem after the rehabilitation was a severe stiff knee gait, but because of the trauma of the (continued)

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surgery, neither she nor her mother was willing to have another operative procedure unless it was absolutely needed; they felt she was doing much better and they were happy. This case is also a good example of a family that is happy because of the excellent gains, even though the surgeon would grade this outcome as disappointing because of the severe stiff knee gait, which should have been treated at the initial procedure.

Cross-References ▶ Dystonia and Movement Disorders in Children with Cerebral Palsy ▶ Gait Analysis Interpretation in Cerebral Palsy Gait: Developing a Treatment Plan ▶ Muscle Performance in Children and Youth with Cerebral Palsy: Implications for Resistance Training

References Bowen TR, Lennon N, Castagno P, Miller F, Richards J (1998) Variability of energy-consumption measures in children with cerebral palsy. J Pediatr Orthop 18:738–742 Bowen TR, Miller F, Mackenzie W (1999) Comparison of oxygen consumption measurements in children with cerebral palsy to children with muscular dystrophy. J Pediatr Orthop 19:133–136 Boyd R, Fatone S, Rodda J (1999) High-or low-technology measurements of energy expenditure in clinical gait analysis? Dev Med Child Neurol 41:676–682 Davids JR, Rowan F, Davis RB (2007) Indications for orthoses to improve gait in children with cerebral palsy. J Am Acad Orthop Surg 15:178–188

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Gorter JW, Ketelaar M, Rosenbaum P, Helders PJ, Palisano R (2009) Use of the GMFCS in infants with CP: the need for reclassification at age 2 years or older. Dev Med Child Neurol 51:46–52 Karol LA, Chambers C, Popejoy D, Birch JG (2008) Nerve palsy after hamstring lengthening in patients with cerebral palsy. J Pediatr Orthop 28:773–776 Lebiedowska MK, Gaebler-Spira D, Burns RS, Fisk JR (2004) Biomechanic characteristics of patients with spastic and dystonic hypertonia in cerebral palsy. Arch Phys Med Rehabil 85:875–880 Liggio F, Kruse R (2001) Split tibialis posterior tendon transfer with concomitant distal tibial derotation osteotomy in children with cerebral palsy. J Pediatr Orthop 21:95–101 Nene AV, Evans GA, Patrick JH (1993) Simultaneous multiple operations for spastic diplegia, outcome and functional assessment of walking in 18 patients. J Bone Joint Surg Br 75:488–494 Novacheck TF, Stout JL, Tervo R (2000) Reliability and validity of the Gillette Functional Assessment Questionnaire as an outcome measure in children with walking disabilities. J Pediatr Orthop 20:75–81 Palisano RJ, Hanna SE, Rosenbaum PL, Russell DJ, Walter SD, Wood EP, Raina PS, Galuppi BE (2000) Validation of a model of gross motor function for children with cerebral palsy. Phys Ther 80:974–985 Piccinini L, Cimolin V, Galli M, Berti M, Crivellini M, Turconi AC (2007) Quantification of energy expenditure during gait in children affected by cerebral palsy. Eura Medicophys 43:7–12 Schutte LM, Narayanan U, Stout JL, Selber P, Gage JR, Schwartz MH (2000) An index for quantifying deviations from normal gait. Gait Posture 11:25–31 Schwartz MH, Rozumalski A (2008) The gait deviation index: a new comprehensive index of gait pathology. Gait Posture 28:351–357 Woratanarat P, Dabney KW, Miller F (2009) Knee capsulotomy for fixed knee flexion contracture. Acta Orthop Traumatol Turc 43:121–127 Wren TA, Otsuka NY, Bowen RE, Scaduto AA, Chan LS, Dennis SW, Rethlefsen SA, Healy BS, Hara R, Sheng M, Kay RM (2013) Outcomes of lower extremity orthopedic surgery in ambulatory children with cerebral palsy with and without gait analysis: results of a randomized controlled trial. Gait Posture 38:236–241

The Evolution of Knee Flexion During Gait in Patients with Cerebral Palsy

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Reinald Brunner

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1544 Current Knowledge . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1546 Assumptions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1549 The Concept of the Development of Knee Flexion Gait . . . . . . . . . . . . . . . . . . . . . . . . . . . Starting with the Foot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Starting with the Knee . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Starting with the Hip . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1553 Limitations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1554 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1555 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1555

Abstract

Walking with flexed knees (knee flexion gait with foot equinus or crouch gait with excessive dorsiflexion) is a common problem in cerebral palsy. Treatment can be difficult and often with unsatisfactory outcome. Descriptive classifications at the moment are of limited help in understanding the pathology as the gait pattern may change over time. The gait disorder gives the impression of being mechanically influenced by spasticity. However, other dimensions such as developmental

R. Brunner (*) Children’s University Hospital Basel, Basel, Switzerland Basel University, Basel, Switzerland e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_221

retardation, body perception, and sensation, which are hard to quantify, may be equally relevant. When considering mechanical and nonmechanical factors, two vicious circles are identified which are mutually dependent; the progressive foot deformity driven by triceps overactivity and inappropriate loading and the knee and hip flexion deformity driven by the hamstrings. The triceps surae is overly activated to provide for more stable knee extension. If knee flexion occurs, even in spite of the triceps activity, control of the center of mass is used to divide up the load between knee and hip extensors in order to avoid collapsing. Both hip and knee joints require extensor activity to maintain upright body posture and support body weight when the hamstrings extend the 1543

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hip in a flexed knee position, vasti counteractivity is required, which leads to longterm failure of the knee extensors by over stretching. These accommodations lead to a perception of insecurity in body support in stance further increasing the activity of the muscles involved and of increasing muscle tone in general reflected as spasticity. This concept including the psychological reaction of the individual offers a wider insight into the biomechanical causes of the individual pathology, which may provide for better treatment and prophylactic strategies. Keywords

Cerebral palsy · Etiology · Gait pattern · Functional equinus · Crouch gait · Flexed knee gait

Introduction Inadequate knee extension during stance phase in gait is a frequent problem in patients with cerebral palsy. Therapists as well as orthopedic surgeons are confronted with this problem, which affects endurance and walking distance and increases the energy cost of walking. The overload of the knee extensors may finally lead to a high-riding patella and knee pain with the danger of significant loss of gait function. Commonly patients are seen by specialists only when the deformity has become severe and starts to affect function. Understanding why and how the patient has arrived at this point is difficult at the time of severe functional loss but crucial to separate primary from secondary deformities in order to avoid further deterioration and enable efficient treatment. The main source of structural and functional deformities is generally seen in the spasticity which is present in the majority of patients with cerebral palsy and similar syndromes. For this reason, spasticity is treated aggressively. The aim besides functional improvement is prevention of further secondary deformities. The connection “spasticity equals functional impairment” becomes the logical assumption as spasticity is visible and the functional impairment obvious.

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There is little doubt that many patients improve when spasticity is treated and many become loss function, but even improvement per se is a weak argument for choosing a treatment option as it does not exclude that there may be superior treatment options. Egger’s hamstring transfer (Eggers 1952) led to improvement as the patients maintain walking but at the cost of dynamic knee flexion and gait velocity. Intramuscular hamstring lengthening as done later maintained the joint motion at the knee and resulted in a superior functional improvement. Gait pathologies in cerebral palsy have been classified according to the kinematic pattern which was present at evaluation without consideration of how the deformity developed. In diplegics, Sutherland and Davids (1993) described the jump knee, recurvatum, crouch, and normal gait which was again used by Lin in 2000 (Lin et al. 2000). For hemiplegics, Winters Jr et al. (1987) brought up a classification in 1987 which was modified and supplemented by Rodda and Graham (2001). These classifications defined clinical entities and aimed to improve decisions for treatment. The idea is that patients with similar kinematic deformities would require similar treatment. However, these classifications consider only the present situation and neglect the fact that the past history and future evolution of the deformity may vary greatly and may diverge significantly between patients who are similar at one instance in time. Therefore, different long-term outcomes should be expected after similar treatment. This problem was a conclusion of a review on classifications of gait patterns by Dobson et al. (2007). The description on gait evolution in CP is usually restricted to details (such as RoM) and is distorted due therapeutic means such as orthotics and surgery (Johnson et al. 1997; Wren et al. 2005). A comprehensive concept on the evolution of gait in cerebral palsy is still missing. There is a tendency to focus on the obvious mechanical problem and to solve this problem to attain functional improvement. One of these consequences is treating muscle imbalance or spastic muscle activity. However, further investigation for the etiology of inadequate muscle

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activity or other whole patient factors are seldom considered. It has taken a long time since the description of the obvious motor disorder until there was acceptance that these patients also have a deficiency in sensory function. Already in 1956, Minear stated the neglect of the sensory system by therapists (Minear 1956). Nasher in 1983 described the deficits in sensory organization and muscle coordination mechanisms (Nasher et al. 1983). More modern investigations found sensory impairment in all children (Hoon Jr et al. 2009). Although reports on sensory dysfunction have a long history (Kenney 1963; Tachdjian and Minear 1958) and a connection to motor dysfunction was seen (Nasher et al. 1983; Bleck 1975; Bobath 1967), only in 2005 was the sensory dysfunction included in the definition of cerebral palsy. Functional MRI with tractography regularly depicts the deficits of the sensory connections in the brain (Trivedi et al. 2010). Considering the fact that feedback is a prerequisite for adequate motor function, it becomes clear that a lack of sensory information will make an appropriate motor response difficult. Thus at least some part of the motor dysfunction can be explained by the sensory affection. This link may be obvious but often is not considered especially during orthopedic treatment, which remains mechanically based. While approaching understanding of the function of an individual as a whole, another level needs to be considered which is even more difficult to assess. The psychological reaction of the individual to his or her dysfunction also impacts the motor function. The patient realizes the difficulties for walking which appear especially when weight bearing. This perception affects the motor response and thus motor function similar to how stress or emotion affects people without CP. The presence of stress has been proven for very young patients with cerebral palsy (Zhao et al. 2015). The result is a rise of tone (Boman 1971). Other sensory deficits such as visual, auditory, or equilibrium impairment further contribute to the reaction of the individual and have an influence on motor development and function (Salavati et al. 2017; Rine et al. 2000). Treating patients with such complex disorders as cerebral palsy requires a global vision

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of the individual, and treatment should be adapted accordingly. In addition and may be secondary to the sensorimotor deficit, many patients show developmental motor control retardation which means that they remain in a state of immature motor control throughout their lifetime. The typical posture control for toddlers with increased plantarflexor activity and more knee extension in stance (Okamoto et al. 2003) can be seen at a higher age and is not necessarily a result of spasticity. Thus functional problems in these patients need to be seen in a much larger context and cannot be restricted to biomechanics and muscle function or to spasticity. Crenna et al. in 1998 already concluded that the gait pattern is due to a large mix of factors including even developmental retardation (“immature concepts”) and even “nonneural components” (Crenna 1998). Other authors have come to a similar conclusion before (Sutherland and Olshen 1988; Bleck 1981; Sussman 1992), but treatment concepts still lack the inclusion of an overall view of deficits in these children. The global motor output is not only a result from sensory input and reactive motor action but also modulated by the perception and subjective psychological reaction of the individual. This last point hence needs to be included for a comprehensive assessment. One major issue is the fact that the deformities, like motor control alterations, are directly and causally linked with the brain damage in cerebral palsy. A foot equinus in these patients is a spastic equinus; knee flexion in stance is seen as a result of spasticity of the hamstrings. However, biomechanics do not differ between various basic diseases: a flexed knee under load requires knee extensor activity independent from its cause, and bending forward reduces this load at the cost of hip extensor activity including the hamstrings. Similar required compensations are required for the nervous system due to the brain damage; however, little credit is given for adequate normal reactions and functions. In functional patients (GMFCS I–III), however, there is no reason why reactions should be different from patients with equal functional problems but with another basic disease. And this further applies to psychological reactions such as stress or fear.

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The characteristics of cerebral palsy affecting function are: – Weakness – Inadequate muscle activity (including spasticity, ataxia, and dystonia) – Reduced motor control and muscle innervation – Reduced sensory function (including proprioception) – Impairments of other senses (vision, hearing) – Difficulty to control posture – Perception of insecurity There are a lot of other diseases which at least partly show similar problems – and similar reactions. In the present chapter, I bring together puzzle pieces from all sides. The problem of proof, however, remains immanent as measuring tools for certain neurological domains such as sensation or developmental age are still lacking and depend on collaboration of the child. Even more difficult is an assessment of psychological factors, especially in these patients who never experienced the real normal situation for comparison. Nevertheless, it is possible to set up a concept which helps in clinical practice and even may introduce prophylactic options. The questions are: – Are pure biomechanical problems a possible reason for the gait disorder? – Where does sensory dysfunction come into play? – What is the role of spasticity?

Current Knowledge The possibility to stand on one leg without collapsing is essential to bring the opposite leg forward. One precondition is dynamic stability of the stance leg which means the optimal balance of shank position and knee (and hip) flexion, the necessary neuromotor control, and muscle strength. The more joint flexion, the higher is the demand on motor and sensory control and strength. The second precondition is the feeling of stability: moving forward becomes

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difficult without the confidence of stance stability. The plantarflexors, especially the triceps surae muscle, produce knee extension under load. This physical connection requiring a strong plantarflexor moment has been described as the plantarflexion-knee extension couple (Zajac and Gordon 1989; Gage 2004; Brunner et al. 2008). It was recently calculated as the plantarflexion-knee extension (PFKE) score (Sangeux et al. 2015) as the plantarflexor moment, not the movement, is relevant. This mechanism is important to maintain knee extension and thus contributes essentially to the dynamic stability. Excessive Achilles tendon lengthening in cerebral palsy is known to risk crouch gait where the balance of joint flexion, motor control, and strength is not maintained anymore. Overactivity of the triceps surae muscle and equinus foot position is commonly ascribed to spasticity and brain damage in patients with cerebral palsy. Treating spasticity thus seems the logical conclusion in order to prevent and correct a functional or structural equinus. It may be an unconventional idea, but there may be a reason for increased plantarflexion apart from spasticity. It has been shown that patients with muscle weakness for any reason more frequently show premature triceps activity than patients with strong muscles (Schweizer et al. 2013b). Inadequate and premature muscle triceps activity was also found in non-neurological conditions (Brunner and Romkes 2008; Klyne et al. 2012; Mokhtarzadeh et al. 2012; Sutherland et al. 1981). These reports suggest that the triceps surae muscle is used to control the knee extension (avoid flexion) in case of weakness or conditions lacking stance stability. Weakness is a general and well-known problem in cerebral palsy, besides spasticity, and weakness alone can cause triceps overactivity as in Duchenne muscular dystrophy. Indeed patients with this disease show the same changes in gastrocnemius and tibialis EMG during gait (Sutherland et al. 1981) (Fig. 1). Similarly, this muscle group gets short over time. Another important point is motor development. In toddlers starting to walk, prolonged triceps activity has been described (Berger et al. 1984). This toddler pattern

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Fig. 1 (a) Superficial raw EMG data of gastrocnemius medialis and tibialis anterior of a patient with Duchenne muscle dystrophy and (b) the same of the affected leg of a patient with spastic hemiplegic cerebral palsy. Both examples show the same pattern: onset already in terminal swing and prolonged gastroc activity in stance together with a shutoff of the tibialis anterior in terminal swing

of EMG activity is lost during maturation of gait. Patients with cerebral palsy, however, remain longer in early developmental stages due to motor retardation. The delay of developing control and confidence in stability may even be reflected in the spontaneous improvement of spasticity after the age of 4 (Hagglund and Wagner 2008). They usually show difficulties to control posture during one-leg stance, even at an age of 10 years or later. Unfortunately, a standardized assessment of the age of motor development in such detail and this respect is not available. Developmental motor retardation thus may be another factor for triceps overactivity besides spasticity. The constant overuse finally results in equinus deformity as it has been described for non-neurological conditions as well (Brunner and Romkes 2008). Due to poor muscle control and spasticity, these patients are more prone to develop early and severe equinus. These considerations show spasticity as one factor only among others. Today in contrast, spasticity is

well accepted as the main cause of triceps surae overactivity and equinus in cerebral palsy. On the other hand, this concept is still not more than another hypothesis which still lacks the causal evidence. Spasticity is defined as an increased resistance against fast movements on a neurological base, apart from muscle contractures. This resistance can present as hyperreflexia (clonus, assessed by the Tardieu test) (Haugh et al. 2006) or as stiffness (Ashworth test) (Pandyan et al. 1999) with less dynamical movements. Spasticity is seen as a consequence of the lesion of the upper motoneuron similar to paraplegia. This concept applies well for hyperreflexia with lacking inhibition of the muscle tendon reflex, but the general increase of tone is not fully understood. Emotions and stress increase spasticity LIT (Boman 1971), especially of the stiff type. Another possible reason can be seen in the feeling of being unsafe which can be considered as stress to keep posture:

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if the stance leg is dynamically unstable or felt to be unstable, swinging of the opposite leg gets difficult. The stance leg hence is stiffened up in order to avoid loss of posture, and the contralateral leg is held extended in order to be ready to accept loading in case of failure of the stance leg. The greater the sensation of instability, the more the patients stiffen up. Indeed, patients with such problems are unable to stand on one leg, the prerequisite for performing an adequate step. Indeed they relax when they perform the same test held by a trusted person. Spasticity on the other side is appealing to explain gait deformities as overactive reflexes inhibit the appropriate joint position. However, walking with reflexes is normal (Duysens et al. 2000; Dietz et al. 1990a, b; Hodapp et al. 2007a, b; Misiaszek 2003; Misiaszek et al. 1998), and the reflexes are well controlled which requires adequate sensation and muscle strength. Patients with cerebral palsy lack adequate sensation, motor control, and strength. This condition can serve as one explanation why compensatory muscle activity, such as the triceps surae muscle, runs out of control, but they do not exclude other possible causes. The triceps surae muscle pulls the patient up on the toes. The reduction of floor contact area and the increasingly flexed position reduces dynamic stability which is perceived by the patients. The result is a rise of tone which is interpreted as (an increase of) spasticity. Hence patients with cerebral palsy are unstable on their legs which may at least to some part explain the stiffness during gait. Toe walking, true (due to foot equinus) or apparent (due to excessive knee flexion at initial contact), is common in bilaterally involved patients with CP. A mild circumduction of the leg in swing is normal. Patients with hemiplegic CP can rely on the stability of the healthy leg and thus have time to prepare the hemiplegic foot for floor contact. In bilateral involvement, however, the leg in stance is less stable due to poor control, especially if it gets into a flexed position. The time for swing of the contralateral leg shortens as a consequence the toes touch due to inadequate knee extension. Initial toe contact due to true or apparent equinus during circumduction pushes the foot in extrarotation with every step. The

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consequences are a flat foot deformity, hallux valgus, and finally an increased external tibial torsion as a reaction of the torque with every step. Jim Gage has raised the term “lever arm disease” for bony malalignment in patients with cerebral palsy which comprises rotational deformities at the hip and tibia and their combinations. It applies to the foot deformity as well: the midfoot break combines external rotation with talar head loading instead of the first metatarsal head which both reduces the mechanical advantage for the triceps surae (Gage and Novacheck 2001). Like pathological external tibial torsion, the foot deformity as well can significantly interfere with the physical advantage of the plantarflexors to control knee extension in mid stance. The second important mechanism to control knee extension is the knee extensors. Roughly 80% of this muscle group is monarticular (vasti) and 20% biarticular (rectus femoris). The vasti are reported to lose efficacy the more the knee reaches extension (Tredinnick and Duncan 1988). Trunk forward lean can reduce the load on the knee extensors (Perry 1992) but requires control by the hip extensors including the hamstrings. Especially in case of weak glutei, the hamstrings are used for compensation. The hamstrings are potent hip extensors but flex the knee as well especially when the knee already is in flexion. Co-contraction between hamstrings and vasti is required in this situation, to help extension at both joint levels (Frigo et al. 2010). Again adequate strength of the knee extensors is required which often is lost due to overlengthening demonstrated by the high-riding patella. The hamstrings are often seen as the main cause of knee flexion in spastic gait disorders as they are found to be short during clinical examination. Musculoskeletal modeling in contrast showed long hamstrings in 80% of patients during crouch gait (Delp et al. 1996). In spite of reports of improved knee extension after hamstring surgery, there is increasing doubt on the effect of this intervention (Adolfsen et al. 2007), and bony surgery is preferred nowadays to hamstring lengthening for recovering knee extension. Indeed botulinum toxin injection or lengthening of the hamstring does not reliably increase knee

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extension, especially not at initial contact (Desloovere et al. 2007; Ackland et al. 2012; Rutz et al. 2010), but has the side effect of an increased anterior pelvic tilt with a consecutive hyperlordosis. This again draws doubt on the reason for hamstring surgery. This is in contrast to the understanding from normal swing phase control where the hamstrings are active in the late swing to avoid knee hyperextension (Arnold et al. 2007).

Assumptions Tone depends on the feeling of stability and feeling at general ease. It is normal that difficult conditions (danger of fall, stress, etc.) make the muscle tone rise. Patients with cerebral palsy are weak and have a lack of posture and motor control and a deficit in proprioception. It should be assumed that they will feel unsafe especially with dynamic motor function. This assumption is almost impossible to prove as there is no objectivity in feelings, and a subjective judgment is only possible if the whole scale was experienced before. Unfortunately, there is hardly any literature on the effect of stability or providing stability on spasticity. Only the paper of Dreher et al. (2013) describes a reduction of muscle tone after correction of the biomechanical condition by a multilevel surgical approach but an increase of tone again over time with deterioration of gait (Dreher et al. 2013). It can be further assumed that a reduction of the loaded area (such as in toe walking) and a reduction of the functional muscle moment due to weakness and/or to a loss of lever arm challenge posture control. The result is an even greater problem with postural stability which leads to an increase of tone again. The less equilibrium and postural control are developed – and developmental motor retardation is a typical concomitant symptom of cerebral palsy – the more the patient feels unsafe. This may be the reason for the usually greater stiffness in more affected tetraparetic patients with a lack of trunk and head control than in diplegics or even hemiplegics. This hypothesis is confirmed by the study of Schweizer et al. where weakness turned out to be the

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deteriorating factor for gait reflected by the GPS in all patients and spasticity only in tetraparetic patients (Schweizer et al. 2013a).

The Concept of the Development of Knee Flexion Gait Knee flexion gait is frequent in bilaterally involved patients with cerebral palsy. It may be combined with excessive plantar or dorsiflexion and hip flexion. There are several mechanisms leading to knee flexion during gait, and they can derive from any lower extremity joint (Fig. 2).

Starting with the Foot One cause of true equinus is weakness and another is poorly controlled reflex activity. A patient feeling of body insecurity leads to more plantarflexor, as a way of providing more stability in the lower extremity with more knee extension. Usually this activity starts in terminal swing probably to prevent the knee from flexing after initial contact. An increase of muscle tone stiffens up the foot in the equinus position which is another reaction of inadequate balance control. Plantarflexor hyperactivity may help initially, as long as there is no or only mild equinus which can be compensated by knee hyperextension. As this response becomes more severe, toe walking further reduces stance stability. The patient needs to balance the center of gravity over a much smaller loading area positioned in the forefoot, which leads to a position with flexed knees and hips. More plantarflexor hyperactivity is produced which increases this vicious circle. Loading the foot only on the toes is poorly tolerated by the soft and growing foot. Muscle control to balance forces within the foot is not yet developed due to developmental motor control retardation and the muscle weakness typical for cerebral palsy. Ligaments give way; bones adapt to this new foot collapsed posture. Under pressure (dorsolateral), bone growth is reduced, whereas under tension, it is stimulated (medioplantar) (Wolff 1899). The result is a foot deformity

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Evolution of knee flexion during gait inpatients with cerebral palsy Weakness

Equilibrium

Sensation of instability

other

Retardation

Triceps hyperactivity Tone rise Rigid spasticity

Equinus

Knee flexion at term. swing Toe walking

Knee hyperextension

Foot deformity

Unstable contralat. leg

Functionally weak triceps

Knee flexion

Knee extensor overload

Trunk inclination Trunk inclination

Hip flexion

Hip extensors include hamstrings

Fig. 2 Schematic depiction of the evolution of the various gait patterns in cerebral palsy. The igniters are in the top row (weakness, poor equilibrium, motor and mental retardation, and other factors) which produce the perception of

instability and insecurity. As a consequence, the individual seeks for stability what drives the locomotor system into deformities and functional disorders (see text)

known as midfoot break which shortens the lever arm for posture control by abduction/external rotation with floor contact mainly occurring at the head of the talus in severe cases. In this situation, the knee extensors need to take over control of knee extension, which means forced extension of an extended knee under load. Even strong knee extensors often fail as the muscle moment decreases dramatically in knees with near full knee extension even in normal people (Tredinnick and Duncan 1988). This decreased moment arm at terminal knee extension is magnified further by weakness which occurs in cerebral palsy. This combination of weakness and reduced moment arm causes the knee to fall into flexion. In order to reduce the load on the knee extensors, the patient uses the anterior displacement of the center of mass by leaning the trunk forward and the moment of inertia from the forward motion. This movement of the center of mass forward of the knee joint axis requires hip flexion which has to be controlled by hip

extensors. Keeping the ground reaction force in front of the knee creates an extension moment at the knee at the cost of hip flexion. The hip joint has to be held fixed without motion which locks the trunk to the thigh. Now as the CoM moves forward faster than the knee during stance phase of gait, knee extension occurs. These mechanisms require hip control by the hip extensors. The primary hip extensors are the glutei which are often weak, and therefore more demand is placed on the hamstring function as hip extensors. The hamstrings control hip flexion under load and extend the hip; however, the knee should be extending at this moment. There is no way to avoid concomitant knee flexion force by the hamstrings when they are recruited to be hip extensors. The more knee flexion is present, the high the moment arm for knee flexion force from the hamstrings, and knee extensor activity is required not only for knee extension but also to shift the effect of the hamstrings to the hip by locking the knee. Co-contracture in this situation hence is not

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pathological but required and indicates adequate motor control (Frigo et al. 2010). The high incidence of high-riding patellae found in these cases shows that this load on the knee extensors is too high for the anatomical structures. They constantly act against gravity, acceleration to flexion, and the knee flexion force of the hamstrings. While the active mainly biarticular hamstrings can work on the hip and thus have a possibility to shorten when contracting, this is not true for the mainly monarticular knee extensors; they are stretched in spite of their contraction which leads to long tendinous structures. First, the hamstrings indirectly help with knee extension by blocking hip flexion; however, as the condition worsens, the hamstrings become progressively more effective knee flexors, and knee flexion during stance phase of gait increases. The second vicious circle between knee extensors and hamstrings is established. The more severe the stance phase knee flexion becomes, the more unstable the posture becomes. This increased feeling of instability leads to more plantarflexor activity again and turns on the vicious cycle creating progressive collapse of the foot in planovalgus. This concept shows the role of the foot deformity and the deterioration of posture over time. It further explains the increase of tone seen as spasticity. Initially, however, spasticity is not the igniter of the pathophysiological chain, but poor control and spasticity help and accelerate the process of deformities. This fits again with the finding of Schweizer et al. where weakness was more important than spasticity for poor gait pattern (Schweizer et al. 2013a). The constant use of the muscles at an inadequate length finally results in contracture or overlengthening. If the patient is seen clinically for the first time in this situation, a correct interpretation of the causal links may be difficult. As spasticity and contractures are predominant symptoms at this moment, linking these two findings causally may be obvious. In contrast to the tonic spasticity which rises in situations of stress and feeling unsafe due to lack of adequate posture control, the hyperreflexic type of spasticity may remain unchanged but also contributes to foot deformity

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by the increased force on a poorly positioned foot with poor motor control. The initially increased plantarflexor activity results in a foot deformity, usually of the midfoot break- type, which secondarily causes knee flexion and compensatory hamstring overactivity. The hamstrings become structurally short and require co-contraction of the knee extensors which finally fail. The increased effort required to maintain posture results in an increase of tone which again deteriorates function at foot and knee level. The result is increasing knee flexion (with secondary hip flexion in order to avoid falling) and foot deformity. Another possibility is increasing equinus which leads to compensatory knee hyperextension. With increasing plantarflexion deformity, with or without knee hyperextension, the support area shifts to the forefoot, and the patient needs to flex forward to maintain equilibrium. The consequence is hip flexion which once fixed requires compensatory knee flexion. The consequence for posture control is the same as described for the midfoot break deformity: knee extensor and hamstring co-contraction is required to control the knee and hip. Knee flexion and hip flexion as a consequence cause functional shortening of the leg in stance with the difficulty for the swing leg: more flexion is needed to let the leg pass by, and more time is required to extend the leg for initial contact in a situation of reduced dynamic instability of the stance leg due to the flexed position. The result is an inadequate knee extension of the swinging leg at terminal swing and initial contact. The deformity may arise on the opposite leg as well. This cycle by itself is not pathologic: it can be observed as a typical reaction of people walking on difficult surfaces. However, in patients with cerebral palsy, it becomes chronic due to poor motor control, sensory lack, poor reflex or tone control, and fix deformities that develop.

Starting with the Knee Knee flexion and crouch gait can also develop on the basis of a primary knee flexion deformity,

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either in mid/terminal stance or in terminal swing. Knee flexion in stance may be caused – apart from local ligament or bone pathologies – by either failure of the knee extensors or by overactive or structurally short hamstrings. Contemporary movement of extension at the knee and flexion at the hip may even override their tolerance to stretch velocity and result in a spastic contraction. Knee flexion results in knee extensor overload. Two mechanisms can be used for compensation of increased knee flexion. The individual can profit from forward transposition of the CoM and from moment of inertia with the higher forward velocity of the CoM compared to the knee joint which again requires extensor activity at the hip in part by the hamstrings. The second mechanism is to use more plantarflexor activity and thus secondary equinus. The first mechanism is prone to hamstring contracture and knee extensor overlengthening and the second to plantarflexor shortness and foot deformity, usually a midfoot break. As a consequence, the plantarflexors fail to help with knee extension, and more tone or plantarflexor activity only increases the foot deformity. In addition, the hip needs to flex to keep balance which again requires hip extensor activity to stabilize the hip joint. The insufficient knee extension at terminal swing results in an initial toe contact. This inverse initial loading of the foot combined with the physiological circumduction drives the foot into the flatfoot-midfoot break deformity as the toes are in floor contact, while the hindfoot still moves medial. The plantarflexors lose their contribution for knee extension and the knee flexes as a consequence. Similar to the description earlier in this paper, hamstrings and knee extensors need to take over control on knee extension and hip position, with the final consequence of knee extensor failure and hamstring shortening. Both plantarflexors and the knee flexors controlling the leg in stance fail, which results in an increase of crouch, feeling of instability, and increased tone. Hip flexion is required to compensate for knee flexion in order to keep balance which may result in secondary contracture. However, it is usually less pronounced than the knee deformity. The foot, toe, and shank deformities are further consequences.

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Starting with the Hip The hip extensors control hip extension during loading response. Glutei and hamstrings have a synergistic activity at this moment. Hip extensor weakness thus may lead to hip flexion especially in early stance which requires compensatory knee flexion to avoid falling backward. This knee flexion occurs secondarily to the hip and is usually less pronounced. In case of gluteal weakness, the hamstrings become more important for hip extension. In case of concomitant knee flexion, the hamstrings flex the knee, and co-contraction of the knee extensors is required. As knee extensor muscle group is relatively insufficient close to full knee extension, there is a risk for failure which then requires hip flexion to compensate. Weakness and unsafe posture due to knee flexion stimulate the plantarflexors to help in knee extension. This mechanism, as described above, is prone to fail due to equinus and secondary midfoot break deformity. Again both vicious cycles, the one at the knee and the one at the foot, develop and result in severe crouch. Not in all cases it is easy to define the level of origin for the final crouch deformity. Possibly factors at several levels may play together, but always the same vicious cycles lead to deterioration. Especially once a severe deformity is present, all the contributing factors at all levels, primary and secondary, need to be tackled to correct the functional problem. The level of origin is of minor importance at this stage. In the beginning when patients are seen early, however, this concept may offer the clue to prevent deterioration. This concept, although hypothetical in some parts, helps to understand the problem of knee flexion and crouch gait in its complexity. The more the patient with equilibrium problems and weakness gets into a posture demanding good motor control and strength, the more the sensation of instability will result in stiffness seen as spasticity. The consequence is an increased stiffness during gait. Indeed knee kinematics show less dynamics and range of motion the more the knee is flexed under load. Spasticity increases and results in a stiff knee gait pattern. This concept does not, however, exclude that

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spasticity may be a problem in some patients. It explains the development of gait disorders on a biomechanical basis, and spasticity is regarded as a second problem apart from biomechanics. The approach of treating gait disorders by treating spasticity hence needs to be checked carefully. The biomechanical alterations may arise in spite of spasticity treatment as the need for orthopedic corrections after SDR indicates (LIT).

Conclusions Two mechanisms to control the leg are described in their interplay: the plantarflexors and the knee extensors. Either mechanism can fail which in the long run leads to failure of the secondary mechanism as well. The result is increasing knee flexion gait, often crouch. These considerations have direct clinical implications as they provide information on prophylaxis and treatment. It is crucial to avoid any foot deformity. If the patient is not capable to keep control on his/her foot, orthotics are required to take over. If the foot is deformed or malrotated, it should be redirected to align correctly with the knee and hip, either with conservative or operative means. It is easier to keep control of the foot if heel contact can be achieved. If an equinus position is necessary to get the foot aligned, it should be compensated by a respective adaptation of the orthosis or shoe. Any knee contracture needs to be prevented or treated. The first sign is a mild elastic resistance against full extension which usually can be overcome by a little pressure from the examiner. This resistance, however, will increasingly impede knee extension at terminal swing even in cases with long hamstrings. Capsular stretch may be the adequate answer in such a situation. If more severe, stretching becomes often unsatisfactory, and surgical correction is required. Often the hamstrings are accused as causing the knee flexion deformity. In these early cases, they are usually not very short but become so later. Lengthening thus may be indicated, but care should be taken not to interfere with their function as hip extensors. The capsular tightness

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often requires an additional supracondylar extension osteotomy to get full knee extension. Co-contraction around the knee joint is a necessary consequence of posture control in flexed knees and cannot be used as an indicator for poor control. If knee extension is restored surgically, a simultaneous correction of lengths of extensors and flexors may be required in addition. Another interesting point comes up when this pathomechanism is related to clinical findings: true biarticular muscles seem to have a tendency to become short in case of overuse (hamstrings, gastrocnemii), whereas monarticular muscles become long (knee extensors being mainly vasti and soleus). Whereas the short muscles may still be functional as shortness compensates for weakness, the monarticular long muscles lead to severe decompensation and deterioration of function. Thus especially overlengthening of the monarticular muscles needs to be avoided. The biarticular muscles in contrast work on a second joint level where force can be transferred to. For this reason, they do not undergo stretch during activity, which may be the reason why they have a tendency to become short. This description of evolution of crouch gait passes through all gait patterns described by Sutherland as well as through stiff knee gait. As the evolution is very individual, a given patient may remain constant with his pattern. However, it is known that usually evolution leads to deterioration, and the final result is walking with bent knees. Thus the gait pattern may change for the individual patient with time. Although the evolution of the flexed knee and crouch gait described in this paper is not proven as a continuous chain of events, the known puzzle pieces set together indicate the probability of this concept. It describes the possible progression and its consequences and thus allows for earlier therapeutic strategies for prevention as well as a change of treatment. Equinus in this context is an overestimated problem as there is a tendency to develop a plantigrade foot with time. Main factors are equilibrium and balance control which both require more attention in treatment and assessment techniques, and developmental motor retardation is the other underestimated issue. Both result in a reduced

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dynamic stability in stance which is perceived and leads to an increase of tone. The result of all these pathogenic factors is an inadequate position of the body segments under load, which requires more efforts for gaining dynamic stability and for movement (Steele et al. 2010). Strengthening programs is one step in this direction of treating the basic problems, but working on balance reaction and motor development should become a major part in the rehabilitation program as well. Variable and demanding activities (climbing, jumping, etc.) may help as far as feasible based on the individual patient. Orthotics and surgery are indicated to help in these problems as they can provide stability but must not hinder dynamics in function if controlled by the patient and the developmental process. The main aim in this context is to optimize the biomechanical conditions of the locomotor system. Another consequence is the need for better diagnostics: gait analysis up to date provides only rough information on muscle function. It derives from computation of kinetics and usually surface EMG of the larger muscle groups. Whereas kinetics only describe net moments (and hide co-contractions), EMG only shows activity of some muscles without a clear information on force or moment and a more thorough overview. Furthermore, even a passive muscle can have a functional impact by its resistance. More information on muscle function during gait is wanted. A sensory assessment especially in poorly collaborative patients is not available, and neither is an assessment for motor development in these cases. These neurological components, however, are essential for understanding the patient’s situation and for optimizing treatment. In the whole concept, spasticity has only a minor role. The sensory deficit, the locomotor retardation, and the psychological reaction of the individual have at least a similar importance. These considerations, however, influence our strategies of interventions on muscles, especially with concerns about muscle weakening. The main goal should be to improve mechanical stability which requires a biomechanically correct joint position and adequate strength. This situation

R. Brunner

needs to be perceived by the patient to have an effect on function and tone. A question mark needs to be put on muscle transfers as the altered action requires proprioception to benefit from motor reprogramming. On the other hand, bony corrections and orthotics, which allow for using more passive structures in a biomechanically more stable situation, seem to be more appropriate. Of course soft tissue surgeries with the same result lead to an improvement as well but at the cost of weakening.

Limitations There are clear limitations of this concept: the whole picture of the development of deformities over time is a combination of biomechanical and clinical puzzle pieces – other combinations may be possible. Some parameters such as feeling unsafe, lack of possible assessment, and developmental motor retardation can be evaluated only very globally. Dynamic instability can be tested, e.g., by letting the patient stand on one leg, but it is not quantified. Even more difficult is the quantification of motor control and development. These two factors are essential as they are one basis for the dynamic instability which causes the chain of compensations. This means that the whole evolution described in this paper starts with a weak point. Further, some references are marginally reliable but are the only ones covering a specific topic. A final proof of this concept using forward modeling is not possible either as only biomechanical consequences can be modeled but the brain reaction leading the compensations is not included. There are too many possibilities and all components may be of different expression. For a longitudinal study, there are too many possibilities, combinations, and even the lack of consequent development, and deterioration requires too large a cohort. Lack of clear evidence of some factors of this concept hence is an issue, but using it as a basis for clinical decision-making and treatment helps at least in our hands and offers possible ways of preventions. The patient should not be reduced to a biomechanical type of robot. We need to consider the patient with all his/her

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aspects although some remain scientifically too difficult to approach.

Cross-References ▶ Gait Analysis Interpretation in Cerebral Palsy Gait: Developing a Treatment Plan ▶ Hip and Pelvic Kinematic Pathology in Cerebral Palsy Gait ▶ Muscle Performance in Children and Youth with Cerebral Palsy: Implications for Resistance Training ▶ Musculoskeletal Physiology Impacting Cerebral Palsy Gait

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treatment in children with cerebral palsy. Dev Med Child Neurol 49:56–61 Dietz V, Discher M, Faist M et al (1990a) Amplitude modulation of the human quadriceps tendon jerk reflex during gait. Exp Brain Res 82:211–213 Dietz V, Faist M, Pierrot-Deseilligny E (1990b) Amplitude modulation of the quadriceps H-reflex in the human during the early stance phase of gait. Exp Brain Res 79:221–224 Dobson F, Morris ME, Baker R et al (2007) Gait classification in children with cerebral palsy: a systematic review. Gait Posture 25:140–152 Dreher T, Brunner R, Vegvari D et al (2013) The effects of muscle-tendon surgery on dynamic electromyographic patterns and muscle tone in children with cerebral palsy. Gait Posture 38:215–220 Duysens J, Clarac F, Cruse H (2000) Load-regulating mechanisms in gait and posture: comparative aspects. Physiol Rev 80:83–133 Eggers GW (1952) Transplantation of hamstring tendons to femoral condyles in order to improve hip extension and to decrease knee flexion in cerebral spastic paralysis. J Bone Joint Surg Am 34 (A):827–830 Frigo C, Pavan EE, Brunner R (2010) A dynamic model of quadriceps and hamstrings function. Gait Posture 31:100–103 Gage JR (2004) The treatment of gait problems in cerebral palsy. Mac Keith, Lavenham Gage JR, Novacheck TF (2001) An update on the treatment of gait problems in cerebral palsy. J Pediatr Orthop B 10:265–274 Hagglund G, Wagner P (2008) Development of spasticity with age in a total population of children with cerebral palsy. BMC Musculoskelet Disord 9:150 Haugh AB, Pandyan AD, Johnson GR (2006) A systematic review of the Tardieu scale for the measurement of spasticity. Disabil Rehabil 28:899–907 Hodapp M, Klisch C, Berger W et al (2007a) Modulation of soleus H-reflexes during gait in healthy children. Exp Brain Res 178:252–260 Hodapp M, Klisch C, Mall V et al (2007b) Modulation of soleus H-reflexes during gait in children with cerebral palsy. J Neurophysiol 98:3263–3268 Hoon AH Jr, Stashinko EE, Nagae LM et al (2009) Sensory and motor deficits in children with cerebral palsy born preterm correlate with diffusion tensor imaging abnormalities in thalamocortical pathways. Dev Med Child Neurol 51:697–704 Johnson DC, Damiano DL, Abel MF (1997) The evolution of gait in childhood and adolescent cerebral palsy. J Pediatr Orthop 17:392–396 Kenney WE (1963) Certain sensory defects in cerebral palsy. Clin Orthop Relat Res 27:193–195 Klyne DM, Keays SL, Bullock-Saxton JE et al (2012) The effect of anterior cruciate ligament rupture on the timing and amplitude of gastrocnemius muscle activation: a study of alterations in EMG measures and their relationship to knee joint stability. J Electromyogr Kinesiol 22:446–455

1556 Lin CJ, Guo LY, Su FC et al (2000) Common abnormal kinetic patterns of the knee in gait in spastic diplegia of cerebral palsy. Gait Posture 11:224–232 Minear WL (1956) A classification of cerebral palsy. Pediatrics 18:841–852 Misiaszek JE (2003) The H-reflex as a tool in neurophysiology: its limitations and uses in understanding nervous system function. Muscle Nerve 28:144–160 Misiaszek JE, Cheng J, Brooke JD et al (1998) Movementinduced modulation of soleus H reflexes with altered length of biarticular muscles. Brain Res 795:25–36 Mokhtarzadeh H, Yeow CH, Malekipour F, et al. (2012) Ankle plantarflexor contribution to knee joint loading and anterior cruciate force during single leg standing. 30th annual conference of biomechanics in sports, Melbourne Nasher LM, Shumway-Cook A, Marin O (1983) Stance posture control in select groups of children with cerebral palsy: deficits in sensory organization and muscular coordination. Exp Brain Res 49:393–409 Okamoto T, Okamoto K, Andrew PD (2003) Electromyographic developmental changes in one individual from newborn stepping to mature walking. Gait Posture 17:18–27 Pandyan AD, Johnson GR, Price CI et al (1999) A review of the properties and limitations of the Ashworth and modified Ashworth scales as measures of spasticity. Clin Rehabil 13:373–383 Perry J (1992) Gait analysis: normal and pathological function. SLACK, Thorofare Rine RM, Cornwall G, Gan K et al (2000) Evidence of progressive delay of motor development in children with sensorineural hearing loss and concurrent vestibular dysfunction. Percept Mot Skills 90:1101–1112 Rodda J, Graham HK (2001) Classification of gait patterns in spastic hemiplegia and spastic diplegia: a basis for a management algorithm. Eur J Neurol 8 (Suppl 5):98–108 Rutz E, Hofmann E, Brunner R (2010) Preoperative botulinum toxin test injections before muscle lengthening in cerebral palsy. J Orthop Sci 15:647–653 Salavati M, Rameckers EA, Waninge A et al (2017) Gross motor function in children with spastic cerebral palsy and cerebral visual impairment: a comparison between outcomes of the original and the cerebral visual impairment adapted gross motor function Measure-88 (GMFM-88-CVI). Res Dev Disabil 60:269–276 Sangeux M, Rodda J, Graham HK (2015) Sagittal gait patterns in cerebral palsy: the plantarflexor-knee extension couple index. Gait Posture 41:586–591

R. Brunner Schweizer K, Romkes J, Coslovsky M, Brunner R (2013a) The influence of muscle strength on the gait profile score (GPS) across different patients. Gait Posture 39(1):80–85 Schweizer K, Romkes J, Brunner R (2013b) The association between premature plantarflexor muscle activity, muscle strength, and equinus gait in patients with various pathologies. Res Dev Disabil 34:2676–2683 Steele KM, Seth A, Hicks JL et al (2010) Muscle contributions to support and progression during single-limb stance in crouch gait. J Biomech 43:2099–2105 Sussman MD (1992) The diplegic child evaluation and management: symposium Charlottesville, Virginia, Nov. 21–24, 1991. American Academy of Orthopaedic Surgeons, Rosemont Sutherland DH, Davids JR (1993) Common gait abnormalities of the knee in cerebral palsy. Clin Orthop Relat Res 288:139–147 Sutherland D, Olshen R (1988) The development of mature walking. Cambridge University Press, Cambridge Sutherland DH, Olshen R, Cooper L et al (1981) The pathomechanics of gait in Duchenne muscular dystrophy. Dev Med Child Neurol 23:3–22 Tachdjian MO, Minear WL (1958) Sensory disturbances in the hands of children with cerebral palsy. J Bone Joint Surg Am 40-A:85–90 Tredinnick TJ, Duncan PW (1988) Reliability of measurements of concentric and eccentric isokinetic loading. Phys Ther 68:656–659 Trivedi R, Agarwal S, Shah V et al (2010) Correlation of quantitative sensorimotor tractography with clinical grade of cerebral palsy. Neuroradiology 52:759–765 Winters TF Jr, Gage JR, Hicks R (1987) Gait patterns in spastic hemiplegia in children and young adults. J Bone Joint Surg Am 69:437–441 Wolff J (1899) Die Lehre von der functionellen Knochengestalt. Archiv für pathologische Anatomie und Physiologie und für klinische Medicin 155:256–315 Wren TA, Rethlefsen S, Kay RM (2005) Prevalence of specific gait abnormalities in children with cerebral palsy: influence of cerebral palsy subtype, age, and previous surgery. J Pediatr Orthop 25:79–83 Zajac FE, Gordon ME (1989) Determining muscle's force and action in multi-articular movement. Exerc Sport Sci Rev 17:187–230 Zhao X, Chen M, Du S et al (2015) Evaluation of stress and pain in young children with cerebral palsy during early developmental intervention programs: a descriptive study. Am J Phys Med Rehabil 94:169–175. quiz 176–179

Part XX Upper Extremity

The Upper Extremity in Cerebral Palsy: An Overview

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1560 Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1560 Normal Development of Function of Children’s Upper Extremities . . . . . . . . . . . . . . . . . . 1561 Classifying Upper Extremity Function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1561 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1562 Specific Treatments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1563 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1564 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1564 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1567 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1567

Abstract

Upper extremity functional impairment is a common problem in individuals with cerebral palsy. The level of the disability is very variable, with many individuals having functional use of the limb but with decreased dexterity. The Gross Motor Function Classification System (GMFCS) defines gross motor function as it primarily relates to the trunk and lower extremities. This does also relate to the upper extremity since the ability to do functional ambulation with an assistive device is also impacted by upper extremity function.

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_106

Manual Ability Classification System (MACS) is a functional assessment similar to the GMFCS for mobility but focused on upper extremity manual use. The MACS evaluates how the upper extremity hand functions for activities of daily living as well as for more sophisticated activities. It considers both hands as a unit; therefore, if one hand is very highly functional, the score may be very high even though one limb has virtually no function. The International Classification of Functioning, Disability and Health (ICF) defines the causes of a disability that would prevent a specific activity from being accomplished. Therefore, based on the ICF guidelines, a child may be able to feed themselves because of the physical impairment caused at 1559

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the level of body function and structure by not having an upper extremity or hand that is functioning at the level required for this activity. Furthermore, there may be individual personal factors such as lack of motivation as the cause of not self-feeding. The upper extremity disability in children with CP may have a significant impact on the individual’s participation and ability to complete activities. The goal of this chapter will be to provide an overview of the disability including expected natural history and a review of treatment options. Keywords

Cerebral palsy · Upper extremity · MACS · SHUEE · Surgery · Botulinum

Introduction Upper extremity functional impairment is a common problem in individuals with cerebral palsy. The level of the disability is very variable, with many individuals having functional use of the limb but with decreased dexterity. The gross motor function classification system (GMFCS) defines gross motor function as it primarily relates to the trunk and lower extremities. This does also relate to the upper extremity since the ability to do functional ambulation with an assistive device is also is impacted by upper extremity function. The upper extremity impact on function is especially significant for GMFCS III level mobility as well as those children who are high functioning GMFCS IV. Manual Ability Classification System (MACS) is a functional assessment similar to the GMFCS for mobility but focused on upper extremity manual use. The MAC scale system evaluates how the upper extremity hand functions for activities of daily living as well as for more sophisticated activities. It considers both hands as a unit; therefore, if one hand is very highly functional, the score may be very high even though one limb has virtually no function. The upper extremity disability and children with cerebral palsy (CP) vary widely based on the individuals need in the context of their environment.

F. Miller

The International Classification of Functioning, Disability and Health (ICF) defines the causes of a disability that would prevent a specific activity from being accomplished. Therefore, based on the ICF guidelines, a child may be on able to feed themselves because of the physical impairment caused at the level of body function and structure by not having an upper extremity or hand that is functioning at the level required for this activity. There may also be environmental factors that preclude selffeeding such as the lack of available proper utensils or food with a texture or structure that the individual can manage. Furthermore, there may be individual personal factors such as lack of motivation as the cause of not self-feeding. The upper extremity disability in children with CP may have a significant impact on the individual’s participation and ability to complete activities. The goal of this chapter will be to provide an overview of the disability including expected natural history and a review of treatment options.

Natural History The GMFCS level of motor involvement also tends to provide some definition of upper extremity involvement. GMFCS I often have hemiplegic pattern CP. The hemiplegic limb may have a very wide variation of motor disability from being a limb that has a little functional ability to a limb with only mild clumsiness. The most common pattern of GMFCS II level functional children diplegic pattern CP. The upper extremity in children with diplegic pattern CP tends to show mild motor disability. Many children have only mild to moderate fine motor difficulties with diplegia. GMFCS level III usually have very functional upper limbs. There are a group of children with very asymmetric diplegic pattern involvement also called triplegic who may have one limb with a significant motor disability. This triplegic pattern involvement often makes functional use of a mobility aid difficult unless the motor function is such that the individual can walk with a single crutch or cane. For

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GMFCS IV level of function, the often have most common difficulty is related to extremity motor problems limiting the use of an assistive device. Often, there is also a combination of trunk coordination problems as well as upper extremity function. GMFCS V level function is the individuals with the most severe upper extremity disability. It is very uncommon to have a GMFCS V level function with good upper extremity function, although there are children whose upper extremity provides very useful function for activities of daily living such as self-feeding and operating a power wheelchair.

Normal Development of Function of Children’s Upper Extremities Upper extremity spastic deformities start out as a clinched fist position with the thumb in the palm under the flexed fingers. This is an especially common posture in the affected limb of the child with hemiplegia but may also be seen with bilateral CP. As children grow, the fingers open first, and as more maturity and development occur, the thumb relaxes out of the palm. Often, in children with hemiplegia, the fingers are out of the flexed position by 2–3 years of age, and over the next several years, the thumb slowly relaxes. By 6–9 years of age, the thumb may be at the level of maximum abduction, and wrist flexion is becoming the predominant position. There is also significant elbow flexion with forearm pronation from early childhood. As children move through middle childhood and into adolescence, the elbow flexion and pronation often slowly decrease but almost never resolves completely but may become insignificant (Riad et al. 2007). By late childhood and early adolescence, the upper extremity deformity has developed the position it will maintain throughout the remainder of individuals’ lives, except some of the contractures such as the contracted finger and wrist flexors may slowly become more fixed and more severe. These progressive contractures seem to be more common in quadriplegia than hemiplegia. Throughout childhood, the

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evaluation of individual children has to focus on their current function, physical deformity in the context of their age, and cognitive abilities. There is a great lack of published data describing the natural development of upper extremity function in children with CP. There is a large interest in correlating the brain imaging (Rose et al. 2011; van der Aa et al. 2013) with later function, and there is a renewed interest in imaging as it relates to enforced use therapy (Cao et al. 2015; Sutcliffe et al. 2009). Although there are theories how this imaging will be able to direct therapy and long-term prognosis (Friel et al. 2014; Holmefur et al. 2013; Spittle et al. 2009), there is not any real live data with longterm follow-up through to maturity to confirm these assumptions.

Classifying Upper Extremity Function The MACS classification system of upper extremity function in children with CP has become the best recognized for overall monitoring. The disadvantage of this system is that it considers both hands as one unit and does not really separate the individual limb function. MACS is useful however to define overall upper extremity function in children with CP. It has good validity and reliability (Eliasson et al. 2006; Jeevanantham et al. 2015) Table 1. We have use an upper extremity functional rating system that is directed at individual limbs. The goal of this system is to define the function and problems related to the individual limb. This classification system has not been validated; however, we find it useful to assess a limb (Table 2). Another popular classification of function was published by House (House et al. 1981) related to function of the thumb and how the whole hand is utilized. The advantage of this classification is that it does focus on the individual hand (Table 3). Other measures of hand function include the Assisting Hand assessment (AHA) and Children’s Hand-use Experience Questionnaire (CHEQ) are instruments used to assess hand functional use

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Table 1 Manual ability classification system (MACS) Level I Level II Level III Level IV Level V

Handles objects easily and successfully Handles most objects but with somewhat reduced quality and/or speed of achievement Handles objects with difficulty, needs help to prepare and/or modify activities Handles a limited selection easily managed objects in adapted situations Does not handle objects and has severely limited ability to perform even simple activity

Table 3 House functional assessment Class 0 1 2 3 4

More detailed instructions are also available for down load: http://www.macs.nu/files/MACS_English_2010.pd

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Table 2 Upper extremity function

6

Functional type: A Extremity is not functional B Can use hand as a paperweight, pressure assist, or posting device; is able to swipe a toy and turn a switch on and off C Hand has mass grasp but poor active control D Hand has active grasp and release and can place an object with some degree of accuracy E Hand has fine pinch useful for holding a pen or pencil, has key pinch with the thumb F Normal function can be used for buttoning and shoestring tying, thumb has fine tripod opposition Within each type, also assess level of contractures: I. No contractures II. Dynamic contractures III. Fixed contractures

over time. These instruments seem to be most useful in the research environment since they are very time intensive to administer (Ryll et al. 2016). Further information on these instruments are available in ▶ Chap. 109, “Upper Extremity Assessment and Outcome Evaluation in Cerebral Palsy”. Another instrument which can be used for diagnostic purposes is the Shriners Hospital for Children Upper Extremity Evaluation (SHUEE). The SHUEE testing is helpful to discriminate specific body impairments at the level of the hand which impact functional use (Davids et al. 2006). In this way, it is helpful for planning surgical treatment, orthotic use, or specific therapy interventions (▶ Chap. 109, “Upper Extremity Assessment and Outcome Evaluation in Cerebral Palsy”).

Designation Does not use Poor passive assist Fair passive assist Good passive assist Poor active assist Fair active assist Good active assist

7

Spontaneous partial use

8

Spontaneous use complete

Activity level Does not use Uses as a stabilizing weight Holds object placed into hand Holds and stabilizes object for use by other hand Actively grasps object and holds weakly Actively grasps object and stabilizes well Actively grasps object and manipulates against other hand Carries out bimanual activities easily and occasional spontaneous use Uses hand independently

As reported in Upper Extremity Chap. by Kozen, Chap. 34, p. 773 based on (House et al. 1981)

Treatment Standard first-line treatment for upper extremity disability involves approaches using occupational therapy to encourage bimanual hand use in age-appropriate method (Charles and Gordon 2006). Constraint-induced movement therapy has become increasingly popular method to encourage the use of a unilateral CP upper limb. There are a wide variety of techniques including temporary splinting to complete long arm cast application of the well limb. Many short-term improvements have been reported (DeLuca et al. 2012); however, dosages and long-term impact are still unclear (▶ Chap. 179, “Constraint-Induced Movement Therapy for Children and Youth with Hemiplegic/ Unilateral Cerebral Palsy”). The use of botulinum toxin has also been extensively reported in children with CP. The functional improvement with injection in the upper limb includes many reports that document short-term benefit (Sanger et al. 2007; Satila et al. 2006; Satila et al. 2006). There is no data on the long-term benefit from the use of botulinum toxin

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in the upper extremity. There are other less commonly used methods to reduce muscle tone such as neurectomy and intrathecal baclofen (▶ Chap. 179, “Constraint-Induced Movement Therapy for Children and Youth with Hemiplegic/Unilateral Cerebral Palsy”). Surgical correction of the deformities has a long and well-established history. There are still however many variations in practice as it relates to surgical corrections of the upper extremity. When making surgical decisions and recommendations, it is very important to continue to consider the goals to be accomplished. It is important not to only focus at the impairment or body level dysfunction but one also needs to be very aware of the patient-specific goals for use of the limb. In the ICF model, we have to consider the personal factors. These personal factors which are very important are the goals of the patient specifically what the activity of functional use they would like to accomplish, are there cosmetic concerns as it relates to the limb, and most importantly does the patient have an interest in changing his current limb position or function. If the child is very young and these decisions are made by proxy of the parent or caregiver. An issue in considering upper extremity surgery should be whether the patient themselves can have input. This raises the question of when is the best age to consider operative surgical reconstruction. One train of thought is that surgery should be done young in 4 or 5-year-old children so they get the benefit of the reconstructed limb to maximize its function. Surgery in the young child risks a higher rate of recurrent deformity. Another approach is to wait until the individual is an adolescent and then involve the individual in the decisionmaking as to what they would like to have accomplished. Surgery at this age also appears to have less risk of recurrent deformity and therefore better long-term maintenance of correction. There are no studies currently that give guidance on the timing related to age, although I favor waiting until the individual child can assist in the decision-making.

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Specific Treatments The current standard of upper extremity surgery in children with CP is to make an evaluation of the whole limb and then combine all the surgical treatments at one setting. This is the single- event multilevel surgery (SEMLS) approach that has become the standard of care for the lower extremity and is now also the standard of care for the upper extremity (Smitherman et al. 2011) (▶ Chap. 112, “Single-Event Multilevel Surgery for the Upper Extremity in Cerebral Palsy”). Specific areas which one should consider for surgical correction include the shoulder and elbow. The most common shoulder problems are contractures that limit abduction and make activities of daily care difficult in patients mostly with GMFCS V level who require attendant care. Improvement in ease of care is greatly facilitated by the release of pectoralis contractures (Domzalski et al. 2007). The primary issue at the elbow is a flexion contracture mainly creating cosmetic concerns except in some children with GMFCS V develop severe contractures which make custodial care and dressing difficult. Release of the biceps or brachialis usually improves this contracture. Children with severe pronation may develop radial head dislocations which seldom become painful and the only treatment should be consideration of radial head resection at skeletal maturity if they are symptomatic (Abu-Sneineh et al. 2003) (▶ Chap. 113, “Shoulder and Elbow Problems in Cerebral Palsy”). Pronation contractures are most common in the distal forearm. These pronation contractures sometimes are helpful in that they allow the hand to be in a position where he can operate a keyboard. These pronation contractures sometimes are helpful in that they allow the hand to be in a position where he can operate a keyboard. Care should be taken to avoid placing the hand and forearm in the supinated position because this is both cosmetically and functionally much less appealing. Wrist flexion deformities and

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contractures are very common. The Green transfer (Green and Banks 1962) of the flexor carpi ulnaris to the extensor carpi radialis brevis continues to be a mainstay treatment for this very common deformity (Beach et al. 1991). For individuals who have a nonfunctional hand, the use of a wrist fusion to place the hand and an optimal cosmetic position also eases care especially for placing the limb in shirt sleeves. The wrist fusion should be avoided in individuals who are using wrist motion to facilitate finger grasp or finger release (Thabet et al. 2012). Thumb adduction is another common disability in the hand of the individual with CP. The thumb adduction may limit the ability to grasp objects in the palm or to use pinch grasp effectively. Most individuals with CP are able to use key pinch but not tripod pinch grasp because of the limited abduction motion present in the thumb. Thumb positions have been classified by House (House et al. 1981) with Type 1 being adduction with normal MCP and IP joints and Type 2 as adduction with flexion of MCP and IP joints that are not fixed. Type 3 has hyperextension of the MCP joint and abduction of the thumb, and Type 4 has a fixed flexion contracture of the MCP joint with severe abduction. Treatment of the thumb deformity requires releasing fixed contractures, stabilizing hypermobile joints, and augmenting weak muscles (▶ Chap. 114, “Forearm, Thumb, and Finger Deformities in Cerebral Palsy”).

Complications The main complication of treatment of the upper extremity in children with CP is that a lot of treatment may be given without much benefit. This is especially related to mild invasive treatments such as botulinum toxin, constraint therapy, and very intense therapy. Research needs to focus especially on the impact of these treatments longterm and also consider the negative impacts on the patient and family. The most major complications of surgical reconstruction are to not clearly defining the expected outcome goals to the patient and

F. Miller

the family; often there is a feeling that expected goals therefore were not achieved. A common scenario is the adolescent very much wants surgery to improve the cosmetic appearance of his hemiplegic limb; however, the parents’ goal is for the adolescent to start using the limb in a more functionally normal way. When this does not happen the parent is not satisfied but the adolescent is. Clearly, another complication risk with upper extremity’s surgery is recurrent deformity. This is especially likely in children who are very young when they have surgery. Another risk is overcorrection of a previous deformity. This is especially true for overcorrection of wrist flexion deformity which may then gradually become wrist extension deformity. Another, possible overcorrected deformity is forearm pronation especially when there is too much correction of the pronation, one can develop a supination contracture or deformity which makes families and patients very unhappy.

Conclusion The classic child with a spastic upper extremity, in whom surgical treatment is considered, has spastic hemiplegia causing posturing of the involved upper extremity with the elbow flexed, forearm pronated, the wrist and fingers flexed, and the thumb adducted and flexed in the palm. Children with movement disorders (athetosis or dystonia) may present with upper extremity involvement; however, surgical correction is rarely indicated. The greatest task is to clearly define the functional difficulties (if any), determine optimum goals for a specific child’s developmental stages, and bring together realistic long-term goals between patients, parents, and orthopedic surgeons. This task requires that surgeons understand the concerns of families and children especially the cosmetic concerns of the extremity and the specific functional concerns. Often, the concerns of patients, especially adolescents, are different from the concerns of parents. Also, orthopedists have to understand each component of the global

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Table 4 Upper extremity algorithm

Upper Extremity What is the pattern of involvement ?

Hemiplegia with good function in the contralateral limb

Quadriplegia with Quadriplegia with functional upper nonfunction extremities upper extremities What is the child’s age?

What is the child’s age?

8 years old

What is the goal?

What is the goal?

Functional gain Occupational therapy Any improvement in the past year?

YES Continue therapy

NO Consider surgery What is the specific functional problem?

Poor hand grip due to thumb in the palm  Thumb adductor release and possible web space Z-plasty

No grip due to wrist flexion  Transfer FCU to ECRB

Cosmetic improvement

Functional gain Carefully explain expectations

Has deformity changed in the last year?

YES Wait another year unless it is getting worse

Cannot see palm due to pronation  Pronator teres release or transfer

NO Child >5 years old ?

Does the child & family still want to proceed?  YES Reconstruction of specific problem, but do not ignore other clear contractures

YES Consider reconstruction of contractures.  Correct elbow flexion, pronation, wrist flexion, and thumb adduction

What is the child’s age?

Cosmetic improvement Consider reconstruction of all contractures  Correct elbow flexion, pronation, wrist flexion, and thumb adduction

NO Wait till >5 years old

(continued)

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F. Miller

Table 4 (continued)

Upper Extremity Quadriplegia (continued)

Quadriplegia with functional upper extremities

Quadriplegia with nonfunction upper extremities

What is the child’s age?

What is the child’s age?

8 years old

Focus on occupational therapy

Identify specific functional goal (often more than one)

With good OT program and no change in past year Identify specific problem  Consider reconstruction

Cannot bring hand toward face because of elbow & shoulder extension  Release of the triceps at the shoulder

Cannot reach out because of elbow flexion contracture  Lengthen Elbow flexors

Cannot hold object in hand because of wrist flexion and poor finger flexion  FCU transfer to dorsum of the wrist (Avoid fusion)

Cannot hold object because of thumb adduction contracture  Thumb adductor release and web space Z-plasty (Avoid overlengthening)

8 years old Is the deformity fixed contracture?

YES Consider reconstruction  May need fusions wrist and thumb

NO Try passive ROM & splinting

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extremity’s impairment and how these impairments evolve with developmental maturation. It is important to have an algorithm conceptually when evaluating the child for surgery (Table 4).

Cross-References ▶ Constraint-Induced Movement Therapy for Children and Youth with Hemiplegic/Unilateral Cerebral Palsy ▶ Forearm, Thumb, and Finger Deformities in Cerebral Palsy ▶ Physical Examination and Kinematic Assessment of the Upper Extremity in Cerebral Palsy ▶ Shoulder and Elbow Problems in Cerebral Palsy ▶ Single-Event Multilevel Surgery for the Upper Extremity in Cerebral Palsy ▶ Spasticity, Dystonia, and Athetosis Management in the Upper Extremity in Cerebral Palsy ▶ Upper Extremity Assessment and Outcome Evaluation in Cerebral Palsy

References Abu-Sneineh AK, Gabos PG, Miller F (2003) Radial head dislocation in children with cerebral palsy. J Pediatr Orthop 23:155–158 Beach WR, Strecker WB, Coe J, Manske PR, Schoenecker PL, Dailey L (1991) Use of the green transfer in treatment of patients with spastic cerebral palsy: 17-year experience. J Pediatr Orthop 11:731–736 Cao J, Khan B, Hervey N, Tian F, Delgado MR, Clegg NJ, Smith L, Roberts H, Tulchin-Francis K, Shierk A, Shagman L, MacFarlane D, Liu H, Alexandrakis G (2015) Evaluation of cortical plasticity in children with cerebral palsy undergoing constraint-induced movement therapy based on functional near-infrared spectroscopy. J Biomed Opt 20:046009 Charles J, Gordon AM (2006) Development of hand-arm bimanual intensive training (HABIT) for improving bimanual coordination in children with hemiplegic cerebral palsy. Dev Med Child Neurol 48:931–936 Davids JR, Peace LC, Wagner LV, Gidewall MA, Blackhurst DW, Roberson WM (2006) Validation of the shriners hospital for children upper extremity evaluation (SHUEE) for children with hemiplegic cerebral palsy. J Bone Joint Surg Am 88:326–333 DeLuca SC, Case-Smith J, Stevenson R, Ramey SL (2012) Constraint-induced movement therapy (CIMT) for

1567 young children with cerebral palsy: effects of therapeutic dosage. J Pediatr Rehabil Med 5:133–142 Domzalski M, Inan M, Littleton AG, Miller F (2007) Pectoralis major release to improve shoulder abduction in children with cerebral palsy. J Pediatr Orthop 27:457–461 Eliasson AC, Krumlinde-Sundholm L, Rosblad B, Beckung E, Arner M, Ohrvall AM, Rosenbaum P (2006) The manual ability classification system (MACS) for children with cerebral palsy: scale development and evidence of validity and reliability. Dev Med Child Neurol 48:549–554 Friel KM, Kuo HC, Carmel JB, Rowny SB, Gordon AM (2014) Improvements in hand function after intensive bimanual training are not associated with corticospinal tract dysgenesis in children with unilateral cerebral palsy. Exp Brain Res 232:2001–2009 Green WT, Banks HH (1962) Flexor carpi ulnaris transplant and its use in cerebral palsy. J Bone Joint Surg Am 44-A:1343–1430 Holmefur M, Kits A, Bergstrom J, Krumlinde-Sundholm L, Flodmark O, Forssberg H, Eliasson AC (2013) Neuroradiology can predict the development of hand function in children with unilateral cerebral palsy. Neurorehabil Neural Repair 27:72–78 House JH, Gwathmey FW, Fidler MO (1981) A dynamic approach to the thumb-in palm deformity in cerebral palsy. J Bone Joint Surg Am 63:216–225 Jeevanantham D, Dyszuk E, Bartlett D (2015) The manual ability classification system: a scoping review. Pediatr Phys Ther 27:236–241 Riad J, Coleman S, Miller F (2007) Arm posturing during walking in children with spastic hemiplegic cerebral palsy. J Pediatr Orthop 27:137–141 Rose S, Guzzetta A, Pannek K, Boyd R (2011) MRI structural connectivity, disruption of primary sensorimotor pathways, and hand function in cerebral palsy. Brain Connect 1:309–316 Ryll UC, Bastiaenen CH, Eliasson AC (2016) Assisting hand assessment and Children’s hand-use experience questionnaire – observed versus perceived bimanual performance in children with unilateral cerebral Palsy. Phys Occup Ther Pediatr 37:1–11 Sanger TD, Kukke SN, Sherman-Levine S (2007) Botulinum toxin type B improves the speed of reaching in children with cerebral palsy and arm dystonia: an openlabel, dose-escalation pilot study. J Child Neurol 22:116–122 Satila H, Kotamaki A, Koivikko M, Autti-Ramo I (2006) Low- and high-dose botulinum toxin a treatment: a retrospective analysis. Pediatr Neurol 34:285–290 Smitherman JA, Davids JR, Tanner S, Hardin JW, Wagner LV, Peace LC, Gidewall MA (2011) Functional outcomes following single-event multilevel surgery of the upper extremity for children with hemiplegic cerebral palsy. J Bone Joint Surg Am 93:655–661

1568 Spittle AJ, Boyd RN, Inder TE, Doyle LW (2009) Predicting motor development in very preterm infants at 12 months’ corrected age: the role of qualitative magnetic resonance imaging and general movements assessments. Pediatrics 123:512–517 Sutcliffe TL, Logan WJ, Fehlings DL (2009) Pediatric constraint-induced movement therapy is associated with increased contralateral cortical activity on functional magnetic resonance imaging. J Child Neurol 24:1230–1235

F. Miller Thabet AM, Kowtharapu DN, Miller F, Dabney KW, Shah SA, Rogers K, Holmes L Jr (2012) Wrist fusion in patients with severe quadriplegic cerebral palsy. Musculoskelet Surg 96:199–204 Van der Aa NE, Verhage CH, Groenendaal F, Vermeulen RJ, de Bode S, van Nieuwenhuizen O, de Vries LS (2013) Neonatal neuroimaging predicts recruitment of contralesional corticospinal tracts following perinatal brain injury. Dev Med Child Neurol 55:707–712

Upper Extremity Assessment and Outcome Evaluation in Cerebral Palsy

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Lena Krumlinde-Sundholm and Lisa V. Wagner

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1570 Mode of Administration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1571 Classifications Versus Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1572 Norm-Referenced Versus Criterion-Referenced Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1572 Assessing Hand Use in Infants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1573 The ICF Framework . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1573 Capacity Versus Performance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1574 Psychometric Properties . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1574 Clinical Utility . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1575 Final Points . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1575 Upper Extremity Evaluation: Examples of Commonly Used Tools . . . . . . . . . . . . . . 1576 Body Function Assessments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1576 Classifications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1576 Body Function Assessments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1576 Activity and Participation Assessments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1588 Classifications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1588 Questionnaires . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1589 Observation Based . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Speed and Dexterity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Quality of Movements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Bimanual Performance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Functional Activity Performance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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L. Krumlinde-Sundholm (*) Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden e-mail: [email protected] L. V. Wagner Shriners Hospitals for Children, Greenvill, SC, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_108

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L. Krumlinde-Sundholm and L. V. Wagner Development of Hand Skills . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1593 Individualized Measures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1593 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1594 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1594

Abstract

Keywords

In both research and clinical practice, clinicians use a multitude of different assessment instruments to evaluate hand function in children with cerebral palsy (CP). Hand function in and of itself is complex with influences from intrapersonal components and environmental issues. Further complications arise from the perspective of different stakeholders who themselves possess differing concepts of what needs to be evaluated. While it would be terrific to have one universal tool useable for all needs and accepted by all stakeholders, the reality is that a single universal instrument does not exist. Selection of the right tool must be based on its ability to address both the question at hand and the various perspectives driving the question. This chapter’s purpose is to guide the process of selecting the most appropriate assessment(s) for a given situation. This chapter will address (i) the modes of administration, (ii) the differences between classifications and tests, (iii) the differences between norm-referenced and criterionreferenced tests, (iv) the assessment of hand use in infants, and (v) the International Classification of Functioning, Disability, and Health (ICF) framework, with definitions of capacity and performance qualifiers. This chapter will also review (vi) psychometric properties distinctive to assessments and (vii) clinical utility considerations. Finally, a brief review of commonly used instruments for the evaluation of upper limb function in children with cerebral palsy as well as a selection of promising newly published instruments is included. It is our hope that this compilation will be useful in assisting the reader to select the right tool for his/her specific intentions.

