Limb Lengthening and Reconstruction Surgery Case Atlas: Pediatric Deformity [2 ed.] 3031773586, 9783031773587

Table of contents :
Preface
Booknotes
Contents
About the Editors
Section Editors
Contributors
Principles of Deformity Correction
Radiological Assessment of Lower Limb Deformities
Mechanical Alignment of the Lower Limbs (Weight-Bearing)
Frontal Plane Alignment
Sagittal Plane Alignment
Joint Orientation Lines Assessment
Frontal Plane
Sagittal Plane
Joint Orientation Angles
Frontal Plane (Fig. 12)
Sagittal Plane (Fig. 13)
Determining the Apex of the Deformity or CORA (Centre of Rotation of Angulation)
Acute Versus Gradual Correction
Gradual Correction
Advantages of Gradual Correction with 8 Plates
Advantages of Gradual Correction with External Fixators
Acute Correction
Combined Gradual and Acute Correction
References and Suggested Reading
Part I: Pediatric Deformity: Pediatric Lower Extremity Trauma
1 Pediatric Trauma: An Introduction
2 Adolescent Unstable Tibial Fracture Treated Initially with Closed Reduction and Plaster Cast Immobilization Converted to TL-...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
3 Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with PRECICE Retrograde Intr...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
4 Correction of Partial Epiphyseal Growth Arrest and 7 cm Shortening in 10 Years Old Girl
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
5 Distal Femoral Fracture Treated Initially with Internal Fixation Converted to Circular External Fixation due to Nonunion and...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
6 Oblique Plane Deformities of Femur and Tibia After Open Fracture Treated with TSF
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
7 Open Femoral Fracture Treated with Internal Fixation Converted to Circular External Fixation due to Infection
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problems List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
8 Post-traumatic Deformity and Bone Loss in 8 Year Old Boy
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
9 Proximal Tibial Growth Arrest with Varus, Recurvatum, and Shortening After ACL Reconstruction. Correction with TSF
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
10 Sixteen Year Old Male with Tibial Malunion Complicated by Pathological Fracture
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 2
References and Suggested Reading
11 Ten Year Old Male with Comminuted Distal Femoral Fracture
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
12 Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique Wire Bone Transp...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
13 Traumatic Physeal Arrest of the Proximal Tibia with Deformity in All 4 Dimensions (Length, Coronal Angulation, Sagittal Ang...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Image During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
14 TSF for Displaced Pediatric Tibia Fractures
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
15 Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral Lengthening with FITBONE Re...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problems List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
16 Valgus/Flexion and Shortening of the Distal Femur from Growth Arrest Treated with a Monolateral Frame
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
17 Vitamin D-Resistant Hypophosphatemic Rickets Treated by Double-Level Femoral Osteotomy with Internal Fixation and Proximal ...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
18 Adolescent with Segmental Bone Defect Secondary to Grade IIIB Open Tibial Fracture Treated by Oblique Wire Bone Transport
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problems List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 2
References and Suggested Reading
19 Comminuted Type I Open Distal Femur Fracture
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
Part II: Pediatric Deformity: Growth Plate Injuries
20 Growth Plate Injuries: An Introduction
21 Correction of Post-Traumatic Medial Growth Arrest of the Distal Tibia (Common Problem, but Often Neglected)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
Part III: Pediatric Deformity: Congenital Pseudarthrosis of the Tibia and Fibula
22 Congenital Pseudarthrosis of Tibia and Fibula: An Introduction
23 Congenital Pseudarthrosis of the Fibula
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
24 Congenital Pseudarthrosis of the Fibula: Direct Surgical Repair
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
25 Congenital Pseudarthrosis Tibia (El-Rosasy - Paley Type 3)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
26 Congenital Pseudarthrosis Tibia (El-Rosasy - Paley Type 2)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
27 Congenital Pseudoarthrosis of the Tibia Repaired with Massive Autograft and Tibia-Fibula Cross-Union
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
28 Congenital Tibial Pseudarthrosis Managed with Ipsilateral Vascularized Fibula Transfer
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
29 Congenital Tibial Pseudoarthrosis Treated with Internal and External Fixation Using the 4 in 1 Technique
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
30 Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteoto...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
31 Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
32 Recalcitrant Congenital Pseudarthrosis of the Tibia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
33 Congenital Pseudarthrosis of Tibia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
Part IV: Pediatric Deformity: Congenital Lower Limb Deficiencies
34 Congenital Lower Limb Deficiencies: An Introduction
Fibular Hemimelia
Tibial Hemimelia
Posteromedial Bowing of the Tibia
Congenital Short Femur and PFFD
References and Suggested Reading
35 Absent Knee Extensor Mechanism, Fixed Flexion Deformity, Leg Length Inequality and Foot Deformity in an 11 Year Old Girl Tr...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
36 Avoiding Amputation and Prosthetics in Children with Complex Lower Limb Deformities
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
37 Congenital Femoral Deficiency: Paley Type 1b and Paley Type 1c
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
38 Congenital Femoral Deficiency: Paley Type 2a
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Superhip2 Procedure
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
39 Congenital Femoral Deficiency: Paley Type 2c
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
40 Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral Lengthening Associated with...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
41 Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
42 Congenital Short Femur and Fibular Hemimelia Staged Approach Frame and Nail
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs (4-5)
Avoiding and Managing Problems
References and Suggested Reading
43 Congenital Short Femur - Lengthening Over a Rush Pin with Knee Ligament Reconstruction
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
44 Correction of Recurvatum Deformity with Osteotomy, External Fixator, and Epiphysiodesis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
45 Distal Femoral Deformity Correction Using MAC
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
46 Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral Deficiency and Femor...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
47 Femoral Lengthening with MAC External Fixation System
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
48 Femoral Shortening (14 cm) and Deformity Treated with Acute Correction and Two Consecutive Retrograde Fitbone Applications
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
49 Femur Lengthening with an Extramedullary Nail
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
50 Fibular Hemimelia and Congenital Short Femur-Staged Approach in Child
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs (4-5)
Avoiding and Managing Problems
References and Suggested Reading
51 Fibular Hemimelia: Paley Type 3
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Case 1
Case 2
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
52 Guided Growth and Syme Amputation in a Thirteen-Year-Old Boy with Type 2 Congenital Fibular Deficiency
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
53 Intraoperative SSEP Monitoring of Circular External Fixation for Revision of Brown Rotationplasty
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problems List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
54 Knee Subluxation During Femoral Lengthening in a Six Year Old Boy with Congenital Coxa Vara and Congenital Short Femur
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
55 Proximal Tibial Recurvatum and Ipsilateral Short Femur Treated with a MAC External Fixation System
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
56 Proximal Tibial Recurvatum Corrected Using the Ilizarov Technique in a 14 Year Old Boy
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
57 Sequential Deformity Correction and Tibial Elongation Using Dormant Magnetic Nail Technique
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References
58 Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot Equinus and LLD
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
59 Simultaneous Knee and Hip Dislocation During Femoral Lengthening in a Patient with Congenital Short Femur
Brief Clinical History
Chief Complaint: Right Limb Shortening
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Problems that can Occur During Lengthening
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
60 Staged Lengthening of 20 cm in the Femur and Tibia to Equalize Leg Lengths in a Growing Child
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
61 Tibia Valga Treated with Tibial and Fibular Osteotomy and Application of a Multi-axial Correcting External Fixation System
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
62 Tibial Hemimelia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
63 Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Case 1
Case 2
Technical Pearls
Type 5a
Type 5b
Outcome Clinical Photos and Radiographs
Case 1
Case 2
Avoiding and Managing Problems
Case 1, Paley 5a/Weber Patelloplasty
Case 2, Paley 5b
Cross-References
References and Suggested Reading
64 Tibial Hemimelia Type II in a 6 Year Old Child Treated with Fibular Transfer and Centralization and Symes Amputation
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
65 Tibial Hemimelia: Staged Treatment with External Fixation and Then Internal Lengthening Nail
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
66 Tibial Lengthening Over a Free Vascularized Fibular Autograft After Ewing´s Sarcoma Resection
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Technical Pearls
Avoiding and Managing Problems
References and Suggested Reading
67 Treatment of Recurrent Patellar Dislocation Using Langenskiold Reconstruction of the Patella, Grammont Patellar Slide, and ...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References
Suggested Reading
68 Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
69 Delayed Regenerate Bone Formation in a Seven Year Old Boy with Fibular Hemimelia Undergoing Tibial Lengthening
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
70 Septic Destruction of the Hip and Significant LLD Treated by Pelvic Support Osteotomy and Femoral Lengthening
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
Part V: Pediatric Deformity: Pediatric Blount Disease
71 Pediatric Blount Disease: An Introduction
72 Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
73 Adolescent Blounts Disease Treated with MAC External Fixation System
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
74 Correction of Adolescent Tibia Vara Without Fibular Osteotomy and Without Fixation of the Fibula Using the Taylor Spatial F...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
75 Correction of Juvenile Blounts Disease
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
76 Correction of Multi-planar Deformity in 120 kg 13 Year Old Patient with Blounts Disease
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
77 Guided Growth Treatment for Early-Onset Blount Disease
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
78 Hemiplateau Elevation for Early-Onset Blount Disease
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Current Considerations
Future Considerations
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
79 Infantile Blount Disease with Plateau Depression
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
80 Morbidly Obese Teenager with Significant Blounts Treated with Taylor Spatial Frame
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
81 One-Stage Hemi-Plateau Elevating Osteotomy in Advanced Blount´s Disease
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy (Figs. 4 and 5)
Basic Principles
Images During Surgery
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
82 Proximal Tibial Osteotomy in a 4 Year Old Child with Blount Disease
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
83 Simultaneous Correction of Medial Proximal Tibial Plateau Depression and Tibia Vara in an Obese Child with Blounts
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
84 13 Year Old with Unilateral Late-Onset Blount Disease
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
Part VI: Pediatric Deformity: Pediatric Arthrogryposis
85 Pediatric Arthrogryposis: An Introduction
References and Suggested Reading
86 Bilateral Congenital Vertical Talus and Dislocated Hips in a Child with Very Severe Arthrogryposis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
87 Correction of Arthrogrypotic Clubfoot Deformities with the Ponseti Method of Serial Casting
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
88 Correction of Severe Contractures, Pterygium and Lower Limb Deformities Caused By Arthrogryposis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
89 Journey of a Child Born with Severe Arthrogryposis and Lower Limb Deformities
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
90 Recurrent Knee Flexion Contractures in a 10 Year Old with Arthrogryposis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
91 Simultaneous Correction of Hip and Knee Flexion Contractures in a 5 Year Old with Arthrogryposis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
92 Synchronization of Surgical Interventions for Multiple Deformities in a Four Year Old with Arthrogryposis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
93 Talectomy for Correction of Severe Rigid Clubfoot in a Patient with Arthrogryposis
Brief Clinical History
Pre-operative Clinical Pictures and Radiographs
Pre-operative Problems List
Treatment Strategy
Basic Principles
Technical Pearls
Images During Treatment
Outcome Clinical Pictures and Radiographs
Further Reading
94 Correction of Severe Arthrogrypotic Knee Flexion Contractures
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
Part VII: Pediatric Deformity: Pediatric Skeletal Dysplasias
95 Pediatric Skeletal Dysplasias: An Introduction
96 Bilateral Genu Valgum due to Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
97 Correction of Lower Limb Deformities in Multiple Hereditary Exostosis (MHE)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
98 Correction of Tibia Recurvatum and Shortening in Skeletal Dysplasia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
99 Genu Valgum and Limb Length Discrepancy in Multiple Enchondromatosis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
100 Guided Growth Treatment for Genu Valgum Secondary to Juxtaphyseal Recurrent Aneurysmal Bone Cyst of the Distal Femur
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
101 Management of Distal Femur Deformity Following Ablation of Aneurysmal Bone Cyst, by Corrective Osteotomy and Elongation
Brief Clinical History
Pre-operative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References
102 Migration of the Fibula During Tibial Lengthening in Achondroplasia
Brief Clinical History
Chief Complaint: Short Stature
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
103 Multiple Lower Limb Deformities in a 14 Year Old Girl with Multiple Epiphyseal Dysplasia and Low Lumbar Spina Bifida
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
104 Refracture, Soft Tissue Contracture, and Angular Deformity After Femoral Lengthening in Achondroplasia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 2
References and Suggested Reading
105 Retrograde Insertion of a SLIM Nail in a Femur in a Patient with Osteogenesis Imperfecta
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
106 Revision of Bilateral Tibial Fassier-Duval Rods in an Eleven Year Old Girl with Osteogenesis Imperfecta
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
107 Revision of Femur Fassier-Duval Rod for Intertrochanteric Fracture Nonunion with Lytic Cyst and Coxa Vara in Osteogenesis ...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
108 Revision of Telescoping Rod and Plate Complicated with Nonunion and Metallosis in a Girl with Osteogenesis Imperfecta Type...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Radiographs
Avoiding and Managing Problems
References and Suggested Reading
109 Severe Genu Valgum in Skeletal Dysplasia: Acute Versus Gradual Correction
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Gradual Correction: Growth Modulation Versus External Fixators
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
110 Spondyloepiphyseal Dysplasia Treated by Bi-lateral Proximal Tibial Osteotomy Followed by Gradual Deformity Correction
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
111 Staged Femur and Tibial Lengthening During Childhood for Russell Silver Syndrome
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs (4-5)
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
112 Telescopic Rodding in Combined Technique After Transphyseal Elastic Nailing in a Boy with Type IV Osteogenesis Imperfecta
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
113 The Use of Gap Nail for Tibia Fracture in Skeletally Mature Osteogenesis Imperfecta Patient
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
114 The Use of SLIM (Simple Locking Intra Medullary) Nail in the Lower Extremities of Skeletally Immature Patients with Osteog...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
115 Type IV Tibial Dysplasia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
116 Varus Deformity of the Distal Femur and LLD Secondary to Olliers Disease Corrected by Gradual Deformity Correction and Len...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
117 Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with Telescoping Fass...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
Part VIII: Pediatric Deformity: Pediatric Metabolic and Vascular Disorders
118 Pediatric Metabolic and Vascular Disorders: An Introduction
119 Deformity Correction in Child with X-Linked Hereditary Hypophosphatemic Rickets by Combined Technique (External Fixation a...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
120 Genu Varum Secondary to Rickets Treated with Femoral and Tibial Osteotomy and Application of a Multi-axial Correcting Exte...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
121 Infantile Myofibromatosis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
122 Hypophosphatemic Rickets with Bilateral Severe Genu Varum. Retrograde Fixator Assisted Nailing for Femurs and Double Level...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
Part IX: Pediatric Deformity: Pediatric Hip Deformities
123 Pediatric Hip Deformities: An Introduction
124 Healed Slipped Capital Femoral Deformity
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References
125 Hinged Arthrodiastasis for Avascular Necrosis of the Hip
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
126 Hip Fusion with an External Fixator
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
127 Ilizarov Hip Reconstruction for Post Infective Femoral Head Destruction
Brief Clinical History
Preoperative Clinical Photos and Radiographs
X-Rays
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Intraoperative X-Rays
Technical Pearls
Outcome Clinical Photos and Radiographs
Postoperative X-Rays and Photographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
128 Percutaneous Osteotomy of the Proximal Femur for Slipped Capital Femoral Epiphysis
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Rationale
Preoperative Planning and Surgical Tips
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
129 Perthes Hip Treated with Articulated Hip Distraction and Small Diameter Core Decompression
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
130 Perthes: Femoral Head Reduction Osteotomy
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
131 Coxa Vara in a Nine-Year-Old Boy with Osteogenesis Imperfecta
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 3
References and Suggested Reading
Part X: Pediatric Deformity: Pediatric Foot and Ankle Deformities
132 Pediatric Foot and Ankle Deformities: An Introduction
133 Complex Foot Deformity in a Child with Aperts Syndrome, Treated with a Miter Frame
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
134 Correction of Severe Foot Deformity in Arthrogryposis with Midfoot Osteotomy and Ilizarov Frame
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
135 Fourteen Year Old Female with Residual Clubfoot Deformity Treated with Taylor Spatial Frame
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
136 Recalcitrant Clubfeet in a 4 Year Old with Arthrogryposis Treated with External Fixation
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
137 Severe Equino-Varus Foot and Ankle Deformity from Compartment Syndrome. TSF to Correct Deformities followed by Tendon Tran...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 2
References and Suggested Reading
138 Severe Equinus Secondary to Linear Scleroderma Treated by Gradual Deformity Correction via Circular External Fixation With...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
See Also in Vol. 2
References and Suggested Reading
139 Step by Step Approach to Cavus Foot Deformity
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
140 Treatment of Complex Clubfoot Using Midfoot Osteotomy and Taylor Spatial Butt Frame (TSF)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 2
References and Suggested Reading
141 Foot Stump Lengthening
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
Part XI: Pediatric Deformity: Pediatric Chronic Osteomyelitis
142 Pediatric Chronic Osteomyelitis: An Introduction
Cross-References
143 Cable Bone Transport for Segmental Bone Loss Secondary to Grade IIIB Open Tibial Fracture
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problems List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vols. 2 and 3
References and Suggested Reading
144 Chronic Osteomyelitis and 5 cm Bone Defect Treated with Masquelet Technique Followed by Ilizarov
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
The Masquelet Technique
Distraction of a Hypertrophic Nonunion
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
145 Correction of Post-infectious Partial Proximal Tibia Growth Arrest with Taylor Spatial Frame in a Child
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
146 Post-infection Loss of the Lateral Half of the Upper Tibial Epiphysis with Deformity and LLD
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
147 Radical Excision and Lengthening of Chronic Sclerosing Osteomyelitis of Garre (10 Years of Follow-Up)
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
148 Trifocal Distraction Osteogenesis in the Management of Sequelae of Chronic Osteomyelitis of the Tibia (Pseudarthrosis, Def...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 2
References and Suggested Reading
149 Deformity and Lengthening of Tibia Following Meningococcal Septicemia
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
Cross-References
References and Suggested Reading
Part XII: Pediatric Deformity: Pediatric Rotational Deformities
150 Derotational Osteotomies of the Femur and Tibia for Tetratorsional Malalignment
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
References and Suggested Reading
Part XIII: Pediatric Deformity: Pediatric Rotational Deformities (Miserable Malalignment)
151 Pediatric Rotational Deformities (Miserable Malalignement Syndrome): An Introduction
Cross-References
152 Adolescent with Bilateral Femoral and Tibial Rotational Deformity (Miserable Malalignement Syndrome) Combined with Proxima...
Brief Clinical History
Preoperative Clinical Photos and Radiographs
Preoperative Problem List
Treatment Strategy
Basic Principles
Images During Treatment
Technical Pearls
Outcome Clinical Photos and Radiographs
Avoiding and Managing Problems
See Also in Vol. 3
References and Suggested Reading
Index

Citation preview

S. Robert Rozbruch Reggie C. Hamdy Austin T. Fragomen Mitchell Bernstein Editors

Limb Lengthening and Reconstruction Surgery Case Atlas Pediatric Deformity

Second Edition

Limb Lengthening and Reconstruction Surgery Case Atlas

S. Robert Rozbruch • Reggie C. Hamdy • Austin T. Fragomen • Mitchell Bernstein Editors

Limb Lengthening and Reconstruction Surgery Case Atlas Pediatric Deformity – Reggie C. Hamdy and Jason S. Hoellwarth Second Edition

With 1347 Figures and 16 Tables

Editors S. Robert Rozbruch Department of Orthopedic Surgery Hospital for Special Surgery New York, NY, USA Austin T. Fragomen Department of Orthopedic Surgery Hospital for Special Surgery New York, NY, USA

Reggie C. Hamdy Department of Pediatric Surgery Shriners Hospital - Canada Montreal, QC, Canada Mitchell Bernstein Departments of Surgery & Pediatric Surgery (Division of Orthopaedics) McGill University Health Center & Shriners Hospital for Children - Canada Maywood, IL, USA

ISBN 978-3-031-77358-7 ISBN 978-3-031-77359-4 (eBook) https://doi.org/10.1007/978-3-031-77359-4 © Springer Nature Switzerland AG 2015, 2024 This work is subject to copyright. All rights are solely and exclusively licensed 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. Cover illustration: Artist: Yonina Jacobs, “New Growth” 2010, Assemblage: acrylic on canvas, 3300  1500 This Springer imprint is published by the registered company Springer Nature Switzerland AG. The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland If disposing of this product, please recycle the paper.

To my wife, Yonina, and our children, Jason and Nicole and Libby and Brett, whose love and support make me smile and give me strength every day. S. Robert Rozbruch I dedicate this second edition of the Atlas to my wife Sylvie and my son Sebastien for their relentless and unwavering support during the completion of this new edition. To my late parents, who will always remain my guiding light. To my mother, whose sweet and comforting voice will forever resonate in my ears. To my father, the best mentor I would ever have dreamed of having. He will not see this Atlas, but I am sure he would have been very proud of it. To all my colleagues for their invaluable help and especially to all the children and adolescents I have treated, you have been the most amazing inspiration to me. Reggie C. Hamdy I would like to dedicate this book to my mentors, John Denton, Eugene Wolf, and S. Robert Rozbruch. These giants shaped me as a surgeon and helped me hone in on my career path. It has been my greatest honor to join my close friend Rob Rozbruch and journey together to shape this world of limb lengthening and reconstruction. I thank my family for supporting my demanding clinical academic career. And most importantly, I thank my patients for trusting me and teaching me the true art of medicine. Austin T. Fragomen To my wife, Neilly: I love you dearly, and thank you for your continued support and encouragement. To our two wonderful children, Asher and Avery, whose boundless curiosity and joy remind me daily of the magic of learning. This work is dedicated to you, with all my gratitude. Mitchell Bernstein

Preface

Nine years after publication of the first edition, we are pleased to present the second edition of the Limb Lengthening and Reconstruction Surgery Case Atlas. The clinical work presented and the amazing subspecialty of orthopedic surgery it represents has come a long way since our first edition in 2015. Now well known internationally and in the USA, the limb lengthening community is recognized as a distinct and critical area of orthopedics despite its apparent overlap with other subspecialties. No longer entirely reliant on external fixators, we routinely use internal fixation for complex deformity correction, lengthening, bone defect management, and limb salvage. The precision we achieve in surgery is based on the core principles and the new techniques that have been developed. The evolution to the new often borrows from the old knowledge using circular external fixation. We have transitioned from the sole use of external fixation to integrated techniques and to the frequent use of internal fixation. While we have a variety of powerful tools available to tackle complex limb reconstruction cases, different surgeons will use varying approaches and techniques based on both their preferences and also the resources available in their locations. For that reason, none of the cases presented in this book are out of date. Techniques presented include classic circular external fixation, hexapod external fixation, integrated techniques such as lengthening and then nailing (LATN), lengthening over a nail (LON), bone transport over a nail, bone transport and then nailing, and fully internal techniques of internal lengthening nailing, opening and closing wedge osteotomies with plates, deformity correction with intramedullary nailing and blocking screws, plate-assisted bone segment transport, and internal bone transport nail. Also included is amputation reconstruction with osseointegration limb replacement. The second edition of the atlas has a total of 481 cases in 6 sections presented in 3 volumes. Limb Lengthening and Reconstruction Surgery Case Atlas—Trauma • Foot and Ankle—135 cases Limb Lengthening and Reconstruction Surgery Case Atlas—Pediatric Deformity—152 cases Limb Lengthening and Reconstruction Surgery Case Atlas—Adult Deformity • Tumor • Upper Extremity—194 cases We have found case-based learning to be especially enjoyable and educationally effective. As orthopedic deformity surgeons, we are visual, practical, and creative. It is natural for most of us to teach and learn by assembling a case for presentation. The chapter format used in this case atlas mimics my own personal approach to evaluating and treating a patient, which includes a detailed history and physical examination, analysis and measuring radiographs, and synthesis of a problem list. This exercise enables analysis of a very complicated case, first by breaking it down to its composite parts. The patient then becomes less complicated enabling the formation of a treatment strategy based on principles to address all component parts of the problem list. This often demands creativity and some courage but since the plan is based on sound principles, it is less intimidating. With increasing experience, I have been able to utilize technical tricks and these “pearls” are most helpful in surgery. I have learned vii

viii

Preface

to think a couple steps ahead and have a backup plan or two in order to avoid and manage complications. I diligently document cases in a photo database to be used for patient and colleague education as well as research. This has been my approach to limb deformity practice over the last 25 years. The format of this case atlas continues to be uniform and is aligned with my approach to evaluating and treating patients. Each case/chapter contains this flow: 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11.

Abstract Basic clinical history Preoperative photos and x-rays Problem list Treatment strategy Basic principles Photos and x-rays during treatment Technical pearls Outcome photos and x-rays Avoiding and managing complications References and additional reading

I want to thank our brilliant contributors from around the world. With a large collection of cases, the reader will be exposed to an array of limb lengthening and reconstruction surgery. You will also notice similar cases presented by different authors who use differing approaches. Our field has less uniformity and more creativity than our sibling subspecialties. Furthermore, I want to thank my friends and co-editors, Reggie Hamdy, Austin Fragomen, and Mitchell Bernstein and section editors, Taylor Reif and Jason Hoellwarth without whom this project would not have been possible. I am pleased that this publication is endorsed by the Limb Lengthening and Reconstruction Society (LLRS) of North America, an organization that we have all been active in. Additional gratitude goes to the fantastic Springer team, including Kristopher Spring who helped initiate the project and our wonderful, energetic, and organized editorial team led by Swetha Varadharajan. I also want to thank my mentors Dror Paley, John Herzenberg, Munjed AlMuderis, Russ Warren, Tom Sculco, and David Helfet from whom I have learned so much. I am grateful for having had the opportunity to teach and train many fellows and residents who have taught and energized me over the last 25 years. Finally, a special thanks to my wife, Yonina and my children, Jason and Nicole and Libby and Brett whose love and encouragement make me smile and give me strength every day. New York, USA December 2024

S. Robert Rozbruch, MD

Booknotes

This book is part of a set consisting of three volumes: Volume 1: “Pediatric Deformity” Section: Pediatric Deformity – Reggie C. Hamdy and Jason S. Hoellwarth Contents: Pediatric Lower Extremity Trauma Growth Plate Injuries Congenital Pseudarthrosis of the Tibia and Fibula Congenital Lower Limb Deficiencies Pediatric Blount Disease Pediatric Arthrogryposis Pediatric Skeletal Dysplasias Pediatric Metabolic and Vascular Disorders Pediatric Hip Deformities Pediatric Foot and Ankle Deformities Pediatric Chronic Osteomyelitis Pediatric Rotational Deformities Pediatric Rotational Deformities (Miserable Malalignment) Volume 2: “Trauma • Foot and Ankle” Section: Trauma – Mitchell Bernstein Contents: Femoral Reconstruction Tibial Reconstruction Intentional Deformation Tibia Bone Defect—Integrated Fixation Acute trauma—External Acute trauma—External Fixation Bone Defect—External Fixation Bone Defect—Internal Fixation Malunion—External Fixation Malunion—Internal Fixation Nonunion—External Fixation Nonunion—Internal Fixation Osteomyelitis

ix

x

Section: Foot and Ankle – Austin T. Fragomen Contents: Ankle Arthrodesis Charcot Neuroarthropathy Complex Foot Deformity Contracture Distraction Arthroplasty Metatarsal Reconstruction Supramalleolar Osteotomy (SMO) Volume 3: “Adult Deformity • Tumor • Upper Extremity” Section: Adult Deformity – S. Robert Rozbruch Contents: Internal Lengthening Nail Femoral Deformity Tibial Deformity Multiple Segment Deformity Integrated Fixation Hip Knee Knee Fusion Bone Defect Amputation Reconstruction Section: Tumor – Taylor J. Reif Contents: Benign Bone Tumor Malignant Bone Tumor Failed Initial Treatment Sequela of Resection Section: Upper Extremity – John E. Herzenberg Contents: Upper Limb: Humerus Upper Limb: Humerus Upper Limb: Elbow and Forearm Upper Limb: Hand and Wrist Upper Limb: Trauma Upper Limb: Amputation

Booknotes

Contents

Part I

Pediatric Deformity: Pediatric Lower Extremity Trauma . . . . . . . . . . . . .

1

1

Pediatric Trauma: An Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

3

2

Adolescent Unstable Tibial Fracture Treated Initially with Closed Reduction and Plaster Cast Immobilization Converted to TL-Hex External Fixation due to Residual Displacement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Haim Shtarker and Mikhail Samchukov

3

4

5

6

7

Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with PRECICE Retrograde Intramedullary Nail . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . John Birch, Alexander Cherkashin, and Mikhail Samchukov Correction of Partial Epiphyseal Growth Arrest and 7 cm Shortening in 10 Years Old Girl . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mark Eidelman Distal Femoral Fracture Treated Initially with Internal Fixation Converted to Circular External Fixation due to Nonunion and Hardware Failure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Lane Wimberly, Alexander Cherkashin, and Mikhail Samchukov

7

15

23

29

Oblique Plane Deformities of Femur and Tibia After Open Fracture Treated with TSF . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Marie Gdalevitch

35

Open Femoral Fracture Treated with Internal Fixation Converted to Circular External Fixation due to Infection . . . . . . . . . . . . . . . . . . . . . . . . . . Lane Wimberly, Alexander Cherkashin, and Mikhail Samchukov

41

8

Post-traumatic Deformity and Bone Loss in 8 Year Old Boy . . . . . . . . . . . . . Mark Eidelman

9

Proximal Tibial Growth Arrest with Varus, Recurvatum, and Shortening After ACL Reconstruction. Correction with TSF . . . . . . . . . . . . . S. Robert Rozbruch

53

Sixteen Year Old Male with Tibial Malunion Complicated by Pathological Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Karl Rathjen, Alexander Cherkashin, and Mikhail Samchukov

59

10

11

Ten Year Old Male with Comminuted Distal Femoral Fracture . . . . . . . . . . . Karl Rathjen, Alexander Cherkashin, and Mikhail Samchukov

47

67

xi

xii

12

13

Contents

Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique Wire Bone Transport . . . . . . Karl Rathjen, Alexander Cherkashin, and Mikhail Samchukov Traumatic Physeal Arrest of the Proximal Tibia with Deformity in All 4 Dimensions (Length, Coronal Angulation, Sagittal Angulation, and Rotation) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Christopher Iobst

14

TSF for Displaced Pediatric Tibia Fractures . . . . . . . . . . . . . . . . . . . . . . . . . Christopher Iobst

15

Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral Lengthening with FITBONE Retrograde Intramedullary Nail . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . John Birch, Alexander Cherkashin, and Mikhail Samchukov

16

17

18

19

Valgus/Flexion and Shortening of the Distal Femur from Growth Arrest Treated with a Monolateral Frame . . . . . . . . . . . . . . . . . . . . . . . . . . . S. Robert Rozbruch Vitamin D-Resistant Hypophosphatemic Rickets Treated by Double-Level Femoral Osteotomy with Internal Fixation and Proximal Tibial Osteotomy with Gradual Deformity Correction . . . . . . . . . . . . . . . . . . John Birch, Alexander Cherkashin, Marina Makarov, and Mikhail Samchukov Adolescent with Segmental Bone Defect Secondary to Grade IIIB Open Tibial Fracture Treated by Oblique Wire Bone Transport . . . . . . Lori Karol, Alexander Cherkashin, and Mikhail Samchukov Comminuted Type I Open Distal Femur Fracture . . . . . . . . . . . . . . . . . . . . . Christopher Iobst

Part II

75

85 89

95

103

111

123 129

Pediatric Deformity: Growth Plate Injuries . . . . . . . . . . . . . . . . . . . . . .

133

20

Growth Plate Injuries: An Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

135

21

Correction of Post-Traumatic Medial Growth Arrest of the Distal Tibia (Common Problem, but Often Neglected) . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mark Eidelman, Nadav Rinott, and Pavel Kotlarsky

Part III Pediatric Deformity: Congenital Pseudarthrosis of the Tibia and Fibula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

139

145

22

Congenital Pseudarthrosis of Tibia and Fibula: An Introduction . . . . . . . . . . Reggie C. Hamdy

147

23

Congenital Pseudarthrosis of the Fibula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Charles E. Johnston

149

24

Congenital Pseudarthrosis of the Fibula: Direct Surgical Repair . . . . . . . . . . John Birch

155

25

Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 3) . . . . . . . . . . . . . Mahmoud A. El-Rosasy

161

26

Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 2) . . . . . . . . . . . . . Mahmoud A. El-Rosasy

167

Contents

xiii

27

28

29

30

31

Congenital Pseudoarthrosis of the Tibia Repaired with Massive Autograft and Tibia-Fibula Cross-Union . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Taylor J. Reif and S. Robert Rozbruch

173

Congenital Tibial Pseudarthrosis Managed with Ipsilateral Vascularized Fibula Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Michael Aiona

179

Congenital Tibial Pseudoarthrosis Treated with Internal and External Fixation Using the 4 in 1 Technique . . . . . . . . . . . . . . . . . . . . . . . . . Pablo Wagner and John E. Herzenberg

185

Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application . . . . . . . . . . . . . . . . . . Reggie C. Hamdy and François Fassier Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

191

197

32

Recalcitrant Congenital Pseudarthrosis of the Tibia . . . . . . . . . . . . . . . . . . . . Robert A. Hill

205

33

Congenital Pseudarthrosis of Tibia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dror Paley and Craig A. Robbins

211

Part IV

Pediatric Deformity: Congenital Lower Limb Deficiencies . . . . . . . . . .

219

34

Congenital Lower Limb Deficiencies: An Introduction . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

221

35

Absent Knee Extensor Mechanism, Fixed Flexion Deformity, Leg Length Inequality and Foot Deformity in an 11 Year Old Girl Treated with Knee Disarticulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Elizabeth Ashby and Reggie C. Hamdy

36

37

229

Avoiding Amputation and Prosthetics in Children with Complex Lower Limb Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . David S. Feldman and Adam M. Kurland

233

Congenital Femoral Deficiency: Paley Type 1b and Paley Type 1c . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dror Paley and Craig A. Robbins

241

38

Congenital Femoral Deficiency: Paley Type 2a . . . . . . . . . . . . . . . . . . . . . . . . Dror Paley and Craig A. Robbins

251

39

Congenital Femoral Deficiency: Paley Type 2c . . . . . . . . . . . . . . . . . . . . . . . . Dror Paley and Craig A. Robbins

261

40

Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral Lengthening Associated with Flexible Intramedullary Nailing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dmitry A. Popkov and Pierre Lascombes

267

xiv

41

42

43

44

Contents

Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Robert A. Hill

275

Congenital Short Femur and Fibular Hemimelia Staged Approach Frame and Nail . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Jason S. Hoellwarth and S. Robert Rozbruch

279

Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Robert A. Hill

285

Correction of Recurvatum Deformity with Osteotomy, External Fixator, and Epiphysiodesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . David S. Feldman and Adam M. Kurland

291

45

Distal Femoral Deformity Correction Using MAC . . . . . . . . . . . . . . . . . . . . . Patrick J. O’Toole and Richard S. Davidson

46

Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral Deficiency and Femoral Retroversion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pablo Wagner, Renee Hunter, and John E. Herzenberg

47

Femoral Lengthening with MAC External Fixation System . . . . . . . . . . . . . . Nariman Abol Oyoun and Richard S. Davidson

48

Femoral Shortening (14 cm) and Deformity Treated with Acute Correction and Two Consecutive Retrograde Fitbone Applications . . . . . . . . Metin Kucukkaya

49

Femur Lengthening with an Extramedullary Nail . . . . . . . . . . . . . . . . . . . . . Claire E. Shannon

50

Fibular Hemimelia and Congenital Short Femur-Staged Approach in Child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Emilie-Ann Downey and S. Robert Rozbruch

297

301 307

311 317

327

51

Fibular Hemimelia: Paley Type 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dror Paley and Craig A. Robbins

52

Guided Growth and Syme Amputation in a Thirteen-Year-Old Boy with Type 2 Congenital Fibular Deficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . Elizabeth Ashby and Reggie C. Hamdy

347

Intraoperative SSEP Monitoring of Circular External Fixation for Revision of Brown Rotationplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Marina Makarov, Charles E. Johnston, and Mikhail Samchukov

351

Knee Subluxation During Femoral Lengthening in a Six Year Old Boy with Congenital Coxa Vara and Congenital Short Femur . . . . . . . . . . . . Reggie C. Hamdy

359

Proximal Tibial Recurvatum and Ipsilateral Short Femur Treated with a MAC External Fixation System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Nariman Abol Oyoun and Richard S. Davidson

367

53

54

55

335

Contents

xv

56

57

58

59

60

61

Proximal Tibial Recurvatum Corrected Using the Ilizarov Technique in a 14 Year Old Boy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Elizabeth Ashby, Reggie C. Hamdy, and François Fassier

373

Sequential Deformity Correction and Tibial Elongation Using Dormant Magnetic Nail Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Alan Katz and Ehud Lebel

379

Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot Equinus and LLD . . . . . Alexander Cherkashin, John Birch, and Mikhail Samchukov

385

Simultaneous Knee and Hip Dislocation During Femoral Lengthening in a Patient with Congenital Short Femur . . . . . . . . . . . . . . . . . Hae-Ryong Song and Kwang-Won Park

397

Staged Lengthening of 20 cm in the Femur and Tibia to Equalize Leg Lengths in a Growing Child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . S. Robert Rozbruch

407

Tibia Valga Treated with Tibial and Fibular Osteotomy and Application of a Multi-axial Correcting External Fixation System . . . . . . . . . R. Jay Lee and Richard S. Davidson

413

62

Tibial Hemimelia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Muayad Kadhim and Richard S. Davidson

63

Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Dror Paley and Craig A. Robbins

429

Tibial Hemimelia Type II in a 6 Year Old Child Treated with Fibular Transfer and Centralization and Syme’s Amputation . . . . . . . . . . . . Elisabeth Leblanc and Reggie C. Hamdy

445

Tibial Hemimelia: Staged Treatment with External Fixation and Then Internal Lengthening Nail . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mark Eidelman and Pavel Kotlarsky

451

Tibial Lengthening Over a Free Vascularized Fibular Autograft After Ewing’s Sarcoma Resection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Barak Rinat, Marc Isler, and Panagiotis (Peter) Glavas

461

64

65

66

67

68

69

70

Treatment of Recurrent Patellar Dislocation Using Langenskiold Reconstruction of the Patella, Grammont Patellar Slide, and Percutaneous Distal Femur and Distal Tibia/Fibula Osteotomies . . . . . . . . . . . . . . . . . . . . . Raymond W. Liu

419

469

Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD) . . . . . . . . . . . . . . . . . . . . . . . . J. Ivan Krajbich

479

Delayed Regenerate Bone Formation in a Seven Year Old Boy with Fibular Hemimelia Undergoing Tibial Lengthening . . . . . . . . . . . . . . . . Reggie C. Hamdy

485

Septic Destruction of the Hip and Significant LLD Treated by Pelvic Support Osteotomy and Femoral Lengthening . . . . . . . . . . . . . . . . . . . Mikhail Samchukov, John Birch, and Alexander Cherkashin

491

xvi

Contents

Part V

Pediatric Deformity: Pediatric Blount Disease . . . . . . . . . . . . . . . . . . . .

501

71

Pediatric Blount Disease: An Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

503

72

Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Peter Calder

73

Adolescent Blount’s Disease Treated with MAC External Fixation System . . . . Anish G. R. Potty, Michael M. Kheir, and Richard S. Davidson

74

Correction of Adolescent Tibia Vara Without Fibular Osteotomy and Without Fixation of the Fibula Using the Taylor Spatial Frame (TSF) . . . . Mark Eidelman

75

Correction of Juvenile Blount’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mark Eidelman

76

Correction of Multi-planar Deformity in 120 kg 13 Year Old Patient with Blount’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Haim Shtarker and Mikhail Samchukov

505 511

519 525

529

77

Guided Growth Treatment for Early-Onset Blount Disease . . . . . . . . . . . . . . Sanjeev Sabharwal

539

78

Hemiplateau Elevation for Early-Onset Blount Disease . . . . . . . . . . . . . . . . . Folorunsho Edobor-Osula and Sanjeev Sabharwal

547

79

Infantile Blount Disease with Plateau Depression . . . . . . . . . . . . . . . . . . . . . . Robert A. Hill

557

80

Morbidly Obese Teenager with Significant Blount’s Treated with Taylor Spatial Frame . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Craig A. Robbins

563

One-Stage Hemi-Plateau Elevating Osteotomy in Advanced Blount’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Ixchel Montoya and Panagiotis (Peter) Glavas

569

Proximal Tibial Osteotomy in a 4 Year Old Child with Blount Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

575

Simultaneous Correction of Medial Proximal Tibial Plateau Depression and Tibia Vara in an Obese Child with Blount’s . . . . . . . . . . . . . Craig A. Robbins

583

81

82

83

84

13 Year Old with Unilateral Late-Onset Blount Disease . . . . . . . . . . . . . . . . . Folorunsho Edobor-Osula and Sanjeev Sabharwal

Part VI 85

589

Pediatric Deformity: Pediatric Arthrogryposis . . . . . . . . . . . . . . . . . . .

599

Pediatric Arthrogryposis: An Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

601

Contents

xvii

86

87

88

89

90

91

92

93

94

Bilateral Congenital Vertical Talus and Dislocated Hips in a Child with Very Severe Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Noémi Dahan-Oliel and Reggie C. Hamdy

605

Correction of Arthrogrypotic Clubfoot Deformities with the Ponseti Method of Serial Casting . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Harold J. P. van Bosse

611

Correction of Severe Contractures, Pterygium and Lower Limb Deformities Caused By Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . David S. Feldman and Adam M. Kurland

617

Journey of a Child Born with Severe Arthrogryposis and Lower Limb Deformities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Harold J. P. van Bosse

623

Recurrent Knee Flexion Contractures in a 10 Year Old with Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy and Noémi Dahan-Oliel

631

Simultaneous Correction of Hip and Knee Flexion Contractures in a 5 Year Old with Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy and Noémi Dahan-Oliel

637

Synchronization of Surgical Interventions for Multiple Deformities in a Four Year Old with Arthrogryposis . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy and Noémi Dahan-Oliel

643

Talectomy for Correction of Severe Rigid Clubfoot in a Patient with Arthrogryposis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Saleh Alfaisali and Reggie C. Hamdy

647

Correction of Severe Arthrogrypotic Knee Flexion Contractures . . . . . . . . . . Harold J. P. van Bosse

Part VII

653

Pediatric Deformity: Pediatric Skeletal Dysplasias . . . . . . . . . . . . . . .

661

95

Pediatric Skeletal Dysplasias: An Introduction . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

663

96

Bilateral Genu Valgum due to Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Lori Karol, Mikhail Samchukov, and Alexander Cherkashin

667

Correction of Lower Limb Deformities in Multiple Hereditary Exostosis (MHE) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mark Eidelman

675

Correction of Tibia Recurvatum and Shortening in Skeletal Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Samantha A. Spencer

679

Genu Valgum and Limb Length Discrepancy in Multiple Enchondromatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hae-Ryong Song and Kwang-Won Park

685

97

98

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Contents

Guided Growth Treatment for Genu Valgum Secondary to Juxtaphyseal Recurrent Aneurysmal Bone Cyst of the Distal Femur . . . . . . . . Sanjeev Sabharwal

693

Management of Distal Femur Deformity Following Ablation of Aneurysmal Bone Cyst, by Corrective Osteotomy and Elongation . . . . . . . . Alan Katz, Amos Peyser, and Ehud Lebel

701

Migration of the Fibula During Tibial Lengthening in Achondroplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Hae-Ryong Song and Kwang-Won Park

707

Multiple Lower Limb Deformities in a 14 Year Old Girl with Multiple Epiphyseal Dysplasia and Low Lumbar Spina Bifida . . . . . . . . . . . Elizabeth Ashby and Reggie C. Hamdy

721

Refracture, Soft Tissue Contracture, and Angular Deformity After Femoral Lengthening in Achondroplasia . . . . . . . . . . . . . . . . . . . . . . . Hae-Ryong Song and Kwang-Won Park

725

Retrograde Insertion of a SLIM Nail in a Femur in a Patient with Osteogenesis Imperfecta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Khaled Abu Dalu, Yousef Marwan, and Mitchell Bernstein

733

Revision of Bilateral Tibial Fassier-Duval Rods in an Eleven Year Old Girl with Osteogenesis Imperfecta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Elizabeth Ashby, Reggie C. Hamdy, and François Fassier

739

Revision of Femur Fassier-Duval Rod for Intertrochanteric Fracture Nonunion with Lytic Cyst and Coxa Vara in Osteogenesis Imperfecta Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Khaled Abu Dalu, Mitchell Bernstein, and Reggie C. Hamdy

743

Revision of Telescoping Rod and Plate Complicated with Nonunion and Metallosis in a Girl with Osteogenesis Imperfecta Type VIII . . . . . . . . . Dmitry Popkov and Pierre Lascombes

749

Severe Genu Valgum in Skeletal Dysplasia: Acute Versus Gradual Correction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy and Neil Saran

753

Spondyloepiphyseal Dysplasia Treated by Bi-lateral Proximal Tibial Osteotomy Followed by Gradual Deformity Correction . . . . . . . . . . . John Birch, Alexander Cherkashin, and Mikhail Samchukov

763

Staged Femur and Tibial Lengthening During Childhood for Russell Silver Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Amber A. Hamilton, Danya M. Jacobs, and S. Robert Rozbruch

773

Telescopic Rodding in Combined Technique After Transphyseal Elastic Nailing in a Boy with Type IV Osteogenesis Imperfecta . . . . . . . . . . Dmitry Popkov, Pierre Lascombes, and Sergey Khmyzov

777

The Use of Gap Nail for Tibia Fracture in Skeletally Mature Osteogenesis Imperfecta Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yousef Marwan, Khaled Abu Dalu, and Mitchell Bernstein

785

The Use of SLIM (Simple Locking Intra Medullary) Nail in the Lower Extremities of Skeletally Immature Patients with Osteogenesis Imperfecta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Yousef Marwan and Reggie C. Hamdy

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Type IV Tibial Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Robert A. Hill

116

Varus Deformity of the Distal Femur and LLD Secondary to Ollier’s Disease Corrected by Gradual Deformity Correction and Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . B. Stephens Richards, Alexander Cherkashin, and Mikhail Samchukov

117

Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with Telescoping Fassier-Duval Rods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy and François Fassier

Part VIII Pediatric Deformity: Pediatric Metabolic and Vascular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118

Pediatric Metabolic and Vascular Disorders: An Introduction . . . . . . . . . . . Reggie C. Hamdy

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Deformity Correction in Child with X-Linked Hereditary Hypophosphatemic Rickets by Combined Technique (External Fixation and Flexible Intramedullary Nailing) . . . . . . . . . . . . . . . Dmitry A. Popkov and Pierre Lascombes

120

Genu Varum Secondary to Rickets Treated with Femoral and Tibial Osteotomy and Application of a Multi-axial Correcting External Fixation System . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . R. Jay Lee and Richard S. Davidson

121

Infantile Myofibromatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Gamal Ahmed Hosny

122

Hypophosphatemic Rickets with Bilateral Severe Genu Varum. Retrograde Fixator Assisted Nailing for Femurs and Double Level Tibial Osteotomies with TSF . . . . . . . . . . . . . . . . . . . . . . . . . . Marie Gdalevitch

Part IX

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815

823 825

827

833 837

841

Pediatric Deformity: Pediatric Hip Deformities . . . . . . . . . . . . . . . . . . .

849

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Pediatric Hip Deformities: An Introduction . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

851

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Healed Slipped Capital Femoral Deformity . . . . . . . . . . . . . . . . . . . . . . . . . Neil Saran

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Hinged Arthrodiastasis for Avascular Necrosis of the Hip . . . . . . . . . . . . . . David S. Feldman and Adam M. Kurland

857

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Hip Fusion with an External Fixator . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Neil Saran

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Ilizarov Hip Reconstruction for Post Infective Femoral Head Destruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Robert A. Hill

869

Percutaneous Osteotomy of the Proximal Femur for Slipped Capital Femoral Epiphysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Heather Kong and Sanjeev Sabharwal

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Perthes Hip Treated with Articulated Hip Distraction and Small Diameter Core Decompression . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Marie Gdalevitch

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Perthes: Femoral Head Reduction Osteotomy . . . . . . . . . . . . . . . . . . . . . . . Dror Paley and Craig A. Robbins

131

Coxa Vara in a Nine-Year-Old Boy with Osteogenesis Imperfecta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . François Fassier, Elizabeth Ashby, and Reggie C. Hamdy

Part X

885 891

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Pediatric Deformity: Pediatric Foot and Ankle Deformities . . . . . . . . .

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Pediatric Foot and Ankle Deformities: An Introduction . . . . . . . . . . . . . . . . Reggie C. Hamdy

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Complex Foot Deformity in a Child with Aperts Syndrome, Treated with a Miter Frame . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . David B. Frumberg

907

Correction of Severe Foot Deformity in Arthrogryposis with Midfoot Osteotomy and Ilizarov Frame . . . . . . . . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy and Noémi Dahan-Oliel

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Fourteen Year Old Female with Residual Clubfoot Deformity Treated with Taylor Spatial Frame . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Craig A. Robbins

919

Recalcitrant Clubfeet in a 4 Year Old with Arthrogryposis Treated with External Fixation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Craig A. Robbins

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Severe Equino-Varus Foot and Ankle Deformity from Compartment Syndrome. TSF to Correct Deformities followed by Tendon Transfer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Marie Gdalevitch Severe Equinus Secondary to Linear Scleroderma Treated by Gradual Deformity Correction via Circular External Fixation Without Osteotomies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Alexander Cherkashin, John Birch, and Mikhail Samchukov

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Step by Step Approach to Cavus Foot Deformity . . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

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Treatment of Complex Clubfoot Using Midfoot Osteotomy and Taylor Spatial Butt Frame (TSF) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mark Eidelman

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Foot Stump Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Gamal Ahmed Hosny

Part XI

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939 947

953 959

Pediatric Deformity: Pediatric Chronic Osteomyelitis . . . . . . . . . . . . . .

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Pediatric Chronic Osteomyelitis: An Introduction . . . . . . . . . . . . . . . . . . . . Reggie C. Hamdy

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Cable Bone Transport for Segmental Bone Loss Secondary to Grade IIIB Open Tibial Fracture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mikhail Samchukov, John Birch, Alexander Cherkashin, and Antony I. Riccio

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Contents

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Chronic Osteomyelitis and 5 cm Bone Defect Treated with Masquelet Technique Followed by Ilizarov . . . . . . . . . . . . . . . . . . . . . . . . . . Marie Gdalevitch

975

Correction of Post-infectious Partial Proximal Tibia Growth Arrest with Taylor Spatial Frame in a Child . . . . . . . . . . . . . . . . . . . . . . . . Craig A. Robbins

983

Post-infection Loss of the Lateral Half of the Upper Tibial Epiphysis with Deformity and LLD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mahmoud A. El-Rosasy

989

Radical Excision and Lengthening of Chronic Sclerosing Osteomyelitis of Garre (10 Years of Follow-Up) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Mark Eidelman and Nadav Rinott

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Trifocal Distraction Osteogenesis in the Management of Sequelae of Chronic Osteomyelitis of the Tibia (Pseudarthrosis, Deformity and LLD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1001 Mahmoud A. El-Rosasy

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Deformity and Lengthening of Tibia Following Meningococcal Septicemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1007 Peter Calder

Part XII 150

Pediatric Deformity: Pediatric Rotational Deformities . . . . . . . . . . . . . 1013

Derotational Osteotomies of the Femur and Tibia for Tetratorsional Malalignment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1015 Bridget K. Ellsworth, Erik J. Geiger, and S. Robert Rozbruch

Part XIII Pediatric Deformity: Pediatric Rotational Deformities (Miserable Malalignment) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1023

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Pediatric Rotational Deformities (Miserable Malalignement Syndrome): An Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1025 Reggie C. Hamdy

152

Adolescent with Bilateral Femoral and Tibial Rotational Deformity (Miserable Malalignement Syndrome) Combined with Proximal Tibial Varus and Recurvatum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1027 Haim Shtarker and Mikhail Samchukov

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1037

About the Editors

Dr. S. Robert Rozbruch is Chief of the Limb Lengthening and Complex Reconstruction Service and Director of the Osseointegration Limb Replacement Center at Hospital for Special Surgery. He is Professor of Clinical Orthopedic Surgery at Weill Cornell Medical College, and is a member of several national medical societies including fellowship in the American Academy of Orthopaedic Surgeons, Orthopaedic Trauma Association, and ASAMI—The Limb Lengthening & Reconstruction Society of which he was President (2012–2013). He has presented his clinical and research works at numerous national and international medical meetings and has authored over 200 articles in medical journals and chapters in orthopedic textbooks. He edited two authoritative textbooks on limb lengthening and reconstruction. Dr. Rozbruch was educated at the University of Pennsylvania graduating Magna Cum Laude in 1985, and he attended Weill Cornell Medical College of Cornell University, from which he graduated with honors in research in 1990. Residency training at Hospital for Special Surgery in orthopedic surgery (1991–1995) was followed by two fellowships. He did specialized training in Trauma as an AO fellow at the University of Bern in Switzerland. Additional training in adult and pediatric limb lengthening followed at the Maryland Center for Limb Lengthening & Reconstruction. Reggie C. Hamdy is a pediatric orthopedic surgeon, graduated from the University of Alexandria and completed his residency at the University of Ottawa, and his fellowship training at the Hospital for Sick Children, Toronto, and Brown University in Providence, Rhode Island. He joined the Shriners Hospitals for Children—Canada in 1994, and was appointed Chief of Staff and Head of Division of Paediatric Orthopaedics, McGill University in 2010 (till 2020). He was President of LLRS Morth America 2015. He is a tenured Professor of Surgery. He was appointed Associate Chair, Department of Paediatric Surgery (Continuous Professional Development), McGill University in 2024 and is currently the Medical Director of the Motion Analysis Laboratory, Director of the Limb Lengthening and Deformity Unit, Director of the Fellowship Training Program in Paediatric Orthopaedics, and Surgical Director of The Osteogenesis Imperfecta Program. His main clinical interests include the management of children with complex

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About the Editors

conditions such as Osteogenesis Imperfecta (OI), skeletal dysplasias, neuromuscular conditions specifically arthrogryposis, and children with limb deformities. He is the Chairman of the Limb Reconstruction Committee of the SICOT 2024/2025. He has authored or co-authored over 160 peer review publications and 8 chapters, and he is the Editor of a book on Pelvic and Femoral Osteotomies in Children (Springer) and the Co-Editor of an Atlas on Limb Lengthening and Reconstruction (First Edition and the present Second Edition). Dr. Austin T. Fragomen is Professor of Clinical Orthopedic Surgery at Weill Cornell Medical School and HSS. He has excelled as an educator. He is the director of the LLCRS fellowship program which includes recruitment and year-long training of exceptional young surgeons interested in pursuing a career in the subspecialty of Limb Deformity. In addition, surgeons from various regions in the United States and from around the globe come to New York to rotate with Dr. Fragomen for month-long visiting-professor observer-ships to broaden how they approach complex orthopedic challenges. Feedback from these observers includes amazement with the volume of Limb Deformity patients treated in Dr. Fragomen’s clinic and his ability to spend time with each person to ensure that the patient remains the focus of the practice. With over 150 publications, research has been central to ensuring the highest quality patient care including surgical decision making and has given Dr. Fragomen a voice nationally and worldwide. Authoring textbook chapters requires not only a profound knowledge of the field of Limb Deformity but also demands meticulous scouring of publications, new and old, relevant to the topic, providing further information and perspective to the author. Dr. Fragomen maintains a prospective database of all the surgeries he has performed for use in later analysis to find trends in patient care. This data then drives future decision making to optimize surgery and post-operative protocols ensuring the best possible outcomes and patient satisfaction. Dr. Fragomen is invited to speak regularly at orthopedic programs around the United States, Canada, and abroad to share his research and personal experiences for the advancement of successful outcomes in these other centers. As the president of the national Limb Lengthening & Reconstruction Society (LLRS) from 2019 to 2021, Dr. Fragomen launched several initiatives that have inspired current members, drawn in new members, and gained traction at the American Academy of Orthopedic Surgeons (AAOS) where he also serves on the Board of Subspecialty Societies. Dr. Fragomen takes pride in resolving the previously “unsolvable” problems from which his patients suffer, and he welcomes collaboration with other HSS surgeons who have complimentary skill sets in an effort to guarantee that all of his patients have been treated by the best of the best.

About the Editors

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Mitchell Bernstein is Associate Professor of Surgery at McGill University in Montreal, Canada. He specializes in adult and pediatric trauma, limb lengthening, and deformity correction surgery. Dr. Bernstein serves as the Director of the Orthopaedic Residency Program, Head of the Orthopaedic Trauma Unit at the Montreal Children’s Hospital, and Co-Director of the Limb Deformity Unit at Shriners Hospital for Children—Canada.

Section Editors

Jason Hoellwarth Director of Research Limb Lengthening and Complex Reconstruction Service Hospital for Special Surgery New York, NY, USA Weill Cornell Medical College Cornell University New York, NY, USA Taylor J. Reif Director of Education Limb Lengthening and Complex Reconstruction Service Hospital for Special Surgery New York, NY, USA Weill Cornell Medical College Cornell University New York, NY, USA

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Contributors

Nariman Abol Oyoun Orthopaedic Surgery, Assiut University, Assiut, Egypt Khaled Abu Dalu Shriners Hospitals for Children, Montreal, QC, Canada McGill University Health Centre, McGill University, Montreal, QC, Canada Michael Aiona Shriners Hospital for Children, Portland, OR, USA Saleh Alfaisali Shriners Hospitals for Children, Montreal, QC, Canada Elizabeth Ashby Division of Orthopaedics, Shriners Hospital, Montreal, QC, Canada Mitchell Bernstein Departments of Surgery & Pediatric Surgery (Division of Orthopaedics), McGill University Health Center & Shriners Hospital for Children - Canada, Maywood, IL, USA John Birch Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA Peter Calder Royal National Orthopaedic Hospital (RNOH), Brockley Hill, Stanmore, London, UK Alexander Cherkashin Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA Noémi Dahan-Oliel Clinical Outcomes, Shriners Hospital for Children, Montreal, QC, Canada Richard S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Emilie-Ann Downey Department of Orthopaedic Surgery, Pierre-Boucher Hospital, QC, Canada Folorunsho Edobor-Osula Pediatric Orthopaedic Surgery, Rutgers – New Jersey Medical School, Newark, NJ, USA Mark Eidelman Technion Faculty of Medicine, Rambam Health Care Campus, Meyer’s Children Hospital, Haifa, Israel Ruth Rappoport Children’s Hospital, Rambam Healthcare Campus, Haifa, Israel Bridget K. Ellsworth Hospital for Special Surgery, New York, NY, USA Mahmoud A. El-Rosasy Faculty of Medicine, Department of Orthopaedic Surgery, University of Tanta, Tanta, Al-Ghrabeya, Egypt François Fassier Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada David S. Feldman Pediatric Orthopedic Surgery, NYU Langone Medical Center, New York, NY, USA xxix

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David B. Frumberg Department of Orthopaedics & Rehabilitation, Yale School of Medicine, New Haven, CT, USA Marie Gdalevitch Verdun Hospital, Montreal, QC, Canada Erik J. Geiger University of Miami, Miami, FL, USA Panagiotis (Peter) Glavas Sainte-Justine University Hospital Center, QC, Canada Reggie C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada Amber A. Hamilton Hospital for Special Surgery, New York, NY, USA John E. Herzenberg Rubin Institute for Advanced Orthopedics, International Center for Limb Lengthening and Reconstruction, Sinai Hospital of Baltimore, Baltimore, MD, USA Robert A. Hill Portland Hospital for Women and Children, London, UK Jason S. Hoellwarth Limb Lengthening and Complex Reconstruction Service, Hospital for Special Surgery, New York, NY, USA Gamal Ahmed Hosny Benha Faculty of Medicine, Cairo, Egypt Renee Hunter Rubin Institute for Advanced Orthopedics, International Center for Limb Lengthening and Reconstruction, Sinai Hospital of Baltimore, Baltimore, MD, USA Christopher Iobst Department of Orthopedic Surgery, Nemours Children’s Hospital, Orlando, FL, USA Marc Isler Sainte-Justine University Hospital Center, QC, Canada Danya M. Jacobs Hospital for Special Surgery, New York, NY, USA Charles E. Johnston Texas Scottish Rite Hospital for Children, Dallas, TX, USA Muayad Kadhim Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA Lori Karol Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA Alan Katz Pediatric Orthopedic Surgeon, Department of Orthopedic Surgery, Shaare Zedek Medical Center, Faculty of Medicine, Hebrew University, Jerusalem, Israel Michael M. Kheir Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA Sergey Khmyzov Sitenko Institute of Spine and Joint Pathology, Kharkiv, Ukraine Heather Kong Rutgers – New Jersey Medical School, Newark, NJ, USA Pavel Kotlarsky Ruth Rappoport Children’s Hospital, Rambam Healthcare Campus, Haifa, Israel J. Ivan Krajbich Shriners Hospitals for Children, Portland, OR, USA Metin Kucukkaya Florence Nightingale Hospital, Istanbul, Turkey Adam M. Kurland Pediatric Orthopedic Surgery, NYU Langone Medical Center, Hospital for Joint Diseases, New York, NY, USA Pierre Lascombes Division of Pediatric Orthopaedics, University of Geneva, Geneva, Switzerland University of Nancy, Nancy, France

Contributors

Contributors

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Ehud Lebel Head of Pediatric Orthopedics Unit, Department of Orthopedic Surgery, Shaare Zedek Medical Center, Faculty of Medicine, Hebrew University, Jerusalem, Israel Elisabeth Leblanc Division of Orthopaedics, University of Sherbrooke, Sherbrooke, QC, Canada R. Jay Lee Johns Hopkins Bloomberg Children’s Center, Baltimore, MD, USA Raymond W. Liu Case Western Reserve School of Medicine, Rainbow Babies and Children’s Hospital, Cleveland, OH, USA Marina Makarov Texas Scottish Rite Hospital for Children, Dallas, TX, USA Yousef Marwan Shriners Hospitals for Children, Montreal, QC, Canada McGill University Health Centre, McGill University, Montreal, QC, Canada Department of Surgery, College of Medicine, Health Sciences Centre, Kuwait University, Kuwait City, Kuwait Ixchel Montoya Sainte-Justine University Hospital Center, QC, Canada Patrick J. O’Toole Children’s Hospital of Philadelphia, Philadelphia, PA, USA Dror Paley Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA Kwang-Won Park Department of Orthopedic Surgery, Korea University Medical Center, Guro Hospital, Seoul, South Korea Amos Peyser Shaare Zedek Medical Center, Jerusalem, Israel Dmitry A. Popkov National Ilizarov Medical Research Center for Traumatology and Orthopaedics, Kurgan, Russia Anish G. R. Potty Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA Karl Rathjen Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA Taylor J. Reif Director of Education, Limb Lengthening and Complex Reconstruction Service, Hospital for Special Surgery, New York, NY, USA Weill Cornell Medical College, Cornell University, New York, NY, USA Antony I. Riccio Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA B. Stephens Richards Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA Barak Rinat Sainte-Justine University Hospital Center, QC, Canada Nadav Rinott Ruth Children’s Hospital, Rambam Health Care Campus, Haifa, Israel Craig A. Robbins Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA S. Robert Rozbruch Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA Sanjeev Sabharwal Department of Orthopaedics, Rutgers – New Jersey Medical School, Newark, NJ, USA

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Mikhail Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA Neil Saran Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada Claire E. Shannon Paley Orthopedic and Spine Institute, West Palm Beach, FL, USA Haim Shtarker Western Galilee Hospital, Nahariya, Israel Hae-Ryong Song Department of Orthopedic Surgery, Korea University Medical Center, Guro Hospital, Seoul, South Korea Samantha A. Spencer Orthopaedic Surgery, Harvard Medical School, Boston, MA, USA Harold J. P. van Bosse Shriners Hospital for Children, Philadelphia, PA, USA Pablo Wagner Rubin Institute for Advanced Orthopedics, International Center for Limb Lengthening and Reconstruction, Sinai Hospital of Baltimore, Baltimore, MD, USA Lane Wimberly Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA

Contributors

Principles of Deformity Correction Reggie C. Hamdy1 and S. Robert Rozbruch2 1

Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA

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Patients with lower limb deformities constitute a large proportion of referrals to both pediatric and adult orthopedic clinics. A practical approach to assessing and treating lower limb deformities should include four distinct steps. First, know what is normal: determine if there a deformity or if it is part of the normal development of the child. Second, define the characteristics of this deformity (type, site, plane, and magnitude). Third, decide if the deformity needs to be treated (depending on the clinical picture). Fourth, generate a treatment plan. In this atlas, more than 300 cases of lower and upper limb deformities of various etiologies are presented. A detailed analysis of these deformities is a prerequisite for appropriate planning of surgical correction. This chapter presents a practical approach to the assessment of lower limb deformities. When a skeletally immature patient presents with a lower limb deformity, the first priority is to rule out physiological “deformity.” Almost all newborn babies have a symmetrical angular genu varum deformity of the legs that changes to a genu valgum deformity around 18–24 months of age. This partially corrects, gradually, until the age of 7–8 years old, when the adult anatomic valgum value of 7 is reached (Fig. 1). Similarly, the vast majority of rotational deformities in children (in-toeing and out-toeing) is physiological and usually corrects with age. However, in some patients, these deformities are not physiological but secondary to pathological conditions (congenital or acquired) and should be fully investigated. History taking. The presenting symptoms should be clearly identified: what is the reason for the consultation? Is this a cosmetic or functional problem? What are the functional limitations this deformity inflicts on the patient? Is pain present? What are the characteristics of this pain (when did it start, is it acute or chronic, any associated symptoms, any systemic manifestations, what are the relieving and precipitating factors, localization, radiation of the pain, character of the pain)? Are daily activities affected? In certain conditions, such as skeletal dysplasias and metabolic disorders, other nonskeletal systems may be affected, and it is important to recognize these. The patient may have more than one deformity. It is imperative to identify which deformity (or deformities) is the cause of the symptoms. It is also imperative to determine the cause of the deformity as this may have an impact on the surgical planning. In skeletally immature patients, the deformity may be progressive. This is especially true for partial growth arrest deformities. In addition, the possibility of recurrence of the deformity after a successful correction has to be explained to the patient and family. Physical examination is very important for the assessment of not only bony deformities but also other musculoskeletal pathologies such as joint contractures and soft tissue problems, including the condition of the skin and soft tissues surrounding the deformity. These factors will have an impact in the decision-making process regarding acute versus gradual correction and bony stabilization. A complete neurovascular assessment should be performed. More than one type of deformity may be present, including angular (in frontal, sagittal, or oblique planes), rotational, translational, axial (limb length discrepancy), or multiplanar deformity. The deformities could be unilateral or bilateral and may be associated with other musculoskeletal problems, as well as non- musculoskeletal problems. Physical examination is of specific

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Fig. 1 Normal age limits for physiological genu varum and genu valgum

Fig. 2 (a–d) (a) Normal rotational alignment of the lower limbs. (b) Bilateral Internal rotation deformity of the lower limbs (in-toeing), partly due to exaggerated femoral anteversion, as the patella are

“kissing” each other. (c) Right-side external rotation deformity due to external tibial torsion (patella is pointing forwards). (d) Sitting position showing external rotation deformity of the right tibia

importance in the identification and assessment of rotational deformities and limb length discrepancies. For rotational deformities, clinical examination is usually sufficient to quantify, plan, and correct the rotational deformity (Figs. 2 and 3). For a more precise measurement of rotational deformities, a CT scan can be performed (Fig. 4). Plain X-rays may suggest a rotational malalignment, but cannot quantify the magnitude of the deformity. For limb length discrepancies, a wooden block placed under the shorter leg to level the pelvis usually gives a good clinical measure of the magnitude of shortening (Fig. 5). When there are angular deformities in the limbs, it is important to differentiate true from apparent LLD (Fig. 6). Each deformity should be identified and characterized separately in order to decide which one (or ones) needs to be addressed and which need not be corrected.

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Fig. 3 Rotational alignment in the prone position. (a) Hip external rotation. (b) Hip in neutral position. (c) Hip internal rotation

Fig. 4 CT scan measurement of rotational profile, using transverse cuts through. (a) Proximal femur (femoral anteversion) – normal 12 . (b) Distal femur (femoral condyle alignment). (c) Proximal tibia. (d) Distal tibia

Radiological assessment of bony deformities should address six key questions: – What is the plane of the deformity (frontal, sagittal, or oblique)? – What is the direction of the deformity (varus/valgus, anterior/posterior)?

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Fig. 5 Measurement of LLD with wooden blocks. (a) Without block, the pelvis is oblique. (b) With wooden block elevation equal to the LLD, the pelvis is level. This method takes into consideration the heel height, but cannot be used in the presence of knee or hip contractures

Fig. 6 Causes of apparent limb length discrepancy

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– – – –

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Which bone and/or joint is affected (femur, tibia, knee, or ankle joint)? Which segment of the bone is affected (epiphysis, metaphysis, or diaphysis)? Where is the apex of the deformity? What is the magnitude of the deformity?

The next step is to decide whether or not the deformity needs to be corrected. In other words, what are the indications for surgery? The decision to surgically address a deformity should take into consideration symptomatology, radiological assessment, the functional limitations of that deformity, the potential psychosocial impact of surgery, and the ability of surgery to improve these problems. In general, if function is affected, then surgery is indicated. The goal of correcting any deformity is to improve function and restore normal mechanical alignment of the lower limbs and thus prevent degenerative changes in the articular cartilage. Having decided to proceed with surgical correction of the deformity, the next question is how to correct the deformity: is it by acute or gradual correction? If it is decided to proceed with an acute correction, then what type of osteotomy should be performed: closing, opening, neutral wedge, dome, or oblique osteotomy. One must consider how to stabilize the osteotomy: by internal or external fixation. To increase the accuracy and ease of acute correction, fixator-assisted plating or nailing techniques could be enlisted. The second option is gradual correction of the deformities by either growth modulation (in skeletally immature patients) or with external fixators or internal devices. The third option is combined acute and gradual correction, where one or more deformities are acutely corrected and then gradual correction is used for the remaining deformities, usually limb length discrepancy.

Radiological Assessment of Lower Limb Deformities The first step is to obtain appropriate X-rays of the entire lower limbs standing with patellae pointing forward (usually the patella can be seen on the X-rays), as well as AP and lateral views of affected bones. The following parameters are analyzed: (I) Mechanical and anatomical alignment of the whole lower limb as well as that of individual bones (II) Joint orientation lines (III) Joint orientation angles (IV) Apex of the deformity as determined by the CORA (center of rotation of angulation)

Mechanical Alignment of the Lower Limbs (Weight-Bearing) Frontal Plane Alignment Each long bone has two axes: mechanical and anatomical (Fig. 7). The mechanical axis of a long bone is represented by a line joining the centers of the joints proximal and distal to that bone. The anatomical axis passes through the center of the diaphysis and is represented by a line joining the center of the transverse diameter of the diaphysis through several points along the bone: • Mechanical Axis of the whole lower limb (Fig. 7a). – Is a straight line from the center of the femoral head to the center of the ankle or middle of tibial plafond.

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Fig. 7 (a) Mechanical axis of the whole lower limb. (b) Mechanical and anatomical axes of the femur and tibia

– This is always a straight line. – Falls just medial to the center of the knee (up until 8 mms). – Measures varus/valgus angulation. • Mechanical and anatomical axes of individual bones (Fig. 7b): (a) Femur mechanical axis From center of the femoral head to center of the knee (different from the anatomical axis) (b) Femur anatomical axis Is a mid-diaphyseal line: straight in the frontal plane Curved in the sagittal plane (c) Tibia mechanical axis Line from the center of the knee to the center of the ankle (d) Tibia anatomical axis Mid-diaphyseal line In the tibia, anatomical and mechanical axes are parallel and for practical purposes are the same • Mechanical axis deviation (MAD) or malalignment (Fig. 8). – Represents loss of colinearity of the hip, knee, and ankle in the frontal plane. – Is the distance between the mechanical axis of the whole lower limb and the center of the knee. Could also be expressed in percentage.

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Fig. 8 (a) Valgus mechanical axis deviation (valgus malalignment). (b) Varus mechanical axis deviation (varus malalignment)

– As mechanical axis falls medial to the center of the knee, MAD up to 8 mm is considered normal. – Any MAD greater than 8 mm (some authors consider 15 mm) medial to the knee center is considered varus malalignment. – Any MAD lateral to the center of the knee is considered valgus malalignment. – In general, malalignment in the frontal plane is much more serious than in the sagittal plane. The consequences of frontal plane malalignment on the knee (varus/valgus) are well described and documented and may lead to degenerative osteoarthritis, while the consequences of malalignment in the sagittal plane are less well defined. The knee joint is a uniaxial joint allowing no motion in the frontal plane, and any degree of malalignment in the frontal may be detrimental. That is not the case in the sagittal plane, where motion in the sagittal plane occurs all the time during walking, sitting, and other activities. – MAD can occur secondary to bony deformities in the femur or tibia and joint deformities in the knee or ankle (due to ligamentous laxity or joint incongruity such as medial plateau depression in Blount’s disease).

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Fig. 9 Sagittal plane alignment. (a) Entire lower limb (falling through anterior part of the knee). (b) Anatomical axis of the femur (not a straight line) and tibia (straight line). (c) Mechanical axis of femur and tibia

Sagittal Plane Alignment Whole lower limb falls just in front of the center of the knee joint line (Fig. 9) (a) Femur mechanical axis From center of the femoral head to center of knee (b) Femur anatomical axis Is a mid-diaphyseal line: curved in the sagittal plane (c) Tibia mechanical axis Line from the center of the knee to the center of the ankle (d) Tibia anatomical axis Line from the anterior one fifth of the proximal tibia to the center of the ankle joint

Joint Orientation Lines Assessment This represents the relation of the joint axis to the anatomic and mechanical axis of a bone. Joint orientation lines are drawn on both frontal and sagittal planes.

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Fig. 10 Joint Orientation lines in coronal (frontal plane). (a) Normal lines and alignement. (b) Varus malalignement due to ligamentous laxity. (c) Varus malalignement due to joint incongruity

Frontal Plane (a) Hip joint orientation (Fig. 10a): 1. Trochanteric – femoral head line: line from the tip of greater trochanter to center of the femoral head 2. Femoral neck line: line drawn from the hip joint center to several points that bisect the diameter of the femoral neck (b) Knee joint orientation: Represents the relation of the knee joint line to the distal femur and proximal tibia – Normally is in valgus 3 . – This is the most important measurement. – Represented by lines joining the two femoral condyles and a line joining the two tibial plateaus. These 2 lines should be parallel (more than 2 considered abnormal and a source of MAD). – Joint line congruence angle (JLCA) is the angle between these two lines. If not parallel, this is caused either by joint laxity (such as in children with achondroplasia) (Fig. 10b) or by articular surface incongruity (such in Blount’s disease with depressed medial tibial plateau) (Fig. 10c).

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Fig. 11 Joint orientation lines in sagittal plane. (a) posterior slope of the tibial plateau (normal 7–9 ) and anterior slope of the tibial plafond (normal 7–10 ). (b) Tibia recurvatum with anterior slope of 7 . (c) Tibia procurvatum with exaggerated posterior slope of 14

(c) Ankle joint orientation: – Line drawn along the tibial plafond – Parallel to the floor or slight valgus (up to 8 )

Sagittal Plane (a) Knee joint orientation (Fig. 11): – A line drawn along the tibial plateau – Normally 7–9 posterior slope (b) Ankle joint orientation line: – A line drawn through the most distal points of the anterior and posterior distal tibia – Normally, about 7–10 anterior slope

Joint Orientation Angles These are angles formed between the joint lines and either the mechanical or anatomic axis of separate bones.

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Fig. 12 Joint orientation angles in frontal plane. (a) Normal values. (b) mLDFA of 77 causing distal femoral valgus. (c) mMPTA of 80 causing proximal tibia varus

Frontal Plane (Fig. 12) • LDFA (Lateral Distal Femoral Angle). This measures the angle between the distal articular femoral surface and either the mechanical or anatomical axis of the femur. The distal femoral articular surface is in slight valgus of one or two degrees when mechanical LDFA is measured and in about 7 of valgus when the anatomical LDFA is measured (Fig. 12a). • MPTA (Medial Proximal Tibial Angle). This measures the angle between the proximal tibial articular surface and the mechanical or anatomical axis of the tibia. The proximal tibial articular surface is in about 1 – 3 varus with both mechanical and anatomical axis. • LDTA (Lateral Distal Tibial Angle). This measures the angle between the distal tibial articular surface and the anatomical or mechanical axis of the tibia (both are the same). It measures about 90 . Abnormal joint orientation angles are demonstrated in Fig. 12, showing distal femoral valgus (Fig. 12b) and proximal tibia vara (Fig. 12c).

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Fig. 13 Joint orientation angles in sagittal plane: normal values

Sagittal Plane (Fig. 13) • PPFA (Posterior Proximal Femoral Angle). This is measured between the mid-diaphyseal line of the neck of the femur and the line across the physis or physeal scar of the femoral head. Normally 90 . • ANSA (Anterior Neck Shaft Angle). It is measured between the sagittal plane middiaphyseal line of the proximal femur and the mid-diaphyseal line of the neck of the femur. About 170 . • PDFA (Posterior Distal Femoral Angle). This is measured between the sagittal distal femoral joint line and the mid-diaphyseal line of the distal femur. Usually about 83 (Fig. 13). • PPTA (Posterior Proximal Tibial Angle). This represents the angle between the proximal tibial surface and the mechanical axis of the tibia. It is usually about 81 . • ADTA (Anterior Distal Tibial Angle). This represents the angle between the distal articular surface and mechanical axis of the tibia. It is usually about 81 .

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Fig. 14 Uni-apical deformity at the mid-diaphyseal region of tibia. CORA is located at the intersection of the anatomical axis of the proximal and distal segments of the tibia

Fig. 15 Multi-apical deformities of tibia. Double CORA present at the intersection of the anatomical axes of the proximal, middle, and distal segments of the tibia

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Fig. 16 When apex of the deformity and CORA do not correspond, a translational deformity is present

Fig. 17 Presence of multiplanar deformities in frontal plane (a), oblique plane (b), and sagittal plane (c). In the presence of multiplanar deformities, the deformity in the oblique plane is always greater than either the frontal or sagittal plane deformity

Determining the Apex of the Deformity or CORA (Centre of Rotation of Angulation) – Used to define where the apex of the deformity is located within the bone. – Determining the CORA is crucial for surgical planning of correction of any angular deformity and will serve as the site of osteotomy or hinge. – If deformity is diaphyseal, the anatomical axis should be used. The apex of the deformity or CORA is the intersection of the diaphyseal lines of proximal and distal fragments (Fig. 14).

Principles of Deformity Correction

Fig. 18 Types of osteotomies and the impact of axis of correction position. All osteotomies in this figure are placed upon CORA and apex of deformity. (a) Mid-diaphyseal deformity. CORA and apex of deformity are the same. (b) If axis of correction is situated on the concave side of the deformity, a closing wedge osteotomy is produced. (c) If axis of correction is situated in the mid-diaphyseal area (the same

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as CORA and apex of the deformity), a neutral osteotomy is produced. (d) If axis of correction is situated on the convex side of the deformity, an opening wedge osteotomy is produced. (e) If the axis of correction is situated outside the diaphysis, distraction is produced, together with the opening wedge osteotomy. The further the axis of correction is from the diaphysis, the greater the distraction is

– If the deformity is metaphyseal or juxta-articular (epiphyseal), the CORA is determined by the intersection of a line perpendicular to the joint orientation line and the mid-diaphyseal line of the deformed segment or bone (Figs. 20 and 21). – More than one CORA may exist in the same bone (multi-apical deformity) (Fig. 15). – If CORA and apex of the deformity do not coincide, then there is an additional translational deformity (Fig. 16). – If the CORA is present on both the frontal and sagittal planes, then an oblique deformity exists. The magnitude of an oblique plane deformity is always greater than what it measures in either the frontal or sagittal planes (Fig. 17). – The impact of the anatomical location of the axis of correction on the type of osteotomy – closed, open, and neutral – is shown in Fig. 18. – The impact of placement of the osteotomy relative to CORA and axis of correction on the resulting translation is shown in Fig. 19. Malalignment test. This includes performing all the previously mentioned measurements: mechanical alignment of the whole lower limb and of separate bones, calculating MAD, drawing joint orientation lines, measuring joint orientation angles, and determining the site and amount of the CORA or CORAs. It should be emphasized, however, that all of these measurements are static and not dynamic.

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Fig. 19 The impact of placement of osteotomy relative to CORA and axis of correction. (a) Mid-diaphyseal deformity. CORA, and apex of deformity are the same. (b) Osteotomy performed upon CORA and axis of correction. (c) Osteotomy performed distal to CORA and axis of

Principles of Deformity Correction

correction. Mechanical axis is corrected because of the translation. (d) Osteotomy and axis of correction are in a different location than CORA. Mechanical axis not corrected, because there is no translation

By using this systematic approach, the core 6 questions (previously mentioned) could then be answered and the deformity fully appreciated: – – – – – –

What is the plane of the deformity (frontal, sagittal, or oblique)? What is the direction of the deformity (varus/valgus, anterior/posterior)? Which bone and/or joint is affected (femur, tibia, knee, or ankle joint)? Which segment of the bone is affected (epiphysis, metaphysis, or diaphysis)? Where is the apex of the deformity? What is the magnitude of the deformity?

Examples of step-by-step approach in the assessment of various lower limb deformities are shown in Figs. 20, 21, 22, and 23: – – – –

Figure 20: Distal femoral valgus deformity Figure 21: Proximal medial tibial deformity Figure 22: Uni-apical mid-diaphyseal deformity of tibia Figure 23: Double apical diaphyseal deformity of tibia

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Fig. 20 Example of distal femoral valgus deformity – step-by-step approach. (a) Diagram showing the deformity. (b) Mechanical axis of entire limb showing varus malalignment. (c) Mechanical axis of femur and tibia. (d) Joint orientation line of knee. (e) Joint orientation angles: mLDFA 77 , MPTA 88 . (f) Red dotted lines showing two different levels of osteotomy. (g) If osteotomy performed upon CORA and axis of

correction (situated on lateral cortex on the same line as the CORA), then an opening wedge is produced, and there is no translation. (h) If osteotomy performed proximal to CORA and axis of correction is on the lateral cortex on same line as osteotomy, then translation is required to maintain mechanical alignment

Fig. 21 Example of proximal medial tibial deformity – step-by-step approach. (a) Diagram showing the deformity. (b) Mechanical axis of entire limb showing varus malalignment. (c) Mechanical axis of femur and anatomical axis of the tibia. (d) Joint orientation lines of the knee and ankle. (e) Joint orientation angles: mLDFA 90 , MPTA 65 , MDTA

90 . (f) Red dotted line showing line of osteotomy at CORA. (g) Correction of the deformity causing an opening wedge osteotomy as apex of correction is on the convex side of the deformity (shown by the red dot)

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Fig. 22 Example of uni-apical mid-diaphyseal deformity of the tibia – step-by-step approach. (a) Diagram showing the deformity. (b) Anatomical axis of distal tibial segment. (c) Anatomical axis of proximal tibial segment and CORA. (d) Joint orientation lines of the knee and

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ankle. (e) Joint orientation angles LPTA 89 and MDTA 89 showing that the deformity is diaphyseal. (f) Osteotomy performed upon CORA. Axis of correction lies on the convex side same line as CORA. (g) Opening wedge osteotomy with restoration of anatomical axis of tibia

Acute Versus Gradual Correction Several factors should be taken into consideration before deciding on whether an acute or gradual correction should be performed. These include: – Age of the patient. Uniplanar deformities in very young children can be safely and successfully corrected by gradual correction (growth modulation) with 8 plates. – Amount to be corrected. It is generally accepted that deformities less than 15 – 20 can be adequately and safely addressed with an acute correction, while deformities greater than this are better addressed by gradual correction. – Site of the deformity. Excessive stretching of the surrounding neurovascular structures is always a concern when performing acute correction, specially if these structures are anatomically located on the concave site of the deformity, as in the case of valgus deformities of the distal femur and proximal tibia. In these cases, the common peroneal nerve is vulnerable and at risk of neuropraxia; therefore, prophylactic decompression of this nerve should be performed prior to the acute correction. The same applies around the ankle, where a tarsal tunnel release may be indicated before proceeding with an acute correction. On the

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Fig. 23 Example of double apical diaphyseal deformity of the tibia – step-by-step approach. (a) Diagram showing deformity. (b) Anatomical axis of proximal segment. (c) Anatomical axis of middle segment. (d) Anatomical axis of distal segment with double CORA. (e) Joint orientation lines. (f) Joint orientation angles. (g) Lines of osteotomies upon

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both CORA. (h) Deformity correction at distal CORA causing an opening wedge osteotomy as axis of correction is on the convex side of deformity. (i) Deformity correction at proximal CORA also causing an opening wedge osteotomy as axis of correction is on the convex side of the deformity

other hand, acute correction of diaphyseal deformities of the femur is less likely to cause neurovascular problems. – Status of the surrounding soft tissues. Previous surgeries or scarring due to burns or trauma often lead to soft tissue fibrosis and disturbed anatomy. In these cases, extensive dissection should be avoided as this may further damage the soft tissues. Furthermore, the dissection may be technically difficult as all the anatomy is abnormal making it challenging to recognize the vital structures.

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– Quality of the bone to be corrected. In some cases of severe osteopenia (such as osteogenesis imperfecta), the bones are so weak and soft that they may be unable to withstand the stresses of external fixators, and in such cases an acute correction and intramedullary stabilization may be indicated. – Associated limb length discrepancy. If there is an associated limb length discrepancy, then a gradual correction that could address all the deformities simultaneously is indicated. – Amount of bone to be resected. If an acute correction is contemplated, then shortening of the bone at the site of the deformity – in order to accommodate the soft tissue contractures – will probably be necessary, and this should be planned preoperatively and clearly explained to the patient and family. This is especially true for rigid foot deformities that are corrected with osteotomies and acute correction. Preoperative planning should address all the above mentioned issues, and each case should be individualized.

Gradual Correction In the skeletally immature patient, this could be carried out either by growth modulation with 8 plates or external fixators. In skeletally mature patients, osteotomies and gradual distraction with various types of unilateral, circular, or hybrid fixators are the only techniques available.

Advantages of Gradual Correction with 8 Plates “The best osteotomy is NO osteotomy” (John Herzenberg). Growth modulation has radically changed our approach to the correction of long bone deformities in the skeletally immature patient. Whenever possible, it is always better to use growth modulation. It is a minimally invasive surgery compared to osteotomy. It can be performed at a very young age (as young as the age of 2 years). When correction is obtained, the plate can be removed, or one of the screws, generally the metaphyseal one, can be removed leaving the plate and epiphyseal screw in place so that if the deformity recurs it can be easily reapplied percutaneously. Most importantly, it is not a permanent correction. This may avoid more extensive surgery in the form of osteotomies whether for gradual or acute correction. However, as seen in several cases of the atlas, growth modulation is not always successful and may not yield satisfactory results in cases of severe and/or multiple deformities (adolescent Blount’s, skeletal dysplasias, rickets). Furthermore, the correction with 8 plates takes a more prolonged time than with the use of external fixators. In addition, growth modulation has its maximum effect in the first decade and may be less reliable when it is applied near skeletal maturity. Advantages of Gradual Correction with External Fixators These are numerous and include: 1. Minimal invasive surgery and minimal soft tissue dissection. This is mostly a percutaneous technique. 2. Can generate new bone (in cases of gradual open wedge osteotomy). 3. Extremely versatile. 4. Allows adjustments during correction of the deformities. The inability to obtain a standing hip to ankle radiograph during surgery limits the precision of an intraoperative correction. With external fixation stabilization, the position can be changed acutely or gradually after a standing hip to ankle radiograph is obtained and the appropriate mechanical axis analysis is performed. 5. Allows certain amount of axial loading, which is beneficial for bone healing. 6. Can be used in the presence of acute and chronic infection.

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7. It is easier to obtain an accurate reduction with gradual correction, specifically if multiplanar deformities are present. 8. In severe deformities, a bone resection can be avoided. 9. Less risk of neurovascular complications and compartment syndrome that may occur with acute corrections. 10. Simultaneous limb lengthening is possible 11. The use of external fixators allows immediate full weightbearing and allows functional use of the limb. The disadvantages of gradual correction include all the problems associated with prolonged time needed to wear the external fixator while the newly formed bone consolidates, necessitating multiple clinic visits and missing school days, potential for psychosocial problems, prolonged rehabilitation, and all other medical problems including pin site infection, pain, and swelling. Therefore, we believe gradual correction using external fixators is indicated if there is an associated limb length discrepancy requiring lengthening by distraction osteogenesis, extensive soft tissue scarring from previous surgeries, trauma or burns, multiple complex deformities and severe bony deformities.

Acute Correction Acute correction offers several advantages. Usually all the deformities are corrected in one surgery only, a quicker return to daily activities is usually possible (although the patient may be non-weight-bearing or toe touching) and the absence of any of the problems associated with external fixation. The use of fixator-assisted acute correction could increase the accuracy of acute correction. Disadvantages of acute correction include a lack of post-op adjustability: No further adjustment of the correction could be made. Furthermore, any associated LLD could not be addressed at the same time.

Combined Gradual and Acute Correction In some cases of multiple deformities, especially when both femur and tibia are affected, a viable alternative is acute correction of the deformities in one bone (usually the femur) and gradual correction of the other, as discussed in several cases in the atlas. The femoral deformities are addressed by acute correction (whether standard technique or fixator assisted), and the tibial deformities are addressed by gradual correction with external fixators. This avoids having two external devices (on femur and tibia) and allows simultaneous correction of all deformities in the affected limb, with the least “cumbersome” fixation.

References and Suggested Reading Brinker MR, O’Connor DP (2012) Principles of malunions. In: Rockwood CA, Bucholz RW, James C-B, Heckman JD, Paul T (eds) Rockwood and Green’s fractures in adults, 7th edn, vol 1. Lippincott Williams & Wilkins, Philadelphia, pp 641–664 Hamdy RC, McCarthy JJ (2011) Management of limb-length discrepancies. Monograph series, vol 45. American Academy of Orthopaedic Surgeons, Rosemont Paley D (2002) Principles of deformity correction. Springer, Heidelberg/Berlin Rozbruch SR, Ilizarov S (2007) Limb lengthening surgery. Informa Health Care, NewYork/London

Part I Pediatric Deformity: Pediatric Lower Extremity Trauma

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Pediatric Trauma: An Introduction Reggie C. Hamdy

Abstract

Most fractures in skeletally immature patients can be successfully treated conservatively by closed reduction and cast application. In some cases, however, open reduction and stabilization of the fracture with various techniques may be necessary, including K-wires, elastic nails, standard plates, or locking pediatric plates. The use of standard external fixators (that do not allow gradual correction of deformities) may be indicated in the management of open fractures, fractures with bone loss, comminuted fractures, certain metaphyseal and physeal injuries, infected fractures, polytrauma patients, and those with head injuries. In such cases, the use of standard external fixation is usually temporary and is typically converted to other methods of stabilization once the condition permits. On the other hand, in some specific conditions, the use of external fixation that allows deformity correction is indicated. The advantages of these fixators over other modalities of treatment, include functional use of the limb and weight bearing during the whole period of treatment, access to the fracture site for treatment of soft tissues, correction of any type and any amount of deformity (acutely or gradually), and the possibility of lengthening in cases of bone shortening (Table 1). Most fractures in skeletally immature patients can be successfully treated conservatively by closed reduction and cast application. In some cases, however, open reduction and stabilization of the fracture with various techniques may be necessary, including K-wires, elastic nails, standard plates, or locking pediatric plates. The use of standard external fixators (that do not allow gradual correction of deformities) may be indicated in the management of open fractures, fractures with bone loss, comminuted fractures, certain metaphyseal and R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

physeal injuries, infected fractures, polytrauma patients, and those with head injuries. In such cases, the use of standard external fixation is usually temporary and is typically converted to other methods of stabilization once the condition permits. On the other hand, in some specific conditions, the use of external fixation that allows deformity correction is indicated. Some of these conditions are discussed in this section, emphasizing the advantages of these fixators over other modalities of treatment, including functional use of the limb and weight bearing during the whole period of treatment, access to the fracture site for treatment of soft tissues, correction of any type and any amount of deformity (acutely or gradually), and the possibility of lengthening in cases of bone shortening (Table 1). As shown in Table 1, indications for the use of external fixators in cases of trauma in skeletally immature patients include: – Displaced fractures with extensive soft tissue damage and swelling (case 1). In such cases, immediate manipulation would further compromise soft tissues. Hence, it would be more appropriate to apply an external fixator in order to stabilize the bone segments temporarily and allow for healing of the soft tissues. Once the swelling has subsided, gradual or acute reduction of the fracture could be obtained. – Open fractures with minimal bone loss (case 2). In such cases, acute shortening and compression would allow the bone ends to be approximated for healing to occur. The resulting LLD could then be addressed later by osteotomy (proximal or distal to the fracture site) and lengthening after healing of the bony and soft tissues has occurred. – Comminuted fractures (case 3) open or closed – specifically metaphyseal or physeal injuries, in which reduction and stabilization of the fracture would be technically difficult by standard conservative or operative treatment. The use of external fixation is indicated in such cases to bridge the fracture without further compromising the fracture site and later allowing gradual correction of any deformity if required.

© Springer International Publishing Switzerland (outside the USA) 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_369

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4

R. C. Hamdy

Table 1 Details of the 11 Pediatric Trauma cases discussed in this Atlas Case Diagnosis 1 14 year old female, displaced tibial fracture 2 10 year old male, grade III open comminuted fracture distal femur 3 13 year old male, comminuted Type I open distal femur fracture 4 8 year old boy, open fracture Type IIIB 5

6

7 8 9 10

11

15 year old male, unstable displaced diaphyseal tibial fracture 7 year old female, comminuted open Type IIIA fracture femur 13 year old male, grade IIIB open tibial fracture 3 year old female, grade IIIB tibial fracture 13 year old male, grade IIIB open tibial fracture 14 year old male, comminuted closed Salter IV fracture of the distal femur 16 year old male, tibial diaphyseal malunion

Problems Significant soft tissue swelling, displaced fracture Open fracture, bone loss, potential for infection

Surgery & key points TSF application, fracture left slightly unreduced to allow swelling to subside. Then reduction after few days Acute shortening and compression with TrueLok fixator to allow healing of soft tissues. Deal with LLD later

Comminuted fracture close to physis. Plates and nails difficult to apply

Avoid exposing the fracture site with TSF application. Correction of alignment could be obtained later

Large soft tissue defect anteriorly, bone loss 40 mm

Acute shortening and posterior angulation with TSF application, to allow soft tissue healing, then gradual deformity correction and lengthening Failed closed reduction with cast application. Gradual correction and realignment with TrueLok fixator Displaced fracture. Open growth plate Infection after initially reduced with locking plate. Loss of fixation

TrueLok application. Bridge construct. Reduce fracture. Allow debridement and treatment of infection

Segmental bone loss 4 cms, large soft tissue defect Segmental bone loss 4.0 cms, large soft tissue defect Segmental bone loss 10 cms, large soft tissue defect Hypertrophic nonunion 12 months after initial treatment with open reduction and locking plate Valgus recurvatum tibial deformity, translational component, LLD 2.5 cms, nonunited distal pathological fracture

Vastus lateralis flap. Cable bone transport 3 months after initial trauma with Truelok fixator Soleus flap. Oblique wire bone transport with TrueLok after healing of soft tissues Rectus abdominis flap. Oblique wire transport with TrueLok fixator Acute deformity correction and compression with TrueLok fixator

– Open fractures with extensive soft tissue damage (case 4). In some of these cases, acute shortening and temporary posterior angulation will allow re-approximation of the bone ends and at the same allow the soft tissues to heal (with or without various soft tissue coverage techniques). Gradual realignment of the fracture is performed once all tissues have healed satisfactorily. – Failure of closed reduction, or cases with loss of reduction (case 5), where the presence of open growth plates precludes the application of other operative techniques. Open reduction would necessitate extensive soft tissue dissection, while application of external fixation (specifically circular fixators) with percutaneous technique and minimal invasive surgery would avoid compromise to the soft tissues around the fracture site and at the same time would allow gradual or acute correction of any malalignment and deformity, including limb length discrepancy. – Infected fractures (case 6), whether acutely in cases of open fractures or following internal fixation. Application of external fixators in such cases would allow management of the infection by allowing easy access to the infected site and repeated debridements if necessary and

Two stage correction with TrueLok fixator. First stage, distal osteotomy through pathological nonunion. Second stage, osteotomy through malunion to correct remaining deformities

at the same time allow correction of any deformity (at the same time or after resolution of the infection). – Traumatic bone loss leading to critical sizedefects. In such cases, the technique of bone transport would be indicated in the management of these defects (other options include autografts, allografts, bone graft substitutes, vascularized fibular bone graft and most recently the Masqueletinduced membrane technique). Bone transport is considered a bifocal distraction/compression technique, and in cases with large defects, a double bone transport technique could be used (using two intercalary bone transported fragments). Three cases (Cases 7, 8, 9) are presented in this section, demonstrating two popular techniques of bone transport: the cable technique developed by Weber and the oblique wire technique originally described by Ilizarov. Oblique versus horizontal wires are more convenient for transport of the intercalary segment. It is strongly recommended that, once the transport is terminated, the docking site is debrided and bone grafted, in order to avoid the problem with healing of the docking site – which remains one of the main problems with this technique.

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Pediatric Trauma: An Introduction

– Nonunions (which are very rare in children) and malunions of fractures (case 11) are other indications for the use of external fixators. In this section, a case of hypertrophic nonunion of the distal femur following open reduction and stabilization with a locking plate

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and successfully treated with realignment and compression of the nonunion site with a circular fixator is discussed (case 10). The topic of nonunions is extensively discussed in the other sections of this case atlas.

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Adolescent Unstable Tibial Fracture Treated Initially with Closed Reduction and Plaster Cast Immobilization Converted to TL-Hex External Fixation due to Residual Displacement Haim Shtarker and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13

Abstract

Fifteen-year-old male with an unstable displaced tibial middle/distal shaft fracture treated initially by closed reduction and plaster cast fixation that was converted to TL-Hex circular external fixation due to residual lateral translation and recurvatum.

by closed reduction and plaster cast immobilization. However, the fracture remained unstable and displaced with a half-ofdiameter lateral translation and 10 of recurvatum (Fig. 2).

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Brief Clinical History The patient is a 15 year old male who was hit by a car while crossing a street and sustained a closed tibial shaft fracture at the level of middle/distal thirds (Fig. 1). He was treated initially H. Shtarker (*) Western Galilee Hospital, Nahariya, Israel e-mail: [email protected]; [email protected] M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]

Preoperative Problem List • Unstable displaced tibial shaft fracture with half-of-diameter lateral translation of the distal bone fragment and 10 of recurvatum deformity. • Unsuccessful closed reduction and plaster cast immobilization resulted in remaining distal fragment displacement and additional tibial recurvatum. • Adolescent patient with open growth plates preventing application of conventional treatment for adult patients.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_68

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H. Shtarker and M. Samchukov

Treatment Strategy Because of unsuccessful attempt of nonsurgical treatment and still open proximal tibial growth plate preventing the use of conventional fracture fixation methods in adults, the treatment strategy for reduction and stabilization of his unstable displaced midshaft tibial fracture was based on circular external fixation. Hexapod-type TL-Hex external fixation system was utilized for fracture reduction and stabilization. This method provides anatomical reduction of the fracture while restoring the limb alignment and preserving its length. In addition, stable fixation of bone fragments with supportive compression permits full weight bearing of the injured limb and physical therapy of the knee and ankle joints during consolidation.

Basic Principles

Fig. 1 Preoperative AP and LAT radiographs after injury demonstrating a displaced tibial middle/distal shaft fracture. Note half-of-diameter lateral translation and mild anterior translation of the distal fragment

Fig. 2 AP and LAT radiographs of the tibia after initial closed reduction and application of the plaster cast for immobilization revealing remaining half-of-diameter lateral translation of the distal bone fragment and 10 of recurvatum in addition to its initial anterior translation

Several treatment options currently exist for displaced or comminuted tibial shaft fractures ranging from closed reduction and plaster cast immobilization to open reduction followed by internal fixation [6]. Unfortunately, the majority of those methods have limitations when used for stabilization of unstable fractures in adolescent patients with open growth plates. Primary closed reduction with plaster cast fixation often fails to maintain bone fragments’ position, resulting in axial deviation and rotational malalignment in addition to prolonged joint immobilization. Open reduction and internal fixation (ORIF) with plates and screws is an invasive procedure usually reserved for treatment of noncomminuted fractures [9]. Although less invasive intramedullary nail (IMN) fixation is considered a preferred method of choice for adults, application of the rigid nail in growing adolescents may cause significant physeal injury potentially resulting in growth plate damage and development of genu recurvatum [3, 7]. Flexible nails (e.g., elastic titanium nail) allow intramedullary fixation without growth plate injury [1, 4, 5]. However, they do not provide reliable fixation of unstable midshaft tibial fractures and often require additional immobilization. Monolateral pinto-bar external fixators are also not stable enough for definitive treatment of such fractures. Alternatively, circular external fixation allows for precise fracture reduction and provides very stable fixation of bone fragments without injury to the growth plate [2, 8]. In addition, it preserves the normal length of the tibia and fibula while restoring the alignment of the limb.

Images During Treatment See Figs. 3, 4, 5, 6, 7, 8, and 9.

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Adolescent Unstable Tibial Fracture Treated Initially with Closed Reduction and Plaster Cast. . .

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Fig. 3 Intraoperative photograph of the tibia visualizing displaced midshaft fracture and preoperative planning of frame application. Note marked levels for external supports and required length for connecting rods

resulting in spatial repositioning of one ring (moving ring) relative to the other ring (reference ring). Unique key design features of the TL-Hex rings (light anodized aluminum, tab location on the side of the ring, two-strut attachment with a single set screw, etc.) and struts (metal–plastic interface at the claw-type universal joints, independent acute and gradual length adjustment with a single length adjustment scale, lockable pull-and-turn adjustment knob, etc.) allow to maintain Ilizarov principles of stable bone fragment fixation using a combination of crossing wires and half pins while performing complex multiplanar deformity correction sequentially or simultaneously in a single spiral movement. The frame has tremendous overall stability during and between the strut length adjustments throughout the entire period of treatment, thereby allowing immediate weight bearing and maintenance of joint range of motion. Similar to other hexapod systems, the TL-Hex is integrated with web-based computer software that generates a prescription of strut length adjustment to achieve the desired final position of bone fragments.

Outcome Clinical Photos and Radiographs Fig. 4 Intraoperative photograph demonstrating preassembled circular external fixator. Note proximal and distal TL-Hexrings blocked to TrueLok 5/8 ring and full ring, respectively, and interconnected by six telescoping struts in neutral acute/gradual adjustment length position

See Figs. 10, 11, 12, and 13.

Avoiding and Managing Problems Technical Pearls TL-Hex is a computer-driven circular fixation system consisting of two external supports interconnected by six telescoping struts, which are arranged in a standard hexapod configuration. As with any other hexapod-type external fixators, those struts can be lengthened or shortened

One of the most common complications of circular external fixation is pin tract infection. In our department, we developed a protocol for the prevention and management of pin-related complications in patients with external fixation. Intraoperatively, all skin tenting around the pins should be released using a small-blade scalpel. This is followed by application of 3% syntomycin (chloramphenicol) ointment

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H. Shtarker and M. Samchukov

Fig. 5 Intraoperative photograph during frame application showing positioning of the preassembled fixator relative to the proximal reference wire. This will be followed by stabilization of the frame to the proximal fragment with anterior-medial half pin and second, more distal, medial-face wire and to the distal fragment using three crossing wires

Fig. 6 Intraoperative AP radiographs during fracture reduction. Note position of bone fragments relative to each other before reduction (left), after acute reduction of lateral translation (center), and after gradual reduction of residual lateral translation followed by axial compression (right)

Fig. 7 Intraoperative photograph after closed reduction of the fracture and compressive stabilization demonstrating final frame position on the tibia

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Adolescent Unstable Tibial Fracture Treated Initially with Closed Reduction and Plaster Cast. . .

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Fig. 8 Postoperative AP and LAT radiographs of the tibia after closedreduction and stabilization of displaced tibial shaft fracture. Note restored alignment of bone fragments in both coronal and sagittal planes and frame orientation relative to the anatomical axis of the tibia

Fig. 9 AP and LAT radiographs of the tibia 2.5 months after surgery demonstrating maintained tibial alignment and mineralized fracture callus. The frame was removed 1 week later

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Fig. 10 AP and LAT radiographs of the tibiaimmediately after frame removal demonstrating restored anatomical axis of the tibia and healed midshaft tibial fracture

Fig. 11 Front view photograph of the lower extremities illustrating clinical appearance 1 year after frame removal

H. Shtarker and M. Samchukov

Fig. 12 Lateral view photograph of the left lower extremity 1 year after frame removal. The patient has no complaints and is happy with the appearance of his lower extremities. His function is completely restored and he has returned to competitive sport

Fig. 13 AP and LAT radiographs of the tibia 1 year after frame removal. Note complete remodeling of the fracture callus with corticalization and proper alignment of the limbs

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Adolescent Unstable Tibial Fracture Treated Initially with Closed Reduction and Plaster Cast. . .

around the wire and pin sites and skin stabilization using VAC system sponges that are usually removed 4–5 days later. The patient takes a shower with an antiseptic fluid soap (e.g., septal scrub or polydine cleanser) once daily and sprays 70% alcohol on and around the pin tracks three times per day. In addition, swimming in the pool is recommended if possible. In case of skin redness around pin sites, localized pain, and appearance of nonpurulent discharge, oral antibiotics (e.g., cephamysine 0.5 g  3 per day) are prescribed for 7–10 days. If the superficial pin tract infection is associated with purulent discharge, we perform a small incision under local anesthesia and apply bandages with 1% solution of chlorine in addition to antibiotics. Usually, the local infection resolves in 2–3 days. If the treatment described above is unsuccessful, the infected wire/pin is removed and replaced with a new wire/pin later after the infection is resolved.

Cross-References ▶ TSF for Displaced Pediatric Tibia Fractures

See Also in Vol. 2 C3.3 Pilon Fracture Closed. Ilizarov Fixation with Limited Open Reduction of Joint Surface and Distal Tibia Bridging Distraction of Ankle Joint Gunshot Tibia Fracture

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References and Suggested Reading 1. Bienkowski P, Harvey EJ, Reindl R, Berry GK, Benaroch TE, Ouellet JA. The locked flexible intramedullary humerus nail in pediatric femur and tibia shaft fractures: a feasibility study. J Pediatr Orthop. 2004;24(6):634–7. 2. Gregory RJ, Cubison TC, Pinder IM, Smith SR. External fixation of lower limb fractures in children. J Trauma. 1992;33(5):691–3. 3. Henley MB, Chapman JR, Agel J, Harvey EJ, Whorton AM, Swiontkowski MF. Treatment of type II, IIIA, and IIIB open fractures of the tibial shaft: a prospective comparison of unreamed interlocking intramedullary nails and half-pin external fixators. J Orthop Trauma. 1998;12(1):1–7. 4. Holbrook JL, Swiontkowski MF, Sanders R. Treatment of open fractures of the tibial shaft: ender nailing versus external fixation. A randomized, prospective comparison. J Bone Joint Surg Am. 1989;71 (8):1231–8. 5. O’Brien T, Weisman DS, Ronchetti P, Piller CP, Maloney M. Flexible titanium nailing for the treatment of the unstable pediatric tibial fracture. J Pediatr Orthop. 2004;24(6):601–9. 6. Schmidt AH, Finkemeier CG, Tornetta P. Treatment of closed tibial fractures. Instr Course Lect. 2003;52:607–22. 7. Sledge SL, Johnson KD, Henley MB, Watson JT. Intramedullary nailing with reaming to treat non-union of the tibia. J Bone Joint Surg Am. 1989;71(7):1004–19. 8. Tucker HL, Kendra JC, Kinnebrew TE. Management of unstable open and closed tibial fractures using the Ilizarov method. Clin Orthop Relat Res. 1992;280:125–35. 9. Van der Linden W, Larsson K. Plate fixation versus conservative treatment of tibial shaft fractures. A randomized trial. J Bone Joint Surg Am. 1979;61(6):873–8.

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Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with PRECICE Retrograde Intramedullary Nail John Birch, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21

Abstract


A 12 year old male with 10 distal femoral varus and 7.5 cm of leg length discrepancy of unknown cause underwent distal femoral osteotomy followed by acute angular deformity correction and femoral lengthening using PRECICE retrograde intramedullary nail.

deceleration of the right distal femur, proximal tibia, distal tibia, and left distal femur (Fig. 1). MRI and CT scans confirmed the plain radiographic findings but without identifying etiology. There is a rare disorder of cutis dysplasia congenita characterized by scalp lesions at birth and similarly appearing spontaneous physeal growth disturbance, but this patient had no history for skin abnormality. He had full range of motion of all lower extremity joints and was neurovascularly intact.

Brief Clinical History Preoperative Clinical Photos and Radiographs The patient is a 12 year old male with 10
distal femoral varus and 7.5 cm of leg length discrepancy of unknown cause. There was no history of trauma or infection. The patient was otherwise clinically well. Radiographic evaluation revealed irregular idiopathic asymmetric physeal growth J. Birch (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]

See Fig. 1.

Preoperative Problem List • Limb length inequality (7.5 cm) with right leg shorter than the left (6.8 cm in the femur and 0.7 mm in the tibia) • Mild (10
) varus deformity of the right distal femur

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_88

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• Complete destruction of the right distal femoral physis • Multilevel asymmetric physeal growth deceleration (right tibia and probably left femur), etiology indeterminate • Progressive leg length discrepancy with future growth potentially requiring contralateral epiphysiodesis and/or repeated limb lengthening

Treatment Strategy Since the current major limb shortening was in the right femur and exceeded 7 cm, a right femoral lengthening was recommended as a first stage of treatment. Because complete closure of the distal femoral physis was confirmed by MRI/CT and the patient had an aversion to external fixation, PRECICE retrograde intramedullary nail with a stroke of 65 mm was selected for femoral lengthening. A preoperative planning of distal femoral osteotomy followed by acute varus

J. Birch et al.

deformity correction and subsequent femoral lengthening was made using the Reverse Planning Method (Fig. 2) as described by Baumgart [1]. Because of the widespread, asymmetric growth deceleration of physes in both lower extremities, etiology indeterminate, the extent of anticipated recurrence of leg length inequality with further growth was deemed unpredictable and, therefore, will be managed in the future by contralateral epiphysiodesis and/or ipsilateral femoral and/or tibial lengthening at the surgeon’s recommendations and patient’s preference. Response to initial limb lengthening procedure will help guide to appropriate management for subsequent leg length inequality treatment.

Basic Principles PRECICE intramedullary lengthening nail consists of a magnetic motor imbedded in the telescopic body [2–4]. The nail is available in 10.7-mm and 12.5-mm diameter with a maximal stroke of 65 mm. Distraction is achieved by manipulation of the internal motor using external electromagnetic actuator that held over the nail magnet. The patient usually activates the device three to four times daily in 0.25-mm increments after a latency period corresponding with bone formation in the distraction gap and soft tissue tolerance. The device has polarity and can be reversed to compression providing an important utility during the lengthening with developing joint contracture or poor bone formation in the distraction regenerate.

Images During Treatment See Figs. 2, 3, 4, 5, 6, 7, 8, and 9.

Technical Pearls

Fig. 1 Standing AP radiograph of lower extremities demonstrating right distal femoral varus (LDFA ¼ 97
, MPTA ¼ 87
, JLCA ¼ 0
), leg length inequality (7.5 cm), and multifocal physeal growth disturbance including complete disruption of the right distal femoral physis

Intramedullary lengthening requires careful attention to preexisting deformities and, in the case of femoral lengthening, medial translation of the knee secondary to obligate lengthening along the anatomic femoral axis. The Reverse Planning Method as described by Baumgart [1] is a valuable preoperative planning tool for angular deformity correction accounting for axis translation induced by lengthening of the femur along the anatomic axis. At surgery, angular and rotational deformity correction can be controlled by using a pair of half pins inserted into the proximal and distal segments prior to osteotomy perpendicular to their anatomic axis and out of the way of the intended tract of the nail. Distraction during intramedullary lengthening with PRECICE nail occurs by placing the electromagnetic actuator over the imbedded magnet of the nail. This area should be marked with a water-

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Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with. . .

Fig. 2 Diagram illustrating the “Reverse Planning Method” for correction of distal femoral varus deformity: (a) initial outline of the femur (black) after distal femoral osteotomy; (b) the desired location of the proximal segment (red) at the end of lengthening; (c) translation of the

Fig. 3 Intraoperative photograph showing femoral medullary canal reaming using straight reamers

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proximal segment (green) along the axis of the nail into position at the beginning of lengthening; (d), position of the proximal segment and the nail at the end of surgery. Note correction of varus deformity with translation of opposed surfaces of bone segments

18 Fig. 4 Intraoperative photograph demonstrating PRECICE intramedullary lengthening nail

Fig. 5 Intraoperative photographs showing test of nail elongation using external electromagnetic actuator

Fig. 6 Intraoperative fluoroscopic views of the femur before (left) and after (right) test of nail elongation. Note 1-mm gap on the right illustrating nail elongation

J. Birch et al.

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Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with. . .

Fig. 7 Postoperative AP and LAT radiographs of the femur demonstrating nail orientation and position of bone segments after acute deformity correction. The postoperative regimen included gradual distraction 0.25 mm four times daily, guided physical therapy concentrating on joint motion, maintenance of muscle strength, and compliance with a partial weight bearing

resistant rectangular mark at the end of surgery using fluoroscopy. The patient (parents) must be educated as about the polarity of the actuator and the location of the nail magnet. Activations of the nail motor take approximately 2 min per session.

Outcome Clinical Photos and Radiographs

19

Fig. 8 Standing AP radiograph of lower extremities after completion of limb lengthening

treatment modality before initiating treatment. Because the actuator has polarity, the patient must be carefully instructed in the proper use of the actuator to prevent unintended reverse activation of the nail motor, causing nail compression. Finally, the patient must be carefully monitored at least biweekly during distraction period to confirm maintenance of full knee extension, hip extension and abduction, adequate total joint range of motion, compliance with partial (approximately 20 kg) weight-bearing restrictions, and radiographic monitoring of appropriate distraction and bone formation in the distraction gap.

See Figs. 10, 11, 12, and 13.

See Also in Vol. 3 Avoiding and Managing Problems Patients undergoing intramedullary limb lengthening are subject to the same risk of joint stiffness, joint subluxation/ dislocation, and prolonged consolidation of the distraction regenerate, among other potential complications. Patients should be carefully screened and counseled regarding this

Femoral Lengthening (12 cm) with Two Precice Nail Lengthenings. Management of a Broken Precice Nail During the First Lengthening Femur Lengthening with Precice Internal Lengthening Nail Tibial Lengthening Using a PRECICE Nail

20 Fig. 9 LAT radiograph of the femur after completion of limb lengthening

Fig. 10 Front and side view photographs showing clinical appearance of lower extremities during consolidation

J. Birch et al. Fig. 11 Standing AP radiograph of the lower extremities during consolidation demonstrating active remodeling of the distraction regenerate with corticalization

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Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with. . .

21

Fig. 13 LAT radiograph of the femur demonstrating knee flexion

References and Suggested Reading

Fig. 12 LAT radiograph of the femur during consolidation

1. Baumgart R. The reverse planning method for lengthening of the lower limb using a straight intramedullary nail with or without deformity correction: a new method. Oper Orthop Traumatol. 2009;21:221–33. 2. Harris M, Prince D, Paley D. New implantable lengthening nail. In: Limb lengthening and reconstruction society. ASAMI-North America. New York; 2013. 3. Herzenberg J, Standard S, Conway J. Limb Lengthening with a new, controllable, internal device in patients with congenital shortening. In: Limb lengthening and reconstruction society. ASAMI-North America. New York; 2013. 4. Kirane Y, Fragomen A, Rozbruch R. Precision of internal lengthening nail. In: Limb lengthening and reconstruction society. ASAMI-North America. New York; 2013.

4

Correction of Partial Epiphyseal Growth Arrest and 7 cm Shortening in 10 Years Old Girl Mark Eidelman

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27

Abstract

Growth arrest of the physis is a well-known complication after damage to the physis from trauma, infection, or tumor. The outcome of complete growth arrest is shortening of the affected limb, but partial growth arrest of the epiphysis will lead to shortening and angulation. The amount of deformity and limb length discrepancy (LLD) is directly related to the age of the child at the time of the physeal damage. The treatment strategy in a growing child with partial growth arrest is different from the strategy in a mature patient.

Brief Clinical History A 16 year old girl presented with limping, valgus deformity, and shortening of the left femur (Fig. 1a–c). Seven years earlier, she was hospitalized due to osteomyelitis of the left

distal femur. Leg length discrepancy is 7 cm, projected leg length discrepancy based on the multiplier formula [4] is 10 cm, and projected height at maturity is 161 cm.

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List 1. Partial growth arrest of the left distal femur 2. Valgus deformity of the distal femur 3. Leg length discrepancy

M. Eidelman (*) Technion Faculty of Medicine, Rambam Health Care Campus, Meyer’s Children Hospital, Haifa, Israel e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_64

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M. Eidelman

Fig. 1 (a) Clinical picture before treatment. (b) Preoperative X-rays demonstrate valgus deformity and shortening of the femur. (c) Close-up view of the epiphysis demonstrated that the medial part of the physis of the left distal femur is open, while the lateral part is closed

Treatment Strategy Percutaneous medial epiphysiodesis was performed first in order to prevent further progression of the distal femoral valgus (Fig. 2a). One year later (Fig. 2b), estimation of projected LLD was done, and the distal femoral valgus was corrected in addition to lengthening of 50 mm using a Taylor spatial frame (TSF). A second lengthening of 50 mm using TSF was performed after maturity 3 years afterward.

Basic Principles Established partial growth arrest will lead to progressive limb angulation; therefore, the first treatment mode should be completion of the epiphysiodesis. Failure to do this in immature patients will result in recurrence of the deformity [2]. Correction of leg length discrepancy of more than 60 mm is a difficult task, and the results might be unpredictable. A staged program consisting of first lengthening and an epiphysiodesis of the long limb is an acceptable and well-known treatment mode. An alternative solution to treat LLD is to perform a double-stage lengthening of the short limb. Parents should be informed about height at maturity and available treatment options. Correction of femoral deformities and shortening might be performed

using a monolateral or circular external fixation or fixatorassisted femoral nailing [1].

Images During Treatment See Figs. 2, 3, and 4.

Technical Pearls CORA (center of rotation of angulation) was close to the knee joint (Fig. 2b); therefore, in order to prevent translation, osteotomy was performed as close as possible to the knee joint. For better knee flexion, a 2/3 distal ring was applied. Only one Ilizarov wire was inserted in order to eliminate muscle irritation. This wire was removed 6 weeks after completion of correction. Two additional 6 mm half pins were inserted, one from posterolateral and second from posteromedial directions. At the time of the second lengthening, the femur was straight, which explains why a midshaft osteotomy (Fig. 4a) was performed (less stress and stiffness to the knee joint). Both lengthening procedures were performed using two 2/3 rings (distal ring was open posteriorly and the proximal open medially).

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Correction of Partial Epiphyseal Growth Arrest and 7 cm Shortening in 10 Years Old Girl

25

Fig. 2 (a) Percutaneous epiphysiodesis using 1.8 Ilizarov wire and 6 mm cannulated drill. (b) X-rays show leg length discrepancy after epiphysiodesis (image taken 1 year after X-rays on Fig. 1b). (c) Sagittal alignment of left limb

Fig. 3 (a) X-rays at the end of first lengthening and deformity correction. (b–c) Coronal and sagittal alignment before removal of TSF

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M. Eidelman

Outcome Clinical Photos and Radiographs

Avoiding and Managing Problems

See Fig. 5.

A 50mm femoral lengthening through a distal femoral osteotomy may cause stiffness of the knee joint. Remove Ilizarov wires in case of muscle irritation. Oblique half pins

Fig. 4 (a) Intraoperative view shows location of the osteotomy far away from the knee joint. Note that distal fragment was fixed with three 6 mm half pins and one 1.8 Ilizarov wire. (b) Long X-rays

demonstrate mechanical axis at the end of the second lengthening. (c) Sagittal view before removal of TSF

Fig. 5 (a) After removal of the external fixator, the femur is straight and final LLD is 2 mm (right longer than left). (b–d) Clinical pictures at age 16 demonstrate straight limb with full range of knee motions

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Correction of Partial Epiphyseal Growth Arrest and 7 cm Shortening in 10 Years Old Girl

inserted posterolaterally and posteromedially do not irritate quadriceps and hamstring muscles when inserted properly. Fascia lata is the “enemy of femoral lengthening” and should be released during the lengthening through a small incision.

Cross-References ▶ Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral Lengthening with FITBONE Retrograde Intramedullary Nail

27

References and Suggested Reading 1. Marangoz S, Feldman DS, Sala DA, Hyman JE, Vitale MG. Femoral deformity correction in children and young adults using Taylor spatial frame. Clin Orthop Relat Res. 2008;466:3018–24. 2. Ogden JA. The evaluation and treatment of partial physeal arrest. J Bone Joint Surg. 1987;69:1297–302. 3. Paley D. Principles of deformity correction. Berlin: Springer; 2002. 4. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. J Bone Joint Surg Am. 2000;82:1432–6.

5

Distal Femoral Fracture Treated Initially with Internal Fixation Converted to Circular External Fixation due to Nonunion and Hardware Failure Lane Wimberly, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33

Abstract

A 14 year old male with a comminuted Salter-Harris IV fracture of the distal femur was treated initially by open reduction and internal fixation and was converted to circular external fixation after nonunion, infection, deformity, and implant failure.

Brief Clinical History The patient is a 14 year old male who fell onto his knee while jumping a fence and sustained a comminuted, closed SalterHarris IV fracture of the distal femur (Fig. 1). He was treated

initially with open reduction and internal fixation using a 4.5 pre-contoured locking plate proximal to the physis and cannulated screws across the epiphyseal fracture. He was placed into a long leg cast for additional immobilization (Fig. 2). Six weeks after surgery, his cast was removed as early callus was noted on routine radiographs. At his 12-week follow-up, the plate was broken, and a varus-procurvatum deformity had occurred. This angulation was deemed clinically acceptable and a trial to achieve union was begun. Twelve months after injury, the patient complained of persistent, mild pain at the knee, and the radiographic appearance of the fracture was consistent with nonunion (Fig. 3).

Preoperative Clinical Photos and Radiographs L. Wimberly (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected]

See Figs. 1, 2, and 3.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_77

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Fig. 1 Preoperative radiograph demonstrating a comminuted SalterHarris IV distal physeal femoral fracture

L. Wimberly et al.

Fig. 3 AP and LAT radiographs of the femur 12 months after surgery revealing broken plate and a varus-procurvatum deformity of the distal femur

• Potential osteomyelitis • Varus and procurvatum deformity of the distal femur

Treatment Strategy The preoperative plan included examination under anesthesia, implant removal, wound culture, nonunion biopsy, debridement of the nonunion site, and application of a TrueLok circular external fixator for acute deformity correction and stabilization in compression. His wound unexpectedly grew clindamycin-resistant methicillin-resistant Staphylococcus aureus (MRSA). He was treated with Bactrim at the recommendation of the infectious disease service for 5 months until clinical and radiographic union was achieved. Fig. 2 Postoperative radiograph of the femur after initial stabilization of the fracture with a lateral pre-contoured distal locking plate and cannulated screws across the epiphyseal fracture

Preoperative Problem List • Implant (broken locking plate) failure • Nonunion of the comminuted distal femoral metaphyseal fracture

Basic Principles Nonunion of the distal femoral metaphysis in healthy patients after open reduction and internal fixation should be considered an infectious complication until sterile cultures are obtained. With clinical suspicion for a low-grade infection contributing to the nonunion, implant removal and nonunion debridement were planned. External fixation is the preferred method of reduction and stabilization in infectious cases.

5

Distal Femoral Fracture Treated Initially with Internal Fixation Converted to Circular External Fixation. . .

Stabilization of bone in compression at the nonunion often achieves union despite active infection. In addition, external fixation permits acute or gradual correction of associated deformities to ensure acceptable anatomic alignment as local anatomy may be distorted due to resorption of bone or formation of hypertrophic callus [1–3]. Upon examining the nonunion site in our patient, the ends of the femur appeared to be viable and we proceeded with acute deformity correction and compression. As no significant shortening occurred from a large resection mass, there was no need for subsequent lengthening or bone transport.

Fig. 4 Intraoperative photograph of the femur with an applied TrueLok circular external fixator after acute deformity correction and compression. A proximal femoral arch 5/8 ring block with three half pins and a distal ring with two cross wires and one lateral-posterior half pin were connected with four threaded rods. Note half pin (gray) and wire (red) perforated rubber stoppers to secure dressings (e.g., 22 in. drain sponges) and stabilize soft tissues around the wires and half pins

Fig. 5 Intraoperative AP and LAT radiographs of the distal femur after acute deformity correction and compression demonstrating restored alignment and bone apposition at the nonunion site

31

Images During Treatment See Figs. 4, 5, 6, and 7.

Technical Pearls We use preassembled circular external fixators for correction of most bone deformities. However, in periarticular nonunions, our preference is to independently stabilize the proximal and distal segments and then select connection modules according to the residual deformity after debridement and acute correction. If the deformity was completely corrected acutely and the proximal and distal external supports are parallel to each other, then 3–4 regular threaded rods may be used for compression. When the rings are not parallel, rapid adjust struts are used as they allow significant freedom in alignment, more precise repositioning of bone segments, and interconnection of nonparallel external supports followed by acute and/or gradual compression. If the bone deformity cannot be corrected acutely and gradual correction is required, opposing external supports are interconnected by either two hinges with an angular distractor or by six struts of the hexapod-type frame. To stabilize the soft tissues and secure dressings such as 2  2 in. drain sponges around the wires and half pins, two types of perforated rubber stoppers are utilized postoperatively. In case of limb edema or local swelling, those stoppers will slide along the wire or half pin, thereby preventing soft tissue pressure necrosis.

Outcome Clinical Photos and Radiographs See Figs. 8, 9, and 10.

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Avoiding and Managing Problems Conversion to circular external fixation after failed internal fixation of this comminuted fracture presented several challenges related to soft tissue anatomy, osteopenia, and changes in bone structure. Our standard frame configuration for the proximal femur uses half pin fixation. Two half pins attached directly to the arch are inserted anterolateral and posterolateral, and a third half pin is inserted distal to the arch bisecting the angle between first two half pins and secured to the arch using the fixation post or cube (Fig. 4). Our typical fixation of the distal femur consists of one horizontal olive wire and two half pins inserted posterolateral and posteromedial. This pattern is often modified in distal comminuted femoral fractures, and we will primarily use tensioned olive wires. Two crossing olive wires are inserted, tensioned, and secured to the ring, providing side-to-side compression between the bone segments. An additional wire or half pin is introduced to adjust the ring orientation relative to the distal femur and improve the overall stability of the construct.

Fig. 6 Photograph of the lower extremities during the treatment. Frame configuration and stability of bone segment fixation allowed the patient to stand and walk with full weight bearing

Fig. 7 AP and LAT radiographs of the distal femur 5 months after surgery demonstrating solid mineralized callus. The frame was soon removed

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Distal Femoral Fracture Treated Initially with Internal Fixation Converted to Circular External Fixation. . .

33

Fig. 8 Radiographs of the pelvis and lower extremities 1 year after frame removal demonstrating complete remodeling of the callus with proper alignment of the limbs

Fig. 10 Flexion of the knee 1 year after frame removal. The patient has no complaints and is happy with the appearance of his lower extremities. With his function completely restored, he has returned to competitive soccer

Cross-References ▶ Comminuted Type I Open Distal Femur Fracture ▶ Ten Year Old Male with Comminuted Distal Femoral Fracture

See Also in Vol. 3 Pathologic Femur Fracture Close to Distal Femoral Physis

References and Suggested Reading 1. Maini L, Chadha M, Vishwanath J, Kapoor S, Mehtani A, Dhaon BK. The Ilizarov method in infected nonunions of fractures. Injury. 2000;31(7):509–17. 2. Marsh DR, Shah S, Elliott J, Kurdy N. The Ilizarov method in nonunion, malunion and infection of fractures. J Bone Joint Surg (Br). 1997;79(2):273–9. 3. Sabharwal S. Role of Ilizarov external fixator in the management of proximal/distal metadiaphyseal pediatric femur fractures. J Orthop Trauma. 2005;19(8):563–9. Fig. 9 Photograph of the lower extremities illustrating clinical appearance 1 year after frame removal

6

Oblique Plane Deformities of Femur and Tibia After Open Fracture Treated with TSF Marie Gdalevitch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40

Abstract

This case is an example of post-traumatic growth arrest of the distal femur with an unrecognized injury to the tibial tubercle growth plate at the time of the injury. Careful analysis of the radiographs and drawing the joint orientation angles demonstrated that the patient developed a distal femoral valgus and limb length discrepancy as well as a proximal tibia varus and recurvatum deformity. The patient required gradual correction using a six-axis circular external fixator for the tibial and the femoral deformities.

