Comprehensive Pediatric Hospital Medicine [2 ed.] 9780071829298

Table of contents :
Cover Page
Title Page
Copyright Page
Dedication Page
Contents
Section Editors
Contributors
Foreword
Preface
Part I Inpatient Pediatric Medicine
1 Understanding the Value of Pediatric Hospitalists: Studies of Efficiency, Education, Care Processes, and Quality of Care
2 Variation in Healthcare
3 Evidence-Based Medicine
4 Overview: Quality of Care
5 Infection Control for Pediatric Hospitalists
6 Electronic Health Records and Clinical Decision Support
7 Family-Centered Care
8 Medical Comanagement and Consultation
9 Child Development: Implications for Inpatient Medicine
10 Palliative Care
11 Communication and Discharge Planning
12 Ethical Issues in Pediatric Hospital Practice
13 Medicolegal Issues in Pediatric Hospital Medicine
14 Hospitalist Professional Organizations and Models of Care
15 Careers in Hospital Medicine
Part II Common Presenting Signs and Symptoms
16 Abdominal Mass
17 Abdominal Pain
18 Acidosis
19 Altered Mental Status
20 Chest Pain
21 Cyanosis
22 Diarrhea
23 Failure to Thrive
24 Fever
25 Gastrointestinal Bleeding
26 Hypertension
27 Hypoglycemia
28 Hypoxemia
29 Irritability and Intractable Crying
30 Limp
31 Lymphadenitis
32 Oral Lesions and Oral Health
33 Neck Pain
34 Petechiae and Purpura
35 Respiratory Distress
36 Shock
37 Syncope
38 Vomiting
Part III Systems Approach
Section A: Abuse and Neglect
39 Cutaneous Injuries of Concern for Nonaccidental Trauma
40 Abusive Head Trauma
41 Imaging of Child Abuse
42 Medical Child Abuse: Münchausen Syndrome by Proxy and Other Manifestations
43 Legal Issues
Section B: Adolescent Medicine
44 Eating Disorders
45 Sexually Transmitted Infections in Adolescents and Young Adults
46 Abnormal Uterine Bleeding
Section C: Allergy and Immunology
47 Anaphylaxis
48 Drug Allergy
49 Primary Immunodeficiency Diseases
50 Immunoglobulin
Section D: Cardiology
51 The Cardiac Examination
52 Electrocardiogram Interpretation
53 Congenital Heart Disease
54 Infective Endocarditis
55 Myocarditis and Cardiomyopathy
56 Pericarditis
57 Acute Rheumatic Fever
Section E: Dermatology
58 Purpura
59 Vesicles and Bullae
60 Vascular Anomalies
61 Atopic Dermatitis
62 Ecthyma Gangrenosum
63 Drug-Associated Rashes
64 Erythema Multiforme
65 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
66 Skin Disease in Immunosuppressed Hosts
67 Epidermolysis Bullosa
Section F: Endocrinology
68 Diabetes Mellitus and Hyperglycemia
69 Disorders of Thyroid Hormone
70 Disorders of Pituitary Function
71 Disorders of Calcium Metabolism
72 Disorders of the Adrenal Gland
Section G: Fluids and Electrolytes
73 Dehydration
74 Fluid and Electrolyte Therapy
Section H: Gastroenterology and Nutrition
75 Biliary Disease
76 Constipation
77 Dyspepsia
78 Disorders of Gastric Emptying
79 Liver Failure
80 Inflammatory Bowel Disease
81 Malnutrition
82 Pancreatitis
83 Feeding Issues
Section I: Genetics and Metabolism
84 Genetic Syndromes Caused by Chromosomal Abnormalities
85 Hyperammonemia
86 Hypoglycemia
87 Metabolic Acidosis
Section J: Hematology
88 Anemia
89 Management of Sickle Cell Disease
90 Neutropenia and Bone Marrow Failure
91 Thrombocytopenia
92 Disorders of Coagulation and Thrombosis
93 Transfusion Medicine
Section K: Infectious Diseases
94 Empirical Treatment of Bacterial Infections
95 Fever
96 Prolonged Fever and Fever of Unknown Origin
97 Fever and Rash
98 Central Nervous System Infections
99 Complications of Acute Otitis Media and Sinusitis
100 Neck and Oral Cavity Infections
101 Middle Respiratory Tract Infections and Bronchiolitis
102 Lower Respiratory Tract Infections
103 Gastrointestinal Infections
104 Urinary Tract Infections in Childhood
105 Bone and Joint Infections
106 Skin and Soft Tissue Infections
107 Device-Related Infections
108 Human Immunodeficiency Virus
109 Infections in Special Hosts
Section L: Nephrology
110 Acute Renal Failure
111 Chronic Renal Failure
112 Glomerulonephritis
113 Hemolytic Uremic Syndrome
114 Interstitial Nephritis
115 Nephrotic Syndrome
116 Renal Tubular Acidosis
117 Renal Venous Thrombosis
Section M: Neurology
118 Seizures
119 Headache
120 Hypotonia and Weakness
121 Stroke, Arteriopathy, and Vascular Malformations
122 Demyelinating Disease
Section N: Newborn Medicine
123 Delivery Room Medicine
124 The Well Newborn
125 Birth Injury
126 Congenital Anomalies
127 Transient Tachypnea of the Newborn and Persistent Pulmonary Hypertension
128 Congenital and Perinatal Infections
129 Hypoglycemia and Infants of Diabetic Mothers
130 Neonatal Hyperbilirubinemia
131 Neonatal Abstinence Syndrome
Section O: Oncology
132 Childhood Cancer
133 Oncologic Emergencies
134 Hematopoietic Stem Cell Transplant
Section P: Psychiatry
135 Depression and Physical Illness
136 Assessment and Management of the Suicidal Patient
137 Conversion and Pain Disorders
138 Agitation
139 New-Onset Psychosis
Section Q: Pulmonology
140 Apparent Life-Threatening Event, Infant Apnea, and Pediatric Obstructive Sleep Apnea Syndrome
141 Asthma
142 Aspiration
143 Bronchopulmonary Dysplasia and Chronic Lung Disease of Infancy
144 Cystic Fibrosis
145 Choking and Foreign Body Aspiration
146 Pulmonary Function Testing
Section R: Rheumatology
147 Kawasaki Disease
148 Henoch-Schönlein Purpura
149 Juvenile Dermatomyositis
150 Juvenile Idiopathic Arthritis
151 Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome
152 Infection-Associated Arthritis
153 Systemic Lupus Erythematosus
Section S: Surgical Issues
154 Gastrointestinal Obstruction: Pyloric Stenosis, Malrotation and Volvulus, and Intussusception
155 Appendicitis
156 Hernias
157 General Trauma
158 Ear, Nose, and Throat
159 Neurosurgical Issues
160 Ophthalmology
161 Orthopedics
162 Burns and Other Skin Injuries
163 Pneumothorax and Pneumomediastinum
164 Urology
Section T: Toxins, Substance Abuse, and Environmental Exposures
165 Stabilization and Hospitalization of the Poisoned Child
166 Toxicity of Over-the-Counter Medications and Oral Hypoglycemic Agents
167 Hazardous Household Chemicals: Hydrocarbons, Alcohols, and Caustics
168 Lead, Other Metals, and Chelation Therapy
169 Drugs of Abuse
170 Withdrawal Syndromes
171 Fire-Related Inhalational Injury
172 Heat Disorders
173 Hypothermia and Cold-Related Injuries
174 Drowning
175 Human and Animal Bites
176 Envenomation
177 Infant Botulism
178 Anticoagulants and Antithrombotics
Section U: Care of the Child with Medical Complexity
179 Introduction to the Child with Medical Complexity
180 Acute Care of the Child with Medical Complexity
181 Managing Comorbidities in Children with Severe Neurologic Impairment
182 Technologic Devices in the Child with Medical Complexity
183 Do-Not-Attempt-Resuscitation Orders
Part IV Procedures
184 Procedural Sedation
185 Radiology for the Pediatric Hospitalist
186 Ultrasonography for the Pediatric Hospitalist
187 Lumbar Puncture
188 Cerebrospinal Fluid Shunt Assessment
189 Bladder Catheterization
190 Arterial Blood Gas
191 Vascular Access
192 Intraosseous Catheters
193 Umbilical Artery and Vein Catheterization
194 Phlebotomy
195 Noninvasive Positive-Pressure Ventilation
196 Emergent Airway Management
197 Replacing a Tracheostomy Tube
198 Thoracentesis
199 Arthrocentesis
Index