Cerebral palsy · Upper extremity · Assessments · Classifications

Introduction Measures are tools used to perform specific tasks . . . It is therefore essential that we be clear about what we wish to accomplish before beginning to measure and then seek the tools that will enable us to do that task. (Rosenbaum 1998)

In both research and clinical practice, clinicians use a multitude of different assessment instruments to evaluate hand function in children with cerebral palsy (CP). Often, assessments are selected because they are “available” or have “traditionally be used.” Instead of specifying the purpose for performing the test, or even which aspect of hand function is to be tested, clinicians choose assessments regarded as general tests of “hand function.” Another difficulty when choosing an appropriate tool is the large number of assessments in use. For example, in a review of 23 randomized controlled studies of constraint-induced movement therapy (CIMT) in children with unilateral CP, 48 different assessments were used (Eliasson et al. 2014). Such inconsistency in the use of assessments makes it difficult to compare results between studies. The clinician must learn to choose a useful assessment based on critical analysis of the tool, relevant evidence of psychometric properties for use with the targeted age and diagnostic group, and correct purpose and type of assessment to answer the question (Rosenbaum et al. 1990). Hand function in and of itself is complex with influences from intrapersonal components and environmental issues. Further complications arise from the perspective of different stakeholders who themselves possess differing

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concepts of what needs to be evaluated. As clinicians, it is imperative to have assessment tools and classification systems that help guide treatment planning, measure the efficacy of an intervention to improve hand function, and prevent secondary complications and deterioration while also discriminating between subjects and providing longitudinal monitoring (Wagner and Davids 2012). Caregivers and patients often consider assessments/classifications as a way to help them describe their function more effectively, to help them set and identify goals, and to give them the motivation to advance to the next level. Policymakers and administrations possess yet another perspective in the use and selection of assessments often looking toward assessments as a means of providing accountability for service and program effectiveness. Assessments may thus help determine allocation of resources or program emphasis. While it would be terrific to have one universal tool, useable for all needs and accepted by all stakeholders, the reality is that a single universal instrument does not exist. Selection of the right tool must be based on its ability to address both the question at hand and the various perspectives driving the question. This process of selecting an assessment can be approached in a myriad of ways. This chapter’s purpose is to guide the process of selecting the most appropriate assessment (s) for a given situation. This chapter will address (i) the mode of administration, (ii) the differences between classifications and tests, (iii) the differences between norm-referenced and criterionreferenced tests, (iv) the assessment of hand use in infants, and (v) the International Classification of Functioning, Disability, and Health (ICF) framework, with definitions of capacity and performance qualifiers. This chapter will also review (vi) psychometric properties distinctive to assessments and (vii) clinical utility considerations. Finally, a brief review of commonly used instruments for the evaluation of upper limb function in children with cerebral palsy as well as a selection of promising newly published instruments is included. It is our hope that this compilation will be useful in assisting the reader to select the right tool for his/her specific intentions.

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Mode of Administration Assessments use different forms for gathering information. They can be patient reported, observation based, or individualized. Patient-reported outcome measures (PROM) are important to reflect the child’s (or parent’s) perspective on the assessed feature, their personal perceived opinion. Such assessments are often questionnaires, and in the context of hand function, they ask about accomplishment of certain activities (e.g., can, with difficulty, or cannot), rather than how the hands are used to execute the activities. Examples of such questionnaires are the ABIHAND-KIDS (Arnould et al. 2004) and the Children’s Hand Use Experience Questionnaire (CHEQ) (Sköld et al. 2011; Amer et al. 2016). Observation-based tests are commonly executed by therapists and consist of a number of tasks that the child performs for the therapist. These tests score on quality, speed, or the accomplishment of specific tasks such as the Box and Block (Mathiowetz et al. 1985a), the QUEST (DeMatteo et al. 1992), or on joint positional analysis such as the SHUEE (Davids et al. 2006). Individualized assessments identify and evaluate the patient’s perceived performance difficulties, establishing self-identified goals for interventions and to evaluating the level of that goal achievement. Such instruments, although not originally meant to measure upper limb function, can assist the patient/parent in identifying areas of concern or activities they would like to accomplish with a more functional hand. Examples of such instruments are the Canadian Occupational Performance Measure (COPM) (Law et al. 1990) which identifies difficulties in everyday life and rates them for performance ability and satisfaction on a 0–10 scale, respectively. Another example is the Goal Attainment Scaling (GAS) (Kiresuk et al. 1994), in which the successive improvement of a specific performance is formulated and given measurable grades of goal achievement on a 5-level scale from 2 to +2, where the goal is to reach level 0. Results from patient-reported measures (perceived ability) often differ from observation-

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based measures (observed ability), which makes it important to report results from both aspects. The combined approach could assist the family with the identification of problem areas they would like to be addressed, thus guiding the clinician in the identification of possible intervention approaches. By the use of individualized outcome measures, the achievement of a variety of different personal goals can be evaluated and compared between patients.

Classifications Versus Tests The terms assessment/test and classification system are often used interchangeably, but in fact, there is a distinct difference between tests and classification systems. A test/assessment refers to instruments that use a standardized procedure to measure a specific attribute within a given population. Tests are scored on a number of items using a rating scale or timed performance. Special test materials, score forms, and a detailed manual are typically provided. Test results are often reported as the sum of scores expressed as a raw score commonly converted to different scaled scores. A test should have the ability to detect differences in the population with which it is used and must consider ranging levels of difficulty in order to differentiate between individuals at both low and high ability levels. The purpose of classifications is to group persons according to common characteristics; this can be general classifications such as overall function and bimanual use or specific classifications such as analysis of joint deformity. A classification is, essentially, a one-item test. The usefulness of a classification is in describing the common characteristics of individuals. An example of a general classification is the Manual Ability Classification System (MACS) (Eliasson et al. 2006). The MACS classifies children’s ability to handle objects in daily activities (one item) in five levels (categories). The five levels describe the complex concept of manual abilities in children within the wide severity spectrum of CP, from very mild to very severe disability. The five levels each include children with a

L. Krumlinde-Sundholm and L. V. Wagner

range of manual abilities grouped together by the common characteristics as defined in the classification. Since classes are crude, the MACS is not expected to detect change, and most persons will stay within the same level over time (Öhrvall et al. 2014). The exception to this is in individuals whose ability is close to the border, between two levels, the effects of intervention or growth may allow them to change one level. An example of a specific classification is the Zancolli classification (Zancolli et al. 1983). Designed as a pre- and postoperative four-level scale, the Zancolli describes the single characteristic of simultaneous extension of the wrist and fingers. As such, the Zancolli classification may be able to guide intervention and describe change after surgical intervention targeting this specific characteristic.

Norm-Referenced Versus CriterionReferenced Tests There are two main types of observation-based standardized test results: norm-referenced and criterion-referenced. Norm-referenced tests have the purpose of being descriptive. Descriptive tests typically answer the question “does this individual differ in the feature of interest from others with typical development and, if so, how much?” The child’s performance is compared to the average performance of a normative sample of typically developed age-matched peers. Typical development is commonly defined to fall between 2 standard deviations. Results are reported by standard scores, t-scores, z-scores, percentiles, or as age-equivalent score. Norm-referenced tests often have a general content covering a variety of skills. Intended to compare to typical development, the test items can be clinically irrelevant and far too advanced for a child with an identified disability such as cerebral palsy. Furthermore, the framework of norm-referenced tests does not account for the fact that children with disabilities do not always follow typical developmental patterns. Using norm-referenced scores to measure change over time may be completely misleading.

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For example, a young child with CP may receive results showing values just below two standard deviations. Repeating the assessment 1 year later, the same child may show a larger discrepancy from the norm, even though the child has actually learned new skills. Due to a slower learning pace, distance to the typically developing children has increased. Thus, norm-referenced scores are not helpful in measuring outcomes of intervention or change over time. The most important contribution of the norm-referenced tests for children with disabilities is their use in identifying a child’s need and eligibility for therapy services by determining the extent of the child’s delay or dysfunction. In contrast, criterion-referenced tests have the purpose of being evaluative. Evaluative measures typically answer the question “How does this individual’s performance, at this specific time, compare to pre-set criteria?” The evaluative test tends to compare individuals against a criterionreferenced specification. They provide information about how a child performs on specific tasks, i.e., whether the child’s performance meets the criteria stipulated for successful performance rather than if his or her performance is age-appropriate. Typically, a rating system is used to score a progression of skills, as opposed to relating skills to age levels. Interpretation is based on raw scores, sometimes expressed as the percent of the total sum or as a scaled score. If, e.g., Rasch measurement model analysis was used to develop the scale, the child’s raw scores are converted to equal interval scaled scores. Criterion-referenced tests often measure functional skills and are useful to identify change since the score is based on the number of tasks passed according to the criteria, not taking into account the age of the child. Criterion-referenced tests, in particular those with interval level scales, should be used to quantify change.

Assessing Hand Use in Infants The need for early assessment of children at risk for CP has been highlighted in several papers, e.g., in Novak et al. (2017), asserting the importance of early intervention to take advantage of the

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substantial brain plasticity in infants. To evaluate treatment effects, as well as evaluate development with age, there is a need for relevant evaluative, criterion-referenced assessment tools with evidence of strong psychometric properties. A number of assessments of general development for infants exist, and there are also assessments of neurological signs. These tests provide important information but typically do not provide facts specifically about upper limb function. The Peabody Developmental Motor Scales version 2 (PDMS-2) (Folio and Fewell 2000) and Bayley Scale of Infant and Toddler Development version III (BSID-III) (Bayley 2006) are two normreferenced tests which include separate fine motor scales. The fine motor tasks are to be performed with the preferred hand, which makes it irrelevant for evaluating hand function in children with signs of unilateral CP. Only a few assessment tools were found to be useful for infants at risk of CP (Krumlinde-Sundholm et al. 2015) and none that would be relevant for infants at risk of developing unilateral CP. The recently developed Hand Assessment for Infants (HAI) (Krumlinde-Sundholm et al. 2017) can be used with infants at risk of CP to measure their ability to use each hand separately, as well as both hands together. The HAI provides a measure of bimanual hand use, an each-hand sum score, and quantifies a possible asymmetry between hands.

The ICF Framework The International Classification of Functioning, Disability, and Health (ICF) (World Health Organization 2001) and the ICF-CY for children and youth provide a standard language and framework for the description of health and health-related states. The ICF emphasizes the importance of measuring or addressing an individual’s function not only in terms of body structure and body function but also in terms of activities, participation, and environmental factors. In the ICF, body functions are the physiological functions of body systems, while body structures refer to the anatomical construct of the body. Problems in this domain are referred to as

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impairments. Basic components of motor and sensory functions, e.g., range of motion, skeletal alignment, grip strength, muscle tone, and tactile sensibility, are regarded as aspects of body functions (coded in ICF classification chapters b2 and b7). Activity is the execution of tasks or actions of an individual, while participation refers to involvement in life situations. Difficulties in these areas are referred to as activity limitations and participation restrictions. Skills such as grasping, picking up, manipulating, reaching, turning, and releasing objects are listed under the activity and participation domain in the (ICF chapter d4 Mobility, codes d430-d449). This domain also incorporates more complex, hand-related activities like eating and grooming (ICF chapter d5 Self-care, codes d510-570) and cooking and cleaning (ICF chapter d6, Household tasks d630–649). This wide definition of activity and participation makes the ICF more complex for the purpose of clarifying differences of focus in assessments. Various authors scrutinizing test items have linked the same items to different ICF domains (Hoare et al. 2011; Klingels et al. 2010a). Even though improved functional performance is the ultimate goal of most interventions, to know if body function-focused interventions (such as hand surgery or tone-reducing botulinum toxin injections) give the expected effect on the targeted body area, the relevant body function measures need to be completed. It should be recognized that the relationship between body function capacity and activity performance is weak, as noted by many authors, e.g., Klingels and collaborators (2012). However, in order to determine if the intervention influenced functional performance, it is imperative that measures covering the domains of activity and performance be performed. The intervention and evaluation must match in order to determine the success of the intervention, and most importantly, the final goal for the intervention must be evaluated.

Capacity Versus Performance When assessing hand function, another concept from the ICF classification is the difference between the qualifiers of capacity and performance.

L. Krumlinde-Sundholm and L. V. Wagner

Capacity is referred to as the person’s ability to execute a task or action on the highest possible level of functioning in a standardized environment (e.g., in a typical clinical test setting). Transferred to a hand function test situation, capacity is examined in any test where the person is asked to use the hand, e.g., to grasp, press, lift, or transport objects, demonstrating the best ability. Analyzing the persons’ capacity, the clinician is able to assess what the person “can do” with respect to hand function. The concept of performance incorporates the real-life environment and thus social, environmental, and habitual aspects in completion of activities. Hand function assessments are not usually executed in a person’s normal environment. Questionnaires describing perceived hand use in daily life may be viewed as representing the performance aspect. Questionnaires also have the ability to cover a broader scope of activity than do traditional observed tests. Assessing the persons’ performance, the clinician is able to understand what the person actually “does do” with respect to functionally using his hand. The distinction between these two qualifiers is clinically relevant. Just because a person can use the hand in a task does not mean a person does use the hand in the given task. For example, when asked, a child with hemiplegic cerebral palsy can pick up objects from the table with the affected hand (the capacity). However, his typical performance is to use the better hand to pick up objects from the table, thereafter grasping them from the better hand with the hemiplegic hand, which is easier and quicker, i.e., more functional for him (the performance). Although these concepts are vastly different, the majority of hand function tests only measure capacity.

Psychometric Properties In order to be useful, outcome measures need to be standardized, have a stated purpose, have to be clinically applicable, and produce valid and reliable measures. The tool in and of itself does not possess a general attribute of validity or reliability in the sense that it is valid and reliable for any population. Rather, the psychometric properties are only applicable when the assessment is used

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with persons within the age and diagnoses that it is intended for and when it is used as stated in the manual, e.g., using all test items in the stipulated context. If the assessment is applied differently, e.g., to a different population, the user needs to show that psychometric properties are reevaluated for that purpose (Streiner et al. 2015). Reliability refers to the trustworthiness or reproducibility of a measure and whether or not the same results can be obtained from the same rater (intrarater), from different raters (inter-rater), or for repeated test occasions (test-retest). That is, how true or accurate is the outcome of a specific test? How much does the result vary within and between raters? Can the result be trusted and be used to interpret if the patient has really changed, e.g., after intervention? For calculations of reliability, the use of kappa, weighted kappa, or intraclass correlation coefficients is recommended (Streiner et al. 2015). The interpretation of the meaning of the reliability coefficients (e.g., high, moderate, low correlation) varies in the literature, and even if a coefficient of 0.75 is often labeled as “good,” it is, in fact, the minimal requirement for a useful instrument (Streiner et al. 2015, p 184.) A practical implication of the use of reliability coefficients is given by Nunnally (1978) stating that a coefficient between 0.70 and 0.80 is sufficient for group-level comparisons (e.g., in research) and a coefficient of 0.90 or more is required for establishing whether a change has occurred or not, in an individual patient (e.g., for clinical use for individuals). With this guideline in mind, clinicians should critically review the reliability results of chosen outcome measures. Validity refers to the extent to which an instrument actually measures what it is supposed to measure and yields scores that can be validly interpreted (Streiner et al. 2015). Validity is influenced by the purpose and the construct of a measure, the item content, and the rationale for the item selection. The validity may be the most important aspect of an instrument; however, it is often not thoroughly evaluated or described. Validity testing documents that there are valid uses for the outcome measure, but it does not document that the outcome measure is valid for all inquiries. Different psychometric properties of assessments tools are important for specific areas of use (Rudman and Hannah 1998). For example, if

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the question is whether a child’s hand function is normally developed, a descriptive test with good internal consistency, content and construct validity, and well-researched norms is important. However, if the question is whether change has occurred after a treatment period, the test chosen must be evaluative, and it must have good intrarater and test-retest reliability, content and construct validity, and sensitivity to change. Perhaps the most important aspect of an outcome measure is to detect change. To evaluate sensitivity to change information about the smallest/minimal detectable difference/change is useful. Based on such information, it can be determined whether a change between two measurement occasions is exceeding the error variance for the scale. If so, the change can be regarded to be a true change. A cutoff value for a minimal clinically important change is another question, much more difficult to determine (Streiner et al. 2015) and seldom mentioned for clinical measures of hand function.

Clinical Utility A final component to assess when choosing an upper extremity assessment tool is the feasibility of using the tool for the patient population, the administering therapist, and the clinic environment. The client or the caregiver must be able to participate in the activities. The clinic setting/environment must be conducive to completing tested activities, and the test results must be relevant to the clinical practice. Adequate time to complete the test and necessary equipment or evaluation kits must be obtained, and the cost of the assessment must be considered. (Some measures are free webaccessible assessments, while some require a financial investment with a certification process.)

Final Points When selecting a tool for measuring outcome of interventions, a medical professional should: • Use an evaluative criterion-referenced measure. • Use a measure that is related to the nature and goal for the intervention.

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• Confirm that the choice of instrument is a good match of ICF levels with the patient’s goals as well as to the expected effects of intervention. • Confirm that the assessment selected produces valid and reliable measures for people with CP in the targeted ages. • Confirm that the selected assessment for the analysis of change has evidence of high testretest reliability. • Be aware that the frequent use of an outcome measure is not synonymous with the measures validity or reliability. • Remember to use all test items in the stipulated manner – A scale should not be adapted since it is only valid when used according to administration instructions.

Upper Extremity Evaluation: Examples of Commonly Used Tools Having reviewed the characteristics of upper limb outcome measures such as type, purpose, and psychometric properties, the following pages give a short presentation of each tool, in text and in Table 1. Identification of the most appropriate outcome tool(s) requires consideration of all characteristics in order to address the task in question. Ultimately, medical professionals must always consider the questions: what do we want to know and why do we need the information? The reason for doing the assessment must guide the choice of instruments. A tool intended to evaluate and plan intervention will not serve the same purpose as a tool intended to measure outcomes. Likewise, a tool intended to describe eligibility for therapy will not measure a patient’s perceived use of hand function. In the paragraphs below, the assessment tools, which all rate upper limb functioning, are arranged according to their ICF domain body function or activity and participation. Further, they are divided into classifications or tests with subheadings describing their main focus. Psychometric properties for each of the specific instruments should be carefully reviewed in the literature to determine the appropriate reliability and validity for the intended use of the instrument. We hope this compilation can assist

L. Krumlinde-Sundholm and L. V. Wagner

clinicians and researchers when choosing and using tests of upper limb function.

Body Function Assessments Classifications House Thumb in Palm Classification (House et al. 1981) was designed to classify the static and dynamic deformities of the thumb when contemplating surgical intervention. House describes the metacarpal adduction contracture and the metacarpophalangeal deformity of the thumb. When reviewing the reliability of motor and sensory impairments in children with hemiplegic cerebral palsy, (Klingels et al. 2010b) found a low interrater agreement with Ƙw 0.73 for two experienced raters classifying 30 children with unilateral CP. Tonkin et al. (2001) reported a modification to the thumb deformity classification with reference to the deforming forces as they relate to the thumb position. Zancolli Classification (Zancolli et al. 1983) is designed to assist with determining surgical intervention; Zancolli classified the patient’s ability to extend the fingers in different wrist positions into four levels. The four levels range from best ability, simultaneous finger extension and wrist extension to more than 20 , to the most severe ability, no ability to extend fingers, not even with wrist flexed. Matsuo et al. (2001) presents an adjusted version with added sub-ratings in the highest level. Thus, the Zancolli classification can describe positions of the wrist and fingers; however, whether it is sensitive to change has yet to be determined. Klingels and collaborators (2010b) reported excellent inter-rater reliability and testretest reliability from a sample of 30 children 5–15 years old with unilateral CP.

Body Function Assessments Australian Spasticity Assessment Scale (ASAS): Love et al. (2016) further developed the Tardieu scale (see below) to quantify spasticity in children

Short description

Patient/parent reported questionnaire describes ease or difficulty with manual activity performance on 21 unimanual and bimanual tasks Parent describes ease or difficulty with global activity performance questionnaire of activates of daily living Measures how well a person with unilateral CP use of the assisting hand together with the well functioning hand to perform bimanual activities Quantifies the presence of spasticity

Key references

Arnould et al. 2004

Bleyenheuft et al. 2017

KrumlindeSundholm et al. 2007; Holmefur and KrumlindeSundholm 2016; Louwers et al. 2016

Love et al. 2016

Name of tool

ABILHANDkids

ACTIVLIMCP

Assisting Hand Assessment 5.0 (AHA 18–18)

Australian Spasticity Assessment Scale (ASAS)

PROM

PROM

Observation

Observation

2–18 years All CP

18 months UCP to 18 years

4–19 years All CP

Type of results

Test

Test

Criterion

Criterion

Questionnaire Criterion

Questionnaire Criterion

Mode of Type of administration assessment

6–15 years All CP

Age for use

Type of CP or other diagnosis

Evaluative

Evaluative

Evaluative

Evaluative

Purpose

Table 1 A short presentation of a selection of commonly used or newly developed Upper Extremity Evaluation tools

Activity and Performance participation Does not measure hand function but task performance

To measure tone

Body function

(continued)

Activity and Performance participation

Activity and Performance participation

Unimanual and bimanual

Measure

ICF domain

Nature of test items

Bimanual To measure bimanual hand use guide intervention toward effective assisting hand use

To measure perceived ability to performance daily activities

To measure perceived ease of hand use in daily life activities

Reason for use

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Both Hands Assessment (BoHA)

Bimanual Fine Motor Function Classification (BFMF)

Besta Scale

Name of tool

Short description

Fedrizzi et al. 2003; RosaRizzotto et al. 2014

Describes the capacity and the performance of the upper limb in bimanual play activities and ADL activities Elvrum et al. Classifies fine motor function 2016; Elvrum et al. to 5+5 levels according to 2017a the child’s best ability to grasp, hold, and manipulate objects for each hand separately Elvrum et al. A new test of 2017b hand function for children with bilateral CP, at MACS levels I–III, measuring bimanual performance, unilateral performance for each hand separately and provides an asymmetry index quantifying a possible side difference between hands

Key references

Table 1 (continued)

Observation

PROM, observation

PROM

3–18 years All CP

18 months BCP to 12 years

Test

Classification

Test

Mode of Type of administration assessment

18 months UCP to 12 years

Age for use

Type of CP or other diagnosis

Criterion

Criterion

Criterion

Type of results

Evaluative

Descriptive

Evaluative

Purpose Unimanual and bimanual

Nature of test items

To measure bimanual performance

Bimanual

To classify hand Unimanual function and bimanual

To measure hand use

Reason for use

Measure

Activity and Performance participation

Activity and Capacity participation

Activity and Capacity participation and performance

ICF domain

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Mathiowetz et al. 1985a; Mathiowetz et al. 1985b

Law et al. 1990, 2000; Cusick et al. 2006; Wallen et al. 2007

Sköld et al. 2011; Amer et al. 2016

Mathiowetz et al. 1986; Klingels et al. 2010; Damiano et al. 2002a; Dekkers et al. 2014

Box and Block

Canadian Occupational Performance Measure (COPM)

Children’s Hand-use Experience Questionnaire 2 (CHEQ)

Grip/Pinch Strength

Observation

PROM

PROM/ individualized

Not age specific

Generic

Observation

6 years and Generic older

6–18 years UCP Describes the perceived efficacy to use of the affected hand during bimanual daily activities on three scales: effectiveness of grasp, time use in comparison to peers, and experience of feeling bothered while doing the activity Quantifies the Not age Generic specific grip or the various types of pinches of the hand in pounds or kg

Assesses unilateral gross manual dexterity, the number of blocks (side 2.5 cm) moved in 60 sec Clients identify and record perception of occupational performance in the domains of self care, productivity, and leisure Criterion

Norm and criterion

Test

Norm and criterion

Questionnaire Criterion

Test

Test

Descriptive, evaluative

Descriptive, evaluative

Evaluative

Descriptive, evaluative

Unimanual

Activity and Capacity participation

Determine strength of grip or pinch

Unimanual

Body function

Upper Extremity Assessment and Outcome Evaluation in Cerebral Palsy (continued)

Capacity

Individualized Activity and Performance To identify participation client focused problem areas to measure change in perceived ability and satisfaction with performance Bimanual Activity and Performance To identify participation client’s perceived ability to performance bimanual tasks concerning effectiveness of grasp, time taken, and sense of botheredness

To measure gross dexterity

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House Functional Classification System for Cerebral Palsy

Hand Assessment for Infants (HAI)

Goal Attainment Scale (GAS)

Name of tool

Quantifies the 3–12 months quality and frequency of hand use in infants at risk of CP for three outcomes; unilateral hand use for each hand separately sum score; an asymmetry index, and bimanual hand use measure House et al. Describes 2–20 years 1981; spontaneous Waters et al. use of the 2004; involved UE in Koman et al. nine categories 2008; from not used, Geerdink passive hand, et al. 2014 active hand, and complete use

Not age Client identifies client specific goals and measurement criteria

Kiresuk et al. 1994; Steenbeek et al. 2007, 2010; Palisano et al. 1992, Palisano 1993; Cusick et al. 2006 KrumlindeSundholm et al. 2017

Age for use

Short description

Key references

Table 1 (continued)

Classification

Observation

UCP

Test

Test

PROM/ individualized

Mode of Type of administration assessment

UCP and Observation validation is ongoing for BCP

Generic

Type of CP or other diagnosis Evaluative

Purpose

Criterion

Descriptive, evaluative

Criterion Descriptive, and norm evaluative

Criterion

Type of results

Measure

Activity and Performance participation

Activity and Performance participation

Goal setting to determine measures of current ability and change

Unimanual To measure and bimanual hand use in infants to identify possible deviations or asymmetry between hands, related to norms, evaluate interventions, follow development over time, assist diagnosing of CP Unimanual Describe functional status

ICF domain

Capacity or performance

Nature of test items Individualized

Reason for use

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Manual Muscle Testing

Manual Ability Classification System (MACS) and Mini-MACS

Jebsen and Taylor Test of Hand Function (JTTH)

House Thumb in Palm Classification

Describes the static and dynamic deformity of the thumb by four groups according to which muscles are contract or spastic Jebsen et al. Timed test of 1969; Taylor speed and dexterity in et al. 1973; seven tasks Gilmore et simulating al. 2010 everyday activities. Psychometrics not investigated for children with CP Classifies Eliasson children’s et al. 2006, manual ability 2017 to handle objects in everyday life for five levels, a lower number indicates a higher ability. Available in many languages at www.MACS. nu Klingels Quantifies et al. 2010a; strength of individual Damiano et al. 2002a; muscle or

House et al. 1981; Klingels et al. 2010b; Tonkin et al. 2001

Observation

PROM

Observation

6 years and Generic older

4–18 years All CP and MiniMACS 120 ), vertebral column resection is also an option and gives excellent correction for severe pelvic obliquity and coronal and sagittal plane deformity (Fig. 9). Sponseller and colleagues reported on a series of 23 children undergoing vertebral resection for severe neuromuscular scoliosis with excellent

Fig. 8 Anterior release: wedge resections of the discs are performed around the apical vertebrae if a stiff spinal deformity exists, usually greater than 100

K. W. Dabney and M. W. Shrader

correction but a risk of major complications (Sponseller et al. 2012).

Surgical Outcomes The goals of spinal surgery in patients with cerebral palsy are to enhance quality of life by improving balanced sitting posture, reducing pain, and deformity while minimizing both intraoperative and postoperative complications. However, neuromuscular surgery may be rather challenging and most reports show higher rates of complication compared to idiopathic scoliosis surgery. In a large multicenter study reviewing all cases of pediatric scoliosis, neuromuscular scoliosis had 17.9% complications (Reames et al. 2011). In our own series of 107 cerebral palsy patients undergoing scoliosis surgery utilizing the unit rod as fixation, complications included 14 deep infections, 3 deaths, 15 cases of prominent hardware, and 1 pseudarthrosis (Tsirikos et al. 2008). We concluded that unit rod instrumentation is simple to use and considerably less expensive than most instrumentation systems, associated with low reoperation rates, and achieves successful long-term correction of 70–80% of the preoperative curvature magnitude and 80–90% correction of pelvic obliquity. Caretakers reported

Fig. 9 This year-old female, GMFCS level 5, had a (a) 130 curvature and (b) underwent a vertebral resection of the L2 vertebrae with (c) contoured rods and pedicle screw instrumentation showing good coronal and (d) sagittal alignment

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Surgical Treatment of Scoliosis Due to Cerebral Palsy

a 96% satisfaction rate (Tsirikos et al. 2008). Three studies have looked at preoperative risk factors for postoperative complications (Lipton et al. 1999; Samdani et al. 2016; Nishnianidze et al. 2016). Non-ambulatory status preoperatively, dependence on G-tube feeding, and curve magnitude of greater than or equal to 60 were directly associated with increased risk of major complications. One study indirectly associated with increased length of stay (Samdani et al. 2016). A recent study compared neuromuscular scoliosis complication rates from 2004 to 2015 which included bleeding, infection neurologic deficit, respiratory complications, and mortality (Cognetti et al. 2017). Encouraging results showed a 3.5-fold decrease in the complication rate from 2004 to 2015, especially, infection rates, respiratory complications, and implant-related complications (Cognetti et al. 2017). Finally, Jain and colleagues subclassified children with Gross Motor Function Classification System 5 (GMFCS 5) undergoing spinal fusion according to their number of neuromotor impairments such as the presence of gastrostomy tube, tracheostomy, seizure disorder, and nonverbal status (Jain et al. 2016b). The rate of major complications was proportional to the number of neuromotor impairments (subclassification GMFCS levels 5.0 thru 5.3). Five out of seven of the patients who died within their follow-up period were level 5.3. The authors also utilized the CPCHILD to rate quality of life which decreased significantly from GMFCS levels 5.0 to 5.3, both preoperatively and postoperatively; however there was no difference between GMFCS levels in the improvement of CPCHILD scores from preoperative to follow-up evaluation (Jain et al. 2016b). Historically, the possibility of ambulatory cerebral palsy patients losing their ambulatory status due to spinal fusion with instrumentation was a concern. Tsirikos et al. showed that 24 ambulatory patients with cerebral palsy who had posterior spinal fusion using unit rod instrumentation maintained their ambulatory status postoperatively (Tsirikos et al. 2003). We have also observed this preservation of ambulatory status in children undergoing posterior spinal fusion with modular instrumentation utilizing pre-contoured rods. This

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supports the principle that instrumentation that preserves normal spinal sagittal contour also preserves preoperative ambulatory function.

Functional Outcomes and Quality of Life Important aspects of postoperative assessment also include overall function, appearance, ease of care, and improved quality of life. Outcome measures should differentiate treatment effects from underlying disease functional impairments (Bowen et al. 2012). Some might question whether spinal deformity surgery is truly beneficial for neuromuscular patients, particularly those most severely involved. In one survey of 190 parents and caretakers assessed for functional improvement of children with cerebral palsy after spinal fusion, 95.8% of parents and 84.3% of caretakers would recommend spinal surgery again (Tsirikos et al. 2004). Several other reviews report caretakers, families, and patients with severely involved neuromuscular diseases are most often satisfied with the surgical correction, improved appearance, and enriched quality of life (Dias et al. 1996; Larsson et al. 2005; Jones et al. 2003; Watanabe et al. 2009). A few studies have examined quality of life pre- and postoperatively following spinal fusion. An earlier literature review of patients undergoing spinal fusion with neuromuscular scoliosis concluded that quality of life improved in neuromuscular patients with cerebral palsy (Mercado et al. 2007). More recently, a prospective study was used to evaluate changes in quality of life and caregiver burden in CP children with GMFCS levels IV and V following spinal fusion (DiFazio et al. 2017). While quality of life appeared to improve at 1 year postoperatively, it regressed at 2 years, and caregiver burden did not change after spinal fusion. In a retrospective study of a multicenter prospective registry, caregiver perceptions such as qualitative changes in global quality of life, comfort, and health, relative valuation of spine surgery versus other interventions in children with cerebral palsy, and quantitative changes in healthrelated quality of life scores were accessed (Jain

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et al. 2018). Spinal surgery was ranked as the most beneficial procedure in patients’ lives by 75% of 212 caretakers who were surveyed, second only to gastrostomy tube insertion. Health-related quality of life improved over a 2-year follow-up across several quality of life domains. As surgeons recommending surgery for scoliosis, we must always evaluate the risks versus benefits of surgery, especially in the totally involved child with CP. In a final study, investigators looked at the risk-benefit ratio of undergoing scoliosis surgery in cerebral palsy by evaluating the benefits of health-related quality of life measures using the CPCHILD questionnaire as well as looking at improvements and the effects of complications on outcomes over 1-, 2-, and 5-year follow-up evaluations (Miyanji et al. 2018). The investigators found significant improvements in ease of personal care, positioning, and comfort domains at each follow-up time period. While the 1-year complication rate was 46.4%, it fell to 4.3% at 2–5 years postoperatively, and there was no apparent correlation between complications and the CPCHILD scores at each time period. Given that health-related quality of life scores improved significantly and were maintained over the 5 years, the authors concluded that the benefits of scoliosis surgery outweighed the risks despite the high rate of complications. Further studies are needed to solidify whether the risk-benefit ratio is worth scoliosis surgery in all cerebral palsy patients despite existing motor involvement, comorbidities, etc. Ultimately, determining the risk-benefit ratio according to increasing risk factors may further define who will benefit most from surgery.

Cross-References ▶ Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management ▶ Early-Onset Scoliosis in Cerebral Palsy ▶ Infections and Late Complications of Spine Surgery in Cerebral Palsy ▶ Pelvic Alignment and Spondylolisthesis in Children with Cerebral Palsy

K. W. Dabney and M. W. Shrader

▶ Spinal Procedure Atlas for Cerebral Palsy Deformities ▶ Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy ▶ Windblown Hip Deformity and Hip Contractures in Cerebral Palsy

References Abousamra O, Nishnianidze T, Rogers KJ, Bayhan IA, Yorgova P, Shah SA (2016) Correction of pelvic obliquity after spinopelvic fixation in children with cerebral palsy: a comparison study with minimum two-year follow-up. Spine Deformity 4(3):217–224 Abousamra O, Sullivan BT, Samdani AF, Yaszay B, Cahill PJ, Newton PO, Sponseller PD (2018) Three methods of pelvic fixation for scoliosis in children with cerebral palsy: differences at 5-year follow-up. Spine (Phila Pa 1976). https://doi.org/10.1097/BRS.0000000000002761. [Epub ahead of print Auerbach JD, Spiegel DA, Zgonis MH, Reddy SC, Drummond DS, Dormans JP, Flynn JM (2009) The correction of pelvic obliquity in patients with cerebral palsy and neuromuscular scoliosis: is there a benefit of anterior release prior to posterior spinal arthrodesis? Spine (Phila Pa 1976) 34(21):E766–E774 Bell DF, Moseley CF, Koreska J (1989) Unit rod segmental spinal instrumentation in the management of patients with progressive neuromuscular spinal deformity. Spine (Phila Pa 1976) 14(12):1301–1307 Boachie-Adjei O, Lonstein JE, Winter RB, Koop S, vanden Brink K, Denis F Management of neuromuscular spinal deformities with Luque segmental instrumentation Bonnett C, Brown JC, Grow T (1976) Thoracolumbar scoliosis in cerebral palsy. Results of surgical treatment. J Bone Joint Surg Am 58:328–336 Bowen RE, Abel MF, Arlet V et al (2012) Outcome assessment in neuromuscular spinal deformity. J Pediatr Orthop 32:792–798 Brenn BR, Theroux MC, Dabney KW, Miller F (2004) Clotting parameters and thromboelastography in children with neuromuscular and idiopathic scoliosis undergoing posterior spinal fusion. Spine (Phila Pa 1976) 29(15):E310–E314 Brown JC, Swank S, Specht I (1982) Combined anterior and posterior spine fusion in cerebral palsy. Spine 7:570–573 Cognetti D, Keeny HM, Samdani AF, Pahys JM, Hanson DS, Blanke K, Hwang SW (2017) Neuromuscular scoliosis complication rates from 2004 to 2015: a report from the Scoliosis Research Society morbidity and mortality database. Neurosurg Focus 43(4):E10 Comstock CP, Leach J, Wenger DR (1998) Scoliosis in total-body involvement cerebral palsy. Analysis of surgical treatment and patient and caregiver satisfaction. Spine 23:1412–1424

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Dhawale AA, Shah SA, Sponseller PD, Bastrom T, Neiss G, Yorgova P, Newton PO, Yaszay B, Abel MF, Shufflebarger H, Gabos PG, Dabney KW, Miller F (2012) Are antifibrinolytics helpful in decreasing blood loss and transfusions during spinal fusion surgery in children with cerebral palsy scoliosis? Spine (Phila Pa 1976) 37(9):E549–E555 Dias RC, Miller F, Dabney K, Lipton G, Temple T (1996) Surgical correction of spinal deformity using a unit rod in children with cerebral palsy. J Pediatr Orthop 16(6): 734–740 DiCindio S, Theroux M, Shah S, Miller F, Dabney K, Brislin RP, Schwartz D (2003) Multimodality monitoring of transcranial electric motor and somatosensoryevoked potentials during surgical correction of spinal deformity in patients with cerebral palsy and other neuromuscular disorders. Spine (Phila Pa 1976) 28(16):1851–1855 DiCindio S, Arai L, McCulloch M, Sadacharam K, Shah SA, Gabos P, Dabney K, Theroux MC (2015) Clinical relevance of echocardiogram in patients with cerebral palsy undergoing posterior spinal fusion. Paediatr Anaesth 25(8):840–845 DiFazio RL, Miller PE, Vessey JA, Snyder BD (2017) Health-related quality of life and care giver burden following spinal fusion in children with cerebral palsy. Spine (Phila Pa 1976) 42(12):E733–E739 Edebol, Tysk K (1989) Epidemiology of spastic tetraplegic cerebral palsy in Sweden. I. I. Impairments and disabilities. Neuropediatrics 20:41–45 Erickson MA, Oliver T, Baldini T et al (2004) Biomechanical assessment of conventional unit rod fixation versus a unit rod pedicle screw construct: a human cadaver study. Spine (Phila Pa 1976) 29:1314–1319 Ferguson RL, Allen BL Jr (1988) Considerations in the treatment of cerebral palsy patients with spinal deformities. Orthop Clin North Am 19(2):419–425 Fuhrhop SK, Keeler KA, Oto M, Miller F, Dabney KW, Bridwell KH, Lenke LG, Luhmann SJ (2013) Surgical treatment of scoliosis in non-ambulatory spastic quadriplegic cerebral palsy patients: a matched cohort comparison of unit rod technique and all-pedicle screw constructs. Spine Deformity 1(5):389–394 Gau YL, Lonstein JE, Winter RB, Koop S, Denis F (1991) Luque-Galveston procedure for correction and stabilization of neuromuscular scoliosis and pelvic obliquity: a review of 68 patients. J Spinal Disord 4(4):399–410. J Bone Joint Surg Am. 1989;71(4):548–62 Hagglund G, Pettersson K, Czuba T, Persson-Bunke M, Rodby-Bunke M (2018) Incidence of scoliosis in cerebral palsy: a population-based study of 962 young individuals. Acta Orthop 89(4):443–447 Jackson TJ, Yaszay B, Pahys JM, Singla A, Miyanji F, Shah SA, Sponseller PD, Newton PO, Flynn JM, Cahill PJ, Harms Study Group (2018) Intraoperative traction may be a viable alternative to anterior surgery in cerebral palsy scoliosis 100 degrees. J Pediatr Orthop 38(5):e278–e284 Jain A, Njoku DB, Sponseller PD (2012) Does patient diagnosis predict blood loss during posterior spinal

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fusion in children? Spine (Phila Pa 1976) 37(19): 1683–1687 Jain A, Kebaish KM, Sponseller PD (2016a) Sacral-alariliac fixation in pediatric deformity: radiographic outcomes and complications. Spine Deform 4(3):225–229 Jain A, Sponseller PD, Shah SA, Samdani A, Cahill PJ, Yaszay B, Njoku DB, Abel MF, Newton PO, Marks MC, Narayanan UG, Harms Study Group (2016b) Subclassification of GMFCS Level-5 cerebral palsy as a predictor of complications and health-related quality of life after spinal arthrodesis. J Bone Joint Surg Am 98(21):1821–1828 Jain A, Sponseller PD, Shah SA, Yaszay B, Njoku DB, Miyanji F, Newton PO, Bastrom TP, Marks MC, Harms Study Group (2017a) Incidence of and risk factors for loss of 1 blood volume during spinal fusion surgery in patients with cerebral palsy. J Pediatr Orthop 37(8):e484–e487 Jain A, Sullivan BT, Kuwabara A, Kebaish KM, Sponseller PD (2017b) Sacral-alar-iliac fixation in children with neuromuscular scoliosis: minimum 5-year follow-up. World Neurosurg 108:474–478 Jain A, Sullivan BT, Shah SA, Samdani AF, Yaszay B, Marks MC, Sponseller PD (2018) Caregiver perceptions and health-related quality-of-life changes in cerebral palsy patients after spinal arthrodesis. Spine (Phila Pa 1976) 43(15):1052–1056 Jones KB, Sponseller PD, Shindle MK et al (2003) Longitudinal parental perceptions of spinal fusion for neuromuscular spine deformity in patients with totally involved cerebral palsy. JPO 23:143–114 Karatas AF, Miller EG, Miller F, Dabney KW, Bachrach S, Connor J, Rogers K, Holmes L Jr (2013) Cerebral palsy patients discovered dead during sleep: experience from a comprehensive tertiary pediatric center. J Pediatr Rehabil Med 6(4):225–231 Ko PS, Jameson PG 2nd, Chang TL, Sponseller PD (2011) Transverse-plane pelvic asymmetry in patients with cerebral palsy and scoliosis. J Pediatr Orthop 31(3):277–283 Koop SE (2009) Scoliosis in cerebral palsy. Dev Med Child Neurol 4(Suppl):92–98 Kuklo TR, Bridwell KH, Lewis SJ et al (2001) Minimum 2-year analysis of sacropelvic fixation and L5–S1 fusion using S1 and iliac screws. Spine (Phila Pa 1976) 26:1976–1983 Larsson EL, Aaro SI, Normelli HC et al (2005) Long-term follow-up of functioning after spinal surgery in patients with neuromuscular scoliosis. Spine (Phila Pa 1976) 30:2145–2152 Lipton GE, Miller F, Dabney KW, Altiok H, Bachrach SJ (1999) Factors predicting postoperative complications following spinal fusions in children with cerebral palsy. J Spinal Disord 12(3):197–205 Lonstein JE, Koop SE, Novachek TF et al (2012) Results and complications after spinal fusion for neuromuscular scoliosis in cerebral palsy and static encephalopathy using Luque-Galveston instrumentation. Spine (Phila Pa 1976) 37:583–591 Madigan RR, Wallace SL (1981) Scoliosis in the institutionalized cerebral palsy population. Spine 6:583–590

1740 Majd ME, Muldowny DS, Holt RT (1997) Natural history of scoliosis in the institutionalized adult cerebral palsy population. Spine 22:1461–1466 Mercado E, Alman B, Wright JG (2007) Does spinal fusion influence quality of life in neuromuscular scoliosis? Spine (Phila Pa 1976) 32:S120–S125 Miyanji F, Nasto LA, Sponseller PD, Shah SA, Samdani AF, Lonner B, Yaszay B, Clements DH, Narayanan U, Newton PO (2018) Assessing the risk-benefit ratio of scoliosis surgery in cerebral palsy: surgery is worth it? J Bone Joint Surg Am 100(7):556–563 Modi HN, Hong JY, Mehta SS et al (2009) Surgical correction and fusion using posterior-only pedicle screw construct for neuropathic scoliosis in patients with cerebral palsy: a three year follow up study. Spine (Phila Pa 1976) 34:1167–1175 Nishnianidze T, Bayhan IA, Abousamra O, Sees J, Rogers KJ, Dabney KW, Miller F (2016) Factors predicting postoperative complications following spinal fusions in children with cerebral palsy scoliosis. Eur Spine J 25(2):627–634 Peelle MW, Lenke LG, Bridwell KH et al (2006) Comparison of pelvic fixation techniques in neuromuscular spinal deformity correction: Galveston rod versus iliac and lumbosacral screws. Spine (Phila Pa 1976) 31:2392–2398 Rappaport DI, Pressel DM (2008) Pediatric hospitalist comanagement of surgical patients: challenges and opportunities. Clin Pediatr (Phila) 47(2):114–121 Rappaport DI, Cerra S, Hossain J, Sharif I, Pressel DM (2013a) Pediatric hospitalist preoperative evaluation of children with neuromuscular scoliosis. J Hosp Med 8(12):684–688 Rappaport DI, Adelizzi-Delany J, Rogers KJ, Jones CE, Petrini ME, Chaplinski K, Ostasewski P, Sharif I, Pressel DM (2013b) Outcomes and costs associated with hospitalist comanagement of medically complex children undergoing spinal fusion surgery. Hosp Pediatr 3(3):233–241 Reames DL, Smith JS, Fu KM et al (2011) Scoliosis Research Society morbidity and mortality committee. Complications in the surgical treatment of 19,360 cases of pediatric scoliosis: a review of the Scoliosis Research Society morbidity and mortality database. Spine (Phila Pa 1976) 36:1484–1491 Rinsky LA (1990) Surgery of spinal deformity in cerebral palsy. Twelve years in the evolution of scoliosis management. Clin Orthop Relat Res 253:100–109 Saito N, Ebar S, Ohotsuka K, Kumeta H, Takaoka K (1998) Natural history of scoliosis in spastic cerebral palsy. Lancet 351:1687–1692 Samdani AF, Belin EJ, Bennett JT, Miyanji F, Pahys JM, Shah SA, Newton PO, Betz RR, Cahill PJ, Sponseller PD (2016) Major perioperative complications after spine surgery in patients with cerebral palsy: assessment of risk factors. Eur Spine J 25(3):795–800

K. W. Dabney and M. W. Shrader Sees JP, Sitoula P, Dabney K, Holmes L Jr, Rogers KJ, Kecskemethy HH, Bachrach S, Miller F (2016) Pamidronate treatment to prevent reoccurring fractures in children with cerebral palsy. J Pediatr Orthop 36(2):193–197 Sponseller PD, Shah SA, Abel MF et al (2009) Scoliosis surgery in cerebral palsy: differences between unit rod and custom rods. Spine (Phila Pa 1976) 34:840–844 Sponseller PD, Shah SA, Abel MF, Newton PO, Letko L, Marks M (2010) Infection rate after spine surgery in cerebral palsy is high and impairs results: multicenter analysis of risk factors and treatment. Clin Orthop Relat Res 468(3):711–716 Sponseller PD, Jain A, Lenke LG, Shah SA, Sucato DJ, Emans JB, Newton PO (2012) Vertebral column resection in children with neuromuscular spine deformity. Spine (Phila Pa 1976) 37(11):E655–E661 Strauss D, Brooks J, Rosenbloom L, Shavelle R (2008) Life expectancy in cerebral palsy: an update. Dev Med Child Neurol 50:487–493 Sussman MD, Little D, Alley RM, McCoig JA (1996) Posterior instrumentation and fusion of the thoracolumbar spine for treatment of neuromuscular scoliosis. J Pediatr Orthop 16:304–313 Takeshita K, Lenke LG, Bridwell KH, Kim YJ, Sides B, Hensley M (2006) Analysis of patients with nonambulatory neuromuscular scoliosis surgically treated to the pelvis with intraoperative halo-femoral traction. Spine (Phila Pa 1976) 31(20):2381–2385 Thometz JG, Simon SR (1988) Progression of scoliosis after skeletal maturity in institutionalized adults who have cerebral palsy. J Bone Joint Surg Am 70(9): 1290–1296 Tsirikos AI, Mains E (2012) Surgical correction of spinal deformity in patients with cerebral palsy using pedicle screw instrumentation. J Spinal Disord Tech 25(7): 401–408 Tsirikos AI, Chang WN, Shah SA, Dabney KW, Miller F (2003) Preserving ambulatory potential in pediatric patients with cerebral palsy who undergo spinal fusion using unit rod instrumentation. Spine (Phila Pa 1976) 28(5):480–483 Tsirikos AI, Chang WN, Dabney KW, Miller F (2004) Comparison of parents’ and caregivers’ satisfaction after spinal fusion in children with cerebral palsy. J Pediatr Orthop 24(1):54–58 Tsirikos AI, Lipton G, Chang WN, Dabney KW, Miller F (2008) Surgical correction of scoliosis in pediatric patients with cerebral palsy using the unit rod instrumentation. Spine (Phila Pa 1976) 33(10): 1133–1140 Vitale MG, Riedel MD, Glotzbecker MP, Matsumoto H, Roye DP, Akbarnia BA, Anderson RC, Brockmeyer DL, Emans JB, Erickson M, Flynn JM, Lenke LG, Lewis SJ, Luhmann SJ, LM ML, Newton PO, Nyquist

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AC, Richards BS 3rd, Shah SA, Skaggs DL, Smith JT, Sponseller PD, Sucato DJ, Zeller RD, Saiman L (2013) Building consensus: development of a Best Practice Guideline (BPG) for surgical site infection (SSI) prevention in high-risk pediatric spine surgery. J Pediatr Orthop 33(5):471–478 Watanabe K, Lenke LG, Daubs MD et al (2009) Is spine deformity surgery in patients with spastic cerebral palsy

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truly beneficial? A patient/parent evaluations. Spine (Phila Pa 1976) 34:2222–2232 Yoshido K, Kajiura I, Suzuki T, Kawabata H (2018) Natural history of scoliosis in cerebral palsy and risk factors for progression of scoliosis. J Orthop Sci 23(4):649–652

Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1744 Etiology/Pathogenesis/Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1744 Patient Assessment and Preoperative Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1745 Nonoperative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1746 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Medical/Anesthesia Considerations (Anesthesia for Cerebral Palsy Spine Fusion Surgery) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Operative Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Preoperative Planning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Current Preferred Surgical Treatment Methods (Spinal Procedure Atlas for Cerebral Palsy Deformities) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Intraoperative Positioning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Instrumentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Fusion to the Pelvis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Kyphosis Correction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Lumbar and Thoracolumbar Kyphosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Thoracic Kyphosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hyperlordosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Rigid Kyphotic and Hyperlordotic Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Evidence-Based Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1758 Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1759 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1759 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1759

K. W. Dabney (*) Department of Orthopedics, Nemours/AI DuPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_116

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Abstract

Sagittal plane spinal deformities, excessive kyphosis or lordosis may occur in the child with cerebral palsy (CP) and may be seen either with or without scoliosis. These spinal deformities may cause difficulty with seating and/or pain, especially when the deformity is greater than 70 degrees. And hyperlordosis has also been reported to cause superior mesenteric artery syndrome. Pain and difficulty with seating are the most common indications for correcting sagittal plane spinal deformities in the child with CP. Mild and some moderate sagittal plane deformities can be treated by wheelchair modifications and bracing. Symptomatic moderate and severe deformity may require surgical treatment. More flexible kyphosis and hyperlordosis can be corrected by posterior spinal fusion and segmental instrumentation alone while rigid deformity may require posterior osteotomies (for kyphosis) or anterior discectomies (for hyperlordosis). Instrumentation and correction techniques vary from screw/rod constructs using distraction/ compression correction to wire or screw/rod constructs using cantilever correction. Overall, natural history and surgical outcome studies focused solely on sagittal plane spinal deformities in the patient with CP are limited. Those authors that do measure functions report improvements in pain, sitting balance, head and neck control, breathing, and hand use. Patients with kyphosis undergoing spinal fusion with instrumentation are at risk for loss of proximal and/or distal fixation. Patients with hyperlordosis appear to be at greatest risk for postoperative complications. Keywords

Cerebral palsy · Neuromuscular · Kyphosis · Hyperlordosis · Sagittal plane · Spinal deformity · Spinal fusion

Introduction Sagittal plane spinal deformities, excessive kyphosis or lordosis may occur in the child with cerebral palsy (CP) and may be seen either with or

K. W. Dabney

without scoliosis. These spinal deformities may cause difficulty with seating and/or pain, especially when the deformity is greater than 70 degrees. And hyperlordosis has also been reported to cause superior mesenteric artery syndrome (Lipton et al. 2003; Karampalis and Tsirikos 2014). Pain and difficulty with seating are the most common indications for correcting sagittal plane spinal deformities. Separate surgical strategies are necessary to correct each deformity (Dabney et al. 2004). Similar to scoliosis, the surgical outcomes of corrective spine surgery in the CP child may be adversely affected by associated comorbidities in the CP child. Optimum medical management is therefore important to minimize adverse postoperative outcomes.

Etiology/Pathogenesis/Natural History Similar to scoliosis in cerebral palsy, sagittal plane deformities are a consequence of muscle imbalance. Iliopsoas contracture can cause lumbar hyperlordosis, severe anterior pelvic tilting, and a horizontal sacrum. On the other hand, McCarthy et al. showed an association between loss of lumbar lordosis or even frank lumbar kyphosis with hamstring contracture that may cause a posteriorly tilted pelvis and a prominent vertically oriented sacrum (McCarthy and Betz 2000; Dabney 2018). Lumbar kyphosis can result in a greater weightbearing load shifted on to the sacrum resulting in a sacral pressure sore. Truncal hypotonia and poor head control can initially result in postural thoracic kyphosis eventually resulting in a more rigid kyphosis over time. Currently, there is insufficient research to ascertain whether or not the severity of motor involvement is directly proportional to the severity of sagittal plane deformity in CP, although our clinical experience tells us that this is likely the case. Understanding whether or not quality of life is impacted by the sagittal plane spinal deformity should be the main determinant and rationale for treatment. No current natural history studies exist for either kyphosis or hyperlordosis. On the other hand, the CP child’s life expectancy is largely dependent on the number of comorbidities.

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Accordingly, the surgeon must recognize if the sagittal plane spinal deformity is having a significant impact on the child’s quality of life or not. In addition, the number and severity of comorbidities may impact the surgical timing and perioperative risks associated with spinal surgery.

Patient Assessment and Preoperative Considerations After a shared decision-making process between the medical care provider and the family to determine whether to proceed with surgery, all children with CP should have a detailed preoperative medical evaluation similar to that of scoliosis in the child with CP. The surgeon and interdisciplinary medical team must ensure that all associated comorbid conditions are optimized medically. Common comorbidities that exist include gastroesophageal reflux, aspiration pneumonia and reactive airway disease, poor nutrition, seizure disorders, and low bone mineral density and may be risk factors for postoperative complications (Lipton et al. 1999; Samdani et al. 2016; Nishnianidze et al. 2016). Risk factors should be identified preoperatively and comanaged medically (Rappaport et al. 2013a, b; Rappaport and Pressel 2008). The child with CP should also have a detailed neurological examination, including sensory and motor testing, the assessment of upper and lower extremity reflexes as well as abdominal reflexes. This helps to establish the child’s baseline neurological function and serves to assess for any undiagnosed intraspinal pathology such as tumor, tethered cord, or syringomyelia which may also occasionally occur in the child with CP. The orthopedic preoperative assessment should evaluate sitting and/or standing ability, as well as coronal, sagittal, and rotational deformity of the pelvis. Also, flexibility of the spinal and pelvic deformity is very important to evaluate. The flexibility of kyphosis can be assessed by either physical and/or radiographic examination by placing the child in the supine position and placing a bolster directly under the apex of the kyphosis to determine how well the kyphosis

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corrects. The flexibility of a hyperlordosis is more difficult to assess, which, however, can be assessed by gently flexing both hips and thighs to the chest with the child in the supine position. Radiographs can also be taken in this position to evaluate flexibility. For better accuracy, the surgeon should position the patient for these radiographs in the x-ray suite instead of allocating them to the x-ray technician. The surgical treatment of more rigid versus stiffer sagittal plane deformities will be discussed later in the chapter. The assessment for the coexistence of sagittal spinal deformity, hip flexion contracture, and/or adduction contracture is also important. This can be done by performing the Thomas Test, done by stabilizing the pelvis in a neutral position by flexing the opposite hip and assessing for hip flexion contracture on the opposite side. Alternatively, assessing for the presence of hip adduction contracture can be achieved by measuring the amount of abduction achieved with the pelvis in neutral obliquity. If these contractures are present, the parents should be advised that muscle releases may be needed 4–6 months after corrective spine surgery in order to balance infra-pelvic deformity. Also, assessment with an AP radiograph for the presence of hip subluxation should always be done in patients with spinal deformity. The surgeon should also assess if the pelvis is part of the kyphosis (posterior pelvic tilt) or lordosis, anterior pelvic tilt in order to determine whether instrumentation and fusion to the pelvis will be necessary. In the nonambulatory patient with cerebral palsy (GMFCS VI and V), fusion to the pelvis is almost always necessary to prevent distal extension of the deformity especially when lumbar kyphosis or hyperlordosis are present. Also, patients with poor head control and/or thoracic kyphosis should be considered for instrumentation and fusion up to T1 or T2 to prevent a junctional kyphosis at the cervical-thoracic junction. Patients with very proximal kyphotic deformities may even require instrumentation and fusion into the lower cervical spine. Another special high-risk group is children who develop severe lumbar lordosis following posterior dorsal rhizotomy for spasticity management. The lordosis component is often very stiff

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combined with a dense posterior surgical scar. Often the intrathecal nerve roots are also scarred. Correction with strong posterior stretching forces a center of rotation of deformity correction to be in the anterior aspect of the lumbar vertebra requiring large excursion of the nerve roots which will lead to nerve root nerve pain. It is strongly advised in the situation to first focus on anterior spinal shortening with large disk vertebral end plate wedge resections or vertebral body resection. This spinal shortening will allow lordosis correction without stretching the intrathecal neural elements.

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patients and/or those with stiff kyphotic deformities are generally not amenable to bracing (Dabney 2018). Alternatively, these patients can be treated nonoperatively with a custom-molded seat back if the kyphosis is not causing pain, recognizing that this only accommodates the kyphotic deformity. Conversely, hyperlordosis is not amenable to bracing. Spinal orthotics may not be tolerated in the child with CP due to discomfort, excessive sweating in warm weather, pressure sores, restriction of the child’s breathing, and abdominal constraint when feeding the child. The latter can be alleviated by removing the brace during and an hour after feedings.

Nonoperative Treatment Surgical Treatment Postural (flexible) kyphosis treatment in CP may be accomplished initially by using wheelchair modifications such as a tilt-in-space wheelchair with a chest harness to stop the trunk from leaning forward (Fig. 1a). In addition, a wheelchair tray table (Fig. 1b) may help prevent forward lean, while a Hensinger or soft cervical collar may assist with head control. As thoracic kyphosis becomes more rigid, a clamshell orthosis that is high in the front of the trunk and lower in the back, usually below the scapulae (Fig. 1c), may provide some assistance with upright seating. Larger

The only treatment that can make a permanent impact on the correction of CP sagittal plane deformity is spinal instrumentation and fusion. The standard surgical procedure is a posterior spinal fusion with segmental instrumentation from T1 or T2 down to the sacrum if the pelvis is part of the deformity. The pelvis and lumbar spine is almost always involved in hyperlordosis. Even if the pelvis is not part of the deformity, in the nonambulatory patient (GMFCS level IV and V) or ambulatory patient with poor balance

Fig. 1 Nonoperative methods for flexible kyphosis: (a) Tilt-in-space chair can be used to reduce a flexible neuromuscular kyphosis by tilting the chair back and (b) in

children with flexible kyphosis, the tray table prevents the child from leaning too far forward. (c) Clamshell brace used for kyphosis with high anterior extension

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(GMFCS level III), the surgeon should strongly consider fusion to the pelvis to prevent late pelvic deformity (Dabney 2018). In the past, the gold standard to instrument the correction of neuromuscular scoliosis was Luque rod instrumentation and sublaminar wires with Galveston instrumentation to the pelvis (Ferguson and Allen 1988). This was later improved upon with the Unit Rod (Surgical Treatment of Scoliosis due to Cerebral Palsy) or the cross-linkage of separate rods to prevent rod shift and rotation (Bell et al. 1989; Rinsky 1990; Dias et al. 1996; Sponseller et al. 2009; Peelle et al. 2006; Sink et al. 2003) However, this surgical approach has had mixed outcomes in managing hyperkyphosis and hyperlordosis (Lipton et al. 2003; Karampalis and Tsirikos 2014; Sink et al. 2003).

Medical/Anesthesia Considerations (Anesthesia for Cerebral Palsy Spine Fusion Surgery) The general medical status of the CP child should be evaluated using a multidisciplinary approach prior to spinal fusion with instrumentation. These considerations are described in more detail in the scoliosis ▶ Chap. 78, “Medical Evaluation for Preoperative Surgical Planning in the Child with Cerebral Palsy”. Increased perioperative morbidity, including increased blood loss, and postoperative infection are associated with increasing lumbar lordosis (Karampalis and Tsirikos 2014; Sponseller et al. 2010a; Sponseller et al. 2013; Jain et al. 2012). Our experience has also shown increased blood loss in patients with hyperlordosis. Guidelines to prevent infection are described in the scoliosis section and should also be followed for the correction of sagittal plane deformity (Vitale et al. 2013) (Surgical Treatment of Scoliosis due to Cerebral Palsy). In addition, the utilization of tranexamic acid (TXA) should be followed to reduce blood loss, especially in hyperlordotic deformity (Dhawale et al. 2012). Even with the use of TXA, the surgeon should take additional measures to prepare excessive blood loss when correcting hyperlordosis which include the availability of: typed and cross-matched blood

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(up to twice the patient’s blood volume), freshfrozen plasma, and platelets (Brenn et al. 2004). The use of cell-saver blood should be considered. Vascular access through the use of a central line should also be considered in patients with poor peripheral access (Anesthesia for Cerebral Palsy Spine Fusion Surgery). In patients with poor nutrition, a central venous catheter can also be used for postoperative hyperalimentation. We place a central venous catheter in high-risk patients with severe sagittal plane deformity. The use of pedicle screws instead of sublaminar wires has also been reported to have lower blood loss in the correction of scoliosis in CP, and this may also be true in sagittal plane deformity as well (Fuhrhop et al. 2013). Another concern in the treatment of sagittal plane spinal deformity is the neurologic risk to the spinal cord. While spinal cord injury is a risk during the correction of spinal deformity in all children with CP, rigid thoracic lordosis and kyphosis may present greater risk. Hyperlordosis may cause the spinal cord to shift more posteriorly over the apex of the lordosis which causes greater neurologic risk for posterior spinal cord injury during the corrective process and also greater risk during the passing of sublaminar wires (Fig. 2), especially in thoracic hyperlordosis where the space for the cord may be narrowed. In rigid hyperlordotic deformity, anterior release may be helpful in reducing this risk by shortening the spine and decreasing the amount of tension on the spinal cord at the apex of the lordosis. Alternatively, a rigid thoracic kyphosis may result in stretching the anterior thoracic spine over the apex of the kyphosis during correction. In severe rigid kyphosis, posterior multilevel vertebral osteotomies which shortens the spinal column can help lessen neurologic risk of spinal cord stretch. In severe rigid combined anterior release, posterior osteotomies or vertebral resection may also be helpful to lessen tension on the spinal cord in very rigid higher magnitude deformity. Many children with mild to moderate cerebral palsy (those with less severe motor cortex involvement) can have successful spinal cord monitoring using a combination of somatosensory and motor-evoked potentials (DiCindio et al. 2003). DiCindio et al. showed approximately 60% of children with cerebral palsy to be monitorable with only severe quadriplegic cerebral palsy children

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Fig. 2 This patient with spastic quadriplegic cerebral palsy (GMFCS 5) and severe thoracolumbar hyperlordosis has narrowing of the spinal canal between the lamina and the vertebral bodies especially in the thoracic spine. This places increased neurologic risk during wire passage. Using current modular fixation, curve correction may be more safely achieved using pedicle screws as fixation

with poor motor function unable to be monitored (DiCindio et al. 2003) (Surgical Spinal Cord Monitoring in Cerebral Palsy). As a general rule, somatosensory and motor evoked potential monitoring should be attempted for the child with CP who has ambulatory function and the capability to assist with standing transfers. There may also be some efficacy in monitoring neuromuscular patients with intact sensation and bowel and bladder control. Related to this, the child with CP and a neurogenic bladder preoperatively should be carefully evaluated for urinary tract infection, and, if present, should be treated prophylactically to clear the urine prior to surgery. The next important consideration in the correction of sagittal plane deformity in the child with CP is whether the child has low bone mineral density, especially prevalent in the child with CP with: greater motor involvement (GMFCS IV and V), patients on anticonvulsant medications, and patients who are nutritionally deprived (Sees et al. 2016). Adequate bone density is especially important during the cantilever correction of the sagittal plane deformity. These are highest at the apex in lordosis posteriorly and very high posteriorly at the distal- and proximal-most ends of the spine in kyphosis. In both the deformities, wire pull-out

when wires are used or screw plowing when pedicle screws are used may occur when low bone mineral density is present. Any nonambulatory child with low-impact long bone fractures should be checked for low bone density using dual energy x-ray absorptiometry (DEXA scan). Intravenous pamidronate is recommended for the child with CP with bone density two or more Z-scores below the mean and with frequent fractures (Sees et al. 2016). Children on seizure medication should have preoperative calcium, phosphorous, and vitamin D levels measured (Managing Bone Fragility in the Child with CP).

Operative Principles The principles of spinal deformity correction for sagittal plane spinal deformity in CP are to: (1) correct coronal, sagittal, and transverse plane pelvic deformity with the sitting or standing surface as a reference plane. (2) Restore coronal and sagittal truncal balance in order to center the head over the trunk and pelvis and correct anatomic sagittal alignment of the trunk and pelvis (average sacral slope of approximately 40 , pelvic tilt 13 , and lumbar lordosis of 40–60 ) (SW1 et al.

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2013). Nonambulatory children with CP should be corrected to a slightly greater than anatomic lumbar lordosis to balance the child’s body weight over the posterior thigh muscle mass. This helps to prevent sacral decubitus ulcers from occurring. Ambulatory children with CP should have relatively equal lumbar lordosis and thoracic kyphosis in order to optimize standing balance. Suh and colleagues showed a significant difference between sagittal spinopelvic parameters in the CP child compared to normal control children and that these abnormal parameters may be related to the symptoms seen in CP children (SW1 et al. 2013). The correction of these parameters during spine surgery is therefore critically important in the child with primary sagittal plane deformity. (3) Maximize segmental fixation in the face of what is often osteoporotic bone and (4) minimize operative time since children with CP often have multiple comorbidities, excessive bleeding, and a greater risk for wound infection (Sponseller et al. 2010a; Sponseller et al. 2013; Jain et al. 2012).

Preoperative Planning When planning preoperatively for surgery, three technical questions deserve careful thought: (1) Should fusion include the pelvis? (2) Is there a rotational component to the spinal deformity that is affecting sitting or standing balance that will definitively require pedicle screw fixation over sublaminar wires? (3) Is there poor flexibility of the sagittal deformity that will warrant: preoperative or intraoperative traction, anterior release, concurrent posterior-only osteotomies, or total vertebral resection?

Current Preferred Surgical Treatment Methods (Spinal Procedure Atlas for Cerebral Palsy Deformities) Intraoperative Positioning The patient is positioned prone with the abdominal area left free in order to minimize abdominal pressure and therefore bleeding. Patients with lumbar kyphosis should have their hips and

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knees flexed to create maximum lordosis, while patients with hyperlordosis should have their legs left to hang freely, minimizing excessive lordosis (Fig. 3). This position also helps to minimize the stress on the wire or screw/bone interface during the correction maneuver for hyperlordosis. All bony prominences should be well padded and minimal tension/pressure should be placed on contracted extremities. Urinary catheters should be free flowing, especially in children with neurogenic bladder, vesicostomy, and/or other bladder reconstruction.

Instrumentation The Unit Rod was previously shown to be effective in the correction of both hyperlordosis and kyphosis; however, the pelvic limbs are difficult to insert, especially with hyperlordosis, making the risk of pelvic limb penetration through the pelvic wall high (Lipton et al. 2003; Dabney et al. 2004). Newer methods of instrumentation allow modularization of the Unit Rod concept and cantilever correction by combining wires or pedicle screws with two pelvic screws placed independently into the pelvis and connected to two rods pre-contoured in the sagittal plane to restore sagittal alignment and a proximal connector (Fig. 4). The pelvic screws have varying diameter of 7–10 mm and length between 65 mm and 100 mm that can be selected according to the pelvic size of the child. Because the pelvic screws can be positioned separately into the pelvis, there is less risk of penetration through the inner pelvic wall as did the Unit Rod, especially in hyperlordotic spinal deformity (Fig. 5). Pelvic screws also provide better fixation into the pelvis, decreasing distal screw pull-out (Erickson et al. 2004). Achieving proper sagittal balance is critical as described earlier which may require recontouring the lumbar bend to have greater or less lordosis than the manufactured contour. Either sublaminar wires or pedicle screw fixation can be used; however, many surgeons prefer to use pedicle screws given their greater rigidity instead of wires for segmental fixation, especially if there is a severe kyphotic or lordotic deformity. On the other hand, sublaminar wires/tapes may be

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Fig. 3 Positioning of the patient should allow the abdomen to be free and in cases of hyperlordosis, the hips are flexed to 90 degrees with the trunk and the legs hang freely to allow as much passive correction of the hyperlordosis as possible

Fig. 4 This modular system consists of (a) two rods with a sagittal contour which are (b) connected by a closed connector proximally and a cross-link at the thoracolumbar

junction. The pelvic screws are anchored into the pelvis separately which allows easier pelvic placement than the Unit Rod

just as efficient with correcting a hyperlordotic lumbar spine into a more anatomic alignment (Dabney et al. 2004). In hyperlordotic spines, great caution

should be taken to prevent pedicle screw pull-out (“plowing”) if using screws, and laminar fracture if using wires, especially with osteopenic bone. If

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Fig. 5 Placement of the pelvic limbs of the unit rod is difficult in hyperlordosis due to the far anterior start point required for the pelvic limbs to enter the pelvis. The drill hole and rod limbs (the latter of which must be crossed in order to enter the pelvis properly) must aim just in front of

the sciatic notch and aim distal and posterior. Failure to do so may cause the rod limb to penetrate the inner pelvic table as shown. Placing separate pelvic screws attached to pre-contoured rods is technically easier

significant sagittal plane stiffness is present on either physical or radiographic examination, preoperative halo-femoral/intraoperative traction, posterior only osteotomies, anterior discectomies, and/or total vertebral resection should be considered. The choice of these procedures is determined by the magnitude and stiffness of the deformity and the type of sagittal plane deformity and will be discussed later in this chapter.

with less complication than the Unit Rod construct (Fig. 6). Pelvic screws can be inserted into the pelvis at the traditional posterior superior iliac spine entrance or can be placed using an S2 iliac approach. A pedicle probe is used for either approach to enter the pelvis. In the former, the pedicle probe enters at the posterior superior iliac spine and aims just above the sciatic notch using intraoperative fluoroscopy. This is the region where the pelvis is most dense for pelvic screw fixation (Miller et al. 1990). By not fully exposing the sciatic notch as previously done with the Unit Rod procedure, blood loss is minimal. Intraoperative AP and “tear drop” fluoroscopic views are taken to confirm the placement of the probe to make sure that there is no penetration through the inner or outer pelvic table, or into the sciatic notch (Fig. 7). A pelvic screw with the largest diameter possible (usually 7–10 mm) is placed in this trajectory and should be an adequate length to pass the sciatic notch by at least 1 cm. The author prefers to use a closed polyaxial screw

Fusion to the Pelvis The extension of fixation and fusion to the pelvis should be considered in every patient with CP with a sagittal plane spinal deformity that extends to the pelvis. Similar to the correction of pelvic obliquity in scoliosis, cantilever correction is an excellent method to correct both anterior and posterior pelvic tilt in the sagittal plane using pelvic screws connected to dual pre-contoured rods connected to a proximal connector as described

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Fig. 6 This patient with spastic quadriplegic cerebral palsy and severe lumbar hyperlordosis underwent posterior spinal fusion with Unit Rod instrumentation. Due to the inability to achieve the proper trajectory of the Unit Rod pelvic limbs into the pelvis, the correction of anterior pelvic tilt was insufficient. The introduction of separated pelvic screws and pedicle screws in the lumbar spine would have obviated the failure to correct this severe anterior pelvic tilt

head which maximizes the rigidity of the final rod-pelvic screw construct. Usually, only two pelvic screws alone are used for pelvic fixation; however, if additional fixation is needed to improve the correction of a rigid pelvic deformity, S1 screws can be added to the construct. Alternatively, pelvic screws can be placed using the medial portal (S2-iliac approach) as described by Chang et al. and Sponsellar (Chang et al. 2009; Sponseller et al. 2010b). Advocates for this method claim less exposure time, less bleeding, and that the pelvic screw head is less prominent and more in line with the rod, making the need for a separate connection to the rod unnecessary. While we have not found bleeding or exposure time to be less in our hands, the screw

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is less prominent using this approach and lines up more directly with the pre-contoured rods, avoiding the need for lateral rod connectors. If the traditional PSIS start point is used, notching the ilium at the entrance point with a rongeur and countersinking the screw prevents screw head prominence. A fixed lateral rodded connector, 10–20 mm in length) is used to connect each pelvic screw to a pre-contoured rod. Critical to the correction is to attach and secure each of the pre-contoured rods to the iliac screws with the fixed lateral connectors so that each of the rods are perfectly perpendicular to the horizontal axis of the pelvis and that the sagittal contour of the rods are parallel with one another and aligned with the sacrum (Figs. 4 and 8) (Dabney 2018). The sagittal bend of each rod should be identical from proximal to distal. In addition, the sagittal contours for lumbar lordosis and thoracic kyphosis of the rod should match the length of the lumbar and thoracic spine, respectively. If these steps are not meticulously followed, the sagittal alignment of the pelvis and thoracolumbar spine may not be optimally corrected with the cantilever maneuver. Once the correction is obtained, the set screws on both the pelvic screws can be tightened and torqued down onto the rod. A proximal connector is added at the top of the construct which strengthens the proximal construct. A cross connector should be added at the thoracolumbar junction to augment the stability of the construct. Only if the patient preoperatively has a level pelvis, a correct sagittal pelvic position, and adequate balance, should the surgeon consider ending the fusion and fixation more proximally at the L4 or L5 vertebrae. If fixation to the pelvis is not done, distal pedicle screw fixation in the lumbar spine at a minimum of four levels is recommended. In severe lumbar lordosis, pedicle screws should be considered at each level. Cantilever correction with fixation using pedicle screws or sublaminar wires to correct the remainder of the sagittal alignment can then be done to complete the thoracic spine correction. Hyperlordosis correction requires another corrective technique that will be described.