M. Gdalevitch (*) Verdun Hospital, Montreal, QC, Canada e-mail: [email protected]

Brief Clinical History This is the case of a healthy 18 year old male who suffered a significant trauma to his left knee at the age of 10. He had an open Salter-Harris II fracture of the distal femur with the skin opening on the lateral side. He was treated with irrigation, debridement and closed reduction, and casting at an outside hospital. Two years later, he represents to the outside hospital with a progressive knee deformity and a small area of redness over the previous open fracture site. The incision was opened and a small superficial abscess was debrided. The knee deformity was later treated with a distal medial femoral eight plate for guided growth. He presents to our hospital 6 years later with a deformity of his knee. On exam, his left leg is in valgus alignment and is 3 cm shorter when standing. He has recurvatum of 20 in the left knee compared to 3 on the right. The skin at the previous open fracture site was slightly red. He denies any fevers, chills, or constitutional symptoms, and the remainder of the exam was normal.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_17

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Preoperative Clinical Photos and Radiographs

M. Gdalevitch

1. Tibia vara of 5 and recurvatum deformity of 15 2. Potential osteomyelitis or femoral abscess at old open fracture site 3. Femoral valgus of 17 4. Limb length inequality of 3.5 cm

superficial abscess was found at the time exploration, and it was debrided and closed, and the patient was placed on 2 weeks of antibiotic treatment. Tibial correction for the recurvatum and varus deformity took 3 weeks, and once the regenerate was healed, the TSF was removed and the patient was placed in a Sarmiento patellar bearing cast. A few months later, the patient returned for treatment of the LLD and femoral deformity. The previous abscess site remained clean and dry without any redness. A femoral TSF was positioned and the correction began 5 days later. The patient was permitted to weight bear the entire duration of treatment, and the fixator was removed once the correction was achieved.

Treatment Strategy

Basic Principles

Due to the uncertainty with regard to a possible femoral osteomyelitis or abscess at the previous open fracture site, we decided to begin with gradual correction of the tibial deformity and exploration of the femoral wound. A preoperative MRI demonstrated no osteomyelitis in the leg. A small

Distal femoral growth plate injuries can often lead to growth arrest [6] and an associated injury at the tibial tubercle can be missed [1, 3]. When assessing patients with growth arrests around the knee, it is important to do a thorough physical exam and obtain coronal and sagittal plane radiographs.

See Figs. 1, 2, and 3. See Table 1.

Preoperative Problem List

Fig. 1 Clinical photographs demonstrating the valgus and recurvatum deformity of the knee

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Fig. 2 Weight-bearing anteroposterior and lateral radiographs of the patient’s lower limbs demonstrating a 3.5 cm limb length discrepancy with a valgus femur and varus and recurvatum of the tibia

Fig. 3 Two coronal cuts from the patient’s MRI performed a few years after his accident, demonstrating the malunion of the distal lateral femur and subsequent growth arrest

Drawing joint orientation angles helps delineate the origin of the deformity and allows the surgeon to then identify the apex of the deformity (center of rotation and angulation). Some small abnormalities in joint orientation angles can be

acceptable, and the surgeon can compensate for these when correcting the other bone. Other times, both bones need to be addressed separately, such as in this case. Tibial recurvatum deformities can be challenging to treat acutely, and gradual

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Table 1 Joint orientation angles in the coronal and sagittal planes as well as limb assessments of the limbs demonstrating the marked abnormalities in the left LDFA, MPTA and PPTA Coronal LPFA LDFA MPTA LDTA JLCA Femur (cm) Tibia (cm) Total LLD Sagittal aPDFA aPPTA aADTA

Right 86 87 86 87 2 50 40.1 Right 83 83 80

Left 87 71 81 89 0 47.5 39.5 3.1 Left 85 98 84

correction may facilitate the accuracy of correction and the healing of the bone [2, 4]. Femoral valgus deformities can be treated acutely depending on their severity; however, the surgeon needs to be wary of stretching the peroneal nerve. Decompressing the nerve and shortening the bone is necessary to avoid stretch when performing acute corrections. In this case, had the tibia only had a slight varus deformity, the surgeon could have decided to under-correct the femoral valgus to avoid operating on the tibia. This would have left the patient with a varus tibia and valgus femur. This is a bad deformity combination for the knee and theoretically would increase the risk of arthritis [5]. However, given the tibial recurvatum deformity along with the varus, treating the tibial and femoral deformities seemed more reasonable. Furthermore, given the limb length discrepancy and 17 valgus deformity of the femur, a gradual correction was the best treatment option for both bones.

Images During Treatment See Figs. 4, 5, and 6.

Technical Pearls • When dealing with a recurvatum deformity of the proximal tibia, the surgeon can use the distal ring as the reference to facilitate planning the correction. Furthermore, the proximal ring can be placed to mimic the deformity and therefore help visualize the correction. • The configuration of the fixation for the distal femoral ring is to mount it on a wire and then add one posteromedial and one posterolateral half pin. • Two 2/3 rings are used when doing a femoral frame to allow for comfort for the patient.

Fig. 4 Weight-bearing anteroposterior and lateral radiographs of the corrected tibia with the use of the Taylor Spatial Frame (TSF). Note the overall valgus alignment of the limb looks worse since the varus of the tibia was corrected

• When using the planning software for a femoral TSF, a distal reference is preferred with a rotatory frame offset of 60 of external rotation.

Outcome Clinical Photos and Radiographs See Fig. 7.

Avoiding and Managing Problems • When correcting a recurvatum deformity, clinically assessing knee extension is important to avoid overcorrecting and creating a knee flexion deformity. Therefore, it is important to always ensure that the patient maintains full knee extension during correction. • If there is concern about stretching the peroneal nerve, the nerve decompression should be done before mounting the

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Fig. 5 Intraoperative photographs of the femoral TSF

Fig. 6 Weight-bearing anteroposterior and lateral radiographs during the femoral lengthening and correction using the TSF

Fig. 7 Weight-bearing anteroposterior and lateral radiographs of the patient a year post removal of the femoral external fixator

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frame. Doing a peroneal nerve decompression at a later date becomes more difficult with the frame in place. Therefore, in cases where a large valgus deformity is being corrected (even gradually), it is safer to perform the decompression at the time of initial surgery.

Cross-References ▶ Correction of Partial Epiphyseal Growth Arrest and 7 cm Shortening in 10 Years Old Girl

See Also in Vol. 2 Computer Assisted External Fixation at Femur Malunion Accompanied with Complex Deformity

References and Suggested Reading 1. Bowler JR, Mubarak SJ, Wenger DR. Tibial physeal closure and genu recurvatum after femoral fracture: occurrence without a tibial traction pin. J Pediatr Orthop. 1990;10(5):653–7. 2. Choi IH, Chung CY, Cho TJ, Park SS. Correction of genu recurvatum by the Ilizarov method. J Bone Joint Surg (Br). 1999;81(5):769–74. 3. Domzalski M, Mackenzie W. Growth arrest of the proximal tibial physis with recurvatum and valgus deformity of the knee. Knee. 2009;16(5):412–6. 4. Manohar Babu KV, Fassier F, Rendon JS, Saran N, Hamdy RC. Correction of proximal tibial recurvatum using the Ilizarov technique. J Pediatr Orthop. 2012;32(1):35–41. 5. Paley D, Herzenberg J. Principles of deformity correction. Springer. Chapter 14; 2005. p. 455. 6. Wall EJ, May MM. Growth plate fractures of the distal femur. J Pediatr Orthop. 2012;32(Suppl 1):S40–6.

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Open Femoral Fracture Treated with Internal Fixation Converted to Circular External Fixation due to Infection Lane Wimberly, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41 Preoperative Problems List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46

Abstract

Seven year old female sustained a Salter-Harris II comminuted distal type IIIA open femoral fracture with associated bone loss. She was treated initially with locking plate internal fixation and converted to a circular external fixator because of an acute infection and loss of fixation.

with a lateral distal femoral locking plate that was placed proximal to the physis (Fig. 2). The wound was closed primarily. She returned to the hospital with pain, swelling, erythema, fever, and elevated infectious indices 2 weeks after her injury and fixation.

Preoperative Clinical Photos and Radiographs Brief Clinical History The patient is a 7 year old female who was a rear-seat passenger ejected from a golf cart sustaining a Salter-Harris II comminuted distal open type IIIA fracture of the femur with bone loss (Fig. 1). Initial treatment included urgent irrigation, debridement, open reduction, and internal fixation L. Wimberly (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]

Figs. 1 and 2.

Preoperative Problems List • • • •

Comminuted distal femoral fracture with bone loss Growth plate injury with the potential for physeal arrest Postoperative polymicrobial wound infection Loss of fracture fixation

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_76

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Treatment Strategy The patient underwent several irrigation and debridement procedures with wound VAC applications to control the acute infection. Once the wound appeared clean, the implants were removed, and the wound was closed following the application of a TrueLok circular external fixator. She was managed with amoxicillin-clavulanate for a polymicrobial wound infection. In conjunction with the infectious disease service, it was decided she should remain on antibiotic therapy until fracture union and frame removal.

Basic Principles

Fig. 1 Preoperative radiograph demonstrating a comminuted distal Salter-Harris II femoral fracture

The application of an external fixator as a bridge construct permits extraphyseal implant placement and maintains the anatomic length of the thigh, the soft tissue, and the residual bone segments. Available internal fixation options are not suitable for comminuted physeal fractures complicated by infection. The use of bone graft is generally contraindicated in the presence of an acute polymicrobial wound infection. In cases with bone loss that fail to achieve union, bone transport, bone grafting, or bone lengthening after acute shortening can be performed in the future. Limitation of knee motion and contribution to post-injury knee stiffness are drawbacks to the intracapsular implants of the external fixator [3, 5]. We encourage aggressive physical therapy to maintain range of motion during treatment and to limit the anticipated knee stiffness.

Images During Treatment Figs. 3, 4, 5, and 6.

Technical Pearls

Fig. 2 AP and LAT radiographs of the femur after initial stabilization with a lateral distal locking plate

The key features of the TrueLok Trauma (TL-T) bridging fixator assembly are the circular and semicircular external supports that are connected by rapid-adjust struts (RAS). Compared to traditional pin-to-bar spanning frames, TL-T external supports are secured using combinations of tensioned wires and half pins. The TL-T frame provides superior stabilization of the injury, improves the ability to make controlled adjustments, and increases options for fixation of comminuted fractures. During assembly, proximal and distal external supports are provisionally connected with one or two RAS. Each strut has a telescoping body with an independent acute/gradual adjustment dial and a claw-and-socket lockable

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Fig. 3 Postoperative AP and LAT radiographs of the femur after conversion to circular external fixation. A TrueLok Trauma spanning frame was assembled with a proximal femoral arch connected to the femur by 2 half pins and a distal 5/8 ring-half ring block with 3 tensioned epiphyseal olive wires. The proximal and distal external supports were bridged by 3 rapid-adjust struts. The frame was placed as a bridge construct without shortening or bone graft

universal joint at each end. Temporarily releasing the telescoping body and all hinges allows for reduction. Tightening of the preliminary rapid-adjust struts provides temporary stabilization of bone segments in the reduced position. If necessary, the position of the bone segments can be finely tuned using gradual distraction or compression through the struts. Once acceptable alignment is achieved, extra struts are added to the frame to improve the rigidity of the construct. Dynamization of the struts can be performed to allow axial motion and to improve fracture callus maturation.

Outcome Clinical Photos and Radiographs Figs. 7, 8, 9, and 10.

Avoiding and Managing Problems The loss of motion after external fixation of femoral fractures has been previously described in the literature [1, 2, 4]. Most surgeons would consider some loss of knee motion as inev-

Fig. 4 Photograph of the lower extremities during the treatment

itable in the setting of a large traumatic wound with soft tissue injury, acute infection, and multiple procedures to achieve union. In this injury, no internal fixation device would allow adequate fixation and alignment given the infectious complication and fracture location. Distal femoral epiphyseal fixation using opposing olive wires is necessary to control the fracture after reduction. The epiphyseal olive wires are intra-articular and penetrate the joint capsule and therefore limit knee motion. In addition, the proximal half pins penetrate the quadriceps to exacerbate this motion loss. While stressing physical therapy to maintain knee motion during treatment, sometimes the joint stiffness requires further surgical intervention. Although the intraoperative improvement in motion in our patient after lysis of adhesions and quadriceps release was modest, it may allow her to begin to progress again with physiotherapy. We would recommend aggressive physical therapy and early surgical intervention in patients with an inability to regain expected joint motion.

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Fig. 5 The frame configuration and the position of external supports allowed the patient to sit comfortably in a chair

Fig. 6 AP and LAT radiographs of the femur demonstrating proper alignment and maturing fracture callus. She achieved radiographic union 9 months after application of the external fixator. Her subsequent frame removal was uneventful

Fig. 7 Photograph of the lower extremities illustrating the clinical appearance 1 month after frame removal. Because of a knee extension contracture and stalled physical therapy gains, she underwent an arthroscopic lysis of adhesions and quadriceps release from the femur 1 month later

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Fig. 10 LAT radiograph of the knee with maximum flexion 6 months after frame removal. After the lysis of adhesions and release of the quadriceps, her intraoperative knee flexion was 80

Fig. 8 AP and LAT radiographs of the femur 6 months after frame removal demonstrating remodeling of the callus with proper alignment of the limb. The possible sequela of distal femoral physeal arrest is being followed with sequential radiographs

Fig. 9 LAT radiograph of the knee with maximum extension 6 months after frame removal

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Cross-References

References and Suggested Reading

▶ Comminuted Type I Open Distal Femur Fracture ▶ Ten Year Old Male with Comminuted Distal Femoral Fracture

1. Babar IU. External fixation in closed comminuted femoral shaft fractures in adults. J Coll Physicians Surg Pak. 2004;14(9):553–5. 2. Marangoz S, Feldman DS, Sala DA, Hyman JE, Vitale MG. Femoral deformity correction in children and young adults using Taylor Spatial Frame. Clin Orthop Relat Res. 2008;466(12):3018–24. 3. Marsh DR, Shah S, Elliott J, Kurdy N. The Ilizarov method in nonunion, malunion and infection of fractures. J Bone Joint Surg (Br). 1997;79(2):273–9. 4. Pavolini B, Maritato M, Turelli L, D’Arienzo M. The Ilizarov fixator in trauma: a 10-year experience. J Orthop Sci. 2000;5(2):108–13. 5. Sabharwal S. Role of Ilizarov external fixator in the management of proximal/distal metadiaphyseal pediatric femur fractures. J Orthop Trauma. 2005;19(8):563–9.

See Also in Vol. 3 Pathologic Femur Fracture Close to Distal Femoral Physis

8

Post-traumatic Deformity and Bone Loss in 8 Year Old Boy Mark Eidelman

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52

Abstract

An 8 year old boy with a Gustilo–Anderson type 3 open fracture of the tibia and large soft tissue defect was treated with debridement and temporary external fixation. After acute shortening and posterior angulation his fixation was converted to a Taylor spatial frame (TSF) with gradual deformity correction and lengthening. He underwent several interventions up to maturity to address his residual deformities.

Brief Clinical History The patient is an 8 year old boy who was involved in a traffic accident, sustaining a Gustilo–Anderson type 3b open tibial fracture with a large soft-tissue anterior leg defect and 40 mm of bone loss after debridement (Fig. 1a–c). Additionally, he had an ipsilateral closed fracture of the femur (Fig. 1d).

Preoperative Clinical Photos and Radiographs See Fig. 1.

M. Eidelman (*) Technion Faculty of Medicine, Rambam Health Care Campus, Meyer’s Children Hospital, Haifa, Israel e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_66

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Fig. 1 (a) Clinical picture demonstrated soft-tissue defect and bone loss. (b, c) Anteroposterior (AP) and lateral X-rays of the leg. (d) X-ray of ipsilateral fracture of the proximal femur

Preoperative Problem List

Treatment Strategy

1. Extensive soft-tissue defect 2. Bone loss of the tibia and fibula 3. Ipsilateral femoral fracture

After plating of the femur and temporary external fixation his soft-tissue and bone defects were treated using an acute shortening and a “posterior angulation” technique with two Taylor spatial frame (TSF) rings. After correction of the deformation, the length was restored after proximal tibial osteotomy and gradual lengthening of the tibia.

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Post-traumatic Deformity and Bone Loss in 8 Year Old Boy

Basic Principles Closure of an extensive soft-tissue defect is a difficult task and may need microvascular intervention and free flap coverage. Post-traumatic bone loss with stripping of the periosteum is another challenging problem. Posterior leg angulation and shortening using a circular frame was described in order to facilitate wound closure and bone apposition [1–3]. In our

Fig. 2 (a) X-ray after debridement and preparation of the tibia and fibula for shortening and angulation. (b, c) X-rays after posterior angulation and shortening of the tibia and fibula. (d) Clinical picture

49

case, a combined approach of intentional angulation and bone compression was the only way to achieve wound closure without free flap transplantation.

Images During Treatment See Figs. 2 and 3.

demonstrated healing of the wound and temporary deformation of the leg. (e, f) AP and lateral X-rays demonstrated deformity correction of the tibia and fibula

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Fig. 3 (a) Clinical picture before application of the third ring and proximal tibia and fibula osteotomy. (b, c) AP and lateral X-rays after proximal tibial and fibular osteotomy

Technical Pearls

Outcome Clinical Photos and Radiographs

Meticulous debridement and excision of all devitalized tissues are prerequisites before bone surgery. Fibular fixation to the distal tibia is important to prevent later ankle malalignment. Two rings of the TSF should be applied relatively close to the site of compression (Fig. 2d) to give additional space for the third proximal ring and a proximal tibial osteotomy (Fig. 2b, c).

See Fig. 4.

Avoiding and Managing Problems Open physes in immature patients may cause residual deformity. Monitoring of the mechanical axis should be continued until maturity (Fig. 4c–e).

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Post-traumatic Deformity and Bone Loss in 8 Year Old Boy

Fig. 4 (a, b) AP and lateral X-rays after 40 mm of proximal tibial lengthening before removal of the frame. (c) Residual valgus of the tibia 7 years after the trauma. (d) A Taylor spatial frame (TSF) was applied to

51

correct valgus of the tibia. (e) Final X-rays after maturity demonstrated a normal mechanical axis and equal length

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Cross-References

References and Suggested Reading

▶ Ten Year Old Male with Comminuted Distal Femoral Fracture

1. Lerner A, Fodor L, Soudry M, Peled IJ, Herer D, Ullman Y. Acute shortening: modular treatment modality for severe combined bone and soft tissue loss of extremities. J Trauma. 2004;57:603–8. 2. Nho SJ, Helfet DL, Rozbruch SR. Temporary intentional leg shortening and deformation to facilitate wound closure using Ilizarov/ Taylor spatial frame. J Orthop Trauma. 2006;20:419–24. 3. Rozbruch SR, Pugsley JS, Fragomen AT, Ilizarov S. Repair of tibial nonunions and bone defects with the Taylor spatial frame. J Orthop Trauma. 2008;22:88–95. 4. Spiegel P, Cooperman D, Laros G. Epiphyseal fractures of the distal ends of the tibia and fibula. J Bone Joint Surg Am. 1978;60:1046–50.

See Also in Vol. 2 Acute Shortening and Then Lengthening

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Proximal Tibial Growth Arrest with Varus, Recurvatum, and Shortening After ACL Reconstruction. Correction with TSF S. Robert Rozbruch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58

Abstract

This is a case of a tibial growth arrest that occurred after anterior cruciate ligament (ACL) reconstruction with a transepiphyseal tunnel done at age 12. This resulted in LLD, varus, and recurvatum deformity. We describe this case and the correction using distraction osteogenesis in conjunction with a Taylor Spatial Frame (TSF) (Smith & Nephew Inc, Memphis,Tennessee, USA) using the Ilizarov method.

Brief Clinical History A 17 year old male patient had an acquired leg-length discrepancy (LLD) and deformity after ACL reconstruction performed at age 12 after a ski injury. Reconstruction was performed using an Achilles allograft by an experienced surgeon. The graft was

fixed in an anatomically placed tibial tunnel in which a plug was countersunk and tied over a button for secure fixation. No tunnel was drilled on the femoral side, and the graft was placed intra-articularly and over the top of the lateral-femoral condyle. The Achilles allograft was then put on tension and fixed to the lateral femur using two small staples. Over the course of several years, there progressively emerged a deformity of the right tibia associated with an LLD. The patient lived overseas and there was little follow-up with the ACL surgeon. He was referred to our service and came for treatment at age 17, which was 4.5 years after initial ACL surgery.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

S. R. Rozbruch (*) Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_57

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Preoperative Problem List 1. 2. 3. 4. 5. 6.

LLD ¼ 4.5 cm Predicted LLD ¼ 5.5 cm Varus deformity 15 Recurvatum deformity 28 External rotation deformity 15 Damaged proximal tibial growth plate

Treatment Strategy 1. Close remaining proximal tibial growth plate to avoid additional deformity. 2. Calculate the growth remaining from the proximal tibial growth plate which in this case was 1 cm. 3. Perform osteotomy at proximal tibia and fibula to correct varus, recurvatum, and external rotation. 4. Lengthen tibia a total of 5.5 cm (4.5 cm preoperative LLD + 1 cm growth remaining that was stopped). 5. Use distraction osteogenesis and the TSF for complex deformity correction and bone lengthening.

Fig. 1 (a, b) Front and side standing views of showing knee deformity and LLD. Growth plates are still open despite his age of 17

Fig. 2 Preoperative X-rays of the knee. (a) AP view showing varus. (b) Lateral view showing PPTA of 110 and recurvatum deformity. Note anterior subluxation of the femur on the tibia related to the recurvatum deformity. Note the anteromedial bone plug from the ACL graft. (c)

Surgical planning on the lateral view. Proximal joint orientation line is drawn (brown). Distal axis line is drawn (maroon). Intersection between lines is the CORA with a magnitude of 28

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Proximal Tibial Growth Arrest with Varus, Recurvatum, and Shortening After ACL Reconstruction. Correction. . .

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4. Current LLD is 4.5 cm. Using growth remaining calculations for the proximal tibial growth plate, another 1 cm of growth is expected to occur. (His bone age was 15.) 5. Close proximal tibial growth plate so no additional deformity will occur after the correction.

Images During Treatment See Figs. 4, 5, and 6.

Technical Pearls 1. Make the proximal ring the reference ring since it is close to the apex of deformity and the origin. 2. Use a two third ring proximally so the knee can bend beyond 90 . 3. Apply the TSF to match the deformity. 4. Stabilize the tibia and fibula both proximal and distal with tensioned wire. This will prevent fibula migration. 5. Use TSF planning to determine the necessary coronal and sagittal plane translation needed during the lengthening and deformity correction. Fig. 3 Preoperative 51 in. erect leg X-ray with a 3 cm block under the right foot. (a) Note the medial MAD and the LLD of 4.5 cm as well as the external rotation of the ankle relative to the knee. (b) Surgical planning is shown. The femur does not have deformity. The femoral mechanical axis line (brown) is extended across the knee. The distal tibial axis line (maroon) is drawn in a retrograde fashion and the intersection is the CORA with a magnitude of 15 of varus

Outcome Clinical Photos and Radiographs

Basic Principles

Avoiding and Managing Problems

1. Growth arrest of the anteromedial aspect of the proximal tibial growth plate (presumably related to the tibial bone tunnel and ACL graft) led to a varus and recurvatum deformity. 2. The rotational deformity is more difficult to explain but likely occurred from the same etiology. 3. The oblique plane deformity (apex posterolateral) and rotational deformity will be corrected gradually with distraction osteogenesis using the TSF.

1. Avoid recurrence of deformity by closing damaged proximal tibial growth plate. 2. Avoid LLD by calculating the predicted LLD and overlengthening the affected side. 3. Do not remove frame until three cortices are seen on biplanar X-rays. 4. Apply long leg cast after frame removal as a transitional phase to prevent fracture.

See Figs. 7 and 8.

56 Fig. 4 (a, b) Front and side view of the TSF frame mounted to the leg to match the varus, recurvatum, and external rotation deformity

Fig. 5 (a, b) Side view and lateral X-ray at the end of distraction showing correction of deformity and proximal tibial lengthening

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Proximal Tibial Growth Arrest with Varus, Recurvatum, and Shortening After ACL Reconstruction. Correction. . .

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Fig. 6 Erect leg standing X-ray at the end of distraction showing equal leg lengths and correction of the MAD line

Fig. 7 (a, b) Patient as an 18 year old with equal leg lengths and correction of deformity

Fig. 8 (a, b) AP and lateral X-rays showing healed and remodeled proximal tibial lengthening and deformity correction. The PPTA on the lateral X-ray is normal

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Cross-References

References and Suggested Reading

▶ Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with PRECICE Retrograde Intramedullary Nail ▶ Guided Growth Treatment for Genu Valgum secondary to Juxtaphyseal Recurrent Aneurysmal Bone Cyst of the Distal Femur ▶ Oblique Plane Deformities of Femur and Tibia After Open Fracture Treated with TSF ▶ Valgus/Flexion and Shortening of the Distal Femur from Growth Arrest Treated with a Monolateral Frame

1. Goldman V, McCoy TH, Harbison M, Fragomen AT, Rozbruch SR. Limb lengthening in children with Russell-Silver syndrome: a comparison to other etiologies. J Child Orthop. 2013;7:151–6. Epub 2013 Jan 5 2. Rozbruch SR, Fragomen A, Ilizarov S. Correction of tibial deformity using the Ilizarov/Taylor spatial frame. J Bone Joint Surg Am. 2006;88-A(Suppl 4):156–74. 3. Rozbruch SR, Segal K, Ilizarov S, Fragomen AT, Ilizarov G. Does the Taylor spatial frame accurately correct tibial deformities? Clin Orthop Relat Res. 2010;468(5):1352–61. 4. Rozbruch SR, Schachter L, Bigman D, Marx R. Growth arrest of the Tibia after ACL reconstruction: lengthening and deformity correction with the Taylor spatial frame. Am J Sports Med. 2013;41(7):1636–41. Published online before print April 25, 2013. https://doi.org/10.1177/ 0363546510369318.

Sixteen Year Old Male with Tibial Malunion Complicated by Pathological Fracture

10

Karl Rathjen, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66

Abstract

A 16 year old male sustained an acute pathological tibial fracture, while playing basketball, distal to previously established midshaft tibial malunion with valgusrecurvatum deformity, lateral translation, and 2.5 cm leg length discrepancy (LLD). The fracture was treated initially by a long-leg cast immobilization converted to TrueLok circular external fixation to establish the union at the pathological fracture site, equalize limb length, and correct complex multi-planar tibial deformity.

Brief Clinical History The patient is a 16 year old male with a well-established midshaft tibial malunion and complex valgus-recurvatum deformity with a one-diameter lateral translation and 2.5 cm proximal translation secondary to tibial/fibular injury 2 years ago in a motocross accident. He sustained an acute pathological tibial fracture distal to the malunion while playing basketball. His soft tissue envelope was intact, and the patient was otherwise healthy. He was treated initially nonoperatively with a long-leg cast immobilization. Three months later, no acceptable healing was observed at the pathological fracture site requiring conversion of the long-leg cast immobilization to circular external fixation to establish union of the pathological fracture and correct complex multi-planar deformity of the tibia.

K. Rathjen (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_82

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Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List • Well-established midshaft tibial malunion • Complex valgus-recurvatum tibial deformity with one-diameter lateral translation • LLD 2.5 cm due to proximal translation at the malunion • Non-united pathological fracture 3.0 cm distal to the malunion • Insufficient 3-month plaster cast immobilization

Treatment Strategy Due to insufficient stabilization of bone segments which resulted in substantial increase of tibial recurvatum deformity, the long-leg cast immobilization was converted to TrueLok circular external fixation with sequential correction of

Fig. 1 AP and LAT radiographs demonstrating an established midshaft tibial malunion with complex deformity (valgus, 5 ; recurvatum, 15 ; one-diameter lateral translation; and 2.5 cm proximal translation) and transverse pathological tibial fracture approximately 3.0 cm distal to the malunion

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components of the complex tibial deformity. Treatment planning included two stages: (1) distal tibial osteotomy through the non-united pathological fracture followed by acute recurvatum deformity correction and compression and (2) secondary coronal-plane oblique osteotomy through the malunion followed by frame modification for acute/gradual correction of residual deformities, lateral translation and LLD.

Basic Principles Different circular external fixator configurations can be utilized for translation deformity correction [1, 6]. In Ilizarov-type frames, translation displacement of the bone fragments can be eliminated either within the rings by tensioning of the horizontally inserted stopper wires or outside the rings by repositioning the ends of the connecting threaded rods to the appropriate-length plates. In addition, various translation

Fig. 2 AP and LAT radiographs 3 months after long-leg cast immobilization illustrating non-united pathological fracture of the distal tibia with increased magnitude of the sagittal plane deformity (recurvatum 30 )

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Sixteen Year Old Male with Tibial Malunion Complicated by Pathological Fracture

modules for gradual correction can be built from multiple frame components and attached between the rings. Ilizarov introduced several types of module assemblies for gradual horizontal bone segment transportation based on the orientation of the threaded rods included into the module assembly.

Fig. 3 First-stage computerized preoperative planning of tibial recurvatum deformity correction using the LegPerfect software

Fig. 4 Intraoperative photograph of the tibia with an applied TrueLok circular external fixator prior to acute recurvatum deformity correction and compression. Proximal and distal tibial bone segments were stabilized in the associated proximal and distal ring blocks with 3 half pins and 3 cross wires, respectively. Two double-ring blocks of the fixator were interconnected by a pair of uniplanar hinges posteriorly and angular distractor anteriorly

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Preassembled translation modules can also be applied. Alternatively, hexapod-type external fixators do not require frame modification for sequential correction of complex deformity. The same six struts can be utilized for either sequential or simultaneous correction. Depending on the chosen protocol,

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this type of external fixators requires only recalculation of the daily strut adjustment to produce a new prescription and perform modification of the movement path for bone segments.

Images During Treatment See Figs. 3, 4, 5, 6, 7, 8, 9, 10, and 11.

Technical Pearls Although the limb realignment can be achieved gradually, we prefer to correct isolated translation deformities under the somatosensory evoked potentials (SSEP) monitoring acutely in several steps, followed by gradual correction of any remaining malalignment if necessary. Our standard frame

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configuration includes 3–4 preassembled translation modules positioned parallel to the correction plane. In cases when both interconnected rings are parallel to the path of bone segment movement, each module consisted of the two posts attached to the opposing rings and one threaded rod with double square distraction/compression nuts placed parallel to the vector of distraction or compression. When the rings are not parallel to the vector of distraction or compression, translation modules are represented by the TrueLok rapid adjust struts, which can be oriented obliquely relative to the ring surface (see Figs. 10 and 11) and allow interconnection of nonparallel external supports. Struts provide very precise translation of bone segments either acutely or gradually.

Outcome Clinical Photos and Radiographs See Figs. 12 and 13.

Avoiding and Managing Problems

Fig. 5 Intraoperative photograph of the tibia immediately after the tibial/fibular osteotomy and acute recurvatum deformity correction with compression demonstrating restored alignment in the sagittal plane. Note that both hinges and angular distractor were replaced by 4 threaded rods for compressive stabilization

Fig. 6 Intraoperative LAT radiographs before and immediately after acute recurvatum deformity correction and compression

Typically, tibial deformity in patients with post-traumatic diaphyseal malunion is an oblique-plane deformity combining coronal/sagittal plane angulation with horizontal and axial displacement and/or external/internal rotation. Correction of this multiaxial displacement always required detailed preoperative planning and, in majority of cases with no external/internal rotation, can be achieved in the simple movement path (simultaneous angular and translational displacement) following to osteotomy, which is performed at the area of the malunion (e.g., just below the former fracture callus). Correction of translation component of the deformity in well-established malunion associated with a secondary pathological fracture, however, presents several challenges

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related to location of the fracture relative to the malunion area. If the pathological fracture occurred at the distance from the malunion, correction of the deformity through the fracture site may result in a cosmetically unacceptable horizontal translation of the distal fragment due to too distal level of the pathological fracture. In addition, this approach may lead to prolonged consolidation time and even development of secondary malunion/nonunion. To overcome those potential complications, surgeons may consider achieving a healing of the pathological fracture first followed by acute/gradual deformity correction at the more appropriate level. Alternatively, treatment planning can be divided in several stages with correction of deformity components at the different levels. In our patient, for example, we elected to correct procurvatum tibial deformity acutely at the pathological fracture level, followed by acute/gradual correction of the lateral/ proximal displacement via separate oblique osteotomy through the malunion area.

See Also in Vol. 2

Fig. 7 AP and LAT radiographs 1 month after surgery demonstrating maintained alignment in coronal and sagittal planes and gradually mineralizing callus

Fig. 8 Second-stage computerized preoperative planning of tibial lateral horizontal translational deformity correction and lengthening using the LegPerfect software

Gradual Correction of Distal Tibia Malunion (Varus with Shortening) Spatial Frame Correction of an Infected Distal Metaphyseal Tibial Nonunion/Malunion

64 Fig. 9 Intraoperative AP radiographs after secondary coronal plane oblique osteotomy through the malunion prior to and immediately after acute correction of the one-diameter lateral horizontal translation. Note that the newly formed middle bone segment was stabilized by one olive-stopper wire and one half pin attached to the additional ring interconnected with the proximal ring block with two rapid adjust struts (RAS) oriented parallel to the direction of the osteotomy and path of coronal plane translation

Fig. 10 AP and LAT radiographs 2.5 months after surgery during the consolidation period. Note perfect limb alignment in both coronal and sagittal planes and progressive consolidation at the pathological fracture and osteotomy sites

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Sixteen Year Old Male with Tibial Malunion Complicated by Pathological Fracture

Fig. 11 Photograph of the lower extremities at 2.5 months of the consolidation period. Frame configuration and stability of bone segment fixation allowed the patient to stand and walk with full weight bearing. Two weeks later, the frame was destabilized by removing half pin from the middle bone segment and medial/lateral threaded rods and RAS for dynamization. After 5 weeks of dynamization, frame was removed. Total time in the circular fixator was 6 months

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Fig. 12 AP and LAT radiographs of the tibia 6 months after frame removal (1 year after pathological fracture) illustrating perfect final limb alignment and actively remodeling calluses with corticalization

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Fig. 13 Clinical appearance of lower extremities after treatment. Patient has no complaints and is happy with the appearance of his lower extremities. His has no residual LLD and his function completely restored

References and Suggested Reading 1. Ilizarov GA. Basic principles of transosseous compression and distraction osteosynthesis. Ortop Traumatol Protez. 1971;32:7–15. 2. Ilizarov GA. Transosseous osteosynthesis: theoretical and clinical aspects of the regeneration and growth of tissues. New York: Springer; 1992. 3. Marangoz S, Feldman DS, Sala DA, Hyman JE, Vitale MG. Femoral deformity correction in children and young adults using Taylor Spatial Frame. Clin Orthop Relat Res. 2008;466(12):3018–24.

4. Marsh DR, Shah S, Elliott J, Kurdy N. The Ilizarov method in nonunion, malunion and infection of fractures. J Bone Joint Surg (Br). 1997;79(2):273–9. 5. Taylor JC. Tibial malunion. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: Informa Healthcare USA, Inc.; 2007. p. 167–76. 6. Tetsworth K, Prodger S. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: Informa Healthcare USA, Inc.; 2007. p. 177–84.

Ten Year Old Male with Comminuted Distal Femoral Fracture

11

Karl Rathjen, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 70 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 74

Abstract

A 10 year old male sustained a Grade III open comminuted distal femoral shaft fracture in a high-speed motor vehicle collision. The fracture was treated initially with a closure of the wounds without debridement and a long-leg splint immobilization converted 1 week later to TrueLok circular external fixation due to high risk of potential infection.

fracture, facial fractures, and fracture of the hand. He was managed initially with closure of his wounds without any debridement and application of a long-leg splint for immobilization (Fig. 1). One week later, he presented for definitive treatment requiring conversion of the long-leg splint immobilization to circular external fixation due to high risk of potential infection.

Preoperative Clinical Photos and Radiographs Brief Clinical History See Fig. 1. The patient is a 10 year old male who was involved in a highspeed motor vehicle collision with fatalities in Mexico and sustained Grade III open comminuted distal femoral shaft

Preoperative Problem List

K. Rathjen (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected]

• Comminuted Grade III open distal femoral shaft fracture • LLD due to potential bone loss • Possible growth plate injury with potential for physeal arrest

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_83

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Fig. 1 Preoperative AP and LAT radiographs demonstrating a Grade III open comminuted distal femoral shaft fracture immobilized with significant posterior translation in the longleg splint

• Insufficient long-leg splint immobilization • High risk of potential infection due to initial wound closure without prior irrigation and debridement and late presentation

Treatment Strategy After elliptical excision of the previous wound, the patient underwent superficial to deep debridement circumferentially and irrigation through the partially split muscles. All bone segments without soft tissue attachment were removed (Fig. 2), creating an approximately 3–4 cm segmental bone defect. Due to insufficient stabilization of bone segments and high risk of potential infection, the long-leg splint immobilization was converted to TrueLok circular external fixation followed by fracture reduction, rapid shortening until the contact between the opposing bone segments, and compression.

Basic Principles Different circular external fixation strategies are applied for initial stabilization and subsequent management of the severe pediatric open fractures. Those strategies include (1) fracture stabilization with compression until the adequate soft tissue coverage in cases with a minimal bone loss, (2) rapid shortening until bone end apposition followed by subsequent limb lengthening, and (3) gradual transportation of the intercalary

Fig. 2 Intraoperative photograph after irrigation and debridement (I and D) demonstrating removed bone fragments with no soft tissue attachment

bone segment through the bone defect area or bone transport [1, 2, 5, 7–9]. Typically, circular external fixation in cases with rapid segmental shortening followed by limb lengthening consists of two stabilization modules attached to the proximal and distal segments, respectively, and interconnected by fracture reduction modules allowing for bone segment reposition, compression, and gradual distraction. In Ilizarov-type frames, those connection modules are represented by three–four telescopic rods with two universal hinges [3, 4] while in alternative hexapod-type external fixators, fracture reduction, compression, and subsequent gradual distraction are achieved with six struts [6, 10].

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Ten Year Old Male with Comminuted Distal Femoral Fracture

Images During Treatment See Figs. 2, 3, 4, 5, 6, 7, 8, 9, and 10.

Technical Pearls The TrueLok Trauma (TL-T) circular external fixation system for the management of severe comminuted femoral fractures consists of the proximal and distal external fixation modules. The proximal module is assembled from the proximal femoral arch and midshaft full ring or open medially 5/8 ring and attached to the proximal femur with three half pins. The distal external fixation module is represented by the Fig. 3 Intraoperative photograph of the femur after conversion of the long-leg splint immobilization into circular external fixation. The TrueLok Trauma spanning frame consisted of a proximal femoral arch 5/8 ring block attached to the proximal femur by three half pins and a distal ring secured to the distal femur by one lateral olive wire and two half pins and interconnected with the proximal external fixation module by four rapid adjust struts

Fig. 4 Intraoperative AP and LAT radiographs immediately after rapid shortening realignment of the bone segments and compression. Note limited contact surface area and significant anteromedial bone defect at the docking site

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distal femoral full ring or open posteriorly 5/8 ring secured to the distal femur with one olive wire and two half pins. Proximal and distal fixation modules are interconnected by four rapid adjust struts (RAS) providing superior stabilization of the bone segments and allowing for precise rapid fracture reduction and gradual compression as well as gradual distraction for limb lengthening. Initially, proximal and distal fixation modules are interconnected by two struts functioning as a temporary circular external fixator. After achieving the acceptable alignment, two more struts are attached to the system followed by gradual correction of any remaining malalignment if necessary and compression between the bone segments.

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Outcome Clinical Photos and Radiographs See Figs. 11, 12, and 13.

Avoiding and Managing Problems Delayed fracture healing is one of the most common problems in cases with open comminuted fractures. Despite known stimulation effects of distraction osteogenesis, limb lengthening in those patients can be postponed until successful soft tissue coverage, resolution of the infection, and realistic prediction of the final leg length discrepancy. To prevent those potential complications in our patient, for example, we elected to achieve soft tissue coverage and fracture healing first followed by LLD dynamics observation. Depending on growth plate involvement in the original injury and progression of the residual femoral shortening, several treatment options should be considered including simple LLD compensation by shoe insert or lift, opposite leg epiphysiodesis, and femoral lengthening at the appropriate level.

Fig. 5 AP radiograph of the femur 2 months after surgery showing actively mineralizing callus posteromedially with no positive dynamics at the anteromedial docking site bone defect requiring bone graft Fig. 6 Intraoperative photograph of the femur after secondary debridement of the nonviable tissues at the docking site demonstrating anteromedial partial bone defect filled in with the iliac crest bone-morselized autograft and allograft

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Ten Year Old Male with Comminuted Distal Femoral Fracture

Fig. 7 Postoperative AP radiograph of the femur illustrating maintained limb alignment and anteromedial partial bone defect filled in with the bone graft

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Fig. 8 AP and LAT radiographs 2.5 months after iliac crest bone grafting (4.5 months after external fixator application). Note perfect limb alignment in both coronal and sagittal planes and the abundance throughout the fracture site

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Fig. 9 Photograph of the lower extremities at 4.5 months of external fixation. Frame configuration and stability of bone segment fixation allowed the patient to stand and walk with full weight-bearing. The frame was removed at this time

Fig. 11 AP radiograph of the lower extremities 1 year after frame removal demonstrating remodeled fracture callus and proper alignment of the limb

Fig. 10 AP and LAT radiographs of the femur 2 weeks after frame removal illustrating maintained limb alignment and dimensions of the fracture callus

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Ten Year Old Male with Comminuted Distal Femoral Fracture

Fig. 12 LAT radiographs of the knee with maximum flexion and extension 1 year after frame removal

Fig. 13 Clinical appearance of the lower extremities 1 year after frame removal. Patient has no complaints and is happy with the appearance of his lower extremities. He has approximately 110 of the knee range of motion and 2 cm of LLD due to residual femoral shortening. The possible sequela of LLD is being followed

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Cross-References ▶ Comminuted Type I Open Distal Femur Fracture ▶ Open Femoral Fracture Treated with Internal Fixation Converted to Circular External Fixation due to Infection

See Also in Vol. 3 Pathologic Femur Fracture Close to Distal Femoral Physis

References and Suggested Reading 1. Blondel B, Launay F, Glard Y, Jacopin S, Jouve JL, Bollini G. Hexapodal external fixation in the management of children tibial fractures. J Pediatr Orthop B. 2010;19:487–91. 2. Eidelman M, Katzman A. Treatment of complex tibial fractures in children with the Taylor spatial frame. Orthopedics. 2008;31(10):pii. www.orthosupersite.com/view.asp?rID=31513 3. Ilizarov GA. Basic principles of transosseous compression and distraction osteosynthesis. Ortop Travmatol Protez. 1971;32:7–15.

K. Rathjen et al. 4. Ilizarov GA. Transosseous osteosynthesis: theoretical and clinical aspects of the regeneration and growth of tissues. New York: Springer; 1992. 5. Lerner A, Fodor L, Soudry M, Peled IJ, Here D, Ullmann A. Acute shortening: modular treatment modality for severe combined bone and soft tissue loss of the extremities. J Trauma. 2004;57:603–8. 6. Marangoz S, Feldman DS, Sala DA, Hyman JE, Vitale MG. Femoral deformity correction in children and young adults using Taylor Spatial Frame. Clin Orthop Relat Res. 2008;466(12):3018–24. 7. Nho SJ, Helfet DL, Rozbruch SR. Temporary intentional leg shortening and deformation to facilitate wound closure using the Ilizarov/ Taylor spatial frame. J Orthop Trauma. 2006;20:419–24. 8. Saleh M, Rees A. Bifocal surgery for deformity and bone loss after lower-limb fractures. Comparison of bone-transport and compression-distraction methods. J Bone Joint Surg B. 1995;77:429–34. 9. Sen C, Kocaoglu M, Eralp L, Gulsen M, Cinar M. Bifocal compression-distraction in the acute treatment of grade II open tibial fractures with bone and soft-tissue loss: a report of 24 cases. J Orthop Trauma. 2004;18:150–7. 10. Taylor JC. Tibial malunion. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: Informa Healthcare; 2007. p. 167–76.

Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique Wire Bone Transport

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Karl Rathjen, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84

Abstract

Three year old female sustained multiple fractures in a rollover motor vehicle collision including bilateral closed femoral shaft fracture, stabilized internally with Rush rods and grade IIIB open left tibial/fibular shaft fractures with segmental bone loss and substantial soft tissue defect fixed externally with the Agee WristJack monolateral fixator following to irrigation and debridement (I and D). Four days postoperative, soft tissue necrosis necessitated second I and D with rotational soleus flap and split-thickness skin graft. Following soft tissue coverage, bone stabilization was converted to TrueLok circular external fixator to allow for proximal tibial osteotomy and 4-cm obliquewire segmental bone transport.

Brief Clinical History The patient is a 3 year old female who was a passenger in a rollover motor vehicle collision and sustained multiple fractures including bilateral closed diaphyseal femoral fracture and Gustilo-Anderson grade IIIB open left distal tibial/fibular shaft fractures with segmental bone loss and large soft tissue defect (Fig. 1). She was treated initially with intramedullary Rush rod fixation of the femoral fractures and irrigation and debridement (I and D) of the open tibial fracture followed by staple wound closure and Agee WristJack monolateral external fixation (Fig. 2). Four days later, she developed a soft tissue necrosis, underwent second I and D with a rotational soleus flap and split-thickness skin graft (Fig. 3), and subsequently was transferred for treatment of a 4-cm segmental bone defect.

K. Rathjen (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_80

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• Potential for post-traumatic distal tibial growth disturbance (partial or complete physeal arrest) requiring repeated deformity correction and/or limb lengthening at skeletal maturity

Treatment Strategy Although acute tibial shortening until contact between the opposite ends of bone segments followed by osteotomy and limb lengthening is a predominant method of bone loss management, substantial (4 cm) bone defect with intact length of the fibula and presence of rotational soleus flap and split-thickness skin graft for large soft tissue defect coverage would be associated with a high risk of complications. Therefore, the classic Ilizarov bone transport method was selected for the definitive treatment of segmental bone defect. Treatment planning included: (1) soleus flap mobilization, debridement of segmental bone loss area, and resection of the tapered bone spikes; (2) conversion of the insufficient temporary monolateral fixation to more stable circular external fixation; (3) proximal tibial osteotomy followed by oblique-wire bone transportation; and (4) replacement of the oblique wires with transverse wires followed by debridement of the proximal and distal bone ends, acute correction of residual deformity, bone grafting, and docking site compression. Due to potential distal tibial growth plate injury, the long-term treatment planning considered periodic distal tibial deformity correction during the patient growth with the final residual deformity correction and limb lengthening at skeletal maturity. Fig. 1 Preoperative AP radiograph demonstrating bilateral displaced femoral shaft fracture and left tibial/fibular shaft fractures with segmental bone loss

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Preoperative Problem List • • • •

Segmental 4-cm tibial bone defect Stabilized fibular fracture with intact fibular length Insufficient fixation of bone segments Presence of rotational soleus flap and split-thickness skin graft • Very young-aged (3 year old) patient with unknown response to prolonged treatment

Basic Principles Three distinct strategies using the circular external fixation are utilized in the management of the severe open tibial fractures in children [1, 2, 5–8]. Fractures with minimal bone loss can be stabilized with compression to allow for soft tissue coverage and fracture healing. If soft tissue would allow, the tibia can be shortened acutely to achieve bone end apposition with subsequent limb lengthening at a site remote from the fracture (bone defect). Finally, in patients whose soft tissues would not allow acute shortening to achieve apposition, tibial fracture can be stabilized with or without minimal shortening for soft tissue coverage followed by bone transport. According to Ilizarov [3, 4], bone transport technique includes osteotomy forming a free intercalary segment of bone (transport segment) from one of the residual bone segments (host segment) followed by its gradual movement toward the opposite residual bone segment (target segment)

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Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique. . .

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Fig. 2 AP and LAT radiographs demonstrating intramedullary Rush rod fixation of both femoral fractures (a) and temporary stabilization of the open tibial fracture using Agee WristJack monolateral external fixator (b)

Fig. 3 Clinical photographs of the tibia at the time of rotational soleus flap (a) and split-thickness skin graft coverage of soft tissue defect (b)

across the bone defect. Once the transport segment reaches the residual target segment, compressive forces are applied at the docking site until the solid fusion between the transport and target segments. Basic frame configuration for Ilizarov bone transport includes proximal and distal circular external supports (rings or double-ring blocks) for proximal and distal residual bone fixation, respectively, interconnected by four long threaded rods. Intercalary transport segment is stabilized with two oblique olive wires attached to threaded bone transport modules connected to one of the external supports. In

addition, one floating ring is inserted between the proximal and distal external supports for securing the transverse wires that will be inserted into the transport bone segment after its docking with the residual target segment for compression.

Images During Treatment See Figs. 4, 5, 6, 7, 8, 9, 10, 11, 12, and 13.

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Fig. 4 Intraoperative AP and LAT radiographs after frame application and proximal tibial osteotomy for bone transport. Note that the TrueLok circular external fixator consisted of the proximal ring and distal doublering block attached to the related bone fragments by 2 crossing wires with 1 half pins and 3 crossing wires, respectively, and interconnected by 4 threaded rods. Note 2: oblique olive wires inserted into the intercalary fragment and “floating” middle ring, which will be used for docking between the transport and target bone fragments later

Fig. 5 Intraoperative (a) and postoperative (b) photographs showing the initial and final position of oblique wires and soft tissue coverage in the beginning and at the completion of bone transport

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Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique. . .

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Fig. 6 AP and LAT radiographs at the end of bone transport. Note the perfect axial alignment of the residual host and transport and residual target bone segments. At that time, the patient was returned to the OR for replacement of the oblique wires with transverse wires followed by debridement of the bone ends, local bone grafting, and docking site compression

Technical Pearls

Outcome Clinical Photos and Radiographs

The main advantages of oblique wires when compared to transverse crossing wires are the absence of long, longitudinal, soft tissue cutting scars and better preservation of the flap during bone transport. On the other hand, oblique wires require more attention during their insertion and careful manipulation with the transported bone segment. In addition to insertion of those oblique wires through both bone cortices at the same level and at the same angle relative to the limb axis, we are cutting the ends of the wires behind the olive stoppers (see Fig. 4), thereby further avoiding the longitudinal scar formation. When the transported bone segment reaches the residual target bone segment, both oblique wires are removed through two small incisions by pushing them back toward the olive side with slight rotation. Although not employed in this case, small washers can be placed between the olive stoppers and contacting bone surface to increase the stability of oblique-wire fixation in cases with severe osteopenia of the transported bone.

See Figs. 14, 15, and 16.

Avoiding and Managing Problems Delayed docking site consolidation is one of the most common complications of bone transport. Therefore, serious consideration should be given at the time of surgical transition from the gradual transportation of the intercalary bone segment through the defect area to the acute compression between the transport and residual target segments at the docking site. To achieve successful docking site healing, our bone transport protocol for all patients includes secondary debridement of contacting bone segment surfaces with placement of iliac crest bone graft prior to acute docking site compression. During debridement, all fibrous tissues surrounding the transport and target bone segments should be removed followed by the adaptation of opposing bone

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Fig. 7 AP and LAT radiographs during the consolidation period. Note the perfect final alignment, active distraction regenerate remodeling, and progressive consolidation at the docking site. The total time in the circular fixator was 80 days and comprised of a 6-day latency period, 29 days of bone transportation, and 45 days for regenerate consolidation and docking site union

surfaces to achieve the maximum contact area between the ends of both those segments. All slender fragments of retained bone that may compromise the contact between the transport and residual target bone fragments should be resected also. Next, iliac crest bone graft is harvested and placed between the contacting bone surfaces and around the docking site, followed by adequate acute (approximately 5–10 mm) docking site compression. Based on the shape of

resultant bone segments after contacting surface adaptation, either longitudinal or side-to-side horizontal (or both) compression should be utilized. The second is usually applied for oblique or steplike contacting bone surfaces using opposing olive-olive stopper wires. Moreover, docking site compression should be checked regularly and maintained by gradual or periodical additional acute compression (about 1–2 mm every 2 weeks).

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Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique. . .

Fig. 8 AP and LAT radiographs 2 years after frame removal illustrating distal tibial procurvatum due to post-traumatic physeal growth disturbance requiring several subsequent stages of treatment during the patient growth including physeal bar excision, three repeated corrective distal tibial osteotomies with internal fixation, and distal tibial and proximal fibular epiphysiodesis

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Fig. 9 AP and LAT radiographs 8 years after frame removal before the final stage of treatment demonstrating distal tibial varus-recurvatum deformity and 5 cm of residual LLD. Final treatment included application of the circular external fixator followed by distal tibial osteotomy with hinge-guided acute deformity correction and proximal tibial/distal fibular osteotomies for limb lengthening

Fig. 10 Intraoperative LAT radiograph showing the location of the hinge axis relative to the apex of the deformity in the sagittal plane for hinge-guided acute deformity correction

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Fig. 11 Postoperative AP and LAT radiographs after distal tibial osteotomy followed by acute deformity correction and proximal tibial/distal fibular osteotomies for limb lengthening

Fig. 12 AP and LAT radiographs during the consolidation period. Note the final alignment of bone segments and active distraction regenerate remodeling

Fig. 13 AP and LAT radiographs 6 months after frame removal. Note the perfect bone segments alignment, complete remodeling of distraction regenerate with corticalization, and progressive callus mineralization distally

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Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique. . .

Fig. 14 AP standing radiograph 3 years after final frame removal (11 years after bone transport) illustrating final limb alignment

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Fig. 15 Clinical appearance of lower extremities after treatment. Patient has no residual LLD and ambulates with a fairly coordinated gait without pain

See Also in Vol. 2 Distal Tibial Bone Defect Treated with Bone Transport Using Two Proximal Osteotomy Sites GIII B Pilon Fracture with Segmental Bone Loss

References and Suggested Reading

Fig. 16 Side-view photographs after treatment illustrating a preserved knee and ankle range of motion

Cross-References ▶ Ten Year Old Male with Comminuted Distal Femoral Fracture

1. Blondel B, Launay F, Glard Y, Jacopin S, Jouve JL, Bollini G. Hexapodal external fixation in the management of children tibial fractures. J Pediatr Orthop B. 2010;19:487–91. 2. Eidelman M, Katzman A. Treatment of complex tibial fractures in children with the Taylor spatial frame. Orthopedics. 2008;31(10). pii: orthosupersite.com/view.asp?rID=31513 3. Ilizarov GA. Basic principles of transosseous compression and distraction osteosynthesis. Ortop Traumatol Protez. 1971;32:7–15. 4. Ilizarov GA. Transosseous osteosynthesis: theoretical and clinical aspects of the regeneration and growth of tissues. New York: Springer; 1992. 5. Lerner A, Fodor L, Soudry M, Peled IJ, Here D, Ullmann A. Acute shortening: modular treatment modality for severe combined bone and soft tissue loss of the extremities. J Trauma. 2004;57:603–8. 6. Nho SJ, Helfet DL, Rozbruch SR. Temporary intentional leg shortening and deformation to facilitate wound closure using the Ilizarov/ Taylor spatial frame. J Orthop Trauma. 2006;20:419–24. 7. Saleh M, Rees A. Bifocal surgery for deformity and bone loss after lower-limb fractures. Comparison of bone-transport and compression-distraction methods. J Bone Joint Surg (Br). 1995;77:429–34. 8. Sen C, Kocaoglu M, Eralp L, Gulsen M, Cinar M. Bifocal compression-distraction in the acute treatment of grade II open tibial fractures with bone and soft-tissue loss: a report of 24 cases. J Orthop Trauma. 2004;18:150–7.

Traumatic Physeal Arrest of the Proximal Tibia with Deformity in All 4 Dimensions (Length, Coronal Angulation, Sagittal Angulation, and Rotation)

13

Christopher Iobst

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Image During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88

Abstract

A 13 year old male suffers a fracture to the left proximal tibia involving the physis. He develops a four-dimensional deformity (short, external rotation, valgus, and recurvatum) over time. Simultaneous correction of all four planes of deformity was achieved using the Taylor Spatial FrameTM.

demonstrates 8 of valgus, 2 cm of shortening, 10 of asymmetric external rotation compared to the opposite side, and 25 of recurvatum (apex posterior). See Fig. 2a, b.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Brief Clinical History A 13 year old male sustains a displaced left proximal tibial Salter-Harris II fracture (Fig. 1a, b). The fracture was immediately fixed with two smooth wires and healed uneventfully. Over the next 3 years, however, the patient slowly developed a noticeable deformity of the left leg. A partial proximal tibial physeal arrest was noted. Evaluation of the limb at that time C. Iobst (*) Department of Orthopedic Surgery, Nemours Children’s Hospital, Orlando, FL, USA e-mail: [email protected]

Preoperative Problem List 1. Approaching skeletal maturity – guided growth not an option 2. Near-complete arrest of the left proximal tibial physis – resection of the physeal bridge not possible 3. Leg length discrepancy 4. External rotation deformity 5. Genu valgum 6. Proximal tibial slope 25 in the wrong direction

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_96

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Fig. 1 (a, b) AP and lateral radiographs of the left tibia demonstrate a displaced SalterHarris II fracture of the proximal tibia in a 13 year old male

Fig. 2 (a–c) Standing full-length lower extremity radiograph demonstrates the valgus deformity with lateral deviation of the mechanical axis and a 2 cm leg length discrepancy. The AP radiograph highlights the

valgus deformity that has developed in the proximal tibia. The lateral radiograph of the tibia demonstrates a posterior proximal tibia angle of 105

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Traumatic Physeal Arrest of the Proximal Tibia with Deformity in All 4 Dimensions. . .

Treatment Strategy In a multi-planar deformity such as this one, the Taylor Spatial FrameTM is an excellent management option because it allows simultaneous gradual correction of each plane of deformity without having to make additional trips to the operating room. Other than one planned strut change, no further alteration of the initial frame was necessary to achieve the complete correction. After comprehensive deformity analysis is completed, the deformity parameters are placed in the Taylor Spatial Frame software program. The origin is chosen at the level of the proximal tibial physis. Multi-planar fixation spread over the length of each segment is carefully placed with a minimum of three fixation points to each segment. The deformities can be corrected sequentially (one at a time) or simultaneously. In this case, one half of the expected length was corrected simultaneously with the rotation and angular deformities. Once the limb was completely realigned, the final amount of necessary lengthening was performed.

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Fig. 3 Standing full-length film demonstrates correction of alignment and length. Note the distal tibial segment is fixed with a two-ring block for added stability

Basic Principles The surgeon should be cognizant of the peroneal nerve in tibial valgus to varus corrections, especially if there is a concomitant rotational correction needed. The peroneal nerve should be decompressed at the time of surgery if any portion of the deformity is going to be corrected acutely. If the entire deformity correction is going to be done gradually, then the nerve decompression is not mandatory but peroneal nerve function should be monitored. If nerve symptoms arise during the correction, then it should be decompressed as soon as possible.

Image During Treatment

Therefore, do not put the entire amount of desired lengthening in the initial “define deformity” page of the Taylor Spatial FrameTM software program. Once the leg is straightened, the surgeon has a more accurate impression of the remaining length needed to balance the leg lengths. The remainder of the correction can then be performed as a straight lengthening.

See Fig. 3.

Outcome Clinical Photos and Radiographs Technical Pearls 1. The frame is constructed to limit the number of anticipated strut changes. A rough draft of the software program can be run before surgery to anticipate the direction and amount of excursion necessary for each strut. Then, after applying the frame in the operating room, the appropriate sized struts can be added to the frame that will allow the correction to proceed with the minimum number of strut changes. 2. It is often difficult to anticipate the amount of length gained by straightening a multi-planar deformity.

See Fig. 4.

Avoiding and Managing Problems 1. Decompress the peroneal nerve if there is concern about correcting the valgus deformity of the tibia. 2. Place the origin in the center of the bone in both the AP and sagittal planes when performing gradual rotational correction. This will prevent unwanted secondary translation of the tibia from occurring.

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Fig. 4 (a, b) Final AP and lateral radiographs demonstrate healing of the regenerate bone at the osteotomy site with correction of the tibial slope and the leg length discrepancy. Note the translation of the distal tibia necessary to maintain the mechanical axis because the osteotomy is not at the same level as the CORA (the physis)

3. Dynamize the frame by gradually removing points of fixation to train the bone to accept weight. The patient should be comfortably fully weight bearing in the frame before it is removed.

Cross-References ▶ TSF for Displaced Pediatric Tibia Fractures

References and Suggested Reading 1. Herzenberg JE, et al. The art of limb alignment. Baltimore: Rubin Institute for Advanced Orthopedics; 2013. 2. Paley D. Deformity correction techniques. Oper. Tech Orthop. 2011;21(2):103–202.

TSF for Displaced Pediatric Tibia Fractures

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Christopher Iobst

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 91 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93

Abstract

Acute pediatric deformity (trauma) can be corrected successfully using a Taylor Spatial Frame (TSF). Common indications include open fractures and delayed fracture management. This case illustrates the ability to use the frame to stabilize a displaced tibia fracture with significant soft tissue swelling and prevent a potential compartment syndrome.

Brief Clinical History A 14 year old female presents with a closed right diaphyseal tibia and fibula fracture after colliding with another player during a soccer game. The right leg is swollen and has an obvious deformity. She has an intact neurovascular exam in

her right lower extremity. She did not have any other injuries. She had appropriate pain with passive range of motion of her right toes. Due to the amount of swelling, it was decided that closed reduction and casting would not be the safest initial treatment method for her injury. Therefore, external fixation of the injury with the Taylor Spatial FrameTM (Memphis, TN) was chosen as the primary treatment. Using this external fixator, the fracture could be left slightly unreduced to allow her swollen anterior and lateral compartments to stay relaxed. The fracture would then be reduced fully in a few days when her swelling had subsided and the risk of compartment syndrome had passed.

Preoperative Clinical Photos and Radiographs See Fig. 1.

C. Iobst (*) Department of Orthopedic Surgery, Nemours Children’s Hospital, Orlando, FL, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_94

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Fig. 1 (a) Anteroposterior and (b) lateral radiographs of the right tibia demonstrating a displaced diaphyseal right tibia and fibula fracture in a 14 year old female. She has been immobilized in situ in a posterior splint for comfort

Preoperative Problem List 1. Acute, traumatic deformity of the tibia 2. Swelling of the right leg with concern for developing compartment syndrome

adjustment schedule to begin the gradual correction of the fracture 1 week later. Once anatomic reduction was achieved, the fixator was locked in place with a threaded rod and full weight bearing was started. The frame was then gradually dynamized by removing fixation points as the fracture healed. The frame was removed when the patient demonstrated she could walk comfortably, fully weight bearing with all struts loose, and there was sufficient callus on the radiographs.

Treatment Strategy A circular external fixator (Taylor Spatial Frame) was applied with each ring mounted orthogonal to its respective fracture fragment. Three points of fixation were used on each fragment with the pins spread in multiple planes over the length of the fragment. The rings were mounted at a measured distance from the fracture to allow the frame to be affixed at the neutral strut length. For medium fast Fx struts, this distance between the ring and the fracture is approximately 8.5 cm on each fragment. This allows the struts to have enough excursions to permit the necessary movements to achieve reduction of the fracture without binding. In this case, the fracture was purposefully only partially reduced to keep from overstretching the anterior and lateral compartments of the leg. The origin was placed at the tip of the fracture on the reference fragment (proximal tibia), and the corresponding point was chosen as the coincident point of the fracture on the distal fragment. The patient was given a strut

Basic Principles 1. Place three points of fixation on each fragment. The fixation should be spread over the length of the fragment and placed in multiple planes. 2. The rings should be centered on the limb to allow one to two fingerbreadths of clearance circumferentially. This is important in a fracture that may have significant swelling. 3. Predrill each half pin with a sharp drill bit. Do not heat the bone while drilling. Pause frequently while drilling and remove the drill to clean the flutes if necessary. Make sure each drill path is bi-cortical. 4. Mount each ring orthogonal to its respective fracture fragment in both the coronal and sagittal planes. 5. Dynamize the frame during the course of treatment to train the bone to accept increasing loads.

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TSF for Displaced Pediatric Tibia Fractures

Images During Treatment See Figs. 2, 3, and 4.

Technical Pearls 1. The surgeon can lay a rancho cube directly on the anterior tibial diaphysis to guide the orthogonal placement of the first half pin for each fracture fragment. 2. Fast Fx struts are helpful to achieve manual reduction of the fracture. However, once the fracture has satisfactory alignment, consider adding a threaded rod or exchanging the fast Fx struts for standard struts to prevent accidental collapse of the construct. If one locking sleeve of the fast Fx struts becomes dislodged by the patient (the posterior struts are at risk when rolling around in the bed), the entire reduction will be lost. Make sure to check that all of the sleeves of the fast Fx struts are completely pushed up in the locked position. 3. Be sure to slide the proximal ring onto the limb before adding any fixation. If you place the half pins first, they will prevent the proximal ring from being able to slide up to the correct level on the limb. 4. Leaving the fracture in a purposely deformed position with delayed gradual reduction is possible with the Taylor

Fig. 2 (a, b) The immediate postoperative radiographs reveal the fracture has only been reduced partially. The fracture was left in slight valgus to relax the anterior and lateral compartments of the leg. Note the threaded rod has been added to prevent loss of stability in case one of the struts gets accidentally released

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Spatial Frame. This allows injuries to the soft tissue envelope such as open wounds or swelling to be treated more effectively. The bone can then be gradually realigned when it is safe to manipulate the soft tissues. 5. Once the fracture healing is underway, the frame should be continuously dynamized to gradually transfer more load from the frame to the bone. Half pins can be detached from the frame without having to be removed from the patient. Start by releasing the central of the three half pins on each side of the fracture. Do not dynamize the Taylor Spatial Frame by releasing one strut. This will produce shear at the fracture site. Releasing all six struts at the appropriate time is a more effective method of dynamization than releasing any combination of struts.

Outcome Clinical Photos and Radiographs See Figs. 5 and 6.

Avoiding and Managing Problems 1. Placing a threaded rod (or two) between the rings after the reduction is complete will provide additional stability when using a frame construct with Fast Fx struts. The

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Fig. 3 (a, b) After 1 week, the swelling has subsided and the fracture was then gradually reduced using the Taylor Spatial Frame strut adjustment prescription generated by the computer software. There is now anatomic alignment of the fracture in both planes

Fig. 4 (a, b) After 3 months, the fracture is sufficiently healed to allow frame removal

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Fig. 6 (a, b) Final radiographs demonstrate excellent healing of the fracture and restored alignment in both the coronal and sagittal planes Fig. 5 (a, b) The frame was removed and the patient was placed in a walking cast for 3 weeks

struts can occasionally become unlocked in overactive patients and the threaded rods prevent catastrophic collapse of the frame. Once early callus is noted, the threaded rods can be removed and the dynamization process can be started. 2. Pin sites closer to the joints (metaphysis) above and below the fracture site tend to have more excursion of the skin with movement. Stabilizing the skin with a dressing or sponge around these pin sites will help to minimize irritation and potential pin track infection.

Cross-References ▶ Adolescent Unstable Tibial Fracture Treated Initially with Closed Reduction and Plaster Cast Immobilization Converted to TL-Hex External Fixation due to Residual Displacement

▶ Traumatic Physeal Arrest of the Proximal Tibia with Deformity in All 4 Dimensions (Length, Coronal Angulation, Sagittal Angulation, and Rotation)

References and Suggested Reading 1. Al-Sayyad MJ. Taylor Spatial Frame in the treatment of pediatric and adolescent tibial shaft fractures. J Pediatr Orthop. 2006;26(2):164–70. 2. Lahoti O, Findlay I, Shetty S, Abhishetty N. Intentional deformation and closure of soft tissue defect in open tibial fractures with a Taylor spatial frame – a simple technique. J Orthop Trauma. 2013;27 (8):451–6. 3. Nho SJ, Helfet DL, Rozbruch SR. Temporary intentional leg shortening and deformation to facilitate wound closure using the Ilizarov/ Taylor spatial frame. J Orthop Trauma. 2006;20(6):419–24.

Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral Lengthening with FITBONE Retrograde Intramedullary Nail

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John Birch, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Preoperative Problems List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 102

Abstract


Sixteen year old male with 11 distal femoral valgus and 4.5 cm of leg length discrepancy secondary to posttraumatic physeal growth arrest underwent distal femoral osteotomy followed by acute angular deformity correction and gradual femoral lengthening using FITBONETM TAA retrograde intramedullary nail.

Brief Clinical History The patient is a 16 year old male presented with an intermittent pain and clicking sensation in the right knee, moderate genu valgum, and LLD secondary to post-traumatic physeal growth arrest. He had suffered a closed distal femoral physeal

fracture at the age of 11, treated by closed reduction and percutaneous pin fixation. A progressive deformity and LLD were noted since injury. He has also had a lateral meniscus repair performed arthroscopically 4 years after initial injury. The patient was otherwise healthy. All of his lower extremity joints were stable with a full range of motion. He was neurovascularly intact but had right knee joint line tenderness and crepitus with motion. Radiographic evaluation revealed distal femoral valgus 11
and 4.5 cm of right femoral shortening (Figs. 1 and 2).

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

J. Birch (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_87

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Fig. 2 Front view photograph showing clinical appearance of lower extremities before the treatment

• LLD 4.5 cm in skeletally mature patient • Mild (11
) valgus deformity of the distal femur • S/p arthroscopic repair of the lateral meniscus tear

femoral shortening with internal fixation, gradual angular correction and femoral lengthening using circular external fixation, and acute angular deformity correction and femoral lengthening using intramedullary lengthening nail. The patient and his family opted for intramedullary lengthening. Because of the complete closure of the distal femoral physis, the motorized FITBONETM TAA retrograde intramedullary nail was selected for femoral lengthening. A preoperative planning of distal femoral osteotomy, acute valgus deformity correction, intramedullary nail orientation, and subsequent femoral lengthening was done (Fig. 3) using the Baumgart reverse planning method [1]. The postoperative regimen included guided physical therapy concentrating on joint motion, maintenance of muscle strength, and compliance with a partial weight-bearing (20 kg).

Treatment Strategy

Basic Principles

Several treatment options are available for correction of femoral deformity and leg length inequality in skeletally mature patients including acute angular deformity correction and contralateral

FITBONETM TAA intramedullary lengthening nail (Fig. 4) contains an electric motor incorporated within the telescopic body [2–4]. The motor is activated by radiofrequency wave

Fig. 1 Standing AP radiograph of lower extremities demonstrating right distal femoral valgus (LDFA ¼ 76
, MPTA ¼ 87
, JLCA ¼ 2
varus) and leg length inequality (4.5 cm)

Preoperative Problems List

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Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral. . .

Fig. 3 Diagram illustrating the “reverse planning method” for correction of distal femoral valgus deformity: (a) initial outline of the femur (black) after distal femoral osteotomy; (b) the desired location of the proximal segment (red) at the end of lengthening; (c) translation of the

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proximal segment (green) along the axis of the nail into position at the beginning of lengthening; (d) position of the proximal segment and the nail at the end of surgery. Note correction of valgus deformity with translation of opposed surfaces of bone segments

Fig. 4 FITBONETM TAA device for intramedullary lengthening

pulses transmitted to subcutaneously implanted receiver (antenna) that converse those pulses into electric signals. This version of nail is available with an outer-rod maximum diameter of 12 mm and stroke of 40–80 mm. During distraction period, the patient activates the device three times daily by placing the transmitter over the receiver. Monitoring of device activation is accomplished by listening of the “whir” of the motor using a stethoscope placed over the knee. The nail cannot be reversed for compression, and periodic clinical and radiographic assessment is essential, with the distraction rate adjusted appropriately (by the number of activations). The FITBONETM device is not approved by FDA and can be applied in the USA only on an individual, compassionate-use basis.

Images During Treatment See Figs. 3, 4, 5, 6, 7, 8, 9, 10, and 11.

Technical Pearls Combination of acute deformity correction of the preexisting distal femoral deformity and gradual lengthening along the anatomical axis of the femur requires careful preoperative planning of bone fragment orientation as well as accurate execution of the planned position of bone fragments intraoperatively. A radiopaque “grid” of horizontal and vertical lines placed on the operative table under the patient allows accurate intraoperative assessment of limb alignment relative to its mechanical axis during surgery. Distraction during intramedullary lengthening with FITBONETM nail occurs three times daily by the radiofrequency wave activation of the electric motor. The patient must carefully listen with a stethoscope and count the number of “whirs” of the motor during device activations. Typically, motor is activated nine times per each distraction session (27 activations daily) producing approximately 1 mm of distraction per day. Therefore, if the patient does not hear the motor activate, the patient should reactivate the transmitter and keep it

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Fig. 5 Intraoperative photograph showing femoral medullary canal reaming using straight reamers

Fig. 6 Intraoperative photograph demonstrating insertion of the FITBONETM TAA intramedullary lengthening nail

over the antenna until nine activations are heard; the patient then removes the transmitter from the vicinity of the antenna to avoid overlengthening.

Outcome Clinical Photos and Radiographs See Figs. 12, 13, 14, and 15.

Avoiding and Managing Problems The most important component of successful intramedullary femoral lengthening is adequate correction of distal femoral valgus/varus as well as other associated deformities. Using the reverse planning method and careful critical analysis of

intraoperative bone fragment orientation allow to avoid inadequate correction of the initial deformity or prevent development of genu valgum at the completion of lengthening in most of the cases. In some patients, a separate stage of deformity correction is required in preparation to intramedullary lengthening. In addition, the surgeon should carefully access and monitor bone fragment stability during the lengthening. Although not used in this case, placement of “block screws” in consideration of deforming forces (varus and procurvatum) will enhance nail fixation in the medullary canal thereby preventing fragment migration during femoral lengthening. Finally, patients with a distal femoral physeal fractures have a very high (>30 %) incidence of subsequent growth disturbance, irrespective of Salter-Harris fracture pattern. Therefore, all patients with these injuries must be carefully followed to skeletal maturity.

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Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral. . .

Fig. 7 Composite intraoperative fluoroscopic views on a radiopaque grid, allowing the surgeon to confirm adequate angular deformity correction and fragment translation: (a) before acute deformity correction; (b) after correction of genu valgum

Fig. 8 Intraoperative photograph showing testing of proper nail function by activation using the transmitter placed over the subcutaneous receiver. Note stethoscope placed over the knee to monitor device activation

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Fig. 10 AP and LAT radiographs of the femur at the completion of lengthening Fig. 9 Postoperative AP and LAT radiographs of the femur demonstrating nail orientation and position of bone segments after acute deformity correction

Fig. 11 Side view photographs of the lower extremity illustrating knee range of motion at the completion of lengthening

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Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral. . .

Fig. 12 Standing AP radiograph of the lower extremities during consolidation demonstrating active remodeling of the distraction regenerate with corticalization

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Fig. 13 Front view photograph showing clinical appearance of lower extremities during consolidation

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Fig. 15 LAT radiograph of the femur demonstrating knee flexion

See Also in Vol. 3 Femoral Stump (Residual Limb) Lengthening with a Motorized Intramedullary Lengthening Rod

Fig. 14 LAT radiograph of the femur during consolidation

Cross-References ▶ Femoral Shortening (14 cm) and Deformity Treated with Acute Correction and Two Consecutive Retrograde Fitbone Applications

References and Suggested Reading 1. Baumgart R. The reverse planning method for lengthening of the lower limb using a straight intramedullary nail with or without deformity correction: a new method. Oper Orthop Traumatol. 2009;21:221–33. 2. Baumgart R, Bürklein D, Hinterwimmer S, Thaller P, Mutschler W. The management of leg-length discrepancy in Ollier’s disease with a fully implantable lengthening nail. J Bone Joint Surg (Br). 2005;87:1000–4. 3. Krieg AH, Speth BM, Foster BK. Leg lengthening with a motorized nail in adolescents: an alternative to external fixators? Clin Orthop Relat Res. 2008;466:189–97. 4. Krieg AH, Lenze U, Speth BM, Haster CC. Intramedullary leg lengthening with a motorized nail: indications, challenges, and outcome in 32 patients. Acta Orthop. 2011;82(3):344–50.

Valgus/Flexion and Shortening of the Distal Femur from Growth Arrest Treated with a Monolateral Frame

16

S. Robert Rozbruch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 108 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110

Abstract

This is a case of traumatic growth arrest of the distal femur that caused LLD of 3.6 cm and valgus/flexion deformity. Treatment consisted of percutaneous osteotomy of the distal femur, acute correction, and gradual lengthening using a monolateral rail frame. The patient went on to play and excel at competitive soccer.

deformity in her right leg. She had played soccer for her school but was having difficulty playing at a high level because of her symptoms.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Brief Clinical History The patient is a 15 year old girl who sustained a fracture of the distal femur as an adolescent. This was treated at another medical center with a cast. This resulted in deformity and shortening that worsened over the last 3 years. Her symptoms included knee and low back pain. She was unhappy with the

Preoperative Problem List 1. 2. 3. 4. 5.

LLD 3.6 cm. Valgus deformity 10 . Flexion deformity 8 . Post-traumatic growth arrest. Patient is an athlete.

S. R. Rozbruch (*) Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_56

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Fig. 1 (a) Fifty-one inch standing X-ray showing lateral MAD, valgus deformity and LLD of 3.6 cm. (b) The LDFA is 77 (normal is 85–90). The valgus deformity is in the femur. (c) Preoperative planning. Extend distal mechanical axis line (black line) from the ankle through the center of the knee. Draw anatomical axis line of the proximal femur and then a parallel line starting from the center of the femoral head (orange lines). Draw 7 line off the orange line (blue line). This represents the proximal mechanical axis. The intersection between the blue and black lines is the CORA, and the magnitude of the valgus deformity is 10 . (d) Schematic drawing reviewing the steps of deformity planning [4]

Treatment Strategy

Basic Principles

Apply monolateral fixator pins to match the valgus and flexion deformity using the variable angle distal pin clamp. Then, perform percutaneous osteotomy and acutely correct the valgus and flexion deformities (Fig. 4). Apply the neutral monolateral frame. Start distraction on postoperative day 7 at rate of 1 mm per day.

1. Applying the fixator in line with the mechanical axis of the femur is most correct for lengthening. However, the frame will fit the leg better if frame is applied in line with the anatomical axis of the femur, and this works well in practice.

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Valgus/Flexion and Shortening of the Distal Femur from Growth Arrest Treated with a Monolateral Frame

Fig. 2 Front (a), back (b), and side (c) views showing LLD 3.6 cm, valgus deformity, and apparent lack of full knee extension

Fig. 3 (a) AP X-ray of both knees. The right distal femur is short and in valgus. Distal growth plate is closed. (b) Lateral X-ray showing flexion deformity. The growth plate is fully fused posteriorly but still somewhat open anteriorly. The growth arrest was more advanced in the posterolateral quadrant accounting for the valgus, flexion, and shortening deformity

105

106 Fig. 4 Intraoperative (a) AP and (b) lateral images after acute correction of the valgus and flexion deformity. Note the distal fragment is in varus and extension relative to the proximal fragment

Fig. 5 Radiographs during consolidation: (a) AP view shows some deficient healing of the lateral cortex and some heterotopic bone prominent laterally and (b) lateral view showing extension of the distal fragment

S. R. Rozbruch

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Valgus/Flexion and Shortening of the Distal Femur from Growth Arrest Treated with a Monolateral Frame

2. Increase latency phase after acute correction. The acute correction disrupts the vascularity of the bone at the osteotomy site to a greater degree than leaving the osteotomy non-displaced. 3. The axis of rotation of the distal angulator clamp should be placed at same level as the CORA (or as close as possible).

Images During Treatment

2. Use three pins in both the proximal and distal clamps when lengthening. 3. Insert the distal three pins to match the flexion deformity of the distal segment. Pin clamp is removed from the rail during this application. 4. After percutaneous osteotomy, use the pins as joysticks and acutely correct the biplanar deformity. The pins will all be parallel and collinear. 5. Apply a neutral frame to the pins. Straight lengthening then follows. Residual deformity correction can be done with the distal angulator/translation clamp as needed.

See Figs. 4, 5, and 6.

Outcome Clinical Photos and Radiographs Technical Pearls See Figs. 7, 8, and 9. 1. Apply the fixator to match the valgus deformity by dialing in 10 of valgus into the distal angulator clamp. Fig. 6 (a) Front view during the consolidation phase showing equalization of leg lengths and correction of deformity. (b) Fiftyone inch standing X-ray showing equal leg lengths, deformity correction, and symmetrical MAD measurements

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Fig. 7 Front (a), back (b), and side (c) views at age 17 showing equal leg lengths, correction of valgus, and flexion deformities

Avoiding and Managing Problems 1. Make sure the knee flexes fully before leaving the operating room by flexing the knee after the frame is on. This will loosen the iliotibial band around the pin sites. 2. If gradual correction of varus or valgus is needed, length will be affected since the hinge is several centimeters

lateral to the bone. With correction of varus, there will be shortening (closing wedge effect). With correction of valgus, there will be lengthening (opening wedge effect). 3. Most distal pin should be placed just proximal to the flare of the condyle. Avoid intra-articular pin placement to avoid risk if joint sepsis.

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Valgus/Flexion and Shortening of the Distal Femur from Growth Arrest Treated with a Monolateral Frame

Fig. 8 (a) AP and (b) lateral X-rays showing bony union and correction of valgus and flexion deformities. Note the absence of the heterotopic bone which was removed and distal pin sites were excised. The patient had developed hypertrophic granulation tissue at the distal pin sites

Fig. 9 One year after removal of the fixator, the patient excelled at soccer in her senior year of high school and was recruited to play soccer in college

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Cross-References ▶ Valgus Deformity of the Distal Femur and LLD Secondary to Posttraumatic Physeal Arrest: Femoral Lengthening with FITBONE Retrograde Intramedullary Nail ▶ Varus Deformity of the Distal Femur and LLD Secondary to Ollier’s Disease Corrected by Gradual Deformity Correction and Lengthening

References and Suggested Reading 1. Fabricant PD, Camara JM, Rozbruch SR. Femoral deformity planning: intentional placement of the apex of deformity. Orthopedics. 2013;36(5):e533–7.

S. R. Rozbruch 2. Goldman V, McCoy TH, Harbison M, Fragomen AT, Rozbruch SR. Limb lengthening in children with Russell-Silver syndrome: a comparison to other etiologies. J Child Orthop. 2013;7:151–6. Epub 2013 Jan 5 3. Palatnik Y, Rozbruch SR. Femoral reconstruction using external fixation. Adv Orthop. 2011; https://doi.org/10.4061/2011/967186. Article ID 967186, 10 pp. http://www.sage-hindawi.com/journals/ aop/2011/967186/ 4. Paley D. Principles of deformity correction. Berlin: Springer; 2002. 5. Seah KT, Shafi R, Fragomen AT, Rozbruch SR. Distal femoral osteotomy: is internal fixation better than external? Clin Orthop Relat Res. 2011;469:2003–11.

Vitamin D-Resistant Hypophosphatemic Rickets Treated by Double-Level Femoral Osteotomy with Internal Fixation and Proximal Tibial Osteotomy with Gradual Deformity Correction

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John Birch, Alexander Cherkashin, Marina Makarov, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 112 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 112 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 112 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121

Abstract

Fourteen year old female with left leg varus (femur and tibia) associated with significant tibial torsion and 2 cm Leg Length Discrepancy (LLD) secondary to vitamin D-resistant rickets previously treated with bilateral 8-plate growth modulation and double-level femoral closing wedge osteotomy with retrograde intramedullary nail fixation. The patient underwent proximal tibial and midshaft fibular osteotomies followed by gradual varus deformity correction and acute tibial derotation via circular external fixation. J. Birch (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] M. Makarov Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]

Brief Clinical History The patient is a skeletally mature 14 year old female with a known personal and family history of vitamin D-resistant (hypophosphatemic) rickets. She had been treated medically since infancy. She had previously undergone attempted correction of a windswept lower extremity deformity (right genu valgum and left genu varum) by insertion of growth modulation 8-plates. These had adequately corrected the right leg deformity, but the left genu varum persisted (Figs. 1, 2, and 3). Subsequently, she had undergone double-level left femoral closing wedge osteotomy with acute correction of the femoral varus deformity and internal retrograde IM nail fixation (Fig. 4). Six months after that surgery, she complained of residual bowing (varus) and shortening of the left leg and nonspecific knee pain. On examination, she had radiographically confirmed varus deformity of the left proximal tibia with significant internal tibial torsion and approximately

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Fig. 1 Front view photograph showing clinical appearance of lower extremities at age 10 + 8 before initial treatment. Note windswept deformity (right genu valgum and left genu varum)

2.5 cm shortening (Fig. 5). She was neurovascularly intact and had no evidence of intra-articular abnormalities of the hips, knees, or ankles.

Preoperative Clinical Photos and Radiographs

Fig. 2 Standing AP radiograph of lower extremities demonstrating bilateral complex deformities of the femur and tibia with right genu valgum 25
(Lateral Proximal Femoral Angle (LPFA) ¼ 110
, Lateral Distal Femoral Angle (LDFA) ¼ 75
, Medial Proximal Tibial Angle (MPTA) ¼ 95
, Joint Line Congruence Angle (JLCA) ¼ 0
) and left genu varum 30
(LPFA ¼ 105
, LDFA ¼ 120
, MPTA ¼ 80
, JLCA ¼ 0
, midshaft femoral varus 30
) with characteristic pathologic changes of the growth plates

See Figs. 1, 2, 3, 4, and 5.

Treatment Strategy Preoperative Problem List • Persistent complex genu varum deformity (25
) due to distal femoral varus (10
), proximal tibial varus (10
), and incongruent knee joint (5
varus) • Associated significant internal tibial torsion (40
) • Mild (2.5 cm) LLD (L < R) • Nonspecific intra-articular knee pain without detected structural abnormalities • Systemic skeletal dysplasia (VDRR) controlled on replacement therapy • Propensity to delayed union associated with VDRR

Based on radiographic analysis of the left lower extremity including coronal plane malalignment test, genu varum consisted of three components including 10
distal femoral varus, 10
proximal tibial varus, and 5
joint line incongruence. Due to moderate deformity of the distal femur and the presence of the retrograde intramedullary nail, our approach for coronal plane limb realignment was based on proximal tibial osteotomy followed by 20
gradual deformity correction and 1 cm lengthening using circular external fixation. Computer-assisted preoperative planning was utilized for this stage using the Leg Perfect software. The next stage of limb realignment comprised of correction of 40
internal tibial

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Fig. 3 Standing AP radiographs of lower extremities illustrating attempted growth modulation of the right medial distal femur/ proximal tibia and left lateral distal femur using 8-plates (radiograph on the right is at skeletal maturity). Note that growth modulation has effected correction of the right genu valgum (LPFA ¼ 110
, LDFA ¼ 88
, MPTA ¼ 88
, JLCA ¼ 0
), but the left genu varus persists (LPFA ¼ 105
, LDFA ¼ 122
, MPTA ¼ 85
, JLCA ¼ 4
varus, midshaft femoral varus 30
) associated with internal tibial torsion

torsion, which was done acutely under sedation using somatosensory-evoked potential (SSEP) monitoring. Finally, limb length equalization was completed by gradual distraction. The hope was that this strategy would equalize loading on both sides of the knee during weight bearing, thereby restoring congruency of the contacting surfaces of the joint and eliminating knee pain. Because of known risk of delayed healing/nonunion associated with VDRR, adequate management of the patient’s vitamin D metabolism was confirmed with metabolic specialists to allow reconstructive surgery requiring osteotomy and gradual/acute manipulation of the tibial fragments.

Basic Principles Vitamin D-resistant rickets (VDRR), also known as hereditary or familial hypophosphatemic rickets, comprises a group of diseases in which normal dietary intake of vitamin D is insufficient to achieve normal bone mineralization due to pathologic renal phosphate wasting. The radiographic

features include complex and multi-apical recurrent deformities (typically, femoral varus procurvatum and tibial varus internal rotation) with metaphyseal osteopenia and characteristic growth plate medial widening [3, 4]. Management of patients with VDRR requires multimodal medical replacement therapy by metabolic specialists to avoid hypercalcemia and delayed union and to identify renal dysfunction. Prolonged healing time should also be expected in those patients, thereby influencing decision-making in the overall medical therapy, rehabilitation of functional impairment, and deformity correction strategies using stable bone fragment fixation [1, 2, 5 ,6]. In addition, patients with VDRR often have complaints of polyarticular pain without specific clinical or radiographic findings, even when medical management and deformity correction are deemed adequate.

Images During Treatment See Figs. 6, 7, 8, 9, 10, 11, 12, 13, 14, and 15.

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Fig. 5 Front view photograph demonstrating clinical appearance of lower extremities at presentation for tibial deformity correction. Note varus deformity of the left tibia and significant internal torsion

Fig. 4 Standing AP radiograph of lower extremities after double-level left femoral closing wedge osteotomy with retrograde intramedullary nail fixation. Note persistent 25
genu varum due to combined residual distal femoral and proximal tibial varus deformity (LPFA ¼ 95
, LDFA ¼ 98
, MPTA ¼ 77
, JLCA ¼ 5
varus), internal tibial torsion, and 2.5 cm LLD

preference in those cases is to correct rotation deformities acutely in several steps using SSEP monitoring, followed by gradual correction of any remaining rotation if necessary.

Outcome Clinical Photos and Radiographs See Figs. 16, 17, and 18.

Technical Pearls The level, nature, and severity of each identified deformity must be carefully assessed in patients with VDRR to develop an appropriate correction strategy. In this patient, acute correction of the femoral deformity and internal fixation were chosen, while the proximal tibial varus deformity was deemed more appropriate for gradual correction and circular external fixation. In addition, the associated internal tibial torsion was also corrected using the circular external fixator. Several frame configurations are available for rotational deformity correction. In Ilizarov-type frames, different rotation modules can be assembled and attached between the external supports. Preassembled translation-rotation components can also be utilized during frame modification. In hexapod-type fixators, only recalculation of the daily strut adjustment is required without modification of the frame. Our

Avoiding and Managing Problems Deformities in patients with VDRR are routinely bilateral, multi-segmental, multi-planar, and multi-apical. Therefore, correction of deformity in one segment immediately affects the biomechanics of the deformed segment across the joint often increasing the magnitude of its deformity. Ideally, correction of all deformities should be included in preoperative planning. In some arch-shaped multi-apical limb deformities, however, correction of all those deformities is very difficult or impossible. In addition, the potential for prolonged healing time and development of malunion/nonunion must always be kept in mind. To overcome those difficulties, surgeons often must consider a compromise strategy of significant bone fragment translation at more than one level, in order to

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Fig. 6 Computerized preoperative planning of deformity correction using the Leg Perfect software

Fig. 7 Intraoperative photograph demonstrating standard TrueLok frame configuration for angular deformity correction. The frame consisted of proximal ring and distal double-ring block interconnected by two hinges and angular distractor and secured to the proximal tibia using one horizontal lateral olive with two half pins and distal tibia by two cross wires with one half pin

Fig. 8 Postoperative AP radiograph of the tibia after proximal tibial osteotomy and frame application. Note the level of the osteotomy located below the deformity apex and the axis of the hinges translated along the bisector line laterally

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Fig. 9 AP and LAT radiographs after completion of angular deformity correction. Note wedge-shaped 1 cm gap between the tibial bone fragments and substantial remaining internal torsion of the distal tibia

Fig. 10 Intraoperative photograph showing location of SSEP monitoring electrodes on the affected and contralateral (control) legs

minimize the total number of osteotomies required. In our patient, for example, the varus deformity of the distal femur was not corrected; we elected to place the proximal tibia in valgus position. As a result, while the overall left lower extremity alignment and joint congruity were achieved, the

joint line ended up in substantial varus orientation relative to the mechanical axis. Moreover, correction of this periarticular deformity now can only be done in two levels including correction of distal femoral varus and proximal tibial valgus.

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Fig. 11 Intraoperative photographs illustrating acute correction of internal tibial torsion. Under SSEP monitoring, the telescopic connection rods between the middle and distal rings were disconnected, tibia was externally de-rotated in two 20
increments acutely, and external supports were reconnected using the same rods

Fig. 12 Intraoperative SSEP traces demonstrating stable peroneal (left) and tibial (right) nerve recordings at the lumbar spine (L1-T8) and popliteal fossa during 40
of acute external tibial de-rotation. Note stable SSEP waveforms during adjustment of stimulation parameters

(1), initial 20
external rotation (2), and additional 20
external rotation (3). To identify potential changes, the resultant signals were constantly compared to preoperative baseline waveforms (4)

118 Fig. 13 AP and LAT radiographs of the tibia after acute correction of internal tibial torsion and completion of the remaining limb lengthening

Fig. 14 Front view photograph showing clinical appearance of lower extremities during consolidation period

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Fig. 15 AP and LAT radiographs of the tibia at the end of consolidation period. Note improved limb alignment and mineralized distraction bone regenerate

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Fig. 16 Standing AP radiograph of the lower extremities 2 years after frame removal demonstrating slight 7
residual left genu valgum and the congruent but mal-orientated joint line (LPFA ¼ 95
, LDFA ¼ 107
, MPTA ¼ 107
, JLCA ¼ 0
). Potential realignment of the extremity will require distal femoral and proximal tibial osteotomies followed by correction of varus and valgus deformity, respectively Fig. 17 Front and lateral view photographs after treatment. Despite slight valgus deformity of the knee radiographically, patient is pleased with the appearance of her lower extremities

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Fig. 18 Lateral view photographs after treatment demonstrating knee and ankle joint function

Cross-References

References and Suggested Reading

▶ Deformity Correction in Child with X-Linked Hereditary Hypophosphatemic Rickets by Combined Technique (External Fixation and Flexible Intramedullary Nailing) ▶ Genu Varum Secondary to Rickets Treated with Femoral and Tibial Osteotomy and Application of a Multi-Axial Correcting External Fixation System ▶ Hypophosphatemic Rickets with Bilateral Severe Genu Varum. Retrograde Fixator Assisted Nailing for Femurs and Double Level Tibial Osteotomies with TSF

1. Choi IH, Kim JK, Chung CY, Cho TJ, Lee SH, Suh SW, Whang KS, Park HW, Song KS. Deformity correction of knee and leg lengthening by Ilizarov method in hypophosphatemic rickets: outcomes and significance of serum phosphate level. J Pediatr Orthop. 2002;22:626– 31. 2. Ferris B, Walker C, Jackson A, Kirwan E. The orthopaedic management of hypophosphataemic rickets. J Pediatr Orthop. 1991;11:367. 3. Hamosh A (2011) Hypophosphatemic rickets, X-linked dominant. Recourse document. Online Mendelian inheritance in man (OMIM), #307800. http://omim.org. Accessed 3 Mar 2014. 4. Herring JA. Limb deficiencies. In: Herring JA, editor. Tachdjian’s pediatric orthopedics. 5th ed. Philadelphia: Elsevier Saunders; 2014. p. 738–9. 5. Rubinovitch M, Said SE, Glorieux FH, Cruess RL, Roqala E. Principles and results of corrective lower limb osteotomies for patients with vitamin D-resistant hypophosphatemic rickets. Clin Orthop Relat Res. 1988;237:264. 6. Song HR, Soma Raju VV, Kumar S, Lee SH, Suh SW, Kim JR, Hong JS. Deformity correction by external fixation and/or intramedullary nailing in hypophosphatemic rickets. Acta Orthop. 2006;77:307–14.

See Also in Vol. 3 Complex Four Segment Multiapical Lower Extremity Deformities in Rickets Treated with Fixator Assisted Intramedullary Nailing

Adolescent with Segmental Bone Defect Secondary to Grade IIIB Open Tibial Fracture Treated by Oblique Wire Bone Transport

18

Lori Karol, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Preoperative Problems List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128

Abstract

Thirteen-year-old male sustained multiple fractures in a rollover bus accident including Gustilo-Anderson grade IIIB open right tibial fracture as well as severe traumatic brain injury necessitating a prolonged ICU stay. Initial treatment consisted of repeated irrigation and debridement, followed by insertion of flexible stainless steel Ender’s nails. Ten days postoperatively, infection necessitated aggressive bony debridement which resulted in a 10-cm bone defect and placement of a temporary spanning frame. Later, pin-to-bar external fixator was converted to TrueLok circular external fixator followed by proximal tibial osteotomy and 10-cm oblique wire bone transport.

L. Karol (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected]

Brief Clinical History The patient is a 13 year old male who was a passenger in a rollover bus accident and sustained multiple fractures including Gustilo-Anderson grade IIIB open right distal tibial/fibular segmental fracture and closed fractures of the left femoral shaft, distal tibia and fibula, and distal radius, as well as nonedisplaced fracture of the pelvis, T11 compression fracture, mandibular fracture, and severe brain injury, requiring intracranial pressure monitoring. He was treated initially with intramedullary fixation of the femoral fracture and irrigation and debridement (I&D) of the open tibial fracture. Four days following injury, patient underwent I&D and insertion of two Ender’s flexible stainless steel nails for stabilization of his open tibia fracture. Six days later, the edges of the overlying wound were necrotic; repeated I&D revealed gross purulence and necrosis within the segmental tibia fracture (Fig. 1). The implants were removed, and eventually a bridging pin-to-bar frame was applied with an antibiotic methyl methacrylate spacer spanning 10 cm (Fig. 2). In addition, a rectus abdominus free flap was performed 20 days after injury and

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patient was transferred to a rehabilitation facility for further treatment of his brain injury (Fig. 3). The patient remained nonambulatory 3.5 months following injury and dependent for all care.

L. Karol et al.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Preoperative Problems List • • • • • • •

Segmental 9.5-cm tibial bone defect Intact fibular length History of postoperative infection Insufficient fixation of bone segments Post-immobilization osteopenia Presence of free rectus abdominus flap Poor ability to rehabilitate due to traumatic brain injury rendering unable to ambulate

Treatment Strategy

Fig. 1 Intraoperative photograph showing necrotic edges of the overlying wound and necrosis within the segmental tibia fracture

Fig. 2 AP and LAT radiograph demonstrating 10-cm segmental bone loss. Note antibiotic methyl methacrylate spacer between the bone fragments, which are stabilized with spanning external fixator

Two distinct strategies using circular external fixation are utilized in the management of segmental bone loss including (1) acute shortening until contact between the opposite ends of bone segments followed by osteotomy and limb lengthening and (2) bone transport. Due to substantial bone defect

Fig. 3 Photograph during temporary fixation showing location and clinical appearance of the rectus abdominus free flap and location of the spanning frame

18

Adolescent with Segmental Bone Defect Secondary to Grade IIIB Open Tibial Fracture Treated by Oblique Wire. . .

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with intact fibular length and prolonged stabilization of bone segments with the presence of the free flap, acute shortening of the limb would be associated with a high risk of complications. Therefore, defect management of this case was based on bone transport. Basic frame configuration included three levels of external supports for fixation of residual proximal and distal segments using wires and half pins and stabilization of the created intercalary transport segment with two oblique olive wires. Because proximal and distal bone segments were already bridged by the spanning fixator, utilization of previous half pins was considered. Moreover, circular external fixator was assembled intraoperatively around the existing temporary fixator followed by half pin disconnection and reattachment to the rings of the frame.

Basic Principles Bone transport is a distraction osteogenesis technique that was introduced by Ilizarov for treatment of long bone defects resulting from trauma, oncologic resection, and other severe congenital or acquired deformities [4]. According to his classification, bone transport is a bifocal distraction-compression osteosynthesis technique involving formation of a free segment of bone (transport segment) from one of the residual bone segments (host segment) and its gradual movement toward the opposite residual bone segment (target segment) across the osseous defect. Originally, two oblique wires were utilized to transport the intercalary segment through the bone defect [5]. Later, other methods of bone segment transportation were applied including sliding of the ring with attached transport segment along the threaded rods (transverse wire bone transport), cable bone transport, transport along the intramedullary rod, and bone segment transportation using motorized intramedullary lengthening nail [1–3, 6–8]. During this movement, distraction osteogenesis occurs and a typical bone distraction regenerate is formed between the residual host bone segment and the trailing end of the transport segment. Once the transport segment reaches the residual target segment, compressive forces are applied at the docking site until the bony margins of the transport and target segments are fused.

Images During Treatment See Figs. 4, 5, 6, 7, 8, and 9.

Fig. 4 Intraoperative photograph demonstrating assembly of TrueLok circular external fixator over the spanning frame

Fig. 5 Intraoperative photograph showing half pin reattachment to the rings and conversion of pin-to-bar fixator to circular external frame

Technical Pearls Although oblique wire bone transport avoids longitudinal scar cutting of soft tissues produced by transverse wire bone transport, does not compromise the flap, and did not resulted in long scars, it requires more careful manipulation with the transported bone segment. Ideally, oblique wires should be inserted through both bone cortices at the same level and penetrate only distal third of the transport segment being at the same angle relative to the limb axis. Both olive wires should be straightened after insertion but not over tensioned preventing over distraction at the osteotomy. If one of the wires is bent after surgery, it can be straightened gradually by increasing the rate of distraction for 5–10 days.

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Fig. 6 Intraoperative AP radiograph after frame application and proximal tibial osteotomy for bone transport. TrueLok fixator consisted of the proximal and distal rings attached to the related bone fragments by two half pins with lateral olive wire and two olive wires with one half pin, respectively, and interconnected by four threaded rods. Note two oblique olive wires inserted into the intercalary fragment and “floating” middle ring, which will be used for docking between the transport and target bone fragments later

Fig. 7 Intraoperative photograph illustrating frame extension to the foot to increase overall stability of short distal tibial fragment fixation. Note oblique wire attachment to special threaded rods for bone transport

L. Karol et al.

Fig. 8 AP radiograph during oblique wire bone transport. Note two distraction regenerates due to premature consolidation of the primary gap after 10 mm of distraction (patient stops doing distraction) requiring re-osteotomy to continue bone transport

Fig. 9 AP and LAT radiographs during consolidation after docking between the transport and target distal segments at the end of 9.7 cm of total bone transport. Note that oblique wires are removed and transport segment is stabilized by two transverse olive wires secured to the “floating” middle ring interconnected with the proximal and distal rings by two sets of four telescopic rods for independent axial distraction proximally and compression at the docking site distally. Frame was removed 10 months after initial injury without further sequelae

18

Adolescent with Segmental Bone Defect Secondary to Grade IIIB Open Tibial Fracture Treated by Oblique Wire. . .

Due to oblique orientation of the wires, the rate of distraction should be slightly higher (1.25–1.5 mm/day depending on the wire orientation angle) in order to produce 1 mm of distraction along the axis of the bone. When transported bone segment reaches the residual target bone segment, both oblique wires should be removed and replaced by two transverse tensioned wires attached to the middle (initially “floating”) ring providing more reliable compression between the bone segments at the docking site.

Outcome Clinical Photos and Radiographs See Figs. 10 and 11.

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correction of angular deformities in the coronal plane (valgus/varus), the rate of distraction should be adjusted (increased/decreased) on one of the wires until the transported bone fragment returns to its original movement path. Angular deviations in the sagittal plane (procurvatum/ recurvatum) should be managed acutely by reattaching both oblique wire distraction rods anteriorly or posteriorly depending on the angle between the axis of the transported segment and the common vector of distraction. Horizontal migration of the transport segment (anterior/posterior and medial/lateral) is corrected acutely at the docking site during replacement of the oblique wires with horizontal wires. In our institution, all patients undergo debridement of contacting bone segments with placement of the iliac crest bone graft followed by acute compression at the docking site.

Avoiding and Managing Problems See Also in Vol. 2 Oblique wires provide well-controlled transportation of the intercalary segment across the bone defect. Any angular deviation of the transported segment from the planned vector should be corrected by manipulation with the wires. For

GIII B Pilon Fracture with Segmental Bone Loss IIIB Segmental Open Tibial Plafond Fracture Treated with Ankle Joint Salvage and Bone Transport

Fig. 10 AP and LAT radiographs 1.5 years after frame removal demonstrating good alignment and complete remodeling of the distraction regenerate and docking site with corticalization

Fig. 11 Clinical appearance of lower extremities after treatment. At his final follow-up 4 years after frame removal, patient has no pain and ambulates with a fairly coordinated gait despite 1.5 cm of residual LLD and a mild foot drop due to initial injury

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References and Suggested Reading 1. Baumgart R, Hinterwimmer S, Kettler M. Central bone transport system optimizes reconstruction of bone defects. Results of 40 treatments. Unfallchirurg. 2005;108:1011–21. 2. Catagni R, Catagni M, Johnston EE. The treatment of infected nonunions and segmental defects of the tibia by the methods of Ilizarov. Clin Orthop Relat Res. 1992;280:143–52. 3. Eralp L, Kosaoglu M, Yusof MN. Distal tibial reconstruction with use of a circular external fixator and an intramedullary nail. J Bone Joint Surg Am. 2007;89:2218–24.

L. Karol et al. 4. Ilizarov GA. Basic principles of transosseous compression and distraction osteosynthesis. Ortop Traumatol Protez. 1971;32:7–15. 5. Ilizarov GA. Transosseous osteosynthesis: theoretical and clinical aspects of the regeneration and growth of tissues. New York: Springer; 1992. 6. Kucukkaya M, Armagan R, Kuzgun U. The new intramedullary cable bone transport technique. J Orthop Trauma. 2009;23(7):531–6. 7. Paley D, Maar DC. Ilizarov bone transport treatment for tibial defects. J Orthop Trauma. 2000;14:76–85. 8. Rozbruch SR, Weitzman AM, Watson JT. Simultaneous treatment of tibial bone and soft-tissue defects with the Ilizarov method. J Orthop Trauma. 2006;20:197–205.

Comminuted Type I Open Distal Femur Fracture

19

Christopher Iobst

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132

Abstract

A 13 year old male suffered a comminuted type I open left distal femoral metaphyseal fracture. The fracture pattern and location made traditional treatment methods difficult to use. A “no touch” closed reduction of the left distal femoral fracture was performed using a Taylor Spatial Frame (TSF). This technique avoids the zone of injury and does not further devascularize any of the fracture fragments. With a stable frame, weight bearing can be started immediately. The comminuted fracture healed anatomically and without bone grafting in 4 months.

Brief Clinical History A 13 year old male suffered a type I open left distal femoral metaphyseal fracture after falling off of an all-terrain vehicle. His type I open wound was treated by irrigation in the emergency department and immediate administration of intravenous antibiotics (Ancef). The left lower extremity was splinted temporarily and taken to the operating room the following morning. Due to the large amount of comminution of the medial distal femoral metaphysis, it was decided to avoid the zone of injury and manage the injury by using the “no touch” method of closed reduction and external fixation. Because of the distal nature of the femur fracture, the fixator was constructed to span the knee to provide more stable fixation.

C. Iobst (*) Department of Orthopedic Surgery, Nemours Children’s Hospital, Orlando, FL, USA e-mail: [email protected] © Crown 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_95

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Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List 1. 2. 3. 4.

Type I open fracture wound Comminuted distal femoral fracture Proximity of fracture to physis Choice of fracture fixation

C. Iobst

felt to be the best option for this injury. The Taylor Spatial Frame allowed stable, multi-planar fixation to be used. This frame also allowed the fracture to be reduced anatomically without having to open the fracture site. The Taylor Spatial Frame was gradually adjusted until the two main fragments were in proper alignment. No attempt to reduce the comminuted fragments was performed. In order to maximize stability, it was decided to extend the frame across the knee to the proximal tibia. The knee was placed in full extension, and the joint capsule was distracted approximately 5 mm in the operating room.

Treatment Strategy

Basic Principles

The type I open wound was managed by irrigation in the emergency department and immediate administration of intravenous antibiotics. Other than covering the wound with a sterile dressing, no further treatment was needed for the open wound. The fracture had significant comminution medially with a large zone of injury. The fracture was too distal for nailing. Plating would require a large exposure through the zone of injury. Subcutaneous bridge plating would be difficult because of the physis. Therefore, external fixation was

1. By avoiding the zone of injury, the blood supply to the bone fragments will not be disturbed any further. External fixation allows the fixation to be placed outside the zone of injury and still maintain excellent stability of the fracture fragments. 2. The “no touch” closed reduction relies on ligamentotaxis to obtain the alignment. No significant violation of the soft tissue envelope should be attempted.

Fig. 1 AP (a) and lateral (b) views demonstrate the comminuted type I open left distal femoral fracture with significant bone crush to the medial distal metaphysis. The physis appears to be intact. There was a less than 5 mm-sized poke hole on the lateral distal thigh over the fracture site

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Comminuted Type I Open Distal Femur Fracture

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Fig. 2 A tracing attempts to depict the amount of missing bone in the medial distal left femur

Fig. 4 Radiographs at 10 weeks show excellent alignment of the distal femur and consolidation of the comminuted fracture fragments

3. Small periarticular fracture fragments may require the surgeon to span the adjacent joint in order to achieve adequate stability. A small amount of distraction of the joint at the time of fixation is helpful to prevent significant contractures from developing when the frame is removed. 4. Multi-planar fixation spread out over the length of the bone creates the most stable frame construct.

Images During Treatment See Fig. 3.

Technical Pearls

Fig. 3 Fixator construct with two opposing olive wires in the distal femoral epiphysis and three half pins in the proximal femur. The two main femoral fragments are reduced. An extra ring is used to span the knee and with two half pins in the proximal tibia. The knee joint is distracted approximately 5 mm using threaded rods

1. All pediatric type I open fractures do not need formal open irrigation and debridement in the operating room. Immediate initiation of intravenous antibiotics is the most important variable in managing these injuries. 2. The fracture pattern dictated placing fixation in the distal femoral epiphysis. The surgeon should be aware that this intra-articular hardware placement may potentially increase the risk of developing a septic knee joint.

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Fig. 5 AP (a) and lateral (b) radiographs at 3-year follow-up show complete reconstitution of the medial femoral metaphysis

3. The knee joint was spanned to provide increased stability to the distal femoral fracture fragment. The knee joint should be distracted 5–10 mm at the time of fixation. This should help prevent significant contractures from developing when it is time to remove the fixator. 4. The tibial portion of the fixator was removed once callus formation was noted on the radiographs. Callus indicates the fracture has developed enough inherent stability that the spanning ring can be removed. The less time the joint is immobilized, the less difficulty there should be in restoring range of motion. 5. The “no touch” technique can be used for fractures with large areas of comminuted bone. Rodding, submuscular bridge plating, or external fixation can utilize this method.

Outcome Clinical Photos and Radiographs See Figs. 4 and 5.

Avoiding and Managing Problems 1. Spanning the knee joint with the fixator provided extra stability to the distal femoral fracture fragment. 2. Distracting the knee joint when spanning with an external fixator helps prevent knee contractures. 3. Prompt removal of the spanning portion of the fixator at the first sign of callus formation helps to prevent knee contractures. 4. Gradual reduction using a “no touch” technique avoids further injury to the blood supply to the comminuted bone

fragments. Avoid making any unnecessary incisions in the zone of injury. 5. Choose a Taylor Spatial Frame ring large enough to keep the struts off of the skin of the thigh. 6. Make sure there is enough posterior clearance between each ring and the thigh. Placing towels between the ring and the skin may help keep the frame adequately positioned intraoperatively. 7. Taylor Spatial Frame rings should be arranged with patient comfort in mind. 2/3 rings can be used with the opening medially at the proximal ring and with the opening posterior with the distal femoral ring. This allows maximal clearance proximally so the patient can close his/her legs together. This also allows maximal knee flexion distally because the ring is not impinging on the soft tissues.

Cross-References ▶ Distal Femoral Fracture Treated Initially with Internal Fixation Converted to Circular External Fixation due to Nonunion and Hardware Failure ▶ Ten Year Old Male with Comminuted Distal Femoral Fracture

References and Suggested Reading 1. Marangoz S, Feldman DS, Sala DA, Hyman JE, Vitale MG. Femoral deformity correction in children and young adults using Taylor Spatial Frame. Clin Orthop Relat Res. 2008;466(12):3018–24. 2. Sabharwal S. Role of Ilizarov external fixator in the management of proximal/distal metadiaphyseal pediatric femur fractures. J Orthop Trauma. 2005;19(8):563–9.

Part II Pediatric Deformity: Growth Plate Injuries

Growth Plate Injuries: An Introduction

20

Reggie C. Hamdy

The growth plate is a specialized cartilaginous structure situated at the proximal and distal ends of long bones and is responsible for longitudinal growth of bones. As the child is growing, the cartilage on the metaphyseal side is gradually replaced by mineralized tissue that ossifies through the process of endochondral ossification. It is the weakest part of the bone and thus vulnerable to various types of injuries or insults. In this section, 17 cases of growth plate injuries are discussed, most of them caused by trauma, but also other pathologies including infection (cases 15 & 16, two other cases of proximal femoral destruction are discussed in the Hip section), meningococcemia (case 12), curettage of cystic lesion adjacent to the growth plate (case 13), and post ACL repair (case 20). In some cases of fractured femur, a delayed appearance of a proximal tibial deformity (case 21), most often recurvatum deformity, may occur long after the initial trauma, and this is due to an undiagnosed injury to the proximal tibial growth plate. This possibility should be considered when addressing femur fractures. Furthermore, limb deformities can develop in some genetic conditions, like skeletal dysplasias, where one side of the physis is “sick” with slower growth than the other half of the physis, leading to angular (genu valgum/varum) or more complex deformities (this is more extensively discussed in Skeletal Dysplasia section). A “sick” physis is also typically seen in cases of Blount’s disease, where the medial proximal tibial physis grows at a slower rate than the lateral half of the physis (this is discussed in Blount Disease section). As the growth plate is entirely cartilaginous, it has very limited potential to regenerate following damage, and healing occurs either through osseous tissue formation or decreased growth (dysfunctional or sick physis). As such, any injury to the growth plate, irrespective of the cause, may have serious consequences on its structure and function. If partial growth R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

plate arrest occurs, in the form of a bony bar (cases 21, 22 and 26) or dysfunction (a “sick” physis, if only growth retardation is present without bar) (cases 13, 18, 23, 24), this usually leads to asymmetric growth and subsequent angular/rotational deformities. If complete growth plate arrest ensues, then longitudinal growth ceases from that growth plate, and a limb length discrepancy gradually develops (case 12). Combined angular deformity (with or without a rotational component) and limb length discrepancy often occur together as seen in most cases of this section. Management of growth plate injuries warrants a high index of suspicion and a close follow-up to detect any early dysfunction of the growth plate, usually evident clinically by the appearance of an angular deformity or LLD. Radiological assessment (plain X-rays) will show evidence of any malalignment and LLD. MRI and bone scans may be necessary to evaluate the function of the growth plate and the presence of a bony bar. Important factors that should be considered in the planning of any treatment for growth plate injuries include: • Age at time of the insult. This is probably the most important factor that will determine the magnitude of the deformity and length discrepancy. The younger the patient, the more potential for growth and the greater the LLD and deformity can be expected if no treatment is instituted. On the other hand, a growth plate injury occurring near skeletal maturity would probably have no or minimal consequences regarding LLD or development of deformities and therefore may not need any treatment. In three cases (12, 15, 26), the injury occurred at a very young age, with a large predicted LLD. In such cases, it should be clearly explained to the parents that if they opt for correction of the LLD with lengthening, this will necessitate multiple lengthenings. Multiple short lengthenings are usually preferred and considered safer than a single large lengthening. • Nature and severity of the trauma. Any type of physeal injury can lead to a growth arrest; however, crush injuries, Salter-Harris types IV and V, are more susceptible to

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_370

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develop growth plate arrest. Furthermore, forceful reduction of these injuries could lead to iatrogenic damage to the growth plate. In cases of fulminant meningococcemia (case 12), the parents should realize that this disease usually affects multiple growth plates with various degrees of severity and in many cases, a combination of partial and complete growth arrest, resulting in both angular/rotational deformities and LLD. • Anatomical location of the growth plate. The knee is the most common anatomical site for clinical problems arising from growth plate injuries and this is the site affected in all 17 cases in this section (all by different authors). This emphasizes the importance of a very close follow-up for all distal femoral physeal injuries. The growth plates affected are either the distal femur, proximal tibia, or both. The reason is that the distal femoral growth plate is the fastest growing growth plate in the human body followed by the proximal tibia and the proximal humerus. In some cases (12 and 15), the growth plates of the tibia only are damaged and not those of the fibula, leading to an overgrowth of the fibula, and this may necessitate isolated lengthening of the tibia only to restore the normal anatomy of the leg. • Is the growth plate damage complete or partial? If a partial injury, is it caused by a bony bar or by dysfunction of part of the physis? (a) In cases of complete growth arrest, it becomes an isolated LLD problem, and this should be managed based on the predicted LLD at skeletal maturity (options include no treatment, shoe lift, timely contralateral epiphysiodesis, one or multiple lengthenings, contralateral shortening after skeletal maturity, or a combination of any of the above).

R. C. Hamdy

(b) In cases of partial growth arrest, it is important to determine if that is caused by a dysfunction of the growth plate or by a bony bar. Further radiological investigations with MRI may be necessary to identify the presence of a bar, and if present, the extent of this osseous bar (mapping of the bar) to help determine if bar resection is indicated. Management of partial growth plate arrest in the skeletally immature patient includes three basic principles, as shown in Fig. 1: 1. How to correct the deformity – acute versus gradual correction 2. How to maintain the correction 3. How to address the LLD In case the injury consists only of a sick physis, with growth potential present but at a slower rate, growth modulation with hemi-epiphysiodesis using a staple or plate is indicated (case 13). However, this may not be successful in every case (as shown in case 15). In cases with combined angular/rotational deformities and LLD (cases 17, 19 and 20), a logical approach could be a combined acute and gradual correction: acute correction of angular/rotational deformities and then gradual lengthening to address the LLD. In such cases, the latency period before starting distraction for lengthening should be longer than the standard 7–10 days, as acute correction may cause increased damage to the blood supply at the site of correction. In all cases of partial growth arrest (cases 21, 22, 26), correction of the deformity only is not enough in skeletally immature patients, it is also important to maintain the correction by performing hemi-epiphysiodesis of the remaining physis (Table 1).

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Growth Plate Injuries: An Introduction

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Treatment of Partial Growth Arrest Sequelae

No bony bridge

Bony bridge

1. Correct the malalignment

Osteotomy

Acute correction by osteotomy

2. Maintain the Continue correction hemiepiphysiodesis & prevent progression of deformity 3. Correct the limb length discrepancy

• Nothing • Shoe lift • Contralateral epiphysiodesis • Lengthening

Osteotomy

Osteotomy

Gradual correction with external fixator

Gradual correction of angular deformity & LLD

Continue hemiepiphysiodesis

Continue hemiepiphysiodesis

• Nothing • Shoe lift • Contralateral epiphysiodesis • Lengthening

Both angulation deformity and leg length discrepancy correct simultaneously

Fig. 1 Diagram showing management guidelines for cases of partial growth arrest in skeletally immature patients associated with deformities. If a bony bridge is present and cannot be resected, options include either acute deformity correction and internal fixation or gradual correction with external fixation. The limb length discrepancy is addressed at a later date. A third option is gradual correction of the deformities and

Stapling

• Nothing • Shoe lift • Contralateral epiphysiodesis

simultaneous lengthening for the limb length discrepancy. In all three scenarios, hemi-epiphysiodesis of the unaffected part of the physis is recommended in order to maintain the correction and prevent progression of the deformity. If a bony bridge is not present (sick physis), then hemi-epiphysiodesis of the healthy part of the physis is usually recommended if the growth plate is still open

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R. C. Hamdy

Table 1 Details of the 17 cases of Growth Plate Injuries discussed in this Atlas Case 12

Diagnosis Fulminant meningococcemia at the age of 5 months

Problems 6 year old boy, right distal femoral, proximal and distal tibial growth arrest. Overgrowth fibula. LLD of 14 cm

13

Recurrent aneurysmal bone cyst distal femur, treated with curettage Proximal tibial recurvatum

11 year old male, progressive genu valgum after curettage of ABC distal femur. MRI showed no bony bridge, but sick physis 14 year old boy, with progressive proximal tibial recurvatum 8 year old male, proximal tibial growth arrest, valgus deformity. Failed hemi-epiphysiodesis medial side, relative overgrowth fibula

14 15

Proximal tibia osteomyelitis at the age of 5 years

16

16 year old girl, previous osteomyelitis, distal femur 7 years prior to presentation

Valgus deformity distal femur. Projected LLD 10 cms

17

Fracture tibia at 13 years old. Presenting 3 years later with multiple deformities

16 year old male, with proximal tibial arrest, valgus, recurvatum, external rotation deformity. LLD 2 cm

18

12 years old, LLD, angular deformity. Unknown etiology

12 year old male, LLD 7.5 cm due to physeal growth deceleration. Varus distal femur 10

19

Fell from height at 7 years of age. Physeal arrest of distal femur Proximal tibial arrest after ACL reconstruction Salter II distal femur fracture at 13 years of age Proximal tibial fracture at 10 years of age, deformity proximal tibia

14 year old male, LLD 14 cm from femur. Varus 6 and 30 rotational deformity, distal femur

20 21 22

17 year old male, LLD 4.5 cm and 15 varus, recurvatum 28 , external rotation deformity 18 year old male, bony bar proximal tibia, LLD 5.5 cm femur, 26 recurvatum, proximal tibia 12 year old girl, anterior physeal arrest tibia, proximal tibia recurvatum

23

Post-traumatic physeal growth arrest distal femur at 12 years of age

15 year old male, valgus/flexion deformity distal femur, LLD 3.6 cm

24

Post-traumatic physeal growth arrest distal femur at 11 years of age Post-traumatic physeal injury to distal femur and proximal tibia at 10 years of age

16 year old male, 11 valgus, LLD 4.5 cm

25

26 27

28

4 year old girl, open fracture proximal tibial epiphysis 13 year old boy, posttraumatic Salter I and IV proximal tibia 12 year old boy, presenting with left valgus deformity. No clear history of trauma

18 year old male, distal femoral valgus 17 . Proximal tibia varus 5 and recurvatum deformity 15 . LLD 3.5 cm Infection, destruction of part of the epiphysis and physis. Valgus proximal tibia 40 and LLD Partial growth arrest, tibia valga. Asymmetric growth tibia and fibula Distal femoral valgus 35 , LLD 1.5 cms

Surgery and key points Isolated lengthening of tibia first with TSF to restore normal relationship between tibia and fibula, then simultaneous lengthening of both bones. Timely contralateral epiphysiodesis Guided growth distal femur with eight-plate

Opening wedge osteotomy proximal tibia and gradual correction with standard Ilizarov frame Proximal tibial osteotomy, gradual correction of valgus, isolated lengthening tibia with TSF to restore relation between tibia and fibula. Decompression of common peroneal nerve Distal medial femoral hemi-epiphysiodesis. Two stage correction. First, distal femoral osteotomy and gradual correction and lengthening 5 cms with TSF. Second lengthening of 5 cms 3 years later Proximal tibial osteotomy and gradual correction with TSF of all deformities and then address limb length discrepancy with lengthening. Decompression of common peroneal nerve Acute angular correction and lengthening with PRECICE retrograde intramedullary nail. Plans to address remaining LLD by second lengthening or contralateral epiphysiodesis Acute correction of deformities and two consecutive retrograde femoral lengthenings with Fitbone IM nails Proximal tibial osteotomy and gradual correction with TSF Double MAC external fixation device Proximal tibial osteotomy, gradual correction with TSF. After removal of fixator, bilateral proximal tibial epiphysiodesis to prevent recurrence of deformity or LLD Acute correction of deformities through osteotomy distal femur. Gradual lengthening using monolateral frame. After acute correction, increase the latency period as it disrupts the circulation. Apply frame along anatomical axis Acute correction of deformity. Gradual lengthening with Fitbone retrograde intramedullary nail Decompression of common peroneal nerve before application of frame. Correction first of tibial deformities with TSF, followed by femoral deformities with TSF Medial hemi-epiphysiodesis proximal tibia, gradual correction with Ilizarov fixator Gradual correction with MAC fixator, epiphysiodesis proximal fibula, syndesmotic screw distally. Fasciotomies Medial distal femoral hemi-epiphysiodesis. Distal femoral osteotomy and gradual correction with MAC device

Correction of Post-Traumatic Medial Growth Arrest of the Distal Tibia (Common Problem, but Often Neglected)

21

Mark Eidelman, Nadav Rinott, and Pavel Kotlarsky

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143

Abstract

Varus deformity of the ankle joint secondary to epiphyseal fractures of the distal tibia is quite common. Damage to the medial part of the distal tibial physis may lead to partial growth arrest that can cause a varus deformity with some shortening of the tibia. In the long term, malalignment of the ankle joint can lead to irreversible damage due to ankle and subtalar arthritis. Several options to correct this deformity were described in the literature. The proposed surgeries usually include a supramalleolar osteotomy, which can be either a medial open wedge or lateral closing wedge of the distal tibia, and fixation with a variety of internal and external fixation devices. External fixation devices can be used to perform acute as well as gradual correction. Completion of the closure of the affected physis should be done M. Eidelman (*) · N. Rinott Ruth Children’s Hospital, Rambam Health Care Campus, Haifa, Israel e-mail: [email protected] P. Kotlarsky Ruth Rappoport Children’s Hospital, Rambam Healthcare Campus, Haifa, Israel e-mail: [email protected]

as early as possible, to prevent progression of angular as well as rotational deformity of the distal tibia. In addition, closure of the physis of the distal fibula should be performed to prevent fibular overgrowth and lateral impingement of the ankle. In immature patients epiphysiodesis of the contralateral distal tibia and fibula should be considered, to prevent development of leg length discrepancy. Our correction protocol includes medial opening wedge supramalleolar osteotomy with insertion of structural cortical allograft, to restore normal ankle orientation, and fixation with anatomic locking plate. An oblique distal fibular osteotomy is a crucial part of the procedure, to prevent the talus being squeezed between the medial and the lateral malleolus at the time of correction.

Brief Clinical History 11 years old boy, after right ankle fracture-dislocation, with Salter Harris (SH) 4 fracture of the medial malleolus, initially treated by closed reduction and epiphyseal screw fixation. Eighteen months after the injury developed a severe varus

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_398

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deformity of the ankle – with a mechanical lateral distal tibial angle (LDTA) of 103 (normal 86 –92 ).

Preoperative Clinical Photos and Radiographs See Fig. 1.

Fig. 1 (a, b) Radiographs right after the injury: (a) Anteroposterior (AP) x-ray view, (b) lateral x-ray view. The images show an SH4 fracture dislocation. (c, d) Three months post reduction and fixation x-rays: (c) AP view, (d) lateral view. Complete fracture union and proper

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Preoperative Problem List • • • •

Partial medial growth arrest of the right distal tibia Varus deformity Mild overgrowth of the fibula relatively to tibia Expected leg length discrepancy at maturity as a result of impaired growth of the right distal tibial physis (the

alignment of the distal tibia are seen. (e) Radiograph of both legs, 18 months after injury shows medial growth arrest of the right distal tibial physis with associated varus deformity (LDTA – 103 )

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Correction of Post-Traumatic Medial Growth Arrest of the Distal Tibia (Common Problem, but Often Neglected)

projected LLD can be calculated using the Multiplier method).

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Images During Treatment See Fig. 2.

Treatment Strategy Technical Pearls The first step when approaching this type of deformity is prevention of deformity progression. Therefore, completion of distal tibia and fibula epiphysiodesis on the injured side are needed, and contralateral epiphysiodesis should be considered in young patients. The second step is correction of the deformity. We achieve correction in a single stage procedure by an open wedge osteotomy. This is easily accessible area, safe, and has the advantage of providing mild lengthening to the affected limb which is usually slightly shorter.

Basic Principles All the deformity components need to be addressed. Firstly, completion of the epiphysiodesis of the distal tibia needs to be performed, as the lateral side of the physis is still active, thus preventing recurrence of the deformity. Secondly, epiphysiodesis of the distal fibula is required to prevent further fibular overgrowth and lateral impingement. Oblique medial open wedge osteotomy allows for correction of the deformity, followed by insertion of a cortical structural allograft and internal fixation with a medial locking plate, maintaining the correction and providing stability. Oblique osteotomy of the distal fibula relieves the pressure from the talar dome during and following correction, and intramedullary fixation of the fibula adds stability to the ankle. Contralateral epiphysiodesis of the distal tibia and fibula prevent development of limb length discrepancy in the future.

Oblique osteotomy of the tibia should be performed as close to the apex of deformity as possible. The angle of the osteotomy depends on the severity of the varus deformity. The osteotomy is started using an oscillating saw, however, should be completed with an osteotome. We recommend leaving a few mm of the lateral tibial cortex intact and serve as a hinge during correction, thereby increasing the stability of the whole construct. Fibular osteotomy should be performed early during the procedure, usually just after marking the tibial osteotomy with a wire, thereby preventing compression of the talus during correction. The fibular osteotomy is done at the level of the lateral edge of the tibial osteotomy. Distracting the tibial osteotomy provides for the correction of the distal tibial alignment. A cortical allograft in combination with a locking plate are used to keep the correction. The cortical allograft is wedge shaped. Its size can be estimated by 1 mm for every degree of desired correction (based on preoperative measurements), and intraoperatively adjusted by measuring the gap at distraction site that achieves anticipated ankle alignment. To increase stability on the lateral side, the fibula should be fixed with an intramedullary wire.

Outcome Clinical Photos and Radiographs See Fig. 3.

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Fig. 2 (a) Completion of the closure of the distal tibial physis using a cannulated drill. (b) Osteotomy of the distal tibia and fibula (marked with a red arrow). (c, d) Achieving correction of the distal tibial Fig. 3 (a, b) Radiographs showing the final distal tibial alignment 4 months after correction. The osteotomy sites are united and remodeling is seen

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alignment by performing distraction of the osteotomy site using a Hintermann distractor. (e) Insertion of a cortical bone graft into the gap. (f) Fixation with anatomically countered locking plate

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Correction of Post-Traumatic Medial Growth Arrest of the Distal Tibia (Common Problem, but Often Neglected)

Avoiding and Managing Problems The decision to perform contralateral epiphysiodesis should be based on the estimated leg length discrepancy. On average, the distal tibial physis grows 5 mm yearly, therefore, in girls younger than 11 and boys younger than 13 this step should always be considered. Precise correction must be achieved on both planes intraoperatively. Stable fixation using locking anatomically precountered plate is important. We do not recommend this technique for very large deformities, with LDFA more than 20 of normal. The high risk for

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complication (e.g., nonunion, Neuropraxia) with these deformities should be considered for gradual rather than acute correction.

References and Suggested Reading 1. Kotlarsky P, Abu Dalu K, Eidelman M. Correction of posttraumatic medial growth arrest of the distal tibia in adolescents. Foot Ankle Spec. 2022. https://doi.org/10.1177/19386400211029130. 2. Knupp M, Bolliger L, Hintermann B. Treatment of posttraumatic varus ankle deformity with supramalleolar osteotomy. Foot Ankle Clin. 2012;17(1):95–102.