Citation preview

Comprehensive Pediatric Hospital Medicine

NOTICE Medicine is an ever-changing science. As new research and clinical experience broaden our knowledge, changes in treatment and drug therapy are required. The authors and the publisher of this work have checked with sources believed to be reliable in their efforts to provide information that is complete and generally in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical sciences, neither the authors nor the publisher nor any other party who has been involved in the preparation or publication of this work warrants that the information contained herein is in every respect accurate or complete, and they disclaim all responsibility for any errors or omissions or for the results obtained from use of the information contained in this work. Readers are encouraged to confirm the information contained herein with other sources. For example and in particular, readers are advised to check the product information sheet included in the package of each drug they plan to administer to be certain that the information contained in this work is accurate and that changes have not been made in the recommended dose or in the contraindications for administration. This recommendation is of particular importance in connection with new or infrequently used drugs.

Comprehensive Pediatric Hospital Medicine Second Edition Editors

Lisa B. Zaoutis, MD Associate Professor of Clinical Pediatrics Department of Pediatrics Perelman School of Medicine of the University of Pennsylvania Director, Pediatrics Residency Program The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Vincent W. Chiang, MD Chief, Inpatient Services Boston Children’s Hospital Associate Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Associate Editors

Brian Alverson, MD Director, Division of Hospital Medicine Hasbro Children’s Hospital Professor of Pediatrics Alpert Medical School Brown University Providence, Rhode Island

Sanjay Mahant, MD, FRCPC, MSc Staff Paediatrician, Division of Paediatric Medicine Associate Professor Department of Paediatrics University of Toronto Hospital for Sick Children Toronto, Canada

Samir S. Shah, MD, MSCE Director, Division of Hospital Medicine James M. Ewell Endowed Chair Attending Physician in Hospital Medicine & Infectious Diseases Cincinnati Children’s Hospital Medical Center Professor, Department of Pediatrics University of Cincinnati College of Medicine Cincinnati, Ohio

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COMPREHENSIVE PEDIATRIC HOSPITAL MEDICINE, Second Edition Copyright © 2018 by McGraw-Hill Education. All rights reserved. Printed in the China. Except as permitted under the United States Copyright Act of 1976, no part of this publication may be reproduced or distributed in any form or by any means, or stored in a data base or retrieval system, without the prior written permission of the publisher. 1 2 3 4 5 6 7 8 9 DSS 22 21 20 19 18 17 ISBN 978-0-07-182928-1 MHID 0-07-182928-8 e-ISBN 978-0-07-182929-8 e-MHID 0-07-182929-6 This book was set in Minion Pro by Cenveo® Publisher Services The editors were Andrew Moyer and Christie Naglieri The production supervisor was Richard Ruzycka Project management was provided by Neha Bhargava The cover designer was Randomatrix The index was prepared by Susan Hunter RR Donnelley Shenzhen was the printer and binder This book is printed on acid-free paper.

Library of Congress Cataloging-in-Publication Data Names: Zaoutis, Lisa B., editor. | Chiang, Vincent W., editor. | Shah, Samir S., editor. | Alverson, Brian, editor. | Mahant, Sanjay, editor. Title: Comprehensive pediatric hospital medicine / editors, Lisa B. Zaoutis, Vincent W. Chiang ; associate editors, Samir S. Shah, Brian Alverson, Sanjay Mahant. Description: Second edition. | New York : McGraw-Hill Education, [2017] | Includes bibliographical references and index. Identifiers: LCCN 2016051100| ISBN 9780071829281 (hardcover : alk. paper) | ISBN 0071829288 (hardcover : alk. paper)

Subjects: | MESH: Pediatrics | Hospitalists | Child, Hospitalized Classification: LCC RJ45 | NLM WS 100 | DDC 618.92—dc23 LC record available at https:// na01.safelinks.protection.outlook.com/?url=https%3A%2F%2Flccn.loc.gov%2F2016051100&data= 01%7C01%7Cjessica.gonzalez%40mheducation.com%7C1ccf4ef0dd404ca9704808d40cb7ecf2%7Cf919b1efc0c3473 JfsZWGscM78%2FO2e5Qoykzt9XpKHflhC5XPRn%2BYcsqQ8%3D&reserved=0 McGraw-Hill Education books are available at special quantity discounts to use as premiums and sales promotions, or for use in corporate training programs. To contact a representative, please visit the Contact Us pages at www.mhprofessional.com.

I am grateful for the wisdom and support of my mentors, colleagues, learners, friends, and family. LBZ

Deepest thanks to all of the hospitalists with whom I have had the privilege to work on CHIPS—I am forever in your debt. I am most appreciative of Drs Fleisher, Schuster, Bachur, and Churchwell for letting me do what I do. And finally, thanks and love to my family who both support me and put up with me; especially Susanne without whom none of this would work. I am eternally grateful. VWC

Contents Section Editors Contributors Foreword Preface

PART I

Inpatient Pediatric Medicine

1 Understanding the Value of Pediatric Hospitalists: Studies of Efficiency, Education, Care Processes, and Quality of Care Rajendu Srivastava and Christopher P. Landrigan 2 Variation in Healthcare Lisa McLeod and Ron Keren 3 Evidence-Based Medicine Jonathan M. Mansbach 4 Overview: Quality of Care Jeffrey Simmons, Paul Hain, and Michele Saysana 5 Infection Control for Pediatric Hospitalists Julia S. Sammons and Susan E. Coffin 6 Electronic Health Records and Clinical Decision Support Levon Utidjian, Eric Shelov, Bimal R. Desai, and Christopher P. Bonafide 7 Family-Centered Care Vineeta Mittal and Michael T. Vossmeyer

8 Medical Comanagement and Consultation Erin Stucky Fisher 9 Child Development: Implications for Inpatient Medicine Deirdre A.L. Caplin 10 Palliative Care Tammy Kang, Lindsay Burns Ragsdale, Daniel J. Licht, Oscar H. Mayer, Gina Santucci, Malinda Ann Hill, Jennifer Hwang, and Chris Feudtner 11 Communication and Discharge Planning Daniel A. Rauch and David Zipes 12 Ethical Issues in Pediatric Hospital Practice Jennifer K. Walter, Sarah Hoehn, and Chris Feudtner 13 Medicolegal Issues in Pediatric Hospital Medicine Steven M. Selbst and Joel B. Korin 14 Hospitalist Professional Organizations and Models of Care Daniel A. Rauch and David Zipes 15 Careers in Hospital Medicine Karen Smith and Mary C. Ottolini

PART II

Common Presenting Signs and

Symptoms 16 Abdominal Mass Daniel A. Rauch 17 Abdominal Pain Timothy Gibson 18 Acidosis Chén Kenyon 19 Altered Mental Status Fabienne C. Bourgeois 20 Chest Pain David A. Kane 21 Cyanosis