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Fig. 7 Intraoperative AP (a, b) and oblique (c, d) views showing proper placement of pelvic screw. Note the AP view shows the trajectory of the pedicle probe from the PSIS to just superior and adjacent to the sciatic notch and the final screw position at least 1 cm lateral to the notch.

Kyphosis Correction Lumbar and Thoracolumbar Kyphosis Cantilever correction is very effective in correcting both lumbar and thoracolumbar kyphosis in the child with CP. Each is effectively corrected utilizing a distal-to-proximal cantilever correction with the modular dual contoured rod construct described, beginning with fixation to the pelvis similar to the cantilever correction described with the Unit Rod (Dabney et al.

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The oblique view is taken parallel with the probe and shows the probe and the final screw position between the inner and outer cortex just superior to the sciatic notch which appears as a “teardrop”

2004). The surgeon places the pelvic screws, attaches the pre-contoured rods, and then begins cantilever correction (Fig. 9). Next, the surgeon progressively pushes the rod down to each vertebra at a time, securing each vertebral level with sublaminar wires or screws. The surgeon should not use the fixation to pull the rod to the spine, as this may cause loss of fixation (either wires cutting through the laminae or pedicle screw pullout). The process of securing the rod to the fixation at each level begins at the L5 vertebral level and progresses gradually up to the T2 or T1 vertebral level. The placement of pedicle screws with

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Fig. 8 Pelvic fixation is performed first with any distal to proximal cantilever correction. (a) The pelvic screws are placed as shown in this anteroposterior radiograph. (b) The construct is then assembled from distal to proximal securing the rods to the pelvic screws using the rodded

connectors shown if using a traditional PSIS entrance into the pelvis. A proximal closed connector and crosslink at the thoracolumbar junction connect the two pre-contoured rods which should be parallel to one another

Fig. 9 In lumbar and thoracolumbar kyphosis, a distal to proximal cantilever correction is performed, first fixing the rod to distal vertebrae and then pushing down (anterior) on the rod after the rod is anchored to the apical vertebrae. The sagittal placement of the rod should initially be parallel to

the pre-corrected sagittal alignment of the sacrum which is tilted posterior along with the pelvis in kyphosis. As the rod is moved to the spine using cantilever correction, the sagittal alignment of the pelvis and spine will correct to the sagittal contour of the rod

reduction posts at the proximal ends of the spine is helpful to capture the proximal rod ends as the kyphosis is gradually being corrected. Pedicle screws along the entire spine may also be helpful

to perform compression of the vertebrae posteriorly to further correct the kyphosis. The pelvis which is typically posteriorly tilted should also be corrected as a part of the cantilever correction.

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Thoracic Kyphosis Thoracic kyphosis is difficult to correct using a distal to proximal cantilever correction technique because the lever arm remains too short above the apex of the kyphosis to provide an adequate cantilever correction. Accordingly, thoracic kyphosis is difficult to correct with the Unit Rod since it requires distal fixation into the pelvis first. With this type of curvature, a proximal to distal cantilever correction is preferred when using the more modular system (Fig. 10). After exposing the spine and pelvis, the pre-contoured rods are connected using a proximal closed rod connector at the T1 level and a cross connector in the lumbar spine. The rods should be parallel from proximal to distal with respect to their contour. Next, pelvic screws and sublaminar wires are placed as previously described. The top of the rod construct is then secured to the spine using sublaminar wires or pedicle screws from T1 down to the apex of the kyphosis. In thoracic kyphosis, great care should be taken to preserve the spinous process ligaments in order to prevent a junctional kyphosis (Fig. 11).

Fig. 10 It is difficult to cantilever thoracic kyphosis using the Unit Rod due to insufficient lever arm. This diagram shows a proximal to distal cantilever technique that can be used for thoracic kyphosis. The rod is preassembled and

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After the apical vertebrae is secured to the rod, cantilever correction can be performed by gradually pushing the rod down to the next more distal vertebrae, tightening the sublaminar wire or securing it to the pedicle screw, performing the same maneuver progressively down the spine until the rod is secured to each of the two pelvic screws. Pelvic screws with reduction posts are helpful to capture the distal-most end of the rods. Similar to lumbar or thoracolumbar kyphosis, pedicle screws placed along the kyphosis can be helpful to compress and further correct the kyphosis. The fixed rodded lateral connectors are then utilized to connect the rod to the pelvic screws or directly to the pelvic screw if the S2 iliac technique is utilized. Ambulatory patients without pelvic deformity can be fused short of the pelvis and secured to pedicle screws at the L4 or L5 vertebrae. In thoracic kyphosis, it is critical that fixation be completed up to at least the T1 vertebral level and occasionally the C7 level to prevent “dropoff” at the cervicothoracic junction. Firm fixation at the proximal-most end with two wires, hooks, or screws is recommended (Dabney 2018).

secured proximally and then delivered into lumbar pedicle screws if there is no pelvic sagittal misalignment. Preoperative and postoperative radiographs are shown

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Fig. 11 (a) This patient with quadriplegic CP (GMFCS 5) and thoracic kyphosis with poor head control (b) underwent posterior spinal fusion with Unit Rod instrumentation. (c) By 3 months post-op, he developed a

significant junctional kyphosis at the cervical-thoracic junction. (d) This eventually necessitated extension of the fusion up to the occiput

Hyperlordosis

for additional fixation if screw pull-out is beginning to occur. After the hyperlordosis is corrected, the rest of the spinal instrumentation can be completed using cantilever correction.

Isolated neuromuscular lumbar hyperlordosis does occur but is more frequently seen in combination with scoliosis or thoracic kyphosis. Pelvic screws are placed first followed by pedicle screw fixation with reduction posts in the vertebrae within the hyperlordosis (usually in the lumbar spine) (Fig. 12) (Dabney et al. 2004). After securing the pre-contoured rods to the pelvic screws, the rods are pushed down into the reduction posts and secured set using screws. Reduction of the hyperlordosis cannot be solely achieved using cantilever correction alone but requires incrementally screwing down the set screws, gradually increasing the load share over each of the screws, a small amount at a time. Great care is taken to notice any evidence of posterior plowing of the pedicle screws. Maximizing the diameter of the screws may help with improved pedicle fixation. In addition, the supplementation of sublaminar wires can be used at the same level as the screw

Rigid Kyphotic and Hyperlordotic Deformities Rigid thoracic and thoracolumbar kyphosis may be difficult to correct using posterior spinal fusion with instrumentation alone. Some authors have shown that multiple level posterior-only (Ponte, vertebral, or Smith-Petersen) osteotomies with or without anterior discectomies as a first stage followed by posterior instrumentation is successful to correct severe kyphotic deformity. Corrective osteotomies may also decrease the excessive corrective forces required by shortening of the vertebral column (Diab et al. 2011; Dorward and Lenke 2010; Geck et al. 2007; Auerbach et al. 2009). It may also lessen neurologic risk to the cord during

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Fig. 12 (a) Correction of hyperlordosis can be achieved using pedicle screws with reduction posts in the hyperlordotic region of the deformity after pelvic fixation of the

rod is done. Screw tightening should be gradual and incremental to share the load across all screws (b) Preoperative and postoperative photographs are shown

correction. Preoperative or intraoperative traction has been recommended by some as an alternative to anterior release for rigid spinal deformities,

specifically scoliotic deformity (Takeshita et al. 2006; Keeler et al. 2010; Jackson et al. 2018). Little is written about halo-femoral traction and

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Fig. 13 (a) Severe lordoscoliosis (with primary lordosis) which underwent (b) a staged anterior vertebrectomy followed by (c) posterior completion to a total vertebrectomy with posterior instrumentation and fusion

its use in sagittal plane deformity. Rigid hyperlordotic deformity may require staged anterior release (multiple anterior discectomies) at the rigid apex of the lordosis followed by posterior spinal fusion with instrumentation (Lipton et al. 2003; Dabney et al. 2004; Geck et al. 2007). In severely rigid hyperlordotic and kyphotic deformity, vertebral column resection can produce excellent curve correction and restoration of sitting balance (Helenius et al. 2012; Sponseller et al. 2012; Modi et al. 2011). This can be achieved as a staged anterior/posterior vertebral resection or posterior only vertebral resection (Modi et al. 2011) (Fig. 13).

Evidence-Based Outcomes Lipton et al. was the first to describe a series of 24 children with cerebral palsy with isolated sagittal plane spinal deformity (8 with hyperlordotic deformity, 14 with kyphotic deformity, and 2 with both) (Lipton et al. 2003). Each sagittal plane deformity underwent posterior spinal fusion and cantilever correction using Unit Rod instrumentation. The indications for surgery included back pain, seating problems despite wheelchair modifications, and two cases of superior mesenteric

artery syndrome refractory to conservative treatment in children with hyperlordosis. In children with kyphotic deformity, the mean preoperative kyphosis of 93.8 was corrected to a mean postoperative kyphosis of 35.8 , while the mean preoperative hyperlordosis of 91.8 was corrected to a mean postoperative lordosis of 43.6 in children with hyperlordosis. Postoperatively, caregivers reported improvements in: sitting balance, head control, pain relief, and physical appearance. Both cases of superior mesenteric artery syndrome resolved after spinal deformity correction. Karampalis and Tsirikos reported on 13 patients with lumbar hyperlordosis and lordoscoliosis who underwent posterior spinal fusion with instrumentation (Karampalis and Tsirikos 2014). The mean lumbar lordosis was corrected from 108 to 62 postoperatively. Sacral slope (horizontal sacral inclination) improved from 79 to 50 . Sagittal imbalance was improved from a mean of 8 cm to 1.8 cm. Preoperative lumbar lordosis and sacral slope had an increased risk of perioperative morbidity. Reduced lumbar lordosis and increased thoracic kyphosis were associated with improved sagittal balance at follow-up. Postoperative questionnaires at the final follow-up showed relief of severe preoperative back pain and improvements in physical appearance and function. There were

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also improvements in head control, breathing, and hand use (Dabney 2018). Sink et al. looked at a retrospective case series of 24 patients with patients had preoperative kyphotic deformities (Sink et al. 2003). Preoperative thoracic, thoracolumbar, and lumbar kyphosis were risk factors for loss of proximal and distal sagittal fixation and therefore correction. The authors stated that increased forces at the proximal- and distal-most end (Galveston fixation) of the instrumentation during kyphosis correction resulted in the greatest potential for failure. They recommended reinforcing these ends with stronger fixation. We prefer to use the largest diameter pelvic screw fixation which in our experience is less likely to pull-out compared to the Unit Rod or Galveston fixation. Proximal loss of correction occurred in 11 patients who developed a junctional kyphosis. Securing fixation proximally with two wires, screws, or hooks provide a more secure proximal fixation.

Summary Sagittal plane spinal deformities (kyphosis and hyperlordosis) are uncommon by themselves in cerebral palsy; however, when present can interfere with proper sitting and standing balance. Sagittal plane spinal deformity in conjunction with scoliosis is more common and must be treated surgically as a component of the scoliosis. Mild and some moderate sagittal plane deformities can be treated by wheelchair modifications and bracing. Symptomatic moderate and severe deformity may require surgical treatment. More flexible kyphosis and hyperlordosis can be corrected by posterior spinal fusion and segmental instrumentation alone while rigid deformity usually requires posterior osteotomies (for kyphosis) and/or anterior discectomies (for hyperlordosis). Instrumentation and correction techniques vary from screw/rod constructs using distraction/compression correction to wire or screw/rod constructs using cantilever correction. Overall, natural history and surgical outcome studies focused solely on sagittal plane spinal deformities in CP are limited. Those authors that do measure functions report improvements in pain, sitting balance, head and neck control,

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breathing, and hand use. Patients with kyphosis undergoing spinal fusion with instrumentation are at risk for loss of proximal and/or distal fixation. Patients with hyperlordosis appear to be at greatest risk for postoperative complications.

Cross-References ▶ Anesthetic Management of Spine Fusion ▶ Managing Bone Fragility in the Child with Cerebral Palsy ▶ Spinal Procedure Atlas for Cerebral Palsy Deformities ▶ Surgical Treatment of Scoliosis Due to Cerebral Palsy

References Bell DF, Moseley CF, Koreska J (1989) Unit rod segmental spinal instrumentation in the management of patients with progressive neuromuscular spinal deformity. Spine (Phila Pa 1976) 14(12):1301–1307 Brenn BR, Theroux MC, Dabney KW, Miller F (2004) Clotting parameters and thromboelastography in children with neuromuscular and idiopathic scoliosis undergoing posterior spinal fusion. Spine (Phila Pa 1976) 29(15):E310–E314 Chang TL, Sponseller PD, Kebaish KM, Fishman EK (2009) Low profile pelvic fixation: anatomic parameters for sacral alar-iliac fixation versus traditional iliac fixation. Spine (Phila Pa 1976) 34(5):436–440 Dabney K (2018) Sagittal plane spinal deformity in patients with neuromuscular disease. In: Samdani AF et al (eds) Neuromuscular spine deformity. https://doi. org/10.1055/b-0038-162475, Part II: Disease Specific Dabney KW, Miller F, Lipton GE, Letonoff EJ, McCarthy HC (2004) Correction of sagittal plane spinal deformities with unit rod instrumentation in children with cerebral palsy. J Bone Joint Surg Am 86-A(Suppl 1(Pt 2)):156–168 Dhawale AA, Shah SA, Sponseller PD et al (2012) Are antifibrinolytics helpful in decreasing blood loss and transfusions during spinal fusion surgery in children with cerebral palsy scoliosis? Spine (Phila Pa 1976) 37(9):E549–E555 Diab MG, Franzone JM, Vitale MG (2011) The role of posterior spinal osteotomies in pediatric spinal deformity surgery: indications and operative technique. J Pediatr Orthop 31(1 Suppl):S88–S98 Dias RC, Miller F, Dabney K, Lipton G, Temple T (1996) Surgical correction of spinal deformity using a unit rod in children with cerebral palsy. J Pediatr Orthop 16(6):734–740

1760 DiCindio S, Theroux M, Shah S, Miller F, Dabney K, Brislin RP, Schwartz D (2003) Multimodality monitoring of transcranial electric motor and somatosensoryevoked potentials during surgical correction of spinal deformity in patients with cerebral palsy and other neuromuscular disorders. Spine (Phila Pa 1976) 28(16):1851–1855 Dorward IG, Lenke LG (2010) Osteotomies in the posterior-only treatment of complex adult spinal deformity: a comparative review. Neurosurg Focus 28(3):E4 Erickson MA, Oliver T, Baldini T et al (2004) Biomechanical assessment of conventional unit rod fixation versus a unit rod pedicle screw construct: a human cadaver study. Spine (Phila Pa 1976) 29:1314–1319 Ferguson RL, Allen BL Jr (1988) Considerations in the treatment of cerebral palsy patients with spinal deformities. Orthop Clin North Am 19(2):419–425 Fuhrhop SK, Keeler KA, Oto M, Miller F, Dabney KW, Bridwell KH, Lenke LG, Luhmann SJ (2013) Surgical treatment of scoliosis in non-ambulatory spastic quadriplegic cerebral palsy patients: a matched cohort comparison of unit rod technique and all-pedicle screw constructs. Spine Deform 1(5):389–394 Geck MJ, Macagno A, Ponte A, Shufflebarger HL (2007) The Ponte procedure: posterior only treatment of Scheuermann's kyphosis using segmental posterior shortening and pedicle screw instrumentation. J Spinal Disord Tech 20(8):586–593 Helenius I, Serlo J, Pajulo O (2012) The incidence and outcomes of vertebral column resection in paediatric patients: a population-based, multicentre, follow-up study. J Bone Joint Surg Br 94(7):950–955 Jackson TJ, Yaszay B, Pahys JM, Singla A, Miyanji F, Shah SA, Sponseller PD, Newton PO, Flynn JM, Cahill PJ, Harms Study Group (2018) Intraoperative traction may be a viable alternative to anterior surgery in cerebral palsy scoliosis 100 degrees. J Pediatr Orthop 38(5):e278–e284 Jain A, Njoku DB, Sponseller PD (2012) Does patient diagnosis predict blood loss during posterior spinal fusion in children? Spine (Phila Pa 1976) 37(19):1683–1687 Auerbach JD, Spiegel DA, Zgonis MH, Reddy SC, Drummond DS, Dormans JP, Flynn JM (2009) The correction of pelvic obliquity in patients with cerebral palsy and neuromuscular scoliosis: is there a benefit of anterior release prior to posterior spinal arthrodesis? Spine (Phila Pa 1976) 34(21):E766 Karampalis C, Tsirikos AI (2014) The surgical treatment of lordoscoliosis and hyperlordosis in patients with quadriplegic cerebral palsy. Bone Joint J 96-B(6):800–806 Keeler KA, Lenke LG, Good CR, Bridwell KH, Sides B, Luhmann SJ (2010) Spinal fusion for spastic neuromuscular scoliosis: is anterior releasing necessary when intraoperative halo-femoral traction is used? Spine (Phila Pa 1976) 35(10):E427–E433 Lipton GE, Miller F, Dabney KW, Altiok H, Bachrach SJ (1999) Factors predicting postoperative complications following spinal fusions in children with cerebral palsy. J Spinal Disord 12(3):197–205

K. W. Dabney Lipton GE, Letonoff EJ, Dabney KW, Miller F, McCarthy HC (2003) Correction of sagittal plane spinal deformities with unit rod instrumentation in children with cerebral palsy. J Bone Joint Surg Am 85-A(12):2349–2357 McCarthy JJ, Betz RR (2000) The relationship between tight hamstrings and lumbar hypolordosis in children with cerebral palsy. Spine (Phila Pa 1976) 15:211–213 Miller F, Moseley C, Koreska J (1990) Pelvic anatomy relative to lumbosacral instrumentation. J Spinal Disord 3:169–173 Modi HN, Suh SW, Hong JY, Yang JH (2011) Posterior multilevel vertebral osteotomy for severe and rigid idiopathic and nonidiopathic kyphoscoliosis: a further experience with minimum two-year follow-up. Spine (Phila Pa 1976) 36(14):1146–1153 Nishnianidze T, Bayhan IA, Abousamra O, Sees J, Rogers KJ, Dabney KW, Miller F (2016) Factors predicting postoperative complications following spinal fusions in children with cerebral palsy scoliosis. Eur Spine J 25(2):627–634 Peelle MW, Lenke LG, Bridwell KH et al (2006) Comparison of pelvic fixation techniques in neuromuscular spinal deformity correction: Galveston rod versus iliac and lumbosacral screws. Spine (Phila Pa 1976) 31:2392–2398 Rappaport DI, Pressel DM (2008) Pediatric hospitalist comanagement of surgical patients: challenges and opportunities. Clin Pediatr (Phila) 47(2):114–121 Rappaport DI, Cerra S, Hossain J, Sharif I, Pressel DM (2013a) Pediatric hospitalist preoperative evaluation of children with neuromuscular scoliosis. J Hosp Med 8(12):684–688 Rappaport DI, Adelizzi-Delany J, Rogers KJ, Jones CE, Petrini ME, Chaplinski K, Ostasewski P, Sharif I, Pressel DM (2013b) Outcomes and costs associated with hospitalist comanagement of medically complex children undergoing spinal fusion surgery. Hosp Pediatr 3(3):233–241 Rinsky LA (1990) Surgery of spinal deformity in cerebral palsy. Twelve years in the evolution of scoliosis management. Clin Orthop Relat Res 25(3):100–109 Samdani AF, Belin EJ, Bennett JT, Miyanji F, Pahys JM, Shah SA, Newton PO, Betz RR, Cahill PJ, Sponseller PD (2016) Major perioperative complications after spine surgery in patients with cerebral palsy: assessment of risk factors. Eur Spine J 25(3):795–800 Sees JP, Sitoula P, Dabney K, Holmes L Jr, Rogers KJ, Kecskemethy HH, Bachrach S, Miller F (2016) Pamidronate treatment to prevent reoccurring fractures in children with cerebral palsy. J Pediatr Orthop 36(2):193–197 Sink EL, Newton PO, Mubarak SJ, Wenger DR (2003) Maintenance of sagittal plane alignment after surgical correction of spinal deformity in patients with cerebral palsy. Spine (Phila Pa 1976) 28(13):1396–1403 Sponseller PD, Shah SA, Abel MF et al (2009) Scoliosis surgery in cerebral palsy: differences between unit rod and custom rods. Spine (Phila Pa 1976) 34:840–844

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Sponseller PD, Shah SA, Abel MF, Newton PO, Letko L, Marks M (2010a) Infection rate after spine surgery in cerebral palsy is high and impairs results: multicenter analysis of risk factors and treatment. Clin Orthop Relat Res 468(3):711–716 Sponseller PD, Zimmerman RM, Ko PS et al (2010b) Low profile pelvic fixation with the sacral alar iliac technique in the pediatric population improves results at two-year minimum follow-up. Spine (Phila Pa 1976) 35(20):1887–1892 Sponseller PD, Jain A, Lenke LG et al (2012) Vertebral column resection in children with neuromuscular spine deformity. Spine (Phila Pa 1976) 37(11):E655–E661 Sponseller PD, Jain A, Shah SA et al (2013) Deep wound infections after spinal fusion in children with cerebral

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palsy: a prospective cohort study. Spine (Phila Pa 1976) 38(23):2023–2027 Suh SW, Suh DH, Kim JW, Park JH, Hong JY (2013) Analysis of sagittal spinopelvic parameters in cerebral palsy. Spine J 13(8):882–888 Takeshita K, Lenke LG, Bridwell KH, Kim YJ, Sides B, Hensley M (2006) Analysis of patients with nonambulatory neuromuscular scoliosis surgically treated to the pelvis with intraoperative halo-femoral traction. Spine (Phila Pa 1976) 31(20):2381–2385 Vitale MG, Riedel MD, Glotzbecker MP et al (2013) Building consensus: development of a Best Practice Guideline (BPG) for surgical site infection (SSI) prevention in high-risk pediatric spine surgery. J Pediatr Orthop 33(5):471–478

Early-Onset Scoliosis in Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1764 Natural History and Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1764 Treatment Options for Early-Onset Scoliosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Early Short Fusion as an Option . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Growing Rod Constructs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Spinal Deformity in Very Small Children Who Are Older . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Recommendation for Early-Onset Spine Fusion in Children with CP . . . . . . . . . . . 1766 Long-Term Outcome of Early Spine Fusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1767 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1767 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1767 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1776 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1776

Abstract

There is a group of children with cerebral palsy who developed very-early-onset scoliosis before the age of 7. This scoliosis may be associated with hip dislocation or asymmetric hip contractures as the primary driving force of the scoliosis. In children with hip dislocation or asymmetric contractures and scoliosis under the age of 7 years, first the hip problems should be addressed. If the hip problems are corrected

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_117

and the patient has been monitored with increasing scoliosis curve size and stiffness, then surgery should be considered. The surgical options to consider include short apical fusion, a growing rod construct using either MAGEC rod or a classic growing rod construct. Growing rods have reported very high complication rates; however, there are no reports at this time of the MAGEC rod use in children with spasticity. Children between the ages of 7 and 9 years whose scoliosis becomes large and stiff can be monitored with radiographs every 6 months. When the scoliosis curve reaches 90 or becomes very stiff, complete spine (T1 to pelvis) fusion is recommended. Children who 1763

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develop early-onset scoliosis tend to have severe medical problems and generally are fragile with most of them having seizures and requiring gastrostomy tubes for feeding. Approximately a quarter of the children require tracheostomies. The mortality in this group of patients is approximately 25% 10 years after the spine fusion. This mortality is not related to the spinal fusion or surgery but due to the many underlying medical problems. Keywords

Cerebral palsy · Early-onset scoliosis · Scoliosis · Growing rods · Kyphosis · Spine fusion · Pelvic obliquity

Introduction The term early-onset scoliosis has been defined by the Scoliosis Research Society as occurring in children before the age of 10. This is the age that scoliosis starts to develop in children with cerebral palsy with the most common onset time between the ages of 8 and 10. Therefore this definition includes many of the children with CP. The vast majority of children with cerebral palsy including those with gross motor function classification system (GMFCS) levels IVand V have spines that are very flexible although they tend to lean over and have the appearance of scoliosis before the age of 8–10. On careful physical examination however, these spines tend to be very flexible, and they can just as easy side bend to the opposite direction. This concept of early-onset scoliosis in CP should specifically focus on those children who developed a very significant structural curve which is felt to be due to structural spinal deformity, not a collapsing flexible curve. It is also important to make sure the scoliosis is not being driven by infrapelvic pelvic obliquity causing a compensatory scoliosis. This compensatory scoliosis is really part of the syndrome of asymmetric hip contractures often with hip subluxation or dislocation and should be seen as part of that treatment algorithm. In these children, the hips need to be addressed first, and then the scoliosis almost always will stabilize or even have significant

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correction (Case 1). The group of children who classify as early-onset scoliosis are those in whom the pelvic obliquity is not being driven by deformities at the hips, but they have a fixed deformity of the spine which may be causing the pelvis to tilt. Often a component of the scoliosis in young children includes a significant kyphosis which needs to be monitored. Many of these early-onset scolioses also are not the typical long C-shaped curves that are more commonly seen with the adolescent developing scoliosis of cerebral palsy. The group of children who develop true early-onset scoliosis with cerebral palsy often are part of congenital syndromes or have congenital syndromic features. This makes this group of children very heterogeneous and in many ways quite difficult to classify. The goal of this chapter will be to review the various presentations that developed in earlyonset scoliosis in children with cerebral palsy.

Natural History and Etiology Early-onset scoliosis in children with cerebral palsy should be classified into those children who really have the very-early-onset scoliosis that this is significant fixed structural deformity before the age of 7 years. Children with this extreme early-onset pattern of scoliosis tend to be very rare. In my experience they only occur in approximately 1 out of every 200–400 children with scoliosis. The pattern of scoliosis in these very young children tends to be sharper, and more stiff scoliosis curves often with significant kyphosis and often involving more the thoracic spine than the typical long curve are seen in adolescents with cerebral palsy scoliosis (Case 2). There is a second group of children whose scoliosis develops early defined as those between the ages of 7 and 10. Many of these children are at the upper end of this age range and in fact fall into the typical pattern of cerebral palsy scoliosis development. There are some children who are very small at that upper end who may be similar to the very-early-onset type. The diameter of the chest wall continues to grow, and over time the ribs tend to grow down over the pelvis; however, this does not seem to cause any problem (Case 3). This

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usually occurs in child fused before age 9; however, in some children fused at a later age, this may still become evident. The life expectancy of these children with cerebral palsy and very-earlyonset scoliosis fused before the age of 9 years is more limited than the children who developed scoliosis later. In one review the mortality rate was 28% after 10 years and predicted to be 50% at 15 years (Sitoula et al. 2016). It is our belief that the cause of the increased mortality is due to the severity of the cerebral palsy and not due to the early spine fusion; however, there is no definitive evidence to support this. Early-onset kyphotic deformity may also occur, almost always it is a flexible collapsing kyphosis in sitting, and when the child lies supine, they will be flat or near flat. It is only in the very rare case that this becomes stiff before the age of 9 or 10 years. Paying attention to good wheelchair adjustment especially keeping the lap tray high will tend to be able to manage this until adolescent growth. If the curve is becoming stiff in a very young child under age 7 years, it is always associated with scoliosis in my experience. The management then follows the scoliosis protocol. Symptomatic hyperlordosis is never been seen in my experience in the young child.

Treatment Options for Early-Onset Scoliosis The treatment of early-onset scoliosis is focused at preventing severe and progressive curve progression to the point where restrictions on respiratory function and gastrointestinal function. There is a general concern in the treatment of early-onset scoliosis to preserve spinal growth potential because spinal growth potential can impact on the development of pulmonary function especially on lung volume. In the children with cerebral palsy whose physical function usually is extremely limited because of their very severe neurologic disability, the concern for preserving pulmonary volume may be less important, and furthermore these children have normal potential for lateral chest wall growth. They do not have the restrictions that many congenital syndromes have

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with regard to lateral chest wall growth. The option to consider in treating early scoliosis is first observation. When the scoliosis is initially identified, observation should always be the first choice. Sometimes these curves will be fairly substantial; however, they will remain stable when the child is not growing very fast. Therefore, one can monitor them with x-rays every 4–6 months to either document progression or document that the curve is stable. Other options for treating early scoliosis include bracing and casting which are more commonly used for idiopathic early-onset scoliosis. These options have very limited possibility in the children who are medically extremely fragile as the ones with cerebral palsy almost always are. The primary option is surgical treatment which includes early fusion and growing rod constructs.

Early Short Fusion as an Option Occasionally, children develop a spinal curve that is very stiff with a severe magnitude as early as age 3–5 years. These curves may approach 90 in magnitude and may become very stiff, making orthotic management difficult. Orthotic management also usually fails because the children have difficulty tolerating the orthosis. If the curve is in the thoracic area only, a limited anterior and posterior fusion in the thoracic area using sublaminar wires or pedicle screw construct is the recommended treatment (Cases 2 and 5). The goal of this treatment is to stabilize the sharp curve section and then extend the fusion to the pelvis in case the curve deteriorates as the children grow. This extension will at least allow children to gain height through growth from the lumbar vertebrae. In one child, this growth continued substantially, and a scoliosis in the lumbar spine has not developed; therefore, no additional treatment was required (Case 4). In another example, growth continued for 6 years, and then a rather severe curve extending into the lumbar spine and pelvis developed, requiring a revision surgery. Substantial height was gained, however, and treatment was successful (Case 5). Another option is to implant a spinal rod that is fixed with sublaminar

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wires but does not have a fusion. It is hoped that children will continue to get taller, growing off the superior end of the rod; however, our success with the concept has not worked well. The growth phenomena has been documented to occur in children with muscular dystrophy who were not fused and who continued to grow into their adolescent years (Miller et al. 1992)

Growing Rod Constructs There has been increased interest in the use of growing rod constructs over the past 10–15 years. These systems usually have fixation at one end often in an area of short fusion and then have an extending rod connecting these areas. Every 4–6 months, the rods are distracted. This allows the spine to grow and to correct the spinal deformity over time. Because these systems require frequent surgeries, they have a very high rate of complications especially in the complex child with early-onset CP scoliosis. One report of 28 growing rod patients noted a complication rate of 84% and mortality rate of 18%. CP was not separately reported (Phillips et al. 2013). One study has reported on a series of 27 children with CP with an acceptable outcome; however, only 25% had completed treatment, and there was already a 30% deep wound infection rate (McElroy et al. 2012). The newer version of the growing rod construct is the MAGEC rod which is magnetic motor that allows lengthening without the need for doing additional surgery. At this time there are no reports specific to children with CP. This might have the potential for using the growing concept; however, the problems of weak bones and spasticity will likely continue to make this a high complication procedure (Case 7).

Spinal Deformity in Very Small Children Who Are Older Growth inhibition in children with severe neurologic disability may be significant with children being only 15 kg in weight at 10 years of age but also having severe scoliosis. These children

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should be instrumented and fused, but instead of using the regular 6.5-mm-diameter Unit rod as used in the normal larger child, the thinner 5.0mm Unit rod should be used. The large Unit rod can be used in most children up to 15 kg in size; however, it is extremely difficult, as the rod gets shorter and because of its severe stiffness, to be able to manage it in the small thin osteoporotic pelvis. The smaller Unit rod is available up to 330 mm in length and is much easier to use and has sufficient strength for these small children. The thinner rod should not be used in taller children because of the risk of rod fracture and the development of pseudarthrosis, which would subsequently require a revision. The use of small screw and rod systems is another option. Another area related to the very small child is the increasing interest in growth attenuation or suppression of young children 3–5 years old, so they will remain small in size and stature to make care easier. This means giving growth and androgenic hormones to drive them through puberty when they are 3–5 years old and stopping their growth. One of alleged benefits of this treatment is that it will prevent scoliosis. This benefit has not been confirmed due to this practice still being relatively infrequent. We have not seen a child that developed scoliosis after this treatment.

Recommendation for Early-Onset Spine Fusion in Children with CP Children less than age 7 years who develop a structural scoliosis over 60 which has substantial stiffness fall into the very young group. Always the first additional review should be to evaluate the hips, and if there is hip dysplasia or asymmetric contractures driving an infrapelvic pelvic obliquity, this should be addressed first (Case 1). If the scoliosis is flexible, almost always the scoliosis will improve after correcting the hips. This improvement will be temporary, and the curve will typically start the normal increase again at 9–10 years of age. If the hips are normal and the spine is developing increasing stiffness as documented with multiple examinations over 1 year, intervention is indicated (Case 6). During

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this year of observation or till progression is clearly documented, body jacket bracing can be attempted if the child can tolerate. When progression is documented to be structural by also having increased stiffness, consider surgical intervention. If the child is relatively healthy and has adequate bone density, a growing rod construct, maybe a MAGEC rod, can be considered with recognition of the likely high complication rate. If the child is medically fragile and/or has severe bone fragility, a short segment fusion at the apex of the curve can be performed (Case 2). Children with cerebral palsy between the ages of 7 and 9 years old fall into the technical definition of early-onset scoliosis; however, these children can almost always be managed with wheelchair seating adjustment and comfort orthotics until the sitting scoliosis reaches 90 . Almost all curves in this age range stay flexible until around 90 . The whole spine should then be fused. Most of the children who are developing scoliosis in this early time also tend to be very medically fragile, and some may have hip subluxation. If there is a structural scoliosis and a dislocated hip, it usually means the pelvic obliquity has a combined infra- and suprapelvic pelvic obliquity. In these cases it is better to wait and correct the spine and then 6 months later address the hips (Case 6). Children with cerebral palsy who are over 10 years old even if they are small in their body size should be considered for full spine fusion when the scoliosis approaches 90 or the stiffness makes it impossible to bend the child to the midline (Case 3). These are the indications for the child who has significant remaining growth, not for the individual who is skeletally mature (▶ Chap. 118, “Surgical Treatment of Scoliosis Due to Cerebral Palsy”).

Long-Term Outcome of Early Spine Fusion We have reviewed our children who were fused between 4.4 and 9.9 years of age with a mean of 8.3 years. They were reviewed at a mean 10-year follow-up. These were very medically involved children with 94% having seizures, 88% having

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gastrostomy tubes for feeding, and 27% having permanent tracheostomies. There was 28% mortality at 10 years of follow-up. None of the deaths were related to the spinal surgery or due to restrictive lung disease (Sitoula et al. 2016). This review suggests that this is a very fragile group of children, and we need to also focus on quality of life. For this review we did not have any quality of life measures, but parents report children being more comfortable and better able to sit after the spinal surgery.

Conclusion Early-onset scoliosis in children with CP tends to fall roughly into two groups, the very early group with deformity before age 7 years and those 7–9 years of age. For the early group, clinically monitoring to document progression in curve size and magnitude is the first treatment. The 7–9-yearolds are almost always similar to the older children with CP and can be managed with monitoring followed by fusion at approximately 90 scoliosis.

Cases

Case 1 Clarissa

Clarissa, an 8-year-old girl with severe spastic quadriplegia, presented for a second opinion concerning her progressive scoliosis. Her parents were most concerned about her increasing problems with sitting, which they perceived came primarily from her scoliosis. She had been prescribed a spinal orthosis to help with sitting and control her scoliosis. She was fed orally and was small for her age but appeared well nourished. She was taking Tegretol for seizure control and had not had a seizure for 6 months. She was a dependent sitter and had minimal function in her hands. On physical examination she was noted to be diffusely spastic with mild shoulder contractures. The spine (continued)

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had a flexible scoliosis, and the hips were limited to 10 of abduction on the left side and 50 of abduction with some limited adduction on the right side. The knees had a popliteal angle of 60 bilaterally, and the feet were controlled with solid ankle-foot orthotics with minimal fixed deformity. Observation of her sitting demonstrated rather poorly adjusted chest laterals, as she was hanging over the lateral on the right side. A radiograph of the spine demonstrated 48 of scoliosis (Fig. C1.1), and the right hip was dislocated, and the left hip appeared to be abducted in the classic windblown deformity (Fig. C1.2). Based on this assessment, it was concluded that she had a primary infrapelvic pelvic obliquity due to the spastic hip disease. It was recommended to her parents that she have a repair of the hips by bilateral femoral shortening derotation, varus osteotomy, adductor muscle lengthening, and peri-ilial pelvic

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osteotomy (Fig. C1.3). Following this procedure, she could sit much better until age 12 years when her sitting again deteriorated, and the pelvic obliquity now was caused by suprapelvic pelvic obliquity coming from a progressive 74 scoliosis (Figs. C1.4 and C1.5). This was corrected with a Unit rod instrumentation, and she was again comfortable as a sitter (Fig. C1.6). This case demonstrates the importance of making the correct diagnosis of the pelvic obliquity, because correcting the spine will not help treat the symptoms of infrapelvic pelvic obliquity and vice versa. When in doubt, the spine should be corrected first if there is a significant scoliosis.

Case 2

Hammie is a girl with severe GMFCS V spastic quadriplegia who has seizures, has (continued)

Fig. C1.2

Fig. C1.1

Fig. C1.3

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Fig. C1.4

Fig. C1.6

scoliosis in the lumbar spine became so severe that she had difficulty sitting at age 10 (Fig. C2.3). At this time the spinal fusion was extended to the pelvis with correction of the pelvic obliquity (Fig. C2.4).

Case 3 Fig. C1.5

severe respiratory impairment, and has severe osteoporosis. She has had multiple fractures and has been treated with pamidronate. By age 6, she developed a severe thoracic scoliosis (Fig. C2.1). We elected to treat this with a localized thoracic spine fusion using screws at every level because of the osteoporosis (Fig. C2.2). We continued to follow her until the

Georg is now a 21-year-old man with GMFCS V quadriplegic CP who had early-onset scoliosis, developing a severe curve with pelvic obliquity at age 8. This was treated with a whole spine fusion, and during the following 12 years, he has completed his growth with a significant amount of circumferential chest growth. This has resulted in the ribs growing out over the pelvis (Fig. C3.1), but because the spine is fused, there is not enough movement against the pelvis to cause pain. He has (continued)

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Fig. C2.3 Fig. C2.1

Fig. C2.2

Fig. C2.4

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Fig. C3.1 Fig. C4.1

also had no appreciated respiratory impairment or other effect of this short spine.

Case 4 David

David, a 5-year-old boy, presented being unable to ambulate with mild lower extremity spasticity, mental retardation, and very poor motor control. A relatively stiff thoracic scoliosis was noted. Because of his young age and relatively straight lumber spine, he was fused only to T12 (Fig. C4.1). Over the next 10 years, he completed his growth to a height of 170 cm, gradually developing the ability to do assisted ambulation in the home. His spine remained straight (Fig. C4.2).

Case 5 Roger

Roger, a 4-year-old boy with severe spastic quadriplegia, presented with his mother

with a concern about his increased scoliosis. He also had grand mal seizures with poor seizure control, was a poor feeder, and had gastroesophageal reflux, which was being medically managed. He was scheduled to have a gastrostomy tube inserted. A radiograph demonstrated a 60 very stiff scoliosis. Because he had many gastrointestinal problems, he was a poor candidate for spinal bracing; therefore, we agreed to see him again in 4 months. During that time, he was fed by a gastrostomy tube and had gained 2 kg in the previous 2 months. A spine radiograph showed a scoliosis that had progressed to 80 and was very stiff (Fig. C5.1). He was instrumented from T1 to T12 with sublaminar wires because the majority of the scoliosis was in the thoracic spine (Fig. C5.2). He was then followed for 6 years as he grew, until he again developed (continued)

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Fig. C4.2

Fig. C5.2

Fig. C5.1

Fig. C5.3

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Fig. C5.4

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Fig. C6.1

increasing deformity distal to his previous instrumentation (Fig. C5.3). He had an anterior lumbar release and was instrumented to the pelvis, attaching to the proximal rod (Fig. C5.4). He had good trunk balance and had gained height by taking this two-step approach. Because of his seizures and poor feeding history, he would have been a very poor candidate for a subcutaneous growing rod.

Case 6 Buddy

Buddy was a 5-year-old boy with GMFCS V quadriplegia and severe hypotonia who presented for the first visit for a second opinion related to his scoliosis which was 65 , and very flexible (Fig. C6.1). His primary physician recommended growing rods. He did not have a definitive diagnosis for his condition, Fig. C6.2

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Fig. C6.3

Fig. C6.5

Fig. C6.4

and by parental history, he has been in his current condition since birth. There has not been any neurologic change; therefore, we presume it is a static condition although most likely some underlying metabolic problem considering the severe hypotonia. He has recently under gone varus femoral osteotomy for correction of hip subluxation. Our recommendation was for a well-fitting wheelchair with the goal of waiting until he is not able to sit comfortably. The family chose this approach, and 4 years later at age 9, his scoliosis reached 116 , and his seating became very difficult (Fig. C6.2). By this time his right hip had dislocated again (Fig. C6.3), and we intentionally waited to reconstruct

Fig. C6.6

the hip until the severe suprapelvic pelvic obliquity was corrected by the spine fusion (Fig. C6.4). At this time a spinal fusion was performed with excellent correction (Fig. C6.5). After the spine fusion, the right hip became painful, likely due to increased movement and stress caused by the fused spine. Six months after the spinal fusion, hip reconstruction was performed (Fig. C6.6). He has done well with a 4-year follow-up.

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Fig. C7.2 Fig. C7.1

Case 7 Shem

At age 1 year, Shem has severe quadriplegic pattern CP and was already developing scoliosis (Fig. C7.1). He was managed with wheelchair adjustments and orthotics until age 6 years (Fig. C7.2). Because of increasing problems with seating, he was implanted with a growing rod system (Fig. C7.3). Over the next 4 years, he developed several deep wound infections following lengthenings, requiring complete rod removal at age 10. An attempted spinal fusion resulted in reactivation of the infection, again requiring rod removal, and he is currently 11 years old with a 60 scoliosis, which will very likely increase as his growth continues. He also developed skin breakdown from use of spinal orthosis, requiring treatment of the decubitus. This case is representative of the severe complications frequently encountered in children with severe Fig. C7.3

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References quadriplegic pattern CP utilizing growing rods. The use of the magnetic lengthening rods may decrease these risks; however, there is still not enough experience to document this.

Cross-References ▶ Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management ▶ Surgical Treatment of Scoliosis Due to Cerebral Palsy

McElroy MJ, Sponseller PD, Dattilo JR, Thompson GH, Akbarnia BA, Shah SA, Snyder BD, Group Growing Spine Study (2012) Growing rods for the treatment of scoliosis in children with cerebral palsy: a critical assessment. Spine (Phila Pa 1976) 37:E1504–E1510 Miller F, Moseley CF, Koreska J (1992) Spinal fusion in Duchenne muscular dystrophy. Dev Med Child Neurol 34. SRC – GoogleScholar:775–786 Phillips JH, Knapp DR Jr, Herrera-Soto J (2013) Mortality and morbidity in early-onset scoliosis surgery. Spine (Phila Pa 1976) 38:324–327 Sitoula P, Holmes L Jr, Sees J, Rogers K, Dabney K, Miller F (2016) The long-term outcome of early spine fusion for scoliosis in children with cerebral palsy. Clin Spine Surg 29(8):E406–E412

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1778 Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Overall Risk Factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Death: Mortality . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Preoperative Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Intraoperative Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Spinal Cord Monitoring: Loss of Motor Evoked Potentials . . . . . . . . . . . . . . . . . . . . . . . . . .

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Postoperative Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hypotension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Thrombophlebitis and Pulmonary Embolism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Coagulopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Respiratory Failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pneumothorax or Hemothorax and Pleural Effusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reflux and Aspiration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pancreatitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Colicystitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Duodenal Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Constipation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Poor Feeding . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Seating Adjustments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hair Loss . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1789 1789 1789 1790 1790 1790 1791 1792 1792 1792 1792 1793 1793 1793

Doing Posterior Spinal Fusion When Families Refuse Blood Transfusions . . . . . 1793 Dealing with Families Who Refuse Spinal Fusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1794 Handling Families and Children When a No Resuscitation Status Is Requested . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1794 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1795 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1799 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1799

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_118

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Abstract

Children with severe cerebral palsy frequently develop scoliosis and other spinal deformities that require spinal fusion for correction. Because many of the children who develop spinal deformities have very severe neurologic deficits, the complication risks during and after the spinal fusion is high. Children with severe cerebral palsy who are not having aggressive nutritional management through gastrostomy tubes may have nutritional deficits. If these nutritional deficits are severe, the ability to tolerate a large surgical procedure and with a large wound is decreased. Management of the nutritional deficit may be with preoperative increased nutrition or the insertion of a gastrostomy or nasogastric tube. Alternatively, this can be managed with diligent attention to immediate postoperative nutritional intake. This acute postoperative nutritional intake may require insertion of a nasogastric or nasal jejunostomy tube for early feeding. In some rare cases, central venous hyperalimentation will be required. Other complications include high blood loss intraoperative requiring a diligent attention to a blood replacement. There are multiple other complications that need to be monitored such as sepsis, pancreatitis, cholangitis, and urinary tract infections in the immediate postoperative period. Gastroesophageal reflux is another common problem that needs to be medically managed before and during the surgical recovery.

Keywords

Cerebral palsy · Spinal fusion · Pancreatitis · Mortality · Complications · Pulmonary embolism · Thrombophlebitis · Nutrition · Pleural effusion · Pneumonia

Introduction Spinal deformity is very common in children with gross motor function classification (GMFCS) IV and V. Most of these children develop deformities that require surgical

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treatment (▶ Chaps. 117, “Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management” and ▶ 118, “Surgical Treatment of Scoliosis Due to Cerebral Palsy”). Complications of spinal fusion in CP are common due the medically compromised condition of the children. Most of these complications are relatively self-limiting and resolve with appropriate management. From an extensive review of 107 spinal fusions in children with CP, the best predictor for risk of complication is severity of the neurologic disability. The level of the neurologic involvement is defined based on a score that combines the ability to speak, walk, eat, presence of seizures, and intelligence. Based on assessing these five areas, there was such a strong correlation to neurologic disability that the presence of additional risk factors such as tracheostomy, tracheal diversion, gastrostomy tubes, seizure drugs, or malnutrition were not correlated with additional risk (Lipton et al. 1999). One other report suggested that poor nutritional indicators, as defined by low absolute lymphocyte count and low albumin, increase the risk of complications, which are defined as urinary tract infection, length of intubation, and length of hospital stay (Jevsevar and Karlin 1993). We have subsequently repeated a very detailed review of another 303 children and found that the primary predictor of complications was the presence of a gastrostomy tube (Nishnianidze et al. 2016). Again we could not find a relationship of albumin level or absolute lymphocyte count. We suspect this is partly because the more neurologically involved children had a more prolonged course of postoperative lack of nutrition. At this time, we believe the overriding risk factor for complications following spine surgery is the severity of the children’s neurologic disability. It is difficult to know how to use this information to counsel families, but physicians have to be honest with this risk. For families who want to aggressively pursue medical care, this risk is usually not a significant concern because the alternative of the child becoming bedfast is difficult for them to manage as well. Another problem when assessing complication rate and specific complication risk factors is most publications report on neuromuscular

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scoliosis, not specifically upper motor neuron disease. These publications often combine upper motor neuron disease such as cerebral palsy with muscular dystrophy, spinal muscular atrophy, and myleomeningocele. The expected complications from each of these general categories tend to be quite different. As an example, myleomeningocele is expected to have a very high risk factor for urinary complications due to the paralytic bladder and much higher infection rate due to poor soft tissue coverage. Respiratory complications associated with flaccid scoliosis such as spinal muscular atrophy and muscular dystrophy, tend to be most related to weakness of the respiratory muscles. Complications related to the pulmonary system in CP are especially higher for aspiration and decreased vital capacity due to spasticity. The goal of this chapter is to review the acute complications occurring in children with CP who have a spinal fusion.

Natural History Overall Risk Factors The overall risk for developing complications after spinal surgery in CP is primarily related to the severity of the child’s neurologic disability. This is the same risk factor for the children developing scoliosis in the first place as almost all the spinal deformity occurs in children with GMFCS IV and V. Most patient series that have done comprehensive evaluation of risk factors for complications report the primary risk factor as being the severity of the neurologic disability. It is however quite difficult to segment the severity of the neurologic disability further then the GMFCS. An analysis of risk factors and separately assessing the complication risk based on the severity of the neurologic disability found a history of pulmonary hospitalizations and severity of neurologic disability correlated to complication rate based on our early review (Lipton et al. 1999). However, when we applied those same risk factors to a more recent different group of patients, the combination risk factor scores no longer were found to be significant (Nishnianidze et al. 2016); in this latest study, the

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most significant risk factor was the presence of the feeding gastrostomy tube. It seems most likely that the presence of the G-tube is actually a marker separating the severity of our patients especially as it relates to oral motor function. Another study reported a much higher incidence of complications in children who were unable to ambulate (Master et al. 2011). Since the severity of the individual child’s neurologic disability is strongly related to the incidence of complications, it makes it very difficult to compare different patient series in the literature. In some centers, they have relatively strict criteria to only correct scoliosis in patients who have fewer medical problems who may be able to interact or are felt to be at lower risk. All of these selection biases mean that the incidence of complications will be reduced if they only operate on healthier and less at-risk patients. The overall incidents of complications is usually reported between 40% (Samdani et al. 2016) and 50% (Duckworth et al. 2014); however, a recent review found reported complication rates being 10–70% (Legg et al. 2014); this high rate of complications in children with cerebral palsy means that there needs to be a good medical management of the child both before and after the surgical event. Children or adolescents who have a spinal fusion for severe scoliosis usually also have many other multiple system problems. These problems, combined with the large magnitude of the surgical procedure, mean that these children are at risk for almost any medical problem that can be imagined. This discussion of complications will focus only on serious complications that have been encountered or that are encountered frequently. In reality, this covers almost every complication encountered in over 700 children with CP in whom we have done a spine fusion. Several notable areas are missing from this complication list, mainly thromboembolic disease. We have never seen pulmonary embolus or deep venous thrombosis in the lower extremity in children or adolescents undergoing spinal surgery. If thromboembolic disease was considered because of asymmetric swelling in the lower extremity, it has always been heterotopic ossification (▶ Chap. 137, “Complications of Hip Treatment in Children with Cerebral Palsy”) or a

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fracture. Therefore, the concern about thromboembolic disease rarely needs to be raised and then only after every other alternative has been ruled out.

Death: Mortality The most significant complication of spinal surgery is death. This is the only surgery in CP where there is a definite risk of mortality, although all surgery carries this risk at some level. There are no literature reviews that specifically evaluate the mortality rate, although series reporting mortality range from 31% (Onimus et al. 1992) to reports that include one death (Boachie-Adjei et al. 1989; Krismer and Bauer 1990). The recent review found 2.8–19% risk of mortality (Legg et al. 2014). Also, there are many series of small numbers of patients that report no deaths. In the last 28 years, we have performed approximately 750 spinal fusions in children with CP and have had 8 deaths in the acute intraoperative and postoperative period. This result translates into a rate of approximately 1% mortality; however, these deaths occurred in the most neurologically involved population. Therefore, a more accurate risk maybe 2–5% in a select group of the most severely involved children. This high rate of mortality for this group of children who have the most severe neurologic involvement with no speech, no self-feeding, requiring gastroesophageal tube feeding, and who are fully dependent sitters is hard to assess from the literature. To maintain a low morbidity rate in these severely disabled children, high-volume experience and good protocols with multidisciplinary care are required. It is as important when comparing mortality rates as it is when comparing general complication rates to consider the severity of the children’s neurologic deficit. It is also important to do a careful case evaluation of all deaths, as often the death may have been due to a preventable cause and lessons can be learned. Specific causes of intraoperative death are usually bleeding that was not appropriately managed. With proper preparation and anesthesia, as well as surgical management, this should almost never

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happen, and every death due to excessive blood loss needs very careful attention to identify the causes of treatment failure. Another reported intraoperative cause of death is air embolism, (Krismer and Bauer 1990; Nectoux et al. 2010) and we are aware of two other cases that have not been reported. The usual scenario of an air embolus is a child whose blood pressure is dropping from hypovolemia, in which the anesthesia team responds by decreasing the anesthesia level. At a time when many epidural spaces and epidural veins are open, this child under light anesthesia starts breathing on their own, drawing air in through the open venous sinuses. Air embolism is totally preventable in that children should always be under full neuromotor paralysis during this procedure to avoid inadvertent negative pressure in the chest cavity. If neural monitoring is being done, then the anesthesia team needs to be extremely vigilante to prevent spontaneous respiration. Other causes of intraoperative death, such as dislodgment of the endotracheal tube and loss of vascular and arterial access, are all preventable by appropriate preoperative preparation. Cardiac arrhythmias and hypotension may also be related to low body temperature, which should be maintained above 34 at all times.

Transition Time Another high-risk time is in the transition from the end of the operative procedure until children are completely set up in the intensive care unit. For two deaths, the initiating event began in this time frame. One child had a difficult anteroposterior surgery followed by a required revision at the distal end of the Unit rod because of pelvic perforation. This child had initially been moved from the operating table to the hospital bed and a radiograph was obtained. Multiple radiographs were obtained because of concern of the rod placement. Over a period of approximately 30 min, it was decided that the rod needed to be revised and preparations were made to move the child back onto the operating table. During this time, the child’s blood pressure dropped somewhat and fluid resuscitation was initiated. The child was placed back on the operating table and a short 30-min procedure was performed to revise the

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rod. At the end of this short revision, the patient had a sudden drop of blood pressure and there was also extensive bleeding from the surgical site. It was concluded at this time that the child was in a coagulopathy, and aggressive resuscitation with blood products was begun. During the time, the child was moved back over to the hospital bed, the arterial line became dislodged, and it was some time before it was possible to get further blood pressure readings. During this time, the child had a severe hypotensive event and continued with bleeding. The child was resuscitated and taken to the intensive care unit but continued to bleed into the chest; however, the mother requested that no further resuscitation be performed. In another case, the child had a very uneventful anterior and posterior procedure with exceptionally low blood loss. Again, the child was transferred to the hospital bed. Some time was consumed in obtaining appropriate radiographs, and over a 30–45-min period, the child was transferred to the intensive care unit. As the child was being moved into the intensive care unit, the portable monitor showed that the blood pressure had dropped and there was a concern that there might have been a monitor malfunction; however, with a short review, it was determined that the child had a cardiac arrest. The child was then returned to the operating room, aggressively resuscitated, and returned to the intensive care unit. No source of bleeding was found and the cardiac arrest was due to a combination of hypovolemia and anemia. Again, the mother requested that no further resuscitation efforts be made, and 8 h later, the child had another drop in blood pressure and a cardiac arrest and no resuscitation was performed. These two cases demonstrate the extreme importance of maintaining a high state of vigilance in this period from the end of the operative procedure until children are safely in the intensive care unit with full monitoring.

Immediate Postoperative Period Immediate postoperative deaths may occur if there is not an aggressive intensive care unit management of electrolyte balance, coagulopathy, hypovolemia, and respiratory support. We had one death in the first 24 h after

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surgery in which the girl developed a rapid coagulopathy followed by a cardiac arrest from which she could not be resuscitated. The postmortem examination showed severe hemorrhagic pancreatitis for which there was no explanation for the cause. Risks of death after the acute postoperative period are mainly due to respiratory compromise. After discharge from the hospital, the risk may be higher in the first 6 months but not substantially. Again, this risk involves those children with the most severe neurologic disability. We also had 12 deaths of children who were scheduled for surgery but died before the spine surgery could be performed. All of these were severely involved children in whom the caretakers noted increasing problems from the scoliosis and desired aggressive comfort management. The treating physician did not perceive that these children were having any more medical problems than many similar children who do well and make significant improvements following surgery. We also had three children die in the first 3 months after surgery after discharge from the hospital. One of these children was admitted to the hospital with what was initially thought to be severe constipation; however, she quickly became septic and was believed to have an acute surgical abdomen. The family refused surgical treatment of the acute abdomen because the spinal fusion had been performed under a no resuscitation order. The family desired only comfort care. When the child died, the postmortem examination showed a ruptured Meckel’s diverticulum that was completely unrelated to the spinal fusion. Another child developed pneumonia 6 weeks after discharge and was admitted to another hospital, where again the family refused to have the child intubated, and she died. The spinal fusion may have been related in the development of her pneumonia; however, one of the goals of the spine surgery was to try to improve her respiratory function, which had been getting progressively worse. A third child was found dead in bed in the morning by the caretaker 4 weeks after discharge. No postmortem examination was done; however, this is how death most commonly occurs in this group of severely involved children.

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Preoperative Problems If families have decided to move ahead with the posterior spinal fusion, it is important that their children’s whole medical treatment be under maximal therapy.

Poor Nutrition As previously noted, the importance of good nutrition is well understood but how nutritionally fit is required is totally unknown at this time. The goal is to have children in as good nutrition as their families will allow; however, we would almost never refuse surgery because of malnutrition unless the children are in the severe stages of starvation and malnutrition. A full diet history should be done and evaluated by a dietitian. Using this data and a history of the family dynamics, the dietitian can usually recommend appropriate feeding supplements and better nutrition. Nighttime feeding with a nasogastric tube is also recommended occasionally. The primary focus on nutrition, however, is in the postoperative period. A prolonged course focused on nutrition for a year in a child whose scoliosis is increasing, will likely incur more risk from the progressive spinal deformity than the gain obtained from the nutrition. Gastroesophageal reflux, gastritis, and stomach and duodenal ulcers are quite common in this population. Children should be under maximum medical management of these conditions; however, if the gastroesophageal reflux is of such severity that it requires surgical repair, we prefer to do the spine fusion first. The effect of the spine fusion on the gastroesophageal reflux is very unpredictable, ranging from very significant improvement, to no effect, to significant deterioration (Case 1). This unpredictability appears to be due to the poorly understood anatomical changes that the spinal deformity causes to the diaphragm and gastroesophageal junction. The assessment of our surgical colleagues is that it is also easier to address the gastroesophageal reflux when the spine is straight and there is not a severe distortion of the anatomy. Seizure drug levels should be checked preoperatively, and a consultation with a neurologist is encouraged if the seizures are not well controlled.

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However, there is little concern about managing seizures postoperatively because they are seldom a problem. If a grand mal seizure occurs in the postoperative phase, the Unit rod is strong enough to resist failure and we have never seen any related problems. If the child has a ventricular peritoneal shunt, it should be checked because significant changes in body shape may affect its function. Sometimes the shunt has a fracture however there may be a wellformed track that is functioning to connect the two catheters. After the spine is corrected, this track will be stretched and will likely not function. Consideration for preoperative shunt replacement may be needed in selected cases (Lai et al. 2014).

Intraoperative Complications Respiratory Problems Many children with severe neurologic involvement have some level of aspiration, which may lead to reactive airway disease. As children are anesthetized, asthma may become more noticeable. Appropriate treatment with inhalers and steroids should be started, and if the patients respond quickly, the surgery can proceed. If there are prolonged periods of hypoxia or difficulty with ventilation, the surgery should be canceled if it has not been started, and if this occurs during the operative procedure, very rarely surgery may need to be abandoned. Dislodgment of the endotracheal tube is a serious respiratory emergency and the whole team must understand the protocol in the event this occurs. Children need to be turned emergently into a supine position on a stretcher that should always remain immediately accessible to the operating room. The endotracheal tube may also occasionally move distally into the right mainstem bronchus and cause hypoventilation on the left side. If there is hypoxia and decreased breath sounds on one side during surgery, the movement of the endotracheal tube should be the first thing to check. If the tube is fine, then an acute pneumothorax on the side with decreased breath sounds should be considered. It is very difficult to get a good chest radiograph in the prone position on a

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spine frame, so if the problem persists, it is better to prophylactically place a chest tube on the side with decreased breath sounds. This placement is relatively easy to perform. By only minimal movement of the surgical drapes, the midthoracic level of the posterior axillary line is accessible and a tube can be easily inserted from the surgical field. If no pneumothorax is present, no damage is done; however, this can potentially avoid a very serious complication. The tube can be left open to the air until the end of the case when it is placed under water seal. Bleeding Problems (Chapter ▶ “Anesthetic Management of Spine Fusion”) Intraoperative bleeding is a well-recognized problem in children with CP. This bleeding is worse than for other neuromuscular conditions and may be made even worse if children are on valproate sodium for seizure control. Another factor may involve chronic dehydration and contraction of the intravascular fluid volume, which many children have. This intravascular fluid space, if rapidly expanded under the stress of surgery, may cause acute dilution of the coagulation factors. However, this increased risk for bleeding is multifactorial, as there has been little effect with attempts to raise specific coagulation factors with desmopressin acetate (Theroux et al. 1997). The key is not to allow a coagulopathy to develop. Often, there are situations where children appear to be doing very well and then have a sudden decrease in the ability of clot formation and increased bleeding begins. Ideally, the coagulopathy can be treated before it becomes this obvious by the early administration of freshfrozen plasma when approximately one half of the blood volume has been lost. In children on valproate sodium, phenobarbital, or other drugs known to cause increased bleeding, as well as children with severe neurologic involvement, early transfusion of fresh-frozen plasma may be considered. Large volumes of crystalloid should be avoided in favor of plasma and red cell transfusion. Periodic blood samples should be obtained, especially as one blood volume of loss is approached, to monitor platelet count. Hemoglobin levels also need to be monitored.

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If the platelet count falls below 100,000 during surgery, platelet transfusion should be given. Hemoglobin should be maintained above 8 grams during the surgical procedure, and the goal is to maintain blood pressure at a mean of between 60 and 80 mmHg. More aggressive hypotensive anesthesia is not beneficial because most of the bleeding is venous in origin. Maintaining a low venous pressure is beneficial in decreasing blood loss, but this can be very dangerous. Children may go from maintaining a blood pressure of 60 mmHg, and if the intravascular volume is being maintained low to help with bleeding, they may suddenly drop to a systolic pressure of less than 30 mmHg. It is better to have a little more margin of safety even if there may be a little more bleeding. Surgeons must be prepared to handle high blood loss (Case 1). The value of blood salvage in this group of children is uncertain because most of the blood loss tends to come at the end of the procedure, especially with bone decortication and facetectomy. To most adequately use blood salvage, the blood needs to be obtained through suction and there should be no wound coagulant, such as thrombin and Gelfoam, used in the wound. In our facility, there is not much difference in the amount of blood lost and the amount of donor transfusion, whether blood salvage is used or not. Also, there is debate about how much electrocautery should be used, with some surgeons doing much of the dissection with electrocautery and others using it only to control points of bleeding. Again, there is not much difference in blood loss. Surgeons must be aware that some children with CP have high blood loss with surgery and some have very minimal blood loss. Except for children with the most severe neurologic deficit and possibly those on seizure medications, it is impossible to predict exactly which children will have high blood loss. This means the surgical team must be prepared for large volume blood loss with arterial monitors and central vein access. The use of antifibrinolytics should also be considered. In one study tranexamic acid (TXA) was compared to epsilon-aminocaproic acid (EACA) and saline (Dhawale et al. 2012). There was less

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blood loss with TXA so it is a good additional assist to reduce blood loss. Another study found a significant reduction in transfusion after using EACA (Thompson et al. 2008). EACA however is now not recommended for children.

Epidural Bleeding Opening of the epidural space may cause the most blood loss. In most children, this part of the procedure involves very little or no bleeding. Sometimes one level will have a slight amount of venous bleeding, which is easily controlled. However, in a few rare children, approximately 1 in 75, there will be exuberant bleeding from almost every epidural space at every level. This bleeding can make wire passing stressful; however, with proper preparation, it can always be performed. The technique for managing this exuberant bleeding is to open the epidural space, then pack it with Gelfoam and neural strip sponges, putting gentle pressure on the interspace. Almost all this bleeding is venous, and no attempt should be made to find the vein as these epidural veins are very circuitous and hard to control directly. After all the interspaces have been opened and packed, start passing wires at each interspace, removing only the pack at that interspace. If substantial bleeding occurs during passing of wires, the interspaces are immediately packed again with Gelfoam, neural strips, and a sponge, sometimes requiring someone to hold pressure over the area. When this type of bleeding is encountered in the surgical field, it is mandatory to communicate with the anesthesia team to ensure that enough blood has been typed and cross-matched and that coagulation factors are being transfused. Our worst experience with this type of bleeding occurred in a girl with relatively good motor function who was cognitively normal but had many previous abdominal procedures and severe hyperlordosis. It is our impression that this combination of abdominal procedures and hyperlordosis increased the risk of this venous bleeding. It is likely that the vena cava had a partial obstruction and that the blood flow from the lower extremities was coming, in part, through the epidural veins, which had become dilated. In some of these children, each interspace seems like passing a wire

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through the vena cava itself. In our most severe case, 10 liters of blood was lost during the procedure, most in passing wires and controlling the epidural bleeding. However, this case is an ideal example that the volume of bleeding has little to do with postoperative recovery, as this girl had an excellent postoperative recovery and has had no perceptible effects of this blood loss.

Bone Bleeding The second major source of bleeding is from bone veins during decortication and facetectomies. We prefer to control this bleeding with packing with bone graft that has been embedded with thrombin immediately after decorticating and doing facetectomies. Also, packing the wound with sponges helps to control this venous bleeding if there is not a concomitant coagulopathy. We also prefer to do the decortication and facetectomies after the wires have been passed but before the rod is inserted. This approach allows for the best decortication and removal of the facets; however, there is then a longer period of time of bone bleeding until the wound is closed. The problem with doing decortication after the rod is inserted is that it is very difficult to do any substantial decortication and facet removal. However, in rare patients in whom there are severe problems with bleeding early in the case, we prefer to do the decortication after the rod has been inserted. If significant bleeding occurs, it is important for surgeons and anesthesiologists to keep communicating. In general, the protocol we use is to try to maintain a mean blood pressure of 60–80 mmHg. If the mean pressure drops below 60 mmHg, volume is replaced with crystalloid and donor packed cells because few of these children are able to donate blood. If the mean pressure drops below 40 mmHg and is not quickly responding to volume replacement, the surgical wound should be packed and held under pressure to stop all wound bleeding temporarily. If part of the problem is coagulopathy, clotting factors and platelet replacement should be given as soon as they can be obtained from the blood bank. It is also important for anesthesiologists and surgeons to always be aware of how much blood is

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available for immediate transfusion. During the operative procedure, the amount of blood available for immediate transfusion should never drop below two units. By the time the fifth unit of transfusion is needed, the blood bank should be in the process of cross-matching more blood. It must be recognized that children with CP have a tendency for sudden drops in blood pressure, especially if the intravascular volume is too low and they become suddenly coagulopathic. This tendency for sudden drops in blood pressure may occur because these children have very poor general conditioning, as they never get any exercise. Also, if there is a substantial drop of the blood pressure below a systolic of 30 or 40 mmHg, preparation should be made to emergently turn patients into a supine position in the event a cardiac arrest should occur. However, cardiac arrest with a substantial blood pressure drop is rare, and aggressive fluid replacement will almost always reverse the situation. The most important aspect in managing intraoperative blood loss is for the operative team to be prepared and expect the worst. If blood loss is managed properly and the operation is completed properly, the amount of blood loss has very little impact on children’s recovery or outcome. (Lipton et al. 1999) Surgery done poorly because of blood loss usually means another return to the operating room with the same bleeding problems encountered again, which can be well demonstrated by an individual case (Case 1).

Dural Leak While opening the epidural space, or during passing of sublaminar wires, a dural tear may occasionally be created, although this is very rare in individuals who have not had previous spine surgery. In these situations, the tear is usually small, and with slightly more opening at the interspace, it is possible to repair the dura directly with a small 6–0 nylon suture. Gelfoam can then be placed on top of the repair, and the dural leak is adequately controlled. However, it is much more common to get dural openings when there has been previous surgery, such as following a dorsal rhizotomy. Again, most of these dural tears tend to be small and are fairly easy to repair with direct suture.

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If the dura is extremely friable and cannot be directly repaired, it can be left open if this open area is large enough that a one-way valve is not created. Usually, this means that the open area should be approximately 0.5 cm2, which is left covered with Gelfoam, and then the spinal procedure is continued. At the end of the procedure, the fascia is closed very tightly in the usual fashion, being especially careful that the area over the dural tear is closed with good substantial soft tissue. Occasionally, it may be appropriate to place some crushed cortical bone directly over the tear so long as there is no pressure pressing this bone into the spinal canal. The most common circumstance where substantial tears of the dura are encountered is during spinal osteotomy, when there has been substantial previous spinal surgery. Another option for repairing these dural tears is to obtain a small bit of lumbar fascia and then suture it over the dural defect. All these children are kept supine for 2–3 days because of the large magnitude of the surgical procedure; therefore, this area usually seals or becomes part of the surgical wound. We have never had any problems with persistent dural leaks or problems with spinal headaches in children in whom we have left large defects in the dura open.

Perforation of the Pelvis with Unit Rod One of the potential complications of Unit rod instrumentation is perforation of the pelvic wall with the pelvic limb of the rod or iliac screw placement. This complication occurs primarily in individuals whose deformity has a major component of lumbar hyperlordosis and is especially common in children who are very short in stature and have thin osteoporotic pelvic bone. These specific criteria are not contraindications to using the Unit rod but should raise the concern about possible perforation. Decreasing the lordosis by maximum hip flexion and using an anterior abdominal block can all help to prevent this problem. Also, getting a good visual perception of the angle of the hole in the pelvis and making sure that the pelvic rod stays in line with this hole during insertion is important. Using the fluoroscope, the ilium can be imaged and one can confirm that the rod is in the bone and not perforating. This tear

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perforating leg was cut and removed. The hole in the pelvis where the rod was supposed to go was identified, and the rod was cleaned and reinserted. The wound was well irrigated, and the child was maintained on antibiotics for 4 weeks and had an uneventful recovery with no recurrent infection in the spine after more than 5 years of follow-up.

Fig. 1 By obtaining an oblique and distally oriented fluoroscopy image, the intramedullary canal and of the ilium appears like a tear drop

drop shape should allow excellent imaging (Fig. 1).

Medial Pelvic Perforation Three types of perforation of the pelvis may occur. Perforation of the medial wall of the pelvis has the highest risk of causing significant problems (Case 2). We had one patient in whom the rod perforated the pelvis and subsequently caused a colon perforation. This perforation occurred at a time early in our experience when we did not think there would be much risk from colon injury because we thought the rod would lie on the medial side of the ilium and the colon would simply move away. We are aware of one other case of colon perforation in which the rod was allowed to lie on the inside of the pelvis. We now believe that this is not an appropriate position to leave the rod or screws long term and recommend revision when it lies substantially medially to the pelvis. Both these colon perforations occurred late, 7 weeks after surgery in our case. This colon perforation responded well to draining and closure of the colon perforation initially, then, after 5 days, the posterior spine was opened at the distal end and the rod on the side of the

Lateral Pelvic Perforation The iliac rod may perforate the pelvis laterally as well, and this almost never causes a problem and generally can be ignored. We had one such perforation that was close to the sciatic nerve and caused neuritic pain, requiring eventual resection of the rod leg on that side. In another child, just the tip of the rod perforated laterally and developed heterotopic ossification over the tip of this rod, which formed a painful bursa and required removal of the heterotopic bone and tip of the rod (Fig. 2). Theoretically, it is possible for the Unit rod to perforate the normal acetabulum. However, if the entrance of the drill hole is started in the inferior aspect of the posterosuperior iliac spine and the drill guide ensures that the hole is drilled just above the sciatic notch, this almost never occurs. Acetabular Perforation The rods are also sized so that the pelvic legs of the Unit rod are not long enough to reach into the acetabulum. However, if children have a severely dysplastic acetabulum, which is migrated posterosuperiorly, it is possible for the leg of the rod to reach and enter this false acetabulum. This rod perforation has occurred in one of our children. We removed the tip of the rod by opening the ilium just anterior and superior to the acetabulum and cut off the tip of the rod, drawing it out of the acetabulum but leaving the remainder of the leg with good fixation in the ilium (Givon and Miller 1999). Wires Pulling Through Laminae In many children with CP, the bones of the laminae are not of normal strength. Occasionally, a lamina fractures during wire tightening. With one or two laminae fractured, they can be bypassed and there

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Fig. 2 Protrusion of the pelvic leg of the Unit rod may occur on the lateral side (a). This usually does not cause any problem; however, it may cause ossification of the muscle (b). Over 6–12 months, this usually matures and becomes asymptomatic; however, in a few cases, persistent bursitis remains after complete maturity (c). Excision of the ossification and rod are then required, and this would usually be treated with one dose of radiation to prevent recurrence (d). Inferior protrusion into the sciatic notch may cause sciatic nerve irritation, requiring revision of the pelvic limb of the rod. Medial protrusion into the abdomen should be avoided because colon perforation may occur

is no problem. The most common lamina to fracture is L5, which is often quite thin and weak. L5 is probably the least important lamina, although it is at a transitional level. The most important laminae that should not fracture are the top two or three levels. The laminae of T1, T2, and C7 are very strong and will almost always be the ending fixation, especially when significant osteoporosis or osteopenia has been encountered. In spite of even severe osteoporosis, almost all lamina fractures are caused by technical error by surgeons. These errors can be avoided first by absolutely never using the wire to pull the rod to the spine. The rod must always be pushed against the spine, and then the wire is tightened until it just contacts with the rod. There must be a very gentle touch to using the wire twister in children who have osteoporosis and osteopenia, being specifically careful to avoid jerking movements and stopping as soon as the wire twist is in contact with the rod.