Part III Pediatric Deformity: Congenital Pseudarthrosis of the Tibia and Fibula

Congenital Pseudarthrosis of Tibia and Fibula: An Introduction

22

Reggie C. Hamdy

Management of congenital pseudarthrosis of the tibia (CPT) is most challenging. Almost half of the cases are associated with neurofibromatosis type 1. The problems include a recalcitrant pseudarthrosis of the tibia, deformity, limb length discrepancy, concomitant affected fibula in many cases, ankle valgus, and a notorious tendency for refracture after having healed. These problems are due to biological causes in the form of unhealthy avascular and scarred soft tissue environment with very poor potential for healing, mechanical causes due to difficulty in obtaining stable fixation of the distal fragment which is often thin, short, and osteoporotic, as well as other associated causes including deformities of the fibula, ankle valgus, and limb length discrepancy. The goal in treating this condition is not only to obtain union but equally important to maintain union and address all associated deformities in order to achieve a functional limb. There is no universally accepted classification of this disease as the radiological types described in the various classifications often change during treatment and with progression of the disease. As there is no standard and generally accepted treatment for this condition, multiple surgeries and techniques have been described in the management of this condition, and often the end result is a frustrated adolescent who had numerous surgeries, several refractures, and a limb that may be nonfunctional. It is imperative, as soon as the diagnosis of CPT is established, to have a transparent and detailed discussion with the patient and the family regarding all the potential problems of this condition and the expected numerous surgeries including the possibility of an amputation. Poor prognostic factors include first surgery at a very young age, residual ankle valgus, and fibular pseudarthrosis. In this section, ten cases (eight cases of CPT, two cases of CPF) with different scenarios and various modalities of treatment are discussed, including the basic principles of R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

treatment. Regarding the management of CPT, there is almost universal agreement that excision of the pseudarthrosis site and the surrounding hamartomatous tissue is the first step in the surgical treatment of this condition. An exception could be when there is a stiff pseudarthrosis of the tibia, in which case the site is not exposed and distraction is applied (case 31). In all other cases, excision of the pseudarthrosis site is followed by realignment of the bone and correction of all deformities. To maintain the correction with stable fixation is often a challenge. Circular external fixation is most commonly used to provide stability and compression, prevent disengagement of the bone ends, allow concomitant functional use of the limb during the duration of treatment, control rotation, and allow the simultaneous correction of any residual deformity. The use of intramedullary fixation alone as with the Fassier Duval rod is another option although this implant does not usually provide adequate rotational stability (cases 29, 32, 33 and 36). When there is a very short distal tibial segment, crossing the ankle with an intramedullary rod may be the only solution to provide the necessary stability (cases 32, 33, 34 and 35). Another option to provide stability is to insert the female component of the Fassier Duval rod through the ankle in a retrograde fashion (case 32). Optimizing the healing environment with autogenous iliac crest bone graft is almost a requirement in these cases, specifically in initial cases after excision of the pseudarthrosis site. The use of BMPs to augment bone grafts has been described by many authors (cases 29, 32, 36). Internally fixing the fibula with intramedullary rods (case 32) provides enhanced stability, specifically rotational, and is generally recommended. Periosteal grafting has been recently described by Paley and seems to enhance healing (cases 33 and 36) (Table 1). If a large segment of bone has to be resected, the available options include Ipsilateral or contralateral vascularized fibular transfer (case 35) bone transport by distraction osteogenesis, acute shortening and then lengthening at the proximal tibia (cases 32, 34), and the Masquelet induced membrane technique. Although it is believed that the whole tibia may be

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_368

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Table 1 Details of the 10 CPT and CPF cases discussed in this Atlas Case 29

35

Diagnosis 8 year old boy, CPT, bowing presented with non-displaced fracture 3 year old female, CPT, El-Rosasy Type, mobile pseudarthrosis 3.5 year old boy, stiff pseudarthrosis, El-Rosasy Paley Type 3 14 year old female, neglected CPT, severe deformity 5 year old, female, CPT, first surgery at age 1 year, failure to heal 8 year old male. El-Rosasy type 2 7 year old, CPT

36

1 year old, CPT

37

12 year old male, congenital pseudarthrosis fibula 13 year old male, congenital pseudarthrosis fibula

30

31

32

33

34

38

Problems Pathological fracture at site of bowing. Neurofibromatosis Type I

Surgery and key points Double osteotomy, excision of pseudarthrosis site, stabilized with Fassier-Duval rod. Op-1 used, iliac crest graft

NF-1, antero-lateral bowing. Previous surgery failed

Acute shortening, impalement distal fragment in split proximal segment, fibular osteotomy, rush rod through ankle, Ilizarov fixator Site of pseudarthrosis NOT exposed. Distraction with Ilizarov frame. Osteotomy proximally to correct bowing, rush rod Multiple surgeries. Excision of pseudarthrosis, hybrid TSF and Ilizarov, OP-1, iliac crest graft, persistent nonunion, solid nail (reversed female FD rod through ankle). Healed Rush rod across ankle, Ilizarov, grafting technique 4 in 1, tibio-fibular synostosis, fibular wire, Zolendronate. Fassier-Duval rod, periosteal graft Excision site of pseudarthrosis. Acute shortening. Rod. Ilizarov/TSF fixator. BMP. Solid nail Ipsilateral vascularized fibular transfer Resection pseudarthrosis site. IM rod through ankle. Brace

Established stiff pseudarthrosis, deformity, LLD. No NF-1 Severe deformity, huge LLD, Short distal fragment, contractures. No NF-1 Persistent pseudarthrosis Failure of previous surgeries Atrophic pseudarthrosis NF-1, failure to heal previous surgeries At age of 9 months, CPT presented. Brace. At 3 years, excision plus rod. Previous surgery failed Previous McFarland bypass surgery, failed NF-1. Pseudarthrosis fibula, ankle valgus, short fibula Isolated pseudarthrosis fibula. No NF-1. Progressive ankle valgus

abnormal, lengthening at the proximal tibia has been successful (case 34). It has been described that the hamartomatous tissue surrounding the pseudarthrosis site exhibits increased osteoclastic activity and bone resorption, and therefore in an attempt to block these resorptive effects, the use of Bisphosphonates (Zoledronic acid) has been suggested as an adjunct to treatment (cases 33, 36). In cases 33 and 36, a special type of bone grafting technique called 4–1 technique, where massive bone grafting is performed between the distal tibia and fibula to achieve a tibiofibular synostosis, seems to address both the biological problem of poor healing environment as well as the mechanical problem of instability. Persistent ankle valgus has been described as being a poor prognostic sign and needs to be addressed. Newer modalities such as periosteal grafting, tibiofibular synostosis, use of bisphosphonates to inhibit the increased osteoclast activity, and the Masquelet technique

Comprehensive treatment plan: excision of diseased site, re-alignment, autogenous bone graft, BMP, periosteal graft, tibio-fibular synostosis, Bisphosphonates Distal tibia-fibula synostosis. Correct ankle valgus with hemi-epiphysiodesis medial distal tibia Direct repair. Excision pseudarthrosis. Intercalary tri-cortical iliac crest bone graft to bridge gap, internal fixation dynamic compression plate (DCP)

seem promising. A comprehensive approach to CPT described by Paley and including most of the abovementioned techniques seems very successful in obtaining union and, most importantly, maintaining the union without refracture (case 36). In adolescents and skeletally mature patients and after healing is achieved, attempts at preventing refracture include the use of a locked intramedullary nail and a full-contact Sarmiento type of orthosis (case 34). Congenital pseudarthrosis of the fibula (CPF), as an isolated entity, is a much less common condition and rarely leads to CPT. Treatment is indicated if there is progressive ankle valgus. This may be addressed either by growth modulation of the medial malleolus or by supramalleolar osteotomy. The fibular defect is stabilized either by direct repair (case 38) or by distal tibiofibular synostosis with permanent hemiepiphysiodesis of the distal tibia medially (case 37).

Congenital Pseudarthrosis of the Fibula

23

Charles E. Johnston

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153

Abstract

Brief Clinical History

Isolated congenital pseudarthrosis of the fibula (CPF) may be associated with progressive ankle valgus and rarely progresses to tibial insufficiency fracture and potential pseudarthrosis. An isolated CPF with ankle valgus is therefore indicated for treatment to correct ankle alignment and avoid late development of tibial pseudarthrosis. Growth modulation (hemi-epiphyseodesis of the medial distal tibia) is preferred if feasible (immature patient), or supramalleolar osteotomy (acute or gradual), if mature, may be employed. Stabilization of the fibula defect should be accomplished by either direct repair or distal tibiofibular synostosis.

A 12 year old male with neurofibromatosis type 1 presented with increasing limp, external rotation, and pain in his left lower leg. The gait changes and pain have insidiously increased over the preceding several months, but the patient had had external rotation of the left foot and ankle for several years without symptoms. Ankle range of motion was normal except that the foot excessively everted with dorsiflexion. The affected leg was clinically 1 cm shorter than the normal right side. NF-1 diagnosis was obvious due to multiple cafe au lait spots and positive family history.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3. C. E. Johnston (*) Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_331

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Fig. 1 Significant left ankle valgus seen on AP radiographs of the bilateral ankles

Preoperative Problem List 1. Discontinuity of the distal fibula, with short lateral malleolus 2. Ankle valgus with talar subluxation

Treatment Strategy 1. Obtain stable ankle joint by distal tibiofibular synostosis (Langenskiold procedure). 2. Correct ankle valgus by growth modulation or osteotomy.

Basic Principles Treatment of CPF first requires that the shortening of the fibula be stabilized in order to provide an adequate buttress to the talus laterally and prevent further talar subluxation. If

C. E. Johnston

Fig. 2 Close-up of the left ankle showing fibular pseudarthrosis, ankle valgus due to lateral growth inhibition of the distal tibial epiphysis (wedge-shaped epiphysis), and lateral talar subluxation due to shortened distal fibula

there is no ankle valgus and fibula pseudarthrosis exists alone, pseudarthrosis repair with internal fixation can be curative and prevent development of ankle valgus later. However, if fibular shortening is established and ankle valgus already exists, as in this case, pseudarthrosis repair alone will not correct the mechanical malalignment, which must be pursued in order to prevent development of tibial stress insufficiency fracture, an ominous development as it may lead to established tibial pseudarthrosis. Ankle valgus may be corrected in this case by medial distal tibial hemiepiphyseodesis, either transiently by staple, eight plate, or transphyseal screw or by permanent hemi-epiphyseodesis, curetting, and bone grafting the medial 1–1.5 cm of the distal tibial physeal plate. In order to stabilize the ankle mortise while correcting the valgus alignment, the distal fibula should be synostosed to the tibia (Langenskiold procedure), so that the two components of the mortise are re-aligned simultaneously.

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Fig. 3 Clinical appearance

Images During Treatment See Figs. 4 and 5.

Fig. 4 Date of surgery – distal tibiofibular synostosis with autogenous bone graft and screw fixation. Also the medial 1 cm of the distal tibial physis has been curetted to perform permanent hemi-epiphyseodesis. Permanent hemi-epiphyseodesis was chosen due to the patient’s age, approaching maturity

Technical Pearls Avoiding and Managing Problems Distal tibiofibular synostosis: although not described by Langenskiold, the use of a trans-mortise fixation is highly recommended, as well as cast immobilization for 6–8 weeks, in order to gain a permanent cross union between the two bones. The bed for the bone graft (iliac crest cancellous) should be decorticated with a high-speed burr. Permanent medial hemi-epiphyseodesis is achieved by direct curettage of the physis to a depth of 1–1.5 cm and then the packing with cancellous bone to achieve rapid physeal arrest.

Outcome Clinical Photos and Radiographs See Figs. 6, 7, and 8.

Hemi-epiphyseal arrest, as with any growth modulation method, must be carefully monitored to assure that the closed portion of the physis is in fact tethering the remaining growth and correction occurring. If lack of correction is recognized early, revision of the hemi-epiphyseodesis, with either fixation (screw, plate) and/or re-curretage, can be accomplished in time to produce the modulation desired, assuming enough physeal growth remains. If modulation correction is unsuccessful, then supramalleolar osteotomy may be eventually required. One must ensure that the tibiofibular synostosis is united if osteotomy for valgus correction is chosen.

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Fig. 5 Distal tibiofibular synostosis technique (Langenskiold) Fig. 6 Nine-month postoperative, gradual correction of ankle valgus occurring. Medial physeal arrest is noted

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Congenital Pseudarthrosis of the Fibula

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Fig. 7 Final correction at maturity age 17

Fig. 8 Final clinical appearance

Cross-References

References and Suggested Reading

▶ Congenital Pseudarthrosis of the Fibula: Direct Surgical Repair

1. Cho TJ, Choi IH, Chung CY, Yoo WJ, Lee SH, Lee SH, Suh SW. Isolated congenital pseudarthrosis of the fibula: clinical course and optimal treatment. J Pediatr Orthop. 2006;26:449. 2. Langenskiold A. Pseudarthrosis of the fibula and progressive valgus deformity of the ankle in children: treatment by fusion of the distal tibial and fibular metaphyses. Review of three cases. J Bone Joint Surg. 1967;49-A:463. 3. Martus JE, Johnston CE II. Isolated congenital pseudarthrosis of the fibula. J Pediatr Orthop. 2008;28:825–30.

Congenital Pseudarthrosis of the Fibula: Direct Surgical Repair

24

John Birch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159

Abstract

Isolated congenital pseudarthrosis of the fibula (not associated with congenital dysplasia of the tibia or systemic disorders such as neurofibromatosis type I or fibrous dysplasia) is an uncommon entity. It can lead to local symptoms, progressive valgus deformity of the ankle and fibular hypoplasia, and mild leg length inequality. We describe here a case of direct surgical repair of an isolated congenital pseudarthrosis of the fibula by a combination of pseudarthrosis excision, intercalary iliac crest bone grafting, and internal fixation with a standard dynamic compression plate (DCP). The graft incorporated uneventfully, and the implant was subsequently removed electively. At 3-year follow-up, the ankle was clinically normal, valgus deformity of the distal tibial epiphysis had normalized, and the patient was without complaint.

Brief Clinical History A 13 year old boy presented with a complaint of limping, favoring the left leg, and mild intermittent achy pain over the lateral aspect of his lower leg. There was no history of injury, infection OR systemic illness, NOR family history for any significant neuromusculoskeletal abnormalities. The patient’s overall appearance was of a healthy, well-developed young man. There were no cutaneous stigmata suggesting neurofibromatosis. Positive findings were limited to the left lower extremity. There were mild distal tibial/foot valgus and mild tenderness to palpation of the distal fibula. The muscles of the lower leg were slightly atrophic (Fig. 1). He had less than 1 cm of leg length inequality, his left leg shorter than his right. There was no evidence of tibial deformity other than the mild ankle valgus. He was otherwise neurovascularly intact. Anteroposterior and lateral radiographs of the left tibia and fibula (Fig. 2a, b) demonstrated isolated

J. Birch (*) Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected] © Springer International Publishing Switzerland (outside the USA) 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_360

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pseudarthrosis of the fibula, mild hypoplasia of the fibula demonstrated by distal migration of the proximal fibula and proximal migration of the lateral malleolus, and a wedgeshaped distal tibial epiphysis producing a mild valgus deformity. There was no evidence of tibial involvement.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List – Symptomatic (isolated) congenital pseudarthrosis of the fibula – Progressive ankle valgus – Increasing fibular hypoplasia – Mild leg length inequality

J. Birch

Treatment Strategy Treatment options which include observation (no treatment, for stable and asymptomatic lesions); direct repair by resection, grafting, and fixation; and cross-union of the distal fibular segment to the lateral tibia with osteotomy or growth modulation of the distal tibia have all been described [1–5]. In this patient, since the affected fibular segment was small, the patient was symptomatic, and early deformity evident, direct pseudarthrosis repair was recommended.

Basic Principles At surgery, the pseudarthrosis lesion was exposed subperiosteally through a direct lateral approach (Fig. 3a). The portion of the fibula to be resected was marked (Fig. 3b), indicating the size of ipsilateral tricortical iliac crest graft that would be required. After resection of the pseudarthrosis (Fig. 3c), the gap was measured. A few millimeters of “oversizing” was anticipated, to ensure a snug interpositional fit of the graft within the fibular remnants and to judiciously lengthen the fibula. After careful shaping of the graft within the defect, the fibula and graft were stabilized with a 3.5 DCP and neutral screws. Intraoperative radiographic appearance is demonstrated in Fig. 4.

Images During Treatment See Figs. 3 and 4.

Technical Pearls

Fig. 1 Clinical photograph of the legs at presentation. Note the mild muscular atrophy of the lower leg and mild valgus deformity of the left foot and ankle

The patient should first be carefully assessed for the presence of comorbidities, specifically, for family history, cutaneous abnormalities, or musculoskeletal disorders associated with neurofibromatosis type I. In addition, radiographs of the lower leg should be carefully assessed for evidence of tibial involvement by some variation of congenital tibial dysplasia (congenital pseudarthrosis of the tibia variant). These conditions are not contraindications to direct surgical repair of congenital pseudarthrosis of the fibula, but likely result in a poorer prognosis for surgical success. We recommend filling the gap with a shaped tricortical iliac crest autograft. Gentle distraction of the fibular gap after resection of the pseudarthrosis may result in some immediate correction of the proximal migration of the distal fibula. Great care should be taken to size the autograft

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Congenital Pseudarthrosis of the Fibula: Direct Surgical Repair

157

Fig. 2 AP and lateral radiographs of the left tibia before treatment: (a) anteroposterior radiograph of the tibia. Note the established pseudarthrosis of the left fibula. There is relative fibular hypoplasia compared to the tibia, identified as distal migration of the proximal

fibular epiphysis and proximal migration of the lateral malleolus. In addition, the distal tibial epiphysis has become wedge shaped, producing a mild ankle valgus deformity. (b) Lateral radiograph

Fig. 3 (a–d) Intraoperative photographs at the time of direct repair, grafting, and fixation of pseudarthrosis site: (a) appearance of the fibular pseudarthrosis after subperiosteal exposure. (b) Marking of the pseudarthrotic segment to be excised. (c) Appearance of the residual

fibular segments after resection of the pseudarthrosis. An approximate 2 cm gap exists. (d) Appearance after insertion of tricortical autograft from the ipsilateral iliac crest, with fixation of the proximal and distal segments of the fibula, and the graft, with a 3.5 standard DCP

such that a snug, keyed-in construct results. We prefer fixation that allows controlled axial loading of the construct, i.e., with intramedullary rod or standard dynamic compression plate, rather than a “locking” plate.

to normal activities. Radiographs at 1 year (Fig. 5) demonstrated incorporation of the graft, with improvement in the radiological appearance of the distal tibia and fibula. The implant was subsequently removed electively. Three years postoperatively, at skeletal maturity, the patient was without complaints or detectable abnormalities. Radiographs demonstrated normalization of the fibula, with improvement of the radiological appearance of the proximal and distal fibula, and the distal tibial epiphysis (Fig. 6).

Outcome Clinical Photos and Radiographs The patient had an uneventful postoperative course. He was kept in a walking boot, toe-touch weight-bearing with crutches for 2 months and then gradually allowed to return

158

Fig. 4 Intraoperative appearance of the fibula on fluoroscopy after grafting and fixation

J. Birch

Fig. 5 Internal rotation (“mortise”) view of the left ankle 1 year after surgery. Note the incorporation of the graft in the pseudarthrosis gap, with apparent improvement of the distal tibial epiphysis wedging, and favorable distal migration of the lateral malleolus relative to the tibia

Avoiding and Managing Problems Isolated congenital pseudarthrosis of the fibula is an uncommon entity and is more typically seen in association with congenital pseudarthrosis of the tibia. Therefore, the clinician must evaluate the patient with congenital pseudarthrosis of the fibula for evidence of tibial dysplasia or deformity suggestive of congenital tibial pseudarthrosis variants and for evidence of neurofibromatosis type I. Treatment options to

consider are observation only (appropriate for very young or older patients who are asymptomatic with stable lesions), direct repair (when the size of the pseudarthrosis and residual fibular segments make such procedure technically feasible), and distal fibular-to-distal tibial cross-union (usually with bone grafting and fixation; distal tibial valgus deformity can be managed by distal tibial osteotomy or growth modulation as appropriate).

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Congenital Pseudarthrosis of the Fibula: Direct Surgical Repair

Fig. 6 Anteroposterior radiograph of the tibia 3 years postoperatively. The implant has been removed electively. Note the resolution of the pseudarthrosis and the improvement in the radiographic appearance of the distal tibial epiphysis, the proximal fibula, and the lateral malleolus. The patient is asymptomatic, without evidence of deformity

159

▶ Congenital Pseudarthrosis of the Fibula ▶ Congenital Pseudarthrosis of Tibia ▶ Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 2) ▶ Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 3) ▶ Congenital Tibial Pseudoarthrosis Treated with Internal and External Fixation Using the 4 in 1 Technique ▶ Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application ▶ Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia ▶ Recalcitrant Congenital Pseudarthrosis of the Tibia

References and Suggested Reading 1. Bitan F, Rigault P, Padovani JP, Finidori G, Touzet P. Congenital pseudarthrosis of the tibia and fibula in children. Results of the treatment of 18 cases with nails and bone grafts. Rev Chir Orthop Reparatrice Appar Mot. 1987;73(7):552–60. [French]. 2. Cho TJ, Choi IH, Chung CY, Yoo WJ, Lee SH, Lee SH, Suh SW. Isolated congenital pseudarthrosis of the fibula: clinical course and optimal treatment. J Pediatr Orthop. 2006;26(4):449–54. 3. Lampasi M, Antonioli D, Di Gennaro GL, Magnani M, Donzelli O. Congenital pseudarthrosis of the fibula and valgus deformity of the ankle in young children. J Pediatr Orthop B. 2008;17(6):315–21. 4. Martus JE, Johnston CE 2nd. Isolated congenital pseudoarthrosis of the fibula: a comparison of fibular osteosynthesis with distal tibiofibular synostosis. J Pediatr Orthop. 2008;28(8):825–30. 5. Trigui M, de Billy B, Metaizeau JP, Clavert JM. Treatment of congenital pseudarthrosis of the fibula by periosteal flap. J Pediatr Orthop B. 2010;19(6):473–8.

Cross-References ▶ Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum)

Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 3)

25

Mahmoud A. El-Rosasy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166

Abstract

Brief Clinical History

Congenital pseudarthrosis of the tibia (CPT) type 3 (after El-Rosasy-Paley classification of CPT) has the best prognosis. In this type the bone ends are wide and the pseudarthrosis is not mobile and with/without past history of surgical interference (El-Rosasy et al., Congenital pseudarthrosis of the tibia. In: Rozbruch SR, Ilizarov S (eds) Limb lengthening and reconstruction surgery. Informa Healthcare Publisher, New York/London, pp 485–493, 2007). Closed distraction of this type of CPT leads to stimulation of new bone formation across the distraction gap, along the lines of distraction, and consolidation of the pseudarthrosis.

A three-and-a-half year old male child is known to have neurofibromatosis type 1 (NF-1) (manifested by widespread “café au lait” patches on the trunk and extremities). He was born with a slight anterior curvature of his right leg. Within the last year his parents noticed gradual increase in the curvature and limping and the child complained of leg pain. Radiographs confirmed the diagnosis of CPT type 3 which developed in a dysplastic segment of the tibia. Previous treatment was only above-knee cast in a trial to get the pseudarthrosis to unite.

Preoperative Clinical Photos and Radiographs See Fig. 1. M. A. El-Rosasy (*) Faculty of Medicine, Department of Orthopaedic Surgery, University of Tanta, Tanta, Al-Ghrabeya, Egypt e-mail: [email protected] © Crown 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_280

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Fig. 1 (a) Preoperative clinical photo shows “café au lait” patches of NF-1 and anterior angulation of the leg. (b–d) Preoperative radiographs show pseudarthrosis of the tibia type 3 (after El-Rosasy-Paley

Preoperative Problem List 1. Stiff pseudarthrosis of the tibia with deformity 2. Anterior tibial angulation proximal to the pseudarthrosis site

M. A. El-Rosasy

classification of CPT). Atrophic pseudarthrosis of the fibula is also seen. Mobility of the pseudarthrosis is being tested under image intensifier [2]

external fixator is left in place until consolidation of the osteotomy. The IM rod is left in situ and a protective splint is to be used until the follow-up radiographs show complete consolidation, bone remodeling, and medulla formation.

Images During Treatment Treatment Strategy Following the treatment protocol based on our classification system, the procedure includes the following: (1) application of a preconstructed Ilizarov frame for closed distraction of the tibial pseudarthrosis without surgical disturbance of the pseudarthrosis site; (2) after consolidation of the pseudarthrosis, an intramedullary rod is inserted and retained as a permanent splint to protect against refracture; (3) corrective osteotomy is to be performed to correct proximal tibial angulation and allow insertion of the intramedullary rod; and (4) a knee-ankle-foot orthosis (KAFO) with fixed ankle will be used as an external splint [3].

See Figs. 2, 3, and 4.

Technical Pearls 1. The fixator should be kept until full consolidation of the osteotomy. 2. Residual deformity could be safely corrected through an osteotomy in a healthy bone segment to allow insertion of intramedullary rod. 3. If the intramedullary rod is to be removed, to allow free ankle range of motion, then the tibia should be protected by an external splint.

Basic Principles After consolidation of the pseudarthrosis, any residual deformity in the tibial diaphysis should be corrected to avoid refractures and to allow insertion of intramedullary rod. The

Outcome Clinical Photos and Radiographs See Fig. 5.

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Fig. 2 (a, b) Postoperative clinical photos show the application of the external fixator without disturbance of the pseudarthrosis site

Fig. 3 (a–c) Radiographs during treatment show early consolidation of the pseudarthrosis and new bone formation along the lines of distraction (longitudinally arranged). The anterior angulation at the pseudarthrosis is being corrected; however, another anterior angulation is present

proximal to the pseudarthrosis and needs corrective osteotomy to allow insertion of an intramedullary rod. (d) Clinical photo after deformity correction

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Fig. 4 (a, b) Radiographs during treatment after percutaneous corrective osteotomy and insertion of intramedullary rod in the presence of the fixator

Avoiding and Managing Problems Preconstruction of the Ilizarov frame should consider application of the hinge so as to produce a neutral wedge correction to avoid tibial overlengthening at the end of deformity

correction. Insertion of an IM rod could be facilitated by acute correction of the other deformities while the frame is in place. A protective removable splint should be used until skeletal maturity when the tibial bone is well formed and remodeled to avoid refracture.

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Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 3)

Fig. 5 (a–c) Follow-up radiographs show the consolidation of the pseudarthrosis and the retained intramedullary rod as a permanent splint after fixator removal. (d, e) Follow-up clinical photos show full weight bearing and overlengthening of the tibia as a result of distraction which is compensated for by a shoe raise on the normal side. (f) Follow-up radiographs after removal of the rod to allow free range of motion of the

Cross-References ▶ Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum) ▶ Congenital Pseudarthrosis of Tibia ▶ Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 2)

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ankle joint. The pseudarthrosis is consolidated but the tibia is still dysplastic and needs protection in a splint until full remodeling and medulla formation. Proximal tibial valgus deformity is planned to be corrected by temporary medial hemi-epiphysiodesis for gradual deformity correction

▶ Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application

166

References and Suggested Reading 1. El-Rosasy MA, Paley D, Herzenberg JE. Congenital pseudarthrosis of the tibia. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York/London: Informa Healthcare Publisher; 2007. p. 485–93.

M. A. El-Rosasy 2. Rozbruch SR, Helfet DL, Blyakher A. Distraction of hypertrophic nonunion of tibia with deformity using Ilizarov/Taylor Spatial Frame. Report of two cases. Arch Orthop Trauma Surg. 2002;122(5):295–8. 3. Thabet AM, Paley D, Kocaoglu M, Eralp L, Herzenberg JE, Ergin ON. Periosteal grafting for congenital pseudarthrosis of the tibia: a preliminary report. Clin Orthop Relat Res. 2008;466(12):2981–94.

Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 2)

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Mahmoud A. El-Rosasy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 171

Abstract

Congenital pseudarthrosis of the tibia (CPT) is defined as a nonunion of a tibial fracture that develops spontaneously or after trivial trauma in a dysplastic bone segment of the tibial diaphysis. The condition is associated with NF-1 in over 50% of cases; however, no neurofibromatosis tissue has been found at the CPT site. Pathologically, a fibrous hamartoma surrounds the bone at the CPT site. Histobiochemical studies have revealed increased osteoclastic activity in the periosteum surrounding the CPT. CPT is both a biological and mechanical problem; the surgical treatment should address both aspects of the condition. The recommended protocol of treatment includes complete excision of the pathological periosteum, insertion of autogenous iliac crest bone graft (ICBG), and combined fixation using intramedullary rod (IMR) and Ilizarov external fixator. Leg length discrepancy, due to tibial M. A. El-Rosasy (*) Faculty of Medicine, Department of Orthopaedic Surgery, University of Tanta, Tanta, Al-Ghrabeya, Egypt e-mail: [email protected]

shortening, in a walking child has been noticed to induce femoral overgrowth which compensates for the discrepancy and may obviate the need for tibial lengthening in young children during treatment of tibial shortening.

Brief Clinical History A 3 year old female child is diagnosed as having congenital pseudarthrosis of the tibia associated with neurofibromatosis-1 (NF-1). Previous surgical treatment using intramedullary rod and Ilizarov frame for bifocal compression–distraction of the pseudarthrosis and proximal tibial osteotomy were performed. No bone grafting was done, and the pseudarthrosis failed to unite.

Preoperative Clinical Photos and Radiographs See Fig. 1.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_279

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Fig. 1 (a) Preoperative radiographs show the result of previous surgical treatment using intramedullary rod and Ilizarov frame for compression– distraction of the pseudarthrosis and proximal tibial osteotomy. (b, c)

Preoperative Problem List 1. Atrophic bone ends and mobile pseudarthrosis indicate poor biological environment at the site of pseudarthrosis. 2. Previous surgery invites fibrous tissue formation and scarring and adds to the compromised blood supply. 3. Short bone segments and the presence of tibial physes which make fixation difficult. 4. The expected limb shortening after approximation of bone ends. 5. The presence of an intact but dysplastic fibula holding the tibial bone ends wide apart.

M. A. El-Rosasy

Radiographs after removal of IMR show CPT type 2 (after El-Rosasy– Paley classification of CPT ), atrophic bone ends, mobile pseudarthrosis, and previous surgical interference

into the longitudinally split end of the proximal segment so as to increase the area of bone contact and increase the crosssectional diameter of the tibia. (2) Fibular osteotomy is done to allow leg shortening. The fibular osteotomy is to be done through the healthy diaphysis above the level of the dysplastic segment. No fibular resection is done, and the bone ends are overlapped to unite and enforce the dysplastic segment. (3) Tibial fixation is performed using a transcalcaneal Rush rod. (4) Autogenous iliac crest bone graft is applied around the pseudarthrosis. (5) A two-ring Ilizarov frame is applied for axial compression and to prevent rotational and shear strains.

Basic Principles Treatment Strategy According to the treatment protocol based on our classification system, the procedure includes the following: (1) Acute limb shortening is performed to wedge the tip of the distal segment

1. 2. 3. 4.

Excision of soft tissue fibromatosis. Stimulation of bone healing by autogenous ICBG. Proper fixation under compression. Protect healed bone by retaining the IMR as a permanent splint.

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Images During Treatment

Outcome Clinical Photos and Radiographs

See Figs. 2 and 3.

See Figs. 4 and 5.

Technical Pearls

Avoiding and Managing Problems

1. Avoid aggressive bone resection, only debridement of dead bone. 2. The IMR used should span the whole length of the tibia to avoid refracture at the tip of a short rod. 3. Due to the short bone segments in small children, a proximal lengthening osteotomy is not desirable; moreover, such leg shortening would be compensated in walking children due to femoral overgrowth as evidenced by the different knee levels and coxa valga, so the total limb lengths would be equal. 4. The Rush rod could be left a little long in the heel so that, when the tibia starts to outgrow the rod, the distal end of the rod could be hammered through a small stab incision in the heel to avoid rod exchange while the tibial union is still attenuated.

In such young children, a proximal tibial lengthening for bone transport would complicate the procedure due to small bone segments and osteoporotic bone. Leaving intact fibula does not allow good tibial bone contact; however, a fibular osteotomy should be performed in a healthy segment, and the bone ends are allowed to overlap. The use of half pins should be avoided except in the metaphysis of the tibia in older children, to avoid stress fracture after pin extraction due to the weak healing of the tibia in these cases.

Fig. 2 (a, b) Postoperative radiographs show (1) fibular osteotomy and overlap of bone ends, (2) impalement of the distal tibial bone end into the proximal one, (3) insertion of a Rush rod spanning the whole tibia with extra length in the heel, and (4) application of a two-ring Ilizarov frame for axial compression with an extra ring below the foot for weight bearing

Fig. 3 (a, b) Follow-up radiographs show consolidation of the pseudarthrosis maintained by the Rush rod, continued growth of the tibia (evidenced by the outgrown rod). (c) Composite X-rays show overgrowth of the ipsilateral femur (evidenced by the coxa valga and different knee levels) which compensated for the 3 cm shortening of the tibia with equal total limb lengths

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Fig. 4 (a, b) Radiographs after hammering the rod from the heel without disturbance of the united pseudarthrosis. The pseudarthrosis is consolidated, and the tibial cortex is well formed and remodeled

Fig. 5 (a, b) Clinical photos show a fully active child with well-aligned leg and equal limb length

M. A. El-Rosasy

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Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 2)

171

Cross-References

References and Suggested Reading

▶ Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum) ▶ Congenital Pseudarthrosis of Tibia ▶ Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 3) ▶ Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application

1. El-Rosasy MA, Paley D, Herzenberg JE. Congenital pseudarthrosis of the tibia. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York/London: Informa Healthcare Publisher; 2006. p. 485–93. 2. Onwuasoigwe O. Longitudinal overgrowth of the femur stimulated by short-leg ambulation in unilateral partial tibia hemimelia. J Pediatr Orthop B. 2013;22(4):357–62. 3. Thabet AM, Paley D, Kocaoglu M, Eralp L, Herzenberg JE, Ergin ON. Periosteal grafting for congenital pseudarthrosis of the tibia: a preliminary report. Clin Orthop Relat Res. 2008;466(12):2981–94.

Congenital Pseudoarthrosis of the Tibia Repaired with Massive Autograft and Tibia-Fibula Cross-Union

27

Taylor J. Reif and S. Robert Rozbruch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177

Abstract

This case presents a skeletally mature patient with an ongoing pseudoarthrosis of the right tibia after several unsuccessful attempts at obtaining union during his childhood. He was treated with revision fixation and massive bone grafting of the right tibia and fibula to obtain not only tibia and fibula union but additionally a cross-union between the bones for enhanced and durable stability. The bone graft was supplemented with catabolic suppression using zoledronic acid and anabolic support using BMP-2.

T. J. Reif (*) Director of Education, Limb Lengthening and Complex Reconstruction Service, Hospital for Special Surgery, New York, NY, USA

Brief Clinical History The patient is a 16-year-old male with neurofibromatosis type 1 with congenital pseudoarthrosis of the right tibia. He had underwent multiple attempts at bone union at the pseudoarthrosis site over his childhood, with attempts at bone grafting and nonlocked intramedullary fixation. He also had the right tibia lengthened proximally with an external fixator to manage a large limb length discrepancy. He ambulated with a Charcot restraint orthotic walker (CROW)-type kneeankle-foot orthosis (KAFO) with 8 cm external lift with no pain but worsening deformity of the foot and ankle.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Weill Cornell Medical College, Cornell University, New York, NY, USA e-mail: [email protected] S. R. Rozbruch Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA e-mail: [email protected] © Springer International Publishing Switzerland (outside the USA) 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_401

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174 Fig. 1 (a, b) Standing photographs of the patient, demonstrating 8 cm LLD, healed incisions from prior surgeries, and procurvatum and valgus of the distal tibia

Fig. 2 (a, b) AP and lateral radiographs of the distal tibia, demonstrating prior tibial intramedullary rod with associated blocking screws, retained broken fibular wire, and valgus and procurvatum deformity of the distal tibia through the tibial and fibular nonunions

T. J. Reif and S. R. Rozbruch

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Congenital Pseudoarthrosis of the Tibia Repaired with Massive Autograft and Tibia-Fibula Cross-Union

175

Preoperative Problem List 1. 2. 3. 4. 5. 6.

Neurofibromatosis type 1 Pseudoarthrosis of right tibia and fibula Multiplanar deformity of the distal tibia and fibula Retained implants Multiple scarred areas along leg from prior surgeries 8 cm LLD

Treatment Strategy The pseudoarthrosis of the distal tibia needs additional biology and stability in order to heal. To accomplish this, the hamartoma around the tibia is excised, and massive bone grafting and periosteal membrane harvest from the ipsilateral pelvis are used to create a tibiofibular cross-union stabilized with a locked intramedullary nail. Zoledronic acid is used to reduce bone graft resorption, and BMP-2 is also used to promote maturation and incorporation of the autograft to the cross-union construct. The deformity is corrected with a new intramedullary nail and blocking screws. The leg length discrepancy will be addressed in a staged manner after solid cross-union is obtained.

Basic Principles • The pseudoarthrosis of the tibia is a periosteal disease, so excision of the hamartoma surrounding the tibia and addition of healthy periosteum (from the ilium) is critical to facilitate bone healing [1]. • The periosteum also has increased osteoclastic activity and reduced osteoblastic response to bone morphogenic protein (BMP), so perioperative treatment with systemic zoledronic acid and local BMP-2 reduce bone catabolism and increase the anabolic stimulus, respectively [2]. • To provide sufficient bone graft for the cross-union, the ipsilateral ilium is opened and cancellous graft harvested. • Stability of the bone is achieved with the largest intramedullary implant feasible; in this case, a locked solid nail was able to be utilized.

Images During Treatment See Figs. 3, 4, and 5.

Fig. 3 To correct the deformity, the fracture was opened and the small distal bone segment was centrally reamed over a wire to allow proper positioning of the nail

Technical Pearls • A wide exposure is needed to remove all the hamartoma around the tibia and fibula. • In a small bone segment like the distal tibia in this case, use all locking holes and enhance stability further by using blocking screws in the coronal and sagittal plane. When placed prior to the nail, the blocking screws can also guide the nail to the optimal position for anatomic alignment. • Enhancing fibular stability with an intramedullary wire or plate is recommended to support the lateral aspect of the construct. • Harvest the iliac periosteum from the inner table prior to the cancellous bone harvesting. It can be meshed to increase its coverage area. • Given the large amount of graft harvested from the iliac wing, it can be backfilled with cancellous allograft chips to support new bone growth.

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T. J. Reif and S. R. Rozbruch

Fig. 4 (a–d) Graphic rendering of the bone graft strategy, including placement of the meshed iliac periosteum (a), placement of cancellous graft (b), placement of BMP-2 collagen sponges (c), and axial representation of layers (d)

Fig. 5 (a, b) Postoperative AP and lateral radiographs demonstrating the tibial locked intramedullary nail with associated blocking screws and lateral fibular plate. The leg was placed in a cast for the first 3 months with strict non-weightbearing

Outcome Clinical Photos and Radiographs

Avoiding and Managing Problems

See Figs. 6 and 7.

• The anterior compartment and associated neurovascular bundle must be fully mobilized to facilitate safe hamartoma removal and subsequent bone grafting.

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Fig. 7 AP radiograph 6 months postoperative demonstrating further consolidation of the crossunion and healing of the tibial and fibular nonunions

Fig. 6 (a, b) AP and lateral radiographs 4 months after surgery demonstrating healing of the cross-union and excellent alignment of the distal tibia

• Zoledronic acid is given in an IV infusion, once prior to surgery, and another dose 3 months later. • Given the periosteum and bone is abnormal in a pseudoarthrosis, expect longer time to consolidation than typical reconstructive procedures, even with autograft and BMP-2 application. This patient was kept NWB in a cast for 3 months and then transitioned back to a CROW boot with shoe lift for ongoing support. • This is a lengthy reconstructive procedure with many steps, so communicate with anesthesia prior to surgery

so the appropriate patient safety measures are in place prior to incision.

References and Suggested Reading 1. Paley D. Congenital pseudarthrosis of the tibia: biological and biomechanical considerations to achieve union and prevent refracture. J Child Orthop. 2019;13:120–33. https://doi.org/10.1302/1863-2548. 13.180147. 2. Schindeler A, Birke O, Yu NYC, et al. Distal tibial fracture repair in a neurofibromatosis type 1-deficient mouse treated with recombinant bone morphogenetic protein and a bisphosphonate. Bone Joint J. 2011;93-B:1134–9.

Congenital Tibial Pseudarthrosis Managed with Ipsilateral Vascularized Fibula Transfer

28

Michael Aiona

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183

Abstract

Congenital pseudarthrosis is a difcificult clinical challenge with upwards of 40–50 % amputation rate. Multiple procedures are available to the clinician for management. The area of nonunion is thought to be “avascular” and contributes to the failure of traditional bone grafting techniques. Many reports describe the use of a contralateral vascularized fibula transfer to achieve union. This requires operative intervention on the normal limb with its inherent potential complications. This case describes the use of an ipsilateral vascularized fibula, avoiding the potential morbidity on the contralateral limb as the source of the graft and the greater technical difficulty with a free transfer. The requirement of a “good” ipsilateral fibula limits patient eligibility.

M. Aiona (*) Shriners Hospital for Children, Portland, OR, USA e-mail: [email protected]

Brief Clinical History The patient presented at 9 months of age with a deformity of the tibia first noted by the mother at 4 months of age. No history of trauma was noted. No known family history of neurofibromatosis or similar deformity was elucidated. Physical exam demonstrated the anterior lateral bow of the tibia along with knee, thigh, and trunk café-au-lait spots noted. The foot was plantigrade and no pain was elucidated with palpation (Fig. 1a, b). No spinal deformity was present. X-rays of the tibia demonstrate the classic tibia pseudarthrosis, associated bowing, and deformity (Fig. 2a, b). Initial management was a clamshell orthosis to provide support and stability. The patient managed well and was ambulatory with the brace. At almost 3 years of age, because of difficulty in managing the deformity, several options for management were discussed [2]. The chosen surgical treatment was pseudarthrosis resection, bone grafting, and IM rodding as reported by Dobbs et al. [1]. In spite of bracing, failure of IM rod occurred secondary to failure of healing of pseudarthrosis repair (Fig. 3).

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_329

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180 Fig. 1 Clinical photograph, frontal (a) and side (b) view of depicting the shortening and angulation of the leg

Fig. 2 Initial radiograph at presentation, AP (a) and lateral (b) views demonstrating the classic appearance of tibial pseudarthrosis with deformity. Note the intact fibula

M. Aiona

28

Congenital Tibial Pseudarthrosis Managed with Ipsilateral Vascularized Fibula Transfer

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Preoperative Problem List – Persistent pseudarthrosis despite resection, grafting, and IM nail – Angular deformity of the tibia – Limb length inequality

Treatment Strategy The persistent nonunion with now failure of the rod poses questions for further management. Multiple options are potentially available to manage the persistent pseudarthrosis [2]. Among them are: • Repeat grafting (use of BMP) and IM rod replacement [4, 6]

181

• Resection of pseudarthrosis, proximal tibial osteotomy, and bone transport with external fixation device • Vascularized contralateral fibula transfer with IM stabilization [3] • Ipsilateral vascularized fibular transfer [5] • Syme amputation Since failure of the previous intervention of resection and grafting occurred, an alternative approach was considered. As the child was only 7 years old, it was felt that the advantage of lengthening with the bone transport added a more complicated and prolonged treatment timeline as the limb length inequality could be addressed in the future because of the amount of remaining growth. Unique to this case is the intact fibula despite the tibial nonunion. One could argue that this was preventing union, but the fibula was osteotomized at the initial resection and grafting. Though most reports in the literature describe the contralateral fibula as the donor site for a vascularized transfer, this case afforded the opportunity to operate solely on the pathologic leg.

Basic Principles – – – – –

Resect pseudarthrosis Stabilize and realign tibia IM rodding spanning at minimum tibial–talar joint Bone grafting at site of pseudarthrosis Continued use of postoperative clamshell orthosis with solid ankle

Images During Treatment See Fig. 4.

Technical Pearls • Adequate resection of tibial pseudarthrosis site, including the surrounding soft tissues. • IM rodding performed retrograde by placing rod from the distal tibial fragment through the ankle and foot. Important to hold the ankle in correct position to avoid too much dorsiflexion of the calcaneus. • Stabilization of the ankle with internal fixation. • Remove above knee cast at 6 weeks, measure for new clamshell orthosis, place below the knee walking cast for 4 weeks, remove and wear orthosis. Fig. 3 A 20-month postoperative radiograph – AP (right) and lateral (left) X-rays demonstrating persistent pseudarthrosis and rod failure. Once again, note the intact fibula

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M. Aiona

Fig. 4 Immediate postoperative X-ray – AP (a) and lateral (b) views demonstrating the transposition of the fibula to the tibia, stabilized by IM rod and cerclage wires

Outcome Clinical Photos and Radiographs See Figs. 5 and 6.

Avoiding and Managing Problems Continued careful follow-up and adherence to brace wear are imperative till skeletal maturity. As the patient grows, the rod should migrate proximally as noted in this case. The internal fixation of the ankle has also migrated proximal and ankle valgus is now present. As the patient is immature, guided growth can realign the ankle without the need for osteotomy.

Fig. 5 Five-year follow-up X-ray – AP (b) and lateral (a) views demonstrating incorporation of graft, spanning the pseudarthrosis, though ankle valgus is now noted

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Fig. 6 Clinical photograph – frontal (a) and back (b) views of the leg, note the well-aligned leg with no evidence of bowing though muscle atrophy and shortness still are present

Cross-References ▶ Avoiding Amputation and Prosthetics in Children with Complex Lower Limb Deformities ▶ Three Year Old Female with Segmental Bone Defect due to Grade IIIB Open Tibial Fracture Treated by Oblique Wire Bone Transport

See Also in Vol. 3 Acute Shortening and Re-lengthening to Bridge Bone Defects

References and Suggested Reading 1. Dobbs MB, Rich MM, Gordon JE, Szymanski DA, Schoenecker PL. Use of an intramedullary rod for treatment of congenital

pseudarthrosis of the tibia. A long-term follow-up study. J Bone Joint Surg Am. 2004;86-A(6):1186–97. 2. Grill F, Bollini G, Dungl P, Fixsen J, Hefti F, Ippolito E, Romanus B, Tudisco C, Wientroub S. Treatment approaches for congenital pseudarthrosis of tibia: results of the EPOS multicenter study. European Paediatric Orthopaedic Society (EPOS). J Pediatr Orthop B. 2000;9(2):75–89. 3. Iamaguchi RB, Fucs PM, da Costa AC, Chakkour I. Vascularised fibular graft for the treatment of congenital pseudarthrosis of the tibia: long-term complications in the donor leg. Int Orthop. 2011;35 (7):1065–70. https://doi.org/10.1007/s00264-010-1103-8. 4. Spiro AS, Babin K, Lipovac S, Stenger P, Mladenov K, Rupprecht M, Rueger JM, Stuecker R. Combined treatment of congenital pseudarthrosis of the tibia, including recombinant human bone morphogenetic protein-2: a case series. J Bone Joint Surg (Br). 2011;93 (5):695–9. 5. Tan JS, Roach JW, Wang AA. Transfer of ipsilateral fibula on vascular pedicle for treatment of congenital pseudarthrosis of the tibia. J Pediatr Orthop. 2011;31(1):72–8. 6. Woo EJ. Adverse events after recombinant human BMP2 in nonspinal orthopaedic procedures. Clin Orthop Relat Res. 2013;471 (5):1707–11. https://doi.org/10.1007/s11999-012-2684-x. Epub 2012 Nov 7.

Congenital Tibial Pseudoarthrosis Treated with Internal and External Fixation Using the 4 in 1 Technique

29

Pablo Wagner and John E. Herzenberg

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189

Abstract

A 5 year old female presents with idiopathic congenital pseudarthrosis of the tibia and fibula (CPT). Four years ago, she had ORIF with bone graft, an internal rod, and a circular external fixator. The tibial nonunion failed to heal and she underwent revision surgery 3 years ago with bone graft, Rush rod application across the foot, external fixation, and bone grafting using the 4 in 1 technique, supplemented with postoperative zoledronate. Solid healing was achieved. One year later, the Rush rod was removed to free the ankle joint, and it was replaced with a Fassier-Duval growing rod. At 12 months follow-up, she had 20 of dorsiflexion and 45 of plantar flexion with solid healing of the tibia and fibula and cross union.

P. Wagner (*) · J. E. Herzenberg Rubin Institute for Advanced Orthopedics, International Center for Limb Lengthening and Reconstruction, Sinai Hospital of Baltimore, Baltimore, MD, USA e-mail: [email protected]; [email protected]; [email protected]

Brief Clinical History Patient is a 5 year old female with congenital tibial pseudarthrosis (CPT). At 1 year of age, she had a surgery for a tibial nonunion using massive cancellous iliac crest bone graft, BMP, internal rodding, and external fixator application. This failed to unite. During the postoperative phase, the massive cancellous bone graft resorbed. One year after that surgery, a revision surgery was performed using the 4 in 1 bone grafting technique, with IM rodding across the ankle, repeat ring external fixator application, and zoledronate supplementation.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_313

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186

Fig. 1 Initial presentation

P. Wagner and J. E. Herzenberg

Fig. 3 AP tibia X-ray: first treatment with external fixator, BMP, iliac crest graft

Fig. 2 Lateral tibia X-ray: first treatment with external fixator, BMP, iliac crest graft

Fig. 4 AP tibia X-ray after external fixator removal, A tibio-fibular non union can be seen

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Preoperative Problem List Tibial and fibular nonunion (CPT)

Treatment Strategy • • • • •

Bicortical iliac crest bone harvest Nonunion resection Axial stabilization with tibio-talo-calcaneal Rush rod Bone graft application, “4 in 1” technique External fixation application

Basic Principles CPT is difficult to treat, having frequent refractures and nonunions resistant to treatment. Combining external and internal fixation methods, a high rate of union and a low rate of refracture can be achieved. The external fixation provides rotational stability and the internal fixation provides axial stability. Other important keys to success include a large volume of bone graft, BMP/periosteal graft to encourage bone healing, and zoledronate to inhibit bone resorption. Fig. 5 Intraoperative image: reaming of tibia from the calcaneus, wire in the fibula

Images During Treatment See Figs. 5 and 6.

Technical Pearls Place a tibio-talo-calcaneal Rush rod and a K-wire for the fibula. The tibio-talo-calcaneal (TTC) internal rod provides a greater stability than just a tibial rod. The downside of the TTC construct is that it blocks ankle motion. However, we have found that the TTC rod can be removed after solid healing of the nonunion. It can be replaced by a FassierDuval nail and ankle motion can be restored. Harvest a 5 cm tricortical iliac crest graft and split it to obtain 2 unicortical grafts. Position them anteriorly and posteriorly between the tibia and fibula surrounded by a large cancellous bone graft (4 in 1 technique). Drill 2 small holes in the cortical graft with K-wires to suture the graft in position.

Outcome Clinical Photos and Radiographs See Figs. 7, 8, 9, and 10.

Fig. 6 Postoperative X-ray: internal fixation, external fixation, 4 in 1 graft technique [2]

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Fig. 7 Healed tibia on lateral view

P. Wagner and J. E. Herzenberg

Fig. 9 After external fixator removal and Fassier-Duval application: lateral view Fig. 10 After external fixator removal and Fassier-Duval application: AP view

Fig. 8 Healed tibia on AP view

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Congenital Tibial Pseudoarthrosis Treated with Internal and External Fixation Using the 4 in 1 Technique

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Avoiding and Managing Problems

Cross-References

The risk of nonunion and refracture can be minimized with the use of combined pharmacologic and surgical strategy. Resect the hamartoma/periosteum until normal appearance. The larger bone segment can be split and the smaller segment can be invaginated into it, creating a wide cross-sectional area for healing. Autogenous graft harvested from the iliac crest is taken as a bicortical solid graft, which is split into inner and outer tables. These tables are left intact and are placed anteriorly and posteriorly around the nonunion, like a “sandwich.” A cancellous bone is packed inside the sandwich along with BMP. The periosteum is harvested from the iliac crest inner table, meshed, and wrapped around the nonunion. Zoledronic acid is administered to prevent graft resorption given the hyperactivity of osteoclasts present in CPT/NF. An internal rod is left to prevent refracture and an external fixator is placed to control rotation and to add stability to aid with bone healing. The final goal in CPT is not just to achieve bone union of the tibia and fibula but to achieve a crosssectional union with the widest possible diameter of bone, to prevent future fractures.

▶ Congenital Pseudarthrosis of the Fibula ▶ Congenital Pseudarthrosis of Tibia ▶ Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 2) ▶ Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 3)

References and Suggested Reading 1. Agashe MV, Song SH, Refai MA, Park KW, Song HR. Congenital pseudarthrosis of the tibia treated with a combination of Ilizarov’s technique and intramedullary rodding. Acta Orthop. 2012;83(5):515–22. 2. Choi IH, Lee SJ, Moon HJ, Cho TJ, Yoo WJ, Chung CY, Park MS. “4-in-1 osteosynthesis” for atrophic-type congenital pseudarthrosis of the tibia. J Pediatr Orthop. 2011;31(6):697–704. 3. Khan T, Joseph B. Controversies in the management of congenital pseudarthrosis of the tibia and fibula. Bone Joint J. 2013;95-B (8):1027–34. 4. Thabet AM, Paley D, Kocaoglu M, Eralp L, Herzenberg JE, Ergin ON. Periosteal grafting for congenital pseudarthrosis of the tibia: a preliminary report. Clin Orthop Relat Res. 2008;466(12):2981–94.

Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application

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Reggie C. Hamdy and Franc¸ois Fassier

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 191 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 191 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 193 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 193 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196

Abstract

Brief Clinical History

This is a case of congenital pseudarthrosis of the tibia in an 8 year old boy who presented with a non-displaced fracture of his tibia. Surgical treatment included realignment of the tibia with double osteotomies, excision of the pseudarthrosis site, internal fixation of the tibia with a Fassier-Duval rod, cancellous bone graft from the iliac crest, and OP-1 (BMP7) application to both osteotomy sites. At the last follow-up, 7 years following the surgery, both sites remain healed with good alignment. The patient is completely asymptomatic and functioning well with a full-contact AFO. He is scheduled for a contralateral distal femoral epiphysiodesis to correct a limb length discrepancy of 2.5 cm.

An 8 year old boy was admitted to the hospital for surgical treatment of congenital pseudarthrosis of his left tibia. He was first seen at the age of 2 because of anterolateral bowing of his tibia. He was completely asymptomatic at that time. A workup revealed that he has neurofibromatosis Type 1 with typical skin manifestations of café au lait spots. He was prescribed a full-contact ankle-foot orthosis with no restrictions of his activities. He was followed on a yearly basis until he was admitted at the age of 8 because of increasing pain in his left leg and inability to weight bear after he sustained a non-displaced fracture of his tibia.

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

F. Fassier Shriners Hospitals and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_22

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Fig. 1 At 2 years of age, AP and lateral view of the left leg showing anterolateral bowing and intact fibula

Preoperative Problem List 1. Double-level anterolateral bowing with non-displaced fracture as well as pseudarthrosis of the distal fibula 2. Limb length discrepancy of 2.0 cm 3. Expected difficulties in obtaining union in cases of congenital pseudarthrosis of the tibia 4. Expected problems in maintaining the union until skeletal maturity with possibility of refracture

Treatment Strategy After the patient was first seen at the age of 2, it was decided to postpone surgical intervention until he fractures or until the deformity becomes so severe that it interferes with his function. Once he fractured, then the same basic principles used in the management of any case of frank CPT were followed and included:

Fig. 2 At 4 years of age, AP and lateral views of the left leg showing essentially no increase in the deformity. However, distal fibula is starting to develop dysplastic changes

1. Double-level osteotomy of the tibia at the proximal tibia and junction of middle to distal third of the tibia (where the bone is most affected). 2. Perform the proximal osteotomy percutaneously as no excision is necessary at that site. 3. Perform excision of the pseudarthrosis site at the second distal osteotomy. 4. Addition of cancellous bone grafting from the iliac crest with cortico-cancellous chips. 5. Addition of biological stimulator (OP-1). 6. Realignment with a Fassier-Duval rod. We have decided to use an intramedullary rod as the primary technique of stabilization, as we felt that minimal excision would be required and hence approximation of the two fragments would not present a problem. Furthermore, the distal fragment was long enough to provide good purchase

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Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity. . .

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Fig. 3 (a) At age of 8 years, AP and lateral views showing a fracture at the middle – distal tibia and frank pseudarthrosis of the distal fibula. (b) AP view showing double-level deformity at the proximal and distal tibia

and stability with the FD rod. Also, with a lengthening rod, such as FD, it is not necessary to change the rod with growth and the rod could stay permanently, providing stability and preventing refracture.

Basic Principles The basic principles in the treatment of congenital pseudarthrosis include: 1. Excision of the pseudarthrosis site and surrounding tissues. 2. Realignment and stable fixation of the tibia. 3. Autogenous cancellous bone grafting as well as biological augmentation with BMP2 or BMP7 (OP-1).

4. Internal fixation of the fibula. 5. Prolonged external support with cast or full-contact brace until union is achieved. 6. After healing is obtained, maintain intramedullary nail. 7. Continuation of external support with a full-contact AFO until skeletal maturity. 8. Correction of any associated problems other than the healing of the pseudarthrosis such as ankle valgus, limb discrepancy and fibular nonunion.

Images During Treatment See Figs. 4, 5, and 6.

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Fig. 4 (a, b) Showing AP and lateral views of the tibia following a double-level osteotomy and internal fixation with a FassierDuval rod – size 4 mm rod

Technical Pearls

Outcome Clinical Photos and Radiographs

1. Once a fracture occurs through the pseudarthrosis site, even if non-displaced, the site needs to be excised. 2. When using the FD rod, the female rod has to be cut shorter in order to allow some compression of the bony fragments following the realignment osteotomy. If this is not taken into consideration, then this collapse will cause the female rod to protrude in the knee – as was the case in this patient. 3. As the Fassier-Duval rod does not provide enough rotational stability, the leg should be immobilized either in a cast or a slab and, once fusion occurs, in a total contact AFO.

See Figs. 7 and 8.

Avoiding and Managing Problems 1. Whether fibular osteosynthesis should have been done or not could be debated. Advantages of fibular osteosynthesis include: (1) giving more stability to the rodding and (2) providing lateral support, thereby decreasing or preventing the development of ankle valgus. 2. If the distal fragment is too short, the use of a FD rod is contraindicated as this may cause instability. 3. In retrospect, prophylactic posteromedial bypass grafting could have been performed. This may have avoided the fracture.

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Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity. . .

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Fig. 7 (a, b) One year and 5 months after the initial surgery, showing complete healing of the proximal osteotomy but incomplete healing of the distal osteotomy Fig. 5 (a, b) AP and lateral views 4 months after the surgery, showing incomplete healing at both levels and protrusion of the female rod in the knee

Fig. 6 Lateral view showing reinsertion of the female rod

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Cross-References ▶ Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with Telescoping Fassier-Duval Rods ▶ Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia

References and Suggested Reading 1. Dohin B, Dahan-Oliel N, Fassier F, Hamdy R. Enhancement of difficult nonunion in children with osteogenic protein-1 (OP-1). Clin Orthop Relat Res. 2009;467:3230–8. 2. Johnston CE, Birch J. A tale of two tibias: a review of treatment options for congenital pseudarthrosis of the tibia. J Child Orthop. 2008;2:133–49. 3. Khan T, Joseph B. Instructional review: children’s orthopaedics. Controversies in the management of congenital pseudarthrosis of the tibia and fibula. Bone Joint J. 2013;95-B(8):1027–34. 4. Vander KL, Hensinger RN, Caird M, Johnston C, Farley F. Congenital pseudarthrosis of the tibia. J Am Acad Orthop Surg. 2008;16:228–36.

Fig. 8 (a, b) Two years and 5 months after the initial surgery, showing complete healing of both osteotomy sites

Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia

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Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 198 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 198 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 201 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 201 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 203 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 203

Abstract

This is a case of a 14 year old patient with a severe, neglected congenital pseudarthrosis of the tibia (CPT). She did not have any prior treatment for this condition. She underwent excision of the pseudarthrosis, application of a hybrid Ilizarov – Taylor Spatial Frame, autogenous bone grafting, and OP-1 application. She had five subsequent surgeries until bony fusion was obtained. The last surgery included the insertion of an intramedullary female Fassier-Duval rod through the ankle joint. Associated problems included a limb discrepancy of 14 cms. After obtaining fusion at the pseudarthrosis site, she underwent femoral lengthening of 8 cms and is functioning now with a shoe lift. This case exemplifies the complexity and numerous challenges in the management of CPT.

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

Brief Clinical History A 14 year old patient presented to the outpatient clinic with a severe angular deformity of her tibia. She had not had any previous treatment. A genetics workup revealed that she had neurofibromatosis type 1. The patient underwent excision of the pseudarthrosis, Ilizarov reconstruction, autogenous bone grafting, and OP-1 application. Subsequently, the patient was taken back twice to the operating room for irrigation and debridement of an acute infection (at 14 and 20 days after the index surgery). Furthermore, threaded rods were added to allow further stabilization between the distal and the most proximal rings. The patient subsequently had two other revision surgeries with application of OP-1 (as well as a TCP granule chronos grafting) for failure of healing at the pseudarthrosis site. The Taylor Spatial Frame was removed and a female Fassier-Duval rod was inserted through the os calcis in order to provide rigid fixation. Following this, the pseudarthrosis site completely healed and the patient was able to fully weight bear with a lift. She subsequently had a femoral lengthening and remains with an LLD of 6.0 cms.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_23

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Preoperative Clinical Photos and Radiographs

Preoperative Problem List

See Figs. 1 and 2.

1. Severe pseudarthrosis of the tibia and fibula with severe angular deformity and atrophy of the bone ends. This situation may necessitate excision of a large amount of bone which may subsequently leave a large gap.

Fig. 1 (a and b) Showing the severe angular deformity as well as the severe contractures of the posterior structures and tendo Achilles

Fig. 2 (a) Severe atrophic type of pseudarthrosis. (b) Size of limb length discrepancy

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Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia

2. The pseudarthrosis is in the distal tibia – rendering fixation of the distal fragment problematic. This may require fixation of the ankle. 3. Poor skin condition over the tip of the pseudarthrosis as the patient was bearing partial weight on the tip of the pseudarthrosis. This increases the likelihood of infection. 4. There is a significant LLD of 14 cm. 5. Soft tissue contractures of the posterior structures are present. 6. The ankle joint may have poor function as this has been subjected to abnormal pressures for many years.

Treatment Strategy This is not an easy case! The presence of such a severe deformity, further complicated by the late age at presentation, renders the management of this particular patient quite challenging. The first goal was to obtain union. The basic principles in the management of any case of CPT, listed below, were followed meticulously. 1. Resection of the pseudarthrosis and the surrounding fibrous and hamartomatous tissue. 2. Soft tissue release posteriorly. 3. Realignment of the leg by trying to get an acute correction and apposition of the bone ends. 4. Cancellous bone grafting from the iliac crest together with cortico-cancellous chips of bone. 5. Biological augmentation with OP-1. 6. Stabilization of the tibia with a hybrid Ilizarov/TSF fixator. We decided to use an Ilizarov reconstruction technique for the following reasons: • Need for correction of the limb discrepancy by lengthening the proximal tibia – either simultaneously or at a second setting. • If the proximal and distal fragments could not be approximated or compressed after excision of the pseudoarthrosis, then these two rings could be used for bone transport. • Further correction of any residual deformities at the pseudarthrosis site, specifically procurvatum (apex anterior) of the distal fragment and ankle valgus. • Extension of the frame to include the ankle joint in order to obtain further stabilization of the distal tibial fragment. It was expected that the distal fragment would be too short to obtain stable fixation without spanning the ankle joint.

199

Basic Principles The basic principles in the management of CPT are, first, to obtain union, second, to maintain union, and, third, to have a functional extremity. There are many techniques used in the surgical treatment of CPT; however, regardless of the technique used, the basic principles to obtain union remain the same and should include: 1. Excision of the pseudarthrosis site and all the surrounding diseased tissue (fibrosed and hamartomatous tissue). It may be difficult to judge how much to excise. 2. Autogenous cancellous bone grafting with or without cortico-cancellous bone chips. 3. Stable fixation of the tibia. 4. Fixation of the fibula should be performed. 5. After obtaining union, the permanent insertion of an intramedullary rod and the permanent use of a brace are strongly recommended to maintain union and prevent refracture. 6. Associated deformities (limb discrepancy and ankle valgus) should be addressed either simultaneously or later.

Images During Treatment See Figs. 3, 4, 5, and 6.

Technical Pearls 1. This is a severe atrophic type of pseudarthrosis where a large resection is needed. The decision of when to stop the excision may not be easy and clinical judgment is needed. On one hand, it is important to obtain bleeding bone. On the other hand, care must be taken not to remove bone unnecessarily which may leave a huge gap to reconstruct. 2. One of the key points to promote successful healing is to obtain a large healing contact area after the resection is complete. 3. Use everything (all resources) possible in order to maximize the chances for union. 4. In this specific case, it is better to apply an external fixator whether it is a standard Ilizarov circular fixator, TSF, or a hybrid Ilizarov/TSF fixator in order to allow correction of residual deformities specifically procurvatum of the distal fragment and any residual ankle valgus.

200 Fig. 3 Intraoperative pictures showing the tibia after resection of the pseudarthrosis (a) and after filling the defect with cancellous bone graft and with OP-1 (b)

Fig. 4 Details of surgical technique used: excision of the pseudarthrosis site (a), filling the gap with cancellous bone graft from the iliac crest mixed with OP-1 (recombinant BMP-7) (b), application of cortico-cancellous bone chips around the gap (c), and cerclage vicryl sutures to try to hold the bone graft in place (d)

R. C. Hamdy

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Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia

201

5. It is essential to stabilize the ankle and include it in the frame as the distal fragment is too small to allow for any stability without having to immobilize the ankle. The other alternative is to use an intramedullary nail through the ankle. (This was done later once all the deformities had been corrected.) 6. Stabilization of the fibula. 7. After removal of the fixator, insertion of an intramedullary rod that will remain permanent. 8. The lifelong use of a brace.

Outcome Clinical Photos and Radiographs See Figs. 7, 8, and 9.

Fig. 5 The two fragments are re-approximated with a K-wire prior to the application of the external fixator

Fig. 6 Showing radiographs with good alignment and correction of the deformity

Avoiding and Managing Problems The first thing to do in the management of such cases is to have a long discussion with the patient and family, explaining very clearly the numerous challenges with this condition, the expected difficulties in obtaining union, the difficulties in maintaining union, and the difficulties in obtaining a functional limb. Numerous surgeries may be required to achieve these goals. Follow meticulously the basic principles and have a clear preoperative plan. In severe, neglected cases like the present one with multiple associated problems, it is better to use an Ilizarov reconstruction technique. This allows the numerous issues to be addressed at the same setting. It also provides a construct to manage any unexpected problems that may arise during the treatment.

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Fig. 7 (a, b and c) External fixator construct used: middle segment consisting of a TSF with the distal ring attached to the distal fragment with a 1.8 olive K-wire and a half pin, proximal segment consisting of TSF rings connected with standard Ilizarov rods for proximal

Fig. 8 (a and b) Final X-rays showing healing of the pseudarthrosis site and intramedullary fixation of the tibia with a female Fassier-Duval rod and intramedullary fixation of the fibula

R. C. Hamdy

lengthening, and distal segment or foot frame including an open TSF ring connected to the middle segment with standard Ilizarov hinges and attached to the os calcis with a 4 mm half pin and a 1.8 olive K-wire

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Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia

203

Fig. 9 (a–d) Showing significant amount of limb length discrepancy and full correction of the deformity

Cross-References ▶ Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application

References and Suggested Reading 1. Choi IH, Cho T-J, Moon HJ. Ilizarov treatment of congenital pseudarthrosis of the tibia: a multi-targeted approach using the Ilizarov technique. Clin Orthop Surg. 2011;3:1–8.

2. Dohin B, Dahan-Oliel N, Fassier F, Hamdy R. Enhancement of difficult nonunion in children with Osteogenic Protein-1 (OP-1). Clin Orthop Relat Res. 2009;467:3230–8. 3. Johnston CE, Birch J. A tale of two tibias: a review of treatment options for congenital pseudarthrosis of the tibia. J Child Orthop. 2008;2:133–49. 4. Khan T, Joseph B. Instructional review: children’s orthopaedics “Controversies in the management of congenital pseudarthrosis of the tibia and fibula”. Bone Joint J. 2013;95-B(8):1027–34. 5. Pannier S. Congenital pseudarthrosis of the tibia. Orthop Traumatol Surg Res. 2011;97:750–61. 6. Vander KL, Hensinger RN, Caird M, Johnston C, Farley F. Congenital pseudarthrosis of the tibia. J Am Acad Orthop Surg. 2008;16:228–36.

Recalcitrant Congenital Pseudarthrosis of the Tibia

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Robert A. Hill

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210

Abstract

Congenital pseudarthrosis of the tibia (CPT) is a rare condition with an incidence of 1 in 190,000 live births. The pseudarthrosis usually develops in the first 2 years of life although cases have been diagnosed antenatally and in adult life. Although the etiology remains unclear, there is a strong association with neurofibromatosis (NF-1). CPT is variable not only in its clinical presentation and appearance but also in the radiological appearance. Histologically, there is hyperplasia of fibroblasts and dense, almost invasive, fibrous tissue surrounding the site of the pseudarthrosis and between the bone ends if a frank pseudarthrosis has developed. The bone is usually thin and sclerotic, and X-rays or MRI may show abnormal bone at some distance from the focus of the disease. The fibula may be involved, and occasionally a fibula

R. A. Hill (*) Portland Hospital for Women and Children, London, UK e-mail: [email protected]

pseudarthrosis is present with a relatively normal tibia. As yet there is no unifying classification based on all these features that offers any therapeutic or prognostic guide to management. CPT is challenging to treat because of the triad of dysplastic poorly vascularized bone; deformity, including shortening; and a propensity for refracture after union is initially obtained. Multiple surgical interventions are common. Maintenance of union is as important as achieving union, and it is also important to consider ankle function. This case has been chosen to illustrate some of the difficulties in managing this condition and the principles that need to be considered.

Brief Clinical History An 8 year old boy was referred with a pseudarthrosis of the right tibia; there had been a minor injury which had precipitated attendance at the local hospital although some bowing

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_52

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of the lower leg had been previously noted. X-rays (Fig. 1) showed features consistent with CPT. Genetic investigations revealed no evidence of NF-1. The patient then underwent a series of operations which illustrate some of the difficulties in managing this condition. During the course of this patient’s treatment, we were evolving a management strategy for CPT which is reflected in this patient’s management [2].

R. A. Hill

Treatment Strategy The initial treatment planned was to excise the pseudarthrosis to viable bone, to acutely compress the pseudarthrosis (and subsequently maintain compression), and to correct deformity and lengthen above to restore leg length and stimulate healing at the pseudarthrosis site. This first operation was carried out in July 2005, but as will be discussed below treatment continued till 2012 – 7 years.

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List • An established tibial pseudarthrosis in an area of abnormal, poorly vascularized bone • Deformity • Leg length difference • Known risk of recurrence

Basic Principles The extent of resection of the pseudarthrosis is debatable; it is not the author’s practice to resect the entire area of abnormal bone but to resect sufficiently back to definitely bleeding bone and allow stability of contact between the bone ends – usually a “pen and inkwell” type of compression. To excise the entire area of abnormality may mean a wide excision which will then necessitate reconstruction by transport with its attendant difficulties. It is reasonable to assume that most, if not all, of the tibia between the growth plates is abnormal in some way. The next principle is to correct the mechanical alignment and get mechanics on your side; as will be seen if this is not achieved and maintained, then there may be recurrence. The frame must be very stable, and with distal pseudarthroses this may necessitate extension to the foot. Ankle position and function must not be forgotten as these patients are at risk of developing valgus if the fibula is involved as well as of soft tissue contractures developing during treatment. The X-rays that follow show the problems that ensued and how the issues were managed.

Images During Treatment See Figs. 2, 3, 4, and 5.

Technical Pearls

Fig. 1 AP and lateral X-rays of tibia at presentation showing tibial pseudarthrosis with broad bone ends and some evidence of healing. This is a stiff pseudarthrosis. There is a typical anterolateral bow and the fibula is intact. Note abnormal appearance of tibia more proximally

While the initial treatment was successful in correcting the deformity and healing the pseudarthrosis, there was fairly rapid recurrence of the bowing which lead onto a stress fracture. It can be very difficult to assess healing of a pseudarthrosis by X-ray, and it is wise to progressively destabilize the frame prior to removal to check on the stability of union. Once significant deformity recurs, problems are almost inevitable and the mechanical axis needs to be restored and the bone protected against further deformity by intramedullary fixation. This was done here (Fig. 5) by the use of a Rush pin. Unfortunately, the bone did not heal,

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Recalcitrant Congenital Pseudarthrosis of the Tibia

207

Fig. 2 (a) AP and (b) lateral X-rays after first operation. The pseudarthrosis has been resected and will be compressed by the Ilizarov frame; there is a proximal osteotomy for lengthening, and alignment has been corrected

Fig. 4 Stress fracture of tibia due to recurrent deformity and dysplastic bone Fig. 3 Lateral and AP views 7 months after frame removal. 4.5 cm of lengthening achieved, but there has been some recurrence of deformity which inevitably leads to a stress fracture (Fig. 4)

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Fig. 5 Lateral X-ray showing correction of alignment by Rush pin insertion

Fig. 6 (a) AP and (b) lateral views during revision treatment with a spatial frame. Frame could be criticized for not being stable enough as only one ring distal to pseudarthrosis when original Ilizarov frame had two (Fig. 2)

R. A. Hill

despite functional bracing, and there was rotational instability. At this point there was a full discussion with the family of the options, including amputation, but they elected to continue with reconstruction. To achieve rehealing of the pseudarthrosis, it was elected to apply a spatial frame after resection of the pseudarthrosis site and again relengthen but over a shorter pre-placed Rush pin with a view to maintaining a track for rodding or nailing after frame removal (Fig. 6). There were some problems with tightness of the Achilles tendon necessitating extra physiotherapy, and the family also required psychology support. Healing at the pseudarthrosis site was slow, and EXOGEN ultrasound treatment was given. This may have been helpful, and the frame was removed approximately 9 months after application. It was intended to rod the tibia on frame removal, but in practice it proved very difficult to pass a rod through the narrow sclerotic segment, and only a small-diameter pin could be passed. This was less stable than had been desired. Healing and stability at the pseudarthrosis site remained problematic, and further treatment was advised which was carried out 17 months after the second frame had been removed. In Limb Reconstruction, particularly nonunion, if a treatment has not been successful, it is wise to consider the possible reasons for failure and what factors can be addressed. In this case it was felt that the second frame had not been successful because it was not stable enough and because of the bone biology. The concept of protecting against deformation after frame removal with a rod was considered sound provided the practical issues could be overcome. A spatial frame was reapplied ensuring good stability,

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and a biological stimulus was applied by a bone graft wrap combined with bone morphogenic protein (BMP 2 Induct Os. Wyeth Europa) (Fig. 7). This frame appeared more successful than the previous one; care was taken to progressively destabilize the frame when healing appeared to be occurring. It is sometimes useful to screen the pseudarthrosis site in the radiology department as with so much metal around the leg it is difficult to get good pictures of the pseudarthrosis site. The frame was removed after 6 months, and a plaster cast used for 8 weeks followed by a protective removable splint. An epiphysiodesis of the opposite proximal tibial and distal femoral growth plates was carried out to prevent the leg length discrepancy from deteriorating. Fig. 7 (a) AP and (b) lateral views showing third frame – note more stable than previous frame as there are two rings distal to the pseudarthrosis. The position of the bone graft wrap is shown by the wires around the pseudarthrosis

Fig. 8 (a) AP and (b) lateral views showing a fracture just distal to the healed pseudarthrosis site which has been thickened by the BMP and bone graft wrap

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The bone graft wrap and BMP had successfully healed the pseudarthrosis and thickened the bone, perhaps analogous to the concept of a bypass graft; however, it had not been possible to insert an intramedullary nail. As was remarked by a colleague (Robert Simonis, Consultant Orthopedic Surgeon, Rowley Bristow Orthopaedic Hospital, Surrey, UK), in pseudarthrosis the bone is not filled with blood but with black ingratitude, and the ingratitude was shown by a stress riser fracture in the more osteoporotic bone just distal to the thickened, healed pseudarthrosis site (Fig. 8). This was the opportunity to insert a locked intramedullary nail, with BMP, and the bone is now healed, straight, and protected (Fig. 9).

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R. A. Hill

Fig. 9 (a) AP and (b) lateral views 15 months after nailing showing healed, aligned, and protected tibia

Outcome Clinical Photos and Radiographs

recurrent deformities and possible fracture with a locked intramedullary nail is attractive.

See Figs. 6, 7, 8, and 9.

Cross-References Avoiding and Managing Problems Many of the problems that can occur did occur with this case. In this condition problems have to be anticipated, and it is wise to have a careful discussion with the family before treatment commences and to ensure good psychology and physiotherapy support is in place. Multiple procedures are often required, and the family needs to be prepared for this. It is not just a question of getting the pseudarthrosis to heal; it is also necessary to maintain that healing through to skeletal maturity. Alignment is critical, the frame needs to be stable, and in selected cases BMP has a definite role but in the author’s opinion in combination with a bone graft wrap to thicken and perhaps bypass the pseudarthrosis segment. At the conclusion of treatment, protecting the tibia against

▶ Congenital Pseudarthrosis of Tibia ▶ Congenital Pseudarthrosis Tibia (El-Rosasy – Paley Type 3) ▶ Congenital Tibial Pseudoarthrosis Treated with Internal and External Fixation Using the 4 in 1 Technique ▶ Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia

References and Suggested Reading 1. El-Rosary M, Paley D, Hertzenberg J. Chapter 34: Congenital pseudarthrosis of the tibia. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: Informa Healthcare; 2007. 2. Nicolaou N, Ghassemi A, Hill RA. Congenital pseudarthrosis of the tibia: the results of an evolving protocol of management. J Child Orthop. 2013;7:269–76.

Congenital Pseudarthrosis of Tibia

33

Dror Paley and Craig A. Robbins

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 213 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 218 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 218

Abstract

The treatment of congenital pseudarthrosis of the tibia (CPT) is challenging with variable results. Refracture, need for multiple operations, persistent nonunion, and residual limb length discrepancy are common. This chapter describes a comprehensive new treatment strategy with proven results of 100% union and no refractures.

Brief Clinical History This is a 14 month old child born with congenital pseudarthrosis of the tibia. She had previous failed treatment at an outside facility by McFarland bypass graft. She has significant procurvatum deformity and a flail pseudarthrosis. D. Paley (*) · C. A. Robbins Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA e-mail: [email protected]; [email protected]; [email protected]

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List • Flail pseudarthrosis (Paley-El-Rosasy type 2) • Leg length discrepancy • Procurvatum-Valgus tibial deformity

Treatment Strategy Paley Kitchen Sink Treatment Regimen Steps (Fig. 2) 1. Zoledronic acid (ZA) infusion 2 weeks preoperative. 2. Resect hamartoma of the tibia and fibula.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_35

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Fig. 1 AP (left) and lateral (right) radiograph of CPT tibia and fibula. She had one previous failed attempt at treatment. Note the significant procurvatum

Lateral view

a

b

c Bones docked

Bones split

d Rods inserted

e

Periosteal graft Bone graft applied wrapped around to docking sites tibial docking site

AP view fixator f External applied

a

b

Fig. 2 Paley “kitchen sink” treatment for CPT

c

d

e

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Congenital Pseudarthrosis of Tibia

3. Split one bone end or square both bone ends (A). 4. If split, invaginate ends; if square, oppose ends (B). 5. Insert Fassier-Duval (FD) telescopic nail to the tibia and wire to the fibula (C). 6. Harvest periosteal graft from undersurface of the iliacus. 7. Decancelization of the entire iliac wing (anterior and posterior) to harvest bone graft (see Fig. 4). 8. Place periosteal graft around tibial docking site (D) covered by ICBG (E) and then BMP. 9. Bone graft between the tibia and fibula to achieve a wide cross union (E). 10. Close wound over drain. 11. Apply circular frame to compress and stabilize rotation (F). 12. Repeat ZA infusion at the time of removal surgery. 13. Replace nail as needed according to growth. 14. Use plate hemi-epiphysiodesis to correct residual knee or ankle valgus deformities.

213

BMP. The BMP needs to be adjacent to the vascularized soft tissues to recruit and induce new bone formation. • The FD nail (Pega Medical, Montreal, Canada) allows for rodding from epiphysis to epiphysis and permits growth to occur. The fibula should also be stabilized with an intramedullary wire to add rotational stability. • The cross union to the fibula is one of the most important goals of treatment to prevent refracture as it greatly increases the cross-sectional area of the bone. • The external fixator is important to neutralize the rotation forces on the leg since the FD nail cannot do this. It also adds some compression to the bone ends and prevents them from disengaging.

Images During Treatment See Figs. 3, 4, 5, and 6.

Basic Principles • CPT patients have a periosteal disease. The periosteum has over abundant overactive osteoclasts. The periosteal thickening is called a hamartoma. It has cells wrapping themselves around the blood vessels. The bone is affected secondarily which is why we resect the diseased hamartoma but not the bone. • Due to increased osteoclasts, we use bisphosphonate treatment. By giving the bisphosphonate before surgery, it gets into the bone graft making it non-resorbable. Since bisphosphonate may inhibit osteoblasts, it is best to give it 2 weeks before surgery. • Periosteal grafting replaces the diseased periosteum with a healthy periosteum from the underside of the iliacus muscle. This tissue can be expanded using a skin graft mesher. • NF patients’ bone produces less BMP which is why we use BMP. • Very young patients present a problem harvesting enough bone graft. There is ample bone graft in the ilium if one splits the tables of the ilium and harvests down to the supra-acetabular and SI joint regions under fluoroscopy. The cortical bone should be left behind to regenerate the iliac bone. • The bone ends of the CPT should not be resected except as needed to either square them off or split one atrophic end to invaginate the other atrophic end into it. • The grafting sequence should be periosteal graft, cambium layer down, followed by cancellous bone, and finally by

Technical Pearls This treatment regimen was developed sequentially by the primary author over the last two decades. Its success is based on the multimodal approach and all steps should be followed. • In some cases, the tibia and fibula can be approached through a single incision; otherwise, leave an adequate soft tissue bridge. • Resect all pathologic hamartomatous periosteum circumferentially. • Only resect as much bone as needed to allow tension-free contact. • Place intramedullary hardware prior to grafting. • The periosteal graft shrinks after it is removed; remove adherent muscle and expand with 1:1.5 mesher and place it cambium side down. • Split the apophysis along its entire length, and then split between the inner and outer tables completely with a thin osteotome prior to harvesting any bone graft to avoid fracturing the thin cortices. • Adequate bone must be harvested to obtain cross union between the tibia and fibula. • BMP is the last layer of grafting between the ICBG and soft tissues. • The Ilizarov is placed with two opposed olive wires proximal and distal ensuring neither wire touches the intramedullary implant to decrease risk of deep infection.

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Fig. 3 Intraoperative fluoro showing AP tibia and fibula after hamartoma resection (left), AP and lateral tibia after FD rodding of the tibia and K-wire insertion to the fibula, and grafting (middle and right)

Fig. 4 Intraoperative fluoro of the left ilium showing special technique of cancellous bone graft harvest. After splitting the apophysis, the iliac wing tables are split (left upper) using a very thin sharp osteotome (Pega Medical, Montreal, Canada). The cancellous bone of the ilium is curetted from the supra-acetabular region all the way to the subchondral bone and triradiate cartilage (upper three right). The osteotome is used to split the tables extending toward the sacroiliac joint posteriorly and

down to the sciatic notch (left lower). A curette is used to scrape out the bone between the tables (lower two middle). This method allows harvesting a large amount of bone from children as young as 1 year, through one bikini line incision. Radiograph of ilium 4 months after the bone graft harvest. There is no negative effect on the hip joint or triradiate cartilage. No weight-bearing restrictions are needed

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Congenital Pseudarthrosis of Tibia

Fig. 5 Intraoperative photographs of CPT site: periosteal graft is meshed and wrapped around the CPT site and secured with a suture (left); iliac crest bone graft is placed around the tibia and between the

215

tibia and fibula (middle); BMP2 (Infuse, Medtronics, Memphis, TN) is applied between the bone graft and the soft tissues (right)

Fig. 6 AP (left) and lateral (right) radiographs after surgery. The bone graft is seen around the tibia and between the tibia and fibula. The Ilizarov device is stabilizing the tibia externally while neutralizing the foot forces

Outcome Clinical Photos and Radiographs See Figs. 7, 8, and 9.

Avoiding and Managing Problems I published four studies on CPT [1–4]. In the first in 1992, I used the Ilizarov and iliac crest bone grafting of the tibia but no IM rod [3]. We obtained union in 100% but had a 68% refracture rate.

In the second, done in 1998 but published in 2007, we added IM rodding of all cases [1]. The union rate was 100%, but the refracture rate dropped to 29%. In the third published in 2008, I added periosteal grafting and BMP [4]. The union rate stayed at 100%, but the refractures dropped to 20%. In the fourth study published in 2012, I added the cross union to the fibula and zoledronic acid infusion [2]. The union rate was 100%, but the refracture rate dropped to zero. Since using this protocol which is outlined in this case, I have had no refractures in over 30 CPT cases since 2007 (7 year follow-up). Successful cross union was achieved in all cases [2].

216 Fig. 7 The external fixator was removed 4 months after surgery. The CPT of the tibia and fibula is healed. There is an excellent cross union of the bone from the tibia to the fibula over a wide area. The FD tibial nail and K-wire fibular nail remain to protect the two bones from refracture

D. Paley and C. A. Robbins

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Congenital Pseudarthrosis of Tibia

Fig. 8 AP of left hemi-pelvis 4 months after bone graft harvest; there is no negative effect on the hip joint or triradiate cartilage. No weightbearing restrictions were needed

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Fig. 9 AP (left) and lateral (right) radiographs 2 years after surgery showing excellent remodeling of the bones, cross union intact, no CPT, and obvious tibial and fibular growth with telescoping of the FD nail

Cross-References ▶ Congenital Tibial Pseudarthrosis Managed with Ipsilateral Vascularized Fibula Transfer ▶ Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application ▶ Fourteen Year Old Patient with Severe Congenital Pseudarthrosis of the Tibia

References and Suggested Reading 1. El-Rosasy MA, Dror P, Herzenberg JE. Congenital pseudarthrosis of the tibia. In: Limb lengthening and reconstruction surgery. New York: Informa Healthcare; 2007. p. 485–93.

2. Paley D (2012) Congenital pseudarthrosis of the tibia: combined pharamcologic and surgical treatment using biphosphonate intravenous infusion and bone morphogenic protein with periosteal and cancellous autogenous bone grafting, tibio-fibular cross union, Intramedullary rodding and bone grafting. (ed: Alessandro Zorzi). InTech. ISBN: 978-953-51-0324-0. http://www.intechopen.com/ books/bonegrafting/treatment-of-congenital-pseudarthrosis-with-peri osteal-and-cancellous-bone-grafting 3. Paley D, Catagni M, Argnani F, Prevot J, Bell D, Armstrong P. Treatment of congenital pseudoarthrosis of the tibia using the Ilizarov technique. Clin Orthop Relat Res. 1992;280:81–93. 4. Thabet AM, Paley D, Kocaoglu M, Eralp L, Herzenberg JE, Ergin ON. Periosteal grafting for congenital pseudarthrosis of the tibia: a preliminary report. Clin Orthop Relat Res. 2008;466(12):2981–94.

Part IV Pediatric Deformity: Congenital Lower Limb Deficiencies

Congenital Lower Limb Deficiencies: An Introduction

34

Reggie C. Hamdy

Contents Fibular Hemimelia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 222 Tibial Hemimelia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223 Posteromedial Bowing of the Tibia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 224 Congenital Short Femur and PFFD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 224 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 228

Cheated of feature by dissembling nature, Deformed, unfinished, sent before time Into this breathing world, scarce half made up, And that so lamely and unfashionable, That dogs bark at me as I halt by them (in Richard III, from Mercer’s Orthopaedic Surgery, 7th Edition)

Is there a more vivid description of the anguish and despair that some patients with major limb deficiencies and their families live in? The birth of a child with a deformity could be one of the most traumatic events for a parent. No one is better positioned than we, as treating physicians, to offer hope for these parents, often desperate, often disillusioned, hope that their child could function as normal as possible and that they will not be ridiculed. As limb reconstruction surgeons, we have the unique privilege of taking care of these children and, with the knowledge, skills, and tools that we possess, of being able to offer a brighter future. It’s a testimony that today we can indeed make a difference in the lives of these children. Congenital lower limb deficiencies include a wide spectrum of anomalies with various degrees of severity. They are complex conditions due to the presence of a multitude of problems, including LLD that is often severe, multiple-joint, bony, and soft tissue anomalies, unstable joints, and in some cases upper limb deficiencies that may have an impact on the R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

ability of the patient to have adequate postoperative rehabilitation following lower limb surgery. These children are best followed in multidisciplinary clinics, where appropriate resources are available, including psychologists, social workers, physiotherapists, and occupational therapists. During the first visit, we get to know the family, gain their confidence, and appease their apprehension. There is rarely a need to decide for surgery the first time we see these patients. During the ensuing visits, a comprehensive management plan, always including the parents, is formulated based on the predicted deformities at skeletal maturity. The goal in the management of these children is to obtain a functional and aesthetically acceptable limb. In some severe cases of fibular hemimelia, tibial hemimelia, and congenital short femur, the question often arises: is amputation and prosthetic fitting a better alternative to heroic attempts at limb salvage and reconstruction? Many parents, understandably, have difficulty accepting an amputation for their child. It is our role to explain to them and help them understand that amputation is a reconstructive procedure to optimize function and should not be considered a failure of treatment. We should give the parents the choice between amputation and limb salvage, and if they refuse amputation, then we should do everything possible to respect that decision and proceed with limb salvage reconstructive surgery or refer them to a surgeon with the appropriate expertise. Seeing and meeting with other children who have had amputation may help them in their decision-making.

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With evolving techniques available for limb lengthening, severe LLD should not be considered an indication for amputation, unless associated with other severe deformities. There is no generally agreed maximum amount of LLD beyond which an amputation is recommended, and for correction of an isolated LLD deformity, there is no absolute limit. Multiple staged lengthenings for severe LLD is a safer approach than one single extensive lengthening. The optimal age at which to proceed with the first lengthening still remains controversial. Many authors agree that it is better to wait before proceeding with the first lengthening, until functional problems start to appear, while others prefer to perform the first lengthening at a relatively young age of 2–4 years. Others do not recommend lengthening between the ages 4 and 7, as lengthening during these years may not be well tolerated by many children for psychosocial reasons. Over the last two decades, the general approach to severe congenital deformities has greatly evolved in large part from the creative contributions of Dror Paley. His innovative classifications and revolutionary surgical techniques for correction of the most severe deformities, have truly pushed the envelope into “unchartered waters!” With the advent of new imaging modalities and a better understanding of the pathogenesis of these conditions came new classifications for fibular hemimelia, tibial hemimelia, and proximal femoral focal deficiency (PFFD) that address some of the weaknesses present in previous classifications. The superhip, superknee, Fig. 1 Achterman-Kalamchi classification of fibular deficiency

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and superankle procedures are slowly changing our approach to these difficult problems. In this section, 24 cases are discussed: Congenital short femur and PFFD (12 cases), tibial hemimelia (6 cases), Fibular hemimelia (3 cases), congenital posteromedial bowing of the tibia (1 case), absent quadriceps mechanism (1 case) and generalized short femur and tibia (1 case). Congenital pseudarthrosis of the tibia is discussed in another separate section. From the numerous associated anomalies, patients with congenital deficiencies are more susceptible to develop complications than those with noncongenital deformities, including poor regenerate bone formation (case 39), knee subluxation (cases 51, 54), hip subluxation (case 51), and contracture (case 53). The importance of protecting the hip, knee, and ankle during lengthening procedures cannot be overemphasized (with physiotherapy, bracing, or by spanning the joints with the external fixator).

Fibular Hemimelia This is the most common lower limb deficiency. The classification by Achterman-Kalamchi (Fig. 1) is still widely used, although based only on the presence or absence of the fibula, completely ignoring the foot and ankle anomalies. Realizing the significant role of the functional status of the foot and

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ankle and that of the upper limb in the surgical decisionmaking, Birch et al. in 2008, proposed a classification based on whether the foot is functional or not, the percentage LLD, and the status of the upper extremities (Table 1). The ability to preserve the foot was based on the number of rays present, and the recommendation was that at least three rays should be present for foot salvage. Paley then built on this and proposed a new classification (case 41) based on the anomalies present in the ankle and subtalar joints. Regarding the surgical treatment, in cases with a functional foot (case 39), irrespective of the LLD, there is almost unanimous agreement that the optimal treatment is reconstruction of the limb, with one or multiple staged lengthenings (with or without contralateral epiphysiodesis). On the other hand, controversy still exists as to the best approach in cases of complete absence of the fibula with severe anomalies

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of the foot and ankle. While many authors still recommend Syme’s or Boyd amputation (case 40), Paley challenged this approach and favors preservation of the foot and reconstructive procedures in most cases (case 41). Valgus deformity of the distal femur (case 41) remains a concern even after a Syme’s amputation (case 40) and is best approached by medial distal femoral hemi-epiphysiodesis.

Tibial Hemimelia The main problem for children with tibial hemimelia is not the extent of the LLD but rather the severe anomalies that may be present at the knee (Jones Type I) and ankle (Jones Type IV) joints. The Jones classification (Fig. 2) is widely used, although Jones Type III is extremely rare. In cases with

Table 1 Birch classification of congenital fibular deficiency Type Characteristic Type 1 (foot can be salvaged) 1A 30% inequality Type 2 (foot cannot be salvaged) 2A Functional upper extremity 2B Nonfunctional upper extremity

Treatment Anticipated No treatment or orthosis or epiphysiodesis Epiphysiodesis  lengthening 1 or 2 lengthenings  epiphysiodesis or extension orthosis >2 lengthenings or amputation or extension orthosis Early amputation Consider salvage

Shortening is calculated as a percentage relative to the contralateral limb. In bilateral cases, the longer limb is classified as Type 1A, and the shorter limb is classified as is done for unilateral cases.

Fig. 2 Jones classification of Tibial Hemimelia

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completely absent extensor mechanism and patella, the standard treatment still remains knee disarticulation (case 43). However, extensive and complex reconstructive surgery has been described by Paley (case 44a). In cases with an existing extensor mechanism that may not be apparent at birth (Jones Type Ib), reconstructive procedures such as the Weber arthroplasty are discussed (cases 43 and 44b). Prior to any type of surgery, it is important to determine the presence or absence of the extensor mechanism with physical exam, ultrasound and MRI. In Jones Type II cases, it is generally recommended to perform proximal tibiofibular synostosis or centralize the fibula under the proximal tibial remnant (case 45). Simultaneous amputation of the foot may be performed, depending on the severity of the anomalies. Jones Type IV cases are extremely challenging to treat, and there is no uniformly accepted approach (cases 46 and 47) (Table 2). Staged reconstruction is described in case 47, where the foot is first translated distally and then positioned and stabilized plantigrade by means of a tibio-talar fusion. Then the LLD is addressed separately.

Posteromedial Bowing of the Tibia This case was included in this section, although it is not considered a true congenital limb deficiency (case 42). But it demonstrates that in some cases, the tibial deformity does not always correct spontaneously as is usually expected and the LLD may be significant. Sometimes, a calcaneo-valgus deformity is present.

Congenital Short Femur and PFFD This represents a spectrum of disorders that extends from mild discrepancy in length of the femur only to very severe cases with almost complete absence of the proximal femur and acetabulum. The most commonly used classification is that described by Aitken (Fig. 3). Paley has also proposed a classification that is described in cases 55–57. An associated fibular deficiency is present in almost half of children. In cases of congenital short femur with normal or almost normal hip joints, single or staged lengthening remains the standard approach with standard Ilizarov fixator (case 48), MAC device (case 49), Precice internal lengthening nail (case 50), lengthening over elastic nails with a hybrid TSF/Ilizarov fixator (case 52), and lengthening over solid nails with the LON technique (case 53). Release of the iliotibial band should routinely be performed in almost all cases of femoral lengthening. Prior to any lengthening, careful assessment of the hip joint for the presence of any acetabular dysplasia, uncovering

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of the hip, and coxa vara is key for prevention of hip dislocation. If these deformities are present, proximal femoral and/or pelvic osteotomies are indicated prior to lengthening (case 54). Maintaining full extension of the knee with bracing or spanning with the fixator is also key for prevention of knee subluxation (cases 51, 53 and 54). True PFFD is discussed in cases 55–58. There is no standard treatment for these severe cases. Each case should be individualized. However, with the development of new reconstructive techniques, the approach to these severe cases is evolving. Both the deficiencies and deformities have to be fully assessed in detail. Deficiencies include absent parts of the proximal femur and/or acetabulum leading to hip instability and deficient or absent knee joint ligaments in addition to all the manifestations of fibular hemimelia, if present. Deformities include muscular contractures around the hip and severely distorted proximal femur deformities. In most cases of Aitken Type A and Paley Type 1b, there is delay in the ossification of the proximal femur, and this makes it difficult to formulate an early plan of treatment during the first few years of life. For this reason, an MRI of the hip is helpful for evaluation of the status of the hip joint before undertaking any surgical procedures. In general, if there is continuity between the proximal femur and femoral head (even in the presence of pseudarthrosis at the neck or subtrochanteric level (Paley Type 1b and Type 1c)), then reconstruction of the hip with a superhip procedure (Paley, cases 55a and 55b) is becoming increasingly popular. Hip surgery is usually delayed until the age of 3–5 years. Staged multiple lengthenings could then be subsequently performed. The knee joint could be stabilized and reconstructed with a superknee procedure (case 55a). Besides preservation of the limb and multiple lengthenings, other strategies include converting the affected lower limb into one single bone by performing a Syme amputation and knee fusion so that it functions as an above-knee or knee disarticulation amputee. An alternative option is to perform a Van Nes rotationplasty (case 58) with the goal of preserving the ankle joint so that it functions as a knee joint and thus converting the patient into a below-knee amputee. A prerequisite of that procedure is the presence of a functional ankle joint. In both of these scenarios – knee fusion with Syme amputation and rotationplasty – reconstructive hip surgery could be performed at a later date if indicated (Aitken Type A or Paley Type 1b and 1c). In cases where there is no continuity between the proximal femur and femoral head (Aitken Type B and C, Paley Type 2a), a superhip 2 procedure could be performed (Paley, case 56). Other alternatives for these severe cases include Femoral-pelvic arthrodesis with a modified rotationplasty (case 59) as described by Brown (Brown procedure, Fig. 4) or simply prosthetic fitting in the form of an ischial weight-bearing prosthesis that will address the hip instability. A pelvic support osteotomy (case 57) is another

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Table 2 Details of the 24 Congenital Lower Limb Deficiencies cases discussed in this Atlas Case 39

Diagnosis 11 year old boy, fibular hemimelia Kalamchi Type IA, congenital short femur Paley Type IA

Problems LLD 4.0 cm, predicted at skeletal maturity 6.2 cm, equally between femur and tibia, unstable knee, valgus distal femur. Poor regenerate

40

11 months, Type II Kalamchi Fibular hemimelia, non-functional foot. Had Syme amputation 18 months old boy, severe fibular hemimelia, Type II Kalamchi, Paley Type 3

At 12 years age, valgus distal femur LDFA 74

42

9 year old boy, postero-medial bowing tibia followed since age of 2

LLD 7.0 cm, persistent postero-medial bowing tibia

43

10 days old, bilateral tibial hemimelia; Type IA left, Type IB right side (ultrasound showed presence proximal tibia cartilage)

Bilateral Tibial Hemimelia: right side, presence of quadriceps, left side: no quadriceps

44a

Tibial hemimelia Type IA Jones, Paley 5b

Severe equino-varus foot, flexion contracture knee, no quadriceps, dislocation fibula over femur

44b

Tibial Hemimelia Type IB Jones, Paley 5a

45

Type II tibial hemimelia, short proximal tibial stump

46

Type IV Tibial Hemimelia, diagnosed at 5 weeks of age

Severe equino-varus foot, knee flexion contracture 6 year old male, LLD 8.0 cm (expected to be 14 cm at skeletal maturity), 30 rigid equino-varus deformity At 5 years: LLD 4.5 cm, equinovarus abnormal ankle, prominent fibula proximally and distally, unstable knee

47

7 year old boy, Type IV Tibial Hemimelia

Rigid equinovarus foot, absence of functional ankle, LLD, refused Syme’s amputation

48

9 year old boy, congenital short femur Paley Type I

LLD 6 cm, fibular hemimelia, distal femoral valgus, unstable knee

49

18 year old girl, congenital short femur Paley Type I 9.5 year old boy, congenital short femur Paley Type I

LLD 7.2 cm, valgus distal femur

51

10 year old, congenital short femur, psoriasis

LLD 5 cm, mild acetabular dysplasia. Hypoplastic lateral femoral condyle and cruciate ligaments. Underwent femoral lengthening with monolateral fixator. Knee subluxation and hip dislocation

52

7.5 year old boy, congenital short femur Paley Type I

LLD 4 cm, valgus distal femur LDFA 70

41

50

Fixed equino-valgus, LLD 5 cm complete absence fibula, tibia diaphyseal procurvatum and valgus

LLD 5 cm, predicted 8 cm, retroversion

Surgery and key points Tibial lengthening 4.5 cm, poor regenerate developed. Strategy how to address poor regenerate. Accordion technique. Careful monitoring regenerate. Contra-lateral epiphysiodesis planned Growth modulation with 8-plate distal femur. Plate removed after correction of valgus Superankle procedure at 18 months, lengthening 5 cm. Second lengthening 8 cm at 8 years, third lengthening femur and tibia at 12 years, hemi-epiphysiodesis distal femur for valgus Osteotomy tibia, TSF gradual correction and lengthening. Second lengthening of 4.5 cm at skeletal maturity On left side, knee disarticulation at 10 months. On right side, at age 4.5 years, proximal tibio-fibular synostosis. At age 8 years, correction foot deformity with MAC device, followed by talo-fibular centralization and fusion with MAC device Fusion of foot to distal fibular epiphysis. Knee distraction, missing quadriceps restored by transfer hamstrings. Cruciate reconstructed with biceps Weber patellar arthroplasty, ankle fused. Circular fixator Fibular transfer and centralization with Ilizarov fixator. Syme’s amputation At 5 years age, initially differential lengthening of tibia only to bring fibula distally, and then fibular osteotomy and lengthening. Soft tissue release to obtain plantigrade foot Staged reconstruction. Soft tissue releases, TrueLok fixator to gradually translate foot distally to reposition foot. Acute correction of foot. Tibio-talar fusion. Second stage osteotomies pf tibia and fibula to correct LLD. Lengthening over rush pin, by standard Ilizarov fixator, spanning the knee with hinged construct. Consider MacIntosh extra-articular cruciate reconstruction. Acute correction of deformity, Lengthening with MAC device Release iliotibial band. Acute rotational correction with ex. Fixator. Femoral lengthening with Precice nail Open reduction of hip, soft tissue releases, conversion to Ilizarov fixator extended to pelvis. Knee subluxation treated with hamstring release, extension of frame across knee, hinged distraction of knee with hinges placed anteriorly Lengthening femur over elastic intramedullary nails, using Hybrid TSF/Ilizarov frame (continued)

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Table 2 (continued) Case 53

Diagnosis Congenital short femur and tibia. Presented age 9 years with LLD 5.5 cm

Problems Predicted LLD 20 cm

54

6 year old male, congenital short femur and coxa vara

LLD 7 cm, predicted 13 cm, unstable hip, coxa vara, acetabular dysplasia, unstable knee

55a

PFFD Paley Type 1c (femoral neck region delayed ossification)

2 year old girl, delayed ossification femoral neck. Acetabular dysplasia. Absent cruciate ligaments. Severe LLD

55b

PFFD Paley Type Ib (subtrochanteric region delayed ossification)

2 year old girl, delayed ossification subtrochanteric region. Severe LLD

56

3 year old girl, PFFD Paley Type 2a

Complete pseudarthrosis femoral neck

57

10 year old girl, PFFD Paley Type 2c

Complete absence of femoral head and neck and acetabulum. Unstable hip, flexion contracture

58

3 year old girl, Aitken Type B PFFD Paley Type 1b

Severe LLD, foot opposite normal knee. Dysplastic hip. Dysplastic knee with flexion contracture

59

7 year old female, severe PFFD Aitken C (Paley Type 2c). Previously had femoralpelvic fusion with rotationplasty and rotational osteotomies

60

11 year old girl, generalized atrophy entire lower limb

1 year post-operatively, had inadequate position of the limb with femoral component 90 externally rotated, 50 abduction, knee laxity, prosthetic wear difficult Absent quadriceps, severe LLD of 11 cm, recurrent knee flexion deformity of 80 after initial correction by gradual correction with Ilizarov fixator, rigid equinovarus foot

option in cases of complete absence of the femoral head and acetabulum. For bilateral cases, prosthetic fitting only is usually recommended. The management of children with congenital absence of the quadriceps can be very challenging, as shown in case 60. In this patient, multiple and severe deformities include complete absence of the quadriceps mechanism, severe knee flexion contracture, severely atrophied lower limb with almost no functional muscle groups, large LLD of 11 cm, and rigid equinovarus deformity of the foot. After extensive

Surgery and key points Three lengthenings (5 cm femur at age 9 with monolateral frame, 7 cm tibia at age 12 with TSF/Ilizarov, 8 cm at age 15 with LON, lengthening over nail technique), contralateral epiphysiodesis distal femur and proximal tibia Quadricepsplasty for knee extension contracture Femoral valgus osteotomy and DEGA pelvic osteotomy to correct hip dysplasia. Femoral lengthening with monolateral fixator. Knee subluxation during lengthening Superhip and superknee procedures at age 2. BMP2 inserted in femoral neck. Fixation with blade plate. First lengthening 8 cm at age 4 with monorail system, spanning the knee. Second lengthening age 8 years 8.5 cm with Precice nail. Hemiepiphysiodesis for distal femoral valgus Superhip procedure age 2. First lengthening of 6 cm at age 4. Insertion of rush rod post removal fixator Superhip 2 procedure at age 3. First lengthening at age 6, second one age 10. Contra-lateral epiphysiodesis distal femur age 9. Third femoral lengthening age 14. Still has 4 cm discrepancy Pelvic support osteotomy by valgus / extension osteotomy proximally distal varus and lengthening osteotomy of 13 cm. Remodeling occurred, necessitating repeat pelvic osteotomy at age 16 with 10 cm lengthening Modified Van Nes Rotationplasty combined with knee fusion, followed by hip reconstruction by valgus osteotomy. Functioning as below knee amputee. Normal ankle is a pre-requisite Revision rotationplasty with Repeat rotation osteotomy of the femur and acute repositioning with circular fixators. Intraoperative SSEP monitoring of the peroneal and tibial nerves Knee disarticulation and prosthetic fitting, distal femoral epiphysiodesis to allow satisfactory prosthetic fitting

discussion with the family of the available options (reconstruction versus amputation), the parents opted for surgical reconstruction with an Ilizarov frame. However, despite obtaining initial success in the correction of the flexion deformity, this recurred, and it was then decided to proceed with a knee disarticulation. At the latest follow-up 5 years later, the parents and child were very happy with the functional outcome of the knee disarticulation and prosthetic fitting. This demonstrates that amputation may be the best reconstructive approach in certain cases.

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Fig. 3 Aitken classification of PFFD

Fig. 4 Brown procedure

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References and Suggested Reading 1. Achterman C, Kalamchi A. Congenital deficiency of the fibula. J Bone Joint Surg (Br). 1979;61(2):133–7. 2. Aitken GT. Proximal femoral focal deficiency – definition, classification, and management. In: Aitken GT, editor. Proximal femoral focal deficiency. A congenital anomaly. Washington, DC: National Academy of Sciences; 1969. p. 1–22. 3. Birch GJ, Lincoln TI, Mack PW, Birch CM. Congenital fibular deficiency: a review of thirty years’ experience at one institution and a proposed classification system based on clinical deformity. J Bone Joint Surg Am. 2011;93(12):1144–51.

R. C. Hamdy 4. Brown KL. Resection, rotationplasty, and femoropelvic arthrodesis in severe congenital femoral deficiency. J Bone Joint Surg Am. 2001;83A(1):78–85. 5. Hamdy RC, McCarthy JJ. Management of limb length discrepancies, AAOS monographs (45). Rosemont: American Academy of Orthopaedic Surgeons; 2011. 6. Hamdy RC, Makhdom AM, Saran N, Birch JG. Congenital fibular deficiency. J Am Acad Orthop Surg. 2014;22:246–55. 7. Naudie D, Hamdy RC, Fassier F, Morin B, Duhaime M. Management of fibular hemimelia: amputation or limb-lengthening. J Bone Joint Surg. 1997;79(B):58–65. 8. Jones D, Barnes J, Lloyd-Roberts GC. Congenital aplasia and dysplasia of the tibia with intact fibula: classification and management. J Bone Joint Surg Br. 1978;60:31–9.

Absent Knee Extensor Mechanism, Fixed Flexion Deformity, Leg Length Inequality and Foot Deformity in an 11 Year Old Girl Treated with Knee Disarticulation

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Elizabeth Ashby and Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 229 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 230 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 230 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 230 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 230 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 232

Abstract

This is a case of absent knee extensor mechansim, servere flextion deformity of the knee, leg length inequality and foot deformity in an 11 year old girl. Reconstructive surgery using the llizarov technique was attempted at the age of 2.5 years and was initially successful. Unopposed hamstring activity lead to deformity recurrence following frame removal. Three treatment options were then considered: supracondylar femoral ostetomy with hamstring transfer, knee fusion and knee disarticulation. The first two options would have required multiple limb lengthening’s together with reconstruction of the foot. The aims of treatment were to achieve normal weight-bearing, legs of equal length and a normal gait. It was decided that an

E. Ashby (*) Division of Orthopaedics, Shriners Hospital, Montreal, QC, Canada e-mail: [email protected] R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

amputation was the best way to achieve this. This girl now walks well and is a kneen cyclist.

Brief Clinical History This girl was born with a right foot deformity and atrophy of the entire right leg. The foot was treated with Ponseti serial casting followed by tendo-achilles lengthening at the age of 6 weeks. With growth, a leg length inequality (LLI) became apparent, and a progressive flexion contracture of the right knee developed. MRI revealed complete absence of the rectus femoris with hypoplasia of the vastus medialis and vastus lateralis. There was no patella and no patella tendon. An Ilizarov frame was applied at the age of 2.5 years to gradually extend the knee. After 6 months the frame was removed and the child was fitted with an articulated KAFO. The knee was straight and the child was able to walk. Due to unopposed hamstrings, the flexion deformity recurred. By the age of 11 years there was a knee flexion deformity of 80 , a leg length inequality of 11 cm, and a stiff right foot with fixed ankle equinus. The hip had a normal range of motion. It was

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decided to proceed with a knee disarticulation. The initial stump was too long so a distal femoral epiphysiodesis was performed. This young lady now walks well with her prosthetic limb and has just had a cage fitted to her bicycle pedal to enable her to cycle.

Preoperative Clinical Photos and Radiographs See Fig. 1.

E. Ashby and R. C. Hamdy

Treatment Strategy The flexion contracture of the knee was originally corrected using an Ilizarov frame. The deformity recurred once the frame was removed due to unopposed hamstring activity. This is important to remember when planning revision surgery: either the activity of the hamstrings has to be balanced or the knee has to be permanently immobilized in a functional position. The following three strategies were discussed: 1. Supracondylar femoral osteotomy with hamstring transfer 2. Knee fusion 3. Knee disarticulation

Preoperative Problem List 1. Severe fixed flexion deformity of the knee 2. LLI of 11 cm 3. Deformity of the right foot and ankle with 10 fixed equinus

It was decided that hamstring transfer was not possible. There was no patella and patella tendon making rerouting of transferred tendons almost impossible. Knee fusion was discounted due to a severe LLI combined with a deformed foot and ankle. It was therefore decided to proceed with a knee disarticulation.

Basic Principles The basic principle in this case is to achieve a child with a normal gait, normal weight-bearing, and no leg length discrepancy. This could be achieved through reconstructive procedures (knee fusion plus multiple limb lengthenings plus foot reconstructive surgery) or amputation. In this case, there was an 11 cm LLI. Knee fusion would have shortened the right leg further creating a greater LLI. Taking this into account, together with the foot and ankle deformity, it was decided that amputation was the best option to achieve the treatment goals of normal weight-bearing and normal gait.

Fig. 1 Preoperative lateral radiograph of the right knee in maximum extension showing absent patella, hypoplastic quadriceps, and severe flexion deformity

Fig. 2 Immediate postoperative photograph showing severe fixed flexion of the knee with the Ilizarov frame in situ

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Absent Knee Extensor Mechanism, Fixed Flexion Deformity, Leg Length Inequality and Foot Deformity in an 11. . .

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Fig. 3 (a–c) Photographs during treatment showing progressive deformity correction

Images During Treatment See Figs. 2, 3, and 4.

Technical Pearls There are no technical pearls for this case. The main learning point is that limb amputation and disarticulation should not be presented to the patient as a salvage procedure. It should be presented as one form of limb reconstruction. In this case, we believe that knee disarticulation and prosthesis fitting were the best option to reconstruct the leg.

Outcome Clinical Photos and Radiographs See Fig. 5.

Avoiding and Managing Problems

Fig. 4 Photograph of the knee just prior to frame removal

1. To avoid knee level asymmetry, a timely distal femoral epiphysiodesis should be considered to allow space for the prosthetic knee. 2. In a child older than 1 year of age, consultation with a psychologist should be considered prior to any amputation.

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References and Suggested Reading 1. Duerksen F, Rogalsky RJ, Cochrane IW. Knee disarticulation with intercondylar patellofemoral arthrodesis. An improved technique. Clin Orthop Relat Res. 1990;256:50–7. 2. Jeans KA, Browne RH, Karol LA. Effect of amputation level on energy expenditure during overground walking by children with an amputation. J Bone Joint Surg Am. 2011;93:49–56. 3. Lampasi M, Antonioli D, Donzelli O. Management of knee deformities in children with arthrogryposis. Musculoskelet Surg. 2012;96:161–9. 4. Loder RT, Herring JA. Disarticulation of the knee in children. A functional assessment. J Bone Joint Surg Am. 1987;69:1155–60. 5. Thomas B, Schopler S, Wood W, Oppenheim WL. The knee in arthrogryposis. Clin Orthop Relat Res. 1985;194:87–92.

Fig. 5 Postoperative long-leg standing radiograph showing the knee disarticulation and limb prosthesis

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David S. Feldman and Adam M. Kurland

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 233 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 234 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 235 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 235 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 235 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 236 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 236 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 236 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 236 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 239

Abstract

Hemangiomatosis is a rare bone disorder that may cause limb length inequality and asymmetrically affect the growth plate resulting in angular deformity. This patient is a 14 year old female with hemangiomatosis of the right tibia and fibula with a severe tibial deformity and limb length discrepancy. She was treated with tibial osteotomies, a Taylor Spatial Frame (TSF), bone marrow injections, bone graft, and open reduction internal fixation with William’s Nail & Synthes Locking Plate. In treating this disorder one needs to address the biology of the bone as well as the deformity itself. In doing so we were able to

D. S. Feldman (*) Pediatric Orthopedic Surgery, NYU Langone Medical Center, New York, NY, USA e-mail: [email protected] A. M. Kurland Pediatric Orthopedic Surgery, NYU Langone Medical Center, Hospital for Joint Diseases, New York, NY, USA e-mail: [email protected]

achieve an excellent outcome even in the presence of this aggressive disease.

Brief Clinical History The patient is a 14 year old female with a past history of Venous hemangiomatosis that affected her entire right tibia and damaged her knee, ankle, and physes. She underwent multiple minimally invasive interventional radiographic embolization procedures in addition to osteotomies and plate implantation to stabilize and correct the deformity of the affected bones. The damage to her physes resulted in proximal and distal tibial valgus and recurvatum deformities (Figs. 1 and 2). She underwent a peroneal nerve decompression and an acute angular correction with a tibial osteotomy and insertion of a rush rod and an A-O small fragment locking plate to achieve rotational stability. As well she underwent a medial distal tibial hemiepiphysiodesis to allow for gradual correction of her distal tibial valgus (Figs. 3 and 4). Her limb length discrepancy of 7.6 cm was corrected over the course of

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_301

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Fig. 1 Preoperative anteroposterior radiograph demonstrating proximal and distal tibial valgus as well as the moth-eaten appearance of the tibia and fibula consistent with the patient’s known hemangiomatosis

Fig. 3 Anteroposterior radiograph of the right tibia with intramedullary rod and locking plate in place demonstrating valgus knee, malformation of the tibia, and distal tibial valgus

2 years with proximal and distal osteotomies, a bifocal Taylor Spatial Frame (Figs. 5 and 6), a bone marrow injection, and a bone graft (Figs. 7, 8, and 9). After the TSF was removed, the bone demonstrated poor and delayed healing (Figs. 10 and 11). Ultimately, open reduction internal fixation (ORIF) with a William’s Nail and Synthes locking plate was utilized to stabilize the tibia (Figs. 12 and 13).

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

Preoperative Problem List

Fig. 2 Preoperative lateral radiograph revealing an anterior bowing of the tibia

• • • •

Hemangiomatosis of the tibia with severe deformity Bifocal deformity Shortening of the right tibia Delayed union of proximal and distal tibial lengthening sites

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235

Treatment Strategy Tibial osteotomies were performed both proximally and distally in an attempt to correct a combination of proximal and distal tibial valgus and recurvatum deformities while also achieving approximately 5 cm of length. Injections of bone marrow, bone grafts, and bone morphogenetic protein (BMP2) were later performed to promote bone growth and aid in uniting the tibial osteotomy sites. The patient also underwent an ORIF to realign the bone edges of the osteotomy sites which were then held in position with a William’s Nail and Synthes locking plate.

Basic Principles The primary goal in complex deformity correction of a biologically abnormal bone is restoration of the mechanical axis, regardless of the extent of the deformity. Delayed bone healing and poor regenerate are likely. Be prepared to utilize adjuvant treatments to achieve complete healing of the bone.

Images During Treatment See Figs. 5, 6, 7, 8, 9, 10, and 11. Fig. 4 Lateral radiograph of the right tibia showing a proximal recurvatum deformity

Fig. 5 Clinical photograph of the TSF applied to the right tibia

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Fig. 6 Radiograph of the right tibia 3 weeks after surgery showing the osteotomy proximally and distally

Technical Pearls

D. S. Feldman and A. M. Kurland

Fig. 7 Radiograph of the right tibia 4 months after surgery showing a delayed union of the regenerate bone at the osteotomy site. Two weeks later, 20 cc of bone marrow was taken from the patient’s right iliac crest and mixed with demineralized bone matrix and Hypaque. 16 cc of the bone marrow mixture was injected into the proximal site, and 2 cc were injected into the distal site

Even with careful and slow lengthening, the biology of the bone or the state of the patient’s health may impede bone healing and regeneration. The use of biologics and internal devices may aid in healing and by no means should be assumed to be a failure.

her crutch in the future. While it’s possible that the patient’s lower right limb may never be as strong as her left, treatment has allowed her to avoid the need for an above-knee amputation and prosthesis.

Outcome Clinical Photos and Radiographs

See Also in Vol. 3

See Figs. 12 and 13.

Complex Deformity After PFFD Treatment – Multifocal, Gradual Correction on Femur and Tibia with Monolateral External Fixation Treatment of Complex Deformity of the Lower Extremities in Hypophospatemic Rickets Using a Six-Pod Frame and Fixator Assisted Plating

Avoiding and Managing Problems A 1 cm shoe lift was recommended for better balance and symmetry in addition to the ongoing physical therapy for strengthening which should allow the patient to walk without

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Fig. 8 Radiograph of the right tibia 2 months after the bone marrow injection showing continued insufficient bone formation. Two months later, a graft was taken from the patient’s iliac crest, the nonunion tissue and bone at the distal site were debrided, and the bone graft and an insertion of BMP2 were applied to the distal nonunion site

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Fig. 9 Radiograph of the right tibia 2 months after the bone graft and BMP2 insertion

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Fig. 10 Anteroposterior radiograph of the right tibia following the removal of the TSF demonstrating delayed healing and weakness of the bone

Fig. 11 Lateral radiograph of the right tibia after removal of the TSF demonstrating delayed healing and weakness of the bone

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Fig. 12 Anterior radiograph of the tibia with William’s Nail and Synthes locking plate in place

Fig. 13 Lateral radiograph of the tibia with William’s Nail and Synthes locking plate in place

References and Suggested Reading 1. Idei N, Soga J, Hata T, Fujii Y, Fujimura N, Mikami S, Maruhashi T, Nishioka K, Hidaka T, Kihara Y, Chowdhury M, Noma K, Taguchi A, Chayama K, Sueda T, Higashi Y. Autologous bone-marrow mononuclear cell implantation reduces long-term major amputation risk in patients with critical limb ischemia: a comparison of atherosclerotic

peripheral arterial disease and Buerger disease. Circ Cardiovasc Interv. 2011;4(1):15–25. 2. Singh AK, Shetty S, Saraswathy JJ, Sinha A. Percutaneous autologous bone marrow injections for delayed or non-union of bones. J Orthop Surg (Hong Kong). 2013;21(1):60–4. 3. Weiner SD, Leeson MC. Hemangioma of the tibia. Orthopedics. 1990;13(12):1397–400.

Congenital Femoral Deficiency: Paley Type 1b and Paley Type 1c

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Dror Paley and Craig A. Robbins

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 242 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 242 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 242 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 248 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 248 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 250 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 250 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 250 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 250

Abstract

Congenital femoral deficiency (CFD) is a spectrum of severity of femoral deficiency and deformity. Deficiency implies a lack of integrity, stability, and mobility of the hip and knee joints. Deformity refers to bone malorientation, bone malrotation, and soft tissue contractures of the hip and knee. Both deficiencies and deformities are present at birth, nonprogressive, and of variable degree (Paley D, Standard SC, Treatment of congenital femoral deficiency, Chapter 28. In: Operative techniques in pediatric orthopaedics, p 177, 2010). The Paley classification system (Fig. 1) is based on the factors that influence lengthening reconstruction of the congenitally short femur. Type 1 has an intact femur with a mobile hip and knee with subtype 1a having normal ossification and type 1b having delayed ossification of the D. Paley (*) · C. A. Robbins Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA e-mail: [email protected]; [email protected]; [email protected]

subtrochanteric region and type 1c delayed ossification of the femoral neck (Paley D, Standard SC, Treatment of congenital femoral deficiency, Chapter 28. In: Operative techniques in pediatric orthopaedics, p 177, 2010). This chapter illustrates the staged reconstructive treatment of two patients with Paley type 1b CFD.

Brief Clinical History Case 1 This is a 2 year old child born with Paley 1c neck-type CFD. She has an intact femur with delayed ossification of the femoral neck. She has severe flexion, varus, and external rotation deformities of the hip and a large leg length discrepancy. Case 2 This child was born with Paley 1b subtrochanteric-type CFD. She has an intact femur with delayed ossification of the subtrochanteric region. She has severe flexion, varus, and

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Paley Classification for Congenital Femoral Deficiency Type 1: Intact Femur with Mobile Hip and Knee

a. Normal ossification

b. Delayed ossification Subtrochanteric type

Type 2: Mobile Pseudarthrosis with Mobile Knee

c. Delayed ossification Neck type

Type 3: Diaphyseal Deficiency of Femur

a. Knee motion ≥ 45°

a. Femoral head mobile in acetabulum

b. Femoral head absent or stiff in acetabulum

Type 4: Distal Deficiency of Femur

c. b. Knee motion < 45° Complete absence of femur

Cartilage Bone

Fig. 1 Paley classification for CFD (Copyright 2007, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

external rotation deformities of the hip and a large leg length discrepancy. See Fig. 1.

• Flexion, varus, external rotation deformity of femur and hip joint • Acetabular dysplasia • Hypoplastic or absent cruciate ligaments

Preoperative Clinical Photos and Radiographs Treatment Strategy Case 1 See Figs. 2, 3, 4, and 5. Case 2 See Figs. 6 and 7.

Preoperative Problem List Cases 1 and 2 • Intact proximal femur with delayed ossification of the neck (case 1) or subtrochanteric region (case 2) • Leg length discrepancy

Beginning with the initial consultation, a limb reconstruction plan is formulated. An MRI is scheduled prior to surgery to allow classification and visualization of the relevant anatomy. Around 18 months of age, SuperHip and, often, SuperKnee procedures are performed [1, 2]. These involve comprehensive bone and soft tissue reconstruction to restore normal anatomic alignment and joint stability to allow for futurestaged limb lengthening.

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Fig. 2 Full-length standing X-ray of a 2 year old patient with Paley 1c neck-type CFD. Note the delayed ossification of the femoral neck, significant femoral shortening, acetabular dysplasia, and hypoplastic distal femoral and proximal tibia epiphyses

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Fig. 4 MRI cut showing an intact cartilaginous (delayed ossification) femoral neck (Copyright 2007, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

Fig. 5 Drawing of Paley 1c neck-type CFD; notice the greater trochanter is in close proximity to the pelvis; the palpable portion of the proximal femur laterally is known as the “bump”

Fig. 3 Close-up view of the right hip showing acetabular dysplasia and delayed ossification of the femoral neck

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Fig. 6 Full-length standing AP (left) and lateral (right) X-rays of a child with Paley 1b subtrochanteric-type CFD. Note the delayed ossification of the subtrochanteric region, severe angular deformity, femoral shortening, and acetabular dysplasia. The femoral neck is ossified

Fig. 7 MRI cut showing the cartilaginous tissue at the apex of the subtrochanteric angular deformity

Fig. 8 Surgical drawing: AP and lateral placement of guidewires. Perform a hip arthrogram. In the lateral c-arm position, rotate the femur to obtain a “bull’s-eye” with the ossific nucleus centered within the femoral neck and acetabular shadows. On the AP view, the superior wire is placed from the tip of the greater trochanter to the center of the femoral head. The neck wire is placed 45 to the first wire and is centered in the femoral head. Note the flexion deformity of the proximal femur

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Fig. 9 Surgical drawing: Cannulated chisel inserted over neck guidewire perpendicular to posterior edge of greater trochanter which is parallel to the floor

Fig. 12 Surgical drawing: the distal femur is internally rotated

Fig. 10 Surgical drawings: The 130 cannulated infant blade plate is inserted in the chisel track

Fig. 13 Surgical drawing: The bone ends are overlapped to determine how much shortening is required

Basic Principles

Fig. 11 Surgical drawing: A bone wedge is cut parallel and perpendicular to the plate

• Obtain MRI to allow classification of the proximal femoral anatomy. • Classify the CFD using the Paley classification. • For Paley type 1b cases, perform a SuperHip and, if needed, a SuperKnee in preparation for staged limb lengthening surgery.

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Fig. 14 Surgical drawing: A segment of bone is removed and the femur brought adjacent to the plate

Fig. 15 Surgical drawing: The plate is fixed with screws. BMP2 is inserted into a drill hole in the femoral neck superior to the blade plate to promote ossification of the cartilage

D. Paley and C. A. Robbins

Fig. 16 Surgical drawing: A Dega osteotomy of the pelvis is done to cover the femoral head. The previously resected diaphyseal bone is used to fill the gap

Fig. 17 Postoperative radiograph following SuperHip combined with SuperKnee procedure. BMP2 was inserted into the cartilaginous femoral neck. Fixation with 130 cannulated infant blade plate

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Fig. 18 Standing (a and b): AP (a) and lateral (b) X-rays 18 months after surgery. The neck is fully ossified. Note the growth of the distal femur comparing the distance of the distal end of the plate to the physis compared with image 17

Fig. 19 AP right femur: First lengthening performed at age 4 with Smith and Nephew Modular Rail System (MRS) external fixator with articulation across the knee joint and fixation to the tibia to protect the knee from subluxation. Note the blade portion of the plate protecting the femoral neck

Fig. 20 Standing AP right lower extremity after 8 cm of lengthening achieved

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Fig. 21 Standing AP both lower extremities after removal of external fixator with Rush rodding of femur to prevent fracture

• Use the Paley multiplier method to predict limb length discrepancy at skeletal maturity and, based on this discrepancy, determine the number of lengthenings and epiphysiodesis timing needed to equalize limb length discrepancy. • Perform serial lengthenings about 4 years apart with a goal of 5–8cms each time. • To succeed, ensure that the patient undergoes daily aggressive physical therapy to maintain hip and knee range of motion. • Clinical follow-up including radiographs are needed every 2 weeks during the distraction phase. • Always stabilize the knee joint during femur lengthening; use hinged external fixation articulated to the tibia (or an HKAFO with internal lengthenings at older ages). • Rod or plate the femur at the time of ex-fix removal to prevent refracture.

D. Paley and C. A. Robbins

Fig. 22 Standing AP X-ray both lower extremities 1 year after SuperHip for Paley 1b subtrochanteric-type CFD. The deformity is fully corrected and the femur and pelvis are fully healed

Images During Treatment Case 1 See Figs. 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, and 21. Case 2 See Figs. 22 and 23.

Technical Pearls The SuperHip and SuperKnee are technically demanding reconstructive procedures for patients with CFD. The details have been previously published [1, 2] and should be familiar to the surgeon. A critical concept is that femoral lengthening cannot proceed until the hip and knee joints have been stabilized with these “preparatory” procedures.