Jonathan D. Hron 22 Diarrhea Amy Fleming and Carrie B. Lind 23 Failure to Thrive Stephen D. Wilson 24 Fever Sarah E.S. Parker and Kris P. Rehm 25 Gastrointestinal Bleeding April Buchanan 26 Hypertension Avram Z. Traum and Michael J.G. Somers 27 Hypoglycemia Erica Chung 28 Hypoxemia Bryan L. Stone 29 Irritability and Intractable Crying Karen E. Schetzina 30 Limp Robert Sundel 31 Lymphadenitis Amanda S. Growdon 32 Oral Lesions and Oral Health Suzanne Swanson Mendez 33 Neck Pain Matthew T. Lister and Nicole E. St Clair 34 Petechiae and Purpura Susan H. Frangiskakis 35 Respiratory Distress Juliann Lipps Kim 36 Shock Raina Paul and Andrew M. Fine 37 Syncope Sarah C. McBride

38 Vomiting Anne K. Beasley

PART III

Systems Approach

Section A: Abuse and Neglect 39 Cutaneous Injuries of Concern for Nonaccidental Trauma Celeste R. Wilson 40 Abusive Head Trauma Alice W. Newton 41 Imaging of Child Abuse Jeannette M. Pérez-Rosselló and Paul K. Kleinman 42 Medical Child Abuse: Münchausen Syndrome by Proxy and Other Manifestations Genevieve Preer 43 Legal Issues Lisa Santos Spector

Section B: Adolescent Medicine 44 Eating Disorders Zachary McClain, Daniel H. Reirden, Donald F. Schwarz, and Rebecka Peebles 45 Sexually Transmitted Infections in Adolescents and Young Adults Nadia Dowshen, Sarah Wood, and Jessica Fowler 46 Abnormal Uterine Bleeding Alison J. Culyba and Michele L. Zucker

Section C: Allergy and Immunology 47 Anaphylaxis Jordan Scott and David Riester 48 Drug Allergy Jordan Scott and David Riester 49 Primary Immunodeficiency Diseases

Manish J. Butte and Jolan E. Walter 50 Immunoglobulin Joseph D. Hernandez and Manish J. Butte

Section D: Cardiology 51 The Cardiac Examination Robert L. Geggel 52 Electrocardiogram Interpretation Douglas Y. Mah and Laura M. Bevilacqua 53 Congenital Heart Disease Barry A. Love 54 Infective Endocarditis Michael H. Gewitz 55 Myocarditis and Cardiomyopathy Robert N. Vincent, Margaret J. Strieper, and Kenneth J. Dooley 56 Pericarditis Nicole Sutton 57 Acute Rheumatic Fever David R. Fulton

Section E: Dermatology 58 Purpura Bernard A. Cohen and Anna L. Grossberg 59 Vesicles and Bullae Andrea L. Zaenglein 60 Vascular Anomalies Lily Changchien Uihlein and Marilyn G. Liang 61 Atopic Dermatitis Albert C. Yan, Christine Lauren, Paul J. Honig, and Jonathan M. Spergel 62 Ecthyma Gangrenosum Julie V. Schaffer and Mary Wu Chang 63 Drug-Associated Rashes Cynthia M.C. DeKlotz and Lawrence F. Eichenfield

64 Erythema Multiforme Leslie Castelo-Soccio 65 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Reena Jethva and James R. Treat 66 Skin Disease in Immunosuppressed Hosts Emily M. Berger and Marissa J. Perman 67 Epidermolysis Bullosa Benjamin S. Bolser and Kara N. Shah

Section F: Endocrinology 68 Diabetes Mellitus and Hyperglycemia Christine T. Ferrara, Amanda M. Ackermann, and Andrew A. Palladino 69 Disorders of Thyroid Hormone Katherine Lord and Andrew A. Palladino 70 Disorders of Pituitary Function Rachana Shah 71 Disorders of Calcium Metabolism Lisa Kenigsberg and Chhavi Agarwal 72 Disorders of the Adrenal Gland Jeffrey D. Roizen and Andrew A. Palladino

Section G: Fluids and Electrolytes 73 Dehydration Philip R. Spandorfer 74 Fluid and Electrolyte Therapy Daniel T. Coghlin

Section H: Gastroenterology and Nutrition 75 Biliary Disease Amethyst C. Kurbegov 76 Constipation Michelle W. Parker 77 Dyspepsia Oren L. Koslowe and Denesh K. Chitkara

78 Disorders of Gastric Emptying Richard J. Noel 79 Liver Failure Scott A. Elisofon 80 Inflammatory Bowel Disease Michael C. Stephens and Subra Kugathasan 81 Malnutrition Jennifer Maniscalco 82 Pancreatitis Amit S. Grover and Menno Verhave 83 Feeding Issues Beth D. Gamulka

Section I: Genetics and Metabolism 84 Genetic Syndromes Caused by Chromosomal Abnormalities Jennifer M. Kalish and Elaine H. Zackai 85 Hyperammonemia Karen Smith 86 Hypoglycemia Rebecca D. Ganetzky 87 Metabolic Acidosis David Adams and Charles P. Venditti

Section J: Hematology 88 Anemia Ian J. Davis and Rupa Redding-Lallinger 89 Management of Sickle Cell Disease Matthew M. Heeney and Venée N. Tubman 90 Neutropenia and Bone Marrow Failure John D’Orazio and Akshay Sharma 91 Thrombocytopenia Samuel Volchenboum 92 Disorders of Coagulation and Thrombosis Vlad Calin Radulescu

93 Transfusion Medicine Jeremy Ryan Andrew Peña and Steven R. Sloan

Section K: Infectious Diseases 94 Empirical Treatment of Bacterial Infections Talene A. Metjian and Jeffrey S. Gerber 95 Fever Samir S. Shah and Elizabeth R. Alpern 96 Prolonged Fever and Fever of Unknown Origin Samir S. Shah and Elizabeth R. Alpern 97 Fever and Rash Angela M. Statile and Corinne L. Bria 98 Central Nervous System Infections Kimberly C. Martin and José R. Romero 99 Complications of Acute Otitis Media and Sinusitis Karen E. Jerardi and Samir S. Shah 100 Neck and Oral Cavity Infections Andrew Shriner and Benjamin Bauer 101 Middle Respiratory Tract Infections and Bronchiolitis Todd A. Florin and Samir S. Shah 102 Lower Respiratory Tract Infections Derek J. Williams and Samir S. Shah 103 Gastrointestinal Infections Jaya Goswami and Karen Kotloff 104 Urinary Tract Infections in Childhood Nader Shaikh and Alejandro Hoberman 105 Bone and Joint Infections Sanjeev K. Swami and Stephen C. Eppes 106 Skin and Soft Tissue Infections Keith W. Morley and Howard B. Pride 107 Device-Related Infections Russell McCulloh and Joanna Thomson 108 Human Immunodeficiency Virus

Ari Bitnun 109 Infections in Special Hosts Kevin J. Downes and Lara A. Danziger-Isakov

Section L: Nephrology 110 Acute Renal Failure Lindsay Chase 111 Chronic Renal Failure Lindsay Chase 112 Glomerulonephritis Michelle A. Lopez 113 Hemolytic Uremic Syndrome Sowdhamini Wallace 114 Interstitial Nephritis Jorge F. Ganem 115 Nephrotic Syndrome Joyee G. Vachani 116 Renal Tubular Acidosis Vanessa L. Hill 117 Renal Venous Thrombosis Vanessa L. Hill