Also, as the major deformity is corrected, it is important to not decrease pressure on the rod pusher or the zipper effect may be encountered. The zipper effect happens when the end lamina has too high a pressure and starts to fail with all laminae pulling out to the apex of the curve. It is important to maintain pressure on the rod holder until all the wires are twisted; in this way, the force is distributed over many laminae and this kind of failure will not occur. If a zipper effect does occur, it is important to have at least three good stable laminae above this area. The rod can be pushed to these laminae, and then all three should be tightened down with pressure on the rod, which should be released slowly. The zipper effect happens very rarely and if the laminae at T1, T2, and C7 are utilized, good proximal strength can usually still be obtained. If there is failure of the last three lamina in a construct, fixation should be switched to pedicle screws in the upper spine.

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Rod Either Too Long or Too Short One of the most difficult technical challenges in using the Unit rod is choosing the correct length of the rod (Fig. 3). The use of the modular rod makes this length selection much easier. Surgeons must predict how much length will be gained as the deformity is corrected. This prediction is complicated by correction of scoliosis and lordosis, which add length to the spine in the instrumented area and correction of kyphosis, which shortens the instrumented section. In general, it is not a major problem if the rod is one level too short because the wires from T1 still will provide a significant corrective force. We have never had a rod that was too short. Even if the rod ended between the T2 and T3 interspace, the wires still provided significant corrective force and could be brought to the end of the rod without difficulty. If the rod is too long, bending the tip of the rod forward so that it is not too prominent posteriorly is helpful. With the rod slightly bent forward, it may be left at a level as high as C5 or C6 without causing any problems. If the rod is too long, another option is to cut off the top of the rod; however, before the rod is cut, it is very important that the two rods be cross connected with two strong cross-connecting elements. If the rods are not cross connected first, they will shift when they are cut and some correction will be lost, as the rods tend to twist into the deformity. If the rod is prominent after

Fig. 3 The correct length of the Unit rod is the most difficult decision during the surgical procedure. The best method is to set the chosen rod upside down on the spine with the distal corners right over the pelvic drill holes. The proximal end should come to the T1 level. If the child has severe scoliosis, the spine may lengthen and a rod one size

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surgery and causes pain from the development of a bursa, it can be cut off as an outpatient procedure at the level of T2 or T3. However, it is recommended that this should not be done until a fusion has occurred, and we try to encourage individuals to wait at least 1 year postoperatively before the rod is cut off superiorly.

Spinal Cord Monitoring: Loss of Motor Evoked Potentials The goal of surgery for children with total body involvement is to correct the spinal deformity so they can sit well. The risk of poor sitting and decubitus formation is such that, in the worst cases where children are completely paralyzed, they will still be better off with a corrected body posture. The use of spinal cord monitoring has much less benefit in severely involved children. The treatment we would consider is to increase the blood pressure if it is low however, the risk–benefit ratio of this would have to be seriously considered. We always raise the blood pressure if it goes below a mean of 60 mmHg, and spinal cord monitoring would not provide additional information, as this is part of our required protocol without the spinal cord monitoring. In 30 patients, 20 of whom were monitored, 3 false positives of the spinal cord monitoring occurred. None of these children had any noticeable

longer can be chosen. If the child has severe kyphosis as the primary curve, there will be significant spinal shortening and a shorter rod should be chosen. It is important to not try to determine if the rod is the correct length after it has been inserted into the pelvis by pushing it down onto the spine. This action risks causing a pelvic fracture

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neurologic change, and except for giving corticosteroids to 2 of these children, no change in the treatment was made. For children with functional standing or ambulation, spinal cord monitoring is important. The criteria for monitoring and response to changes should be similar to those of idiopathic scoliosis. Based on this experience, we believe intraoperative monitoring of the spinal cord in GMFCS V nonambulatory children with CP adds no beneficial gain to the care of these children and, as a consequence, is not indicated.

Postoperative Complications Many reports in the literature evaluate the outcome of spinal fusions in children with CP. Many of these authors report major and minor complications. It is impossible to determine any reasonable rate of complication from these reports, because there is no clear definition of what constitutes a major or a minor complication. In general, most surgeons would consider a deep wound infection a major complication, because it significantly delays children’s recovery and requires much effort from surgeons. Conversely, it is unlikely that surgeons would consider children who required endotracheal intubation and positive pressure ventilation for 2 days postoperatively to have had a complication. However, if children need ventilation for 7 days, is it then a complication? If children need ventilation for 4 weeks and then are converted to a tracheostomy, it would probably be considered a complication. However, if children were considered for possible tracheostomy before spinal surgery, and the hope was that the spinal surgery would make the tracheostomy unnecessary, then it may not have been a complication of surgery, just a failure of the surgery to accomplish the desired goals. Because these children often have multiple system pathologies, there are many outcomes that may or may not be considered a complication. Because of this complexity, there will be no attempt to summarize all complications reported in the literature.

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Hypotension In the acute postoperative period in the intensive care unit, fluid status has to be monitored very carefully with the goal of maintaining urinary output at a minimum of 0.5 ml/kg/h. The arterial blood pressure should be maintained at or above a mean of 60 mmHg and the hemoglobin should not drop below 9 g in the first 48 h postoperatively. Often, the hemoglobin will drop steadily for the first 12–24 h for a total drop of as much as 3–5 g from the immediate postoperative hemoglobin level. Significant additional fluid is almost always required because of third spacing with the accumulation of a significant amount of edema. This edema generally starts to resolve as children start to diuresis on the second and third postoperative day. This accumulation of fluid is believed to be due to the syndrome of inappropriate antidiuretic hormone (SIADH). Also during this period, careful monitoring of the electrolytes, magnesium, and calcium is required. In occasional patients, the blood pressure and lack of diuresis may need to be treated with low levels of dopamine. A welldefined intensive care unit protocol to monitor laboratory values and treat critical levels should be part of the postoperative management protocol. With proper intensive care unit management, these acute postoperative problems rarely have any long-term consequences, although these problems can raise families’ anxieties. Families need to be reassured constantly that these are temporary problems that should resolve without any long-term consequences.

Thrombophlebitis and Pulmonary Embolism In over 750 cases of posterior spinal fusion in children with CP, we have had only one thromboembolic event. In this case, she clotted her whole central venous system and required embolectomy. On work up she was found to have a congenital hypercoagulate state. There are no reports of pulmonary embolism after spine fusion in children with CP. Based on this, there is no indication to

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use any anticoagulation or other preventive mechanisms as prophylaxis against thromboembolic events.

Coagulopathy Just as careful intraoperative monitoring of the clotting cascade was important in the immediate postoperative period, especially for the first 12 h, this careful monitoring must be continued with an attempt to normalize all clotting parameters. If the clotting cascade is not managed aggressively as children continue to have a decrease in hemoglobin level requiring large volumes of crystalloid, a severe coagulopathy can develop quickly in the intensive care unit. Protime and prothrombin time should be brought to the normal range with freshfrozen plasma, and the platelet count should be kept above 75,000 during this period. With appropriate aggressive postoperative care, no children have developed a coagulopathy in the postoperative period except for one child who died of hemorrhagic pancreatitis 12 h after surgery. This girl’s coagulation studies were never fully corrected in the postoperative period, although she received a substantial amount of blood factors.

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some children who have such marginal control of their oral pharynx that tracheostomy may be considered. If these children have severe scoliosis, it is our practice to do the spinal fusion first, then if they cannot be extubated at 3–4 weeks after surgery, a tracheostomy is done. The response to the spine fusion is often such that children are slightly better and can avoid having the tracheostomy. In the immediate first 5 days of the postoperative period, fevers often occur and are almost always of pulmonary origin. Full fever workup is indicated if the fever spikes to 40  C or greater, or if the temperature remains over 39  C after good pulmonary suction and therapy. If the fever remains at 39  C for 8 h, a respiratory cause is presumed. Full culture of these children is indicated, and while the culture results are pending, they should be started on a broad-spectrum antibiotic against respiratory organisms. Also, if chest radiographs suggest an infiltrate, antibiotic treatment should be started. If the children respond to the antibiotics by becoming afebrile, the antibiotic treatment is generally continued for 7 days as empiric treatment unless other specific culture results are obtained.

Pneumothorax or Hemothorax and Pleural Effusion Respiratory Failure As noted earlier, some children may need to be intubated and ventilated for the first 12–24 h postoperatively; however, we feel an aggressive attempt at early extubation allows for a quicker recovery with fewer complications. Clearly, however, many high functioning children with good oral motor control can be extubated safely in the operating room. There are also those children with poor oral motor control who will need to be intubated for 5–7 days until the fluid shifts have all stabilized, until they have little need for pain medication, and they are close to their preoperative motor function. It is very important that this preoperative oral motor function information be communicated to the physicians managing these children in the intensive care unit. These physicians will often not have seen the children before their arrival in the intensive care unit. There are

Another occasional problem in the postoperative period is the occurrence of fluid or air in the chest cavity, most commonly a pleural effusion. This effusion usually becomes apparent on an upright chest radiograph 5–7 days after surgery. It most likely occurs slowly in the postoperative period, during rapid fluid shifts and periods of generalized edema (Fig. 4). During these times, there are seldom good upright chest radiographs so the fluid may be present but not seen if the amount is small. Only rarely does the effusion get large enough to impair breathing, which is the only indication to treat by tube drainage. Most of the time when drainage is required, it is a pink-tinged to serous fluid. Rarely, the drainage will contain a significant amount of blood, which presumably drained into the chest from the posterior surgical wound. This effusion usually resolves quickly with drainage over several days. If an effusion

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Fig. 4 Pleural effusions are relatively common after Unit rod instrumentation. If the effusion becomes very large and impacts the ability to ventilate the child (a), a chest tube may need to be inserted (b). Usually, there is serous or serosanguinous drainage for 3 or 4 days until the child is

diuresing well. On rare occasions, a hemothorax may be seen, and it is then presumed that the posterior wound is draining into the chest. Chest tube drainage and correction of coagulopathies will always stop this bleeding

develops in a chest which also has the VP shunt draining, it is possible that the VP shunt may obstruct causing hydrocephalus (Chaudhry 2012). Pneumothorax may also be noted, sometimes occurring as late as 10 days postoperatively. If children are having respiratory problems or difficulty with hypoxemia, an upright chest radiograph should be obtained any time during the hospital stay. If the pneumothorax is relatively small and minimally symptomatic, it may be carefully monitored. However, if children are having significant respiratory problems or the pneumothorax involves more than 30% of the volume of the chest, it should be drained with a tube. The origin of these pneumothoraces may come from positive pressure ventilation, incidental opening of the chest during posterior spinal surgery, or from the insertion of the central line. However, these pneumothoraces are usually relatively minor and insignificant in the overall recovery of children.

(Bohmer et al. 1999). Our protocol is to do the spinal fusion before any surgical correction of the gastroesophageal reflux or aspiration. Some children will have a dramatic postoperative improvement in the reflux; (Case 1) however, some will have no change and some will become significantly worse. These outcomes are in approximately equal proportions, although we do not have good objective data to make this evaluation. Clearly, these children can be managed safely through the spinal fusion, and then the response can be assessed and appropriate treatment instituted following recovery from the spine surgery. These children need to be monitored very carefully, especially in the intensive care unit immediately after extubation and then again when feeding is begun. Feeding should be with the children in an upright position with careful monitoring to make sure there is no reflux and aspiration. If there is any evidence of reflux, feeding should be stopped immediately and the respiratory status should be monitored carefully. If there is any suggestion of aspiration of the stomach contents, children should be treated for aspiration pneumonia. Most children with severe quadriplegic pattern CP have some posterior aspiration and run a risk of aspiration during the initiation of feeding. This aspiration can lead to very severe and rapid respiratory compromise.

Reflux and Aspiration Many children with CP have gastroesophageal reflux(GERD) and chronic aspiration. The presence of scoliosis has also been associated with an increased incidence of these problems

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There is one report suggesting the presence of GERD may raise the risk of wound infection (Chidambaran et al. 2013). Some children with tracheal malacia develop a redundant and collapsing trachea as the scoliosis increases, sometimes with collapse and compression between the sternum and spine. In two of our patients, the response to correcting the spinal deformity was complete resolution of the symptoms of tracheal collapse and compression. There was concern that these children might have been made worse.

Pancreatitis Chemical pancreatitis, as expressed by a rise in the serum amylase, is relatively common and is present in approximately 50% of children in the postoperative period. A much smaller number, approximately 15–20%, has some symptomatic pancreatitis that may rarely become very severe (Borkhuu et al. 2009; Nishnianidze et al. 2016). One of the deaths in our patients was from acute hemorrhagic pancreatitis. The cause of pancreatitis is unknown; however, it has been recognized as a risk of most spine surgery even in otherwise healthy adolescents who have idiopathic adolescent scoliosis (Leichtner et al. 1991; Shapiro et al. 2001). When children are symptomatic from pancreatitis, medical management includes maintaining adequate liquids and nutrition with central venous hyperalimentation and resting of the gastrointestinal system.

Colicystitis Most of our children are managed with aggressive postoperative nutrition with central venous hyperalimentation on day 2 or 3, and by day 5 or 7, when they have bowel sounds but are not tolerating feeding, the workup should include an ultrasound of the gallbladder. Often, some sludge is noted in the gallbladder, occasionally with some inflammation of the wall of the gallbladder. Sometimes stones are found as well, leading to this inflammation. Children with severe

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disabilities are at increased risk of developing colicystitis and cholangitis. When colicystitis is diagnosed in the postoperative period, medical management includes gastrointestinal rest and antibiotics. Following full recovery, children may be scheduled for colicystectomy.

Duodenal Obstruction Obstruction at the second part of the duodenum where it is trapped between the superior mesenteric artery and the spine may occur in malnourished children with CP, even without any surgical insult (Beccaro et al. 1991). After correction of major scoliosis curves, especially those that involve significant lordotic deformity, duodenal obstruction is even more common. These children present with good bowel sounds; however, their stomachs become very distended when fed. Severe stomach distension leading to death can occur (Beccaro et al. 1991). The treatment is to drain the stomach, rest the bowel, and provide nutrition. This obstruction is definitively diagnosed by a swallow study with dilute barium. If the first part of the duodenum fills but the barium does not continue to pass, there is a duodenal obstruction. Some children will have a partial obstruction, which can be managed by giving small amounts of fluid, and a jejunal tube can be passed through the area of the obstruction in some children. The final treatment of this problem is getting the child to gain weight, which may require prolonged central venous hyperalimentation. One of our children required hyperalimentation for more than 2 months. Parents must be informed that some of these children are at risk for the obstruction returning if they do not eat adequately and start to lose weight in the months following surgery. In rare chronic cases, jejunal tube feeding may be needed for prolonged periods to prevent recurrence of the obstruction.

Constipation Constipation is a persistent and chronic problem for many children. This constipation is not

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affected much either positively or negatively by the spine fusion; however, families should be instructed on methods to avoid prolonged impactions postoperatively, which tend to decrease the children’s interest in eating.

Poor Feeding As mentioned before, good postoperative nutrition is important. Good nutrition is especially important for children who have little reserve to heal the very large wound created by doing a posterior spinal fusion, which involves all of the spine and posterior pelvis. The goal is for children to take in 1.2–1.5 times the daily caloric requirement in the first postoperative month. This intake may be accomplished with oral nutritional supplements and occasionally with short-term nasogastric tube feeding in children who are not eating enough and who do not have a gastric tube. Our experience is that many parents who refuse to use a nasogastric tube preoperatively can be convinced to use it for a short time in the postoperative period when the tube may be seen as part of the surgical treatment.

Seating Adjustments One positive or negative effect of major deformity correction in teenagers with severe spinal curves is a dramatic change in their sitting height. Although this is very dependent on the specific deformity, sitting height gains of 10–15 cm are common. These major changes in the children’s body shapes also require that their wheelchairs have major adjustments before they are allowed to sit in them for a significant amount of time. Also, parents should be warned about this significant gain in height, especially if they are transporting these children in vans with wheelchair lifts, as frequently these vans were adapted when the children were smaller or during a time when significant scoliosis was already present. Parents may need to make plans for modification of their vans or for a different wheelchair. This planning should occur before attempting to place children

Fig. 5 Following posterior spinal fusion, many children are reported by parents to lose hair. Some children develop a completely bald spot, sometimes in an area of inflammatory response. In almost all children, the hair regrows completely over the next 6–12 months. The cause of the hair loss is not known but is probably a stress response to the surgery

in the van after discharge following spinal fusion so that the parents will not suddenly realize that they can no longer transport the child in their van.

Hair Loss Most children have increased hair loss from the stress of a large operation such as a posterior spinal fusion. Some children will develop spots of alopecia, usually 2–3 cm in diameter. Usually the parents can be assured that the hair will grow back in 6–12 months (Fig. 5).

Doing Posterior Spinal Fusion When Families Refuse Blood Transfusions Some families will refuse a blood transfusion because of religious beliefs but still desperately want their children to have a spinal fusion. In general, we strongly advise these parents to leave the children alone and accept the consequence of the scoliosis. However, we have done an instrumentation and fusion without transfusion when families strongly insisted on two occasions. This technique requires that only the spinous processes be exposed to the point that the interspaces can be opened and the sublaminar wires passed.

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The distal (pelvic) end of the wound is exposed and the Unit rod is inserted into the pelvis, and then the wires at the distal half of the wound are tightened onto the rod, leaving the rod protruding proximally. Bone graft is then applied over the rod and the wound is closed around this packed bone graft as far proximally as possible. Another alternative is to use pedicle screws with minimal dissection; however, the risk of nonunion will be high either way. If the blood loss is less than 5% of the blood volume, the superior half of the wound is similarly opened, the rod wired into place, and the wound closed. The goal of the surgery is to complete the whole operation with less than 10% loss of the blood volume. If 10% of the blood volume loss is encountered, the rod should be cut off and the wound closed at the level of surgery. The operation can then be completed in 2–3 weeks when children have made new blood. This is a very inadequate surgery because there is no decortication and no facetectomy, with only very minimal bone exposure. A high rate of pseudarthrosis is expected.

Dealing with Families Who Refuse Spinal Fusion There are families who choose not to have a spinal fusion even when it is the best choice for their children. It is important for families to have a good understanding of what it means for their children to continue to grow as their scoliosis gets worse. It is important to offer the family support through hospice care and palliative medical approaches. Occasionally, families refuse to have the surgery in cognitively highfunctioning children with excellent long-term functional potential in educational and occupational endeavors. Again, these families need to be appraised of the consequence of continued worsening of the scoliosis, and that later correction of the scoliosis would have to include an anterior spinal release as well. It is a family’s legal right to choose not to have the spinal fusion, and physicians should make an effort to maintain a good relationship with these families. Often, as the children develop more deformity and more

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difficulty over time, these families will choose to have the spinal deformity corrected when the risks are higher and the correction is more difficult.

Handling Families and Children When a No Resuscitation Status Is Requested An ethically and philosophically difficult situation may arise for families who choose to provide children only comfort care. Comfort care means families want to provide all care to make their children as comfortable and as happy as they can but do not want treatment that will prolong their children’s lives. Many medical professionals feel that a spinal fusion goes beyond comfort care; however, for families who are managing and caring for their children at home, the ability to spend long periods of time in a wheelchair is an important part of the comfort care. This spinal fusion allows families to take their children grocery shopping or to church, etc. As these children become more scoliotic, they have to spend more of their time reclined in a lying position. It is very difficult to take near adult-sized individuals out in public in a device that looks like a rolling bed. Also, there are families who perceive that the children are very uncomfortable with the increasingly severe scoliosis and want to do something to make them more comfortable. Clearly, this is a high-risk surgical group, and most of our acute deaths have occurred in this group. This is a situation with a high philosophical and ethical dilemma. If physicians are not comfortable with this dilemma, families should be referred for other opinions. If children really are too high risk to consider, and the families talk to two or three additional surgeons who all think this way, they will likely accept these opinions, or they will continue to look for someone who is willing to help them. This ethical and philosophical problem is often hard for operative anesthesia and nursing staff, as well as intensive care unit physicians and nursing staff, to understand. A case example is Craig, a 19-year-old boy with severe spastic GMFCS V quadriplegia and severe cognitive disability. He was cared for in a group home sponsored by his parents. The parents continued to be

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very involved in Craig’s care. He developed severe scoliosis and a spinal fusion was performed under a no resuscitation order, which was agreed to by the operative team. The surgical event and recovery were uneventful; however, 4 weeks after discharge he was brought back to the hospital with a temperature of 40.2  C, vomiting, and not tolerating any food. An examination determined that the source was likely an acute abdominal process, and surgical exploration was recommended. The family refused because they felt this would be a life-saving procedure, not a life quality enhancement procedure. Within 12 h of the family making this decision, he died of sepsis. An autopsy demonstrated a ruptured Meckel’s diverticulum. There was significant frustration among the surgeons and nurses and difficulty understanding how the family could agree to a very large operative procedure, such as the spine fusion, but refuses a simpler life-saving laparotomy. Other professionals should also be able to decide that they are comfortable with the ethical decisions of the families, or they should be allowed to not participate in the care of these children. When problems arise and families decide that they want no further intervention, physicians in most circumstances should not be surprised, as this issue should have been addressed with families preoperatively. It is difficult after working hard on a surgical procedure for families then to say they want everything stopped. This may be especially hard for consultants to understand who have not had the extensive family contact or experience in dealing with this population of individuals with severe disabilities as the neuro-orthopedist has.

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Fig. C1.1 Scoliosis has reached 95

Cases

Case 1 Eric

Eric, a 14-year-old boy with severe quadriplegia and severe mental retardation, was evaluated because of his increasing scoliosis, which had reached 95 (Fig. C1.1) and

Fig. C1.2 Scoliosis at 95

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Fig. C1.3 As a result of the coagulopathy, and the surgical technique at that time in which the pelvic holes were drilled just before to rod insertion, pelvic fixation was abandoned and he was only instrumented to L5

made sitting very difficult (Fig. C1.2). He was only comfortable lying in one position. He was also in treatment for severe gastroesophageal reflux, and was taking tegretol to treat seizures. He was very thin and weighed 23 kg. After the reflux was under maximum medical management, he had spinal surgery with a Unit rod instrumentation to correct the scoliosis. During surgery, he had a high blood loss, totaling four blood volumes, due to a coagulopathy that was not treated aggressively enough early in the case. As a result of the coagulopathy, and the surgical technique at that time in which the pelvic holes were drilled just before to rod insertion, pelvic fixation was abandoned and he was only instrumented to L5 (Fig. C1.3). Postoperative radiographs

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Fig. C1.4 Increased pelvic obliquity and lumbar scoliosis

showed good but not complete correction of the pelvic obliquity. His postoperative recovery was uneventful with greatly diminished gastroesophageal reflux. Immediately after surgery, sitting was much improved. He again presented 9 months following surgery with increased sitting difficulty and increased gastroesophageal reflux. He had increased pelvic obliquity and lumbar scoliosis (Fig. C1.4). He was then taken back to surgery for an anterior release, followed by posterior osteotomies, and connection to the proximal end of the rod with rod connectors (Fig. C1.5). For the first 3 months after surgery, he was again much better with decreased gastroesophageal reflux but then had a sudden onset of reflux and the parents felt his body shape changed. Repeat examination demonstrated (continued)

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Fig. C1.5 Anterior release, followed by posterior osteotomies, and connection to the proximal end of the rod with rod connectors

that the rod connectors had failed, which required a third procedure with rod replacement (Fig. C1.6). Following the third operation, his reflux was again under easy control (Fig. C1.7). This case demonstrates how responsive reflux is to spine deformity correction in a few children. Some get worse and many are unchanged. This case also demonstrates two major errors. One is that the procedure needs to be planned for progressive increase in blood loss, which the team must be prepared to address; that means the pelvic holes should be drilled early in the case when there is little blood loss. The second error is that end-to-end rod connectors located at the same level have a high failure rate and this should be avoided. We had three such failures until we learned this lesson.

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Fig. C1.6 Repeat examination demonstrated that the rod connectors had failed, which required a third procedure with rod replacement

Case 2 Hyon

Hyon, a 12-year-old girl with severe spastic quadriplegia, developed a lordoscoliosis that required instrumentation and correction. The pelvis was noted to be very osteoporotic and thin. With care, the rod was inserted and the abdominal examination was thought to be normal. There was significant bleeding present during the case and, after she was turned into the supine position, the abdominal examination suggested a possible protrusion on the right side, although it was not definitive. An oblique radiograph of the pelvis demonstrated the rod outside the confines of the pelvis (Fig. C2.1). After some discussion, it was decided to leave the rod in this position. She did well postoperatively and was discharged from the intensive care unit on (continued)

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Fig. C2.2 The rod inserted and connected with an end-toend rod connector

Fig. C1.7 Reflux is again under easy control

Fig. C2.1 An oblique radiograph of the pelvis demonstrated the rod outside the confines of the pelvis

the fourth postoperative day. On the fifth postoperative day, she was returned to the operating room where the rod was cut off on the right side, the correct hole identified, and the rod inserted and connected with an

Fig. C2.3 This case demonstrates an acceptable approach, although a second crosslink would be preferred

end-to-end rod connector (Fig. C2.2). This case demonstrates an acceptable approach, although a second crosslink would be preferred (Fig. C2.3). Also, we prefer to do the revision before leaving the operating room whenever possible.

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Cross-References ▶ Anesthetic Management of Spine Fusion ▶ Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management ▶ Complications of Hip Treatment in Children with Cerebral Palsy ▶ Surgical Treatment of Scoliosis Due to Cerebral Palsy

References Del Beccaro MA, McLaughlin JF, Polage DL (1991) Severe gastric distension in seven patients with cerebral palsy. Dev Med Child Neurol 33:912–916 Boachie-Adjei O, Lonstein JE, Winter RB, Koop S, vanden Brink K, Denis F (1989) Management of neuromuscular spinal deformities with Luque segmental instrumentation. Joint Surg Am 71:548–562 Bohmer CJ, Niezen-de Boer MC, Klinkenberg-Knol EC, Deville WL, Nadorp JH, Meuwissen SG (1999) The prevalence of gastroesophageal reflux disease in institutionalized intellectually disabled individuals. Am J Gastroenterol 94:804–810 Borkhuu B, Nagaraju D, Miller F, Moamed Ali MH, Pressel D, Adelizzi-Delany J, Miccolis M, Dabney K, Holmes L Jr (2009) Prevalence and risk factors in postoperative pancreatitis after spine fusion in patients with cerebral palsy. J Pediatr Orthop 29:256–262 Chaudhry MS (2012) Intracranial hypotension caused by dural-pleural fistula. J Neuroimaging 22:208–209 Chidambaran V, Gentry C, Ajuba-Iwuji C, Sponsellar PD, Ain M, Lin E, Zhang X, Klaus SA, Njoku DB (2013) A retrospective identification of gastroesophageal reflux disease as a new risk factor for surgical site infection in cerebral palsy patients after spine surgery. Anesth Analg 117:162–168 Dhawale AA, Shah SA, Sponseller PD, Bastrom T, Neiss G, Yorgova P, Newton PO, Yaszay B, Abel MF, Shufflebarger H, Gabos PG, Dabney KW, Miller F (2012) Are antifibrinolytics helpful in decreasing blood loss and transfusions during spinal fusion surgery in children with cerebral palsy scoliosis? Spine (Phila Pa 1976) 37:E549–E555 Duckworth AD, Mitchell MJ, Tsirikos AI (2014) Incidence and risk factors for post-operative complications after scoliosis surgery in patients with Duchenne muscular dystrophy: a comparison with other neuromuscular conditions. Bone Joint J 96-B:943–949 Givon U, Miller F (1999) Shortening of a unit rod protruding into the hip joint: case report and description of a surgical technique. J Spinal Disord 12:74–76

1799 Jevsevar DS, Karlin LI (1993) The relationship between preoperative nutritional status and complications after an operation for scoliosis in patients who have cerebral palsy [published erratum appears in Joint Surg Am; 1256]. Joint Surg Am 75:880–884 Krismer M, Bauer R (1990) The Luque-Galveston operation in the treatment of neuropathic pelvic tilt. Orthopade 19:309–314 Lai LP, Egnor MR, Carrion WV, Haralabatos SS, Wingate MT (2014) Ventricular peritoneal shunt malfunction after operative correction of scoliosis: report of three cases. Spine J 14:e5–e8 Legg J, Davies E, Raich AL, Dettori JR, Sherry N (2014) Surgical correction of scoliosis in children with spastic quadriplegia: benefits, adverse effects, and patient selection. Evid Based Spine Care J 5:38–51 Leichtner AM, Banta JV, Etienne N (1991) Pancreatitis following scoliosis surgery in children and young adults. J Pediatr Orthop 11:594–598 Lipton GE, Miller F, Dabney KW, Altiok H, Bachrach SJ (1999) Factors predicting postoperative complications following spinal fusions in children with cerebral palsy. J Spinal Disord 12:197–205 Master DL, Son-Hing JP, Poe-Kochert C, Armstrong DG, Thompson GH (2011) Risk factors for major complications after surgery for neuromuscular scoliosis. Spine (Phila Pa 1976) 36:564–571 Nectoux E, Giacomelli MC, Karger C, Herbaux B, Clavert JM (2010) Complications of the LuqueGalveston scoliosis correction technique in paediatric cerebral palsy. Orthop Traumatol Surg Res 96:354–361 Nishnianidze T, Bayhan IA, Abousamra O, Sees J, Rogers KJ, Dabney KW, Miller F (2016) Factors predicting postoperative complications following spinal fusions in children with cerebral palsy scoliosis. Eur Spine J 25:627–634 Onimus M, Manzone P, Lornet JM, Laurain JM (1992) Surgical treatment of scoliosis in bed-ridden patients with cerebral palsy. Rev Chir Orthop Reparatrice Appar Mot 78:312–318 Samdani AF, Belin EJ, Bennett JT, Miyanji F, Pahys JM, Shah SA, Newton PO, Betz RR, Cahill PJ, Sponseller PD (2016) Major perioperative complications after spine surgery in patients with cerebral palsy: assessment of risk factors. Eur Spine J 25:795–800 Shapiro G, Green DW, Fatica NS, Boachie-Adjei O (2001) Medical complications in scoliosis surgery. Curr Opin Pediatr 13:36–41 Theroux MC, Corddry DH, Tietz AE, Miller F, Peoples JD, Kettrick RG (1997) A study of desmopressin and blood loss during spinal fusion for neuromuscular scoliosis: a randomized, controlled, double-blinded study. Anesthesiology 87:260–267 Thompson GH, Florentino-Pineda I, Poe-Kochert C, Armstrong DG, Son-Hing J (2008) Role of Amicar in surgery for neuromuscular scoliosis. Spine (Phila Pa 1976) 33:2623–2629

Neuromonitoring and Anesthesia for Spinal Fusion in Cerebral Palsy

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Sabina Dicindio, Anthony DiNardo, and Mary C. Theroux

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1802 Personnel and Practical Aspects of Neuromonitoring . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1802 Technical Aspects and Interpretation of IONM . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Somatosensory Evoked Potentials (SSEP) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Motor Evoked Potentials (MEP) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Electromyography (EMG) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Stagnara Wake Up Test . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1805 Physiological Application and Risk of Neuromonioring . . . . . . . . . . . . . . . . . . . . . . . . . . . 1806 Blood Supply to the Spinal Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1806 Risk of Neuromonitoring . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1806

S. Dicindio (*) Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Department of Pediatrics, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA e-mail: [email protected]; [email protected] A. DiNardo SpecialtyCare, Nashville, TN, USA e-mail: [email protected] M. C. Theroux Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA Department of Pediatrics, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA Department of Anesthesiology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, USA e-mail: [email protected]; Mary. [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_119

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S. Dicindio et al. Anesthesia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1807 Inhaled Anesthetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1807 Intravenous Anesthetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1807 Nonanesthetic Intraoperative Influences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1808 Clinical Application . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1809 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1810

Abstract

Intraoperative neuromonitoring (IONM) is used to help prevent spinal cord injury, a potential complication of spinal surgery. Neuromonitoring is challenging in patients with cerebral palsy and requires close communication among the anesthesiologist, neurophysiologist, and the surgical team. Factors such as the pathophysiology of cerebral palsy, preexisting neurologic deficits, types of monitoring modalities available, the expertise of the monitoring personnel, anesthetic effects, and surgical maneuvers all have an impact on intraoperative neuromonitoring (IONM) and must be considered in order to safely care for these patients. Keywords

Cerebral palsy · Spine fusion · Neuromonitoring · Anesthesia · Evoked potentials

Introduction Pediatric patients diagnosed with cerebral palsy (CP) have scoliosis with a reported incidence of 5–75% (Balmer and MacEwen 1970; Benson et al. 1998b; Master et al. 2011). Progression of the scoliotic curve interferes with general health and wellbeing. This incidence increases with the severity of the spinal deformity (Sarwark and Sarwahi 2007). A major complication of surgical correction of scoliosis or kyphosis is the potential injury to the spinal cord resulting in neurological deficit. Injury when it occurs often results in partial or complete loss of sensory and motor function. The risk may be attributed to a variety of factors including degree and type of curve as well as conditions

that result in hemodynamic instability during the procedure. Patients with neuromuscular scoliosis have a higher risk for neurologic injury, 1.1%, while having corrective spinal fusion surgery (Lipton et al. 1999; Reames et al. 2011). Overall complication rate reported is also higher for neuromuscular scoliosis (17.9–74%, while the reported mortality rate is as high as, 0.34–1.6% (Barsdorf et al. 2010; Reames et al. 2011). Intraoperative neuromonitoring (IONM) is the use of physiological techniques to assess neural integrity as well as map neural structures to minimize the risk of injury during the surgical procedure. It was first used for spinal deformity surgery in the early 1980s and has evolved to become part of the standard of care. Significant improvement in efficacy and reliability of IONM were made possible by two discoveries: successful stimulation of the motor cortex through an intact cranium and the ability of Anesthesia providers to use a total intravenous anesthetic technique in order to facilitate best possible monitoring environment (Merton and Morton 1980; Sloan and Heyer 2002). IONM as it is done today is not possible without a knowledgeable anesthesia team, who must consider the physiological factors, surgical maneuvers, and anesthetic drugs, all of which have an effect on the evoked potentials, the EMG, and the EEG. This highlights the need for close communication between the surgeon, the neurophysiologist, and the anesthesia team.

Personnel and Practical Aspects of Neuromonitoring Similar to the more established clinical services, neuromonitoring is a clinical service, which establishes a relationship between provider and patient. Neuromonitoring services employed by a particular

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facility may differ in their scope of practice and the level of responsibility they are willing to undertake. Similar to many clinical practices especially in United States, the actual establishes a relationship between provider and patient. Neuromonitoring services employed by a particular facility may differ in their scope of practice and the level of responsibility they are willing to undertake. Similar to many clinical practices especially in United States, the actual make-up of the neuromonitoring team differs based on surgeon and hospital preferences as well as the complexity of the patient population. The Scoliosis Research Society has reported neurologic injury to have an incidence of 0.34–1.1% in patients with neuromuscular scoliosis (Reames et al. 2011). The patients at greatest risk for neurologic injury are pediatric patients, cases involving implants, and those who present with severe and rigid deformities including those with kyphoscoliosis, neuromuscular scoliosis, and combined anterior posterior repair (Ecker et al. 1996; Glover and Carling 2014; Reames et al. 2011). Iatrogenic causes of neurologic injury can be due to direct trauma to the cord by instrumentation, reduction of spinal cord blood flow by stretching or compressing of vessels, direct interruption of radicular blood flow, distraction injury of the spinal cord or epidural hematoma (Gibson 2004). When discussing patients with CP, it is important to realize that there is a continuum of disability present, and the most severely affected patients have limited or no volitional movement or purposeful activity. Questions have been raised in the past regarding the risk/benefit or cost/benefit ratios as they apply to this most profoundly affected group (Stecker 2012). It is legitimate to ask if they actually derive benefit from being monitored. It is our opinion that all neuromuscular scoliosis patients, including those with profound deficits, should be monitored or have monitoring at least attempted during spinal deformity surgery. This should be done for the same reasons all scoliosis surgeries are monitored: to identify and attempt to preclude impending spinal cord injury as well as preserve any and all existing neural function to the extent possible, including upper extremity function. Cerebral Palsy is defined as a static encephalopathy, which is a nonprogressive brain injury.

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These patients do not typically have a concomitant spinal cord injury. They can little afford to sustain injury to any additional functional neural structures or pathways. Additional injury could not only offset the qualitative gains they receive from the successful surgery but could also interfere with any clinical improvement that might result from long-term neuroplastic processes. Additionally, in our experience and perhaps representing something of a paradigm shift, we have found that neuromonitoring under anesthesia can provide information about the functional integrity of the corticospinal and motor pathways not achievable by other means. This can provide a basis for establishing an electrophysiological profile of a patient, which can be used to further subclassify a diagnosis with information based on the functional neurophysiology. For example, we have used it to correlate the patient’s neurophysiological status with brain MRI findings. Other potential uses exist; it may contribute to the planning of the patient’s rehabilitation, as we have seen many cases where the quality of the evoked potentials was better than expected, and it may be useful as a biomarker of therapeutic efforts and, finally, as a stimulus for future research hypotheses.

Technical Aspects and Interpretation of IONM The monitoring is performed with electrical stimulation of peripheral nerves and/or the motor cortex with the response to the stimulus measured as an evoked potential. Somatosensory evoked potentials (SSEP) record spinal responses with epidural electrodes or cortical responses with scalp electrodes. Motor evoked potentials (MEP) transcranially stimulate the motor cortex and record the response at the spinal level with epidural electrodes or more commonly, from muscles as a compound muscle action potential (CMAP). SSEPs monitor the dorsal portion of the spinal cord and the MEPs monitor the anterior two thirds of the spinal cord. SSEP and MEP used concurrently allow detection of injury to the motor and sensory pathways of the spinal cord. SSEP and MEP monitoring are considered the standard of

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care for spinal surgery (Deletis and Sala 2008; Padberg et al. 1998; Schwartz et al. 2007). Evoked potentials are evaluated in terms of measured amplitude (microvolts), latency (milliseconds), and morphology (shape, biphasic or poly phasic). Amplitude is the primary criterion used for evaluating the intraoperative signals, but changes in any of these parameters are a basis for suspicion of an evolving pathophysiologic process or injury. In spinal surgery for scoliosis correction, neurologic complications can occur at different levels and involve different pathways. Evoked potential efficacy relies on the ability to detect the lack of conduction or a decrease in evoked response to stimulation. Pathophysiology due to trauma, ischemia, or distraction of the spinal cord results in loss of synchronization of volleys or actual blockade of axonal transmission and decrease in evoked response to stimulation. This is manifested as a voltage drop in evoked potential amplitude (Emerson 1988).

Somatosensory Evoked Potentials (SSEP) The sensory tracts are monitored by SSEPs. Stimulation of a peripheral nerve in the upper and lower extremities monitors the dorsal column of the spinal cord. The lower extremity nerves, which include the posterior tibial nerve or the peroneal nerve, allow most of the length of the entire spinal cord, brainstem, and somatosensory cortex of the brain to be monitored. The sacral roots and sacral portion of the spinal cord are not monitored with SSEP’s because the posterior tibial nerve enters the spinal column at the L5 cord level. Stimulation of the upper extremity nerves, the ulnar or median nerves, monitors the cervical spinal segments down to T1 level and acts as a control for the lower somatosensory evoked potentials. The median nerve can act as a control for the lower extremities but its limitations are that it only covers to the C6 nerve root level. The ulnar nerve, which is more prone to stretch or compression injury because of arm positioning for the surgery gives a better indication of

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overall brachial plexus function. In addition, it avoids medial placement of stimulus electrodes, which would be in the way of the anesthesia lines. The stimulus travels from the peripheral nerve afferents to the dorsal root ganglia, and then ascends the dorsal column spinal pathways to the medullary nuclei in the brainstem where it crosses midline and ascends in the medial lemniscal pathways. The signal synapses in the thalamic nuclei and projects up to the sensorimotor cortex. Any SSEP changes with amplitude reduction of more than 50% and a latency increase of 10% or greater should be considered significant. These changes can be due to perfusion issues or associated with a specific surgical intervention, such as during placement of spinal instrumentation or during correction of a spinal deformity (Glover and Carling 2014; Gonzalez et al. 2009). SSEP specificity is 27–99% and sensitivity is 0–100%; studies have shown SSEP monitoring to reduce neurologic deficit (Fehlings et al. 2010; Gonzalez et al. 2009; Nuwer et al. 1995). Its limitations lie in that it only monitors the dorsal spinal cord function; it does not provide information regarding the motor tracts (Glover and Carling 2014; Deletis and Sala 2008).

Motor Evoked Potentials (MEP) Motor tracts are monitored by MEPs. Stimulation of the motor cortex causes the spinal cord and peripheral muscles to produce a recordable potential. MEPs can be magnetically or electrically elicited. Magnetic stimulation is generally not used for IONM because it is more affected by anesthesia and the stimulating coils are large and not practical for use on an operating room bed and will not be discussed. Transcranial electric motor evoked potentials (tcMEP) are commonly used to assess the motor function of the spinal cord. A multi-pulse technique, using 59 electrical impulses of 300–700 milliamps with a pulse duration of 75 microseconds and 1000 pulses/second (p/s) and a stimulation rate of 1 hertz (HZ), is less sensitive to anesthetic agents compared to single

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pulse technique (Szelenyi et al. 2007). Subdermal needle electrodes initiate stimulation of the motor cortex and this stimulation is then transmitted through the internal capsule to the caudal medulla. The majority of the fibers cross midline within the pyramidal tract and descend into the lateral and anterior tracts of the spinal cord. An initial D (direct wave) is followed by several I (indirect) waves. D waves are due to direct excitation of the corticospinal tract neurons; I (indirect) waves are due to indirect depolarization and result from synaptic transmission of the stimulus. The I waves cause stimulation of the anterior horn cells with subsequent motor nerve transmission to the muscle. A compound muscle action potential (CMAP) is due to muscle depolarization or direct stimulation of a nerve root or the spinal cord. D waves are less influenced by anesthetics as compared to I waves because I wave propagation involves a greater number of synapses (Macdonald et al. 2013). Commonly monitored CMAPs include those from the first dorsal interosseii, rectus abdominis, iliopsoas, quadriceps, tibialis anterior, and adductor hallucis muscles. An “Alert” of a potential neurological compromise is given to the surgeon and the Anesthesiologist when a significant drop in MEP amplitude compared to baseline occurs; this may include approximately 80% decrease when using CMAP or 50% decrease when recording D waves. Other warning criteria include a labile response indicated by either intermittent ability to record or a significant change drop in MEP amplitude compared to baseline occurs; this may include approximately 80% decrease when using CMAP or 50% decrease when recording D waves. Other warning criteria include a labile response indicated by either intermittent ability to record or a significant change in evoked potential threshold if not related to anesthetic effects (Deletis and Sala 2008; Macdonald et al. 2013; Pajewski et al. 2007). TcMEPS are considered more sensitive than SSEP with a sensitivity of 100% and specificity of 81–96% (Fehlings et al. 2010; Gonzalez et al. 2009; Schwartz et al. 2007). However, they are very sensitive to blood supply and volatile anesthetics (Jameson and Sloan 2012; Macdonald 2006a). They also have a large inherent, trace-to-trace variability, which can be very

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problematic for interpretation especially if the patient is not in a relatively steady state of anesthesia.

Electromyography (EMG) Electromyography (EMG) is used as an adjunct to SSEP to monitor for spinal nerve root injury. Because SSEPs assess several nerve roots at the same time, they are neither sensitive nor specific for identifying an injury to a particular spinal nerve root(s). They are rarely used alone if monitoring for spinal nerve root injury, and it is typical for them to be paired with motor evoked potentials for this purpose. When transpedicle screws are used, EMG stimulation allows detection of incorrect screw placement. If an electric stimulus applied to the pedicle screw with a current of less than 7 milliamps elicits a CMAP, then it may be interpreted as a possible crack or fracture of the pedicle wall while a current of 4 milliamps or less indicates the likelihood of a frank breach of the pedicle. An advantage to EMG is that it is not influenced by anesthetics (Glover and Carling 2014). The introduction of the intraoperative CT scan is replacing the manual stimulation of the pedicle screws as the scan shows the location of the pedicle screw within the pedicle and if it is in the proper location.

Stagnara Wake Up Test The Stagnara wake-up test is still the gold standard for intraoperative assessment of neurologic status. It awakens the patient during the procedure in order to assess lower extremity movement when neurological injury is suspected or after the correction of the spinal deformity. It is a tool, which continues to have value as an adjunct but due to its limitations, which include disruption of the surgery, potential harm to the patient, and its lack of providing data in a continuous and real time assessment of the spinal cord, its use, as a routine monitoring tool is impractical. There are also the issues in the cerebral palsy population of the patient’s cognitive ability to follow directions and their ability to produce volitional movement. The

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wake-up test can be used when the IONM information is equivocal such as if there is an unresolvable equipment issue, or the evoked potential signals have drifted so close to the noise threshold as to be nonreliable. Currently, IONM has become widely accepted as a continuous assessment tool of the integrity of the spinal cord (Deletis and Sala 2008; Schwartz et al. 2007).

Physiological Application and Risk of Neuromonioring Blood Supply to the Spinal Cord The spinal cord receives dual blood supply. The vertebral arteries give rise to paired posterior spinal arteries (PSA) and one anterior spinal artery (ASA). The ASA supplies the anterior two thirds to four fifths of the anterior spinal cord and the two PSAs supply the posterior one third of the spinal cord. The ASA predominantly supplies the spinal cord motor tracts while the PSA primarily supplies the sensory tracts. Radicular arteries from cervical, intercostal, thoracic, and lumbar arteries provide collateral blood flow to the spinal cord. The PSA receives more collateral blood flow than the ASA. The fewest radicular arteries are within the motor distribution of the thoracic spinal cord, and it is thus considered at highest risk for ischemia during hypotension. Due to the fact that motor and sensory components of the spinal cord are separated and because of the difference in their blood supply, SSEPs and MEPs are used simultaneously to provide a global status of the spinal cord. Also, because of the limited collateral flow to the anterior spinal cord, MEPs are more sensitive to ischemia and are considered an earlier predictor of impending damage to the cord. However, when multimodal monitoring is not possible, the individual modalities can be used to maximize patient safety (Jameson and Sloan 2012).

Risk of Neuromonitoring The risks of neuromonitoring include tongue and lip laceration (0.19%) due to electrical

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stimulation, scalp burns (0.013%), and cardiac dysrhythmias (0.033%) (Legatt 2002; MacDonald 2002). Of note, endotracheal tube rupture has been reported (Macdonald et al. 2013). In addition, there exists concern for induction of seizures with tcMEP. The risk of inducing seizures with tcMEP in patients undergoing spine procedures is reported as 0–0.2% (Salem et al. 2016; Schwartz et al. 2007; Ulkatan et al. 2017). The reported incidence of inducing seizures in those with and without a history of seizures is 0.7–1.5% and 1.1–4.4%, respectively (Salem et al. 2016). The risk of seizures provoked by motor evoked potentials used to be an absolute contraindication to and currently is not considered a contraindication. We have monitored many children with intractable seizure disorders with no intraoperative seizure complications and no reports of postoperative change or increase in seizure activity. Any risk for seizure is highest during the transition of cerebral states of consciousness such as with induction or emergence. It is prudent to avoid evoking the motor potentials at those points. In cases with the highest risk of seizures, the neurophysiologist may consider placing additional EEG leads, the temporal lobes, for example, which are not usually placed, for upper motor neuron spinal deformity cases. In general, the stimulation required by many of the CP patients is quite high and the attendant spasticity and hyperreflexia can cause a lot of passive patient movement as the muscles contract to the stimulus. In addition to the risks discussed above, this high stimulation causes episodic increase in bleeding as a result of increase in overall muscle and body tone which leads to a “pumping” of blood onto the surgical field with every stimulus, resulting in a relatively small but additionally appreciated blood loss. Stimulus should therefore be titrated as low as possible and the experienced surgical neurophysiologist should always try to limit the stimulations to the actual need, based on the individual patient, instead of relying on a “cookbook” neuromonitoring approach. Risks such as tongue and lip laceration may be easily avoided with careful placement of soft bite blocks. Risks such as inducing cardiac

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arrhythmias and seizures are rare. The Scoliosis Research Society in 2009 favored multimodal monitoring (SRS 2009) in spite of the risks involved.

Anesthesia All anesthetic agents are known to influence neuromonitoring by altering neuronal excitability by inhibiting synaptic function or slowing axonal conduction by changing the balance of neurotransmitters (Toleikis and American Society of Neurophysiological Monitoring 2005). The greater the number of synapses within the pathway, the greater the effect of anesthetic agents (Deletis et al. 1993). Generally, MEPs are more sensitive than SSEPs and cortical potentials are more sensitive than subcortical potentials. The motor pathways and MEPs are moderately susceptible to anesthetics at the motor cortex, severely affected at the anterior horn cell, and only affected at the neuromuscular junction if neuromuscular blockade is used. The D waves are unaffected by anesthetics because no synapses are involved in their production. However, I waves, because they arise from interneurons and require synaptic activity for their production, are more affected by anesthetics. The mechanics of depression varies depending on the anesthetic agent.

Inhaled Anesthetics All volatile anesthetics produce a dose-dependent increase in cortical SSEP latency and reduction in amplitude (Kalkman et al. 1991; Sloan and Heyer 2002; Zentner et al. 1992). This is due to a change in the receptor or changes in the receptor ion channels. The most potent are isoflurane, desflurane, and sevoflurane. These agents have less of an effect on subcortical SSEP because of the lower number of synaptic interactions. The volatile anesthetics depress synaptic transmission in the anterior horn cells or the cortex. With tceMEP, the suppressive effects of the inhalation agents can be partially compensated for by high

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voltage, multiple pulse transcranial stimulation (Lotto et al. 2004). Monitoring of responses allows the use of 0.5 minimal alveolar concentration (MAC) of inhaled anesthetics; however, much of this early work was based on SSEP data and is not accurate for the motor evoked potential. Because tcMEPs can be abolished at therapeutic concentrations of volatile agents, it may result in degradation of the CMAP to the point it cannot be monitored (Macdonald et al. 2013). It must also be appreciated that the CP population already has a motor deficit and signals are compromised going into the surgery, so any other factors, which reduce signal quality, contribute to neuromonitoring challenges. Nitrous oxide, which is an inhaled, but not a volatile anesthetic, reduces cortical SSEP amplitude and increases latency alone or when combined with volatile anesthetics or opioids. TceMEP are most affected by nitrous oxide (Sloan et al. 2010). Similar to the inhaled anesthetics, subcortical responses are minimally effected (Lotto et al. 2004).

Intravenous Anesthetics Opioids depress electrical excitability by a G protein mechanism. They produce minimal effect on spinal or sub-cortically recorded SSEP, and in fact, the SSEP responses benefit from the decreased EMG activity. Cortical responses display only mild depression of amplitude and increased latency. Subcortical responses are less affected. MEPs show a larger but still a relatively mild decrease in amplitude and increased latency (Kakinohana et al. 2002; Kalkman et al. 1991; Scheufler and Zentner 2002). Opioids can have significant effect on MEPs when given at doses which cause burst suppression on EEG. As a bolus or continuous infusion, fentanyl produces the greatest reduction in MEP followed by alfentanil then sufentanyl. Remifentanil is the least suppressive of all narcotics and the muscle MEPs are preserved at infusion rates of 0.6 ug/kg/ min (Lotto et al. 2004; Scheufler and Zentner 2002). The neurophysiologist should communicate the narcotic(s) effect on the responses to the anesthesia team so they can titrate the anesthetic to

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meet the patients’ needs yet retain a good signal to monitor. Barbiturates affect both motor and sensory potentials. SSEPs are variable in their response and depend on dose and type; thiopental decreases amplitude and increases latency transiently whereas phenobarbital does not affect SSEP. Motor potentials are more sensitive and CMAPs can be abolished for a prolonged period with administration of barbiturates (Lotto et al. 2004). Benzodiazepines can mildly depress cortical SSEP amplitude with minimal change in latency (Kalkman et al. 1991; Scheufler and Zentner 2002). Administered as a premedication, diazepam has little effect on MEP amplitude or latency. Midazolam, as a bolus or continuous infusion, has no significant effect with multi-pulse stimulation (Kalkman et al. 1991; Lotto et al. 2004; Scheufler and Zentner 2002). Ketamine and etomidate can increase as well as depress potentials. Ketamine, an n-methyldaspartate receptor antagonist, blocks glutamic acid – an excitatory neurotransmitter – and enhances cortical SSEP and MEP. Etomidate, via gaba-aminobutyric receptor inhibition, increases the amplitude of cortical SSEP and decreases MEP amplitude with no change in latency. Dexmedetomidine, an alpha 2 adrenergic agonists, has been administered with IONM. Dexmedetomidine when carefully titrated to depth of anesthesia has been used as part of a total intravenous anesthetic (TIVA) without affecting monitoring (Bala et al. 2008; Tobias et al. 2008). There are case studies which report decrease or loss of MEP with and without bolus dosing, SSEPs remained unchanged. If the dexmedetomidine dose is sufficient to cause a loss of the MEP, this effect is prolonged and extends over a few hours even if the dexmedetomidine infusion is turned off (Mahmoud et al. 2010). More studies are needed on the effects of dexmedetomidine on neuromonitoring and its regular use is not yet recommended. Propofol suppresses the activation of the alpha motor neurons at the spinal gray matter as well as enhances the inhibitory neurotransmitter, gamma aminobutyric acid, causes synaptic inhibition (Kakinohana et al. 2002). It causes amplitude depression of cortical SSEP and tceMEP

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(Kawaguchi et al. 2000; Nathan et al. 2003). However, propofol better preserves the cortical SSEP and MEP signal as compared to halogenated agents by themselves or with nitrous oxide (Macdonald et al. 2013; Pechstein et al. 1998). With propofol infusions, studies have demonstrated that the higher the infusion dose, the greater the number of stimuli which are needed to elicit a CMAP (Scheufler and Zentner 2002). While propofol does causes structural changes in the waveform, the changes are predictable and stable. Neuromuscular blocking agents (NMBA) have no effect on SSEP and, in fact, improve the response by removing competing EMG activity; however, they greatly influence the ability to monitor MEPs depending on degree of motor blockade (Pajewski et al. 2007). NMBAs bind at acetylcholine receptors within the neuromuscular junction and with profound blockade can prevent recording of CMAP. There are methods to record CMAP in presence of neuromuscular blockade; these require careful titration of neuromuscular blockade to a train of four or comparison of CMAP amplitude before and after NM blockade (Lotto et al. 2004).

Nonanesthetic Intraoperative Influences Nonanesthetic patient factors that can affect IONM include spinal cord blood flow, hematocrit, body temperature, and minute ventilation. A decrease in mean arterial pressure below the auto regulatory threshold decreases both MEP and SSEP amplitude with minimal effect on latency (Macdonald et al. 2013; Pajewski et al. 2007). In our experience, the adequacy of the MEP amplitude and stability of the wave morphology is a good indicator of adequate hemoregulation by the patient and is useful if the surgeon requests a decrease in mean arterial pressure to decrease blood loss. Decreases in blood flow can also occur during spinal derotation, tissue retraction, ligation of a perfusing artery, or with direct spinal cord injury from instrumentation or stretch. Decreased blood flow to the cord has less effect on subcortical responses compared with cortical responses.

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In addition, MEPs are more sensitive to ischemia than the SSEPs. In addition to blood flow, SSEPs increase in amplitude and latency with hematocrit 10–15% (Nagao et al. 1978; Pajewski et al. 2007). This is most likely due to decreased oxygen and decreased nutrient supply. In the animal model, hyercarbia, PaCO2 greater than 100 mmHG, produced decreased amplitude and increased latencies of SSEP and MEP. Hypocapnia has shown minimal effect on evoked response (Browning et al. 1992; Lotto et al. 2004). Temperature changes also effect SSEP and MEP. Studies have demonstrated that hypothermia decreases amplitude, increases latency, and increases stimulation threshold (Browning et al. 1992; Lotto et al. 2004). Below 25 C, SSEP and MEP disappear. These effects are believed to be due to slowed axonal and synaptic conduction as well as decreased release of neurotransmitters (Oro and Haghighi 1992). Hyperthermia decreases latency and decreases amplitude of MEP and has a biphasic effect on SSEP with an initial decrease in latency then increase with increasing temperature. At temperatures greater than 42 C, cortical SSEP disappear and spinal MEP deteriorate irreversibly at temperatures greater than 45 C (Oro and Haghighi 1992; Sakamoto et al. 2003; Yamane et al. 1992). Patient characteristics also influence the ability to monitor. Children under 6 years of age have an immature central nervous system, which can make MEP monitoring challenging although myelination is normally completed by 12 months. As well, patients with neurologic deficits from brain injury, as with CP, can make MEP monitoring difficult. MEPs are not recordable in some patients with desynchronization of corticospinal tracts (Sloan et al. 2008). Cerebral palsy is a nonprogressive brain injury due to a disorder of the brain cortex formation sustained during early childhood. Synaptic remodeling can involve upper motor and lower motor neurons. Evoked potential monitoring relies on synaptic transmission of electrical stimuli to provide data regarding the integrity of the spinal cord. Any physical parameter, which changes impulse conduction along an axon, may change the evoked potential

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waveform. At baseline, such patients have modulation of their brain, which may be mild or severe and can present challenges in the ability to provide neuromonitoring for these patients.

Clinical Application Patients with cerebral palsy have been successfully monitored intraoperatively with both SSEP and MEP. Previous studies have shown as many as 53–82% of CP patients studied had SSEP monitored whereas 35–63% of the same patients studied had their MEPs successfully monitored. The success of monitoring has been correlated to the severity of cerebral palsy (DiCindio et al. 2003; Ecker et al. 1996; Hammett et al. 2013). The anesthetic for a patient with CP undergoing a spinal fusion must be carefully tailored to maximize patient safety and allow measurement and stability of the neurologic waveforms to minimize negative outcomes. When using SSEP and MEP, the modality with the most restrictive requirement will dictate the choice of anesthetic. It is essential that the choice of anesthetic take into consideration the need for neuromonitoring, allowing for minimal anesthesia related influence. Hypothermia is the most common intra. It is essential that the choice of anesthetic take into consideration the need for neuromonitoring, allowing for minimal anesthesia-related influence. Hypothermia is the most common intra operative complication of CP patients, and to avoid a sudden drop in their temperature once anesthetized, we warm patients in the preoperative area with a forced air-warming device (Nolan et al. 2000; Pajewski et al. 2007; Wass et al. 2012). The patient is actively warmed throughout the preoperative and intraoperative period. Choice of induction of anesthesia may follow as in any other surgical procedures. It is common practice for us to proceed with an inhaled anesthetic using sevoflurane with oxygen and nitrous oxide because spasticity, anxiety, and preoperative fasting make these patients difficult intravenous (IV) access. As soon as the first peripheral IV is obtained, the nitrous and sevoflurane are shut off and TIVA using propofol is started. This is done because while inhaled anesthetics may be used up

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to 0.5% MAC for SSEP, the greater depressive effect on MEPs by inhaled anesthetics may affect the ability to successfully monitor patients with CP (Macdonald et al. 2013). A bolus dose of propofol, 1-2 mg/kg, is given along with a narcotic of choice to facilitate tracheal intubation. Soft bite blocks are placed bilaterally to reduce the risk of tongue laceration. After the trachea is secured, the neurophysiologist begins application of monitoring leads while working alongside the anesthesia team. SSEP and tceMEP are attempted in all patients including those with seizures (Salem et al. 2016; Schwartz et al. 2007). Our experience with these patients indicates that preoperative evaluation of brain MRI and the cognitive status of the patient, weight bearing capability, and in particular, their ability to interact with their environment, even at a rudimentary level, are very useful predictors that monitorable signals will be present. If still questionable, the neurophysiologist may use a modified set up to record motor evoked potentials prior to positioning the patient prone. The set up typically includes an upper extremity muscle group and a sampling of lower extremity muscle groups that typically show a robust response under anesthesia, comparing them, for example, with responses expected from an adolescent idiopathic scoliosis population. . Determination of the ability to obtain SSEP and/or MEP dictates the use of a TIVA with propofol and narcotic with oxygen and air combination. If the patient does have recordable evoked potentials, neuromuscular blockade, rocuronium 0.5 mg/kg, is administered once for exposure. [13] If the patient cannot be monitored, then an inhaled anesthetic, using isoflurane with a narcotic of choice is elected. Regardless of ability to neuromonitor the patient, we maintain systolic blood pressure greater than 50 mmHG and the hemoglobin greater than 8 g/dl (see ▶ Chap. 78, “Medical Evaluation for Preoperative Surgical Planning in the Child with Cerebral Palsy”). Good communication is critical as the inhalation agent needs to be turned off as soon as possible and neuromuscular blockade withheld until accurate interpretation of the supine baselines is achieved.

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Care of a patient with cerebral palsy involves a thorough understanding of patient disease. IONM is used intraoperatively primarily to avoid iatrogenic neural injury during spinal fusion surgery. It can also provide information regarding the electrophysiological profile of a patient, including the status of functional motor and somatosensory pathways. This requires a multidisciplinary approach and should be tailored to each patient. It is our position that all patients presenting to the operating room for scoliosis surgery should be monitored.

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Neurophysiological Monitoring. Clin Neurophysiol 124:2291–2316 Mahmoud M, Sadhasivam S, Salisbury S, Nick TG, Schnell B, Sestokas AK, Wiggins C, Samuels P, Kabalin T, Mcauliffe J (2010) Susceptibility of transcranial electric motor-evoked potentials to varying targeted blood levels of dexmedetomidine during spine surgery. Anesthesiology 112:1364–1373 Master DL, Son-Hing JP, Poe-Kochert C, Armstrong DG, Thompson GH (2011) Risk factors for major complications after surgery for neuromuscular scoliosis. Spine (Phila Pa 1976) 36:564–571 Merton PA, Morton HB (1980) Stimulation of the cerebral cortex in the intact human subject. Nature 285:227 Nagao S, Roccaforte P, Moody RA (1978) The effects of isovolemic hemodilution and reinfusion of packed erythrocytes on somatosensory and visual evoked potentials. J Surg Res 25:530–7 Nathan N, Tabaraud F, Lacroix F, Moulies D, Viviand X, Lansade A, Terrier G, Feiss P (2003) Influence of propofol concentrations on multipulse transcranial motor evoked potentials. Br J Anaesth 91:493–497 Nolan J, Chalkiadis GA, Low J, Olesch CA, Brown TC (2000) Anaesthesia and pain management in cerebral palsy. Anaesthesia 55:32–41 Nuwer MR, Dawson EG, Carlson LG, Kanim LE, Sherman JE (1995) Somatosensory evoked potential spinal cord monitoring reduces neurologic deficits after scoliosis surgery: results of a large multicenter survey. Electroencephalogr Clin Neurophysiol 96:6–11 Oro J, Haghighi SS (1992) Effects of altering core body temperature on somatosensory and motor evoked potentials in rats. Spine (Phila Pa 1976) 17:498–503 Padberg AM, Wilson-Holden TJ, Lenke LG, Bridwell KH (1998) Somatosensory- and motor-evoked potential monitoring without a wake-up test during idiopathic scoliosis surgery. An accepted standard of care. Spine (Phila Pa 1976) 23:1392–1400 Pajewski TN, Arlet V, Phillips LH (2007) Current approach on spinal cord monitoring: the point of view of the neurologist, the anesthesiologist and the spine surgeon. Eur Spine J 16(Suppl 2):S115–S129 Pechstein U, Nadstawek J, Zentner J, Schramm J (1998) Isoflurane plus nitrous oxide versus propofol for recording of motor evoked potentials after high frequency repetitive electrical stimulation. Electroencephalogr Clin Neurophysiol 108:175–181 Reames DL, Smith JS, Fu KM, Polly DW Jr, Ames CP, Berven SH, Perra JH, Glassman SD, Mccarthy RE, Knapp RD Jr, Heary R, Shaffrey CI, Scoliosis Research Society Morbidity and Mortality Committee (2011) Complications in the surgical treatment of 19,360 cases of pediatric scoliosis: a review of the Scoliosis Research Society morbidity and mortality database. Spine (Phila Pa 1976) 36:1484–1491 Sakamoto T, Kawaguchi M, Kakimoto M, Inoue S, Takahashi M, Furuya H (2003) The effect of hypothermia on myogenic motor-evoked potentials to electrical stimulation with a single pulse and a train of pulses

1812 under propofol/ketamine/fentanyl anesthesia in rabbits. Anesth Analg 96:1692–1697. table of contents Salem KM, Goodger L, Bowyer K, Shafafy M, Grevitt MP (2016) Does transcranial stimulation for motor evoked potentials (TcMEP) worsen seizures in epileptic patients following spinal deformity surgery? Eur Spine J 25:3044–3048 Sarwark J, Sarwahi V (2007) New strategies and decision making in the management of neuromuscular scoliosis. Orthop Clin North Am 38:485–496 Scheufler KM, Zentner J (2002) Motor-evoked potential facilitation during progressive cortical suppression by propofol. Anesth Analg 94:907–912. table of contents Schwartz DM, Auerbach JD, Dormans JP, Flynn J, Drummond DS, Bowe JA, Laufer S, Shah SA, Bowen JR, Pizzutillo PD, Jones KJ, Drummond DS (2007) Neurophysiological detection of impending spinal cord injury during scoliosis surgery. J Bone Joint Surg Am 89:2440–2449 Sloan T, Sloan H, Rogers J (2010) Nitrous oxide and isoflurane are synergistic with respect to amplitude and latency effects on sensory evoked potentials. J Clin Monit Comput 24:113–123 Sloan TB, Heyer EJ (2002) Anesthesia for intraoperative neurophysiologic monitoring of the spinal cord. J Clin Neurophysiol 19:430–443 Sloan TB, Janik D, Jameson L (2008) Multimodality monitoring of the central nervous system using motorevoked potentials. Curr Opin Anaesthesiol 21:560–564 Stecker MM (2012) A review of intraoperative monitoring for spinal surgery. Surg Neurol Int 3:S174–S187 Szelenyi A, Kothbauer KF, Deletis V (2007) Transcranial electric stimulation for intraoperative motor evoked

S. Dicindio et al. potential monitoring: Stimulation parameters and electrode montages. Clin Neurophysiol 118:1586–95 Tobias JD, Goble TJ, Bates G, Anderson JT, Hoernschemeyer DG (2008) Effects of dexmedetomidine on intraoperative motor and somatosensory evoked potential monitoring during spinal surgery in adolescents. Paediatr Anaesth 18:1082–1088 Toleikis JR, American Society of Neurophysiological Monitoring (2005) Intraoperative monitoring using somatosensory evoked potentials. A position statement by the American Society of Neurophysiological Monitoring. J Clin Monit Comput 19: 241–258 Ulkatan S, Jaramillo AM, Tellez MJ, Kim J, Deletis V, Seidel K (2017) Incidence of intraoperative seizures during motor evoked potential monitoring in a large cohort of patients undergoing different surgical procedures. J Neurosurg 126:12961302 Wass CT, Warner ME, Worrell GA, Castagno JA, Howe M, Kerber KA, Palzkill JM, Schroeder DR, Cascino GD (2012) Effect of general anesthesia in patients with cerebral palsy at the turn of the new millennium: a population-based study evaluating perioperative outcome and brief overview of anesthetic implications of this coexisting disease. J Child Neurol 27:859–866 Yamane T, Tateishi A, Cho S, Manabe S, Yamanashi M, Dezawa A, Yasukouchi H, Ishioka K (1992) The effects of hyperthermia on the spinal cord. Spine (Phila Pa 1976) 17:1386–1391 Zentner J, Albrecht T, Heuser D (1992) Influence of halothane, enflurane, and isoflurane on motor evoked potentials. Neurosurgery 31:298–305

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1814 Treatment: Cervical Spine Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Extensor Posturing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Occipital Subluxation, Posturing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Atlantoaxial Instability and Subluxation with or without Os Odontoideum . . . . . . . . . . Congenital Atlantoaxial Displacement with Os Odontiodeum . . . . . . . . . . . . . . . . . . . . . . . . Cervical Spine Spondylosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Inability to Hold up the Head . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Severe Upper Thoracic Kyphosis with Lower Cervical Lordosis . . . . . . . . . . . . . . . . . . . . .

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Complications of Cervical Spinal Deformity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1819 Cervicothoracic Junction Kyphosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1819 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1820 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1820

Abstract

Cervical spine problems in children with cerebral palsy are uncommon. Some children with severe movement disorder and those with extensor posturing, however, may develop occipital cervical instability. Atlantoaxial instability can also occur in patients with torsional dystonia. Cervical extensor contractures occur in those children with severe extensor posturing. Symptoms of cervical problems are usually pain or occasionally related to spinal cord compression. Spinal cord compression

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_120

symptoms cause either increase spasticity in the legs or sudden loss of spasticity and decreased movement in the lower extremities. Mid-cervical spinal stenosis and degenerative arthritis occur in adults with movement disorders especially those with athetosis. Lower cervical spine problems occur primarily as a residual of high kyphosis. The treatment for this drop off kyphosis requires surgical correction if it is symptomatic. Other spinal problems that occur after posterior spinal fusion are relatively uncommon although on rare occasions children will develop pseudarthrosis which become symptomatic and require repair. When the spinal instrumentation is not strong enough or solidly connected, deformities may occur around the instrumentation. Treatments 1813

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of these complex problems are discussed in this chapter. Keywords

Cerebral Palsy · Cervical spine · Kyphosis · Spinal stenosis · Pseudoarthosis

Introduction Children with cerebral palsy have frequent spinal deformities primarily thoracolumbar scoliosis or kyphosis. Children who have severe movement disorder either dystonia or athetosis are also at risk for cervical spine instability (Koop et al. 1984). Although this is much less common in childhood, cervical instability is more common in adulthood following degenerative changes due to constant movement from athetosis and dystonia is a well-recognized syndrome (Fuji et al. 1987). Cervical thoracic junctional kyphosis following posterior spinal fusion is another relatively common deformity; if the deformity is symptomatic, revision surgery is required to correct the kyphosis. Other revision surgery may be required for patients who did not have the initial deformity adequately corrected in the thoracocervical spine or the instrumentation which was used was insufficient to maintain the deformity in corrected position. The goal of this chapter is to review deformities related to the cervical spine and those special spinal deformities which may require a revision after the initial procedure has been performed.

Treatment: Cervical Spine Problems Extensor Posturing Extensor posturing at the cervical spine is almost always associated with generalized extensor posturing, either with generalized dystonia or opisthotonic posturing. In some children, this is definitely a dystonia with major torsional elements of the head and neck. These children have no contractures, and when they are broken out of the extensor posturing and

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during sleep will lie in normal positioning, often sleeping in a flexed position. The etiology of the neurologic deficit in children with relatively pure dystonia is usually a chemical insult such as glutaric aciduria acidosis affecting primarily the basal ganglia. Other children tend to remain in the extended position most of the time and develop neck and back extension contractures (Fig. 1). In the typical spastic opisthotonic posturing, the most common etiology of the neurologic deficit tends to be from severe anoxia, such as near drowning, or severe residuals of septic meningitis, which produces severe diffuse brain injury. Treatment of both types of extensor posturing should first focus on having a properly adjusted wheelchair with all caretakers instructed on proper seating of the children. These children should be placed in 90 of hip flexion and knee flexion with their necks in a neutral position. Management of the movement disorder should also consider insertion of an intrathecal baclofen pump, bringing the catheter high into the cervical spine around the C3 to C5 level (Albright 1996). If contractures preclude this in spastic children, muscle release or lengthening of the hip extensors should be performed. Occasionally, lengthening of the knee extensors is required. This type of muscle lengthening is rarely needed in purely dystonic patients because they do not have muscle contractures. Botulinum toxin injection into the neck extensor muscles also provides excellent relief in spastic and dystonic children; however, these usually lead to major disappointment in families. The first injection gives an excellent result, the second injection usually gives a good result, and then by the fourth injection 1–1.5 years later, there is no longer any benefit. The use of intrathecal baclofen has been reported to work in children with extensor posturing (Albright 1996). In general, a very high dose is required and the catheter should be placed at least to the upper cervical spine level. The outcome of intrathecal baclofen use is not as dramatic as it is with the more generalized lower extremity spasticity. It has also been our experience that relatively pure dystonia has a better outcome than the opisthotonic spastic posturing pattern. Another treatment for

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Fig. 1 This 10-year-old boy developed severe scoliosis and increased respiratory problems. He underwent a Unit rod instrumentation and fusion. During the postoperative period, the respiratory condition did not improve, making extubation difficult. He eventually required a tracheostomy but continued with bradycardia, and an MRI of the brain, which demonstrated compression of the upper cervical

spinal cord by a rotatory subluxation of C1 on C2, was performed (a). He had opisthotonic posturing for many years and as the scoliosis got worse, this too seemed to increase (b, c). We have seen three patients with a similar history, and we now workup all children with any evidence of neurologic change who also have long-term severe opisthotonic posturing

children with relatively pure dystonia that has been promoted in some centers is pallidotomy (Lin et al. 1999). We have no experience with pallidotomy for this patient population; however, personal communication with physicians experienced with pallidotomy report excellent results in

some children and very minor benefit in others. The latest development is to insert deep brain stimulator, which has been reported to have positive effects (Bhanpuri et al. 2014; Koy et al. 2014). The effects are somewhat unpredictable at this time.