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Fig. 23 AP X-ray left femur after 6 cm femoral lengthening with MRS; note hinged knee joint with fixation to the tibia to prevent knee subluxation

Fig. 24 Case 1: Standing AP both lower extremities during second lengthening at age 8 using 8.5 mms Precice implantable lengthening nail (Ellipse Technologies, Irvine, CA). Note hemi-epiphysiodesis for valgus

• Drape the entire hemi-pelvis from the ribcage superiorly and midline anteriorly and posteriorly. • Place a removable bump under the hip and use loupes to allow adequate exposure and visualization during the soft tissue releases; remove the bump for the bone procedures. • Subcutaneous dissection follows a single plane to the fascia in order to raise full-thickness flaps. • Perform the soft tissue releases prior to the bone procedures. • Obtaining perfect AP and lateral fluoroscopic “bull’s-eye” views of the hip with arthrography is critical to correct placement of the femoral neck guidewire. • The chisel and blade plate must be placed centrally within the femoral neck; align the chisel perpendicular to the posterior border of the greater trochanter. • Place a guidewire into the cannulated blade plate to help guide screws, BMP placement, and determine femoral anteversion. • Consider doing the femoral shortening osteotomy after the pelvic osteotomy because the amount of femoral shortening may change.

• Resect the superior portion of the iliac wing as needed to allow reapproximation of the apophysis. • If the fascia lata is not used for knee ligament reconstruction (SuperKnee), it must be completely removed or released. • The first femoral lengthening can be performed 12 months after the preparatory surgeries, assuming the femoral neck has ossified and the femur is of sufficient length for an external fixator. • A lateral view knee arthrogram is helpful in children to visualize and overlap the posterior condyles of the femur to identify the center of rotation of the knee for the knee hinge. • Ideally the first lengthening is before 5 years and the second after 7 years of age because children between 5 and 7 years may have psychological difficulties with limb lengthening. • The knee joint must be protected during lengthening; span a femoral fixator to the tibia with a hinge or use an HKAFO with a drop-lock knee in cases of internal lengthening.

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Avoiding and Managing Problems • Formulate a comprehensive limb reconstruction plan prior to treatment and ensure patient/family compliance and surgeon technical expertise. • Stabilize the hip and knee joints prior to lengthening. • Protect the knee joint during lengthening with a fixator or a brace to avoid subluxation. • Monitor the hip and knee joint during lengthening. • Knee joint mobility/deficiency rather than hip joint mobility/deficiency is the most important determining factor for functional outcome and reconstructibility. • Maintain hip and knee motion during treatment with physical therapy.

Cross-References

Fig. 25 Case 2: Standing AP both lower extremities at age 6 years; the rush rod was inserted at the time of fixator removal to prevent fracture

• Closely monitor the hip and knee joint stability during lengthening. • Early nerve irritation may be treated by slowing the rate of lengthening; persistent symptoms warrant nerve decompression. • Internally stabilize the femur (rush rod or plate) at the time of external fixator removal to avoid fracture.

Outcome Clinical Photos and Radiographs See Figs. 24 and 25.

▶ Congenital Femoral Deficiency: Paley Type 2a ▶ Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral Lengthening Associated with Flexible Intramedullary Nailing ▶ Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction ▶ Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral Deficiency and Femoral Retroversion ▶ Femoral Lengthening with MAC External Fixation System ▶ Knee Subluxation During Femoral Lengthening in a Six Year Old Boy with Congenital Coxa Vara and Congenital Short Femur ▶ Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD)

References and Suggested Reading 1. Paley D, Standard SC. Lengthening reconstruction surgery for congenital femoral deficiency, Chap 29. In: Robert RS, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: CRC Press; 2006. 2. Paley D, Standard SC. Treatment of congenital femoral deficiency, Chapter 28. In: Flynn JM, Wiesel SW, editors. Operative techniques in pediatric orthopaedics. Lippincott Williams & Wilkins; 2010. p. 177.

Congenital Femoral Deficiency: Paley Type 2a

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Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 251 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 252 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 252 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 252 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253 Superhip2 Procedure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 253 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 256 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 256 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 256 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 259

Abstract

Congenital femoral deficiency (CFD) is a spectrum of severity of femoral deficiency and deformity. Deficiency implies a lack of integrity, stability, and mobility of the hip and knee joints. Deformity refers to bone malorientation, bone malrotation, and soft tissue contractures of the hip and knee. Both deficiencies and deformities are present at birth, nonprogressive, and of variable degree (Paley D, Standard SC Lengthening reconstruction surgery for congenital femoral deficiency. In: Rozbruch SR, Ilizarov S (eds) Limb lengthening and reconstruction surgery, ch 29. Informa Healthcare, New York, pp 393–428, 2007). The Paley classification system is based on the factors that influence lengthening reconstruction of the D. Paley (*) · C. A. Robbins Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA e-mail: [email protected]; [email protected]; [email protected]

congenitally short femur. Type 2 has a mobile proximal femur pseudarthrosis with a mobile knee. Type 2a has a femoral head mobile in the acetabulum, 2b has a femoral head fused to the acetabulum, and type 2c has absent femoral head and acetabulum. The SuperHip 2 (SH2) procedure for types 2a and some 2b combines bone and soft tissue reconstruction to reliably achieve union between the upper femur and the femoral head with the creation of a new femoral neck. The treatment for types 2c (absent femoral head) and 2b fused femoral heads that are not candidates for SH2 is a pelvic support osteotomy.

Brief Clinical History This is a child born with congenital femoral deficiency Paley 2a with severe leg length discrepancy and hip flexion, adduction, and external rotation deformity. MRI showed that there was a true pseudarthrosis of the femoral neck. She has a 30


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Paley Classification for Congenital Femoral Deficiency Type 1: Intact Femur with Mobile Hip and Knee

a. Normal ossification

b. Delayed ossification Subtrochanteric type

Type 2: Mobile Pseudarthrosis with Mobile Knee

c. Delayed ossification Neck type

Type 3: Diaphyseal Deficiency of Femur

a. Knee motion ≥ 45°

b. Femoral head absent or stiff in acetabulum

Type 4: Distal Deficiency of Femur

c. b. Knee motion < 45° Complete absence of femur

Fig. 1 Paley Classification of Congenital Femoral Deficiency. Type 1 has an intact femur with mobile hip and knee, type 2 has a mobile pseudarthrosis and a mobile knee with the greater trochanteric apophysis present, type 3 has diaphyseal deficiency with an absent greater

a. Femoral head mobile in acetabulum

Cartilage Bone

trochanteric apophysis, and type 4 has distal deficiency of the femur (Copyright 2007, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

fixed flexion deformity of the knee. At age 3 she was treated by the SuperHip2 procedure to construct a femoral neck connecting the femoral head to the femoral shaft (Fig. 1).

Preoperative Clinical Photos and Radiographs See Figs. 2 and 3.

Preoperative Problem List • Femoral neck pseudarthrosis • Leg length discrepancy • Flexion, varus, external rotation deformity of proximal femur • Knee flexion deformity

Treatment Strategy Beginning with the initial consultation, a limb reconstruction plan is formulated. An MRI is scheduled prior to surgery to allow classification and visualization of the relevant anatomy. After age 2 a SuperHip2 is performed [2, 3]. This reconstructs

Fig. 2 Preoperative lateral (left) and AP (right) of right femur. The femoral head is mobile in the acetabulum making this a type 2a. There is a 30
fixed flexion deformity of the knee joint (Copyright 2007, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

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the femoral neck and converts the Paley CFD 2a into a type 1a (intact femur) and allows further treatment as for a 1a. •

Basic Principles • Obtain MRI to allow classification of the proximal femoral anatomy. • Classify the CFD using the Paley classification. • For Paley type 2a and some 2b cases perform a SH2 in preparation for limb lengthening surgery. An external

• • • • •

fixator is needed in addition to internal fixation to neutralize the forces on the femoral head-neck junction. Use the Paley multiplier method to predict limb length discrepancy at skeletal maturity and based on this determine the number of lengthenings and epiphysiodesis timing needed to equalize limb length discrepancy. Perform serial lengthenings about 4 years apart with a goal of 5–8 cm each time. To succeed ensure the patient undergoes daily aggressive physical therapy to maintain hip and knee range of motion. Clinical follow-up including radiographs are needed every 2 weeks during the distraction phase. Always stabilize the knee with external fixation articulated to the tibia. Rod or plate the femur at the time of ex fix removal to prevent refracture.

Images During Treatment Superhip2 Procedure See Figs. 4, 5, 6, 7, 8, 9, 10, and 11.

Technical Pearls

Fig. 3 Preoperative MRI of the right hip showing the femoral head in the acetabulum

Fig. 4 The proximal femur is mobilized on a soft tissue pedicle. It is rotated 135
to create a femoral neck connecting the femoral head to the femoral shaft. The greater trochanter remains in place as the attachment for the hip abductors. The femoral head is fixed to the new femoral neck with threaded k-wires

The SuperHip2 is a technically demanding reconstructive procedure for patients with CFD 2a. A critical concept is that femoral lengthening cannot proceed until the hip and knee joints have been stabilized with “preparatory” procedures such as the SuperHip and SuperKnee.

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Fig. 5 The femoral neck is fixed to the femoral shaft with a Rush rod and tension band wire for compression and rotational control

Fig. 6 The limited fixation of the femoral head to neck is neutralized by a spanning external fixator from the pelvis to the femur to the tibia. This is left in place for 4 months until union occurs

• The same approach as the SuperHip is used (see ▶ Chap. 37, “Congenital Femoral Deficiency: Paley Type 1b and Paley Type 1c”). The operation remains the same until the subtrochanteric osteotomy. • The hip capsule is opened anteriorly. Femoral head cartilage is removed in 1 mm slices to expose the ossific nucleus. • The abductors are released off of the proximal femur. The external rotators are removed supraperiosteally. The

Fig. 7 Postoperative radiograph following SuperHip2 (SH2) procedure with posterior capsulotomy of knee joint to achieve full knee extension. The knee is temporarily fixed fully extended by a transarticular k-wire. A screw was used to fixate the femoral head instead of k-wires

vastus lateralis and medial femoral circumflex artery serve as the pedicle for this bony segment. • The proximal lateral cortex is partially decorticated, as it will eventually be oriented inferiorly and attached to the femoral shaft.

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Congenital Femoral Deficiency: Paley Type 2a

Fig. 8 The femoral head is united to the femoral neck and the femoral neck is united to the shaft of the femur. Note the growth of the distal femur that has occurred following the surgery by comparing the distance of the distal end of the Rush rod to the physis on this radiograph compared to figure 7

• The first bone cut is made about 4–5 cm long, perpendicular to the femoral shaft, creating the new femoral neck and this segment is rotated 135
on its soft tissue pedicle; the greater trochanter moves distal and lateral, and the distal cut end of the subtrochanteric osteotomy is rotated superiorly to fix to the ossific nucleus of the femoral head. • The trochanteric segment is predrilled with a 1.5 mm drill in four places around its cortical periphery. 5/6400 threaded K-wires are run retrograde through the femoral neck and set to the same length. This bone is then drilled at 45
to its long axis for insertion of a Rush rod. This is drilled in a way to miss the path of the four wire holes and into the area previously decorticated. The distal

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Fig. 9 Radiograph following the first lengthening (8 cm) at age 6. A contralateral epiphysiodesis was done at age 9

femur intramedullary diaphysis is prepared with a 3.2 mm drill bit. Dental wire is also placed around this segment underneath the soft tissue pedicles, in preparation for fixation. • The new femoral neck is then attached to the head. The four threaded K-wires are advanced carefully, holding the head with the dental wire. Verify that the wires are not intra-articular with fluoroscopy and a Freer elevator. If happy with the wire fixation, carefully tighten down the dental wire to fix the two segments together. • The second subtrochanteric osteotomy is then made at 45
to the shaft of the femur, removing a trapezoidal piece to provide adequate shortening of the femur. The cut should be made with the leg internally rotated to create femoral neck anteversion. The rush rod is then placed through the trochanteric piece into the femoral shaft, and the dental wire tightened down. The abductor slide is performed as

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Avoiding and Managing Problems • Formulate a comprehensive limb reconstruction plan prior to treatment and ensure patient/family compliance and surgeon technical expertise. • Preserve soft tissue attachments and blood supply to the mobilized proximal femur segment. • Ensure femoral head mobility within the joint (resect the cartilage bridge if needed). • Use dental wire as needed for additional stability of head to neck and neck to shaft. • Neutralize forces across the hip and knee with an external fixator for 4 months. • Stabilize the hip and knee joints prior to limb lengthening. • Protect the knee joint during lengthening with a fixator or a brace to avoid subluxation. • Monitor the hip and knee joint during lengthening. • Maintain hip and knee motion during treatment with physical therapy.

Cross-References

Fig. 10 AP radiograph during second lengthening (8 cm) at age 10

needed, and the abductors are repaired to the greater trochanter. To neutralize the forces on the femoral head and neck, an external fixator is applied from the pelvis to the tibia for 4 months.

Outcome Clinical Photos and Radiographs See Figs. 12, 13, 14, 15, 16, and 17.

▶ Congenital Femoral Deficiency: Paley Type 1b and Paley Type 1c ▶ Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral Lengthening Associated with Flexible Intramedullary Nailing ▶ Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction ▶ Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral Deficiency and Femoral Retroversion ▶ Femoral Lengthening with MAC External Fixation System ▶ Knee Subluxation During Femoral Lengthening in a Six Year Old Boy with Congenital Coxa Vara and Congenital Short Femur ▶ Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD)

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Congenital Femoral Deficiency: Paley Type 2a

Fig. 11 AP (left) and lateral (right) radiographs following second lengthening with varusization of femoral neck

Fig. 12 Final AP (a) and lateral (b) radiographs after third lengthening at age 14. A submuscular locking plate was used to prevent refracture at the time of fixator removal. A plate was chosen to avoid infections since the pins site infections at the time precluded rod insertion

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Fig. 15 Knee extension after SH2, capsulotomy and first lengthening. Note the 90
of hip flexion

Fig. 13 Clinical photograph at age 8 after recovery from first lengthening

Fig. 14 Knee and hip flexion after SH2, capsulotomy and first lengthening

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Congenital Femoral Deficiency: Paley Type 2a

259

Fig. 16 Hip abduction after SH2 and first lengthening

Fig. 17 Hip internal and external rotation after SH2 and first lengthening

References and Suggested Reading 1. Herzenberg JE, Branfoot T, Paley D, Violante FH. Femoral nailing to treat fractures after lengthening for congenital femoral deficiency in young children. J Pediatr Orthop B. 2010;19(2):150–4. 2. Paley D, Standard SC. Lengthening reconstruction surgery for congenital femoral deficiency. In: Rozbruch SR, Ilizarov S, editors. Limb

lengthening and reconstruction surgery, ch 29. New York: Informa Healthcare; 2007. p. 393–428. 3. Paley D, Standard SC. Treatment of congenital femoral deficiency. In: Wiesel S, editor. Operative techniques in orthopaedic surgery. Philadelphia: Lippincott Williams & Wilkins; 2010. p. 1202–23. 4. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. J Bone Joint Surg Am. 2000;82:1432–46.

Congenital Femoral Deficiency: Paley Type 2c

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Dror Paley and Craig A. Robbins

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 261 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 263 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 264 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 264 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 264 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 266

Abstract

Congenital femoral deficiency (CFD) is a spectrum of severity of femoral deficiency and deformity. Deficiency implies a lack of integrity, stability, and mobility of the hip and knee joints. Deformity refers to bone malorientation, bone malrotation, and soft tissue contractures of the hip and knee. Both deficiencies and deformities are present at birth, nonprogressive, and of variable degree (Paley D, Standard SC Lengthening reconstruction surgery for congenital femoral deficiency. In: Rozbruch SR, Ilizarov S (eds) Limb lengthening and reconstruction surgery, ch 29. Informa Healthcare, New York, pp 393–428, 2007). The Paley classification system is based on the factors that influence lengthening reconstruction of the congenitally short femur. Type 2 has a mobile proximal femur D. Paley (*) · C. A. Robbins Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA e-mail: [email protected]; [email protected]; [email protected]

with a mobile knee. The previous classification had only 2a and 2b which both had a mobile pseudarthrosis. Type 2b included fused and absent femoral head. Since the SuperHip (SH) 2 procedure (see Chap. 31, “Congenital Femoral Deficiency: Paley Type 2a”) can be done for some type 2bs, I separated it into type 2c where there is no femoral head. Paley type 2c have a greater trochanter present but no femoral head. The treatment for type 2b fused femoral heads that are not candidates for SH2, and for cases that have no femoral head, what I now refer to as 2c, is a pelvic support osteotomy.

Brief Clinical History This is a 10 year old girl with Paley type 2c congenital femoral deficiency (CFD, Figs. 1 and 2). Her opposite femur is short such that her total limb length discrepancy is not as large as it otherwise would have been. She walks with a significant Trendelenburg gait.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_32

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Paley Classification for Congenital Femoral Deficiency Type 1: Intact Femur with Mobile Hip and Knee

a. Normal ossification

b. Delayed ossification Subtrochanteric type

c. Delayed ossification Neck type

Type 3: Diaphyseal Deficiency of Femur

a. Knee motion ≥ 45°

c. b. Knee motion < 45° Complete absence of femur

Type 2: Mobile Pseudarthrosis with Mobile Knee

a. Femoral head mobile in acetabulum

b. Femoral head absent or stiff in acetabulum

Type 4: Distal Deficiency of Femur

Cartilage Bone

Fig. 1 Paley classification of congenital femoral deficiency. Type 2 have a mobile hip and knee. In this older classification, (a) has a mobile femoral head and (b) has either a fused or absent femoral head. There is now a type (c) in which the femoral head is absent

Fig. 2 Paley CFD Type 2 (a) (left), (b) (middle), and (c) (right). The femoral head is mobile in (a), fused and stiff in (b), and absent in (c)

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Congenital Femoral Deficiency: Paley Type 2c

Preoperative Clinical Photos and Radiographs See Fig. 3.

263

allows the hip to lock in extension, and prevents Trendelenburg by using up all the available adduction. Lengthening and varusization to align the extremity are performed through the distal osteotomy. Technical details are well described in the literature [2, 6, 7].

Preoperative Problem List • • • •

Absent femoral head and acetabulum Unstable hip with proximally migrated femur Leg length discrepancy Weak abductors with Trendelenburg gait

Treatment Strategy The pelvic support osteotomy (PSO) is an old operation used to stabilize the hip in the absence of a femoral head. The valgus-extension position of the proximal femur places it in maximum adduction and eliminates fixed flexion deformity. This increases the abductor lever arm, creates a fulcrum,

Basic Principles • Classify the CFD using the Paley classification. • Use the Paley multiplier method to predict limb length discrepancy at skeletal maturity, and based on this determine the number of lengthenings and epiphysiodesis timing needed to equalize limb length discrepancy. • Perform serial lengthenings about 4 years or more apart with a goal of 5–8 cms each time. (In this case we exceeded this limit because the patient traveled from Australia and was limited in her ability to have more than two lengthenings. She also maintained 90 of knee motion throughout both lengthenings.) • To succeed ensure the patient undergoes daily aggressive physical therapy to maintain hip and knee range of motion. • Clinical follow-up including radiographs is needed every 2 weeks during the distraction phase. • Always stabilize the knee with external fixation articulated to the tibia. • It is best to defer pelvis support osteotomy until skeletal maturity to avoid remodeling of the bend in the femur. To prevent proximal migration of the femur, extend the external fixation to the pelvis.

Images During Treatment See Figs. 4, 5, and 6.

Technical Pearls

Fig. 3 Preoperative AP, both femurs. The left femur is short and proximally migrated. There is no femoral head or acetabulum present

• The preoperative supine maximum adduction AP pelvis X-ray dictates level of proximal osteotomy. • Add 15 to the preoperative femoro-pelvic adduction angle measured on the AP single-leg stance X-ray to determine proximal valgus angulation. • Place the proximal external fixator pins to allow valgus, extension, and internal rotation through the proximal osteotomy. • Place the distal osteotomy at the level that allows extremity realignment and lengthening.

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D. Paley and C. A. Robbins Fig. 5 The pelvic support angulation remodeled and the leg length difference was 10 cms at skeletal maturity

Fig. 4 A pelvic support osteotomy combined with a distal lengthening was performed. A total of 13 cms of lengthening was achieved while maintaining good knee range of motion

Outcome Clinical Photos and Radiographs See Figs. 7 and 8.

Avoiding and Managing Problems • Obtain the requisite preoperative radiographs to plan the level and degree of proximal osteotomy correction. • Place enough hip extension proximally to allow the hip to lock.

• Consider removing and replacing the proximal pins one at a time through a new skin incision to release tethered soft tissues after the acute correction. • Flex and extend the hip and knee fully after the fixator is applied to release tight soft tissues (e.g., iliotibial band).

Cross-References ▶ Ilizarov Hip Reconstruction for Post Infective Femoral Head Destruction ▶ Septic Destruction of the Hip and Significant LLD Treated by Pelvic Support Osteotomy and Femoral Lengthening

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Congenital Femoral Deficiency: Paley Type 2c

Fig. 6 At age 16 she underwent a second pelvic support osteotomy with 10 cms lengthening

Fig. 8 Final knee range of motion at age 26, 10 years after the second lengthening

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Fig. 7 AP standing final radiograph showing limb length equalization and good alignment

266

References and Suggested Reading 1. Herzenberg JE, Branfoot T, Paley D, Violante FH. Femoral nailing to treat fractures after lengthening for congenital femoral deficiency in young children. J Pediatr Orthop B. 2010;19(2):150–4. 2. Inan M, Bowen RJ. A pelvic support osteotomy and femoral lengthening with monolateral fixator. Clin Orthop Relat Res. 2005;440:192–8. 3. Paley D, Standard SC. Lengthening reconstruction surgery for congenital femoral deficiency. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery, ch 29. New York: Informa Healthcare; 2007. p. 393–428.

D. Paley and C. A. Robbins 4. Paley D, Standard SC. Treatment of congenital femoral deficiency. In: Wiesel S, editor. Operative techniques in orthopaedic surgery. Philadelphia: Lippincott Williams & Wilkins; 2010. p. 1202–23. 5. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. J Bone Joint Surg Am. 2000;82:1432–46. 6. Rozbruch S. Robert: Ilizarov Hip reconstruction. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery, ch 26. New York: Informa Healthcare; 2007. p. 357–68. 7. Rozbruch SR, Paley D, Bhave A, Herzenberg JE. Ilizarov hip reconstruction for the late sequelae of infantile hip infection. J Bone Joint Surg. 2005;87(5):1007–18.

Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral Lengthening Associated with Flexible Intramedullary Nailing

40

Dmitry A. Popkov and Pierre Lascombes

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 268 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 268 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 268 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 268 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 271 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 272 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273

Abstract

Congenital femoral length discrepancy (Pappas type VIII) is associated with valgus deformity of the knee joint caused by hypoplasia of the lateral condylar and slight femoral neck anteversion or retroversion. Femoral lengthening and deformity correction can be successfully performed with the combined technique: circular external fixator and flexible intramedullary nailing. In this case the application of the abovementioned method is described. It enabled the period of external fixation to be shortened significantly while correcting the length discrepancy and deformity. The chosen moment of lengthening before

D. A. Popkov (*) Russian Ilizarov Scientific Center for Restorative Traumatology and Orthopaedics, Kurgan, Russia e-mail: [email protected]

prepubertal growth acceleration provided equal growth of the lengthened and intact femurs.

Brief Clinical History The initial consultation of the patient was at 6.5 years of age, when the parents presented to the hospital with shortening of the right lower limb and lameness. On physical examination, the upper limbs and spine were unremarkable, as well as the left lower extremity. It was decided to perform surgical lengthening and correction at the age of 7 years and 6 months. At that time, the length discrepancy of the right femur was 3 cm and the tibia – 1 cm. Valgus deformity of the knee (aLDFA: 70 ), limited internal rotation of the hip joint (Netter angle: 5 ) were observed. Instability of the knee joint was not noted.

P. Lascombes Division of Pediatric Orthopaedics, University of Geneva, Geneva, Switzerland e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_346

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Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List – – – –

Right lower limb length discrepancy (Pappas type VIII) 4 cm LLLD (expected to be 6.5 cm at skeletal maturity) Knee valgus deformity (aLDFA: 70 ) External torsion of the femur

Treatment Strategy Taking into account the patient’s age (a long course of the consequent growth and over 2 years before the prepubertal growth spurt), insignificant contribution of the tibia in the total length discrepancy, and location of the deformity including the torsional one, it was suggested to perform lengthening of the femur and correction of associated deformities. The parents rejected epiphysiodesis of the left leg to equalize the

Fig. 1 Image of both legs, affected and normal

D. A. Popkov and P. Lascombes

length (surgery on the intact extremity and loss of 5 cm of the final height were considered unacceptable). To reduce the duration of external fixation, the use of the combined technique was planned. A unique external fixation was to be applied combining the Taylor Spatial Frame with the Ilizarov frame components (proximal short arch) to make the external fixator less bulky.

Basic Principles – In progressive limb lengthening with any type of external fixator, the principles of the Ilizarov method should be respected (stable elastic osteosynthesis, percutaneous osteotomy aimed to preserve periosteal and intramedullary blood supply of the bone, distraction initiated on the 5th postoperative day, optimal rate and rhythm of distraction, early weight bearing, and mobilization of adjacent joints).

Fig. 2 Radiograph of legs; length discrepancy is mostly in femur, moderate hypoplasia of the lateral femoral condylar

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Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral. . .

– Flexible intramedullary nailing is performed according to the basic principles, but the diameter of the nails should be 20–25 % of the medullar canal diameter. – Femoral growth should be preserved.

•

Images During Treatment

• •

See Figs. 3, 4, and 5.

• •

Technical Pearls • Using the short Ilizarov arch in addition to the TSF rings enables the surgeon to increase the fixation stability of the long proximal fragment and to make the external fixator less bulky. • Initially, just two wires or half-pins were inserted in the proximal and distal systems, and then the external fixator was assembled and intramedullary nails were inserted

Fig. 3 Image of the limb in fixator. Note: distraction and correction were performed between the 5/8 ring proximally and the ring of the hexapodal Taylor Spatial Frame distally. In proximal femoral metaphysis a short Ilizarov arch with two halfpins were placed

•

269

after the osteotomy, only after all the other wires and half-pins of the external fixator were added. Verticalization and walking with crutches were allowed since the 2nd or 3rd postoperative day; at the same time, mobilization of adjacent joints was initiated. Beginning of the distraction – on the 5th day. Rate and rhythm of distraction were defined according to radiographic markers of the reparative bone regeneration. Walking with full weight bearing was obligatory during fixation period. Disappearance of the “growth zone” and continuity of cortices at least on three out of four sides of the regenerate were indications for frame removal. Intramedullary nails were removed at an average of 6 months after frame removal, when at least 50 % of range of motion of adjacent joints was restored.

Outcome Clinical Photos and Radiographs See Figs. 6 and 7.

270 Fig. 4 Radiographs of the femur (AP, lateral) before distraction

Fig. 5 Radiographs of the femur during treatment: (a) end of distraction (56 days), lengthening gain is 4.5 cm, and average distraction rate around-the-clock is 0.8 mm/day. Notice: displacement of intramedullary wires distally caused by lengthening; (b) end of fixation (33 days), corticalization at the lengthening site, and “growth zone” of the regenerate disappeared

D. A. Popkov and P. Lascombes

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Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral. . .

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Fig. 6 After removal of the external fixator. flexible intramedullary nails remained. Final amount of lengthening is 4.5 cm and healing index is 21.1 days/cm

Avoiding and Managing Problems – Consultations with monitoring of the surgery are held at least one time a week during distraction period and at least one time every 2 weeks during fixation. – Intramedullary nails can bind in the proximal fragment if they are advanced to the level of the half-pins. This may

cause migration of the intramedullary nails into the medullar canal. To prevent it, the flexible nails should be inserted up to the upper third of diaphysis and halfpins should be placed more proximally in the metaphysis. – The combined technique requires systemic practice and experience of the surgeon in both methods: the external fixation and the flexible intramedullary nailing.

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Fig. 7 In 2 years after treatment: (a) complete recovery of range of motion of the knee and symmetrical axes of both limbs; (b) length of lower limbs remained equal, no deformity of the femur, and the right tibia still remained shorter than the intact one; (c) diagrams of femoral

Cross-References ▶ Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction ▶ Femoral Lengthening with MAC External Fixation System

D. A. Popkov and P. Lascombes

growth (Héchard and Carlioz graph), dashed line marks the lengthening period of the right femur. Apparently, growth of the lengthened femur is consistent with the intact one during the first 2 years after frame removal

▶ Staged Lengthening of 20 cm in the Femur and Tibia to Equalize Leg Lengths in a Growing Child

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Congenital Femoral Length Discrepancy Pappas Type VIII in a 7 Year Old Child Treated by Femoral. . .

References and Suggested Reading 1. Ilizarov GA. Clinical application of the tension-stress effect for limb lengthening. Clin Orthop. 1990;250:8–26. 2. Lascombes P. Flexible intramedullary nailing in children. Berlin/ Heidenberg: Springer; 2010. 3. Pappas AM. Congenital abnormalities of the femur and related lower extremity malformations: classification and treatment. J Pediatr Orthop. 1983;3:5–60.

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4. Popkov D, Popkov A, Haumont T, Journeau P, Lascombes P. Flexible intramedullary nail use in limb lengthening. J Pediatr Orthop. 2010;30(8):910–8. 5. Popkov D, Journeau P, Popkov A, Pedeutour B, Haumont T, Lascombes P. Analysis of segmental residual growth after progressive bone lengthening in congenital lower limb deformity. Orthop Traumatol Surg Res. 2012;98(6):621–8.

Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum)

41

Robert A. Hill

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 275 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 276 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 276 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 276 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 277 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 278 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 278 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 278

Abstract

Brief Clinical History

Congenital posteromedial bowing of the tibia is a rare, unilateral condition of variable severity that is usually associated with a calcaneovalgus foot at birth. Congenital posteromedial bowing is considered to be benign in that spontaneous improvement may be expected (Fig. 1(a–b)). In contrast, the opposite deformity pattern (anterolateral bowing) is more worrying because it is usually associated with pathologic conditions such as pseudarthrosis of the tibia, osteogenesis imperfecta, neurofibromatosis, and fibula dysplasia. The etiology of posteromedial bowing is unknown. The foot deformity responds quickly to stretching exercises but the bony deformity takes longer to improve. The remodeling of the tibial bowing may be incomplete and is usually associated with a variable leg-length discrepancy that may require limb reconstruction techniques. The size of the discrepancy may be related to the severity of the initial bowing.

The case illustrated is a male patient with isolated posteromedial bowing of the tibia. Initially, stretches and a cast were tried for a few months after birth but were ineffective and discontinued. The patient was kept under observation and some improvement of the bowing, particularly the medial bow, was noted. A 2 cm leg-length difference became apparent at the age of 4½ years and increased to approximately 7 cm by age 8. At the age of 9 the patient underwent application of a Taylor spatial frame for deformity correction and lengthening. Admission for inpatient physiotherapy was required because of knee and ankle contractures that developed during treatment. Overall, 5.4 cm of length was obtained with a satisfactory result. A second frame was applied at the age of 14 for residual leg-length discrepancy (the family declined epiphysiodesis). A further 4.5 cm of lengthening was achieved (Fig. 2).

R. A. Hill (*) Portland Hospital for Women and Children, London, UK e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_53

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Fig. 1 (a–b) Serial X-rays showing spontaneous improvement in the tibial and fibular bowing (the images do not represent the operative case described below). (a) Age 2 months. (b) Age 2 years. (c) Age 4 years

Preoperative Clinical Photos and Radiographs See Fig. 2.

Preoperative Problem List • Leg-length difference of 7 cm • Oblique plane deformity including rotation • CORA in diaphyseal bone with potential consequences for regenerate formation

Treatment Strategy This sort of limb reconstruction problem is best addressed with a spatial frame or similar because of the complex nature of the deformity. Posteromedial bowing sometimes has poor regenerate formation. This may be because the typical CORA for posteromedial bowing is in the diaphysis which is not an ideal site for lengthening. In a patient with a major length discrepancy, it may be wise to concentrate on fully correcting the deformity and limit the amount of desired lengthening. Any residual discrepancy can be dealt with later by

Fig. 2 Preoperative photograph prior to first lengthening

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Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum)

epiphysiodesis or a second lengthening with the osteotomy in a more favorable location.

277

Images During Treatment See Fig. 3 (a–d)

Basic Principles Technical Pearls Because of the diaphyseal location, it is important to perform a low-energy osteotomy. Stable fixation should be used since the frame will be used for several months. Careful, close follow-up with regular radiographs is recommended during the lengthening to monitor the regenerate formation (Figs. 3, 4, and 5).

If the surgeon pays attention to detail and follows the principles of limb reconstruction and circular fixator use, then the treatment of this condition can be predictably successful. The most common difficulty is poor regenerate formation. Therefore, it is important to carry out a low-energy osteotomy and

Fig. 3 (a–b) Treatment X-rays. (a) AP view after application of spatial frame. Note the level of the osteotomy. (b) Lateral X-ray. (c, d) AP and lateral X-rays at the completion of lengthening and deformity correction prior to consolidation of the regenerate

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Fig. 4 Clinical result after the first lengthening (Compare to Fig. 2)

not be too ambitious with the lengthening. If necessary, carry out a two-stage correction with the second lengthening being at or close to skeletal maturity.

Outcome Clinical Photos and Radiographs See Figs. 4 and 5.

Avoiding and Managing Problems Since the principal problem in the management of this condition is poor regenerate formation, the measures suggested above should be taken. The rotational correction may also be a factor in the poor regenerate formation. If the second lengthening is done at skeletal maturity and poor regenerate formation is encountered that does not respond to the usual measures, then the surgeon has the option of inserting a locked intramedullary nail. This can be a good way to deal with persistent failure of consolidation in a patient

Fig. 5 AP and lateral X-rays of the tibia after the second lengthening close to skeletal maturity. Note the lengthening has been carried out in the proximal tibia

increasingly disenchanted with the frame. If pin-site infection is an issue then subsequent nailing carries a risk of creating an intramedullary infection. An aggressive program of preoperative pin cleaning and preoperative intravenous antibiotics is usually effective at preventing this complication.

References and Suggested Reading 1. Kaufman SD, Fagg JA, Jones S, Bell MJ, Saleh M, Fernandes JA. Limb lengthening in congenital posteromedial bow of the tibia. Strateg Trauma Limb Reconstr. 2012;7(3):147–53. https://doi.org/ 10.1007/s11751-012-0145-4. Published online 16 Oct 2012. PMCID: PMC3482434

Congenital Short Femur and Fibular Hemimelia Staged Approach Frame and Nail

42

Jason S. Hoellwarth and S. Robert Rozbruch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 280 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 281 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 281 Outcome Clinical Photos and Radiographs (4-5) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 281 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 281 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284

Abstract

This case illustrates the management of leg length discrepancy and mechanical axis malalignment in the context of a skeletally immature patient with femoral deficiency and fibular hemimelia. This patient’s care featured principles of growth prediction, staged intervention with attention to preserving physeal growth, and the use of external hexapod and intramedullary nail lengthening. A noteworthy point for this patient is that despite his tibial valgus being corrected gradually, he nonetheless developed a peroneal nerve palsy; prompt release relieved symptoms and allowed corrections to goal.

Brief Clinical History This is a case of 12-year-old male with left congenital femoral deficiency and left fibular hemimelia. He had two prior foot surgeries. His chief complaint was left shorter than right leg discrepancy. He used a 5 cm left shoe lift and denied pain. His left limb deformity featured 7.5 cm discrepancy (contributions: 12 mm femur, 34 mm tibia, and 21 mm foot), mechanical axis 30 mm lateral, and grade 2 anterior-posterior knee instability.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2. J. S. Hoellwarth (*) Limb Lengthening and Complex Reconstruction Service, Hospital for Special Surgery, New York, NY, USA e-mail: [email protected] S. R. Rozbruch Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA e-mail: [email protected]

Preoperative Problem List 1. Left shorter than right leg length discrepancy (LLD) 7.5 cm (contributions: 12 mm femur, 34 mm tibia, and 21 mm foot)

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_399

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Fig. 1 (a) Clinical photograph of patient at initial consultation. The substantial left leg length deficiency and genu valgum are clearly visible (b)The long standing xray identifies the valgus mechanical axis and short left leg

2. 30 mm lateral mechanical axis 3. Lateral distal femoral angle (LDFA) 82 , medial proximal tibia angle 92 4. Unstable left knee 5. Predicted skeletal maturity LLD 8.8 cm

Treatment Strategy 1. Two-staged lengthenings to correct current LLD now and a future lengthening near skeletal maturity. 2. Left tibia-fibula lengthening and correction of valgus using an external fixator with simultaneous gastrocnemius tendon release.

3. Femoral lengthening with a motorized intramedullary nail to equalize leg lengths. 4. Consideration of distal medial femoral guided growth to address femur-based genu valgum at the time of tibia lengthening (this was not done). 5. Choose the bones to be lengthened based on its contribution to LLD and deformity; in this case, both femur and tibia contributed to LLD and valgus deformity.

Basic Principles 1. This patient required mechanical axis correction along with current limb length equalization and subsequent equalization at skeletal maturity.

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Congenital Short Femur and Fibular Hemimelia Staged Approach Frame and Nail

281

Images During Treatment See Figs. 3, 4, and 5.

Technical Pearls

Fig. 2 This radiograph illustrates the deformity planning for a hexapod fixator. The CORA is identified at the intersection of the proximal and distal tibial anatomic axis lines (10 valgus) and is 115 mm distal to the tibial plateau. Half-pin and wire/ring levels are further illustrated and measured before surgery. Establishing a complete outline prior to the surgery not only helps the surgeon prepare but also the operating assistants

2. The tibia length and alignment were corrected before the femur, using a hexapod fixator in order to preserve the proximal tibial growth plate. 3. The femur could have length and alignment corrected near skeletal maturity with a retrograde nail using fixatorassisted blocking screw technique. 4. Tibial valgus correction is best done gradually to minimize the risk of injury to the peroneal nerve.

Tibia 1. Fix tibia and fibula proximally and distally with a wire to avoid migration of fibula. 2. Correct valgus gradually to minimize the risk of peroneal nerve entrapment. 3. Decompression of the peroneal nerve was necessary here. A peroneal release at the index surgery could have been considered. Femur 1. Acute correction of valgus with fixator-assisted blocking screw technique. 2. Blocking screws are to be placed in the deformity concavity adjacent to the osteotomy based on preoperative planning. 3. The retrograde nail entry point in the distal femur fragment is critical and based on preoperative planning. 4. Acute deformity correction is stabilized with intraoperative fixator prior to reaming.

Outcome Clinical Photos and Radiographs (4-5) See Fig. 6.

Avoiding and Managing Problems 1. Neurologic changes can occur unexpectedly. This patient developed a peroneal nerve palsy two weeks after frame application. An immediate peroneal nerve decompression at the fibular neck level resolved his symptoms and lengthening successfully continued to goal. 2. Knee flexion contracture can usually be avoided with physical therapy; in this case an orthotic extension from the hexapod frame was helpful to correct the contracture.

282 Fig. 3 AP (a) and lateral (b) of the tibia 5 months after application, having completed distraction and consolidation. The distraction gap is clearly identifiable and the regenerate is robust and mineralizing. The diaphyseal tibial valgus has been corrected to make a straight tibia. Two distal rings were used to increase construct stability

J. S. Hoellwarth and S. R. Rozbruch

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Congenital Short Femur and Fibular Hemimelia Staged Approach Frame and Nail

Fig. 4 Long-leg radiograph of patient at skeletal maturity (age 15 years). The physes are all closed. His final LLD was measured at 60 mm (following tibial correction) with a lateral mechanical axis deviation of 30 mm valgus. The lateral distal femoral angle measured 82 with a medial proximal tibial angle of 90

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Fig. 5 Final long-leg radiograph after left femur lengthening before hardware removal. The acetabular sourcils are clinically equivalent distances from the ground, 2 mm different left and right. His left mechanical axis matches his right, at the position of the medial tibial spine. Both his knee joints are level with the ground. The left knee joint is 2.5 cm lower than the right and the patient does not report any concerns or activity limitations

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References and Suggested Reading 1. Iobst CA, Rozbruch SR, Nelson S, Fragomen A. Simultaneous acute femoral deformity correction and gradual limb lengthening using a retrograde femoral nail: technique and clinical results. JAAOSJournal of the American Academy of Orthopaedic Surgeons. 2018;26(7):241–50. 2. Rozbruch SR, Birch JG, Dahl MT, Herzenberg JE. Motorized intramedullary nail for management of limb-length discrepancy and deformity. JAAOS-J Am Acad Orthop Surg. 2014;22(7):403–9. 3. Fragomen AT, Rozbruch SR. Lengthening and deformity correction about the knee using a magnetic internal lengthening nail. Sicot-J. 2017;3:25.

Fig. 6 Upon completion of both procedures, the patient has straight mechanical axes on each leg. Although his knee heights are approximately 3 cm unequal, he is very active with sports

Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction

43

Robert A. Hill

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 285 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 286 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 286 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 286 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 288 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289

Abstract

Congenital shortening of the femur is a spectrum of deformity ranging from proximal focal femoral deficiency to a normal or almost normal femur which is a little short. There are a number of classifications mainly based on radiological appearance, but more recently classifications have been devised of more relevance to reconstruction surgery as they take into account some of the other features of this condition such as joint stability. These other features, together with the abnormal bone, have rightly given reconstruction and lengthening of congenital short femur a reputation for difficulty and complications. This case illustrates some of these difficulties and how they may be overcome.

Brief Clinical History A 9½ year old boy with congenital shortening of the left femur with a 6 cm leg-length difference was referred for limb reconstruction surgery. The hip was stable and the patient was classified as Paley type 1 – intact femur with mobile hip and knee. However, as is usually the case there were associated abnormalities, and preoperative assessment showed an associated fibula dysplasia, a ball-and-socket ankle joint, a minor degree of distal femoral valgus (Fig. 1), an increased external rotation at the hip, and an AP instability of the knee.

Preoperative Clinical Photos and Radiographs See Fig. 1.

R. A. Hill (*) Portland Hospital for Women and Children, London, UK e-mail: [email protected] © Crown 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_51

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follow-up during the lengthening process. Knee subluxation is a serious complication that can result in long-term morbidity [3], and in the congenital bone dysplasias, the main challenge has switched from achieving length to correcting alignment and preserving and maintaining joint function in joints which from the outset are abnormal in shape and often unstable due to ligamentous deficiency. In addition the preoperative assessment needs to take account of any social, domestic, and psychological issues which could affect the treatment program which is likely to take at least 6 months with some difficulties along the way.

Treatment Strategy

Fig. 1 Preoperative long-leg standing film

Large lengthenings in congenital short femur are more likely to be associated with complications. Therefore, the treatment strategy is to lengthen the femur 5–6 cm using a frame design that anticipates potential complications. The patient was seen in a preadmission clinic by a multidisciplinary team so that appropriate measures could be put in place for post-surgery physiotherapy, attendance at school, and psychological support. In this case it was felt the long-leg standing film did not show sufficient deviation of the mechanical axis to justify any treatment of the distal femoral valgus, but the knee was sufficiently unstable to consider ligament reconstruction for the anterior cruciate ligament. This is a relatively new strategy based on knee problems noted in the long-term follow-up of patients who underwent lengthening for congenital bone dysplasias – particularly congenital short femur. At present in these patients a MacIntosh extra-articular ligament reconstruction as described by Paley and Standard [4] is being used.

Preoperative Problem List Basic Principles Lengthening of congenital short femur is difficult because of the high incidence of complications. In this patient the potential or actual problems include: • Poor regenerate formation and fracture after frame removal • Knee stiffness and subluxation • Malalignment – distal femoral valgus and external rotation of the femur • Equinovalgus contracture of the ankle developing during lengthening • Pin site infections Potential problems need to be anticipated and where possible prevented by appropriate frame design and careful

The frame design is based on the author’s experience of lengthening Paley type 1 congenital short femur [1]. A proximal osteotomy is carried out over a pre-placed Rush pin; some acute correction of the external rotation can be carried out. The Rush pin is valuable in protecting the regenerate against fracture or loss of alignment as is demonstrated in this case (Fig. 4). Proximal fixation is by a combination of half pins and wires attached to an angled Russian arch; distal fixation uses one wire and two half pins inserted in such a way to avoid impaling the quadriceps as far as possible because this will cause knee stiffness. The knee is hinged with a multi-axial Russian-style hinge to protect against knee subluxation during lengthening yet permitting knee movement.

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Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction

Images During Treatment See Figs. 2, 3, 4, and 5.

Technical Pearls This is a “Russian-style” frame and it is important to pay attention to the alignment of the rings. Initially the site of the osteotomy is determined and predrilled; the Rush pin is then inserted down the intact femur. The first ring to be attached is the distal femoral ring which must be correctly aligned in both the AP and lateral views. Although the Rush pin is flexible, this is essentially an anatomical axis lengthening and the rings are aligned to this axis. The Russian arch has to be carefully positioned and sized to avoid impingement, and to facilitate this and the operation in general, it is essential the patient is positioned in such a way that there is free access all around the femur. In practice this is done by building up the table cushions under the torso but removing them from under the leg and supporting the tibia on a rest. A special table can also be used.

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Do not start the operation until free access is confirmed including image intensifier access. The Russian arch is then attached with wires and half pins. Russian arches are designed to permit the use of proximal femoral wires, but some knowledge of proximal femoral anatomy is required. In practice patients do not like the anterolateral to posteromedial wire, and I usually use an anteromedial to posterolateral wire and lateral half pins. Unfortunately the presence of the Rush pin can make it difficult to insert fixation. The femur in this condition is quite flat front to back, and it is in fact easier to insert the wires – not only because they are smaller but because of the shape of the femur. The frame design can be changed to an Italian arch fixed solely with half pins if there is anxiety about the use of a Russian arch (see below Fig. 7).

Fig. 3 Lateral view during lengthening. The multi-axial hinge can be seen between the distal femoral and the tibial ring. In follow-up it is important to check that there is no subluxation of the knee, and a good lateral X-ray of the knee must be obtained every time the patient attends for follow-up

Fig. 2 AP view during lengthening showing lengthening occurring over the Rush pin. Note the proximal Russian arch which is set at an angle and that the rings are widely spaced to recruit as much tissue as possible into the lengthening segment

Fig. 4 AP X-ray during consolidation phase. Note that there has been loss of fixation proximally, but the Rush pin has maintained alignment and it was felt unnecessary to add fixation at this stage

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Positioning the knee hinges can be difficult for a number of reasons. It is debatable whether simple hinges can be used instead of the multi-axial hinges, but the anatomy is not normal and the multi-axial hinge permits some tolerance in placement. The center of rotation of the knee should be marked by insertion of a wire which is cut short at the start of the procedure; this is mainly to prevent the surgeon using a hole on the ring that is subsequently needed for the hinge. When the hinge is placed, it must be tested to ensure the knee

R. A. Hill

moves smoothly and does not start to sublux; adjust the hinge position as necessary (Fig. 5).

Outcome Clinical Photos and Radiographs See Fig. 6.

Fig. 5 (a) Hinge being tested with knee in full extension (different patient, same pathology). (b) Knee flexed to 90 on hinge to check no subluxation

Fig. 6 (a) Final result of AP view. Note the broken half pins to be removed later. The Rush pin can be left in situ and may be useful if there is a further lengthening. It is of small diameter (3.4 mm) and not likely to cause a stress riser. (b) Final result of lateral view

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Avoiding and Managing Problems In this condition it is best to anticipate problems by attention to good technique of wire and half pin insertion, to protect the knee against subluxation (and protect a ligament reconstruction if this has been done). The reason for doing the osteotomy proximal is that it is then above the bulk of the quadriceps and this may be helpful in preventing knee stiffness. Carefully monitor the knee during follow-up; if subluxation is noted then the patient needs to be returned to theater (OR), and the knee reduced by removing the hinges and reducing the knee by manipulating the distal femoral and proximal tibial rings and then joining them with threaded rods. The knee will then have to stay locked in extension (avoid locking it in hyper extension in error), during the treatment. When the frame is removed, knee exercises are very important. If the knee remains very stiff after 12 months of rehabilitation, then a modified quadricepsplasty usually gives good results [2]. The other common complication is regenerate fracture or bending after frame removal. The Rush pin will prevent this, but if lengthening has been carried out without a Rush pin then intra medullary fixation or plating and grafting will be required. The Russian arch can be difficult to position, and if proximal wires are not going to be used then it can be substituted for an Italian arch as shown in Fig. 7. Proximal femoral wires on a Russian arch can be a useful technique in difficult cases with very short proximal femoral segments, and the arch itself is often used in the Ilizarov hip reconstruction. The level of the osteotomy for lengthening is a matter for discussion. There may be some concern about a proximal osteotomy as described here because of the risk of poor regenerate formation and many surgeons prefer a distal osteotomy. I have not experienced issues with poor bone formation, and having a proximal osteotomy allows the use of the Rush pin which has been very helpful in reducing the incidence of fracture or regenerate deformation post-frame removal. As noted above it can make it difficult to insert fixation, but the use of a Rush pin has made a big difference to our results [1]. Femoral lengthening in cases of growth arrest, where the bone and soft tissues are intrinsically normal, is much less prone to complications, and in these cases I do use a distal lengthening site as this makes the frame less cumbersome for the patient. Femoral frames of whatever design are awkward, and the introduction of suitable intramedullary lengthening devices may well change treatment strategies in the future.

Fig. 7 Congenital short femur lengthening with proximal Italian arch. Note the middle ring is empty, and an eight plate is being used to correct distal femoral valgus

Cross-References ▶ Ilizarov Hip Reconstruction for Post Infective Femoral Head Destruction

References and Suggested Reading 1. Aston W, Calder P, Baker D, Hartley J, Hill RA. Lengthening of the congenital short femur using the Ilizarov technique. J Bone Joint Surg (Br). 2009;91-B:962–7. 2. Hosalker H, Jones S, Chowdhury M, Hartley J, Hill R. Quadricepsplasty for knee stiffness after femoral lengthening in congenital short femur. J Bone Joint Surg. 2003;85B:261–4. 3. Jeong C, Inan M, Riddle EC, Gabos PG, Bowen JR. Knee arthritis in congenital short femur after Wagner lengthening. Clin Orthop Relat Res. 2006;451:177–81. 4. Paley D, Standard S. Lengthening reconstruction surgery for congenital femoral deficiency, Chap 29. In: Ilizarov S, Rozbruch R, editors. Limb lengthening and reconstruction surgery. New York: Informa Healthcare USA Inc; 2007.

Correction of Recurvatum Deformity with Osteotomy, External Fixator, and Epiphysiodesis

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David S. Feldman and Adam M. Kurland

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 291 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 294 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 294 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 294 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295

Abstract

Angular deformities with limb length inequality are difficult to treat with acute correction. Gradual correction is therefore often utilized to treat these deformities. A 12 year old female had a left proximal femoral chondrosarcoma that was resected and reconstructed with a left free fibular osseous microvascular flap. She later developed an insidious anterior proximal tibial growth arrest which resulted in a limb length inequality of 4.5cm and a 20 recurvatum deformity. These were treated simultaneously with the application of a multiplanar external fixator (Taylor Spatial Frame, “TSF”) and D. S. Feldman (*) Pediatric Orthopedic Surgery, NYU Langone Medical Center, New York, NY, USA e-mail: [email protected] A. M. Kurland Pediatric Orthopedic Surgery, NYU Langone Medical Center, Hospital for Joint Diseases, New York, NY, USA e-mail: [email protected]

epiphsyiodesis of the ipsilateral posterior proximal tibia. Sagittal plane deformities, such as recurvatum or procurvatum, with concomitant limb length inequality can readily be treated with gradual correction.

Brief Clinical History A 12 year old competitive female gymnast sustained a right proximal tibia fracture at the age of 10 after landing incorrectly on her right leg. She was taken to a local emergency room where her leg was set and placed in a long leg cast for 8 weeks followed by 6 weeks of physical therapy after the cast was removed. Intermittent pain over the next year and 4 months led her family to seek additional treatment. X-rays revealed that the tibial fracture had damaged her physis causing it to close prematurely, resulting in a mild limb length discrepancy, her left limb being longer than her right, a recurvatum deformity that caused hyperextension of her

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right knee of 20 (Figs. 1 and 2), a limb length inequality of 4.5 cm, and persistent pain with activity.

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was removed after 3 months and bilateral tibial epiphysiodesis was performed to prevent the recurrence of the deformity or a further limb length inequality.

Preoperative Clinical Photos and Radiographs Basic Principles See Figs. 1 and 2.

Preoperative Problem List • Anterior physeal arrest of the right proximal tibia • Right tibia recurvatum deformity • Lower limb length discrepancy of 4.5 cm

Occult physeal fractures should be suspected when patients have persistent pain or dysfunction. Deformity correction should involve all aspects of the malalignment, i.e., deformity and length.

Images During Treatment See Figs. 3, 4, and 5.

Treatment Strategy An osteotomy was performed on the right tibia along with a fibular osteotomy to correct the recurvatum deformity to achieve deformity correction and equal limb length. A preconstructed Taylor Spatial Frame affixed to the tibia and fibula was used to achieve gradual correction. The frame

Technical Pearls Understanding the area of deformity to allow for correction around the angulation can recreate a normal limb. Fig. 2 Radiograph of the right lower limb showing recurvatum deformity

Fig. 1 Clinical photograph of the lower limbs showing recurvatum of the right knee

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Fig. 3 Clinical photograph (a) and lateral radiograph (b) of the Taylor Spatial Frame applied to the right leg with a wire and five half-pins showing the osteotomy and distraction of the limb to correct the length discrepancy Fig. 4 Anteroposterior and lateral radiographs of the right lower extremity 3 weeks postoperatively displaying the proximal osteotomy, limbs of equal length, and a straight right lower limb. Instructions were given at this point for full extension of the leg in physical therapy, a prescription for an EXOGEN machine to be used twice daily, and weaning off of any antibiotics

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Fig. 5 Anteroposterior and lateral radiographs of the right lower limb 1 week prior to the removal of hardware showing no limb length inequality and a mostly healed and corrected recurvatum deformity

Outcome Clinical Photos and Radiographs

2. Be certain that deformity with growth will not recur. Perform epiphysiodesis if needed.

See Figs. 6 and 7.

Cross-References Avoiding and Managing Problems 1. Maintain ankle range of motion, dorsiflexion, through physical therapy during deformity and length correction.

▶ Proximal Tibial Recurvatum Corrected Using the Ilizarov Technique in a 14 Year Old Boy

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Fig. 6 Clinical photograph (a) and lateral radiograph (b) of the right lower limb showing corrected recurvatum deformity Fig. 7 Anteroposterior radiograph of lower limbs showing lower extremities of equal length

See Also in Vol. 3 Femoral and Tibial Rotational Deformity Treated with Fixator Assisted Nailing and Gradual Correction with The Taylor Spatial Frame

References and Suggested Reading 1. Domzalski M, Mackenzie W. Growth arrest of the proximal tibial physis with recurvatum and valgus deformity of the knee. Knee. 2009;16(5):412–6. 2. Feldman DS, Madan SS, Koval KJ, van Bosse HJ, Bazzi J, Lehman WB. Correction of tibia vara with six-axis deformity analysis and the Taylor Spatial Frame. J Pediatr Orthop. 2003;23(3):387–91. 3. Moroni A, Pezzuto V, Pompili M, Zinghi G. Proximal osteotomy of the tibia for the treatment of genu recurvatum in adults. J Bone Joint Surg Am. 1992;74(4):577–86.

Distal Femoral Deformity Correction Using MAC

45

Patrick J. O’Toole and Richard S. Davidson

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 297 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 298 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 298 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 298 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 298 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 298 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 299 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 299 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 299 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300

Abstract

Distal femoral deformities are challenging and can be treated in a variety of different ways (1). For skeletally immature patients, guided growth plates can be used to correct deformities, especially in the coronal plane (2). When there is a complex deformity present especially when there is a need to lengthen the femur, then an external fixator is usually required. The MAC (multiaxial correction) device allows correction of displacement and angulation in all three planes and also allows the bone to be lengthened. Preoperative planning is essential to define the different parameters of the deformity. This device is placed laterally on the femur and is attached with hydroxyapatite-coated half pins proximal and distal to P. J. O’Toole (*) Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected] R. S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected]; [email protected]

the proposed osteotomy site. Deformity correction is usually started by the patient at 5–7 days post-application. Removal of the external fixator can only occur once a satisfactory consolidation of the regenerate bone has been achieved.

Brief Clinical History This 12 year old male presented with a shortened left femur associated with a valgus deformity of the knee (Fig. 1). Further analysis, by way of long-leg radiographs, revealed that the femur was 1.5 cm short with an approximately 35 valgus deformity. The CORA (center of rotation of angulation) of the valgus deformity was located in the distal femur. Clinical examination revealed a normal range of motion of the hip, knee, and ankle on the affected left side, with a normal peripheral neurovascular examination. The patient walked with a significant limp, which was only partially corrected with a shoe-lift orthotic. The leg length discrepancy was significant, and considering the malalignment of the

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mechanical axis on the left side, the risk of early knee osteoarthritis was also a concern for the patient and his family. After a discussion with the family, informed consent was obtained for application of a multiaxial correction (MAC) device with a concomitant distal femoral osteotomy and iliotibial band release.

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3. Osteotomy of the distal femur to allow deformity correction to occur. 4. Confirmation of completed osteotomy both clinically and radiologically using the image intensifier.

Basic Principles Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List 1. Short left femur 2. Significant valgus deformity of the left distal femur 3. Tight lateral knee soft tissue structures

Treatment Strategy 1. Application of a MAC external fixator device to allow correction of the distal femoral valgus deformity. Lengthening of the short femur can also be achieved at the same time. 2. Open release of the tight distal iliotibial band to allow and maintain correction of the distal femoral fragment.

Fig. 1 Preoperative clinical image of the shortened valgus left leg

1. The goals of treatment are to restore the length of the shortened femur and to improve the biomechanics of the knee by restoring the normal mechanical axis of the lower limb. 2. The MAC device is applied to the femur as a monolateral external fixator but allows for correction of displacement and angulation in all three planes. Its unique design also allows the limb to be lengthened at the time of deformity correction. 3. The distal femur must be osteotomized to allow the required deformity correction. This is usually performed using drill holes and an osteotome.

Images During Treatment See Fig. 2.

Fig. 2 Intraoperative image of the MAC device attached to the left femur revealing the location of the primary hinge over the CORA

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Technical Pearls 1. The primary hinge is placed over the CORA of the distal femoral deformity (Fig. 2). 2. A temporary spacer can be placed over the CORA on the patient’s skin to allow the MAC device to rest in an appropriate position before the initial fixation to the bone is achieved. 3. Care is taken when placing the diaphyseal femoral half pins to avoid “missing” the femur with the most distal or most proximal diaphyseal pins. This can be avoided by using the proximal half-pin clamp and placing it parallel to the bow of the femur at the time of half-pin insertion. 4. The tight lateral soft tissue structures can act to prevent distal femoral deformity correction. We regularly make an open incision to release the iliotibial band. 5. Pin care routinely starts on postoperative day one and lengthening can start at 5–7 days depending on the age of the patient (Fig. 2).

Fig. 3 Radiographs of the left leg (AP (a) and lateral (b)) showing completion of the angular correction

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6. Postoperatively the patient is followed in the office and correction continues until the mechanical axis had reached normal and the valgus angulation is corrected (Fig. 4). 7. Once consolidation of the lengthened section occurs, the external fixator can be removed.

Outcome Clinical Photos and Radiographs See Figs. 3 and 4.

Avoiding and Managing Problems 1. Patient selection is important. The goals of correction should be to restore the normal mechanical axis of the limb and to address any leg length discrepancy issues. 2. Consideration should be paid to releasing the tight lateral structures about the knee, namely, the distal iliotibial band.

300 Fig. 4 Long-leg radiograph showing a neutral mechanical axis post-deformity correction

P. J. O’Toole and R. S. Davidson

3. The follow-up of these patients is critical. Once deformity correction and the desired leg length have been achieved, then an appropriate consolidation time should be allowed prior to removing the external fixator to avoid complication.

Cross-References ▶ Femoral Lengthening with MAC External Fixation System ▶ Proximal Tibial Recurvatum and Ipsilateral Short Femur Treated with a MAC External Fixation System

References and Suggested Reading 1. Birch JG, Samchukov ML. Use of the Ilizarov method to correct lower limb deformities in children and adolescents. J Am Acad Orthop Surg. 2004;12:144–54. 2. McCarthy JJ, Ranade A, Davidson RS. Pediatric deformity correction using a multiaxial correction fixator. Clin Orthop Relat Res. 2008;466(12):3011–7. 3. Stevens PM. Guided growth for angular correction: a preliminary series using a tension band plate. J Pediatr Orthop. 2007;27(3):253–9.

Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral Deficiency and Femoral Retroversion

46

Pablo Wagner, Renee Hunter, and John E. Herzenberg

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 301 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 301 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 301 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 302 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 304 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305

Abstract

This is a case of limb length discrepancy (LLD) in a child with congenital femoral deficiency and femoral retroversion. External and internal fixations are feasible treatment options. We describe the rotational correction and lengthening using a Precice intramedullary nail. The femur was lengthened 5 cm and was internally rotated 25
. Good final outcome was obtained with no complications.

with external fixation. Femoral retroversion was noted along with grade 1 knee instability.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Brief Clinical History

Preoperative Problem List

A 9.5 year old male patient with a limb length discrepancy of 5 cm on the right due to a diagnosis of congenital femoral deficiency. The patient has undergone previous lengthenings

1. LLD ¼ 5 cm 2. Predicted LLD ¼ 8 cm 3. Retroversion right femur

P. Wagner (*) · R. Hunter · J. E. Herzenberg Rubin Institute for Advanced Orthopedics, International Center for Limb Lengthening and Reconstruction, Sinai Hospital of Baltimore, Baltimore, MD, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_312

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302

P. Wagner et al. Fig. 2 Long lateral X-ray view of the right lower extremity

Fig. 1 AP view of the entire leg showing the LLD

Treatment Strategy 1. Femoral lengthening with Precice nail 2. Acute rotational correction using the Precice nail 3. Use of temporary external fixation for rotational correction and stabilization

Daily physical therapy is recommended to keep an appropriate joint range of motion, preventing joint flexion contractures. An intramedullary nail is a feasible option in skeletally immature patients older than 9 years of age. There is no femoral neck deformity noted in the medium-term followup of these patients.

Images During Treatment Basic Principles See Figs. 3, 4, 5, and 6. Lengthening through an intramedullary nail has a decreased infection rate in comparison with external fixation as well as a better postoperative rehabilitation and higher patient satisfaction. Iliotibial band release is always part of the congenital femoral lengthening surgery to avoid knee subluxation and contracture during lengthening. Rotational corrections have to be always performed under external fixator control to achieve the desired correction. Weight bearing as tolerated is only allowed once three out of four cortices are healed.

Technical Pearls In cases needing rotational correction, make sure to document the lower extremity rotational profile preoperatively. Before performing the osteotomy, place an external fixator, using one pin on the lesser trochanter and the second one on the distal femur. Make sure that both pins are parallel to each other. After performing the osteotomy, correct the rotation (depending on the extremity rotational profile) by manipulating the external

46

Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral. . .

303

Fig. 5 Lateral femur X-ray: an over-reaming of the posterior cortex is noted

Fig. 3 Temporary external fixation to help with the rotational correction

Outcome Clinical Photos and Radiographs See Figs. 7 and 8.

Avoiding and Managing Problems

Fig. 4 AP view of the femur during the lengthening stage

fixator pins. This can be quantified by using a goniometer or inclinometer to confirm that the new position of the pins mirror the correction that was planned preoperatively. Some surgeons use Steinmann pins or K wires to measure rotation. However, 6 mm half pins are a more stable alternative.

1. Do not try to correct a rotational malalignment without an external fixator. It is difficult to maintain accuracy without one. Make sure that a rotational profile is obtained preoperatively. 2. Mark the skin where the nail magnet is located. Ask the patient to refresh the marking as needed. If it appears that the bone is not lengthening, you could place a metallic item on this line when taking X-rays to confirm an appropriate line position. 3. In cases of delayed or partial union, a nail dynamization can be performed (as in this case) together with an injection of stem cells. This stimulates bone healing. 4. The starting point should be located just lateral to the tip of the trochanter to avoid any damage to the femoral head vascular supply.

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Fig. 6 AP view of the femur during consolidation phase

Fig. 7 AP view of the femur post-dynamization: healed

5. The size of the nail used in this case is slightly big for this patient. As shown in the lateral X-ray, the posterior cortex was over-reamed during the surgery. This could have been avoided with the newer smaller Precice nail (diameter 8.5 mm), not available at the time of the surgery. 6. A frequent complication during limb lengthening is the loss of knee extension. To prevent this, daily physical therapy and knee bracing are recommended. 7. Remain partial weight bearing until three of the four cortices are healed.

See Also in Vol. 3 Congenital Femoral and Tibial Shortening Internally Lengthened with an ISKD and a Precice Nail Femur Lengthening with Precice Internal Lengthening Nail

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Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral. . .

305

References and Suggested Reading 1. Kirane YM, Fragomen AT, Rozbruch SR. Precision of the PRECICE ® internal bone lengthening nail. Clin Orthop Relat Res. 2014;472(12):3869–78. 2. Rozbruch SR, Birch JG, Dahl MT, Herzenberg JE. Motorized intramedullary nail for management of limb-length discrepancy and deformity. J Am Acad Orthop Surg. 2014;22(7):403–9. https://doi. org/10.5435/JAAOS-22-07-403. 3. Schiedel FM, Vogt B, Tretow HL, Schuhknecht B, Gosheger G, Horter MJ, Rödl R. How precise is the PRECICE compared to the ISKD in intramedullary limb lengthening? Acta Orthop. 2014;85 (3):293–8. 4. Shabtai L, Specht SC, Standard SC, Herzenberg JE. Internal lengthening device for congenital femoral deficiency and Fibular Hemimelia. Clin Orthop Relat Res. 2014;472(12):3860–8. 5. Wang K, Edwards E. Intramedullary skeletal kinetic distractor in the treatment of leg length discrepancy – a review of 16 cases and analysis of complications. J Orthop Trauma. 2012;26(9):e138–44.

Fig. 8 Entire leg, AP view

Femoral Lengthening with MAC External Fixation System

47

Nariman Abol Oyoun and Richard S. Davidson

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 308 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 310 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 310 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 310

Abstract

This case demonstrates the result of lengthening of a congenitally short femur with the MAC external fixation system. A limb length discrepancy (LLD) of 7.2 cm was corrected with a single osteotomy and callotasis over a period of 5 months at the age of 18 years using a multiaxial correcting (MAC) monolateral external fixation system (Biomet, Warsaw, IN).

Disclosure: Dr. Davidson is a consultant for Biomet and receives royalties on the MAC external fixator of which he is a coinventor. N. Abol Oyoun (*) Orthopaedic Surgery, Assiut University, Assiut, Egypt e-mail: [email protected]; [email protected] R. S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected]; [email protected]

Brief Clinical History A 2.5 year old girl presents with congenital left femoral bowing. Whether this was related to a congenital fracture is uncertain. Bowing had remodeled from 45 at birth to 30 at 2.5 years of age. Projected final limb length discrepancy, based on repeated measurements and calculations using the multiplier method, was 8 cm. The child had normal bone age as determined by wrist bone radiographs. At the age of 6 years, the radiographic LLD was measured to be 5.2 cm. At the time of surgery, aged 18, the patient had a total of 7.2 cm difference with the right lower extremity (LE) longer than the left, the shortening being almost exclusively in her left femur (90.2%) (Fig. 1 and Table 1). There was slight valgus deformity (10 ) of the distal femur with a normal looking proximal femur and minimal laxity in the left knee. She has full range of motion of the left hip, knee, and ankle. The LLD was managed temporarily with a shoe lift of 4 and then 6 cm as the difference increased. At the age of 18, the patient finally decided to have

© Springer International Publishing Switzerland (outside the USA) 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_308

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Treatment Strategy The MAC was assembled with a compression distraction device proximally, the multi-axial hinge in the middle and a distraction and compression rail distally. The screws used were hydroxyapatite coated. Lengthening started 10 days following the osteotomy at a rate of 0.25 mm (90 or ¼ of a turn) four times a day. Distraction phase lasted for 5 months due to the premature consolidation of the medial regenerate, which resulted in ineffective distraction, varus angulation, and pin bending. Distraction eventually came to a stop, and osteoclasis was necessary to resume lengthening with gradual correction of the resultant varus deformity. Consolidation phase until tricortication on radiographs lasted for another 3 months.

Basic Principles

Fig. 1 Preoperative scanogram showing shortening of the left femur Table 1 Values of measured lengths of both lower extremities (LEs) preoperatively Extremity Right Left

Femoral length (cm) 50.4 43.9

Tibial length (cm) 41.1 40.4

Total (cm) 91.5 84.3m

The multi-axial correcting (MAC) monolateral external fixation system was designed to treat muliplanar complex deformities without returning to the operating room [2], but is still capable of efficiently and reliably achieving callotasis via distraction as a monolateral fixator. The adult threaded rail of the MAC is capable of 8 cm of lengthening. A full turn (360 ) equals 1 mm. The MAC system was used as a distraction device, knowing that mere distraction would impose some varus stress [3, 5]. This would automatically correct the preoperative 10 -valgus deformity of the femur. The MAC system is otherwise capable of obtaining as much as 80 angular correction if that were necessary. The presence of multi-axial hinges (Fig. 2) allowed alignment of the bone fragments in the sagittal plane, as well as the easy correction of the varus resulting from premature consolidation medially that occurred 2 months postoperatively (Fig. 3).

Images During Treatment

surgery. With 7.2 cm LLD, femoral lengthening using the MAC external fixation system was performed.

See Figs. 2, 3, and 4.

Preoperative Clinical Photos and Radiographs

Technical Pearls

See Fig. 1 and Table 1.

The presence of the multi-axial hinges as part of the MAC external fixation system allows acute and subsequent adjustment of varus/valgus as well as procurvatum/recurvatum elements of any deformity. Hydroxyapatite-coated screws decrease the rate of loosening and infection and improve the stability of the bone-pin interface. They also demonstrate higher extraction torque [4] and by microstructural analysis show more ingrowth of bone [1].

Preoperative Problem List • A total of 7.2 cm LLD, left shorter than right, almost exclusively in the left femur (90.2%) • Slight valgus deformity (10 ) of the distal femur

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Fig. 2 Double hinge (placed at 90 ) in the MAC external fixation system allowing correction of varus/valgus as well as procurvatum/recurvatum elements of any deformity

Fig. 3 Premature consolidation medially with varus and stress over the pins with distraction occurred 2 months after the osteotomy

Outcome Clinical Photos and Radiographs See Figs. 5, 6, and 7.

Avoiding and Managing Problems Hydroxyapatite screws were used to avoid loosening. At the time of their removal, they had a very strong grip in the bone. The osteotomy site developed premature

Fig. 4 Osteoclasis of the premature consolidation by manipulation under general anesthesia with MAC hinges adjusted to correct the varus deformity

consolidation in varus 2 months postoperatively, and was treated by manipulation of femur and external fixator with osteoclasis of the femur under general anesthesia (Fig. 4). The patient was reluctant to do physical therapy (PT) immediately after surgery and during the distraction and consolidation phases, which resulted in some loss of flexion of the knee, but full extension, both passive and active, was maintained (Fig. 7). Ninety-five degrees of knee flexion is generally maintained 6 months after removal of the fixator.

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Fig. 5 Equal limb length achieved as seen on an orthoroentgenogram

Fig. 7 Lower extremity after removal of the MAC external fixation system showing clinical correction and full knee extension

Cross-References ▶ Proximal Tibial Recurvatum and Ipsilateral Short Femur Treated with a MAC External Fixation System

See Also in Vol. 3 Radial Clubhand

References and Suggested Reading 1. Cimerman M, et al. Microstructural analysis of implant-bone interface of hydroxyapatite-coated and uncoated Schanz screws. J Mater Sci Mater Med. 2005;16(7):627–34. 2. Davidson RS. The MAC (Multi-Axial Correcting) monolateral external fixation system (Biomet/EBI) technique: an easier way to correct deformity. Oper Tech Orthop. 2011;21(2):113–24. 3. Guidera KJ, et al. Extremity lengthening: results and complications with the Orthofix system. J Pediatr Orthop. 1991;11(1):90–4. 4. Moroni A, et al. A comparison of hydroxyapatite-coated, titaniumcoated, and uncoated tapered external-fixation pins. An in vivo study in sheep. J Bone Joint Surg Am. 1998;80(4):547–54. 5. Schlenzka D, et al. Metaphyseal distraction for lower limb lengthening and correction of axial deformities. J Pediatr Orthop. 1990;10 (2):202–5.

Fig. 6 Tricortication of the regenerate marking consolidation is needed before frame removal

Femoral Shortening (14 cm) and Deformity Treated with Acute Correction and Two Consecutive Retrograde Fitbone Applications

48

Metin Kucukkaya

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 311 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 311 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 313 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 314 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 314 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 314 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 315

Abstract

The new-generation lengthening nails have clear advantages for correcting deformities and lower limb lengthening. The amount, speed, and rate of distraction can be controlled completely and can be modified according to bone healing and joint contracture. Other advantages of these nails are little scar tissue formation and easy rehabilitation and pain management, which increase patient compliance. These features allow us to achieve extreme bone lengthening without any serious complications. This section presents a case of a 14 year old male with 14-cm femoral shortening resulting from physeal arrest who was treated with two consecutive motorized lengthening nails.

Brief Clinical History A 14 year old male was admitted with femoral shortening and deformity. He had fallen from a height when he was 7 years old, and a fracture around the distal femoral physis was treated with closed reduction and percutaneous pinning. As he grew, a leg-length difference (LLD) developed because of the physeal arrest. He could walk on his toes without using a shoe lift and he walked with his leg in external rotation. However, he complained of limping and back pain after walking. There was no soft tissue contracture at the hip.

Preoperative Clinical Photos and Radiographs See Fig. 1.

M. Kucukkaya (*) Florence Nightingale Hospital, Istanbul, Turkey e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_29

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M. Kucukkaya

Fig. 1 (a, b) Clinical photos and preoperative long-standing radiograph (LSR) with the leg-length difference compensated for using plates and the patellae oriented anteriorly. (c) Preoperative anteroposterior and lateral views of the right femur. (d) External and internal rotation of the hip. Note the limited internal rotation as compared with the external rotation of the hip

Preoperative Problem List

Basic Principles

• Femoral shortening of 14 cm. • Varus of 6 and a 30 external rotational deformity.

The LLD and angular and rotational deformities were determined from the physical examination and plain radiographs. The malalignment test [4] and the reverse planning method [1] were used for preoperative planning. The required deformity correction, entry site, canal diameter, osteotomy site, and positions of the blocking screws were determined on the LSR. When using an intramedullary nail, the femoral lengthening occurs along the anatomical axis. It results in coronal plane translation of the knee relative to the mechanical axis [1].

Treatment Strategy Acute angular and rotational correction and simultaneous lengthening using two consecutive motorized lengthening nails (Fitbone-TAA 24.5 cm, maximum stroke 80 mm, Wittenstein, Igersheim, Germany) [2].

48

Femoral Shortening (14 cm) and Deformity Treated with Acute Correction and Two Consecutive Retrograde. . .

To prevent this malalignment, the required acute lateral translation of the distal fragment was planned.

Images During Treatment See Fig. 2.

Fig. 2 (a) Intraoperative X-rays show the standard entry point of the K-wire, the medullary canal reaming using rigid reamers, and a temporary distal Schanz pin. (b) Reaming the dense posterior cortical wall using rigid reamers. Note that the surgeon pushes down on the femur with one hand. (c) Before the osteotomy, two parallel 6-mm Schanz pins were inserted into the proximal and distal segments. After the osteotomy, a 30 acute rotational correction was completed. (d) Position

313

Technical Pearls • The operation was performed with the patient in the supine position on a radiolucent operating table with the knee in a semi-flexed position. • The standard entry point for retrograde femoral nailing was at the medial part of the intercondylar notch in the

of the blocking screw and intraoperative alignment control using a grid plate. (e) The X-rays in the early postoperative period and 6 months after 8 cm of distraction. (f) The first lengthening nail was replaced with a new one after the first 8-cm distraction period, and the femur was distracted a further 6 cm. A temporary external fixator was used intraoperatively to protect the distraction site

314

anteroposterior view and at the apex of Blumensaat’s line, which was approximately 1 cm anterior to the posterior cruciate ligament in the lateral view (Fig. 2a) [3]. • Insertion of two rotational Schanz pins. The distal Schanz pin was inserted first and placed posterior to the reamer and nail passage. The second Schanz pin was placed at the level of the lesser trochanter (Fig. 2d). • While reaming the dense posterior cortical wall using rigid reamers, it helps to push down on the femur with one hand (Fig. 2b). • If rotational correction is necessary, an osteotomy must be performed before reaming the proximal segment.

Outcome Clinical Photos and Radiographs See Fig. 3.

Avoiding and Managing Problems • To prevent malrotation after the osteotomy, two parallel 6-mm Schanz pins were inserted into the proximal and distal segments before the osteotomy. • Motor stop and cable breakage are usually caused by technical mistakes. The nail can be damaged during insertion if it is hammered, or the cable unit can break when it is introduced through the tunnel and connected to the subcutaneous antenna. The prepared medullary canal must permit smooth, easy insertion of the nail dummy.

M. Kucukkaya

• Excessive reaming and high-speed reaming of the medullary canal using a rigid reamer can cause necrosis of the bone and result in poor bone regeneration at the distraction site. The medullary canal should be over-reamed 0.5 mm larger than the nail (the largest part of the nail is 12 mm). The reaming should be performed gently to avoid compromising the bone circulation. • After the distal metaphyseal osteotomy, the short distal femur fragment is influenced by deforming muscle forces, and the distal metaphyseal bone does not allow the intramedullary nail to have cortical contact. With the lengthening nail, the alignment tends to be displaced or angulated after distraction (Fig. 2d, e). If necessary, distal and proximal femur blocking screws should be used in both the distal and proximal segments to prevent malalignment and loss of correction [3]. • Insufficient reaming of the posterior wall of the medullary canal inclines the tip of the sharp rigid reamer anteriorly during reaming. This might cause perforation or weakening of the anterior cortical femur. Meticulous reaming of the dense posterior cortical wall using rigid reamers is important for preventing a subtrochanteric fracture around the proximal locking screw.

See Also in Vol. 3 Combined Deformities of the Femur and Tibia with 9 cm Shortening Treated with a Retrograde Femoral Motorized Lengthening Nail and a Tibial Plate

Fig. 3 (a) LSR after 14 cm of lengthening and deformity correction. (b, c) Clinical pictures after treatment

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Femoral Shortening (14 cm) and Deformity Treated with Acute Correction and Two Consecutive Retrograde. . .

Ipsilateral Secondary Hip Osteoarthritis and Leg Length Discrepancy: Treated Simultaneously with Total Hip Replacement and Motorized Lengthening

References and Suggested Reading 1. Baumgart R. The reverse planning method for lengthening of the lower limb using a straight intramedullary nail with or without deformity correction. Oper Orthop Traumatol. 2009;21:221–33. 2. Baumgart R, Hinterwimmer S, Krammer M, et al. A fully implantable, programmable distraction nail (Fitbone) – new perspectives for

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corrective and reconstructive limb survey. In: Leung KS, Taglang G, Schnettler R, editors. Practice of intramedullary locked nails. New developments in techniques and applications. Heidelberg: Springer; 2006. p. 189–90. 3. Kucukkaya M, Karakoyun O, Kuzgun U. Lengthening over a retrograde nail using 3 Schanz pins. J Orthop Trauma. 2013;27:13–7. 4. Paley D. Radiographic assessment of lower limb deformities, frontal plane mechanical and anatomical axis planning. In: Paley D, editor. Principles of deformity correction. Berlin/Heidelberg: Springer; 2002. p. 31–98.

Femur Lengthening with an Extramedullary Nail

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Claire E. Shannon

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 317 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 317 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 317 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 318 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 318 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 319 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 319 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 319 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 320 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 326

Abstract

This case illustrates a 5 cm femoral extramedullary internal limb lengthening (EMILL) in a young child with a 5.3 cm limb length discrepancy (LLD) due to congenital limb deficiency.

Brief Clinical History The patient is a 4-year-old boy with left Fibular Hemimelia (FH) Paley type 3c and Congenital Femoral Deficiency (CFD) Paley type 1a [1, 2]. He underwent a SUPERankle [1] and 5 cm tibial lengthening using external fixation 2 years prior. The current LLD is 5.3 cm total and predicts to be 21.5 cm at skeletal maturity using the Multiplier Method [3]. The left femur is 3.8 cm short and predicts to be 7.8 cm short at the end of growth. The hip and knee are stable, making him a good candidate for internal lengthening. The femur is too

small in diameter to accommodate an intramedullary (IM) lengthening nail (Precice, Nuvasive Specialized Orthopedics, San Diego, CA), so EMILL using the Precice nail was chosen [4, 5]. Additionally, the patient has valgus of the distal femur which will be addressed with a medial distal femur hemiepiphysiodesis.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List Left CFD Left LLD (3.8 cm femur and 1.5 cm below the knee) Left genu valgum

C. E. Shannon (*) Paley Orthopedic and Spine Institute, West Palm Beach, FL, USA e-mail: [email protected] © Her Majesty the Queen in Right of Australia, as represented by Queensland Health 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_377

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C. E. Shannon

Treatment Strategy 1. Insertion of a small antegrade intramedullary rod (SLIM, Orthopediatrics, Warsaw IN) 2. Osteotomy of femur at apex of deformity if deformity present. 3. Femur lengthening using an extramedullary lengthening rod 4. Release of Iliotibial Band (ITB) and proximal Fascia Lata 5. Medial distal femur hemiepiphysiodesis

Basic Principles

Fig. 1 Preoperative erect leg (EL) (left) and long lateral (right) x-ray demonstrates LLD of 5.3 cm with shortening of both femur and tibia. The femur length is adequate (165 mm), but the diameter (13 mm) is too small for an IM lengthening nail. There is valgus of the distal femur

Fig. 2 AP pelvis radiograph showing a stable left hip with adequate coverage

1. The femur must be a minimum length of 150 mm between the femoral neck and the distal femoral physis for the shortest available nail to obtain stable fixation. 2. A SLIM rod is inserted into the femur using an antegrade trochanteric start point to provide stability during lengthening. This is done prior to making the osteotomy. If a distal osteotomy is required or the proximal femur is not accessible, a retrograde SLIM rod can be considered. 3. The osteotomy level is chosen to correct deformity if needed, ideally in an area of unviolated periosteum. The osteotomy should be made at a level such that the SLIM rod will bridge the regenerate through the entire lengthening. The SLIM rod is backed out of the bone to complete the osteotomy and then reinserted. Any rotation of the femur can be done at this time. Care should be taken to avoid unwanted varus or valgus. 4. The proximal incision from the SLIM rod is extended 1–2 cm distal and a cobb or long Precice trocar is used to make a dilated submuscular path along the lateral femur. Antegrade insertion of the nail leaves the larger diameter end of the nail proximal, where there is better soft tissue coverage. 5. The ITB should be released distally to prevent valgus deformity and pain in cases of congenital limb shortening where the ITB has not been excised in a prior surgery. The distal incision is made laterally at the level of the superior pole of the patella, which coincides with the distal end of the nail. The ITB is split transversely, including the intermuscular septum, and the fascia of the biceps femoris is recessed [6]. Proximal release of the fascia lata should also be considered. The anterior and posterior edges of the fascia lata can be felt at the level of the greater trochanter with mayo scissors through the existing incision. The anterior and posterior fascia is split transversely [6]. 6. The distal end of the nail should be docked into the lateral femoral condyle to avoid valgus deformity while lengthening, improve the stability of the lengthening construct, and to keep a lower profile of the nail. This is done by inserting a guidewire into the condyle where the nail

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Femur Lengthening with an Extramedullary Nail

contacts the bone and passing an ACL reamer 1 mm larger than the nail to make a hole. The distal end of the nail is then nested into the condyle [4]. 7. The nail is fixed with fully threaded Precice screws to minimize the risk of pull out. These should be positioned posterior to the SLIM rod. To minimize the profile of the screws, the proximal threads can be burred off to allow the head of the screw to sit flush against the nail. 8. Any residual varus or valgus deformity can be addressed with insertion of hemiepiphysiodesis plates as needed.

Images During Treatment See Figs. 3, 4, 5, 6, and 7.

Technical Pearls 1. Rotation of the femur should be assessed preoperatively. Rotation can be corrected through the osteotomy when the SLIM rod is inserted. A goniometer or inclinometer can be used to place half-pins with the amount of desired correction. When the SLIM is inserted, the pins are rotated until parallel to correct the rotation.

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2. Femoral bowing can be corrected with the SLIM rod. The osteotomy should be held in extension while the SLIM rod is inserted to maintain the correction. 3. An 8.5 mm diameter straight nail is most commonly used in pediatric cases. When possible, a longer length nail with two screws at each end is preferred for stronger fixation. 4. A custom molded HKAFO brace is worn 18–22 h a day to protect the leg, and the knee should be locked in full extension at night time. 5. Due to the limited fixation, the patient is kept touch-down weight-bearing until adequate consolidation is achieved. Lengthening proceeds at a rate of 0.75 mm/day in 0.25 mm increments. Physical therapy is performed daily to avoid hip or knee contractures or dislocations. 6. Removal of the lengthening nail is only performed once the regenerate bone is fully consolidated, typically 6–12 months after insertion. The SLIM rod is left in the femur to protect the bone from fracture.

Outcome Clinical Photos and Radiographs See Figs. 8, 9, and 10.

Fig. 3 After preparing the path for the SLIM rod, multiple drill holes are made at the chosen osteotomy level. This is completed with an osteotome (left). The SLIM rod is then fully inserted into the femur (middle and right)

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C. E. Shannon

Fig. 4 AP (left) and lateral (right) guidewire placement in the lateral distal femoral condyle. An ACL reamer is passed over this to make a docking site for the end of the Precice nail

Avoiding and Managing Problems 1. The hip and knee must be stable prior to internal lengthening. Preparatory surgery should be considered prior to lengthening if intability or deformity is present: SUPERhip with or without a pelvic osteotomy for a dysplastic hip, SUPERknee for absent cruciate ligaments or rotational instability [2]. 2. Nesting of the nail into the lateral condyle will prevent distal screw pull-out and increase the stability of the construct.

3. Cannulated technique for screw insertion allows precise placement of the fixation. Avoid placing screws anterior to the SLIM rod when possible to minimize the risk of femur fracture with forced knee bending. 4. The SLIM rod should be the maximum length of the femur, stopping at the distal femoral physis. A larger rod (3.2–4 mm) is preferred, provided the nail fixation screws can be properly positioned. 5. X-rays of the femur, including the hip and the knee, as well as a thorough physical exam, should be performed every 2 weeks to assess lengthening progress, hip and knee ROM, and joint stability.

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Femur Lengthening with an Extramedullary Nail

Fig. 5 The proximal threads of the fully threaded screws are burred down so that the head of the screw can sit flush to the nail (left). The lateral view shows both screws are posterior to the SLIM rod (right)

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Fig. 6 Immediate post-operative AP (left) and lateral (right) radiographs showing the placement of the extramedullary lengthening nail. A hemiepiphysiodesis plate was applied to the medial distal femur for genu valgum

C. E. Shannon

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Femur Lengthening with an Extramedullary Nail

Fig. 7 AP (left) and lateral (middle) of the patient’s femur after 6 weeks of lengthening at 0.75 mm/day. He has achieved 3 cm of distraction and the alignment of the leg is maintained. EL x-ray after completing 5 cm of femoral lengthening with good early regenerate formation (right)

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Fig. 8 EL (left) and long lateral (right) radiographs showing full consolidation 6 weeks after the end of lengthening

C. E. Shannon

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Femur Lengthening with an Extramedullary Nail

Fig. 9 AP (left) and lateral (right) postoperative x-rays after removal of the EM nail and hibernation of the hemiepiphysiodesis plate 8 weeks after completion of lengthening. The SLIM rod is left in place to protect the femur

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References and Suggested Reading 1. Fuller CB, Shannon CE, Paley D. Lengthening reconstruction surgery for fibular Hemimelia: a review. Children. 2021;8:467. https://doi. org/10.3390/children8060467. 2. Paley D, Shannon C. Treatment of congenital femoral deficiency. In: Wiesel SW, editor. Operative techniques in orthopaedic surgery. Philadelphia: Lippincott Williams & Wilkins; 2021. Chapter 37 In Press. 3. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. JBJS. 2000;82:1432. 4. Shannon C, Paley D. Extramedullary internal limb lengthening. Tech Orthop. 2020;35:195–200. 5. Dahl MT, Morrison SG, Laine JC, Novotny SA, Georgiadis AG. Extramedullary motorized lengthening of the femur in young children. J Pediatr Orthop. 2020;40:e978–83. https://doi.org/10.1097/ BPO.0000000000001593. 6. Paley D, Harris M, Debiparshad K, Prince D. Limb lengthening by implantable limb lengthening devices. Tech Orthop. 2014;29:72–85. https://doi.org/10.1097/BTO.0000000000000072.

Fig. 10 EL x-ray taken 1 year after completion of lengthening

Fibular Hemimelia and Congenital Short Femur-Staged Approach in Child

50

Emilie-Ann Downey and S. Robert Rozbruch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 327 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 327 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 327 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 328 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 329 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 329 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 330 Outcome Clinical Photos and Radiographs (4–5) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 330 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 330 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 334

Abstract

Although fibular hemimelia (FH) is a rare congenital malformation, it is the most common congenital long bone deficiency. It varies in severity and includes fibular shortening and a possible constellation of findings, mainly femur and tibia shortening, knee valgus deformity and flexion contracture, and anteroposterior instability of the knee and ankle, as well as tarsal coalition and deficiency of the lateral rays of the foot. Foot function and extent of shortening of the limb at birth play a role in determining the best treatment option, mainly limb salvage or amputation.

Brief Clinical History This is a 19-month-old infant born with fibular hemimelia Paley type IIIC. The foot is in fixed equinovalgus (from the ankle joint and subtalar joint), and there is a leg length discrepancy.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List

E.-A. Downey (*) Department of Orthopaedic Surgery, Pierre-Boucher Hospital, QC, Canada S. R. Rozbruch Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA

1. 2. 3. 4.

Limb length discrepancy Diaphyseal tibia deformity Short fibula Fixed equinovalgus foot deformity from the ankle and possibly the subtalar joint 5. Ankle joint instability 6. Lateral foot ray absence

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_400

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Fig. 1 (a-c) Clinical front (a), side, (b) and posterior (c) photographs. Note right equinovalgus ankle, limb length discrepancy, diaphyseal deformity, and absence of lateral foot ray

Fig. 2 (a-c) AP and lateral tibial X-rays (a-b), and hip to ankle standing alignment X-ray (c) demonstrating leg length discrepancy (65 mm), diaphyseal tibial deformity, shortened fibula, equinovalgus foot and ankle, and absence of one lateral foot ray

Treatment Strategy Reconstructive surgery involves foot deformity correction and limb length equalization. It is important to correct the fixed foot and ankle deformity early to allow the patient to walk with a plantigrade foot and to be able to wear shoes properly. It is important to correct this deformity either before or at the time of

tibial lengthening. The SuperAnkle procedure (a combination of supramalleolar osteotomy with extra-articular lengthening of the Achilles and peroneal tendons with resection of the fibrocartilaginous fibular anlage) should be performed early. To correct the leg length discrepancy, it is generally advised to split the lengthening surgeries into manageable amounts per time with a few-year period separating each lengthening session, to minimize the complications.

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Fibular Hemimelia and Congenital Short Femur-Staged Approach in Child

Basic Principles

329

4. Perform serial lengthening procedures (5–8 cm each session), a few years apart. 5. Use growth plate modulation as needed to realign the knee.

1. Classify the type of FH deformity using the Paley classification. 2. Using the Paley multiplier method [1], determine the predicted limb length discrepancy at skeletal maturity. Then based on the total length required, determine how many lengthenings (and/or epiphysiodeses) will be required to achieve the goal. 3. If it is a fixed deformity (Paley Classification Type 3), the SuperAnkle procedure is used to achieve correction and prevent recurrence of deformity.

Images During Treatment

Fig. 3 (a-c). Intraoperative views performed at 2 years and 5 months old demonstrating SuperAnkle procedure, including supramalleolar osteotomy. The ankle joint can be further defined intraoperatively

using an arthrogram (a). Note distal tibia valgus and short fibula. The supramalleolar osteotomy is stabilized with 2 k-wires, crossing the subtalar and ankle joints

See Figs. 3, 4, 5, 6, 7, 8, and 9.

Fig. 4 (a-d) Clinical photographs (a, b) and radiographs (c, d) demonstrating first tibial distraction procedure achieving a total of 60 mm. The healed supramalleolar osteotomy is also seen

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E.-A. Downey and S. R. Rozbruch

Fig. 5 (a-d) Clinical photographs of a 6-year-old patient (a, b) and radiographs (c, d) demonstrating femur lengthening surgery using a circular external fixator spanning the knee joint to avoid dislocation. Growth modulation was also performed to correct genu valgum

Technical Pearls 1. The SuperAnkle procedure was performed in the initial surgery to achieve a plantigrade foot, and combined with a tibial lengthening procedure with external fixation. 2. The foot should be corrected to make the plantar aspect of the foot perpendicular to the distal tibia. 3. Break up the lengthening procedures into multiple manageable sessions (example 5 cm per lengthening). 4. The diaphyseal deformity can be corrected gradually with external fixator and/or growth modulation. 5. In younger children, less than 8 years old, expect rebound of the valgus deformity. 6. Spanning the knee joint with circular external fixator femur-lengthening procedures may prevent knee dislocation, especially in presence of clinical anteroposterior knee instability.

7. Foot and ankle coalitions may only become apparent at an older age, requiring future foot and ankle surgical correction to maintain a plantigrade foot.

Outcome Clinical Photos and Radiographs (4–5) See Fig. 10.

Avoiding and Managing Problems 1. Avoid knee flexion contractures 2. Avoid excessive lengthening amount per lengthening to minimize the risk of complications and limit the time in frame per session.

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Fibular Hemimelia and Congenital Short Femur-Staged Approach in Child

331

Fig. 6 Hip to ankle standing alignment X-rays (a-c) demonstrate recurrence of valgus deformity of the femur as well as leg length discrepancy at age 7 years old (a). Further growth modulation was performed (b) with correction of the mechanical axis of the right leg 10 months later (c)

332 Fig. 7 Radiographs (a, b) at age 10 years demonstrate a further 5 cm femur lengthening with antegrade femur lengthening nail

E.-A. Downey and S. R. Rozbruch

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Fibular Hemimelia and Congenital Short Femur-Staged Approach in Child

Fig. 8 (a-d). Radiographs, AP ankle (a), lateral ankle (b), and Saltzman view (c), at age 12 demonstrate fixed equinus deformity of the ankle, distal tibia and hindfoot valgus, short fibula, and lateral ray absence. A

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coronal CT scan slice of the ankle (d) demonstrates a talocalcaneal coalition contributing to fixed ankle and hindfoot equinovalgus deformity

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Fig. 9 (a-c) Radiographs demonstrating medial distal tibia hemiepiphysiodesis and medial slide calcaneal osteotomy for correction of distal tibia and hindfoot valgus alignment, as well as gastrosoleus recession for correction of equinus

E.-A. Downey and S. R. Rozbruch

Fig. 10 (a) Most recent hip to ankle standing alignment X-ray at age 13. Current indirect leg length discrepancy of 41 mm. The patient has undergone 16 cm total right lower extremity lengthening to date (10 cm in the femur, and 6 cm in the tibia). The predicted leg length discrepancy at skeletal maturity is 48 mm. She will undergo one final right tibial lengthening at skeletal maturity

References and Suggested Reading 1. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. J Bone Joint Surg Am. 2000;82: l432–1446.

Fibular Hemimelia: Paley Type 3

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Dror Paley and Craig A. Robbins

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 336 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 337 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 337 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 338 Case 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 338 Case 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 338 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 338 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 338 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345

Abstract

Fibular hemimelia (FH) is the most common lower extremity congenital longitudinal deficiency. It is associated with a constellation of deformities including foot ray deficiencies, coalitions, ankle and hindfoot joint malorientation, diaphyseal deformity, and limb length discrepancy. Severe rigid and intractable equinovalgus deformity of the foot in combination with a limb length discrepancy has been the limiting factor for a successful outcome and the primary indication for ablative surgery. The Paley fibular hemimelia classification (Fig. 1) is based on the ankle joint morphology and stability and is helpful in planning surgical treatment. Reconstruction surgery for fibular hemimelia involves foot deformity D. Paley (*) · C. A. Robbins (*) Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA e-mail: [email protected]; [email protected]; [email protected]

correction and limb length equalization. The SuperAnkle is a combination of bone and soft tissue procedures that stabilizes the foot and addresses all deformities.

Brief Clinical History Case 1: This is an 18 month old child born with fibular hemimelia Paley type IIIa. The foot is in fixed equinovalgus and there is a limb length discrepancy. Case 2: This is an 18 month old child with fibular hemimelia Paley type IIIb. The foot is in fixed equinovalgus and there is a limb length discrepancy.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, 4, 5, and 6.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_33

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Paley Classification of FH Type I - Stable

Type II - Dynamic Valgus

ADTA = 80 - 85°

LDTA = 85 - 90°

Type III - Fixed Equino-valgus a) ankle type

Type III - Fixed Equino-valgus b) subtalar type

ADTA ≥ 90°

Fig. 1 Paley classification of fibular hemimelia. Type I has a stable ankle; type II has dynamic ankle valgus; and type III has fixed equinovalgus with subtypes based on the location of deformity: (a)

ADTA ≤ 90°

Type III - Fixed Equino-valgus c) combined ankle and subtalar type ADTA ≥ 90°

ankle, (b) subtalar, and (c) combined (Copyright 2003, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

Fig. 2 Paley type IIIa fixed equinovalgus type fibular hemimelia; the equinovalgus deformity is related to the malorientation of the distal tibia (Copyright 2003, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

Preoperative Problem List • • • • •

Fixed equinovalgus foot deformity. Limb length discrepancy. Diaphyseal tibia deformity. Case 1 has ankle joint malorientation. Case 2 has subtalar joint malorientation.

Fig. 3 Case 1: clinical posterior (left) and side (right) photograph; note equinovalgus ankle, limb length discrepancy, and diaphyseal deformity (Copyright 2003, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

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Treatment Strategy The SuperAnkle procedure was developed by Paley in 1996. It combines a supramalleolar and/or subtalar osteotomy with extra-articular soft tissue lengthenings of the Achilles and peroneal tendons with resection of the fibrocartilaginous fibular anlage. It is the only procedure that prevents recurrence of foot deformity.

Basic Principles

Fig. 4 Case 1: standing AP of both lower extremities (left) and lateral (right) X-rays of the right leg; note complete absence of the fibula, equinovalgus ankle, limb length discrepancy, diaphyseal deformity, hypoplasia of distal femur and proximal tibia epiphyses Fig. 5 Case 2: lateral X-ray (left) and coronal MRI (right) of the ankle; note overlap of calcaneus and talus on lateral view; note cartilaginous coalition between the talus and calcaneus on MRI

• Classify the FH using the Paley classification. • If there is fixed equinovalgus (type III), it will require a SuperAnkle procedure. • The SuperAnkle procedure can be combined with lengthening. • The SuperAnkle combines correction of ankle and/or subtalar bony deformities with extra-articular soft tissue releases. • Use the Paley multiplier method to predict limb length discrepancy at skeletal maturity, and based on this, determine the number of lengthenings and epiphysiodesis timing needed to equalize limb length discrepancy. • Perform serial lengthenings about 4 years apart with a goal of 5–8 cm each time. • To succeed, ensure the patient undergoes daily aggressive physical therapy. • Clinical follow-up including radiographs is needed every 2 weeks during the distraction phase. • Use hemi-epiphysiodesis as needed to realign the knee.

338

Fig. 6 Paley type IIIb fixed equinovalgus type fibular hemimelia; the equinovalgus deformity is related to the subtalar coalition

Images During Treatment Case 1 See Figs. 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, and 17.

Case 2 See Figs. 18, 19, and 20.

Technical Pearls • A long lateral incision is made parallel to the posterior tibial cortex in distal part of leg and extended to the calcaneus. • Identify and dissect free the superficial peroneal nerve; protect the sural nerve distally. • Suture peroneal tendons together proximally and distally and cut the longus proximally and brevis distally to allow lengthening; Z-lengthen a single tendon. • A second proximal incision is made parallel to oblique course of the peroneal nerve which is identified and decompressed. • Dissect and resect the fibular anlage as a single unit from distal incision and resect interosseous membrane distally. • Decompress posterior tibial neurovascular structures into the tarsal tunnel, and then Z-lengthen Achilles tendon approximately 4 cm in an infant.

D. Paley and C. A. Robbins

Fig. 7 Fibular anlage resection with peroneal nerve decompression; the peroneal tendon(s) is identified, and if both are present, they are sutured together proximally and distally and the longus cut proximally and the brevis distally. If only one peroneal tendon is present, a Z-lengthening of this tendon is performed

• For type IIIa to prevent recurrent deformity, the ankle and subtalar joints are pinned in the position of equinovalgus; then the supramalleolar osteotomy (SMO) is performed obliquely from posterolateral to anteromedial converging on the anteromedial distal tibia physis; the K-wires stabilize the hindfoot, ankle joint, and osteotomy. • The foot should only be corrected to make the plantar aspect perpendicular to the distal tibia diaphysis. • For type IIIb, the osteotomy is made through the coalition of the posterior facet of the calcaneus obliquely from superolateral to inferomedial; leverage the calcaneus distally (to correct equinus and increase foot height) and medially translate; the osteotomy ends in the sinus tarsi. • In some cases, both osteotomies are needed. • The purpose of the osteotomies is to medialize the ground reactive forces to prevent valgus recurrence. • The diaphyseal deformity can be corrected acutely with bone resection or gradually with external fixator. • In children under 8, varusize the distal tibia to compensate for the expected valgus drift with growth.

Outcome Clinical Photos and Radiographs See Figs. 21 and 22 (case 1).

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Fig. 8 The ankle and subtalar joints are pinned in their equinovalgus position just short of the proposed supramalleolar osteotomy level

Avoiding and Managing Problems Preoperative MRI is helpful to define ankle and hindfoot morphology. Use a tourniquet for the soft tissue portion of the procedure. Dissection and decompression of neurovascular structures is mandatory. In type IIIa, stabilize the hindfoot in equinovalgus with 1.5 mm wires prior to the SMO, and then correct the deformity until the foot is plantigrade. In type IIIb, the calcaneus is medialized and rotated to correct equinus and medialize the ground reactive force vector to prevent valgus recurrence. The final result in treatment is dependent on the foot position after reconstruction and lengthening. It is essential to obtain a plantigrade stable foot. Some cases will require ankle fusion to maintain this result.

Fig. 9 The Achilles and peroneal tendons are lengthened and a supramalleolar opening wedge osteotomy is performed to reorient the ankle joint. Allograft can be inserted into the opening wedge

340 Fig. 10 Intraoperative lateral (left) and AP (right) X-rays showing opening wedge supramalleolar osteotomy stabilized with K-wires

Fig. 11 The Achilles and peroneal tendons are repaired; the resected anlage can be used to augment the Achilles tendon

D. Paley and C. A. Robbins

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Fibular Hemimelia: Paley Type 3

Fig. 12 The diaphyseal tibial deformity is corrected combined with lengthening through a separate osteotomy at the apex of diaphyesal deformity. This is done gradually. Up to 5 cm lengthening is done between ages 18 months and 4 years

Fig. 13 AP (left) and lateral (right) radiograph during distraction (age 18 months)

341

342

Fig. 14 AP (left) and lateral (right) radiograph after lengthening of 5 cm. The foot is plantigrade. The tibia is overcorrected into varus to prevent valgus rebound Fig. 15 Standing AP both lowers (right) and lateral (left) during second lengthening (8 cm) at age 8 with hemi-epiphysiodesis of the valgus knee

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Fibular Hemimelia: Paley Type 3

343 Fig. 17 Standing radiograph during third and final lengthening; femur (4 cm) and tibia (6 cm)

Fig. 16 Standing radiograph after consolidation of second lengthening (age 9)

Fig. 18 The soft tissue dissection proceeds as for the type IIIa. Tendon releases are combined with subtalar osteotomy. The osteotomy is angled at 45 to combine medialization with increase in heel height

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Fig. 19 The foot is fixed with K-wires and the tendons repaired; the Achilles tendon can be augmented with the resected anlage

Fig. 21 Standing AP radiograph after completion of third lengthening (age 12). The right side was overlengthened by 1 cm in expectation of future discrepancy; note the rush rodding of the femur that was performed at the time of femoral fixator removal

Fig. 20 The diaphyseal deformity is gradually corrected with lengthening through an osteotomy at the apex of deformity

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Fig. 22 Clinical photographs (age 16). Leg lengths are equal. The foot is plantigrade. There has never been any recurrence of deformity after the SuperAnkle procedure

Cross-References

References and Suggested Reading

▶ Delayed Regenerate Bone Formation in a Seven Year Old Boy with Fibular Hemimelia Undergoing Tibial Lengthening ▶ Guided Growth and Syme Amputation in a Thirteen-YearOld Boy with Type 2 Congenital Fibular Deficiency

1. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. J Bone Joint Surg Am. 2000;82:1432–46. 2. Sabharwal S, Paley D, Bhave A, Herzenberg JE. Growth patterns after lengthening of congenitally short lower limbs in young children. J Pediatr Orthop. 2000;20:137–45.

Guided Growth and Syme Amputation in a Thirteen-Year-Old Boy with Type 2 Congenital Fibular Deficiency

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Elizabeth Ashby and Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 347 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 347 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 350 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 350

Abstract

This is a case of limb length inequality and severe foot deformity in a boy with congenital fibular deficiency. A Syme amputation was performed at age 11 months. He subsequently developed a progressive valgus deformity at the knee that was successfully treated with guided growth at the age of 12 years.

Brief Clinical History A boy with congenital deficiency of the fibula was born with leg length inequality (LLI) of 25% and a severely deformed three-ray foot. Foot reconstruction was not deemed possible E. Ashby (*) Division of Orthopaedics, Shriners Hospital, Montreal, QC, Canada e-mail: [email protected]

so a Syme amputation was performed. The boy made an excellent recovery and began to ambulate with a prosthesis. A hypoplastic lateral femoral condyle, together with apex medial tibial bowing, contributed to a progressive genu valgum. The Hueter-Volkmann principle states that compression of the physis leads to growth inhibition, partially explaining deformity progression in this case. By the age of 12 years, the LDFA (lateral distal femoral angle) was 74 and it was decided to proceed with guided growth using an eightplate (Orthofix). Within 11 months the deformity had corrected and there was a normal mechanical axis of the leg. The 8-plate was removed. This 13 year old boy now leads a full life and is an avid soccer and hockey player.

Preoperative Clinical Photos and Radiographs See Fig. 1.

R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_337

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Fig. 1 Anteroposterior X-ray of both legs and lateral view of the left tibia before the Syme amputation. Anterior tibial bowing is demonstrated with complete absence of the fibula. A three-ray foot is seen

Preoperative Problem List

Basic Principles

1. Severe limb length inequality 2. Severe foot and ankle deformities including the absence of two rays 3. Anterior bowing of the tibia with absent fibula 4. Cruciate ligament-deficient knee

The basic principles of this case are to achieve normal weight bearing, normal gait, and equal leg length. There are two possible treatment strategies: multiple limb lengthenings together with foot and ankle reconstruction with possible epiphysiodesis of the opposite leg or amputation (Syme or Boyd) and prosthetic fitting [6, 8]. For many years the Kalamchi classification system [1] for congenital fibular deficiency was used; however, this classification was based solely on the anatomy of the fibula. Foot and ankle function and anatomy were not considered, both of which constitute important issues for planning the treatment. Birch et al. [2] recently proposed a new classification system for congenital fibular deficiency based on the functionality of the foot, degree of LLI, and functionality of the upper limbs. The system can be used to guide management. In this case, we did not believe the foot could be preserved and the upper limbs were functional, giving a Birch classification of type 2A, and therefore, based on this classification, the recommended treatment for this type of deficiency is amputation. In this case, we believe (and agree) that amputation is the preferred option over reconstruction.

Treatment Strategy 1. The decision was made that the foot could not be reconstructed in this case. It was therefore decided to proceed with a Syme amputation. This was performed at age 11 months when the child first attempted to walk. This avoided interference with normal developmental milestones. 2. Congenital fibular deficiency is associated with other limb abnormalities such as acetabular dysplasia, shortening and deformity of the femoral neck, lateral femoral condyle hypoplasia, and absent cruciate ligaments. Problems attributable to these abnormalities can present at any time during childhood. Therefore follow-up until skeletal maturity is essential.

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349

Images During Treatment

Outcome Clinical Photos and Radiographs

See Fig. 2.

See Fig. 3.

Technical Pearls

Avoiding and Managing Problems

1. If the tibial bow is less than 30 , this can be accommodated in the prosthesis. If the tibial bow is greater than 30 , a corrective tibial osteotomy is needed. 2. If the Achilles tendon is tight, perform a percutaneous tenotomy through a small stab incision to help expose the calcaneus and aid excision. 3. The plantar flap should be long enough to ensure the scar is located on the anterior aspect of the tibia. There should be no scars over the weight-bearing area of the stump.

1. Remove the calcaneus extraperiosteally. Do not leave fragments of cartilage and bone in the foot since these can grow and cause pain. 2. Protect the posterior tibial nerve and artery at all times. Ligate the nerve and vessels as distal as possible. This will ensure the stump is sensate and minimizes the chance of tissue necrosis. 3. Always place a drain in the stump to prevent pressure necrosis.

Fig. 2 Images showing skin incision for Syme amputation. The plantar incision traverses the midpoint of the metatarsals. The dorsal incision extends from the tip of the lateral malleolus to a point 1 cm distal to the tip of the medial malleolus. If the lateral malleolus is not present, this point must be estimated which can be difficult. Careful planning of the incisions is extremely important to avoid deficient skin flaps

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Fig. 3 (a) Anteroposterior standing radiograph of both legs at age 12 years demonstrating a left genu valgum. (b) Anteroposterior standing radiograph of both legs 11 months after insertion of 8-plate demonstrating correction of the genu valgum

Cross-References ▶ Fibular Hemimelia: Paley Type 3

References and Suggested Reading 1. Achterman C, Kalamchi A. Congenital deficiency of the fibula. J Bone Joint Surg (Br). 1979;61:133–7. 2. Birch JG, Walsh SJ, Small JM, et al. Syme amputation for the treatment of fibular deficiency: an evaluation of long-term physical and psychological functional status. J Bone Joint Surg Am. 1999;81:1511–8. 3. Birch JG, Lincoln TL, Mack PW, Birch CM. Congenital fibular deficiency: a review of thirty years’ experience at one institution

and a proposed classification system based on clinical deformity. J Bone Joint Surg Am. 2011;93:1144–51. 4. Catagni MA, Radwan M, Lovisetti L, Guerreschi F, Elmoghazy NA. Limb lengthening and deformity correction by the Ilizarov technique in type II fibular hemimelia: an alternative to amputation. Clin Orthop Relat Res. 2011;469:1175–80. 5. Gyr BM, Colmer HG, Morel MM, Ferski GJ. Hemiepiphysiodesis for correction of angular deformity in pediatric amputees. J Pediatr Orthop. 2013;33:737–42. 6. Hamdy RC, Makhdom AM, Saran N, Birch JG. Congenital fibular deficiency. J Am Acad Orthop Surg. 2014;22:246–55. 7. McCarthy JJ, Glancy GL, Chang FM, Eilert RE. Fibular hemimelia: comparison of outcome measurements after amputation and lengthening. J Bone Joint Surg Am. 2000;82:1732–5. 8. Naudie D, Hamdy RC, Fassier F, Morin B, Duhaime M. Management of fibular hemimelia: amputation or limb-lengthening. J Bone Joint Surg. 1997;79(B):58–65.

Intraoperative SSEP Monitoring of Circular External Fixation for Revision of Brown Rotationplasty

53

Marina Makarov, Charles E. Johnston, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 352 Preoperative Problems List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 352 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 352 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 356 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 356 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 356

Abstract

A 7 year old female born with proximal focal femoral deficiency (PPFD) underwent femoral rotation/adduction osteotomy and circular external fixation to revise unsuccessful femoro-pelvic fusion rotationplasty and subsequent rotation osteotomies. Due to high risk of neurovascular complications, intraoperative somatosensory evoked potential (SSEP) monitoring of tibial and peroneal nerves was utilized to control safety of acute repositioning of the lower limb during surgery.

M. Makarov (*) · C. E. Johnston Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]

Brief Clinical History A 7 year old female with Aitken type III PFFD had previously undergone femoro-pelvic arthrodesis and rotationplasty. Her femoral remnant was shortened, externally rotated, and fused to the pelvis to allow the anatomical knee and ankle function as hip and knee joints, respectively [1]. In addition, Chiari-type pelvic osteotomy with medial displacement of the distal fragment was performed. One year postoperatively, insufficient rotation of the limb in combination with knee laxity made prosthesis wear and ambulation uncomfortable and required additional femoral and tibial rotation osteotomies. Subsequent growth of the distal femur produced 50 abduction position of the femur with 90 external rotation of the lower extremity (Figs. 1 and 2).

M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_93

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Fig. 1 Preoperative appearance of the patient with an insufficient position of the right lower extremity after rotationplasty

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problems List • Inadequate position of the limb (50 abduction and 90 external rotation) after modified Van Nes rotationplasty and subsequent femoral and tibial rotation osteotomies • Difficulties with prosthesis wear and ambulation • High risk for neurovascular impairment during acute repositioning of lower extremity

Treatment Strategy Fig. 2 Preoperative radiograph of the pelvis illustrating iliofemoral fusion on the right with abducted orientation of the femur and almost 90 external rotation of the proximal tibia

Rotation osteotomy of the femur and circular external fixation followed by acute repositioning of the limb were elected for revision of rotationplasty. Intraoperative SSEP

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Fig. 3 Intraoperative SSEP monitoring demonstrating stable recordings after osteotomy and frame application and 4 episodes of nerve response deterioration. First significant alteration of the peroneal response occurred immediately after 90 of acute external rotation and 10 mm of medial translation. Despite partial internal de-rotation of the limb, second episode of complete disappearance of peroneal and tibial waveforms had occurred. Following recovery of both responses after returning the extremity to pre-corrected position, there was third complete loss of tibial SSEPs after attempted acute medial translation

without concurrent external rotation. Partial release of this correction produced an unstable recovery followed by fourth complete loss of tibial SSEPs. Complete elimination of medial translation resulted in restoration of evoked potentials back to normal, which allowed incremental 75 external rotation of the distal femur with no detrimental effect on SSEPs. The remaining 15 of external rotation and 15 mm of medial translation were performed gradually in postoperative period without any neurological deficit

monitoring of peripheral nerve function was utilized to control maximum allowable amount of acute medial translation and adduction of the distal femur and external rotation of the lower extremity. The remaining correction of the limb malposition was performed gradually in the postoperative period.

signal-to-noise ratio, each SSEP waveform represents the average of responses from 50 to 800 stimuli. Nerve stimulation continues throughout the entire surgical procedure with constant comparison of resulting signals with the baseline recordings. In addition, stimulation of nerves on the opposite extremity serves as control for SSEP changes resulting from anesthesia, physiological factors, and technical problems. Reduction of SSEP waveform amplitude >50% or prolongation of its latency >10% relative to baseline values are considered significant and indicative of neurological compromise. Those critical values were first utilized for spinal cord monitoring and subsequently proved to be reliable for peripheral nerve monitoring during external fixation procedures [4].

Basic Principles The protocol of intraoperative SSEP monitoring of external fixation on the femur includes stimulation of peroneal nerve at the dorsal surface of the ankle and tibial nerve at the medial malleolus with recording of resulting responses at the lumbar and cervical regions [2, 3]. Stimulation parameters include a pulse width of 0.1–0.3 ms and repetition rate of 2.35–3.24 stimulations/s. Stimulus intensity is adjusted individually, ranging from 20 to 80 mA. The recording parameters characterized by 30–2,000 Hz bandpass filter, 100 ms analysis time, and 10 μV/div gain. Depending on the

Images During Treatment See Figs. 3, 4, 5, 6, and 7.

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Fig. 4 Intraoperative photograph of the lower extremity after femoral remnant osteotomy, application of the TrueLok circular external fixator, and completion of acute repositioning of the limb. The frame consisted of a pelvic arch attached to the pelvis by two half pins, femoral arch secured to the distal femur by one half pin, and double-ring block fixated the tibia with one half pin and two crossing wires. Pelvic arch and double-ring tibial block with the femoral arch were attached to two larger diameter rings interconnected by three horizontal translation modules for gradual external rotation

Fig. 6 Photographs of the lower extremity at the completion of gradual external rotation demonstrating function of the ankle in the sagittal plane as a future “knee joint.” Horizontal translation modules were placed between pelvic and femoral arches for gradual medial translation

Technical Pearls

Fig. 5 Radiograph of the pelvis and lower extremity at the completion of gradual external rotation. Note medial position of the fibula at the end of correction

Several technical aspects need to be considered for reliable SSEP monitoring. Stimulus intensity should be adjusted for each patient to yield stable reproducible responses without excessive muscle twitch. In some children (e.g., with congenital anomalies), detailed preoperative examination may be required to identify proper position for stimulating and recording electrodes. All electrodes have to be securely fixed to the skin by disposable transparent dressings. Accidental dislodgment of any of the electrodes makes comparison of acquired responses after their repositioning inaccurate. Cooperation between the surgeon, anesthesiologist, and neurophysiologist is critically important throughout the entire surgical procedure and especially during the acute correction of limb deformity. The neurophysiologist must provide rapid feedback to the surgeon immediately after identifying any SSEP abnormalities. In turn, the surgeon has to inform the neurophysiologist about critical manipulations with the extremity and allow a sufficient time for SSEP recovery in cases of significant alterations.

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Outcome Clinical Photos and Radiographs See Figs. 8, 9, 10, and 11.

Avoiding and Managing Problems

Fig. 7 Radiograph of the pelvis and lower extremity at the completion of 15 mm gradual medial translation

Fig. 8 Clinical appearance of lower extremities 2 years postoperatively. Note 180 rotation of the ankle after revision of rotationplasty allowing the joint to function as a “knee”

Brown rotationplasty [1] for severe congenital femoral deficiency was developed to provide stability in patients with congenital absence of an anatomical hip joint and still benefit from rotationplasty [5]. The latter is known for difficulties such as incomplete intraoperative rotation of the limb and a high rate of postoperative de-rotation when the procedure was performed in children younger than 6 years of age. Preservation of peroneal nerve integrity is one of the most crucial components of the surgical procedure because peroneal paralysis will prevent active foot dorsiflexion, namely, “knee flexion.” The use of intraoperative SSEP monitoring allows acute repositioning of the limb to the maximum tolerable limit without risk of neurological compromise. Being readily adjustable at all stages of treatment, external circular fixation offers obvious benefit for rotationplasty procedures providing reliable stabilization of short bone segments, possibility of their incremental acute movements during the surgery and gradual correction of bone segment position postoperatively.

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Fig. 9 Radiograph of the pelvis 3 years postoperatively illustrating optimal orientation of the femoral remnant and stable position of the de-rotated tibia

Fig. 11 Radiograph of the pelvis and lower extremities at 12-year follow-up revealing solid femoro-pelvic fusion and perfect alignment of the de-rotated knee and ankle joints

Cross-References ▶ Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD)

See Also in Vol. 3 Intraoperative SSEP Monitoring of Circular External Fixation in Achondroplasia Treatment of Posterior Tibial Nerve Impingement After Tibial Lengthening Fig. 10 Clinical appearance of the patient’s lower extremities at 12-year follow-up. The patient can ambulate in prosthesis without limit in distance. Her anatomical knee is stable, and the anatomical ankle functions as a knee. She is a freshman at the university participating in all activities without special modifications

References and Suggested Reading 1. Brown KL. Resection, rotationplasty, and femoropelvic arthrodesis in severe congenital femoral deficiency. A report of the surgical technique and three cases. J Bone Joint Surg Am. 2001;83-A:78–85.

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2. Makarov MR, Delgado MR, Birch JG, Samchukov ML. Intraoperative SSEP monitoring during external fixation procedures in the lower extremities. J Pediatr Orthop. 1996;16:155–60. 3. Makarov MR, Samchukov ML, Birch JG, Johnston CE, Delgado MR, Rampy PL, Van Allen EM. Acute deformity correction of lower extremities under SSEP-monitoring control. J Pediatr Orthop. 2003;23:470–7.

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4. Makarov MR, Samchukov ML, Birch JG, Cherkashin AM, Sparagana SP, Delgado MR. Somatosensory evoked potential monitoring of peripheral nerves during external fixation for limb lengthening and correction of deformity in children. J Bone Joint Surg (Br). 2012;94-B:1421–6. 5. Van Nes CP. Rotation-plasty for congenital defects of the femur. Making use of the ankle of the shortened limb to control the knee joint of a prosthesis. J Bone Joint Surg (Br). 1950;32-B:12–6.

Knee Subluxation During Femoral Lengthening in a Six Year Old Boy with Congenital Coxa Vara and Congenital Short Femur

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Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 360 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 360 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 360 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 366 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 366

Abstract

A 6 year old boy with congenital short femur and coxa vara underwent staged correction of his deformities. First he had hip reconstruction with a proximal femoral valgus osteotomy and Dega pelvic osteotomy that was followed 6 months later by femoral lengthening. During the course of his lengthening, he developed a flexion contracture of his knee of 45 and subluxation of his knee. The distraction was stopped after obtaining 3.5 cm lengthening. He was admitted to the hospital, and after 2 weeks in traction and intense physiotherapy that failed to reduce the subluxation, he was taken to the operating room and had release of his Tensor Fascia Lata (TFL),” following which the subluxation was reduced. He was then put in a special type of Knee Ankle Foot Orthosis (KAFO) with hinges at the knee and a turnbuckle to maintain the knee in full extension. R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

Brief Clinical History This is a case of a 5 year old boy who was referred to our clinic for the management of congenital coxa vara and short femur. He had an unremarkable past medical history. Clinical examination revealed a limb length discrepancy (LLD) of 7.0 cm (from the right femur), decreased hip abduction on the right side compared to the left (30 vs. 45 ), and anterior laxity of the right knee. There was no evidence of associated fibular hemimelia. His LLD at skeletal maturity was estimated by the multiplier method developed by Paley to be 12.8 cm (7.0 cm times 1.83 + 12.8). Radiological examination showed a congenital coxa vara with a neck shaft angle of 95 and mild acetabular dysplasia.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

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1. Congenital coxa vara 2. Acetabular dysplasia 3. Congenital short femur with a limb length discrepancy of 7.0 cm, expected to be 12.8 cm at skeletal maturity 4. Valgus deformity of the distal femur 5. Congenital deficiency of the anterior cruciate

Treatment Strategy

Fig. 1 Plain X-ray of both hips showing coxa vara

The treatment strategy is based on the LLD at skeletal maturity and any associated deformities, specifically at the hip joint. The hip dysplasia should be addressed first and then the LLD. It was decided to proceed first with a reconstruction of the right hip in the form of a femoral valgus osteotomy to restore the neck shaft angle to 120 at least (abduction on the affected was 30 and adduction was 50 ) and a DEGA pelvic osteotomy and then to address the LLD. It was explained that – based on the expected LLD at skeletal maturity – there are several options. The first option is to correct the LLD with multiple lengthenings (at least two), the first one soon after correction of the hip dysplasia and the second one at skeletal maturity to address all the remaining LLD. Other options include huge shoe lift, one lengthening and a contralateral epiphysiodesis and shortening at skeletal maturity.

Basic Principles Congenital femoral deficiency is a group of disorders with a large spectrum of severity extending from mild shortening of the femur to severely deficient and abnormal hip and femur. It is associated with fibular deficiency in about half the cases. The treatment of any congenital limb deficiency is very challenging due to the numerous abnormalities present and in this case, specifically, abnormal hip anatomy, unstable knee, and severe LLD. The treating surgeon should be aware of all these problems and anticipate them first and, most importantly, beware of lengthening in congenital limb deficiencies!

Fig. 2 X-rays of the lower limbs standing showing short femur

Preoperative Problem List This is a case of congenital limb deficiency with all the challenges and problems associated with it, specifically the much higher complication rate than in non-congenital cases. Specific problems include:

– Always examine carefully – by both clinical and radiological examination – the joint above and the joint below the bone to be lengthened before you proceed with the lengthening procedure. This will prevent joint subluxation and will prevent or minimize soft tissue contractures. – In femoral lengthenings, first restore normal anatomy to the hip joint if any sign of dysplasia is present. – The knee should be protected from potential subluxation. The key thing here is to maintain the knee in full extension for prolonged periods of time – this will maintain the hamstrings in tension.

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Images During Treatment

Outcome Clinical Photos and Radiographs

See Figs. 3 and 4.

See Figs. 5, 6, 7, 8, 9, 10, and 11.

Technical Pearls

Avoiding and Managing Problems

• Decide first if a monolateral or circular fixator will be used. Always check for the presence of any deformity in the sagittal plane in order to plan for the type of external fixator to be used. • If a monolateral fixator is used (more comfortable for the patient), then: – First, insert the or wire parallel to the knee joint and then the most proximal pin – this will allow lengthening along the mechanical axis. – Application of the orthofix of the osteotomy side – Site of the osteotomy: try to, as distal osteotomies are more prone to lead to quadriceps stiffness. – Low-energy osteotomy – gigli saw or multiple drill holes followed by completion of the osteotomy with an osteotome through a mini-incision. – In femoral lengthenings, always release the TFL. Consider lengthening the medial hamstrings if any knee flexion contracture. – After insertion of the pins, always flex the knee completely, and at the end of the surgery, also flex the knee completely. You should obtain full flexion before the patient is awakened.

– It is well known that lengthening in cases of congenital deficiencies has a higher incidence of complications than lengthening in non-congenital cases. Always anticipate these complications and look for them. To have multiple small lengthenings is safer than one large lengthening. – Muscle contractures remain the number one enemy of any lengthening. Intensive physiotherapy, regular exercises by the patient (supervised by the family), and careful monitoring for the development of any contractures cannot be overemphasized. – How to avoid knee subluxation during the lengthening process? The key point is to maintain full extension of the knee. This will maintain continuous tension on the hamstrings and thus prevent flexion contracture. If the knee is in flexion, this leads to unopposed action of the hamstrings and predisposes to the development of knee flexion contracture and subsequent subluxation, especially if there is associated knee laxity due to cruciate deficiency. • There are several methods to keep the knee in full extension for prolonged periods of time including

Fig. 3 Femoral and pelvic osteotomies to correct the coxa vara

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Fig. 4 Patient with the orthofix

Fig. 5 (a, b) X-rays showing the knee in severe flexion with subluxation

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intense physiotherapy, simple braces with hinges at the knee joint, dynasplints, and crossing the knee with the frame with special hinges at the knee to maintain the knee in full extension while at the same allowing periodical flexion of the knee. • Weekly clinical examination of the range of motion of the knee during the whole distraction period and once monthly during the consolidation is a must. If any suspicion of knee subluxation specifically when associated with loss of full knee extension, radiological examination of the knee (lateral view) is warranted. – How to manage knee subluxation ? Once a diagnosis of knee subluxation has been established, the following should be instituted immediately: • The distraction should be slowed or stopped (some authors would even consider shortening). • Intense physiotherapy, traction, and braces to keep the knee in as much extension as possible. • A special type of brace with hinges at the knee (anterior and distal to the center of rotation of the knee which is situated at the junction of the posterior cortex with the physis) can be used to allow for distraction first and then translation of the distal segment. • If this fails, then surgical lengthening of the hamstrings and TFL may be necessary. • Extension of the fixator across the knee joint to reduce the subluxation.

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Fig. 6 (a–e) The effects of hamstring pull on knee stability (Modified from Paley [4]). (a) In full extension, any adverse effect of the hamstrings is opposed by the bony apposition of the femur and tibia. (b, c) During flexion of the knee, there is unopposed pull of the hamstrings and a tendency towards subluxation of the knee. However, this is partly opposed by stabilizing effects of the cruciate ligaments. (d, e) In absence or deficiency of the cruciates, any flexion of the knee, favors knee subluxation

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Fig. 7 Articulated KAFO showing the hinge placed anteriorly

Fig. 8 A patient wearing the articulated brace (KAFO), and a turnbuckle at the posterior part of the knee, to apply gradual distraction

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Fig. 9 Knee subluxation almost completely corrected

Fig. 10 X-ray showing good regenerate bone formation

Fig. 11 Final X-ray with a residual limb length discrepancy of 4.0 cm

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Cross-References

References and Suggested Reading

▶ Congenital Femoral Deficiency: Paley Type 1b and Paley Type 1c ▶ Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction ▶ Femoral Lengthening and Rotational Correction with a Precice Nail in a Patient with Congenital Femoral Deficiency and Femoral Retroversion ▶ Femoral Lengthening with MAC External Fixation System

1. Aston WJS, Calder PR, Baker D, Hartley J, Hill RA. Lengthening of the congenital short femur using the Ilizarov technique a single surgeon series. J Bone Joint Surg (Br). 2009;91-B:962–7. 2. Birch J. Lengthening for congenital lower limb deficiencies, Chapter 13. In: Hamdy RC, McCarthy J, editors. Management of limb length discrepancies, Monograph series 45. Rosemont: AAOS; 2012. p. 95–107. 3. Conway JD. Complications of distraction osteogenesis, Chapter 10. In: Hamdy RC, McCarthy J, editors. Management of limb length discrepancies, Monograph series 45. Rosemont: AAOS; 2012. p. 65–76. 4. Paley D. Problems, obstacles, and complications of limb lengthening by the Ilizarov technique. Clin Orthop Relat Res. 1990;250:81–90.

Proximal Tibial Recurvatum and Ipsilateral Short Femur Treated with a MAC External Fixation System

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Nariman Abol Oyoun and Richard S. Davidson

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 367 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 367 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 368 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 368 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 368 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 372 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 372

Abstract

An 18 year old male patient undergoes simultaneous bifocal correction of a post-traumatic double-hit growth disturbance of the right femur and tibia using two MultiAxial Correction (MAC) External Fixation Systems (Biomet, Warsaw, IN) with correction of both a limb length discrepancy (LLD) and an angular deformity.