Section M: Neurology 118 Seizures Christelle Moufawad El Achkar, Paul E. Manicone, and Annapurna Poduri 119 Headache Christelle Moufawad El Achkar, Annapurna Poduri 120 Hypotonia and Weakness Sabrina W. Yum 121 Stroke, Arteriopathy, and Vascular Malformations Lauren A. Beslow and Lori Billinghurst 122 Demyelinating Disease Sona Narula and Amy T. Waldman

Section N: Newborn Medicine 123 Delivery Room Medicine Kelley Shultz 124 The Well Newborn Mfon Ekong 125 Birth Injury Mark D. Hormann 126 Congenital Anomalies Mary Beth Madonna and Jonathan Frederick Bean 127 Transient Tachypnea of the Newborn and Persistent Pulmonary Hypertension Ebony Beaudoin 128 Congenital and Perinatal Infections Neera Goyal 129 Hypoglycemia and Infants of Diabetic Mothers Laura Placke Ward 130 Neonatal Hyperbilirubinemia M. Katherine Loudermilk 131 Neonatal Abstinence Syndrome Kathy E. Wedig

Section O: Oncology 132 Childhood Cancer Barbara Degar and Michael Isakoff 133 Oncologic Emergencies Andrew E. Place 134 Hematopoietic Stem Cell Transplant Christine N. Duncan

Section P: Psychiatry 135 Depression and Physical Illness Harsh K. Trivedi and Katherine A.S. Gallagher 136 Assessment and Management of the Suicidal Patient Robert L. Kitts and Patricia I. Ibeziako

137 Conversion and Pain Disorders Ashley K. Storrs and Patricia I. Ibeziako 138 Agitation Colleen A. Ryan and Michael L. Trieu 139 New-Onset Psychosis Georgina Garcia

Section Q: Pulmonology 140 Apparent Life-Threatening Event, Infant Apnea, and Pediatric Obstructive Sleep Apnea Syndrome Craig C. DeWolfe, Angela M. Statile, and Aaron S. Chidekel 141 Asthma Sigrid Payne DaVeiga, and Jonathan M. Spergel 142 Aspiration Mark I. Neuman 143 Bronchopulmonary Dysplasia and Chronic Lung Disease of Infancy Ian MacLusky 144 Cystic Fibrosis Marie E. Egan 145 Choking and Foreign Body Aspiration Aaron S. Chidekel and Natalie Hayes 146 Pulmonary Function Testing Andrea. S. Garrod and Daniel J. Weiner

Section R: Rheumatology 147 Kawasaki Disease Elizabeth Ang and Robert Sundel 148 Henoch-Schönlein Purpura Elizabeth Ang and Robert Sundel 149 Juvenile Dermatomyositis Susan Kim 150 Juvenile Idiopathic Arthritis Sampath Prahalad

151 Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome Melissa M. Hazen 152 Infection-Associated Arthritis Jonathan S. Hausmann and Melissa M. Hazen 153 Systemic Lupus Erythematosus Mindy S. Lo

Section S: Surgical Issues 154 Gastrointestinal Obstruction: Pyloric Stenosis, Malrotation and Volvulus, and Intussusception Julie Story Byerley 155 Appendicitis Eric Sundel 156 Hernias William H. Peranteau and Peter Mattei 157 General Trauma Anupam Kharbanda 158 Ear, Nose, and Throat Catherine K. Hart 159 Neurosurgical Issues Sarah F. Denniston and Tamara D. Simon 160 Ophthalmology Alison E. Niebanck and Robin Ray 161 Orthopedics Martin J. Morrison, III, David A. Spiegel, and B. David Horn 162 Burns and Other Skin Injuries Marisa B. Brett-Fleegler 163 Pneumothorax and Pneumomediastinum Joshua Nagler 164 Urology Bartley G. Cilento, Jr.

Section T: Toxins, Substance Abuse, and Environmental

Exposures 165 Stabilization and Hospitalization of the Poisoned Child Kevin C. Osterhoudt 166 Toxicity of Over-the-Counter Medications and Oral Hypoglycemic Agents Diana Felton and Michele M. Burns 167 Hazardous Household Chemicals: Hydrocarbons, Alcohols, and Caustics Kirstin Weerdenburg and Yaron Finkelstein 168 Lead, Other Metals, and Chelation Therapy Rahul Kaila and Jeffrey P. Louie 169 Drugs of Abuse Heather J. Becker and Carl R. Baum 170 Withdrawal Syndromes Robert J. Hoffman and Adhi N. Sharma 171 Fire-Related Inhalational Injury George Sam Wang and Jeffrey Brent 172 Heat Disorders Lise E. Nigrovic and Michele M. Burns 173 Hypothermia and Cold-Related Injuries Jeffrey P. Louie 174 Drowning Khoon-Yen Tay and Frances M. Nadel 175 Human and Animal Bites Rebecca G. Carlisle and Phyllis Lewis 176 Envenomation Christine S. Cho and Kevin C. Osterhoudt 177 Infant Botulism Michael Del Vecchio 178 Anticoagulants and Antithrombotics Michael Levine

Section U: Care of the Child with Medical Complexity

179 Introduction to the Child with Medical Complexity Rishi Agrawal and Nancy Murphy 180 Acute Care of the Child with Medical Complexity David E. Hall, Laurie Glader, and Sangeeta Mauskar 181 Managing Comorbidities in Children with Severe Neurologic Impairment Laurie Glader, Sangeeta Mauskar, and David E. Hall 182 Technologic Devices in the Child with Medical Complexity Jeremy Friedman and Michael Weinstein 183 Do-Not-Attempt-Resuscitation Orders Armand H. Matheny Antommaria

PART IV

Procedures

184 Procedural Sedation Mythili Srinivasan and Michael Turmelle 185 Radiology for the Pediatric Hospitalist Lindsay S. Baron, Horacio Padua, Frederick Grant, and Jeanne S. Chow 186 Ultrasonography for the Pediatric Hospitalist Matthew Garber and J. Kate Deanehan 187 Lumbar Puncture Timothy Gibson 188 Cerebrospinal Fluid Shunt Assessment Christine S. Cho and Jill C. Posner 189 Bladder Catheterization Sandra Schwab 190 Arterial Blood Gas Esther Maria Sampayo and Mirna M’farrej 191 Vascular Access Frances M. Nadel, Suzanne Beno, and Anne Marie Frey 192 Intraosseous Catheters Eron Y. Friedlaender and Lauren E. Marlowe

193 Umbilical Artery and Vein Catheterization Bryan Upham, revised by Julianne Prasto 194 Phlebotomy Christine S. Cho and Jill C. Posner 195 Noninvasive Positive-Pressure Ventilation Garrett Keim, Pamela A. Mazzeo, Raj Padman, and Vanda Passi 196 Emergent Airway Management Timothy Gibson 197 Replacing a Tracheostomy Tube Kathleen M. Cronan revised by Emily Roumm Kane 198 Thoracentesis Manoj K. Mittal 199 Arthrocentesis Eron Y. Friedlaender and Adelaide E. Barnes Index

Section Editors Christopher P. Landrigan, MD, MPH [Part I] Research Director, Boston Children’s Hospital Inpatient Pediatrics Service Director, Sleep and Patient Safety Program Brigham and Women’s Hospital Associate Professor of Pediatrics and Medicine Harvard Medical School Boston Children’s Hospital Boston, Massachusetts

Amanda S. Growdon, MD [Part II] Assistant in Medicine, Boston Children’s Hospital Instructor, Harvard Medical School Boston, Massachusetts