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Fig. 2 Cervical spine instability is not common in children with CP; however, it does occur. This 10-year-old boy had progressive loss of hand function, stance ability, and respiratory problems. A lateral spine radiograph showed anterior subluxation of C1 on C2 (a). This was followed up with an MRI scan that showed substantial compression of

the spinal cord in the area of the instability (b). The subluxation could not be reduced; therefore, he had a posterior C1 laminectomy followed by posterior instrumentation and fusion (c). By 2 months following surgery, the required deficits had recovered

Occipital Subluxation, Posturing

airway, and this may be a secondary posturing response (Tsirikos et al. 2003). This posturing response may also be secondary to symptomatic gastroesophageal reflux, in which case it is usually intermittent and associated with discomfort.

A small group of children have a torticollis pattern of spasticity, which may be part of a scoliosis pattern or a residual of a spastic opisthotonic posturing in which the extension has diminished (Fig. 1). Occasionally, this posturing may lead to rotatory subluxation. The residuals of these posturing events may lead to neurologic deficits (Amess et al. 1998; Haslam 1975; Thompson et al. 1985). We had one case in which increasing respiratory problems prompted us to do a workup that found a significant myelopathy from a compression (from a rotatory subluxation at C1–C2). Children who have cervical posturing deformities and have a change in their neurologic function, or a dramatic change in their breathing, should have an MRI scan of the cervical spine to make sure that a cervical myelopathy is not causing the neurologic change. If myelopathy is demonstrated, fusion and stabilization are usually indicated (Fig. 2). We have had one child who was found dead in bed after myelopathy was identified and surgical stabilization was recommended. If the primary complaint is respiratory difficulty, children may also be posturing to open an obstructed

Atlantoaxial Instability and Subluxation with or without Os Odontoideum Atlantoaxial instability may develop in the child with spastic cerebral palsy but usually occurs in patients with dystonia or athetosis. It is a rare complication often very difficult to diagnose. The most common presenting symptoms are secondary to obstructive respiratory symptoms or change in movement patterns or spasticity (Behari et al. 2002; Juhl and Seerup 1983). Patient may present with a change in gait or a complaint of losing gait function (Hojo et al. 1994a). Patients may also present with an acute change in head posture which is uncomfortable or painful. This posture is usually the cock robin head position typical in rotatory atlantoaxial subluxation with head tilt to the side and head rotation toward the

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Fig. 3 A 9-year-old boy, GMFCS V with severe movement disorder presented with increasing neck pain. The radiologic workup included radiographs (a) C1-2 rotatory subluxation with anterior subluxation. The displacement

was reduced in the operating room and he was fixed in place with transarticular screws and autogenous ileac crest bone graft (b). A solid fusion developed and he has been followed for 12 years with no further neck pain

opposite side of the head tilt combined with mild cervical flexion. There may or may not be an associate traumatic event. This acquired atlantoaxial instability is easy to recognize in normal individuals; however, in patients with CP with significant movement disorder it can be very difficult to recognize as this posture and change in movement may be similar to their response to generalized pain. Early recognition and obtaining the correct imaging starting with standard radiographs is important. Almost always a CT scan is required to obtain the appropriate imaging usually under general anesthesia. This allows the head to be rotated side to side which will most clearly demonstrate the fixed nature of the subluxation. Although the acquired atlantoaxial subluxation is rare, it is likely largely unrecognized and may be more common. The treatment of choice is C1–2 fusion, since the patients who develop this have severe movement disorder and would not tolerate long-term orthotic management. Since this also can cause neural impingement and respiratory suppression and obstruction, the fixation should be a relative emergency (Akpolat et al. 2015). We have had one patient who died while waiting for his scheduled surgery (Tsirikos et al.

2003). It is not clear which is the best fixation (Sumi et al. 1997), although we favor C1–2 transarticular screw fixation when then lateral masses are large enough (Fig. 3). We still would use a halovest immobilization if the patient has severe dystonia or athetosis. In small number of children, the long-term outcome has been excellent as demonstrated by this case.

Congenital Atlantoaxial Displacement with Os Odontiodeum Some children may have congenital malformations such as os odontoideum which cause early cervical instability. There is some continued controversy if the os odontoideum is congenitial or acquired in children with severe dyskinetic movement disorders (Trabacca et al. 2011). This early instability may cause mylopathy leading to spasticity. If this occurs in a child with other risk factors such as prematurity or periventricular leukomalacia, the spasticity maybe presumed to have a recognized cause (Thompson et al. 1985). It is important to remember that one condition is not immunity to other conditions and both may be present (Akpolat et al. 2015; Kim et al. 2013). Careful clinical

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examination should include cervical spine motion and observation of head posture. If the child has torticollis or limited cervical motion, or any neck pain a further workup of the cervical spine is required. Often these head changes are ascribed to some other cause especially when they come and go (Yoshimura et al. 2014). They should not be ignored. When the instability is identified, the usual treat is stabilization and fusion of the unstable segments.

decompression and stabilization, relatively high rates of recurrent deformity and neurologic symptoms may occur (Azuma et al. 2002). One problem in stabilizing the cervical spine in adults is the strength of the continued movement disorder. This has led to high rate of hardware failure (Nakashima et al. 2012; Kim et al. 2014). This problem occurs exclusively in adulthood, as we have never seen the problem in the pediatric population.

Cervical Spine Spondylosis

Inability to Hold up the Head

Degenerative arthritis with disk degeneration and herniation are common in middle-aged and older individuals with athetoid pattern CP. Multiple reports have documented this process, (Mallakh et al. 1989; Epstein 1999; Hanakita et al. 1989; Harada et al. 1996; Hojo et al. 1994b; Racette et al. 1998), which is due to hypermobility from the athetoid movement disorder (Amess et al. 1998). Although acute episodes of pain often respond to conservative treatment, if neurologic deficit is present, localized decompression and fusion are often needed (Racette et al. 1998; Nishihara et al. 1984). Following the initial

Severe weakness due to hypotonia so that the head cannot be held upright in children with CP may be caused in part by severe thoracic kyphosis. Correction of the kyphosis usually leads to dramatic improvement in the ability to hold the head upright or for wheelchair seating to be adjusted so the head can be held in a normal position (Fig. 4). There are children with severe weakness, mostly children with muscular dystrophy and spinal muscular atrophy, for whom this is much more of a problem and in whom consideration of inclusion of the cervical spine in the posterior fusion is a reasonable option.

Fig. 4 Severe kyphosis makes head control and seating very difficult. This 11-year-old boy had lost complete head control as his kyphosis became more severe (a). With correction of the kyphosis, he developed good head control

in sitting as early as 1 week after the surgery (b). Correction of the kyphosis places the head in a mechanically more advantageous position, making head control easier

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Fig. 5 This 15-year-old boy with quadriplegic involvement had severe kyphosis corrected, which placed his head into much more extension (a). Because he had a fixed cervical lordosis that was not recognized preoperatively, this neck extension was now fixed and he could no longer

look forward. This boy required a surgical release of the cervical lordosis followed by orthotic control (b). This case illustrates the importance of examining the mobility of the cervical spine before correcting kyphosis

In adults, this flexed neck positon may become fixed and in rare cases requires surgical correction (Ogihara and Kunogi 2015; Singh et al. 2008). We have never seen this in children or adolescents.

indicated. Rarely, if children are unable to flex sufficiently at the cervical spine after correction of the kyphosis, a delayed paraspinal muscle lengthening and removal of the posterior nuchal ligament may be required.

Severe Upper Thoracic Kyphosis with Lower Cervical Lordosis Some children with severe thoracic kyphosis will make all efforts to hold their heads upright so they can see forward, and as a consequence, develop significant cervical lordosis or extension contractures. Often, these individuals with thoracic kyphosis sit in wheelchairs for prolonged periods. When the thoracic kyphosis is corrected without the cervical extension contracture being recognized, the children will only be able to gaze at the ceiling when they are sitting upright (Fig. 5). Checking the range of motion of the cervical spine before correcting thoracic kyphosis is important. If a fixed cervical lordosis is present, soft-tissue release of the cervical extensor muscles may be

Complications of Cervical Spinal Deformity Cervicothoracic Junction Kyphosis Children with CP who develop a spinal deformity that includes thoracic kyphosis are at risk of developing junction drop off kyphosis at the superior aspect of the spinal fusion (Sink et al. 2003). The two risk factors for developing this deformity are stopping the fusion too short or having failure of the upper aspect of the fixation. The upper fusion level for children with CP should in almost all cases be to at least T2. When the thoracic kyphosis extends high to the upper thoracic spine, the fusion should extend to C7. These

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Fig. 6 A 16-year-old boy, GMFCS III, had a posterior spinal fusion with a unit rod and sublaminar wires. He was full adult size with a relatively high kyphosis. The instrumentation was a little short with wires under tension to assist in correcting the upper kyphosis. The wires fractured before a fusion occurred and a drop off kyphosis developed

drop-off curves may cause a significant problem by making it hard for the child to hold up their head and require revision (Lonstein et al. 2012). A revision of the drop off curves usually requires using pedicle screws and often extending to C6 level. Other fixation such as the polyesters bands may be considered (Desai et al. 2015). In our experience, the most common cause of junctional kyphosis is failure of the wire fixation especially in adult-sized patients with high thoracic kyphosis (Sink et al. 2003). When this occurs and is symptomatic, it requires correction best done with pedicle screws (Fig. 6).

Cross-References ▶ Cerebral Palsy Spinal Deformity: Etiology, Natural History, and Nonoperative Management ▶ Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy

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which was painful and made it hard to hold his head up (a). This was repaired with fusion to C6, using pedicle screws (b). Sublaminar wires for high kyphosis in very large patients are likely not strong enough, and stronger fixation such as pedicle screws should be considered at the initial fixation

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evoked potentials may relate to clinical benefit in childhood dystonia. Brain Stimul 7:718–726 Desai SK, Sayama C, Vener D, Brayton A, Briceno V, Luerssen TG, Jea A (2015) The feasibility and safety of using sublaminar polyester bands in hybrid spinal constructs in children and transitional adults for neuromuscular scoliosis. J Neurosurg Pediatr 15:328–337 Epstein NE (1999) Circumferential cervical surgery for spondylostenosis with kyphosis in two patients with athetoid cerebral palsy. Surg Neurol 52:339–344 Fuji T, Yonenobu K, Fujiwara K, Yamashita K, Ebara S, Ono K, Okada K (1987) Cervical radiculopathy or myelopathy secondary to athetoid cerebral palsy. J Bone Joint Surg Am 69:815–821 Hanakita J, Suwa H, Nagayasu S, Nishi S, Ohta F, Sakaida H (1989) Surgical treatment of cervical spondylotic radiculomyelopathy with abnormal involuntary neck movements, report of three cases. Neurol Med Chir (Tokyo) 29:1132–1136 Harada T, Ebara S, Anwar MM (1996) The cervical spine in athetoid cerebral palsy, a radiological study of 180 patients. J Bone Joint Surg Br 78:613–619 Haslam RHA (1975) Progressive cerebral palsy’ or spinal cord tumor? Two cases of mistaken identity. Dev Med Child Neurol 17:232–237 Hojo M, Nakahara I, Tanaka M, Oda Y, Kikuchi H (1994a) Surgical treatment of atlanto-axial dislocation in a patient of athetoid cerebral palsy. No Shinkei Geka 22:887–891 Hojo M, Nakahara I, Tanaka M, Oda Y, Kikuchi H (1994b) Surgical treatment of atlantoaxial dislocation in a patient of athetoid cerebral palsy. No Shinkei Geka 22:887–891 Juhl M, Seerup KK (1983) Os odontoideum. A cause of atlanto-axial instability. Acta Orthop Scand 54:113–118 Kim JH, Kim JH, Jang SY, Kong MH (2013) Combined chronic Occipito-atlantal and Atlanto-axial rotator fixation with cerebral palsy. Korean J Spine 10:192–194 Kim KN, Ahn PG, Ryu MJ, Shin DA, Yi S, Yoon DH, Ha Y (2014) Long-term surgical outcomes of cervical myelopathy with athetoid cerebral palsy. Eur Spine J 23:1464–1471 Koop SE, Winter RB, Lonstein JE (1984) The surgical treatment of instability of the upper part of the cervical spine in children and adolescents. J Bone Joint Surg Am 66:403–411 Koy A, Pauls KA, Flossdorf P, Becker J, Schonau E, Maarouf M, Liebig T, Fricke O, Fink GR, Timmermann L (2014) Young adults with dyskinetic cerebral palsy improve subjectively on pallidal stimulation, but not in formal dystonia, gait, speech and swallowing testing. Eur Neurol 72:340–348 Lin JJ, Lin GY, Shih C, Lin SZ, Chang DC, Lee CC (1999) Benefit of bilateral pallidotomy in the treatment of generalized dystonia, case report. J Neurosurg 90:974–976

1821 Lonstein JE, Koop SE, Novachek TF, Perra JH (2012) Results and complications after spinal fusion for neuromuscular scoliosis in cerebral palsy and static encephalopathy using Luque Galveston instrumentation: experience in 93 patients. Spine (Phila Pa 1976) 37:583–591 Mallakh RS, Rao K, Barwick M (1989) Cervical myelopathy secondary to movement disorders: case report. Neurosurgery 24:902–905 Nakashima H, Yukawa Y, Imagama S, Kanemura T, Kamiya M, Yanase M, Ito K, Machino M, Yoshida G, Ishikawa Y, Matsuyama Y, Ishiguro N, Kato F (2012) Complications of cervical pedicle screw fixation for nontraumatic lesions: a multicenter study of 84 patients. J Neurosurg Spine 16:238–247 Nishihara N, Tanabe G, Nakahara S, Imai T, Murakawa H (1984) Surgical treatment of cervical spondylotic myelopathy complicating athetoid cerebral palsy. J Bone Joint Surg Br 66:504–508 Ogihara S, Kunogi J (2015) Single-stage anterior and posterior fusion surgery for correction of cervical kyphotic deformity using intervertebral cages and cervical lateral mass screws: postoperative changes in total spine sagittal alignment in three cases with a minimum followup of five years. Neurol Med Chir (Tokyo) 55:599–604 Racette BA, Lauryssen C, Perlmutter JS (1998) Preoperative treatment with botulinum toxin to facilitate cervical fusion in dystonic cerebral palsy, report of two cases. J Neurosurg 88:328–330 Singh K, Samartzis D, Somera AL, An HS (2008) Cervical kyphosis and thoracic lordoscoliosis in a patient with cerebral palsy. Orthopedics 31:276 Sink EL, Newton PO, Mubarak SJ, Wenger DR (2003) Maintenance of sagittal plane alignment after surgical correction of spinal deformity in patients with cerebral palsy. Spine (Phila Pa 1976) 28:1396–1403 Sumi M, Kataoka O, Ikeda M, Sawamura S, Uno K, Siba R (1997) Atlantoaxial dislocation. A follow-up study of surgical results. Spine (Phila Pa 1976) 22:759–763, discussion 63–64 Thompson GH, Likavec MJ, Archibald I, Rush T (1985) Atlantoaxial rotatory subluxation, congenital absence of the posterior arch of the atlas, and cerebral palsy: an unusual triad. J Pediatr Orthop 5:232–235 Trabacca A, Dicuonzo F, Gennaro L, Palma M, Cacudi M, Losito L, De Rinaldis M (2011) Os odontoideum as a rare but possible complication in children with dyskinetic cerebral palsy: a clinical and neuroradiologic study. J Child Neurol 26:1021–1025 Tsirikos AI, Chang WN, Shah SA, Miller F (2003) Acquired atlantoaxial instability in children with spastic cerebral palsy. J Pediatr Orthop 23:335–341 Yoshimura A, Kibe T, Yokochi K (2014) Cervical myelopathy associated with os odontoideum after botulinum toxin treatment in a patient with cerebral palsy. No To Hattatsu 46:307–310

Pelvic Alignment and Spondylolisthesis in Children with Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1824 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pelvic Malalignment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pelvic Obliquity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Anterior Pelvic Tilt . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pelvis Rotational Malalignment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Spondylolisthesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1828 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1828 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1832 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1832

Abstract

Children with cerebral palsy may have malalignments related to their lumbar spine and pelvis. Pelvic obliquity is a common problem having two primary causes. Suprapelvic pelvic obliquity occurs due to scoliosis, and infrapelvic pelvic obliquity is due to asymmetric contractures of the hips. The treatment of pelvic obliquity requires first to define the etiology; if it is suprapelvic, correct the scoliosis, and if it is infrapelvic, correct the hip contractures. Anterior pelvic tilt is due to hyperlordosis or hip flexion contracture. Posterior

pelvic tilt is due to lumbar kyphosis or contractures of the hip extensors. Abnormal pelvic rotation maybe due to asymmetric rotation at the hips or to abnormal fixed rotation through spine. Treatment to correct abnormal pelvic alignment requires identifying the etiology and then treating the primary cause. Lumbar spondylolysis and spondylolisthesis also occur in children with cerebral palsy who are ambulatory. The highest incidence is in ambulatory children who have had dorsal rhizotomy. The primary initial treatment for painful defects is immobilization, and if this is not successful, surgical stabilization is required.

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_121

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Keywords

Cerebral palsy · Pelvic obliquity · Anterior pelvic tilt · Posterior pelvic title · Rotated pelvis · Spondylolysis · Spondylolisthesis · Rhizotomy

Introduction Spinal deformities are very common in children with cerebral palsy. Since the spine ends at the pelvis, deformities which involve the pelvis are also very common. The scoliosis may extend into the pelvis causing pelvic obliquity and making seating difficult. Correction of pelvic obliquity, which is due to the scoliosis, requires primarily addressing the scoliosis. Deformities involving the pelvis can also include anterior pelvic tilt or posterior pelvic tilt which also impacts seating and can make standing and walking difficult in some children. The pelvis can develop malrotated positions in which its transverse plane alignment with the shoulders is abnormal. This will cause difficulties when the child is sitting, because the shoulders and pelvis are not in the same plane often giving the impression that the child is either sitting sideways or that one leg is longer than the other. Another deformity at the level of the pelvis is primarily related to the low lumbar spine causing spondylolysis or spondylolisthesis. It is a much more common problem in children who have had dorsal rhizotomy, but ambulatory children may also develop spondylolisthesis. The goal of this chapter will be to review the pelvic malalignments and abnormalities in the low lumbar spine.

Natural History and Pathophysiology Pelvic Malalignment Problems of the pelvis fall between the hips and spine; however, we prefer to think of the pelvis as part of the spinal segment because the only direct way of approaching pelvic malalignment is in considering the pelvis as the caudal end of the spine. This thinking

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allows correcting pelvic malalignments during spinal instrumentation.

Pelvic Obliquity The definition and measurement of pelvic obliquity depend on whether children are standing or sitting. Often, pelvic obliquity has been defined based on the horizontal plane of the space in which the children are placed. This definition works well for flexible pelvic obliquity, especially in standing children in whom the radiograph is made with them standing upright or the pelvis is measured from markers placed on the anterosuperior iliac crest during gait analysis. However, the definition does not work well for children who are dependent sitters and have a combination of flexible and fixed pelvic obliquity. The pelvisto-room horizontal plane may be much more reflective of how the technician seated a child for the radiograph than any real measure of pelvic obliquity as defined by the pelvis’s position relative to the spine and trunk. In these children, it is better to measure the obliquity of the pelvis from a line drawn between the centers of T1 to L5 as a straight vertical line. Measuring the alteration of the pelvis from a 90 angle to this vertical line is much more representative of a true measure of children’s functional pelvic obliquity (Fig. 1).

Etiology The cause of abnormal pelvic obliquity is divided into suprapelvic and infrapelvic etiologies. The suprapelvic pelvic obliquity is caused by an extension of the scoliosis into the pelvis. The degree of flexibility of the pelvic obliquity is determined by the flexibility of the scoliosis. Fixed infrapelvic pelvic obliquity is caused by an asymmetric contracture of the hip abductors in sitting children and in some standing children. This obliquity is a part of the windblown hip deformity discussed in more detail in (▶ Chap. 136, “Windblown Hip Deformity and Hip Contractures in Cerebral Palsy.”) This fixed deformity is caused by some combination of hip joint and muscle contractures with hip adduction on the one side and hip abduction on the other

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The flexible deformity follows its specific etiology completely.

Fig. 1 The best way to measure pelvic obliquity is by drawing a line across the iliac crest and then drawing a perpendicular line at the midline of the iliac crest. Next draw a line from T1 to the crossing point pelvic lines. This measures pelvic obliquity relative to the body posture, and the goal from surgical correction should be to have this corrected to less than 5

side. There is also a flexible pelvic obliquity seen in ambulatory children and best measured on gait analysis. This deformity may be secondary to functional or actual limb length inequality or severe asymmetric weakness of the hip abductors. It may also be combined with a mild fixed deformity secondary to contractures.

Natural History The natural history of pelvic obliquity tends to follow the course of the primary etiology. Therefore, if the etiology of the pelvic obliquity is scoliosis that continues to become increasingly more severe, the pelvic obliquity also increases until the ilium rides inside the chest, often causing significant pain from the formation of bursitis between the ribs and the ilium. The infrapelvic pelvic obliquity tends to follow the contractures, which often stabilize after growth is completed.

Treatment Treatment of pelvic obliquity is based on diagnosing the specific etiology causing the obliquity. If the cause is a suprapelvic pelvic obliquity from scoliosis, then correcting the scoliosis is required. If the primary cause is infrapelvic, correcting the fixed deformities at the hip level is required. If the cause is limb length inequality, the exact reason for the limb length inequality needs to be determined and then addressed. If the problem is muscle weakness or hip joint instability, these have to be evaluated as the possible treatments. If a definite primary source can be identified, the treatment is usually very clear cut. However, there are often two causes that are both causative and often additive. A frequent combination is children who have a suprapelvic cause from scoliosis and an infrapelvic cause from a windblown hip secondary to spastic hip disease. In these situations, carefully assessing the stiffness of the spine is important, as some younger children will have a suprapelvic aspect only as a secondary adaptive deformity for what is primarily an infrapelvic etiology. If the spinal deformity is very flexible, then the hip should be considered the primary etiology and should be addressed first with the goal of waiting several years to correct the spine, allowing further growth (Case 1). If this assessment is correct, the scoliosis will partially correct after the hips have been corrected, and children will do well in the short term. However, if this judgment was in error, then the pelvis will stay very oblique, and there will be problems seating children that require the scoliosis to be corrected in the short term, usually in 4–6 months after the hip surgery. If the evaluation determines that the hip and spine are equally involved, or the spine is the primary etiology, then the spine should be corrected first with the hips corrected 4–6 months later. Earlier hip surgery increases the risk of severe heterotopic ossification. By correcting the suprapelvic cause of the pelvic obliquity, the pelvis becomes a stable base in which the hip surgery will be accomplished more easily and more successfully.

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Seating Adjustment The outcome of pelvic obliquity treatment should be an 80–90% correction of all pelvic obliquity, and as a consequence, there should never be a need to make seating accommodations for pelvic obliquity after treatment. Children who have uncorrected pelvic obliquity only rarely have problems with skin breakdown because of their normal sensation. They do, however, develop discomfort in seating. The best seating adaptations are the use of closed-cell foams, in which the seat is partially built up to accommodate the pelvic obliquity. If an attempt is made to completely accommodate children with some flexibility, the pelvic obliquity will often just get worse, which is not the goal of the seating adaptations. Surgical Correction of Pelvic Obliquity Surgical correction of pelvic obliquity is always a part of correction of the scoliosis when it is suprapelvic pelvic obliquity. Therefore the discussion of surgical correction will occur in (▶ Chap. 118, “Surgical Treatment of Scoliosis Due to Cerebral Palsy.”) If the etiology is infrapelvic, the discussion of surgical correction will be in (▶ Chap. 136, “Windblown Hip Deformity and Hip Contractures in Cerebral Palsy.”)

Anterior Pelvic Tilt Increased anterior pelvic tilt is common in children with CP and is present in both ambulatory and nonambulatory individuals. Measurements using radiographs and measuring the sacrofemoral angle, which measures the angle between the L5 and S1 disk and the femur, are seldom used. The most common measurement of anterior pelvic tilt is from gait analysis. This measurement uses the angle formed by the anterosuperior iliac crest to the posterosuperior iliac crest relative to room horizontal plane.

Etiology Just as with pelvic obliquity, there are suprapelvic and infrapelvic causes of the abnormal pelvic tilt. The most common cause of increased anterior pelvic tilt is fixed increase in lumbar lordosis

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(Case 2). Another suprapelvic cause is severe abdominal muscle weakness, which is less common in children with CP than in some other neurologic diseases. Fixed posterior pelvic tilt is rare in children with CP; however, it may be due to decreased lumbar lordosis or lumbar kyphosis that is fixed. The infrapelvic cause of fixed increased anterior pelvic tilt is hip flexion contracture, and the flexible increased anterior pelvic tilt may result from either spastic hip flexors or weakness of the hip extensors. The infrapelvic cause of the posterior pelvic tilt is primarily contracted hip extensors or, in the worst case scenario, the type 1 anterior hip dislocation (▶ Chap. 133, “Anterior Dislocation of the Hip in Cerebral Palsy”). Flexible posterior pelvic tilt is most commonly due to spastic and contracted hamstrings, causing the posterior pelvic tilt in seating.

Natural History The natural history very much follows the specific cause. If the cause worsens, so does the anterior pelvic tilt, which may get very severe to the point of causing pain at the place where the anterosuperior iliac spine is in contact with the anterior thigh. In an insensate child, this may cause a decubitus ulcer, although, in sensate children with CP, pain develops so that they will not tolerate sitting. In most severe cases of posterior pelvic tilt, adaptive compensatory thoracolumbar kyphosis may develop and become a fixed deformity in itself. A common example of this is the child who is primarily sitting with severe lumbar lordosis without pain; however during growth the pain worsens, and all of a sudden, the pain is so severe the adolescent has only limited sitting tolerance. In our experience the only treatment of this is spinal fusion reducing the anterior pelvic tilt by decreasing the lumbar lordosis and fusing the spine to the pelvis. Treatment The treatment of abnormal pelvic tilt requires a clear definition of the exact etiology. If the cause is a suprapelvic anterior pelvic tilt caused by increased lumbar lordosis, the lordosis will need to be corrected if the goal is to improve the

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anterior pelvic tilt. If the problem is an infrapelvic cause, the specific etiology should also be corrected. A very common cause of posterior pelvic tilt in a child with GMFCS IV or V function during sitting in childhood is the spastic or contracted hamstring. These spastic, contracted hamstrings respond very well to lengthening or to seating children with increased knee flexion, which inactivates the hamstring.

Pelvis Rotational Malalignment Rotational malalignment relative to the rest of the trunk occurs in two situations. It occurs secondary to the scoliosis in which the pelvis rotates anteriorly on the elevated side in sitting. Pelvic rotation also has an infrapelvic cause due to asymmetric hip rotation in which the hip that is internally rotated causes the ipsilateral side of the pelvis to rotate posteriorly. These malrotations are not often noticed by families or children as primary problems, but in the suprapelvic cause commonly seen in GMFCS IVand V, the family usually complains of the child sitting with a long leg on the side that is rotated forward. For children who walk and have an infrapelvic rotational problem, complaints tend to be directed more at intoeing. Treatment for the suprapelvic cause is by correction of the scoliosis, whereas correction of the infrapelvic cause requires gait analysis and often unilateral femoral derotation.

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2009). From our experience, spondylolysis and spondylolisthesis probably occur more frequently in athetosis, especially in individuals who are so severe that they can barely ambulate. One study of 61 patients found 41 had significant back pain and 30 patients had radiographs, of which 28 had clear bone defects (Sakai et al. 2006). As noted in the section on dorsal rhizotomy, there is definitely a well-defined increase in the incidence of both spondylolysis and spondylolisthesis in individuals who had multiple-level lumbar laminectomies and who are ambulatory with diplegia (Hennrikus et al. 1993; Peter et al. 1990, 1993; Peter and Arens 1993; Langerak et al. 2009; Li et al. 2008; Spiegel et al. 2004). There is some uncertainty if laminectomy has higher risk than laminoplasty; however in one small series, there was no difference as both had high incidence (Johnson et al. 2004).

Spondylolisthesis

Natural History The natural history for most spondylolysis and spondylolisthesis is asymptomatic development, and it is usually an incidental finding on a spine radiograph done for another reason. These children definitely follow a course similar to a lesion seen in a child without CP and primarily remain asymptomatic. Another group of children present with low back pain of an acute nature that lasts more than several days. A workup usually defines a spondylolysis, and conservative treatment provides symptomatic relief. Again, the natural history in this group is very similar to similar-aged normal individuals, although to date there is no reported series documenting the natural history.

The etiology of lumbar spondylolysis and spondylolisthesis is due to the stress of upright standing and probably is made worse with hyperlordosis. Thus, spondylolysis and spondylolisthesis are rarely seen in individual who are nonstanding quadriplegia GMFCS IV and V (Rosenberg et al. 1981); however, there is one case report in an adolescent with athetosis (Carl et al. 2007). Pars defects are more common in ambulatory diplegia, although it is not clear that the incidence is higher than in normal similaraged individuals (Hennrikus et al. 1993; Murphy

Treatment If the discovery of the spondylolysis and spondylolisthesis is an incidental finding on a radiograph performed for unrelated reasons, such as minor trauma or abdominal radiographs, no treatment or follow-up is indicated (Hennrikus et al. 1993). For children who present with an acute episode of low back pain that has lasted for more than 1 week, a workup is indicated. If the children’s neurologic examination is unchanged from previous examinations, then a lumbar spine radiograph, including oblique

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views, should be obtained. If these radiographs show a spondylolysis or spondylolisthesis, treatment with a lumbar sacral flexion orthosis is indicated. If the radiographs are normal, then a technetium bone scan should be ordered. If the spondylolysis is demonstrated on the bone scan, treatment is indicated. The primary treatment for the acute pain is fitting children with a lumbar sacral flexion orthosis (LSO), which decreases the lumbar lordosis. This orthotic usually makes children much more comfortable immediately; however, a short course of an anti-inflammatory and activity modification may also be added as indicated by symptoms. The orthosis is prescribed to be worn during all waking hours except for bathing. The LSO is used for 3 months and then gradually weaned off over a 1-month period. If the children still have pain, or the pain recurs, the orthosis is again worn for another 3-month period. In almost all children, the pain resolves, and only very rarely is there any progression of the spondylolisthesis. We only monitor children whose spondylolisthesis is more than a 25% slip or who continue to have bouts of pain. Surgical treatment is indicated only in children who have failed several courses of orthotic treatment or in whom the spondylolisthesis progresses to 50% slippage. This indication occurs only rarely, probably at a rate similar to the normal population. In our experience less than 10% needed a fusion of those who were treated for the acute symptoms of spondylolysis or spondylolisthesis (Case 3). The high rate of spondylolysis and spondylolisthesis in ambulatory children with diplegia who have had dorsal rhizotomies is worrisome. Although there are reports of high incidence of spondylolisthesis and spondylolysis following rhizotomy, there are no reported series of surgical treatments. Because many of these children are not yet skeletally mature individuals at the time of the reports, it is difficult to predict the significance of these lesions through adulthood. Our experience is limited to several surgical treated cases, with one case having a very severe archanoiditis following the spine fusion which caused her function to drop from GMFCS II to GMFCS IV and become wheelchair dependent.

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This is the worse complication we have seen secondary to rhizotomy.

Conclusion To manage pelvic malalignments, it is important to identify the etiology with the treatment requiring correction the primary etiology. Spondylolysis and spondylolisthesis do occur in ambulatory children with cerebral palsy but almost never in individuals how do not walk or ambulate. Ambulatory individuals who have had dorsal rhizotomy are at highest risk. Individuals with athetosis or other movement disorders are also at increased risk.

Cases

Case 1 Clarissa

Clarissa, an 8-year-old girl with severe spastic quadriplegia, presented for a second opinion concerning her progressive scoliosis. Her parents were most concerned about her increasing problems with sitting, which they perceived came primarily from her scoliosis. She had been prescribed a spinal orthosis to help with sitting and control her scoliosis. She was fed orally and was small for her age but appeared well nourished. She was taking Tegretol for seizure control and had not had a seizure for 6 months. She was a dependent sitter and had minimal function in her hands. On physical examination she was noted to be diffusely spastic with mild shoulder contractures. The spine had a flexible scoliosis, and the hips were limited to 10 of abduction on the left side and 50 of abduction with some limited adduction on the right side. The knees had a popliteal angle of 60 bilaterally, and the feet were controlled with solid ankle-foot orthotics with minimal fixed deformity. (continued)

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Observation of her sitting demonstrated rather poorly adjusted chest laterals, as she was hanging over the lateral on the right side. A radiograph of the spine demonstrated 48 of scoliosis (Fig. C1.1), and the right hip was dislocated, and the left hip appeared to be abducted in the classic windblown deformity (Fig. C1.2). Based on this assessment, it was concluded that she had a primary infrapelvic pelvic obliquity due to

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Fig. C1.3

Fig. C1.4

Fig. C1.1

Fig. C1.2

the spastic hip disease. It was recommended to her parents that she have a repair of the hips by bilateral femoral shortening derotation, varus osteotomy, adductor muscle lengthening, and peri-ilial pelvic osteotomy (Fig. C1.3). Following this procedure, she could sit much better until age 12 years when her sitting again deteriorated, and the pelvic obliquity now was caused by suprapelvic pelvic obliquity coming from a progressive 74 scoliosis (Figs. C1.4 and C1.5). This was corrected with a Unit rod instrumentation, and she was again comfortable as a sitter (Fig. C1.6). This case demonstrates the importance of making the correct diagnosis of the pelvic obliquity, because correcting the spine will not help treat the symptoms of infrapelvic pelvic obliquity and vice versa. When in doubt, the spine should be corrected first if there is a significant scoliosis.

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Fig. C2.1 Fig. C1.5

Case 2 Sherrill

Sherrill, a 13-year-old girl with severe spastic quadriplegia, limited upper extremity function, and normal cognitive function, presented with a complaint of severe back pain from sitting. Further history demonstrated that for many years she had increased lumbar lordosis during sitting, which the physical therapist thought was due to hip flexion contractures. Over the past year, she had grown rapidly and the lordosis had increased significantly. In the past 3 months, there had been a significant increase in her back pain, especially related to sitting time. There had been no change in her bowel or bladder control. On physical examination she had a fixed lumbar lordosis with hip flexion contractures, and the Thomas test was positive at 30 . A radiograph demonstrated lumbar lordosis of 105 with significant thoracic kyphosis (Fig. C2.1). Following an anterior wedge excision of disks from T12 to L4, posterior Fig. C1.6

(continued)

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Fig. C3.1 Fig. C2.2

spinal fusion was performed with the Unit rod (Fig. C2.2). Sitting balance and comfort increased greatly, although she complained of neuritic type pain in her legs postoperatively. This was treated with electrical stimulation, anti-inflammatories, and antidepressant medications with slow resolution. By 1 year after the surgery, the neuritic pains had resolved and she remained comfortable.

Fig. C3.2

Case 3 Antonio

Antonio, a 12-year-old ambulatory boy with severe athetosis, complained of low back pain that was worse after he walked a long distance or at the end of the day. A physical examination showed no significant contractures except for increased femoral

anteversion, but he had some tenderness with forced extension of the lumbar spine. A radiograph demonstrated a chronic spondylolisthesis with a grade 1–2 slip (continued)

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(Fig. C3.1). His gait pattern showed the high variability of the athetoid movement disorder. After treatment with a lumbar flexion lumbosacral orthosis for 5 months, the pain immediately returned. He then had an in situ fusion of L4 to the sacrum (Fig. C3.2). After the fusion healed, all the pain resolved.

Cross-References ▶ Anterior Dislocation of the Hip in Cerebral Palsy ▶ Surgical Treatment of Scoliosis Due to Cerebral Palsy ▶ Windblown Hip Deformity and Hip Contractures in Cerebral Palsy

References Carl RL, Noonan KJ, Nemeth BA (2007) Isthmic spondylolisthesis in a nonambulatory patient: a case report. Spine (Phila Pa 1976) 32:E723–E724 Hennrikus WL, Rosenthal RK, Kasser JR (1993) Incidence of spondylolisthesis in ambulatory cerebral palsy patients. J Pediatr Orthop 13:37–40. SRC – GoogleScholar Johnson MB, Goldstein L, Thomas SS, Piatt J, Aiona M, Sussman M (2004) Spinal deformity after selective

F. Miller dorsal rhizotomy in ambulatory patients with cerebral palsy. J Pediatr Orthop 24:529–536 Langerak NG, Vaughan CL, Hoffman EB, Figaji AA, Fieggen AG, Peter JC (2009) Incidence of spinal abnormalities in patients with spastic diplegia 17 to 26 years after selective dorsal rhizotomy. Childs Nerv Syst 25:1593–1603 Li Z, Zhu J, Liu X (2008) Deformity of lumbar spine after selective dorsal rhizotomy for spastic cerebral palsy. Microsurgery 28:10–12 Murphy KP (2009) Cerebral palsy lifetime care - four musculoskeletal conditions. Dev Med Child Neurol 51(Suppl 4):30–37 Peter JC, Arens LJ (1993) Selective posterior lumbosacral rhizotomy for the management of cerebral palsy spasticity, a 10 year experience. S Afr Med J 83:745–747. Peter JC, Hoffman EB, Arens LJ, Peacock WJ (1990) Incidence of spinal deformity in children after multiple level laminectomy for selective posterior rhizotomy. Childs Nerv Syst 6:30–32 Peter JC, Hoffman EB, Arens LJ (1993) Spondylolysis and spondylolisthesis after five-level lumbosacral laminectomy for selective posterior rhizotomy in cerebral palsy. Childs Nerv Syst 9:285–287; discussion 2878. Rosenberg NJ, Bargar WL, Friedman B (1981) The incidence of spondylolysis and spondylolisthesis in nonambulatory patients. Spine 6:35–38 Sakai T, Yamada H, Nakamura T, Nanamori K, Kawasaki Y, Hanaoka N, Nakamura E, Uchida K, Goel VK, Vishnubhotla L, Sairyo K (2006) Lumbar spinal disorders in patients with athetoid cerebral palsy: a clinical and biomechanical study. Spine (Phila Pa 1976) 31:E66–E70 Spiegel DA, Loder RT, Alley KA, Rowley S, Gutknecht S, Smith-Wright DL, Dunn ME (2004) Spinal deformity following selective dorsal rhizotomy. J Pediatr Orthop 24:30–36

Infections and Late Complications of Spine Surgery in Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1834 Pathology of Long-Term Complication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1834 Postoperative Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1834 Mechanical Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1837 Special Problems with Spinal Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1841 Doing Revision Spinal Surgery in Children with Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . 1841 Mortality . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1843 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1843 Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1843 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1849 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1849

Abstract

Children with cerebral palsy at the GMFCS IV and V levels often develop spinal deformity requiring surgical correction. A very common concern of parents is to understand the expected complications. The most common significant postoperative complication is a deep wound infection which usually requires a prolonged period of wound care and intravenous antibiotics. However, with proper management seldom do the rods need to be removed, and as a consequence, the benefit of

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_226

the surgery will almost always be maintained. In the long term, there is a small chance of a late infection in the spine around the instrumentation. When a late infection occurs, almost always removal of all the hardware is required. Another problem which may occur is pseudarthrosis, with the two most common sites being the thoracolumbar junction and lumbosacral junction. The most common presenting sign of a pseudarthrosis is fracture of the spine rod. If there is pain or progressive deformity at the site of the pseudarthrosis, a localized repair is required. When patients report back pain 6 months after spine fusion, chronic low-grade infection or pseudarthrosis should be considered. Workup is performed with CT scan, technetium bone scan, and 1833

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testing for inflammatory markers. Long-term mortality is another concern that is not well defined; however, children with the most severe neurologic impairments develop spinal deformities and do have a reduced life expectancy compared to age-matched peers. There is not good data to show that correcting the spinal deformity increases life expectancy.

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expectancy compared to age-matched peers. There is not good data to show that correcting the spinal deformity increases life expectancy. The goal of this chapter is to review postoperative infections after spinal fusion and other late complications occurring in children with cerebral palsy.

Pathology of Long-Term Complication Keywords

Cerebral palsy · Complications · Spinal fusion · Scoliosis · Pseudoarthrosis · Infection

Introduction Children with cerebral palsy at the GMFCS IV and V levels often develop spinal deformity requiring surgical correction. A very common concern of parents is to understand the expected complications. The most common significant postoperative complication is a deep wound infection which usually requires a prolonged period of wound care and intravenous antibiotics (Sponseller et al. 2010). However, with proper management seldom do the rods need to be removed, and as a consequence, the benefit of the surgery will almost always be maintained. In the long term, there is a small chance of a late infection in the spine around the instrumentation. (Szoke et al. 1998). When a late infection occurs, almost always removal of all the hardware is required. Another problem which may occur is pseudarthrosis, with the two most common sites being the thoracolumbar junction and lumbosacral junction. The most common presenting sign of a pseudarthrosis is fracture of the spine rod. If there is pain or progressive deformity at the site of the pseudoarthrosis, a localized repair is required. When patients report back pain 6 months after spine fusion, chronic low-grade infection or pseudarthrosis should be considered (Dias et al. 1997). Workup is performed with CT scan, technetium bone scan, and testing for inflammatory markers. Long-term mortality is another concern that is not well defined; however, children with the most severe neurologic impairments develop spinal deformities and do have a reduced life

Postoperative Infections Major postoperative infections are among the most serious complications that occur following surgery. Infections may cause neurologic deficits after spinal fusion, and they are the most severe insults that delay recovery and have the potential for causing death, paralysis, or loss of hardware. The initial increase in fevers, which typically occurs in the first 5 days after surgery, is almost always respiratory based. As such, these fevers should be treated with broad-spectrum antibiotics if these children remain febrile, even if there are no positive cultures. From 3 days postoperatively until discharge, urinary tract infections should always be considered as a possible source of febrile events or sepsis. As long as children have intravenous lines, these lines need to be cultured and observed as possible sources of sepsis. All intravenous lines and arterial catheters should be removed as soon as they are no longer being used. The central venous line can also be a source of sepsis; however, it is crucial to providing nutrition and should not be removed unless there is cultured evidence that it is infected and a likely source of the ongoing infection. If these central lines are routinely removed as soon as there is a fever, these children will hardly ever get adequate intravenous feeding, and a new problem will result. The goal of this aggressive management of postoperative sepsis is to avoid septicemia, which might seed the large operative wound and hematoma.

Persistent Fevers Persistently high fevers, usually starting between days 7 and 14 postoperatively, sometimes with elevation of the white blood cell count to 20,000, are occasionally encountered.

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These high temperatures can rise to over 40  C, and an aggressive search for a source of infection should include chest radiographs, sputum cultures, urine cultures, and blood cultures. The wound should be aspirated with a long 18-gauge needle and the hematoma fluid sent for culture. This aspiration is performed by going through healthy paraspinal muscle and not through the operative wound. Aspirating through the operative wound may leave a draining injection site that can itself become the source of an infection. Abdominal ultrasound and blood tests should be performed to rule out pancreatitis and cholecystitis. Broad-spectrum antibiotics should be started. If the fever rises above 40  C to 40.5  C and does not decrease with antipyretics, body cooling should be instituted using cooling blankets or ice packing. A small group of children are never positive on any culture but continue to have very high fevers. If these high fevers continue for 5–7 days, all antibiotics should be discontinued and temperature control maintained as needed with antipyretics and external cooling but also with a persistent awareness of the possibility of a missed infectious source. The longest time we have seen for this persistent febrile course was 4 weeks; the fever then resolved without any residual effects. More commonly, the high temperatures persist for 3–5 days and then slowly decrease with a pattern of afternoon spikes. The source of these idiopathic fevers is unknown, but it may be a rejection phenomenon from the large volume of bone graft that is used or may be related to poor central neurologic temperature control, which many children with severe quadriplegic pattern CP also have.

Superficial Wound Infections Superficial wound infections are defined as those which do not violate the deep fascia plane. Usually a small localized area of the wound opens and a subcuticular suture becomes exposed. There are occasionally larger areas of erythema in which the superficial area opens more. These superficial wound problems can be treated easily with local wound care and with oral antibiotics if there is erythema. These superficial wounds do not have much drainage. They can be covered with dry

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gauze, and in 12–24 h, they should not soak a thin gauze sponge but should leave a circular area of drainage somewhat larger than the wound that it was covering. These children do not have significant systemic signs of infection, such as fever and leukocytosis. If the drainage is larger or the children have systemic symptoms, it is important to make sure that a deep wound infection, meaning an infection beneath the muscle fascia, is not present. It is very important to do a watertight wound closure of the deep fascia at the time of surgery, because if any open areas in the fascia are left, almost every minor superficial wound problem will become a deep wound infection. This careful closure is undoubtedly the most important technical thing that surgeons can do to avoid deep wound infections in spine surgery, especially in children with CP.

Acute Deep Wound Infection Acute deep wound infection is the most common serious complication occurring in spinal surgery in children with CP. The incidences from the literature vary because many reports of spinal surgery, even those directed at studying wound infection rates, combine all neuromuscular patients, including spina bifida patients. This is not appropriate because children with spina bifida are insensate and have poor skin and will always have a higher infection rate than children with CP. Children with CP probably have the next highest infection rate, although this is difficult to determine definitely (Terjesen et al. 2000; Mohamed Ali et al. 2010; Sponseller et al. 2013). The severity of the child’s neurologic involvement is probably the highest risk factor for the development of a deep wound infection. However, we have had deep wound infections in ambulators as well as in children with the most neurologic involvement. In an early review of our center’s results (Szoke et al. 1998), we reported 6 deep wound infections in 172 cases for an incidence of 3.7%. Our latest report found a 4.2% rate (Tsirikos et al. 2008). This incidence rate is similar to other reports when an attempt is made to separate out the various underlying diagnoses that have been mixed together. The most recent review paper reported a range between 2.5% and 57%

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(Legg et al. 2014). Sometimes, making the diagnosis is very easy because the wound is grossly open and infected and the children appear generally septic, being febrile with leukocytosis. However, more commonly, children have a small superficial wound that is draining more than expected, and they are not really septic. Gentle probing of this superficial wound should give a definite resistance at the level of the deep fascia. If during this probing the deep fascia is easily breached and the probe reaches to the fusion site, a deep wound infection is present by definition. Sometimes, the fascial defect is proximal to the more superficial wound and will be missed in this initial probing. An aspiration of the deep wound using a long 18-gauge needle should also be performed if there is still concern. If no hematoma is aspirated, it may mean that the hematoma has decompressed itself and a deep wound infection is present. The aspiration is only definitive if good hematoma is aspirated and is found to be sterile. If it is unclear whether children have deep wound infections, it is generally best to just observe the children over several days and allow the wound to declare itself. A superficial wound infection or wound opening will gradually have decreasing drainage; however, a deep wound infection will continue with copious amounts of drainage with fluid being expressed proximal or distal to the superficial wound. These deep wound infections become clarified with time through this careful observation. All scans, such as bone scans and white blood cell-labeled scans, are of no use in this acute phase after spinal fusion. Both scans respond to inflammation; however, the spinal fusion and the use of bank bone grafts always induce a very large inflammatory response which cannot be separated from the inflammation of an infection. Sometime between 30 and 90 days following the procedure, the surgical inflammation is greatly reduced. By 3 months postoperatively, both bone scans and white blood cell-labeled scans are very useful to detect infection. Obtaining scans to determine the presence of an acute deep wound infection in the first 30 days postoperatively is a waste of time. By careful observation over time, the deep wound infection will become defined clearly (Case 1).

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Approximately one third of acute deep wound infections are due to an infection from outside through an open superficial wound. These infections usually occur at the far distal end where a small wound opens and then becomes soiled during a bowel movement (Mohamed Ali et al. 2010). It is often difficult to get a firm, tight fascial closure with good subcutaneous and skin closure in this area. If small wound drainage starts and the wound is contaminated with feces, a multiple bacterial species infection with fecal bacteria will result. These have been the worst infections, with one child becoming severely septic. In addition to careful wound closure, as the initial dressing is removed from the caudal end of the wound, it is important to keep an occlusive dressing on the distal third of the wound to prevent this type of contamination from minor wound leakage. We had two cases where the clear cause of the deep wound infection was dehiscence of the deep fascial closure, which then allowed communication with a minor superficial skin opening. This is a completely avoidable complication. Another third of our deep infections were linked to sepsis at other sites, such as urinary or respiratory infections causing septicemia. These infections are usually single organisms, either gram positive or gram negative. The last third of our infections, most of which are gram positive and probably occur as contamination of the wound intraoperatively, occur without a clear source. Prevention of deep wound infections should include careful sterile technique in the operating room and very tight closure of the deep fascia, subcutaneous layer, and skin, followed with an occlusive wound dressing. Also using depo antibiotic in the bone graft and wound is recommended. We reduced our infection rate using gentamicin which comes in a liquid form (10 mg/kg of body weight) absorbed into the dry bank bone graft (Borkhuu et al. 2008). Some surgeons are using vancomycin powder by directly placing the powder into the wound.

Treatment Acute deep infections. Treatment of deep wound infections should be standardized because there is a tendency for surgeons to want to deny the

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severity of the infection and not approach it with the level of care the wound needs. All deep wound infections should be taken to the operating room and aggressively debrided, irrigated, and packed with an open wound vac dressing sponge. Alternatively, if this is not available, packing with betadine-soaked sponge also will work well. The whole spine wound usually does not need to be opened; however, the area of the abscess does need to be opened to the full extent of the abscess. All reports of other treatments have reported significant rates of failure, meaning that the hardware needs to be removed up to 50% of the time (Sponseller et al. 2000). The packing then should be changed daily starting the day after surgery. This can almost always be done on the ward; however, if the wound is very large, another return to the operating room for dressing change and debridement under general anesthesia may be required. The debridement may continue on the ward as the necrotic tissue separates and is then removed. After all the necrotic tissue has been removed, the wound is allowed to close by secondary intention from the bottom up. The packing should be very loose with a saline-soaked sponge or the wound vac dressing using continuous suction; however, it should be clear that the granulation tissue closes over the rod and that it does not close leaving a fluid-filled cavity as the skin closes over the top. Managing this closure requires that physicians continue to check the wound every day or two. This need for frequent wound checks and intravenous antibiotics means that these patients are kept in the hospital for 4–5 weeks of treatment until granulation tissue has covered the rod, which is the criterion for discharge to outpatient and home nursing care. Typically, intravenous antibiotics specific to the results of the culture are continued at full doses for 6 weeks. After 6 weeks, children are kept on antibiotic suppression therapy with one antibiotic orally if a simple antibiotic is available against the specific organism. This suppression therapy is continued for 6–12 months. Except for one patient with a very severe infection of the whole spine combined with meningitis, all our other deep wound infections have cleared, and the hardware has remained covered and

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in place without evidence of any late infections (Szoke et al. 1998). Late deep wound infections. A late deep infection is one that presents with no previous evidence of infection more than 3 months after spine surgery. We have had late deep wound infections occurring 2–10 years after the original surgery. One of these was directly related to a concurrent urinary tract infection; however, the other was a staphylococcus epidermidis infection that was not directly related to any known concurrent problem. These infections typically involved the whole spinal rod and required removal of the rod and all wires. Solid fusions are usually present. After removal of all the hardware, direct wound closure over a deep wound drain is often possible if there is no active acute evidence of infection. These usually have a slime film material covering the hardware. These wounds typically healed well. One needs to inform the family of the risk of gradual bending of the fusion mass as shown in this child 1.5 years after hardware removal who now has an additional 20 of scoliosis (Case 2). The other boy healed his wound well and had almost closed the deep wound when he had a sudden period of shortness of breath followed by a cardiac arrest at home. No autopsy was done. We have never seen a case of late sterile drainage requiring removal of the hardware, which has been reported with other spinal hardware systems (Yazici et al. 2000).

Mechanical Problems The mechanical problems occurring with spinal fusions in children with CP are specific to the instrumentation system used. The current state of the art is the use of the unit rod or similar devices; therefore, the multitude of mechanical problems that are specific to other individual systems is not addressed. In general systems with many rods, connections have a risk of disconnection or connection failures.

Pain in the Spine Complaints of pain in the back after the acute postoperative period occur mainly from either

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the distal or proximal end. At the proximal end, there is often a 3-month period of some discomfort at the cervicothoracic junction. This discomfort has never become a chronic problem in any of our patients. If the rod is too long or prominent, a bursa can form over the end of the rod and cause chronic discomfort. If this discomfort persists for more than 1 year, the top of the rod can be excised at approximately the T3 level, and the discomfort will disappear. This proximal junction problem is also reported in other series and is likely due to the difficulty in choosing the correct rod length (Sponseller et al. 2009). At the distal end, children may occasionally develop very severe halos around the pelvic leg of the rod, which most typically occur 1–3 years after surgery (Fig. 1). If these individuals are having pain, especially if the pain is increasing, there may be a low-grade infection in this area. Of the six children in whom we have removed the pelvic legs, infections were present in half. This infection can be treated by excising the pelvic end of the rod followed with antibiotics. In two children, the rest of the rod did not develop any signs of infection, and the rod was solidly encased in fusion mass at the time of removal of the distal end. Many children get some halo effect around the rod, but it is not painful and probably represents movement of the sacroiliac joint. The halo effect should be of concern only if there is

Fig. 1 Large halos occasionally develop around the pelvic legs of the unit rod. These halos are due to motion in the sacroiliac joint, as can be seen in this example of a child with a solid fusion at the lumbosacral level. If the halos do not form, the rod will sometimes fracture as it enters the ilium

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significant pain. Then, if the surgery was more than 1 year ago, the ends can simply be removed; however, it is always important to take a culture from the area of the pelvis where the rod end was removed. One of our children had persistent sciatica from a rod that perforated the pelvis laterally. This perforation responded to removal of the end of the rod. Also, when correcting severe lordosis, several of our children complained of neuritic symptoms in the legs, which resolved over the first postoperative year. We feel this mostly represented stretching of the sciatic nerve and the nerve roots during lordosis correction.

Proximal End Prominence or Wire Prominence As previously mentioned, if the rod is too long, it will often appear prominent and may develop a symptomatic chronic bursitis. This bursitis can be easily treated by cutting the rod at the T3 level and removing the proximal end. Treating the chronic bursitis should be done after the spine has fused; therefore, we like to wait at least until children are 1 year postoperative. If there is an acute need for treatment, the rod should be exposed more distally and two rigid connecting devices used to connect the two individual rods to prevent rod motion. We had three children who have presented with open decubitus ulcers over the prominent proximal end of the rod. The decubitus ulcer was excised, and the proximal end of the rod was excised with good healing of the wounds with no persistent infection occurring. We also had one child in whom a wire that was not bent down toward the rod perforated through the skin. Under low-dose local anesthetic, an additional small incision was made, and the wire was cut off at the level of the fascia. The skin was allowed to heal over without any development of infection or problems on follow-up of more than 5 years. Nonunion–Pelvic Leg Halos–Rod Fracture Pseudoarthrosis, which was a significant problem in the early days of spinal fusion in children with CP, has disappeared. In an earlier report of the outcome of the unit rod instrumentation

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Fig. 2 A common cause of nonunion is the use of 3/16inch rods that are not rigidly connected. This 17-year-old ambulatory girl was seen in consultation 2 years after being instrumented with 3/16-inch rods and clear pseudarthrosis with rod fracture. She presented with severe back pain. Treatment of this pseudarthrosis requires compression instrumentation and repeat bone grafting

(Bell et al. 1989), there were two rod fractures, both occurring at the thoracolumbar junction with nonunions (Fig. 2). In the time period reflected in this report (Bell et al. 1989), the instrumentation was performed without the use of allograft, using only the bone harvested from the spinous process resections (Fig. 3). There have been few reports of nonunions in children with CP with unit rod instrumentation when copious amounts of allograft were used. In our center, where we have done 750 posterior spinal fusions with the unit rod using copious amounts of allograft, decorticating, and doing facetectomies, there have been 5 nonunions. There may have been some nonunions that were not recognized; however, there were only two rod fractures, both at the

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Fig. 3 Nonunion and unit rod fractures are very uncommon if there is complete facetectomy, transverse process decortication, and application of a large amount of bank bone. This boy is a large ambulatory boy with diplegia who suddenly developed pain 3 years after surgery. He has minimal fusion mass at the thoracolumbar junction with probable pseudarthrosis. We have not had a documented pseudarthrosis in over 300 cases, although there are likely pseudarthroses that have not become symptomatic. This case shows that it takes a lot of force for 3 years to cause rod failure

lumbopelvic junction in very large individuals. These individuals did not develop any halo effect in the pelvis, and we believe that the rigidly fixed leg of the pelvic end of the rod fractured because of micromovement of the sacroiliac joint (Fig. 4). These individuals remain asymptomatic.

Crankshaft Late progression of scoliosis, especially in young children, was a common cause for the need for revision surgery in the early era of spinal fusion in children with CP. As many as 20% of children with the first versions of the Luque system had substantial late progression of their scoliosis (Comstock et al. 1998; Gau et al. 1991). This

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on top of the shoulders without much force. Some children perceive this change as being unnatural because they were so used to the force required to hold their head upright. This complaint almost always resolves after 3–4 months postoperatively as these children get used to the new sense of their head position and the soreness from surgery usually subsides.

Fig. 4 Rod fracture can occur at the distal bend on the unit rod when there is solid fixation of the pelvic limb in the pelvis (a). The spine radiograph demonstrates excellent fusion mass along the whole spine, and the rod fracture was found inadvertently on a 2-year surgical follow-up radiograph (b). This girl is asymptomatic

progression has been attributed to continued anterior growth along the anterior aspect of the spine, even after a solid posterior fusion, and it is called the crankshaft effect. Twenty-nine children, who were fused posteriorly only using the unit rod before closure of the triradiate cartilage, were reviewed. At skeletal maturity, none of these children had any measurable loss of correction compared with their immediate postoperative position. Crankshaft does not occur when unit rod instrumentation is used; therefore, there usually is no concern for prevention (Smucker and Miller 2001). This instrumentation is so strong that it prevents this anterior growth from distorting the spine.

Neck Stiffness Almost all children who can communicate will complain of neck stiffness after spinal surgery. Stiffness is due to soreness and decreased range of motion from the surgery at the thoracocervical junction. Often, these children’s necks feel stiff because their heads are in a very different and much more stable position. With the spine fully corrected, the head sits naturally at a correct angle

Decreased Floor or Bed Mobility There is a risk of substantial functional loss in children who cannot sit independently but can move on the floor by rolling, often using considerable trunk action. These children can often roll in bed as well to change their position by using this combination of trunk extension and trunk torsion. The most common complaint of loss of function from parents and caretakers is that these children can no longer turn in bed and no longer have floor mobility. Most of these individuals are at a stage when they are becoming young adults, and the difficulty of getting them up off the floor, in addition to the socially unacceptable posture of rolling around on the floor at home, makes this loss of floor mobility a relatively minor problem. However, the loss of ability to turn in bed is a major problem because caretakers now must attend to these individuals every time they need to change their position. Some children will slowly regain this ability over 1–2 years after surgery; however, other individuals can never regain the ability to change their position in bed. A good effort should be made, with intensive physical therapy to try to teach these individuals to turn themselves as well as provide them with adaptive equipment such as rails or overhead bars if these devices can be demonstrated to be useful. These individuals usually make other gains, such as dramatic improvement in sitting ability, which allow the caretakers to see this loss of function as a negative part in an overall greater improvement. If caretakers express overall dissatisfaction with posterior spinal fusion, this loss of function is the most common reason for the dissatisfaction. This small group of children can be preoperatively identified, and parents and caretakers should be warned of this possible loss of function.

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Special Problems with Spinal Surgery Doing Revision Spinal Surgery in Children with Cerebral Palsy Doing revision surgery in children with CP is often a difficult task; however, this depends on the specific problems that are to be addressed. Usually, there should be a very specific goal to be accomplished, such as getting children into better sitting positions, treating painful nonunions, or improving respiratory function. Based on a well-defined problem, a careful plan to achieve these specific goals should be outlined (Dias et al. 1997). The need for revision surgery has greatly decreased with the advent of better instrumentation and the recognition that children with CP need to have the fusion extend from T1–T2 to the pelvis.

Fall-Off from a Short Fusion If the identified problem is that the previous fusion was performed too short, leaving severe pelvic obliquity and an increased residual curve, it is often possible to remove the distal part of the implant and use the proximal end as fixation for the new instrumentation. Using this proximal end for fixation avoids having to remove all the old instrumentation and provides a source for rigid proximal fixation. The apex of the curve must be identified, and osteotomies of the previous fusion mass must extend at least past the apex of this curve if correction of the deformity is to be accomplished. If anterior instrumentation was used, it usually needs to be removed, and the anterior disk spaces need to be completely cut through to allow for correction. If the deformity is a proximal fall-off into severe kyphosis or scoliosis, the anterior disks may need to be excised to T2–T3 if at all possible, and the diskectomy should extend to at least T6–T7 because posterior osteotomies will need to be performed this far distally. The instrumentation should use pedicle screws to C7 and lateral mass screws above. Sharp, short, high thoracic curves are extremely stiff and hard to correct; therefore, a significant amount of the correction needs to be obtained in

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the midthoracic area. If a rod is present distally, it too can usually be cut off, and then the proximal rod can be attached to the distal remaining rod.

Torsional Collapse Another reason for requiring revision in the past has been severe torsional collapse causing respiratory restriction when the unconnected independent rods twisted across each other. This problem is mainly of historical interest because these unconnected rods are no longer used. At the time when unconnected rods and wires were used, some patients had a straight spine, but the shoulders were rotated 90 relative to the pelvis. This whole instrumentation system has to be removed, and multiple osteotomies and pseudarthrosis levels have to be taken down with the insertion of a new rod. Wires can sometimes be salvaged in this construct and used with the new rods. Pedicle screws should be used for fixation since it is hard to pass wires in a scarred epidural space. Pseudarthrosis Pseudarthrosis has been a problem in the past with other instrumentation systems, and if it does occur, the pseudarthrosis must be cleaned and copious amounts of bone graft applied, followed by rigid compression fixation across the pseudarthrosis site. Bone grafting alone, especially in children with CP, is not likely to work (Fig. 2) (Dias et al. 1997). If the pseudarthrosis occurs at the distal L5S1 level, an anterior interbody fusion can be performed. The most common site of nonunion is the thoracolumbar region which can be easily addressed by posterior repair. Hardware Failure Another indication requiring revision surgery is when there has been acute failure of the hardware. In the unit rod, this usually occurs after the rod has been cut and then connected with connecting devices. These rod-connecting devices, especially if only one level of connection is used, have had a high failure rate in the past. Although the connectors are now much better, based on our past failure rate, we still always try

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to get at least two levels of connections, either one end-to-end connector and one side-to-side connector or two side-to-side connectors. However, as soon as this failure is recognized, the patient should be returned to the operating room and the instrumentation repaired, especially if the failure is relatively acute, before any bone fusion has occurred. Again, this complication can be avoided with proper rod connection.

Correcting Deformity Posterior Dorsal Rhizotomy Posterior dorsal rhizotomy was popular in the late 1980s and the early 1990s and has left a group of children with significant spinal deformities who have no posterior laminae for fixation. As longer follow-up has developed, it is clear that some of these adults will now have significant back and leg pain. In a report of follow-up from short term at 4 years to longer term at 21 years, 23% complained of disabling back and leg pain, while they had none at early follow-up. Also 37% developed hyperlordosis (Langerak et al. 2009). Hyperlordosis which is relatively common requires the use of pedicle screws for fixation. The incidence of spinal deformity after dorsal rhizotomy is probably higher than that reported in the literature since none of the studies followed children to completion of growth and spinal deformity increases as children complete growth (Turi and Kalen 2000; Langerak et al. 2009). Spinal deformity after dorsal rhizotomy likely has a higher incidence in nonambulatory children although this is not well defined since the practice of doing rhizotomy in GMFCS IV and V is now very uncommon. The most common severe deformity is hyperlordosis, which can occur with scoliosis or as an isolated deformity (Case 3). If the Fazano technique of thoracolumbar laminectomy is utilized, then a thoracolumbar junctional kyphosis tends to develop. In recent years this technique seems to be more common. Treatment of this deformity is as previously outlined in the specific deformity sections (▶ Chap. 119, “Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy”). Spondylolysis and spondylolisthesis occur in children who had the five-level lumbar

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laminectomies at a significantly higher rate than the normal population. In ambulatory children with diplegia who had these dorsal rhizotomies, approximately 20% will have asymptomatic spondylolysis and spondylolisthesis (Hennrikus et al. 1993; Peter et al. 1990, 1993). It is unclear how great a problem these asymptomatic lesions will be over the lifetime of these children; however, they seem to increase in incidence in adults (Langerak et al. 2009). There are occasional children who develop severe multiple level spondylolysis and spondylolisthesis requiring surgical stabilization. The risk factor for developing spondylolysis and spondylolisthesis is mainly in active ambulatory children with diplegic pattern involvement (Peter et al. 1993). One of the very serious complications following rhizotomy and spondylolisthesis which required surgical stabilization due to increasing back pain and radicular leg pain was arachnoiditis. This complication has not been reported in the literature; however, we have seen one case. This patient developed severe back pain with two levels of spondylolysis requiring spinal fusion. Following the fusion she developed severe arachnoiditis. The debilitating pain and neuropathy caused her function to drop from GMFCS II to GMFCS IV long term.

Correcting Spinal Deformity in Ambulatory Children Occasionally, children with GMFCS I or II level with either hemiplegic or diplegic pattern CP develop scoliosis with a pattern similar to idiopathic adolescent scoliosis. This scoliosis may be idiopathic adolescent scoliosis; however, there is no way of knowing for sure. If the scoliosis has a pattern consistent with idiopathic adolescent scoliosis and children are ambulatory with diplegia or hemiplegia, then indications for treatment similar to idiopathic adolescent scoliosis should be applied. There are also ambulatory children with CP who clearly have long curves with an apex at the thoracolumbar region with significant truncal malalignment. These more typical neuromuscular curves often extend well into the lumbar spine and often have a significant element of hyper- or hypolordosis. These neuromuscular curves should

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be treated as CP spinal deformities, with instrumentation going to the pelvis (Case 4). There is a widely circulated orthopedic myth that fusion to the pelvis in ambulatory children with CP will prevent them from walking postoperatively. This myth has never been substantiated in the literature and probably originated in the early days of posterior spinal surgery with the use of Harrington rods, which removed all these children’s lumbar lordosis when it was used for a fusion to the pelvis. Removing all this lumbar lordosis and utilizing the old Harrington rod instrumentation to the pelvis would make it hard for children to walk after fusion. We instrumented many ambulatory children to the pelvis using the unit rod, and none had any substantial change in their ambulatory ability (Tsirikos et al. 2003a).

Mortality Long-term mortality has been reported at 30% at 11-year follow-up (Tsirikos et al. 2003b). For children with early-onset scoliosis, there was 28% mortality at 10 years postoperative and a projected 50% mortality at 15 years postoperative (Sitoula et al. 2016). We feel this high predicted mortality is related to the severe level of the neurologic impairment. There is no data comparing the mortality rate in children with spinal deformity correction compared to those who have severe spinal deformity. Another study reported 18% mortality after a short 2-year follow-up (Phillips et al. 2013). In another study of 117 patients, there were no deaths in the first 4 years, 91% survival at 10 years, and 72% projected survival at 20 years

Fig. C1.1

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(Asher et al. 2012). This likely represents a selection bias toward having less involved individuals at the time of surgery.

Conclusion Postoperative wound infections are the most common major complications encountered in 3–5% of patients. With proper management the instrumentation rarely needs to be removed, and almost all have a long-term good outcome. Late deep infections do usually require hardware removal and can have longer-term bending of the fusion mass. Pseudoarthrosis does occur and is sometimes asymptomatic and in other children requires a repair.

Cases

Case 1 Jordan

Jordan, a 14-year-old boy with severe spastic quadriplegia, had an uneventful spinal fusion and was discharged home to his mother’s care on the tenth postoperative day. Two weeks after he was discharged, his mother felt that he was warm, and she was concerned that his wound seemed to be a little warmer. She took him to see the pediatrician who thought he might have some viral syndrome and started him on oral antibiotics. Jordan returned to school (continued)

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and over the next week started to have some drainage from the back wound (Fig. C1.1). His mother returned to see the pediatrician, where his antibiotic dose was increased. He continued to have fever spikes to 40.5  C and seemed ill to the school personnel. The school nurse referred him back to the CP clinic where an evaluation showed that he had a temperature of 39.4  C and a white blood cell count of 14,300. The erythrocyte sedimentation rate (ESR) was 127. The back wound had a small area of approximately 5 mm in length that was draining a purulent material (Fig. C1.2). When pressure was placed on the wound 10 cm distal to the draining, a significant increase of the

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drainage occurred. Blood cultures were sent and he was taken to the operating room. When the distal 25% of the wound was opened, it was filled with purulent material surrounding the rod and bone graft. No purulent material could be expressed from the proximal end of the wound. Tissue biopsy was sent for culture and sensitivity, and the wound was copiously irrigated and packed with betadine-soaked sponges. A central line was inserted in expectation of needing long-term antibiotics, and he was started on cefazolin. The following day the dressing was changed on the ward under sedation, and dressing changes were started three times a day. The culture grew Staphylococcus aureus, and he was continued on high-dose nafcillin. After 10 days, the wound had less purulent material and was getting drier, so the dressings were changed to saline-soaked packing. After 2 more weeks and then 3.5 weeks after drainage, the wound was developing excellent granulation tissue, and the packing was discontinued in favor of a loose wet-to-dry cover dressing. After 3–4 weeks, healthy granulation tissue covers most of the hardware (Fig. C1.3); however, some gray necrotic fascia may need to be debrided (Fig. C1.4). He did not have a fever for 3 weeks, and the ESR was 82. At 4.5 weeks after the drainage, the hardware

Fig. C1.2

(continued) Fig. C1.3

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Fig. C1.4

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was covered, and he was discharged home to receive the remaining 10 days of intravenous antibiotic and to continue the wet-todry cover dressing. Jordan returned 6 weeks after drainage, and the central line was removed, and he was switched to oral trimethoprim/sulfamethoxazole twice a day. The ESR had decreased to 53. The wound was still open, 15 cm in length and 4 cm wide. Three weeks after this, he was seen in the outpatient clinic with the wound completely healed. Our current choice would be to use a wound vac drainage system which is easier for the family to manage and is equally effective compared to dressing changes as noted above. There is no evidence that the wound heals faster with the wound vac dressing. The antibiotic was decreased to once a day, and his mother was informed that she was to continue with this for 6 months. Radiographs showed good healing and formation of fusion mass 4 months after the original fusion.

Case 2 Charles

Fig. C2.1

Charles, an 18-year-old male with severe spastic quadriplegic CP, 5 years after a successful posterior spinal fusion with unit rod instrumentation, presented with sepsis (Fig. C2.1). He was noted to have mild erythema along his spine, which was aspirated and grew Proteus. The same bacteria also cultured out from his urine, so this was believed to be a hematogenous infection from his urinary tract. The spine was explored, and the whole rod was found to be involved. All hardware was removed (Fig. C2.2). With dressing changes and antibiotic, the infection cleared. No pseudarthrosis was present when the hardware was removed. However, by 18 months after rod removal, the scoliosis curve (continued)

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Fig. C2.3 Fig. C2.2

had increased 20 , and he had a noticeable increase in his physical position (Fig. C2.3). This increased scoliosis was thought to be caused by bending of the fusion mass. This problem should not progress further; however, he has been lost to further followup.

Case 3 Lemika

Lemika, a 14-girl-old girl, presented 6 years after having had a dorsal rhizotomy. Her main complaint was difficulty sitting (Fig. C3.1). She had moderate spastic quadriplegia, self-fed, and had mild cognitive impairment. On physical examination, the spinal deformity appeared to be relatively fixed, especially the lordotic component

Fig. C3.1

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Fig. C3.2 Fig. C3.4

Fig. C3.3

Fig. C3.5

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Fig. C3.6

(Fig. C3.2). She was otherwise in good health and took no medications. She was taken to the operating room where the anterior release was performed first, followed by the posterior instrumentation using a unit rod with pedicle screws. A short rod was used on the convex side of the scoliosis and attached to the unit rod with rod connectors. The pedicle screws on the concave side were set into the unit rod directly. She had an uneventful recovery and by the fifth postoperative day was sitting with excellent balance (Figs. C3.3 and C3.4). Radiographs also showed good correction of the deformity (Figs. C3.5 and C3.6). There has been no change in this spinal alignment over a 5-year follow-up.

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Fig. C4.1

Case 4 Allison

Allison, a 10-year-old girl with mild cognitive impairment and ambulatory spastic diplegia, presented with a severe neuromuscular-type scoliosis that her mother felt was impacting her ability to walk. An examination showed no change in her spasticity, and she was not taking any medications. Based on the severity of the scoliosis, it was recommended that she have a full spinal fusion with the unit rod instrumentation (Fig. C4.1), which she had without problems. Six months after surgery, she was walking as well or better than preoperatively, and by 1 year after surgery, her gait was more stable than preoperatively according to her mother.