Brief Clinical History Salter-Harris type II fracture of the distal right femur at the age of 13.5 was reduced and fixed with two pins. Nine months after the fracture, radiographic signs of premature physeal closure were observed at the distal femur, and a bony bridge developed at the anterior end of the proximal tibial physis at the tibial tuberosity (Fig. 1). At the age of 18, the LLD had reached 5.5 cm, mostly in the femur (Fig. 2 and Table 1), and surgery was planned. The tibial slope was reversed and had in total 26 recurvatum (Figs. 3, 4, and 5).

Disclosure: Dr. Davidson is a consultant for Biomet and receives royalties on the MAC external fixator of which he is a co-inventor.

Preoperative Clinical Photos and Radiographs

N. Abol Oyoun (*) Orthopaedic Surgery, Assiut University, Assiut, Egypt e-mail: [email protected]; [email protected]

See Figs. 1, 2, 3, 4, and 5, Table 1.

R. S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_304

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Fig. 1 Left: premature fusion of the distal femoral physis. Middle: bony bar formation, yet without deformity, at the anterior end of the proximal right tibial physis. Right: open left tibial physis

Preoperative Problem List • Shortening of the right femur and tibia with a total LLD of 5.5 cm • 26 recurvatum of the proximal right tibia

Treatment Strategy Surgery was performed at the age of 18 + 8 years: osteotomies of the right femur, tibia, and fibula were performed in addition to iliotibial band release, as well as anterior and lateral compartment fasciotomies of the leg after the application of two Multi-Axial Correction (MAC) External Fixation Systems, one at the femur with hinges opposite the osteotomy, to correct any secondary deformity if needed, and another at the tibia with the hinge at the CORA and angulated to conform to the deformity of the tibia. Lengthening was done at each osteotomy at a rate of 0.25 mm (90 or ¼ of a turn) four times a day. The patient was instructed not to bear any weight during the distraction phase and to continue physical therapy for the preservation of knee range of motion. The distraction phase lasted for 2 months, where a lengthening of 3.5 and 1.5 cm was achieved in the femur and tibia, respectively. Consolidation phase lasted for about 3 months.

Basic Principles

Fig. 2 Preoperative scanogram with LLD of 5.5 cm Table 1 Values of measured lengths of both lower extremities preoperatively Extremity Right Left

Femoral length 45.9 cm 50.0 cm

Tibial length 39.6 cm 41.0 cm

Total 85.5 cm 91.0 cm

The MAC External Fixation System is a monolateral external fixator that is applicable coaxially with the CORA or along the bisector, permitting fixation through a sufficient number of bone screws at safe anatomic locations, yet permits multiaxial correction, including angulation, translation, lengthening (compression and distraction), and rotation [1]. In this case,

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Fig. 3 Total premature closure of the femoral and tibial physes with resultant deformity of the proximal tibia

Fig. 5 Clinical photograph showing recurvatum at the proximal right tibia

two MAC frames were used concomitantly to address each deformity, which could have been done separately. At the tibia, lengthening was performed at first to disengage the fragments, then angulation was started for gradual correction of the recurvatum over 5 days. Fig. 4 Posterior proximal tibial angle (PPTA) with the bisector of the shaft is 107 (solid line). The normal PPTA is 81 (dotted line) [2]. Bisector line of a normal tibia starts one fifth of the way back from the anterior edge of the joint (dashed line). The CORA is where the normal and abnormal bisectors meet

Images During Treatment See Figs. 6, 7, and 8.

370 Fig. 6 Femoral osteotomy after the start of distraction

Fig. 7 Tibial osteotomy undergoing distraction, regenerate visible

N. Abol Oyoun and R. S. Davidson

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Technical Pearls The versatile and less cumbersome design of the MAC External Fixation System allows the simultaneous application of two devices on the same limb. By applying an arc to the MAC device, in this case to the proximal tibia, it was possible to apply the hinge opposite the CORA close to the knee joint and safely fixate to the bone above and below the planned osteotomy. Weekly follow-up during distraction, with AP & lateral radiographs, enables the early detection and timely reversal of secondary deformities during distraction using the multiaxial hinges without the need for major readjustment of the system or return to the OR.

Outcome Clinical Photos and Radiographs See Figs. 9 and 10.

Avoiding and Managing Problems

Fig. 8 Orthoroentgenogram two months into the distraction phase, showing 3 and 1 cm gains in the right femur and tibia, respectively Fig. 9 Femur lengthening of 3.5 cm after consolidation and removal of the MAC External Fixation System

Hydroxyapatite-coated screws were used to avoid loosening and infection. Femoral osteotomy developed varus during the distraction phase, so using the multiaxial hinge, correction of the varus was added to the regular lengthening done by the patient at home. During distraction, the right knee developed 25 flexion contracture and quadriceps weakness, which improved with vigorous physical therapy. During subsequent follow-up visits after the fixators were removed, the patient had full active knee extension and flexion to 110 with full muscle power. The patient later

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Fig. 10 Right tibia lengthening of 1.5 cm and correction of recurvatum after consolidation and removal of the MAC External Fixation System

on returned to jogging, and 6 months after fixator removal, he was back into complete normal activity.

Cross-References ▶ Femoral Lengthening with MAC External Fixation System

References and Suggested Reading 1. Davidson RS. The MAC (multi-axial correcting) monolateral external fixation system (Biomet/EBI) technique: an easier way to correct deformity. Oper Tech Orthop. 2011;21(2):113–24. 2. Paley D. Normal lower limb alignment and joint orientation. In: Principles of deformity correction. Berlin/Heidelberg: Springer; 2002. p. 1–18.

Proximal Tibial Recurvatum Corrected Using the Ilizarov Technique in a 14 Year Old Boy

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Elizabeth Ashby, Reggie C. Hamdy, and Franc¸ois Fassier

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 373 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 373 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 373 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 374 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 374 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 374 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 374 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 374 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377

Abstract

This is a case of idiopathic proximal tibial recurvatum in a 14 year old boy. The deformity was successfully corrected using the Ilizarov technique.

with the Ilizarov technique. The osteotomy was performed below the tibial tubercle so patella height was not affected. Normal mechanical axis was achieved. Six months following frame removal the boy was back to full sporting activities.

Brief Clinical History

Preoperative Clinical Photos and Radiographs

A 14 year old boy presented with pain and recurvatum in his left knee. There was no history of trauma or infection. The source of recurvatum was the proximal tibia. The deformity was treated

See Figs. 1 and 2.

E. Ashby (*) Division of Orthopaedics, Shriners Hospital, Montreal, QC, Canada e-mail: [email protected] R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

Preoperative Problem List 1. Proximal tibial recurvatum with normal patella height and no leg length inequality. 2. The physes remain open which influences placement of the osteotomy site.

F. Fassier Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_351

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Treatment Strategy This deformity can be corrected acutely with an osteotomy or corrected gradually using the Ilizarov technique. The advantages of the Ilizarov technique are the following: the correction can be “fine-tuned,” multi-planar correction can be achieved, any leg length inequality can be treated simultaneously, the height of the patella is not affected and the patient can weight-bear immediately after surgery. Disadvantages of the Ilizarov technique include the use of an external fixator for a prolonged period of time, risk of pin-site infection and risk of collapse, deformity, or fracture of the regenerate. The Ilizarov technique also requires close monitoring with regular outpatient appointments and X-rays throughout treatment. This is time-consuming for both the physician and patient. In this case the advantages of the Ilizarov technique were thought to outweigh the disadvantages.

Basic Principles

Fig. 1 Preoperative photograph showing a lateral view of the left leg

The basic principle in this case is to achieve normal lateral mechanical alignment of the leg by correcting the tilt angle of the tibial plateau while maintaining a normal patella height. Traditionally, an opening wedge osteotomy was used to treat tibial recurvatum. The osteotomy could be performed above, below, or at the level of the patella tendon. Each method corrected the tibial recurvatum deformity but created a secondary deformity (Figs. 3, 4, and 5). This problem is not encountered with the Ilizarov technique (Fig. 6).

Images During Treatment See Figs. 7, 8, and 9.

Technical Pearls 1. When applying the Ilizarov frame, placement of the hinges is crucial to achieving a good result. The hinges must lie at the apex of the deformity. 2. In a skeletally immature child the osteotomy site must be planned carefully to ensure the physis is not damaged.

Outcome Clinical Photos and Radiographs See Figs. 10 and 11. Fig. 2 Lateral radiograph of the left knee showing 66 tilt of the tibial plateau (angle between line of the lateral tibial plateau and lateral tibial mechanical axis). The normal tibial plateau tilt angle is 96 1

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Proximal Tibial Recurvatum Corrected Using the Ilizarov Technique in a 14 Year Old Boy

Fig. 3 An opening wedge osteotomy above the patella tendon insertion leads to patella baja

Fig. 4 An opening wedge osteotomy at the level of the patella tendon requires detachment of the tibial tubercle and reattachment, risking injury to the physis

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Fig. 5 An osteotomy below the level of the tibial tubercle translates the tibial plateau posteriorly the lateral mechanical axis of the tibia anteriorly. To achieve a neutral lateral mechanical axis, the tibial plateau must translate posteriorly

Fig. 6 An Ilizarov correction incorporates both angulation and translation and thus does not create any secondary deformity

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Fig. 7 Immediate postoperative lateral radiograph of the proximal tibia

Fig. 9 Lateral radiograph of the proximal tibia just before frame removal showing consolidation of regenerate bone

Fig. 8 Lateral radiograph of the proximal tibia 6 weeks after frame application. Deformity correction has been achieved, but consolidation of regenerate bone is not complete

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Proximal Tibial Recurvatum Corrected Using the Ilizarov Technique in a 14 Year Old Boy

377

Avoiding and Managing Problems 1. To prevent neurovascular injury, always protect the posterior aspect of the tibia while performing the osteotomy. 2. To minimize the chances of compartment syndrome, perform prophylactic fasciotomies, release the tourniquet prior to wound closure to ensure good hemostasis and insert a drain. 3. Do not use an epidural and ensure regular postoperative neurovascular observations. These measures will ensure early detection of compartment syndrome if it does develop.

Cross-References ▶ Correction of Tibia Recurvatum and Shortening in Skeletal Dysplasia ▶ Proximal Tibial Growth Arrest with Varus, Recurvatum, and Shortening After ACL Reconstruction. Correction with TSF ▶ Proximal Tibial Recurvatum and Ipsilateral Short Femur Treated with a MAC External Fixation System Fig. 10 Postoperative photograph of left leg showing correction of deformity

References and Suggested Reading 1. Babu M, Fassier F, Rendon JS, Saran N, Hamdy RC. Correction of the proximal tibial recurvatum using the Ilizarov technique. J Pediatr Orthop. 2012;32:35–41. 2. Choi IH, Chung CY, Cho TJ, et al. Correction of genu recurvatum by the Ilizarov method. J Bone Joint Surg (Br). 1999;81:769–74. 3. Herzenberg JE, Waanders NA. Calculating rate and duration of distraction for deformity correction with the Ilizarov technique. Orthop Clin North Am. 1991;22:601–11. 4. Moroni A, Pezzuto V, Pompili M, et al. Proximal osteotomy of the tibia for the treatment of genu recurvatum in adults. J Bone Joint Surg Am. 1992;74:577–86. 5. O’Dwyer KJ, Mac Eachern AG, Pennig D. Corrective tibial osteotomy for genu recurvatum by callus distraction using an external fixator. Chir Organi Mov. 1991;76:355–8.

Fig. 11 Postoperative lateral radiograph of left knee showing normalization of the tilt of the tibial plateau

Sequential Deformity Correction and Tibial Elongation Using Dormant Magnetic Nail Technique

57

Alan Katz and Ehud Lebel

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

379 380 380 381 381 381

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 384

Abstract

A 17-year-old female with Achondroplasia presented for the final phase of her elective leg elongation process and correct her left tibia deformity. The left leg was shorter and had an ankle varus deformation. Two stages were used to treat the deformity, the first to correct the coronal deformity and the second to address the length discrepancy. A distal osteotomy was performed followed by the insertion of a Precice elongating nail, which stabilized the correction. The Precice nail was left inactive until several months later when another osteotomy was performed in the proximal tibia (around the nail) and elongation was initiated. A careful multistep process was used to correct the varus deformation and limb length discrepancy while using a novel dormant nail technique.

Brief Clinical History The patient was a 17-year-old female with Achondroplasia who 9 years prior underwent limb lengthening of her femur and tibia bilaterally with Ilizarov frames. She recently (within previous 1.5 years) underwent elective re-elongation of both femurs and right tibia with elongating Precice nails. The procedure yielded a lengthening of 5 cm in both femurs as well as in the right tibia. She currently presents to complete the process and correct her left tibia deformity. The left leg has noticeable shortening and an ankle varus deformation.

Preoperative Clinical Photos and Radiographs See Images 1 and 2.

A. Katz (*) Pediatric Orthopedic Surgeon, Department of Orthopedic Surgery, Shaare Zedek Medical Center, Faculty of Medicine, Hebrew University, Jerusalem, Israel e-mail: [email protected] E. Lebel (*) Head of Pediatric Orthopedics Unit, Department of Orthopedic Surgery, Shaare Zedek Medical Center, Faculty of Medicine, Hebrew University, Jerusalem, Israel e-mail: [email protected]

Preoperative Problem List Varus deformity after Ilizarov frame Limb shortening Preventing return to varus after correction

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_381

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Treatment Strategy A two-stage process was used to treat the deformity, first to correct the coronal deformity and then address the length discrepancy. In the first phase, a distal osteotomy was

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performed followed by the insertion of a Precice elongating nail. Varus correction was executed with the use of a temporary blocking screw adjacent to the Precice nail (and a partial midshaft fibulectomy). The Precice nail, with its locking peg, stabilized the correction. Three months after the procedure, there were radiographic signs of tibial union. In the second phase, an osteotomy was made in the proximal tibia around the Precice nail using a Gigli saw. The upper and lower syndesmoses were fixated with screws, and elongation was initiated.

Basic Principles

Image 1 (a, b) After elongation of both femurs, and right tibia, the left leg has a limb length discrepancy and a varus deformity above the ankle

Image 2 (a–c) Preoperative planning broken down into two phases. Images demonstrate Phase 1 to distally correct the varus deformity with an opening osteotomy and the “insertion” of the Precice nail

Achondroplasia is a genetic bone growth disorder. Additionally, it can cause varus deformity of the lower legs because of the unusual growth of the fibula than that of the tibia. Currently, there is no cure for achondroplasia, but there are treatment options for the symptoms of the disorder. Limblengthening surgery can be discussed when the patient is old enough to weigh the risks and benefits. It can increase height in one or more lengthening steps of about 5–8 cm each [1]. The Precice nail is a magnet-operated telescopic internal lengthening device. It is normally used for one-time lengthening. The “dormant nail technique” describes the placement of the elongating intramedullary nail (Precice nail), with its activation occurring at a later time. This is performed to allow a one plane deformity adjustment, followed by elongation. This “dormant nail technique” is similar but different to

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Sequential Deformity Correction and Tibial Elongation Using Dormant Magnetic Nail Technique

Image 3 (a, b) Images above demonstrate intraoperative radiographs at the start and end of the first phase surgery. Post-osteotomy of the distal tibia, large section middle fibulectomy, and insertion of Precice nail with locking screws. At this point, the elongating nail was not activated

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Image 4 X-rays at patient follow-up after Phase 1 surgeries. Waiting for signs of callus and union of distal tibia

another method, labeled the “sleeper” nail concept. It has previously yet scarcely been discussed in the literature. It describes an additional limb lengthening using the same Precice nail after a period of inactivity [2, 3].

• The upper and lower syndesmoses were locked with screws to prevent migration of the fibula during tibia elongation.

Images During Treatment See Images 3, 4, 5, and 6.

Outcome Clinical Photos and Radiographs See Image 7.

Technical Pearls Avoiding and Managing Problems • After varus correction and healing, the tibia osteotomy before elongation was executed in the proximal tibia. It was carefully and circumferentially performed with a Gigli saw in order to prevent damage to the elongating Precice nail. • A long segment fibulectomy was performed to prevent a tether effect acting as a deforming factor. Additionally, it prevented the need for second fibulectomy during the second stage of the deformity correction.

Osteotomy around the nail was not performed with osteotomes, rather a Gigli saw, so as not to compromise the Precice nail. Surgeons and patients should be aware of the small chance the elongating Precice nail will not activate after its inactivity or be damaged from the osteotomy in its proximity.

382 Image 5 (a–c) Clinical photos and radiograph of the osteotomy cautiously performed with a Gigli saw around the Precice nail

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Image 6 (a, b) Intraoperative radiographs of the upper and lower syndesmoses, which were locked with screws to prevent migration of the fibula during tibia elongation

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References 1. Al Kaissi A, et al. Treatment of varus deformities of the lower limbs in patients with achondroplasia and hypochondroplasia. Open Orthop J. 2013;7:33–9. https://doi.org/10.2174/1874325001307010033. http://pubmed.ncbi.nlm.nih.gov/23459260/ 2. Eltayeby HH, et al. Post-retrieval functionality testing of PRECICE lengthening nails: the ‘sleeper’ nail concept. J Clin Orthop Trauma. 2020;14:151–5. https://doi.org/10.1016/j.jcot.2020.06.005. www. ncbi.nlm.nih.gov/pmc/articles/PMC7920018/ 3. Alrabai HM, et al. Limb lengthening reactivation with ‘sleeper’ Precice nails. Annual meeting of the Limb Lengthening and Reconstruction Society, July 2019. www.limblength.org/wp-content/ uploads/Limb-Lengthening-Reactivation-with-Sleeper-PRECICENails-ILLRS.pdf

Image 7 An EOS AP full leg X-ray showing the end of elongation and varus correction of the left tibia

Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot Equinus and LLD

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Alexander Cherkashin, John Birch, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 385 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 389 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 393 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 395 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 395 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 395

Abstract

Seven-year-old male with type IV tibial deficiency and severe rigid equinovarus foot deformity underwent sequential limb reconstruction involving minimal soft tissue release and tendon Achilles lengthening, gradual soft tissue stretching and translation of the foot distally, acute correction of foot equinus deformity and internal rotation, followed by tibiotalar fusion in plantigrade position with fibular fixation and separate tibial/fibular osteotomies followed by acute tibial varus deformity correction using circular external fixation.

A. Cherkashin (*) · J. Birch · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected]

Brief Clinical History This patient was referred as an otherwise healthy male infant with a unilateral clubfoot deformity (Fig. 1). In addition to a rigid equinovarus deformity, he was noted on initial orthopedic examination to have shortening of the right lower leg. His foot deformity was recalcitrant to manipulation and casting. The reconstructive options of foot ablation at walking age (Syme’s amputation) or staged reconstruction to bring the foot plantigrade and manage the limb length inequality were discussed with the mother, who declined amputation as a treatment option. The patient was therefore initially managed conservatively with a soft shoe and orthosis until deemed adequately developed and prepared to proceed with reconstruction. At 7 years of age, he presented with foot fixed in severe equinus position and 4 cm LLD (Fig. 2). Radiographic examination revealed tibiofibular “diastasis” with shortening (Fig. 3) corresponding to type IV congenital tibial deficiency of Jones classification [2] and type II of Weber classification [3].

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Preoperative Clinical Photos and Radiographs See Figs 1, 2, 3.

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• Leg length discrepancy partially compensated by rigid equinus deformity • Expected progressive increase in LLD with growth • Refusal of Syme’s amputation

Preoperative Problem List Treatment Strategy • Rigid equinovarus foot deformity and internal rotation recalcitrant to conservative treatment • Absence of functional ankle • Moderate midshaft tibial varus Fig. 1 Back view photograph demonstrating clinical appearance of the patient at 1 year 4 months of age. Note clubfoot-type of deformity initially managed conservatively with a soft shoe and orthosis

Fig. 2 Front and back view photographs showing clinical appearance of the patient at age 7. Note internal rotational and severe equinus deformity of the foot masking the leg length inequality

Limb reconstruction in type IV tibial deficiency usually involves several stages of treatment. The patient was initially managed conservatively until the distal tibial ossific nucleus

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Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot. . .

Fig. 3 AP and LAT radiographs at age 7 revealing type IV congenital tibial deficiency. Note distal tibiofibular “diastasis,” 20 midshaft tibial varus, 45 internal rotation, and severe 80 foot equinus position

developed. Initial sequential reconstruction was done at the age of 7 and consisted of (1) tendon Achilles lengthening (TAL); (2) gradual distraction through the “ankle” for soft tissue stretching and to bring the foot to the distal end of the tibia using TrueLok circular external fixation; (3) acute correction of the equinus position and internal rotational of the foot; (4) open tibiotalar arthrodesis combined with fibular fixation and midshaft tibial and fibular osteotomies followed by acute tibial varus deformity correction. A second and, possibly, third stages of treatment involving tibial lengthening were anticipated during subsequent growth of the patient. However, he remained without symptoms or functional limitation, and opted for epiphysiodesis of the contralateral proximal tibia and fibula at age 13.

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Fig. 4 Intraoperative photographs illustrating first stage of limb reconstruction. After limited soft tissue release (open TAL), partial correction of the foot position was performed acutely and TrueLok circular external fixator was applied for gradual foot repositioning distally. Note double plastic square nuts at the distal tibial ring for distraction and hinges with angular distractor positioned at the level of mid-tibia for the future varus deformity correction

Basic Principles Several treatment options are available for treatment of type IV tibial deficiency [1]. Foot ablation (Syme’s amputation) should always be considered and discussed with the family. This single-stage procedure performed at walking age will manage both severe foot deformity and leg length inequality simultaneously producing a stable end-bearing residual limb. Prosthetic replacement, however, is required. Occasionally, the severe clubfoot-like deformity of the foot can be corrected by Ponseti-type serial manipulation and casting. In type IV tibial deficiency cases, the foot malposition is usually much more severe than typical clubfoot deformity and, therefore,

388 Fig. 5 Postoperative radiographs before beginning of distraction. Note position of the “articulating surface” of the talus in-between distal tibial and fibular bone ends

Fig. 6 AP and LAT radiographs at the end of gradual foot repositioning. Note approximately 1 cm gap between the “articulating surface” of the talus and distal end of the tibia

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Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot. . .

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Fig. 7 Medial view photograph demonstrating clinical appearance of the limb at the end of gradual foot repositioning and soft tissue stretching. Note amount of distraction along the threaded rods (marked in red)

Fig. 8 Medial view photograph illustrating clinical appearance of the limb after acute correction of foot equinus and internal rotation. Under sedation, the distraction rods between the foot support and tibial frame were disconnected, foot was brought to plantigrade position acutely and external supports were reconnected using threaded rods and universal lockable hinges

conservative treatment is rarely “definitive” for the foot deformity correction, because patient has no functional ankle mortise. Correction of the foot equinus will unmask the associated leg length inequality. Therefore, a strategy for definitive management of the LLD must be developed. In our case, significant soft tissue obstruction to tibiotalar fusion of the foot in a plantigrade position existed including a very tight tendon Achilles holding the foot in rigid equinus and a band of ligamentous tissue encasing the end of the tibia. The former was released during the first stage of treatment (application of the tibial-foot apparatus for gradual reduction of the

foot deformity) and the latter was resected during the tibiotalar fusion. Gradual soft tissue stretching using incremental distraction can also be utilized to bring the talus underneath the distal tibial end and create a slack of soft tissue necessary to achieve plantigrade foot position without extensive soft tissue release.

Images During Treatment See Figs. 4, 5, 6, 7, 8, 9, 10, and 11.

390 Fig. 9 Lateral view photograph after acute foot repositioning. Note soft tissue creases in the mid-foot demonstrating tissue “reserve” created by gradual distraction

Fig. 10 AP and LAT radiographs after tibiotalar fusion performed 10 days after acute foot deformity correction. Simultaneously, separate tibial and fibular osteotomies were done followed by acute mid-tibial varus deformity correction using previously attached hinges and angular distractor

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Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot. . .

Fig. 11 Back and lateral view photographs demonstrating clinical appearance of the lower extremities at the end of consolidation period after tibiotalar fusion and mid-tibial varus deformity correction

Technical Pearls Very significant and rigid foot deformity including plantar flexion (60 or more) and internal rotation of the foot (up to 90 ) are typical for type IV tibial deficiency. Those deformities usually prevent normal shoe-wear, and temporary accommodating orthotics may be needed in preparation to limb reconstruction. The severity of foot deformity virtually obligates gradual tissue stretching using incremental distraction to prevent neurocirculatory embarrassment of the foot during deformity correction. Soft tissue releases should be judicious and performed through limited exposures to prevent skin breakdown during correction of deformity. Because the equinus deformity compensates for the LLD until at least 5 years of age, correction of the deformity (with tibiotalar fusion) will unmask that limb shortening plus prevent the patient from compensating the LLD by toe-walking. A broad band of tissue (resembling a confluent anterior tibial and posterior tibial tendons draped over the end of the distal tibial epiphysis) will prevent contact between the tibial epiphysis and talus. Therefore, this band should be resected to allow attempted tibiotalar arthrodesis.

Outcome Clinical Photos and Radiographs See Figs. 12, 13, 14, 15, 16, 17, and 18.

Fig. 12 AP radiograph 1 year after surgery showing good alignment and complete remodeling of bone regenerate with corticalization

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392 Fig. 13 LAT radiograph 1 year after surgery demonstrating maintenance of the plantigrade foot position. Note preservation of the distal tibial growth plate

Fig. 14 Front and medial view photographs illustrating clinical appearance of lower extremities 1 year after surgery

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Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot. . .

Fig. 15 AP standing radiograph at the age of 15 (7.5 years after surgery). Contralateral proximal tibial and fibular epiphysiodesis was performed at the age of 13

Avoiding and Managing Problems One of the goals of limb reconstruction in type IV tibial deficiency is tibiotalar arthrodesis to maximize maintenance of the foot in a neutral, plantigrade position. Accomplishing this goal required a strategy of delaying the first stage of “definitive” reconstruction until the distal tibial ossific nucleus had appeared (in our patient at age 7). The severity and rigidity of the foot equinus position and internal rotational must be recognized preoperatively. Although gradual correction of

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Fig. 16 LAT radiograph 7.5 years after surgery demonstrating tibiotalar fusion and plantigrade foot position

those deformities is advisable, it may be difficult to perform using circular external fixators with hinges or hexapod systems. Therefore, initial axial gradual distraction may produce soft tissue stretching thereby creating sufficient mobility to perform acute deformity correction with foot realignment followed by tibiotalar arthrodesis. Limited soft tissue exposures will minimize the risk of wound breakdown during correction. In some patients, distal fibula will project beyond the distal tibia. In such cases, “differential” tibial/fibular lengthening and/or distal fibular resection/epiphysiodesis may need to be incorporated into the treatment strategy.

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Fig. 17 Front, medial, and back view photographs showing clinical appearance of lower extremities 7.5 years after surgery. Note plantigrade foot position. Despite 4-cm LLD, patient is without complains and has no functional limitations. He is very active and participates in school football team

Fig. 18 Frames from the video demonstrating foot function during the gait

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Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot. . .

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Cross-References

References and Suggested Reading

▶ Type IV Tibial Dysplasia

1. Herring JA. Limb deficiencies. In: Herring JA, editor. Tachdjian’s pediatric orthopedics. 5th ed. Philadelphia: Elsevier Sanders; 2014. p. 951–1006. 2. Jones D, Barnes J, Lloyd-Roberts GC. Congenital aplasia and dysplasia of the tibia with intact fibula: classification and management. J Bone Joint Surg (Br). 1978;60:31–9. 3. Weber M. New classification and score for tibial hemimelia. J Child Orthop. 2008;2:169–75.

See Also in Vol. 2 Ankle Arthrodesis with Tibial Lengthening for Failed Pilon Fracture Ilizarov Ankle Fusion

Simultaneous Knee and Hip Dislocation During Femoral Lengthening in a Patient with Congenital Short Femur

59

Hae-Ryong Song and Kwang-Won Park

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397 Chief Complaint: Right Limb Shortening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398 Problems that can Occur During Lengthening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 399 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 400 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 400 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 404 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 404 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405

Abstract

Congenital short femur (CSF) is a type of bone dysplasia with an incidence of about 1 in 50,000 births. It can range from simple hypoplasia to proximal femur deficiency. Recently hip stability and anatomic variance have also been described in diagnosing and classifying this disease. It is the malformation most commonly associated with fibular hemimelia, present in 50% of such patients. CSF can have multiple associated deformities including acetabular dysplasia, coxa vara, femoral head retroversion, dysplasia of the lateral condyle of the femur, hypoplasia of tibial H.-R. Song (*) Department of Orthopedic Surgery, Korea University Medical Center, Guro Hospital, Seoul, South Korea e-mail: [email protected] K.-W. Park Department of Orthopedic Surgery, Korea University Medical Center, Guro Hospital, Seoul, South Korea e-mail: [email protected]

eminence, flattening of tibial plateau, anteroposterior knee instability, and ball and socket deformation of the ankle joint. We present the case of a patient with CSF who underwent femoral lengthening but during the course of treatment, suffered ipsilateral posterior hip and knee dislocation. Treatment was complicated by concomitant psoriasis, a disease which is known to exacerbate when a patient is under stress.

Brief Clinical History Chief Complaint: Right Limb Shortening A 10 year old patient presented with a 5 cm leg length discrepancy. The patient also had numerous scaly pink excoriations over the medial aspect of both thighs, which were psoriatic lesions. Expected limb length discrepancy at skeletal maturity using the Paley method was 6 cm. There was full range of motion of both knees and hips. The plan for the patient was femoral lengthening using a monolateral external fixator.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_75

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Preoperative Clinical Photos and Radiographs

Treatment Strategy

See Figs. 1, 2, 3, 4, 5a, b, and 6a, b.

1. Consider if a pelvic osteotomy must be performed for adequate acetabular coverage prior to lengthening of the congenital short femur. 2. Dysplastic hip joints must be investigated and monitored closely for the development of avascular necrosis. 3. During application of the external fixator, adductor and rectus femoris tenotomies can be performed to minimize chances of hip dislocation during lengthening. 4. MRI studies must be done to investigate the ligamentous structures surrounding the knee to determine presence of instability, which can predispose to dislocation. 5. Hamstring lengthening can be done simultaneously to prevent posterior subluxation of the knee. 6. Knee extension must be maintained during distraction to help prevent subluxation of the knee [4].

Preoperative Problem List Problems that can Occur During Lengthening 1. Hip joint dislocation due to acetabular dysplasia or femoral head retroversion 2. Knee joint dislocation due to hypoplastic femoral condyles, hypoplastic cruciate ligaments, as well as flattening of the tibial plateau 3. Exacerbation of psoriatic flares while patient is undergoing the lengthening process 4. Limitation of hip and knee range of motion while Ilizarov fixator is attached

Fig. 1 Ten-year-old patient with psoriatic lesions on the pelvis and the lower extremities

Fig. 2 Preoperative standing radiograph shows a leg length discrepancy of 5 cm

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Simultaneous Knee and Hip Dislocation During Femoral Lengthening in a Patient with Congenital Short Femur

Fig. 3 Hip radiograph showing mild acetabular dysplasia on the right hip

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Fig. 4 Anteroposterior radiograph of both knees, showing mild lateral condylar hypoplasia of the distal femur. There is absence of the lateral tibial spine, indicating hypoplasia of the posterior cruciate ligament

Fig. 5 (a + b) Lateral radiographs also showing congruent right and left knee joints

Basic Principles 1. Patients with congenital short femur may have concomitant acetabular dysplasia. This may predispose to hip dislocation during femoral lengthening. They may also have hypoplastic femoral condyles or hypoplastic cruciate ligaments, which may predispose to knee subluxation. 2. A preoperative acetabular index of more than 20 and center-edge angle less than 25 in a patient with congenital short femur predispose to hip dislocation.

3. During femoral lengthening, there is increased longitudinal distraction force transmitted to the acetabulum through the femoral head [1]. 4. For gradual correction of the angular deformity as well as translation during knee dislocation, the hinge must be placed anterior to the knee. If this is not done, there will be anterior compression of the femoral condyles during correction. 5. To determine placement of the hinge, get the longitudinal axes of both the femur and the tibia. A line perpendicular to the point where these two intersect is the location of the

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Fig. 6 (a + b) Ankle radiographs showing ball and socket deformation of the ankle joint and subtalar coalition

hinge. Placement of the hinge anterior to the joint enables simultaneous correction of translation and angulation (Fig. 7a, b). The figure below demonstrates the translation system for gradual reduction of the knee. Hinge placement is at a point where the posterior cortex of the femur and Blumensaat’s line intersect. With the help of the rod placed posterior to the knee, anterior translation can be done. A hinge placed at the joint center can then assist in the correction of the angular deformity (Fig. 8a, b).

Images During Treatment See Figs. 9a, b, 10a, b, 11, 12, 13a, b, 14, 15, and 16.

Technical Pearls 1. Patients with congenital short femur often have soft tissue contractures as well. Hip flexors, adductors, and hamstrings must be released as needed prior to lengthening to prevent hip and knee subluxation. 2. A monolateral fixator can be used for femoral lengthening. This provides sufficient stability as well as comfort for the patient.

Fig. 7 (a + b) Saw bone model and X-ray diagram showing location of the hinge anterior to joint center. This allows for correction of both translation and angular deformities

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Fig. 8 (a + b) Saw bone model of translation system. This allows for correction of translation. Afterwards, a hinge can be placed at the joint center, which is determined by the point at which Blumensaat’s line and a line parallel to the posterior cortex of the femur intersect

Fig. 10 (a + b) Immediate postoperative radiograph shows that the ipsilateral hip and knee joints are congruent. There is 36 of varus angulation after 3 cm of femoral lengthening

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Fig. 9 (a + b) Clinical photo showing exacerbation of psoriatic lesions after application of external fixator

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Fig. 12 Radiograph showing dislocation of ipsilateral hip after 4 cm of lengthening Fig. 11 Acute correction of the varus deformity was done, as well as application of an Ilizarov fixator

Fig. 13 (a + b) After open reduction of the hip, two half pins were placed on the pelvis for stability, and an intramedullary nail was inserted

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403

Fig. 14 Posterior subluxation of the knee joint after 5 cm of lengthening, one month after the hip dislocation

Fig. 16 A hinge was placed anteriorly, and a rod was placed posteriorly, and through gradual distraction, reduction of the knee was achieved Fig. 15 Placement of a hinge and gradual distraction before anterior translation

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3. When the diagnosis of a hip dislocation is made during lengthening, closed reduction can be attempted. If this fails, open reduction, with a femoral shortening osteotomy and a pelvic osteotomy, can be done [2]. 4. When the diagnosis of a hip dislocation is made, open reduction can be done with concomitant application of two half pins in the pelvis as an extension of the fixator to maintain the reduction. 5. The anterolateral approach can be used for open reduction of the hip. 6. When a knee dislocation is diagnosed during lengthening, medial and lateral hamstring releases can be done followed by closed reduction and maintaining the knee in extension for a few weeks [4]. 7. In the event of a knee dislocation, the knee can be reduced using the Ilizarov fixator and placing a hinge [3]. Knee extension must be maintained afterwards using a ring fixator.

Outcome Clinical Photos and Radiographs See Figs 17, 18a, b, and 19.

Avoiding and Managing Problems Fig. 17 At 4 months after the initial surgery, after removal of the fixator, there is a residual leg length discrepancy of 4 cm

Fig. 18 (a + b) Residual acetabular dysplasia at 5 months after surgery, although the hip has remained reduced

1. In patients with psoriatic lesions, gradual lengthening should be avoided to prevent pin track infections and skin problems. Instead, acute femoral shortening or epiphysiodesis may be a better option for correcting limb length discrepancy.

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Simultaneous Knee and Hip Dislocation During Femoral Lengthening in a Patient with Congenital Short Femur

Fig. 19 At 8 years postoperative, there is a residual leg length discrepancy of 3 cm

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2. Serial radiographs must be taken every week during lengthening to monitor for knee or hip subluxation. 3. Tenotomy of the adductors and rectus femoris is indicated when there is evidence of hip subluxation during lengthening. 4. Tenotomy should be followed by a decrease in lengthening rate and an intensive physical therapy program [5].

Cross-References ▶ Congenital Femoral Deficiency: Paley Type 1b and Paley Type 1c ▶ Congenital Short Femur – Lengthening Over a Rush Pin with Knee Ligament Reconstruction

References and Suggested Reading 1. Bowen R, Kumar SJ, Orellana C, Anderacchio A, Cardona J. Factors leading to hip subluxation and dislocation in femoral lengthening of unilateral congenital short femur. J Pediatr Orthop. 2001;21:354–9. 2. Dhawale A, Johari A, Nemade A. Hip dislocation during lengthening of congenital short femur. J Pediatr Orthop. 2012;21:240–7. 3. Hazra S, Song HR, Jajodia N, Biswal S, Modi HN, Srinivasalu S. Hip and knee dislocation during femoral lengthening in congenital short femur: a rare case report. Arch Orthop Trauma Surg. 2009;129:425–9. 4. Jones D, Mosely C. Subluxation of the knee as a complication of femoral lengthening by the Wagner technique. J Bone Joint Surg (Br). 1985;67(1):33–5. 5. Salai M, Chechick M, Ganel A, Blankstein A, Horoszowksi H. Subluxation of the hip joint during femoral lengthening. J Pediatr Orthop. 1985;5:642–4.

Staged Lengthening of 20 cm in the Femur and Tibia to Equalize Leg Lengths in a Growing Child

60

S. Robert Rozbruch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 407 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 408 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 408 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 409 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 409 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 412 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 412

Abstract

Although this child initially appeared to be a typical case of congenital LLD, it became apparent that with dysfunction of all the right lower extremity growth plates, a massive LLD would ensue. The LLD was coming from both the femur and tibia. Our plan after the initial femur lengthening was to do a lengthening of the tibia/fibula and then to come back and repeat a femur lengthening. Also planned was an epiphysiodesis of the contralateral side to prevent additional LLD after the last lengthening at age 15. The growth plate shutdown and dysplasia were not well defined despite additional consultation with several pediatric orthopedic colleagues and a geneticist. The main practical issue of LLD was, however, addressed with three well-timed lengthenings and an epiphysiodesis of the long

S. R. Rozbruch (*) Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA e-mail: [email protected]

side. This case illustrates an excellent functional and aesthetic outcome following 20 cm (8 in.) of limb lengthening in three stages. Adjuvant procedures including epiphysiodesis, quadricepsplasty, and gastrocnemius recession were all used to achieve limb salvage in this child.

Brief Clinical History J.S. presented to us at age 9 years with a 5.5 cm LLD (Fig. 1a, b). There was no history of infection or trauma, and growth plates looked normal on X-ray. This appeared to be a congenital LLD, and the predicted LLD at maturity was thought to be 7.5 cm using standard prediction methods. He underwent a right femur lengthening of 5 cm using a monolateral frame on the femur (Fig. 2a, b). A minimal incision quadricepsplasty was done to treat a knee extension contracture (40 ) with an excellent functional result (Fig. 3). This was uneventful, and we planned to do another small lengthening in a young teenager.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_55

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Fig. 2 (a) and (b) After 5 cm of femur lengthening using a monolateral frame (just before frame removal)

Fig. 1 (a) and (b) At age 9 showing 5.5 cm LLD

However, during follow-up, the growth plates were noted to be increasingly irregular. At age 12 years, the patient was noted to have 7 cm of additional LLD and trochanteric overgrowth at the hip (Fig. 4). The radiographs now demonstrated abnormal closure of all growth plates of the right lower extremity including the proximal and distal femur as well as the proximal and distal tibia. A poorly defined dysplasia affecting the growth plates was apparent. At age 12 years, he underwent a 7 cm lengthening of the tibia and fibula using an Ilizarov/Taylor Spatial Frame (TSF) (Fig. 5). Gastrocnemius recession was done to treat an equinus contracture of 20 . Closure of the proximal femur trochanteric growth plate was also done to prevent progression of hip deformity. This resulted in equal leg lengths at age 13 (Fig. 6). At age 15 years, he had an additional 8 cm of LLD. At that time, he underwent right femur lengthening over a nail (LON) to gain 8 cm and achieve equal leg lengths (Fig. 7). A contralateral left distal femur and proximal tibia epiphysiodesis was also performed to prevent additional

LLD. In total, he underwent 20 cm of right lower extremity lengthening in three stages (Fig. 8). The patient is now 21 years of age and has equal leg lengths, a normal gait, and normal hip, knee, and ankle range of motion. He has no functional limitations.

Preoperative Clinical Photos and Radiographs See Fig. 1a, b.

Preoperative Problem List 1. 2. 3. 4.

Unilateral lower extremity growth plate shutdown Massive predicted LLD Varus deformity of the hip Good condition of the hip, knee, ankle, and foot and no interest from family in amputation and prosthetic fitting 5. Extension contracture of the knee after first femur lengthening 6. Equinus contracture of the ankle after tibia/fibula lengthening

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Fig. 3 (a-c) Function after first femur lengthening and miniquadricepsplasty (age 9)

Treatment Strategy Our plan after the initial femur lengthening was to do a lengthening of the tibia/fibula and then to come back and repeat a femur lengthening. Also planned was an epiphysiodesis of the contralateral side to prevent additional LLD after the last lengthening at age 15.

Basic Principles Initially with a presumed diagnosis of congenital LLD, the predicted LLD was 7.5 cm using the multiplier method. Once it became apparent that the diagnosis was not a typical congenital case but rather that there was growth plate dysfunction, we made calculations using the growth remaining component of the multiplier method. Practically speaking, we tried to keep up with the progressive LLD by performing three staged lengthenings staggering the long bones of the lower extremity (femur-tibia-femur) followed by an epiphysiodesis of the distal femur/proximal tibia. Soft tissue contractures were treated with soft tissue releases. During the first femur lengthening and during the tibial lengthening, soft tissue contractures developed that were recalcitrant to physical therapy. Our minimal incision quadricepsplasty was

Fig. 4 (a) and (b) Age 12 with 7 cm LLD

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410 Fig. 5 (a) At age 12, after a 7 cm tibial/fibula lengthening with a TSF. (b) Recalcitrant equinus contracture (20 ) developed, and this was treated with gastrocsoleus recession

Fig. 6 (a–c) AP and lateral X-ray of the tibia and clinical photo after 7 cm tibia/fibula lengthening

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Fig. 7 (a) and (b) At age 15, during an 8 cm femur lengthening using LON technique

effective in treating the knee extension contracture at the end of femur distraction. Gastrocnemius recession was used to treat the ankle equinus contracture during the tibial lengthening. Well-timed soft tissue releases have been very effective in our practice to preserve normal joint range of motion. Varus deformity of the hip was addressed with epiphysiodesis of the greater trochanter in a growing child as part of a guided growth approach.

infection and impingement between rod and external fixation pins which could cause failure of the lengthening to progress. The external fixator pins were placed posterior and distal to the IM nail. At the end of distraction which was 3 months in this case, the IM rod was locked and the fixator was removed. This technique has been very effective for decreasing the time in external fixation and preventing fracture.

Outcome Clinical Photos and Radiographs Images During Treatment See Fig. 8. See Figs. 2, 3, 4, 5, 6, and 7.

Avoiding and Managing Problems Technical Pearls The second lengthening at age 15 was done using LON. This is a hybrid technique in which the femur is lengthened over an IM nail with an external fixator. There is no contact between internal and external fixation in order to avoid

Extension contracture of the knee during femur lengthening that is not responsive to physiotherapy can be managed with a mini-quadricepsplasty. Equinus contracture of the ankle that is not responsive to physiotherapy can be managed with a gastrocsoleus recession.

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Cross-References ▶ Adolescent with 7-cm Femoral Shortening due to Physeal Growth Deceleration: Femoral Lengthening with PRECICE Retrograde Intramedullary Nail ▶ Correction of Partial Epiphyseal Growth Arrest and 7 cm Shortening in 10 Years Old Girl ▶ Femoral Shortening (14 cm) and Deformity Treated with Acute Correction and Two Consecutive Retrograde Fitbone Applications

References and Suggested Reading

Fig. 8 (a) and (b) At age 16, front view and standing X-ray showing equal leg lengths after 3 right lower extremity lengthenings totaling 20 cm. Left side has undergone epiphysiodesis

1. Goldman V, McCoy TH, Harbison M, Fragomen AT, Rozbruch SR. Limb lengthening in children with Russell-Silver syndrome: a comparison to other etiologies. J Child Orthop. 2013;7:151–6. 2. Kahkaria S, Bigman D, Fragomen AT, Rozbruch SR. Comparison of PACS and hardcopy 51-inch radiographs for measuring leg length and deformity. Clin Orthop Relat Res. 2011;469(1):244–50. 3. Khakharia S, Fragomen AT, Rozbruch SR. Limited quadricepsplasty for contracture during femoral lengthening. Clin Orthop Relat Res. 2009;467(11):2911–7. 4. Mahboubian S, Seah M, Schachter L, Fragomen AT, Rozbruch SR. Femoral lengthening with lengthening over a nail has fewer complications than intramedullary skeletal kinetic distraction. Clin Orthop Relat Res. 2012;470(4):1221–31. Epub 6 Dec 2011 5. Paley D, Bhave A, Herzenberg JE, Bowen JR. Multiplier method for predicting limb-length discrepancy. J Bone Joint Surg. 2000;82-A (10):1432–46. 6. Rozbruch SR, Zonshayn S, Muthusamy S, Borst EW, Nguyen JT. What risk factors predict usage of gastrocsoleus recession during tibial lengthening? Clin Orthop Relat Res. 2014;472(12):3842–51.

Tibia Valga Treated with Tibial and Fibular Osteotomy and Application of a Multi-axial Correcting External Fixation System

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R. Jay Lee and Richard S. Davidson

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 413 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 414 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 414 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 414 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 415 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 415 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 418 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 418

Abstract

A 13 year old male developed tibia valga secondary to a tibial fracture resulting in physeal closure. He was treated with a left tibia and fibula osteotomy with application of multiaxial correcting (MAC) external fixation system for gradual correction of angular deformity and length. In addition, a left distal tibiofibular syndesmosis screw, a left proximal fibular epiphysiodesis, and an anterior and lateral compartment release were performed.

Disclosure: Dr. Davidson is a consultant for Biomet and receives royalties on the MAC external fixator of which he is a coinventor.

Brief Clinical History The patient is a 13 year old male who was a bicyclist struck by a motor vehicle. He sustained a combination Salter I and IV proximal tibia fracture. The Salter IV fragment was on the lateral side. There was also complete separation of the proximal epiphysis from the metaphysis (Fig. 1). He was treated initially with open reduction and internal fixation. The lateral fragment was addressed with partially threaded screws and the epiphyseal separation with smooth Steinmann pins. However, reduction was lost 1 week after surgery (Fig. 2). A revision open reduction with internal fixation was required (Fig. 3). The fracture healed, but tibial physeal arrest followed, with development of tibial valga and asymmetric growth between the tibia and fibula (Fig. 4).

R. J. Lee (*) Johns Hopkins Bloomberg Children’s Center, Baltimore, MD, USA e-mail: [email protected]; [email protected] R. S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_45

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Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

Preoperative Problem List • Post-traumatic tibial valga with asymmetric growth between the tibia and fibula • Open proximal fibular physis in the setting of proximal tibial physeal arrest

Treatment Strategy

Fig. 1 AP radiograph of the knee, demonstrating the initial fracture affecting the proximal tibial physis

Fig. 2 Radiographs of the knee demonstrating loss of reduction following treatment of the initial injury with open reduction and internal fixation. The AP radiograph (a) shows the tibia valga deformity and widening of the medial tibial physis. The two percutaneous pins have

The preoperative plan included identification of the center of rotation of angulation (CORA) for placement of the tibial osteotomy, to enable gradual correction of the tibia valga and lengthening with the MAC. The tibial osteotomy was placed just below the CORA and the tibial tubercle to prevent patella baja. The fibula was also osteotomized to avoid a bony tether to the correction, while the proximal fibular physis was closed to prevent further asymmetric growth. A syndesmotic screw was placed to maintain the proper relationship of the distal tibia and fibula at the ankle joint. Fasciotomies were performed prophylactically. After gradual correction of the

backed out and are not holding the fracture reduced. The lateral radiograph (b) also demonstrates how the percutaneous pins do not cross the physis and have allowed the proximal tibial epiphysis to slip anteriorly

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Fig. 3 Radiographs of the knee, AP (a) and lateral (b), demonstrating revision of the open reduction and internal fixation, with percutaneous pins that now cross the fracture site and hold the epiphysis reduced Fig. 4 Preoperative radiograph demonstrating the post-traumatic tibia valga

deformity, and healing of 3 of 4 sides of the tibial osteotomy, the external fixator and the syndesmotic screw were removed.

Basic Principles The goal of this procedure is to obtain a neutral mechanical axis with a horizontal knee joint. The MAC external fixation system in the management of lower extremity deformity allows the gradual correction of the deformity in two planes of angulation and two planes of translation, rotation, and lengthening. In this case, correction of valgus angulation and lengthening was obtained. This mono-lateral fixator allows correction without circumferential rings or computer-based calculations. The osteotomy of the fibula is necessary to remove the bony tether. If the fibula is left intact during correction, the proximal or distal tibial-fibular articulations may be disrupted. The syndesmotic screw prevents disruption of the distal tibia-fibula relationship. Finally, the epiphysiodesis of the proximal fibula will prevent further asymmetric growth once final correction is obtained.

Images During Treatment See Fig. 5.

416

Fig. 5 (a) AP radiograph of the proximal tibia, demonstrating how the proximal MAC construct is aligned with the knee joint (b) AP radiograph of the distal tibia, demonstrating how the distal MAC construct is aligned with the tibial shaft. (c) Radiograph of the syndesmotic screw; note the trajectory of the screw from its starting point in the tibia to the fibula. This angle provides a stronger anchor against potential deforming

R. J. Lee and R. S. Davidson

forces of the proximal correction than a transverse trajectory. (d) Lateral radiograph of the proximal tibia, demonstrating the spread of the screws in the proximal tibial fragment and the bi-cortical screw placement in the distal fragment. (e) Lateral radiograph of the distal tibia, with the syndesmotic screw aimed medial to lateral, proximal to distal, and anterior to posterior

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Technical Pearls

Avoiding and Managing Problems

Preoperative planning for correction of this deformity is the key to achieving the desired outcome. Appropriate radiographs must identify the CORA and any sagittal plane deformities, so that they may also be addressed simultaneously. The axis of correction must be placed purposefully, as placing the hinge directly over the CORA minimizes translation. Placing the hinge proximal or distal to the CORA produces translation. Placing the hinge on the convex or concave side of the deformity lengthens or shortens.

One must be mindful of the cross-sectional anatomy of the lower leg and place the external fixation pins in the safe zones, avoiding neurovascular structures. Prophylactic fasciotomies and careful neurovascular exams are performed to avoid and detect compartment syndrome. Postoperative care should include detailed instructions on the prescribed turning of the apparatus. Also, close follow-up should be maintained to detect and avoid malalignment during attempted corrections. As with all external fixation devices, pin site infection should be monitored, and removal of the external fixation device should not be attempted until 3 of 4 cortices are healed.

Outcome Clinical Photos and Radiographs See Fig. 6.

Fig. 6 (a) Postoperative AP radiograph demonstrating the gradual correction of the tibia valga at 4 months. Note how the proximal MAC construct is now perpendicular to the distal MAC construct, as the knee joint is brought perpendicular to the tibial shaft. (b) Postoperative lateral radiograph demonstrating the bony formation at the osteotomy site

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Cross-References

References and Suggested Reading

▶ Adolescent Blount’s Disease Treated with MAC External Fixation System ▶ Genu Varum Secondary to Rickets Treated with Femoral and Tibial Osteotomy and Application of a Multi-axial Correcting External Fixation System

1. Davidson RS. The MAC, (multi-axial correcting) monolateral external fixation system (Biomet/EBI) technique: an easier way to correct deformity. Oper Tech Orthop. 2011;21:113–24. 2. Paley D, Herzenberg JE, Tetsworth K, McKie J, Bhave A. Deformity planning for frontal and sagittal plane corrective osteotomies. Orthop Clin North Am. 1994;25:425–65. 3. Steel HH, Sandrow RE, Sullivan PD. Complications of tibial osteotomy in children for genu varum or valgum. J Bone Joint Surg Am. 1971;53A:1629–35.

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Tibial Hemimelia Muayad Kadhim and Richard S. Davidson

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 419 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 420 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 420 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 420 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 422 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 422 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 422 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 427 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 427 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 427

Abstract

Tibial hemimelia is a complex deformity that can cause knee joint instability, angular deformity of the leg, and deformity of the foot. The case presented here is of a young boy who was treated since birth for tibial hemimelia and underwent multiple surgeries for deformity correction. Multiaxial monolateral fixation system (MAC. Biomet) was utilized

Disclosure: Dr. Davidson is a consultant for Biomet and receives royalties on the MAC external fixator of which he is a coinventor. M. Kadhim (*) Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA e-mail: [email protected] R. S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected]; [email protected]

two times, initially to correct a clubfoot deformity and, later, for tibial angular correction.

Brief Clinical History A 10 days old boy presented to the clinic with bilateral knee dislocation and clubfeet. Radiographs demonstrated bilateral absent tibiae. Physical examination and bilateral lower extremity ultrasound examination, however, demonstrated that the patient had complete absent tibia on the left side and subtotal absence on the right side with lateral subluxation of the patella. The left knee with an absent tibia was unstable and was treated with knee disarticulation at age 10 months. Right Achilles tendon lengthening was done at the same time. The surgical plan was to do right tibiofibular synostosis when the proximal tibial remnant was well ossified. At age 5.5 years, the patient underwent tibial and fibular osteotomy and synostosis with a rush rod and anterior compartment release. The rush rod was maintained until good union was achieved. At age 8 years, the

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_47

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patient underwent surgery for clubfoot release with application of external fixator for gradual correction of the clubfoot deformity and gradual centralization of the foot under the fibula. After 9 months, the patient underwent surgery for talofibular centralization and fusion with allograft, using the same MAC external fixator construct. Four months following to that (13 months after the initial surgery), the MAC external fixator was removed. The patient was able to ambulate with a walker and a brace. He had full knee flexion, 45 lag of full knee extension, and the tibia was translating anteriorly with knee extension due to congenital anterior cruciate ligament (ACL) deficiency. He also had knee varus deformity. At age 10 years, the patient underwent surgery for ACL reconstruction to enhance knee stability. Eighteen months later, the patient underwent right tibiofibular osteotomy with application of MAC external fixator for varus deformity correction. In addition, he underwent right proximal fibular epiphysiodesis to prevent further fibular head prominence. The external fixator was removed after 3.5 months.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

Fig. 1 Radiographs of the right leg at age 7 months represent apparent complete absence of the tibia on X-ray, but on examination there was a small portion of the upper tibia with active full flexion and extension

M. Kadhim and R. S. Davidson

Preoperative Problem List 1. 2. 3. 4. 5.

Tibial hemimelia Persistence clubfoot deformity Achilles tendon contracture Knee joint instability due to congenital ACL deficiency Knee varus deformity with prominent fibular head

Treatment Strategy 1. After reconstruction of the limb with tibiofibular synostosis, the plan was to perform gradual clubfoot deformity correction and talar fibular centralization to achieve ankle joint fusion. 2. MAC external fixation was utilized for equinus and foot adduction correction. Gradual ankle joint distraction and talar fibular centralization was simultaneously achieved utilizing the same MAC construct. 3. Knee varus deformity was addressed later with proximal tibiofibular osteotomy and gradual correction with MAC system.

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Fig. 2 Radiographs at age 5 years represent primitive ossification of the proximal tibial remnant and persistent clubfoot deformity

Fig. 3 Intraoperative radiographs of tibiofibular synostosis done at age 5.5 years

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Fig. 4 Radiographs healed tibiofibular synostosis at 3 years after surgery

Basic Principles

Images During Treatment

1. The goal of treatment was to achieve gradual correction of the clubfoot and the angular deformity to improve functional motion of the knee and the ankle and achieve plantigrade foot for better weight bearing. 2. Gradual correction of angulation in different planes can be accomplished using multiaxial monolateral external fixation device. The rotational deformity can be addressed with a rotation arc. This fixation system provides easier treatment (turning one hinge at a time), no computer assessment and no rings. Correction with the MAC is as accurate as with any external fixation devices as the end point of correction is determined by final radiograph showing appropriate correction. All the basic principles of external fixation apply.

See Figs. 5, 6, 7, 8, 9, 10, and 11.

Technical Pearls The application of MAC external fixator is less complicated than circular external fixators. Potential risk of surgery is less with MAC system as screw insertion sites can be chosen to avoid neurovascular structures. The MAC external fixation can be extended across the joints as was done in the presented case to provide correction to the ankle and the foot. In this case, a rotational arc was added to the fixator across the ankle for correction.

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423

Fig. 5 Intraoperative radiographs represent the application of the external fixation after performing soft-tissue release of the clubfoot. (a) Tibial pins insertion. (b) Foot pins insertion. (c) Adding rotation arc to the fixator

Outcome Clinical Photos and Radiographs See Fig. 12.

Avoiding and Managing Problems Patients with tibial hemimelia have complex bone and softtissue deformity which can be corrected gradually to avoid neurovascular and skin complications.

In this particular case, the correction of the clubfoot deformity with external fixator was delayed until the proximal tibiofibular synostosis was accomplished. In addition, fibular to talar fusion was performed after the foot was corrected with the external fixator, and the ankle joint was gradually distracted with the external fixator. Proximal tibiofibular osteotomy was done after addressing knee instability with ACL reconstruction to avoid inappropriate bony correction due to ligamentous laxity.

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Fig. 6 Radiographs demonstrating the external fixation and the talofibular centralization Fig. 7 Radiographs at 8 months after surgery represent healed fusion of the ankle joint

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Fig. 8 Radiographs demonstrating the right knee joint after ACL reconstruction and proximal tibia fibular varus deformity

Fig. 9 Intraoperative radiographs demonstrating proximal tibiofibular osteotomy and MAC external fixator system application for varus deformity correction

425

426 Fig. 10 Radiographs at 3 weeks after surgery demonstrating gradual osteotomy site distraction

Fig. 11 Radiographs at 5 weeks after surgery demonstrating gradual varus deformity correction

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Fig. 12 Radiographs at 5 months after external fixator removal. The patient underwent surgery for anterior cruciate ligament reconstruction to enhance knee stability

Cross-References

References and Suggested Reading

▶ Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a) ▶ Tibial Hemimelia Type II in a 6 Year Old Child Treated with Fibular Transfer and Centralization and Syme’s Amputation

1. Carranza-Bencano A, González-Rodríguez E. Unilateral tibial hemimelia with leg length inequality and varus foot: external fixator treatment. Foot Ankle Int. 1999;20(6):392–6. 2. Davidson RS. The MAC (Multi-axial correction) monolateral external fixation system (Biomet.EBI) technique: an easier way to correct deformity. Oper Tech Orthop. 2011;21(2):113–24.

See Also in Vol. 2 Older Clubfoot with Osteotomy Treated with MAC External Fixator

Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)

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Dror Paley and Craig A. Robbins

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 431 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 431 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 431 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 431 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 432 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 432 Case 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 432 Case 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 436 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 436 Type 5a . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 438 Type 5b . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 438 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439 Case 1 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439 Case 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 439 Case 1, Paley 5a/Weber Patelloplasty . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 441 Case 2, Paley 5b . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 441 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443

Abstract

Tibial hemimelia (TH) often appears as a shortened leg with knee and ankle deformity. Most often, there are a varus deformity and knee flexion contracture, though multidirectional instability may also be present due to a lack of collateral ligaments. A patella and quadriceps mechanism may be present or absent and may have D. Paley (*) Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA e-mail: [email protected]; [email protected] C. A. Robbins Paley Advanced Limb Lengthening Institute, West Palm Beach, FL, USA e-mail: [email protected]

limited function if present. There may be cutaneous manifestations such as dimples. The tibia may be shortened, dysplastic, or absent; it may also present as an anlage invisible to radiographs. The fibula can be normal or dysplastic and often subluxated or dislocated proximally or distally. The ankle is often in varus and equinus, and the foot may be adducted and supinated. The medial side of the foot may be missing rays. In the current literature, the treatment of choice for severe tibial hemimelia with complete absence of the tibia has historically been biased toward amputation, though it is dependent upon the classification of the deformity. Certainly, the presence of a strong quadriceps, patella, and proximal tibia or anlage seems

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_34

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to favor reconstruction, and tibia-fibular arthrodesis has met with good results. Their absence may be indicated by the absence of a patella, which can be difficult to find in young children. Thus, current imaging options such as MRI and ultrasound are a useful method of determining the presence of the patella or tibia anlage. The Weber patelloplasty was published in 2002 and described a surgical procedure in which the patella was converted into a tibial plateau. Dissatisfied with the existing classifications and prior to Weber’s publication, Paley proposed a new

classification in 2003 (Fig. 1). As with his previous classifications, each type and subtype has a different surgical treatment. The progression of deficiency is from least to greatest, and the classification assumes that TH is a progressive spectrum of deficiency from distal to proximal. Therefore, the Jones type 3 proximal deficiency has been eliminated, not only for its rarity, but since it is a proximal instead of a distal deficiency of the tibia, it belongs in the congenital deficiency of the knee classification (unpublished).

Fig. 1 Paley Tibial Hemimelia Classification. Type 1: valgus proximal tibia (genu valgum), overgrowth of the fibula proximally, plafond present and normal. Type 2: tibial plafond missing. Medial and lateral malleolus present; the distal fibula (lateral malleolus) normally aligned to lateral talus and together with the foot internally rotated around the tibia. Talus sits between the bones due to absence of tibial plafond. Relative proximal fibular overgrowth present. Type 3a: well-formed distal tibial physis and separate from proximal growth plate of the tibia. Plafond present but dysplastic. Major relative overgrowth of the fibula. Type 3b: delta tibia: proximal and distal growth plates connected through bracket epiphysis. Malorientation of ankle and knee joint ends of the tibia. Major relative overgrowth of the fibula. Type 4a: delayed

ossification of part, or all, of the tibia. Ankle joint present but distal physis absent. Major relative overgrowth of the fibula. Type 4b: diaphyseal deficiency of the tibia. Complete absence of the distal tibia at the level of the diaphysis; the proximal tibia has atrophic, pointed distal bone end often covered by separate skin pouch. Major relative overgrowth of the fibula. Type 4c: epiphysis of the proximal tibia present but absent proximal physis. Delayed ossification of the epiphysis. Major relative overgrowth of the fibula. Type 5a: complete absence of the tibia; the patella present; flexion contracture of the knee. Type 5b: complete absence of the tibia; no patella present; (i) fibula centralized, (ii) fibula dislocated; flexion contracture of the knee present

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Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)

Brief Clinical History Case 1: This is a child with Paley type 5a/Jones 1b tibial hemimelia. There is complete tibial agenesis with quadriceps and patella present. She had previous failed ankle surgery at an outside facility. Case 2: This is a child with Paley type 5b/Jones 1a tibial hemimelia. There is complete tibial agenesis with no quadriceps and no patella present.

Preoperative Clinical Photos and Radiographs See Figs. 2 and 3.

Preoperative Problem List • Absent tibia (both cases) • Quadriceps present or absent (Case 1 present, Case 2 absent) • Patella present or absent (Case 1 present, Case 2 absent) • Dislocated knee (both cases) Fig. 2 AP (a) and lateral (b) radiographs of Paley type 5a TH. The foot was previously treated and remains in an equinovarus deformity. Note the presence of the patella (Copyright 2006, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

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• Dislocated ankle (Case 1 ankle previously treated but with malunited fusion, Case 2 present) • Flexion contracture of knee (both cases) • Equinovarus contracture of ankle (both cases)

Treatment Strategy • Consider whether this is unilateral or bilateral TH. • Consider extent of any upper limb involvement that may alter treatment plan for lower limbs. • Investigate family history since TH has a strong genetic background. • Identify presence of any tibial anlage, patella, and quadriceps using physical exam, X-ray, ultrasound, and MRI. • Classify with Paley Classification based on the amount of tibial deficiency and presence or absence of quadriceps and patella. • For unilateral cases, predict leg length difference using the Paley Multiplier method. • If a patella is present, it can be used to reconstruct the knee using the Weber patellar arthroplasty:

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Fig. 3 AP (a) and lateral (b) radiograph of Paley type 5b TH. There is a dislocation of the fibula on the femur and the foot on the fibula. There is a severe equinovarus of the foot and flexion contracture of the knee. No patella is present

• If no patella is present, the fibula can be centralized by distraction followed by centralization and stabilization with an intra-articular femuro-fibular ligament. Obtain a stable ankle with a plantigrade foot. • Protect the proximal and distal fibular physes.

Basic Principles Complete absence of the tibia presents the biggest challenge for reconstruction because there is no knee joint. While ankle fusion gives good function with little disability, knee fusion leads to significant disability for sitting and climbing stairs. It is preferable to avoid a knee fusion. Even if active knee motion cannot be achieved, a mobile knee joint supported by a brace is preferable to a knee fusion. This is not dissimilar to a paralytic knee from polio. The reconstruction for tibial hemimelia type 5 is staged between limb realignment with an external fixator and later joint reconstruction. Prior to application of the external fixator, percutaneous intramedullary wires are placed through the entire fibula, bent, and buried in the proximal and distal epiphysis to protect the physis during distraction (see Fig. 21a, b). An external fixator ring is applied to the femur and foot (see Fig. 20a, b). A transverse stirrup wire is placed

through the fibula and attached to the distal foot ring so that the fibula and foot move as a unit. The initial TSF program serves to distract and correct the knee flexion contracture and align the proximal fibula epiphysis beneath the distal femur. At that point, the fibula stirrup wire is disconnected from the distal foot ring and is then attached to the proximal femur ring. This now stabilizes the fibula, in its corrected position, to the femur. A second TSF program is then created to distract and correct the ankle contracture and align the foot beneath the distal fibula epiphysis (see Figs. 20a, b). The bent fibular epiphysiodesis wires protect the physes during these distractions. After both the knee and ankle joints have been aligned, the joints can be reconstructed as outlined in this article. In the presence of an extensor mechanism with patella and quadriceps, a Weber patellar arthroplasty is performed. In their absence, a knee ligament reconstruction is performed. At the foot, the goal is fusion of the distal fibular epiphysis to the talar ossific nucleus in a plantigrade position.

Images During Treatment Case 1 See Figs. 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, and 19.

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Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)

Fig. 4 Weber patellar arthroplasty incision (Copyright 2006, Rubin Institute for Advanced Orthopedics, Sinai Hospital of Baltimore)

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Fig. 5 The underlying fascia is exposed over the patella, femur, and tibia

Fig. 6 The peroneal nerve should be exposed and decompressed and the biceps tendon lengthened

Biceps tendon to be lengthened Peroneal nerve is exposed and then decompressed

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Fig. 7 The two visor flaps are marked out

Proximal flap incision is made and Z-lengthening of quadriceps tendon is performed Proximal flap

Distal flap Flaps converge incompletely leaving a pedicle for the circulation of the flap

Fig. 8 The visor flaps are incised, and the posterior capsule is cut with great care to identify the vessels posteriorly so as not to injure them

Knee joint capsule is released posteriorly

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Fig. 9 The two visor flaps are flipped so that the proximal one goes distal and the distal one goes proximal

Fig. 11 H-flaps are incised into the perichondrium of the fibular head

Fig. 10 The quadriceps tendon is repaired and the visor flaps sutured together. The patella is now located inferior to the femoral condyles

Fig. 12 The cartilage overlying the proximal fibula is exposed

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Case 2 See Figs. 20, 21, 22, and 23.

Technical Pearls • Protect the fibular physes with temporary epiphysiodeses to prevent fracture during distraction. • One fixator allows consecutive treatment of knee and ankle deformities: place one fixator ring on the femur and one ring on the foot, and capture the fibula with a stirrup wire; attach the stirrup wire to the foot ring when correcting the knee deformity; attach the stirrup wire to the femur ring to correct the foot deformity. • For the femur ring, place 2 half pins for the femoral ring: one up the femoral neck and one transverse at the level of the lesser trochanter; place one transverse olive wire across the femur ring. • At the time of staged ankle and knee reconstruction, remove the foot wires and ring, and leave the femoral ring in place; the fixator will then be reapplied after the procedure. Fig. 13 The ossific nucleus of the fibular head is exposed by resecting some of the overlying cartilage Fig. 14 Corresponding H-flaps are incised into the perichondrium of the patella, and the ossific nucleus is exposed by resecting some of the overlying cartilage

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Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)

Fig. 16 The H-flaps are sutured together Fig. 15 The patella and fibular head are mated to each other

Fig. 17 AP (a) and lateral (b) radiographs after the Weber patellar arthroplasty were performed and the patella joined to the fibula. The ankle was fused in a plantigrade position for the foot. The circular external fixator stabilized the knee and the ankle

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Fig. 18 AP (a) and lateral (b). After distraction, the knee is straight. Not the stirrup wire stabilizing the patella during the flexion contracture correction

Type 5a

Type 5b

• Find and protect the popliteal vessels before completing the posterior capsulotomy when raising flaps for the Weber arthroplasty. • At the medial and lateral ends of the “visor” flaps, keep the pedicles as wide as possible. • In the Paley modification, the distal visor flap includes a small part of cartilage of the inferior pole of the patella which allows a new patella to form anterior to the distal femur. • Consider using BMP into drill holes in the cartilage of the patella and fibular epiphysis to ensure bone-to-bone healing. • Because the knee and ankle were predistracted, they can both be fixed by one axial wire across the ankle and knee joint after staged stabilization. • Reapply the foot ring and use the TSF as a static holding frame to ensure full knee extension and plantigrade foot for 3 months. • After 3 months, remove the fixator and shorten the transknee wire to span only the fibula and foot, and place a cast for month; then use a KAFO for several years until the knee is stable.

• Through midline anterior incision, the distal femur and proximal fibula are exposed. • Liberate and decompress the peroneal nerve. • Split biceps tendon with half left attached to the fibula to be used as a lateral collateral ligament; the other part is transferred to the fibula anteriorly to rebuild a quadriceps muscle. • Medially, the semitendinosis and adductor magnus muscles are transferred anteriorly; laterally, the TFL and ITB are harvested and transferred; all four are connected to the quadriceps which usually ends in the mid-thigh; balance the medial and lateral muscles, and centralize them to the ITB which acts as a central rib for the connection of the muscles and is eventually attached to the front of the fibula to act as a patellar tendon. • Before connecting the transferred muscle, anchor the fibular head to the femur by creating an interosseous ligament between the femur and fibula with half of the biceps tendon as autograft; this prevents subluxation of the fibula from the femur. • Fuse the ankle. • A wire is inserted from the foot through the fibula and across the knee joint.

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Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)

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• Reapply the foot ring, and place a static TSF, and hold the foot plantigrade and knee in full extension for 3 months. • After fixator removal, leave the medullary wire across the knee and ankle, and place a cast for 1 month; remove the wire 6 months later, and start knee rehab. • Then use a KAFO for several years.

Outcome Clinical Photos and Radiographs Case 1 See Figs. 24 and 25.

Case 2 See Fig. 26.

Avoiding and Managing Problems

Fig. 19 After removal of the fixator, an intramedullary wire was used to help protect the fibula

Fig. 20 AP (a) and lateral (b) X-rays: A Taylor Spatial Frame is applied from the femur to the foot. A temporary epiphysiodesis wire is in place in the fibula. A transverse stirrup wire is placed through the fibula and attached to the distal ring

• Accurately classify the congenital deficiency to plan the appropriate staged surgical reconstruction. • Through-knee amputation is a reliable option that should be considered in unilateral cases. In bilateral cases of Paley type 5, complete tibial aplasia reconstruction by one of the above-described techniques should be considered, especially if a side has a patella.

440 Fig. 21 AP (a) and lateral (b). After 3 months of distraction, the knee contracture is eliminated, and the fibula is reduced below the end of the distal femur. The stirrup wire through the fibula allowed it to be pulled distally with the foot ring. After the knee was aligned, the stirrup wire was detached from the foot ring and attached to the proximal ring. Then a new TSF program was performed to align the foot beneath the distal fibula

Fig. 22 AP (a) and lateral (b). After the limb is aligned, the next stage is knee and ankle joint reconstruction. The foot is fused to the distal epiphysis of the fibula. The biceps, semitendinosis, tensor fascia lata, and adductor magnus muscles are all transferred to the knee to substitute for the missing quadriceps muscles. A temporary arthrodesis wire is placed across the knee and ankle. The external fixator was removed three months after the knee-ankle surgery. The wire across the knee and ankle is left in place for six months to allow better loading and minimize the risk of recurrence. It is then removed

D. Paley and C. A. Robbins

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Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)

• Place the external fixator rings as described with a transverse fibular stirrup wire to allow gradual consecutive correction and distraction of knee and ankle deformities.

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Case 1, Paley 5a/Weber Patelloplasty • Predistract and correct the knee flexion contracture prior to the Weber patelloplasty to allow the knee to be held in full extension after the arthroplasty. • Identify the popliteal vessels prior to elevating the distal flap with the capsulotomy. • Create corresponding H-flaps perpendicular to each other on the patella and fibula to allow them to be sewn together; place the posterior, deepest sutures first. • Consider BMP at the fibula-patellar junction to encourage bone-to-bone fusion. • The foot must be plantigrade after the ankle fusion.

Case 2, Paley 5b

Fig. 23 The biceps tendon is used to create a cruciate ligament to tether the fibula to the femur

Fig. 24 Lateral of the knee and tibia in extension (a) and flexion (b) after healing from the Weber patelloplasty. Note how well remodeled the knee appears. Due to leaving a small fragment of the patella behind, a new patella developed. The knee has excellent active range of motion

• Predistract and correct the knee flexion contracture prior to the knee ligament reconstruction to allow the knee to be held in full extension after the reconstruction. • Transfer the medial and lateral muscles to the anterior femur and attach to the shortened quadriceps. • Use allograft or half of the biceps tendon to create an interosseous ligament between the fibula and femur to prevent subluxation. • Even if active knee motion cannot ultimately be achieved, a mobile knee joint supported by a brace is preferable to a knee fusion.

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Fig. 25 (a, b) Clinical photos of the leg after one lengthening. Note the active knee flexion and extension

Fig. 26 AP (a) and lateral (b). The dysplastic hip was treated with femur and pelvic osteotomies. The talus failed to fuse in a plantigrade position. This was revised using a fibula-calcaneal plate. This plate will be removed to liberate the distal fibular physis. The foot is plantigrade.

The knee is stable and mobile. The brace serves to allow hypertrophy of the knee while the knee can flex and extend. The transferred hamstring tendons have replaced the absent quadriceps function

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Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a)

Cross-References ▶ Tibial Hemimelia ▶ Tibial Hemimelia Type II in a 6 Year Old Child Treated with Fibular Transfer and Centralization and Syme’s Amputation ▶ Type IV Tibial Dysplasia

References and Suggested Reading 1. Brown FW. The brown operation for total hemimelia tibia. In: Aitken GT, editor. Selected lower-limb anomalies. Washington, DC: National Academy of Sciences; 1971. p. 20–8. 2. Brown FW, Pohnert WH. Construction of a knee joint in meromelia tibia. A fifteen-year follow-up study. Abstract. J Bone Joint Surg Am. 1972;54:1333.

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3. Christini D, Levy EJ, Facanha FA, Kumar SJ. Fibular transfer for congenital absence of the tibia. J Pediatr Orthop. 1993;13(3):378–81. 4. Jones D, Barnes J, Lloyd-Roberts GC. Congenital aplasia and dysplasia of the tibia with intact fibula. Classification and management. J Bone Joint Surg (Br). 1978;60(1):31–9. 5. Paley D, Herzenberg JE, Gillespie R. Limb deficiency. In: Staheli LT, editor. Pediatric orthopaedic secrets. 2nd ed. Philadelphia: Hanley & Belfus; 2003. p. 406–16. 6. Weber M. A new knee arthroplasty versus Brown procedure in congenital total absence of the tibia: a preliminary report. J Pediatr Orthop B. 2002;11(1):53–9. 7. Weber M. Congenital leg deformities: tibial hemimelia. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: Informa Healthcare USA; 2007. 8. Weber M. New classification and score for tibial hemimelia. J Child Orthop. 2008;2(3):169–75. https://doi.org/10.1007/s11832-0080081-5. Epub 2008 Mar 6

Tibial Hemimelia Type II in a 6 Year Old Child Treated with Fibular Transfer and Centralization and Syme’s Amputation

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Elisabeth Leblanc and Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 445 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 448 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 448 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 450 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 450

Abstract

Partial tibial deficiency can be successfully treated with reconstruction of the affected leg and Syme’s amputation. In this case, we describe a hybrid technique with knee reconstruction and Syme’s amputation in a 6 year old male with isolated type II tibial hemimelia (Jones Classification). The knee reconstruction was performed through a fibular transfer and centralization with Ilizarov fixation, associated with a Syme’s amputation. At a follow-up of 3 years, the patient was fully functional with a Syme’s prosthesis.

E. Leblanc (*) Division of Orthopaedics, University of Sherbrooke, Sherbrooke, QC, Canada e-mail: [email protected]

Brief Clinical History The patient initially presented at 5 years of age, 6 months after having being adopted. No past medical or family history were available at that time. He presented with an isolated short right lower extremity. On physical examination, upper limbs and spine were unremarkable, as well as the left lower extremity. On the right side, the hip was found to be stable and normal in terms of range of motion. The knee had good range of motion with strong quadriceps. There was an LLD of 8 cm, mostly coming from the right lower leg, and the right foot was in a rigid 30 equinovarus deformity.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_325

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Fig. 1 (a) and (b) showing the affected and normal legs

Preoperative Problem List – Tibial hemimelia type II with presence of a short proximal tibial stump – 8 cm LLD (expected at skeletal maturity to be 14.0 cms) – Rigid severe equinovarus deformity – Nonfunctional and severely deficient ankle joint

Treatment Strategy

Fig. 2 Showing X-ray of the affected leg with proximal migration of the fibula and proximal tibia stump with absence of the rest of the tibia and grossly abnormal ankle joint

Based on Jones’ classification, type II tibial hemimelia is a partial deficiency with an ossified proximal tibia and functional quadriceps. As per physical exam and initial X-rays, there was a clear reconstruction potential. Initial visit was important to discuss the treatment options with the parents. They were offered three options: knee reconstruction with fibular centralization and Syme’s amputation, knee and ankle reconstruction with multiple lengthenings, and the last option was knee disarticulation. As part of the pre-op assessment, a psychology consultation was arranged, and the family was also referred to the War Amputees to help them in their decision. After consideration of the three options, they decided to opt for the first option. The treatment plan was to perform the fibula centralization – transfer along with the Syme’s amputation in a single setting, with the use of a standard Ilizarov frame for stabilization of the fibular transfer.

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Tibial Hemimelia Type II in a 6 Year Old Child Treated with Fibular Transfer and Centralization . . .

Basic Principles – – – –

Preserve extension mechanism. Preserve proximal tibial growth. Preserve lateral collateral ligament. Promote good bone biology for healing – with cancellous bone grafting at the site of the transfer. – Preserve normal and stable alignment – with the use of an external fixator. – Solid fixation allowing early weight bearing. – Well padded and viable stump.

•

• •

Images During Treatment See Figs. 3 and 4.

Technical Pearls • Under tourniquet, first step is to prepare the fibular osteotomy. Through an oblique anteromedial incision at the level of the tibial stump and muscle retraction, sub-

Fig. 3 (a–d) Diagrammatic representation of the surgical steps. (a) Showing the proximal cut prior to transfer of the fibula. (b) Showing the transfer of the fibula and impaction in the tibial stump. (c) Showing application of an Ilizarov frame to stabilize the transfer, with the proximal ring fixed to the distal frame with hinges (not shown in the

• • • • •

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periosteal dissection of the proximal fibula is performed and a Vosteotomy of the fibula is performed at the level of the distal end of the tibial stump. Then, the Syme’s amputation is done following the standard technique with care to preserve the vascularity of the heel pad which is attached and sutured to the anterior compartment fascia. Calcaneus cancellous bone is kept for grafting at the site of the transfer. Fibular centralization transfer is then completed by impacting the V-shaped proximal end of the fibula into the distal tibial stump, grafting the site and completing fixation with an Ilizarov frame. One proximal 5/8 ring in the distal femur, one full ring in the tibial stump, and two full rings in the distal fibular stump were used. To correct the final alignment, fluoroscopy images were obtained. A prophylactic fasciotomy is done before closing. Weight bearing on the frame is allowed after 6 weeks. As it is very difficult to assess healing, frame removal should wait until at least 3 months after surgery. After frame removal, a cylinder cast is applied for 2 weeks and then prosthesis fitting is started.

diagram) at the knee joint to allow motion in that joint, the middle fragment is fixed to the proximal tibial stump, and the two distal rings are fixed to the transferred fibula. (d) Final result at 3 years follow-up, showing thickening of the transferred fibula and proximal tibiofibular synostosis

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Fig. 4 (a) and (b) showing the Ilizarov frame during the treatment. Universal joints were placed at the level of the knee joint, to allow for motion at the knee while, at the same time, give more stability to the proximal tibial segment. Universal joints were also placed between the middle ring (in the tibial stump) and the two distal rings, to allow for correction of any residual malalignment of the transferred fibula

Outcome Clinical Photos and Radiographs See Figs. 5, 6, and 7.

Avoiding and Managing Problems – The protruding proximal fibula seems to be a recurrent problem in the literature. Some authors recommend proximal fibula resection. However, long-term knee instability

could be a concern, and preservation of the lateral collateral ligament is important. Prosthetic adaptation and/or proximal fibula epiphysiodesis could address these problems. – Solid fixation with an Ilizarov frame and early weight bearing are important to promote good bone healing. – Good prosthetic fitting is the key to maintaining good function.

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Fig. 6 X-rays at 3 years follow-up

Fig. 5 X-ray of reconstructed knee after frame removal, 3 months after the initial surgery

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450 Fig. 7 Long-standing films at 3 years follow-up showing good alignment with a Syme’s prosthesis

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Cross-References ▶ Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot Equinus and LLD ▶ Tibial Hemimelia ▶ Tibial Hemimelia Paley Type 5a (Jones Type 1b) and Paley Type 5b (Jones Type 1a) ▶ Type IV Tibial Dysplasia

References and Suggested Reading 1. Birch JG. Lengthening for congenital lower limb deficiencies, Chapter 13. In: Hamdy RC, JJ MC, editors. Management of limb-length discrepancies. Monograph series (45). American Academy of Orthopaedic Surgeons; 2011. p. 95–107. 2. Carranza-Bencano A, Gonzalez-Rodriguez E. Unilateral tibial hemimelia with leg length inequality and varus foot: external fixator treatment. Foot Ankle Int. 1999;20(6):392–6. 3. Jones D, Barnes J, Lloyd-Roberts GC. Congenital aplasia and dysplasia of the tibia with intact fibula. Classification and management. J Bone Joint Surg. 1978;60(1):31–9. 4. Schoenecker PL, Capelli AM, Millar EA, et al. Congenital longitudinal deficiency of the tibia. J Bone Joint Surg Am. 1989;71(2):278–87.

Tibial Hemimelia: Staged Treatment with External Fixation and Then Internal Lengthening Nail

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Mark Eidelman and Pavel Kotlarsky

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 451 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 451 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 451 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 452 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 452 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 460

Abstract

Tibial Hemimelia is a relatively rare congenital preaxial longitudinal deficiency. The current case deals with Jones type 4 deformity (also called ankle diastasis). Treatment options range from amputation to tibiotalar arthrodesis with subsequent lengthening procedures. We present a patient with tibial hemimelia Jones type 4 treated by ankle-sparing reconstruction with serial tibial and fibular lengthening procedures.

Brief Clinical History The patient was born in 2001 with a short right leg and a clubfoot-like foot appearance. He was initially examined in our institution at the age of 18 months. The limb length discrepancy (LLD) then was 5 cm, and the foot was in rigid equinovarus position, not correctable by passive stretching.

On X-rays, a clear ankle diastasis was seen, and the fibula had grown to be significantly longer than the tibia. Projected LLD at maturity calculated using the Multiplier method was 16 cm.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List • Rigid equinovarus foot • Fibular overgrowth (fibula longer than tibia) • Limb length discrepancy (current 5 cm, projected 16 cm)

M. Eidelman (*) · P. Kotlarsky Ruth Rappoport Children’s Hospital, Rambam Healthcare Campus, Haifa, Israel © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_391

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Treatment Strategy There were three main goals in the treatment of this patient: first, achieving a plantigrade shoe-able foot by correction of equinovarus deformity; second, preservation of a reasonable ankle motion by keeping the diastasis and maintaining a proper tibia to fibula relationship around the ankle; and third, equalization of the leg lengths by sequential lengthening procedures and contralateral epiphysiodesis – a total LLD of 13 cm was treated by three lengthening procedures: first two using a circular external fixator and the third using an internal tibial lengthening device, with the rest treated by contralateral epiphysiodesis.

Basic Principles

Fig. 1 Initial clinical appearance of the leg and foot at the age of 18 months (right side affected)

The goals of the first surgery were to create a plantigrade and shoe-able foot and to achieve correct tibiofibular alignment by lengthening the tibia and shortening the fibula. These goals were achieved by percutaneous Achilles tendon lengthening, application of the Ilizarov apparatus with simultaneous correction of the foot alignment, resection of

Fig. 2 a-b. Initial radiographic appearance of the leg and foot: (a) Anterior-posterior (AP) view; (b) lateral view. Ankle diastasis and fibular overgrowth are seen

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25 mm of the fibula, temporary wire fixation between the proximal tibia and fibula, and 4 cm gradual tibial lengthening. Total 2 years later, the fibula had grown significantly longer again with a 5 cm LLD. At this stage, lengthening was performed using the Taylor Spatial Frame ® (TSF, Trademark of Smith & Nephew, US Pat). First, proximal tibial osteotomy and gradual lengthening was performed, and when the length of the tibia achieved fibular length, a midshaft fibular osteotomy was performed with fixation of the fibula to the tibia. A total of 5 cm lengthening was achieved. At the age of 12 years, contralateral epiphysiodesis of the distal femur and proximal tibia were performed. At the age of 16 years, the relationship between the tibia and the fibula was preserved, and the total LLD was 40 mm. Since most of the proximal tibia was completely straight, the final lengthening procedure was performed using the Precice ® magnetic nail (Nuvasive, San Diego, CA, USA). To prevent dissociation between the tibia and the fibula, a transverse screw was inserted above the ankle joint between the two bones.

Images During Treatment See Figs. 3, 4, 5, 6, 7, 8, and 9.

Technical Pearls Foot reconstruction can be performed by a combination of soft tissue procedures (commonly Achilles tendon lengthening) and a circular external fixator that allows for both soft tissue distraction as well as lengthening. Maintenance of the tibia to fibula relationship is crucial for the success of treatment. This can be achieved by any combination of – tibial lengthening, fibular shortening, and epiphysiodesis. When a proper tibiofibular relationship is achieved, if lengthening is

453

performed, a fixation between the tibia and the fibula is of utmost importance as was demonstrated throughout this case at every lengthening step. Staged lengthening procedures allow for correction of the leg length discrepancy and can be performed using a circular external fixator or an internal lengthening device. While internal lengthening is more convenient for the patient, it can be used only when the tibia is straight, and when there are no associated leg alignment deformities that need to be addressed at the time of lengthening.

Outcome Clinical Photos and Radiographs See Fig. 10.

Avoiding and Managing Problems There are alternative treatment options for tibial hemimelia Jones type 4 including amputations, tibiotalar arthrodesis with a subsequent tibial lengthening procedures, and foot reconstruction procedures. The importance of this case is to show a possibility for foot salvage and maintenance of ankle range of motion. The first step of foot salvage is achieving a well-balanced plantigrade foot. Only after that goal is achieved lengthening procedures can commence. We do not recommend lengthening of more than 5 cm for each lengthening step. Moreover, during each lengthening stage a close follow-up, both clinical and radiologic, for the knee and ankle function and range of motion is required, as any subluxation and/or associated loss in the range of motion may require halting the lengthening process. Physiotherapy is important to preserve motion of the knee and the ankle both during each lengthening stage as well as between them.

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Fig. 3 a-c. Leg and foot alignment after first surgery that included foot balancing, fibular shortening, and tibial lengthening (overall 4 cm lengthening): (a) Final frame configuration at the end of initial correction; (b) clinical appearance of the foot after frame removal showing a

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plantigrade foot; and (c) radiographic appearance of the leg showing restored tibia-fibular relationship. The patient was able to wear regular shoes

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Fig. 4 Total 2 years after the initial procedure, the fibula has grown significantly longer, and the LLD was 5 cm

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456 Fig. 5 Second lengthening procedure. After tibial length reached fibular length, fibular osteotomy was performed, and the fibula was fixed to the tibia. Overall, 5 cm lengthening was achieved

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Fig. 6 The radiographic appearance after the second lengthening. Note the restored tibia-fibular relationship with stable ankle

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Fig. 7 X-ray taken after contralateral growth plate closure at the age of 12 years. The left distal femoral epiphysiodesis was achieved using eight plates on both sides of the distal femoral growth plate, and epiphysiodesis of the left proximal tibia was performed by a percutaneous drilling and curettage technique

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Fig. 8 a-c. Clinical and radiographic appearance at the age of 16 years, before final lengthening procedure: (a) Clinical look of the feet; (b) AP X-ray with a 4 cm block to compensate for leg length discrepancy; and (c) lateral X-ray

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Fig. 9 a-b. Final lengthening using the intramedullary lengthening device, with both bone osteotomy: (a) AP X-ray view; (b) lateral view. Overall, 4 cm lengthening was performed

Fig. 10 a-e. Clinical and radiographic appearance at the age of 19 years: (a) Clinical picture showing prominence of medial malleolus and no limb length discrepancy; (b-c) AP X-ray views; and (d-e) lateral X-ray views. The patient has reasonable ankle motion (300 of

plantarflexion and 150 of dorsiflexion), a plantigrade foot, and prominence of the medial malleolus that does not interfere with any standard shoe wear. He reached skeletal maturity and had no leg length discrepancy

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References and Suggested Reading 1. Eidelman M, Kotlarsky P. Treatment of tibial hemimelia jones type 4 by ankle-sparing reconstruction (from birth to skeletal maturity). J Limb Lengthening Reconstr. 2021;7:52–6. 2. Weber M. New classification and score for tibial hemimelia. J Child Orthop. 2008;2(3):169–75.

M. Eidelman and P. Kotlarsky 3. Paley D. Tibial Hemimelia: new classification and reconstructive options. J Child Orthop. 2016;10(6):529–55. 4. Tokmakova K, Riddle EC, Kumar SJ. Type IV congenital deficiency of the tibia. J Pediatr Orthop. 2003;23(5):649–53. 5. Litrenta J, Young M, Birch JG, Oetgen ME. Congenital tibial deficiency. J Am Acad Orthop Surg. 2019;27(6):e268–79.

Tibial Lengthening Over a Free Vascularized Fibular Autograft After Ewing’s Sarcoma Resection

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Barak Rinat, Marc Isler, and Panagiotis (Peter) Glavas

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 461 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 463 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 465 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 467

Abstract

Pediatric malignant osseous tumors, such as Ewing’s sarcoma, can involve all parts of the growing skeleton. Surgical treatment of these tumors often mandates the resection of large bony segments which may result in significant bone loss and lead to limb length discrepancy (LLD) and/or deformity. We present a case of an 18-yearold male who was diagnosed with Ewing’s sarcoma of the proximal left tibia.After the initial workup, the patient underwent tumor resection and reconstruction with a tibial allograft and an ipsilateral free vascularized fibular autograft. Given the proximity of the physis to the resection, the patient developed a residual LLD of 44 mm. A subsequent fracture of the proximal tibial metaphysis resulted in a varus deformity. Deformity assessment also indicated valgus of the distal femur which was corrected acutely. A monolateral multiaxial external fixator was installed for both lengthening of the tibia and correction of the angular deformity. The regenerate bone healed uneventfully.

B. Rinat · M. Isler · P. P. Glavas (*) Sainte-Justine University Hospital Center, QC, Canada e-mail: [email protected]; [email protected]

There was no significant residual deformity or LLD. Follow-up examination showed satisfactory functional outcome.

Brief Clinical History The patient presented with left leg pain at 10 years of age. Initial x-rays (Fig. 1) raised suspicion for a bony malignancy hence he underwent an MRI (Fig. 2) followed by biopsy. He was diagnosed with a metaphyseal Ewing’s sarcoma. Systemic disease was ruled out with a whole body Tc99 bone scan. He received neo-adjuvant chemotherapy followed by wide resection. Limb salvage involved a 5 cm distal margin. Proximally, to preserve the joint line a negative margin was planned in the epiphysis. Reconstruction involved a freshfrozen, non-irradiated allograft for stability, combined with ipsilateral transfer of the fibula, including the tibial anterior arterial branch to the fibular epiphysis, to try and maintain growth potential. The tibial allograft included its extensor mechanism, which was sutured to the host tendon. The pedicled fibular autograft was placed in the medullary canal distally and inside the allograft. Stable fixation was obtained

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_379

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Fig. 1 X-ray of the left leg demonstrating a permeative bony lesion with ill-defined borders and periosteal reaction

distally. Temporary fixation of the allograft to the epiphysis of the native tibia was to be removed after chemotherapy to allow growth (Figs. 3 and 4). The patient then completed chemotherapy according to protocol. Approximately 1 year after resection, the proximal tibial plates were removed. Unfortunately, a mildly displaced metaphyseal fracture occurred at the grafted site. Conservative treatment ensued which resulted in union, but with residual varus deformity of the proximal tibia. At skeletal maturity, the patient had a significant LLD as well as a varus deformity of the proximal tibia.

Preoperative Clinical Photos and Radiographs Figures 5, 6, 7: Preoperative EOS images (EOS Imaging, France) demonstrated left genu-varum deformity, medially directed joint line obliquity [1] and a mechanical axis deviation at the edge of zone 2 [2]. The mMPTA (mechanical medial proximal tibial angle) measured 70
and the mLDFA (mechanical lateral distal femoral angle) 81
. The LLD measured 44 mm of which 39 mm derived from the tibia. No sagittal malalignment was noted.

Fig. 2 MRI of both legs demonstrating a left tibial meta-diaphyseal lesion and a periosteal mass

Preoperative Problem List – – – –

Tibia varus (mMPTA 70
) Femur valgus (mLDFA 81
) Oblique joint line of the knee LLD of 44 mm

Treatment Strategy The treatment goals are to restore length and correct angular deformity. For the tibial angular deformity and shortening, a metaphyseal tibial osteotomy was performed at the determined apex. The cut was made through the previously grafted site using a Gigli saw. A MAC hybrid external fixator (Biomet Multi-Axial Correction (MAC) Fixation System, Biomet Orthopedics, Indiana, USA) was mounted on the tibia with 3 proximal hydroxyapatite coated Schanz pins (Biomet) and 3 distal pins. To correct the joint obliquity and distal femur valgus, a lateral open wedge osteotomy of

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Figs. 3 and 4 X-ray images of the tibia (antero-posterior and lateral projections) after tumor resection and intercalary tibial allograft and fibular free vascularised autograft implantation as well as extensor mechanism re-attachment using a bone anchor and proximal and distal rigid fixation

6
was performed and stabilized using a 4.5 mm ContourLock plate (Arthrex, Florida, USA) and a tricalcium phosphate bone graft (Figs. 8, 9, and 10). The sequence of correction for this type of fixator involves an initial period of lengthening, followed by angular correction and then further lengthening. Serial follow-ups showed that after achievement of 37 mm of distraction in combination with coronal plane deformity correction in the femur and tibia, the overall limb lengths were symmetrical (Figs. 11 and 12). The external fixator was removed 6 months after the surgery when complete healing of the regenerate was observed.

Basic Principles

Fig. 5 EOS image of the lower limbs demonstrating genu-varum deformity as well as LLD

The patient described in this case underwent a Ewing’s sarcoma resection at the proximal left tibia and an intercalary combination of allograft and autograft. Although, this technique allows for the preservation of the joint surfaces, the resection of the physis may lead to secondary deformity and shortening. In addition, the grafted site is prone to fracturing which may lead to further deformity. Therefore, when performing this technique, one should be able to foresee and manage the sequelae that may arise. A standard malalignment assessment performed on a standing radiograph, with patellae pointing forward and with block correction of the LLD should be done routinely [2]. In our patient, this assessment revealed a residual LLD as well as coronal malalignment in both the femur and tibia. As expected, the apex of the tibial deformity was directly within

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Figs. 6 and 7 Pre-operative x-rays (antero-posterior and lateral) of the tibia demonstrating the coronal angulation as well as normal posterior proximal tibial angle (PPTA ¼ 80˚)

Fig. 8 AP view of tibia after mounting the MAC external fixator

Fig. 9 Lateral view of the tibia osteotomy through the intercalary graft

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Fig. 11 At the end of the lengthening period. A 37mm gap between the osteotomised parts Fig. 10 AP view of the lower limbs at 10 days post-operatively

the intercalary graft. Lengthening of a short limb after a limb salvage procedure is documented [3, 4, 5], but to our knowledge, there have been only a few reports in the literature of lengthening and deformity correction directly over the grafted site. Ilizarov and Rozbruch [5] have described a somewhat similar case, but of a humerus. Performing the osteotomy as close as possible to the apex of the deformity will correct the deformity with minimal secondary translation. A latency period before distraction allows for all the growth factors to accumulate at the osteotomy site. With this type of fixator, a short course of lengthening should be done prior to angular correction followed by lengthening at a rate of 0.75 mm per day. Rotational correction is usually done at the end of distraction, as needed. Joint line obliquity needs to be addressed and osteotomies performed accordingly. Weekly serial visits monitor for bone regenerate formation as well as any change in clinical and neurologic status.

A consolidation period of two times the lengthening phase was allowed. At 6 months post-operatively, the fixator was removed (Figs. 13, 14, and 15).

Technical Pearls At the time of initial resection, foresee any possible deformities and inform the patient and the family. Meticulous preoperative planning on standing x-rays should always be done. With a MAC fixator, a short period of lengthening of about 1 cm will allow for enough distraction to prevent any impingement of the bony fragments with angular correction. If possible, the orthopedic oncologist who performed the initial tumor resection should assist with the dissection.

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Fig. 12 Lateral view of a healthy-appearing regenerate before external fixator removal

Fig. 13 Standing AP view of the final coronal alignment and length

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Avoiding and Managing Problems Before embarking on a reconstructive procedure on an intercalary graft, one should confirm that the proximal and distal docking sites are healed. A CT scan can be performed to document healing. In addition, look for signs of graft hypertrophy as an indication that the entire graft is re-vascularized and growing. Always respect limb lengthening and deformity correction principals.

References and Suggested Reading 1. Babis GC, An KN, Chao EY, Rand JA, Sim FH. Double level osteotomy of the knee: a method to retain joint-line obliquity. Clinical results. J Bone Joint Surg Am. 2002;84(8):1380–8. 2. Paley D, Tetsworth K. Mechanical axis deviation of the lower limbs. Preoperative planning of uniapical angular deformities of the tibia or femur. Clin Orthop Relat Res. 1992;280:48–64. 3. Accadbled F, Thévenin Lemoine C, Poinsot E, Baron Trocellier T, Dauzere F, Sales de Gauzy J. Bone reconstruction after malignant tumour resection using a motorized lengthening intramedullary nail in adolescents: preliminary results. J Child Orthop. 2019;13(3):324–9. 4. Yang Z, Tao H, Ye Z, Jin L, Lin N, Yang D. Bone transport for reconstruction of large bone defects after tibial tumor resection: a report of five cases. J Int Med Res. 2018;46(8):3219–25. 5. Tsuchiya H, Tomita K, Minematsu K, Mori Y, Asada N, Kitano S. Limb salvage using distraction osteogenesis. A classification of the technique. J Bone Joint Surg Br. 1997;79(3):403–11. 6. Ilizarov S, Blyakher A, Rozbruch SR. Lengthening of a free fibular graft after sarcoma resection of the humerus. Clin Orthop Relat Res. 2007;457:242–6.

Figs. 14 and 15 AP and Lateral views of the tibia after removal of the external fixator

Treatment of Recurrent Patellar Dislocation Using Langenskiold Reconstruction of the Patella, Grammont Patellar Slide, and Percutaneous Distal Femur and Distal Tibia/Fibula Osteotomies

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Raymond W. Liu

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 469 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 469 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 470 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 470 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 471 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 471 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 476 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 478

Abstract

Patellofemoral instability in the younger child can be challenging. Patients generally have an underlying condition, and treatment with the Langenskiold and Grammont procedures can be successful. Potential associated deformities including femoral anteversion, distal femoral valgus, patella alta, proximal tibial valgus, and external tibial torsion should all be evaluated and treated.

progressed to the point where she would not run with her friends due to concerns of dislocation. Reconstruction of her right side was performed and a similar staged procedure was recommended on the left. However, after correction of the right side her function was restored and so the family initially chose to observe the left. At age 9 years the child began to have dislocations on the left side, sometimes just with standing, and inability to run. At this point the family did choose to proceed with correction the left side.

Brief Clinical History

Preoperative Clinical Photos and Radiographs

A 6-year-old female child with chromosome 2q37.3 deletion syndrome and previous treatment for bilateral congenital vertical talus presented with right patellofemoral dislocation. This was initially treated with a patellar stabilizing brace and physical therapy with recurrent dislocations, and the child

On evaluation, she had marked laxity of the left patella, elevated Q angle, prone hip internal rotation of 90o (Fig. 1), prone hip external rotation of 15 , external thigh foot angle of 70 (Fig. 2), and external thigh malleolar axis of 80 . An AP hips to ankles radiograph demonstrated no notable coronal plane deformity (Fig. 3), a lateral knee radiograph noted patellar position within normal limits (Fig. 4), and a sunrise patella view showed trochlear dysplasia (Fig. 5).

R. W. Liu (*) Case Western Reserve School of Medicine, Rainbow Babies and Children’s Hospital, Cleveland, OH, USA e-mail: [email protected]

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_390

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Fig. 1 Increased prone left hip internal rotation at age 9 years, nearly three years after correction of the right side

Fig. 3 Standing bilateral AP hips to ankles radiograph at 9 years, showing adequate healing of the right side and coronal plane alignment of both sides. The apparently different proximal tibia-fibula relationship on the right is likely due to the derotational osteotomies as well as difference in stance position, with the patella centered on the right and medial on the left

Treatment Strategy

Fig. 2 Increased prone left thigh foot angle at age 9 years. It is important to avoid any stress at the knee or ankle when assessing this, and concurrent use of thigh malleolar axis is useful to bypass the ankle and foot, particularly with the history of congenital vertical talus

Preoperative Problem List 1. 2. 3. 4.

Left recurrent patellar dislocation with patellar laxity Left femoral anteversion Left external tibial torsion Left elevated Q angle

The Langenskiold procedure allows for reconstruction of patella with a large lateral release, placement of the patella into a new synovial pocket, and medial imbrication. The Grammont procedure allows for a slide of the patella tendon to medialize its insertion without need for osteotomy, and is an option in a child with an open proximal tibial physis. Percutaneous osteotomies of the distal femur and distal tibia/fibula allow for correction of her rotational deformities with the advantage of being able to subsequently remove the implants in clinic.

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Fig. 4 Lateral knee radiograph at 9 years, without any notable patella alta

Fig. 6 Intraoperative placement of 2.0 mm Steinman pins to judge rotational correction in the femur

3. The Grammont procedure is an option for a patient with any significant growth remaining in the proximal tibial physis, while tibial turbercle osteotomy is contraindicated unless a patient closer to maturity has proximal tibial procurvatum where a purposeful anterior partial growth arrest might be preferable. 4. Percutaneous osteotomies can be used in younger children, with the advantages of a quicker procedure with no permanent implants necessitating repeat surgery for removal. Fig. 5 Sunrise patella radiograph at 9 years, showing trochlear dysplasia

Images During Treatment Basic Principles 1. When treating patellofemoral instability all associated deformities should be considered for correction, including: femoral anteversion, distal femoral valgus, patella alta, an elevated Q angle, proximal tibial valgus, and external tibial torsion. This comprehensive approach is important because certain parameters such as ligamentous laxity and trochlear dysplasia are difficult to treat. 2. Although the Langenskiold procedure was initially described for fixed congenital patellar dislocation [1], it is useful for recurrent patellar dislocation in the young child as it allows excellent visualization, control of extensor mechanism positioning, and large medial imbrication when reconstructing the patella.

The femoral osteotomy was performed first because of its expected effect on the Q angle. We placed 2.0 mm guidewires in the proximal and distal femur in order to approximate 35 of planned rotational correction (Fig. 6). We then placed 2.4 mm Steinman pins laterally from proximal and distal to the planned osteotomy site, with an attempt to maximize the eventual spread between these pins when the osteotomy is eventually pinned. A 3.2 mm drill bit was then used to make multiple medial and lateral holes in the femur at the planned osteotomy site (Fig. 7), and a half inch osteotome used to complete the percutaneous osteotomy. The femur was derotated using the 2.0 mm wires as a guide, and the two 2.4 mm pins advanced to fix the osteotomy (Figs. 8 and 9). Using a sterile tourniquet with gravity elevation for exsanguination, we then made an incision beginning just lateral to

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Fig. 9 Lateral view showing cross pinning across the femoral osteotomy

Fig. 7 After placement of the 2.4 mm Steinman pins the osteotomy is started with the 3.2 mm drill bit here, followed by an osteotome to complete

Fig. 8 AP view showing cross pinning across the femoral osteotomy

Fig. 10 Incision for the Langenskiold/Grammont procedures, crossing the knee at the level of the patellar tendon

the proximal aspect of the patella, curving across the patellar tendon, and then extending down the medial tibial crest (Fig. 10). The extensor mechanism was exposed, with an elevated Q angle with the patella tendon oriented toward

the lateral aspect of the proximal tibia (Fig. 11). The medial and lateral capsule and retinaculum were released with care to try to preserve the synovium. This is typically done to the point where only the quadriceps tendon, patellar tendon, and

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Fig. 13 Intraoperative image from a different patient showing the synovial defect corresponding the previous patellar position has been closed with absorbable suture. After distal realignment, the patella now rests in a different position within the synovium

Fig. 11 Exposure of the extensor mechanism, demonstrating the increased Q angle with lateral position of the tibial tubercle

Fig. 12 Intraoperative image from a different patient demonstrates incision of the lateral capsule and retinaculum sparing the underlying synovium. The synovium is separately released from its attachment to the patellar circumferentially

synovium are attached to the patella (Fig. 12). In general, the synovium is then fully dissected from the patella, the resultant synovial defect is closed (Fig. 13), and after distal realignment is complete a new hole is cut into the synovium more medially to accommodate the new patellar position (Fig. 14). Then the synovium is repaired to the patella. Overall, this assures that the synovium accommodates the new patellar position, as with these steps the patella is better held in the proper position. In this patient, the synovium was

Fig. 14 Intraoperative image from a different patient showing creation of a new hole within the synovium, made directly over the center of the trochlea. The patella will now be placed into this new more medial hole and the synovium sutured to it circumferentially. Note the thickness of the synovium, which supports the structural importance of placing the patella within this new position in the synovium

abnormally thin and we were not able to separate it from the capsule. For the Grammont procedure the patellar tendon was elevated off the tibial crest from medial, extending laterally to Gerdy’s tubercle. The distal attachment was left intact. If necessary the anterior compartment can be elevated to facilitate medial translation of the patellar tendon, or the lateral

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Fig. 15 Returning to the current patient, with nonabsorbable sutures placed using pants over vest technique to substantially medialize the patellar tendon insertion. It is important to check patellar tracking with the tourniquet released

aspect of the patellar tendon can be released, depending on where the tightness is noted intraoperatively. The tendon was then medialized such that the extension mechanism was in just slight valgus, typically 15–20 mm. Testing of patellar tracking was done with the tourniquet released. In younger children the patellar tendon can be sutured down to the medial tibial periosteum which is typically quite robust (Fig. 15). In older children closer to or after skeletal maturity suture anchors may be necessary. The knee should be ranged after tying down the sutures, and adjustments made based on patellar tracking. The large sleeve of medial capsule and retinaculum was then sewn to the patella to serve as the medial imbrication (Fig. 16), and even in children with substantial ligamentous laxity the hypermobility of the patella should be substantially improved. The fibula was then exposed with a small incision, and separated with a drill bit followed by an osteotome (Fig. 17). Proximal and distal 2.4 mm wires were placed in the tibia medially in preparation for the osteotomy, followed by a percutaneous osteotomy with a 2.5 mm drill bit and osteotome (Fig. 18). Correction was judged based on intraoperative thigh foot angle and thigh malleolar axis assessment, and the bone cross-pinned (Figs. 19 and 20). A drain was placed in the knee incision and wounds were closed, followed by a long leg splint.

R. W. Liu

Fig. 16 The clamp helps delineate the large medial capsular sleeve that was repaired to the anterior aspect of the patella. It is possible to overtighten which would lead to medial tracking of the patella

Fig. 17 Confirmation that the distal fibula osteotomy is complete

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Fig. 18 Passage of a 2.5 mm drill bit to begin the osteotomy at the distal tibia

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Fig. 20 Lateral view showing cross pinning across the tibial osteotomy

Technical Pearls 1. Femoral rotational correction requires the use of guide pins or intraoperative radiographic assessment. Tibial rotational correction can be judged clinically. 2. The Langenskiold exposure frees up all soft tissue restraints including the synovium, and when combined with the Grammont procedure distally allows one to precisely position the extension mechanism into an ideal position for patellar tracking. This should be checked multiple times intraoperatively after releasing the tourniquet, and adjusted as necessary.

Outcome Clinical Photos and Radiographs

Fig. 19 AP view showing cross pinning across the tibial osteotomy

The left side was recently corrected at the time of writing. For the previous right side the splint was overwrapped into a long leg cast at 2 weeks. At 6 weeks there was adequate healing to remove the pins and allow the child to weight bear in a knee immobilizer and walking boot (Figs. 21, 22, 23 and 24). At 10 weeks she had adequate healing to wean from both braces (Figs. 25, 26, 27 and 28).

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Fig. 21 The remaining images are all follow up images from the previously done right side. AP knee xray at 6 weeks showing adequate healing to remove the Steinman pins in clinic

Avoiding and Managing Problems 1. Adequate pin fixation is important particularly in the femur, which is only partially protected by the long leg splint. It is important that the pins do not cross at the level of the osteotomy. A third pin can be added if any concern, and images of the distal femur were rechecked at the end of the procedure to ensure it remained stable with the manipulations necessary to correct the tibia. 2. Soft tissue issues with the large knee incision can be minimized by avoiding sharp retraction on the skin, and instead using gloved fingers for retraction as necessary. 3. Proper positioning for the Grammont procedure can be checked by holding the patellar tendon in the desired position with a clamp and ranging the knee. After placement of the first suture, position can be mildly adjusted with the second suture, while larger adjustments require removal of the first suture.

Fig. 22 Lateral knee xray at 6 weeks

Fig. 23 AP ankle xray at 6 weeks showing adequate healing to remove the Steinman pins in clinic

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Fig. 24 Lateral ankle xray at 6 weeks

Fig. 26 Lateral knee xray at 10 weeks

Fig. 25 AP knee xray at 10 weeks, showing adequate healing to wean from immobilization

Fig. 27 AP ankle xray at 10 weeks, showing adequate healing to wean from immobilization

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References 1. Langenskiöld A, Ritsilä V. Congenital dislocation of the patella and its operative treatment. J Pediatr Orthop. 1992;12(3):315–23.

Suggested Reading Langenskiöld A, Ritsilä V. Congenital dislocation of the patella and its operative treatment. J Pediatr Orthop. 1992; 12(3):315–23. Ramos O, Burke C, Lewis M, Morrison MJ, Paley D, Nelson SC. Modified Langenskiöld procedure for chronic, recurrent, and congenital patellar dislocation. J Child Orthop. 2020;14(4):318–29.

Fig. 28 Lateral ankle ankle at 10 weeks

Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD)

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J. Ivan Krajbich

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 480 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 480 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 481 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 484

Abstract

We present a case of a 3 year old, otherwise healthy, child born with proximal femoral focal deficiency (PFFD) Aitken type B. The child has severe leg length discrepancy with the foot on the affected side at level just distal to the contralateral knee. The hip was dysplastic with large ossification defect of the proximal femur and marked varus alignment. Her knee was also dysplastic with significant flexion contracture. She was felt not to be a candidate for leg equalization procedures, and since she had relatively normal ankle and foot, modified Van Nes rotationplasty was recommended as the best treatment option. Her treatment consisted firstly of modified Van Nes rotationplasty followed by hip reconstruction osteotomy as staged procedures 1 year apart. She has become functional rotationplasty prosthesis user with below the knee amputation-like function and a stable hip.

Brief Clinical History A female child was born at term by vaginal delivery with low birth weight and diagnosis of right-sided PFFD. Treatment started at age 10 months using simple extension prosthesis to allow her to stand and walk. At age 3, she was referred to our institution for additional evaluation and possible treatment. She was found to be an otherwise healthy female child with right-sided Aitken type B PFFD, ambulating with the help of extension prosthesis. She had abduction flexion contracture in her right hip, flexion contracture in the right knee, full range of motion in her ankle, and normal five-ray foot.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

J. I. Krajbich (*) Shriners Hospitals for Children, Portland, OR, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_342

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Fig. 1 (a, b): Presenting radiographs of a 3 year old female with right-sided PFFD, Aitken classification type B [1, 3] with very short femoral segment, abnormal knee joint, and dysplastic hip joint with ossification defect in intertrochanteric and cervical aspect of the proximal femur and marked varus alignment

Preoperative Problem List 1. Marked leg length discrepancy 2. Dysplastic hip with marked proximal femoral varus deformity with only minimal ossification of the intertrochanteric and cervical area of the proximal femur 3. Knee flexion contracture

Treatment Strategy Two treatment options presented to the family were (a) Syme’s amputation and knee fusion with through-theknee amputation-like function and (b) modified Van Nes rotationplasty combined with knee fusion and potential hip reconstruction. The second option of modified Van Nes rotationplasty [7] addresses majority of the limb problem issues. It allows for restoration of the mechanical axis of the thigh, with one solid, straight thigh bone. It establishes a biological knee joint-like function. It also facilitates hip reconstruction at a later date [1, 4, 6]. The recommended treatment strategy was therefore modified Van Nes rotationplasty combined with a knee fusion. Hip reconstruction osteotomy was planned as a second stage to allow for restoration of normal hip biomechanics and at the same time, fine-tune the length and rotation of the new established thigh. Fig. 2 Coronal (a) and axial (b) MRI cut of the proximal femur. Cartilage enlargement of the proximal femur with base of the neck defect is demonstrated

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Van Nes Rotationplasty and Hip Reconstruction in the Treatment of Proximal Femoral Focal Deficiency (PFFD)

Basic Principles

Images During Treatment

Importance of normal or near-normal knee function for functional energy efficient gait cannot be overstated [5, 8]. In the treatment of severe congenital or acquired lower limb deficiency, the basic principles need to be followed. They can be stated in a point format:

See Figs. 3 and 4.

(a) Establishment of a stable hip with no contractures, good range of motion, and optimal muscle strength particularly in extension and abduction (b) Normal or near-normal biomechanical axis of the limb (c) Stable, functional knee joint with optimal muscle strength particularly in extension (d) Equal or near-equal leg length (e) Stable, non-painful ankle/foot complex If these objectives are not present in the native limb, they can be accomplished by surgical means, prosthetic means, orthotic means, or a combination of these. In PFFD, Aitken type B and more severely involved Aitken type A, the Van Nes rotationplasty combined with knee fusion hip reconstruction allows for the best chance to accomplish these objectives by a combination of surgical intervention and prosthetic fitting. In the Aitken types C and D deficiency, rotationplasty alone with knee fusion minus the hip reconstruction is employed. The rotationplasty establishes normal mechanical alignment of the thigh, converts ankle to a functional biological knee joint, and allows for prosthetic restoration of the ankle/foot complex and limb length. Aitken types A and B’s surgical reconstruction of the stable, functional hip joint is frequently possible, and in our hands best done as a second stage in the overall limb reconstruction. In C and D, ischial weight-bearing socket for a prosthesis is usually the only viable alternative for hip stability.

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Technical Pearls The goal of the operation is a straight solid thigh with ankle rotated 180 into the functional plane of the normal knee joint at the level of the contralateral knee. The operation, as performed by our team, accomplishes all of these goals by excising the knee joint, usually removing both distal, femoral, and proximal tibial physis and obtaining all of the rotation through the knee fusion in the existent knee site. This necessitates adequate mobilization of the neurovascular structures and detachment or division of all muscular and tendinous structures crossing the knee to allow for adequate rotation without neurovascular compromise. This decreases chances of later derotation. In cases where complete 180 rotation cannot be accomplished through the knee fusion (in very short or near-absent femoral segment), secondary osteotomy further down the tibia may be required.

Outcome Clinical Photos and Radiographs See Figs. 5, 6, 7, 8, 9, and 10.

Avoiding and Managing Problems Key to successful outcome is an appropriate patient selection, attention to technical detail during surgery, and adequate access to rehabilitation and prosthetic services. Relatively normal ankle/foot complex is a prerequisite for a successful outcome. Patients with higher-grade fibular deficiency with

Fig. 3 (a) Intraoperative picture of the abnormal knee to be excised. (b) Knee excision with intact neurovascular bundle – arrow

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Fig. 4 Intraoperative fluoro image of completed rotationplasty

Fig. 6 Healed rotationplasty limb radiographs prior to hip reconstruction

Fig. 5 Immediate postoperative limb appearance

inadequate ankle and less than a four-ray foot will unlikely obtain a benefit from this procedure. The major potential complication of the surgical procedure itself is a compromise of the neurovascular structures. This is particularly so in terms of the adequate vascular supply. The peripheral pulses either palpable or clearly detectable by Doppler must be present before the child has woken up from the anesthesia. This occasionally requires additional dissection and mobilization of the femoral vessels or even easing up on a degree rotation. Any loss of pulses in immediate postoperative period necessitates urgent return to the operating room to decompress the vessels and reestablish adequate circulation.

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Fig. 7 Anterior (a) and posterior (b) views of a 3D CT of R hip prior to surgical intervention

Fig. 8 Intraoperative radiograph of the hip reconstruction by shortening valgus osteotomy

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Fig. 9 (a, b) Healed osteotomy radiographs

Fig. 10 (a, b) Clinical images of rotationplasty with rotationplasty prosthesis limb restoration

References and Suggested Reading 1. Ackman J, Altiok H, Flanagan A, Peer M, Graf A, Krzak J, Hassani S, Eastwood D, Harris GF. Long-term follow-up of Van Nes rotationplasty in patients with congenital proximal focal femoral deficiency. Bone Joint J. 2013;95-B:192–8. 2. Aitken GT. Proximal femoral focal deficiency: definition, classification and management. In: Aitken GT, editor. Proximal femoral focal deficiency: a congenital anomaly. Washington, DC: National Academy of Sciences; 1968. p. 1–22. 3. Gillespie R, Torode IP. Classification and management of congenital abnormalities of the femur. J Bone Joint Surg (Br). 1983;65:557–68.

4. Krajbich JI. Van Nes rotationplasty. In: Hamdy R, McCarthy J, editors. Management of limb-length discrepancies. Rosemont: American Academy of Orthopaedic Surgeons; 2011. p. 77–85. 5. McClenaghan BA, Krajbich JI, Pirone AM, Koheil R, Longmuir P. Comparative assessment of gain after limb-salvage procedures. J Bone Joint Surg Am. 1989;71:1178–82. 6. Torode IP, Gillepsie R. Rotationplasty of the lower limb for congenital defects of the femur. J Bone Joint Surg (Br). 1983;65:569–73. 7. Van Nes CP. Rotationplasty for congenital defects of the femur. Making use of the ankle of the shortened limb to control the knee joint of a prosthesis. J Bone Joint Surg (Br). 1950;32:12–6. 8. Waters RL, Perry J, Antonelli D, Hislop H. Energy cost of walking of amputees: the influence of level of amputation. J Bone Joint Surg Am. 1976;58:42–6.

Delayed Regenerate Bone Formation in a Seven Year Old Boy with Fibular Hemimelia Undergoing Tibial Lengthening

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Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 485 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 485 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 486 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 486 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 486 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 486 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 486 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 490 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 490

Abstract

Brief Clinical History

This is a case of an 11 year old boy with Fibular hemimelia Type IA who had been followed for 4 years. He then underwent tibial lengthening with a standard Ilizarov frame. He developed poor regenerate bone formation that was addressed with the accordion technique, and targeted lengthening was reached. However, 2 weeks after removal of the fixator, he sustained a fracture of the regenerate that was treated with elastic nailing. He subsequently underwent distal medial hemi-epiphysiodesis of the femur on the affected side. At the latest follow-up, he had an LLD of 1.5 cm and satisfactory alignment of his lower limbs.

A 7 year old boy presented for the management of limb length discrepancy. He had a negative past medical history and no symptoms. Clinical examination revealed an LLD of 4.0 cms, equally distributed between the leg and the thigh, anterior knee joint laxity and an absent 5th ray in the foot. Plain X-rays confirmed the diagnosis of fibular hemimelia Type 1 (Kalamchi classification) with a congenital short femur (Type I A, Paley classification). The hips were normal clinically and radiologically. Expected LLD at skeletal maturity calculated by the multiplier method was 4
1.56 ¼ 6.2 cms

Preoperative Clinical Photos and Radiographs See Fig. 1. R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_332

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skeletal maturity or (2) if he becomes dysfunctional prior to maturity, to proceed with one lengthening of about 20 % the length of the bone and then address the remaining LLD by a contralateral epiphysiodesis if necessary. At the age of 11 years, he underwent tibial lengthening with a targeted lengthening of 4.5 cms.

Basic Principles

Fig. 1 X-ray showing congenital short femur and fibular hemimelia

Preoperative Problem List – Congenital fibular deficiency, Type 1 with associated mild congenital short femur – LLD of 4.0 cms, expected to be 6.2 at skeletal maturity (almost equally distributed between the tibia and the femur) – Unstable knee (positive Lachman test) – Mild valgus of the distal femur

Treatment Strategy Since the child was completely asymptomatic and functioning very well with a shoe lift of 3.0 cms at the time of the first visit, it was decided that no surgical treatment was necessary at that time. The patient was followed on a yearly basis, and the shoe lift was adjusted as necessary. The expected LLD at skeletal maturity was 6.0 cms. The plan was to address this LLD either by (1) a lengthening when the child reaches

– Lengthening in congenital deficiencies is very challenging and has a high incidence of complications. Hence, the treating surgeon should be aware of these complications and anticipate them. These should be discussed with the patient and family. – Develop a global plan to address all the problems based on the estimated LLD at skeletal maturity. – Start the lengthening when function of the patient is affected and the shoe lift is no longer tolerated. – Do not perform lengthening more than 20 %. – Always look for any signs of instability in the joint above and below the lengthened bone. – Try to avoid the development of poor regenerate by careful preoperative planning, attention to details intraoperatively, and monitoring the new bone formation in the distraction gap. Some new bone formation should appear radiologically within 3 weeks after the start of lengthening. – Once poor regenerate develops, necessary measures should be implemented as discussed below. – Prophylactic intramedullary fixation of the lengthened bone should be considered whenever poor regenerate develops. – The distal femoral valgus could be addressed with an eight-plate hemi-epiphysiodesis.

Images During Treatment See Figs. 2, 3, 4, 5, 6, and 7.

Technical Pearls – Low-energy osteotomy (gigli saw or multiple drill holes joined with osteotome). – Careful monitoring of the regenerate (weekly during the distraction phase). – If any suspicion of delayed or poor regenerate bone formation exists, do not hesitate to slow down the distraction or apply other measures as discussed below.

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Fig. 2 (a and b) Standard Ilizarov frame applied to the tibia, the proximal construct consists of a 5/8 ring and a full ring, the distal construct consists of 2 full rings. Half pins were used, including fixation of the fibula

Fig. 3 (a and b) X-rays showing minimal bone formation in the distraction gap. Accordion technique started in the form of alternating cycles of distraction, compression, and distraction daily

Fig. 4 Four weeks after initiation of the accordion technique, new bone formation in the distraction gap starts to appear

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Fig. 5 X-ray 10 weeks after initiation of accordion technique, new bone formation starts to bridge the distraction gap posteriorly. Targeted lengthening of 4.5 cms was attained

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Fig. 7 (a and b) X-rays showing lengthened tibia post removal of the fixator. No pain on partial-weight bearing

Outcome Clinical Photos and Radiographs See Figs. 8, 9, 10, and 11.

Avoiding and Managing Problems

Fig. 6 (a and b) X-rays 8 months after start of lengthening showing deficient bone formation anteriorly but good bone bridging posteriorly, medially and laterally. It was decided to proceed with removal of the fixator

– Congenital fibular deficiency is not an isolated entity and has numerous associated conditions that should be identified before planning any surgical treatment (coxa vara, congenital short femur, instability of the knee due to deficient anterior cruciate ligament, atrophy of the lateral distal femoral condyle, unstable ankle joint, abnormal or absent tarsal bones and rays). – The key point of this case is how to avoid and manage problems with the regenerate, starting with careful preoperative assessment and intraoperative attention to details. • Careful monitoring of the regenerate by doing X-rays on a weekly basis during the distraction phase • Poor regenerate bone formation before targeted lengthening is reached can be addressed by – Stopping or slowing down the distraction rate – Encouraging weight bearing of the affected limb – Application of the Accordion technique: alternating the distraction with compression cycles

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Fig. 10 X-ray showing hemiepiphysiodesis of the medial distal femur for management of valgus deformity of the distal femur

Fig. 8 (a and b) X-rays 2 weeks after removal of the fixator, showing fracture in the middle of the regenerate

Fig. 9 X-ray showing elastic nailing of the lengthened tibia

(In this case, distraction/compression /distraction) – The use of LUPUS (Low-Pulsed Ultrasound) If, after all these measures are taken and still after 3–4 weeks, no bone appears, then it has to be decided whether to continue the distraction until targeted lengthening is reached and deal with the poor regenerate or stop the lengthening altogether. • Poor regenerate after targeted lengthening is reached can be addressed by several methods, the most popular being autogenous bone graft from the iliac crest. Other techniques include RIA (reamer irrigation aspiration), various bone graft substitutes, and bone marrow and/or platelet-rich plasma injections. In this particular case, prophylactic intra-medullary rodding probably was indicated as there was already poor bone formation.

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Cross-References ▶ Fibular Hemimelia: Paley Type 3 ▶ Guided Growth and Syme Amputation in a Thirteen-YearOld Boy with Type 2 Congenital Fibular Deficiency

References and Suggested Reading 1. Birch JG, Lincoln TL, Mack PW, Birch CM. Congenital fibular deficiency: a review of thirty years’ experience at one institution and a proposed classification system based on clinical deformity. J Bone Joint Surg Am. 2011;93(12):1144–51. 2. Crawford DA, Tompkins BJ, Baird GO, Caskey PM. The long-term function of the knee in patients with fibular hemimelia and anterior cruciate ligament deficiency. J Bone Joint Surg (Br). 2012;94(3): 328–33. 3. Hamdy RC, Makhdom AM, Saran N, Birch JG. Congenital fibular deficiency. J Am Acad Orthop Surg. 2014;22:246–55. 4. Makdom A, Hamdy R. The use of growth factors to accelerate bone formation in distraction osteogenesis. Tissue Eng Part B. 2013; 19(5):442–53. 5. Naudie D, Hamdy RC, Fassier F, Morin B, Duhaime M. Management of fibular hemimelia: amputation or limb-lengthening. J Bone Joint Surg. 1997;79(B):58–65. 6. Sabarhwal S. Enhancement of bone formation during distraction osteogenesis: pediatric applications. J Am Acad Orthop Surg. 2011;19(2):101–11. Review. 7. Saran N, Hamdy RC. The use of Dexa (dual energy X-Ray absorptiometry) in deciding when to remove the external fixator in children undergoing limb lengthening. Clin Orthop Relat Res. 2008; 466(12):2955–61. 8. Saran N, Hamdy RC. Monitoring of regenerate bone. In: Hamdy RC, McCarthy JJ, editors. Management of limb length discrepancies. Monograph. Rosemont: American Academy of Orthopaedic Surgeons; 2011, 2012. Fig. 11 (a and b) X-rays 6 months after insertion of the eight-plate, showing satisfactory alignment of the limb in both the coronal and sagittal planes. The patient is scheduled for removal of the eight-plate

Septic Destruction of the Hip and Significant LLD Treated by Pelvic Support Osteotomy and Femoral Lengthening

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Mikhail Samchukov, John Birch, and Alexander Cherkashin

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 491 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 491 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 491 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 492 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 492 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 492 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 494 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 494 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 495 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 499 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 499

Abstract

Thirteen year old male with septic destruction of the hip and significant LLD underwent two stages of treatment including (1) Ilizarov pelvic support osteotomy and femoral lengthening with limb realignment and (2) staged double-level femoral osteotomy with repeat femoral lengthening at skeletal maturity using TrueLok circular external fixation.

Trendelenburg gait, limited abduction of the femur, painful rotation, and mild genu valgum.

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List Brief Clinical History The patient is a 13 year old male with significant hip instability and progressive 6-cm LLD secondary to septic destruction of the hip (Fig. 1). His physical examination revealed

• • • • •

Unstable hip with limited abduction Associated 6-cm LLD Mild valgus deformity of the distal femur Structural changes of the proximal femur and acetabulum Skeletally immature patient requiring repeated limb lengthening at skeletal maturity

M. Samchukov (*) · J. Birch · A. Cherkashin Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_90

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Fig. 2 AP radiograph of the pelvis with maximal hip adduction illustrating desired position of the proximal femur relatively to new weightbearing surface on the pelvis for pelvic support

support reconstruction (Figs. 2 and 3) was done using overlapping tracings of the affected limb [1, 6].