Katherine A. O’Donnell, MD [ Part II] Attending, Hospital Medicine & Toxicology Boston Children’s Hospital Instructor in Pediatrics Harvard Medical School Boston, Massachusetts

Celeste R. Wilson, MD, FAAP [Section A] Assistant Professor of Pediatrics Harvard Medical School Medical Director, Child Protection Program Boston Children’s Hospital

Boston, Massachusetts

Carol A. Ford, MD, FSAHM [Section B] Past-President, Society for Adolescent Health and Medicine (SAHM) Professor of Pediatrics, University of Pennsylvania Chief, Division of Adolescent Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Manish J. Butte, MD, PhD [Section C] Department of Pediatrics Division of Immunology, Allergy, and Rheumatology Stanford University Stanford, California

Jordan Scott, MD [Section C] UMASS/Memorial and Children’s Hospital Boston Harvard Medical School Northeast Allergy, Asthma and Immunology Leominster, Massachusetts

Robert L. Geggel, MD [Section D] Associate Professor of Pediatrics Harvard Medical School Director, Pediatric Cardiology Consult Service Department of Cardiology Boston Children’s Hospital Boston, Massachusetts

Albert C. Yan, MD, FAAP, FAAD [Section E] Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Pediatric Dermatology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Andrew A. Palladino, MD [Section F] Assistant Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician and Medical Director of Inpatient Endocrinology Division of Endocrinology and Diabetes The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Marc H. Gorelick, MD, MSCE [Section G] Professor of Pediatrics (Affiliate), University of Minnesota President and Chief Operating Officer Children’s Hospital of Minnesota Minneapolis, Minnesota

Menno Verhave, MD [Section H] Clinical Director, Gastroenterology Boston Children’s Hospital Assistant Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Matthew A. Deardorff, MD, PhD [Section I] Associate Professor Perelman School of Medicine at the University of Pennsylvania Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

John D’Orazio, MD, PhD [Section J] Associate Professor in Pediatrics, Hematology-Oncology University of Kentucky College of Medicine Chandler Medical Center Lexington, Kentucky

Samir S. Shah, MD, MSCE [Section K] Director, Division of Hospital Medicine James M. Ewell Endowed Chair

Attending Physician in Hospital Medicine & Infectious Diseases Cincinnati Children’s Hospital Medical Center Professor, Department of Pediatrics University of Cincinnati College of Medicine Cincinnati, Ohio

Ricardo A Quinonez, MD, FAAP, FHM [Section L] Associate Professor of Pediatrics Section Head and Service Chief Pediatric Hospital Medicine Baylor College of Medicine Texas Children’s Hospital Houston, Texas

Dr. Daniel Licht, MD [Section M] Director, June and Steve Wolfson Laboratory for Clinical and Biomedical Optics The Children’s Hospital of Philadelphia Associate Professor of Neurology Perlman School of Medicine of the University of Pennsylvania Philadelphia, Pennsylvania

Neera Goyal, MD, MSc [Section N] Assistant Professor of Pediatrics Division of Neonatology and Division of Hospital Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Jennifer Kesselheim, MD [Section O] Assistant Professor of Pediatrics Dana-Farber/Boston Children’s Cancer and Blood Disorders Center Director, Master of Medical Sciences (MMSc) in Medical Education Harvard Medical School Boston, Massachusetts

Patricia I. Ibeziako [Section P]

Director, Psychiatry Consultation Service Boston Children’s Hospital Assistant Professor of Psychiatry Harvard Medical School Boston, Massachusetts

Aaron S. Chidekel, MD [Section Q] Associate Professor of Pediatrics Sidney Kimmel Medical College at Thomas Jefferson University Chief, Division of Pulmonology Nemours/Alfred I. duPont Hospital for Children Wilmington, Delaware

Melissa M. Hazen, MD [Section R] Assistant Professor of Pediatrics Harvard Medical School Program in Rheumatology and Department of General Pediatrics Boston Children’s Hospital Boston, Massachusetts

Robert Sundel, MD [Section R] Medical Director, Center for Ambulatory Treatment and Clinical Research Program Director, Rheumatology Boston Children’s Hospital Associate Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Erin Shaughnessy, MD, MSHCM [Section S] Division Chief, Pediatric Hospital Medicine Phoenix Children’s Hospital Phoenix, Arizona

Yaron Finkelstein, MD, FACMT [Section T] Divisions of Paediatric Emergency Medicine and Clinical Pharmacology and

Toxicology, Hospital for Sick Children Professor, Departments of Pediatrics and Pharmacology and Toxicology, University of Toronto Adjunct Scientist, Institute of Clinical Evaluative Sciences Toronto, Ontario, Canada

Eyal Cohen, MD, MSc, FRCPC [Section U] Division of Paediatric Medicine, Hospital for Sick Children Associate Professor, Department of Pediatrics, University of Toronto Toronto, Ontario, Canada

Rajendu Srivastava, MD, MPH [Section U] Assistant Vice President of Research Intermountain Healthcare Salt Lake City, Utah

James M. Callaghan, MD [Part IV] Division of Emergency Medicine Children’s Hospital of Philadelphia Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Contributors Amanda M. Ackermann, MD, PhD [68] Division of Endocrinology and Diabetes The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

David Adams, MD, PhD [87] Deputy Director of Clinical Genomics Office of the Clinical Director, NHGRI and Undiagnosed Diseases Program National Institutes of Health Bethesda, Maryland

Chhavi Agarwal, MD [71] Assistant Professor of Pediatrics Albert Einstein College of Medicine Department of Pediatrics Division of Pediatric Endocrinology and Diabetes The Children’s Hospital at Montefiore Bronx, New York

Rishi Agrawal, MD, MPH [179] Ann & Robert H. Lurie Children’s Hospital of Chicago Chicago, Illinois

Elizabeth R. Alpern, MD, MSCE [95,96] Professor of Pediatrics

Division of Emergency Medicine Department of Pediatrics Ann & Robert H. Lurie Children’s Hospital of Chicago Northwestern University, Feinberg School of Medicine Chicago, Illinois

Elizabeth Ang, MBBS, MRCPCH, MMed [147,148] Rheumatology Fellow Boston Children’s Hospital Boston, Massachusetts

Armand H. Matheny Antommaria, MD, PhD [183] Director, Ethics Center Lee Ault Carter Chair of Pediatric Ethics Associate Professor Division of Hospital Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Adelaide E. Barnes, MD [199] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Lindsay S. Baron, MD [185] Commonwealth Radiology Associates Director of Pediatric Imaging and Ultrasound Lowell General Hospital Lowell, Massachusetts

Benjamin Bauer, MD [100] Riley Hospital for Children at Indiana University Health Indianapolis, Indiana

Carl R. Baum, MD, FAAP, FACMT [169]

Professor of Pediatrics and of Emergency Medicine Yale University School of Medicine New Haven, Connecticut

Jonathan Frederick Bean, MD [126] Surgical Research Fellow Department of Pediatric Surgery Ann & Robert H. Lurie Children’s Hospital of Chicago Resident Physician Department of Surgery University of Illinois Hospital and Health Sciences System Chicago, Illinois

Anne K. Beasley, MD [38] Pediatric Hospitalist Phoenix Children’s Hospital Phoenix, Arizona

Ebony Beaudoin, MD [127] Assistant Professor of Pediatrics Houston, Texas

Heather J. Becker, MD [169] Clinical Fellow Pediatric Emergency Medicine Yale-New Haven Children’s Hospital New Haven, Connecticut

Suzanne Beno, MD [191] Assistant Professor, Department of Paediatrics School of Medicine, University of Toronto Fellowship Director, Pediatric Emergency Medicine Division of Emergency Medicine The Hospital for Sick Children (SickKids) Toronto, Ontario, Canada