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Cross-References ▶ Complications of Spine Surgery in Cerebral Palsy ▶ Early-Onset Scoliosis in Cerebral Palsy ▶ Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy ▶ Surgical Treatment of Scoliosis Due to Cerebral Palsy

References Asher MA, Lai SM, Burton DC (2012) Subsequent, unplanned spine surgery and life survival of patients operated for neuropathic spine deformity. Spine (Phila Pa 1976) 37:E51–E59 Bell DF, Moseley CF, Koreska J (1989) Unit rod segmental spinal instrumentation in the management of patients with progressive neuromuscular spinal deformity. Spine 14:1301–1307. SRC – GoogleScholar Borkhuu B, Borowski A, Shah SA, Littleton AG, Dabney KW, Miller F (2008) Antibiotic-loaded allograft decreases the rate of acute deep wound infection after spinal fusion in cerebral palsy. Spine (Phila Pa 1976) 33:2300–2304 Comstock CP, Leach J, Wenger DR (1998) Scoliosis in total-body-involvement cerebral palsy, analysis of surgical treatment and patient and caregiver satisfaction. Spine 23:1412–1424. SRC – GoogleScholar; discussion 14245 Dias RC, Miller F, Dabney K, Lipton GE (1997) Revision spine surgery in children with cerebral palsy. J Spinal Disord 10:132–144 Gau YL, Lonstein JE, Winter RB, Koop S, Denis F (1991) Luque-Galveston procedure for correction and stabilization of neuromuscular scoliosis and pelvic obliquity: a review of 68 patients. J Spinal Disord 4:399–410. SRC – GoogleScholar Hennrikus WL, Rosenthal RK, Kasser JR (1993) Incidence of spondylolisthesis in ambulatory cerebral palsy patients. J Pediatr Orthop 13:37–40. SRC – GoogleScholar Langerak NG, Vaughan CL, Hoffman EB, Figaji AA, Fieggen AG, Peter JC (2009) Incidence of spinal abnormalities in patients with spastic diplegia 17 to 26 years after selective dorsal rhizotomy. Childs Nerv Syst 25:1593–1603 Legg J, Davies E, Raich AL, Dettori JR, Sherry N (2014) Surgical correction of scoliosis in children with spastic quadriplegia: benefits, adverse effects, and patient selection. Evid Based Spine Care J 5:38–51 Mohamed Ali MH, Koutharawu DN, Miller F, Dabney K, Gabos P, Shah S, Holmes L Jr (2010) Operative

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and clinical markers of deep wound infection after spine fusion in children with cerebral palsy. J Pediatr Orthop 30:851–857 Peter JC, Hoffman EB, Arens LJ, Peacock WJ (1990) Incidence of spinal deformity in children after multiple level laminectomy for selective posterior rhizotomy. Childs Nerv Syst 6:30–32 Peter JC, Hoffman EB, Arens LJ (1993) Spondylolysis and spondylolisthesis after five-level lumbosacral laminectomy for selective posterior rhizotomy in cerebral palsy. Childs Nerv Syst 9:285–287. SRC – GoogleScholar; discussion 2878 Phillips JH, Knapp DR Jr, Herrera-Soto J (2013) Mortality and morbidity in early-onset scoliosis surgery. Spine (Phila Pa 1976) 38:324–327 Sitoula P, Holmes L Jr, Sees J, Rogers K, Dabney K, Miller F (2016) The long-term outcome of early spine fusion for scoliosis in children with cerebral palsy. Clin Spine Surg 29:E406–E412 Smucker JD, Miller F (2001) Crankshaft effect after posterior spinal fusion and unit rod instrumentation in children with cerebral palsy. J Pediatr Orthop 21:108–112 Sponseller PD, LaPorte DM, Hungerford MW, Eck K, Bridwell KH, Lenke LG (2000) Deep wound infections after neuromuscular scoliosis surgery: a multicenter study of risk factors and treatment outcomes. Spine 25:2461–2466. SRC – GoogleScholar Sponseller PD, Shah SA, Abel MF, Sucato D, Newton PO, Shufflebarger H, Lenke LG, Letko L, Betz R, Marks M, Bastrom T, Group Harms Study (2009) Scoliosis surgery in cerebral palsy: differences between unit rod and custom rods. Spine (Phila Pa 1976) 34:840–844 Sponseller PD, Shah SA, Abel MF, Newton PO, Letko L, Marks M (2010) Infection rate after spine surgery in cerebral palsy is high and impairs results: multicenter analysis of risk factors and treatment. Clin Orthop Relat Res 468:711–716 Sponseller PD, Jain A, Shah SA, Samdani A, Yaszay B, Newton PO, Thaxton LM, Bastrom TP, Marks MC (2013) Deep wound infections after spinal fusion in children with cerebral palsy: a prospective cohort study. Spine (Phila Pa 1976) 38:2023–2027 Szoke G, Lipton G, Miller F, Dabney K (1998) Wound infection after spinal fusion in children with cerebral palsy. J Pediatr Orthop 18:727–733. SRC – GoogleScholar Terjesen T, Lange JE, Steen H (2000) Treatment of scoliosis with spinal bracing in quadriplegic cerebral palsy. Dev Med Child Neurol 42:448–454. SRC – GoogleScholar Tsirikos AI, Chang WN, Dabney KW, Miller F, Glutting J (2003a) Life expectancy in pediatric patients with cerebral palsy and neuromuscular scoliosis who underwent spinal fusion. Dev Med Child Neurol 45:677–682

1850 Tsirikos AI, Chang WN, Shah SA, Dabney KW, Miller F (2003b) Preserving ambulatory potential in pediatric patients with cerebral palsy who undergo spinal fusion using unit rod instrumentation. Spine (Phila Pa 1976) 28:480–483 Tsirikos AI, Lipton G, Chang WN, Dabney KW, Miller F (2008) Surgical correction of scoliosis in pediatric patients with cerebral palsy using the unit rod instrumentation. Spine (Phila Pa 1976) 33: 1133–1140

F. Miller Turi M, Kalen V (2000) The risk of spinal deformity after selective dorsal rhizotomy. J Pediatr Orthop 20:104–107. SRC - GoogleScholar Yazici M, Asher MA, Hardacker JW (2000) The safety and efficacy of Isola-Galveston instrumentation and arthrodesis in the treatment of neuromuscular spinal deformities. J Bone Joint Surg Am 82:524–543. SRC – GoogleScholar

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1852 Posterior Spinal Fusion with Single Unit Rod or Modular Unit Rod Using Cantilever Correction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Indication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1852 1852 1852 1863

Special Consideration for Correction of Kyphosis and Lordosis . . . . . . . . . . . . . . . . . 1863 Anterior Spinal Release . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Indication . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1865 1865 1865 1870

Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1870 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1870

Abstract

Scoliosis is a common deformity in nonambulatory children with cerebral palsy, and most of the children require surgical correction. The surgical correction of scoliosis in children with cerebral palsy has to involve considerations that are not common for children with idiopathic scoliosis. It is especially important to consider the amount of the spine to fuse since it is very common for deformities

F. Miller (*) · K. W. Dabney Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_122

to occur at the ends of the fusion area if it is not extensive enough. Therefore, almost all children with nonambulatory cerebral palsy (GMFCS IV and V) who have scoliosis should have a fusion from T1–T2 to the pelvis. This corrects the deformity and also assures that the deformity will remain corrected long term with maximum benefit to the patient. The technique of using cantilever correction has been used extensively to make sure that the pelvis will align with the spine. This has been the best documented and most accurate way to get a pelvic alignment corrected. The description of this procedure requires that the rod systems be fixed in the pelvis first and then sequentially the correction is made so that the spine is 1851

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brought to normal alignment with the pelvis. This technique is not well recognized by spine surgeons who do not have familiarity with treating children with cerebral palsy. The goal of this chapter is to describe the technique for using cantilever deformity correction to align the trunk and pelvis. Keywords

Cerebral palsy · Posterior spinal fusion · Anterior spinal release · Unit rod · Modular unit rod · Sublaminar wires · Pedicle screws · Cantilever correction

Introduction Scoliosis is a common deformity in nonambulatory children with cerebral palsy. Since there is no conservative treatment for spinal deformities in children with cerebral palsy who have a progressive spinal deformity, most of the children require surgical correction. The surgical correction of scoliosis in children with cerebral palsy has to involve considerations that are not common for children with idiopathic scoliosis. It is especially important to consider the amount of the spine to fuse since it is very common for deformities to occur at the ends of the fusion area if it is not extensive enough. Therefore, almost all children with (GMFCS IV and V) nonambulatory cerebral palsy who have scoliosis should have a fusion from T1–T2 to the pelvis. This corrects the deformity and also assures that the deformity will remain corrected long term with maximum benefit to the patient (Dias et al. 1996; Smucker and Miller 2001; Tsirikos et al. 2004, 2008). It is also very common to have pelvic malalignments; therefore, the attention should be placed on making sure the pelvis has correct alignment with the spine. This frequently requires correction of pelvic obliquity and anterior pelvic tilt as well as pelvic malrotation. The technique of using cantilever correction has been used extensively to make sure that the pelvis will align with the spine. This has been the best documented and most accurate way to get a pelvic obliquity alignment corrected. The description of this procedure

F. Miller and K. W. Dabney

requires that the rod systems be fixed in the pelvis first and then sequentially the correction is made so that the spine is brought to normal alignment with the pelvis. This technique is not well recognized by spine surgeons who do not have familiarity with treating children with cerebral palsy. The goal of this chapter is to describe the technique for using cantilever deformity correction for alignment of the trunk and pelvis.

Posterior Spinal Fusion with Single Unit Rod or Modular Unit Rod Using Cantilever Correction Indication The primary instrumentation for fusion of cerebral palsy scoliosis is posterior spinal fusion using a unit rod or the modular system that allows cantilever correction of the pelvic obliquity. The primary benefit of the modular system is easier insertion in the pelvis especially when there is significant lumbar lordosis or severe pelvic obliquity. It is also easier to combine this system with pedicle screws. The indications for fusion in the growing child are a curve approaching 90 when sitting or a curve that is becoming stiff such that side bending to the midline is difficult (▶ Chap. 118, “Surgical Treatment of Scoliosis Due to Cerebral Palsy”). The same instrumentation is indicated for kyphosis in the adolescent when the kyphosis is becoming stiff or is a significant impairment to sitting. Surgical correction of lordosis is indicated when sitting is difficult or if there is pain with sitting from the severe lordosis (▶ Chap. 119, “Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy”).

Procedure 1. Preparation of the child should start with insertion of two large-bore peripheral intravenous lines if possible. The child then is intubated with careful attention to having the endotracheal tube well secured (▶ Chap. 82, “Anesthetic Management of Spine Fusion”).

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2. An arterial line is inserted, usually in the radial artery by percutaneous insertion. If it is impossible to obtain a percutaneous peripheral arterial line, cutdown of the radial artery is indicated with insertion of a line. If this is not possible, a cutdown onto the posterior tibial artery at the posterior aspect of the proximal medial malleolus is recommended. 3. A large-bore central line is inserted, typically using a tunneled central line, which will be used postoperatively as a feeding line. Usually, this line is inserted via the subclavian approach with the catheter exiting on the lateral inframammary line or at the medial midline. 4. A Foley catheter is inserted to monitor urinary output, and a nasogastric tube is inserted to continuously keep the stomach decompressed to decrease venous bleeding. 5. The patient is turned prone on the spine frame, making sure that the abdomen is fully dependent to decrease bleeding from increased abdominal venous pressure, and the hips are flexed sufficiently to maximally reduce lumbar lordosis (Fig. 1). 6. Spinal cord monitoring using Somatosensory Evoked Potentials (SSEPs) and Motor Evoked Potentials (MEPs) is recommended for all children with functional lower

Fig. 1 The patient is turned prone on the spine frame, making sure that the abdomen is fully dependent to decrease bleeding from increased abdominal venous

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7.

8.

9.

10.

extremities who are able to stand or ambulate. Most children with this level of function can be monitored. Children with severe cognitive limitation and no lower extremity function often cannot be monitored, and there is little benefit to monitoring this population. After prepping and draping, a posterior incision is made from T1 to the middle of the sacrum. The longitudinal direction of the line is chosen to be halfway between a straight line from T1 to the sacrum and a line that follows the curve of the spinous process (Fig. 2). A small superficial dermal incision is made, and then the subcutaneous tissue is infiltrated with a large volume, up to 500 ml, of a normal saline solution diluted 1–500,000 with epinephrine. An alternative is to use electrocautery to cut through the subcutaneous tissue and dermis. Utilizing lateral pressure from a clamp and a knife, the interspinous ligaments and spinous process apophysis are transected. By staying exactly in the midline where there are few crossing blood vessels, little bleeding is encountered (Fig. 2A). Subperiosteal dissection is performed over each lamina with packing of a sponge at each level (Fig. 2B).

pressure, and the hips are flexed sufficiently to maximally reduce lumbar lordosis

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Fig. 2 Utilizing lateral pressure from a clamp and a knife, the interspinous ligaments and spinous process apophysis are transected. By staying exactly in the midline where there are few crossing blood vessels, little bleeding is encountered (Fig. 2A). Subperiosteal dissection is performed over each lamina with packing of a sponge at each level (Fig. 2B)

Fig. 3 Identify the crest of the posterosuperior iliac spine, and then make a longitudinal incision down the midline of the crest to the inferior aspect of the posterosuperior iliac spine

11. After all the laminae are subperiosteally exposed and packed from T1 to L5, attention is directed to the sacrum, where the sacrum is stripped with exposure of the paraspinal muscles until the posterosuperior iliac crest can be palpated. This stripping and elevation need to occur from L5 to the distal end of the sacrum. 12. While doing periosteal elevation over the sacrum and L5, care should be taken to avoid opening the sacroiliac joints or violating the posterior sacroiliac ligaments, as these will have significant bleeding. 13. Identify the crest of the posterosuperior iliac spine, and then make a longitudinal incision down the midline of the crest to the inferior

14.

15.

16.

17.

aspect of the posterosuperior iliac spine (Fig. 3). Pelvic Instrumentation Option 1 – Subperiosteally strip the lateral aspect of the ilium anterior and inferior. Use a packing sponge; dissect inferior toward the sciatic notch and the posterosuperior iliac spine. Clean the inferior two-thirds of the posterosuperior iliac spine so its medial and lateral border and caudal edge are visible clearly. Insert the drill guide hook into the sciatic notch, and align the drill insertion point at the inferior aspect of the posterosuperior iliac spine. Do not get too close to the inferior

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Fig. 4 Insert the drill guide hook into the sciatic notch, and align the drill insertion point at the inferior aspect of the posterosuperior iliac spine. Do not get too close to the inferior border. Before drilling, make sure that the drill guide is held into the apex of the sciatic notch with its lateral border being flat against the ilium. Also, before drilling, mark the drill bit so that it will protrude 1–2 cm past the distal end of the drill guide. Drill the hole into the pelvis to, or just past, the mark on the drill bit. Always be careful to stabilize the drill guide in the proper position

18.

19. 20. 21.

22.

border. Before drilling, make sure that the drill guide is held into the apex of the sciatic notch with its lateral border being flat against the ilium. Also, before drilling, mark the drill bit so that it will protrude 1–2 cm past the distal end of the drill guide (Fig. 4). Drill the hole into the pelvis to, or just past, the mark on the drill bit. Always be careful to stabilize the drill guide in the proper position (Fig. 4). Using a wire or a thin probe, document that the drillhole is entirely within the bone. Pack Gelfoam into the drillholes to prevent bone bleeding. Pack the lateral side of the iliac crest with a sponge to prevent bleeding. These sponges have to be inserted completely over the edge of the iliac crest or they will become entangled in the rod or wires. These sponges will be removed just before wound closure. Pelvic Instrumentation Option 2 – After exposure of the posterior superior iliac crest, the intramedullary canal of the ilium can be defined using a pedicle probe and fluoroscopy requiring oblique positioning (Fig. 5). Make sure the tip of the probe is just above the sciatic notch and in the middle (Fig. 6) of the teardrop image of the ilium viewed on axis (Fig. 7).

Fig. 5 After exposure of the posterior superior iliac crest, the intramedullary canal of the ilium can be defined using a pedicle probe and fluoroscopy requiring oblique positioning

23. After the probe is confirmed to have developed the correct track, a pedicle screw is chosen so the length goes at least 1–2 cm past the apex of the sciatic notch. The screw

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Fig. 6 Make sure the tip of the probe is just above the sciatic notch and in the middle

Fig. 8 After the probe is confirmed to have developed the correct track, a pedicle screw is chosen so the length goes at least 1–2 cm past the apex of the sciatic notch. The screw position is then confirmed with fluoroscopy

Fig. 7 Make sure the tip of the probe is just above the sciatic notch and in the middle of the teardrop image of the ilium viewed on axis

Fig. 9 After the probe is confirmed to have developed the correct track. The screw position is also confirmed with fluoroscopy with axial view to confirm that the screw is centered in the ilium

position is then confirmed with fluoroscopy (Figs. 8 and 9). 24. Repeat the same procedure on the iliac crest on the opposite side. 25. Remove the sponge packs from the prior exposure of the spine, and clean each vertebra

so that all the soft tissue is removed from the tips of the transverse process over all the laminae and the spinous processes. Make a good clean exposure of the facet joints.

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Fig. 10 Remove the spinous processes by using a bone biter. In the thoracic spine, cut vertically approximately 1 cm distal to the superio aspect of the lamina

Fig. 11 It is important never to violate the superior border of the posterior elements, as this is where the strength for wire fixation occurs. In the lumbar spine, the spinous processes should be transected transversely at their base.

In the lumbar area, the spinous process is cut horizontally; then at the thoracolumbar junction, they are cut at 45 , and in the thoracic area, the process is cut off vertically

26. Remove the spinous processes by using a bone biter. In the thoracic spine, cut vertically approximately 1 cm distal to the superior aspect of the lamina (Fig. 10). By proper removal of the spinous processes, the spinal interspace is opened. It is important never to violate the superior border of the posterior elements, as this is where the strength for wire fixation occurs. In the lumbar spine, the spinous processes should be transected transversely at their base (Fig. 11). In the lumbar area, the spinous process is cut horizontally; then at the thoracolumbar junction, they are cut at 45 , and in the thoracic area, the process is cut off vertically (Fig. 11). 27. Use a rongeur with a serrated end to remove the ligamentum flavum (Fig. 10A). If more

bone removal is indicated, remove the bone from the inferior aspect of the spinous process base and lamina only. Never remove bone from the superior aspect of the lamina because this is the aspect of the lamina that provides strength for the wire (Fig. 10B). 28. Complete the spinal interspace opening with a curette, making sure that the ligamentum flavum is cut a sufficient distance on either side so wire can be passed (Fig. 10C). If epidural bleeding occurs during this time, the interspace should be packed gently with Gelfoam and a neural sponge. There may be substantial bleeding from these epidural veins; however, it is almost impossible to cauterize them without an extremely large exposure that destroys the lamina. The

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Fig. 12 Wires are inserted starting at the distal end at L5. Usually, two double wires are inserted at L5 and T1 and only a single double wire at each other level (Fig. 12A). The wires are bent over the laminae so that the double end of the wire is bent into the midline pointing caudally, and

bleeding can be controlled with gentle pressure, and occasionally wire passing has to be done in the face of some of the epidural bleeding. 29. Wires are inserted starting at the distal end at L5. Usually, two double wires are inserted at L5 and T1 and only a single double wire at each other level (Fig. 12A). The wires are bent over the laminae so that the double end of the wire is bent into the midline pointing caudally, and each beaded lateral single wire is brought out laterally and cross-cranially over the laminae. This double crossing of the wires provides extra protection to prevent the inadvertent protrusion of the wires into the neural canal (Fig. 12B). When passing wires, it is important to roll the wires under the lamina, being especially careful not to roll the wire with the tip caught under the lamina, as this will cause high pressure on the spinal cord (Fig. 13). Also always pull up on the wire, to prevent any wire loop from developing in the spinal canal which will likely cause spinal cord injury (Fig. 14). 30. Utilizing gouges or rongeurs, all facet joints are removed from T1 to the sacrum (Fig. 7). The transverse processes and far lateral borders of the laminae are decorticated. Bone graft then is packed into this decorticated bone. Bleeding that cannot be controlled with electrocautery will occur during this

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each beaded lateral single wire is brought out laterally and cross-cranially over the laminae. This double crossing of the wires provides extra protection to prevent the inadvertent protrusion of the wires into the neural canal (Fig. 12B)

Fig. 13 It is important to roll the wires under the lamina, being especially careful not to roll the wire with the tip caught under the lamina, as this will cause high pressure on the spinal cord

period as the bone is opened, and it should be controlled by packing the wound with bone graft soaked with thrombin and Gelfoam. Pressure from additional sponge packing also will help control the bleeding. If severe bleeding is encountered, this portion of the procedure can be done after insertion

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Fig. 14 Also always pull up on the wire, to prevent any wire loop from developing in the spinal canal which will likely cause spinal cord injury

of the rod, but it is more difficult, and decortication and facetectomy performed after rod insertion will be much less adequate. Some prefer to decorticate and remove facets after rod insertion to decrease the blood loss; however this severely limits the ability to decorticate and do a facetectomy. 31. When using the single unit rod, choose the correct rod length by estimating the rod and laying it upside down with the legs pointing posteriorly. The most caudal end of the rod is now aligned with the holes drilled in the

pelvis. If significant pelvic obliquity is present, choose a midway point between the right and left holes. The cranial end of the rod then is aligned to lie at the level of T1. If there is severe lumbar lordosis or severe scoliosis, one size longer rod may be chosen. If severe kyphosis is present, one size shorter rod should be chosen (Fig. 15). 32. When the modular rods are used (Fig. 16), insert the fixed lateral connectors to the inserted pelvic screws, and then insert the individual rods into the lateral connector.

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Fig. 15 When using the single unit rod, choose the correct rod length by estimating the rod and laying it upside down with the legs pointing posteriorly. The most caudal end of the rod is now aligned with the holes drilled in the pelvis. If significant pelvic obliquity is present, choose a midway

Fig. 16 When the modular rods are used, insert the fixed lateral connectors to the inserted pelvic screws, and then insert the individual rods into the lateral connector

The sagittal bend in the two rods should be aligned, so their contours match (Fig. 17) and the rods are perfectly perpendicular to the horizontal axis of the pelvis. The sagittal contour should align with the sacrum. The

F. Miller and K. W. Dabney

point between the right and left holes. The cranial end of the rod then is aligned to lie at the level of T1. If there is severe lumbar lordosis or severe scoliosis, one size longer rod may be chosen. If severe kyphosis is present, one size shorter rod should be chosen

two rods should then have a cross connector placed at the thoracic spine or proximal end of the rod (Fig. 18). The length of the modular rod is much easier to address, because the rod can be left a little long and then cut off as one progresses with deformity correction. The lumbar cross connector should always be added after partial correction to stabilize the system and always before the thoracic connector is loosened or moved. If the rod is too long, the thoracic connector can be moved distal and the rod cut off in situ. 33. When the single unit rod is used, the caudal end of the rod legs then are crossed over for insertion. The holes drilled in the pelvis should be palpated with a probe and their orientation carefully memorized. The hole that is most vertical has the rod leg inserted first, with that leg of the unit rod having to be anterior to the leg of the unit rod to be inserted last. By memorizing the direction, the leg of the unit rod is inserted for approximately half its length. Attention then is directed to the opposite hole, where it is again probed and its direction carefully memorized, and then the leg is directed in the proper direction. Each of the legs is impacted sequentially until they are driven down completely below the level of the bone of the superior iliac spine. In small children, or those with severe osteoporosis, it is extremely important to very carefully monitor the direction in which the legs of the rod are being impacted, which

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Fig. 17 The sagittal bend in the two rods should be aligned, so their contours match and the rods are perfectly perpendicular to the horizontal axis of the pelvis

Fig. 18 The two rods should then have a cross connector placed at the thoracic spine or proximal end of the rod

Fig. 19 After the rod is inserted, a fluoroscopy image should be obtained to document the correct position in the medullary canal of the ilium

often requires holding onto the rod with a device and helping direct the rod into the correct direction staying on the drilled hole. With weak bones, the rod may cut its own hole if impaction is not performed carefully. The distal end of the rod then is impacted fully into the pelvis until it is below the level of the posterosuperior iliac spine and should be lying in the gutter between the iliac spine and the lateral sacrum. After the rod is inserted, a fluoroscopy image should be obtained to document the correct position

in the medullary canal of the ilium (Fig. 19). If the rod leg is not in the correct position (Fig. 20), it must be repositioned. This usually means cutting the rod and removing the leg that is malpositioned and reinserting it and reattaching it to the remaining rod with rod connectors. 34. The rod is pushed to L5 using a rod pusher, and the wires are twisted and tightened (Fig. 21). It is extremely important to not try to push the rod down to the spine to see if the spine can be corrected and the rod is

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36.

37. Fig. 20 If the rod leg is not in the correct position, it must be repositioned

38.

39.

40.

Fig. 21 The rod is pushed to L5 using a rod pusher, and the wires are twisted and tightened. It is extremely important to NOT try to push the rod down to the spine to see if the spine can be corrected and the rod is the right length at this time

the right length at this time. This maneuver may cause the pelvis to fracture and the rod to lose its distal fixation. 35. The rod is pushed using a rod pusher to each sequential vertebral level, and the wire

41.

42.

is tightened to the rod sequentially (Fig. 22). Do not use the wires to pull the rod to the bone. The cut wires that protrude laterally (Fig. 23A) are now bent to the midline (Fig. 23B), and additional bone graft is added with bank bone until the rod is covered almost completely (Fig. 23C). The dry bank bone may be mixed with liquid undiluted Gentamycin using 10 mg per KG of body weight. This has been shown to reduce infection risk (Borkhuu et al. 2008). An alternative is to add vancomycin powder to the wound. By digital palpation of the rod, make sure there are no laterally protruding wires. A single wire may easily be missed if there is substantial bleeding before wound closure. Closure of the spinal fascia requires suturing so that the closure is watertight and no leaking or bleeding can occur from the deep hematoma. This leaking leads to a high likelihood of developing a subcutaneous hematoma, which causes wound leakage, and then developing an infection from the outside in. The subcutaneous tissue is closed to obliterate all dead space. No wound drains are needed. The skin is subsequently closed, and an occlusive dressing should be carefully applied to prevent wound contamination with stool as many of the children are incontinent of stool (Fig. 24). After dressing is applied, the child is turned into the supine position, and there is careful palpation of the abdomen, especially in the super pubic region and just to the medial side of the ilium. The anterior tip of the rod can be palpated if it has inadvertently cut a new track and is in the lower abdomen and has not stayed in the drilled holes. An anteroposterior pelvic radiograph is obtained, and if there is any question about the position of the rod, additional 30–40 right and left oblique radiographs of the pelvis are obtained to document that the rod is within the pelvis. A chest radiograph is obtained to document that there is no pneumothorax and also

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Fig. 22 The rod is pushed using a rod pusher to each sequential vertebral level, and the wire is tightened to the rod sequentially. Do not use the wires to pull the rod to the bone

to document the position of the rod postoperatively. 43. If the radiographs demonstrate adequate position, the child then is transferred to the intensive care unit. It is very important to continue with diligent, continuous monitoring throughout the whole postoperative period, especially the period of transfer to the intensive care unit. When the child is sitting well, full-length postoperative radiographs are obtained. Figure 25 is the single unit rod and, Fig. 26 is the modular unit rod.

Postoperative Care The child is kept in the intensive care unit and mobilized as tolerated. Oral feeding is initiated as soon as the bowels are functioning, but there should not be prolonged period of fasting. If the child does not tolerate intestinal feeds by the fifth postoperative day, central venous hyperalimentation should be started. No postoperative orthotic immobilization is required, and no special handling is necessary. Wheelchairs must be adjusted before the child uses them postoperatively because the significant change in body shape will cause high skin pressure areas with a risk of skin breakdown, which can then lead to deep infection.

Special Consideration for Correction of Kyphosis and Lordosis Some children with CP develop almost pure thoracic kyphosis or only severe lordosis. The indications for correction of these deformities are not well defined. If the deformity causes pain or difficulty sitting that impacts quality of life, surgical correction is indicated. The same instrumentation with cantilever correction can also be used for these deformities with some modifications. 1. Kyphosis – When kyphosis is isolated to the thoracic or thoracolumbar spine and there is no pelvic obliquity or lumbar scoliosis deformity, the instrumentation level does not need to extend into the pelvis. In this case, the cantilever correction should proceed from proximal to distal with the distal fixation ending in lumbar pedicle screws usually to L4 or L5 (Fig. 27), with the lumbar fixation using extended tab pedicle screws (Fig. 28). 2. Lordosis – Primary lordosis is the most difficult deformity to correct, and it is a clear contraindication for the use of the single unit rod as it is not possible to insert the rod because of the pelvic tilt (Fig. 29). This deformity also makes passing wires very difficult. A common cause of severe lordosis is following dorsal rhizotomy in which there is also poor or no posterior

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Fig. 23 The cut wires that protrude laterally (Fig. 23A) are now bent to the midline (Fig. 23B), and additional bone graft is added with bank bone until the rod is covered almost completely (Fig. 23C)

lamina for fixation. For these multiple reasons, pedicle screws are almost always the better option in the face of severe lordosis. Strong well-seated screws are required because pullout during correction is a common problem (Fig. 30). Very generous anterior release with

wedge resections of the disks is also indicated for severe deformity especially following dorsal rhizotomy as a large posterior correction will cause significant nerve root traction and prolonged sciatic or neuropathic leg pain. The correction of the lordosis will make a major

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Fig. 25 The postoperative radiograph of a the Single Unit rod

Fig. 24 The skin is subsequently closed, and an occlusive dressing should be carefully applied to prevent wound contamination with stool as many of the children are incon tinent of stool

change in the appearance of the child’s sitting posture (Fig. 31). This usually requires lumbar pedicle screws (Fig. 31c).

Anterior Spinal Release

Fig. 26 The postoperative radiograph of the Modular Unit rod

Indication Anterior spinal release is indicated for spinal curves that are excessively large, usually greater than 100 , and for release of severe lumbar lordosis or kyphosis. Very stiff curves of more than 50 , as defined by children who cannot side bend to bring the spinous processes to the midline, also require anterior release. With the use of the unit rod, anterior release is not required because of a concern about crankshaft deformity with growth. No anterior instrumentation is used, as this procedure always is done in combination with a posterior spinal fusion using a singular or modular unit rod. Both the anterior and posterior procedures may be done on the same day if the child is very healthy and the surgeon feels comfortable with this much surgery in 1 day. Our experience suggests that it is safer in very compromised children to separate the procedures by 1 week. Typically the anterior procedure is done first, and then 1 week later, the posterior procedure is performed. However, if there is an experienced team of surgeons doing both anterior

and posterior procedures on the same day, it is also a reasonable option.

Procedure 1. The exposure is determined by the length of the release to be performed. A thoracic

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Fig. 27 When the deformity is primary thoracic kyphosis, the cantilever correction should proceed from proximal to distal with the distal fixation ending in lumbar pedicle screws usually to L4 or L5

2.

3. 4. 5.

6. Fig. 28 The lumbar fixation is provided using extended tab pedicle screws

exposure is adequate for a release that will extend from the T10–T11 disk space up to the T3–T4 disks. A lumbar exposure is adequate for release from L1–T12 disks to the L4–L5 disks. Thoracolumbar exposures are required for releases crossing the T11–T12 disks. The side of the exposure is always

toward the apex of the scoliosis, or if there is no scoliosis, left-side exposure is easier to avoid the vena cava. If thoracic exposure is sufficient, then the exposure should be made through the ribs, which are two ribs cranial to the apex of the curve. Thoracolumbar exposure is made through the 10th rib bed (Fig. 32). A lumbar exposure typically is made through the bed of the 12th rib. After the level is chosen, an incision is made along the rib and carried anteriorly to the border of the rectus abdominis muscle and then longitudinally along the rectus abdominis muscle to the level. The ribs are exposed and subperiosteally dissected free (Fig. 33). The anterior osteocartilaginous junction is separated, and the rib is subperiosteally dissected leaving it attached posteriorly and then stripped as far posteriorly as possible and transected. The thoracic cavity is entered by opening the periosteum and pleura in the middle of the rib bed and extending it anteriorly. The incision is extended posteriorly to the area of the resection of the rib.

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Fig. 29 Primary lordosis is the most difficult deformity to correct, and it is a clear contraindication for the use of the single unit rod as it is not possible to insert the rod because of the pelvic tilt

Fig. 30 For these multiple reasons, pedicle screws are almost always the better option in the face of severe lordosis. Strong well-seated screws are required because pullout during correction is a common problem

7. If this is a thoracolumbar exposure, the chondral cartilage then is sharply transected longitudinally to where it ends, and it is gently opened using a blunt instrument for dissection at its caudal end. This, then, will enter the abdominal cavity, and the peritoneum should be dissected off the undersurface of the abdominal muscles. At the distal end of the 10th rib, the anterior insertion of the diaphragm is encountered beneath the split cartilage.

8. The anterior dissection then is carried down through the abdominal muscles in line with the incision to the lateral border of the abdominis rectus and can be carried along parallel to the rectus as far caudally as is needed. 9. The peritoneum then is dissected by blunt dissection off of the lateral and posterior abdominal cavity to enter the retroperitoneal space. 10. The retroperitoneal space is entered posterior to the kidneys and spleen on the left and posterior to the liver on the right side. At this time the anterior aspect of the spine can be palpated. 11. The retroperitoneal fat then is incised over the vertebrae, and using a blunt dissection, all the anterior longitudinal ligaments of the vertebrae are cleanly exposed. Segmental vessels are identified, hemoclips are applied, and the vessels are transected. 12. In the thoracic cavity, the pleura is incised over the spine, and the retropleural space is opened with gentle dissection over the anterior longitudinal ligament. Segmental vessels are identified, hemoclips are applied, and the vessels are transected (Fig. 34). 13. If a thoracolumbar exposure is required, the anterior origin of the diaphragm is identified under the split anterior cartilage of the 10th rib and incised at the border between the lateral third and medial two-thirds. Marker sutures are placed on each side of the diaphragmatic incision every 2 cm and cut in such a way that they can be identified as

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Fig. 31 The correction of the lordosis will make a major change in the appearance of the child’s sitting posture (Fig. 31a-b ). This usually requires lumbar pedicle screws (Fig. 31c) Fig. 32 Thoracolumbar exposure is made through the 10th rib bed

Fig. 33 The ribs are exposed and subperiosteally dissected free

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Fig. 34 In the thoracic cavity, the pleura is incised over the spine, and the retropleural space is opened with gentle dissection over the anterior longitudinal ligament. Segmental vessels are identified, hemoclips are applied, and the vessels are transected

14.

15. 16.

17.

markers for repair. Usually a pair of sutures is cut short and the next pair is cut long. The diaphragm is cut through its whole circumference, aiming to the middle of the spine so that the separation between the medial and lateral cruz of the diaphragm will be opened (Fig. 35). The spine now can be exposed with the anterior longitudinal ligament for the intended length. Utilizing Cobb elevators, the iliopsoas muscle can be elevated off the insertion on the bone, although care should be taken not to do subperiosteal dissection, which increases the bleeding. Segmental vessels are ligated or clipped at each level. The disk spaces are identified as the large, thicker areas on the spine and are incised anteriorly using a sharp knife (Fig. 36). All the disk material is eliminated with removal of a large wedge of all the end plate and some of the bone on the convex side of the scoliosis (Fig. 37B). Alternatively, if this is a severe lordosis, the anterior-based wedge is resected to allow the spine to close anteriorly. The posterior longitudinal ligament is left intact. For kyphotic deformities, there occasionally is a very thin disk in the front, sometimes even with a bony fusion, so bone burrs or rongeurs are necessary to make an osteotomy of the bone. In the thoracic spine, removal of the rib heads at two or more levels

Fig. 35 The diaphragm is cut through its whole circumference, aiming to the middle of the spine so that the separation between the medial and lateral cruz of the diaphragm will be opened

at the apex of the curve helps to provide flexibility. 18. The disk spaces are packed with Gelfoam material for hemostasis, and very thin pieces of the bone are applied at the borders. No attempt is made to pack the disk spaces with bone graft (Fig. 37C). 19. The wound is closed starting by using a running suture to close the posterior pleura over the spine. A suture is utilized, and the posterior aspect of the diaphragm is closed with

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thoracotomy wound. Muscle, subcutaneous, and skin closure follows. A chest tube is inserted into the thoracotomy wound before closure. Then, the patient is turned into the supine position for the posterior fusion.

Postoperative Care The chest tube is left in place for 2 or 3 days until the chest tube drainage is less than 50 ml per 8 h. There is no special attention except as one manages the posterior fusion, which is the major aspect of this procedure. Fig. 36 The disk spaces are identified as the large, thicker areas on the spine and are incised anteriorly using a sharp knife

Cross-References ▶ Anesthetic Management of Spine Fusion ▶ Surgical Management of Kyphosis and Hyperlordosis in Children with Cerebral Palsy ▶ Surgical Treatment of Scoliosis Due to Cerebral Palsy

References

Fig. 37 All the disk material is eliminated with removal of a large wedge of all the end plate (Fig 37A) and some of the bone on the convex side of the scoliosis (Fig. 37B). The disk spaces are packed with Gelfoam material for hemostasis, and very thin pieces of the bone are applied at the borders. No attempt is made to pack the disk spaces with bone graft (Fig. 37C)

a running suture to close the incision in the diaphragm to its anterior aspect. 20. Sutures are placed around the superior and inferior ribs and used to approximate the

Borkhuu B, Borowski A, Shah SA, Littleton AG, Dabney KW, Miller F (2008) Antibiotic-loaded allograft decreases the rate of acute deep wound infection after spinal fusion in cerebral palsy. Spine (Phila Pa 1976) 33:2300–2304 Dias RC, Miller F, Dabney K, Lipton G, Temple T (1996) Surgical correction of spinal deformity using a unit rod in children with cerebral palsy. J Pediatr Orthop 16:734–740 Smucker JD, Miller F (2001) Crankshaft effect after posterior spinal fusion and unit rod instrumentation in children with cerebral palsy. J Pediatr Orthop 21:108–112 Tsirikos AI, Chang WN, Dabney KW, Miller F (2004) Comparison of parents’ and caregivers’ satisfaction after spinal fusion in children with cerebral palsy. J Pediatr Orthop 24:54–58 Tsirikos AI, Lipton G, Chang WN, Dabney KW, Miller F (2008) Surgical correction of scoliosis in pediatric patients with cerebral palsy using the unit rod instrumentation. Spine (Phila Pa 1976) 33:1133–1140

Part XXII Hip

Hip Problems in Children with Cerebral Palsy: An Overview

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1874 Natural History, Etiology, and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1874 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Typical Posterior Lateral Dislocations in Children with Spasticity . . . . . . . . . . . . . . . . . . . Other Hip Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Femoral Torsional . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1875 1875 1877 1878

Complications in CP Hip Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1878 Surgical Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1878 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1878 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1879 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1879

Abstract

Children with cerebral palsy often develop subluxation or dislocation. It occurs most commonly in children aged 2–8 years but may occur until skeletal maturity. It is usually caused by abnormally strong or spastic pull of the adductor and hip flexor muscles which causes the femur to position in internal rotation, flexion, and adduction. Early identification with yearly hip radiographs which are monitored using the migration percent (MP) will identify the condition early, when

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_124

treatment has a better outcome. Adductor and flexor muscle lengthening in the young child when the MP is between 25% and 60% has a good chance of resolving the subluxation. For those detected later or failed soft tissue lengthening, hip reconstruction with pelvic and femoral osteotomy usually provides a good outcome. For those hips which develop a complete dislocation and severe arthritis, options for treatment include total hip replacement, interposition arthroplasty, or proximal femoral resection. Although most hips are posterior lateral in the direction of subluxation, it is important to diagnose those which are anterior or due to hypotonia. The treatment for these requires a different approach. Some children may also have developmental hip 1873

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dislocation (DDH) which tends to respond very differently than CP hip dislocation. In very young children under age 2 years, it is important to determine if the hip displacement is due to DDH or CP spasticity. Complications from treating CP hip displacement include recurrence as the most common complication, followed by wound infection, delayed femoral union, heterotopic ossification, and avascular necrosis. Keywords

Cerebral palsy · Hip subluxation · Hip dislocation · Hip reconstruction · Femoral anteversion

F. Miller

has a poor description of specific patterns. With the introduction of the Gross Motor Function Classification System (GMFCS) (Wood and Rosenbaum 2000) and the wide acceptance of the migration percent (MP) (Reimers 1980) as the primary radiograph measure, many of the reports have more consistent data reported. A substantial body of this literature addresses the natural history of the problem of hip dysplasia, and its etiology has been fairly well understood, but the relative importance of different factors is still debatable. The goal of this chapter is to provide a general overview of the etiology and management plan for hip problems in children with CP.

Introduction

Natural History, Etiology, and Pathophysiology

The hip joint is the largest joint in the body and is the joint that causes the most problems both from a functional perspective and at the level of walking, sitting, and lying in children with cerebral palsy (CP). Hips in children with CP are normal at birth, and the problems develop slowly as the children grow and deform under the influence of abnormal forces caused by CP. A second group of children with CP does not actually develop deformity; however, the infantile shape of their proximal femur does not resolve because there is not enough normal derotational force present. These children who may have good ambulatory ability develop contractures and increased abnormal forces that lead to dislocation and dysplasia, or alternatively, they fail to resolve the infantile torsional malalignment. After addressing the concerns of equinus contractures in children with CP, hip problems are the next main area of concern to orthopedists treating these children. The treatment of hip problems has the largest literature base in the area of orthopedic management of CP. A review of the abstract listings in the National Library of Medicine revealed 496 references published from 1963 to 2000 that address hip problems in children with CP. From 2000 to 2016, there were an additional 923 further listings. Although the literature is extensive, much of it does not include any standardized control or standardized radiographic measurements and

The etiology of hip problems in CP is directly related to abnormal hip force magnitude and direction (Miller et al. 1999) (see ▶ Chap. 128, “Etiology of Hip Displacement in Children with Cerebral Palsy”). Since these hips are normal at birth, our goal is to monitor and detect the early changes of hip subluxation and dysplasia. The factors which are the highest risk factors for developing hip subluxation are the GMFCS level and the child’s age (Pruszczynski et al. 2015). The process of developing the hip subluxation occurs gradually in the child with spasticity. It is also important to note that children with hypotonia who have increased hip range of motion also develop hip subluxation and must be monitored. The hip displacement in hypotonia is somewhat different, and the natural history is less well defined. In children with spasticity, often but not always, there is restricted hip abduction, followed by lateral migration of the femoral head in the acetabulum, then acetabular dysplasia, followed by complete hip dislocation, and development of severe hip joint degenerative changes (Fig. 1). Pain frequently develops some time during this process although it may be a very late symptom (see ▶ Chap. 129, “Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy”). Based on understanding the natural history and risk factor, population-based surveillance programs can be carried out to identify CP hip subluxation at an

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Fig. 1 The hip of the child at age 2 (a) is almost normal except for normal mild coxa valga. By age 5, the coxa valga is more apparent, and now there is significant lateral subluxation of the femoral head from the acetabulum

which is also developing lateral dysplasia (b). With no treatment, most of these hips will proceed to develop a complete dislocation with severe arthritic changes (c)

early stage (Pruszczynski et al. 2015). The screening programs are usually focused on young children GMFCS III–V under age 8 starting around age 2, which include the high-risk ages. The risk of subluxation, however, may continue through adolescence until skeletal maturity. The current DuPont Hospital Surveillance program is based on the age of the child, GMFCS level, and the child’s previous hip condition (Table 1). The key surveillance measure is getting a supine anteriorposterior pelvis radiograph in a consistent position (Fig. 2). When surveillance programs have been applied and early subluxation has been addressed in a whole population, there is a significant reduction in hip dislocation and in the number of hips needing reconstruction. This has been best demonstrated in Sweden (Hagglund et al. 2014).

in early hip subluxation is identified with a surveillance program, early treatment should be considered. Mild subluxation is defined by MP between 25% and 40%, moderate subluxation is 40–60%, and severe subluxation is 60–90%. More than 90% is usually considered a dislocated hip. Treatment in the early stages of mild and moderate subluxation is considered prophylactic. Adductor, iliopsoas, and hamstring lengthenings as a method to produce better soft tissue balancing are a widely accepted procedure for mild and moderate hip subluxation treatment in young children under age 8 (see ▶ Chap. 130, “Prophylactic Treatment of Hip Subluxation in Children with Cerebral Palsy”) (Table 1). The outcome of early soft tissue balancing procedures varies based on the GMFCS level and the severity of the initial subluxation (Presedo et al. 2005; Shore et al. 2012). Especially in the GMFCS IV and V level of function, the outcome varies widely from a 10% good outcome (Shore et al. 2012) to a 60% good outcome (Presedo et al. 2005). This difference is mostly due to the surgical dose which is applied, meaning not enough muscle release leads to a less good outcome. Another option for managing these early subluxations is to do early femoral varus osteotomy; however, there is no good data to demonstrate a difference in outcome compared to muscle surgery alone. The use of

Treatment Typical Posterior Lateral Dislocations in Children with Spasticity The most common direction of hip dysplasia and dislocation is posterior lateral with the leg’s predominant posture being flexed, adducted, and internally rotated. When this typical pattern

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Table 1 Hip monitoring protocol for cerebral palsy

Hip Monitoring Protocol for Cerebral Palsy Goal: To monitor all children with CP to prevent hip dislocation. Monitoring schedule: GMFCS I and II (full ambulation without assistive device) – One X-ray after 2 years of age – no other needed if normal Exception: Hemiplegia type 4 (with hip involvement, rotation or flexion) X-ray every 2 years from age 8 to skeletal maturity GMFCS III, IV, & V (non-ambulatory to walking with assistive device) Until age 8 – X-ray every year, X-ray every 6 month – for children with severe spasticity or MP >25 Age 8 to skeletal maturity – if previous X-ray normal – every 2 years If previous X-ray MP > then 25% – every year X-ray

MP

Treatment recommendations: Up to age 8 years: MP 30 to 60% and hip abduction less than 30º with hip and knee extended – STR MP 30 to 60% and hip abduction over than 30º with hip and knee extended – observe MP > 60% and healthy child – reconstruction MP > 60% and hip abduction < 30º and child with multiple medical problems – STR Migration Percent (MP)- A single AP supine pelvis x-ray with the child lying in a relaxed, comfortable neutral Over age 8 years: position with about (10°) abduction, may MP > 40% - Abduction less than 45º - reconstruction have a small blanket roll under the knees MP > 50% - Abduction over 45º - reconstruction to slightly flex the hips and knees if this Dislocated hip – Painful but not severe degenerative changes – reconstruction makes the child more comfortable. Hip Dislocated hip – Painful with severe degenerative changes – palliative procedure rotation should be approximately neutral but do not force into any position.

Protocol for CP hip X-ray Migration Percent (MP) – A single AP supine pelvis X-ray with the child lying in a relaxed, comfortable neutral position with about (5-10°) abduction, may have a small blanket roll under the knees to slightly flex the hips and knees if this makes the child more comfortable. Hip rotation should be approximately neutral but do not force into any position.

Fig. 2 Protocol for CP hip X-ray

botulinum toxin has been advocated to manage hip subluxation; however, based on a randomized controlled study, over time, the botulinum toxin did not have a positive impact on hip displacement

(Graham et al. 2008). Another option some surgeons advocate is careful monitoring until the hips reach moderate to severe subluxation and then proceeding with a reconstruction.

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Reconstruction of the hip is required in the older patient, typically above 8 years, and the skeletal maturity limits the amount of acetabular bone remodeling. This requires a femoral varus shortening osteotomy with an acetabular reconstruction (see ▶ Chap. 131, “Hip Reconstruction in Children with Cerebral Palsy”). The indications are hip subluxation in the moderate to severe range or dislocations which do not have severe fixed deformity or degenerative arthritis. The ideal age for this procedure is between 9 and 14 years old in a hip that has moderate to severe subluxation. Hips in this age and severity range tend to have more than 95% good outcome (Oh et al. 2007). Hip reconstruction can be extended to patients with closed growth plates and more severe deformities, with reduction in pain and good patient satisfaction (Rutz et al. 2015). The procedure in this age group is more technically demanding and has a higher failure rate in our hands. There are hips which are too severely degenerated or have too much dysplasia to do a reduction and reconstruction. These hips require a palliative or salvage procedure. There are a large number of possible options. For children who have functional ambulatory potential which they want to maintain, a standard total hip replacement is the best option. This has been done in young and older adults with CP with good long-term outcomes (Raphael et al. 2010). For small children with many medical conditions especially if they have or had decubitus ulcers, a proximal femoral resection with the Castle technique is an option. It may take 1 year to develop full pain relief (Boldingh et al. 2014). Another option is a proximal resection and an interposition arthroplasty using a shoulder prosthesis. This provides quick pain relief, and there is no attempt to keep it reduced in the joint like a standard total hip replacement (Gabos et al. 1999). Other options have included a proximal femoral valgus osteotomy to direct the femoral head away from the acetabulum. If the main problem is poor limb positon, this is a good option. If the problem is pain, this may not provide reliable pain relief. Another variation on the valgus osteotomy was made by McHale in which the femoral head is

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resected and the lesser trochanter is directed into the acetabulum (McHale et al. 1990). There have not been many independent reports on this procedure, and although many surgeons report antidotal cases who do very well, our experience has been colored by seeing a number of failures who do not get pain relief (see ▶ Chap. 134, “Hypotonic and Special Hip Problems in Cerebral Palsy”).

Other Hip Deformities Although more than 95% of the hip problems are due to the typical posterior lateral hip displacement, some hips do go into the anterior direction. The leg posture for these hips may be in external rotation hip and knee extension or external rotation hip abducted, flexed, and knee flexion (see ▶ Chap. 132, “Palliative or Salvage Hip Management in Children with Cerebral Palsy”). The surgical treatment for these hips is quite different, as one has to focus on correcting severe anterior pelvic deficiency. This usually requires a Pemberton-type pelvic osteotomy. The important problem is severe hip abduction asymmetry which leads to pelvic obliquity with a pattern called the windblown condition. This windblown pattern may be primary with the hips usually due to asymmetric contracture, motor control, or tone. When these asymmetric hip contractures cause the windblown deformity and is the cause of the pelvic obliquity, it is called infrapelvic pelvic obliquity. In other patients, the spinal deformity, usually scoliosis, will drive the pelvis into obliquity, and this is called suprapelvic pelvic obliquity. In some patients, there is contribution from both conditions. For those patients who are young, 8 years old, and have suprapelvic pelvic obliquity, the spinal deformity needs to be corrected first, or it will continue to drive more pelvic obliquity and might risk driving the hip out of the socket especially on the upside of the hip, if the hip subluxation is addressed first. There is also a group of children, typically over age 9, that has a

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combination of fixed hip contractures and scoliosis; in this case, the scoliosis should be corrected first followed by correction of the hip problem 4–6 months later (see ▶ Chap. 135, “Femoral Anteversion in Children with Cerebral Palsy”). Children with CP may also develop other hip pathologies such as subluxation and dislocation due to low hip force environment typical in children with hypotonic CP. The management of the hypotonic hip is more variable and less well studied; therefore, the management protocol is less clear. It is important though not to allow the hip to dislocate especially in children who are walking, as this will negatively impact their ability to walk. Usually a reconstruction of both the acetabulum and femur is required (see ▶ Chap. 133, “Anterior Dislocation of the Hip in Cerebral Palsy”). Children with CP may also develop developmental dislocation of the hip (DDH), Perthes disease, and slipped capital femoral epiphysis (SCFE). These conditions are impacted by the underlying neurologic conditions which have to be considered in planning management.

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one reason for the possibility of many different complications, most of which are relatively uncommon. The most common complication is recurrent hip subluxation after the initial treatment. This requires that all hips should continue to be monitored with annual or every 2-year radiographs until they are skeletally mature. Children and adolescents who develop windblown hips are at especially high risk of developing recurrent hip subluxation or dislocation, on rare occasions even after skeletal maturity. Treatment for the recurrent subluxation is usually successful. After the femoral osteotomy, there can be a delayed union and on very rare occasions a nonunion. Sometimes this is associated with a mild infection; however, wound infections which are most common in the femoral osteotomy site, almost never, preclude healing of the osteotomy. These infections are treated by leaving the wound open until the bone has healed, then the plate is removed, and the wound heals quickly. Heterotopic ossification and avascular necrosis are other rare problems that are occasionally encountered (see ▶ Chap. 137, “Complications of Hip Treatment in Children with Cerebral Palsy”).

Femoral Torsional Babies are born with increased femoral anteversion, and under the influence of normal muscle pull as the child starts to walk, the femur develops its normal 15–20% of anteversion. Children with CP often walk late and have abnormal motor control, with muscles increasing the internal rotation of the hip. This internal rotation force may make the femoral internal torsion worse, but usually it just precludes it is getting better. In middle childhood if this internal rotation is still severe, a femoral derotation osteotomy may be required (see ▶ Chap. 136, “Windblown Hip Deformity and Hip Contractures in Cerebral Palsy”).

Complications in CP Hip Management There are many different patterns and severity levels of children with CP which each impacts the hips in different ways. This high variation is

Surgical Procedures The surgical procedures to address the problems at the hips are generally pretty unique to children with CP. For this reason, a detailed surgical technique guide is also included with this text (see ▶ Chap. 138, “Surgical Atlas of Cerebral Palsy Hip Procedures”).

Conclusion Management of the hip problems in children with CP has a large literature base and a welldeveloped treatment algorithm that is most widely followed. The risks and complications are relatively uncommon and when they occur are easy to treat. By careful monitoring with a surveillance program and early intervention, continued monitoring for recurrence, and when prompt response occurs, all young adults should

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have located hips that do not impair sitting or standing function.

Cross-References ▶ Anterior Dislocation of the Hip in Cerebral Palsy ▶ Complications of Hip Treatment in Children with Cerebral Palsy ▶ Etiology of Hip Displacement in Children with Cerebral Palsy ▶ Femoral Anteversion in Children with Cerebral Palsy ▶ Hip Reconstruction in Children with Cerebral Palsy ▶ Hypotonic and Special Hip Problems in Cerebral Palsy ▶ Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy ▶ Palliative or Salvage Hip Management in Children with Cerebral Palsy ▶ Prophylactic Treatment of Hip Subluxation in Children with Cerebral Palsy ▶ Surgical Atlas of Cerebral Palsy Hip Procedures ▶ Windblown Hip Deformity and Hip Contractures in Cerebral Palsy

References Boldingh EJ, Bouwhuis CB, van der HeijdenMaessen HC, Bos CF, Lankhorst GJ (2014) Palliative hip surgery in severe cerebral palsy: a systematic review. J Pediatr Orthop B 23:86–92 Gabos PG, Miller F, Galban MA, Gupta GG, Dabney K (1999) Prosthetic interposition arthroplasty for the palliative treatment of end-stage spastic hip disease in nonambulatory patients with cerebral palsy. J Pediatr Orthop 19:796–804

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Graham HK, Boyd R, Carlin JB, Dobson F, Lowe K, Nattrass G, Thomason P, Wolfe R, Reddihough D (2008) Does botulinum toxin a combined with bracing prevent hip displacement in children with cerebral palsy and “hips at risk”? A randomized, controlled trial. J Bone Joint Surg Am 90:23–33 Hagglund G, Alriksson-Schmidt A, Lauge-Pedersen H, Rodby-Bousquet E, Wagner P, Westbom L (2014) Prevention of dislocation of the hip in children with cerebral palsy: 20-year results of a population-based prevention programme. Bone Joint J 96-B:1546–1552 McHale KA, Bagg M, Nason SS (1990) Treatment of the chronically dislocated hip in adolescents with cerebral palsy with femoral head resection and subtrochanteric valgus osteotomy. J Pediatr Orthop 10:504–509 Miller F, Slomczykowski M, Cope R, Lipton GE (1999) Computer modeling of the pathomechanics of spastic hip dislocation in children. J Pediatr Orthop 19:486–492 Oh CW, Presedo A, Dabney KW, Miller F (2007) Factors affecting femoral varus osteotomy in cerebral palsy: a long-term result over 10 years. J Pediatr Orthop B 16:23–30 Presedo A, Oh CW, Dabney KW, Miller F (2005) Softtissue releases to treat spastic hip subluxation in children with cerebral palsy. J Bone Joint Surg Am 87:832–841 Pruszczynski B, Sees J, Miller F (2015) Risk factors for hip displacement in children with cerebral palsy: systematic review. J Pediatr Orthop 36(8):829–833 Raphael BS, Dines JS, Akerman M, Root L (2010) Longterm followup of total hip arthroplasty in patients with cerebral palsy. Clin Orthop Relat Res 468:1845–1854 Reimers J (1980) The stability of the hip in children. A radiological study of the results of muscle surgery in cerebral palsy. Acta Orthop Scand Suppl 184:1–100 Rutz E, Vavken P, Camathias C, Haase C, Junemann S, Brunner R (2015) Long-term results and outcome predictors in one-stage hip reconstruction in children with cerebral palsy. J Bone Joint Surg Am 97(6):500 Shore BJ, Yu X, Desai S, Selber P, Wolfe R, Graham HK (2012) Adductor surgery to prevent hip displacement in children with cerebral palsy: the predictive role of the gross motor function classification system. J Bone Joint Surg Am 94:326–334 Wood E, Rosenbaum P (2000) The gross motor function classification system for cerebral palsy: a study of reliability and stability over time. Dev Med Child Neurol 42:292–296

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1882 Natural History and Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1882 Posterior-Superior Hip Subluxation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Secondary Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Tertiary Changes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Treatment and Outcomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1889 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1889 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1890

Abstract

The natural history of hip subluxation and dislocation in children with cerebral palsy shows a strong association with increasing severity of cerebral palsy best assessed with Gross Motor Function Classification System (GMFCS) grading. GMFCS IV and V have the highest risk of developing subluxation, and the highest risk is between ages 2 and 6 with reduced risk through adolescent growth. Children who have severe spasticity or are hypotonic also have higher risk. The primary cause of the hip displacement is spastic muscles causing the hip to be positioned most of the time in flexion, adduction, and

internal rotation. This position with the high muscle force of spasticity causes the hip to displace in the typical posterior superior direction causing acetabular dysplasia. As the dysplasia increases, the acetabulum opens further, and the femoral head completely displaces from the hip joint. Without treatment, the hip completely dislocates and then develops progressive deformity and arthritis. This process typically causes pain with movement of the hip initially and may then leads to constant pain. The amount of pain experienced by this process is variable and maybe pain free until there is very severe destructive arthritis in hip joint. Keywords

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_125

Cerebral palsy · Subluxated hip · Dislocated hip · Acetabular dysplasia · Etiology · Hip arthritis · Hip pain 1881

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Introduction

Natural History and Pathophysiology

The etiology of hip displacement in children with CP is multifactorial with the risk of increasing displacement being most closely related to the motor function best characterized by the Gross Motor Function Classification System (GMFCS) level (Pruszczynski et al. 2015). This relationship means that those children with more severe motor disability and the most impaired motor control also have the highest risk of hip displacement. The one exception to this trend is the higher risk at adolescence growth in children with type IV hemiplegia (Abousamra et al. 2015; Rutz et al. 2012). This group of children is mostly GMFCS I or II, and the hip dysplasia develops at an older age. We will discuss the possible mechanical implication of this group later in this chapter. In addition to severe motor involvement as graded by GMFCS, there is also a clear long-recognized association with increased spasticity (16). However, as it has also been demonstrated by several hip surveillance programs, measures of spasticity and contractures are not good screening tools because children with hypotonia and high hip range of motion also develop displacement (Wynter et al. 2011; Pruszczynski et al. 2015; Larnert et al. 2014; Hagglund et al. 2007).The second most important factor in the risk of hip displacement is the age of the child, with the risk decreasing as the child gets older and the skeleton is more mature (Pruszczynski et al. 2015; Wynter et al. 2015). The etiology of the hip displacement in children with CP is multifactorial and is therefore a lot like the weather in which many factors impact the immediate local development at a specific time and place. I will try to address these multiple factors and then make some attempt to conceptualize how they may interact. Conceptualizing the etiology is important to understanding natural history and the importance of surveillance (▶ Chap. 129, “Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy”). The etiology understanding is also the key to understanding importance of dosing and timing in prophylactic treatment (▶ Chap. 130, “Prophylactic Treatment of Hip Subluxation in Children with Cerebral Palsy”).

The risk of developing hip problems in children with CP is clearly most directly related to the severity of the CP as assessed by the GMFCS (Pruszczynski et al. 2015). The pattern and prognosis are also impacted by children’s level of muscle tone-based spasticity or hypotonic. The spastic (hypertonic) group should also include children with movement disorders such as athetosis and dystonia. The hypertonic hips can be subdivided further by the direction of the dysplasia or the abnormal force into posteriorsuperior, anterior, and inferior and, additionally, by several contracture patterns that may be independent of or concurrent with dysplastic hips. These contracture patterns include windblown hips and hyperabducted hips. The hypotonic hips in children with CP are a little more diffuse and are harder to further categorize.

Posterior-Superior Hip Subluxation The most common dysplastic hip problems in children with hypertonia or spasticity are posterosuperior hip subluxation, dislocation, or dysplasia (Cooke et al. 1989). These problems comprise the typical spastic hip dysplasia (SHD), which is discussed in most of the literature. Based on an extensive review by Cooke (Cooke et al. 1989), in which attention was paid to the specific pattern of dislocation, 98–99% of spastic children with hip subluxation or dislocation have this typical posterosuperior pattern, but this pattern has variation from anterior lateral, direct lateral to posterior lateral.

Etiology The etiology of spastic hip disease has been worked out fairly clearly both through clinical review and, more importantly, through modeling (de Windt et al. 1984; Miller et al. 1999). The concept of an abnormal force caused by adductor muscles was first suggested in a paper by Keats in 1963 (Keats 1972) and was the basis upon which

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he advocated doing adductor lengthening to prevent the spastic hip at risk from dislocating. Since that time, there have been many other clinical studies in which different primary etiologies for spastic hip disease were presented. These etiologies include femoral neck valgus as a primary cause, (Eilert and MacEwen 1977; Fettweis 1979; Fujiwara et al. 1976; Hoffer et al. 1985; Vizkelety et al. 1991) and in one study, the femoral valgus was believed to be the direct cause of the dislocation, but the adductor spasticity and weak gluteus medius were believed to cause the valgus (Heimkes et al. 1992). Femoral anteversion has also been indicated as a primary or contributing cause by numerous authors (Eilert and MacEwen 1977; Fettweis 1979; Fujiwara et al. 1976; Hoffer et al. 1985; Vizkelety et al. 1991). Various muscle contractures and spasticity have been felt to be either contributing or primary cause, with the adductor muscles being mentioned in 59 different articles as the primary cause of spastic hip dislocation. Other muscles that have been indicted in the literature are the iliopsoas (Lundy et al. 1998; Miller et al. 1997a, b) and hamstrings (Miller et al. 1997a, b; Feldkamp and Denker 1989; Mubarak et al. 1992). Another common perception of the cause of hip subluxation is acetabular dysplasia, or malrotation, listed in 12 separate references. Femoral head deformity has been noted as an etiology in the dislocation as well. Based on this literature, the general consensus is that the adductor muscles are the primary etiology in the cause of spastic hip subluxation. The most important clinical evaluation of the etiology of spastic hip subluxation was performed by Reimers and published in 1980 (Reimers 1980). In this study, he evaluated many different types of muscle surgery and concluded that the primary etiology of hip subluxation was abnormal force created by the adductor muscles. This primary etiology was followed by the influence of the hamstrings and, last, by the iliopsoas, which had the least direct effect. To promote a better understanding, modeling studies have been used to better delineate the contributions of specific anatomical aspects of the hip (de Windt et al. 1984; Miller et al. 1999). Computer modeling can determine the impact of the forces by

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constructing a mathematical model of each muscle and having this model in its anatomically correct place in three-dimensional space. The model then allows the muscles to contract to generate force as well as to adjust the muscle’s fiber length to simulate contracture. The muscle model also allows altering femoral neck-shaft angles so that varus and valgus, as well as various degrees of anteversion, can be modeled. Based on this mathematical model, in evaluating a large number of different variations, it is clearly demonstrated that the problems in the spastic hip are due to two specifically different elements of the pathomechanics (Miller et al. 1999). The two elements of abnormality in the spastic hip are a hip joint reaction force that is too high and a force vector in the wrong direction in the spastic hip, placing an abnormal force on the developing acetabulum. The etiology of the high hip joint reaction force appears to occur because there is too much co-contraction of muscles, which is clear to clinicians who examine hips. This co-contraction in which hip flexors, hamstrings, adductors, and abductors are all contracting at the same time generates forces substantially higher than children generate standing on the hip. The etiology of the hip joint reaction force vector being in the wrong direction comes primarily from the position of the femur relative to the acetabulum. The posterosuperior dislocation pattern is caused when the hip is positioned in adduction and flexion (Fig. 1). This finding is completely consistent with the clinical experience in which the vast majority of subluxated hips and dysplastic hips are positioned in adduction and some degree of flexion. The direct cause of this hip positioning is an abnormal pattern of muscle length and contraction force. The primary muscle that causes adduction and flexion is the adductor longus; however, this is closely followed by the gracilis and the medial hamstrings and then the adductor brevis. Based on modeling showing the importance of both hip joint reaction force magnitude and direction, clinical studies would suggest that the net vector direction is likely the most important element. There has been a report comparing children who had rhizotomy or intrathecal baclofen

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Fig. 1 The hip joint reaction force is a vector with both magnitude and direction. Both aspects of the hip reaction force are very sensitive to the position of the hip joint and the level of muscle contraction.3 This hip position and muscle force sensitivity are clearly demonstrated on a mathematical model of the hip force with the normal leg lying in the physiologic position (a, Position B), where the force is low and centrally directed (b, Position B, Vector 1) compared with the spastic hip in the same position (b, Position B, Vector 3), and the spastic hip lying in the typical spastic position (Position A) where the force is higher and directed more posterosuperiorly (b, Position A, Vector 2). This clearly demonstrates a low magnitude and a superomedial direction of the vector in the normal hip (b, Position B, Vector 1). The spastic hip in the typical spastic position has a somewhat higher magnitude, but the direction has shifted to be more posterior and very lateral, clearly showing why these hips dislocate (b, Position A, Vector 2). If the hip is forced into the physiologic position, such as with the use of a strong

orthotic, the magnitude becomes very high although the direction is better than with the spastic position. This high magnitude would likely cause severe damage to the hip joint, and this is the reason forceful bracing should not be used on the hips of young children. The modeling can also be used to evaluate the impact of different combinations of surgery (c). The spastic hip in the spastic position starts with a high force (c, Position A, Vector 1). By doing muscles lengthenings but leaving the hip in the same position, the force has only a slight reduction (c, Position A, Vector 3), and by adding a varus osteotomy but not changing the position, the force is again only slightly reduced but still poorly directed (c, Position A, Vector 4). If the position of the limb is changed after a muscle lengthening procedure, the force vector is reduced and normally directed (c, Position B, Vector 2). This modeling shows the importance of force reduction by muscle lengthening and the importance of correct limb positioning

therapy to reduce tone and thereby reducing the hip joint reaction force. This study found that neither treatment reduced the need for hip reconstruction, although it is not clear if this would make an impact if the tone was reduced very early in life (Silva et al. 2012). Also based on surveillance studies with low tone, those with low hip joint reaction force still develop hip displacement.

Based on modeling of the etiology of posterosuperior subluxation, it is clear that the hip must be positioned so that it is anatomically normal, with some abduction near extension. Also, the hip joint reaction vector must be directed into the central and medial aspect of the hip joint, but in such a way that the hip joint reaction force is not too high or too low. Based on the mathematical model of the muscle, when muscle contractures or

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spasticity of the adductor muscles is modeled, the only way this positioning can be obtained is by lengthening the adductor muscles. If the hip is forced into abduction and placed so that the hip joint reaction force vector is in the proper direction, the very high force magnitude would cause damage to the hip joint. This damage has been reported in a clinical review report of insensate spastic hips in children with spinal injuries (Rink and Miller 1990). In summary, the primary pathology of the most common pattern of posterosuperior subluxation in the spastic hip is confirmed by both clinical evaluation (Reimers 1980) and mathematical modeling (Miller et al. 1999) to be caused by overactivity of the adductor longus and gracilis. Secondary deforming forces are the iliopsoas, hamstrings, and adductor brevis, followed by the much less common but still deforming force muscles, the adductor magnus and pectineus.

Secondary Pathology The primary pathology is the process that initiates the deformity; however, the hip tries to respond to these pathomechanics. The anatomical pathology that develops because of these pathomechanics is the femoral head starts to migrate posteriorly,

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laterally, and superiorly in the acetabulum under the influence of the leg being positioned in adduction, flexion, and often internal rotation. These movement and abnormal force cause the acetabular rim to become deformed, opening up and developing a channel that is directed posterosuperiorly (Brunner et al. 1997). The decreased medial pressure allows the triradiate cartilage to grow wider, thereby causing a widening appearance of the teardrop on radiographs (Kahle and Coleman 1992). Because the force in the femoral epiphysis is increased on the medial aspect of the epiphysis with a large lateral shear force, the femur responds by developing a valgus femoral neck-shaft angle. Therefore, the etiology of the femoral neck-shaft angle is another response to the abnormal pathomechanics and position of the femoral head in the acetabulum; however, it is not a primary cause of hip subluxation (Fig. 2). The etiology of this femoral neckshaft angle has been studied extensively using modeling, specifically finite element analysis of the developing growth plate (Ribble et al. 1998). Based on this finite element analysis, in which it is presumed that the growth plate wants to decrease its shear force, the developing hip joint will grow into valgus so the epiphysis will be at a right angle to the resultant joint reaction force vector. Because of the pathology, the only way that a

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Fig. 2 The degree of femoral neck valgus is largely determined by the force the proximal femur encounters during the childhood growth period. Based on the appearance of a completely flaccid and paralyzed hip, there is probably an approximately 150 neck-shaft angle as the genetic blueprint from which this alternation is made. Also, an infant starts with approximately 150 of femoral neck-shaft

valgus (a). By the time a child has been walking for 1 year at aged 2 years, the femoral neck is about 130 (b); however, for a very spastic nonambulatory child, the femur may increase the valgus to 170 (c). Apparently the femur wants to decrease shear stress in the growth plate so it will grow to be at right angles to the principal force as experienced over time in the capital femoral epiphysis

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femoral head and neck will grow into its anatomically normal degree of varus is through the influence of active standing with active abductor muscle moment (Fig. 2). It is important to note that standing by itself is not enough, and it does require active abductor force to prevent the femoral neck from going into valgus. Some of the most severe coxa valga comes from the hypotonic child who is able to do assisted walking. As the child’s hips become more valgus, the child often increases the wide based gait with hip abduction as a way to compensate, which only drives the neck into more valgus (Fig. 3). Another example of these valgus growth phenomena is the abducted hip in the windblown syndrome, which is always in as much or more valgus then the opposite side which is adducted and dislocated (Fig. 4). It is also clear that the coxa valga, which is caused by the pathologic force milieu, does become another risk factor for hip displacement in the environment where the hip is also in adduction. A continued high degree of anteversion is another aspect of the secondary pathology of hip

Fig. 3 This is an 8-year-old boy with generalized hypotonia who is able to do assisted household walking. This demonstrates the severe coxa valga which occurs when there is weight bearing but no active abductor force

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subluxation. This anteversion is believed to be secondary to the anteversion of infancy, which does not resolve because the normal forces on the hip joint are not present. Documentation that this anteversion gets worse under the influence of spastic muscles is poor. Modeling studies in this area have been difficult to perform and, at this stage, are not very definitive. Clinical studies suggest that the primary cause of hip subluxation is failure to resolve anteversion (Bobroff et al. 1999); however, there is some suggestion that if anteversion is corrected in very young children (less than 4 years) it may recur (Brunner and Baumann 1997). There is also documentation that the coxa valga and amount of anteversion are not related suggesting that the force drivers for the anteversion are different than for the coxa valga (Gose et al. 2010). Another secondary change that progressively becomes worse is when the hip adductor spasticity maintains the hip in a flexed, adducted, and internally rotated position, the muscles gradually develop more contracture. These contractures occur especially in the hip adductors, flexors, internal rotators, and often hamstrings. At the same time, the hip abductors and extensors tend to become overstretched and less effective in their ability to contract. The abnormal force direction also causes eccentric ossification of the femoral epiphysis, often with some medial flattening, especially as the hip starts to subluxate (Fig. 5). The evolution of the spastic hip is one of the progressions especially when the combination of spasticity, anteversion, poor motor control, and coxa valga all foster positioning the hip in flexion adduction and internal rotation. This creates a worsening net posterior superior force vector. When this combination occurs in the young child under aged 6 where the acetabulum has a large component of cartilage, hip subluxation with acetabular deformity occurs. This easily explains the observe risk of child with limited motor control (GMFCS IVor V) and young age (less than 6) being at the highest risk of hip displacement (Pruszczynski et al. 2015). The importance of the weak acetabulum can be appreciated when one considers the type of hip dysplasia which occurs when an otherwise normal child develops

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Fig. 4 This is a 10-year-old boy with spasticity, GMFCS V, who has a severe windblown hip with a dislocation on the right side and a severe fixed abducted hip on the left side. He has had no medical care and is no longer able to be placed into sitting position. Note that he has severe bilateral coxa valga with the abducted left hip

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having more severe coxa valga than the dislocated right hip. This shows that the coxa valga is not the direct cause of the hip dislocation but is a symptom of the severe abnormal mechanical environment driving hips into either extreme of adduction and subsequent hip dislocation or into severe abduction leading to a fixed abducted hip

The etiology of anterior hip dislocation is likely the same as with posterior; however, the posture which becomes predominant is hip extension with external rotation of the lower extremity. This may develop in a child who has hyper extensor posturing for a prolong period of time. This leads to anterior acetabular wall deformity and contractures with either knee flexed and hip external or hip and knee extension with external rotation contractures. The rare inferior hip dislocation is a magnification of this posture in which hyper abduction is added. This can be so severe that the leg appears to be 180 degrees malrotation.