Basic Principles

Fig. 1 Standing AP radiograph of lower extremities demonstrating defect of the proximal femur, pelvic obliquity, and LLD

Treatment Strategy Standard surgical procedures such as open reduction, hip fusion, or total joint arthroplasty may not be applicable in cases with septic hip destruction due to high risk of infection and structural changes in the proximal femur and acetabulum. Instead, a unique Ilizarov pelvic support osteotomy combined with reconstructive angular correction and lengthening of the limb can ameliorate Trendelenburg gait, restore knee alignment, and correct leg length discrepancy [5]. In this case, treatment strategy was based on two phases. The first phase included pelvic support osteotomy and femoral lengthening with limb realignment, followed by distal femoral osteotomy for correction of genu valgum and obtaining additional lengthen. The final phase occurred at skeletal maturity and included staged double-level femoral osteotomy for correction of the residual LLD. Preoperative planning of pelvic

The basic rationale for pelvic support osteotomy is to increase the weight-bearing surface between the periacetabular region of the pelvis and the residual upper femur and minimize excessive pelvic drop during single-limb stance. Ilizarov introduced complex (valgus-extensionderotation) inter- or subtrochanteric osteotomy with increased adduction of the proximal femoral fragment and proximal-medial translation of the distal fragment to form a new (or supplementary) pelvic support [2–4]. In addition, a second femoral osteotomy (usually in the upper/middle diaphysis) is performed, followed by lengthening and gradual angular reposition of the distal fragment to realign the lower limb under the “surrogate” hip with “normal orientation” of the knee relative to the new mechanical axis. In cases when secondary valgus deformity is present in the distal femur, distal metaphyseal femoral osteotomy is performed to further correct the knee orientation and achieve additional limb lengthening [6].

Images During Treatment See Figs. 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, and 15.

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Fig. 3 Diagram of the preoperative planning indicating (a) new mechanical axis drawn perpendicular to the transverse axis of the pelvis from the projected center of the pelvic support, the desired knee level and joint orientation, and the anatomical axis of the distal femoral fragment; (b) second diaphyseal osteotomy and the resultant tertiary fragment overlapped with the anatomical axis of projected distal femoral fragment; and (c) distal metaphyseal osteotomy for an additional limb lengthening along the new mechanical axis

Fig. 4 Intraoperative photograph illustrating position of external supports. Note that angle between the arches is opened laterally and posteriorly

Fig. 5 Intraoperative photograph immediately after proximal femoral osteotomy, acute manipulation of bone fragments, and their stabilization with TrueLok frame. Note parallel orientation of femoral arches with medial shifting of the distal arch

494

Fig. 6 Postoperative standing AP radiograph demonstrating reconstruction of the proximal femur with formation of pelvic support under the lower rim of residual acetabulum. The frame consisted of a proximal femoral arch secured to the proximal fragment by three half pins and midshaft femoral arch attached to the middle fragment by two half pins, connected through a “floating ring” to a distal femoral ring secured to the distal fragment by one wire and two half pins

Technical Pearls Due to the geometrical complexity of the reconstructed femur, all steps of preoperative planning are critically important. Those steps should include (1) location of the weightbearing surface on the pelvis utilized as new (or additional) area for pelvic support, (2) optimal position of the proximal fragment to maximize functional improvement of the hip abductor muscles, (3) proper level of proximal (pelvic support) osteotomy, (4) optimal position of the middle fragment to maximize the contact surface area between the

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Fig. 7 Clinical appearance of the legs after surgery. Note that the operated femur is in an abduction position, which will be gradually reduced in the postoperative period. A block to further adduction indicates the final position of the femur, requiring femoral shaft osteotomy followed by gradual lengthening and limb realignment

reconstructed upper femur and new weight-bearing surface on the pelvis, (5) optimal position of the distal fragment to allow normal orientation of the knee relative to new mechanical axis of the limb, (6) proper level of diaphyseal secondary osteotomy to allow cosmetically acceptable varus of the femoral midshaft, (7) final leg length inequality recognizing that pelvic support osteotomy and diaphyseal varus osteotomy will accentuate femoral shortening, and (8) presence of distal femoral deformity, which may require a third corrective osteotomy to complete the limb realignment.

Outcome Clinical Photos and Radiographs See Figs. 16 and 17.

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Fig. 8 AP radiograph during limb lengthening and realignment following midshaft femoral osteotomy. Note progressive consolidation between the proximal and middle fragments at the pelvic support osteotomy

Avoiding and Managing Problems The functionality of proximal (pelvic support) osteotomy directly correlates with the proper execution of distal (limb reconstruction) osteotomy. Although the latter osteotomy can be performed at the same time, we prefer to delay it until an adequate abduction position of the proximal fragment is established at the pelvic support (typically 3–4 weeks

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Fig. 9 Clinical appearance of lower extremities during limb lengthening and realignment. Note frame modification with addition of hinges and angular distractor

postoperatively) osteotomy. Moreover, gradual realignment of the limb should be followed to modest limb lengthening (typically 2–3 cm) to allow even more time for the proximal segment to finalize its orientation. If the patient is able to adduct beyond neutral, consideration should be made to increase the valgus position of the proximal femur either acutely under anesthesia or gradually after appropriate frame modification. Alternatively, if the patient is unable to adduct to neutral, reduction in the amount of valgus position should be similarly considered.

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Fig. 10 AP radiograph at the completion of sequential limb lengthening after distal metaphyseal femoral osteotomy. Note complete consolidation between the proximal and middle fragments at the pelvic support osteotomy and progressive mineralization of both distraction regenerates

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Fig. 11 Clinical appearance of lower extremities during the consolidation period. At this time, 50% of half pins and wire were removed from the external supports; frame was dynamized for 2 months and then completely removed

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Fig. 12 AP radiograph during second-stage 7 cm of femoral lengthening performed at skeletal maturity as a final stage of treatment. Note 3-cm distraction gap filled with mineralizing bone regenerate. At this time, patient underwent secondary osteotomy above to continue sequential femoral lengthening Fig. 13 AP radiograph at the completion of sequential femoral lengthening. Note second 4-cm distraction regenerate above the area of initial 3-cm lengthening

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Fig. 14 Clinical appearance of lower extremities at the completion of repeated femoral lengthening

Fig. 15 AP radiographs at the completion of consolidation period after sequential femoral lengthening. Note progressive mineralization of both distraction regenerates

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Fig. 17 Clinical appearance in single-limb stance 5 years after pelvic support osteotomy and limb reconstruction. The patient is without complaints and active in sport. He is satisfied with the improvement in his gait and correction of leg length inequality

References and Suggested Reading Fig. 16 Standing AP radiograph 5 years after pelvic support osteotomy with limb lengthening and reconstruction. Note the shape of the femur with complete remodeling of newly formed tissues

Cross-References ▶ Hinged Arthrodiastasis for Avascular Necrosis of the Hip ▶ Hip Fusion with an External Fixator ▶ Ilizarov Hip Reconstruction for Post Infective Femoral Head Destruction

1. Dimitrios P, Nayagam S. The pelvic support osteotomy: indications and preoperative planning. Strateg Trauma Limb Reconstr. 2008;32 (2):83–92. 2. Ilizarov GA, Samchukov ML, Kurtov VM. Ilizarov restorative reconstruction surgery in treatment of hip osteoarthritis, congenital and pathological hip dislocation in children and adults. In: Materials of second international symposium on experimental, theoretical and clinical aspects of transosseous osteosynthesis developed. Kurgan: Kurgan Scientific Research Institute; 1986. p. 100–2. 3. Mahran MA, Elgebeily MA, Ghaly NA, Thakeb MF, Hefny HM. Pelvis support osteotomy by Ilizarov’s concept: is it a valuable option in managing neglected hip problems in adolescents and young adults. Strateg Trauma Limb Reconstr. 2011;6(1):13–20. 4. Rozbruch SR, Paley D, Bhave A, Herzenberg JE. Ilizarov hip reconstruction for the late sequelae of infantile hip infection. J Bone Joint Surg Am. 2005;87:1007–18. 5. Samchukov ML, Birch JG. Restoration of pelvic support and limb length by the Ilizarov method. Bull Hosp Jt Dis. 1992;52(1):7–11. 6. Samchukov MS, Cherkashin AM, Birch JG. Pelvic support osteotomy and limb reconstruction for septic destruction of the hip. Oper Tech Orthop. 2013;23:158–66.

Part V Pediatric Deformity: Pediatric Blount Disease

Pediatric Blount Disease: An Introduction

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Blount disease is a developmental disorder of the proximal tibia that encompasses two types: early onset (includes infantile or juvenile) and late onset (adolescent). In the infantile group, the clinical manifestations first appear as soon as the child begins to ambulate. It usually presents as excessive genu varum (often bilateral) that may be difficult to distinguish from physiological genu varum. Because growth of the posteromedial proximal tibial physis is affected, Blount disease typically creates a multi-planar deformity. Proximal tibial varus, procurvatum (apex anterior), internal tibial torsion, and, in unilateral cases, limb length discrepancy can all occur simultaneously. Medial plateau depression occurs if the disease progresses (usually after the age of 6 years) and indicates the medial physis is nonfunctional or absent due to the development of a bony bridge medially. In the adolescent type, distal femoral valgus may coexist with the varus tibial deformity. The goal of treatment is to restore normal mechanical alignment to the limb in order to prevent the development of degenerative changes in the knee (Table 1). In this section, 12 possible management strategies of Blount’s disease are presented, depending on the age of the patient and the severity of the disease, including neglected and recurrent cases. Several treatment modalities are discussed as well as different types of internal and external fixation. If the diagnosis of infantile Blount disease is made early (less than 3 years of age), conservative treatment in the form of bracing (KAFO) may be tried first. However, the results of bracing are equivocal. Presentation after the age of 3 years or progression of the disease despite bracing (case 61) is usually an indication for surgery. Acute (case 62) or gradual correction of the deformity with an osteotomy can be performed although some reports demonstrate increased accuracy of correction using gradual

techniques. Guided growth of the lateral proximal tibial physis is another option in the growing child (as shown in case 61); however, this will not correct any internal tibial torsion if present. If left untreated (cases 63, 64), the deformity will progressively worsen. Cases of recurrence of the deformities are almost always caused by depression of the medial plateau. Depression of the medial plateau from a medial physeal bar adds an intra-articular deformity to the already existing deformities. The plateau depression can be addressed by elevating the medial plateau using a separate opening wedge osteotomy (cases 63, 64, 65, 66, 67). If depression of the medial tibial plateau is the only deformity, then an elevating osteotomy can be performed alone and stabilized with two screws. If there is concomitant varus, procurvatum, and/or internal tibial torsion, then a second more distal osteotomy may be performed simultaneously to gradually realign the tibia. Cases 63, 64, 65, 66 and 67 demonstrate various techniques that can be used to stabilize the opening wedge osteotomy used for elevation of the tibial plateau. One method uses a half pin (as in case 63) or wire (cases 64, 66) to secure it to the proximal ring of the circular external fixator. Another option is to secure the opening wedge osteotomy with internal fixation using two screws first and then apply the circular external fixator (as in case 65). Fortunately, in the 5 cases with adolescent Blount disease (cases 68, 69, 70, 71 and 72), depression of the tibial plateau rarely occurs. Gradual correction of the alignment with a proximal tibial osteotomy can be obtained with a uniplanar or circular external fixator. Concomitant fibular osteotomy is almost always performed, except in mild cases with minimal internal tibial torsion and procurvatum. External fixators have the advantage of allowing immediate weight bearing even in obese patients. Obese

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_364

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Table 1 Details of the 12 Blount’s disease cases discussed in this Atlas Case 61

Diagnosis 3 year old male, unilateral Blount 4 year old male, bilateral Blount

Problems Proximal tibia varus

63

9 year old female, neglected Blount

Tibia vara, internal tibial torsion, LLD, depression of medial plateau, but no bar

64

6 year old female, neglected Blount

Tibia vara, depression medial plateau with bar

65

8 year old female, recurrent unilateral Blount

66

8 year old female, recurrent unilateral Blount

Depression of medial plateau only. No proximal tibia vara and no ITT (failure of acute correction and hemi-epiphysiodesis) Depression of medial plateau, tibia vara, ITT

67

16 year old male, neglected unilateral infantile Blount. Skeletally mature 15 year old male, adolescent Blount 13 year old male, adolescent Blount, skeletally immature 13 year old female, adolescent Blount, skeletally mature 15 year old female, adolescent Blount, skeletally mature 13 year old male, adolescent Blount, unilateral, skeletally immature

62

68 69

70

71

72

Failure of bracing, tibia vara, and procurvatum, internal tibial torsion

Tibia vara, depression of medial plateau

Surgery and key points Guided growth with 8 plates – extraperiosteal. Overcorrection in valgus Acute correction with the proximal tibia and fibular osteotomies, derotation, overcorrection into valgus, fixation with K-wires One stage correction: elevation of medial plateau with one large half pin connected to TSF, proximal tibial and fibular osteotomies, gradual correction with TSF One stage correction: elevation of medial plateau with wire fixed to TSF, proximal tibial and fibular osteotomies, gradual correction with TSF. Overcorrection into the valgus Medial plateau elevation and fixation with 2 screws. Only intra-articular joint incongruity needed to be addressed Medial plateau elevation and internal fixation with 2 screws. Then application of TSF for gradual correction Medial plateau elevation and fixation to TSF with screw and wire

Tibia vara, procurvatum, LLD, no distal femoral valgus

Proximal tibia osteotomy and TSF. No fibular osteotomy Hemiepiphysiodesis distal femur and proximal tibia. One year later, tibial deformities did not correct: TSF applied MAC (multiaxial correction) external fixator. Tibial and fibular osteotomies

Obese, tibia vara, procurvatum, ITT

Proximal tibial and fibular osteotomies with TSF

Severely obese Tibia vara, procurvatum, ITT

Proximal tibial and fibular osteotomies with TrueLok device

Proximal tibia vara, mild procurvatum, ITT Tibia vara, procurvatum, LLD, distal femur valgus

TSF Taylor spatial frame MAC multiaxial correction

patients require extra fixation points to maintain external fixator stability during the course of treatment and have an increased risk of deep venous thrombosis (cases 71, 72). If distal femoral valgus deformity is identified, it should be

addressed by a medial distal femoral hemiepiphysiodesis or an osteotomy. Fibular osteotomy is almost always performed, except in mild cases specifically with minimal internal tibial torsion (case 68).

Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF

72

Peter Calder

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 506 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 506 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 507 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 507 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 507 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 508 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 509

Abstract

A 16 year old Nigerian boy presented with asymmetrical genu varum. The right knee has both an intra-articular and metaphyseal deformity corrected by hemi-plateau elevation and gradual correction of the distal deformity using a Taylor Spatial Frame.

fixed flexion deformity and flexion to 110 . The preoperative radiographs confirm a displacement of the mechanical axis outside of the medial compartment of the knee. There was a medial tibial plateau depression of approximately 40 and varus deformity of the proximal tibia.

Preoperative Clinical Photos and Radiographs Brief Clinical History A 16 year old Nigerian boy presented with asymmetrical genu varum and knee pain preventing him from walking for long periods. He gives a history of bilateral bow legs from an early age. He had undergone two previous operations on the left leg aged 5 but no previous surgery on the right leg which is the case study. The right knee was in 20 of varus which was partially correctable. There was approximately 5 of

See Figs. 1, 2, and 3.

Preoperative Problem List • Intra-articular deformity with knee instability • Proximal metaphyseal varus deformity

P. Calder (*) Royal National Orthopaedic Hospital (RNOH), Brockley Hill, Stanmore, London, UK e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_13

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Fig. 1 Clinical photograph demonstrating bilateral asymmetrical genu varum Fig. 3 CT scan demonstrating medial tibial plateau depression Fig. 2 Preoperative standing long-leg film

Treatment Strategy The aim was to produce a stable congruent joint and restore the mechanical axis of the leg within the center of the knee. The medial tibial plateau is elevated acutely by performing an osteotomy within the epiphyseal/metaphyseal bone. This is held in place by fibula autograft and wires and a half pin attached to the proximal ring of a Taylor Spatial Frame. A second osteotomy is performed at the CORA of deformity within the metaphysis and the residual varus deformity corrected by distraction osteogenesis.

Basic Principles The depression of the medial tibial plateau results in knee instability and contributes to the lateral thrust seen in gait. A “J”-shaped incision of the medial side of the tibia is made to allow subperiosteal exposure. A ring-handled retractor is placed subperiosteally behind the knee to protect the neurovascular structures. An osteotomy is planned from just below the medial tibial spine in a curved fashion distally to the metaphyseal/diaphyseal junction. The osteotomy is predrilled and completed with a sharp osteotome. A lamina spreader is inserted and the plateau elevated acutely, hinging

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Fig. 4 (a, b) Intraoperative images demonstrating predrilling of the osteotomy and completion with an osteotome

to posteromedial tibial “face” wires and anteromedial half pins to minimize soft tissue tethering. The CORA of the residual deformity is then calculated and a standard osteotomy performed with drill and osteotome. After a latent period of 6 days, the metaphyseal deformity is corrected slowly with distraction osteogenesis. Weight bearing is encouraged as comfort allows and the frame is removed once consolidation of the regenerate bone is achieved.

Images During Treatment See Figs. 4, 5, 6, 7, 8, and 9.

Technical Pearls

Fig. 5 Acute correction of the plateau depression with a lamina spreader

on the subchondral bone. By placing the spreader more posteriorly, an excessive posterior slope may be corrected. The osteotomy position is maintained by placing a fibula graft or tricortical iliac crest graft and the skin incision closed. Bone substitutes such as calcium phosphate may be placed into the residual osteotomy gap. Fixation is maintained by passing wires and a half pin through the medial fragment into the intact lateral aspect of the proximal tibia. The distal tibial fixation is achieved with one or two rings using anterolateral

When completing the proximal osteotomy, rotate the C-arm of the image intensifier to the lateral position so that an accurate knowledge of the depth of the osteotome is known (Fig. 4b). Gentle taps with the hammer allows the posterior cortex to be divided safely. Frame stability is tailored to the individual patient. In the obese patient two distal rings are required. With excessive proximal tissues, the use of wires cannot be tolerated and so half pins alone are used. Protected weight bearing until alignment and regenerate consolidation may be required.

Outcome Clinical Photos and Radiographs See Figs. 10 and 11.

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Fig. 6 (a, b) The osteotomy position is maintained with fibula autograft

Fig. 8 Fixation with three proximal wires and a medial to lateral hydroxyapatite half pin. The osteotomy gap has been filled with calcium phosphate crystals

Fig. 7 Metaphyseal deformity calculated

Avoiding and Managing Problems The treatment process is long and so preoperative counseling is essential with a multidisciplinary team. This should include specialist nurse practitioners and physiotherapists. The frame

time is usually between 4 and 6 months. It is important to lengthen the metaphyseal deformity a little during distraction to allow for translation. In the obese patient it is sometimes difficult to achieve adequate radiographs to confirm correction has been achieved. Ensuring full knee extension in stance and patella pointing forward on each long-leg view is vital to monitor deformity correction.

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Fig. 9 Immediate postoperative frame position

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Fig. 11 Final outcome with mechanical axis restored, a stable horizontal knee, and return to full function pain-free

Cross-References ▶ 13 Year Old with Unilateral Late-Onset Blount Disease ▶ Adolescent Blount’s Disease Treated with MAC External Fixation System ▶ Correction of Juvenile Blount’s Disease ▶ Infantile Blount Disease with Plateau Depression ▶ Morbidly Obese Teenager with Significant Blount’s Treated with Taylor Spatial Frame ▶ Simultaneous Correction of Medial Proximal Tibial Plateau Depression and Tibia Vara in an Obese Child with Blount’s

References and Suggested Reading

Fig. 10 Long-leg radiograph after metaphyseal deformity correction

1. Janoyer M, Jabbari H, Rouvillain JL, Sommier J, Py G, Catonne Y, Colombani JF. Infantile Blount’s disease treated by hemiplateau elevation and epiphyseal distraction using a specific external fixator: preliminary report. J Pediatr Orthop B. 2007;16:273–80. 2. Jones S, Holsalkar HS, Hill RA, Hartley J. Relapsed infantile Blount’s disease treated by hemiplateau elevation using the Ilizarov frame. J Bone Joint Surg (Br). 2003;85:565–71. 3. Schoenecker PL, Johnston R, Rich MM, Capelli AM. Elevation of the medial plateau of the tibia in the treatment of Blount disease. J Bone Joint Surg Am. 1992;74:351–8.

Adolescent Blount’s Disease Treated with MAC External Fixation System

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Anish G. R. Potty, Michael M. Kheir, and Richard S. Davidson

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 511 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 512 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 512 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 512 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 513 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 513 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 513 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 514 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 514 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517

Abstract

This case describes a 13 year old female with adolescent Blount’s disease of the left leg. Blount’s disease causes genu varum and internal tibial torsion. Treatment options are numerous, including nonoperative or operative management depending on the specific clinical presentation and whether there are any relative indications to choose surgery over a nonoperative course. The treatment undergone in this case included a left tibia and fibula osteotomy with application of Biomet Multi-Axial Correction (MAC) External Fixation System with six bone screws and, through separate incisions, anterior and lateral A. G. R. Potty (*) · R. S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] M. M. Kheir Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA e-mail: [email protected]; [email protected]

compartment release as prophylactic fasciotomies. Our operative strategy with the MAC external fixator has been well described for patients with Blount’s disease. The use of the MAC external fixator allows for gradual correction and adjustment of angular deformity and displacement in all three planes, including correction of residual or secondary deformities that occur during lengthening. The fixator can correct lower extremity deformities, including Blount’s disease, and allows the possibility of anatomic alignment within normal radiographic limits.

Brief Clinical History The patient is a 13 year old female with no significant past medical history who presents to the clinic because her mother began noticing varus bowing of her left lower extremity, and patient has been experiencing pain in that extremity (Fig. 1). At surgery, she was treated with left tibia and fibula osteotomy with application of Biomet Multi-Axial Correction (MAC) External Fixation System with six bone screws

© Crown 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_43

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and, through separate incisions, anterior and lateral compartment release (Fig. 6) and perioperative antibiotics. The bones achieved anatomic alignment over the next few months.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Preoperative Problem List • Left tibia vara (Blount’s disease)

Treatment Strategy

Fig. 1 Preoperative photograph demonstrating left lower extremity varus (Blount’s disease)

Fig. 2 Preoperative radiographs demonstrating left lower extremity varus (Blount’s disease). Lateral view of the left lower extremity (a) and lateral view of the left knee. Note the physis still open at tibial tuberosity (b)

The preoperative plan included left tibia and fibula osteotomy with application of the Biomet Multi-Axial Correction (MAC) External Fixation System. The anterior and lateral compartments were examined for fullness, and prophylactic fasciotomy was performed to relieve pressure. All wounds were thoroughly irrigated. Hemostasis was obtained with electrocautery. All wounds were then packed with Xeroform gauze around the bone screws and sterile dressings on each of

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Fig. 3 Preoperative orthoroentgenogram demonstrating frontal plane deformity of the left lower extremity Blount’s disease. AP of bilateral lower extremity (a), AP of bilateral knees (b), and AP of bilateral ankle (c). The right knee shows healed corrective osteotomies (b)

the incisions. The potential infection was managed with perioperative antibiotics.

Basic Principles The use of the MAC external fixator allows for correction and adjustment of angular deformity and displacement in all three planes, including correction of residual or secondary deformities that occur during lengthening. The fixator can correct lower extremity deformities, including Blount’s disease, and allows the possibility of anatomic alignment within normal radiographic limits, with studies demonstrating significant improvements in the mean anatomic medial proximal tibial angle [2]. Our operative strategy with the MAC external fixator has been well described for patients with Blount’s disease with minimal intra- and postoperative complications, decreased wear time, and decreased operative time compared to the traditional Ilizarov circular external fixator [3].

Images During Treatment See Figs. 4, 5, 6, 7, and 8.

Technical Pearls A safe tibial osteotomy is distal to the CORA because of the tibial tubercle and attachment of the patellar ligament (the osteotomy must be placed distal to the attachment or the patella will be displaced distally). Insert proximal screws first. The safe placement of the proximal bone screws is in the metaphysis but distal to the joint and physis. Sometimes the distal screws do not align well with the distal medial shaft. In that case, translate and rotate the compression/distraction mechanism or remove the guide pin and rotate the arc, but one must compensate. A fibular osteotomy is then performed at the middle or distal thirds of the fibula with care not to injure the sensory branch of the peroneal nerve. Anterior and lateral subcutaneous fasciotomies are then performed through the incisions for the osteotomies of the tibia and fibula, respectively. It is important that no corrections be performed acutely, avoiding peroneal nerve stretch injury. In the postoperative period, patients are taught how to do range of motion exercises and how to turn the compression/distraction mechanism. By the third week, after lengthening has been accomplished, angular correction is begun until realignment has been achieved. After these deformities are corrected, translation and any residual angular deformities are corrected.

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Fig. 4 Intraoperative photographs of the left lower extremity after fibula osteotomy, applying the MAC external fixator, with a rotation arc proximally, the multiaxial hinge on the guidewire (which must be in the center of rotation of angulation; CORA), and then a compression

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distraction device distally (a). This was lined up with the deformity of the leg (b). Once all six screws were in solid position, the fixator was tightened to these bone screws. The guidewire was removed (c). The Gigli saw was used to complete the osteotomy of the tibia (d)

Outcome Clinical Photos and Radiographs See Fig. 9.

Avoiding and Managing Problems

Fig. 5 Intraoperative photographs of the left lower extremity after application of the fixator

If there is sufficient growth potential remaining in the patient, a discussion of treatment alternatives, including hemiepiphysiodesis, must be discussed. The most common complications following application of the MAC external fixation system are pin site infections and cellulitis, requiring antibiotics. Though less frequent, recurrence of varus is also a potential complication; however, most of these secondary deformities are able to be corrected using the primary or secondary hinge. Nerve palsy, leg length discrepancy, delayed union/nonunion, and compartment syndrome are rare complications. In general, major complications, including neurovascular complications, are extremely rare given the gradual correction granted by the MAC external fixator compared to an acute correction. Although rare, a careful neurovascular exami-

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Fig. 6 Intraoperative radiographs of the proximal tibia demonstrating osteotomy of the proximal tibial diaphysis with satisfactory alignment, and external fixator is partially visualized. AP view of the proximal tibia (a) and oblique view of the proximal tibia and fibula (b)

nation must still be performed after the procedure. The MAC external fixator appears to have comparable ability to the Ilizarov circular fixator in correcting multiple deformity components of Blount’s disease, with less wear time and less risk of major complications [3]. Patients need to be followed closely during the correction to avoid malalignment. Weekly X-rays are obtained until the deformities are corrected, usually in 2–3 weeks. Monthly X-rays are obtained from then on until consolidation is observed on radiographs, at which time the device can then be removed. As with all fixators, it can affect radiographic evaluation by directly obscuring the osteotomy site, which may lead to errors in measurements [4]. One must take account for these errors; oblique radiographs maybe used to better visualize the osteotomy site, location of the CORA, and coexistent valgus deformities of the femur.

Fig. 7 Postoperative photograph demonstrating patient with external fixator

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2 weeks

2 months

2 weeks

2 months

a

e

c

g

b

f

d

h

Fig. 8 Postoperative radiographs demonstrating gradual correction of the varus deformity and healing osteotomies of the left lower extremity at 2 weeks (a–d) and 2 months (e–h). See Fig. 9 for later outcome

Fig. 9 Radiographs of the left lower extremity after fixator removal, 4 months postoperatively. AP view of the proximal tibia (a) and lateral view of the tibia and fibula (b)

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Cross-References ▶ 13 Year Old with Unilateral Late-Onset Blount Disease ▶ Tibial Hemimelia

References and Suggested Reading 1. Davidson RS. The MAC (multi-axial correcting) monolateral external fixation system (Biomet/EBI) technique: an easier way to correct deformity. Oper Tech Orthop. 2011;21(2):113–24.

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2. McCarthy JJ, Ranade A, Davidson RS. Pediatric deformity correction using a multiaxial correction fixator. Clin Orthop Relat Res. 2008;466:3011–7. 3. Pandya NK, Clarke SE, McCarthy JJ, Horn BD, Hosalkar HS. Correction of Blount’s disease by a multi-axial external fixation system. J Child Orthop. 2009;3:291–9. 4. Sabharwal S, Badarudeen S, McClemens E, Choung E. The effect of circular fixation on limb alignment. J Pediatr Orthop. 2008;28:314–9.

Correction of Adolescent Tibia Vara Without Fibular Osteotomy and Without Fixation of the Fibula Using the Taylor Spatial Frame (TSF)

74

Mark Eidelman

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 519 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 519 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 519 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 520 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 523

Abstract

Adolescent tibia vara can present as a complex threedimensional deformity of the tibia: varus, procurvatum, and internal tibial torsion (Gordon JE et al. J Bone Joint Surg Am 87:1561–1570, 2005). Standard treatment protocol usually includes an osteotomy of the proximal tibia and a fibular osteotomy. When utilizing the Taylor spatial frame (TSF), the origin can be placed at the level of the proximal tibio-fibular joint. This will allow the correction to occur without the need for a fibular osteotomy (Eidelman M et al. J Child Orthop 2:199–204, 2008). We will present the treatment of this condition without a fibular osteotomy and without fixation of the fibula using the TSF.

Brief Clinical History A 15 year old boy with left tibia vara, mild procurvatum of the proximal tibia, and internal tibial torsion (thigh–foot angle measures 5 ).

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List 1. Proximal tibial varus 2. Proximal tibial procurvatum 3. Internal tibial torsion

M. Eidelman (*) Technion Faculty of Medicine, Rambam Health Care Campus, Meyer’s Children Hospital, Haifa, Israel e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_63

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Fig. 1 (a, b) Proximal tibial varus and medial mechanical axis deviation (MAD). (c) Mild proximal tibial procurvatum (PPTA) 74

Treatment Strategy The operation is started by passing a Gigli saw just below the proximal tibial tubercle (Fig. 2a). The proximal portion of the tibia is fixed using a single 1.8-mm Ilizarov wire and three 6-mm half-pins. The distal part of the tibia is then fixed with four half-pins and 1.8 mm of wire (Fig. 2b). After removal of

the two anterior struts of the Taylor spatial frame (TSF), the osteotomy is completed using the Gigli saw. The origin is placed at the level of the proximal tibio-fibular joint (Fig. 2e). Typically, internal rotation is less than 20 ; therefore, there is no need to perform fibular osteotomy because of rotational problems. The correction is started 1 week after the operation with the correction velocity set at 0.75 mm daily.

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Fig. 2 (a) Passing the Gigli saw through two percutaneous incisions. (b) Completion of the fixation. (c) Intraoperative radiograph. (d) Lateral radiograph demonstrates the osteotomy and the center of the distal reference ring. (e) Origin is placed along the mechanical axis of reference fragment at the level of the proximal tibio-fibular joint

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Fig. 3 (a) Completion of the correction with normalization of the mechanical axis. (b) Two years after correction – note the normal mechanical axis and the normal relationship of the tibia and fibula. (c). Clinical appearance 2 years after correction

Basic Principles

Outcome Clinical Photos and Radiographs

Standard treatment of adolescent tibia vara requires a proximal tibial osteotomy and a fibular osteotomy. In patients with proximal tibial varus, the correction can be carried out without fibular osteotomy if the patient does not have significant rotation or significant procurvatum (procurvatum greater than varus) [1, 5].

See Fig. 3.

Images During Treatment

Avoiding and Managing Problems Avoid unstable fixation. It is better to add more half-pins and wires and to remove them if one becomes infected or painful than to “under fix.” Stable fixation is the key to success and helps to avoid loosening, infection, and pain during correction process.

See Fig. 2.

Cross-References Technical Pearls • Avoid unstable fixation • Use 6-mm half-pins whenever possible • Reference ring must be orthogonal relative to the reference fragment

▶ Adolescent Blount’s Disease Treated with MAC External Fixation System

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Correction of Adolescent Tibia Vara Without Fibular Osteotomy and Without Fixation of the Fibula Using the. . .

References and Suggested Reading 1. Eidelman M, Bialik V, Katsman A. The use of the Taylor spatial frame in adolescent Blount disease: is fibular osteotomy necessary? J Child Orthop. 2008;2:199–204. 2. Feldman DS, Madan SS, Koval KJ, van Bosse HJ, Bazzi J, Lehman WB. Correction of tibia vara with six-axis deformity analysis and the Taylor spatial frame. J Pediatr Orthop. 2003;23:387–91.

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3. Gordon JE, Heindrich FP, Carpenter CJ, Kelly-Hahn J, Shoenecker PL. Comprehensive treatment of the late-onset tibia vara. J Bone Joint Surg Am. 2005;87:1561–70. 4. Sachs O, Katzman A, Abu-Johar E, Eidelman M. Treatment of adolescent Blount disease using Taylor spatial frame with and without fibular osteotomy: is there any difference? J Pediatr Orthop. 2014; https://doi.org/10.1097/BPO.0000000000000317. 5. Taylor JC (2013) Correction of general deformity with Taylor spatial frame. http://www.jcharlestaylor.com. Accessed Dec 2013.

Correction of Juvenile Blount’s Disease

75

Mark Eidelman

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 525 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 525 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 525 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 525 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 528

Abstract

Treatment of a patient with neglected juvenile Blount’s disease can be a challenging problem that may need a complete proximal tibia and fibula epiphysiodesis, elevation of medial tibia plateau, proximal tibial osteotomy, preemptive tibial lengthening, and overcorrection into valgus. Despite this comprehensive treatment, parents should know that recurrence of the varus is common, and further correction might be required close to or after skeletal maturity.

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List 1. Severe varus of tibia 2. Langenskiöld stage 5 of medial proximal tibial physis (irreversible changes of the physis, physeal bar) 3. Depression of the medial proximal tibial plateau

Brief Clinical History Treatment Strategy A 6 year old girl with severe varus of the left proximal tibia and Langenskiöld stage 5 changes to the physis (nearcomplete closure of medial proximal tibial growth plate [2]). M. Eidelman (*) Technion Faculty of Medicine, Rambam Health Care Campus, Meyer’s Children Hospital, Haifa, Israel e-mail: [email protected]; [email protected]

• Complete epiphysiodesis of proximal tibia and fibula • Elevation of the medial tibial plateau • Proximal tibial osteotomy and application of circular external fixator (Taylor spatial frame, TSF) • Gradual deformity correction and preemptive tibial lengthening

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_62

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Fig. 1 (a, b) Preoperative X-rays

• Overcorrection into valgus to compensate for anticipated “rebound” deformity

Basic Principles In presence of the physeal bar and medial tibial plateau depression, restoration of the anatomy of the knee joint should be done by plateau elevation [1, 3]. Completion of the tibial epiphysiodesis must be done to prevent recurrence. Concomitant epiphysiodesis of the proximal fibula should be performed to prevent overgrowth of the fibula. In order to prevent shortening of the leg after closure of the epiphysis of proximal tibia, preemptive lengthening should be performed. Recurrence of the proximal tibial varus is the most common complication; therefore, some overcorrection to valgus is desirable.

Images During Treatment

• Use laminar spreader for plateau elevation (Fig. 2d) and iliac autograft. • Gigli saw proximal tibial osteotomy below insertion of the patellar tendon, fibular osteotomy, and fixation of the fibula distally and proximally.

Outcome Clinical Photos and Radiographs Gradual overcorrection was performed combined with a 25 mm lengthening of the tibia. See Fig. 4.

Avoiding and Managing Problems 1. Completion of the epiphysiodesis of the proximal tibia is crucially important for prevention of recurrence of the varus. 2. Medial plateau should be elevated to the level of the lateral tibial plateau.

See Figs. 2 and 3.

Cross-References Technical Pearls • Percutaneous tibial and fibular epiphysiodesis using Kirschner wire and a cannulated drill (Fig. 2a).

▶ Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF ▶ Hemiplateau Elevation for Early-Onset Blount Disease

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Correction of Juvenile Blount’s Disease

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Fig. 2 (a) Percutaneous epiphysiodesis of the proximal tibia and proximal fibula. (b) Completion of epiphysiodesis of the proximal tibia and fibula. (c) Elevation of the medial tibial plateau after medial proximal tibial osteotomy. (d) Use of the laminar spreader for insertion of the iliac bone graft

Fig. 3 (a) Proximal tibial wire should be inserted parallel to the joint. (b) Completion of the fixation and application of TSF

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M. Eidelman

References and Suggested Reading 1. Hefny H, Shalaby S, El-Kawy S, Takeb M, Elmoatasem E. A new double elevating osteotomy in management of severe neglected infantile tibia vara using the Ilizarov technique. J Pediatr Orthop. 2006;26:233–7. 2. Langenskiöld A. Tibia Vara: osteochondrosis deformans tibiae. Blount’s disease. Clin Orthop Relat Res. 1981;158:77–82. 3. Sabharwal S. Blount disease. Current concept review. J Bone Joint Surg. 2009;91:1758–76.

Fig. 4 (a, b) X-rays after removal of TSF. Note overcorrection and preemptive lengthening of the tibia

Correction of Multi-planar Deformity in 120 kg 13 Year Old Patient with Blount’s Disease

76

Haim Shtarker and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 529 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 530 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 531 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 531 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 532 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 532 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 533 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 537 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 537

Abstract

A 13 year old severely obese male with previously untreated Blount’s disease underwent oblique fibular midshaft osteotomy and predrilled proximal tibial osteotomy followed by gradual correction of multi-planar deformity using hexapod-type TL-Hex circular external fixation.

complaining of periodic knee pain, which was increasing with physical activities. His joint range of motion was within the normal limits. He was otherwise healthy and neurovascularly intact.

Preoperative Clinical Photos and Radiographs Brief Clinical History

See Figs. 1 and 2.

The patient is a 13 year old male with unilateral Blount’s disease, multi-planar proximal tibial deformity (25
varus, 30
procurvatum, and 15
internal rotation), severe obesity (120 kg), and no previous treatment (Figs. 1 and 2). He was

Preoperative Problem List

H. Shtarker (*) Western Galilee Hospital, Nahariya, Israel e-mail: [email protected]; [email protected]

• Periodic knee pain increasing with physical activities • Multi-planar proximal tibial deformity (25
varus, 30
procurvatum, 15
internal rotation) due to unilateral Blount’s disease

M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_67

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Fig. 1 Preoperative front view photograph and AP radiograph demonstrating 25
of proximal tibial varus deformity (MPTA ¼62
, JLCA ¼5
varus) and 15
of internal tibial torsion. Note characteristic pathological changes at the proximal medial growth plate

Fig. 2 Preoperative photograph and LAT radiograph demonstrating 30
of proximal tibial procurvatum deformity (PPTA ¼50
)

• Adolescent patient with open growth plates potentially resulting in recurrence of the deformity and requiring slight overcorrection, opposite leg epiphysiodesis, or repeated deformity correction • Severe obesity (120 kg)

Treatment Strategy Because of severe multi-planar deformity without prominent knee joint incongruence in the 13 year old adolescent patient, the treatment strategy was based on proximal tibial corrective osteotomy rather than growth modulation, lateral hemiepiphysiodesis, or tibial plateau elevation. Due to severe

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Correction of Multi-planar Deformity in 120 kg 13 Year Old Patient with Blount’s Disease

obesity of the patient, acute deformity correction with locking plate fixation requires additional plaster cast immobilization to prevent loss of fixation and overstressing the internal hardware. Therefore, the treatment planning included proximal tibial and midshaft fibular osteotomies followed by gradual deformity correction until slight overcorrection into genu valgum utilizing hexapod-type TL-Hex circular external fixation. This method provides for stable fixation of bone fragments and precise restoration of limb alignment while permitting weight bearing and physical therapy of the knee and ankle joints during the treatment.

Basic Principles Blount’s disease is a developmental abnormality of the medial part of the proximal tibia resulting in varus deformity and significant internal rotation. Severe periarticular bony deformity causes noticeable deviation of the mechanical axis with compression forces at the medial compartment of the knee and distraction forces at the lateral knee stabilizers. In addition to prominent cosmetic problems, irreversible degenerative changes will appear in untreated medial knee compartment with time, associated with lateral tract and

Fig. 3 Computerized preoperative planning of frame assembly for correction of multiplanar proximal tibial deformity using the TL-Hex software

531

lateral instability of the knee [7]. The main goal of limb reconstruction in Blount’s disease is to restore the normal lower limb alignment relocating the mechanical axis of the lower extremity to more normal spatial orientation with simultaneous correction of the pathological internal rotation of the tibia [6, 9]. In some severe cases, the knee congruency should also be restored in conjunction with limb realignment by an additional tibial osteotomy and medial tibial plateau elevation [8]. Correction of tibial deformity can be done acutely followed by internal fixation or gradually using external fixation [2–5, 10, 11]. Due to multi-planar nature of tibial deformity especially in severely obese patients with Blount’s disease, utilization of gradual deformity correction using hexapod type of external circular fixation is preferable approach in treatment [1]. This method provides excellent stability of bone fragment fixation with the possibility of early weight bearing and allowed for high-precision deformity correction while keeping the knee joint without additional immobilization.

Images During Treatment See Figs. 3, 4, 5, 6, 7, 8, 9, 10, 11, and 12.

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Fig. 6 Intraoperative photograph during insertion of the second (distal) reference wire orienting the preassembled fixator relative to the tibia. Note additional TrueLok ring blocked to the distal TL-Hex ring to enhance stability of fixation

Technical Pearls Fig. 4 Intraoperative photograph demonstrating preassembled circular external fixator. Note proximal and distal rings interconnected by six telescoping struts in position mimicking the tibial deformity

We prefer to preassemble circular external fixation devices for correction of the majority of bone deformities. Several unique features of the TL-Hex online-based software permit preassembling the hexapod type of external fixator with mimicking the most complicated multi-planar deformities. Built-in preoperative planning module allows entering standard deformity parameters, frame parameters, and planned mounting parameters followed by “frame assembly” with the most efficient software-calculated strut lengths that provides deformity correction with either no or minimal number of strut exchanges. Moreover, surgeon can manipulate with the mounting parameters (e.g., the distance from the deformity apex to the reference ring) until complete satisfaction with computer-generated strut length adjustment prescription. In addition, software allows for manipulation with position of the bone segments relative to each other at the end of deformity correction, thereby modifying strut length adjustment prescription to achieve either under- or overcorrection. This feature is very important for skeletally immature patients (e.g., with Blount’s disease) when deformity correction mat requires overcorrection into the valgus.

Outcome Clinical Photos and Radiographs Fig. 5 Intraoperative photograph of the tibia visualizing tibial deformity and preoperative planning of frame application. Note the anatomic axis of the proximal and distal tibiae as well as marked levels for external supports

See Figs. 13 and 14.

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Correction of Multi-planar Deformity in 120 kg 13 Year Old Patient with Blount’s Disease

533

Fig. 7 Intraoperative AP and LAT radiographs after oblique fibular midshaft osteotomy, predrilled proximal tibial osteotomy, and completion of frame stabilization. Note orthogonal orientation of the centralized proximal reference ring relative to the tibial axis

Avoiding and Managing Problems Identification and correction of multi-planar deformity in obese Blount’s patients present several challenges potentially affecting overall result of the treatment. Large size of the lower extremity around the knee area associated with combination of proximal tibial procurvatum and internal rotation creates difficulties in obtaining sufficient LAT radiograph and calculation of posterior proximal tibial angle. Our preference in those cases is to correct proximal tibial varus and internal rotation first followed by correction of residual tibial procurvatum deformity. In the TL-Hex software, built-in checkup module allows to modify deformity parameters within the existing frame at any time during the deformity correction and, therefore, adjust the strut length adjustment prescription to accommodate correction of additional deformity. Another important requirement for precise deformity correction is obtaining of two standard radiographs (AP and LAT) taking as orthogonal relative to each other as possible. Obtaining of orthogonal X-rays centralized around the reference ring allows to minimize the number of additional prescriptions to correct residual deformities of the limb.

Fig. 8 Intraoperative lateral view and medial view photographs at the end of surgery demonstrating frame orientation on the tibia. Note final frame stabilization to the tibial bone fragments using two crossing wires with two half pins proximally and two crossing wires and one half pin distally

534 Fig. 9 Postoperative AP and LAT radiographs of the tibia before gradual deformity correction

Fig. 10 Front and lateral view photographs of lower extremities during gradual deformity correction

H. Shtarker and M. Samchukov

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Correction of Multi-planar Deformity in 120 kg 13 Year Old Patient with Blount’s Disease

Fig. 11 AP and LAT radiographs of the tibia after gradual deformity correction. Note opening wedge gap in both projections with lateral translation of contacting bone surfaces

Fig. 12 Front and lateral view photographs of lower extremities after gradual deformity correction during the consolidation period. Following 25 days of distraction and 4.5 months of consolidation, the frame was removed with no additional immobilization

535

536 Fig. 13 Front view photograph of the lower extremities and AP radiograph of the lower extremities illustrating clinical appearance 1 year after treatment. Note proper limb alignment and complete remodeling of the distraction regenerated with corticalization

Fig. 14 Lateral view photograph of the left lower extremity and LAT radiograph of the tibia 1 year after treatment. The patient has no complaints and is happy with the appearance of his lower extremities. His joint function is completely restored, and he has returned to normal school activities

H. Shtarker and M. Samchukov

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Correction of Multi-planar Deformity in 120 kg 13 Year Old Patient with Blount’s Disease

Cross-References ▶ 13 Year Old with Unilateral Late-Onset Blount Disease ▶ Adolescent Blount’s Disease Treated with MAC External Fixation System ▶ Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF ▶ Correction of Adolescent Tibia Vara Without Fibular Osteotomy and Without Fixation of the Fibula Using the Taylor Spatial Frame (TSF) ▶ Morbidly Obese Teenager with Significant Blount’s Treated with Taylor Spatial Frame

References and Suggested Reading 1. Clarke SE, McCarthy JJ, Davidson RS. Treatment of Blount’s disease: a comparison between the multi-axial correction system and other external fixators. J Pediatr Orthop. 2009;29(2):103–9. 2. Coogan PG, Fox JA, Fitch RD. Treatment of adolescent Blount’s disease with the circular external fixation device and distraction osteogenesis. J Pediatr Orthop. 1996;16(4):450–4.

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3. El-Rosasy M, Ayoub M. Acute correction of proximal tibial deformities in adolescents using Ilizarov external fixator: focal-dome versus straight-cut osteotomy. J Pediatr Orthop B. 2007;16(2): 113–9. 4. Feldman DS, Madan SS, Ruchelsman DE, Sala DA, Lehman WB. Accuracy of correction of tibia vara: acute versus gradual correction. J Pediatr Orthop. 2006;26(6):794–8. 5. Gilbody J, Thomas G, Ho K. Acute versus gradual correction of idiopathic tibia vara in children: a systematic review. J Pediatr Orthop. 2009;29(2):110–4. 6. Gordon JE, Heidenreich FP, Carpenter CJ, Kelly-Hahn J, Schoenecker PL. Comprehensive treatment of late-onset tibia vara. J Bone Joint Surg Am. 2005;87:1561–70. 7. Jones RE, Herring JA. Blount’s disease after skeletal maturity. J Bone Joint Surg Am. 1982;64:1004–9. 8. Jones S, Hosalkar HS, Hill RA, Hartley J. Relapsed infantile Blount’s disease treated by hemi-plateau elevation using the Ilizarov frame. J Bone Joint Surg (Br). 2003;85:565–71. 9. Sabharwal S, Lee J, Zhao C. Multi-planar deformity analysis of untreated Blount’s disease. J Pediatr Orthop. 2007;27(3):260–5. 10. Smith SL, Beckish ML, Winters SC, Pugh LI, Bray EW. Treatment of late-onset tibia vara using Afghan percutaneous osteotomy and Orthofix external fixation. J Pediatr Orthop. 2000;20(5):606–10. 11. Stanitski DF, Dahl M, Louie K, Grayhack L. Management of lateonset tibia vara in the obese patient by using circular external fixation. J Pediatr Orthop. 1997;17(5):691–4.

Guided Growth Treatment for Early-Onset Blount Disease

77

Sanjeev Sabharwal

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 539 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 540 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 540 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 540 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 540 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 541 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 542 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 542 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 542 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 545 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 545

Abstract

A 3-year-11-month-old male with left-sided genu varum secondary to early-onset Blount disease underwent guided growth treatment using an extraperiosteal non-locking plate across the lateral proximal tibial growth plate. Fourteen months postoperatively, once slight valgus overcorrection of the left lower extremity was confirmed on full-length standing radiographs, the hardware was removed. At the latest follow-up 26 months postoperatively, symmetric alignment of both lower extremities was noted. Further follow-up will be needed to ensure that the limb alignment is maintained over the remaining years of skeletal growth. While there are reports of hardware failure and rebound growth in some patients with Blount disease, guided growth treatment is a viable alternative to a proximal tibial osteotomy in children with mild to S. Sabharwal (*) Department of Orthopaedics, Rutgers – New Jersey Medical School, Newark, NJ, USA e-mail: [email protected]; [email protected]

moderate deformity who have sufficient growth remaining at the ipsilateral proximal medial tibial physis.

Brief Clinical History A 3-year-11-month-old male was brought in by the parents with the chief concern of a painless progressive bowing of the left lower extremity. The child had a normal birth and developmental history and started walking independently at 1 year of age. On physical examination, the patient was thin built and had 5 of varus alignment of the left and 5 of valgus on the right knee (Figs. 1 and 2). He walked with a mild varus thrust on the left side. He had full mobility of the left knee with no joint line tenderness or abnormal ligamentous laxity. His left leg was shorter than the right side by less than 1 cm. Rotational profile at the hip was symmetric bilaterally. Internal tibial torsion was noted on the left side (thigh-foot angle: right 30 external; left 5 internal). Remaining musculoskeletal exam was within normal limits.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_37

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Fig. 1 Anterior view of the left lower extremity. Notice the mild varus alignment of the left knee and physiologic valgus of the right knee

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Preoperative Problem List (I) Genu varum and mild internal tibial torsion on the left side (II) Mild shortening (1 cm) of the left tibia (III) Anticipated increase in multiplanar deformity and gait abnormality with growth

S. Sabharwal

Fig. 2 Posterior view of the same patient at initial presentation

Surgical options include guided growth treatment and a proximal tibial osteotomy with acute or gradual correction. Due to the mild deformity and the child’s young age, guided growth treatment was chosen. While there was a mild varus deformity of the distal femur as well as the proximal tibia, only the proximal tibia was addressed in this case. An extraperiosteal plate with 4.5 mm screws (one epiphyseal, one metaphyseal) was placed across the lateral aspect of the proximal tibial physis. A knee arthrogram was also performed in order to better assess the intra-articular morphology of the knee joint and facilitate accurate placement of the hardware (Figs. 4 and 5). The patient was discharged home the same day with a knee immobilizer and allowed to weight bear as tolerated.

Basic Principles Treatment Strategy The options for treating this child are primarily surgical. The use of full-time bracing with valgus-directed force using a knee-ankle-foot orthosis with a medial upright is of questionable efficacy and not well tolerated in these young patients.

Blount disease is a developmental disorder of the lower extremity that is more common among obese children (although this child was not obese). In patients with earlyonset Blount disease, the onset of tibial bowing is noted before the age of 4 years. Due to the relative growth

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Fig. 4 An intraoperative AP radiograph of the knee demonstrating an extraperiosteal tension band plate placed across the lateral aspect of the proximal tibial physis. A knee arthrogram was also performed in order to better assess the intra-articular morphology of the knee joint and facilitate accurate placement of the hardware Fig. 3 A full-length standing radiograph of the lower extremities revealed a mechanical axis deviation (MAD) of 1 cm lateral on the right and 3.9 cm medial on the left side (Fig. 3). Lateral distal femoral angle (LDFA) was 98 on the right and 102 on the left side. Medial proximal tibial angle (MPTA) was 101 on the right and 85 on the left side. Metaphyseal breaking and changes consistent with mild form of early-onset Blount disease were noted at the left proximal tibial metaphysis. The left hemipelvis was 1 cm lower than the right side, consistent with a mild leg-length discrepancy (LLD)

inhibition of the posteromedial portion of the proximal tibial physis, a multiplanar deformity involving varus, procurvatum, and internal tibial torsion of the affected extremity is noted [2, 3]. While a proximal tibial osteotomy is often indicated for managing such patients, in children with mild deformity with less advanced stages of Blount disease, use of guided growth may allow gradual correction without the need

for an osteotomy [2, 6]. The non-locking, tension band plate is placed extraperiosteally bridging the lateral portion of the proximal tibial physis. The patient is followed with serial full-length standing radiographs to assess the mechanical axis deviation of the limb- and leg-length discrepancy. Given the possibility of recurrent deformity related to the diseased portion of the growth plate, slight overcorrection into valgus alignment is generally recommended prior to hardware removal.

Images During Treatment See Figs. 4, 5, 6, 7, and 8.

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Fig. 5 An intraoperative lateral view of the same patient

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Fig. 6 Clinical appearance (anterior view) 14 months postoperatively demonstrating intentional mild valgus overcorrection of the left lower extremity

Technical Pearls (I) Perform a comprehensive deformity analysis with fulllength standing radiograph to rule out any significant distal femoral (varus or valgus) deformity. Also assess leg-length discrepancy. Do not forget to obtain biplanar radiographs of the entire length of the affected tibia. Besides the more obvious coronal plane proximal tibial deformity, study the lateral view to assess the sagittal plane deformity (procurvatum). (II) If there is greater than 5 of varus deformity at the distal femur (much less common in early-onset than late-onset Blount disease), it is advisable to perform a lateral distal femoral hemiepiphysiodesis (using a tension band non-locking plate). This will prevent creating an iatrogenic knee joint obliquity with potential for increasing the shear stresses at the knee joint. (III) If there is a procurvatum deformity, the plate can be placed slightly off-center anteriorly across the lateral portion of the proximal tibial physis. This may help achieve a biplanar correction of the proximal tibial deformity.

(IV) In larger individuals, avoid short, cannulated screws as these can break, especially on the metaphyseal side. Occasionally, two adjacent plates, H- or I-shaped plates, and/or plates made of stainless steel may be needed in extremely obese children [1, 5].

Outcome Clinical Photos and Radiographs At the latest follow-up 26 months postoperatively: See Figs. 9, 10, and 11.

Avoiding and Managing Problems (I) Avoid any damage to the underlying periosteum when placing the extraperiosteal plate. (II) Ensure compliance with periodic follow-up. In order to diagnose recurrent deformity and also avoid extreme overcorrection, follow these children with serial fulllength radiographs (typically every 4–6 months). Even

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Fig. 7 Clinical appearance (posterior view) 14 months postoperatively demonstrating intentional mild valgus overcorrection of the left lower extremity

after hardware removal, serial follow-up to skeletal maturity is indicated. Recurrent varus deformity secondary to the diseased portion of the medial tibial physis may manifest over time. (III) Assess skeletal age when correcting angular deformities in obese children. Often, these children have precocious puberty and demonstrate an advanced bone age [4]. (IV) Guided growth treatment will not address the shortening and internal tibial torsion that is often associated with a

Fig. 8 Standing fill-length radiograph demonstrating intentional mild valgus overcorrection of the left lower extremity. The hardware from the left proximal tibia was removed as a same-day procedure

limb affected by Blount disease. If clinically significant, these deformities may need further surgical intervention. The family should be counseled regarding the possible need for more surgeries in the future.

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Fig. 9 Anterior view of the lower extremities 26 months postoperatively demonstrating symmetric alignment of both lower extremities

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Fig. 10 Posterior view of the lower extremities at 26 months postoperatively

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Cross-References ▶ 13 Year Old with Unilateral Late-Onset Blount Disease ▶ Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF ▶ Correction of Juvenile Blount’s disease ▶ Hemiplateau Elevation for Early-Onset Blount Disease ▶ Infantile Blount Disease with Plateau Depression

References and Suggested Reading 1. Burghardt RD, et al. Mechanical failures of eight-plateguided growth system for temporary hemiepiphysiodesis. J Pediatr Orthop. 2010;30 (6):594–7. 2. Sabharwal S. Blount disease. J Bone Joint Surg Am. 2009;91 (7):1758–76. 3. Sabharwal S, Lee J Jr, et al. Multiplanar deformity analysis of untreated Blount disease [erratum appears in J Pediatr Orthop. 2007 Jun; 27(4):483]. J Pediatr Orthop. 2007;27(3):260–5. 4. Sabharwal S, Sakamoto SM, Zhao C. Advanced bone age in children with Blount disease: a case–control study. J Pediatr Orthop. 2013;33 (5):551–7. 5. Schroerlucke S, et al. Failure of orthofix eight-plate for the treatment of Blount disease. J Pediatr Orthop. 2009;29(1):57–60. 6. Scott AC. Treatment of infantile Blount disease with lateral tension band plating. J Pediatr Orthop. 2012;32(1):29–34.

Fig. 11 Full-length standing radiograph demonstrating some “rebound” growth following valgus overcorrection. Less than 1 cm of left-sided limb shortening persists. Further follow-up is planned

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Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 547 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 549 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 549 Current Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 549 Future Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 550 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 550 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 550 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 551 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 553 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 553 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 553 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 555 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 555

Abstract

This is a 5 year old female with left-sided genu varum with radiographs consistent with early-onset Blount disease. The patient underwent a proximal tibial osteotomy but subsequently had a recurrence of her deformity with marked clinical genu varum. Subsequently, a CT scan and MRI were obtained that demonstrated a posteromedial bar. The patient was indicated for a proximal medial tibial hemiplateau elevation as well as a proximal lateral tibial drill epiphysiodesis and a proximal fibula drill epiphysiodesis. Postoperatively, the patient did well with F. Edobor-Osula (*) Pediatric Orthopaedic Surgery, Rutgers – New Jersey Medical School, Newark, NJ, USA e-mail: [email protected] S. Sabharwal Department of Orthopaedics, Rutgers – New Jersey Medical School, Newark, NJ, USA e-mail: [email protected]; [email protected]

the restoration of her mechanical alignment. Several years later, the patient required a contralateral tibial epiphysiodesis to prevent future leg length discrepancy.

Brief Clinical History A 4-year 11-month-old female presented with a chief complaint of left lower extremity bowing. She has a normal birth history and a normal developmental history. Initially, the patient’s mother noted bowing of both lower extremities. She stated that overtime the right lower extremity bowing resolved but the left lower extremity deformity persisted. On physical examination, the patient was noted to have a leftsided genu varum and internal tibial torsion with a thigh-foot angle of 15 internal. There was no evidence of genu varum or internal tibial torsion on the right (thigh-foot angle of +5 ). The patient had symmetric hip rotation and normal hip range of motion bilaterally. On a clinical gait analysis, there was no

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evidence of a varus thrust; however, the patient was noted to have intoeing on the left. The rest of the musculoskeletal exam was unremarkable. Preoperative full-length standing radiographs from her initial visit demonstrated a medial proximal tibial angle (MPTA) of 70 on the left. She underwent a left proximal tibial and fibular osteotomy with acute correction. Follow-up radiographs demonstrate healing at the osteotomy site on an AP (Fig. 1) and a lateral view (Fig. 2). One year after the initial surgery, at age 6, radiographs revealed a mechanical axis deviation (MAD) of 15 mm medial on the left with an MPTA of 74 . Despite adequate correction as noted on initial postoperative radiographs, the deformity recurred, and the patient was indicated for lateral proximal tibial hemiepiphysiodesis (Figs. 3 and 4). On serial radiographic and clinical evaluations done over the course of 2 years, the deformity did not improve. An MRI and CT scan of the left knee was obtained that revealed severe medial joint line depression and a posteromedial physeal bar consistent with Langenskiold stage V changes (Figs. 5 and 6). Based on the degree of proximal medial plateau depression, the decision was made to proceed with a left hemiplateau elevation.

Fig. 2 Preoperative lateral view tibia

Fig. 3 AP view after lateral proximal tibial hemiepiphysiodesis Fig. 1 Preoperative AP view tibia

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Fig. 4 Lateral view after lateral proximal tibial hemiepiphysiodesis

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Fig. 6 3D CT reconstruction showing posteromedial physeal bar

Preoperative Clinical Photos and Radiographs Preoperative clinical pictures of the child at age 8, from the front (Fig. 7) and from the back (Fig. 8), demonstrate leftsided genu varum. A full-length standing radiograph of the lower extremities revealed a MAD of 5.5 cm medial on the left and 0 cm on the right (Fig. 9). The Lateral distal femoral angle (LDFA) was 82 on the left and 89 on the right. The MPTA was 63 on the left and 87 on the right. Severe medial plateau depression was noted (Fig. 10), with a depression angle of 35 . On her scanogram, she had a leg length discrepancy measuring 1.1 cm, with the left tibia being shorter than the right.

Preoperative Problem List Current Considerations (I) Recurrent genu varum with advanced (Langenskiold stage V) (Langenskiold and Riska 1964) Blount disease (II) Severe medial joint line depression (III) Mild shortening (1 cm) of the left tibia

Fig. 5 Coronal T1-weighted MRI showing Langenskiold stage V changes

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Fig. 7 Preoperative clinical photo – front view

Fig. 8 Preoperative clinical photo – back view

Future Considerations

screws. Postoperatively, the patient was placed into a bivalved long leg cast to protect the osteotomy site and admitted overnight to observe for compartment syndrome. Future considerations: The need for a contralateral completion epiphysiodesis of the proximal tibia was discussed with the family before proceeding with the hemiplateau elevation. As the patient was 8.5 years at the time of surgery and all growth was halted on the left proximal tibia, contralateral proximal tibial epiphysiodesis at some later time would almost certainly be necessary. Furthermore, skeletal age determination using radiographs is helpful in estimating the appropriate time to perform the contralateral epiphysiodesis. This is of particular importance in children with Blount disease, as they tend to have advanced skeletal maturity [4].

(I) Relative lateral tibial plateau overgrowth (II) Relative proximal fibular overgrowth causing laxity of the fibular collateral ligament (III) Anticipated increased leg length discrepancy

Treatment Strategy This complex case highlights some of the challenges in treating a patient with early-onset Blount disease with advanced Langenskiold stages. Due to the severity of the radiographic changes and the presence of a posteromedial bar over the left proximal tibial physis, a hemiplateau elevation is indicated. The surgical plan included a knee arthrogram in order to have a better intraoperative assessment of the architecture of the medial proximal tibial plateau. To prevent continued angular deformity about the knee, a lateral proximal tibial and fibular drill hemiepiphysiodesis was also performed. The medial joint line depression was addressed with an intra-articular osteotomy. Upon completion of the intra-articular osteotomy, the joint line was elevated; a tricortical allograft was placed at the posteromedial opening wedge osteotomy that was stabilized with two fully threaded

Basic Principles Severe medial proximal tibial plateau depression is associated with irreversible damage to the physis in early-onset Blount disease. The pathogenesis of the depression is likely related to excessive compressive forces on the medial aspect of the proximal tibial physis [3]. Typically, Langenskiold stage V and VI changes are seen in children older than 10 years; however, these changes can be seen in children as

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Fig. 9 Full-length standing radiograph

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young as 6 years [1]. Once evidence of a tibial plateau depression with a physeal bar is confirmed on advanced imaging such as a CT scan or MRI, only an intra-articular osteotomy will help restore the normal articular anatomy. The goal of surgery is to prevent early osteoarthrosis of the knee by reestablishing the normal mechanical alignment of the knee and by correcting the medial plateau depression [1, 5]. The surgical approach to this complex deformity should be comprehensive. The goal should be to correct all components of the deformity, which may include posteromedial tibial plateau depression, asymmetric proximal tibial growth, a second varus deformity of the proximal tibial metaphysis, and possible valgus malorientation of the distal femur [1]. In a study by Sabharwal et al., he noted that on MRI, children with Blount disease had a thicker unossified proximal medial epiphysis and a thicker and wider medial meniscus [3]. This finding is consistent with what is commonly seen during an intraoperative arthrogram of the knee. Commonly, these patients have a component of both intra-articular and extraarticular tibial varus, both of which should be addressed (the majority of our patients’ deformity was intra-articular). One should always assess the deformity in both the coronal and sagittal planes as the tibial plateau depression tends to be posteromedial. This assessment is critical and should be made preoperatively as it will affect the placement of the lamina spreader during the plateau elevation and the placement of the bone graft. In the case of a posteromedial depression, one would want a greater degree of elevation posteriorly than anteriorly, in order to correct the medial joint line depression and the procurvatum deformity simultaneously. Asymmetric growth of the proximal tibia should be addressed by performing a completion proximal lateral tibia plateau epiphysiodesis. Simultaneously, if greater than 2.5 cm of growth remains in the proximal fibula, a drill epiphysiodesis should be performed to prevent relative fibula overgrowth and subsequent recurrence of varus deformity [5]. Finally, a distal femoral guided growth or an osteotomy may be necessary to correct the compensatory distal femoral valgus that may develop as a result of the longstanding tibial deformity [1].

Images During Treatment

Fig. 10 AP tibia demonstrating 35 depression angle

An intraoperative arthrogram (Fig. 11) demonstrating the unossified proximal medial epiphysis An intraoperative fluoroscopy (Fig. 12) demonstrating the placement of the osteotome for the medial plateau elevation An intraoperative fluoroscopy (Fig. 13) showing the medial plateau elevation and the 7.3 fully threaded cannulated screw across the osteotomy site with the tricortical allograft in place Intraoperative photos (Fig. 14) demonstrating the osteotomy site and location of placement of the allograft

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Fig. 11 Intraoperative arthrogram demonstrating the unossified proximal medial epiphysis

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Fig. 13 Intraoperative fluoroscopy showing the medial plateau elevation and the 7.3 fully-threaded cannulated screw across the osteotomy site with the tricortical allograft in place

Fig. 14 Intraoperative photos demonstrating the osteotomy site and location of placement of the allograft Fig. 12 Intraoperative fluoroscopy demonstrating the placement of the osteotome for the medial plateau elevation

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Fig. 15 Intraoperative fluoroscopy demonstrating drill but used as joystick to correct deformity

Technical Pearls (I) Start your osteotomy distal to the insertion of the medial collateral ligament. (II) Always protect the posterior neurovascular structures. (III) Make anterior-posterior drill holes to outline the intended location of the osteotomy, and then complete the osteotomy with a curved osteotome. (IV) Make apex of your osteotomy between the tibial spines and ensure that it is barely cracked, in order to prevent any intra-articular gap or step-off. (V) Place a guide wire at the medial plateau, and drill over the guide wire up to the osteotomy site. This drill bit can now be used as a joystick to correct the deformity (Fig. 15). (VI) Place a lamina spreader at the osteotomy site, and use it to elevate more posterior than anterior to simultaneously correct medial joint line depression and procurvatum deformity (Fig. 16). Once the medial plateau is elevated, provisionally fix with a Kirschner wire, and then use either a plate and/or additional screws for final fixation. (VII) Caution should be taken to avoid iatrogenic injury to the common peroneal nerve when performing the completion epiphysiodesis of the proximal fibula. One should stay anterior on the proximal fibula and use

Fig. 16 Intraoperative fluoroscopy showing lamina spreader used to elevate medial plateau depression and provisional fixation with smooth K-wire

rongeurs and curettes to complete the epiphysiodesis, rather than a drill bit.

Outcome Clinical Photos and Radiographs AP mechanical axis radiograph (Fig. 17) 3 years after surgery showing significant improvement in the overall mechanical axis of the knee [4, 6]. Clinical photos (Figs. 18, 19, and 20) 1 year after surgery demonstrating a good clinical alignment.

Avoiding and Managing Problems (I) Before performing the hemiplateau elevation, the surgeon should have a consultation with the patient and family to discuss the need for future surgery to address the leg length discrepancy that will occur. (II) Close clinical follow-up with serial scanograms (every 4–6 months) is paramount to assess for leg length discrepancy.

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Fig. 18 Front view – One year after surgery

Fig. 17 AP Mechanical Axis radiograph 3 years after surgery

(III) Options for treating leg length discrepancy include contralateral epiphysiodesis of the tibia and/or femur versus lengthening of the ipsilateral lower extremity. (IV) If proximal metaphyseal varus is also present, the surgeon may need to address the deformity either at the same time as the plateau elevation or in a stage manner with or without lengthening of the tibia.

Fig. 19 Back view – One year after surgery

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Cross-References ▶ 13 Year Old with Unilateral Late-Onset Blount Disease ▶ Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF ▶ Guided Growth Treatment for Early-Onset Blount Disease

References and Suggested Reading 1. Johnston C. Tachdjian’s pediatric orthopaedics. 4th ed; 2008. Disorders of the leg: infantile tibia vara, Elsevier 2. Langenskiold A, Riska EB. Tibia vara (osteochondrosis deformans tibiae). A survey of seventy-one cases. J Bone Joint Surg Am. 1964;46-A:1405–20. 3. Sabharwal S, Wenokor C, Mehta A, Zhao C. Intra-articular morphology of the knee joint in children with Blount disease: a case–control study using MRI. J Bone Joint Surg Am. 2012;94(10):883–90. 4. Sabharwal S, Sakamoto SM, Zhao C. Advanced bone age in children with Blount disease: a case–control study. J Pediatr Orthop. 2013;33(5):551–7. 5. Schoenecker PL, Johnston R, Rich MM, Capelli AM. Elevation of the medical plateau of the tibia in the treatment of Blount disease. J Bone Joint Surg Am. 1992;74(3):351–8. 6. Schoenecker P, Rich M. Lovell & Winter’s pediatric orthopaedics, 6th edn. The lower extremity: infantile Blount disease. Lippincott, Williams & Wilkins; 2006. p. 1170–8.

Fig. 20 Side view – One year after surgery

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Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 557 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 558 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 558 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 558 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 559 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 559 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 559 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 560 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 561 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 561 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 561

Abstract

Infantile Blount disease is challenging to treat. In young patients, brace treatment is controversial but may be worthwhile; guided growth by staples or plate may be successful, but historically, treatment has been prefer by osteotomy. Recurrence implies that the disease has been complicated by growth plate arrest or dysfunction and this aspect has to be addressed; otherwise, there will be further inexorable recurrence. The deformity may be a combination of the disease process and also postsurgical deformity from any previous procedures Fig. 1. It is particularly important to recognize medial hemiplateau deformities from those that involve the entire plateau as the management should be different. Those that only or largely involve the medial plateau require some form of hemiplateau elevation as part of the management, whereas

R. A. Hill (*) Portland Hospital for Women and Children, London, UK e-mail: [email protected]

whole plateau involvement requires reorientation of the entire plateau. Provided translation is respected; the whole plateau type is relatively easily corrected by a Taylor Spatial Frame with a metaphyseal osteotomy. Unfortunately most cases of recurrence are of the medial plateau type similar to this case.

Brief Clinical History An overweight female Caucasian patient was noted to have a bow leg, after walking was established at 11 months of age. She was kept under observation, and there was resolution on the right but not the left by the age of 4 years. She presented for specialist treatment at the age of 9 years because of apparent worsening of symptoms. At presentation, there was a unilateral genu varum with 25 of varus clinically on the left associated with 3 cms of shortening and 20 of internal rotation. The patient was otherwise well with normal biochemistry and Vitamin D levels. X-rays and an MRI scan were carried out. The X-rays suggested that the varus

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Fig. 1 Example of complex deformity – a combination of the original Blount disease and postsurgical change (not this case)

deformity was 35 but only the medial plateau was involved and the growth plate looked abnormal but the MRI scan showed no definite evidence of a bar although the appearances were abnormal suggesting some degree of dysfunction. This is a similar appearance to that seen in cases relapsing after previous surgery.

Preoperative Clinical Photos and Radiographs See Fig. 2.

Preoperative Problem List • 35 genu varum deformity due to medial plateau depression (normal lateral plateau) • 20 internal tibial torsion • 3 cms shortening • Probable dysfunction of proximal medial epiphyseal growth plate with consequences for recurrence

R. A. Hill

Fig. 2 Long leg standing film showing alignment and abnormal appearance of left medial proximal tibial growth plate. MRI scan suggests the growth plate is still open albeit abnormal

Treatment Strategy This case is a little unusual in that there is medial plateau depression with a dysfunctional but open medial growth plate, without a history of previous treatment, although this may reflect the long delay in presenting for specialist treatment. It was concluded that medial plateau elevation was indicated, but the disadvantage of this method is that a transphyseal osteotomy, albeit central, is carried out, and this of itself can cause growth arrest. In recurrent cases the growth plate is usually closed so this is not a disadvantage, and in any case in order to prevent recurrent deformity, a lateral growth plate epiphysiodesis is performed. A second distal osteotomy is carried out for the lengthening and correction of any residual deformity such as internal rotation. In most patients further treatment is indicated close to maturity for a residual leg length difference. In this particular patient, it was decided to carry out a medial plateau elevation acutely, fix this with a Taylor Spatial Frame, and then carry out a more distal osteotomy and use the frame to correct the leg length difference and internal rotation. The patient would then be

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kept under observation to see if the medial growth plate recovered, as if it did not, an expeditious lateral proximal tibial epiphysiodesis would be required followed by correction of leg length inequality. In long-standing Blount disease, it is important to assess the alignment of the distal femur as sometimes valgus is present. If this is significant it will require treatment – do not try to correct the overall mechanical axis of the leg by overcorrecting the tibial deformity as this will result in the knee no longer being parallel to the ground or ankle resulting in a shear force.

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Images During Treatment See Figs. 3 and 4.

Technical Pearls

The hemiplateau elevation can be carried out acutely using a fibula graft to hold the osteotomy open supported by a subchondral half pin. The acute method has the advantage of simplicity, but for very severe deformities, gradual elevation can be carried out [2]. It is important to recognize sagittal plane deformity as well as the coronal varus – most commonly a posterior slope, as this may also need correcting by tilting the medial plateau forwards as well as upwards. A 3D CT scan is very useful in assessing the more complex deformities [1]. This was not necessary in this case, and medial plateau elevation alone is described. With the medial plateau elevation secured by additional fixation to a Spatial Frame ring, a distal ring or rings are applied orthogonal to the distal tibia, and a proximal tibial osteotomy is carried out. The concomitant fibula osteotomy is the source of the graft to support the medial plateau elevation. Lengthening and correction of the residual deformity are then carried out.

As shown above, an intraoperative arthrogram is invaluable in determining the true joint line (Fig 3(i)). The medial articular cartilage may be thickened, and it is easy to get a wrong impression of the level of the joint line by relying on X-rays alone. The incision for the plateau elevation should be J shaped with the stem of the J centered in the midline. The periosteum is sharply dissected off the medial side of the tibial metaphysis, and an elevator introduced subperiosteal around the back of the tibia to protect posterior structures. Ideally the osteotomy should be above the medial ligament, as this will then be tightened when the plateau is elevated. The osteotomy is predrilled directly front to back with a small drill (2.5–2.7 mm). Avoid opening the knee joint if possible because keeping the Omnipaque dye in the joint is very helpful when determining the amount of elevation. A preplaced K wire can assist with the orientation of the drill. Note that the subchondral bone is left intact in the midline (as shown in Fig. 3(ii)) – this is where the osteotomy will hinge. Then complete the osteotomy with a narrow sharp Lambotte type osteotome by joining the drill holes. As it is tapped with a hammer, the note will change when the posterior cortex is cut. Gently probe with a blunt McDonald dissector to confirm the posterior cortex is cut. The plateau is elevated using a laminar spreader till the arthrogram shows

Fig. 3 (i) Intraoperative arthrogram to determine true level of medial plateau. (ii) Osteotomy has been cut but left hinging on subchondral bone in mid line. It is being opened up gradually with a laminar spreader. (iii) With the plateau elevated under arthrogram control, the joint line is

restored; fibula graft is used to support the plateau, and a large diameter half pin is used to hold plateau in correct position. This is subsequently attached to a ring. Note a preplaced Gigli saw for the proximal tibial osteotomy

Basic Principles

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the joint line is correct. An appropriate-sized piece of fibula graft is then inserted and a half pin driven across to support the osteotomy (Fig. 3(iii)). The proximal tibial osteotomy for lengthening and residual deformity correction needs to be as high as possible, and it is helpful to pre-insert a Gigli saw before elevating the plateau. This helps ensure there is

R. A. Hill

enough room for the proximal ring and a Gigli saw osteotomy should have no risk of spiraling up into the medial plateau elevation which might occur with a drill and osteotome technique. It is wise to let the tourniquet down at this point to check there has been no posterior vascular injury. The frame is then completed as shown in Fig. 4.

Outcome Clinical Photos and Radiographs

Fig. 4 Frame at conclusion of operation

Fig. 5 AP and lateral X-rays 6 months post frame removal

See Fig. 5. It is important to maintain knee movement within the limitations of the frame; partial weight bearing is permitted until the plateau elevation has healed. As the joint is not entered, knee stiffness is not a common problem. The Spatial Frame is used to correct residual varus – bearing in mind the need to translate to maintain the axis and to de rotate and lengthen the tibia. A major leg length difference is not common, but if it is intended to carry out a proximal tibial epiphysiodesis on frame removal, then the leg can be over lengthened to compensate for this. Estimation of residual growth using bone age can be helpful. Figure 5 shows the result some time after frame removal. At review 7 months post frame removal, the patient was fully active with no complaints with a stable knee with flexion of – 10 to 130 . The left leg is currently 1 cm short and remains under a close review to check for any deformity including an increase in the discrepancy.

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Avoiding and Managing Problems The commonest problems in managing severe Blount deformities in general arise from a failure to appreciate the deformity. Preoperative X-rays can be misleading because of rotation, and it is instructive to screen the knee in the theater before commencing the procedure. If the lateral plateau is normal or relatively normal, it is not logical to reorientate the entire plateau. Distal femoral valgus if present must be corrected in the femur and not the tibia. If the growth plate is open, guided growth can be used. In virtually all late presenting cases or those who have undergone previous treatment, a more distal metaphyseal osteotomy as well as plateau elevation will be required, but there is often a failure to recognize the need to translate to maintain the axis. Although it can be difficult, the more proximal the osteotomy, the less translation will be needed. Cutting the osteotomy for medial plateau elevation can cause some anxiety; placing an elevator subperiosteally around the back of the tibia is helpful; use a sharp drill when drilling the osteotomy, and be sure the drill penetrates the posterior cortex. The drill holes do have to be directly anteroposterior and should be close together. The osteotome will then easily crack the bone between the holes. Use an osteotome as described above but do not lever on the osteotome until the blunt dissector confirms complete division of the posterior cortex. The laminar

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spreader should be opened incrementally, not all at once – let the tissues take up the tension. The common peroneal nerve is not particularly at risk during this procedure unless a lot of lengthening is being contemplated; however, the surgeon may wish to decompress the nerve at the start of the procedure.

Cross-References ▶ Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF ▶ Correction of Juvenile Blount’s Disease ▶ Hemiplateau Elevation for Early-Onset Blount Disease ▶ Simultaneous Correction of Medial Proximal Tibial Plateau Depression and Tibia Vara in an Obese Child with Blount’s

References and Suggested Reading 1. Hosalker H, Jones S, Hartley J, Hill R. Three dimensional tomography of relapsed infantile Blount’s disease. Clin Orthop Relat Res. 2005;431:176–80. 2. Jones S, Hosalker H, Hill R, Hartley J. Relapsed infantile Blount’s disease treated by hemiplateau elevation using the Ilizarov frame. J Bone Joint Surg. 2003;85B:565–71.

Morbidly Obese Teenager with Significant Blount’s Treated with Taylor Spatial Frame

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Craig A. Robbins

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 563 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 563 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 564 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 564 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 565 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 565 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 565 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 566 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 566 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 567 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 567

Abstract

Options for bony deformity correction in a skeletally mature patient include acute or gradual correction with internal or external fixation. Whereas a significant multiplanar deformity may not be amenable to acute correction, external fixation allows for gradual, precise correction of concomitant deformities. As an additional benefit in a morbidly obese patient, external fixation can allow for immediate postoperative weight bearing and joint mobilization. This article describes the surgical treatment of a morbidly obese teenager with a significant multi-planar knee deformity resulting from Blount’s disease. A circular external fixator (Taylor Spatial Frame) was used to gradually and simultaneously correct significant varus, internal torsion, and procurvatum.

Brief Clinical History KG is a morbidly obese 15 year adolescent with significant bilateral knee deformity and pain who presented to my clinic. She reported that her knee pain and limp started progressing in the last few years as she reached skeletal maturity and gained significant additional weight. She also stated that her knees sometimes feel unstable and that the pain was related to the duration of walking and relieved by rest. She has never sought medical treatment. Her mother reported that she had always been overweight and had bowed knees.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

C. A. Robbins (*) Paley Advanced Limb Lengthening Institute, West Palm Beach, FL, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_6

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Fig. 1 Supine clinical picture on the operative table

Fig. 3 Standing AP of the left knee showing significant varus and internal torsion

Fig. 2 Lateral clinical picture of the knee in maximal extension; note the significant curvature distal to the tibial tubercle

Preoperative Problem List • • • •

Tibia varus Internal tibial torsion Tibia procurvatum Morbid obesity

Treatment Strategy The goal of treatment is to allow simultaneous correction of significant multi-planar deformities and allow mobilization. Because of her weight the frame has to be stable and strong. Her body habitus necessitated a redundant construct in the event of hardware failure. A fibular osteotomy was performed.

Fig. 4 Lateral view of the knee showing significant procurvatum deformity

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Morbidly Obese Teenager with Significant Blount’s Treated with Taylor Spatial Frame

Basic Principles An ideal external fixator is stable, strong, and redundant. The osteotomy was made slightly distal to the CORA to provide a longer proximal fragment to capture with the proximal ring. An initial reference wire was placed to mount the proximal ring slightly more distal than usual to allow for clearance of thigh pannus with knee flexion. This wire was removed shortly after surgery because of continued soft tissue irritation. Angled pin clamps were used distally to maximize pin spread and frame stability.

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Expect more pin and wire problems due to the significant amount of adipose tissue and difficulty stabilizing soft tissues. The frame needs to support extreme weight because protected weight bearing is difficult in morbidly obese patients. The osteotomy site needs to be completely healed prior to frame removal because casting or splinting is not possible. With extreme deformity there can be significant translation of the soft tissues within the rings. Therefore, make sure to size and position them appropriately. The

Images During Treatment See Figs. 5, 6, 7, 8, 9, and 10.

Technical Pearls High-BMI patients require additional preoperative and postoperative considerations than their normal-weight peers. Large ring sizes are needed to sufficiently clear the local soft tissues and that of adjacent limb segments with range of motion. Larger frames are inherently less stable because the increased ring diameter increases the distance between the bone and the ring. There is also increased torque on the half pins resulting from the larger distance between the bone and ring. Fig. 6 Lateral intra-operative fluoro showing osteotomy; this was performed prior to placing the last half pin to avoid propagating the osteotomy into the half-pin site

Fig. 5 AP intraoperative fluoro showing reference wire and medial and lateral half pins

Fig. 7 AP clinical view showing final frame construct

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Fig. 8 Lateral clinical view showing the final frame construct; note each ring is orthogonal to its bone segment

Fig. 10 Lateral view during treatment; notice the pin spread on the distal fragment and half pin below the tibial tubercle on the proximal fragment; again note the requisite anterior translation of the distal fragment due to the osteotomy being distal to the CORA

Outcome Clinical Photos and Radiographs See Figs. 11, 12, and 13.

Avoiding and Managing Problems

Fig. 9 AP view during treatment; notice the requisite lateral translation because the osteotomy site was purposely made distal to the CORA

open space posteriorly on the proximal ring needs to be large enough to allow knee flexion. Anticipate and prepare for multiple strut changes with extreme deformities and ensure their availability in clinic for changes when needed. DVT prophylaxis must be considered on an individual basis. Orthogonal X-rays can be difficult.

Careful preoperative consideration of the additional pitfalls inherent in surgery on obese patients is paramount. Expect and aggressively manage wound healing and soft tissue irritation around pins and wires. With extreme magnitudes of correction, consider the use of prophylactic nerve decompression (e.g., from valgus to varus). Although secondary procedures are technically possible when working around a frame in a normal-sized individual, obese patients require much larger incisions and exposure to accomplish the same task. It is also wise to have the primary care provider and anesthesiologist evaluate the patient prior to surgery. Pre- and postoperative weight loss counseling should be provided. Risks and benefits of DVT prophylaxis should be considered on an individual basis.

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Fig. 11 Lateral clinical picture showing full correction of procurvatum

Fig. 13 Full-length standing X-ray of the entire limb showing mechanical axis through the center of the knee joint

References and Suggested Reading

Fig. 12 Lateral X-ray prior to frame removal

Cross-References ▶ Adolescent Blount’s Disease Treated with MAC External Fixation System ▶ Simultaneous Correction of Medial Proximal Tibial Plateau Depression and Tibia Vara in an Obese Child with Blount’s

1. Feldman DS, Madan SS, Kova KJ, van Bosse HJP, Jamal B, Lehman WB. Correction of tibia vara with six-axis deformity analysis and the Taylor Spatial Frame. J Pediatr Orthop. 2003;23(3):387–91. 2. Feldman DS, Madan SS, Ruchelsman DE, Sala DA, Lehman WB. Accuracy of correction of tibia vara: acute versus gradual correction. J Pediatr Orthop. 2006;26(6):794–8. 3. Rozbruch SR, Fragomen AT, Ilizarov S. Correction of tibial deformity with use of the Ilizarov-Taylor spatial frame. J Bone Joint Surg. 2006;88(Suppl 4):156–74. 4. Stanitski DF, Dahl M, Louie K, Grayhack J. Management of lateonset tibia vara in the obese patient by using circular external fixation. J Pediatr Orthop. 1997;17(5):691–4.

One-Stage Hemi-Plateau Elevating Osteotomy in Advanced Blount’s Disease

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Ixchel Montoya and Panagiotis (Peter) Glavas

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 569 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 570 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 570 Treatment Strategy (Figs. 4 and 5) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 570 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 570 Images During Surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 572 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 572 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 573 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 573 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 573

Abstract

Brief Clinical History

Patients presenting with advanced stages of Blount’s disease with medial growth arrest can be treated with a hemiplateau elevation, usually combined with a metaphyseal valgus osteotomy. We present a case of advanced Langeskiöld VI infantile Blount’s disease in a 9-year-old female. She was treated with a single stage medial hemiplateau elevation and valgus producing osteotomy and a concurrent lateral tibial plateau and proximal fibular epiphysiodesis. A second metaphyseal osteotomy was not required. She had subsequent contralateral leg epiphysiodesis for leg length inequality concurrent with the removal of hardware. Follow-up showed satisfactory alignment and leg length.

The patient presented to our clinic at 9 years of age. She had progressive right genu varum since early childhood that was never treated. On exam, she was walking with a right varus thrust. Her hip and knee range of motion were normal. Clinical assessment of her rotational alignment showed normal and symmetric rotation at the hip and a thigh-foot angle of +10 degrees on the left and 0 degrees on the right. Preoperative imaging revealed that the right knee was in varus with a mechanical axis deviation (MAD) at 3 (Figs. 1 and 2). Magnetic Resonance Imaging (MRI) confirmed marked medial plateau depression and proximal tibial physeal growth arrest (Fig. 3).

I. Montoya · P. P. Glavas (*) Sainte-Justine University Hospital Center, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_583

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Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Preoperative Problem List • Marked genu varum and tibial metaphyseal-diaphyseal angle of 23 degrees • Medial physeal growth arrest • Medial plateau depression • Leg length inequality of 9 mm, with left (normal) limb longer than right

Treatment Strategy (Figs. 4 and 5) The patient underwent a hemi-plateau elevation and valgus producing open wedge osteotomy with iliac crest allograft, stabilized using a neutralization plate through a standard anteromedial approach for a high tibial osteotomy. Concomitantly, a lateral tibial and proximal fibular epiphysiodesis ipsilaterally was crucial due to her medial growth arrest. Two years post-operative, at age 11, the limb length inequality had increased to 1.6 cm. The contralateral physis remained open. Therefore, she underwent a proximal tibial and fibular epiphysiodesis of the contralateral side and concomitant removal of hardware. Fig. 1 Preoperative long length film showing a mechanical axis deviation of 3 (dotted line) and Drennan’s metaphyseal-diaphyseal angle of 23 degrees. On this image, the measured mechanical lateral distal femoral angle (mLDFA) was 87 degrees; medial proximal tibial angle (MPTA) was 70 degrees; and lateral distal tibial angle (LDTA) was 85 degrees

Fig. 2 Preoperative AP and lateral knee imaging showing the marked medial hemi-plateau depression and a near normal tibial slope. Langeskiöld VI Blount’s disease

Basic Principles For advanced Langenskiöld VI Blount’s disease, a technique described is a double osteotomy including both a hemiplateau elevation and a second metaphyseal osteotomy.

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571

Fig. 3 MRI showing physeal growth arrest and hemi-plateau depression

Fig. 4 (a, b) AP and lateral of the initial guidewire placed in an anterior to posterior direction, just lateral to the midline of the tibia. It serves as a guide and pivot point for the curved osteotome in the proximal tibial epiphysis. (c, d) Hintermann retractor serves as an external fixator to open and hold the medial tibial plateau osteotomy site. This elevates the

medial hemi-plateau and corrects the varus angulation. An iliac crest allograft is shaped to fit in the osteotomy site. (e, f) Lateral proximal tibia and fibula epiphysiodesis are completed after stabilization of the osteotomy with a standard T-plate

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Fig. 5 (a, b) AP and lateral post-operative images before and after hardware removal showing mechanical axis deviation of +1 and limb length inequality of 1.5 cm. The contralateral epiphysiodesis was performed at the time of hardware removal

The objective of treatment is to restore joint and limb alignment. In our case, the osteotomy allowed for medial hemiplateau elevation to restore the joint anatomy, and also to correct the tibial varus. Our patient had primarily a coronal plane deformity arising from the proximal tibia. The osteotomy was therefore located at the maximal angular deformity site and did not require additional correction for multiplanar deformity. The rotational malalignment and the sagittal plane deformity were deemed to be minimal and did not require correction. Due to her medial hemi-plateau growth arrest, we completed the epiphysiodesis of the lateral proximal tibia and fibula. Due to her age at presentation, we expected a limb length discrepancy of less than 5 cm. Congruent with the family’s wishes, we planned on performing a contralateral tibial and fibular epiphysiodesis at a later date concomitantly with hardware removal.

Images During Surgery See Fig. 4.

Technical Pearls We used a standard proximal anteromedial approach to the tibia, making sure to preserve the pes anserine through subperiosteal dissection. The dissection was extended posteriorly, where a Hohmann retractor was placed to protect the neurovascular bundle. A Kirschner wire was placed in the proximal tibial epiphysis to guide the osteotomy. Multiple holes were drilled using Kirschner wires, to guide the osteotomy trajectory, which was performed with a curved

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osteotome. Gentle stress-relax maneuvers were applied and a Hintermann distractor was used in an external fixator assisted fashion to correct the varus deformity and to elevate the hemi-plateau. A tricortical iliac crest allograft was shaped and placed to fit snuggly in the open wedge and stabilized with a neutralization proximal tibial T-plate. Standard lateral proximal tibial and fibular epiphysiodesis were performed under fluoroscopic guidance. Post-operatively, she was non-weight bearing for 6 weeks: two weeks with a cast, then 4 weeks with a hinged knee brace in extension when mobilizing. Non-weight bearing knee ROM was started at 2 weeks postoperatively and progressive weight-bearing at 6 weeks.

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the joint. A Hintermann retractor acting in a fixator-assisted manner helps to achieve this and maintain the correction while the appropriate allograft wedge is shaped. As the correction is performed through the physis, completion of the epiphysiodesis of the ipsilateral tibia and fibula is mandatory in a skeletally immature patient. Careful planning of the timing of the contralateral epiphysiodesis based on the predicted limb length inequality is required. Another requirement for success is that the deformity be mostly uniplanar because the hinged open wedge osteotomy allows only for single plane correction. A single proximal osteotomy can have the added benefit of reducing the risk of compartment syndrome. Intraoperatively, the neurovascular bundle needs to be protected posteriorly. This can be achieved with subperiosteal placement of a Hohman retractor posteriorly.

Outcome Clinical Photos and Radiographs See Fig. 5.

Avoiding and Managing Problems The success of this osteotomy is dependent upon the preservation of a hinge to effect the correction (both the hemiplateau elevation and varus correction). Opening of the osteotomy site must proceed carefully through multiple cycles of stress relaxation as to avoid propagation through

References and Suggested Reading 1. Gkiokas A, Brilakis E. Management of neglected Blount disease using double corrective tibia osteotomy and medial plateau elevation. J Child Orthop. 2012;6:411–8. 2. Schoenecker PL, Johnston R, Rich MM, Capelli AM. Elevation of the medical plateau of the tibia in the treatment of Blount disease. J Bone Joint Surg Am. 1992;74(3):351–8. 3. van Huyssteen AL, Hastings CJ, Olesak M, Hoffman EB. Doubleelevating osteotomy for late-presenting infantile Blount’s disease: the importance of concomitant lateral epiphysiodesis. J Bone Joint Surg Br. 2005;87(5):710–5.

Proximal Tibial Osteotomy in a 4 Year Old Child with Blount Disease

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Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 575 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 577 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 577 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 581 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 581

Abstract

This is a case of a 4 year old child with bilateral Blount disease that failed to respond to conservative treatment. She was initially seen at the age of 20 months, and a diagnosis of infantile Blount disease was made. Bilateral KAFOs treatment was initiated but she failed to respond and the deformity progressed. She was then referred to our clinic. She underwent high tibial osteotomy with acute correction of all the deformities. At 3 years follow-up, the alignment of her lower limbs was satisfactory with no recurrence of the deformity, and she was functioning normally.

Brief Clinical History This child with an established diagnosis of infantile Blount disease was seen in our clinic after failed conservative treatment in the form of braces. She had an unremarkable past medical history. Clinical examination revealed severe bowing of the lower limbs, internal tibial torsion of both tibiae (about minus 20 ), and a lateral thrust when walking. She also had metaphyseal beaking of both proximal tibiae. Radiological examination confirmed the diagnosis of infantile Blount disease with typical changes in the proximal tibia and some sloping of the medial plateau. Metaphyseal/diaphyseal angle of Drennan was 20 on the right side and 28 on the left. She was scheduled for acute correction of the deformities with a high tibial osteotomy.

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_335

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Preoperative Clinical Photos and Radiographs

Treatment Strategy

See Fig. 1.

Based on the current situation, brace treatment should be abandoned. The need for surgical treatment was discussed with the parents. The plan was to perform bilateral acute correction of the deformities by means of a proximal tibial osteotomy and fixation with Kirschner wires. The potential for recurrence of the deformity and the need for future surgeries were also discussed with the family.

Preoperative Problem List – Failed brace treatment in a 4 year old child with infantile Blount disease (upper limit of brace treatment) – Significant metaphyseal/diaphyseal angle – Internal tibial torsion

Basic Principles – If satisfactory correction with braces has not been obtained by the age of 3–4 years, proceed with surgical intervention. – It is not always necessary to use gradual correction with external fixators for correction of the deformity. Acute correction with proximal osteotomies remains a perfectably viable option. Although some authors have shown that with gradual correction, more accurate correction is obtained, others believe that there is no difference in the final outcome between acute and gradual correction. – Basic principles for acute correction include: • Fibular osteotomy • Arthrogram of the knee joint to visualize the joint line accurately • Proximal tibial osteotomy distal to the tibial tubercle • Derotation of the tibia first • Then correction of the varus (closed or open wedge) • Overcorrection into 10 of valgus

Images During Treatment See Figs. 2, 3, 4, and 5.

Technical Pearls

Fig. 1 X-ray showing the varus deformity of the proximal tibia and the metaphyseal changes and beaking

– The most important points are to prevent the potential complications of proximal tibial osteotomies and these are discussed below.

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– We always recommend to perform a fibular osteotomy. – Overcorrection into 10 valgus is necessary. – Immobilize in long leg casts in full knee extension.

Outcome Clinical Photos and Radiographs See Figs. 6, 7, and 8.

Avoiding and Managing Problems

Fig. 2 (a–c) Schematic representation of surgical steps. (a) Preoperative planning of the closing wedge osteotomy, site of the osteotomy, and approximate amount of bone to be resected. Fibular osteotomy performed (through same oblique anterolateral incision or through a separate incision). (b) Insertion of two parallel K-wires (one proximal and one distal to the osteotomy site) before proceeding with the derotation, to accurately mesasure the amount of derotation. (c) Following the derotation, a wedge of bone is removed with a lateral base to allow overcorrection into 10 of valgus

– Avoid neurovascular injuries by meticulous attention to the details of the osteotomy. The posterior aspect of the proximal tibia needs to be protected while performing the osteotomy. Flexing the knee while doing the osteotomy allows the vascular structures to fall back and away from the osteotomy. Release of the tourniquet before closure is also recommended. – Avoid compartment syndrome by performing a fasciotomy of the anterior compartment and insertion of a drain. The importance of careful monitoring and anticipation of compartment syndrome cannot be overemphasized. – Compartment syndrome remains a clinical diagnosis and should be dealt with immediately on an emergency basis. – Follow-up is absolutely necessary to detect early signs of recurrence, progression of the deformity, or development of a bony bar medially.

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Fig. 3 (a–e) Fluoroscopic pictures showing the various surgical steps. (a) Arthrogram of the knee. (b) Proximal tibial osteotomy and insertion of the 2 K-wires for assessing the degree of derotation. (c) Derotation to correct

Fig. 4 (a–c) Long standing X-rays in cast showing satisfactory alignment

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the internal tibial torsion. (d) Closing wedge osteotomy and fixation with K-wires. (e) Lateral X-rays after correction of the deformity

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Fig. 5 X-rays 2 months after surgery showing good healing of the osteotmies. K-wires ready to be removed

Fig. 6 X-rays at 1 year follow-up

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R. C. Hamdy Fig. 8 X-rays at 4 years followup, showing satisfactory alignement, reconstitution of the medial physis, and no evidence of recurrence or progression of the deformity

Fig. 7 X-rays at 3 years follow-up

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Cross-References

References and Suggested Reading

▶ 13 Year Old with Unilateral Late-Onset Blount Disease ▶ Guided Growth Treatment for Early-Onset Blount Disease ▶ Hemiplateau Elevation for Early-Onset Blount Disease ▶ Infantile Blount Disease with Plateau Depression

1. Birch JG. Blount disease. J Am Acad Orthop Surg. 2013;21(7):408– 18. Review. 2. Burghardt RD, Specht SC, Herzenberg JE. Mechanical failures of eight-plateguided growth system for temporary hemiepiphysiodesis. J Pediatr Orthop. 2010;30(6):594–7. 3. Sabharwal S. Blount disease. J Bone Joint Surg Am. 2009;91 (7):1758–76. Review. 4. Sabharwal S, Wenokor C, Mehta A, Zhao C. Intra-articular morphology of the knee joint in children with Blount disease: a case-control study using MRI. J Bone Joint Surg Am. 2012;94(10):883–90.

Simultaneous Correction of Medial Proximal Tibial Plateau Depression and Tibia Vara in an Obese Child with Blount’s

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Craig A. Robbins

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 583 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 583 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 584 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 584 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 585 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 585 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 585 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 585 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 585 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 587 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 587

Abstract

Older children with advanced stages of infantile Blount’s suffer from numerous current and future deformities. The medial proximal tibia physeal growth disturbance leads to an intra-articular deformity as well as overall genu varus, tibial torsion, and procurvatum. Premature medial physeal closure leads to axial shortening of the tibia and length mismatch with the normal growing fibula. This case details the concommitant treatment of the intra- and extra-articular deformities in an obese 8 year old child with advanced infantile Blount’s. An acute medial hemi-plateau elevation osteotomy was combined with a metaphyseal tibia osteotomy and gradual correction with a Taylor Spatial Frame. Epiphysiodesis was performed on the lateral tibia and proximal fibula physes to prevent future asymmetric growth.

Brief Clinical History AW is a 150 lb (68 kg) 8 year old female with progressive knee deformity from infantile Blount’s. When she was 3.5 years old, she had a valgus-producing metaphyseal tibia ostetomy. Since that time, her deformity has recurred, and she has activity-related knee pain. Clinically, she presents with tibia varus, tibial torsion, and lack of terminal knee extension. Radiographically, she has medial proximal tibia physeal closure with plateau depression and tibia vara.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

C. A. Robbins (*) Paley Advanced Limb Lengthening Institute, St. Mary’s Medical Center, West Palm Beach, FL, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_4

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Fig. 1 Standing AP of both lowers at age 3.5 years showing obesity, tibial torsion, and infantile Blount’s with medial plateau depression Fig. 3 Standing AP of both lowers at age 7.5 showing obesity, retained hardware, medial plateau depression, and medial physeal arrest with wide lateral joint space, tibia vara, and internal tibial torsion

Preoperative Problem List • • • • • • •

Medial physeal growth arrest with plateau depression Tibia vara Internal tibial torsion Proximal tibia procurvatum Retained hardware Obesity Need to capture fibula and perform osteoplasty for lengthening

Treatment Strategy

Fig. 2 AP view of leg at age 3.5 years after valgus overcorrection osteotomy

She has complete medial physeal closure and depression of the medial proximal tibia plateau, but the lateral physis and fibula are still open. The tibia is shorter than the contralateral side. The plan is to realign the joint surface with an acute opening wedge hemi-plateau elevation osteotomy and to gradually correct the tibial torsion, procurvatum, and tibia vara through a metaphyseal osteotomy with a Taylor Spatial Frame. In order to prevent recurrent deformity, a lateral

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proximal tibia and fibula epiphysiodesis will be performed. A fibular osteoplasty and proximal and distal capture of the fibula will allow overlengthening of the tibia and fibula to account for a portion of the predicted axial shortening.

Basic Principles Proximal tibia joint alignment and stability will be obtained through an acute medial hemi-plateau elevation osteotomy. Structural bone graft is used with internal hardware to stabilize the osteotomy. Because of the magnitude of expected axial deficiency, gradual distraction is the preferred treatment rather than contralateral epiphysiodesis. External fixation will also allow correction of tibial torsion, procurvatum, and varus. Capturing the fibula proximally and distally is necessary to allow concurrent lengthening of the fibula previous. Hardware will be removed at this procedure.

Images During Treatment See Figs. 4, 5, 6, and 7.

Technical Pearls It’s important to recognize the multiple sites of deformity: posteromedial depression of medial hemi-plateau, and varus, procurvatum, and internal torsion of the proximal tibia.

Fig. 5 Intraoperative AP fluoro showing medial plateau elevation stabilized with laminar spreader and impacted tri-cortical iliac crest allograft; note the loss of medial dye pool and the colinear tibial plateaus

Although acute correction of the later deformities is theoretically possible, because of her age and predicted limb length discrepancy, the best option is for gradual deformity correction with circular external fixator. The fibula must be captured proximally and distally to allow concommitant fibular lengthening. A consideration for structural bone graft of the medial plateau is fibular diaphyseal autograft taken at the time of fibula osteoplasty. Tensioned wires could be considered to support the medial tibial plateau but, due to her body habitus and fear of soft tissue irritation and wire failure, interal fixation was used. I limited her postoperative weight bearing for 6 weeks to allow healing of the plateau osteotomy.

Outcome Clinical Photos and Radiographs See Figs. 8 and 9.

Avoiding and Managing Problems

Fig. 4 Intraoperative semi-oblique arthrogram showing maximal plane of depression (posteromedial); note the medial dye pooling and the amount of unossified cartilage medially

Preoperative recognition of current and predicted deformities is paramount to successfully planning and correcting complex deformities. Due to the multiple locations and magnitudes of deformities, a double osteotomy solution was chosen. Surgical closure of the unaffected lateral tibia and proximal fibula is required to prevent recurrence and overgrowth, respectively. Protected weight bearing in the post-op period is necessary to allow adequate healing of the medial plateau osteotomy. Allowing for adequate soft tissue

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Fig. 6 Intraoperative AP (a) and lateral (b) fluoro images showing stabilization of the medial plateau osteotomy and lateral proximal tibia hemi-epiphysiodesis; on the lateral, note the more posterior placement of bone graft to elevate the posteromedial depression; the smaller screw is stabilizing the bone graft and capturing the proximal fibula to allow fibular lengthening with the external fixator

Fig. 7 Postoperative AP (a) and lateral (b) images during consolidation phase; note the requisite metaphyseal translation to align the tibial mechanical axis; note the level tibial joint line on the lateral view; note the size of the distal ring to allow soft tissue clearance

clearance with large rings is especially important in obese patients to avoid internal ring impingement as soft tissues migrate within the ring during deformity correction.

Preoperative and postoperative weight loss counseling should be performed as well.

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Fig. 8 Standing AP of both lower extremities; note the level proximal tibia joint line, incorporation of the structural allograft, closure of the lateral proximal tibia physis, normal station of the proximal fibula, correction of the tibial torsion, and overlengthening of the entire leg to account for some of the predicted axial discrepancy. Also note her obesity

Fig. 9 Lateral X-ray of the leg

References and Suggested Reading

Cross-References ▶ Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF ▶ Correction of Adolescent Tibia Vara Without Fibular Osteotomy and Without Fixation of the Fibula Using the Taylor Spatial Frame (TSF) ▶ Hemiplateau Elevation for Early-Onset Blount Disease ▶ Infantile Blount Disease with Plateau Depression

1. Bar-On E, Weigl DM, Becker T, Katz K. Treatment of severe early onset Blount’s disease by an intra-articular and a metaphyseal osteotomy using the Taylor Spatial Frame. J Child Orthop. 2008;2 (6):457–61. 2. Feldman DS, Madan SS, Koval KJ, Van Bosse HJP, Bazzi J, Lehman WB. Correction of tibia vara with six-axis deformity analysis and the Taylor spatial frame. J Pediatr Orthop. 2003;23(3):387–91. 3. Rozbruch SR, Fragomen AT, Ilizarov S. Correction of tibial deformity with use of the Ilizarov-Taylor spatial frame. J Bone Joint Surg. 2006;88(Suppl 4):156–74. 4. Sabharwal S. Blount disease. J Bone Joint Surg. 2009;91(7):1758–76. 5. Stanitski DF, Dahl M, Louie K, Grayhack J. Management of lateonset tibia vara in the obese patient by using circular external fixation. J Pediatr Orthop. 1997;17(5):691–4.

13 Year Old with Unilateral Late-Onset Blount Disease

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Folorunsho Edobor-Osula and Sanjeev Sabharwal

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 589 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 591 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 591 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 591 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 593 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 593 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 593 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 597 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 597 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 598 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 598

Abstract

This is a 13-year 8-month-old non-English-speaking male who recently immigrated to the United States with clinical and radiographic features consistent with late-onset Blount disease of the right lower extremity. The patient underwent lateral hemi-epiphysiodesis of both the distal femur and the proximal tibia 1 year prior to his definitive procedure. Subsequently, the patient underwent a right proximal tibial osteotomy with gradual correction using a circular frame. Postoperatively, the patient was placed on chemical prophylaxis for prevention of deep venous thrombosis and was transferred to an inpatient rehabilitation facility. Approximately 1 month after application of F. Edobor-Osula (*) Pediatric Orthopaedic Surgery, Rutgers – New Jersey Medical School, Newark, NJ, USA e-mail: [email protected] S. Sabharwal Department of Orthopaedics, Rutgers – New Jersey Medical School, Newark, NJ, USA e-mail: [email protected]; [email protected]

the frame, the patient had complete restoration of his mechanical alignment and equalization of limb lengths. Two months later the circular frame was removed.

Brief Clinical History A 13-year 8-month-old male, who recently immigrated to the United States, presented with a chief complaint of right lower extremity bowing and pain. The patient’s father first noted the right lower extremity bowing approximately 2 years prior to the office visit. On physical examination, the patient weighed 112 kg and was 181 cm tall, with a body mass index (BMI) of 34. The patient had an obvious bowing deformity about the right knee with clinical varus measuring approximately 10 compared to 3 of valgus at the contralateral knee (Figs. 1 and 2). He had a 10 flexion deformity at the right knee and a 5 flexion deformity on the left. His clinical leg-length discrepancy was measured to be about 4 cm, with the right leg shorter than the left. His thigh-foot angle was 0 on the right and 30 external on the left. On clinical gait inspection, he

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_38

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Fig. 1 Initial clinical photo – back view

F. Edobor-Osula and S. Sabharwal

was noted to have pelvic obliquity with the right hemipelvis lower than the left and a significant limp. Preoperative fulllength standing radiographs (Fig. 3) from his initial visit demonstrated a Mechanical Axis Deviation (MAD) measuring 9.4 cm medial on the right with a Medial Proximal Tibial Angle (MPTA) of 76 and a Lateral Distal Femoral Angle (LDFA) of 101 . His Joint Line Convergence Angle (JCA) measured 2 lateral on the right and his Proximal Posterior Tibial Angle (PPTA) was 71 (Fig. 4). He also had a scanogram that revealed a 3 cm length discrepancy with the right leg being shorter, and the femoral segment was responsible for 2 cm of that difference. His skeletal age was consistent with his chronologic age. Based on these findings the patient was diagnosed with Late-Onset Blount Disease. Given the patient’s social circumstances, his inability to comply with instructions for postoperative rehabilitation and his remaining growth potential, a staged approach was undertaken. First, lateral drill hemi-epiphysiodesis of the distal femur and proximal tibia (Fig. 5a, b) was used to decrease his genu varum and perhaps obviate the need for a distal femoral osteotomy. Once the patient was closer to skeletal maturity and had adjusted socially, proximal tibial osteoplasty with gradual correction and lengthening of his residual deformity was performed using the Ilizarov technique.

Fig. 3 Initial AP full-length radiograph

Fig. 2 Initial clinical photo – front view

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Preoperative Clinical Photos and Radiographs Preoperative (prior to proximal tibial osteotomy with gradual correction) clinical pictures of the boy at age14 years and 8 months, from the front (Fig. 6) and from the back (Fig. 7), demonstrating right-sided genu varum. A preoperative

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scanogram demonstrated a leg-length discrepancy of 3.3 cm, right shorter than left. A full-length AP standing radiograph (Fig. 8) of the lower extremities revealed a MAD of 7 cm medial on the right and 0.9 cm medial on the left. The LDFA was 96 on the right and 91 on the left. The MPTA was 82 on the right and 88 on the left. The lateral tibial alignment standing radiograph (Fig. 9) of the lower extremities demonstrated a PPTA of 74 on the right.

Preoperative Problem List (I) Pathologic genu varum slightly improved after lateral drill hemi-epiphysiodesis of the distal femur and proximal tibia (II) Leg-length discrepancy with the right lower extremity shorter than the left

Treatment Strategy

Fig. 4 Initial lateral tibia alignment radiograph Fig. 5 (a and b): Intraoperative photo – lateral, femoral, and tibial drill hemi-epiphysiodesis

This case illustrates one approach to a patient with late-onset Blount disease with some residual growth remaining. In this particular case, the patient was a recent immigrant without adequate social support and with a complex problem that is oftentimes better approached in a staged fashion. In this case, at presentation, the patient’s growth plates were still open which offered an opportunity for growth modulation to be used to correct some but obviously not all of the varus deformity. By using this strategy, the surgeon has the opportunity to assess patient and family compliance with this smaller, less invasive surgical technique before embarking on the much more

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Fig. 8 Preoperative AP full-length radiograph Fig. 6 Preoperative clinic photo – front view

Fig. 7 Preoperative clinic photo – back view

Fig. 9 Preoperative lateral tibial alignment radiograph

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extensive proximal tibial osteotomy and the Ilizarov technique. In addition, a lateral hemi-epiphysiodesis of the distal femur may obviate the need for a distal femoral osteotomy. In this case, the patient underwent lateral hemiepiphysiodesis of both the distal femur and proximal tibia, which decreased his deformity while providing the patient and family time to adjust to the new environment and allow a period of close observation for the treating surgeon. Subsequently, once the surgeon and patient were comfortable with the treatment plan and the patient became conversant in English, a proximal tibial osteotomy using gradual correction via the Ilizarov technique was introduced as the definitive treatment plan. The circular frame was left in place for approximately 3 months with restoration of the mechanical axis as well as equalization of limb lengths. Subsequently, after consolidation of the regenerate, the frame was removed.

Basic Principles Late-onset Blount disease is a multifactorial disorder, often associated with obesity [9, 11, 16]. It is characterized by progressive varus deformity of the leg with or without knee pain. The adolescent (late-onset) form of the disease can be unilateral, bilateral, or asymmetric [1]. The clinical features of the disease include varus deformity of the proximal tibia, and often the distal femur, variable internal tibial torsion, and proximal tibial procurvatum deformity. This disease is seen commonly in obese African-American children as well as in children of Scandinavian descent [12]. Several theories have been proposed for the pathogenesis of Blount disease, most notably increased mechanical forces at the proximal tibia physis [1]. Based on the Hueter-Volkmann principle, excessive compressive forces at the proximal tibial physis cause growth inhibition and subsequently the proximal tibial deformity [6, 11]. Evaluation of these patients should include a standing fulllength anteroposterior (AP) radiograph of the entire length of both lower extremities with the patella facing forward to assess the frontal plane alignment [12]. It is important, particularly in the adolescent form of the disease, to carefully assess the contribution of deformity from not only the proximal tibia but also the distal femur [5, 11]. In a study by Gordon et al. [5], varus deformity of the distal part of the femur constituted 30 % of the genu varum deformity in patients with late-onset tibia vara. They concluded that when correction of late-onset tibia vara is planned, the surgeon should be aware that distal femoral varus could be an important component of the deformity [5]. In a subsequent study, we confirmed these findings (Sabharwal 2007). In addition to the AP radiograph, a fulllength lateral radiograph should also be obtained to assess for any sagittal plane deformity. Additionally, a scanogram and an assessment of bone age are also necessary for assessment of current and future limb-length discrepancy [12].

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Management options for an adolescent with late-onset Blount disease should be customized based on the patient’s age at presentation, magnitude of the deformity, limb-length discrepancy, surgeon’s training and expertise, and psychosocial factors [11]. According to Birch [1], no documented report of attempted bracing for adolescents with late-onset Blount disease exists in the literature [1]. In theory, based on the larger size of these patients, it seems bracing would be ineffective. Surgical management of these patients should include a combination of the following techniques: Lateral hemi-epiphysiodesis (guided-growth) and proximal tibial metaphyseal osteotomy with either acute correction with internal versus external fixation or gradual correction with an external fixator. The choice of surgery and implant is again influenced by the patient’s age, magnitude of the deformity, whether a limb-length discrepancy is present, surgeon’s preference, and social factors. The Taylor Spatial Frame (Smith and Nephew, Memphis Tennessee) allows the surgeon to perform six-axis deformity correction on the basis of a computer-generated schedule and has improved the accuracy of correction [2, 3, 7, 11]. Several studies have shown that when compared to acute correction, gradual correction with a circular frame allows for less residual mechanical axis deviation, sagittal plane angulation, translational deformity, and limb-length discrepancy [4, 8]. While this patient did have mild residual varus malorientation of the femur (LDFA 96 ) following lateral distal femoral hemiepiphysiodesis, we decided to overcorrect the proximal tibia varus instead of performing a concomitant distal femoral osteotomy. With any greater distal femoral varus, we typically perform a distal femoral osteotomy as well (to avoid excessive joint obliquity and generation of shear forces at the knee joint).

Images During Treatment See Figs. 10, 11, 12, 13, 14, 15, and 16.

Technical Pearls (I) Position patient on a fully radiolucent operating room table. (II) Prior to prepping and draping, place a bump under the ipsilateral hip to ensure that the ipsilateral patella is facing toward the ceiling. (III) Use a sterile tourniquet. (IV) If greater than 2 cm of lengthening of the tibia is planned, the authors’ preference is to stabilize the distal tibiofibular joint with a fully threaded syndesmotic screw to prevent proximal fibula migration with gradual lengthening of the tibia. (V) Exsanguinate the extremity with an elastic wrap and inflate the tourniquet to 100 mmHg above the patient’s systolic blood pressure.

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Fig. 10 (a and b) Intraoperative fluoroscopy demonstrating 4.5 mm fully threaded cannulated screw across the distal tibiofibular joint and subsequent distal fibula osteotomy

Fig. 11 (a and b) Intraoperative fluoroscopy demonstrating preparation for multiple drill hole proximal tibial osteotomy

(VI) Osteotomize the distal fibula at the junction of middle and distal third. The authors’ preference is to remove a 1 cm piece of bone to prevent premature consolidation of the distal fibula osteotomy. (VII) Make a 3–4 cm longitudinal incision centered over the proximal tibial osteotomy site. Incise the periosteum sharply using a cruciate periosteal incision to

allow for repair after completion of the osteotomy. Avoid damaging the periosteum. (VIII) Start the transverse proximal tibial osteotomy by placing multiple drill holes (front to back) across the osteotomy site using a 4.8 mm drill bit and tissue protector. Keep the trajectory of the drill holes essentially at right angles to the posterior cortex of the

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Fig. 14 Intraoperative fluoroscopy demonstrating completion of proximal tibial osteotomy

Fig. 12 Intraoperative fluoroscopy demonstrating insertion of proximal tibial reference wire essentially parallel to the tibial plateau

Fig. 13 Intraoperative fluoroscopy demonstrating application of proximal ring with appropriate centering of the proximal (reference) ring on the proximal tibia

proximal tibia. The author finds radiolucent Hohmann retractors helpful during this step. Keep the knee semi-flexed with a bump underneath the knee, so as to let the popliteal neurovascular bundle fall back from the proximal tibia. (IX) Do not complete the proximal tibial osteotomy until later, after you have mounted the frame and set the mounting parameters. (X) Deflate the tourniquet at this time and pack a salinesoaked sponge at the osteotomy site. (XI) Insert proximal reference wire parallel to the proximal tibial joint line and distal to the physeal scar. (XII) Align proximal ring orthogonal to limb axis with the master tab in line with the patella and two threaded rods mounted centrally on the anterior and posterior tabs (for rotational alignment). (XIII) Ensure that the proximal ring is centered on the proximal tibia in frontal plane (ring should appear as a single line on AP fluoroscopy). (XIV) Avoid the tibial tubercle and patellar tendon when placing half pins anteromedially and anterolaterally on to the proximal ring. The use of hydroxyapatite (HA)-coated half pins are preferable. (XV) Ensure that the proximal tibial osteotomy is distal to the proximal half pins to avoid pin cutout. (XVI) Do not forget to document the strut lengths of each of the 6 struts prior to completion of the proximal tibial osteotomy. We prefer to use fast fix struts. (XVII) With adequate protection of the surrounding soft tissues, use a ½ inch straight osteotome to complete

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Fig. 15 (a and b) Postoperative AP and lateral radiographs 1 month after surgery

Fig. 16 (a–c) Clinical photos taken 3 months after surgery

the multiple drill hole osteotomy. Confirm that the proximal tibial osteotomy is complete by gentle translation of the two fragments under direct visualization. (XVIII) Confirm that osteotomy ends are realigned back to the pre-osteotomy position at the time of wound

closure. Avoid any distraction or translation at the osteotomy site. (XIX) Prophylactic anterior compartment fasciotomy is an option that some surgeons perform routinely. (XX) Insert a subcutaneous drain prior to wound closure, including re-approximation of the periosteum.

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(XXI) Place the patient into a posterior splint or a postoperative shoe with an elastic band attached to the external frame with the ankle in neutral dorsiflexion to prevent postoperative equinus contracture. (XXII) The patient is allowed to mobilize toe-touch weight bearing on the operated extremity postoperatively.

Outcome Clinical Photos and Radiographs See Figs. 17, 18, and 19.

Avoiding and Managing Problems (I) In obese adolescent patients, strong consideration should be given to giving postoperative chemical prophylaxis for the prevention of deep venous thrombosis and pulmonary embolism (15, 16). (II) Postoperatively inpatient rehabilitation is helpful prior to discharge home until the patient is able to independently ambulate and the caretakers have learned how to do strut manipulation based on the schedule provided to them at the time of discharge from the hospital. (III) Gradual correction of deformity is initiated 5–10 days postoperatively. (IV) Strut education and the strut turning schedule should be explained to the patient and family preoperatively and reinforced prior to discharge from the hospital and at each postoperative visit. Fig. 18 (a and b) AP and lateral radiographs, showing the final tibial alignment

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(V) The author provides the patient with a prescription for a 1-week course of oral antibiotics (first-generation cephalosporin) at the time of discharge, with instructions to start the oral antibiotics in case signs of a pin track Fig. 17 AP mechanical axis radiograph 1 year after surgery showing restoration of mechanical axis

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Fig. 19 (a and b) Clinical photos taken 8 months after surgery demonstrating good clinical alignment

infection appear. This allows the patient to begin a course of antibiotics at the earliest signs of infection. (VI) Pin care instructions (author’s preference is soap and water and use of half-strength hydrogen peroxide with clean cotton swabs daily) and a prescription for supplies should be given to the patient and family prior to discharge from the hospital. (VII) Close follow-up (weekly during the distraction phase) with serial radiographs should be obtained to assess progress and to ascertain whether adjustments need to be made to the strut turning schedule.

Cross-References ▶ Adolescent Blounts Treated with Acute Plateau Elevation and Metaphyseal Correction with TSF ▶ Guided Growth Treatment for Early-Onset Blount Disease

References and Suggested Reading 1. Birch JG. Blount disease. J Am Acad Orthop Surg. 2013;21:408–18. 2. Fadel M, Hosny G. The Taylor spatial frame for deformity correction in the lower limbs. Int Orthop. 2005;29:125–9. 3. Feldman DS, Madan SS, Koval KJ, van Bosse HJ, Bazzi J, Lehman WB. Correction of tibia vara with six-axis deformity analysis and the Taylor Spatial Frame. J Pediatr Orthop. 2003;23(3):387–91. 4. Feldman DS, Madan SS, Ruchelsman DE, Sala DA, Lehman WB. Accuracy of correction of tibia vara: acute versus gradual correction. J Pediatr Orthop. 2006;26:794–8.

5. Gordon JE, King DJ, Luhmann SJ, Dobbs MB, Schoenecker PL. Femoral deformity in tibia vara. J Bone Joint Surg Am. 2006;88:380–6. 6. Johnston CE 2nd. Infantile tibia vara. Clin Orthop Relat Res. 1990;255:13–23. 7. Kristiansen LP, Steen H, Reikeras O. No difference in tibial lengthening index by use of Taylor spatial frame or Ilizarov external fixator. Acta Orthop. 2006;77:772–7. 8. Matsubara H, Tsuchiya H, Sakurakichi K, Watanabe K, Tomita K. Deformity correction and lengthening of lower legs with an external fixator. Int Orthop. 2006;30:550–4. 9. Montgomery CO, Young KL, Austen M, Jo CH, Blasier RD, Ilyas M. Increased risk of Blount disease in obese children and adolescents with vitamin D deficiency. J Pediatr Orthop. 2010;30(8):879–82. 10. Raffini L, Horn D, Dormans J, Manno C. Deep vein thrombosis and pulmonary embolism after orthopaedic surgery in children’s hospital. Presented as an E-poster exhibit at the annual meeting of the Pediatric Orthopaedic Society of North America, 29 Apr–3 May 2008; Albuquerque. 2008. 11. Sabharwal S. Blount’s disease. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: Taylor and Francis; 2006. p. 511–20. 12. Sabharwal S. Current concepts review: Blount disease. J Bone Joint Surg Am. 2009;91:1758–76. 13. Sabharwal S, Passannante MR. Venous thromboembolism in children: preliminary results of a survey of POSNA members. J Pediatr Orthop. 2013:1–5. 14. Sabharwal S, Zhao C, McClemens E. Correlation of body mass index and radiographic deformities in children with Blount disease. J Bone Joint Surg Am. 2007a;89(6):1275–83. 15. Sabharwal S, Lee J Jr, Zhao C. Multi-planar deformity analysis of untreated Blount disease. J Pediatr Orthop. 2007b;27(3):260–5. 16. Thompson GH, Carter JR, Smith CW. Late-onset tibia vara: a comparative analysis. J Pediatr Orthop. 1984;4(2):185–94.

Part VI Pediatric Deformity: Pediatric Arthrogryposis

Pediatric Arthrogryposis: An Introduction

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Reggie C. Hamdy

Arthrogryposis is a group of disorders characterized by multiple joint contractures, muscle atrophy, and muscle weakness present at birth. The word “arthrogryposis” is derived from the Greek language. Arthro, which means joint, and gryp, which means curved, describes the multiple congenital contractures. Most individuals with arthrogryposis have normal or above normal intelligence and normal sensation. Arthrogryposis can be seen in isolation or in association with other congenital abnormalities as part of a syndrome with or without central nervous system involvement. There are over 300 different syndromes associated with congenital contractures, the most common being amyoplasia. This term translates to “no muscle growth” (a ¼ no, myo ¼ muscle, plasia ¼ growth). It is the most common form of arthrogryposis and represents one third of all cases. Most affected individuals have all four limbs involved, including the feet (most common site affected), knees, and hips. The contractures are usually most severe at birth. The goals of treatment are to improve the function of these children specifically regarding mobility, self-care, and daily activities and to render them as independent as possible. Management of these children should be in multidisciplinary clinics. Treatment should start immediately after birth and includes intensive physiotherapy and bracing for both upper and lower limb deformities. Aggressive surgery is warranted as many of these have the potential to improve their function, even if the contractures have a known tendency for recurrence. In general, mild contractures can be addressed with intense physiotherapy and bracing. Moderate deformities can be addressed with soft tissue releases and growth modulation, especially around the knees. Severe deformities are best corrected with bony procedures. Very severe and recurrent deformities may also be addressed with gradual correction with external fixators. R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

In this section, 11 cases of Arthrogryposis with various scenarios are discussed (Table 1). Foot problems are very common in this condition (73, 74, 75, 76, 77). Like idiopathic clubfoot, management of clubfoot deformities in children with arthrogryposis should start immediately after birth. Tenotomy of the tendo-Achilles is now recommended before the start of manipulation and casting. Even when the child presents late (9 years old in case 73), it is still advisable to try the modified Ponseti techniques first before open surgery. If manipulation and casting fail, then a posteromedial release (PMR) is the next logical step (case 82). With recurrence of the deformities, the foot becomes more rigid and a repeat PMR is generally not recommended because it can be dangerous. If the foot is rigid but the deformity is mild, then acute correction through osteotomies can be performed. However, most recurrent cases have rigid deformities and extensive scarring. In this situation, gradual correction with the use of external fixators remains the best option. This gradual correction may be performed through soft tissues only without osteotomies (case 74) or in more severe cases through a midfoot osteotomy using a standard Ilizarov frame (case 75) or Taylor Spatial Frame (TSF) (case 76). Further surgical interventions may be necessary after removal of the frame, such as a calcaneal osteotomy for residual heel varus (case 76). In cases of congenital vertical talus, the reverse Ponseti technique described by Dobbs is the first line of treatment, and if unsuccessful, then a one-stage extensive release is necessary. However, if the deformities are very rigid, talectomy could be an option (case 77). Knee flexion contractures are much more common than extension contractures. Their management is extensively discussed, either as an isolated entity (cases 78, 79) or as part of a generalized plan for the treatment of multiple deformities (cases 80, 81, 83). Initial treatment of knee flexion contractures should start with physiotherapy, bracing, and corrective casting. Growth modulation by anterior hemi-epiphysiodesis of the distal femur is not always successful (case 78). Acute correction with supracondylar osteotomies (with or without

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Table 1 Details of the 11 Pediatric Arthrogryposis cases discussed in this Atlas Case 73

75

Diagnosis 9 year old girl, severe clubfeet deformities 4 year old girl, severe clubfeet deformities 9 year old girl, rigid feet deformities

76

12 year old boy, rigid feet deformities

77

18 month old girl, severely involved, very weak muscles, bilateral vertical tali, and dislocated hips 10 year old boy, severe knee flexion contractures 16 year old female, pterygium syndrome

74

Problems Severe relapses post posteromedial releases Severe recurrent deformities after two posteromedial releases Recurrence after posteromedial releases, rigid supination deformity Recurrence after PMR and Ilizarov correction

80

14 year old male, pterygium right lower limb

81

5 year old girl, severe bilateral knee and hip flexion contractures

Rigid vertical tali, problems wearing shoes, rigid dislocated hips with very limited abduction Recurrence after supracondylar osteotomies at age of 5 years Knee flexion contractures, patellar subluxation, rotatory knee subluxation Severe knee flexion contracture. Dislocated patella. Multiple upper limb and feet deformities Unable to stand because of severe flexion contractures

82

4 year old boy, bilateral clubfeet and hip dislocation

Rigid clubfeet, unilateral dislocation left hip

83

4 year old girl, no previous surgeries

Clubfeet, knee flexion contractures, hip flexion contractures

78 79

shortening) is the next option (case 81). Remodeling at the site of the correction occurs rapidly and recurrence of the deformity is almost the rule (case 78). Gradual correction with external fixators remains the last option. This could be performed using the fixator only without simultaneous posterior soft tissue releases (case 78) or with soft tissue releases including hamstrings and posterior knee capsule (case 79). Placement of the hinges anterior to the center of the knee joint is crucial in order to prevent posterior subluxation of the knee and also to prevent compression of the articular cartilage of the femur and tibia. Initial distraction of the knee joint prior to proceeding with correction of the flexion deformity is recommended. This gradual correction could be performed with standard Ilizarov frame or a TSF (cases 78, 79, 80). Pterygium deformities are the most challenging of the knee contractures to treat. Options include gradual correction with soft tissue releases (case 79) or extensive surgery including excision of the fibrous band (the pterygium) from the ischium to the ankle, multiple Z-plasties of the skin, and then gradual correction with a circular frame (case 80). In such severe and long-standing knee deformities, the patella may be dislocated and require to be relocated (cases 79, 80). Hip problems in children with arthrogryposis include contractures (cases 77, 81, 83) that may interfere with ambulation

Surgery and key points Successfully treated with modified Ponseti technique of casting Gradual correction through soft tissues only, with pediatric Ilizarov and TSF. No bony procedures Midfoot osteotomy and gradual correction with Ilizarov frame Midfoot osteotomy with Gigli saw, gradual correction with TSF, fixation of toes to frame, calcaneal osteotomy to correct heel varus after removal of TSF Bilateral talectomies at age of 18 months and bilateral proximal femoral valgus osteotomies at age of 5 years, to help toileting and hygiene Gradual correction with Ilizarov fixator. No soft tissue releases Soft tissue releases, gradual correction with Ilizarov frame Hinges placed anteriorly to prevent subluxation of knee Gradual correction of knee flexion contracture with excision of the pterygium from ischium to ankle, soft tissue releases, and Ilizarov fixator Simultaneous correction of all deformities with proximal femoral extension osteotomies and distal femoral supracondylar osteotomies Treatment of feet first, tenotomy, and Ponseti technique. Failed, PMR. At age of 18 months, open reduction hip, femoral shortening, and DEGA osteotomy Feet first, at age 18 months, address hip with proximal femoral osteotomies, knees addressed at 4 years, Ilizarov

and function. They are best addressed with proximal femoral osteotomies. Hip dislocations are usually teratologic in nature and most often fail to respond to Pavlik harness or closed reduction (case 81). In unilateral cases, an open reduction with femoral shortening and pelvic osteotomy is often required (case 82). In cases with bilateral dislocated hips, the treatment depends on the degree of stiffness. If the dislocations are very rigid, then open reduction is generally not indicated. Surgical intervention is only indicated if there is a functional problem specifically caused by this deformity as in case 77, where the loss of abduction causing difficulty with hygiene and toileting. In such cases, only proximal femoral valgus osteotomy – without attempting to relocate the hip – is indicated. In cases with contractures affecting several joints and several limbs (cases 77, 80, 81, 82, 83), it is important to put forward a plan of treatment and define the goals. There is almost universal agreement that the feet should be addressed first, while continuing physiotherapy and bracing for the knees and hips (cases 82, 83). Surgery for hip dislocations – if indicated – is usually performed around the age of 18 months, and knee flexion contractures are addressed around the age of four years (case 83). Physiotherapy and bracing remain the cornerstone for the successful management of any child with arthrogryposis.