Emily M. Berger, MD [66] Department of Dermatology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Lauren A. Beslow, MD, MSCE, FAHA [121] Assistant Professor of Neurology and Pediatrics Perelman School of Medicine at the University of Pennsylvania The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Laura M. Bevilacqua, MD [52] Department of Cardiology Boston Children’s Hospital Department of Pediatrics Harvard Medical School Boston, Massachusetts

Lori Billinghurst, MD, MSc, FRCPC [121] Clinical Assistant Professor of Neurology Perelman School of Medicine at The University of Pennsylvania Attending Physician, Division of Neurology Neurology Director, Neonatal Neurocritical Care Program The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Ari Bitnun, MD, MSc, FRCPC [108] Associate Professor, Department of Pediatrics, University of Toronto Staff physician, Division of Infectious Diseases, Hospital for Sick Children Toronto, Ontario, Canada

Benjamin S. Bolser, MD [67] Attending Physician, Division of Hospital Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Christopher P. Bonafide, MD, MSCE [6] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician, Division of General Pediatrics The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Fabienne C. Bourgeois, MD, MPH [19] Pediatric Hospitalist Children’s Hospital Inpatient Service Boston Children’s Hospital Instructor in Pediatrics Harvard Medical School Boston, Massachusetts

Jeffrey Brent, MD, PhD [171] Distinguished Clinical Professor of Medicine Clinical Professor of Pediatrics University of Colorado School of Medicine Denver, Colorado

Marisa B. Brett-Fleegler, MD [162] Attending Physician Division of Emergency Medicine Boston Children’s Hospital Assistant Professor Division of Emergency Medicine Harvard Medical School Boston, Massachusetts

Corinne L. Bria, MD, MEd [97] Assistant Professor of Pediatrics University of Cincinnati College of Medicine Attending Physician Division of Pediatric Emergency Medicine Cincinnati Children’s Hospital Medical Center

Cincinnati, Ohio

April Buchanan, MD, FAAP [25] Assistant Dean for Academic Affairs and Clinical Clerkship Education Associate Professor of Pediatrics University of South Carolina School of Medicine Greenville Pediatric Hospitalist Children’s Hospital of the Greenville Health System Greenville, South Carolina

Michele M. Burns, MD, MPH [166,172] Fellowship Director: Harvard Medical Toxicology Medical Director: Regional Center for Poison Control & Prevention serving MA & RI Staff Physician: Emergency Medicine Boston Children’s Hospital Boston, Massachusetts

Manish J. Butte, MD, PhD [49,50] Associate Professor and Division Chief Division of Immunology, Allergy, and Rheumatology, Dept. of Pediatrics Director, Jeffrey Modell Diagnostic and Research Center University of California, Los Angeles

Julie Story Byerley, MD, MPH [154] Professor General Pediatrics and Adolescent Medicine Vice Dean for Education, School of Medicine Vice Chair for Education, Department of Pediatrics University of North Carolina at Chapel Hill

Deirdre A.L. Caplin, MS, PhD [9] Associate Professor Division of Pediatric Behavioral Health Department of Pediatrics University of Utah School of Medicine

Intermountain Primary Children’s Medical Center Salt Lake City, Utah

Rebecca G. Carlisle [175] Assistant Professor of Pediatrics George Washington University College of Medicine Children’s National Medical Center Washington, DC

Leslie Castelo-Soccio, MD, PhD [64] Assistant Professor of Pediatrics and Dermatology, Perlman School of Medicine at the University of Pennsylvania and Attending Physician, Section of Pediatric Dermatology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Mary Wu Chang, MD [62] Associate Clinical Professor of Dermatology and Pediatrics University of Connecticut School of Medicine West Hartford, Connecticut

Lindsay Chase, MD [110,111] Associate Professor Director of the Section of Hospital Pediatrics Division of General Pediatrics and Adolescent Medicine University of North Carolina and NC Children’s Hospital Chapel Hill, North Carolina

Aaron S. Chidekel, MD [140,145] Associate Professor of Pediatrics Sidney Kimmel Medical College at Thomas Jefferson University Chief, Division of Pulmonology Nemours/Alfred I. duPont Hospital for Children Wilmington, Delaware

Denesh K. Chitkara, MD [77]

Pediatric Gastroenterology and Nutrition Goryeb Children’s Hospital Morristown, New Jersey

Christine S. Cho, MD, MPH, MEd [176,188,194] Associate Professor of Pediatrics USC Keck School of Medicine Fellowship and Education Director Division of Emergency Medicine Children’s Hospital Los Angeles Los Angeles, California

Jeanne S. Chow, MD [185] Associate Professor Harvard Medical School Director of Fluoroscopy and Uroradiology Department of Radiology Boston Children’s Hospital Boston, Massachusetts

Erica Chung, MD [27] Assistant Professor of Pediatrics The Warren Alpert Medical School of Brown University Pediatric Hospitalist Hasbro Children’s Hospital Providence, Rhode Island

Bartley G. Cilento, Jr. [164] Assistant Professor of Surgery (Urology) Harvard Medical School Department of Urology Boston Children’s Hospital Boston, Massachusetts

Susan E. Coffin, MD, MPH [5] Professor of Pediatrics UPENN School of Medicine

Associate Chief, Division of Infectious Diseases The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Daniel T. Coghlin, MD [74] Associate Professor of Pediatrics, Clinician Educator The Warren Alpert Medical School of Brown University Pediatric Hospitalist Hasbro Children’s Hospital Providence, Rhode Island

Bernard A. Cohen, MD [58] Professor of Dermatology and Pediatrics Johns Hopkins School of Medicine Director of Pediatric Dermatology Johns Hopkins Children’s Center Baltimore, Maryland

Kathleen M. Cronan, MD [197] Nemours/Alfred I. duPont Hospital for Children Wilmington, Delaware

Alison J. Culyba, MD, MPH [46] Adolescent Medicine Fellow Craig Dalsimer Division of Adolescent Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Lara A. Danziger-Isakov, MD, MPH [109] Professor, Department of Pediatrics University of Cincinnati College of Medicine Director, Immunocompromised Host Infectious Diseases Division of Infectious Diseases Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Sigrid Payne DaVeiga, MD [141] Attending Physician Division of Allergy and Immunology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Ian J. Davis, MD, PhD [88] G. Denman Hammond Associate Professor, Division of Hematology/Oncology Department of Pediatrics Department of Genetics Lineberger Comprehensive Cancer Center University of North Carolina at Chapel Hill Chapel Hill, North Carolina

J. Kate Deanehan, MD, RDMS [186] Assistant Professor, Pediatric Emergency Medicine Director of Emergency Ultrasound Associate PEM Fellowship Director Johns Hopkins Children’s Center Baltimore, Maryland

Barbara Degar, MD [132] Associate Director of Inpatient Oncology Boston Children’s Hospital Assistant Professor in Pediatrics Harvard Medical School Boston, Massachusetts

Cynthia M.C. DeKlotz, MD [63] Pediatric and Adult Dermatologist MedStar Washington Hospital Center/Georgetown University Hospital Assistant Professor of Clinical Medicine and Pediatrics Georgetown University School of Medicine Washington, DC

Michael Del Vecchio, MD [177] Professor, Clinical Pediatrics Department of Pediatrics Lewis Katz School of Medicine at Temple University Philadelphia, Pennsylvania

Sarah F. Denniston, MD [159] Assistant Clinical Professor of Pediatrics Department of Pediatrics Division of Hospital Medicine University of Washington School of Medicine Attending Physician Seattle Children’s Hospital Seattle, Washington