Tertiary Changes

Fig. 5 The eccentric loading of the femoral head causes the epiphysis to develop eccentrically with medial flattening and increased lateral growth

severe anoxic insult and is severely spastic. In this case, the acetabulum is strong enough to resist deforming however the femoral head develop defects (Fig. 6).

The late changes of spastic hip disease include an acetabulum that becomes very shallow because of lateral growth of the triradiate cartilage. In addition to developing a very wide teardrop, this triradiate cartilage may actually form somewhat of a ridge in the center of the acetabulum because there are no opposing forces. Also, the posterosuperior aspect of the acetabular labrum opens up and becomes a fairly deep trough or channel through which the femoral head is migrating further

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Fig. 6 This girl had a severe asthmatic attack at age 6 years, which caused a severe anoxic static encephalopathy. Due to severe quadriplegic spasticity, the hip slowly subluxated, but due to a combination of her changing spasticity pattern and aggressive adductor release the hip did not completely dislocate. As the hip joint reaction force focused on the center of the femoral head and edge of the acetabulum, both sides became deformed, however the acetabulum had relatively less deformity then the femoral head. This pattern is seen when the spasticity onset is in middle childhood or adolescence

superiorly, laterally, and posteriorly. As the femoral head is migrating through this channel, almost all its force is on the medial side; therefore, the femoral head often develops some flattening along its medial side. Concurrently, there is no force on the lateral aspect of the femoral head except for some soft tissue force; therefore, the lateral aspect of the femoral head often becomes quite osteoporotic (Fig. 7). As the osteoporosis increases, a deep channel from the reflected head of the rectus and the hip joint capsule may develop. As the femoral head either migrates further or stays in this severely abnormal position, the cartilage of the femoral head gradually becomes degenerated and develops deep pitting, and the femoral head has the appearance of latestage degenerative arthritis. As this deformity continues, the femoral head becomes very triangular in shape from the collapse caused by the severe lateral osteoporosis and compression of the medial side due to high force. The femoral head

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Fig. 7 With reduced range of motion, the lateral side of the femoral head gets no weight-bearing stimulation, develops severe osteoporosis, and is at risk for collapsing from pressure of the reflected head of the rectus or after being reduced into the acetabulum, if there is not enough decompression

then takes on a wedge shape. Also, during this later stage, in addition to contractures developing in the adductor muscles, the medial aspect of the hip joint capsule becomes further contracted as well (Fig. 8). The etiology of the hypotonic hip subluxation is likely very similar to the spastic hip; however, the data and reports are almost nil. The hips are also much more variable, and there are no clear patterns that can be appreciated by the spastic hip. Most of the children with a hypotonic hip tend to have coxa valga and a shallow acetabulum, but not a deformed acetabulum. These hips often have the appearance of a femoral head that is too large for the acetabulum. The natural history also suggests that these hips tend to become uncovered laterally as measured by the MP of 40 to 50% but do not progress through childhood. This likely suggests that the reduced force is a major contributing factor, but the net force vector remains stable.

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Fig. 8 The anatomical pathology in the spastic hip develops when the femoral head is forced posterolaterally and superiorly (b). These bends open the lateral rim, labrum, and the acetabulum (c). Because the femoral head no longer compresses the medial wall of the acetabulum, the triradiate cartilage grows laterally, thereby widening the medial wall of the acetabulum and decreasing the depth of the acetabulum (a) (Fig 8a). As the femoral head

continues to be laterally displaced, the lateral side of the femoral head is no longer weight bearing and develops severe osteoporosis. The weakened osteoporotic femoral head may then collapse under the tension of the reflected head of the rectus tendon, causing an indentation in the lateral aspect of the femoral head (Fig 8b). On the gross anatomical specimen, this defect is seen as a crater exposed bone with no cartilage (Fig 8c)

Treatment and Outcomes

Palsy” and ▶ 133, “Anterior Dislocation of the Hip in Cerebral Palsy”)

Treatment and outcomes of the hip dysplasia in children will be further discussed in subsequent chapters. (▶ Chaps. 130, “Prophylactic Treatment of Hip Subluxation in Children with Cerebral Palsy,” ▶ 131, “Hip Reconstruction in Children with Cerebral Palsy,” ▶ 132, “Palliative or Salvage Hip Management in Children with Cerebral

Conclusion The hip in the child with cerebral palsy is at risk for subluxation and acetabular dysplasia which may progress to total dislocation. This process may be pain free in its evolution; however, as

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the degenerative process increases, many of these hips will become very painful. The etiology of this process may be either too high and mal-directed joint reaction force or too low hip joint reaction force. Based on understanding the pathology, it is possible to consider early screening and intervention before the hip degenerative change becomes severe.

References Abousamra O, Er MS, Rogers KJ, Nishnianidze T, Dabney KW, Miller F (2015) Hip reconstruction in children with unilateral cerebral palsy and hip dysplasia. J Pediatr Orthop 36(8):834–840 Bobroff ED, Chambers HG, Sartoris DJ, Wyatt MP, Sutherland DH (1999) Femoral anteversion and neck-shaft angle in children with cerebral palsy. Clin Orthop Relat Res 364:194–204 Brunner R, Baumann JU (1997) Long-term effects of intertrochanteric varus-derotation osteotomy on femur and acetabulum in spastic cerebral palsy: an 11- to 18-year follow-up study. J Pediatr Orthop 17:585–591 Brunner R, Picard C, Robb J (1997) Morphology of the acetabulum in hip dislocations caused by cerebral palsy. J Pediatr Orthop B 6:207–211 Cooke PH, Cole WG, Carey RP (1989) Dislocation of the hip in cerebral palsy. Natural history and predictability. J Bone Joint Surg 71:441–446 de Windt FH, Spoor CW, Huson A, Duijfjes F (1984) A model for the study of hip dysplasia in the spastic child. Acta Orthop Belg 50:143–147 Eilert RE, MacEwen GD (1977) Varus derotational osteotomy of the femur in cerebral palsy. Clin Orthop Relat Res 125:168–172 Feldkamp M, Denker P (1989) Importance of the iliopsoas muscle in soft-tissue surgery of hip deformities in cerebral palsy children. Arch Orthop Trauma Surg 108:225–230 Fettweis E (1979) Spasm of the adductor muscles, pre-dislocations and dislocations of the hip joints in children and adolescents with cerebral palsy. Clinical observations on aetiology, pathogenesis, therapy and rehabilitation. Part II. The importance of the iliopsoas tendon, its tenotomy, of the coxa valga antetorta, and correction through osteotomy turning the hip into varus (author's transl). Zeitschrift fur Orthopadie und ihre Grenzgebiete 117:50–59 Fujiwara M, Basmajian JV, Iwamoto M (1976) Hip abnormalities in cerebral palsy: radiological study. Arch Phys Med Rehabil 57:278–281 Gose S, Sakai T, Shibata T, Murase T, Yoshikawa H, Sugamoto K (2010) Morphometric analysis of the femur in cerebral palsy: 3-dimensional CT study. J Pediatr Orthop 30:568–574

F. Miller Hagglund G, Lauge-Pedersen H, Wagner P (2007) Characteristics of children with hip displacement in cerebral palsy. BMC Musculoskelet Disord 8:101 Heimkes B, Stotz S, Heid T (1992) Pathogenesis and prevention of spastic hip dislocation. Zeitschrift fur Orthopadie und ihre Grenzgebiete 130:413–418 Hoffer MM, Stein GA, Koffman M, Prietto M (1985) Femoral varus-derotation osteotomy in spastic cerebral palsy. J Bone Joint Surg Am 67:1229–1235 Kahle WK, Coleman SS (1992) The value of the acetabular teardrop figure in assessing pediatric hip disorders. J Pediatr Orthop 12:586–591 Keats S (1972) Early preventive surgery in the modern management of the preschool child. Need a cerebral palsied child be a crippled child? Int Surg 57:398–405 Larnert P, Risto O, Hagglund G, Wagner P (2014) Hip displacement in relation to age and gross motor function in children with cerebral palsy. J Child Orthop 8:129–134 Lundy DW, Ganey TM, Ogden JA, Guidera KJ (1998) Pathologic morphology of the dislocated proximal femur in children with cerebral palsy. J Pediatr Orthop 18:528–534 Miller F, Girardi H, Lipton G, Ponzio R, Klaumann M, Dabney KW (1997a) Reconstruction of the dysplastic spastic hip with peri-ilial pelvic and femoral osteotomy followed by immediate mobilization. J Pediatr Orthop 17:592–602 Miller F, Cardoso Dias R, Dabney KW, Lipton GE, Triana M (1997b) Soft-tissue release for spastic hip subluxation in cerebral palsy. J Pediatr Orthop 17:571–584 Miller F, Slomczykowski M, Cope R, Lipton GE (1999) Computer modeling of the pathomechanics of spastic hip dislocation in children. J Pediatr Orthop 19:486–492 Mubarak SJ, Valencia FG, Wenger DR (1992) One-stage correction of the spastic dislocated hip. Use of pericapsular acetabuloplasty to improve coverage. J Bone Joint Surg Am 74:1347–1357 Pruszczynski B, Sees J, Miller F (2015) Risk factors for hip displacement in children with cerebral palsy: systematic review. J Pediatr Orthop 36(8):829–833 Reimers J (1980) The stability of the hip in children. A radiological study of the results of muscle surgery in cerebral palsy. Acta Orthop Scand Suppl 184:1–100 Ribble T, Santare M, Miller F (1998) Response of the femoral epiphysis to force: a finite element modeling study. University of Delaware, Newark Rink P, Miller F (1990) Hip instability in spinal cord injury patients. J Pediatr Orthop 10:583–587 Rutz E, Passmore E, Baker R, Graham HK (2012) Multilevel surgery improves gait in spastic hemiplegia but does not resolve hip dysplasia. Clin Orthop Relat Res 470:1294–1302 Silva S, Nowicki P, Caird MS, Hurvitz EA, Ayyangar RN, Farley FA, Vanderhave KL, Hensinger RN, Craig CL (2012) A comparison of hip dislocation rates and hip containment procedures after selective dorsal rhizotomy versus intrathecal baclofen pump insertion in

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nonambulatory cerebral palsy patients. J Pediatr Orthop 32:853–856 Vizkelety T, Rényi-Vámos A, Szöke G (1991) Surgical treatment of the hip in cerebral palsy. Acta Chir Hung 32:215–224 Wynter M, Gibson N, Kentish M, Love S, Thomason P, Kerr Graham H (2011) The development of Australian Standards of Care for Hip Surveillance in Children with

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Cerebral Palsy: how did we reach consensus? J Pediatr Rehabil Med 4:171–182 Wynter M, Gibson N, Willoughby KL, Love S, Kentish M, Thomason P, Graham HK, Group National Hip Surveillance Working (2015) Australian hip surveillance guidelines for children with cerebral palsy: 5-year review. Dev Med Child Neurol 57:808–820

Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy

129

Freeman Miller

Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1894 Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Childhood . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Adolescence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Adult . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1894 1894 1896 1897

Diagnostic Evaluations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hip Radiograph . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Computed Tomography Scans . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Ultrasound . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Bone Scan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Arthrography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1898 1899 1900 1901 1901 1901

Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1901 Surveillance Algorithm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1901 Surveillance Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1903 Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1904 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1904

Abstract

Children with cerebral palsy (CP) very commonly develop subluxation or dislocation of the hip joint. This dislocation leads to difficulty sitting, problems with custodial care, and hip pain. The hip in the child with CP is normal at birth, and because of the abnormal muscle pull and growth, it slowly becomes displaced moving lateral and posterior superior. This process

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_126

typically begins around age 2 and has the highest risk between 3 and 5 years old. The subluxation is measured with anteriorposterior supine pelvic radiographs, and the amount of subluxation is approximately one degree a month which means radiographs are required annually from age 2 to 8 years old and then every 2 years till completion of growth. The high-risk group of children are those who cannot walk or need to use assistive devices like walkers or crutches (GMFCS III–V). For children who can walk (GMFCS I–II), there needs to be only one radiograph in early childhood 2–4 years old; if it is normal (30% or MP>33% 0.8

0.6

0.4

0.2

0 GMFCS I

Fig. 8 The percent of the total population studied who had dislocated hips at the specific age (green line) and the percent of the total population who had subluxated hip at a defined age (blue line). This shows that the peak dislocation by age risk occurs 2–3 years after the peak subluxation risk. This includes 182 subluxated hips and 38 dislocated hips in which the data can define when it occurred

GMFCS II

GMFCS III

GMFCS IV

GMFCS V

% of total subluxation (MP>30% or MP>33%) occurred at certain age % of total dislocation occurred at certain age

30

22.5

15

7.5

0

1

2&3

4

5

5&6

8

9

11&12 15&16

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was then used to indicate the need for radiograph. The frequency was based on the work of Reimers who showed that hips progressed at a rate of approximately one degree a month in the early stages of subluxation (Reimers 1980). The hip monitoring program at AI DuPont Hospital started in 1988 based on the data of Rang and Reimers but has evolved to include the current information. It is now based on age and GMFCS, with exception for the child with type IV hemiplegia. The population-based data on the child with hemiplegia is still very weak, since it is small group to start with and only a small number develops hip subluxation. Our surveillance program is outlined in table below (Fig. 9).

Surveillance Results A surveillance program for early detection of hip subluxation in children with CP only makes sense

1903

if there is early intervention that is successful in preventing later hip dislocation. By careful monitoring of the surveillance results and encouraging early intervention, there has been a dramatic reduction in the number of children who need hip reconstruction based on the data from the Swedish and Australian population screening (Dobson et al. 2002; Elkamil et al. 2011; Hagglund et al. 2014). Although there is no agreed universal treatment protocol for the hip with mild subluxation, there are several generally accepted strategies. The need for some raised level of concern is between 25 and 40% subluxation. The first-level response is increased frequency of observation based on other factors such as severe spasticity, nonambulatory, and windblown posture. This is also the time to initiate the conversation with the parents that some surgical intervention may be needed in the future which helps them get psychologically prepared. Children with spasticity who are GMFCS III–V and

Hip Monitoring Protocol for Cerebral Palsy Goal: To monitor all children withCP to prevent hip dislocaon. Monitoring schedule: GMFCS I and II (full ambulation without assistive device) – One X-ray after 2 years of age – no other needed if normal Exception: Hemiplegia type 4 (with hip involvement, rotation or flexion) X-ray every 2 years from age 8 to skeletal maturity GMFCS III, IV, & V (non-ambulatory to walking with assistive device) Until age 8 – X-ray every year, X-ray every 6 month – For children with severe spasticity or MP >25 Age 8 to skeletal maturity – if previous X-ray normal – every 2 years If previous X-ray MP > then 25% – every year X-ray

MP

Treatment recommendaons: Up to age 8 years: MP 30 to 60% and hip abduction less than 30º with hip and knee extended – STR MP 30 to 60% and hip abduction over than 30º with hip and knee extended – observe MP > 60% and healthy child – reconstruction MP > 60% and hip abduction < 30º and child with multiple medical problems – STR Migration Percent (MP)- A single AP supine pelvis X-ray with the child Over age 8 years: lying in a relaxed, comfortable neutral position with about (10°) abduction, may MP > 40% – abduction less than 45º – reconstruction have a small blanket roll under the knees MP > 50% – abduction over 45º – reconstruction to slightly flex the hips and knees if this Dislocated hip – painful but not severe degenerative changes – reconstruction makes the child more comfortable. Hip Dislocated hip – painful with severe degenerative changes – palliative procedure rotation should be approximately neutral but do not force into any position.

Fig. 9 This is the poster we have in each clinic to remind and explain the CP hip surveillance program

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whose migration is increasing toward 40% should then be prepared for surgical intervention based on the physical examination and tone pattern. For the child with spasticity and hip abduction less than 30 degrees, adductor lengthening is usually considered. For children with hypotonia, further observation to 50% subluxation would be reasonable. The important factors in making a treatment algorithm require knowing the age, the degree of abduction of the hip, and the MP from a radiograph. The hip treatment is then divided into three areas, as follows. The first area is prevention, in which the treatment is addressed at the root cause of the spastic hip disease. The second area is reconstruction, in which the treatment is primarily directed at reversing the secondary effects of spastic hip disease. The third, or palliative, area is the treatment directed at addressing the tertiary symptoms and deformities of spastic hip disease.

Conclusion The natural history for hip displacement in children with cerebral palsy is well documented in the childhood age range and is heavily influenced by GMFCS functional level of the children. The natural history in adolescence and in adulthood is less clearly defined. Surveillance program based on radiographic imaging of the hips in a scheduled program has demonstrated the ability to maintain the hips’ location and reduce the amount of surgery. The best time and type of intervention based on this early identification are still not clear.

References Abousamra O, Er MS, Rogers KJ, Nishnianidze T, Dabney KW, Miller F (2015) Hip reconstruction in children with unilateral cerebral palsy and hip dysplasia. J Pediatr Orthop 36:834 Brunner R, Robb JE (1996) Inaccuracy of the migration percentage and center-edge angle in predicting femoral head displacement in cerebral palsy. J Pediatr Orthop B 5:239–241 Buckley SL, Sponseller PD, Magid D (1991) The acetabulum in congenital and neuromuscular hip instability. J Pediatr Orthop 11:498–501

F. Miller Chung CY, Choi IH, Cho TJ, Yoo WJ, Lee SH, Park MS (2008) Morphometric changes in the acetabulum after Dega osteotomy in patients with cerebral palsy. J Bone Joint Surg Br 90:88–91 Connelly A, Flett P, Graham HK, Oates J (2009) Hip surveillance in Tasmanian children with cerebral palsy. J Paediatr Child Health 45:437–443 Dobson F, Boyd RN, Parrott J, Nattrass GR, Graham HK (2002) Hip surveillance in children with cerebral palsy. Impact on the surgical management of spastic hip disease. J Bone Joint Surg Br 84:720–726 Elkamil AI, Andersen GL, Hagglund G, Lamvik T, Skranes J, Vik T (2011) Prevalence of hip dislocation among children with cerebral palsy in regions with and without a surveillance programme: a cross sectional study in Sweden and Norway. BMC Musculoskelet Disord 12:284 Frischhut B, Krismer M (1990) Pelvic tilt in neuromuscular diseases. Der Orthopade 19:292–299 Gordon GS, Simkiss DE (2006) A systematic review of the evidence for hip surveillance in children with cerebral palsy. J Bone Joint Surg Br 88:1492–1496 Graham HK, Baker R, Dobson F, Morris ME (2005) Multilevel orthopaedic surgery in group IV spastic hemiplegia. J Bone Joint Surg Br 87:548–555 Hagglund G, Lauge-Pedersen H, Wagner P (2007) Characteristics of children with hip displacement in cerebral palsy. BMC Musculoskelet Disord 8:101 Hagglund G, Alriksson-Schmidt A, Lauge-Pedersen H, Rodby-Bousquet E, Wagner P, Westbom L (2014) Prevention of dislocation of the hip in children with cerebral palsy: 20-year results of a population-based prevention programme. Bone Joint J 96-B:1546–1552 Haspl M, Bilic R (1996) Assessment of femoral neck-shaft and antetorsion angles. Int Orthop 20:363–366 Heinrich SD, MacEwen GD, Zembo MM (1991) Hip dysplasia, subluxation, and dislocation in cerebral palsy: an arthrographic analysis. J Pediatr Orthop 11:488–493 Hermanson M, Hagglund G, Riad J, Rodby-Bousquet E, Wagner P (2015a) Prediction of hip displacement in children with cerebral palsy: development of the CPUP hip score. Bone Joint J 97-B:1441–1444 Hermanson M, Hagglund G, Riad J, Wagner P (2015b) Head-shaft angle is a risk factor for hip displacement in children with cerebral palsy. Acta Orthop 86:229–232 Hodgkinson I, Vadot JP, Metton G, Berard C, Berard J (2000) Prevalence and morbidity of hip excentration in cerebral palsy: review of the literature. Revue de chirurgie orthopedique et reparatrice de l'appareil moteur 86:158–161 Hodgkinson I, Jindrich ML, Duhaut P, Vadot JP, Metton G, Berard C (2001) Hip pain in 234 non-ambulatory adolescents and young adults with cerebral palsy: a crosssectional multicentre study. Dev Med Child Neurol 43:806–808 Horstmann H, Mahboubi S (1987) The use of computed tomography scan in unstable hip reconstruction. J Comput Tomogr 11:364–369

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Huh K, Rethlefsen SA, Wren TA, Kay RM (2011) Surgical management of hip subluxation and dislocation in children with cerebral palsy: isolated VDRO or combined surgery? J Pediatr Orthop 31:858–863 Jung NH, Pereira B, Nehring I, Brix O, Bernius P, Schroeder SA, Kluger GJ, Koehler T, Beyerlein A, Weir S, von Kries R, Narayanan UG, Berweck S, Mall V (2014) Does hip displacement influence health-related quality of life in children with cerebral palsy? Dev Neurorehabil 17:420–425 Kentish M, Wynter M, Snape N, Boyd R (2011) Five-year outcome of state-wide hip surveillance of children and adolescents with cerebral palsy. J Pediatr Rehabil Med 4:205–217 Kim HT, Wenger DR (1997) Location of acetabular deficiency and associated hip dislocation in neuromuscular hip dysplasia: three-dimensional computed tomographic analysis. J Pediatr Orthop 17:143–151 Mahboubi S, Horstmann H (1986) Femoral torsion: CT measurement. Radiology 160:843–844 McHale KA, Bagg M, Nason SS (1990) Treatment of the chronically dislocated hip in adolescents with cerebral palsy with femoral head resection and subtrochanteric valgus osteotomy. J Pediatr Orthop 10:504–509 Miller F, Bagg MR (1995) Age and migration percentage as risk factors for progression in spastic hip disease. Dev Med Child Neurol 37:449–455 Miller F, Liang Y, Merlo M, Harcke HT (1997) Measuring anteversion and femoral neck-shaft angle in cerebral palsy. Dev Med Child Neurol 39:113–118 Pons C, Remy-Neris O, Medee B, Brochard S (2013) Validity and reliability of radiological methods to assess proximal hip geometry in children with cerebral palsy: a systematic review. Dev Med Child Neurol 55:1089–1102

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Pruszczynski B, Sees J, Miller F (2015) Risk factors for hip displacement in children with cerebral palsy: systematic review. J Pediatr Orthop 36:829 Reimers J (1980) The stability of the hip in children. A radiological study of the results of muscle surgery in cerebral palsy. Acta Orthop Scand Suppl 184:1–100 Rutz E, Passmore E, Baker R, Graham HK (2012) Multilevel surgery improves gait in spastic hemiplegia but does not resolve hip dysplasia. Clin Orthop Relat Res 470:1294–1302 Silver RL, Rang M, Chan J, de la Garza J (1985) Adductor release in nonambulant children with cerebral palsy. J Pediatr Orthop 5:672–677 Smigovec I, Ethapic T, Trkulja V (2014) Ultrasound screening for decentered hips in children with severe cerebral palsy: a preliminary evaluation. Pediatr Radiol 44(9):1101 Terjesen T, Lofterod B, Myklebust G (2004) Orthopaedic problems in adults with cerebral palsy. Tidsskr Nor Laegeforen 124:156–159 Wynter M, Gibson N, Kentish M, Love S, Thomason P, Kerr Graham H (2011) The development of Australian standards of care for hip surveillance in children with cerebral palsy: how did we reach consensus? J Pediatr Rehabil Med 4:171–182 Wynter M, Gibson N, Willoughby KL, Love S, Kentish M, Thomason P, Graham HK, Group National Hip Surveillance Working (2015) Australian hip surveillance guidelines for children with cerebral palsy: 5-year review. Dev Med Child Neurol 57:808–820 Zimmermann SE, Sturm PF (1992) Computed tomographic assessment of shelf acetabuloplasty. J Pediatr Orthop 12(5):581

Prophylactic Treatment of Hip Subluxation in Children with Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1908 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1908 Specific Prophylactic Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1909 The Outcome of Preventative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Other Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Iliopsoas Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Adductor Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Botulinum Toxin Injection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Intrathecal Baclofen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dorsal Rhizotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Abduction Orthosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1910 1912 1912 1912 1913 1913 1913 1914

Complications of Preventative Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hyperabduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1914 1915 1915 1915

Cases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1915 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1921

Abstract

Hip subluxation is a common problem in children with cerebral palsy. It is more common and more severe as the child’s motor function decreases. The highest risk is in children ages 2–6 years old with GMFCS IV and V level function. Screening programs using routine radiographs are the most important element in

F. Miller (*) Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA e-mail: [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_127

the early identification of the subluxation since it is usually pain free with no symptoms. The best documented early intervention is surgical lengthening of the hip adductor muscles with an expected good outcome in 90% of children with GMFCS II and III and 10–60% of children with GMFCS IV and V. The amount of muscle lengthening is important since the best reported outcomes are in series with open lengthenings (not percutaneous) that include adductor longus, gracilis, and iliopsoas, with some patients also needing to have adductor brevis and proximal hamstring lengthenings. Physical therapy, 1907

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abduction orthosis, botulinum toxin, dorsal rhizotomy, and intrathecal baclofen have documented benefits in the prevention of hip subluxation in children with cerebral palsy.

goal of this chapter is to define the methods of early intervention, the indications, and the expected outcomes.

Keywords

Treatment

Hip subluxation · Adductor lengthening · Botulinum toxin · Hip dislocation · Abduction orthosis · Rhizotomy · Intrathecal baclofen

Introduction Hip subluxation and dysplasia are two of the most common problems in children with cerebral palsy (CP) affecting mainly those children who are unable to ambulate (Gross Motor Function Scale Classification IV & V (Ailon et al. 2015)) (▶ Chap. 128, “Etiology of Hip Displacement in Children with Cerebral Palsy”). The data from population surveillance is now very clear that there is a very strong increasing risk of hip subluxation as the motor function decreases (Hagglund et al. 2007). The primary measure of hip subluxation is the Reimers migration percent, which measures the amount of lateral uncoverage of the femoral head or femoral epiphysis to the total width of the femoral head (Reimers 1980). Although both children with spasticity and hypotonia develop hip subluxation, the early treatment is different. Likely the hypotonic hip with increased hip abduction develops subluxation from insufficient hip joint reaction force; although the data in support of this is minimal, what is known is that hip abduction is not a good and reliable screening tool (Hagglund et al. 2007). Hip abduction does however help to separate those children who have spastic hips from those with hypotonic hips. The spastic hips are defined as those with limited hip abduction. A third monitoring item most recently defined is the femoral head-shaft angle which adds to the predictive value for defining who will progress. For 10 increase of head-shaft angle, the risk of subluxation increases 1.6 times (Hermanson et al. 2015). There is a currently an APP developed to calculate risk of subluxation at the CPUP web site: http:// cpup.se/in-english/what-is-cpup-in-english/. The

The primary prophylactic measure for prevention of hip subluxation is early identification through surveillance using a schedule of pelvic radiographs (▶ Chap. 129, “Natural History and Surveillance of Hip Dysplasia in Cerebral Palsy”). At this time there is no widely agreed upon specific indication as to when intervention is indicated. The most objective measure we have to follow is the Reimers migration (MP). Normal as defined by Reimers is under 25% at most ages (Reimers 1980). However we know many children with CP will come close to this or even go over and then reverse and get better. Based on this normal report, our current recommendation is to increase surveillance to every 6 months hip radiograph if there are at least two other risk factors, such as young age under age 6, GMFCS IV or V, high head-shaft angle (>150 ), or acetabular changes such a blunting of the lateral acetabular edge. As MP increases, there is also higher risk that it will continue to increase especially in children with spasticity with MP 33–39% (Hagglund et al. 2007); however, this risk is not well defined as separate risk factor for high MP. There is data to show that there is high risk for further progression even in mature adult once the MP reaches 60% (Miller and Bagg 1995). Based on the above data, recommendation for prophylactic intervention based on MP is between 25 and 35% with increased surveillance or adductor lengthening based on other risk factors. For MP greater than 35% and less then age 8 years old, adductor lengthening should be considered based on the amount of hip abduction and considering risk factors. For hips with MP greater than 60%, there is a high risk of failure, but it may still be a viable option as a delaying procedure if the child is very young such as less than 6 years old and GMFCS IV or V (Case 1) (Table 1). There is no data to support adductor lengthening as a prevention or treatment of further progression in either

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Table 1 Indications for adductor lengthening. The indications for adductor lengthening are not clearly defined, but it can be used as a procedure to delay reconstruction or as the definitive treatment, and this table tries to consider the variables that are known to be risk factors for progression. MP is migration percentage, GMFCS is Gross Motor Function Classification System, Age is in years, Hip ABD is hip abduction with hips and knees extended, H-S angle is the head-shaft angle measures on a supine AP pelvis radiograph, and Acetabulum means considering if the acetabulum has a normal configuration or if the lateral corner is blunted or turned up or if the whole acetabulum is shallow with the femoral head seated against the medial wall. Treatment terms: Surveillance means returning to the recommended MP 60 >60 >60

GMFCS I–V I–II III–V III–V III–V I–II I–II III–V III–V III–V III–V I–II III–V III–V

Age 15 years

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Lawrence et al. (2016) Lawrence et al. (2016) Lawrence et al. (2016) Lawrence et al. (2016) Lawrence et al. (2016)

Adults with CP Adults with CP Adults with CP Adults with CP Adults with CP

Lawrence et al. (2016) Lawrence et al. (2016) Lawrence et al. (2016)

Adults with CP Adults with CP Adults with CP

Lawrence et al. (2016)

Adults with CP

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Fig. 1 (a) At 27 years of age, left peak knee flexion range of motion in swing at 45–50 degrees, similar to pre- and postoperative values post rectus femoris transfer as a child. Yellow band is normal. (b) At 27 years of age, right peak knee flexion range of motion in swing at 45–50 degrees,

similar to pre and postoperative values post rectus femoris transfer as a child. Yellow bland is normal. (c) At almost 35 years of age, right (red) and left (blue) knee flexion peak knee flexion range of motion in swing is 55 degrees. Green band is normal

Sports Medicine (ACSM) Exercise Management for People with Chronic Disease and Disability (Moore et al. 2016), and known dosing parameters for children with CP (Gannotti 2017) is best evidence for dosing parameters for adults with CP (see Table 3). Combining rehabilitation strategies listed in Table 1 with rehabilitation techniques listed in Table 4 provides a variety of options for a rehabilitation plan that is individualized and person centered. Table 4 lists the best evidence to support rehabilitation techniques. In some instances, the best evidence has been not determined for adults with CP, specifically but for adults with chronic pain, fatigue, or cardiovascular disease. One systematic review synthesizes interventions published to date with effectiveness for adults with CP (Lawrence et al. 2016). Exercise and physical activity are the cornerstone of any rehabilitation program for adults with chronic disease and disability (Pescatello et al. 2004a). Using

dosing parameters of adequate frequency, intensity and time, a variety of forms of exercise, physical activity, and rehabilitation techniques show promise for adults with CP. Forms of aerobic exercise such as treadmill training or conventional therapy have improved endurance, gait speed, and gross motor skills in adults with CP (Lawrence et al. 2016). Clinicians and consumers are challenged to find the type of exercise or physical activity that is best suited for each individual and their social context.

Conclusion Clinical registries, patient reported registries, and more intervention research is needed to better meet the needs of adults with CP (Lungu et al. 2016). Nonetheless, there is ample evidence from the general population and other populations from which rehabilitation needs, assessments,

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strategies, and techniques can be developed. Consumers and clinicians should be aware of the possibilities and potential of rehabilitation interventions to improve the health, function, wellbeing, and quality of life of adults with CP and advocate for physical activity programs for all people with mobility disorders. Case Studies

Andrew is 40 years old and has spastic diplegic CP, GMFCS II. He was fortunate to have some of the best care available when he was young. He had three-dimensional gait analysis and single event multilevel surgery when he was 9 years old. He walked with bilateral ankle foot orthoses, walked more slowly than other kids, and walked

Fig. 2 (a) At 27 years of age, left ankle kinematics. Yellow band is normal. (b) At 27 years of age, right ankle kinematics. Yellow bland is normal. (c) At almost

M. Gannotti and D. Frumberg

noticeably different. He had difficulty climbing stairs without using rail and could not run very well. He had the derotational osteotomy on the left femur (femoral derotational osteotomy), and soft tissue surgery on bilateral hamstrings, rectus femoris muscles, and heel cords. He had intense physical therapy after his surgery, and had postoperative gait analysis that demonstrated improvement in foot progression and toe clearance, but no change in the amount of knee flexion in swing. In middle school or high school, he rarely saw a physical therapist. He went to college and tried to keep up with his peers. After graduating and moving (continued)

35 years of age, right (red) and left (blue) ankle kinematics. Green band is normal. No changes in kinematics between two evaluations

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Fig. 3 (a) At 27 years of age, left ankle kinetics- solid line barefoot; dotted line shoes. Yellow band is normal. Arrow indicates excessive absorption. (b) At 27 years of age, right ankle kinetics- solid line barefoot; dotted line shoes. Yellow bland is normal. Arrow indicates power generation

timing off. (c) At almost 35 years of age, right (red) and left (blue) ankle kinetics, barefoot Green band is normal. Timing and amount of power absorption on left and generation on right more normalized

out to the West Coast to start a business, at age 27, he began to trip and fall as he began to have difficulty clearing his right foot. He had a follow-up gait analysis that identified changes in range of motion at the hip, knee, and ankle, decreased gait speed, poor foot clearance bilaterally. Andrew began an intensive 2–3 week physical therapy program that consisted of a multimodal approach – strengthening, motor control, core stability, and vestibular training. The program transitioned after a year or so to an athletic trainer, who began to train Andrew like an athlete. He asked Andrew to identify a sport that he desired to do. Andrew had never done a sport, he was interested literature and science, and picked something he thought was unattainable. He said rock climbing. The

athletic trainer and Andrew worked on this goal of gradually leaving the training program with the athletic trainer and moving to a gym where he could rock climb and exercise independently. After several years of work, Andrew joined a rock climbing gym. He currently engages in his own exercise program of resistance training, core stability, vestibular training, and power training. Additionally, he rock climbs, and he teaches children to rock climb. He had a repeat gait analysis after several years of his current lifestyle change, and he improved his peak knee flexion in swing and his foot clearance, by improving control of the timing and amplitude of his ankle power burst in pushoff (see Figs. 1, 2, and 3). His rock climbing (continued)

2536 Photo C1.1 Andrew begins the climb and his calf muscles are in an elongated position and generate power to lift him up the wall. (photography by Devan Perez)

Photo C1.2 Continued ascent up the wall requires positioning of the foot and ankle and generation of muscle power to continue to climb. (photography by Devan Perez)

Photo C1.3 Ankle balance and positioning are key to staying on the route. (photography by Devan Perez)

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Photo C1.4 Total body strength and coordination required to ascend. Although asymmetrical positioning of the body is a challenge persons with bilateral spastic diplegia, the position is functional on the rock wall! (photography by Devan Perez)

Photo C1.6 Ian’s spine at 15 years old with greater than 70 degrees of scoliosis, osteopenia, and chronic pain. He was not a good candidate for surgery due to his fragile health Photo C1.5 Andrew made it to the top! (photography by Devan Perez)

carried over to functional changes in his gait (see Video C1.1). Ian is 30 years old, and he has spastic quadriplegia, GMFCS IV, with a more than

70 scoliosis. Ian’s case study is well described elsewhere (Gannotti et al. 2015). Ian became disenchanted with traditional school and medical physical therapy services as a teenager and beseeched (continued)

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his parents to provide him with the opportunity to become a boxer, a wheelchair boxer. Ian’s engagement in his sport changed a young man with a G-tube,

Photo C1.7 Ian’s hip x-ray after bilateral hip osteotomies reveal osteopenia at an early age

Photo C1.8 (left upper corner) Ian shadow boxing in preparation to hit the heavy bag. (lower left corner) Home adaptation of lazy Susan with foam to assist with

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failure to thrive, poor grades, incontinence, and upper extremity contractures, into a more vibrant, confident, healthy person. After working out aggressively two times a week from age 15 years until age 23, Ian was able to increase his appetite and eliminate his G-tube, regulate his bowels and form stools, attend college, improve his upper extremity speed of movement and range of motion, and decrease his back painzation with people with and without disabilities. His exercise program has been a vital part of keeping him healthy. He has avoided spine surgery, kept active and managed his pain, avoided pneumonia or other respiratory infections, and is able to enjoy life living with his romantic partner and working part time (see Photos 1, 2, 3 and 4; Video C1.2, heavy bag and Video C1.3 – pull-ups) .

trunk rotation with punching. (Right) Ian performing knee extension with assist of Tyrone Burris, his personal care assistant. (photography by Pete Pyzik)

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Cross-References ▶ Aging with Cerebral Palsy: Adult Musculoskeletal Issues ▶ Community Engagement for Adults with Cerebral Palsy ▶ Community Resources: Sports and Active Recreation for Individuals with Cerebral Palsy ▶ Life Care Planning for the Child with Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2543 Goals and Environment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2544 Techniques and Evidence of Effectiveness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2545 Transportation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2545 Home Health and Support Services . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2548 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2561

Abstract

Adults with cerebral palsy have the same desires as those without disabilities to create their own path to becoming independent, valued, and engaged members of their communities. Their goals can be difficult to achieve given the everyday challenges they face. As they transition out of the protected world of high school, they need to learn and manage a new set of laws, rules, and obstacles to achieving their goals. This chapter explores four key areas for adults with cerebral palsy as they pursue community engagement and independence. The four areas are transportation, home

M. N. Orlin (*) Department of Physical Therapy and Rehabilitation Sciences, Drexel University, Philadelphia, PA, USA e-mail: [email protected] S. Tachau Pennsylvania Assistive Technology Foundation (PATF), King of Prussia, PA, USA e-mail: [email protected] # Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_159

health and support services (including attendant care), postsecondary education and work, and vocational services. Each section includes scientific literature and many other resources for both consumers and providers. A case scenario is also included that integrates each of these four areas, including challenges and solutions, from the perspective of an adult with cerebral palsy. Keywords

Adults with cerebral palsy · Community participation · Independent living · Employment · Assistive technology

Introduction Adults with cerebral palsy (CP) have the same desires as those without disabilities to create their own path to becoming independent, valued, and engaged members of their communities. Their 2543

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goals can be difficult to achieve given the everyday challenges they face. As they transition out of the protected world of high school, they need to learn and manage a new set of laws, programs, and rules for achieving their adult life goals. There are various obstacles but there are also many private and public programs and social supports that are available to adults with CP to assist them with their goals. However, accessing them successfully requires knowledge, advocacy skills, coordination, and perseverance. Adults with CP have grown up with a disability, so most received services as children and adolescents, which are often (although not always) more coordinated than those available to adults. Transition from high school to the adult world for those with CP provides an opportunity for services and programs to be introduced or initiated but the adult service system is not as coordinated as the one that the youth or adolescent leaves behind at high school graduation. Some people with CP can manage these systems and programs independently or with occasional guidance, but others need more episodic or ongoing support. These are the same systems often needed by adults with disabilities that have been acquired in adulthood, but since this is all new and unfamiliar to them, they can be even more difficult for them to understand and manage. In some situations, adults with an acquired disability are involved with comprehensive discharge planning from medical and rehabilitation facilities that can be very helpful and can refer people to needed services. There are a number of key areas that people with CP and their caregivers need to consider when planning what is needed to achieve desired life goals. This chapter explores four of these key areas for adults with CP as they pursue community engagement and independence. These are transportation, home health and support services (including attendant care), postsecondary education and work, and vocational services. Each section includes scientific literature and many other resources for both consumers and providers. A case scenario is also included that integrates each of these four areas, including challenges and solutions, from the perspective of an adult with CP.

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Goals and Environment Social services and programs for adults with CP are designed to offer education, resources and opportunities for individuals to live their best lives based on their interests, strengths, and abilities. These services should, ideally, maximize the person’s potential and abilities by providing the supports needed to participate fully within their community. This is true for both adults with CP and other lifelong disorders, as well as for those who have acquired disabilities in adulthood. However, adults with CP have experienced and worked within many programs and systems long before graduating from high school and entering adult life as opposed to people with disorders acquired in adulthood. Adults with CP have experienced more coordinated pediatric health care and education, often entering the service system through early intervention and children’s hospitals and outpatient clinics. They and their families have had the advantage of having many “eyes” on them at various time points, at home, in school, and in the community. People with CP have a wide variety of strengths and needs. They have had years to learn about both of these as they have evolved over time. They and their families and other caregivers have had opportunities to learn about advocacy and how to work with the pediatric, educational, and governmental systems. This is in contrast to people who have acquired disabilities in adulthood, who are beginning, as adults, to learn what is available to them, and how to advocate successfully for themselves and access appropriate programs, at the same time as they are learning about their disability. The adult service world is very different from the pediatric one. It is less comprehensive, more complex, and often fragmented, and takes time, knowledge, and skill to navigate. Service needs often change over time, suggesting that they should be revisited over time and reconfigured when necessary to maximize abilities; however, not all programs have that flexibility. Young adults with motor disabilities described how social services and programs intended to assist them can actually be detrimental

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by emphasizing a disability-based approach rather than focusing on the environmental adaptations and services needed to maximize their ability to work toward their goals (Darrah et al. 2010). Despite these issues, there are many groups – both governmental (local, state and federal) and private – that offer supports to individuals with CP depending on what they need to meet their goals of living meaningful and satisfying lives. Each has its own focus, personnel and requirements but all should empower the person by using an individual strength-based approach that has some flexibility depending on current needs. It is important for adults with CP to be involved in the conversations about services for them and to advocate for the services they need, using a service coordinator or other advocate to assist them if necessary. There are various advocacy agencies that can provide this help. Services and programs also change over time, so it is also important to seek out updated information. This chapter will focus on certain key social and vocational services, while recognizing that there are others that are beyond the scope of this chapter. The social services that will be discussed include transportation, home health and support services, assistive technology, and home and workplace accommodations. The vocational services discussed are training and supported employment and postsecondary education. Each of these life issues for individuals with CP will focus on how the service can impact community engagement and independence. The chapter will also discuss current literature and end with a case study to illustrate how an adult with CP and his caregivers navigate the social service and vocational systems to put appropriate and needed supports in place for him to achieve the quality of life that is important to him.

Techniques and Evidence of Effectiveness Transportation Having accessible and reliable transportation is a key requirement for adults with CP to live with personal choice and independence in and beyond

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their own communities. For many people with CP, the ability to travel for work, school, and medical appointments and to participate in social, leisure, and community and civic activities, such as voting, is dependent upon the availability of appropriate transit resources. Transportation is also critical for developing and sustaining social relationships, and for achieving happiness and a good quality of life. This is especially important because loneliness has been identified as a concern for some adults with CP (Balandin et al. 2006). In 2015, the US Department of Transportation (DOT) issued a policy statement affirming a commitment to the development of an accessible transit system so that all Americans can have equal access (https://www.transportation.gov/ sites/dot.gov/files/docs/accessibility-policy-state ment-July-29-2015.pdf). However, appropriate transportation can be difficult to find, coordinate and fund and is often described as an obstacle to community participation and independent living, rather than a facilitator (Scheer et al. 2003). This can be particularly true in rural areas where there are fewer public transit lines, and people may need to travel further to work and other social or medical appointments. A national study conducted by the United States Department of Transportation, Bureau of Transportation Statistics in 2003, indicated that 1.9 million people with disabilities never leave their homes, and of those, over 25% (560,000) indicated that they are homebound due to transportation difficulties (Bureau of Transportation Statistics 2003). Since the passage of the Americans with Disabilities Act (ADA), accessibility to transportation has improved; nevertheless, significant barriers are still reported. Access to transportation for people with disabilities is different depending on geographical location. Bezyak et al. (2017) surveyed 4161 people with disabilities who described a number of barriers to transportation. In urban areas, they reported issues with lack of knowledge by drivers, a failure to alert passengers of upcoming stops, poor driver attitudes, and lack of space in wheelchair accessible areas. In rural areas, the main problem was difficulty becoming eligible for paratransit. Almost half (47%) of those surveyed indicated that public transportation systems were

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inadequate to meet their needs for timely and efficient transportation (Bezyak et al. 2017). Both users and their caregivers often cite transportation as a barrier to attending medical appointments and social engagements, to arriving and leaving work on time, or getting to work at all. Transportation services may be provided by State Vocational Rehabilitation (VR) agencies to enable individuals to participate in work services and training. If an individual has a vehicle or access to a vehicle, the state VR agency, under the rehabilitation technology service provision, may provide funding to adapt that vehicle to enable the individual to be transported. Examples of these adaptations are hand controls, swivel chairs, lowered floors, tie downs, and ramps. State VR agencies do not purchase the vehicles themselves for individuals, and obtaining the financing to buy the vehicles can also be a substantial barrier. Those seeking employment need to carefully consider transportation needs when looking for a job in a particular location because the lack of appropriate transportation can be a barrier to employment (Cook and Burke 2002; Magill-Evans et al. 2008), even if the person desires to work in that particular job (Rutkowski and Riehle 2009). It is often difficult to find accessible public transportation for those who are unable to drive, or can drive but do not have access to a vehicle, or who do not have family members or caregivers who can drive them. Depending on where the individual lives, public transportation, although mandated to be accessible through the Americans with Disabilities Act, is often problematic. Even if individuals can use public transportation lines, buses and trains are not always equipped for wheeled mobility and stops may not be near destinations, like medical offices and work. Adults with a family friend, direct support care staff, or other adult to drive them describe the need to schedule their own appointments around those individuals’ schedules, making unexpected medical or other visits particularly difficult (Scheer et al. 2003). People who use transportation services designed specifically for those with disabilities, such as the paratransit system, often describe them as unreliable. Then can require several days to a

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week of advance booking, effectively restricting social and work flexibility (Darrah et al. 2010; Scheer et al. 2003). Adults with significant restrictions in motor skills and leisure activities indicated that adapted transportation is essential to the accomplishment of these activities (Boucher et al. 2010), and the lack of transportation can also be a barrier to starting new leisure activities (Usuba et al. 2015). For those using wheelchairs, education about how to use them in and around transportation is critical for safety and efficiency, and not all wheelchair users, caregivers, and other healthcare providers have the education they need to use best practices for wheelchair transportation safety (Brinkey et al. 2009). Standards for wheelchairs and wheelchair use during transport are available through several sources including RESNA (www.resna.org), University of Michigan Transportation Research Institute (http://wc-transportation-safety.umtri. umich.edu/wts-standards) and the Transportation Code of Federal Regulations (45 C.F.R. § 37 (2017)). There are a number of funding or partial funding options for transportation solutions depending on the specific service needed and the individuals’ eligibility. Public transit lines often offer reduced fares for people with disabilities, but such fares are frequently limited to off-peak hours. There are also cases in which individuals with CP may not be able to access any funding for transportation and may need to be creative with sharing or contracting for rides with transportation companies. For all of these services, there are federal rules and regulations as well as additional rules at the state level. Therefore, it is important for individuals to learn what is available in their own state. For public transit and paratransit systems, fares are posted online and may be paid by the rider, or in some cases, by Medicaid, if the rider is enrolled. States may have reduced fares available. Non-emergency medical transportation (NEMT) is a benefit for eligible Medicaid beneficiaries for transport to and from non-emergent Medicaid providers for medical appointments. Again, it is often reported that this transportation can be unreliable. The Centers for Medicare & Medicaid Services (CMS) provides multiple

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documents on their website www.cms.gov that explain benefits, programs, and eligibility requirements. Transportation costs may also be covered by a Home and Community Medicaid waiver, so those individuals who currently have waiver funding, or are planning to apply for it, should check the waivers available in their state for transportation services and funding. A Medicaid waiver, which is funded by a combination of federal and state dollars, provides an individual with a disability the supports that are needed to live successfully in the community thereby “waiving” the old rules that provided those supports only in institutional settings. All 50 states have waiver programs, but each state offers different waivers and waiver services for various populations. The Medicaid website lists all state waiver programs at: https://www. medicaid.gov/medicaid/section-1115-demo/dem onstration-and-waiver-list/index.html. Eligibility is determined by functional and financial criteria. People with CP might qualify for waiver services if they need services to live independently and safely in a community setting that could otherwise be provided in a long-term care facility. When a person enrolls in a waiver, they receive a supports coordinator, who is an individual who helps the waiver participant define and access the supports and services they need to live independently. Transportation is one of a number of available waiver services. For those pursuing the purchase of an adapted van, there may be state grants, low-cost loans, or other programs available such as the Alternative Financing Programs which will be further discussed in the Assistive Technology section of this chapter. These programs are open to all people regardless of the type of disability or health condition. However, programs will vary as to the amount of money that can be borrowed as well as the terms and conditions of borrowing. It is also possible for an individual to obtain a low-interest loan from their bank or credit union depending on their credit worthiness. There are currently no state grants available to fund the purchase of the vehicle chassis, but there may be grants for the adaptations needed. To find such programs it is important for individuals to do a thorough search at the time they look to make a

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purchase, either by themselves or while working with the assistance of a supports coordinator or case manager. Another good starting reference for information about transportation is the Coordinating Council on Access and Mobility (CCAM) (www.transit.dot.gov/ccam). This is a federal governmental interagency initiative established in 2004, to bring transportation services together under one website to improve services for people with disabilities, those with low income and older adults. The CCAM website provides an extensive resource library for those with disabilities and their caregivers to explore and find federal agencies and programs that can support transportation needs. However, actual services can vary widely across different geographical areas because funds for these programs are administered by states and local municipalities, potentially limiting availability. The United Cerebral Palsy Association (www. ucp.org) also provides a number of informational websites related to travel and transportation including air, land, wheeled mobility, and accessibility. The ADA requires that public transportation organizations that operate public transit services in a particular geographical area (called fixed route services) also provide complementary accessible services for those with disabilities. These are called paratransit services, which are operated by various transit companies under different names in different states and different regions. There are specific detailed rules and regulations regarding all aspects of the federal administration of paratransit services that can be found at the following link: 49 CFR Part 37- Transportation Services for Individuals with Disabilities (ADA). Specific transit providers in different localities may have some different rules but must follow the basic rules and regulations set forth in the federal guidelines. Paratransit is an “origin to destination service” (49 C.F.R Section 37.129(a)) and is a service that is meant to be more flexible than public transit lines. These services can assist those with CP to get to and from work as well as other community activities and must provide services to adults with disabilities on an individual basis to be certain that the origin to destination

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requirement is met (Federal transit administration https://www.transit.dot.gov). Those seeking paratransit services must be determined to be eligible, which is determined by an application process. The applicant must also have a physician certify the need for paratransit services. A person with a disability may also need a personal care attendant (PCA) to successfully and safely ride complementary paratransit service or a fixed route transit lines. This must be approved through the application process. Personal care attendants are not allowed to be charged a fee on paratransit when riding with a person paying a fare but may be charged when riding a fixed route transit line. The rules regarding PCAs riding fixed route transit vary from state to state. In summary, transportation is a critical service for community participation for adults with CP and is part of the ADA. However, in many communities, delivery and funding of transportation services are problematic. This section provided a summary of the current issues regarding transportation and a number of resources that can help any adult with CP find, apply for, and receive services. It is very important to remember that resources change often and vary state by state. If assistance is needed, information advocacy may be available through social service agencies like The Arc (www.thearc.org), local affiliates of the United Cerebral Palsy Association (www.ucp.org), and other local organizations and disability rights groups.

Home Health and Support Services This section will discuss both health and other support services that may take place in the home but are also important for the individual with CP to access community activities of their choosing, such as work, health and other appointments, volunteer work, visiting friends and family, leisure and recreation, and social events. Although many adults with CP are completely independent in their daily lives, there are others who need support at varying levels. Support may be for activities of daily living (ADL), including basic personal

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activities such as dressing and personal hygiene and/or for instrumental activities of daily living (IADL), which are more complex skills used in everyday life such as using a telephone and making medical appointments (Frisch and Msall 2013). IADLs also include social and recreational activities, either at home or in the community, and are important for meaningful community participation and personal choice. For adults with CP who need these services, they are essential to successfully live in their homes and participate in their communities safely and efficiently. For some individuals with more significant mobility limitations, particularly those people whose motor skills are classified at GMFCS level IV or V, support services are needed for many daily life activities as soon as they transition out of school and their family homes into community living or to postsecondary education. In the United States, most of the long- term care and support is delivered by unpaid family members or friends (www. KFF.org), but there are other avenues for longterm care as family members age or are otherwise unable to provide the care that their adult children with CP may need. These support services are known by several designations, including Personal Care Attendants (PCA), Assistant Attendant Care, Direct Support Personnel (DSP), or home health aides. There is abundant literature about the positive effects of participation in the community for mental and physical health as well as independence and selfefficacy (Crawford et al. 2008; Dattilo et al. 2008). Usuba and colleagues (Usuba et al. 2015) reported the second most common barrier to participation in leisure time physical activity by adults with CP who live in the community was lack of time with attendant care. Some adults with CP indicate that they are lonely, particularly if they do not work or have never worked (Balandin et al. 2006). Some of the other factors that have been suggested to impact social interaction are communication difficulties (Balandin et al. 2006, Ballin and Balandin 2007; Cooper et al. 2009), lack of satisfactory community participation and social connectivity, and, potentially, a change in residence that can take an individual away from family and friends (Ballin and Balandin 2007). Social

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support can take a number of forms and has been shown to be important to adults with CP (Horsman et al. 2010). Having friends and social networks at home and in community venues include family, housemates, church, and clubs. Adults with CP who were interviewed about loneliness indicated that these social networks at home and in the community are important for socialization and support (Ballin and Balandin 2007) and may be dependent upon their attendant care to access successfully. Some of these individuals with CP, but not all, talked about their care staff as friends, as people they socialize with while still acknowledging that they are paid as attendant care staff. Others felt that developing friendships with staff can be difficult and risky particularly if there is significant turnover (Ballin and Balandin 2007). Being a PCA is a very important and demanding position. It is critical that attendant care staff allow individuals with CP to make their own choices regarding important decisions in their lives. Attendant care staff must also treat individuals with CP with dignity and respect. There are no federal training requirements for direct care support staff who are paid by public funds, which is mainly through Medicaid, so training requirements vary from state to state (Marquand and Chapman 2014). However, PCAs should receive ongoing and appropriate education and training about the general issues of disability as well as the specific issues related to CP, and particularly for the individual they are supporting. There are many potential areas of education and training and different curricula that should be included for staff depending on the needs of the client. Examples of these for the caregiver working with someone with CP are how to lift clients safely and comfortably; how to use and care for adaptive equipment; how to administer medication and control infections; how to handle emergency situations; how to provide basic personal care such as bathing, eating, dressing, and oral hygiene; how to communicate effectively with clients with intellectual disabilities and/or communication difficulties; how to manage behaviors that may arise; how to respect client choice and client privacy; and how to take clients out into the community for appointments, social recreation,

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and leisure. Staff may work for an agency or can be hired directly by clients or their families. Training should be ongoing and revisited over time. If a direct support staff member is hired directly by the client or family, either the staff member or client will need to seek out training and education, which can be provided by a variety of human services agencies or state agencies. State certifications may also be available for direct care staff and may be required to work for agencies that bill Medicaid or Medicare for their services. Generally, skilled home health care, such as nursing, physical, occupational, or speech therapy, is provided on a temporary basis and the amount of service provided and payment depends on the funding source. This may be for a specific episode of care, such as postsurgical or postinjury care, or can be for episodes of increased pain, decreasing function or other needs that may arise as the individual ages. Either Part A or Part B of Medicare can pay for home health care, both skilled and nonskilled, but only on a temporary basis and not for long-term care (also called “custodial” care), which includes ADL needs like dressing and bathing. If Medicare will be paying for these services, they will generally be provided by individuals who are employed by agencies who must be Medicare-approved. Adults with disabilities who are on the Social Security Disability Insurance (SSDI) program also have Medicare benefits even though they are younger than age 65, which is the time when people without disabilities are eligible. There are some individuals who have both Medicare benefits and Medicaid benefits, the latter because of they are low income or eligible for waiver services. These people have what is termed “dual eligibility,” so benefits coordination between these plans, which can be complex, is important for individuals or caregivers to understand and put into place. Advocates who are employed by agencies and service coordinators who are part of the Medicaid Managed care plans that cover the individuals can help with this benefits coordination. For intermittent health care support in the home, private insurance plans paid for by the consumer or though employment, or insurance such as Medicare advantage plans that are paid

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for by the consumer, can fund these services. Since Medicaid services are different from state to state, it may also pay for these services depending on in which state the person is living. Medicare does not pay for long-term or 24-h care, or personal care or homemaker services which adults with CP who have significant disabilities may need (www.medicare.gov). Medicare also does not pay for attendants to take people out of the home to participate in their communities. In these cases, a different funding mechanism is necessary. For those individuals with CP who have low incomes, Medicaid is the primary funder of this kind of long-term care and support (Reaves and Musumeci 2015) through waivers, as discussed in the last section. For those people who are not covered by waivers, there may be other funding mechanisms such as through Medicaid or individual budgets funded by State Divisions of Developmental Disabilities (DDD). Similar to waivers, there is generally a supports coordinator the individual meets with to map out services and supports necessary to live in the community. Not all states have this funding mechanism, but for those that do (legislatively designated as “Developmental Disability” states as opposed to Intellectual Disability states), individuals can qualify depending on their level of disability. These services and the funding for the cost plays a very important role in the lives of people with CP who want to live as independently as possible but who may need ongoing support in the home and in the community.

Assistive Technology and Home and Workplace Accessibility Accommodations Assistive Technology Assistive technology (AT) is another important component for supporting a rewarding life for many adults with CP. AT can make it possible for someone to live more independently at home, participate in the community, go to school or work, and excel in those activities. Individuals with disabilities can exercise choice in their daily lives with more autonomy and be less dependent on family members and caregivers with the use of

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AT. There are many types of AT, but this section of the chapter will focus on AT laws and funding, AT solutions for the home and the workplace, and vehicle adaptations. AT has been shown to be effective to improve independence, function, and community participation in people with disabilities in the USA and other parts of the world (World Health Organization Press 2011) and to be beneficial for those living in the community using AT for everyday activities (Hammel et al. 2002). In older adults with physical disabilities, AT has been shown to assist in preventing functional decline and decreasing caregiver support. Adults with physical disabilities, including people with CP, were given AT for home ADLs and IADLs and compared to a group not provided with AT at both 12 and 24 months. Results indicated a slower progression in functional decline in those using AT and significantly more independence with tasks which decreased the need for personal care support (Wilson et al. 2009). Mortenson et al. (2013) found similar results after providing AT to a group of older individuals with physical disabilities and their caregivers. Those users who received AT reported significantly higher accomplishment and performance satisfaction and decreased difficulty with selected activities performed with their caregivers. Caregivers reported a decreased burden during the same selected activities with users (Mortenson et al. 2013). Adults with physical disabilities and mobility limitations may use mobility devices such as walkers and canes. These devices have also been shown to improve walking ability and feelings of safety while walking in this group of adults; however, they need to be used properly to avoid an additional risk of falling due to their potential destabilizing effect and the physical demands of using them (Bateni and Maki 2005). Although this study included elderly people with physical mobility deficits and did not include adults with cerebral palsy, it is reasonable to consider that some of these issues may be similar for adults with CP who wish to walk and need assistance. Children with CP are required to be provided with AT at no cost, if needed to assist them to meet

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their education-related goals and objectives in school, as part of their Individual Educational Plans (IEP) under the Individuals with Disabilities Education Act (IDEA) as of 2004. This legislation also provides for AT training for school personnel who are working with children who are using AT. Students who are not eligible to receive special education under IDEA, may still be able to receive AT in school under Section 504 of the Rehabilitation Act of 1973 (known as the Rehab Act), if it is needed to meet their educational goals. However, adults who have aged out of school need to find other mechanisms for funding their AT. The federal Assistive Technology Act of 1998, amended in 2004 (also known as the “AT Act”) defines assistive technology as “any item, piece of equipment, or product system, whether acquired commercially, modified, or customized, that is used to increase, maintain, or improve functional capabilities of individuals with disabilities” [PL 108–364, 118 Stat, 1710 § 3(4)] (Public Law 108–364: Assistive Technology Act of 1998, 2004). This law also defines assistive technology services as including evaluation, acquisition, maintenance, repair, coordination with other service providers, and training and technical assistance among others [PL 108–364, 118 Stat, 1710 § 3(5)] (Public Law 108–364: Assistive Technology Act of 1998, 2004). There is a wide range and scope of AT devices – so wide so that it can be difficult for a practitioner to figure out which device(s) can best meet the needs of the individual. The solution is to examine the functional need that is identified and then look for the AT that will best address that need. AT can be categorized into different types. Categories include (1) Durable Medical Equipment (DME), such as wheelchairs, scooters, walkers, lifting devices, hospital beds, and bath chairs; (2) Home adaptations or modifications, such as widened doorways, roll-in showers, ramps; (3) Vehicle adaptations, such as tie-downs for wheelchairs, ramps, lowered floors, and hand controls; and (4) general AT devices, such as hearing aids, augmentative communication systems, stair glides, seat lift chairs, phones that are compatible with hearing aids or have large buttons or are voiceactivated, and smart home technology.

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These distinctions are important to understand because there are different possible funding resources depending on the types of AT needed. For example, health insurance policies, including Medicaid and Medicare, will often pay for Durable Medical Equipment (DME). Waiver programs, vocational rehabilitation, and some nonprofit foundations may pay for home adaptations or vehicle modifications. Smart home devices, if classified as AT, are a covered service under many of the waivers because these devices help individuals with disabilities to live safely and more independently in their home. AT should not be planned, ordered, or used in isolation. The adult with CP and family members and/or other caregivers should be part of the conversation about the AT before it is ordered and during the time it is being used. Additionally, there are AT services that can and should be used when purchasing and using AT. Each state and territory in the United States has an AT program created and funded under the federal AT Act of 1998, and amended in 2004, as mentioned above. These programs are administered through the Administration for Community Living’s (ACL) Center for Integrated Programs, Office of Consumer Access and Determination within the US Department of Health and Human Services. ACL maintains a richly sourced website at https://www. acl.gov/programs with information on programs for community living, assistive technology, employment, grants for AT for State Programs, and many other disability-related topics. Basic information on state and territory AT programs, their activities and programs, leadership activities, and state partners can be accessed through ACL and are listed at: www.catada.info. Each state has a somewhat different focus, but all states have a combination of AT demonstration programs, AT equipment lending libraries, AT reuse programs, and state financing activities. Most programs provide training opportunities as well as Information and Assistance services. As part of the AT Act, Congress also provides annual grant opportunities for states and nonprofit organizations to establish and expand Alternative Financing Programs (AFPs). Alternative financing includes several mechanisms for attaining AT

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such as low interest or no interest loans. Currently 42 states and US territories operate such programs. Alternative financing programs are important because they can provide loans to people who would not qualify for financing from traditional banks or who could not afford the high-interest rates and short repayment periods associated with such loans on those rare occasions when they are made available. Programs vary from state to state. Here are two examples of the types of programs states may provide: the Pennsylvania Assistive Technology Foundation (PATF) (https://patf.us) is a nonprofit Community Development Financial Institution that provides education and financing opportunities for the purchase of AT. PATF provides Information and Assistance about possible funding resources. It also has three loan products, including credit-building “mini-loans” (0% interest with no fees) for purchases under $2000 and low-interest, guaranteed and non-guaranteed loans, with no fees, for larger AT purchases (up to $60,000). PATF also reports repayments to the credit reporting bureaus so that borrowers have an opportunity to increase their credit-worthiness. PATF also provides financial education opportunities for people with disabilities and their families so that they can learn new skills and be more in control of their financial future. PATF and the National Disability Institute (NDI) work with the FDIC, Consumer Financial Protection Bureau, and the National Endowment for Financial Education (NEFE) to provide scenarios and disability-specific information in their financial education materials. The Idaho Assistive Technology Project (IATP) (https://idahoat.org) is housed within their state AT program. The IATP Finance Program also has a low-interest, guaranteed and non-guaranteed, loan program for amounts that range from $500 to $10,000. Staff also provide consumers with financial education opportunities. There is a downloadable listing of each state APF at: https://patf.us/who-we-are/ Other potential sources of funding for AT include State vocational rehabilitation (VR) services, private insurance, Medicaid and Medicare, or other human services charitable organizations. State VR offices fund AT that is related to improving the ability of an individual to prepare for,

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attain, and keep employment. Depending on the specific plan, private insurance may pay for DME that is deemed “medically necessary.” Medicaid can be a funding source for people who are eligible due to low income and resources but generally only for equipment that is “medically necessary.” Medicaid can also fund AT for individuals on waiver services as already discussed or through the Supports Programs through the State Divisions of Developmental Disabilities (DDD) or Human Services. But again, these programs and what they provide are different from state to state and waiver to waiver, so consumers, family members, or other caregivers need to find out what is available in the state in which they live. If a person with CP is already on Medicaid for health insurance, on a waiver, or a client of a state DDD, case managers in those programs can assist with more specific information about Assistive Technology. Medicare may fund AT under the category of DME under Part B. For a detailed list of funding sources, the following website will be a helpful place to start: https://www.atia.org/at-resources/ what-is-at/resources-funding-guide/ Home Modifications and Accessibility Adaptations Home modifications, by definition, make a physical or structural change to a home and can include many different types of AT depending on the needs of the user. Examples of modifications for inside the home are lowered kitchen counters, stair railings, stair lifts, roll-in showers for wheelchair users, expanded doorways, and automatic door openers and door sensors. These modifications can range from the simple and inexpensive to the complex and expensive. Examples of modifications outside the home include ramps, porch lifts, and railings for steps into and out of the house. These are important to enable access to the neighborhood and transportation. After examining data on the effectiveness of home modifications for 266 individuals who used public funding in England, Wales, and Northern Ireland, researchers confirmed that these expenditures made users feel more independent and confident and improved their health and well-being. Benefits came from both minor (handrails and

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grab bars) and major adaptations (bathroom modifications). The authors did find that there can be problems with home modifications, particularly with larger changes, but the research demonstrated that good results can be obtained even with major home modifications when all family members, including the individual with a disability, were part of the planning decision-making process and the integrity of the home was considered (Heywood 2001). Other Assistive Technology Devices Other AT devices, including sensory devices, tablets, and other communication devices, are an integral part of life today. These devices make it possible for people to hear and see, communicate and interact with others, learn, and play. Telecommunication devices can remove barriers to participation in everyday life. They provide opportunities for people to access and relay information, keep in touch with family and friends, access medical personnel, search for and apply for employment and educational opportunities, and keep safe in the event of an emergency. In 2010, Congress passed P.L. 111–260, the TwentyFirst Century Communications and Video Accessibility Act (CVAA). This law was passed to ensure that persons with disabilities have access to and can fully use technology for communication and equipment that is current, including video programming. This includes online video closed captioning, access to the internet, use of mobile and other phones, mobile phone internet browsers, and other communication devices and services. This also includes electronic messaging, instant messaging, and video programming and communications (Federal Communications Commission Consumer Guide 2016). The law also required that the FCC keep a clearing house for information on accessible devices, which can be found at https://ach.fcc.gov/products-and-ser vices/mobile-devices/region-na/all-manufacturer/ all-blind-features/all-cognitive-features/all-hearingfeatures/all-mobility-features/all-physical-features/ page-1-of-5/show-100/. Consumers can use this website to look for specific features they are interested in or to look at the specifications for products such as wireless phones, smart TVs, and

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mobile apps. This law also provides for manufactures and service providers to develop options for consumer accessibility or to ensure that products are compatible with equipment already used by the consumer with a disability and for a complaint process if consumers have accessibility issues. This does not mean that there is funding for all of these devices and services for people with disabilities; however, there are programs for people with low income to access services such as support services for telecommunication accessibility issues. Consumers with disabilities should contact their service providers for assistance for specific issues. Technology is changing rapidly and there are now devices that are affordable, easy to purchase, and can make lives much easier that were not available even 5 years ago. This is evident in the types of smart home devices an individual with CP can use to improve the quality of life at home and make home living more efficient and safer. Examples of these are the Amazon Echo (aka Alexa) ®, Google Home®, and the Nest ® devices used for home or office device control. These are voice- or app-controlled devices that can be used to perform daily tasks and can be used in conjunction with smart home appliances and devices already on the market. Smart home appliances can be purchased and paired with the Echo ® for the person who has limited mobility or manual dexterity. Some of the tasks these devices can do are playing music, making telephone calls, controlling the TV, answering questions, and contacting a friend or relative in case of a fall or in the event that the person needs help. Some of these devices require additional apps and set up, but they can still be installed fairly easily (Metcalf 2017; Sperling 2017). There are also several different voice-activated adaptive telephones to allow people to use the phone hands free. Workplace Modifications The ability to find and sustain employment in the community is critical for people with CP for a myriad of reasons. Work makes people feel like a valued contributor to society; it allows people to learn skills, gain independence, and to lessen their economic dependence on public funding and

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programs. It also provides opportunities for community inclusion and socialization. This is true for adults who have acquired a disability in adulthood as well. Depending on the type of disability, a change in work life may need to be considered. People with CP, as a group, have high un- and underemployment, which will be discussed in the next section in more detail. But rehabilitation technology in the workplace – which can include AT devices and services and rehabilitation engineering services to reduce barriers in work areas – have been shown to increase the odds of employment (Huang et al. 2013). Title I of the Americans with Disabilities Act (ADA) requires an employer to provide reasonable accommodations in the workplace so the person with the disability can have an equal opportunity to secure employment and to be successful in their work environments. In 2012, the US Government asked the National Academies of Sciences, Engineering and Medicine to organize a group of experts to collect data from the existing literature, the public, and recognized experts to analyze specific devices and services to better understand: (1) their availability to consumers with disabilities, (2) how they are being selected and used, (3) whether employers provide access to them, (4) costs for all aspects of equipment use, (5) who is using the AT devices and services and to what extent, and (6) how they have impacted success in the workplace. The published report (Jette et al. 2017) is available as either a summary or a full pdf report at no cost or can be purchased in paperback or E-book formats, at this website: http://nationala cademies.org/hmd/reports/2017/promise-of-assis tive-technology-to-enhance-activity-and-workparticipation.aspx. There are a number of important conclusions from this report. To summarize, AT devices that provide accommodations can be positive moderating factors for success in the workplace, but they need to be correctly prescribed and used. Even so, depending on other environmental factors and personal factors, AT may not fully remove the limitations experienced by the user. The Academy also found significant variability in availability of AT both in terms of geography and funding, causing some individuals to lack access, which can affect workplace

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performance. Funding models also vary, with some agencies providing a greater range of devices and technologies than others. They also stressed the importance of education on AT, but AT development is advancing at such a rapid rate that it difficult for service providers and others to stay informed. Additionally, to best understand how successful a device is for a particular individual, professional evaluation needs to consider the personal, environmental, and societal factors at play (Jette et al. 2017). The report also discussed the need for more research to better understand the impact of AT on inclusion and work. There are many people with CP who are working successfully and need few, if any, accommodations. However, for those who do need them, accommodations can be physical, such as accessible restrooms, enlarged doorways and ramps, or related to technology, such as accessible communication and software. Companies may also need to make policy adaptations that can include work hours, break times, and schedules (Accommodations 2017). The Job Accommodation Network (JAN), a technical assistance organization funded by the US Department of Labor, Office of Disability Employment Policy (ODEP), lists potential accommodations by disability, by topic, and by limitation on their website at https:// askjan.org/links/atoz.htm. Prior to working out accommodations, there are a number of questions that need to be asked, including the specific type (s) of limitation(s), how the work and specific job tasks are affected, and how much education the employee’s supervisor and other personnel need about CP and necessary and appropriate accommodations. Employers often need to know the best ways to provide accommodations for people with CP specifically, especially for those who have mobility and communication needs (Huang et al. 2013). It is also important to be sure that the employee has been a part of the conversation about modifications. They are the most knowledgeable about their needs. If there is a job coach or advocate, that individual should also be involved in the discussion. Many accommodations do not require complex, expensive solutions, but there are some that do. Some of the accommodations listed by the JAN are for things like

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parking spaces closer to the door to the workspace, motorized carts for mobility over long distances, and close proximity to restrooms and lunch rooms. People with CP may also need the services of a job coach or personal care attendant, and this person would need physical space in the workplace near the individual they are serving. In some cases, reduced work time and extra rest times may be necessary if fatigue is a problem. Other accommodations that are listed include alternative keyboards, desk ergonomics, and adaptive telecommunications as discussed above. For individuals with cognitive impairments, accommodations could include specialized instructions, “carved out” parts of jobs (when the individual could not do the entire job), extra structure, and an onsite job coach to assist both the employee and employer. These support persons are funded by Offices of Vocational Rehabilitation for individuals who are eligible for their services. This will be discussed in more detail in the next section.