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References and Suggested Reading 1. Brunner R, Hefti F, Tgetgel JD. Arthrogrypotic joint contracture at the knee and the foot: correction with a circular frame. J Pediatr Orthop B. 1997;6(3):192–7. 2. Eidelman M, Katzman A. Treatment of arthrogrypotic foot deformities with the Taylor Spatial Frame. J Pediatr Orthop. 2011;31(4):429–34. 3. Fassier A, Wicart P, Dubousset J, Seringe R. Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity. J Child Orthop. 2009;3(5):383–90.

603 4. Hamdy RC, Dahan-Oliel N. Arthrogryposis. In: Sabharwal S, editor. Pediatric lower limb deformities: principles and techniques of management. New York: Springer; 2016. 5. VanBosse HJP, Feldman DS, Anavian J, et al. Treatment of knee flexion contractures in patients with arthrogryposis. J Pediatr Orthop. 2007;27:930–7. 6. Yang SS, Dahan-Oliel N, Montpetit K, Hamdy RC. Ambulation gains after knee surgery in children with arthrogryposis. J Pediatr Orthop. 2010;30(8):863–9.

Bilateral Congenital Vertical Talus and Dislocated Hips in a Child with Very Severe Arthrogryposis

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Noe´mi Dahan-Oliel and Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 605 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 606 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 606 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 607 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 607 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 607 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 607 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 607 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 607 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 608 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 609

Abstract

This case is not about surgical techniques, but rather about the indications for surgery and the general approach to children with severe arthrogryposis. This is a child with very severe arthrogryposis who almost did not have any active movements in her lower limbs. She had bilateral congenital vertical tali that were very rigid and bilateral stiff dislocated hips. Her potential for ambulation was very poor, and she was operated only to improve her quality of life. At the age of 18 months, she underwent bilateral talectomies to enable her to wear braces and shoes. At the age of 5 years, she underwent bilateral valgus

N. Dahan-Oliel (*) Clinical Outcomes, Shriners Hospital for Children, Montreal, QC, Canada e-mail: [email protected] R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

osteotomies of the proximal femur to facilitate toileting and local hygiene.

Brief Clinical History This patient was seen initially in our clinic, soon after birth with a diagnosis of arthrogryposis. She had generalized upper and lower limb stiffness, bilateral congenital vertical talus that were very rigid, and very stiff bilateral dislocated hips. Aggressive physiotherapy was immediately initiated. However, she exhibited almost no active movements in her lower limbs. The prognosis for independent ambulation was very poor, and it was decided to proceed with surgery only if that surgery could improve her quality of life. At the age of 18 months, extensive soft tissue release of the right foot was performed as well as right talectomy. The left foot was then treated with the same procedure 2 weeks later. At the age of 5 years, bilateral valgus osteotomies were performed to improve abduction and hygiene care. The patient had no movement of the lower extremities, could not ambulate, and

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Fig. 1 (a–c) Preoperative radiographs showing severe congenital vertical tali

was dependent for all transfers. At 13 years of age, she had posterior spinal instrumentation and fusion for a severe progressive scoliosis. At the age of 15 years, the patient is pain free, wheelchair dependent, and has no difficulties wearing shoes or braces and has no problems with toileting and local hygiene. She ambulates in a motorized wheelchair.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List

Fig. 2 Radiograph showing high dislocation of both hips with no abduction

• During infancy and early childhood: • Bilateral very rigid congenital vertical talus, with difficulty wearing braces • Bilateral stiff dislocated hips with no abduction, rendering toileting a challenge • Generalized upper and lower limb stiffness

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• During adolescence: • Lumbosacral pelvic deformity

Treatment Strategy The goal in the management of this severely handicapped child is not to treat the multiple deformities and dislocations simply because they are present. In such patients, with almost no potential for ambulation (whether dependent or independent) and poor upper limb function, surgical interventions are indicated only to improve quality of life and basic daily needs. Furthermore, in wheelchair-dependent patients, correction of knee flexion deformities is indicated only if such deformities interfere with positioning and sitting. Therefore, in this patient, the two main problems were the severe foot deformities that precluded the uses of braces and shoes and the severe hip deformities that interfered with local hygiene (no abduction). The treatment plan was to address both of these issues, to render the feet plantigrade, and to increase the abduction of the hips.

Basic Principles – Bilateral dislocated stiff hips in arthrogryposis usually are not surgically reduced. – However, if there is limited abduction of the hips interfering with personal care, then valgus osteotomies are indicated, as presented in this case. – The management of vertical talus in arthrogryposis often requires talectomy if the deformity cannot be fully corrected by soft tissue releases. – In very stiff feet and recurrent deformities, surgical options include excision of the navicular, talectomy, multiple osteotomies, and gradual correction with an external fixator. – If the patient is near skeletal maturity and has severe deformities, then a triple arthrodesis may be considered. – This case shows treatment with valgus osteotomies for the hips and talectomy for the feet.

Fig. 3 Intraoperative X-ray showing satisfactory position of the calcaneum underneath the tibia

– If there is difficulty with local hygiene, then a simple valgus osteotomy to allow toileting should be performed, with no attempts at reducing the dislocation. – If the feet are very rigid, do not hesitate to perform a talectomy. The goal is to obtain a plantigrade, braceable foot. Attention to operative details, specifically the positioning and stabilization of the os calcis beneath the tibia, is important.

Outcome Clinical Photos and Radiographs Images During Treatment

See Figs. 5 and 6.

See Figs. 3 and 4.

Avoiding and Managing Problems Technical Pearls – In this case, bilateral stiff dislocated hips should not be reduced, as they will remain stiff after the reduction.

Discuss the treatment strategy with the family, specifically so that they do not have unrealistic expectations. Avoid being overaggressive with hip surgery and do not attempt to reduce the very stiff hips.

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Fig. 5 (a, b) At 5 years follow-up, showing satisfactory position of the tibia over the calcaneum

Fig. 4 (a, b) Intraoperative X-rays showing valgus osteotomy of both hips

Cross-References ▶ Recurrent Knee Flexion Contractures in a 10 Year Old with Arthrogryposis ▶ Simultaneous Correction of Hip and Knee Flexion Contractures in a 5 Year Old with Arthrogryposis

Fig. 6 At 3 years post-valgus osteotomy, showing increased abduction at the hips

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References and Suggested Reading 1. Asif S, Umer M, Beg R, Umar M. Operative treatment of bilateral hip dislocation in children with arthrogryposis multiplex congenita. J Orthop Surg (Hong Kong). 2004;12(1):4–9. 2. Cassis N, Capdevila R. Talectomy for clubfoot in arthrogryposis. J Pediatr Orthop. 2000;20(5):652–5.

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3. Daher YH, Lonstein JE, Winter RB, Moe JH. Spinal deformities in patients with arthrogryposis. A review of 16 patients. Spine (Phila Pa 1976). 1985;10(7):609–13. 4. Fassier A, Wicart P, Dubousset J, Seringe R. Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity. J Child Orthop. 2009;3(5):383–90. 5. Hamdy RC, Dahan-Oliel N. Arthrogryposis. In: Sabharwal S, editor. Lower limbdeformities in children: principles and techniques of management. New York: Springer; 2016.

Correction of Arthrogrypotic Clubfoot Deformities with the Ponseti Method of Serial Casting

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Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 611 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 611 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 612 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 612 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 613 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 614 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 614 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 615 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 616 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 616 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 616

Abstract

A 9 year old girl with arthrogryposis multiplex congenita was adopted from China with bilateral clubfoot deformities and severe relapses following prior posterior medial releases. She had 30–40 equinus contractures, with moderate to severe heel varus, and >45 supination and adductus and was wheelchair dependent. She was successfully treated with a modified Ponseti technique of serial casting. The modifications included an earlier percutaneous Achilles tenotomy, generalizing pressure over the anterolateral ankle rather than over just the lateral head of the talus, and specialized posttreatment AFOs, to slow the possibility of relapse.

Brief Clinical History The patient was 9 years old when we first met her. She was brought to the USA from a Chinese orphanage by her adoptive family a month prior. Her bilateral clubfoot deformities had undergone extensive soft tissue releases at about 3 years of age. Up until a few months prior to the initial clinic visit, she had been ambulatory but had become wheelchair dependent. She had 30–40 equinus contractures bilaterally, with heel varus >20 on the right, >45 on the left. Supination and adduction were both >45 bilaterally, with a deep cavus.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

H. J. P. van Bosse (*) Shriners Hospital for Children, Philadelphia, PA, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_358

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Fig. 1 (a–e) Precasting photographs of bilateral feet. (a–c) Multiple views of bilateral feet, demonstrating the severe heel varus, midfoot supination, and adductus. (d, e) Plantar and medial views of the left foot

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with the deep medial crease and cavus. Note the resolving lateral midfoot callus from prior weightbearing over this aspect of the foot. The prior longitudinal surgical scars are visible bilaterally

Fig. 2 (a–b) Precasting radiographs. (a) Lateral view of bilateral feet. Note the severe cavus. (b) AP views of the left foot. The forefoot is medially rotated approximately 90 compared to the talus

Preoperative Problem List

Treatment Strategy

• Rigid clubfoot deformities – Arthrogrypotic clubfoot deformities – History of prior complete soft tissue clubfoot releases • Short foot length • Moderate to severe equinus contractures

The objective in correcting the arthrogrypotic clubfoot is to convert a rigid deformity into a plantigrade, braceable foot, suitable for weightbearing, with only limited surgical intervention. The Ponseti method of serially casting has proven to be highly effective in correcting idiopathic clubfoot deformi-

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Fig. 3 (a, b) A bone model showing how, in a severe clubfoot, pressure directed laterally on the medial side of the foot, perpendicular to the axis of the tibia, will serve initially to dorsiflex the first metatarsal, necessarily exaggerating the midfoot supination to match the hindfoot varus

ties [2] and is among the best options for relapsed clubfeet, even after surgical releases [3]. The underlying concept of the technique is to rotate the calcaneo-pedis block of the foot externally relative to the talus, using the lateral talar head as a fulcrum. A percutaneous Achilles tenotomy is performed once the only remaining deformity is the ankle equinus. For the arthrogrypotic clubfoot, the technique is modified by performing an Achilles tenotomy earlier, even initially, if the equinus is severe enough to limit the heel from rolling out of the varus; a second Achilles tenotomy is then occasionally necessary at the end of treatment to complete equinus correction.

Basic Principles The initial manipulation is to externally rotate the forefoot, pushing at the mid-level of the first metatarsal, with the force perpendicular to the shaft of the tibia (Fig. 3a and b). Initially, due to the severe supination, that force will actually seem to exaggerate the supination but will actually be correcting the medial foot cavus by addressing the relative plantar flexion of the first metatarsal. In children older than babies, especially those with the increased rigidity of a teratologic clubfoot and/or surgical scarring, direct pressure over the lateral head of the talus may cause skin breakdown. I prefer instead using the ankle mortise to control the talus, placing the flat of my hand over the anterolateral ankle to act as the fulcrum, avoiding pressure over the fibular (to prevent skin injury) and the calcaneocuboid joint (to prevent inadvertent blocking of correction) (Fig. 4). As treatment progresses, precasting

Fig. 4 To prevent excessive skin pressure over the lateral head of the talus, in older children and/or stiffer feet, pressure is generalized over the anterolateral ankle while molding the cast

stretching focused at realigning the lateral talonavicular joint may help to increase the efficiency of casting (Fig. 5a and b). Classically, once the heel rolls into the valgus, a percutaneous Achilles tenotomy is performed through a stab incision just medial to the most palpable region of the Achilles tendon. In the severe clubfoot, the tenotomy is performed earlier if it is determined that the calcaneus is showing no progression toward rolling out of the varus.

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Images During Treatment See Figs. 3, 4, 5, and 6.

Technical Pearls The casting is divided into a short leg cast first, to allow for focused molding of the foot, followed by extending to a long leg cast. Only once the heel has rolled into valgus can a short Fig. 5 (a, b) Precasting stretching of the lateral talonavicular joint. In the images, the practitioner’s left thumb is over the lateral head of the talus, the right thumb over the lateral navicular, and the right hand is abducting and sliding the forefoot laterally

Fig. 6 (a–d) Photographs after eight sets of casts. (a) Bilateral medial feet and (b, c) AP and plantar view of the left foot just prior to Achilles tenotomy. Note the continued heel severe varus. (d) Photograph of the left foot just after Achilles tenotomy, with improvement of the heel varus. Percutaneous incision is visible

H. J. P. van Bosse

leg cast be considered. After the completion of the casting series, the foot should be braced with a custom-molded ankle-foot orthosis (AFO) with correction of the hindfoot varus and forefoot adductus; the anterior ankle strap should start inside laterally on the brace, to help roll the heel into the valgus. Nighttime dorsiflexion straps are added, to help maintain the ankle stretch. These are used throughout the growing years, to limit relapses. Relapses of arthrogrypotic clubfeet are common, regardless of the mode of correction. Relapses following casting, though, are easier to treat with repeat

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casting followed by bracing. Casting can be scheduled as often as twice per week or once every 2–4 weeks, per family convenience.

Fig. 7 (a, b) After a total of 16 casts and 4 months, casting is complete, and brace wear is started, with the dorsiflexion AFOs at night and leaf spring AFOs during the day

Fig. 8 (a–d) 12 months after completing casting. Patient is 10 years old and ambulating fully independent with AFOs. (a) Frontal view of the feet showing a mild recurrence of the forefoot adductus. (b, c) Lateral and medial views of both feet, demonstrating their plantigrade shape. (d) Plantar view of the left foot showing the mild adductus relapse and heel varus, neither of which is hindering ambulation. In the future, she may require repeat casting, probably prior to molding for her next set of braces

Outcome Clinical Photos and Radiographs See Figs. 7 and 8.

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Avoiding and Managing Problems

References and Suggested Reading

The key to success with Ponseti casting for severe clubfoot deformities is gentle persistence and an early Achilles tenotomy when progress seems to plateau. Skin injury should be treated with iodoform gauze, extra cast padding, and avoidance of repeat direct pressure over that site. Plaster or Gypsona casts are preferred, as these can be molded better to maintain the correction obtained by stretching the foot.

1. Boehm S, Limpaphayom N, Alaee F, Sinclair MF, Dobbs MB. Early results of the Ponseti method for the treatment of clubfoot in distal arthrogryposis. J Bone Joint Surg Am. 2008;90:1501–7. 2. Ponseti IV. Congenital clubfoot: fundamentals of treatment. Oxford: Oxford University Press; 1996. 3. van Bosse HJP. Treatment of the neglected and relapsing clubfoot. Clin Podiatr Med Surg. 2013;30:513–30. 4. van Bosse HJP, Marangoz S, Lehman WB, Sala DA. Correction of arthrogrypotic clubfoot with a modified Ponseti technique. Clin Orthop Relat Res. 2009;467:1283–93.

Cross-References ▶ Journey of a Child Born with Severe Arthrogryposis and Lower Limb Deformities

Correction of Severe Contractures, Pterygium and Lower Limb Deformities Caused By Arthrogryposis

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David S. Feldman and Adam M. Kurland

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 617 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 618 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 619 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 619 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 619 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 620 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 620 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 620 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 621 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 622 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 622

Abstract

Arthrogryposis is a disease that has variable associated joint contractures and muscular weaknesses. Treatment of this disorder is often determined by the underlying strength of the various muscle groups. A 14 year old male diagnosed with arthrogryposis at birth had been wheelchair bound since the age of six. He had severe contractures of the upper and lower extremities and limb deformities caused by arthrogryposis. He presented with the inability to ambulate and was unable to bring his hand to his mouth. For his lower extremity he underwent multiple releases, pterygium excision, patellar relocation, osteotomies, and external fixation. For his upper D. S. Feldman (*) Pediatric Orthopedic Surgery, NYU Langone Medical Center, New York, NY, USA e-mail: [email protected] A. M. Kurland Pediatric Orthopedic Surgery, NYU Langone Medical Center, Hospital for Joint Diseases, New York, NY, USA e-mail: [email protected]

extremities he underwent radial head excision, an outerbridge procedure, and ulnar nerve transposition. Understanding the patient’s underlying strength and potential allowed us to create a treatment plan that would ultimately allow him to independently ambulate and bring his hand to his mouth.

Brief Clinical History A 14 year old male from Bari, Italy, was diagnosed with arthrogryposis at birth and wheelchair bound since age 6. The patient had a strong desire to walk and obtain better control of and movement in the upper limbs. X-rays and physical exams revealed: • Lower extremities demonstrated active hip flexion and extension of 4/5 strength. There were 20 hip flexion contractures with the ability to flex the hip to 90 . • Severe knee flexion contractures with pterygium on the right. The left knee ranged from 45 to 95 , and the right

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_303

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knee ranged from 85 to 110 . Active flexion and extension indicated that he had enough strength to overcome gravity. Left foot had a severe fixed equinovalgus deformity, and the right ankle/foot had a fixed 30 equinus deformity with talar dome irregularity on X-ray. Shoulders atrophied leaving him unable to reach forward or above his head but he had shoulder forward elevation passively to 70 . Triceps functioned 4/5, but there was no bicep function. The left elbow was fixed in full extension, and the right elbow had a range of motion of 0 – 20 . X-rays revealed elbow irregularities and minimal joint space. Left wrist was in approximately 80 of flexion with minimal grasp strength and could be passively extended to about 50 of flexion. The right wrist was in 60 of flexion and could be extended passively to 30 . Finger grasp function was weak on the right but better than the left, and he was able to write with his right hand by maneuvering his body. He was unable to walk independently and needed full assistance.

Preoperative Clinical Photos and Radiographs See Figs. 1–4. Fig. 1 Clinical photograph of a 14 year old male with lower limb deformities and severe contractures caused by arthrogryposis

Fig. 2 Radiograph of right and left knees, tibias, and fibulas

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Fig. 3 Radiograph of left foot with fixed equinovalgus deformity and right foot and ankle with 30 equinus and talar dome deformity

Preoperative Problem List • • • • • •

45 left knee flexion contracture/deformity Chronic dislocation of right patella Left fixed equinovalgus Right ankle fixed equinus deformity Right knee pterygium and 85 contracture/deformity Severe extension contracture/deformity of the right and left elbow

Treatment Strategy Posterior knee releases were performed to bring the left knee from an approximately 45 flexion contracture to 10 of being straight and the right knee from an approximately 85 flexion contracture to 60 . A quadricepsplasty was performed on the right knee to relocate the chornically dislocated patella which was performed by extending the leg and shifting the patella into proper position. A hinged circular external fixator

was then applied to the right femur and tibia with two hinges at the knee axis. Gradual correction of the flexion contracture/ deformity was performed after excision of the pterygium and Z-plasty of the skin. Osteotomies and soft-tissue releases of the peroneus longus and brevis were performed on the left foot to resolve a severe fixed equinovalgus deformity and achieve a plantigrade foot. Soft-tissue releases of the right Achilles tendon and posterior capsule were performed to resolve the right foot equinus deformity. The right elbow contracture was released, the radial head excised, and an outerbridge procedure was performed with transposition of the ulnar nerve in order to achieve passive motion of the right elbow.

Basic Principles Assess the patient’s underlying strength prior to creating goals. Active extension of the knee is the most important function and cannot be substituted. Excision of the pterygium

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Fig. 5 Clinical photograph of the lower extremities with the Ilizarov fixator in place on the right lower limb

Fig. 4 Radiograph of right elbow showing irregularities and minimal joint space

was important to achieve lasting extension of the right knee. A peroneal nerve neurolysis and release into around the fibular head and into the anterior compartment is essential when treating severe deformities. Acute or gradual correction of the knee is possible even in long-standing deformities.

Images During Treatment See Figs. 5–9.

Technical Pearls • Utilizing distraction and gradual correction is possible even with severe contractures. • External fixation is not always required. • Excise the pterygium from the ischium to the heel. • Skin Z-plasties. • Release the peroneal nerve into the anterior compartment.

Fig. 6 Clinical photograph of the right lower extremity with the Ilizarov fixator

Outcome Clinical Photos and Radiographs See Fig. 10. Several years after treatment, the patient is wearing braces and is now able to walk without assistance. In addition, his

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Correction of Severe Contractures, Pterygium and Lower Limb Deformities Caused By Arthrogryposis Fig. 9 Radiograph of lower limbs with braces after removal of hardware

Fig. 7 Clinical photograph of the lower limbs after removal of hardware

Avoiding and Managing Problems

Fig. 8 Clinical photograph of the lower limbs in braces

upper extremities have maintained passive motion which allows him to utilize his hand in ways he could not before. To date, we have not performed any additional procedures to achieve active motion of the upper extremity.

• Peroneal nerve release • Complete excision of pterygium • Realistic goals based on muscle strength The patient had no complications.

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Cross-References ▶ Pediatric Arthrogryposis: An Introduction ▶ Simultaneous Correction of Hip and Knee Flexion Contractures in a 5 Year Old with Arthrogryposis ▶ Synchronization of Surgical Interventions for Multiple Deformities in a Four Year Old with Arthrogryposis

References and Suggested Reading 1. Amor CJ, Spaeth MC, Chafey DH, Gogola GR. Use of the pediatric outcomes data collection instrument to evaluate functional outcomes in arthrogryposis. J Pediatr Orthop. 2011;31(3):293–6. 2. Spencer HT, Bowen RE, Caputo K, Green TA, Lawrence JF. Bone mineral density and functional measures in patients with arthrogryposis. J Pediatr Orthop. 2010;30(5):514–8. 3. Van Bosse HJ, Feldman DS, Anavian J, Sala DA. Treatment of knee flexion contractures in patients with arthrogryposis. J Pediatr Orthop. 2007;27(8):930–7.

Fig. 10 Photograph of the patient standing independently

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Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 623 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 624 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 624 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 625 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 625 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 629

Abstract

A 4 year old girl with arthrogryposis was adopted from abroad, having had previous clubfoot surgery. She presented with clubfoot relapses, severe hip contractures, and 70–90 knee flexion contractures. Her clubfoot deformities were treated solely by serial casting and percutaneous Achilles tenotomies. Her multiplanar hip contractures were corrected by proximal femoral reorientational osteotomies (described for the first time in print in this chapter). Her knees were then straightened by posterior knee releases and application of knee spanning Ilizarov external fixators. At 7 years of age, she became an independent ambulatory in below-knee braces.

Brief Clinical History The patient was a 4 year old girl when we first met her. She was brought to the USA from an Eastern European orphanage by her adoptive family at 3 years of age, where her only prior orthopedic treatment apparently was bilateral clubfoot releases. At the time of presentation to us, she was only able to scoot on the floor in a sitting position. Her lower limb positioning was characteristic of children with severe arthrogryposis, with the hips flexed and widely abducted and knees flexed, into a “frog-leg”-like position (Fig. 1a, b). Her hips flexed 120 with 65 flexion contracture (Fig. 2a, b); knee range of motion was from 70 to 90 on the right, and 90–100 on the left (Fig. 3a, b), for a total arc of motion of 20 and 10 , respectively; both feet had equinocavus clubfoot variant recurrences with heel varus and forefoot adductus and equinus contractures of 35 on the right and 25 on the left.

H. J. P. van Bosse (*) Shriners Hospital for Children, Philadelphia, PA, USA e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_341

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Preoperative Clinical Photos and Radiographs

Preoperative Problem List

See Figs. 1, 2, 3, 4, and 5.

• Relapsed bilateral clubfoot deformities after previous surgical releases

Fig. 1 Preoperative photographs of the patient sitting (a) and lying down (b), demonstrating the hip and knee contractures, and relatively mild relapsed foot deformities

Fig. 2 Preoperative photographs, side views of bilateral hips, demonstrating maximal preoperative hip flexion and contralateral extension. (a) Left hip flexion, right hip extension. (b) Right hip flexion, left hip extension

Fig. 3 Preoperative photographs of left lower extremity showing maximal knee motion range. (a) maximal knee extension. (b) maximal knee flexion

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Fig. 4 Pretreatment lateral radiographs of the feet

Fig. 5 Preoperative radiograph of the pelvis, illustrating the hip contractures

• Bilateral hip flexion/abduction/external rotation contractures • Bilateral severe knee flexion contractures with limited joint total arc of motion

Treatment Strategy Our algorithm for treating children with the typical lower extremity manifestations of arthrogryposis (clubfoot, hip flexion/abduction/external rotation contractures, knee flexion contractures) is to start with serial casting of the feet when first possible. If a child is seen as an infant, then the feet undergo serial casting at that time, with a percutaneous Achilles tenotomy to achieve dorsiflexion once the foot shape is corrected. Then between 12 and 18 months, the

hips are addressed with reorientational osteotomies. The child is then outfitted with knee-ankle-foot orthoses (KAFOs) to help him/her begin upright standing and ambulating. After 4 years of age, the knee flexion contractures are corrected with posterior knee releases and gradual straightening via joint distraction using an Ilizarov external fixator. In the older child, we will still follow that order of correction, merely beginning the process when we first meet the child.

Basic Principles The Ponseti method of clubfoot correction by serial casting has proven to be very efficient and generalizable to the teratologic clubfoot, as seen in arthrogryposis [1, 5].

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Occasionally, in those feet with a severe equinus deformity, an initial or midtreatment Achilles tenotomy is required to unlock the calcaneus from the tibia, allowing it to swing into valgus. We have not needed to do open Achilles tenotomies and posterior or complete releases, even in feet that relapsed after previous soft tissue surgery. The reorientational osteotomy is an intertrochanteric femoral osteotomy, leaving the femoral-acetabulum relationship unchanged but bringing the rest of the lower extremity to align with the body axis. We have been using it for about 8 years and will publish results soon. A guidewire is placed into the very proximal femur, parallel in all planes to a line drawn between the anterior superior iliac spines, with the hip positioned in the manner characteristic for that patient. Over that guidewire, a cannulated seating chisel for a femoral blade plate is advanced, correcting for the flexion contracture as needed by rotating the chisel around the wire. The first osteotomy is just inferior and parallel to the seating chisel. The second osteotomy is just distal to the first and is perpendicular to the shaft of the femur. The two osteotomy surfaces are then married together with the blade plate. Severe knee flexion contractures (greater than 45 ), including those with pterygia, are best corrected by gradual soft tissue distraction with the use of a circular external fixator. A posterior knee release is performed at the time of surgery, allowing for joint distraction with a lessened risk of posterior tibial subluxation. The external fixator allows for precise placement of the medial and lateral knee hinges, which are aligned with the knee’s axis of rotation [2]. On average, each knee requires 4–6 weeks of distraction (1–2 per day), whereafter they are maintained in full extension for another 4–6 weeks, prior to frame removal. In bilateral cases, the knees are corrected sequentially. The limb is then casted for 4 weeks, prior to continuous bracing with a knee-ankle-foot orthosis.

H. J. P. van Bosse

arthrogrypotic clubfoot should be maintained in AFOs, with nighttime dorsiflexion straps to dorsiflex and evert the foot. Reorientational proximal femoral osteotomies – a blade plate gives better flexion/extension control than a hip screw and side plate when correcting flexion contractures by extending across the osteotomy. In cases of extreme flexion correction, an anterior spike off the proximal fragment is created by the first osteotomy cut, which needs to be trimmed to prevent impingement and possible vascular compromise during flexion. Knee flexion contracture – bias the hinge placement anterior and inferior to the actual knee axis, to help prevent posterior tibial subluxation and joint impingement at terminal extension, respectively.

Outcome Clinical Photos and Radiographs See Fig. 9.

Avoiding and Managing Problems

See Figs. 6, 7, and 8.

Clubfoot relapses are common in children with arthrogryposis, regardless of casting or surgical treatment. It is important to recognize that so long as there are no bony fusions, most of these feet can again be corrected to a braceable, plantargrade foot by the Ponseti casting method. Immobilization of the proximal femoral osteotomies in a Petrie cast allows for flexion and extension of the hips, to maintain or improve hip motion. Parents need to be carefully instructed on lifting the patient, maintaining stress-free alignment of the osteotomy sites. Neuropraxias with knee contracture correction are best prevented by not attempting to achieve contracture correction intraoperatively. In young children, insufficiency fractures occur occasionally when removing the external fixator. These are usually nondisplaced and can be treated by extending the post-frame cast wear to 6 weeks total.

Technical Pearls

Cross-References

Clubfoot correction – the emphasis of serial casting should be on forefoot abduction, although in cases of severe heel varus and/or cavus, first ray dorsiflexion may need to be exaggerated until the heel begins to roll into valgus. Post casting, the

▶ Correction of Arthrogrypotic Clubfoot Deformities with the Ponseti Method of Serial Casting ▶ Correction of Severe Arthrogrypotic Knee Flexion Contractures

Images During Treatment

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Fig. 6 Photographs after Ponseti style serial casting followed by bilateral percutaneous Achilles tenotomies. AP view of (a) right and (b) left foot. Medial view of (c) right and (d) left foot

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Fig. 7 Postoperative radiographs after reorientational proximal femoral osteotomies. AP (a) and frog-leg (b) pelvis immediately following the procedure. AP (c) and frog-leg (d) pelvis 6 months postoperative. The

H. J. P. van Bosse

acetabulum is already showing signs of improved acetabular dysplasia from the increased weightbearing

Fig. 9 The patient at 8 years old, standing in her KAFOs. She currently is ambulating fully independently with floor reaction AFOs. Hip motion is 5–90 . Knee motion is 20–40

Fig. 8 Postoperative photograph immediately after removal of left knee Ilizarov external fixator for correction of knee flexion contracture

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References and Suggested Reading 1. Boehm S, Limpaphayom N, Alaee F, Sinclair MF, Dobbs MB. Early results of the Ponseti method for the treatment of clubfoot in distal arthrogryposis. J Bone Joint Surg Am. 2008;90:1501–7. 2. Hollister AM, Jatana S, Singh AK, Sullivan WW, Lupichuk AG. The axes of rotation of the knee. Clin Orthop Relat Res. 1993;290:259–68. 3. van Bosse HJP. Contractures of the knee. In: Rozbruch SR, Ilizarov S, editors. Limb lengthening and reconstruction surgery. New York: Informa Healthcare USA; 2006. p. 345–55.

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4. van Bosse HJP, Feldman DS, Anavian J, Sala DA. Treatment of knee flexion contractures in patients with arthrogryposis. J Pediatr Orthop. 2007;27:930–7. 5. van Bosse HJP, Marangoz S, Lehman WB, Sala DA. Correction of arthrogrypotic clubfoot with a modified Ponseti technique. Clin Orthop Relat Res. 2009;467:1283–93.

Recurrent Knee Flexion Contractures in a 10 Year Old with Arthrogryposis

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Reggie C. Hamdy and Noe´mi Dahan-Oliel

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 631 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 632 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 632 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 632 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 632 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 633 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 633 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 634 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 634 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 635 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 635

Abstract

This case presents a young adolescent male with arthrogryposis who had multiple surgeries for correction of severe knee flexion deformities. The last surgery was at the age of 12 years when he had full correction of his deformities by means of an Ilizarov frame. Despite recurrence of the deformities, he is still ambulating and functioning well. This case examplifies the frustration of managing knee flexion deformities in children with arthrogryposis as these patients have a high recurrence rate of these deformities even after having obtained full correction.

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] N. Dahan-Oliel Clinical Outcomes, Shriners Hospital for Children, Montreal, QC, Canada e-mail: [email protected]

Brief Clinical History This patient is a 10 year old male with arthrogryposis and severe bilateral knee flexion contractures. He was initially seen at 4 years of age. He was first treated with distal femoral supracondylar osteotomies, with complete correction obtained. However, 4–5 years later, the correction was completely lost and the deformities recurred. Growth modulation by hemiepiphysiodesis of the anterior distal femoral physis using eight plates was then attempted but failed to fully correct the deformities. On the right side, knee flexion was from 30 to 90 and from 80 to 130 on the left side. He also had bilateral equinovarus deformities that were treated surgically, with partial recurrence of the deformity of the right foot. The right foot had a residual equinus deformity of minus 30 . Gradual correction of the knee flexion deformities using an Ilizarov frame was then performed. This led to complete correction of the flexion deformities, and the patient was able to ambulate independently with knee-ankle-foot orthoses (KAFOs) and crutches. Almost complete knee extension was obtained with the latter treatment intervention. Despite

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recurrence of the flexion contracture at the last follow-up, this adolescent is ambulating with bilateral KAFOs independently for short distances in the home, at school, and outdoors. He uses a manual wheelchair for long distances. He is able to ascend stairs with both hands on the railing. The patient also presents mild elbow contractures, mild hip flexion contractures of about 20–30 on each side, and an increased thoracolumbar kyphosis, likely to be compensatory for the hip contractures. At the latest follow-up, the knee deformities have recurred, and further surgery in the form of gradual correction with Ilizarov on both sides was discussed at the latest appointment in clinic.

R. C. Hamdy and N. Dahan-Oliel

– One may ask: is it worth correcting the knee flexion deformity, with such a high recurrence rate? The answer is “definitely.” Correction of the deformity will allow the patient to ambulate and, in many cases, continue to ambulate and perform activities of daily living even after recurrence of the deformities. In this specific case, previous management in the form of physiotherapy, casting and bracing, supracondylar osteotomies, and anterior stapling had been attempted. In such a case, gradual correction with an Ilizarov frame was the next logical step.

Basic Principles Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List • Severe recurrent bilateral knee flexion contractures in a child with arthrogryposis • Recurrent equinovarus deformity of the right foot • Bilateral mild hip flexion contractures • Increased thoracolumbar kyphosis • Bilateral mild elbow contractures

Treatment Strategy – Knee flexion contractures in children with arthrogryposis have a known reputation for recurrence, regardless of the surgery performed, as shown in this case. This can be very frustrating for the treating physician, for the patient, and for his family. However, this should be anticipated and clearly explained to the patient and family before embarking on surgical correction.

• Children with arthrogryposis should be followed in a multidisciplinary clinic. • The overall goal in the management of these children is to improve function and ambulation while fostering optimal development. • Early intervention as soon as possible after birth in the form of aggressive physiotherapy (stretching, joint mobilization, and range of motion exercises) can facilitate optimal long-term functional outcome. • Among knee deformities, flexion contractures are the most common in arthrogryposis and present some of the most challenging problems causing gait disturbances. • Treatment of knee deformities should start at birth with intensive stretching exercises and bracing. • Soft tissue releases of the hamstrings only are usually not enough to correct the flexion deformity. • Anterior hemiepiphysiodesis of the distal femur is an excellent option as this technique is minimally invasive and should be used as a first-line treatment of flexion deformities in the skeletally immature patient with mild to moderate deformities (25 that is not balanced by another deformity is an indication for surgery in cases of genu valgum in multiple enchondromatosis. 3. According to the Hueter-Volkmann law, a mass at the lateral aspect of the distal femur causes compression pressure at the lateral aspect and distraction pressure on the medial physis and accelerates growth on this side, creating a wedge-shape deformity of the epiphysis, resulting in knee valgus. To prevent progression of deformity, an ipsilateral hemiepiphysiodesis can be performed. 4. An obstruction to treatment in multiple enchondromatosis is the poor mechanical resistance of the bone, which often results in wire cut through during distraction or prolonged time in the fixator. 5. The use of the Ilizarov device in treating multiple enchondromatosis has led to conversion of chondroma cartilage in normal bone. It can be highly effective in treating the disease [2]. 6. One complication of Ollier’s disease is unilateral physeal arrest at an area near the lesion due to bone bridge formation. The diagnosis of bone bridge formation must be made early, using either CT or MRI.

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7. There is no consensus as to whether distraction osteogenesis at the site of enchondromas affects malignant transformation. However, it has been shown that malignant transformation can occur in 30% of patients with multiple enchondromatosis [3].

Images During Treatment See Figs. 4, 5, 6, 7, 8, and 9.

Technical Pearls 1. If it is unavoidable to insert wires in pathologic bone, secure fixation using more wires and half pins than one would in normal bone will increase stability and decrease incidence of cut through. 2. Callus distraction can be done at the area of diseased bone. However, there is still no consensus as to whether this helps in the conversion of pathologic bone to normal healthy regenerate bone. 3. Lengthen the bone along the anatomical axis of the femur to minimize recurrence of the valgus deformity. 4. For younger children with greater deformity and shortening, a bifocal femoral osteotomy is more appropriate than

Fig. 5 After removal of the fixator, there is correction of both valgus deformity and leg-length discrepancy

Fig. 4 (a–c) Patient underwent monofocal femoral osteotomy and application of an Ilizarov fixator. Gradual correction of the valgus deformity was done

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Fig. 6 (a, b) Fracture of the femur at the osteotomy site, which was intralesional. This occurred 2 years after removal of the fixator. Patient then underwent insertion of flexible IM nails for fracture fixation

Fig. 7 (a, b) Fracture then healed, but there was recurrence of valgus deformity at the age of 10, 4 years after the initial surgery, while the flexible nails were in place, due to premature physeal arrest. Note of progression of valgus deformity

a monofocal osteotomy. In order to decrease the chances of a pathological fracture, there should be more lengthening at the proximal osteotomy site and less lengthening at the distal site, where the osteotomy is intralesional. 5. In older patients, arrest of the ipsilateral growth plate can be done simultaneously with a supracondylar osteotomy. Because of complete growth plate arrest, overcorrection of

the deformity is unnecessary. Contralateral growth plate arrest can then be done to prevent leg-length discrepancy [1]. 6. The use of a unilateral fixator is better for femoral lengthening. For lengthening at the distal aspect, the use of a hybrid of unilateral fixator and two Ilizarov half rings distally provides more stability during correction.

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Fig. 8 (a, b) Patient underwent removal of the flexible IM nails at the age of 10. Patient also underwent bifocal femoral osteotomy with application of monofixator for correction of angulation as well as temporary hemiepiphysiodesis of the medial femoral condyle

Avoiding and Managing Problems

Fig. 9 Lengthening at the proximal osteotomy site is 2.2 cm, while lengthening at the distal site is 2.6 cm

Outcome Clinical Photos and Radiographs See Fig. 10.

1. Application of the Ilizarov fixator with simultaneous intramedullary nailing significantly shortens the time needed with the external fixator in place, which lessens the incidence of pin tract infections, as well as patient discomfort. This also prevents the occurrence of pathologic fractures [4] (Fig. 11). 2. The use of the unilateral fixator for lengthening of the femur provides sufficient stability and comfort for patients. For bifocal osteotomies more lengthening should be done at the proximal osteotomy and less should be done at the distal osteotomy to prevent recurrence of the valgus deformity. 3. Lateral physeal arrest can occur as a mass effect of the lesion. When this happens, hemiepiphysiodesis can be performed to prevent progressive valgus deformity of the knee. 4. Bone bridge formation must be diagnosed early using MRI. If there is bone bridge formation, it should be excised to prevent recurrence of the deformity. 5. Close monitoring of formation of regenerate as well as assessment of regenerate quality must be performed before fixator removal since the regenerate is not always healthy normal bone. 6. To monitor for malignant transformation, one should look for radiographic signs such as endosteal or cortical erosion, frank cortical destruction, and associated soft tissue mass extending beyond the cortical disruption in periodic skeletal surveys.

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Fig. 10 (a, b) The leg-length discrepancy and valgus deformity were improved at the age of 14. Note that normal trabeculation has been obtained after lengthening and correction. Note the disappearance of pathologic lesions at the lengthened portion, and the appearance of normallooking bone

See Also in Vol. 3 Correction of Forearm Deformities in Herediatry Multiple Exostosis (MHE) Correction of Long Bone Deformities due to Ollier’s Disease with Ilizarov Method

References and Suggested Reading

Fig. 11 The use of an intramedullary nail with a hybrid external fixator is only possible when only lengthening will be done and the angular deformity is mild. It will be difficult to insert even a flexible nail with a severe angular deformity

Cross-References ▶ Correction of Lower Limb Deformities in Multiple Hereditary Exostosis (MHE)

1. Chew DK, Menelaus MB, Richardson MD. Ollier’s disease: varus angulation at the lower femur and its management. J Pediatr Orthop. 1998;18:202–8. 2. Jesus-Garcia R, Bongiovanni JC, Korukian M, Boatto H, Seixas MT, Laredo J. Use of the Ilizarov external fixator in the treatment of patients with Ollier’s disease. Clin Orthop. 2001;382:82–6. 3. Liu J, Hudkins PG, Swee RG, Unni KK. Bone sarcomas associated with Ollier’s disease. Cancer. 1987;59:1376–85. 4. Popkov D, Journeau P, Popkov A, Haumont T, Lascombes P. Ollier’s disease limb lengthening: should intramedullary nailing be combined with circular external fixation. Orthop Traumatol Surg Res. 2010;96:348–53. 5. Shapiro F. Ollier’s disease. An assessment of angular deformity, shortening, and pathological fracture in twenty-one patients. J Bone Joint Surg Am. 1982;64:95–103. 6. Stokes IA. Mechanical effects on skeletal growth. J Musculoskelet Neuronal Interact. 2002;2(3):277–80. 7. Watanabe K, Tsuchiya H, Sakurakichi K, Yamashiro T, Matsubara H, Tomita K. Treatment of lower limb deformities and limb-length discrepancies with the external fixator in Ollier’s disease. J Pediatr Orthop. 2007;12:471–5.

Guided Growth Treatment for Genu Valgum Secondary to Juxtaphyseal Recurrent Aneurysmal Bone Cyst of the Distal Femur

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Sanjeev Sabharwal

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 693 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 694 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 694 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 695 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 695 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 696 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 697 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 697 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 698 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 700 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 700

Abstract

An 11 year 6 month old male with a history of recurrent aneurysmal bone cyst involving the distal metaphysis of the left femur presented with increasing asymmetric genu valgum affecting the left lower extremity. Plain radiographs and MRI scan confirmed the distal femoral valgus related to the recurrent juxtaphyseal lesion without any discrete bony bar. He underwent guided growth treatment with application of an extraperiosteal non-locking plate across the ipsilateral medial distal femoral physis as a same-day procedure. Over the next several months, progressive correction of his angular deformity was noted. After mild intentional overcorrection, the plate was removed 2 years postoperatively. At a 5-year follow-up, he was skeletally mature with equal leg lengths and symmetric physiologic alignment of his lower extremities. In a skeletally immature patient with a “sick” but viable S. Sabharwal (*) Department of Orthopaedics, Rutgers – New Jersey Medical School, Newark, NJ, USA e-mail: [email protected]

physis, guided growth treatment using an extraperiosteal plate is a viable alternative to more invasive osteotomies.

Brief Clinical History An 11 year 6 month old male was seen as an outpatient with the chief concern of progressive left-sided genu valgum. His past history was pertinent for a diagnosis of recurrent aneurysmal bone cyst (ABC) involving the distal femoral metaphysis. He had previously undergone two surgeries on the left lower extremity. These procedures included an initial curettage and bone grafting of the lesion about 2 years earlier, followed by a repeat procedure 8 months prior to presentation due to recurrence of the lesion. There was no history of any postoperative infection or fracture. On physical exam, he had asymmetric genu valgum, left greater than right side. His leg lengths were equal within a centimeter. The range of motion of the left knee was 5–130 compared to 0–140 on the unaffected right side. Lateral

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_41

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maltracking of the left patella was noted. The torsional profile of the lower extremities was symmetric.

Preoperative Clinical Photos and Radiographs Preoperative clinical picture of the patient from the front (Fig. 1) and back (Fig. 2) demonstrating the left-sided genu valgum. A full-length standing radiograph of the lower extremities revealed a mechanical axis deviation (MAD) of 1.3 cm lateral on right and 6 cm lateral on left side (Fig. 3). Lateral distal femoral angle (LDFA) was 86 on right and 67 on left side. Medial proximal tibial angle (MPTA) was 89 on right and 86 on left side. Scanogram revealed a 3 mm shortening of the left side, consistent with a leg length discrepancy (LLD). His skeletal age was consistent with his chronologic age.

Fig. 1 Preoperative clinical picture of the patient from the front demonstrating the left-sided genu valgum

S. Sabharwal

The MRI scan revealed two small cystic lesions located at the lateral aspect of the distal femoral metaphyses. The largest one measured approximately 1.5  1.2  1 cm in greatest dimension. The lesions had regressed in size and had become more solid in appearance since the second procedure. The lesion extended up to the lateral aspect of the distal femoral growth plate. However, a distinct physeal bar was not apparent on the MRI (Figs. 4 and 5). See Figs. 1, 2, 3, 4, and 5.

Preoperative Problem List (I) Genu valgum left side, secondary to juxtaphyseal aneursymal bone cyst affecting the growth of the lateral physis of the distal femur (II) Mild, asymptomatic lateral maltracking of the left-sided patella

Fig. 2 Preoperative clinical picture of the patient from the back demonstrating the left-sided genu valgum

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Fig. 4 The MRI scan (T1 sequence) revealed two small cystic lesions located at the lateral aspect of the distal femoral metaphyses. The largest one measured approximately 1.5  1.2  1 cm in greatest dimension. The lesions had regressed in size and had become more solid in appearance since the second procedure. The lesion extended up to the lateral aspect of the distal femoral growth plate. However, a distinct physeal bar was not apparent on the MRI Fig. 3 A full-length standing radiograph of the lower extremities revealed a mechanical axis deviation (MAD) of 1.3 cm lateral on right and 6 cm lateral on left side. Lateral distal femoral angle (LDFA) was 86 on right and 67 on left side. Medial proximal tibial angle (MPTA) was 89 on right and 86 on left side

(III) Anticipated increase in angular deformity, patellar maltracking, and gait abnormality with future growth (IV) Need to follow distal femoral lesion for recurrence

Treatment Strategy The options for treating this child are primarily surgical. Due to his growth remaining and lack of a distinct physeal bar, we performed guided growth treatment with an extraperiosteal plate placed across the medial aspect of the distal femoral physis. In order to address the mild lateral maltracking of his patella, an open lateral retinacular release was also performed at that time. The patient was mobilized, weight bearing was tolerated with crutches postoperatively, and discharged home the same day.

Basic Principles This skeletally immature patient developed genu valgum related to asymmetric growth of the distal femur due to a “sick” lateral physis secondary to the juxtaphyseal position of the recurrent ABC [2]. Preoperative evaluation in such a patient includes assessment of the magnitude and location of the angular deformity and growth remaining (using skeletal age) with radiographs. Advanced imaging such as an MRI (or CT scan) helps delineate the status of the physis and presence of a discrete physeal bar. In this case, since a discrete physeal bar was not present, there was a potential for the lateral physis to continue growing, although at a somewhat slower rate than the medial side. Based on the principles of guided growth, an extraperiosteal non-locking plate was placed across the medial distal femoral physis, in order to “balance” the growth across the distal femur [1, 3]. Given the poor bone stock due to the recurrent ABC at the distal femoral metaphysis and the increased surgical morbidity, a

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Fig. 6 Intraoperative AP fluoroscopic image demonstrating placement of an extraperiosteal non-locking two-hole plate across the medial aspect of the distal femoral physis Fig. 5 The T2 sequence MRI scan confirms the above noted findings in Fig. 4

distal femoral osteotomy was not recommended. Furthermore, given the lack of a discrete physeal bar, a “bar resection” was not recommended.

Images During Treatment Intraoperative AP (Fig. 6) and lateral (Fig. 7) fluoroscopic images demonstrate placement of an extraperiosteal non-locking two-hole plate across the medial aspect of the distal femoral physis. Full-length standing radiographs 8 months later demonstrate mild correction of the left-sided genu valgum with some divergence of the screws (Fig. 8). After another 3 months, further correction is noted on clinical exam (Fig. 9) and radiographs (Fig. 10). Serial follow-up with clinical examination and full-length standing radiographs of the lower extremities revealed progressive improvement in the genu valgum and patellar tracking. Approximately 2 years following the guided growth treatment, he had achieved neutral alignment of his left lower extremity (intentional mild overcorrection) and had 5 of genu valgum on the right side. A full-length standing radiograph of the lower extremities revealed a MAD of

Fig. 7 Intraoperative lateral fluoroscopic image demonstrating placement of an extraperiosteal non-locking two-hole plate centered over the distal femoral physis

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Fig. 8 Full-length standing radiographs 8 months later demonstrate mild correction of the left-sided genu valgum with some divergence of the screws

0.9 cm lateral on right and 0 cm on left side (Fig. 11). The distal femoral plate was removed as a same-day procedure at that time. See Figs. 6, 7, 8, 9, 10, and 11.

Technical Pearls (I) Rule out a physeal bar preoperatively, using advanced imaging such as a CT scan or MRI. (II) Confirm that there is growth remaining using plain radiographs of the affected physis and determining the skeletal age of the patient. (III) Ensure that the underlying periosteum is not damaged during plate insertion.

Fig. 9 At 11 months following insertion of the guided growth implant, further correction is noted on clinical exam

(IV) In order to avoid any iatrogenic sagittal plane deformity (procurvatum or recurvatum), center the implant on the bone on the lateral view.

Outcome Clinical Photos and Radiographs At a recent visit, about 5 years following plate insertion, the patient had maintained symmetric knee alignment and equal leg lengths based on physical exam (Figs. 12 and 13) and full-length standing radiographs (Fig. 14). He had reached skeletal maturity, and progressive healing of the ABC was noted. See Figs. 12, 13, and 14.

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Fig. 10 Full-length standing radiographs corresponding to Fig. 9 (11 months postoperatively) demonstrate progressive correction of the left-sided genu valgum with further divergence of the screws

Avoiding and Managing Problems (I) Avoid any damage to the underlying periosteum when placing the extraperiosteal plate. (II) Ensure compliance with periodic follow-up. In order to diagnose recurrent deformity and also avoid extreme overcorrection, follow these children with serial fulllength radiographs (typically every 4–6 months). (III) Even after hardware removal, serial follow-up to skeletal maturity is indicated in order to avoid recurrent defor-

Fig. 11 Approximately 2 years following the guided growth treatment, the patient had achieved neutral alignment of his left lower extremity (intentional mild overcorrection) and had 5 of genu valgum on the right side. A full-length standing radiograph of the lower extremities revealed a MAD of 0.9 cm lateral on right and 0 cm on left side. The distal femoral plate was removed as a same day procedure at that time

mity related to rebound growth or overcorrection secondary to unanticipated permanent injury to the underlying physis [1].

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Fig. 12 Five years following plate insertion, the patient had maintained symmetric knee alignment and equal leg lengths based on physical exam (frontal view)

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Fig. 13 At 5-year follow-up, the patient had maintained full knee mobility with no sagittal plane deformity

700 Fig. 14 Full-length standing radiographs at 5-year follow-up. The patient had reached skeletal maturity and progressive healing of the ABC was noted

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Cross-References ▶ Guided Growth Treatment for Early-Onset Blount Disease

References and Suggested Reading 1. Ballal MS, Bruce CE, Nayagam S. Correcting genu varum and genu valgum in children by guided growth: temporary hemiepiphysiodesis using tension band plates. J Bone Joint Surg (Br). 2010;92(2):273–6. 2. Lin PP, Brown C, Raymond AK, Deavers MT, Yasko AW. Aneurysmal bone cysts recur at juxtaphyseal locations in skeletally immature patients. Clin Orthop Relat Res. 2008;466(3):722–8. 3. Stevens PM, Klatt JB. Guided growth for pathological physes: radiographic improvement during realignment. J Pediatr Orthop. 2008;28(6):632–9.

Management of Distal Femur Deformity Following Ablation of Aneurysmal Bone Cyst, by Corrective Osteotomy and Elongation

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Alan Katz, Amos Peyser, and Ehud Lebel

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 701 Pre-operative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 702 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 702 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 702 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 702 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 703 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 704 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 704 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 705

Abstract

An adolescent healthy male presented with a left distal femur aneurysmal bone cyst (ABC). The patient’s ABC was managed with evacuation, cryo-ablation, sclerotherapy, and synthetic bone substitute. The patient developed medial distal growth plate arrest leading to a limb length discrepancy and a varus deformation. Two stages were used to treat the deformity: a complete epiphysiodesis of the distal femur and valgus osteotomy, followed by bone elongation on a telescoping nail at a more proximal site.

A. Katz Pediatric Orthopedic Surgeon, Department of Orthopedic Surgery, Shaare Zedek Medical Center, Faculty of Medicine, Hebrew University, Jerusalem, Israel A. Peyser Shaare Zedek Medical Center, Jerusalem, Israel e-mail: [email protected] E. Lebel (*) Head of Pediatric Orthopedics Unit, Department of Orthopedic Surgery, Shaare Zedek Medical Center, Faculty of Medicine, Hebrew University, Jerusalem, Israel e-mail: [email protected]

The process was performed over two stages in order to achieve accurate, precise, and controlled correction on healthy bone only (away from the pathologic bone).

Brief Clinical History An 11-year-old healthy male presented to the outpatient clinic with generalized left knee pain that awakened him at night. After a full diagnostic workup, including X-rays and MRI, an aneurysmal bone cyst (ABC) of the left distal femur was diagnosed. The patient’s ABC was managed with evacuation of the bloody infiltrate, cyst walls were ablated with curettage cryo-ablation and Argon beam sclerotherapy, and synthetic bone substitutes were used to fill the void. A long leg cast was applied and initial non-weight bearing was prescribed. The patient was closely followed and a medial distal growth plate arrest was identified. Ultimately, the patient developed a limb length discrepancy (LLD) and a varus deformation. At age 13, the left leg was 3 cm shorter (expected LLD at maturity 4.0 cm) with an aLDFA (anatomic lateral distal femoral angle) of 106 and a mechanical axis deviation of 4.3 cm medial.

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Sequential femur radiographs were taken with a target length based on the contralateral femur (to correct the full LLD).

See Images 1, 2, 3, and 4.

Basic Principles Preoperative Problem List Healing femur with bone substitute post-ABC curettage and ablation Growth plate injury LLD and varus deformity

Treatment Strategy Two stages were used to treat the deformity. In the first phase (at age 13), a complete ipsilateral epiphysiodesis of the distal femur was performed (to prevent further deterioration). Simultaneously, a valgus osteotomy, deformity correction, and plate fixation with a pediatric distal femur locking plate were executed. After bone healing as well as after contralateral femur growth had stopped (at age 14.5), a second phase was initiated, where the plate was removed and sequential bone elongation on a telescoping nail at a more proximal site was performed. Image 1 (a, b) AP and lateral radiographs of the aneurysmal bone cyst in the left medial distal femur. Note the sharply defined expansile osteolytic lesion

An aneurysmal bone cyst is a locally aggressive bone lesion arising predominantly in the pediatric population that can cause local pain, swelling, and pathologic fractures. The standard treatment plan is curettage with or without bonegraft, depending on the resultant void. Additionally, there are various adjuvants including use of cement, high-speed burr, argon beam, phenol, and cryotherapy to reduce the rate of recurrence [1]. Growth plate involvement, asymmetric growth, and growth arrest are of great concern because of the lesion’s location as well as its treatment [2]. Regarding limb lengthening or deformity correction postABC treatment, there are several elements that could affect the quality of the bone regenerate. Local tissue factors such as the presence of bone pathology (in this case, an ABC) are potential obstacles and barriers in achieving reliable new bone formation [3]. An internal lengthening nail (ILN), a Precice nail in this example, is a good option as it avoids the need for external fixation and requires one less surgical procedure than

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Image 2 (a–c) MRI images of the aneurysmal bone cyst demonstrating cysts with variable signal with a surrounding rim of low T1 and T2 signal

Image 3 (a–c) Six month post-op clinical photo and X-ray after ABC treatment with evacuation, cryo-ablation, sclerotherapy, and synthetic bone substitute

lengthening over a nail (LON) with subsequent static nail removal. Additionally, femoral lengthening with ILN is more accurate than with LON [4].

Images During Treatment See Images 5 and 6.

Technical Pearls Elongation should ideally be performed on healthy bone. Therefore, the length correction was based proximally in the femur, away from the prior ABC and treated bone pathology (as well as the varus correction). A prerequisite for

deformity correction is good bone stock. The process was performed over two stages in order to achieve accurate, precise, and controlled correction. For proximal femur lengthening, a trochanteric entry nail is advised for skeletally immature patients and a piriformis entry nail is advised for skeletally mature patients. In our case, a piriformis entry Precice nail was used, as the patient’s physes were closing and contralateral femur growth had stopped. Additionally, the patient had a narrow femoral canal (making it difficult to use a trochanteric entry nail due to the angle at insertion.) A schedule of 1 mm/day (divided into 2 sessions per day) was started 10 days post-nail insertion. After the surgery, a non-weight bearing protocol was instated and ambulation with crutches was advised. After the completion of the limb lengthening, weight bearing was permitted after 1) three

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regenerate cortexes were visualized on AP and lateral (combined) follow-up X-rays, and 2) the patient felt balanced and pain-free while standing.

Outcome Clinical Photos and Radiographs See Image 7.

Avoiding and Managing Problems

Image 4 Full-leg AP X-ray demonstrating the mechanical axis and varus deformation. The left leg in on blocks. (aLDFA 106 , LLD 3 cm)

Image 5 (a, b) X-rays after the first phase of surgeries: valgus osteotomy, deformity correction, and plate fixation with a pediatric distal femur locking plate

The distal locking screws of the Precice nail were placed close to holes of the prior plate placed on the distal femur during the valgus osteotomy. Caution and attention are advised, as this is a weak point of the bone. Correction of the LLD was performed only after verification that contralateral leg growth had stopped. The manufacturer recommends routine removal of this type of nail at the completion of regenerate consolidation (usually 1–2 years after insertion). Due to the fact that the patient was happy with the results and was asymptomatic at the end of elongation and deformity correction, the patient staunchly refused nail removal and only recently (at the time of publishing) has agreed.

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Image 6 (a, b) Radiographs after the second phase of surgeries demonstrating the healed valgus osteotomy, distal locking plate removal, new proximal valgus osteotomy, insertion of Precice nail, and femur distraction osteogenesis

Image 7 Full-length radiograph of both legs showing almost equal limb length and varus correction

References 1. Park HY, et al. Current management of aneurysmal bone cysts. Curr Rev Musculoskelet Med. 2016;9(4):435–44. https://doi.org/10.1007/ s12178-016-9371-6. 2. Stevens K, Stevens J. Aneurysmal bone cysts. StatPearls, 30 Aug 2021. www.statpearls.com/ArticleLibrary/viewarticle/17552 3. Idulhaq M, et al. Distraction osteogenesis at the site of previously cystic bone lesion of femur: a case report. Int J Surg Case Rep. 2021;84:106153. https://doi.org/10.1016/j.ijscr.2021.106153. www. ncbi.nlm.nih.gov/pmc/articles/PMC8258856/ 4. Fragomen AT, et al. A comparison of femoral lengthening methods favors the magnetic internal lengthening nail when compared with lengthening over a nail. HSS J. 2018;14(2):166–76. https://doi.org/ 10.1007/s11420-017-9596-y.

Migration of the Fibula During Tibial Lengthening in Achondroplasia

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Hae-Ryong Song and Kwang-Won Park

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 707 Chief Complaint: Short Stature . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 707 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 708 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 708 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 708 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 711 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 711 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 711 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 712 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 712 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 720 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 720 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 720

Abstract

Complications arising from tibial lengthening in achondroplasia include (1) delayed consolidation of the fibula, (2) premature consolidation of the fibula, (3) angulation or translation of the fibula, and (4) valgus angulation of the ankle or the knee (Kim et al., Acta Orthop 83:271–5, 2012). These can be attributed to the slower fibular distraction rate compared to the tibial distraction rate, resulting in a lagging behind of the lateral malleolus compared to the distal tibia. A study on the effect of these distractionresisting forces measured the tibiofibular distraction difference, the proximal tibiofibular joint distraction, the

H.-R. Song (*) · K.-W. Park Department of Orthopedic Surgery, Korea University Medical Center, Guro Hospital, Seoul, South Korea e-mail: [email protected]; [email protected]

distal tibiofibular joint distraction, and the mean tibial valgus angulation of patients who underwent tibial lengthening (Shyam et al., J Bone Joint Surg 91:1671–82, 2009). They concluded that if the aim of lengthening is more than 25% of the initial tibia length, one should expect an increase in knee laxity, and if it is more than 50% of the initial length, one should expect an increase in tibial valgus angulation.

Brief Clinical History Chief Complaint: Short Stature This was a 10 year old patient who was diagnosed with achondroplasia and underwent bilateral tibial lengthening. Cut-through of the proximal wire occurred, and this resulted in premature consolidation of the fibula. There was also progressive valgus deformity of the tibia. Correction of the

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valgus deformity and repeat fibular osteotomy was done as well as insertion of an additional half pin at the proximal ring for more stability (Figs. 1, 2, 3, 4, 5, and 6). Case Tibial lengthening with distal migration of fibular head See Fig. 1.

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IV. Distal migration of the proximal fibula associated with increased ligamentous laxity V. Proximal migration of the distal fibula associated with ankle valgus deformity as well as distal migration of the fibular head associated with premature consolidation VI. Epiphyseal separation of the proximal fibula with knee pain VII. Translation of the proximal fibula after monofocal fibular and bifocal tibial osteotomy

Preoperative Clinical Photos and Radiographs Our patient is a 10 year old female who was diagnosed with achondroplasia. On the anteroposterior radiograph of both legs, it can be noted that the fibulae are longer than the tibiae, a common feature of this skeletal dysplasia. This is one factor that predisposes the patient to genu varum. The preoperative tibia length is 16 cm, while the preoperative fibula length is 17 cm.

Preoperative Problem List I. Different distraction rates between the tibia and fibula as an effect of distraction-resisting forces arising from the soft tissues II. Wire cut-through due to increased distraction forces resulting in proximal or distal migration of the fibula. III. Premature consolidation of the fibula due to slower distraction rate than the tibia

Treatment Strategy 1. Make the amount of distraction similar for the tibia and fibula. 2. Consider fibular osteoclasis if with any sign of premature consolidation of the fibula as well as transport of the fibula proximally or distally to restore proximal or distal tibiofibular joint relationships. 3. Gradual correction of the tibial deformity to depending on the consolidation status of the fibula to prevent nonunion of the fibula due to either proximal or distal migration. 4. Consider bifocal osteotomy of the fibula when correcting the tibial deformity with multiple CORA levels to prevent translation or angulation at the osteotomy site. 5. Transfixation of the proximal and the distal tibiofibular joints with wires or screws, to prevent distal migration of the fibular head or proximal migration of the distal fibula, respectively.

Fig. 1 Patient with achondroplasia

Tibia length R: 16 cm L: 16 cm Fibula length R: 17 cm L: 17 cm Talocrural angle R: 20° L: 22° Bimalleolar angle R: 96° L: 100° Proximal tibiofibular joint distraction R: 18 cm L: 16 cm Distal tibiofibular joint distraction R: 1.8 cm L: 1.8 cm Tibiofibular valgus angulation: 5°

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Tibial length L: 19 cm Amount of tibial lengthening: 3 cm Fibula length L: 18 cm Amount of fibular lengthening: 1.5 cm Proximal tibiofibular joint distraction L: 3 cm Distal tibiofibular joint distraction: no change

Fig. 2 Monofocal tibial and fibular osteotomy was done when the patient was 10 years old. Radiograph shows 3 cm of tibial lengthening, 1.5 cm fibular lengthening. There is a difference of 1.5 cm between the amount of lengthening of the tibia and fibula

a

b

Tibial length: 24 cm Amount of tibial lengthening: 6 cm Fibular length: 19 cm Amount of fibular lengthening: 3 cm Proximal tibiofibular joint distraction: 4 cm Distal tibiofibular joint distraction: 2 cm

Fig. 3 There is a difference of 3.2 cm in the distraction of the tibia and the fibula. Cut-through of the proximal tibiofibular wire occurred after 60 mm of lengthening. This caused distal migration of fibular head and premature consolidation of the fibular osteotomy

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Tibial valgus angulation: 27° Tibial length 24 cm Amount of tibial lengthening 7 cm Fibular length 21 cm Amount of fibular lengthening 2 cm Proximal tibiofibular joint distraction: 4 cm Distal tibiofibular joint distraction: no change

Fig. 4 Proximal wires were removed due to loosening and pin track infection. Instability caused progression of valgus angulation after 7 cm of lengthening. This caused increased distal migration of fibular head

Tibial length: 26 cm Amount of tibial lengthening: 8 cm Fibular length: 23 cm Amount of fibular lengthening 2.5 cm Tibiofibular distraction difference: 5 cm Proximal tibiofibular joint distraction: 3 cm Distal tibiofibular joint distraction: 2 cm

Fig. 5 Distal fibular osteotomy and half pin insertion for gradual lengthening of fibula

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a

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b Proximal tibiofibular distraction difference: 1.8 cm Tibiofibular distraction difference R: 4 cm L: 5 cm Pre-op

Post-op

Tibia length R: 16 cm

Tibial length R: 25 cm

L: 16 cm

L: 26 cm

Fibular length R: 17 cm

Fibular length R: 22 cm

L: 17 cm

L: 22 cm

Talocrural angle R: 20°

Talocrural angle R: 32°

L: 22°

L: 26°

Bimalleolar angle R: 96°

Bimalleolar angle R: 89°

L: 100°

L: 82°

Proximal tibiofibular joint

Mechanical axis

distraction R: 18 cm

deviation: 2.5 cm

L: 16 cm

Proximal tibiofibular

Distal tibiofibular joint

joint distraction: 2.8 cm

distraction: R: 1.8 cm

Distal tibiofibular joint

L: 1.8 cm

distraction: R: 1.8cm L: 1,8 cm Valgus angulation of tibia: 7°

Fig. 6 Comparison of pre-op and post-op X-rays

Basic Principles 1. The rate of distraction of the fibula is slower than the rate of distraction of the tibia because of the countertensioning forces brought about by the soft tissues. 2. Lengthening of more than 50% of the original length of the tibia results in a greater tendency for wire cut-through, proximal migration of the distal fibula, and tibial valgus angulation. 3. The proximal reference wires are inserted at the metaphyseal area of the fibula below the area of the peroneal nerve, and this increases the propensity for wire cut-through in children, resulting in distal migration of the fibular head. 4. The use of carbon fiber rings helps in visualizing radiographically the status of the proximal and distal fibula during the distraction period. 5. Weekly follow-up during the distraction period assists in monitoring the occurrence of wire cut-through, distal migration of the fibular head, or proximal migration of the distal fibula. 6. These parameters indirectly measure the distractionresisting forces on the tibia during distraction osteogenesis and hence should be monitored: Tibiofibular distraction difference: (total tibial distractiontotal fibular distraction) (Fig. 7)

Proximal tibiofibular joint distraction (distal migration of the fibular head measured on preoperative and postoperative radiographs with the tibial plateau as reference) (Fig. 18) Distal tibiofibular joint distraction: proximal migration of the distal fibula measured on preoperative and postoperative radiographs (Fig. 19) 7. Use of the talocrural angle (Fig. 20) and bimalleolar angle (Fig. 21) for the evaluation of the proximal migration of the distal fibula. A difference of 2 between either side is significant.

Images During Treatment See Figs. 2, 3, 4, and 5.

Technical Pearls I. The use of transfixation wires at the proximal tibiofibular joint to prevent distal migration of proximal fibula or at the distal tibiofibular joint to prevent proximal migration of the distal fibula. Ilizarov wires can be enough for adolescents with firm metaphyseal cortices, but for younger children with soft metaphyseal bone

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Fig. 7 Patient with achondroplasia who underwent bilateral tibial lengthening. During distraction, 4 months after the initial surgery, proximal migration of the distal fibula occurred, leading to premature consolidation of the fibula osteotomy site

II.

III.

IV.

V. VI.

VII.

quality and a greater tendency for cut-through, an additional half pin is recommended. Even with the additional fixation of a half pin at the proximal fibula, there have been cases of wire cut-through or pin breakage, especially when the amount of lengthening is greater than 50% of the original length of the tibia. The recommendation is that instead of just one additional half pin, two additional half pins should be inserted. Use of transfixation wires at the distal tibiofibular joint to maintain a normal tibiofibular relationship and prevent ankle valgus deformity. An Ilizarov wire is usually sufficient to prevent wire cut-through due to proximal migration during fibular lengthening because of the soft tissues surrounding the joint. More secure fixation is needed for a larger amount of lengthening to prevent cut-through of the fibula due to the increased distraction forces. A half pin can be used in addition to an Ilizarov wire for proximal fixation of the fibula. Medial slanting of proximal wires and rings 10 medially to prevent tibial valgus. Proximal ring block must be oriented in 5–7 of valgus and procurvatum to avoid tibial valgus and procurvatum. Lengthening using Ilizarov over an intramedullary nail will decrease tibial valgus angulation.

Outcome Clinical Photos and Radiographs See Fig. 6. After removal of the fixator, the final tibial length was 25 cm for the right and 26 cm for the left. The final fibula length was 22 cm for both the left and right. There is no residual valgus deformity of the tibia. The total proximal tibiofibular joint distraction was 2.8 cm, while the distal tibiofibular joint distraction did not change. There is complete union of the tibial and fibular osteotomy sites.

Avoiding and Managing Problems 1. Proximal migration of the fibula can cause residual ankle valgus deformity as well as nonunion of the fibular osteotomy site. Our patient, who was 6 years old when he underwent bilateral tibial lengthening, was found to have proximal migration of the distal fibula (Fig. 7). This resulted in valgus deformity of the ankle. Patient underwent repeat fibular osteotomy and revision of the Ilizarov fixator (Fig. 8). He subsequently underwent insertion of allograft mixed with demineralized bone matrix. However, upon removal of the fixator, there was note of nonunion at the fibular osteotomy site (Fig. 9). At the age of 9 years, a closing wedge osteotomy

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Migration of the Fibula During Tibial Lengthening in Achondroplasia

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Tibial length: 18 cm Amount tibial lengthening: 6 cm Fibular length: 16 cm Amount if fibular lengthening: 2 cm Proximal tibiofibular joint distraction: 1 cm Distal tibiofibular joint distraction: 1.5 cm

Fig. 8 Patient also underwent allograft application and injection of demineralized bone matrix Fig. 9 Note of valgus deformity of the tibia at 9 years old due to proximal migration of fibula as well as atrophic nonunion of the fibula even after insertion of allograft chips

Tibial length R: 25 cm L: 25 cm Fibular length: R 20 cm L: 22 cm Talocrural angle 16. 38° Proximal tibiofibular joint distraction: 3 cm Distal tibiofibular joint: 1 cm Tibiofibular valgus angulation R: 24

was done for the tibia to address the valgus deformity. For the fibula, autogenous iliac bone graft was inserted, along with additional half pin insertion for gradual

lengthening (Fig. 10). During the last follow-up, the ankle valgus was corrected and union of the right fibula was observed as well (Fig. 11).

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Case Fibular nonunion and valgus deformity of the ankle after lengthening 2. Fibular translation can occur when a monofocal fibular osteotomy is done with a bifocal tibial osteotomy. One patient who underwent bilateral tibial lengthening was noted to have lateral translation of the distal fibula on follow-up. This was corrected by doing a repeat bifocal fibular osteotomy and application of two half pins at the distal fibula (Fig. 12). Case Fibular translation due to monofocal tibial osteotomy with bifocal tibial osteotomy 3. Physeal separation of the proximal fibula can occur, even when the proximal tibiofibular joint relationship is maintained. In one patient, it was caused by premature consolidation at the fibular osteotomy site (Fig. 14). This was managed with repeated fibular osteotomy, application of half pins on middle of fibula, and gradual proximal transport. After removal of the Ilizarov fixator, there was complete union at the osteotomy site but with some residual distal migration of the fibula (Figs. 15 and 16). Fig. 10 Closing wedge osteotomy to correct tibial valgus deformity as well as autogenous iliac bone graft application and additional half pin insertion for gradual transport of the fibula

Case Epiphyseal separation at the proximal fibula See Figs. 13, 14, 15, 16, 17, 18, 19, 20, and 21.

Pre-op measurements

Post-op measurements

Tibial length R: 11 cm L: 11 cm Fibular length R: 11 cm L: 11 cm Bimalleolar angle R: 64° L: 62° Talocrural angle R: 11° L: 10°

Tibial length R: 22 cm L: 22 cm Amount of tibial lengthening: 11 cm Fibula length R: 20 cm L: 20 cm Amount of fibular lengthening: 9 cm Proximal tibiofibular joint distraction R: 1.7 cm L: 1 cm Talocrural angle R: 21° L: 19° Bimalleolar angle R: 74° L: 66° Distal tibiofibular joint distraction R: 0.9 cm L: 1.9 cm Proximal tibiofibular distraction difference R: 0.3 cm L: 0.6 cm Distal tibiofibular distraction difference R: 0.3 cm L: 0.9 cm Tibiofibular distraction difference R: 2 cm L: 2 cm

Proximal tibiofibular joint distraction R: 1.4 cm L: 1.6 cm Distal tibiofibular joint distraction R: 1.1 cm L: 1.05 cm

Fig. 11 Bilateral tibia X-ray after Ilizarov removal at 10 years old

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Migration of the Fibula During Tibial Lengthening in Achondroplasia

Fig. 12 Patient with achondroplasia and severe genu varum, who underwent bilateral tibial lengthening. He had lateral translation of the fibula due to the monofocal osteotomy accompanying the bifocal tibial osteotomy. To correct the translation, patient underwent bifocal fibular

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osteotomy and addition of two half pins at the distal fibula. After removal of the fixator, there is complete union of the fibular osteotomy site and ankle mortise is neutral

Fig. 13 Patient with achondroplasia who underwent lengthening at the age of 5

Tibial length R: 16 cm L: 16 cm Fibular length R: 17 cm L: 17 cm Talocrural angle R: 22. 56° L: 18. 56° Bimalleolar angle: R: 70° L: 63° Proximal tibiofibular distance R: 1.5 cm L: 1.4 cm Distal tibiofibular distance: R: 1.5 cm L: 1.7 cm

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Tibial length: 24 cm Tibial lengthening: 9.9 cm Fibular length: 22 cm Proximal tibiofibular joint distraction: 1.5 cm Fibular epiphyseal separation distance: 6.3 cm Distal tibiofibular joint distraction: same

Fig. 14 Radiograph shows evidence of physeal separation at the proximal fibula 4 months after the initial surgery, resulting in premature consolidation of the fibular osteotomy site

Tibial length: 24 cm Fibular length: 22 cm Proximal tibiofibular joint distraction: 1.5 cm Epiphyseal separation distance: 6.3 cm Distal tibiofibular joint distraction: same

Fig. 15 Repeated fibular osteotomy, application of half pins on the middle of fibula for gradual fibular transport

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Migration of the Fibula During Tibial Lengthening in Achondroplasia

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Tibiofibular distraction difference R: 3 cm L: 3 cm

Pre-op measurements

Post-op measurements

Tibial length R: 16 cm

Tibial length R: 32 cm

L: 16 cm

L: 32 cm

Fibular length R: 17 cm

Amount if tibial lengthening: 16 cm

L: 17 cm

Fibular length R: 30 cm

Talocrural angle R: 22. 56°

L: 30 cm

L: 18. 56°

Amount of fibular lengthening: 13 cm

Bimalleolar angle: R: 70°

Tibio-fibular valgus angulation 7°

L: 63°

Talocrural angle R: 21°

Proximal tibiofibular

L: 28°

distance R: 1.5 cm

Bimalleolar angle: R: 75°

L: 1.4 cm

L: 75°

Distal tibiofibular

Proximal tibiofibular joint distraction: R: 2°

distance R: 1.5 cm

L: 2.4°

L: 1.7 cm

Distal tibiofibular joint distraction: no change Proximal tibiofibular joint distraction difference: R: 0.6 cm L: 2 cm

Fig. 16 Postoperative X-ray at the age of 13 with complete union of osteotomy sites but with residual inferior fibula migration on the left

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Fig. 17 Tibiofibular distraction difference (total tibial distraction-total fibular distraction)

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Fig. 18 Proximal tibiofibular joint distraction (distal migration of the fibular head measured on preoperative and postoperative radiographs with tibial plateau as reference)

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Migration of the Fibula During Tibial Lengthening in Achondroplasia

Fig. 19 Distal tibiofibular joint distraction proximal migration of distal fibula measured on preoperative and postoperative radiographs

Fig. 20 Talocrural angle Fig. 21 Bimalleolar angle

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Cross-References

References and Suggested Reading

▶ Eight Year Old Child with Congenital Pseudarthrosis Tibia and Severe Deformity. Correction of Deformity with Double Osteotomy, Insertion of Fassier-Duval Rod and OP-1 Application

1. Anderson W. Lengthening of the lower limb: its place in the problem of limb discrepancy. London: Butterworths; 1962. p. 1–22. 2. Hatzokos I, Drakou A, Christodoulou A, Terzidis I, Pournaras J. Inferior subluxation of the fibular head following tibial lengthening with a unilateral external fixator. J Bone Joint Surg Am. 2004;86:1491–6. 3. Ilizarov GA. Clinical application of the tension stress effect for limb lengthening. Clin Orthop Relat Res. 1990;250:8–26. 4. Kim S-J, Agashe MV, Song S-H, Song H-R. Fibula related complications during bilateral tibial lengthening. Acta Orthop. 2012;83(3):271–5. 5. Saleh M, Bashir HM, Farhan MJ, McAndrew AR, Street R. Tibial lengthening: does the fibula migrate? J Pediatr Orthop B. 2002;11:302–6. 6. Shyam AK, Song HR, An H, Isaac D, Shetty GM, Lee SH. J Bone Joint Surg. 2009;91:1671–82.

See Also in Vol. 2 Ankle Distraction Supramalleolar Osteotomy with Ankle Distraction

Multiple Lower Limb Deformities in a 14 Year Old Girl with Multiple Epiphyseal Dysplasia and Low Lumbar Spina Bifida

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Elizabeth Ashby and Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 721 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 722 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 724 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 724 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 724 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 724

Abstract

This is a case of a 14 year old girl with multiple epiphyseal dysplasia (MED) and low lumbar spina bifida presenting with a windswept leg deformity, limb length inequality (LLI), right hip dislocation, left foot and ankle deformity, and long thoracolumbar scoliosis. Each deformity was surgically corrected in a stepwise manner. Several different deformity correction techniques were used including gradual correction with a frame, guided growth and acute correction with an osteotomy.

E. Ashby (*) Division of Orthopaedics, Shriners Hospital, Montreal, QC, Canada e-mail: [email protected]

Brief Clinical History A girl was born with lower lumbar spina bifida and a diastematomyelia. Repair of the split cord was performed at age 5 months. The procedure was repeated at age 12 years for tethered cord symptoms. Over the course of her childhood, she developed a dislocated right hip, an increasing windswept lower limb deformity with bilateral knee pain, leg length inequality (LLI), left foot and ankle deformity, and scoliosis secondary to a tethered cord. She was diagnosed with multiple epiphyseal dysplasia. A management plan was established for each limb deformity and they were surgically corrected in a stepwise manner over 3 years. At age 20 years this young lady is mobilizing with no aids and planning to start a family. She has persistent left knee and bilateral hip pain. A total knee replacement is planned in the near future followed by possible bilateral total hip replacements.

R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_24

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Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List 1. Dislocated right hip 2. Windswept leg deformity with varus deformity of the left knee originating primarily from the proximal tibia and valgus deformity of the right knee originating primarily from the distal femur 3. 6 cm LLI 4. Left foot and ankle deformity 5. Long thoracolumbar scoliosis with Cobb angle of 65

Treatment Strategy Multiple epiphyseal dysplasia (MED) is a rare genetic condition with autosomal dominant and recessive types. It is characterized by abnormal epiphyseal cartilage, particularly

E. Ashby and R. C. Hamdy

in weight-bearing joints. Many patients develop severe hip and knee osteoarthritis by the second or third decades of life. Correcting lower limb deformity will not change the natural history of MED and will not slow down osteoarthritis progression. However, it will improve mobility and appearance in the short term and will facilitate arthroplasty surgery in the long term. The treatment strategy in this case was to correct lower limb alignment in a stepwise manner in the following order: 1. Correct the left varus tibial deformity and LLI using an Ilizarov frame. 2. At the same time treat the right valgus knee deformity with guided growth by using a medial distal femoral hemiepiphysiodesis. 3. Correct the left ankle deformity acutely with an osteotomy stabilized with staples. 4. Perform an open reduction of the right hip, a varus derotational osteotomy of the proximal femur, and a Chiari pelvic osteotomy.

Basic Principles The basic principles of this case are to achieve straight legs, which are equal in length, with stable joints. The main dilemma in this case is the order of deformity correction. There are two possible strategies: start proximal and work distally (spine, then hips, then legs) or start distal and work proximally (legs, then hips, then spine). To correct the long thoracolumbar curve, spinal fusion to the pelvis would be necessary. All ambulatory movement would then come from the hips. In the presence of a dislocated right hip, the ability to ambulate could be lost. Therefore, it was decided to correct the limb and hip deformities before addressing the spine.

Images During Treatment See Figs. 3 and 4.

Technical Pearls

Fig. 1 Standing anteroposterior radiograph of both legs showing the windswept leg deformity, leg length inequality, and dislocated right hip

There are no specific technical pearls for this case. However, the main learning point is the methodical approach to managing a child with multiple lower limb deformities. A surgical plan should be devised for each deformity. The corrections should then be performed in a stepwise, timely manner.

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Multiple Lower Limb Deformities in a 14 Year Old Girl with Multiple Epiphyseal Dysplasia and Low Lumbar. . .

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Fig. 2 (a) Anteroposterior and (b) lateral radiographs of the left foot and ankle

Fig. 3 Immediate postoperative anteroposterior radiograph of the left tibia with Ilizarov frame in situ

Fig. 4 Anteroposterior long-leg standing radiograph prior to frame and staple removal showing correction of knee deformities

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Outcome Clinical Photos and Radiographs See Figs. 5 and 6.

Avoiding and Managing Problems

Fig. 5 Anteroposterior radiograph of the pelvis following reconstruction of the right hip

1. The natural history of MED must be discussed with the patient and family. They must understand that limb realignment surgery will not affect joint disease progression, and joint replacement surgery will almost certainly be needed in the future. 2. We believe that when a knee deformity and dislocated hip are present in the same leg, the knee deformity should be corrected prior to hip reconstruction. We believe that it is almost impossible to plan a proximal femoral osteotomy with a deformed knee. However, once a normal tibiofemoral angle has been achieved, the proximal femoral osteotomy can be planned in a normal manner.

Cross-References ▶ Journey of a Child Born with Severe Arthrogryposis and Lower Limb Deformities ▶ Synchronization of Surgical Interventions for Multiple Deformities in a Four Year Old with Arthrogryposis

References and Suggested Reading 1. Bajuifer S, Letts M. Multiple epiphyseal dysplasia in children: beware of overtreatment! Can J Surg. 2005;48:106–9. 2. Cho TJ, Choi IH, Chung CY, Yoo WJ, Park MS, Lee DY. Hemiepiphyseal stapling for angular deformity correction around the knee joint in children with multiple epiphyseal dysplasia. J Pediatr Orthop. 2009;29:52–6. 3. Li LY, Zhao Q, Ji SJ, Zhang LJ, Li QW. Clinical features and treatment of the hip in multiple epiphyseal dysplasia in childhood. Orthopedics. 2011;34:352. 4. Lim SJ, Park YS, Moon YW, Jung SM, Ha HC, Seo JG. Modular cementless total hip arthroplasty for multiple epiphyseal dysplasia. J Arthroplast. 2009;24:77–82. 5. Sebik A, Sebik F, Kutluay E, et al. The orthopaedic aspects of multiple epiphyseal dysplasia. Int Orthop. 1998;22:417–21. 6. Yilmaz G, Oto M, Thabet AM, Rogers KJ, Anticevic D, Thacker MM, Mackenzie WG. Correction of lower extremity angular deformities in skeletal dysplasia with hemiepiphysiodesis: a preliminary report. J Pediatr Orthop. 2014;34:336–45.

Fig. 6 Anteroposterior radiograph of the left ankle following distal tibial osteotomy

Refracture, Soft Tissue Contracture, and Angular Deformity After Femoral Lengthening in Achondroplasia

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Hae-Ryong Song and Kwang-Won Park

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 725 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 725 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 725 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 726 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 727 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 727 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 727 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 730 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 730 See Also in Vol. 2 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 731 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 731

Abstract

Extensive limb lengthening may be indicated in patients with achondroplasia in order to improve their quality of life. However, excessive lengthening is associated with decreased ROM of the adjacent joints, decreased new bone formation, delayed weight bearing, and fractures of the regenerate bones. Many surgeons have recommended that the goal of lengthening in a bone segment should be limited to 20~30% of its initial length. And fractures of the regenerate bones may be avoided by careful analysis of the regenerate bone in the distraction gap before removal of the apparatus.

Brief Clinical History Eight-year-old boy patient, who diagnosed with achondroplasia, was admitted for limb lengthening surgery. He underwent bilateral 8-cm lengthening of tibia 18 months ago. At the time of visit, he was complaining about gait abnormality caused by disproportionate limb length.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

Preoperative Problem List H.-R. Song (*) · K.-W. Park Department of Orthopedic Surgery, Korea University Medical Center, Guro Hospital, Seoul, South Korea e-mail: [email protected]; [email protected]

I. Angular deformities (Varus tendency at the lengthening site and valgus tendency of mechanical axis) II. Soft tissue contractures of the adjacent joints

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_73

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Fig. 1 Lower extremity AP at the time of initial visit (5 years 10 months). Rt.limb:mLDFA 99
, mMPTA 85
, MAD 13 mm. Lt. limb:mLDFA 84
, mMPTA 86
, MAD 14 mm

(Flexion contracture of the hip, extension contracture of the knee, tightness of the iliotibial band) III. Poor callus formation and refracture of the regenerate bones IV. Difficulty in determining removal timing of the external fixators (Specific problems related with patients with achondroplasia) (i) Relatively short bone segment – The level of osteotomy should be located in mid-diaphyseal area. (ii) Malalignment (coxa vara) of the proximal femur – Prone to fractures of the regenerate bone. (iii) Previous tibial lengthening – Decreased potency of new bone formation in distraction osteogenesis and subsequent lateral instability of the knee joint.

Treatment Strategy I. Align the axis of external fixator according to the mechanical of axis of the lower extremity.

Fig. 2 Clinical photo of the patient

II. Consider release of hip flexor muscles (sartorius, fascia lata, rectus femoris), quadricepsplasty for knee flexion, and Z-plasty of the iliotibial band for treatment of valgus knee. III. Avoid using wires for soft tissue impingement near the joint. Consider half-pin insertion using the intermuscular planes. IV. Evaluate properly of the quality of regenerate bones. Use the Pixel Value Ratio (PVR) method. V. Use PVR to determine proper timing of removal of the external fixators. (Specific treatment strategy related with patients with achondroplasia) (i) Timing of surgery – Consider significant disturbance of growth after extensive lengthening in skeletally immature patients with achondroplasia. (ii) Malalignment of the lower limb – Thorough preoperative evaluation of the patient’s rotational profile and joint orientations.

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Refracture, Soft Tissue Contracture, and Angular Deformity After Femoral Lengthening in Achondroplasia

Basic Principles I. Determining the amount of lengthening Excessive lengthening was associated with decreased new bone formation and delayed weight bearing. Large amount of lengthening more than 50% of their initial femoral length was associated with an increased incidence of concave, lateral, and central callus shapes, adjacent joint stiffness, and fracture [5]. II. Avoiding angular deformity When an external fixator is used to lengthen the femur, the device can be aligned with the mechanical or the anatomical axis. Aligning it with the anatomical axis theoretically leads to medialization of the knee joint (lateralization of the mechanical axis line). Therefore, most femoral external fixators should be aligned with the mechanical axis.

727

III. Management of soft tissue Excessive lengthening is associated with a decreased ROM of the adjacent joints. Knee movement is known to help to prevent muscle atrophy and adhesion of the muscles to the underlying bone regenerate and to increase the blood supply to bone and bone strength [1]. Excessive lengthening affects the thigh muscles by unable to keep pace with the lengthening, resulting in a decreased ROM at the knee and fixed flexion at the hip. IV. Evaluation of the regenerate bone (Pixel Value Ratio, PVR) The pixel values of the proximal, distal, and regeneration areas could be calculated from the mean value of each area using the free line range of interest (ROI) method. V. Timing of the external fixator removal (PVR) A pixel value ratio of 1 indicated corticalization of the regenerate in the lengthening area. The external fixators should be removed based on a PVR of at least 1.0.

Images During Treatment Figs. 4, 5, 6, 7, 8, 9, and 10.

Technical Pearls I. Early physeal closure related to lengthening loss ranging from 1.5 to 4 cm after extensive tibial and femoral lengthening could occur after extensive limb lengthening

Fig. 3 POD 1 year 6 months after 8 cm of lengthening, correctional osteotomy, and external fixator application with Ilizarov apparatus (7 years 4 months). Rt.limb:mLDFA 84
, mMPTA 93
, MAD 10 mm. Lt.limb:mLDFA 87
, mMPTA 97
, MAD 14 mm

Fig. 4 After femoral lengthening osteotomy and external fixation with monolateral fixator (8 years 2 months)

728 Fig. 5 POD 3 months after 9 cm of lengthening (8 years 5 months) and Assessment of Pixel value based on a radiograph in PACS system. Note the red circle showing the pixel value of range of interest (ROI) drawn at each segment. The value M (encircled in red) indicates the mean pixel value for that segment and can be used in the computation for the PVR pixel value ratio ¼ [(pixel value of the proximal segment + pixel value of the distal segment)/ 2]/pixel value of the regenerate [3]

Fig. 6 Clinical photos showing flexion contracture of the hip joint caused by excessive lengthening. He was treated with releasing of sartorius, fascia lata, straight head of rectus femoris, and psoas muscle

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Fig. 7 POD 1 year 3 months after 9 cm of lengthening (9 years 5 months) showing concave and lateral type of poor callus formation

Fig. 8 Third operation for regenerate fracture with additional bone graft (DBM and PRP) and internal fixation (9 years 5 months)

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Fig. 9 POD 1 year (11 years 5 months) after removal of implant on left side. Rt.limb:mLDFA 91
, mMPTA 89
, MAD 2 mm. Lt.limb: mLDFA 102
, mMPTA 95
, MAD 19 mm

Fig. 10 After bifocal femoral osteotomy and monolateral external fixator application on left limb (11 years 5 months) and monofocal femoral osteotomy and monolateral fixator application on right limb (12 years 4 months)

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in skeletally immature patients with achondroplasia [4] and start lengthening after 10 years old. II. Aligning the external fixators with the mechanical axis did not guarantee anatomical alignment after lengthening. There is tendency of varus angulation due to absence of medial distraction when using monofixator and tendency of valgus knee caused by iliotibial band. III. Regular radiological examination, physiotherapy, careful adjustment of the distraction rate, and soft tissue releases should be considered during large amount of femoral lengthening. And regular stretching and the maintenance of a good ROM can help to decrease the pain and muscle spasm during lengthening. IV. The radiological features of the callus in distraction osteogenesis could be classified with regard to shape and type on the basis of the Li classification [2]. The shape was based on the width of the callus compared to the original osteotomy site. The type was based on the callus shapes (cylindrical, fusiform, and concave), three patterns of osteogenesis (homogeneous, heterogeneous, and lucent), and three densities (low, intermediate, and normal). When there is concave or lateral type of poor callus formation, compression is necessary until fusiform or cylinder type of callus is visible (Fig. 11). Description Regeneration wider than the original bone Regeneration same width as the original bone Regeneration narrower than the original bone

Fig. 11

Name Fusiform Cylindrical Concave (continued)

Description Regeneration mainly on one side of the distraction gap Regeneration a thin pillar

Name Lateral Central

V. We can safely rely on PVR values to define the timing of fixator removal, as it exhibits a good correlation in patients with a cylindrical callus pattern regardless of whether the pathway is homogeneous or heterogeneous [3].

Outcome Clinical Photos and Radiographs See Figs. 12 and 13.

Avoiding and Managing Problems I. Treating physicians should include the possibility of early physeal closure in preoperative counseling of the patients and their parents and also should be cautious in determining the amount of lengthening. II. Treating physicians should consider difference of axis between mechanical axis and anatomical axis in coronal plane and axial deviation during femoral lengthening and also their surrounding soft tissue conditions. III. Various soft tissue procedures such as release of the fascia of vastus lateralis, tensor fascia lata, psoas tendon, hamstring tendon, and iliotibial band could be used to correct joint stiffness.

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Refracture, Soft Tissue Contracture, and Angular Deformity After Femoral Lengthening in Achondroplasia

Fig. 12 POD 6 months after femoral correction (13 years). Rt.limb: mLDFA 87
, mMPTA 89
, MAD 2 mm. Lt.limb: mLDFA 95
, mMPTA 100
, MAD 2 mm

See Also in Vol. 2 Femoral Bone Defect

References and Suggested Reading 1. Herzenberg JE, Scheufele LL, Paley D, Bechtel R, Tepper S. Knee range of motion in isolated femoral lengthening. Clin Orthop Relat Res. 1994;301:49–54.

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Fig. 13 Clinical photo at the time of last visit (13 years) 2. Li R, Saleh M, Yang L, Coulton L. Radiographic classification of osteogenesis during bone distraction. J Orthop Res. 2006;24–3:339–47. 3. Song SH, Sinha S, Kim TY, Park YE, Kim SJ, Song HR. Analysis of corticalization using the pixel value ratio for fixator removal in tibial lengthening. J Orthop Sci. 2011;16–2:177–83. 4. Song SH, Kim SE, Agashe MV, Lee H, Refai MA, Park YE, Choi HJ, Park JH, Song HR. Growth disturbance after lengthening of the lower limb and quantitative assessment of physeal closure in skeletally immature patients with achondroplasia. J Bone Joint Surg (Br). 2012;94–4:556–63. 5. Venkatesh KP, Modi HN, Devmurari K, Yoon JY, Anupama BR, Song HR. Femoral lengthening in achondroplasia: magnitude of lengthening in relation to patterns of callus, stiffness of adjacent joints and fracture. J Bone Joint Surg (Br). 2009;91–12:1612–7.

Retrograde Insertion of a SLIM Nail in a Femur in a Patient with Osteogenesis Imperfecta

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Khaled Abu Dalu, Yousef Marwan, and Mitchell Bernstein

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 733 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

733 733 734 734 734 734 735 735

References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 738

Abstract

This is a case of a 17-year-old female patient with a severe form of osteogenesis imperfecta (OI) type IV who had multiple previous surgeries of the lower limbs to correct deformities and treat fractures. She had a BMI of 42. She presented with right femur fracture below a retained Kirschner-type wire implant. This fracture was treated successfully via retrograde application of the SLIM (Simple Locking IntraMedullary nail by Pega Medical, Laval, Canada).

Brief Clinical History The patient is a morbidly obese 17-year-old female who is known to have type IV OI. She sustained a right distal third femoral shaft fracture 6 weeks prior to her presentation with a retained Rush rod from a previous fracture fixation. She is known to be ambulatory; however, she was not able to bear weight on her right lower extremity following the recent injury.

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List K. A. Dalu · Y. Marwan Shriners Hospitals for Children, Montreal, QC, Canada McGill University Health Centre, McGill University, Montreal, QC, Canada e-mail: [email protected] M. Bernstein (*) Departments of Surgery & Pediatric Surgery (Division of Orthopaedics), McGill University Health Center & Shriners Hospital for Children Canada, Maywood, IL, USA e-mail: [email protected]

1. Subacute right distal third femoral fracture (6 weeks) in skeletally mature patient 2. Osteopenic bone 3. OI, type IV 4. Significant mismatch between size of bone and mass of patient, retained previous implant

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_408

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Fig. 1 Preoperative X-rays. (a) Anteroposterior (AP) view of lower limbs, showing the fracture and the retained implant in the right femur; (b) lateral view of the right lower extremity showing posterior displacement of her femur fracture

5. Pseudoarthrosis lesion proximal to the current fracture from a previous fracture 6. BMI 42

Treatment Strategy Treatment strategy included removal of the retained implant, open reduction of the fracture site for accurate intramedullary access. In this case, open reduction of the fracture is critical to ensure safe re-canalization of implant to stabilize the segment. It was decided to utilize a retrograde approach for the SLIM nail through the distal segment due to her obesity to ease the insertion of the nail. Also, performing osteotomies was expected to realign the femur as needed. To ensure appropriate rotational stability of the bony segments, interlocking the rod and the addition of a supplemental plate at the fracture site were planned.

Basic Principles • In patients with OI, avoid using plate and screws as the main fixation implants. Such devices create stress riser points and lead to periprosthetic fractures. • Planning for surgery is crucial. It should include measurement of the canal diameter, determining the levels of the

corrective osteotomies and choosing the appropriate fixation implants. • OI patients require a multidisciplinary approach to their care to optimize the treatment outcomes. In addition to surgical treatment, medical treatment and rehabilitation are important.

Images During Treatment See Figs. 2, 3, and 4.

Technical Pearls 1. For OI cases, transport and position the patient on the operating room (OR) carefully to avoid iatrogenic fractures. 2. Understand the implant options and how to use them very well. Have the company representative available in the OR for assistance. In skeletally mature OI patients, telescoping rods are not required anymore. Therefore, the use of solid nails (e.g., SLIM and Gap nails from Pega medical) can be used. 3. Correct the alignment before inserting the guidewire and start reaming the medullary canal.

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4. Perform percutaneous osteotomies with multiple drill holes and sharp osteotomes to realign the bone. 5. Avoid eccentric reaming of the medullary canal by realigning the bone and proper placement of the guide wire. 6. Avoid using large diameter nails that fit the medullary canal tightly as this leads to bone resorption in cases with osteopenic bone like OI. 7. Ensure proper selection of entry point. Poor nail entry point will result in malalignment. 8. Test the rotational stability of the fracture/osteotomy fixation intraoperatively. Have low threshold to add a supplemental plate to improve the mechanical environment at the fracture/osteotomy site.

Outcome Clinical Photos and Radiographs See Figs. 5 and 6.

Avoiding and Managing Problems

Fig. 2 Clinical image showing the importance of proper positioning especially in a patient with morbid obesity. Free draping the limb on a radiolucent table is important for access. A bump (e.g., a rolled blanket) is placed under the ipsilateral buttock to internally rotate the leg to help with rotational alignment. A radiolucent ramp is placed under the ipsilateral limb to elevate it for “shoot-through” lateral imaging

1. Avoid aggressive manipulation of the bone in patients with OI to prevent iatrogenic fractures. 2. Use interlocking pins with the SLIM nail to prevent implant migration. 3. Supplemental plate fixation would add better mechanical advantages to help promote healing and minimize rotational instability at the fracture and osteotomy sites.

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Fig. 3 Fluoroscopic images showing some of the surgical steps. (a) Identifying the fracture site before making any skin incision; (b) protrusion of the implant proximally through the piriformis fossa; (c) using

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a K-wire to locate the tip of the Rush rod proximally to prepare for removal; (d, e) Identifying the entry point for the new rod; (f, g, e) retrograde reaming and osteotomy planning

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Retrograde Insertion of a SLIM Nail in a Femur in a Patient with Osteogenesis Imperfecta

Fig. 4 Intraoperative fluoroscopic images. (a, b) AP and lateral view showing the initial attempt to realign the proximal and distal segments; (c) creating an intermediate segment using ½ inch osteotome; (d, e) AP

Fig. 5 Final intraoperative fluoroscopic images demonstrating supplemental fixation of the three bony segments with one-third tubular locking plate

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and lateral views after the osteotomy; (f, g) AP and lateral view showing the new intermediate-segment position (arrows); (h, i) insertion of the interlocking K-wire

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Fig. 6 X-rays of the right femur obtained 4 and 7 months postoperatively demonstrating excellent fracture and osteotomy healing with acceptable alignment

References and Suggested Reading 1. Musielak BJ, Woźniak Ł, Sułko J, Oberc A, Jóźwiak M. Problems, complications, and factors predisposing to failure of Fassier-Duval

rodding in children with osteogenesis imperfecta: a double-center study. J Pediatr Orthop. 2021;41(4):e347–52. 2. Mulpuri K, Joseph B. Intramedullary rodding in osteogenesis imperfecta. J Pediatr Orthop. 2000;20(2):267–73.

Revision of Bilateral Tibial Fassier-Duval Rods in an Eleven Year Old Girl with Osteogenesis Imperfecta

106

Elizabeth Ashby, Reggie C. Hamdy, and Franc¸ois Fassier

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 739 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 740 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 740 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 740 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 740 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 740 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 740 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 741 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 741 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 741 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 742

Abstract

This is a case of bilateral tibial bowing in an 11 year old girl with osteogenesis imperfecta type IV with telescopic Fassier-Duval rods in situ. Removal of bowed intramedullary implants is not straight forward. We describe the surgical technique in detail. Once the implants were removed, further osteotomies were performed and a more lateral entry point was used to reinsert each Fassier-Duval rod. This ensured the valgus deformity was fully corrected. E. Ashby (*) Division of Orthopaedics, Shriners Hospital, Montreal, QC, Canada e-mail: [email protected] R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] F. Fassier Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada e-mail: [email protected]

Brief Clinical History An ambulatory girl with osteogenesis imperfecta type IV was treated medically for osteopenia with intravenous bisphosphonates. At the age of 4 years she underwent surgical correction of bilateral tibial and femoral deformities with multiple osteotomies and insertion of telescopic FassierDuval rods. The left tibial rod was exchanged at the age of 6 years due to fracture and deformity. The right femoral rod was exchanged at age of 8 years for the same reason. Both tibial rods extended with growth leading to a decrease in telescoping between the male and female parts. Progressive valgus deformity in the tibiae led to bending of the rods that ultimately led to no further telescoping. At the age of 11 years the intermalleolar distance was 6 cm and increasing. It was therefore decided to proceed with revision bilateral tibial rodding. This involved removal of the previous implants, repeat tibial osteotomies (one on the right and two on the left) and reinsertion of the telescopic Fassier-Duval rods using a more laterally placed entry point.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_336

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Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List 1. Bilateral tibial bowing 2. Implants in situ that require removal 3. Poor bone quality

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1. Perform as many osteotomies as necessary to achieve straight bones. 2. Reinsert expandable Fassier-Duval rods to protect the whole length of the growing tibiae. 3. When reinserting the Fassier-Duval rods, use a more lateral entry point to ensure the deformity is fully corrected. If the same entry point is used, some valgus deformity will persist.

Images During Treatment Treatment Strategy 1. Stop medical treatment a minimum of 48 h prior to surgery and restart a minimum of 4 months after surgery. 2. Obtain clinically straight tibiae. Careful preoperative planning of the osteotomy sites is important. Intraoperative X-ray images obtained in several planes may lead to changes in the plan. 3. Protect the growing tibiae with telescopic FassierDuval rods.

Basic Principles The basic principles of this case are to correct the deformity and prevent future fracture. This is achieved in the following ways:

See Fig. 2.

Technical Pearls 1. Plan the site of the first osteotomy preoperatively. Use a Midas Rex Microsaw (Medtronic) to start the osteotomy and cut through the Fassier-Duval rod. Once the rod is cut, perform the remaining part of the osteotomy using osteotomes. 2. Bone that has been cut with the Midas Rex Microsaw will be damaged. Remove it using a rangeur. 3. Further tibial osteotomies can be performed percutaneously using multiple drill holes and an osteotome.

Fig. 1 Preoperative anteroposterior and lateral X-rays showing bilateral tibial bowing with Fassier-Duval rods in situ

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Fig. 2 Surgical stages to remove a Fassier-Duval rod. (a) Deformed tibia with Fassier-Duval rod in situ. (b) Perform the tibial osteotomy. (c) Remove the distal female rod using pliers. (d) Remove the distal male rod using pliers or a T-handle. (e) Insert a wire (the same size as the male rod) into the distal end of the remaining female rod. Push the male rod into the knee under X-ray guidance. Make an appropriate incision in the

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knee and remove the male rod using pliers. (f) Engage the screwdriver in the female head. While unscrewing, apply pressure at the distal end of the female part using a rod of the same diameter. This will aid the removal. (g) When inserting the new Fassier-Duval rod, use a more lateral entry starting point

See Fig. 3.

4. Protect the osteotomies in above-knee plaster immobilization postoperatively and avoid weight bearing for 3 weeks. At this time, ankle-foot orthoses should be fitted to allow weight bearing.

Avoiding and Managing Problems

See Also in Vol. 3

1. Do not use the largest diameter rod that fits the canal as this can lead to bone resorption. 2. Ensure the threads of the male component are in the epiphysis on the lateral intraoperative X-ray view. The anteroposterior view can be misleading. 3. Ensure the male part is not too long within the knee. This could cause articular cartilage damage.

Deformity of the Humerus in a Four Year Old Boy with Osteogenesis Imperfecta Forearm Deformity in a Fourteen-Year-Old Boy with Osteogenesis Imperfecta

Outcome Clinical Photos and Radiographs

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Fig. 3 Immediate postoperative anteroposterior and lateral radiographs of both tibiae showing correction of deformity

References and Suggested Reading 1. Anam EA, Rauch F, Glorieux FH, Fassier F, Hamdy RC. Osteotomy healing in children with osteogenesis imperfecta receiving biphosphonate treatment. J Bone Miner Res. 2015;30(8):1362–8. 2. Burnei G, Vla C, Georgescu I, Gavriliu T, Dan D. Osteogenesis Imperfecta: diagnosis and treatment. J Am Acad Orthop Surg. 2008;16:356–66. 3. Esposito P, Plotkin H. Surgical treatment of osteogenesis imperfecta: current concepts. Curr Opin Pediatr. 2008;20:52–7.

4. Fassier F, Glorieux FH. Osteogenesis Imperfecta. In: Surgical techniques in orthopaedics and traumatology. Paris: Elsevier SAS; 2003. p. 1–8; 55-050-D-30. 5. Ruck J, Dahan-Oliel M, Montpetit K, Rauch F, Fassier F. FassierDuval femoral rodding in children with osteogenesis imperfecta receiving bisphosphonates: functional outcomes at one year. J Child Orthop. 2011;5:217–24. 6. Shapiro JR, Sponsellor PD. Osteogenesis Imperfecta: questions and answers. Curr Opin Pediatr. 2009;21:709–16.

Revision of Femur Fassier-Duval Rod for Intertrochanteric Fracture Nonunion with Lytic Cyst and Coxa Vara in Osteogenesis Imperfecta Patient

107

Khaled Abu Dalu, Mitchell Bernstein, and Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 743 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 744 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 744 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 744 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 745 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 746 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 746 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 747 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 747 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 747 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 747

Abstract

This is a case of Fassier-Duval (FD) rodding used in revision fixation for a 5-year-old boy with osteogenesis imperfect (OI) type IV who presented with intertrochanteric fracture non-union with cyst development. Deformities of the right femur were corrected with other bone deformities at age 2 years using osteotomy and insertion of FD rod. A fracture non-union with cyst formation at the K. A. Dalu (*) Shriners Hospitals for Children, Montreal, QC, Canada McGill University Health Centre, McGill University, Montreal, QC, Canada e-mail: [email protected] M. Bernstein Departments of Surgery & Pediatric Surgery (Division of Orthopaedics), McGill University Health Center & Shriners Hospital for Children Canada, Maywood, IL, USA e-mail: [email protected] R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

intertrochanteric area developed at age 4 years with accompanied with coxa vara. This was addressed by corrective osteotomy, rod exchange, and fixation of the proximal femur with K-wires and cerclage. This case presents a complex clinical scenario; it highlights the challenges of managing non-union in the context of OI and can further our understanding of its associated complications.

Brief Clinical History A 5-year-old boy with OI type IV was presented with right hip pain and difficulty walking. He was known to the practice from prior treatment. His medical therapy had included bisphosphonates (Zoledronic acid). At age of 2 years he was presented with bilateral lower limb deformities affecting femur and tibia (bowed legs). He underwent corrective osteotomies with FD rodding in four bones of the lower extremities. He subsequently developed in right femur an intertrochanteric cyst and sustained a nondisplaced fracture

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_586

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at the lower border of the cyst, rod protrusion and progressive coxa vara at age of 4 years. (Were there any thoughts regarding why fixation was insufficient, particularly only in one side? Such thought can guide readers who wish to gain insight to their own work to avoid such an issue, and guides the intervention that is planned.) The recommended intervention was: The revision included removal of the FD rod, bone cyst aspiration, corrective osteotomy of the coxa vara, stabilization of the osteotomy with new FD rod, and supplemental K-wires and bone graft.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Fig. 1 (a) Preoperative anteroposterior X-ray of the right femur showing the protrusion of the Fassier-Duval nail proximally and the lytic lesion. (b) Coronal cut of the CT scan showing the boundaries of the lytic lesion. (c) Coronal cut the proximal femur showing a nonhealing

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Preoperative Problem List 1. 2. 3. 4.

Nonunion of right intertrochanteric fracture Cystic bony lesion of the proximal femur Coxa vara deformity Protrusion of the FD rod proximally and progression of the bone deformity

Treatment Strategy 1. To address the intertrochanteric cystic lesion by curettage and bone grafting (how?). 2. To increase the neck-shaft femoral angle to approximately 135 . 3. To stabilize the whole length of the femur with intramedullary fixation nail (how, using what devices?) while maintaining femoral neck angle in a patient with OI.

fracture at the lower edge of the lytic lesion.(d) Axial cut of the CT showing the Fassier-Duval rod outside the bone proximally on the right side

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Revision of Femur Fassier-Duval Rod for Intertrochanteric Fracture Nonunion with Lytic Cyst and Coxa Vara. . .

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Fig. 2 (a, b) Anteroposterior preoperative X-ray of the right femur and the preoperative planned correction lines. (c, d) Coronal cuts of the proximal femur and the preoperative correction lines

Basic Principles The basic principles of this case are to address multiple pathologies, including the active cystic lesion, the nonunion in the intertrochanteric area, and correcting the deformity. This can be achieved as follows: 1. Perform cyst aspiration, curettage, and bone grafting with demineralized bone matrix. 2. Insertion of two K-wires along the femoral neck, to be used as joystick for better adjustment of the alignment of the proximal bony fragment and later as supplemental fixation of the subtrochanteric osteotomy.

3. The K-wires must be advanced through the physis for better purchase of the femoral head. 4. Performing subtrochanteric corrective osteotomy to achieve deformity corrections (mainly the coxa vara) and preventing further deformity at the level of the protruded FD rod. 5. Reinsertion of telescoping Fassier-Duval rod between the two K-wires in appropriate angle to correct the coxa vara. 6. Bending the K-wires and secure against the femoral shaft below the level of the osteotomy using cerclage cables. 7. The ideal fixation method in treating fracture or deformity correction in OI patient achieved with IM nail and not a plate. The IM nail distributes the forces along the entire length of the bone and minimizes the stress concentration

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at the fracture site. Plates can be used as supplemental tool to provide additional rotational stability (what is the specific benefit of the plate as a supplement?).

Images During Treatment See Fig. 3.

Fig. 3 Intraoperative radiographs demonstrating the surgical steps, (a) addressing the lytic lesion, (b) insertion of two K-wires along the femoral neck into the head. (c) Subtrochanteric corrective osteotomy at the lower border of the nonunion site and the planned correction. (d)

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Technical Pearls 1. Perform meticulous aspiration and curettage of the bony lytic cyst to prevent recurrence of the lesion. 2. After achieving good fixation of the deformity correction, the lesion should be filled with bone graft. 3. While inserting the K-wires along the femoral head ensure that one is anterior and the other K-wire inserted posterior

Reaming of the proximal fragment. (e, f) AP and lateral of the final radiographs with the FD rod, bent K-wires. and supplemental cerclage cables

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Fig. 4 (a, b) AP and axial radiographic X-ray views of the right femur 6 months after surgery showing full healing of the lytic lesion and the osteotomy site

to allow the insertion of the FD rod later, in addition the K-wires should cross each other in the axial plane, which creates a truss-like stabiliaing effect. 4. Secure the K-wires with cerclage cables to ensure two-point fixation.

Filling the cyst with DBM should increase the chances of healing and resolving of the lytic lesion. Non-weight-bearing should be the postoperative recommendation until healing is proved radiologically.

Cross-References Outcome Clinical Photos and Radiographs See Fig. 4.

Avoiding and Managing Problems The inserted FD rod should be with reasonable diameter and not the largest that can fit, to prevent bone resorption; a large diameter nail will lead to “disappearing bone effect,” stress is needed for developing and maintaining bone density, and bone reaming should be minimally used before nail insertion. To prevent displacement of the achieved alignment or cut out of the FD rod, K-wires should be hold in place, a nonthreaded K-wires can cross the growth plate without complications and that is needed for better bone purchase and more rigid fixation.

▶ Revision of Telescoping Rod and Plate Complicated with Nonunion and Metallosis in a Girl with Osteogenesis Imperfecta Type VIII

References and Suggested Reading 1. Fassier F, Ashby E, Hamdy RC. Case 86: Coxa Vara in a Nine-YearOld Boy with Osteogenesis Imperfecta. In: Rozbruch S, Hamdy R, (eds) Limb Lengthening and Reconstruction Surgery Case Atlas. Springer, Cham. 2015. https://doi.org/10.1007/978-3-31918023-6_20. 2. Aarabi M, Rauch F, Hamdy RC, Fassier F. High prevalence of coxa vara in patients with severe osteogenesis imperfecta. J Pediatr Orthop. 2006;26:24–8. 3. Fassier F, Sardar Z, Aarabi M, Odent T, Haque T, Hamdy R. Results and complications of a surgical technique for correction of coxa vara in children with osteopenic bones. J Pediatr Orthop. 2008;28: 799–805.

748 4. Iwata H, Sakano S, Itoh T, Bauer TW. Demineralized bone matrix and native bone morphogenetic protein in orthopaedic surgery. Clin Orthop Relat Res. 2002;395:99–109. 5. Boyan BD, Ranly DM, Schwartz Z. Use of growth factors to modify osteoinductivity of demineralized bone allografts: lessons for tissue engineering of bone. Dent Clin. 2006;50(2):217–28.

K. A. Dalu et al. 6. Drosos GI, Touzopoulos P, Ververidis A, Tilkeridis K, Kazakos K. Use of demineralized bone matrix in the extremities. World J Orthop. 2015;6(2):269. 7. Fassier FR. Osteogenesis imperfecta-who needs rodding surgery? Curr Osteoporos Rep. 2021;19(3):264–70. https://doi.org/10.1007/ s11914-021-00665-z. Epub 2021 Mar 1. PMID: 33646506

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Revision of Telescoping Rod and Plate Complicated with Nonunion and Metallosis in a Girl with Osteogenesis Imperfecta Type VIII Dmitry Popkov and Pierre Lascombes

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 750 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 750 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 750 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 750 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 751 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 751 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 751 Outcome Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 752 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 752 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 752

Abstract

This is a case of a 13-year-old girl with Type VIII osteogenesis imperfecta (OI), who required care for femur nonunion and problems with the Fassier-Duval (FD) rod and other local hardware. A revision surgery featuring hardware removal and fixation with intramedullary pins and an external fixator was successful in achieving anatomic union. Telescopic rodding is recognized as the most successful system for long-lasting intramedullary osteosynthesis dedicated to correction of long bone deformities in children with OI. The major limitation of any intramedullary telescopic system is rotational and longitudinal instability. The combined use of telescopic rods and locking plates

with screw fixation is aimed to prevent secondary torsional deformations. However, this method does not enable axial loading on the operated site of the bone. It mandates a second operation to remove the plate after bone union. This combined approach also excludes the possibility of minimally invasive percutaneous osteotomies. Further, the proximity of dissimilar metals may lead to galvanic reaction injuring tissues and compromising hardware. It is well-known, the common metallurgic wisdom cautions against association of dissimilar metals for osteosynthesis in a biologically active environment. This impermissible situation results in corrosion that compromises the implants and leads to aseptic loosening, implant failure, or adverse biological reaction, e.g., aseptic inflammation, in host tissue.

D. Popkov (*) National Ilizarov Medical Research Center for Traumatology and Orthopaedics, Kurgan, Russia P. Lascombes University of Nancy, Nancy, France University of Geneva, Geneva, Switzerland © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_417

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Brief Clinical History

Preoperative Clinical Photos and Radiographs

A girl aged 13 years 3 months presented to our clinic with a chief complaint of inability to walk or stand following prior surgeries performed to address her underlying diagnosis of Type VIII OI. Her issue seemed most reasonably due to pain and gross instability, rather than nerve injury. Her relevant history and interventions were multiple fractures treated conservatively and with pamidronate administrations. Her prior surgeries were performed at outside institutions. At age 7 years the left femur and both tibias had FD rodding, and the right femur had Rush rodding. The right femur went on to nonunion, managed by Ilizarov external fixation combined with intramedullary hydroxyapatite-coated intramedullary nails which achieved union. Five years later (4 months prior to presentation to us), she sustained a fracture of her left femur and FD rod. This was managed with revision FD rodding, and soon after a titanium plate was added to supplement stability. Two months later, the patient then presented to our clinic with the issue as stated above. The expectation was that dissimilar metal osteosynthesis led to loosening of the plate and longitudinal and rotational recurrence. We found no signs of septic complications at admittance.

See Fig. 1.

Fig. 1 Preoperative radiographs: (a) failure of telescopic steel FD rod, pseudarthrosis of the left femur (October 2019); (b) fractured rod has been replaced with another FD rod; longitudinal instability resulted in

increasing interfragmentary gap distance; (c) combination of telescopic rod and plate 2 months after surgery; note the fracture has not united and the screws appear to be backing out of the plate and bone

Preoperative Problem List 1. 2. 3. 4.

Nonunion of the left femur Loosening of the plate and screws Longitudinal instability of the steel telescopic rod High probability of local soft-tissue injury because of metallosis.

Treatment Strategy All elements of the surgery were performed during the same intervention. The treatment started with removal of all hardware. Followed by resection of pseudarthrosis ends which was limited to non-viable bone. The combination of elastic bent intramedullary HA-coated titanium nails and limited external fixation ensured multiple advantages for consolidation of compromised bone tissue: rotational and longitudinal

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stability, early weight-bearing with axial forces stimulating healing, maintenance of compression, avoidance of casting, control for soft tissue healing.

Basic Principles The basic principles of this case are to remove dissimilar metals from the zone of pseudarthrosis and ensure favorable conditions for bone union. This is achieved in the following ways: • Removal of Fassier-Duval steel rod, titanium plate and screws, • Limited pseudarthrosis end resection providing large contact of bone fragments; • Meticulous lavage and removal of particles of metallosis, • Uuse of HA-coated intramedullary nails stimulating union of compromised bone, • Early axial weight-bearing in condition of limited external fixation. Fig. 2 Clinical photos and radiographs during treatment: (a) intraoperative view: nonunion site was approached through the previous incision, Extensive metallosis of multiple charred appearance inclusions were found around the plate in the surrounding soft tissues. Note large holes enlarged due to osteolysis. Culture and histopathology did not reveal infection; (b) after antegrade insertion of elastic HA-nails, proximal short arch with three half-pins and distal ring with three wires were applied; (c) front-view photograph showing clinical appearance of the patient standing with weight-bearing on the operated left lower limb; d—on 62nd day after surgery, bone union was established

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Images During Treatment See Fig. 2.

Technical Pearls It is critical to remove compromised tissue (such as bone) but not excessive amounts. Also important to remove compromised hardware. Reaming the bone at the prior screw sites helped achieve a uniform healthy canal. The flexible intramedullary nails inserted fully to the distal epiphysis helped achieve a straight and stable femur for frame application. The frame should be applied so that no further adjustment is needed through the treatment period. Early weight-bearing by 3–4 days prompts anabolic bone metabolism. Frame removal is indicated upon radiographic evidence of uninterrupted periosteal callus.

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Fig. 3 Radiographs after frame removal: (a) on the day of frame removal. Note uninterrupted periosteal callus and anatomical orientation of the knee joint. External fixation lasted 63 days; (b) in 2 years after

Outcome Radiographs See Fig. 3.

Avoiding and Managing Problems In patients with OI, alignment of bone fragments should be acute. Any progressive manipulations and frame adjustment result in external frame loosening, delayed union, and high risks of infection. The use of dissimilar metals in combined osteosynthesis may cause galvanic reaction, osteolysis, and early loosening of implants.

References and Suggested Reading 1. Eliaz N. Corrosion of metallic biomaterials: a review. Materials (Basel). 2019;12(3):407.

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frame removal. Note anatomical orientation of hip and knee joints, bone union and callus remodeling

2. Franzone JM, Kruse RW. Nailing with supplemental plate and screw fixation of long bones of patients with osteogenesis imperfecta: operative technique and preliminary results. J Pediatr Orthop B. 2018;27(4):344–9. 3. Popkov D, Dolganova T, Mingazov E, Dolganov D, Kobyzev A. Combined technique of titanium telescopic rods and external fixation in osteogenesis imperfecta patients: first 12 consecutive cases. J Orthop. 2020;22:316–25. 4. Popkov D. Use of flexible intramedullary nailing in combination with an external fixator for a postoperative defect and pseudarthrosis of femur in a girl with osteogenesis imperfecta type VIII: a case report. Strateg Trauma Limb Reconstr. 2018;13(3):191–7. 5. Zartman KC, Berlet GC, Hyer CF, Woodard JR. Combining dissimilar metals in orthopaedic implants: revisited. Foot Ankle Spec. 2011;4(5):318–23.

Severe Genu Valgum in Skeletal Dysplasia: Acute Versus Gradual Correction

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Reggie C. Hamdy and Neil Saran

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 753 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 754 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 754 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 755 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 756 Gradual Correction: Growth Modulation Versus External Fixators . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 756 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 757 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 757 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 759 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 759 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 762 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 762

Abstract

In this chapter, we discuss the management of severe genu valgum in the context of skeletal dysplasias. Two issues are stressed: the first is acute versus gradual correction and the second is gradual correction with growth modulation versus the use of external fixation. Two cases of severe genu valgum are presented.

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] N. Saran Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada e-mail: [email protected]; [email protected]

Brief Clinical History Patient A is a 9 year old boy with a known diagnosis of Morquio syndrome who has been followed in our clinic for severe genu valgum. For the last 2 years, he has been mobilizing mostly with the help of a wheelchair, and at home with crutches. He previously had a C1/C2 fusion for cervical spine instability. He also has dilatation of the ascending aorta but his cardiac condition is stable. He has no pain and is asymptomatic. He has severe bilateral genu valgum, mostly coming from the proximal tibia, with an inter-malleolar distance of 11 cm. He underwent bilateral distal femoral and proximal tibial hemiepiphysiodesis (guided growth) with plates that corrected only the femoral deformity and not the tibial deformity. We then decided to proceed with gradual correction of the deformity using osteotomy and a Taylor spatial frame (TSF) was applied at the age of 11 years. Full correction was obtained and the patient is scheduled for surgery with a TSF on the other side. Patient B is a 10 year old with a diagnosis of spondylometaphyseal dysplasia who was seen for severe bilateral genu

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_339

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valgum. She previously had an occiput to C2 fusion for cervical instability. She has increasing difficulty ambulating mostly because of the increasing genu valgum and pain in the knees and in the hips. She has severe bilateral coxa vara with very limited abduction (10 on each side and 15 internal/external rotation, flexion 120 ). She has inter-malleolar distance of 11 cm. She has very osteopenic bones with severely deformed proximal tibial metaphyseal regions as shown in the MRI. Because of the severely affected proximal tibiae, it was decided to proceed with acute correction of the valgus deformities of her proximal tibiae bilaterally and then proceed with correction of the coxa vara. She underwent bilateral acute correction of her tibial deformities with release of the common peroneal nerve. Her genu valgum was satisfactorily corrected, and she was ambulating 3 months after the surgery and is scheduled for correction of the coxa vara.

Preoperative Clinical Photos and Radiographs Patient A: – See Figs. 1 and 2. Patient B: – See Figs. 3, 4, and 5.

Preoperative Problem List Both cases share some common problems, including: – All the problems associated with skeletal dysplasias, whether skeletal (short stature, osteopenic, and bony deformity) and extra-skeletal (e.g., cardiac) Fig. 1 (a, b) Front and back views of patient A with severe bilateral valgus deformities at the knees

Fig. 2 X-rays of same patient showing the severe valgus deformities at the knee level with large lateral mechanical axis deviation

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Fig. 3 (a–d) Clinical photos of patient B with severe deformities, specially the right leg, with external rotation and valgus deformity of the leg

Fig. 4 (a–d) X-rays of patient B, showing severe valgus deformities at the knee level, recurvatum deformities and severe coxa vara

– – – – –

Cervical spine instability that needed to be addressed first Hip problems Severe genu valgum Ligamentous laxity The deformities are usually multiplanar in skeletal dysplasias, in contrast to uniplanar deformities in the coronal plane in most other cases of genu valgum – Difficulty or inability to ambulate because of the increasing deformities – Status of the upper limbs that may preclude the use of crutches or other ambulatory aids and that may have an impact on the planning of the surgical interventions

Treatment Strategy Although severe genu valgum was present in both cases, the treatment strategy differed. In Patient A with Morquio syndrome (which is a spondyloepiphyseal dysplasia), there is relatively good bone in the metaphyseal regions and it was decided first to attempt guided growth correction with plates and if that failed to obtain adequate correction of the deformities, to proceed with gradual correction and osteotomy using a TSF.

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Fig. 5 MRI of knee in patient B, showing poorly ossified metaphyseal regions of the proximal tibia

In Patient B with spondylo-metaphyseal dysplasia, there is deficient and pathological bone in the metaphyseal regions (as shown in the MRI images). It was decided to proceed with acute correction, fully realizing that the correction may not be completely adequate in addressing the deformities in all planes (there was genu recurvatum with downward slope of the tibial plateau and external rotation deformity of the tibia) and that another surgery may be required. That was clearly explained to the parents. It was also decided to address first the genu valgum, followed by the ankle valgus in order to restore the mechanical alignment of the lower limbs with a future plan to address the coxa vara.

Basic Principles There are some basic principles that have to be followed whenever we are dealing with children with skeletal dysplasia and angular deformities of the long bones, including: – The approach has to be multidisciplinary. In many cases, the orthopedic conditions may well be the least important to be addressed at the time the patient is first seen. – Cervical spine instability should always be suspected, very carefully investigated and dealt with as a priority. – Acute versus gradual correction of the genu valgum. Although the final outcome may be not different between acute versus gradual correction, each technique offers distinct advantages as well as disadvantages. Each case should be individualized and several factors should be taken into consideration before deciding on acute versus gradual correction. – Advantages of gradual correction with external fixators include more accurate reduction; specifically if multiplanar

deformities are present, there is no bone resection that is often required in acute correction, and less risk of neurovascular complications that may occur in acute corrections. Disadvantages of gradual correction include the prolonged time wearing the external fixator, necessitating multiple clinic visits and missed school days, potential for social problems, prolonged rehabilitation, and all the problems and complications of external fixation including pin site infection, pain, and swelling. Indications for gradual correction include associated limb length discrepancy that could be addressed simultaneously with limb lengthening. – Advantages of acute correction include a single surgery required no postoperative adjustments, quicker return to daily activities and the absence of any of the problems associated with external fixation. In pathological and osteoporotic bone – such as is the case in Patient B and in patients with osteogenesis imperfecta (OI) – acute correction is usually indicated as the weak and pathological bone may not be able to withstand the stresses of external fixation. However, in contrast to gradual correction, no further fine tuning of the correction could be made and LLD, if present, could not be addressed at the same time.

Gradual Correction: Growth Modulation Versus External Fixators “The best osteotomy is NO osteotomy” (John Herzenberg). Growth modulation has radically changed our approach to correction of long bone deformities in the skeletally immature patient. Whenever possible, it is always better to use growth modulation. This may avoid more extensive surgery in the form of osteotomies – whether for gradual or acute correction. However, growth modulation with plates may not yield satisfactory results in cases of severe and/or multiple

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deformities. Furthermore, the correction with plates takes more time than with the use of external fixators. In addition, growth modulation may not yield satisfactory results in conditions where the physis is sick (e.g., skeletal dysplasias and Blount’s disease). In Patient A, we first attempted to correct the deformities with plates, knowing beforehand that we may not be successful. The plan was then to address all residual deformities with gradual correction with TSF. In Patient B, because of the pathological metaphyseal bone and the social condition of the family, we excluded the use of gradual correction and external fixators and we opted instead for acute correction, knowing also that this may not be ideal in fully correcting all the deformities. Furthermore, because of the severe genu valgum, the patient was not able to ambulate any longer and the priority was to give her back her ambulatory potential. We also decided to address the coxa vara at a second surgery.

Images During Treatment Patient A: – See Figs. 6, 7, 8, and 9. Patient B: – See Fig. 10.

Technical Pearls In Patient A – Arthrogram of the knee is helpful. – Avoid too heavy a construct. We used one ring proximally attached with a K-wire (reference wire) and two

Fig. 6 X-ray patient A showing bilateral distal femoral and proximal tibial medial eight-plates

Fig. 7 (a–d) X-rays of patient A showing gradual correction with TSF 2 years later

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Fig. 8 Front, side, and back view photos of patient A with TSF during treatment

Fig. 9 Clinical photos of Patient A during treatment (in supine position)

half pins (small diameter – 4.0 Ilizarov half pins) and one ring distally with three half pins. In Patient B – Prophylactic decompression of the common peroneal nerve (acute correction of valgus was done)

– Osteotomy of the fibula – Minimal excision of bone – Fixation with K-wires (simplest instrumentation) – Fasciotomies (prophylactic)

and

quickest

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Fig. 10 (a–c) X-rays showing acute correction with K-wire fixation

Outcome Clinical Photos and Radiographs Patient A – See Fig. 11. Patient B – See Figs. 12 and 13.