Bimal R. Desai, MD, MBI, FAAP [6] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Chief Health Informatics Officer The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Craig C. DeWolfe, MD, MEd [140] Director of Pediatric Medical Student Education Assistant Professor of Pediatrics George Washington University School of Medicine Pediatric Hospitalist Children’s National Health System Washington, DC

Kenneth J. Dooley, MD [55] Children’s Healthcare of Atlanta Associate Professor of Pediatrics Emory University School of Medicine Atlanta, Georgia

John D’Orazio, MD, PhD [90] Professor in Pediatrics, Hematology-Oncology University of Kentucky College of Medicine Markey Cancer Center Lexington, Kentucky

Kevin J. Downes, MD [109] Clinical Fellow Division of Infectious Diseases Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Nadia Dowshen, MD, MSHP [45] Director of Adolescent HIV Services Craig-Dalsimer Division of Adolescent Medicine Faculty, PolicyLab The Children’s Hospital of Philadelphia Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Christine N. Duncan, MD [134] Dana-Farber/Boston Children’s Caner and Blood Disorders Center Stem Cell Transplant Center Boston, Massachusetts

Marie E. Egan, MD [144] Director, Yale Cystic Fibrosis Center Professor, Departments of Pediatrics and Cellular and Molecular Physiology Yale University New Haven, Connecticut

Lawrence F. Eichenfield, MD [63] Professor of Dermatology & Pediatrics Vice Chair, Department of Dermatology Chief, Pediatric and Adolescent Dermatology

Rady Children’s Hospital, San Diego University of California, San Diego School of Medicine San Diego, California

Mfon Ekong, MD [124] Assistant Professor of Pediatrics Division of Community & General Pediatrics University of Texas Health Science Center - Houston Houston, Texas

Scott A. Elisofon, MD [79] Attending Physician Division of Pediatric Gastroenterology Boston Children’s Hospital Assistant Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Stephen C. Eppes, MD [105] Director, Pediatric Infectious Diseases Christiana Care Health System Professor of Pediatrics Department of Pediatrics Jefferson Medical College Newark, Delaware

Mirna M’farrej, MD [190] Associate Professor Department of Pediatrics University of Pennsylvania School of Medicine Attending Physician Division of Emergency Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Diana Felton, MD [166]

Toxicology Fellow Harvard Medical Toxicology Fellowship Boston Children’s Hospital Boston, Massachusetts

Christine T. Ferrara, MD, PhD [68] Clinical Fellow in Pediatric Endocrinology and Diabetes Division of Endocrinology and Diabetes The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Chris Feudtner, MD, PhD, MPH [10,12] Professor of Pediatrics, Medical Ethics and Health Policy Perelman School of Medicine, University of Pennsylvania Attending Physician and Director of Research for the Pediatric Advanced Care Team Steven D Handler Endowed Chair of Medical Ethics The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Erin Stucky Fisher, MD, MHM, FAAP [8] Professor of Clinical Pediatrics and Pediatric Hospital Medicine Fellowship Director Department of Pediatrics University of California, San Diego Medical Director for Quality Management Pediatric Hospitalist Rady Children’s Hospital and Health Center San Diego San Diego, California

Andrew M. Fine, MD, MPH [36] Assistant Professor of Pediatrics and Emergency Medicine Harvard Medical School Associate in Medicine Emergency Medicine Boston Children’s Hospital

Boston, Massachusetts

Yaron Finkelstein, MD, FACMT [167] Divisions of Paediatric Emergency Medicine and Clinical Pharmacology and Toxicology, Hospital for Sick Children Professor, Departments of Pediatrics and Pharmacology and Toxicology, University of Toronto Adjunct Scientist, Institute of Clinical Evaluative Sciences Toronto, Ontario, Canada

Amy Fleming, MD, MHPE [22] Associate Dean for Medical Student Affairs Associate Professor, Department of Pediatrics Monroe Carell Jr. Children’s Hospital at Vanderbilt Vanderbilt University School of Medicine Nashville, Tennessee

Todd A. Florin, MD, MSCE [101] Assistant Professor of Pediatrics University of Cincinnati College of Medicine Director, Research Operations Division of Pediatric Emergency Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Jessica Fowler, MD, MPH [45] Chief Resident Pediatric Residency Program The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Susan H. Frangiskakis, MD [34] Pediatrician Wausau, Wisconsin

Anne Marie Frey, BSN, RN, CRNI, VA-BC [191]

Clinical Expert Vascular Access Service: I.V. Team The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Eron Y. Friedlaender, MD, MPH [192,199] Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Emergency Medicine Attending Division of Emergency Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Jeremy Friedman, MB, ChB, FRCPC [182] Associate Paediatrician-in-Chief, Hospital for Sick Children Professor of Paediatrics, University of Toronto Toronto, Ontario, Canada

David R. Fulton, MD [57] Distinguished Tommy Kaplan Chair in Cardiovascular Sciences Chief, Cardiology Outpatient Services, Emeritus Department of Cardiology Boston Children’s Hospital Associate Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Katherine A.S. Gallagher, PhD [135] Assistant Professor of Pediatrics Baylor College of Medicine Psychology Section Texas Children’s Hospital Houston, Texas

Beth D. Gamulka, MDCM, FRCPC [83] Staff Pediatrician

Department of Pediatrics The Scarborough Hospital Division of Pediatric Medicine The Hospital for Sick Children (SickKids) Adjunct Clinical Assistant Professor, Department of Pediatrics University of Toronto Faculty of Medicine Toronto, Ontario, Canada

Jorge F. Ganem, MD, FAAP [114] Assistant Professor Department of Pediatrics University of Texas at Austin Dell Medical School Director Hospital Medicine Dell Children’s Medical Center of Central Texas Austin, Texas

Rebecca D. Ganetzky, MD [86] Assistant Professor of Pediatrics University of Pennsylvania School of Medicine Division of Human Genetics and Metabolic Diseases Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Matthew Garber, MD, FAAP, FHM [186] Professor of Pediatrics, University of Florida COM Division Chief, Hospital Pediatrics UF Hospitalist, Wolfson Children’s Hospital Medical Director, Value in Inpatient Pediatrics Network Jacksonville, Florida

Georgina Garcia, MD [139] Children’s Hospital Boston Department of Psychiatry Cambridge Eating Disorder Center Director of Adolescent Services Cambridge, Massachusetts

Andrea S. Garrod, MD [146] Assistant Professor of Pediatrics University of Virginia Health System Charlottesville, Virginia

Robert L. Geggel, MD [51] Senior Associate in Cardiology and Director of Cardiology Consult Service Boston Children’s Hospital Associate Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Jeffrey S. Gerber, MD, PhD [94] Assistant Professor of Pediatrics and Epidemiology Perelman School of Medicine at the University of Pennsylvania Medical Director, Antimicrobial Stewardship Program Associate Director, Center for Pediatric Clinical Effectiveness Division of Infectious Diseases The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Michael H. Gewitz, MD [54] Physician-in-Chief Director, Pediatric Cardiology Maria Fareri Children’s Hospital Professor and Vice Chairman Department of Pediatrics New York Medical College Valhalla, New York

Timothy Gibson, MD [17,187,196] Clinical Associate Professor of Pediatrics University of Massachusetts Medical School Worcester, Massachusetts

Laurie Glader, MD [180,181]

Associate in Medicine Boston Children’s Hospital Assistant Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Jaya Goswami, MD [103] Pediatric Hospitalist Division of Hospitalist Medicine St. Louis Children’s Hospital St. Louis, Missouri