Postsecondary Education, Work, and Vocational Services Adults with CP can and should be able to enjoy a meaningful and satisfying life after high school graduation. School, work, career, and employment are goals for everyone, including those with CP. Most people want to work in some way. Work and volunteering gives people purpose in life, brings them into their communities, and can provide skills and social outlets. This section will discuss issues related to postsecondary education, career, and work and provide information and strategies on how people with CP can accomplish these life goals. Postsecondary Education Postsecondary education is an appropriate option to build a career and one which is within the reach of many people with CP. Programs can be degree conferring in a university or community college, or more skill related such as in a technical/vocational school or technical skill certifications for specific jobs. Students should be sure to investigate their eligibility for scholarships and loans for certificate programs that do not confer a degree. If

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they are not eligible for scholarships or loans, vocational rehabilitation or waiver services may help to fund those programs. As high school students plan to transition to life after school, they should be planning their futures just like anyone who is nearing graduation. For people with CP, this can be a complex process and require support from the school special education personnel, the VR office, and other relevant agencies to determine the best solutions to any accommodations needed for campus living, mobility, transportation, communication, and academic life. When leaving the public educational system, postsecondary plans should be in place for immediate life after graduation, although they will likely continue to evolve as needs become more apparent and situations change. Some of the important laws that address postsecondary education that can assist students with needed accommodations and modifications are the Americans with Disabilities Act (ADA), The Assistive Technology Act (AT Act), already discussed above, and Section 504 of the Rehab Act. These laws also provide support for people entering the workplace which will be discussed in the next section. In terms of postsecondary education, all of these laws prohibit discrimination on the basis of disability and each covers different aspects of what may be needed by a student with CP on a college campus to be successful. It is helpful for students to be knowledgeable about these laws. In addition, the VR program can provide financial support to assist with educational expenses not otherwise covered, including those that are specifically disability related such as PCAs and other educational support services and personnel (U.S. Department of Education 2017). Schools may not deny an individual admission as long as the person meets the basic requirements for admission, nor can a school charge a person with a disability more for their education than any other student who does not have a disability. Prospective students also need an understanding of their rights and responsibilities in requesting accommodations. First, the student should research schools that meet their academic needs, like any college-bound student; they should also research other issues about the school, including

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building and facility accessibility, physical layout of the campus, campus and surrounding transportation, academic supports, and other disability services and accommodations the schools provide. Students with disabilities talk about the value of having faculty members they can talk to who are knowledgeable about issues facing students with a disability (Dowrick et al. 2005) and having faculty mentors who can help when facing obstacles (Timmerman and Mulvihill 2015). All schools must provide basic disability services, but some may provide more of what an individual student is looking for and may be more welcoming to a student with a physical disability than others. For example, if a student wishes to live on campus and requires a PCA, this needs to be planned out in advance. This process will be different from the pre-college years, when, most often, the family provided most of the needed care. In this situation, the student will be responsible for finding the funding and hiring and training their attendant and working with that person on an everyday basis (Burwell et al. 2015). As was mentioned above, attendant care may be paid for through several funding options like Medicaid, a Medicaid waiver, VR, or privately. Hiring and training can be done through an agency or privately, but in either case, it is important for the student to know what they will need the attendant to do and the university physical environment, so they can hire the appropriate person and make sure they receive the correct training. If the student hires their own attendant, rather than using an agency, they need to be aware that they will need to take a larger role in the hiring process and may not have back up help if the PCA is unable to attend on a given day, unless the student has planned for that. If the student is unable to do all of the hiring and training, this can be accomplished with the help of family and/or an advocate and/or VR counselor prior to the beginning of school. Additionally, if the PCA will be providing 24/7 care, the student should discuss and may need to negotiate where their PCA will live to be accessible to the student during the night hours. That will be illustrated in the case scenario. This is a process that will give students the opportunity to take more responsibility for their own lives, learn

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to self-advocate, and develop positive relationships with their attendants (Burwell et al. 2015). After deciding which schools to apply to, the student must identify himself or herself to the school as a person with a disability in order to request accommodations. This is always a voluntary disclosure and can be difficult for the student, but in order to receive appropriate accommodations, this is an important first step, and an open dialogue is very helpful for successful inclusion into the college environment. This information is confidential and is handled by the school’s Office of Disability Services. Each school will have its own process, but the process usually encompasses meeting with Disability office staff, providing documentation of the disability, and discussing needed accommodations and how they will be accomplished. Schools do not have to provide any and all modifications requested but need to abide by the laws that govern accommodations. Even if the process has gone well from the outset, there may be issues that come up, and students will need to advocate for what they need. If particular issues are not resolved satisfactorily, all schools must have a grievance process. Work and Vocational Services Unfortunately, people with disabilities, including CP, have and continue to have high rates of unemployment and underemployment. The 2016 American Community Survey shows the percentage of employment for persons age 16 and older with disabilities to be 23.4%, compared to 66.8% for those with no disability (US Census Bureau 2016). Additionally, according to the Bureau of Labor Statistics 2016 Census data, adults over age 16 with disabilities are more likely than those without a disability to be employed part-time rather than full-time (Bureau of Labor Statistics 2017). These data include people with CP as well as a number of other disabilities including intellectual disabilities, which sometimes may accompany CP. Adults with acquired disabilities, not just those with lifelong disabilities such as CP, may have these same issues. Some of the factors found to positively impact employment are gender (men are employed more than women), higher cognitive function, access to transportation, positive

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employer attitudes, and physical accessibility (Magill-Evans et al. 2008). Higher educational level has been found to predict employment in some studies (Huang et al. 2013; Murphy et al. 2000) but not others (Magill-Evans et al. 2008). Specific barriers limiting mobility and employment sites were identified by Berbrayer (2016). Participants in this Canadian study identified the lack of timely accessible transportation, limitations in the physical environment such as the dimensions and accessibility of restrooms, elevators and lunchrooms, and computer and telephone access as the main barriers to employment (Berbrayer 2016). Other job services, provided by the VR Offices, were found to be positive factors predicting employment. These were on the job training and support, assistance with job placement, follow-along services, and rehabilitation technology (Huang et al. 2013). Additionally, those who applied for employment after age 25 had a higher likelihood of being employed, suggesting the need for longer periods of education and training for people with disabilities than for those without (Huang et al. 2013). Interestingly, SSI and SSDI cash benefits, along with other government disability benefits, can act as disincentives to accepting competitive employment particularly when the proposed job provides low pay and no, or very limited, employer-sponsored benefits, even if the individual wants to work at that job (Darrah et al. 2010; Murphy et al. 2000; Rutkowski and Riehle 2009). Restrictions on the amount of money individuals are allowed by the government to earn each month and still receive the benefits they need to survive often require that people work low-paying jobs so they will not lose those necessary benefits. In some situations, people may choose to take a higher-paying job, thereby losing most or all of their disability benefits, but they have concluded that higher salary makes the trade-off worthwhile. However, if that same individual loses that higherpaying job, they will need to re-apply for their disability benefits. This applies to cash and supportive benefits like housing and food, but also to medical benefits like Medicare and Medicaid. To assist individuals with these issues, the Social Security Administration has developed work

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incentive programs such as the Ticket to Work program to help individuals stay employed while keeping social security benefits or to expedite reinstatement of benefits should employment be lost. Information about these programs can be found at https://choosework.ssa.gov/about/workincentives/index.html. These are complicated programs and many people with CP and their families are often not adequately knowledgeable about these programs, what questions to ask and how to deal with the changes in the governmental systems, which happen frequently. Unfortunately, some of these programs, while well intentioned, can keep people under- or unemployed, so they won’t lose benefits, even if they have a college degree or other certifications, therefore not attaining their goals of independent quality of life and perpetuating a cycle of poverty (Nye-Lengerman and Nord 2016; Darrah et al. 2010). People with motor disabilities such as CP describe high costs of living with a disability and the difficulty of living on low salaries even with government benefits (Darrah et al. 2010; MagillEvans et al. 2008). It is important to be as knowledgeable as possible and to consult with VR Offices and employment specialists, supports coordinators, and other advocates who can assist people in these areas. Benefits coordinators and counselors can help people weigh the various options they need to think about when planning education and seeking employment. Even those with a college education may need the services of an employment specialist when seeking employment in their chosen field. A new program that many people with CP may be eligible for is the Achieving a Better Life Experience Act (ABLE Act). ABLE accounts are tax-advantaged savings accounts for individuals with disabilities. The monies in an ABLE account, within specific guidelines, are not counted as an asset so they are a “safe” way to save for qualifying expenses (e.g., housing, assistive technology, medical expenses, work, and transportation). If a person does not have an ABLE account, they are not allowed to have more than $2000 in savings to retain their eligibility for SSI benefits or $8000 to maintain their eligibility for waiver services (for most states.)

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With an ABLE account, however, they can save up to $100,000 (with annual contribution limits of $15,000) and still keep their benefits. In late 2017, Congress passed an amendment to the ABLE Act, the ABLE to Work Act. ABLE account owners who are employed are able to contribute an additional $12,060 annually to an ABLE account above the $15,000 annual contribution limit. For more information about the ABLE program, go to the National ABLE Resource Center, www. ablenrc.org. In 2014, President Obama signed The Workforce Innovation and Opportunity Act (WIOA) (P.L. 113–128) (1) . This is a very important law for people with disabilities who are seeking employment or postsecondary education opportunities. WIOA strengthens opportunities for community based, integrated employment and training by providing additional funding to State VR programs to encourage greater coordination between them and other organizations that provide assistance to people with disabilities. WIOA also provides for better transition services for youth exiting high school, including pre-employment activities such as internships and workplace job sampling as well as for assistance with postsecondary education planning and support for tuition and training (The Arc 2015). Many individuals with CP will need and benefit from supported employment to access and maintain a job. Supported employment was introduced in the Rehab Act with the premise that everyone has the right to community employment, to have choice and control in their career and work life, to work with colleagues with and without disabilities, and to have access to the advantages of employment, including salary, benefits, and enhanced personal satisfaction. Individuals must be approved by the state VR office for supported employment services, or services can be approved through the Division of Developmental Disabilities in DDD states. The services are often delivered by community agencies who have spent time developing relationships with the local community employers and know where they may be able to develop job opportunities to meet the needs and choices of the clients they are working with. Sometimes creative

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solutions need to be found to that take advantage of an individual’s specific strengths. This will be discussed in the case study. Service needs are determined individually and include skill evaluations and interest inventories to help employment specialists assist the individual in deciding which jobs to apply for and in finding a good match between the individual and the business (Brooke et al. 1997). Supports can also include applications and interviews and onthe-job coaching as necessary for success in a paid position. The coach acts a liaison between the individual and the workplace to assist the individual with what they will need to be successful. This can include on-the-job training, AT and workplace modifications (as discussed above), assistance with mobility needs, and other job services. Some of the specifics will need to be negotiated between the employer, the client, and the agency. This is one of the reasons that relationship development between the VR agency and employer is an important part of the job of the employment specialist and job coach. There are a number of characteristics of successful relationships between VR agencies and employers that are important to improving opportunities and outcomes for people with disabilities. For example, the employer and the VR agency must trust that each will be competent in their roles and will work well together and maintain the focus on the individual seeking employment. By doing so, they can ensure that the individual gets the support they need, no matter where that support comes from (Buys and Rennie 2001). Once the individual is doing well in the job, ongoing job coaching can be decreased and replaced with follow-along services as part of a job monitoring situation. Eventually, these services should be phased out as the individual becomes more independent. Optimally, as that happens, workplace natural supports, which are informal supports from co-workers, will develop over time. While these supports assist a person with paid employment, they are not able to be used to support a person in a volunteer, unpaid position. However, Medicaid waiver funding may be able to support someone to volunteer in the community, since community integration and inclusion is

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a cornerstone of waiver funding. This can include a job coach, attendant care, and transportation. Volunteering can be an excellent way to integrate into the community, learn skills, and develop social relationships either on its own or as an adjunct to a part time job. The following case scenario illustrates and integrates all the aspects of community living that have been discussed in the chapter.

Clinical Case Example Andrew is 26 years old and recently graduated from college with his Associate’s degree in liberal studies. Andrew has cerebral palsy (spastic quadriplegia) and is a power wheelchair user. He has very limited ability to use his hands and is, therefore, dependent on others for assistance with all activities of daily living (ADLs) and instrumental activities of daily living (IADLs). Andrew was able to attend college because of the supports he received from both the University and the PA Office of Vocational Rehabilitation (OVR). These two entities have a long history of working collaboratively so that students with disabilities have an opportunity to succeed in postsecondary education. Andrew took advantage of many of the services offered to students with disabilities. He was allowed a reduced class load (2 or 3 classes a semester) throughout his time at the University. And the Office for Students with Disabilities provided note takers for each of Andrew’s classes as well as tutors who assisted him with completing class assignments, writing papers, and studying for exams. OVR provided Andrew, and the other students who have disabilities, with attendant care in the dorm and with assistance at mealtime in the University dining halls. OVR also provided all the students with disabilities with transportation services between the dorm, dining halls, and academic buildings – a “must” because of the amount of snow and artic temperatures that occur in this region of PA as a result of the Lake (Erie) effect! But once Andrew finished school, he started planning for the next stage of his life. He recognized that he would miss talking and visiting with his friends (so easy to do when living on campus)

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and attending all of the sporting events. But, Andrew’s goals after graduation were to live in a home of his own in Pennsylvania and work parttime using his talents and interests so that he would have a career, not merely a job. Andrew started to receive Supplemental Security Income (SSI) when he turned 18. It was then that he was considered an adult and only his income – and not his family’s income – determined his financial eligibility for this program. Andrew also enrolled in one of the state’s home and community-based waiver programs (“waiver”) at this time. He met the financial eligibility requirements because he was receiving SSI and he also met the functional eligibility requirements because of the severity of his cerebral palsy. When Andrew enrolled in a waiver, he was automatically enrolled in Medicaid. So how did Andrew begin constructing his life after school? First, Andrew met with his supports coordinator for his waiver services. Together they planned what services Andrew needed to move home with his parents (both of whom work fulltime) while he looked for an apartment. It was determined that he needed personal assistance services (“attendant care”), someone to help him with activities of daily living. He also needed community integration services (someone to help him re-integrate into the community as well as help him with finances) and transportation services (tokens for the Paratransit system.) Andrew’s supports coordinator also advised him as to how to look for attendants and a communityintegration specialist. Second, Andrew met with his Office of Vocational Rehabilitation (OVR) counselor. Because OVR’s mission is to help people with disabilities obtain competitive employment (i.e., a job with a salary of minimum wage or higher), OVR has contracts with agencies that can provide direct employment services. Andrew was quickly connected to one such agency. The employment agency developed a service plan with Andrew that included a skills assessment, career exploration, and job coaching. His employment counselor also recommended that Andrew use all of his networking abilities (as well as those of his parents) to connect with possible leads for a desired job. Andrew and his parents hosted a

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“Let’s Get Andrew a Lead” party. While twenty of their friends munched on goodies, they learned about Andrew’s interests and abilities. Ideas of where Andrew could look, and who he could talk with, flew around! By the end of the afternoon, Andrew and his employment counselor had a plan in place. Andrew followed his new employment plan and he scheduled informational interviews. One of his connections was the local professional basketball team! After several interviews with the team’s vice-president of business operations and the team’s chief statistician, Andrew was offered a part-time job doing statistical research and as well as the opportunity to welcome members of the press before each of the team’s home games! This was a great opportunity! The job played right into Andrew’s strengths – a love of sports, the ability to research, a memory for details, and a joy in developing relationships. So, how could Andrew make this work? First, he wanted to talk with his co-workers about his disability so they would have a greater understanding about cerebral palsy and, therefore, be more comfortable collaborating with him on projects. Andrew wanted to explain what he could do all by himself, and what his attendant would help him with. He also wanted his co-workers’ assistance with the redesign of the office layout so that he and his wheelchair would have enough room so that he could avoid bumping into others whenever he entered or exited the office. Second, Andrew wanted to meet his new supervisors along with his job coach. Andrew wanted to learn the details of his new job responsibilities, including what others had found to be the most reliable research sites and the format he would be expected to use when he submitted his findings. He counted on his job coach to help him set up the systems he would need to do his job well. Next, during the interviewing process, Andrew was told that he could work from home from time to time – particularly when researching and charting previous players’ performances. He had a computer that was loaded with all of the necessary software, but he needed a computer table that his wheelchair could fit under so that he could sit closely and read the small print of newspaper articles and data sets.

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OVR agreed that his was a reasonable accommodation and they funded an adjustable table for Andrew. Fourth, within a few weeks, Andrew discovered that he couldn’t rely on the public transportation system, Paratransit, to get him to workon time! So, Andrew talked with his OVR counselor and his counselor approved the agency’s paying for the adaptations needed for a new vehicle. Andrew was referred to Pennsylvania’s Alternative Financing Program (AFP), Pennsylvania Assistive Technology Foundation, for a low-interest loan for the vehicle’s chassis. Andrew had good, albeit “thin” credit (he was paying back his student loan), so he qualified for a loan. Now, Andrew had the funding package he needed to get a new, reliable, adapted passenger vehicle! Andrew’s attendants are the primary drivers of his van. As part of Andrew’s interviewing process, he makes sure that successful candidates have a valid driver’s license, are good drivers, and have experience driving an adapted vehicle. The attendants don’t have to carry their own vehicle insurance on the van because Andrew’s insurance covers anyone he designates to drive him. Lastly, Andrew’s doctor also recommended that two pieces of equipment be added to his power wheelchair so that he could travel safely in his new van – a chest harness for stability and connectors on the wheelchair frame for the tie-downs to the floor. Both were approved by Andrew’s Medicaid carrier and the modifications were made to the wheelchair before the van was ready for delivery. Now, finally, Andrew could focus on his goal of living in a home of his own! Andrew has a friend with a disability who also wanted to live independently so after talking about their compatibility, they decided to be roommates. His community integration specialist (and parents) helped them develop a budget using a financial education book called Cents and Sensibility: A Guide to Money Management (Pennsylvania Assistive Technology Foundation fifth Edition 2017) so they could figure out how much they could afford for rent and utilities. Andrew and his friend developed a list of “musts” for their new apartment – it must be accessible and located in a safe area that

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has sidewalks; is near a pharmacy, store, restaurants, and a bank; has access to public transportation so that it’s easy for attendants to travel; is nearby to one set of parents; and, finally, is not too far from the sports stadiums. After searching for about 5 months, Andrew found the perfect place! He and his friend signed a multi-year lease – and their supports coordinator for their waiver services was able to amend their plans to include funding for a barrier-free shower and increased hours for attendant care. The increase in hours was necessary now that they were living independently and their parents could no longer provide some of their care. Fast forward. It’s been a couple of years since Andrew transitioned to his new life after college. He has a new job that offers greater flexibility and a higher salary. He also receives SSI, but the amount varies every month depending on how much he earned the previous month. The neighboring business owners know Andrew and cheerfully greet him as he conducts business in their pharmacy, diner, or coffee shop. Finally, there was a recent ruling in federal court that provides a clarification to Title III of the Americans with Disabilities Act that ensures that Andrew, and others like him, are no longer required to pay an additional admittance fee for their attendant in order that he (Andrew) can attend and access sporting events, museums, and other public venues (Anderson 2016). This court decision can be found online at: https://www.paed.uscourts. gov/documents/opinions/16D0367P.pdf. Suffice it to say, Andrew is living the independent life he dreamed of in his chosen community – and he is able to attend as many basketball, football, and baseball games as his budget allows.

References (2014) Workforce Innovation and Opportunity Act Accommodations (2017) Accommodations [Online]. United States Department of Labor. https://www.dol.gov/odep/ topics/Accommodations.htm. Accessed 28 Dec 2017 Anderson M (2016) Why the Franklin Institute can no longer charge disabled guests twice. www2.law.tem ple.edu/voices/category/faculty-commentary/page/3/. Accessed 27 July 2016

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Balandin S, Berg N, Waller A (2006) Assessing the loneliness of older people with cerebral palsy. Disabil Rehabil 28:469–479 Ballin L, Balandin S (2007) An exploration of loneliness: communication and the social networks of older people with cerebral palsy. J Intellect Develop Disabil 32:315–326 Bateni H, Maki BE (2005) Assistive devices for balance and mobility: benefits, demands, and adverse consequences. Arch Phys Med Rehabil 86:134–145 Berbrayer D (2016) Identifying barriers to mobility as it relates to employment in adults with cerebral palsy. J Rehabil Med (Stiftelsen Rehabiliteringsinformation) 48:96–96 Bezyak J, Sabella S, Gattis R (2017) Public transportation: an investigation of barriers for people with disabilities. J Disabil Policy Stud 28:52–60 Boucher N, Dumas F, Maltais DB, Richards CL (2010) The influence of selected personal and environmental factors on leisure activities in adults with cerebral palsy. Disabil Rehabil 32:1328–1338 Brinkey L, Savoie C, Hurvitz EA, Flannagan C (2009) Patients’ and health care providers’ knowledge of wheelchair transportation issues. Assist Technol 21:35–46 Brooke V, Inge K, Armstrong A, Wehman P (1997) Chapter 1: supported employment: a customer-driven approach. In: Supported employment handbook: a customer-driven approach for persons with significant disabilities manual. Virginia Commonwealth University, Richmond Bureau of Labor Statistics (2017) Persons with a disability: labor force characteristics summary. United States Department of Labor, Washington, DC Bureau of Transportation Statistics (2003) Transportation difficulties keep over half a million disabled at home. Washington, DC Burwell NR, Wessel RD, Mulvihill T (2015) Attendant care for college students with physical disabilities using wheelchairs: transition issues and experiences. J Postsecond Educ Disabil 28:293–307 Buys N, Rennie J (2001) Developing relationships between vocational rehabilitation agencies and employers. Rehabil Couns Bull 44:95–103 Cook JA, Burke J (2002) Public policy and employment of people with disabilities: exploring new paradigms. Behav Sci Law 20:541–557 Cooper L, Balandin S, Trembath D (2009) The loneliness experiences of young adults with cerebral palsy who use alternative and augmentative communication. Augment Altern Commun 25:154–164 Crawford A, Hollingsworth HH, Morgan K, Gray DB (2008) People with mobility impairments: physical activity and quality of participation. Disabil Health J 1:7–13 Darrah J, Magill-Evans J, Galambos NL (2010) Community services for young adults with motor disabilities – a paradox. Disabil Rehabil 32:223–229 Dattilo J, Estrella G, Estrella LJ, Light J, Mcnaughton D, Seabury M (2008) “I have chosen to live life

2562 abundantly”: perceptions of leisure by adults who use augmentative and alternative communication. Augment Altern Commun 24:16–28 Dowrick PW, Anderson J, Heyer K, Acosta J (2005) Postsecondary education across the USA: experiences of adults with disabilities. J Vocat Rehabil 22:41–47 Federal Communications Commission Consumer Guide (2016) Twenty-first century communications and video accessibility act [Online]. https://transition.fcc.gov/cgb/ consumerfacts/CVAA-access-act.pdf. Accessed 24 Dec 2017 Frisch D, Msall ME (2013) Health, functioning, and participation of adolescents and adults with cerebral palsy: a review of outcomes research. Dev Disabil Res Rev 18:84–94 Hammel J, Lai JS, Heller T (2002) The impact of assistive technology and environmental interventions on function and living situation status with people who are ageing with developmental disabilities. Disabil Rehabil 24:93–105 Heywood F (2001) Money well spent. The effectiveness and value of housing adaptations. Joseph Rowntree Foundation, Bristol, UK Horsman M, Suto M, Dudgeon B, Harris SR (2010) Ageing with cerebral palsy: psychosocial issues. Age Ageing 39:294–299 Huang IC, Holzbauer JJ, Lee EJ, Chronister J, Chan F, O’neil J (2013) Vocational rehabilitation services and employment outcomes for adults with cerebral palsy in the United States. Dev Med Child Neurol 55:1000–1008 Jette AM, Spicer CM, Flaubert J (2017) The promise of assistive technology to enhance activity and work participation. The National Academies of Sciences, Engineering and Medicine, Washington, DC Magill-Evans J, Galambos N, Darrah J, Nickerson C (2008) Predictors of employment for young adults with developmental motor disabilities. Work 31: 433–442 Marquand A, Chapman S (2014) The national landscape of personal care aide training standards. UCSF Health Workforce Research Center on Long Term Care, San Fransisco Metcalf L (2017) 5 ways to utilize your Amazon Echo. www.eastersealstech.com/2017/01/11/5-ways-utilizeamazon-echo. Accessed 23 Dec 2017 Mortenson WB, Demers L, Fuhrer MJ, Jutai JW, Lenker J, Deruyter F (2013) Effects of an assistive technology intervention on older adults with disabilities and their informal caregivers: an exploratory randomized controlled trial. Am J Phys Med Rehabil 92:297–306

M. N. Orlin and S. Tachau Murphy KP, Molnar GE, Lankasky K (2000) Employment and social issues in adults with cerebral palsy. Arch Phys Med Rehabil 81:807–811 Nye-Lengerman K, Nord D (2016) Changing the message: employment as a means out of poverty. J Vocat Rehabil 44:243–247 Pennsylvania Assistive Technology Foundation 5th Edition (2017) Cents and sensibility, a guide to money management, 5th edn. https://patf.us/wp-content/ uploads/2017/04/PATF_FinanceBooklet_5thEd_FINALALLc_508.pdf. Accessed 7 Jan 2018 Public Law 108-364: Assistive Technology Act of 1998, A. I. (2004) PL 108-364: Assistive Technology Act of 1998, amended in 2004 Reaves E, Musumeci M (2015) Medicaid and long-term services and supports: a primer. https://www.kff.org/ medicaid/report/medicaid-and-long-term-services-andsupports-a-primer/. Accessed 19 Dec 2017 Rutkowski S, Riehle E (2009) Access to employment and economic independence in cerebral palsy. Phys Med Rehabil Clin N Am 20:535–547 Scheer J, Kroll T, Neri MT, Beatty P (2003) Access barriers for persons with disabilities: the consumer’s perspective. J Disabil Policy Stud 13:221–230 Sperling (2017) Amazon Echo and Google Home for the disabled. https://ilcnsca.org/amazon-echo-google-homedisabled/. Accessed 23 Dec 2017 The Arc (2015) WIOA: what it means for people with intellectual and/or developmental disabilities (I/DD). The Arc, Washington, DC Timmerman LC, Mulvihill TM (2015) Accommodations in the college setting: the perspectives of students living with disabilities. Qual Rep 20:1609–1625 US Census Bureau (2016) American Community Survey 5 year estimates, Table S1811; American FactFinder. http://factfinder.census.gov. Accessed 10 Jan 2017 U.S. Department of Education, O. O. S. E. a. R. S. (2017) A transition guide to postsecondary education and employment for students and youth with disabilities. Washington, DC Usuba K, Oddson B, Gauthier A, Young NL (2015) Leisure-time physical activity in adults with cerebral palsy. Disabil Health J 8:611–618 Wilson DJ, Mitchell JM, Kemp BJ, Adkins RH, Mann W (2009) Effects of assistive technology on functional decline in people aging with a disability. Assist Technol 21:208–217 World Health Organization Press (2011) World report on disability. World Health Association Press, Geneva, Switzerland

Part XXVII Body Structure and Functions

Postural Control in Children and Youth with Cerebral Palsy

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Contents Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Systems Underlying Posture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Development and Theory . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Categorizing and Testing Posture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Deficits in Posture in Cerebral Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Goals and Environment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2570 Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Assessments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Principles of Motor Learning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Interventions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Evidence of Effectiveness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2578 Evaluation of Research . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2578 Theoretical Concepts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2579 Professional Practice Reflections with Respect to Device Modifications . . . . . . . . . 2584 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2584 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2584

Abstract

Deficits in movement and postural control are defining characteristics of cerebral palsy. Postural control is defined as the ability to align and adjust body segments against gravity without falling or collapsing. Posture involves complex neural processes that must be coupled to biomechanical and environmental constraints

S. L. Saavedra (*) · A. D. Goodworth Department of Rehabilitation Sciences, University of Hartford, West Hartford, CT, USA e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2020 F. Miller et al. (eds.), Cerebral Palsy, https://doi.org/10.1007/978-3-319-74558-9_161

and can be categorized in terms of static, active (or anticipatory), and reactive control. Because ability to control posture is an integral part of all movement, deficits in the posture system contribute to challenges in body structure and function, daily activities, and participation. There is a very high burden of care for those with severe posture deficits. This chapter (1) defines postural stability from a systems perspective; (2) reviews the impact of posture deficits on body function and structure, daily activities, and participation across levels of the Gross Motor Function Classification Scale (GMFCS); (3) summarizes 2565

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assessments of postural control for sitting and standing; and (4) describes current interventions from the perspective of motor learning principles. The number of published interventions directly aimed at improving postural control is limited. Moreover, the type of intervention, outcome measures, and quality of studies vary significantly between ambulatory and nonambulatory children. For those at GMFCS levels I and II, interventions refine the existing posture. For those at GMFCS level III, children must often choose between task performance and postural control and typically prioritize the functional task. Children at GMFCS levels IV–V have undeveloped postural control and require contextual modifications to enable opportunities for basic acquisition and practice of head and trunk control. Keywords

Balance · Posture · Segmental · Sensory · Motor learning

Introduction Systems Underlying Posture Deficits in postural control have been consistently included in definitions of cerebral palsy (CP) since it was first recognized in 1861 (Rosenbaum et al. 2007). Impairments in CP can exhibit as clumsiness or frequent falls during ambulation, difficulty reaching, or difficultly developing or maintaining an upright sitting position. However, before describing deficits in posture associated with CP, this chapter begins with a brief overview Fig. 1 Postural control refers to the ability to control our bodies above our base of support to hold and adjust a specific position to accomplish a task. Posture requires a complex array of neural processes that function within the constraints of the body

of postural control and how it is typically developed. Postural control refers to the ability to control our bodies above our base of support, to hold and adjust a specific position to accomplish a task. The base of support often refers to the area under the feet in standing or the area in contact with a seat in sitting. However, the base of support can significantly increase if another body segment is in contact with a stable object, such as when a person grabs a rail during stair climbing. Postural control must be maintained within environmental constraints and in response to perturbations. For the purposes of this chapter, we will focus only on upright posture with respect to gravity in sitting, standing, or walking activities. For typically developing populations, postural control is “behind the scenes” and typically involves automatic processes that require little cognitive effort. However, postural control is anything but simple (Fig. 1). For those with severe impairments, posture is not automatic. Posture is considered inherently unstable in that a small deviation from upright results in gravitational forces that further accelerate the body away from upright. There are many body segments that are all under the influence of gravity. Also, the motion of one segment generates an interaction torque on adjacent segments. Thus, all segments must be stabilized for robust postural control. The possible movements from the segments of the body are referred to degrees of freedom. For children with impairments in posture, controlling the many degrees of freedom in the body against gravity is difficult. Postural alignment and stability are further complicated by the fact that muscles used for posture serve multiple purposes. For example,

Muscle activations Selection of muscles Scaling of muscles Timing of muscles

Constraints Muscle strength Base of support Degrees of freedom

Posture Control

Sensory Processing Sensory integration Sensory weighting

Sensory Development Vision Vestibular Somatosensory

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trunk muscles must simultaneously participate in expanding and contracting the ribcage during respiration, maintain stable alignment of the body and head in space, adjust postural activations to counterbalance for positional changes of the extremities, and anticipate and respond to external load requirements. Coordination for these different tasks must allow more than one functional goal to be accomplished at the same time, often by the same muscles (Hodges et al. 2002). Sensory guidance for postural control is also complex. There is not one single sensory input that controls posture. Instead input from multiple sensory systems is integrated and weighted by the structures in the brain in determining the best response (Shumway-Cook and Woollacott 2016). The three systems that most often contribute to posture include: vision, vestibular (inner ear sense of gravity), and somatosensory (touch and muscle/body position) (Peterka 2002; Goodworth and Peterka 2012). Reliable input from these sensory systems is needed for postural stability and for interaction with the environment. The head serves as the frame of reference for motion detection by the visual and vestibular systems. Vision may be our best guide for balance when walking in daylight; however, we are able to remain upright and walk in the dark. In the dark, when vision is less helpful, our brains rely more heavily on input from touch, muscle position sensors, and the vestibular system. The brain’s ability to interpret and shift reliance from one system to a different system is remarkable.

Development and Theory The first year of life represents the most rapid change in postural control. Pathways associated with vision (Hubel and Wiesel 1970), hearing (Tees 1967), and touch (Simons and Land 1987) develop during a critical period of infancy where sensory stimuli are required to calibrate each system. If deprived of sensory stimuli during this period, neural pathways may never develop. Evidence also supports a critical period for refining vestibular processes where exposure to gravity information is needed (Jamon 2014). Similarly, the postural control system requires sensory

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stimuli and practice with gravity to calibrate and integrate multiple sensory systems. Typically developing infants gain postural control from the top-down. Infants first learn to raise their head upright, followed by development of postural control over their trunk, leading to independent sitting, and then pulling to stand and beginning to walk (Shumway-Cook and Woollacott 2016). As infants achieve upright alignment of each new body region, they need to expand their repertoire beyond a static posture by developing stability and freedom of movement according to the new affordances offered by the position. For example, once an infant achieves upright sitting, their hands are free to reach out and interact with objects. This requires adaptation of postural muscle forces as the configuration of the body changes when the arms reach away from the trunk in different directions and distances. Adjustments must be made in the postural muscles before and during reaching in order to stay upright throughout the activity. In addition, our musculoskeletal system also affects the potential for postural control. As infants and children grow into adult size bodies, they must constantly adapt their postural responses to accommodate changes in the distribution of body weight and increasing length of bones and muscles. The complexity of sensorimotor control for posture is phenomenal, yet typically developing infants master the basics of this control during the span of a mere 6–9 months. Beyond 9 months of age, trunk control is so efficient that it is often modeled as a single link in postural control research (Goodworth and Peterka 2012). Neuroscience research has demonstrated that the neural structures involved in motor control are highly plastic at birth and are molded by the child’s interactions with their environment. Infants are born with an abundance of neural connections and during the first years of life the nervous system goes through a period of rapid change based on the child’s interactions with the environment. These changes influence the refinement of sensory structures and strengthen sensorimotor connections for muscle synergies. From a maturational theory of motor development, the severity of the neural lesion would form an impenetrable barrier to behavior change in children with

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moderate to severe disability. A more current approach to motor development, systems theory is consistent with current neuroscience research and considers behavioral outcomes to be flexible, emergent properties that result from interaction of anatomical, physiological, and neurological components within specific task and environmental contexts. We use the general definition of systems theory (as described by Shumway-Cook & Woollcott (Shumway-Cook and Woollacott 2016)) which was first introduced by Bernstein in 1967. According to this theory, patterns of movement self-organize within the environmental and task conditions, based on the body systems and characteristics of the individual. In other words, changes in a subsystem underlying postural control and changes in the environment can enable or constrain a child from achieving postural control (Spencer et al. 2000). It is this systems perspective that we take to explore postural control in children with cerebral palsy.

Categorizing and Testing Posture Postural control can be categorized in several ways (Fig. 2) (Shumway-Cook and Woollacott 2016). Static control refers to the ability to achieve and maintain an upright position within the gravitational field. While muscle strength plays an important role in static control, it should be noted that complex neural processes also underlie static control. For static control, postural stability in quiet sitting or standing is measured. Even during quiet sitting, our bodies are constantly moving with very small oscillations that are referred to as postural sway. As the amplitude or velocity of sway increases, a child will visibly wobble. If the child wobbles far enough that their Fig. 2 Postural control can be subdivided as the ability to maintain static, active/ anticipatory, and reactive control

center of mass approaches the edge of their base of support, the child will need to take a step or reach out to secure their balance or they may fall. Active or Anticipatory control refers to the ability to adjust the postural muscles before or during a movement in anticipation of the expected perturbation. Expected perturbations can include internal (self-initiated motion that perturbs the posture system, such as reaching, walking, and even breathing) or external (predictable forces from the surrounding, such as anticipating a slippery surface or anticipating the forces to pick up an object). Anticipatory responses are learned over time and require practice. Active control is evaluated by examining the child’s ability to prepare in advance for a planned movement. This might include weight shift or activation of muscles prior to the onset of a task like stepping or reaching. Examination of reaching activities is a common method used in both laboratory and clinical tests. Reactive control refers to the ability to respond to an unexpected threat to balance. For example, if an infant is accidentally bumped or jostled, the postural control system must quickly select and activate the correct muscles in a coordinated manner in order to return the body to an upright balanced position before a fall occurs, or if an older child slips or trips, he/she must alter his/her stepping pattern to prevent a fall. The underlying premise of reactive testing is that the participant must first achieve a position of balance either standing or sitting and then an unexpected external perturbation will challenge balance. The movement distance, recovery of position, change in ground reaction forces, or muscle activation patterns are examined and compared across different types and directions of perturbation and between children with typical development and children with cerebral palsy.

Static Ability to quietly sit or stand upright

Active Ability to balance in voluntary movements

Reactive Ability to respond to a perturbation

Force

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For posture testing, environments can be altered to assess the impact of sensory systems. Visual contributions to posture are assessed by comparing balance during different visual conditions such as eyes closed or moving visual surround. Altering the type of support surface or placing vibration on muscles can be used to assess contributions of cutaneous touch and pressure sensors or muscles sensors. Vestibular input to posture is usually assessed by tipping the child sideways and observing if the child corrects their alignment.

Deficits in Posture in Cerebral Palsy Postural deficits in children with CP vary widely based on the level of severity. Therefore, it is helpful to categorize CP across severity. The Gross Motor Function Classification System (Palisano et al. 2008) (GMFCS) was developed as a method of classifying children with CP on the basis of functional abilities and limitations. Since the original publication in 1997, the GMFCS has become widely used around the world as way to describe gross motor function of children with CP. The GMFCS includes five levels of gross motor function across 5 age bands that span from birth through 18 years. In general, children who are in Level I demonstrate relatively “good” postural control and walk without limitations. Children in Level II walk independently but have some limitations based on environmental conditions that present heightened challenges to posture. Children who are in Level III have noticeably impaired postural control and therefore walk using a hand-held mobility device like a walker, cane, or crutches and have self-mobility through crawling or scooting while sitting on the floor. Those children who are classified as Level IV have impairments in trunk postural control. Their limitations typically exhibit impairments in self-mobility and they may use power mobility for function. Children who are in Level V are transported in a manual chair and are unable to accomplish self-mobility. Posture deficits in children at Level V are severe with very little trunk postural control with frequent deficits in their control of head position. For those in Levels III–V, there is

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similarity in postural control between level of severity of CP and typically development infants, where Level V is associated with head control only (similar to 2–3 month old infant) and Level III may have independent sitting but not walking (similar to 7–8 month old infants) (Saavedra and Woollacott 2015). With the advent of motion analysis and computer processing in the last 30 years, research has given more detail into differences in static, active, and reactive postural control in CP compared to typically developing peers (Shumway-Cook and Woollacott 2016). The majority of research has been completed with subjects who can stand and walk independently (typically GMFCS levels I and II) (Woollacott and Shumway-Cook 2005). In these studies, CP is associated with larger sway in static posture, abnormal sensory integration of vision and vestibular cues (more often in spastic diplegia and ataxia compared to hemiplegia), and different patterns of muscle activation. Active posture associated with voluntary reaching has fairly typical anticipatory posture adjustments but have slower and shorter reaches. Reactive postural control is associated with different and delayed functional muscle activations and more stepping or falling. During gait, many of the movement patterns associated with CP pose a heightened challenge to postural control, such as tripping or instability due to deficits in lower extremity alignment, strength or coordination. Impairments in trunk and head control are also present in CP. Trunk control is important because it creates the foundation for most functional skills and is critical for production of speech as well as swallowing, eating (Redstone and West 2004; Stevenson 1995), and reaching (Santamaria et al. 2016). Reactive trunk posture impairments are evident as high co-activation of muscles, poor modulation of posture responses, and different timing patterns of muscles: with a top-down sequence of activation compared to typical development where a bottom-up pattern is prevalent (Brogren et al. 1998). This difference implies that children with CP may prioritize head stability. For those with moderate-to-severe CP (GMFCS levels III–V), impaired trunk posture is a major factor limiting independent standing and walking. These children exhibit delays in static, active, and

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reactive control. For children who are able to sit independently (typically GMFCS level III), ability to align vertically, especially during hands free sitting can be challenging. Static posture typically exhibits a posterior tilt of the pelvis and kyphotic trunk with forward head position when sitting and an anterior tilt with excess lumbar lordosis during standing. These classic postures are likely adopted as compensation to limit the number of degrees of freedom that need to be balanced against gravity (i.e., reducing the available movement in head and spinal segments). For those who cannot sit independently (GMFCS levels IV–V), it is nearly impossible to describe static, active, and reactive postural control using conventional methods. Historically, researchers and clinicians have investigated the trunk as a single unit, typically describing a child as either exhibiting independent sitting or not. This “all or nothing” approach does not capture the segmental developmental spectrum and is not informative for understanding populations with underdeveloped or impaired sitting. Until recently we did not have tools to adequately study posture in those with severe impairments. However, recent research is investigating trunk and head posture using a segmental approach, where external trunk support is provided and postural control is described in body segments above the support. While this is a new area of research, the segmental approach paired with modern technology is opening opportunities to understand postural control in children who face severe motor control challenges (Goodworth et al. 2017).

Goals and Environment Postural control serves as the foundation for all motor skills (Shumway-Cook and Woollacott 2016). As such, posture impacts every aspect of function, encompassing all four components of the World Health Organization’s International Classification of Functioning, Disability and Health (ICF). The impact on (1) body function and structure, (2) activities and participation, (3) personal and (4) environmental factors depend on the severity of the child’s cerebral palsy. If a child is ambulatory, improved postural control

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might make them safer in complex environments but may not have a distinct impact on their potential for participation. Whereas for a nonambulatory child who lacks head control, improved postural control may not alter mobility but could improve participation by improving head stability for better visual interaction with the environment and allowing the child to make eye contact and observe facial gestures for increased social interaction. The burden of care and challenges faced by families of children with CP are directly related to the child’s level of postural control. Children at GMFCS levels I and II have deficits in postural control that may interfere with speed, stability, or agility when walking or running, or cause challenges to safety and attention in complex environments or on uneven surfaces. Children with this level of severity gain independent ambulation before 6 years of age (Palisano et al. 2008). Postural deficits contribute to poor alignment or difficulty coordinating balance responses and this can lead to clumsiness, decreased energy efficiency, and increased fatigue. These challenges can lead to difficulty participating in age-related physical activity on the playground or during sports related activities especially as children reach adolescence and performance requirements for the activities become more demanding. Children who are classified at GMFCS level II may require adaptations to enable participation in physical activities or sports. Nevertheless, deficits are minimal enough to allow children with these levels of severity to move freely in their home and community and to practice postural control throughout their day. Children classified at GMFCS level III learn to sit by 2–4 years of age, frequently using “Wsitting,” and learn to walk using a hand-held mobility device by 6 years of age (Palisano et al. 2008). Deficits in postural control limit the child’s activity and participation not only in the community but also within their homes. These children need to hold on to a person or a support surface if they want to transfer from sit to stand or from the floor to stand. The child often must choose between task performance and postural control. For example, the “W-sit” position reduces the degrees of freedom for the lumbar spine and

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pelvis thus reducing the demand for postural control. This allows the child more freedom for upright head and upper body movement including having at least one hand free to play with toys. Unfortunately, while this helps the child with the current task performance, it is a compensatory strategy that locks the posture system in such a way that the child is not practicing active postural control when reaching. That is, the child is not learning to coordinate posture with voluntary activities. Children with this level of severity often need additional pelvic or trunk support in order to free both hands for bilateral hand activities. Postural deficits also lead to body structure and functional limitations. Muscle imbalance and altered bone growth can arise from abnormal posture alignment, minimal time in standing and walking, and movement patterns used to compensate for inadequate postural control. These secondary deficits restrict the child’s ability to achieve good postural alignment and increase the risk for hip dysplasia, lower extremity surgeries, and medications or injections for muscle tone management. For these reasons, the burden of care is higher for parents of children at GMFCS level III. These children do, however, have adequate postural control and self-mobility for some level of autonomy and independence. They need adaptations (hand-held mobility device, manual or power wheel chair) to enable physical activities and sports in the community. Context modifications can be used in the home or school settings to increase the options for the child to practice active postural control without having to choose between posture or task performance. One example of a home-based contextual modification is the sit-to-stand device shown in Fig. 3 that allows the child to spontaneously practice weight shifts, reaching, sitting, and standing for postural control while engaged in eye-head-hand coordination and mobility skills. For children who are classified at GMFCS level IV, deficits in posture interfere with development of adequate trunk control for independent sitting. The child may be able to floor sit, when placed, but is not able to sit erect and needs to use hands for balance, thus limiting the ability to reach for and interact with toys. Children with this level of severity are able to assist with sit to stand when

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an adult helps them. They may be able to walk short distances with a walker with close adult supervision; however, they have limited selfmobility. Poor sitting balance contributes to delays in mastering skills such as eating, object manipulation, and eye-head-hand coordination for reaching (Redstone and West 2004; Santamaria et al. 2016). With age, these children spend increasingly more of their day in a wheelchair. Community mobility requires adaptations including power wheelchair and/or physical assistance. These children face similar or greater risks for muscle imbalance and boney deformities as those at GMFCS level III. Positioning devices for children with this level of severity are often geared toward ease of care and propping the child upright but are usually not adjusted with the intent of offering the opportunity for practice of postural control. As in the case of children at GMFCS level III, deficits in postural control for children at GMFCS level IV have a significant effect on body structure and function, activity and participation and contribute to a high burden of care for the family. More importantly, children with this level of severity are dependent on others to help set up the context and offer assistance for any opportunity to practice postural control. Figure 4 shows a modification to the home-based sit to stand device that can be used by children at GMFCS level IV or V. The modification includes a sit-to-stand box surrounding the child that allows unlimited practice with sit to stand, weight bearing, and weight shift and can offer the parent some respite. One parent using this device reported “this is the first time since my child was born that I have been able to relax and take time for self-care. I can put him in this device and he is happy to play for 20–25 minutes without needing input from me.” Unlike the child at GMFCS level III, the sit-to-stand box does not allow the child with more severe posture problems to explore postural control. With this modification, children often use excessive trunk stiffness, a compensatory strategy to allow upright alignment that limits variations in posture. Children often need to hold on to a support bar or lean against the box to remain upright and the ability to turn and look around or reach in different directions is limited. To practice posture with variable movements and

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Sit to stand Book or toy

practicing up and down movements

Bolster

Encouraging movement toward edge of support

Fig. 3 Example of a context modification in a sit-to-stand device for a child at GMFCS level III. A firm bolster with diameter approximately the length of the child’s lower leg and long enough to extend through both sides of the box and still provide space outside the box for the child to sit. The bolster is secured by cutting holes to allow it to be inserted through the bottom of a large box. The box serves to stabilize the bolster, provide a play surface for the child, and provide a stable surface the child can use to assist with sit to stand. The bolster encourages sitting with legs abducted and feet on the floor. Sitting or standing with

Addition of surround (e.g., cardboard) for more severe CP

Fig. 4 Example of a modification of a sit to stand device to allow children at GMFCS level IV or V to spontaneously practice sit to stand. A box is placed around the child to offer additional support for safety and to allow independence

control, the child would need external support to the level where postural control is challenged (usually thoracic region for GMFCS level IV) and opportunity to practice active postural control, coordinating head and arm movements in an upright vertical alignment. Another option would be to adjust the trunk support on a gait trainer to allow the child to practice upright control when stepping or standing. This requires attention to

legs straddled across the bolster provides pelvic stability while still allowing freedom of movement for the lower trunk. In this way, the child can practice reaching and playing with toys with bilateral hands free while also practicing postural control. Weight shift and transfers can be promoted as the child gets on and off of the bolster. The child can continuously practice sit to stand easily and spontaneously throughout the day without requiring parental assistance. The child can move toward the edge of his/her base of support and weight shift laterally by reaching for objects on the floor on either side of the bolster

height and firmness of support for optimal upright position. If the support is not high enough or firm enough, the child will have to compromise either posture or stepping. Children at GMFCS level V have the most severe postural deficits with the greatest impact on body structure and function, activity, and participation and the burden of care for families is highest. These children are limited in their ability to achieve or maintain upright head or trunk postures. All mobility and transitions are dependent on caregivers (Palisano et al. 2008). These children require support devices (e.g., wheelchairs, seating systems, standers, gait trainers) for sitting, standing, bathing, and eating and often for communication (Ostensjo et al. 2005). Mobility and functional limitations are not fully compensated by equipment. These children require assistance from others for most functional tasks. Participation in physical activities or sports necessitates physical assistance and/or use of power mobility. Opportunities for practice of postural control are the most limited for children at GMFCS level V and require custom

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adaptations of equipment specifically for the purposes of allowing upright vertical practice. The effort needed to provide this type of opportunity can be rewarding because even small improvements in head control can lead to increase in active participation. If a child gains head control, the potential for eye contact and interaction with others is improved. This can dramatically change the child’s potential for social interaction and communication through facial gestures. It can change participation from the child being “present” to being “actively engaged” with other people. Not only are children at GMFCS level V limited in their ability to practice upright control but they also miss the activity-dependent integration of sensory systems that support the development and control of posture. The biggest changes in visual and vestibular systems occur in typically developing infants during the first 6 months of life, and many of these changes appear to be facilitated and integrated through emergence of active antigravity control of the head and trunk. Children at GMFCS level V often use compensatory strategies for head and trunk posture in order to stabilize their head for visual interaction. For example, one 12-year-old child we worked with had poor head control that resulted in variable movements when trying to keep his head vertical. During our laboratory posture tests, we allow participants to watch a movie and we found this particular child regularly dropped his head to his shoulder in order to stabilize his visual feedback. This compensation unfortunately eliminates any active postural control of the head. Interestingly, when his vision was occluded with a blindfold, he lifted his head up more vertically and was thus practicing postural control of his head. Ideally, he could be encouraged to combine practicing vertical head control during engaging visual tasks. This can be accomplished by offering him a flat surface behind his head. The surface guides him with respect to where vertical is and allows him to press into a stable surface to help stabilize his head upright. An example of this type of positioning is shown in the case study at the end of this chapter.

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Technique Physical therapy interventions for deficits in postural control begin with meaningful assessments, followed by activities to improve function and/or promote motor learning. Therefore, we begin this section by summarizing commonly used posture assessments for various functional levels, then review motor learning principles, and then describe common interventions for postural control for children with CP.

Assessments Figure 5 shows several of the posture assessments that have adequate research support covering a range of static, active, and reactive control for children with CP. Other posture assessments exist with variable levels of research support (Bañas and Gorgon 2014; Saether et al. 2013). The clinical assessments listed here are geared towards quantification of (1) alignment and stability for static control, (2) movement distance and stability using reaching, stepping or turning to assess active control, and (3) responses to brief nudges or being tipped sideways, forward or backward to document reactive control. Another way to document changes in posture is by examining change in motor skills that reflect underlying improvement in posture. The most commonly used global assessment that infers changes in postural control in children with CP is the Gross Motor Function Measure, however the Bruininks Oseretsky Test of Motor Performance (BOT-2) validated in typically developing children, is also used for the higher functioning children with CP (GMFCS levels I–II) and the Alberta Infant Motor Scales (AIMS) or Peabody Developmental Motor Scales 2nd edition (PDMS-2) may be used for younger children with CP. The first two measures listed in Fig. 5 (gray shading) allow evaluation of children across all GMFCS levels because they do not require the ability to sit independently. The Segmental Assessment of Trunk Control (SATCo) is measured during sitting and with arms lifted. External

2574 Fig. 5 Assessment tools used to measure postural control in children with cerebral palsy. Some assessments are performed sitting, standing, or both. Each assessment has research support across specific GMFCS levels and focuses on one or more aspect of postural control

S. L. Saavedra and A. D. Goodworth

Assessments (GMFCS levels) SATCo (I-V)

Reactive ECAB (I-V) LSS (I-IV) SACND (I-IV) PRT (I-IV)

Static / Steady State

TCMS (I-III) TUG (I-III) P-CTSIB (I,II)

support is initially provided high on the trunk where static, active, and reactive control is evaluated in body segments above the level of support. Support is gradually lowered until the patient no longer demonstrates control. Performance is rated on an ordinal scale. A strapping system is required to secure the pelvis and assistance is required to apply the nudges. Test time is about 10–15 min. The Early Clinical Assessment of Balance (ECAB) (Mccoy et al. 2014) was designed as a broad, comprehensive observational measure of overall postural stability in young children with CP less than 7 years of age. It includes a head/ trunk control section that measures head and trunk righting responses for children with more severe CP and incorporates sitting, standing, walking, and turning items for children classified at GMFCS I–III. Performance is a numeric total. Test time is about 15 min. The next group of posture assessments (Fig. 5 tan shading) require that the child be able to safely maintain a sitting position when placed on a bench or stool and thus cannot be used for children at GMFCS level V and may be difficult for those at GMFCS level IV. The Level of Sitting Scale (LSS) rates sitting ability based on amount of manual support required to maintain a sitting position. Performance is rated on an 8-point ordinal scale ranging from “unplaceable” to ability to sit with hands free and reach laterally. No special

Active / Anticipatory

equipment is needed. Test time is about 5–10 min. The Sitting Assessment of Children with Neuromotor Dysfunction (SACND) is an assessment of static and anticipatory trunk control. Sitting posture is videotaped during 5 min of quiet sitting and during 5 min when the child is encouraged to point forward at different objects. Scores are rated on a 4 point ordinal scale for categories of postural tone, alignment, balance, and stability. A Plexiglas board with toy attachments is required. Test time is about 10–15 min. The Pediatric Reach Test (PRT) is a modification of the adult Functional Reach Test incorporating sitting and standing positions and side reaching as far as possible without moving the base of support. Performance is based on reaching in all three directions and measured as a distance. No special equipment is needed. Test time is less than 15 min. Children at GMFCS level IV can be included in the sitting but not the standing portion. For children who are at GMFCS levels I–III, the Trunk Control Measurement Scale (TCMS) can be used to assess static and anticipatory trunk control. This test requires that the child have stable independent sitting on a bench or table with feet unsupported. Performance of sitting is measured while children complete a series of reaches, within and outside their base of support and attempt to remain stable while completing leg movements. Performance is rated on 2, 3,

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or 4 point ordinal scale. No special equipment is required. Test time is about 15 min. The TCMS has been used to help differentiate the effect of trunk postural control on gait in higher functioning children with CP. The Timed Up and Go (TUG) assesses the speed with which a child stands up from a chair, walks three meters, turns, walks back to the chair, and sits down. No physical assistance is given, but an assistive device can be used by the child so this can be used for children at GMFCS levels I–III. No special equipment is required. Test time is about 5–10 min. The Pediatric Clinical Test of Sensory Interaction and Balance (P-CTSIB) focuses on standing posture and the ability to incorporate information from vision, vestibular, and somatosensory feedback. Children with adequate standing balance (GMFCS levels I–II) stand in eyes open or eyes closed, on a firm or foam surface, and with or without looking into a visual dome attached to the head. Each condition modifies the type of sensory feedback available to the child. Performance is rated on an ordinal scale. A foam pad and visual dome is required. Test time is about 5–10 min. While most of these assessments have been tested for reliability (consistency within and between different testers) and validity (results accurately reflect constructs of postural control), there is sparse evidence for responsiveness (sensitivity to changes in postural control). More research is necessary to document this aspect of currently available posture assessments (Saether et al. 2013).

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the child’s focus to be on the external result of their action as opposed to the details of how their movement or posture was carried out. Finally, because most activities need to be carried out in a range of different contexts, and because children with CP have reduced adaptive responses, it is important to generalize learning through variable practice. Four hallmarks of motor learning include: improvement, adaptability, consistency, and retention (Magill and Anderson 2014). For postural control, improvement must be evident in a meaningful outcome measure. Outcome measures include several of the assessments noted above along with more mechanistic findings like quantification of alignment, postural sway, or muscle activation patterns that can be obtained in a research laboratory. After a child achieves improved postural control in a specific situation, adaptability is needed to transfer the improved control into new situations, such as a new environments (e.g., clinic and school; on a smooth and rough surface) or modified movements (e.g., maintain posture with voluntary reaching in multiple directions for different objects). Consistency refers to the child’s ability to maintain adequate performance with posture when repeating an activity, where neither fatigue nor distractions significantly degrade performance. Retention refers to the child’s ability to maintain performance goals weeks, months, and years after the intervention. Retention is particularly difficult to assess in research because longitudinal studies are logistically complex and numerous factors vary across months and years that can influence outcome measures.

Principles of Motor Learning Activities that elicit the highest levels of motor learning include activities with cognitive and motivational factors that help solidify learning (Magill and Anderson 2014). For example, it is best to use activities that are engaging to the individual child, and novel or variable. Children involved with age appropriate problem solving tend to be more engaged. Tasks that are relevant to a child’s everyday life will increase motivation and focus. In these activities, it is typically best for

Interventions Research studies exploring interventions for postural control in CP are minimal; however, the variety and quality of studies has increased over the past 20 years (Dewar et al. 2015). Techniques reported include biomechanical approaches, strength or muscle facilitation techniques, massed practice, techniques to enhance feedback, and perturbationbased approaches. Any of these approaches can

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Biomechanical Strength/Muscle facilitation

Static / steady state

Massed practice

Active / anticipatory

Enhanced feedback

Perturbation

Reactive

Fig. 6 Summary of posture interventions aligned by type of control, for children with cerebral palsy

potentially influence static, active, or reactive postural control. However, most approaches tend to emphasize one more than the other (Fig. 6).

Biomechanical One approach to improving static control and alignment is to provide biomechanical support to the postural control system. A number of studies have evaluated the effects of seating systems on alignment and the effects of external support on posture and upper extremity skills. Methods that abduct the legs and help the pelvis to tilt anteriorly tend to help children align more vertically (Harris and Roxborough 2005; Roxborough 1995). External trunk support improves reach and alignment for young children with mild CP (Saavedra et al. 2009) and children of any age with more severe CP (van der Heide et al. 2004; Saavedra and Woollacott 2015; Santamaria et al. 2016). The immediate change in alignment and upper extremity function due to changes in biomechanical context is important for considering contextual adaptations or positioning devices for children with partial trunk control. However, the most pertinent question is whether or not biomechanical approaches can also be used to promote improved postural control. Butler (1998) (Butler 1998) took the biomechanical approach to the next level when she provided a custom seating or standing device on a rocking base and added home exercises for active and reactive training. The device was adjusted based on the child’s level of trunk control such that the child was working on the edge of where they were gaining postural control and the support was gradually lowered over time as each child gained control. Families used the custom device and completed posture training activities at home 5–7 days per week, 20 min up to 2 h 30 min per day for 12–25 weeks. All 6 children (aged 2–7 years,

GMFCS levels III–IV) gained independent sitting and increased their segmental level of trunk control. Improvements were retained in 2 children who had follow-up testing at 20 weeks and 1 year post-intervention. This protocol was repeated in a recent randomized controlled trial in which 28 children (aged 2–14 years, GMFCS levels III–V) were randomized into intervention or control group. The children trained in the support device either at home or at school for an average of 84 min per week for 6 months and showed significant improvements in head and trunk control, but the improvement was not maintained at follow-up 6 months postintervention (Curtis et al. 2016). Because of the importance of helping children with severe impairment acquire basic components of postural control (head and trunk control), similar studies are on-going. Biomechanical approaches for the lower extremities for children who are ambulatory most often include the use of an ankle foot orthosis (AFOs) to prevent excessive plantar flexion and control spasticity. AFOs can be solid or flexible. By preventing plantar flexion, the AFO may lessen certain types of tripping, but an AFO also limits mobility in the ankle and therefore limits the repertoire of reactive postural control strategies (Burtner et al. 1999). Overall, there are mixed results with respect to improvement of alignment and balance in children with CP through use of AFOs (Butler et al. 1992; Harris and Roxborough 2005).

Strength or Muscle Facilitation Interventions that focus on muscle strength or muscle facilitation techniques have the potential to improve alignment and static control of posture while also contributing to improvements in muscle activation and timing for better active control.

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Motor impairments for children with CP include (1) coordination issues related to co-activation of postural muscles, (2) poor modulation of posture responses, (3) different timing patterns of muscle recruitment, and (4) weakness that can prevent the child from achieving and sustaining upright alignment. Classic strength training and task repetition techniques similar to those used in adults with increasing difficulty and/or repetitions over time have been used for children at GMFCS levels I–III. Protocols varying from 10 to 30 min sessions, 2–5 days per week for 4–12 weeks have shown variable results, some have shown increase in strength but no change in standing balance, while others have shown improvements in standing and walking or in alignment during sitting (Dewar et al. 2015). Very few of these studies have done follow-up testing for retention; one study that used a home-based strengthening program showed results that were maintained at 6-week follow-up. Kinesiotape is often used in sports training to improve muscle performance. Recent randomized controlled studies using this technique have yielded encouraging results for children with CP. Two studies applied kinesiotape, 6 days per week for 12 weeks while attending traditional therapy sessions two or three times per week. Running speed, strength, and balance improved significantly in the study for children at GMFCS levels I–II (Kaya Kara et al. 2015). Children at GMFCS levels III–V, in the other study, showed significant improvements in sitting balance and hand function, but no changes in gross motor function (Şşimşşek et al. 2011). Functional electrical stimulation is used for rehabilitation in adults and a few cases have been reported in children with CP as an aid to specific task practice. Two recent studies have reported improved alignment and sitting control for young children with CP when 30 min of electrical stimulation was administered to trunk muscles 5 days per week during a 6 week in-patient rehabilitation protocol (Dewar et al. 2015). None of the studies with kinesiotape and electrical stimulation included follow-up examinations for retention.

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Massed Practice Intensive massed practice has been effective for upper extremity constraint induced therapy or bilateral arm movement training for children with CP and for lower extremity balance in adults post-CVA. The concept of massed practice applied to postural control has not been explored very extensively. Treadmill training for children with CP is usually focused on improving gait speed or fitness; however, there have also been reports of improved standing balance for children at GMFCS levels I–III (Dewar et al. 2015). One interesting study focused more directly on massed practice of reactive posture to a predictable perturbation in standing conditions. Researchers gave a group of 6 children (age 7–12 years, GMFCS levels I–II) 100 perturbations/day for 5 days on a moveable platform (Dewar et al. 2015). This study showed improved ability to recover stability and the reduced time to recover. The improvements remained 30 days after completion of training. Enhanced Feedback Techniques that provide enhanced feedback include studies with virtual reality, video/computer games, or visual feedback. Engagement and enjoyment is typically increased in children when gaming is coupled to rehabilitation. However, improvements in postural control are less well documented. Five studies were reviewed by Dewar (Dewar et al. 2015). A total of 52 children were included. Four of the five studies included only GMFCS levels I–II. In these three studies, interventions included use of a Nintendo Wii fit balance board (3–5 weeks of 30 min of practice per session), a virtual reality program (5 days with 90 min of practice), and a balance training protocol with visual feedback of the child’s center of pressure (6 weeks with training 3 times per week). The Nintendo Wii studies showed mixed results. The virtual reality and visual feedback programs both reported improvements in standing balance and mobility scores, with retention 1 month later. One of the five studies included subjects with more severe CP. Children played a computer game in their wheelchair that aimed to improve trunk control and smoothness of

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movements (4–5 sessions over 1 week). No significant changes were found.

Perturbation Training We considered hippotherapy (individualized therapy by PT, OT, or Speech therapist using horse riding), therapeutic horse riding (program designed and implemented by a therapeutic riding instructor), and equine simulators to be perturbation approaches. The movements of the horse, while repetitive and reasonably consistent, offer greater variability than the massed practice from a moveable platform or treadmill. Two recent reviews of the effects of hippotherapy for children with CP (Zadnikar and Kastrin 2011; Tseng et al. 2013) along with the reviews listed above (Dewar et al. 2015; Harris and Roxborough 2005) yielded 12 studies that examined the effect of equine seated perturbation training on postural control in children with CP. Across the three studies using therapeutic riding, a total of 27 children (2–12 years, GMFCS levels I–IV) were included. One study involved riding 60 min 2x per week for 10 weeks and showed improved balance for all 11 children. The other two studies involving (1) 60 min 1x per week for 26 weeks and (2) a single session of before and after riding found that 50% of the children improved and 50% did not improve in balance measures and/or alignment. Across the six studies using hippotherapy, a total of 38 children (2–17 years, GMFCS levels I–V) were included. Duration varied from 20 to 50 min per session, 1 or 2x per week for 8–12 weeks. Posture and functional balance improved for children in the five studies who were at GMFCS levels I–II or I–IV and did not improve in the study that was limited to three children who were at GMFCS level V. Across the four studies using hippotherapy simulators, 77 children (3–18 years, GMFCS levels I–V) received 10–40 min simulated riding 1–2 times per week for 4–12 weeks. Posture improved as measured by postural sway tests for children in 3 of the studies. The 4th study showed significant improvements in GMFM Dim B with those children at GMFCS level V showing the strongest response. These results were not maintained at 12 week followup. Overall, these type of approaches offer some

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of the strongest evidence for improved balance in children with CP; however, only one study included follow-up and the results were not maintained at 12 weeks post-intervention (Dewar et al. 2015).

Evidence of Effectiveness In this section, we first review the strength of evidence from published research studies. However, because research is limited, we also apply theoretic concepts into suggested approaches for interventions across GMFCS levels as a guide for families and clinicians.

Evaluation of Research Overall, the research evidence for effectiveness of interventions that directly target postural control for children with CP is limited. As indicated in the previous section, a variety of methods have been created and explored for improving postural control in small numbers of children, but no single approach has risen to the top and more extensive exploration is warranted. For children who have already achieved sitting and standing balance, there are a variety of interventions that have been researched: muscle facilitation techniques (strengthening exercises and kinesiotape), massed practice (treadmill, and standing platform perturbations), perturbation techniques (therapeutic riding, hippotherapy, and riding simulators), and enhanced feedback through video games. In children with the most severe CP (GMFCS levels IV–V), there is sparse but encouraging evidence from a few randomized controlled trials, segmental training (biomechanical approach) resulted in improved head and trunk control, hippotherapy simulator (perturbation approach) showed improved performance on sitting tasks, and kinesiotape and electrical stimulation to trunk muscles (muscle facilitation approach) resulted in improved sitting alignment. The four hallmarks of motor learning (sensitive measures of improvement, adaptability, retention,

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consistency) provide a means to discuss areas that could be improved in research studies.

Improvement Most studies evaluated children before and after intervention for signs of improvement. However, measuring improvements of postural control in and of itself is challenging because most laboratory and clinical measures, while reliable and valid, have not been tested for responsivity. Moreover, children with CP can sometimes have “good” or “bad” days where emotional or behavior factors can interfere with performance measures. Identifying responsive outcome measures is particularly challenging for more severely involved children. The SATCo offers one potential measure; however, responsiveness to change has not yet been established for children with CP. Changes in gross motor function may not be adequate to document changes related to improvement of head or upper thoracic control. Posture studies for children with more severe disability should explore measures that are aligned with development of head and upper trunk control such as “social looking,” eye-head coordination for gaze control, look duration, eating and drinking skills, eye-head-hand coordination during reaching, frequency and coordination of uni-manual or bimanual reaching activities, and wheel chair driving or propulsion. Adaptability In general, the question of adaptability has been addressed by most researchers at a macroscopic level through evaluating carry-over in terms of improvements in functional activities assessed with standardized motor tests. Further studies that explore adaptability as an outcome measure at a micro level are warranted. For example, a number of studies have demonstrated that children with CP show less variability of postural sway (da Costa et al. 2017; Kyvelidou et al. 2013) and less adaptability to changes in perturbation amplitude (Brogren et al. 1998; HaddersAlgra 2008; Carlberg and Hadders-Algra 2005). Thus, interventions that help the posture system to adapt more robustly to contextual changes would be an important step forward.

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Retention In contrast to improvement and adaptability, retention was not tested for most interventions. There have been very few postural intervention studies that include follow-up evaluations to assess for retention. Some of the studies with the positive improvements were not retained. For example, retention tested at 12 weeks for hippotherapy simulator and at 6 months for segmental training did not yield a positive outcome. This is an important area for future work. Consistency While consistency has been studied for anticipatory reactions and reaching performance during a single session of postural testing, we are not aware of any studies that used measures of consistency to document the trajectory of motor learning for postural control in an intervention across hours and days in children with CP. It is expected that when an improvement in postural control is first achieved, it will fluctuate over time, with plateaus and setbacks (especially in a growing child). But eventually the improvement should solidify and become consistent.

Theoretical Concepts Posture is usually controlled at a subconscious level. We only notice our posture if it interferes with a task we hope to accomplish or if someone draws our attention to our alignment. Children are even less disposed to pay attention to posture. They are driven to move and explore their environments. These reasons likely underlie the challenges in studying interventions intended to improve postural control. Therefore, we suggest therapist should treat posture by combining an engaging task with posture demands. We do not suggest practicing posture in isolation in youth and children. When combined with a task, postural control can be “built in” to daily function. This could be accomplished by creating goal-directed assessments and adapting the posture programs with specific emphasis on the child or family’s goals. If the new skills can be incorporated into the child’s daily activities, it is likely to be more

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effective in the long term. This requires some level of individualization with respect to activities that have meaning to the child and family and matching of the intervention to the type of postural control that is most needed by the child. Our proposed model for engaging children in posture training is shown in Fig. 7. Children at GMFCS levels I and II, on the left side of the plot, spontaneously develop upright trunk control for independent sitting and standing. Some care and attention may be needed to adjust the activity for more variability and challenge for postural improvement, but these children have the potential to engage spontaneously and further refine their postural control. For example, a child who wants to play on the local soccer team would benefit from a more perturbation and repetition based balance approach like treadmill training or video games that require lots of leg movements or stepping. A child who seeks improved balance to be able to stand and play drums in the band might need more strengthening and static balance activities like playing video games while standing in one place. Children at GMFCS level III are at a pivotal location. These children face situations where they have inadequate postural control to accomplish many tasks. If environmental supports or contexts can be created that allow the child to

Spontaneous acquisition Refinement

Posture Control

Fig. 7 Schematic of patient behavior and intervention focus across levels of severity in cerebral palsy. GMFCS is the Gross Motor Function Classification System

actively use postural control, there is potential that posture and task performance could simultaneously improve. However, if contextual support is not available, the child will need to alter the task or their position to stabilize the postural component. This can be placing one hand down at all times during sitting or collapsing the trunk into kyphosis. Using external trunk support can bring the child into a range where they have the potential for successful active postural responses while also practicing new functional tasks. For those with more severe CP, one of the major challenges for gaining postural control is the child’s level of trunk control. The lack of control interferes with the child’s freedom to spontaneously practice head and trunk posture during skill performance. A therapist must focus on significant modifications to the context so these children have the opportunity to practice upright postural control. For these children, training posture must often take the form of simplifying the postural control tasks by reducing the degrees of freedom involved (providing trunk or partial head support) or providing additional avenues of support (hand rail). After the simplified posture is achieved, additional challenges can be introduced (lessening support and adding perturbations or larger voluntary movements). In this way, posture is developed incrementally, similar to the typical

posture NOT major limiting factor posture IS major limiting factor

Trade-off between function & posture

Context modifications to enable practice

Undeveloped

Focus on acquisition

I

II

III GMFCS Level

IV

V

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developmental pattern. Even in the more severe children, it is still recommended to keep posture as the underlying focus while the child is interacting with a different functional task. This approach is consistent with a recent systematic review of interventions for children with cerebral palsy. In this review, three types of training topped the list of effective methods for general functional skill performance across subgroups of children with cerebral palsy. These were goal-directed training, home programs, and context-focused therapy (Novak 2014). Goal-directed training is defined as “childactive” repetitive and structured training in selfcare tasks, e.g., dressing, designed to meet a goal meaningful to the child. In goal-directed training, the tasks and the environment are also changed to promote skill acquisition (Ahl et al. 2005). Home programs are defined as “evidence-based home programs of child-active repetitive and structured home-based practice of tasks that are meaningful to the child and their family” (Novak 2014). Context-focused therapy is a compensatory/environmental approach where the environment is adapted to promote successful task performance (Law et al. 2011). However, for children who do not spontaneously develop independent sitting, we advocate for creating an environmental approach that also specifically allows children the opportunity to practice postural control with support optimized based on the child’s segmental level of control.

Case Example

We provide an example to demonstrate the concepts for promoting opportunity to practice postural control for a child with severe CP (GMFCS level V). Much more research is needed to determine if this approach can alter the course of postural development in children with CP, but the immediate improvement in functional skills and opportunity to actively engage in practicing postural control make it a better option than the traditional care that

positions these children throughout most of the day.

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passively

History

JAS was born prematurely at 24 weeks gestation, weighing just 1 pound 13 ounces and in his mother’s words “survived a harrowing five months in the NICU.” His mother reported that “Although he didn’t have any brain bleeds and as a family, we had no indication he might have cerebral palsy, at 17 months actual (13 adjusted) he is unable to sit, roll, or crawl. He has high tone on his right side and very low tone in his core. He falls over when propped in a sitting position and can’t handle sitting in a high chair because he’s very wobbly.”

Examination and Evaluation

On evaluation for our research study at 14 months corrected age, we observed that JAS is an enthusiastic and engaging child whose fine motor skills (5% on Peabody Developmental Motor Scales, Edition 2, PDMS-2, fine motor subtests) were markedly better than his gross motor skills (