Avoiding and Managing Problems In Patient A • Generally, the use of a guided growth plate seems to provide more stability than staples. In this specific case, perhaps the use of double plates would have yielded better deformity correction. • Compartment syndrome and neurovascular complications remain the two most important problems to carefully monitor and try to avoid.

In Patient B • The main problem of acute correction of a genu valgum is common peroneal nerve palsy. This may be prevented by doing a decompression of that nerve just distal to the head of the fibula. There are three structures that need to be released: the posterior crural intermuscular septum underneath the peroneus longus, the anterior crural intermuscular septum between the peroneus longus and the extensor hallucis longus and the third has no name and lies between the extensor hallucis longus and the tibialis anterior muscle. • As with all proximal tibial osteotomies, compartment syndrome should be anticipated and the neurovascular status should be carefully monitored. Prophylactic fasciotomies and insertion of a drain are extremely important. • Long leg bracing, specifically during weight bearing and walking is necessary to try prevent recurrence of the deformity.

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Fig. 11 (a–c) X-rays showing satisfactory alignment of the right corrected extremity. The left side has will undergo tibial osteotomy to correct the residual valgus

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Fig. 12 (a–c) X-rays of patient B, 3 months after acute correction, showing satisfactory alignment in the coronal plane, with some translation in the sagittal plane

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Cross-References ▶ Bilateral Genu Valgum due to Chondroectodermal Dysplasia (Ellis-van Creveld Syndrome) ▶ Genu Valgum and Limb Length Discrepancy in Multiple Enchondromatosis ▶ Guided Growth and Syme Amputation in a Thirteen-YearOld Boy with Type 2 Congenital Fibular Deficiency ▶ Spondyloepiphyseal Dysplasia Treated by Bi-lateral Proximal Tibial Osteotomy Followed by Gradual Deformity Correction

References and Suggested Reading 1. Kopitz S. Orthopaedic complications of dwarfism. Clin Orthop Relat Res. 1976;114:153–79. 2. Saran N, Rathjen KE. Guided growth for the correction of paediatric lower limb angular deformity. J Am Acad Orthop Surg. 2010;18 (9):528–36. 3. White KK, Ted S. Mucopolysaccharide disorders in orthopaedic surgery. J Am Acad Orthop Surg. 2013;21:12–22. 4. Wiemann JM IV, Tryon C, Szalay EA. Physeal stapling versus 8-plate hemiepiphysiodesis for guided correction of angular deformity about the knee. J Pediatr Orthop. 2009;29:481–5. 5. Yilamz G, Oto M, Thabet AM, Rogers KJ, Anticevic D, Thacker MM, Mackenzie WG. Correction of lower extremity angular defornities in skeletal dysplasia with hemiepiphysiodesis: a preliminary report. J Pediatr Orthop. 2014;34(3):336–45.

Fig. 13 X-rays of patient B, 6 months after the acute correction

Spondyloepiphyseal Dysplasia Treated by Bi-lateral Proximal Tibial Osteotomy Followed by Gradual Deformity Correction

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John Birch, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 763 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 763 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 765 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 765 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 766 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 766 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 767 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 770 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 770 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 772 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 772 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 772

Abstract

Thirteen year old female with multiple skeletal deformities secondary to spondyloepiphyseal dysplasia congenita underwent bilateral proximal tibial osteotomy followed by sequential gradual deformity correction via circular external fixation for treatment of progressive genu valgum, recurvatum, and increasing knee pain.

Brief Clinical History The patient is a 13 year old female with multiple skeletal deformities secondary to spondyloepiphyseal dysplasia congenita. She had been treated for C1–C2 cervical instability with myelopathy by in situ fusion 7 years previously. She

presented at this time with complaints of increasing bilateral recurvatum valgus deformity of her legs (Figs. 1, 2, and 3), increasing knee pain, and diminished exercise tolerance. Due to the combination of extensive deformities of her lower extremities, she walked very slowly with a Trendelenburg gait but without using upper or lower extremity aids. Plain radiographs and MRI demonstrated significant proximal tibial recurvatum with valgus, accentuating milder procurvatum, and valgus deformities of the distal femur. She had no obvious neurological deficits nor other significant illnesses or surgeries.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, and 3.

J. Birch (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_86

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764 Fig. 1 Front-view photograph and standing AP radiograph of lower extremities demonstrating severe epiphyseal dysplasia with bilateral coxa vara, genu valgum, and ankle deformities

Fig. 2 Lateral view photograph and radiograph of the right lower extremity before treatment illustrating knee flexion compensating a significant (approximately 60 ) proximal tibial recurvatum. Note that the “lateral” projection is oblique relative to the distal femur and proximal tibia

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Fig. 3 Lateral view photograph and radiograph of the left lower extremity before treatment illustrating knee flexion compensating a significant proximal tibial recurvatum. Note that the “lateral” projection is oblique relative to the distal femur and proximal tibia

Preoperative Problem List Bilateral distal femoral valgus, procurvatum, and internal rotation deformities Bilateral severe proximal tibial valgus and recurvatum Bilateral intra-articular knee abnormalities with increasing nonspecific pain Systemic disorder (spondyloepiphyseal dysplasia) with severe short stature Status post upper cervical fusion due to instability with history of myelopathy

Treatment Strategy The nature of the deformity mandates the use of a very flexible treatment strategy with sequential correction of bilateral multi-planar tibial deformity using very adaptable but stable external circular fixator. The patient’s stable neurological status was first confirmed by careful physical examination and assessment of the in situ fusion on flexion/extension

lateral radiographs of the cervical spine. Since the primary complaint was increasing knee deformity and pain, and the most severe deformity was localized to the tibiae, it was decided to address proximal tibial deformities first. Because of the severity of the deformity, short stature, and proximity of the apex of deformity to the epiphysis, gradual deformity correction using circular external fixation was selected with possible extension of the apparatus across the knee joint. During surgery, a true orthogonal view of the knee was obtained for precise frame orientation relative to bone axis. For each limb, application of circular external fixator and proximal tibial osteotomy immediately below the tibial tubercle were performed under somatosensory evoked potential (SSEP) monitoring followed by gradual recurvatum deformity correction according to computerized LegPerfect preoperative planning. After correction of the recurvatum was completed, the hinges and angular distractor were relocated, and residual valgus deformity of the tibia was corrected. During the consolidation period on the right tibia, the similar surgical procedure, frame configuration, and correction strategy were performed on the left leg.

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Basic Principles Spondyloepiphyseal dysplasia (SED) congenita is an autosomal dominant chondrodysplasia caused by mutation in the COL2A1 locus on chromosome 12 resulting in formation of abnormal type II collagen [3]. This systemic disorder is characterized by disproportionate short-trunk dwarfism with progressive involvement of the spine and long bone epiphyses. Patients have upper cervical instability, hip flexion contractures accentuating a lumbar lordosis, and coxa vara producing a waddling gait. They often have intra-articular pathology with flattened and irregularly shaped epiphyses as well as severe complex angular deformities due to delayed ossification of long bone secondary ossification centers. Over time, the majority of the patients develop activity-related pain and restrictions in joint mobility [1, 2, 4, 7). Orthopedic

Fig. 4 Intraoperative lateral radiographs demonstrating maximal flexion (left) and extension (right) of the right knee. Note severe procurvatum deformity of the distal femur and matching in magnitude recurvatum deformity of the proximal tibia

Fig. 5 Computerized preoperative planning of right tibial deformity correction using the LegPerfect software

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treatment begins with posterior cervical fusion from occiput or C1 to C2 for stabilization of instability and prevention of progression of myelopathic symptoms. Valgus femoral osteotomy may be used for correction of severe coxa vara. Periarticular knee abnormalities including distal femoral procurvatum and proximal tibial recurvatum are also often present as a component of the complex lower limb deformity and should be corrected accordingly, e.g., via distal femoral and/or proximal tibial osteotomy.

Images During Treatment See Figs. 4, 5, 6, 7, 8, 9, 10, 11, 12, and 13.

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Fig. 6 Intraoperative photograph demonstrating TrueLok frame configuration for correction of right tibial recurvatum deformity. The frame consisted of proximal ring and distal double-ring block connected by two hinges and angular distractor and secured to the proximal and distal tibia using two crossed wires and one half pin each

Fig. 7 LAT radiographs of the right tibia during recurvatum deformity correction. Note that the osteotomy level was translated distally relative to the axis of the hinges, thereby resulting in posterior translation of the distal fragment simultaneously with angular deformity correction. After correction of tibial recurvatum, hinges and angular distractor were relocated for correction of genu valgum

Technical Pearls Location of the deformity apex close to the joint, unusual shape, and extremely short stature all create challenges in obtaining sufficient fixation of the proximal tibial segment and frame orientation relative to its anatomical axis in cases with severe recurvatum deformity. Although we prefer to use proximal tibial half pin attached to the ring via connection cube as a reference, a transverse reference wire inserted perpendicular to the knee was utilize in this case for proper

orientation of the fixator to the distorted limb. Instead of using a preassembled frame, the proximal ring was attached to the reference wire, rotated around the wire until properly oriented to the proximal segment, and then stabilized in position with an additional wire and medial-anterior half pin. Next, two uniplanar hinges were connected to the proximal ring with their common axis of rotation at the posterior cortex of the bone along the bisector line. Finally, a doublering tibial block was assembled and attached to the threaded rods of the hinges aligned with the distal tibial anatomic axis. Due to the location of the osteotomy being below the

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Fig. 8 Front-view photograph showing clinical appearance of the right lower extremity and frame configuration during valgus deformity correction

Fig. 9 AP and LAT radiographs of the right tibia after completion of deformity correction and beginning of consolidation period. At that time, we proceeded with a similar correction strategy for the left leg

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Fig. 10 Immediate postoperative LAT radiograph demonstrating TrueLok frame configuration for correction of left tibial recurvatum deformity

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Fig. 11 Front-view photograph showing clinical appearance of the lower extremities during consolidation period on the right tibia and procurvatum deformity correction on the left tibia

Fig. 12 AP and LAT radiographs of the right tibia at the end of consolidation period before frame removal

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Fig. 13 AP and LAT radiographs of the left tibia demonstrating gradual correction of recurvatum deformity (left) followed by hinges and angular distractor relocation and gradual correction of valgus deformity (right)

deformity apex, correction of recurvatum produced a posterior translation of the distal tibial segment, thereby stretching the periosteum and improving the shape of the proximal tibia.

Outcome Clinical Photos and Radiographs See Figs. 14, 15, and 16.

Avoiding and Managing Problems The most important aspect of this patient’s treatment was careful assessment of the functional impact of his/her multiple extensive deformities. Careful evaluation of the nature and severity of each angular deformity and intra-articular

abnormalities coexisting with those complex deformities (e.g., by MRI of the knee) is required. In addition, evaluation of the entire musculoskeletal system and neurological function (e.g., a neurological examination and evaluation of upper cervical spinal stability in our patient) is necessary. The development of a treatment plan based on prioritization of the impact of each deformity is one of the important components of preoperative planning to achieve the functional goals in this patient. Sequential, staged correction of the bilateral deformities facilitated management, physical therapy, and functional status of the patient during the treatment. The potential for a neurogenic cause (upper cervical instability) for the complaint of decreased function must be recognized while treating patients with SED.

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Fig. 14 Front-view photograph and standing AP radiograph of the lower extremities 1 year after frame removal demonstrating significantly improved limb alignment. The most recent examination was 4 years after treatment. She was ambulating without difficulty and satisfied with her appearance and functional improvement. She is working now as a store cashier. She has declined correction of residual left genu valgum and bilateral femoral procurvatum to date

Fig. 15 Lateral view photograph and radiograph of the right lower extremity 1 year after frame removal

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Fig. 16 Lateral view photograph and radiograph of the left lower extremity 1 year after frame removal

Cross-References ▶ Congenital Posteromedial Bowing of the Tibia (Congenital Tibia Recurvatum)

See Also in Vol. 3 Computer Assisted External Fixation for Aesthetic Changing Lower Limb Shape Multiapical Deformity of Knee, Tibia, and Ankle Treated with Osteotomy, Arthrodesis, and Arthroplasty

References and Suggested Reading 1. Bassett GS. Orthopaedic aspects of skeletal dysplasias. Instr Course Lect. 1990;39:381–7.

2. Damignani R, Young NL, Cole WG, Anthony AM, Badley EM. Impairment and activity limitation associated with epiphyseal dysplasia in children. Arch Phys Med Rehabil. 2004;85:1647–52. 3. Hamosh A (2011) Spondyloepiphyseal dysplasia congenital. Resource document. Online Mendelian Inheritance in Man (OMIM), #183900. http://omim.org. Accessed 3 Mar 2014. 4. Herring JA. Spondyloepiphyseal dysplasia. In: Herring JA, editor. Tachdjian’s pediatric orthopedics. 5th ed. Philadelphia: Elsevier Sanders; 2014. p. 394–401. 5. Horton WA, Hall JG, Scott CI, Pyeritz RE, Rimoin DL. Growth curves for height for diastrophic dysplasia, spondyloepiphyseal dysplasia congenital, and pseudoachondroplasia. Am J Dis Child. 1982;136:316–9. 6. Svensson O, Aaro S. Cervical instability in skeletal dysplasia: report of 6 surgically fused cases. Acta Orthop Scand. 1988;59:66–70. 7. Tolo VT. Spinal deformity in short-stature syndromes. Instr Course Lect. 1990;39:399–405.

Staged Femur and Tibial Lengthening During Childhood for Russell Silver Syndrome

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Amber A. Hamilton, Danya M. Jacobs, and S. Robert Rozbruch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 773 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 773 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 774 Outcome Clinical Photos and Radiographs (4–5) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 775 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 775 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 776 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 776

Abstract

This case describes a patient with Russel Silver Syndrome (RSS), a rare congenital syndrome characterized by restricted intrauterine and postnatal growth. Clinically relevant orthopedic findings of RSS include short stature and limb length discrepancy. This case highlights the difficulty of limb lengthening in patients with RSS, as treatment requires careful consideration of nutritional status, healing potentials, and bone strength.

A. A. Hamilton (*) · D. M. Jacobs Hospital for Special Surgery, New York, NY, USA e-mail: [email protected] S. R. Rozbruch Department of Orthopedic Surgery, Hospital for Special Surgery, New York, NY, USA e-mail: [email protected]

Brief Clinical History The patient is a 14-year-old female diagnosed with RSS at age 2. She was treated with growth hormone (GH) and cyproheptadine for short stature and poor growth. This regimen increased linear growth without concomitant increases in limb length discrepancy (LLD). Staged tibia and femur lengthening was planned to achieve symmetric leg length. At age 8, she presented with LLD of 3.6 cm with a predicted discrepancy of 6 cm at skeletal maturity. She underwent tibial lengthening with external fixator. At age 15 she presented with the expected 2.7 cm of additional LLD. An internal lengthening nail (ILN) was used to lengthen the femur. Lengthening was completed without complications.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_576

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Preoperative Problem List 1. Congenital LLD of 3.6 cm 2. Predicted LLD of 6 cm at skeletal maturity 3. Tibia and femur LLD

Treatment Strategy 1. Tibia and fibular lengthening (4 cm) using external fixation 2. Staged Femur lengthening (2.5–3 cm) using ILN close to skeletal maturity 3. Use shoe lift to manage LLD between surgeries

Basic Principles

Fig. 1 Front view showing 3.6 cm LLD at age 8

1. Calculate predicted LLD at skeletal maturity using a formula for congenital LLD. 2. If LLD is split between femur and tibia, then approach with lengthening one bone as child and then the second bone closer to skeletal maturity. 3. Current LLD of 3.6 cm is best treated at current age with external fixation in tibia since there are open growth plates and use of intramedullary rod would damage growth plate. 4. In the future, after or close to skeletal maturity, the femur may be lengthened with an ILN to correct the ultimate LLD. 5. Divide 6–7 cm lengthening between femur and tibia and between childhood and adolescence in 2 stages.

Images During Treatment See Figs. 3, 4, 5, 6, and 7.

Technical Pearls 1. Stabilize tibia and fibula proximal and distal with a wire to avoid fibula migration. 2. Choose osteotomy level in femur at apex of bow on lateral x-ray. 3. Choose femur nail length so that 5 cm of thick nail is left in distal fragment at end of distraction. 4. Perform iliotibial band tenotomy to prevent knee contracture during femur lengthening.

Fig. 2 Preoperative x-rays (a) standing AP x-ray shows LLD of 3.6 cm with 3.5 cm block under left foot at age 8. The discrepancy in the femur was 2 cm. The discrepancy in the tibia/fibula was 1.6 cm

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Outcome Clinical Photos and Radiographs (4–5) See Fig. 7.

Avoiding and Managing Problems 1. Prevent ankle and knee contracture with exercise and stretching program during tibial lengthening. 2. If ankle contracture occurs, then gastroc-soleus release may be done as needed.

Fig. 3 Front view showing external fixator used for tibial lengthening at age Fig. 4 Post-operative x-rays at age 8 (a, b) standing AP and lateral x-ray at the end of tibial/ fibular distraction 78 days showing bone consolidation showing lengthening of 3.9 cm

Fig. 5 Preoperative x-ray at age 15 showing residual LLD in the femur

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Fig. 7 Final clinical photograph of the patient standing showing correction of LLD

References and Suggested Reading Fig. 6 Postoperative x-rays (a, b) standing lateral and AP x-ray at the end of femur distraction (150 days after surgery) showing bone consolidation and equal leg lengths. Note the left tibia and fibula is well remodeled after lengthening done 7 years earlier

3. Avoid damage to growth plates in tibia and fibula with careful wire placement. 4. Use trochanteric entry nail for femur in children over age 8 to avoid injuring blood supply to femoral head. 5. Prevent knee and hip contracture during femur lengthening with exercise and stretching program.

Cross-References ▶ Staged Lengthening of 20 cm in the Femur and Tibia to Equalize Leg Lengths in a Growing Child

1. Abraham E, Altiok H, Lubicky JP. Musculoskeletal manifestations of Russell–silver syndrome. J Pediatr Orthop. 2004;24:552–64. 2. Wakeling EL, Brioude F, Lokulo-Sodipe O, O’Connell SM, Salem J, Bliek J, Canton AP, Chrzanowska KH, Davies JH, Dias RP, Dubern B, Elbracht M, Giabicani E, Grimberg A, Grønskov K, Hokken-Koelega AC, Jorge AA, Kagami M, Linglart A, Maghnie M, Mohnike K, Monk D, Moore GE, Murray PG, Ogata T, Petit IO, Russo S, Said E, Toumba M, Tümer Z, Binder G, Eggermann T, Harbison MD, Temple IK, Mackay DJ, Netchine I. Diagnosis and management of silver-Russell syndrome: first international consensus statement. Nat Rev Endocrinol. 2017;13(2): 105–24. 3. Goldman V, McCoy TH, Harbison MD, Fragomen AT, Rozbruch SR. Limb lengthening in children with Russell-silver syndrome: a comparison to other etiologies. J Child Orthop. 2013;7(2):151–6. 4. Goodbody CM, Buksbaum J, Harbison MD, Fragomen AT, Rozbruch SR. Limb lengthening in Russell-silver syndrome: an update confirming safe and speedy healing. J Pediatr Orthop. 2021; https:// doi.org/10.1097/BPO.0000000000001855. Online ahead of print.

Telescopic Rodding in Combined Technique After Transphyseal Elastic Nailing in a Boy with Type IV Osteogenesis Imperfecta

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Dmitry Popkov, Pierre Lascombes, and Sergey Khmyzov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 777 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 778 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 778 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 779 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 779 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 780 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 780 Outcome Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 780 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 782 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 784 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 784

Abstract

There are some indications for sliding titanium flexible intramedullary nailing (FIN) in selected cases, such as in severe osteogenesis imperfecta (OI) patients under the age of four to six, with narrow medullary canals and severe comorbidities. The FIN is considered as a less invasive and salvage procedure compared to telescopic rodding but is a temporary option. Because no telescopic rod available in our country before 2017, sliding FIN was the only method to manage long bone pathology in children with severe and moderate-to-severe types of OI.

D. Popkov (*) National Ilizarov Medical Research Center for Traumatology and Orthopaedics, Kurgan, Russia P. Lascombes University of Nancy, Nancy, France University of Geneva, Geneva, Switzerland S. Khmyzov Sitenko Institute of Spine and Joint Pathology, Kharkiv, Ukraine

This is a case of a boy with type IV OI, initially treated with FIN at the age of 3.5 years in 2015. During the period from 2018 to 2021, elastic nails were replaced by titanium telescoping rods (TTR). These are not Fassier-Duval rods. Simultaneous surgery was performed for bilateral femurs and left tibia. Only telescopic rod without external fixation was used for right tibia. The main surgical indications for implant replacement were fractures and deformities. We present and discuss surgical techniques applied in this case, pitfalls, and mid-term results.

Brief Clinical History This is a case of a boy with type IV OI. He started to receive pamidronate treatment at the age of 2 years. Recurrent fractures followed by conservative treatment and plaster cast immobilization, angular and torsional deformities at lower limbs resulted in delayed gross motor development and

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_418

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functional disability. He presented to our practice when he was 3 years old. Index surgery consisted of corrective osteotomies and sliding titanium FIN. This approach enabled independent walking. Three years after index operation, the boy was referred to our clinic for the replacement of nails in the left tibia because of malalignment union after fracture.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Fig. 1 Preoperative clinical view and radiographs: front-view photograph showing clinical appearance and standing with weight-bearing radiographs after sliding FIN

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Preoperative Problem List 1. Malalignment union of tibial fracture. 2. Impingement (failure of sliding movement) of several nails which migrated out of physis in both femurs and right tibia. 3. Irregular medullary canal causing difficulty to position male rod in the distal epiphysis. 4. Risk of intraarticular protrusion prone to the conventional technique of rodding.

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Telescopic Rodding in Combined Technique After Transphyseal Elastic Nailing in a Boy with Type IV. . .

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Fig. 2 Standing full-size radiographs demonstrating valgus procurvatum malunion after the fracture of the left tibia (at the age of 6 years)

Treatment Strategy Improvement of mobility, self-care, functional skills, and functional independence are the main objectives enabling better quality of life for children with OI. The multidisciplinary approach based on bisphosphonate therapy, orthopedic treatment, and rehabilitation provides their achievement. Recurrent fractures and deformities interfering with the motor development of children with OI justify orthopedic surgery based on intramedullary telescopic rodding. However, preventive rodding should be discussed with parents and caregivers. A combination of TTR and reduced (both in time and in design) external fixation allows to overcome an inherent rotational and longitudinal instability of any telescopic system in early postoperative period and ensure immediate

weight-bearing on operated leg. The titanium alloy implants do not raise any concerns for magnetic resonance imaging.

Basic Principles The basic principles of this case are to remove previously inserted elastic nails and to perform titanium telescopic rodding ensuring favorable conditions for bone union. This is achieved in the following ways: • • • •

Removal of elastic nails Corrective osteotomies and bony shortening as necessary Use of TTR Limited external fixation ensuring favorable conditions for bone union: early axial charges and weight-bearing

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Images During Treatment See Figs. 3, 4, and 5.

Technical Pearls The first step of surgery is elastic nail removal. The nails pulled out of physis and fully located into medullary canal can be easily removed through corrective osteotomy. If necessary, a limited reaming over guide wire inserted through greater trochanter or proximal tibial epiphysis can be performed for irregular medullary canal. Alternatively, the guide wire may be placed through osteotomy site. The male rod is inserted first or just after reaming. It should be never screwed first into distal epiphysis in order to avoid intraarticular protrusion while insertion of female rod. The female rod is cut to size always intraoperatively and then inserted over the male rod. Female rod was always screwed first in proximal tibial epiphysis/greater trochanter. At the last step of rodding, the male rod was screwed into distal epiphysis under x-ray control (C-arm). The female rod diameter should correspond to the function that the rod serves

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as an internal strut, sharing force with the bone and avoiding resorption and disuse osteoporosis. The aim of reaming is to obtain smooth medullary canal, not to enlarge its diameter. An external frame should be assembled as the final step of surgery. A proximal short arc with two to three half-pins or half-wires and distal full ring or two-third ring with three wires are applied for femur. In tibia, two rings, each with three wires, can be used. Rings and arc are connected in definitive position by the end of surgery. In early postoperative period patients are encouraged for standing up and walking with weight-bearing since third or fourth day using a walker or crutches. No manipulations or external frame adjustment should be performed after surgery. Radiological signs of uninterrupted periosteal and/or endosteal callus usually observed in four to five postoperative weeks justify frame removal. Minimal additional immobilization has psychological benefit.

Outcome Radiographs See Fig. 6.

Fig. 3 Rodding of the left tibia: (a) intraoperative x-rays showing osteotomy and telescopic rodding followed by external fixation (at the age of 6.5 years); (b) frame removal in 29 days, x-rays demonstrating bone union

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Telescopic Rodding in Combined Technique After Transphyseal Elastic Nailing in a Boy with Type IV. . .

Fig. 4 Rodding of the left femur: (a) fracture resulted in procurvatum malunion (x-rays dated October 3, 2019); (b) intraoperative x-rays showing osteotomy and telescopic rodding followed by external fixation (October 8, 2019). Translation of bone fragments ensured accurate

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alignment of the femur, satisfactory positioning of the rod in distal epiphysis, and avoided multilevel osteotomies; (c) frame removal in 35 days, x-rays demonstrating bone union (at the age of 7.5 years); (d) bone remodeling in 5 months, elongation of the rod)

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Fig. 5 Rodding of the right femur: (a) fracture resulted in procurvatum and rotational displacement of bone fragments (8 y.o.); (b) intraoperative x-rays showing alignment of bone fragments, telescopic rodding, and external fixation

Avoiding and Managing Problems In patients with OI, alignment of bone fragments should be acute. Any progressive manipulations and frame adjustment result in early external frame loosening, delayed union, and high risks of sepsis.

In a situation of soft tissue contracture, shortening bones allows to reduce risks related to soft tissue tension related to acute correction of deformities.

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Fig. 6 Radiographs 2.5 years after the first rodding procedure (mid-term results): (a) x-rays (9 y.o. April 14, 2021) demonstrating anatomical alignment of lower limbs and elongation of rods (45 mm for left tibia, 26 mm for left femur, and 13 mm for right femur). There are no titanium telescopic rod failures; (b) corrective osteotomy of only

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right tibia and rodding without external fixation (April 15, 2021); (c) bone union achieved, partial weight-bearing was authorized on May 27, 2021; (d) clinical photos after 6 months of the right tibia rodding.

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Cross-References ▶ Retrograde Insertion of a SLIM Nail in a Femur in a Patient with Osteogenesis Imperfecta

References and Suggested Reading 1. Langlais T, Pannier S, De Tienda M, Dukan R, Finidori G, Glorion C, Péjin Z. ‘In-Out-In’ K-wires sliding in severe tibial deformities of osteogenesis imperfecta: a technical note. J Pediatr Orthop B. 2021;30(3):257–63.

D. Popkov et al. 2. Persiani P, Martini L, Ranaldi FM, Zambrano A, Celli M, Celli L, D’Eufemia P, Villani C. Elastic intramedullary nailing of the femur fracture in patients affected by osteogenesis imperfecta type 3: indications, limits and pitfalls. Injury. 2019;Suppl 2:S52–6. 3. Popkov D, Popkov A, Mingazov E. Use of sliding transphyseal flexible intramedullary nailing in pediatric osteogenesis imperfecta patients. Acta Orthop Belg. 2019;85(1):1–11. 4. Popkov D, Dolganova T, Mingazov E, Dolganov D, Kobyzev A. Combined technique of titanium telescopic rods and external fixation in osteogenesis imperfecta patients: first 12 consecutive cases. J Orthop. 2020;22:316–25. 5. Fassier FR. Osteogenesis imperfecta-who needs rodding surgery? Curr Osteoporos Rep. 2021;19(3):264–70.

The Use of Gap Nail for Tibia Fracture in Skeletally Mature Osteogenesis Imperfecta Patient

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Yousef Marwan, Khaled Abu Dalu, and Mitchell Bernstein

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 785 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 785 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 786 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 786 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 786 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 786 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 787 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 787 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 787 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 790 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 790

Abstract

This is a patient with osteogenesis imperfecta (OI) type 5 who had previous Fassier-Duval (FD) rodding of the right tibia. The patient presented after a tibia fracture with a deformed limb and an FD rod. Revision surgery was performed to remove the nonfunctional FD rod and stabilize the limb using the Gap (Pega Medical, Laval, Quebec, Canada) intramedullary rod.

Y. Marwan (*) Department of Surgery, College of Medicine, Health Sciences Centre, Kuwait University, Kuwait City, Kuwait

Brief Clinical History The patient is a 17-year-old male who is known for OI type 5. At the age of 9 years, due to the presence of multiple long bones deformities and fractures, he underwent telescopic rodding of his femurs and tibias using the Fassier-Duval (FD) rod (Pega Medical, Laval, Quebec, Canada). He remained active and independent, and when he was 17 years old, he presented with right tibia displaced fracture. The FD rod in the right tibia was deformed. The patient was from another country and the local surgeons were not comfortable treating this patient. Consequently, there was a delay in treatment, and when the patient presented, there was an ulcerated open wound over the fracture site with exposed hardware.

K. A. Dalu Shriners Hospitals for Children, Montreal, QC, Canada McGill University Health Centre, McGill University, Montreal, QC, Canada

Preoperative Clinical Photos and Radiographs

M. Bernstein Departments of Surgery & Pediatric Surgery (Division of Orthopaedics), McGill University Health Center & Shriners Hospital for Children Canada, Maywood, IL, USA

See Figs. 1 and 2.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_409

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Treatment Strategy The plan was to stage the treatment since the patient had evidence of an open contaminated wound with exposed hardware. The first stage consisted of hardware removal and debridement of the fracture site, in addition to obtaining tissue samples for microbiology analysis. This was done successfully, and the tissue cultures did not grow any microbe. Stabilization of the limb during stage 1 was performed with a long leg splint. In patients with OI, external fixation is contraindicated due to stress riser at the pin sites. The second stage of the treatment was performed 2 weeks following the first stage and consisted of fracture reduction, osteotomy to correct malalignment/bowing, and bone fixation with the Gap nail. Postoperatively, weight-bearing was restricted for a period of 3 months, and careful fracture healing follow-up was done. Physiotherapy for range of motion exercises was initiated early postoperatively to improve mobility and function.

Basic Principles

Fig. 1 Lower extremity X-rays before initial surgical treatment at the age of 9 years

Preoperative Problem List 1. Skeletally mature patient with osteogenesis imperfecta type 5 2. Right tibia fracture and deformity 3. Right tibia bent FD rod at the male component 4. Osteopenic bone and narrow intramedullary canal 5. Open wound at the fracture site

1. Telescopic rods are not required for skeletally mature OI patients. The preferred method of fixation of long bones for this group of patients is solid interlocking intramedullary nails. 2. Avoid using plates and screws for OI patients. Such implants create stress points and increase the risk of periprosthetic fractures. 3. In OI, external fixation is contraindicated due to the risk of stress fractures at the pin sites. 4. Preoperative surgical planning is critical in such patients. In addition to the medical considerations (e.g., risk of bleeding and airway difficulties), the planning should include: (a) measurements of the length of the bone, (b) measurements of the canal diameter, and (c) deformity analysis and identifying potential osteotomy site(s). 5. In cases of severe deformity, shortening the bone to help regain the alignment should be considered. 6. Preserve the bone as much as possible in patients with osteopenic conditions such as OI by minimal reaming (i.e., smaller diameter nails).

Images During Treatment See Figs. 3, 4, 5, and 6.

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Fig. 2 Clinical and radiographic images of the right tibia at age 17 years

Technical Pearls 1. For OI cases, transport and position the patient on the operating room (OR) carefully to avoid iatrogenic fractures. 2. Understand the implant options and how to use them well. Have the company representative available in the OR for assistance. In skeletally mature OI patients, telescoping rods are not required anymore. Therefore, the use of solid nails (e.g., SLIM and Gap nails from Pega Medical) is required. 3. In order to remove the bent implant, the rod has to be cut. Use a wire cutter or Midas Rex Microsaw (Medtronic) to cut the implant through the fracture or osteotomy site. 4. Perform percutaneous osteotomies with multiple drill holes and sharp osteotomes to realign the bone. Consider avoiding osteotomies that generate heat (e.g., oscillating saws). 5. Avoid eccentric reaming of the medullary canal by realigning the bone and proper placement of the guidewire.

6. Avoid using large diameter nails that fit the medullary canal tightly as this leads to bone resorption in cases with osteopenic metabolic bone disease.

Outcome Clinical Photos and Radiographs See Figs. 7 and 8.

Avoiding and Managing Problems 1. Avoid using bone reduction clamps as these might easily break the bone. 2. In cases of severe deformity/bowing, have a low threshold to shorten the bone to achieve appropriate alignment. 3. Ensure that the proximal threads of the Gap nail are in the epiphyseal region of the bone on both anteroposterior and lateral views. 4. Avoid long-term postoperative immobilization to prevent bone resorption and poor functional outcomes.

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Fig. 3 Postoperative X-rays following stage 1 of the surgical treatment. The FD rod was removed, tissue samples were sent for microbiology analysis, and debridement of the fracture site was done

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Fig. 4 Surgical planning using the X-rays which were obtained between stage 1 and stage 2. In order to obtain proper alignment in the sagittal plane, an osteotomy is needed between the junction of the proximal and middle thirds of the tibia shaft in addition to the fracture reduction

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Fig. 5 Intraoperative fluoroscopic images – Part 1. (a) Guidewire inserted in a retrograde fashion through the fracture site for retrograde reaming of the proximal segment following reaming of the distal segment through the fracture site also. (b) Apex of the deformity in the sagittal plane identified. (c) The guidewire was pulled back to a level below the osteotomy site, and low energy osteotomy was started using a

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sharp drill bit. (d) Osteotomy completed with a sharp osteotome. (e) The osteotomy is completed. (f) Realignment of the bone at the osteotomy site was done using a solid metal surgical tool percutaneously, and the guidewire was passed proximally. (g) The guidewire at the nail entry point in the lateral view. (h) The guidewire at the nail entry point in the anteroposterior view

Fig. 6 Intraoperative fluoroscopic images – Part 2. (a–f) After realigning the bone and reaming the intramedullary canal, the Gap nail (Pega Medical, Laval, Quebec, Canada) was inserted. (g and h) Proximal and distal locking screws were inserted

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Fig. 7 X-rays of the tibia 2 days postoperatively

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Fig. 8 X-rays of the tibia 4 months postoperatively showing excellent alignment and healing

Cross-References ▶ Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with Telescoping Fassier-Duval Rods ▶ Revision of Bilateral Tibial Fassier-Duval Rods in an Eleven Year Old Girl with Osteogenesis Imperfecta

References and Suggested Reading 1. Galban M. Intramedullary rodding in adult with osteogenesis imperfecta developing of a new device. In: Anticevic D, editor. 11th international conference on osteogenesis imperfecta final program & abstract book. Dubrovnik: Croatian Pediatric Orthopaedic Society of Croatian Medical Association; 2011. p. 81. 2. Musielak BJ, Woźniak Ł, Sułko J, Oberc A, Jóźwiak M. Problems, complications, and factors predisposing to failure of Fassier-Duval rodding in children with Osteogenesis Imperfecta: a double-center study. J Pediatr Orthop. 2021;41(4):e347–52.

The Use of SLIM (Simple Locking Intra Medullary) Nail in the Lower Extremities of Skeletally Immature Patients with Osteogenesis Imperfecta

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Yousef Marwan and Reggie C. Hamdy

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 791 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 792 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 792 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 792 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 794 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 795 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 795 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 795 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 795 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 800 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 801

Abstract

This chapter describes the use of the Simple Locking Intra Medullary (SLIM) nail system (Pega Medical, Laval, Quebec, Canada) in four skeletally immature patients with osteogenesis imperfecta (OI). In all patients, the SLIM nail was used to stabilize the tibia or femur for different indications. This nail is a helpful tool to have for surgeons who treat patients with metabolic bone disorders who are known to have narrow medullary canal.

Y. Marwan (*) Department of Surgery, College of Medicine, Health Sciences Centre, Kuwait University, Kuwait City, Kuwait e-mail: [email protected]

Brief Clinical History Case #1: A 19-month-old female with type 4 OI presented with severe deformities and fractures of the lower extremities. The patient was experiencing delayed walking and was only able to pull to stand at that time. Osteotomies and rodding of the femurs and tibias were indicated to improve the alignment of the bones and help the patient to ambulate. The medullary canal was very narrow; therefore, SLIM nail was indicated. Case #2: An 8-year-old male with type 7 OI who had multiple previous rodding of the femurs and the tibias to correct bowing and fractures. His right tibia had a FassierDuval (FD) rod 3 years before the current preoperative visit. This right tibia developed hardware failure, fracture, and bowing. The patient was indicated for revision surgery with FD rod; however, we found that the appropriate length

R. C. Hamdy Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_598

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3.2 mm FD rod was not available in our institution during the surgery after intraoperative measurement of the bone length. Therefore, an appropriate length 3.2 mm SLIM nail was used to stabilize the tibia instead of the FD rod. Case #3: A non-ambulatory 9-year-old female with type 3 OI presented with difficulties in sitting properly in her wheelchair due to severe lower extremities bowing. She had narrow medullary canal of her femurs and tibias. She, therefore, underwent osteotomies of bilateral femurs and tibias with SLIM nail fixation of all the long bones except the left tibia, which was stabilized using a K-wire. Since she is wheelchair dependent, the SLIM nails survived for more than 5 years; however, the K-wire was revised to a SLIM nail 4 years following the primary surgery. Case #4: A 14-year-old male with type 4 OI presented with bilateral lower extremities deformities, limiting his daily function. He was indicated for osteotomies and rodding of both femurs and tibias to optimize his function and quality of life. Since the medullary canal of his tibias was narrow, SLIM nail was used to stabilize the osteotomies.

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Preoperative Problem List 1. Skeletally immature patients with osteogenesis imperfecta. 2. Lower extremity deformities/fractures. 3. Osteopenic bone. 4. Very narrow medullary canal that can’t fit the smallest diameter telescopic rod.

Treatment Strategy The plan was to realign and stabilize the bones using a small diameter rod that could fit the medullary canal. The SLIM nail was used to manage some of these deformities/fractures. Postoperatively, weight bearing was restricted for a period of 2–3 months, and careful fracture healing follow-up was done. Physiotherapy for range of motion exercises was initiated early postoperatively to improve mobility and function.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

Fig. 1 Case 1: Preoperative X-rays of the lower extremities showing bilateral femur and tibia deformities/fractures

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Fig. 2 Case 2: Preoperative X-rays of the lower extremities showing previous rodding of bilateral femurs and tibias. In the right tibia, there is bending of the FD rod with pull out of the anchor from the distal tibia and failure of telescoping. In addition, there is perforation of the tibial

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cortex by the distal tip of the male component of the FD rod. Varus and procurvatum deformity is also seen, with fracture nonunion of the mid-shaft of the right tibia

Fig. 3 Case 3: Preoperative X-rays of the lower extremities showing bilateral femur and tibia deformities/fractures

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Fig. 4 Case 4: Preoperative X-rays of the lower extremities showing bilateral femur and tibia deformities

Basic Principles 1. The SLIM nail is a solid intra medullary nail designed specifically to provide fixation in long bones with small medullary canal that cannot fit other larger solid nails (whether telescoping or non-telescoping). This nail helps protect the bone during growth, and eventually can be revised to a larger telescoping nail. 2. The SLIM nail is indicated in any patient, skeletally mature or immature, with a systemic metabolic bone disorder, specifically OI, where the canal is very small. 3. The advantages of SLIM nail over other types of solid nails (such as Kirshner wires, elastic nails, Rush rods, and Enders nails) is that the proximal part of the nail is threaded, hence allowing anchorage of the nail in the proximal epiphysis. This should, therefore, prevent proximal migration of the nail, which is a common complication of the other small diameter intra-medullary nails. Proximal and distal locking options are also available for some sizes of the SLIM nail to help control rotation stability of the fixation.

4. The SLIM nail is made of stainless steel, and has a diameter ranging from 2.0 mm to 6.4 mm. Regarding the length of the nail, available options range from 80 mm to 400 mm. 5. The SLIM nail is most commonly used in the tibia, followed by the femur, fibula, and humerus. In the femur, fibula, and humerus, it can be inserted in a retrograde fashion. 6. Preoperative surgical planning is critical in patients with OI. In addition to the medical considerations (e.g., risk of bleeding, airway difficulties), the planning should include: (a) measurements of the length of the bone, (b) measurements of the canal diameter, and (c) deformity analysis and identifying potential osteotomy site(s). 7. In cases of severe deformity, shortening the bone to help regain the alignment should be considered. 8. Preserve the bone as much as possible in patients with osteopenic conditions such as OI by minimal reaming (i.e., smaller diameter nails).

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The Use of SLIM (Simple Locking Intra Medullary) Nail in the Lower Extremities of Skeletally Immature. . .

Images During Treatment See Figs. 5, 6, 7, 8, and 9.

Technical Pearls 1. For OI cases, transport and position the patient on the operating table carefully to avoid iatrogenic fractures. 2. Understand the implant options and how to use them well. Have the company representative available in the OR for assistance. 3. Perform percutaneous osteotomies with multiple drill holes and sharp osteotomes to realign the bone. Consider avoiding osteotomies that generate heat (e.g., oscillating saws). 4. Avoid eccentric reaming of the medullary canal by realigning the bone and proper placement of the guide wire. 5. In cases where there is severe bone sclerosis with no medullary canal (or very narrow canal) at the shaft, insertion of the guide pin is not possible. Use solid small size drill bits to create and ream the medullary canal. Fig. 5 The SLIM nail. (a) Two different sizes of the SLIM nail, noting that the smaller nail does not have a distal hole for interlocking pin/peg; (b) Closer look at the proximal threads and the interlocking hole; (c) The longer nail with proximal and distal interlocking pegs, and the short nail with proximal interlocking peg and a distal bullet used for rotational stability and prevention of migration

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6. Avoid using large diameter nails that fit the medullary canal tightly, as this leads to bone resorption in cases with osteopenic metabolic bone disease. 7. In cases of rotational instability at the fracture/osteotomy site with the use of SLIM nail, using the interlocking pins/ pegs and/or distal bullet helps stabilize the bone. Our preferred method for providing rotational stability in such cases is the use of mini-fragment plate and screws.

Outcome Clinical Photos and Radiographs See Figs. 10, 11, 12, and 13.

Avoiding and Managing Problems 1. Avoid using bone reduction clamps, as these might easily break the bone. 2. In cases of severe deformity/bowing, have a low threshold to shorten the bone to achieve appropriate alignment.

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Fig. 6 Case 1: X-rays done at 3 months post-surgery. Both tibias underwent osteotomies and fixation with SLIM nail. Osteotomies and FD rodding were done for the femurs

3. Ensure that the proximal threads of the SLIM nail are in the epiphyseal region of the bone on both anteroposterior and lateral views. 4. If a plate was used for rotational stability, it is advised to remove the plate once osteotomy/fracture union is completed. Leaving it for a long duration will create stress fractures at the ends of the plate. 5. Avoid long-term postoperative immobilization to prevent bone resorption and poor functional outcomes. 6. In a skeletally immature child (especially a very young patient), the bone will continue to grow and outgrow the nail. This will create a certain length of the bone distal to the nail which is not protected, and therefore, may bend or break. Radiographic monitoring of the bone growth is important to plan the revision surgery to a longer nail or a telescoping rod. This should be explained to the caregivers before the primary surgery.

Fig. 7 Case 2: X-rays of the right tibia done during the first day postsurgery. Revision of the FD rod was done using SLIM nail. The deformity was corrected via an osteotomy at the distal third of the tibial shaft. A 2.7 mm plate was implanted at the osteotomy site to provide rotational stability

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Fig. 8 Case 3: X-rays done post-surgery. Top row showing X-rays done 11 months after osteotomies of bilateral femurs and tibias with SLIM nail fixation of all the long bones except the left tibia which was

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stabilized using a K-wire. Bottom row showing X-rays done 4 years after the primary surgery demonstrating survival of the SLIM nails and fracture nonunion of the left tibia around the distal part of the K-wire

Fig. 9 Case 4: X-rays done 5 days post-surgery. Both tibias underwent realigning osteotomies and stabilization with SLIM nail. A 2.7 mm plate was used to add rotational stability at the osteotomy site

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Fig. 10 Case 1: Follow-up X-rays. Top row showing X-rays of both tibias done 16 months postoperatively, demonstrating bone growth, deformity, and rod perforation at the anterior cortex. Bottom row

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showing X-rays of both tibias done 10 months post-revision of the SLIM nails to FD rods showing incomplete union of the bone

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Fig. 11 Case 2: Follow-up X-rays done 3 weeks after the surgery. Alignment is maintained, and no early postoperative complications are seen

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Fig. 12 Case 3: Follow-up X-rays done 5 years after the primary surgery (13 months after the revision surgery of the left tibia). In case of ambulatory patients, revision surgery to correct the deformities and

Cross-References ▶ Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with Telescoping Fassier-Duval Rods

Y. Marwan and R. C. Hamdy

stabilize the bone would be indicated. Since this patient is non-ambulatory and was asymptomatic, revision surgery was not indicated

▶ Retrograde Insertion of a SLIM Nail in a Femur in a Patient with Osteogenesis Imperfecta ▶ Revision of Bilateral Tibial Fassier-Duval Rods in an Eleven Year Old Girl with Osteogenesis Imperfecta

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Fig. 13 Case 4: Follow-up X-rays done 3 months after the surgery. Alignment is maintained, and no early postoperative complications are seen

References and Suggested Reading 1. Spahn KM, Mickel T, Carry PM, Brazell CJ, Whalen K, Georgopoulos G, Miller NH. Fassier-Duval rods are associated with superior probability of survival compared with static implants in a

cohort of children with osteogenesis imperfecta deformities. J Pediatr Orthop. 2019;39(5):e392–6. 2. Yong B, De Wouters S, Howard A. Complications of elongating intramedullary rods in the treatment of lower extremity fractures for osteogenesis imperfecta: a meta-analysis of 594 patients in 40 years. J Pediatr Orthop. 2022;42(3):e301–8.

Type IV Tibial Dysplasia

115

Robert A. Hill

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 803 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 804 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 804 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 804 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 805 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 805 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 805 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 807 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 807 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 808 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 808

Abstract

Tibial dysplasia is very rare. It is commonly classified into four types in the Jones classification: Type I is the total absence of the tibia, in Type II the proximal tibia is present, in Type III the distal tibia only is present, and in Type IV there is a diastasis of the distal tibia and fibula with shortening of the tibia. The case illustrated describes the management of a Type IV case. The rarity of the condition is such that even in specialist centers experience is limited and often treatment has to be individualized for each case although there are some broad principles. The condition of the foot will often dictate management – severe foot deformities and polydactyly are frequent and some form of amputation may offer the best functional result. In Type IV tibial dysplasia, there may be a reasonable foot, and reconstruction is often an option in contrast to Types I and III where because of the absence of a knee, R. A. Hill (*) Portland Hospital for Women and Children, London, UK e-mail: [email protected]

as well as foot abnormalities, amputation gives the best result. Type II may be reconstructible.

Brief Clinical History This patient was born by a normal delivery at term without any family history of congenital bone dysplasia. A right club footlike deformity was noted at birth although the foot could be positioned plantigrade. There was a varus of the forefoot, a short first ray, and a prominent distal fibula. Following radiographs at 5 weeks of age, the diagnosis of type IV tibial dysplasia was made. The initial treatment was conservative with stretching exercises, while a number of opinions were sought regarding definitive management. These included amputation and a talofibular fusion. While there may be a role for amputation or fusion in severe cases, it is possible in many instances, including this one, to preserve the ankle if it is reasonably stable and mobile. At the age of 5, patient was

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_50

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referred for reconstruction was carried out. The clinical findings at this stage were a leg-length difference of 4.5 cm, an equinovarus of the ankle and foot, an overlong fibula, a mild valgus deformity of the distal femur, and a moderate but asymptomatic AP instability of the knee. The patient was asymptomatic and ambulatory with a shoe lift and a mobile but abnormal ankle.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

Preoperative Problem List • Shortening of the limb • Relative discrepancy in length between the fibula and tibia – fibula prominent distally and proximally • Equinovarus foot deformity

Fig. 1 AP X-ray of the right tibia and fibula at about 18 months. Note the length discrepancy, the distal diastasis, and the abnormal ankle

R. A. Hill

• Abnormal ankle in which it was unclear whether it was the tibia or fibula that formed the major articulation with the talus • Probable subtalar fusion Other problems that were present but not requiring treatment at this stage included: • • • •

Valgus deformity of distal femur AP instability of the knee Mild shortening in the femoral segment Shortening of the first ray

Treatment Strategy The aim of the initial reconstruction was to correct the foot and ankle deformity to produce a stable plantigrade foot while retaining as much ankle movement as possible. In addition, the limb length discrepancy was to be corrected as well as the relative discrepancy between the fibula and tibia.

Fig. 2 AP of the tibia and fibula immediately prior to reconstruction

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Fig. 3 AP of the ankle immediately prior to reconstruction. Note abnormal articulation of the fibula – both the tibia and fibula form the proximal side of the ankle joint Fig. 4 Lateral of ankle joint. The ankle is in equinus, the distal fibula and tibia look very similar, and the distal tibia is posterior

Basic Principles While the broad strategy outlined above is unlikely to be a matter of debate, how this is to be achieved may be more controversial. The author’s strategy is to explore the ankle and forefoot via a posteromedial incision as for a soft tissue release of a clubfoot, to lengthen tight posterior structures, and to ascertain by direct inspection whether the fibula or tibia forms the major articulation with the talus bearing in mind that sometimes it is both. It is important to maintain as much movement as possible in the ankle. The talus should be aligned to whichever appears to offer the best articulation, and bear in mind a mobile functional ankle is the aim rather than a “normal” radiographic appearance. This surgery should be done at about age 12-18 months. Conventional forefoot surgery including a possible osteotomy of a conjoined talus and os calcis may be required later. The aim is to get the foot plantigrade and then hold it in position with splintage as appropriate. Subsequently proximal tibial lengthening is then carried out but initially without a fibula osteotomy or proximal fibula fixation so the fibula is drawn down. When the fibula is at the correct level proximally, a

second-stage fibula osteotomy is carried out, and the proximal fibula is incorporated into the frame while the lengthening is continued. In this patient it was necessary to carry out corrective ankle surgery and lengthening simultaneously because of the later referral.

Images During Treatment See Figs. 5, 6, 7, 8, and 9.

Technical Pearls The ankle anatomy is likely to be quite abnormal and early identification of the neurovascular bundle is recommended. It may not be in the expected position. In this patient the orientation of the bundle was abnormal, the tibialis posterior lay between the fibula and tibia, and the tibialis anterior is inserted into the navicular. There was a fusion between the talus and the os calcis, but the soft tissue release alone was

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Fig. 6 The fibula has now been captured by the frame and a fibula osteotomy carried out Fig. 5 AP of the leg during treatment – note the tibia only is lengthened at this stage and the proximal fibula has been drawn down compared to Fig. 2

sufficient to correct hindfoot varus. The corrected foot has to be held with a foot frame. It is unwise to put the foot in the fully corrected position if this causes excessive tension on the wound or neurovascular bundle. In this case, leaving the ankle in some equinus and hinging the frame at the ankle level to allow gradual correction of the equinus is safer. The position of the ankle hinge needs to be carefully placed and the hinge needs to be tested intraoperatively. For the differential lengthening and for ankle stability, the distal fibula needs to be incorporated into the frame. The proximal fibula is not incorporated. The patient will be returned to surgery for a second-stage fibula osteotomy. It is wise to bear this in mind when applying the frame so there will be sufficient access to perform the osteotomy and add fixation to the proximal fibula. In this case, a 4 mm half pin was used which gave sufficient stability. The choice of fixation, however, remains at the surgeon’s discretion bearing in mind that the common peroneal nerve may not be in the expected position.

Fig. 7 Lateral X-ray. Note the foot is incorporated in frame and there is a hinge at the ankle level; residual equinus is present after soft tissue correction

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Outcome Clinical Photos and Radiographs See Figs. 10 and 11.

Avoiding and Managing Problems Ultimately, it will be the maintenance of ankle movement that will determine outcome rather than any leg-length discrepancy. Many of these patients have tarsal coalition and for such patients the “normal” ankle is a ball-and-socket ankle. Therefore, trying to give them a tibiotalar hinge-type ankle is not only likely to fail but will result in stiffness. Some patients may come to ankle fusion in early adult life but this is not the intended initial management. The maintenance of

Fig. 8 Lateral X-ray at conclusion of lengthening and correction of equinus using the frame

Fig. 9 AP X-ray at conclusion of lengthening

Fig. 10 Long-leg standing film 2 years after frame removal. With time there has been some recurrence of the length discrepancy between the tibia and fibula. The shortening at this point measures 2.2 cms. The ankle appears unchanged from pre-reconstruction radiographs

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movement – any movement – is helpful for function. Fusion is reserved as a last resort for pain and intractable deformity.

Cross-References ▶ Seven-Year-Old Male with Lower Limb Reconstruction and Lengthening due to Type IV Tibial Deficiency, Foot Equinus and LLD

References and Suggested Reading 1. Pattinson RC, Fixsen JA. Management and outcome in tibial dysplasia. J Bone Joint Surg (Br). 1992;74(6):893–6. 2. Weber M. Congenital leg deformities: tibial hemimelia, Chap 30. In: Ilizarov S, Rozbruch R, editors. Leg lengthening and reconstruction surgery. New York: Informa Health Care; 2007.

Fig. 11 Clinical appearance 2 years after frame removal. The patient now aged 6½ is active and participates in ballet and uses a 1 cm shoe lift

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Varus Deformity of the Distal Femur and LLD Secondary to Ollier’s Disease Corrected by Gradual Deformity Correction and Lengthening

B. Stephens Richards, Alexander Cherkashin, and Mikhail Samchukov

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 809 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 809 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 810 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 810 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 810 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 811 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 811 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 813 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 813 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 814 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 814 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 814

Abstract

A 9 year old male with progressive varus deformity of the distal femur and LLD secondary to Ollier disease underwent three stages of treatment including (1) distal femoral osteotomy followed by gradual varus deformity correction and lengthening, (2) opposite leg pan-genu epiphysiodesis 2 years later, and (3) staged double-level femoral osteotomy at skeletal maturity and repeated femoral lengthening.

Brief Clinical History A 9 year old male with recurrent progressive 28
genu varum, increasing knee pain and 6 cm femoral shortening secondary to multiple enchondromatosis (Ollier disease), had previously undergone three procedures, each time for deformity correction via closing wedge osteotomy and Steinman pin internal fixation (Fig. 1). With growth, the deformity predictably recurred. His malalignment test revealed 4
varus deformity of the proximal tibia and 24
varus deformity of the distal femur due to the enchondroma.

Preoperative Clinical Photos and Radiographs See Fig. 1. B. S. Richards (*) · A. Cherkashin · M. Samchukov Center for Excellence in Limb Lengthening and Reconstruction, Texas Scottish Rite Hospital for Children, Dallas, TX, USA e-mail: [email protected]; [email protected]; [email protected]; [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_84

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Preoperative Problem List

Treatment Strategy

• Significant (24
) varus deformity of the distal femur combined with slight (4
) varus deformity of the proximal tibia • Associated 6 cm femoral shortening • Structural changes of the distal femur due to enchondroma • High risk of deformity recurrence and limb shortening requiring opposite leg pan-genu epiphysiodesis and repeated deformity correction and/or limb lengthening at skeletal maturity

Treatment strategy included three stages. First, existing varus deformity of the distal femur was gradually corrected and followed by 6 cm of femoral lengthening. Computer-assisted preoperative planning was utilized for this stage using the LegPerfect software (Fig. 2). Initially, the TrueLok circular external fixator was applied with two hinges aligned to correct the femoral deformity with a minimal amount of lengthening. Due to the periarticular location of the deformity, the chosen level of the osteotomy was above the apex of deformity. This should result in mild horizontal translation of the opposed surfaces of the osteotomized bone segments. Completion of the deformity correction was followed by frame modification with replacement of hinges and angular distractor by four telescopic linear distractors to continue limb lengthening. Two years after frame removal, a pan-genu epiphysiodesis of the opposite leg was performed as a second stage to minimize the residual LLD. The final stage of treatment occurred at skeletal maturity and included correction of residual varus deformity and remaining LLD.

Fig. 1 Preoperative standing AP radiograph demonstrating varus deformity of the left lower extremity due to a large enchondroma in the medial aspect of the distal femur (LDFA ¼ 111
, MPTA ¼ 83
, JLCA ¼ 0
)

Basic Principles Ollier disease is often associated with severe bone deformities and progressive shortening of the limbs. Due to extent of the lesions, traditional curettage and bone grafting cannot be used as a treatment modality in the management of multiple enchondromas. Instead, several surgical interventions including corrective osteotomies, epiphysiodeses, gradual angular deformity corrections, and repeated limb lengthening are required to achieve limb realignment and equal limb length at skeletal maturity. Circular external fixation in those cases is the preferred method, as it allows firmly stabilized bone segments and can achieve both angular deformity correction Fig. 2 Computerized preoperative planning of deformity correction using the LegPerfect software. Note medial translation of contacting surface of the distal segment due to location of the osteotomy above the deformity apex

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and limb lengthening simultaneously [2, 3, 6]. Although the presence of enchodromas precludes internal fixation, fully implantable intramedullary lengthening devices were recently introduced for correction of LLD in multiple enchondromatosis cases [1].

Images During Treatment See Figs. 2, 3, 4, 5, 6, 7, 8, and 9.

Technical Pearls Due to large size and periarticular location of lesions, the apex of the deformities in cases with multiple enchondromatosis is often located at the level of the physis. Thus, the osteotomy for angular deformity correction and limb lengthening should be performed at a level above the deformity apex resulting in horizontal translation of the bone segments at the level of the osteotomy. The use of computerized preoperative planning to identify the exact location of the hinge axis is beneficial for successful angular deformity correction in those cases. Although the combination of

Fig. 3 Postoperative AP radiograph of the femur after distal femoral osteotomy and application of the TrueLok circular external fixator. The frame consisted of proximal femoral arch with the floating ring and distal ring interconnected by pair of hinges laterally and angular distractor medially and attached by three half pins and two half pins with lateral olive wire to the proximal and distal segments, respectively

Fig. 4 AP radiograph of the femur after completion of deformity correction demonstrating restored femoral alignment with medial translation of contacting surface of the distal segment according to preoperative planning. Note premature consolidation required re-osteotomy and replacement of bent distal medial half pin. In addition, second medial olive wire was inserted into the distal femoral segment to improve fixation

Fig. 5 AP radiograph of the femur during subsequent femoral lengthening. Note replacement of hinges and angular distractor by four telescopic linear distractors for lengthening

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Fig. 6 AP and LAT radiographs of the femur at the end of consolidation period before frame removal demonstrating solid mineralized distraction regenerate undergoing corticalization

Fig. 7 Standing AP radiograph of the lower extremities at skeletal maturity 4 years after gradual deformity correction with lengthening and 2 years after contralateral pan-genu epiphysiodesis demonstrating restored limb alignment and significant pelvic obliquity due to 6 cm of residual LLD

Fig. 8 AP radiograph of the femur during the repeated femoral lengthening. The frame consisted of proximal femoral arch with the floating ring and distal ring interconnected by four telescopic linear distractors and attached by three half pins and two half pins with lateral olive wire to proximal and distal segments, respectively. Note 3 cm distraction gap filled with mineralizing bone regenerate. At this time, patient underwent secondary osteotomy below to continue staged femoral lengthening

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Fig. 10 Standing AP radiograph of the lower extremities 1 year after frame removal demonstrating restored limb length and improved alignment with complete remodeling of both femoral distraction regenerates

Fig. 9 AP and LAT radiographs of the femur at the completion of sequential femoral lengthening. Note second 3 cm distraction regenerate below the area of initial 3 cm lengthening

intralesionally inserted half pins and multiple wires is usually sufficient for stabilization of the enchondromatous bone, the preference should always be towards using more olive wires rather than half pins to overcome poor mechanical resistance of bone tissue. Presence of enchondroma with intraosseous cartilaginous foci and subperiosteal deposition of cartilage in the area of limb lengthening often alters the normal appearance of distraction regenerate, making it difficult to interpret new bone formation and its maturation. Hence, prolonged consolidation periods for single-level limb lengthening, or sequential osteotomies with staged double-level lengthening, are utilized in those cases.

Outcome Clinical Photos and Radiographs See Figs. 10, 11, and 12.

Avoiding and Managing Problems Unusually rapid mineralization of distraction bone regenerate in cases with multiple enchondromatosis is not uncommon, despite the radiolucent lesions in the region of distraction

[4, 5]. This may result in premature consolidation. Due to the altered radiographic appearance of newly formed bony tissues, typical signs of potential premature distraction regenerate consolidation (hypertrophic callus formation, increased density of mineralizing tissues, and reduction of radiolucent fibrous interzone) are not usually seen in the standard radiographs. Other indicators should be utilized in identification of this complication, such as a disproportion of the amount of distraction to the height of the distraction gap and significant bending of wires and half pins (Fig. 4). Similarly, typical procedures that are used to manage the hypertrophic bone formation to avoid premature consolidation (rapid distraction rate and reduced frequency of distraction) cannot be applied because of potential wire and half pin cutting through the relatively weak bone. Therefore, we prefer immediate release of any distraction forces to preserve existing wires and half pins followed by re-osteotomy and distraction at a different level.

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Fig. 11 Front and side view photographs showing clinical appearance of lower extremities after treatment

Fig. 12 Side view photographs of the lower extremity illustrating preserved knee range of motion after treatment

Cross-References

References and Suggested Reading

▶ Correction of Lower Limb Deformities in Multiple Hereditary Exostosis (MHE) ▶ Genu Valgum and Limb Length Discrepancy in Multiple Enchondromatosis

1. Baumgart R, Bürklein D, Hinterwimmer S, Thaller P, Mutschler W. The management of leg-length discrepancy in Ollier’s disease with a fully implantable lengthening nail. J Bone Joint Surg (Br). 2005;87:1000–4. 2. D’Angelo G, Petas N, Donzelli O. Lengthening of the lower limbs in Ollier’s disease: problems related to surgery. Chir Organi Mov. 1996;81(3):279–85. 3. Jesus-Garcia R, Bongiovanni JC, Korukian M, Boatto H, Seixas MT, Laredo J. Use of the Ilizarov external fixator in the treatment of patients with Ollier’s disease. Clin Orthop Relat Res. 2001;382:82–6. 4. Märtson A, Haviko T, Kirjanen K. Extensive limb lengthening in Ollier’s disease: 25-year follow-up. Medicina (Kaunas). 2005;41(10):861–6. 5. Pandey R, White SH, Kenwright J. Callus distraction in Ollier’s disease. Acta Orthop Scand. 1995;66:479–80. 6. Watanabe K, Tsuchiya H, Sakurakichi K, Yamashiro T, Matsubara H, Tomita K. Treatment of lower limb deformities and limb-length discrepancies with external fixator in Ollier’s disease. J Orthop Sci. 2007;12(5):471–5.

See Also in Vol. 3 Correction of Forearm Deformities in Herediatry Multiple Exostosis (MHE) Correction of Long Bone Deformities due to Ollier’s Disease with Ilizarov Method

Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with Telescoping Fassier-Duval Rods

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Reggie C. Hamdy and Franc¸ois Fassier

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 815 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 816 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 816 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 816 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 817 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 817 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 817 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 818 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 818 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 821 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 821

Abstract

An 11 year old boy with osteogenesis imperfecta type III presented to our clinic with multiple lower limb deformities. He was on bisphosphonate treatment and had no previous surgeries. He was wheelchair bound because of the severe deformities and previous multiple fractures. He successfully underwent femoral and tibial rodding on one side with telescoping Fassier-Duval rods, followed by the other side 3 weeks later. He was immobilized in a posterior slab for 3 weeks and then for another 3 weeks in long leg braces for a total 6 weeks non-weight bearing. Partial weight bearing with intense physiotherapy was then

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] F. Fassier Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada e-mail: [email protected]

started. At 3 months postoperatively he was able to walk independently with long leg braces and a walker. Two years postoperatively, the osteotomies were healed and the rods were telescoping. Since then, he has been ambulating independently with braces and a walker.

Brief Clinical History This is a case of an 11 year old patient with type III osteogenesis imperfecta (OI), unable to stand or walk because of multiple severe lower limb deformities. He was seen in our multidisciplinary OI clinic for further management. Prenatal history was completely normal, with no drug or alcohol abuse and no history of smoking and no family history of brittle bone disease. He was born by C-section and was noted at birth to have a right femur fracture. Since then, he sustained more than 40 fractures which caused delayed gross motor development. He started to pull to stand at the age of 2 but was never able to walk unaided. By the age of 6, he was completely wheelchair

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_21

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dependent. He was started on pamidronate treatment at the age of 24 months at a dose of 1 mg per Kg day for 3 days every 4 months. Once he started bisphosphonate treatment, he sustained no further fractures. On examination, his total height was 110 cms and weight 15.3 kgs. He had blue sclerae and joint hyperlaxity but no dentinogenesis imperfecta. His upper limbs and spine were normal. His lower limbs showed severe deformities in both femora and tibiae.

Preoperative Clinical Photos and Radiographs See Figs. 1, 2, 3, and 4.

Preoperative Problem List • Severe bony deformities with concomitant long-standing soft tissue contractures at the site of deformities that will necessitate shortening of bones whenever surgical reconstruction is contemplated. • Low functioning patient, completely wheelchair bound from the age of 6. • Bone fragility, specifically the osteopenic bones, that renders internal fixation very difficult. Plates should not be used as they cause a stress riser. The use of intramedullary nails is indicated. Since he is still growing, telescoping nails are preferable. • Problems that could be encountered with the use of telescoping nails (bending, failure to telescope, proximal migration of the proximal fragment, and need for exchange rods). • Need for further surgery as the average life span for a telescoping intramedullary nail is about 5 years. • Ensure the availability of postoperative rehabilitation. Fig. 1 (a and b) showing severe deformities of the lower limbs

R. C. Hamdy and F. Fassier

Treatment Strategy – Osteogenesis imperfecta (OI) is a heterogeneous and generalized group of disorders usually caused – in most cases – by a defect in collagen type I synthesis and includes extra-skeletal and skeletal manifestations. OI type III is one of the most severe forms, typically associated with severe deformities as seen in this case. – Surgical treatment should be coordinated with medical treatment and should be part of a multidisciplinary team that includes physiotherapists and occupational therapists. Stop medical treatment at least 48 h before the surgery and do not restart bisphosphonate treatment until 4 months after the surgery. This helps to prevent the bisphosphonate therapy from interfering with healing of the osteotomies. – The indication for surgery in children with OI is the presence of multiple deformities and/or multiple fractures that interfere with function. Prophylactic surgery in the absence of severe deformities or multiple fractures remains questionable and is controversial. – The treatment plan should be discussed in detail with the patient and family. It consists of surgical correction of the lower limb deformities with telescoping FassierDuval rods in order to make the patient more functional, to enable him to walk, and to prevent further fractures. – The plan was to initially perform femoral and tibial rodding on the right side with Fassier-Duval rods. Three weeks later the patient was to have rodding of the left femur and tibia also with Fassier-Duval rods. – Short periods of postoperative immobilization (6 weeks total, non-weight bearing) and intense physiotherapy.

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Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with. . .

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Fig. 2 Anteroposterior X-ray of the lower limbs

Basic Principles – – – –

Use telescoping intramedullary nails (Fassier-Duval). Bony shortening as necessary. Gentle manipulation of the bones. Careful preoperative planning (specifically, the size and length of the Fassier-Duval nails) – One side at a time. – Postoperative rehabilitation extremely important. – Minimal postoperative immobilization (3 weeks in long leg plaster slabs followed by 3 weeks in braces, then partial weight bearing).

Images During Treatment See Figs. 5 and 6.

Fig. 3 Lateral view showing apex of the deformities in the left femur and tibia

Technical Pearls – Perform percutaneous osteotomies for the femur (with multiple drill holes and osteotomes). – Shorten the bone as much as necessary to be able to realign the bone smoothly and get a plantigrade foot. – Be meticulous and follow very carefully all the details and surgical steps described for each technique. – Do not use the largest-diameter rod that fits in the canal, as this may lead to bone resorption. The goal of intramedullary rodding in children with osteogenesis imperfecta is not to replace the bone but rather to serve as a tutor.

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– Insertion of the male rod should be in the center of the epiphysis. – The female rod should slide easily on the male rod. – The duration of postoperative immobilization should be as short as possible; otherwise, more bone resorption will ensue.

Outcome Clinical Photos and Radiographs See Figs. 7 and 8.

Avoiding and Managing Problems

Fig. 4 Lateral view showing apex of the deformities in the right femur and tibia

– Sometimes, the medullary canal is too small to fit the smallest F-D rod (3.2 mm). In such cases, Kirschner wires are used. – In severe cases such as the present one, the use of an Esmarch bandage is not recommended, although a tourniquet can be used for the tibia. – Do not use bone clamps – this may break the fragile bones. – Do not hesitate to shorten the bones in order to reduce the soft tissue tension resulting from long-standing contractures.

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Eleven Year Old Child with Osteogenesis Imperfecta Type III and Multiple Severe Deformities, Treated with. . .

Fig. 5 Surgical technique for insertion of femoral nail. (a) Percutaneous osteotomies at apex of the deformities. Special reamers are used before insertion of the male rod. (b) If necessary, multiple-level osteotomies to allow the nail to slide easily. (c) The threaded end of the male component of the nail should be at the center of the epiphysis in

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both the AP and lateral views. All the threads should be in the epiphysis. (d) The proximal part of the female rod should be threaded in the cartilaginous greater trochanter. The male part is cut leaving about 1.0 cm protruding proximal to the female part

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Fig. 6 Surgical technique for insertion of the tibial nail. (a) Through a standard anteromedial approach, the patellar tendon is retracted laterally and the pre-spinal, extra-articular surface of the tibial plateau is exposed. A small diameter K-wire is inserted antegrade with special

R. C. Hamdy and F. Fassier

attention not to bend the wire. Special reamers are used. (b) After performing the osteotomies, the male rod is inserted. (c) The male rod is centered in the middle of the epiphysis. (d) The male rod is cut and its smoothness checked to allow for telescoping

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Fig. 7 (a and b) X-rays 4 weeks postoperatively

Cross-References ▶ Coxa Vara in a Nine-Year-Old Boy with Osteogenesis Imperfecta ▶ Revision of Bilateral Tibial Fassier-Duval Rods in an Eleven Year Old Girl with Osteogenesis Imperfecta

References and Suggested Reading

Fig. 8 X-ray 2 years postoperatively showing elongation of the nails

1. Anam EA, Rauch F, Glorieux FH, Fassier F, Hamdy RC. Osteotomy healing in children with osteogenesis imperfecta receiving biphosphonate treatment. J Bone Miner Res. 2015;30(8):1362–8. 2. Burnei G, Vla C, Georgescu I, Gavriliu T, Dan D. Osteogenesis imperfecta: diagnosis and treatment. J Am Acad Orthop Surg. 2008;16:356–66. 3. Esposito PW. Multiple percutaneous osteotomies and Fassier-Duval telescoping nailing of long bones in osteogenesis imperfecta. Chapter 37. In: Flynn J, editor. Operative techniques in paediatric orthopaedics. Philadelphia: Wolters Klumer/Lippincott Williams & Wilkins; 2011. p. 259–69. 4. Esposito P, Plotkin H. Surgical treatment of osteogenesis imperfecta: current concepts. Curr Opin Pediatr. 2008;20:52–7. 5. Fassier F, Glorieux F (2003) Osteogenesis imperfecta. Surgical techniques in orthopaedics and traumatology. Paris : Elsevier SAS, pp 1–8, 55–050–D–30 6. Ruck J, Dahan-Oliel N’m, Montpetit K, Rauch F, Fassier F (2011) Fassier–Duval femoral rodding in children with osteogenesis imperfecta receiving bisphosphonates: functional outcomes at one year. J Child Orthop 5:217–224 7. Shapiro JR, Sponsellor PD. Osteogenesis imperfecta: questions and answers. Curr Opin Pediatr. 2009;21:709–16.

Part VIII Pediatric Deformity: Pediatric Metabolic and Vascular Disorders

Pediatric Metabolic and Vascular Disorders: An Introduction

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Reggie C. Hamdy

Rickets is a common disorder of children, caused by inadequate supply or metabolism of calcium, phosphorous, or Vitamin D, all of which are necessary elements for the development of healthy growing bones. While nutritional rickets (poor dietary intake) remains the most common cause of this disease worldwide, sex-linked Vitamin D-resistant hypophosphatemic rickets is believed to be the most common cause of this disorder in North America. Pathologically, rickets is characterized by deficient mineralization of newly formed bone (preformed osteoid is unmineralized), and the endochondral calcification at the growth plate is absent or reduced leading to growth deformity. Clinically, this results in stunted growth and soft and weak bones causing multiple generalized deformities, specifically around the knee in the form of bow legs, knock knees, and windswept knees. As this is a systemic disorder, deformities are typically bilateral, multi-apical, multi-planar, and multi-segmental. Rotational deformities, often severe, are also very common (three of the four cases presented in this section). Medical treatment (oral phosphate and Vitamin D) remains the cornerstone of management of these conditions. However, in some cases, the deformities are not fully corrected despite adequate medical treatment and can even progress or recur. In such cases, surgical treatment is indicated. High recurrence rate of

deformities and a tendency toward delayed and nonunions of osteotomies are two potential complications largely dependent on the age at which surgery was performed and is generally independent of the type of corrective osteotomy or type of fixation used. In this section, four cases of Pediatric Rickets are discussed. All were adolescents with complex deformities around the knee and with significant rotational components in cases 98, 100 and 101 (Table 1). Failed growth modulation with 8-plates and recurrence after acute correction is emphasized in case 101. A combined approach consisting of acute correction of femoral deformities with fixator assisted nailing and gradual correction of tibial deformities with external fixators was used in two cases (100, 101). All four cases used various types of external fixators (TSF, MAC device, TrueLok and hybrid TSF/Ilizarov) and the benefits of using somatosensory evoked potential during surgery are discussed in case 101. Deformities in patients with metabolic disorders are complex and require a multidisciplinary approach, where medical control of the disease remains an essential part of the treatment. If TAR syndrome was diagnosed, the use of biologics (bone marrow injection, bone grafts and BMP7) is important as significant adjuncts in the treatment of delayed bone formation at the sites of distraction (as discussed in case 102).

R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_371

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R. C. Hamdy

Table 1 Details of the 5 cases of Pediatric Metabolic Disorders discussed in this Atlas Case 98

Diagnosis 11 year old child, X-linked hypophosphatemic rickets (XHPR)

Problems Distal femoral varus and procurvatum, tibial recurvatum. Bilateral rotational deformities of femurs and tibiae. Multiapical deformities of both femurs and tibiae

99

13 year old female, hypophosphatemic rickets, worsening of deformities despite medical treatment

Genu valgum from distal femur and proximal tibia. Sagittal plane deformities of the femur and tibia

100

13 year old female, X-linked hypophosphatemic rickets

Distal femoral varus and procurvatum, tibia varus and procurvatum with multiapical deformities, internal tibial torsion 45

101

14 year old female, X-linked hypophosphatemic rickets after failed growth modulation with two hole plates, recurrence after femoral osteotomies and acute correction, controlled medical treatment

Complex genu varum deformity from distal femoral and proximal tibia varus, incongruent knee joint, internal tibial torsion 40 , LLD 2.5 cm Nonspecific polyarticular pain

102

14 year old female, venous haemangiomatosis affecting entire right knee, tibia and ankle

LLD 7.6 cms, bifocal deformity Tibia valgus and recurvatum. Delayed union proximal and distal tibial lengthening sites

TSF Taylor spatial frame MAC multiaxial correction

Surgery and key points Single-event correction of all deformities on one side with TSF and short Ilizarov arch on proximal femur, double osteotomy of the femur and tibia. Nine months later, other side corrected. Flexible intramedullary nails also used with external fixators. No overcorrection performed Femoral and tibial osteotomies and gradual correction with double MAC device, common peroneal nerve release, syndesmotic screw of the distal tibia and fibula One limb at a time, retrograde fixatorassisted nailing of distal femoral deformity (acute correction) followed by double tibial osteotomies and double TSF. After removal of TSF, walking Sarmiento patellar-bearing cast was placed Detailed preoperative planning of these multiplanar deformities is needed. Acute correction of femoral deformity and internal fixation with intramedullary nails; gradual correction of tibial deformities with TrueLok frame utilizing intraoperative SSEP (somatosensory evoked potential) Double proximal and distal tibial osteotomies, gradual correction with TSF. Bone marrow injection, bone graft, BMP7 open reduction internal fixation locking plate, William rod

Deformity Correction in Child with X-Linked Hereditary Hypophosphatemic Rickets by Combined Technique (External Fixation and Flexible Intramedullary Nailing)

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Dmitry A. Popkov and Pierre Lascombes

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 827 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 828 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 828 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 828 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 828 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 828 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 828 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 829 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 829 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 830 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 830 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 831

Abstract

X-linked hereditary hypophosphatemic rickets (XHPR) is the most common form of the hypophosphatemic rickets caused by renal phosphate wasting, impaired renal production of 1,25-dihydroxyvitamin D3, and abnormal bone mineralization. Clinical orthopedic features become obvious at walking age and are manifested by varus or valgus deformities of the lower limb, torsion deformities, impaired gait, short stature, and pathologic fractures. In this case we describe a multilevel, multiplanar deformity correction using the Ilizarov method and Taylor

D. A. Popkov (*) National Ilizarov Medical Research Center for Traumatology and Orthopaedics, Kurgan, Russia e-mail: [email protected] P. Lascombes Division of Pediatric Orthopaedics, University of Geneva, Geneva, Switzerland e-mail: [email protected]

device in association with intramedullary elastic nails in children with XHPR. The combined technique allows correction of lower limb deformities as well as a method to decrease the duration of external fixation.

Brief Clinical History The patient presented at age 11 with bilateral varus procurvatum deformity at the level of the femoral diaphysis, recurvatum of the tibiae, and pathologically decreased aLDFA (70
on the right, 68
on the left). Also, there were low values of anteversion of the neck (2
on the right, 9
on the left) and internal torsion of the tibiae (15
on the right and 11
on the left). The deformities impaired ambulation, and the patient complained about mechanical pain in the knees, ankles, and feet after continuous walking over 30–40 min.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_345

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Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Preoperative Problem List – Varus procurvatum deformities of the femoral diaphyses – Recurvatum of tibiae – Pathologic aLDFA (70
on the right, 68
on the left) and aMPTA (78
on the right, 76
on the left) – External torsion of femurs (anteversion of the neck measures 2
on the right, 9
on the left) – Internal torsion of tibiae (15
on the right and 11
on the left) – MAD 10 mm on the right and 18 mm on the left

Treatment Strategy Plan of surgical treatment was a single-event correction of all deformities in the right and left limbs. A hexapodal Taylor frame was the most convenient instrument for correction of these complex deformities.

D. A. Popkov and P. Lascombes

The location of the CORAs required an osteotomy of the femoral and tibial bones at two levels each. Application of the flexible intramedullary nailing combined with the external fixator proved to be beneficial: secondary displacement was prevented, especially lateral translation while correcting the torsion deformities, and the consolidation period and duration of external fixation were decreased. To make the frame assembly less bulky in the upper third, a short Ilizarov arch with half-pins was used. The interval between the surgeries was 9 months.

Basic Principles – In progressive limb lengthening with any type of external fixator, the principles of the Ilizarov method should be respected (stable elastic osteosynthesis, percutaneous osteotomy aimed to preserve periosteal and intramedullary blood supply of the bone, distraction initiated on the 5th postoperative day, optimal rate and rhythm of distraction, early weight bearing and mobilization of adjacent joints). – Flexible intramedullary nailing is performed according to the basic principles, but the diameter of the nails is 20–25% of the medullary canal diameter. – When double osteotomies of a segment (femur or tibia) are performed, a bipolar sliding intramedullary nailing is recommended. – In XHPR the MAD restoration should be maximally precise without overcorrection.

Images During Treatment See Figs. 3, 4, and 5.

Technical Pearls

Fig. 1 Showing the affected legs

• Using a short Ilizarov arch in the upper third of the femur enables the external fixator to be less bulky. • For immediate deformity correction, Taylor Spatial Frame Fast FX struts can be used even between the Ilizarov arch and Taylor ring. • Ambulation with crutches is allowed starting the 2nd–3rd postoperative day, and mobilization of the adjacent joints is initiated at the same time. • Gradual correction (including torsion deformities) can begin on the 5th day. • During the fixation period walking with full weight bearing on the leg is obligatory. • The intramedullary nails are not removed – they remain as an internal immobilizer.

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Fig. 2 Showing X-ray and CT to demonstrate torsion deformities

Outcome Clinical Photos and Radiographs See Fig. 6.

Avoiding and Managing Problems – Weekly consultations with the surgeon for monitoring are held at least once a week during distraction period and during fixation at least once every 2 weeks.

– Intramedullary nails can become blocked in the proximal fragment at the level of the half-pins during insertion. It may cause migration of the intramedullary nails in the medullary canal. To prevent this, the flexible nails should be inserted up to the upper third of the diaphysis and the half-pins should be placed in the metaphysis. – The combined technique requires practice and experience of the surgeon in both the external fixation and flexible intramedullary nailing techniques.

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Fig. 3 The first stage of treatment – correction of the left lower limb: (a) X-ray of the limb when correction is achieved; (b) showing the limb, osteosynthesis is performed with a Taylor Spatial Frame fixator in combination with Ilizarov components Fig. 5 X-rays of the right leg when the deformity correction is completed

Cross-References ▶ Vitamin D-Resistant Hypophosphatemic Rickets Treated by Double-Level Femoral Osteotomy with Internal Fixation and Proximal Tibial Osteotomy with Gradual Deformity Correction

See Also in Vol. 3 Complex Four Segment Multiapical Lower Extremity Deformities in Rickets Treated with Fixator Assisted Intramedullary Nailing Multiapical Deformity Correction in Bilateral Femur and Tibia in Rickets. LATP Technique to Decrease the Time of External Fixator

Fig. 4 After removal of the external fixator (duration of the external osteosynthesis – 109 days). (a) X-ray of the lower limb. (b) Image of the legs

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Fig. 6 After frame removal from the right lower limb (duration of the external osteosynthesis – 113 days). (a) X-rays of the lower limbs. Notice the correct alignment of the mechanical axes (MAD ¼ 0 on the right and left); there is no relapse of any deformity in the left leg. (b) Image of patient demonstrating that the mobility of the adjacent joints is recovered

References and Suggested Reading 1. Choi IH, Kim JK, Chung CY, Cho TJ, Lee SH, Suh SW, Whang KS, Park HW, Song KS. Deformity correction of knee and leg lengthening by Ilizarov method in hypophosphatemic rickets: outcomes and significance of serum phosphate level. J Pediatr Orthop. 2002;22 (5):626–31. 2. Fucentese SF, Neuhaus TJ, Ramseier LE, Ulrich EG. Metabolic and orthopedic management of X-linked vitamin D-resistant hypophosphatemic rickets. J Child Orthop. 2008;2:285–91.

3. Ilizarov GA. Clinical application of the tension-stress effect for limb lengthening. Clin Orthop. 1990;250:8–26. 4. Popkov D. Use of FIN for correction of deformities in children with familial hypophosphatemic rickets. In: Lascombes P, editor. Flexible intramedullary nailing in children. Berlin: Springer; 2010. p. 291–9. 5. Popkov D, Lascombes P, Berte N, Hetzel L, Baptista BR, Popkov A, Journeau P. The normal radiological anteroposterior alignment of the lower limb in children. Skeletal Radiol. 2014;44:197–206. https://doi. org/10.1007/s00256-014-1953-z.

Genu Varum Secondary to Rickets Treated with Femoral and Tibial Osteotomy and Application of a Multi-axial Correcting External Fixation System

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R. Jay Lee and Richard S. Davidson

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 833 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 833 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 833 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 834 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 834 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 835 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 835 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 835 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 835 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 836 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 836

Abstract

A 13 year old female with genu varum secondary to hypophosphatemic rickets was treated with femoral and tibial osteotomies with application of multiaxial correcting external fixation system (MAC) for gradual correction of the angular deformity. Iliotibial band fasciotomy, a left distal tibiofibular syndesmotic screw, lower leg anterior and lateral compartment releases, and a peroneal nerve release were also performed.

Brief Clinical History The patient is a 13 year old female with hypophosphatemic rickets causing genu varum bilaterally. Despite being managed medically by endocrinology, she continued to have worsening of the deformity. The decision was made to proceed with a staged surgical intervention.

Preoperative Clinical Photos and Radiographs See Figs. 1 and 2.

Disclosure: Dr. Davidson is a consultant for Biomet and receives royalties on the MAC external fixator of which he is a coinventor. R. J. Lee (*) Johns Hopkins Bloomberg Children’s Center, Baltimore, MD, USA e-mail: [email protected]; [email protected] R. S. Davidson Department of Orthopaedics, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA e-mail: [email protected]; [email protected]

Preoperative Problem List • Genu varum, with contributions from the distal femur and proximal tibia. • Increased anterior femoral bow • Increased posterior tibial slope

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_46

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Fig. 1 AP radiograph of the lower extremity, demonstrating a femoral and tibial component to the genu varum

Fig. 2 Lateral radiograph of the lower extremity, demonstrating the increased anterior femoral bowing and the increased posterior slope of the proximal tibial plateau

Treatment Strategy

Basic Principles

The preoperative plan includes identification of the center of rotation of angulation (CORA) for both the femur and tibia, on both the sagittal and coronal planes. The tibial osteotomy is placed just below the CORA at a level just below the tibial tubercle to prevent patella baja. The osteotomies enable gradual correction of the combined deformity. Soft tissue and nerve releases are performed to avoid tethers to the correction and iatrogenic neuropraxia. A syndesmotic screw was to be placed to maintain the proper relationship of the distal tibia and fibula, in the ankle joint. Fasciotomies were to be performed prophylactically. After gradual correction of the deformity, and healing of three of four sides of the tibial osteotomy, the external fixator and the syndesmotic screw will be removed.

The goal of this procedure is to obtain a neutral mechanical axis with a horizontal knee joint. The MAC external fixation system in the management of lower extremity deformity allows the gradual correction of the deformity in two planes of angulation, two planes of translation, rotation, and lengthening. In this case, correction of varus angulation of both the femur and tibia, the excessive anterior bowing of the femur, and the increased posterior slope of the proximal tibia were obtained. This monolateral fixator allows these corrections without circumferential rings or computer-based calculations. A technique guide is available for the MAC fixator [1]. The soft tissue releases are necessary to remove any tether, while the peroneal nerve release is critical as correction of varus ultimately places stretch on the nerve. The syndesmotic

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screw prevents disrupting the distal tibia-fibula relationship. Prophylactic lower leg compartment releases are recommended for prevention of compartment syndrome.

Outcome Clinical Photos and Radiographs

Images During Treatment

Avoiding and Managing Problems

See Figs. 3 and 4.

Medical workup and management of rickets should be maximized before considering surgical intervention. One must be mindful of the cross-sectional anatomy of the lower leg, and place the external fixation pins in the safe zones, avoiding neurovascular structures. Prophylactic fasciotomies and careful neurovascular exams are performed to avoid and detect compartment syndrome. Postoperative care should include detailed instructions on the prescribed adjusting of the apparatus. Close follow-up should be maintained to detect and avoid malalignment during attempted corrections. As with all external fixation devices, pin sites should be monitored, and removal of the external fixation device should not be attempted until three of four cortices are healed.

Technical Pearls Preoperative planning for correction of this deformity is key to achieving the desired outcome. Appropriate radiographs must identify all coronal and sagittal plane deformities, so that they may be addressed simultaneously. The axis of correction must be placed carefully, as placing the hinge directly over the CORA minimizes translation. Placing the hinge proximal or distal to the CORA produces translation, while placing the hinge on the convex or concave side of the deformity lengthens or shortens.

Fig. 3 Intraoperative photo after placement of the external fixator

See Fig. 5.

Fig. 4 Radiograph of the lower leg during gradual correction of the deformity

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Fig. 5 (a–b) Intraoperative photo, demonstrating the angular correction, just prior to external fixator removal

Cross-References

References and Suggested Reading

▶ Adolescent Blount’s Disease Treated with MAC External Fixation System ▶ Distal Femoral Deformity Correction Using MAC ▶ Tibia Valga Treated with Tibial and Fibular Osteotomy and Application of a Multi-axial Correcting External Fixation System

1. Davidson RS. The MAC, (multi-axial correcting) monolateral external fixation system (Biomet/EBI) technique: an easier way to correct deformity. Oper Tech Orthop. 2011;21:113–24. 2. Paley D, Herzenberg JE, Tetsworth K, McKie J, Bhave A. Deformity planning for frontal and sagittal plane corrective osteotomies. Orthop Clin North Am. 1994;25:425–65.

Infantile Myofibromatosis

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Gamal Ahmed Hosny

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 837 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 838 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 838 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 838 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 838 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 838 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 838 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 839 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 839 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 840 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 840

Abstract

A 4 year old child presented to us with 11 cm tibial shortening, 90 anteromedial angulation at the junction of the middle and distal thirds with violent discoloration of the skin over the apex of deformity, and 180 internal rotation of the foot (the heel faces strictly forward and the forefoot directed posteriorly). There was valgus heel and metatarsus adductus. The diagnosis was infantile myofibromatosis. Operative treatment is with application of Ilizarov external fixator and double corticotomy to correct the deformities. The magnitude of lengthening achieved was 11 cm. The angulation was corrected and derotation of the foot was undertaken till the normal foot position was achieved. Besides, the foot deformity was corrected using the bloodless technique. Complications were (1) neurapraxia as the patient could not move his toes postoperatively, (2) pin track infection, and (3) partial recurrence of the deformity. Conclusion: Up to the best of G. A. Hosny (*) Benha Faculty of Medicine, Cairo, Egypt e-mail: [email protected]

our knowledge, this is the first time to report the management of deformities in cases with infantile myofibromatosis.

Brief Clinical History The baby was born as normal vaginal delivery with no specific problem in the perinatal period. This baby weighed 3.4 kg with a head circumference of 32.8 cm and height 50 cm. Clinical examination revealed an isolated lesion of the left leg which involved almost the entire length and circumference of the leg, sparing the upper most part and the ankle. The lesion felt hard and nodular. Ultrasound examination of the viscera revealed no abnormality. Routine blood tests were normal. Plain X-rays revealed an osteolytic lesion of the left tibia. Biopsy was performed after 6 weeks of birth, and the result was infantile myofibromatosis. The patient was referred to us at the age of 4 years. Clinical examination revealed 11 cm tibial shortening, 90 anteromedial angulation, and about 180 f. rotation.

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_58

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Preoperative Clinical Photos and Radiographs See Fig. 1a, b, c.

G. A. Hosny

lengthening and correction of the angulation, we modified the frame into a derotation construct where we derotated the foot at a rate of 1 mm per day. The frame was removed after 12 months in the outpatient clinic and above knee cast was applied for 1 month.

Preoperative Problem List 1. The presence of the benign tumor : infantile myofibromatosis. 2. Tibial deformities: shortening, anteromedial angulation, and foot rotation from the distal tibia. 3. Foot deformities: valgus heel and metatarsus adductus. 4. The limb has one vessel which may be endangered by K-wire introduction.

Basic Principles As the solitary bony lesions of infantile myofibromatosis usually regress spontaneously [1], we did not excise the lesion and we used the site for corticotomy. We corrected the deformity from the CORA site which is the tumor site. Another site of corticotomy was performed in the upper tibia for lengthening to reduce the time in the frame.

Treatment Strategy Ilizarov external fixator in the form of three and half rings was applied to the upper part, middle part, and lower part of the tibia and calcaneus. Corticotomy was performed between the upper and middle rings; another corticotomy was done at the apex of the deformity or the site of the lesion. The fibula was osteotomized at the middle third. We did 30 acute derotation from the lower corticotomy site. However, the patient could not move his toes after the operation due to neurapraxia. Neurapraxia resolved after 3 months. Three days after the operation, distraction started from both corticotomy sites at a rate of half mm per day (we reduced the rate because of the neurapraxia). At the end of

Images During Treatment See Fig. 2a, b, c, d, e.

Technical Pearls The use of the circular frame was important to correct the complex deformities. The frame was modified in the outpatient clinic many times to combat the residual deformities. Arterial mapping was done immediately before the operation to avoid the vascular supply of the limb.

Fig. 1 (a) Plain X-ray taken at birth showing eccentric osteolytic lesion. (b) Photo of the left leg and foot at the age of four. (c) CT scannogram

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Fig. 2 (a) Immediate postoperative X-ray. (b) Postoperative photo before distraction. (c) Postoperative photo at the end of lengthening. (d) Postoperative photo at the end of lengthening and angular correction. (e) Postoperative X-ray before frame removal

Outcome Clinical Photos and Radiographs

Avoiding and Managing Problems

See Fig. 3a, b, c, d.

It is better to avoid any acute correction of the rotational deformity due to the possibility of neurovascular problems.

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Fig. 3 (a) Photo at the end of foot derotation. (b) Photo at the end of foot derotation. (c) X-ray at the end of follow-up. (d) Photo of both lower limbs at follow-up

Cross-References ▶ Tibial Hemimelia

References and Suggested Reading 1. Chung EB, Enzinger FM. Infantile myofibromatosis. Cancer. 1981;48:1807–18.

2. Hausbrandt PA, Leithner A, Beham A, Bodo K, Raith J, Windhager R. A rare case of infantile myofibromatosis and review of literature. J Pediatr Orthop B. 2010;19(1):122–6. 3. Wiswell TE, Davis J, Cunningham BE, Solenberger R, Thomas PJ. Infantile myofibromatosis: the most common fibrous tumor of infancy. J Pediatr Surg. 1988;23(4):315–8. 4. Wu W, Chen J, Cao X, Yang M, Zhu J, Zhao G. Solitary infantile myofibromatosis in the bones of the upper extremities: two rare cases and a review of the literature. Oncol Lett. 2013;6(5):1406–8.

Hypophosphatemic Rickets with Bilateral Severe Genu Varum. Retrograde Fixator Assisted Nailing for Femurs and Double Level Tibial Osteotomies with TSF

122

Marie Gdalevitch

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 841 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 842 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 842 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 842 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 843 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 844 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 845 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 845 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 845 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 845 See Also in Vol. 3 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 845 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 847

Abstract

This is a case of a skeletally mature patient with hypophosphatemic rickets and bowed legs. Rickets patients often present with multi-apical deformities, and this patient had limb deformities in all planes (coronal, sagittal, and rotational). Her case demonstrates the combination of acute and gradual correction techniques when dealing with limb deformities in both the femur and the tibia. In patients with metabolic bone disorders, intramedullary nailing can help prevent recurrence. Therefore, these patient’s femurs were treated with fixator-assisted nailings, and her tibias were treated with double-level osteotomies and circular external fixators for gradual correction.

Brief Clinical History This is the case of a 13 year old girl with familial (x-linked) hypophosphatemic rickets. Two of her other siblings as well as her mother also have this condition. The family has been followed at our hospital for many years and the patient herself was not very compliant with her medication as a child. She presents at skeletal maturity with significantly bowed legs in the coronal and sagittal planes. She is having knee and ankle pains when walking. On exam, she has full range of motion of her hips and ankles. However, she appears to have a 5–10 flexion deformity of both knees and an internal thigh-foot axis of 40 bilaterally. Patient’s radiographs demonstrate varus malalignment and procurvatum deformities of the limbs.

M. Gdalevitch (*) Verdun Hospital, Montreal, QC, Canada e-mail: [email protected] © Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_16

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Preoperative Clinical Photos and Radiographs See Figs. 1 and 2. See Tables 1 and 2.

M. Gdalevitch

2. Tibia varus and procurvatum with two apices at the proximal and distal third junctions 3. Internal tibial torsion of 45 bilaterally

Treatment Strategy Preoperative Problem List 1. Femoral varus and procurvatum with the apex of the deformity located at the distal third of the femur

Fig. 1 (a–d) Clinical standing photographs of the patient’s lower extremities demonstrating severe bowing in the coronal and sagittal planes as well as an internal rotation deformity of the tibias

The patient’s treatment consisted of treating one limb at a time. The preoperative deformity assessment demonstrated a single CORA (center of rotation and angulation) in the femur and two CORAs in the tibia. The patient underwent

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Fig. 2 (a–c) Weight-bearing anteroposterior and lateral radiographs of the patient’s lower extremities demonstrating marked varus and procurvatum deformities of the femurs and tibias

Left 82 124 69 104 4 25.9 26.8 +0.3

used, and she was given several programs to complete her gradual tibial correction. The patient requested a small amount of lengthening; therefore, at each tibial osteotomy, we added 1.5 cm of length during the correction. The distal osteotomy was used for most of the rotational correction. She was permitted to weight bear 6 weeks after the initial surgery to allow the femoral osteotomy to heal. Once the tibial correction was achieved, she was encouraged to weight bear as much as possible. The fixator was removed when bony union was achieved, and the patient was placed in a walking Sarmiento patellar-bearing cast for 4 weeks. Three months later, the patient underwent the same surgery for the other leg.

Left 56 72 81

Basic Principles

Table 1 Joint orientation angles in the coronal plane as well as limb length assessments Angles LPFA LDFA MPTA LDTA JLCA Femur (cm) Tibia (cm) Total LLD

Right 86 116 70 113 6 27.3 25.1

Table 2 Joint orientation angles in the sagittal plane Angles aPDFA aPPTA aADTA

Right 67 59 111

retrograde fixator-assisted nailing of her femur with a distal third osteotomy (at the level of the femoral CORA). A commercially available nail that is available in very short lengths and has a 10 bow used upside down to correct the flexion deformity of the bone was inserted. Once the femoral nail was complete, the patient underwent a double-level tibial osteotomy (at the CORAs) with application of a three-ring external fixator construct. A computer-assisted fixator was

Patients with rickets often present with multi-apical bone deformities secondary to the gradual bowing of the bone over time. Medical treatment during childhood can help decrease the severity of the deformities and is the first priority when these patients present, but these deformities can still persist [2, 4]. Deformity correction during growth yields a high risk of recurrence and guided growth should be considered until skeletal maturity [1–3]. Deformity correction through guided growth can be slower than in other children due to the shorter stature and slower growth of

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these patients [4]. In the skeletally mature patients, correction of deformities using intramedullary nailing is recommended when possible to avoid recurrence [1, 3]. The use of combined acute and gradual deformity correction in the same limb allows one to compensate for slight underor overcorrections of the acutely corrected bone. Furthermore, the flexibility of gradual correction to adjust for limb

length inequalities becomes even more important when treating the second limb.

Fig. 3 Intraoperative (a) and postoperative (b, c) radiographs demonstrating the technique for acute deformity correction using retrograde fixator-assisted nailing of the femur. Note the correction of the deformity

and correction using the external fixator (a) before insertion of the nail. Also note the presence of a blocking screw (b) to prevent postoperative shifting of the osteotomy site over the nail

Images During Treatment See Figs. 3, 4, and 5.

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• The use of the nail in extension helps correct the flexion deformity of the distal femur. • Intraoperative radiographs prior to nail insertion are the key to an accurate correction. Prior to taking intraoperative radiographs, an initial alignment test (either with the bovie cord or a grid on the table) to assess the mechanical axis of the limb is recommended.

Outcome Clinical Photos and Radiographs See Figs. 6 and 7.

Avoiding and Managing Problems • Preoperative planning in such cases is critical. Due to the short stature and the underlying bone pathology, the bones are often short with thick cortices. Measuring the length and diameter of the bones for nailing requires appropriate magnification markers. • It is important to remember that during acute deformity corrections, the surgeon often needs to shorten the bone to ensure that the neurovascular structures are not jeopardized. • The regenerate bone during gradual correction in the rickets patients can take longer to heal, and consulting your metabolic team for medical treatment can be helpful. Fig. 4 (a, b) Standing radiograph of the right limb at the time of final correction demonstrating a mechanical axis through the knee and correction of the knee flexion deformity

Cross-References Technical Pearls • Fixator-assisted nailing can be a challenging technique. For deformities in both the sagittal and coronal planes, two fixators are required (one in each plane). Once the fixator pins are in place in the femur, bending the knee to release the fascia lata around the pins can facilitate correction after the osteotomy is complete. • The short stature of rickets patients requires careful preoperative templating to ensure that you have an intramedullary nail short enough to fit the patient.

▶ Genu Varum Secondary to Rickets Treated with Femoral and Tibial Osteotomy and Application of a Multi-Axial Correcting External Fixation System

See Also in Vol. 3 Multiapical Deformity Correction in Bilateral Femur and Tibia in Rickets. LATP Technique to Decrease the Time of External Fixator

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Fig. 5 Clinical photographs demonstrating the double-level frame at the time of application as well as the corrected limb and frame at the time of removal

Fig. 6 Radiograph prior to the removal of the tibial external fixator on the left side showing the corrected limb alignment

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Hypophosphatemic Rickets with Bilateral Severe Genu Varum. Retrograde Fixator Assisted Nailing for Femurs. . .

Fig. 7 (a–c) Final standing radiographs of both limbs in coronal and sagittal planes demonstrating the corrected alignment of the limbs. Note that the regenerate of the distal osteotomy of the left tibia was slow to

References and Suggested Reading 1. Eralp L, Kocaoglu M, Toker B, Balci HI, Awad A. Comparison of fixator-assisted nailing versus circular external fixator for bone realignment of lower extremity angular deformities in rickets disease. Arch Orthop Trauma Surg. 2011;131(5):581–9. 2. Petje G, Meizer R, Christof Radler C, Aigner N, Grill F. Deformity correction in children with hereditary hypophosphatemic rickets. Clin Orthop Relat Res. 2008;466(12):3078–85.

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heal, and the patient continues to wear a Sarmiento brace until the healing will be complete

3. Song HR, Soma Raju VV, Kumar S, Lee SH, Suh SW, Kim JR, Hong JS. Deformity correction by external fixation and/or intramedullary nailing in hypophosphatemic rickets. Acta Orthop. 2006;77(2):307–14. 4. Stevens PM, Klatt JB. Guided growth for pathological physes: radiographic improvement during realignment. J Pediatr Orthop. 2008;28 (6):632–9.

Part IX Pediatric Deformity: Pediatric Hip Deformities

Pediatric Hip Deformities: An Introduction

123

Reggie C. Hamdy

The hip joint is one of the wonders of the human body. It is the largest joint, and its main function is to support the body’s weight during various daily activities of standing, walking, running, as well as various sports activities. Its ball-and-socket design allows a wide range of motion in several planes: flexion/extension, adduction/abduction, and internal and external rotation, thus allowing movements in almost any direction. The stability of that joint is increased by the presence of a labrum that deepens the acetabulum, a thick capsule, and the presence of very strong surrounding ligaments and muscles. However, the hip joint does have a major weakness, and that is its tenuous blood supply, which mostly comes from the medial circumflex artery supplying the epiphysis and metaphysis of the proximal femur. There is minimal blood supply coming from the nutrient arteries of the femur. Therefore, any insult or injury to that blood supply in the immature skeleton may have serious consequences on the viability and shape of the epiphysis and metaphysis and can result in abnormal anatomy. This may eventually lead to pain, decreased range of motion, abductor weakness, limp, limb length discrepancy, degenerative arthritis, and functional limitations. To help restore the normal anatomy to the hip joint and provide the patient with a painless mobile hip joint that allows him or her to ambulate and function in a satisfactory way remains the ultimate goal of any reconstructive surgery in the pediatric and adolescent population. This is challenging since it requires one to address not only the bony deformities but also to take into consideration the articular cartilage and the surrounding soft tissue envelope, specifically the hip musculature. In this section, eight cases that deal with severe deformities of the femoral head and neck are presented. In patients where the proximal femur is destroyed by sepsis and the R. C. Hamdy (*) Department of Pediatric Surgery, Shriners Hospital - Canada, Montreal, QC, Canada e-mail: [email protected]

proximal femur migrates proximally, the technique of Pelvic Support Osteotomy offers a reasonable alternative to hip fusion or arthroplasty (cases 103, 104). As described initially by Ilizarov, it consists of double femoral osteotomies, the proximal one to stabilize the hip and the distal one to correct the malalignment and the limb length discrepancy. Furthermore, this technique increases the length of the abductor lever arm, leading to improved range of motion, increased abductor muscle power, and improvement of the Trendelenburg gait (Table 1). In cases of advanced Perthe’s disease (case 105) and of post-traumatic avascular necrosis of the femoral head (case 110), articulated hip distraction may be indicated and may lead to restoration of nearly normal anatomy of the femoral head as it offloads the articular cartilage while at the same time allowing movement by placing the hinges at the rotation center of the hip joint. The disadvantage of this technique, however, is that the external fixator has to be kept on for several months to allow remodeling of the femoral head and this may lead to problems. In some cases of Perthe’s disease, coxa magna develops leading to incongruence of the hip joint. The principle of Femoral Head Reduction Technique by surgical dislocation, introduced by Ganz is another possibility to treat femoral head deformity and coxa magna (case 105). Slipped Capital Femoral Epiphysis (SCFE) is the most common hip joint pathology affecting adolescents (cases 107 and 108). Where the management of cases with stable SCFE is uniformly accepted to be pinning in situ, much controversy exists as to the best approach toward cases of unstable SCFE. Emergency pinning in situ and decompression of the hip joint still remains the gold standard and the residual deformity of the femoral neck is addressed at a later stage. Two different techniques for addressing these late deformities are described in this section. The first one is by surgical dislocation, removal of the cam lesion and a subtrochanteric osteotomy (case 107). The second technique is a percutaneous osteotomy of the proximal femur stabilized with an external

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Table 1 Details of the 8 Pediatric Hip Deformity cases discussed in this Atlas Case 103

Diagnosis Septic destruction of the Hip and significant LLD

104 105

Post-infective septic destruction left hip Perthes disease

106

Late onset Perthes disease

107

Healed slipped capital femoral epiphysis with residual deformity Post SCFE deformity

108

Problems 13 year old male with significant hip instability, Trendelenburg gait, limited abduction, painful rotation, LLD of 6 cms 10 year old, hip instability, extensive scarring and muscle wasting left thigh, LLD 7.5 cms 16 year old male, severe limp, painful limited range of motion, Coxa Magna 11 year old female patient, pain, decreased range of motion, limp, femoral head collapse 15 year old male, groin pain, cam lesion proximal femur, coxavara, femoral head retroversion

Surgery and key points Pelvic support osteotomy and femoral lengthening

Percutaneous subtrochanteric osteotomy, stabilized with external fixator Surgical hip dislocation, hip fusion, internal fixation, offloading subtrochanteric osteotomy and iliofemoral external fixation Hinged arthro-diastasis left hip, core decompression, BMP2, calcium/phosphate and bisphosphonates

109

Early degenerative arthritis of the hip, Tonnis 3

12 year old male, painful femor-acetabular Impingement, LLD and decreased range of motion 13 year old male, LLD 1.5 cms, Trendelenburg gait, stiff hip with no range of motion

110

Avascular necrosis hip, post left femoral neck fracture

18 year old male, pain, limping, decreased range of motion hip, avascular necrosis Stage III with early collapse

fixator (case 108). In cases with severe degenerative changes in the hip associated with stiffness and pain, hip fusion remains an excellent option. A combined intra- and extra-articular approach with a subtrochanteric osteotomy stabilized with external fixator is described (case 109).

Pelvic support osteotomy and femoral lengthening Surgical dislocation, femoral head reduction osteotomy Articulated hip distraction with monolateral hip distractor Surgical dislocation, cam resection and femoral osteotomy

Four other cases of severe hip pathology in patients with Proximal Femoral Focal Deficiency (PFFD) are discussed in ▶ Chap. 34, “Congenital Lower Limb Deficiencies: An Introduction” and include the SuperHip Procedures and Van Nes Rotationplasty.

Healed Slipped Capital Femoral Deformity

124

Neil Saran

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 853 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 854 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 854 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 854 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 854 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 854 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 854 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 856 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 856 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 856 References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 856

Abstract

Healed deformity of slipped capital femoral epiphysis (SCFE) is known to be associated with acetabular, labral, and chondral pathology that results in early osteoarthritis of the hip. Furthermore, the metaphyseal bump in addition to proximal femoral anatomy can cause alterations in range of motion of the hip that precludes participation in various activities including some activities of daily living. While the modified Dunn osteotomy allows for an excellent correction of the deformity, recent literature has shown unacceptably high rates of avascular necrosis. Safer forms of treatment include resection of the femoral head-neck junction (metaphyseal) bump or realignment of the proximal femur through intertrochanteric/subtrochanteric osteotomies. In moderate to severe cases, a combined approach is sometimes required. N. Saran (*) Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada e-mail: [email protected]

Brief Clinical History This 15-year and 3-month-old male presented with a 6-month history of left groin pain. He was having difficulty playing a full game of football and would limp for at least a day after playing. He was treated for a stable SCFE 3 years earlier with an in situ pinning. Examination revealed a normal gait and no Trendelenburg sign. He had 95 of flexion, 15 of obligate external rotation at 90 of hip flexion, 80 of external rotation, and 20 of abduction of the left hip compared to 130 of flexion, 30 of internal rotation, 45 of external rotation, and 35 of abduction on the other side. He had groin pain on flexion, adduction, and internal rotation of the hip. Radiographs revealed a cam lesion of the proximal femur along with the typical findings of a moderate slip including coxa vara, decreased articulotrochanteric distance, and femoral head retroversion. It was decided that in order to alleviate the impingement and to improve range of motion, a cam resection and femoral osteotomy were required.

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Preoperative Clinical Photos and Radiographs

Basic Principles

See Fig. 1.

The surgical dislocation enables a safe resection of the cam lesion of the femoral head and treatment of labral pathology and/or peripheral cartilage damage. In cases of mild SCFE, this may be enough; however, in moderate or severe cases of SCFE, the cam resection alone may not result in an adequate range of motion (100 of flexion and 20 of internal rotation) to preclude persistent femoral acetabular impingement. As such, a flexion, valgus, derotation osteotomy of the intertrochanteric region is performed to give the patient a more normal range of motion that is less likely to impinge. Furthermore, the abductor lever arm and mild leg length discrepancy due to the SCFE can be improved by the valgusization.

Preoperative Problem List • Cam lesion • Femoral retroversion, coxa vara, and external rotation

Treatment Strategy The treatment strategy consists of treating the impingement and deformity using a vascular-friendly approach to the femoral head. The surgical hip dislocation [1] allows for a safe approach to the hip and treatment of the femoral cam lesion as well as any anterior or superior labral pathology that may be identified at the time of dislocation. Other options include anterior arthrotomy without dislocation and arthroscopic techniques to remove the bump although if one of these other approaches is utilized, the femoral deformity should be addressed first to facilitate the intra-articular osteoplasty. While the ultimate treatment of the deformity would consist of a femoral neck osteotomy, this approach carries with it a significant risk for femoral head avascular necrosis; therefore, an intertrochanteric osteotomy is preferred. The goal of the osteotomy is to improve the neck shaft angle, articulotrochanteric distance, femoral retroversion, and rotation of the hip.

Fig. 1 (a) Anteroposterior radiograph showing a healed SCFE of the left hip previously treated with an in situ pin. The left hip has a decreased neck shaft angle (115 ), decreased articulotrochanteric distance, and lateral metaphyseal bump (white arrow). (b) The frog-lateral

Images During Treatment See Figs. 2 and 3.

Technical Pearls The procedure is performed in the lateral decubitus position. A surgical hip dislocation is performed. The SCFE screw is removed, and the cam lesion is resected. In the event that the SCFE screw becomes stuck, cut off the screw head and use a reverse trephine to remove the screw. The residual hole can be bone grafted with the bone from the cam resection. If required, labral or anterior acetabular rim work can be done prior to closing the capsule. The trochanter is positioned and temporarily fixed with threaded wires. If after the cam resection, hip flexion is less than 100 or internal rotation is less

pelvis X-ray shows 40 of femoral head retroversion and large anterior metaphyseal bump (white arrow) at the femoral head-neck junction that is limiting hip flexion

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Fig. 2 (a) Intraoperative photograph of the femoral head-neck junction showing the metaphyseal bump (black arrow) and decreased femoral head offset. After resection of the bump (b), offset was restored (block arrow) and the SCFE screw was uncovered and removed. After removal of the bump, internal rotation of the hip improved to approximately 10 .

(c) Photograph of the acetabulum revealing fissures (black arrow) within the anterosuperior acetabular cartilage as well as some areas of complete cartilage loss (white arrow) which were microfractured prior to relocation

Fig. 3 (a) Intraoperative fluoroscopy view showing the temporary fixation of the greater trochanter while the proximal femoral flexion (40 ), valgus (20 ), derotation osteotomy (30 ) was being performed.

(b) Lateral fluoroscopy view showing approximately 40 of flexion correction with 50 % apposition at the osteotomy site

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than 20 , an osteotomy is performed. Either a blade plate or a locking plate can be used to perform the intertrochanteric osteotomy. The entry point must be located posteriorly on the lateral aspect of the proximal femur to prevent excessive translation of the osteotomy. The amount of flexion is based on the extent of femoral head retroversion. Flexion is limited to a maximum of about 40 , any further correction will result in minimal contact of bone at the osteotomy site and will increase the risk of nonunion. The amount of valgus can be determined on an AP radiograph to recreate a normal articulotrochanteric distance. However, be aware that correcting the femoral head retroversion will in fact result in some restoration of the articulotrochanteric distance, and therefore the ideal radiograph to determine the necessary valgus is a prone AP with the affected hip in maximal extension [2]. The amount of derotation is based on the amount of internal rotation a patient has at 90 of flexion after the cam resection has been performed. The goal is to create neutral anteversion (symmetric internal and external rotation); however, a minimum of 20 of internal rotation is desired once the osteotomy is complete. Once the osteotomy is fixed, the final fixation for the trochanter can be performed as is typically done for the surgical hip dislocation technique if it has not already been captured by the plate.

Outcome Clinical Photos and Radiographs See Fig. 4.

Avoiding and Managing Problems The major problem in treating healed SCFE deformity is ensuring adequate fixation and bony contact of the osteotomy. In treating the coxa vara and retroversion, deformity correction results in a translation of the proximal bony fragment because the axis of correction is not in the same plane as the axis of the deformity, and therefore, bony contact between the fragments can be compromised. Firstly, the plate entry should be kept posterior to minimize translation. This usually precludes capturing the epiphysis in the proximal fixation. Rather, the fixation ends in the femoral neck calcar. Secondly, the flexion component should be kept to a maximum of 40 in order to minimize translation. Finally, valgusization must be accompanied by lateralization of the femoral shaft in order to not shift the mechanical axis of the lower extremity, and therefore an appropriate plate must be chosen to allow for lateralization.

Fig. 4 (a) Anteroposterior pelvis radiograph showing restoration of the neck shaft angle and articulotrochanteric distance 6 months postoperatively. (b) Frog-lateral pelvis radiograph showing restoration of the femoral head version and elimination of the cam lesion at the femoral head-neck junction. The patient returned to football the following season with no further episodes of pain. At his 2 year follow up, he remains asymptomatic and has 120 of hip flexion, 35 of hip abduction, 35 of internal rotation, and 45 of external rotation

Cross-References ▶ Percutaneous Osteotomy of the Proximal Femur for Slipped Capital Femoral Epiphysis

References 1. Ganz R, Gill TJ, Gautier E, Ganz K, Krügel N, Berlemann U. Surgical dislocation of the adult hip a technique with full access to the femoral head and acetabulum without the risk of avascular necrosis. J Bone Joint Surg (Br). 2001;83(8):1119–24. 2. Kelley SP, Graham HK. Letter to the editor on “Quantitative evaluation of angular measurements on plain radiographs in patients with slipped capital femoral epiphysis”. J Pediatr Orthop. 2010;30(1):99.

Hinged Arthrodiastasis for Avascular Necrosis of the Hip

125

David S. Feldman and Adam M. Kurland

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 857 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 860 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 860 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 860 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 861

Abstract

Avascular necrosis of the hip with collapse will routinely lead to progressive arthropathy. The treatment of this disorder has ordinarily been protected weight bearing with limited surgical options once collapse has already occurred. An 18 year old male with avascular necrosis of the left hip following a left femoral neck fracture was treated surgically with arthrodiastasis (external fixation of the femur spanning the hip), core decompression, bone morphogenetic protein 2 (BMP2), calcium sulphate/phosphate, and bisphosphonates. This combination of treatment allowed for an excellent outcome and prevented further collapse of the femoral head. D. S. Feldman (*) Pediatric Orthopedic Surgery, NYU Langone Medical Center, New York, NY, USA e-mail: [email protected] A. M. Kurland Pediatric Orthopedic Surgery, NYU Langone Medical Center, Hospital for Joint Diseases, New York, NY, USA e-mail: [email protected]

Brief Clinical History The patient is an 18 year old male with post-traumatic avascular necrosis (AVN) of the left hip. At age 16 he sustained a displaced left femoral neck fracture from a fall while hiking. He continued to hike on the injured hip for 13 miles. He was seen in an emergency room where his X-rays revealed a displaced femoral neck fracture (Fig. 1a, b) and then underwent open reduction and internal fixation (ORIF) of the left femur to treat this fracture. Forty-eight hours postsurgery he underwent aspiration of the left hip due to an intra-articular hematoma. The fracture healed over the subsequent 2 months with protected weight bearing. The patient presented to our institution 4 months postoperatively with symptoms of hip pain. An MRI taken at that time demonstrated stage III AVN of the left hip with early collapse and fracture (Fig. 2a, b). Upon examination the patient had full flexion of the left hip with diminished internal rotation and abduction as well as pain when attempting internal rotation. His right hip had full range of motion with no pain. The patient was placed on crutches to protect weight bearing on the left side. X-rays were taken in

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Fig. 1 Anteroposterior (a) and cross-table lateral (b) X-rays taken in the emergency room at the time of injury revealing a displaced femoral neck fracture

Fig. 2 Coronal (a) and (b) view MRI demonstrating AVN of the superior head with mild collapse of the left hip

anticipation of surgical intervention (Fig. 3a, b). One month later the patient was treated surgically with removal of the hardware, arthrodiastasis with an external fixator spanning the hip and core decompression with injection of a combination including 75% calcium sulphate and 25% calcium phosphate and insertion of BMP2 into the femoral head. The patient’s hip was distracted 5 mm acutely in the operating room (Fig. 4). Postoperatively the patient was allowed to weight-bear as tolerated on the left lower extremity. He was given three 0.01 mg/kg infusions of zoledronate as well. The

first dose was given 2 days prior to surgery. The second and third doses were given 6 weeks and 3 months postoperatively. Follow-up to check range of motion of the hip and distraction was done at 2 weeks, 6 weeks, and 3 months postoperatively (Fig. 5). The frame was removed in the operating room 4 months after surgery. The patient was most recently seen a year postoperatively for follow-up with no complaints of pain or discomfort. There has been no further collapse of the femoral head. He has good motion of the left hip and a normal gait with no pain (Figs. 6a, b and 7a, b).

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Fig. 3 Preoperative anteroposterior (a) and frog lateral (b) radiographs of the hip showing internal fixation post femoral neck fracture with healed femoral neck fracture

Fig. 4 Postoperative radiograph of the left proximal femur and hip 2 weeks after distraction of the left hip. Note that the external fixator spans the pelvis and is distracting the joint

Fig. 5 Anteroposterior radiograph taken 2 weeks prior to removal of the external fixator following completion of treatment demonstrate healing of the avascular segment and asymptomatic heterotopic ossification at the insertion point

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Fig. 6 Anteroposterior (a) and frog lateral (b) radiographs of the left femur 2 weeks after removal of hardware from the left proximal femur and hip (9 months after femoral neck fracture)

Fig. 7 Anteroposterior (a) and frog lateral (b) radiographs of the pelvis 8 months after removal of hardware from the left proximal femur and hip (16 months after femoral neck fracture)

Preoperative Clinical Photos and Radiographs

Treatment Strategy

See Figs. 1, 2, and 3.

The treatment plan included removal of the cannulated screws, core decompression, injection of a combination including 75% calcium sulphate and 25% calcium phosphate, insertion of BMP2 into the femoral head, and distraction of the hip with application of a hinged external fixation crossing the pelvis. The arthrodiastasis was performed to prevent intra-articular collapse as the avascular necrosis healed and allowed for treatment of existing collapse. The patient had three pins placed proximal to the superior acetabulum into the

Preoperative Problem List • Avascular necrosis of the hip • Femoral head collapse confirmed on MRI • Potential for probable further collapse and arthropathy

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iliac wing, two pins applied intra-iliac, and three across the proximal femur for attachment to the hinged fixator (Fig. 4). The hinge was maintained at the level of the center of the femoral head before and after distraction.

Basic Principles • Van Valberg et al., using the anterior cruciate ligament transection (ACTL) model in dogs (a generally accepted model for osteoarthritis (OA) in humans), hypothesized that the absence of mechanical stress in concert with intermittent fluid pressure and flow during joint distraction would allow for cartilaginous repair and an overall improvement of the distracted joint. The study found that articulated joint distraction in the canine osteoarthritic knee resulted in a decrease of secondary inflammation of the synovial tissue that is present in both human OA and canine ACTL. This finding has been additionally supported by studies using cocultures of human OA synovial fluid [6]. Intra-articular intermittent fluid pressure was created during distraction via joint movement. Additionally, following joint distraction, the abnormal cartilage proteoglycan (PG) metabolism had reverted to the levels found in two control groups (the contralateral knee as well as knees in a control population). The adjustment in PG levels suggested normalization of chondrocyte function indicating the possibility for long-term repair of the cartilage as articulating joint distraction counteracted the characteristics of chondrocytes in OA cartilage [7]. • External fixation for arthrodiastasis of the hip in avascular necrosis using the center of the femoral head as the center of rotation unloads the joint thereby reducing the stresses of direct contact by the femoral head on the articular surface while allowing the joint to remain mobile. The mobility of the distracted joint provides the intermittent fluid pressure and flow beneficial to cartilaginous repair of the hip joint. • Bisphosphonates are utilized both pre- and postoperatively to inhibit the rapid resorption of bone. Bisphosphonates encourage osteoclast cells to undergo spontaneous apoptosis, thereby shifting the equilibrium between creation and destruction of bone in favor of osteoblast cells. This may aid more rapid healing of the avascular segment and prevent collapse. • BMP2 stimulates production of bone by the proliferation of osteoblast cell differentiation in undifferentiated perivascular mesenchymal cells through serine-threonine kinase receptors. • Calcium sulphate/calcium phosphate provides a skeletal framework for regenerate bone with a high compressive strength and can be resorbed by the body.

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• Core decompression and reaming of the bone remove devascularized bone, relieving intraosseous pressure and lessening pain, and stimulate angiogenesis, promoting the formation of new, healthy bone and revascularization of the femoral head.

Images During Treatment See Figs. 4 and 5.

Technical Pearls • Align the fixator hinge precisely with the axis of rotation of the femoral head. • The femoral head is used as the center of rotation during arthrodiastasis. • Soft tissue releases become necessary if neutral alignment of the hip cannot be obtained intraoperatively for attachment of the external fixator. If this is not done correctly, there is an increased risk of limited and painful hip movement with loosening of the pins as the most likely possibility [3, 5].

Outcome Clinical Photos and Radiographs See Figs. 6 and 7.

Avoiding and Managing Problems • Abduct the hip 10 while applying the external fixator. • Soft tissue contractures should be treated during the initial procedure. • Check judet views to ensure the pelvic pins do not extend into the sciatic notch and are not anteriorly too long.

Cross-References ▶ Avoiding Amputation and Prosthetics in Children with Complex Lower Limb Deformities ▶ Perthes: Femoral Head Reduction Osteotomy

References and Suggested Reading 1. Bachiller FG, Caballer AP, Portal LF. Avascular necrosis of the femoral head after femoral neck fracture. Clin Orthop Relat Res. 2002;399:87–109. 2. Hosny GA, El-Deeb K, Fadel M, Laklouk M. Arthrodiastasis of the hip. J Pediatr Orthop. 2011;31(Suppl 2):S229–34.

862 3. Scher DM, Jeong GK, Grant AD, Lehman WB, Feldman DS. Hip arthrodesis in adolescents using external fixation. J Pediatr Orthop. 2001;21(2):194–7. 4. Steinberg ME, Larcom PG, Strafford B, Hosick WB, Corces A, Bands RE, Hartman KE. Core decompression with bone grafting for osteonecrosis of the femoral head. Clin Orthop. 2001;386:71–8. 5. Thacker MM, Feldman DS, Madan SS, Straight JJ, Scher DM. Hinged distraction of the adolescent arthritic hip. J Pediatr Orthop. 2005;25(2):178–82.

D. S. Feldman and A. M. Kurland 6. van Valburg AA, Van Roy JLAM, Lafeber FPJG, Bijlsma JWJ. Beneficial effect of intermittent fluid pressure, of a low but physiological magnitude, on cartilage and inflammation in osteoarthritis. J Rheumatol. 1998;25:515–20. 7. van Valburg AA, van Roermund PM, Marijnissen AC, Wenting MJ, Verbout AJ, Lafeber FP, Bijlsma JW. Joint distraction in treatment of osteoarthritis (II): effects on cartilage in a canine model. Osteoarthr Cartil. 2000;8(1):1–8.

Hip Fusion with an External Fixator

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Neil Saran

Contents Brief Clinical History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 863 Preoperative Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 864 Preoperative Problem List . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 864 Treatment Strategy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 864 Basic Principles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 865 Images During Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 865 Technical Pearls . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 865 Outcome Clinical Photos and Radiographs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 865 Avoiding and Managing Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 865 Cross-References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 867 References and Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 867

Abstract

Although the long-term outcomes of total hip arthroplasty continue to improve, their survival in adolescent patients still remains a serious concern. In adolescent patients with severe degenerative joint disease of the hip, it can be argued that hip fusion is still the preferred treatment option. Short-term problems with hip fusion include nonunion, malalignment, and limb length discrepancy. Longterm problems include degenerative low back pain and ipsilateral knee pain as well as a difficult conversion to total hip arthroplasty. While the short-term problems can be minimized using the approach described below, the long-term problems of adjacent segment degenerative joint disease cannot and may in fact necessitate future conversion to total hip arthroplasty. Avoiding the use of complex plating techniques and minimizing trauma to the N. Saran (*) Shriners Hospital and Montreal Children Hospital, Division of Paediatric Orthopaedics, McGill University, Montreal, QC, Canada e-mail: [email protected]

abductor musculature during hip arthrodesis are important factors to consider for future total hip arthroplasty.

Brief Clinical History This adolescent male was first seen at age 13 years and 3 months for a history of worsening right hip pain. The hip pain started insidiously 2–3 years earlier and had been progressively worsening to the point where he had a constant limp and decreased ability to participate in sports. His hip pain was located in the groin. He had start-up pain in the groin and developed lateral hip pain over the course of the day with activities. There was no history of antecedent hip problems. Gait assessment revealed an abductor lurch as well as a 1.5 cm LLD. He had a positive Trendelenburg sign and had virtually no abduction/adduction or internal/external rotation. He had 90 of hip flexion and a minimal hip flexion contracture. Radiographs revealed Tonnis grade-3 osteoarthritis of the right hip with an LLD of 1.5 cm. A discussion regarding possible treatment options including hip fusion and

© Springer Nature Switzerland AG 2024 S. R. Rozbruch et al. (eds.), Limb Lengthening and Reconstruction Surgery Case Atlas, https://doi.org/10.1007/978-3-031-77359-4_343

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total hip replacement was undertaken, and he was sent for a rheumatologic, hematologic, and infectious workup which all came back negative. He returned to the clinic 4 months later with a further decline in function and had stopped participating in all activities due to increasing pain. He was managing to ambulate without an assistive device; however, his limp had worsened quite markedly and he was prescribed a cane at that visit. A further discussion regarding treatment was undertaken and he was booked for a hip fusion by surgical hip dislocation, internal fixation, offloading subtrochanteric osteotomy, and iliofemoral external fixation. In addition, he was booked for growth modulation of the opposite limb to ensure that the LLD was minimized.

Preoperative Clinical Photos and Radiographs See Fig. 1.

Preoperative Problem List • Hip OA of unknown origin in an adolescent patient • LLD 1.5 cm Fig. 1 (a) Anteroposterior pelvis radiograph reveals severe osteoarthritis with lateral subluxation. (b) Anteroposterior lower extremity image performed with a 2 cm left under the right foot demonstrates a 1.5 cm limb length discrepancy secondary to joint collapse. (c) A coronal plane computed tomography cut of the pelvis shows severe joint space loss with subchondral cysts within the acetabulum and femoral head

N. Saran

Treatment Strategy The treatment strategy entails obtaining an adequate fusion of the hip with the leg in a functional position with minimal disruption of the abductor mechanism. In order to achieve an optimal surface preparation for the fusion, the surgical hip dislocation approach [2] is chosen as it gives excellent exposure of the femoral head and acetabulum. Furthermore, this approach is abductor sparing. Rigid compressive fixation is achieved with partially threaded 7.3 mm cannulated screws without the need for complex plating techniques that can further compromise the abductor mechanism either during the initial surgery or at the time of conversion to a total hip. An offloading osteotomy is performed to decrease the lever arm and moment on the hip fusion fixation and thus allow for an adequate fusion and minimize the risk of nonunion [1]. An iliofemoral fixator is applied to stabilize the osteotomy. The external fixator consists of a hinge at the offloading osteotomy site and a rail to allow for realignment and lengthening if desired.

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Basic Principles • • • •

Surface preparation Alignment Rigid compressive fixation Offloading osteotomy

Images During Treatment See Figs. 2 and 3.

Technical Pearls The keys to a good outcome after a hip fusion are obtaining union, ensuring adequate alignment of the leg, and minimizing an LLD to