Neera Goyal, MD, MSc [128] Assistant Professor of Pediatrics Division of Neonatology and Division of Hospital Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Frederick Grant, MD [185] Assistant Professor in Radiology Harvard Medical School Division of Nuclear Medicine and Molecular Imaging Department of Radiology Boston Children’s Hospital Boston Massachusetts

Anna L. Grossberg, MD [58] Assistant Professor of Dermatology and Pediatrics Johns Hopkins School of Medicine Baltimore, Maryland

Amit S. Grover, MB BCh BAO [82] Director, Boston Children’s Hospital Program for Pancreatic Disease Attending Physician Division of Gastroenterology, Hepatology and Nutrition, Boston Children’s Hospital Instructor in Pediatrics

Harvard Medical School Boston, Massachusetts

Amanda S. Growdon, MD [31] Assistant in Medicine, Boston Children’s Hospital Instructor, Harvard Medical School Boston, Massachusetts

Paul Hain, MD [4] Vanderbilt University School of Medicine Residency, Monroe Carell Jr. Children’s Hospital at Vanderbilt Nashville, Tennessee

David E. Hall, MD [180,181] Professor of Clinical Pediatrics Director, Program for Children with Medically Complex Needs Vanderbilt University School of Medicine Monroe Carrel, Jr. Children’s Hospital at Vanderbilt Nashville, Tennessee

Catherine K. Hart, MD [158] Assistant Professor Pediatric Otolaryngology Head & Neck Surgery Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Jonathan S. Hausmann, MD [152] Program in Rheumatology Boston Children’s Hospital Division of Rheumatology Beth Israel Deaconess Medical Center Instructor in Medicine Harvard Medical School Boston, Massachusetts

Natalie Hayes, DO [145]

Department of Pediatrics Division of Pulmonology Wake Forest Baptist Health Brenner Children’s Hospital Winston-Salem, North Carolina

Melissa M. Hazen, MD [151,152] Assistant Professor of Pediatrics Harvard Medical School Program in Rheumatology and Department of General Pediatrics Boston Children’s Hospital Boston, Massachusetts

Matthew M. Heeney, MD [89] Assistant Professor of Pediatrics Harvard Medical School Associate Chief, Hematology Director, Sickle Cell Program Boston, Massachusetts

Joseph D. Hernandez, MD, PhD [50] Clinical Assistant Professor Department of Pediatrics (Immunology, Allergy and Rheumatology) Stanford University, Lucile Packard Children’s Hospital Stanford, California

Malinda Ann Hill, MA [10] Bereavement Coordinator The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Vanessa L. Hill, MD [116,117] Associate Professor Department of Pediatrics Division of Pediatric Hospital Medicine

Baylor College of Medicine Children’s Hospital of San Antonio San Antonio, Texas

Alejandro Hoberman, MD [104] Chief, Division of General Academic Pediatrics Professor of Pediatrics and Clinical and Translational Science Jack L. Paradise Professor of Pediatric Research Vice Chair for Clinical Research Children’s Hospital of Pittsburgh Pittsburgh, Pennsylvania

Sarah Hoehn, MD, MBE [12] Associate Professor of Pediatrics University of Kansas School of Medicine Kansas City, Kansas

Robert J. Hoffman, MS, MD [170] Director, Clinical Toxicology Department of Emergency Medicine Sidra Medical & Research Center Doha, Qatar

Paul J. Honig, MD [61] Professor Emeritus, Pediatrics and Dermatology Children’s Hospital of Philadelphia Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Mark D. Hormann, MD [125] Associate Professor of Pediatrics McGovern Medical School at The University of Texas Health Science Center at Houston Medical Director, Newborn Nursery Children’s Memorial Hermann Hospital Houston, Texas

B. David Horn, MD [161] Associate Professor of Clinical Orthopedic Surgery Perelman School of Medicine at the University of Pennsylvania The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Jonathan D. Hron, MD [21] Pediatric Hospitalist, Division of General Pediatrics Boston Children’s Hospital Instructor of Pediatrics Harvard Medical School Boston, Massachusetts

Jennifer Hwang, MD, MHS [10] Fellowship Director, Pediatric Advance Care Team The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Michael Isakoff, MD [132] Associate Professor of Pediatrics University of Connecticut School of Medicine Clinical Director, Division of Hematology and Oncology Director, Sarcoma and Adolescent and Young Adult Cancer Program Connecticut Children’s Medical Center Hartford, Connecticut

Patricia I. Ibeziako, MD [136,137] Director, Psychiatry Consultation Service Boston Children’s Hospital Assistant Professor of Psychiatry Harvard Medical School Boston, Massachusetts

Karen E. Jerardi, MD, MEd [99] Assistant Professor, Department of Pediatrics University of Cincinnati College of Medicine

Associate Director, Division of Hospital Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Reena Jethva, MD [65] Hackensack University Medical Center Hackensack, New Jersey

Rahul Kaila, MD [168] Assistant Professor Department of Pediatrics University of Minnesota Masonic Children’s Hospital Minneapolis, Minnesota

Jennifer M. Kalish, MD, PhD [84] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician, Genetics Division The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

David A. Kane, MD [20] Assistant Professor of Pediatrics Division of Pediatric Cardiology University of Massachusetts Medical School Worcester, Massachusetts

Tammy Kang [10] Chief, Section of Palliative Care at Texas Children’s Hospital Associate Professor of Pediatrics, Baylor College of Medicine Houston, Texas

Emily Roumm Kane, MD, MS [197] Division of General Pediatrics The Children’s Hospital of Philadelphia

Philadelphia, Pennsylvania

Garrett Keim, MD [195] Fellow, Pediatric Critical Care Department of Critical Care and Anesthesia The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Lisa Kenigsberg, MD [71] Albert Einstein College of Medicine Department of Pediatrics Division of Pediatric Endocrinology and Diabetes The Children’s Hospital at Montefiore Bronx, New York

Chén Kenyon, MD, MSHP [18] Assistant Professor of Pediatrics Perelman School of Medicine, University of Pennsylvania Pediatric Hospitalist The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Ron Keren, MD, MPH [2] Professor of Pediatrics and Epidemiology Perelman School of Medicine at the University of Pennsylvania Gerald D. Quill Distinguished Chair in the Department of Pediatrics Vice President of Quality and Chief Quality Officer The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Anupam Kharbanda, MD, MSc [157] Chief, Critical Care Services Children’s Hospital Minnesota Minneapolis, Minnesota

Juliann Lipps Kim, MD [35]

Department Lead, Pediatric Hospital Medicine Palo Alto Medical Foundation Adjunct Assistant Clinical Professor of Pediatrics Stanford University Palo Alto, California

Susan Kim, MD, MMSc [149] Associate Clinical Professor Pediatric Rheumatology UCSF Benioff Children’s Hospital University of California San Francisco, California

Robert L. Kitts, MD [136] Assistant Professor of Psychiatry, Harvard Medical School Attending Physician Department of Psychiatry, Massachusetts General Hospital Boston, Massachusetts

Paul K. Kleinman, MD, FAAP [41] Professor of Radiology Harvard Medical School Director of Musculoskeletal Imaging Boston Children’s Hospital Boston, Massachusetts

Joel B. Korin [13] Attending Physician Division of Emergency Medicine Nemours/Alfred I. duPont Hospital for Children Wilmington, Delaware

Karen Kotloff [103] Head, Division of Infectious Disease and Tropical Pediatrics

Professor of Pediatrics and Medicine Center for Vaccine Development Institute of Global Health University of Maryland School of Medicine Baltimore, Maryland

Subra Kugathasan, MD [80] Professor of Pediatrics & Human Genetics Marcus Professor of Pediatric Gastroenterology Emory University School of Medicine Atlanta, Georgia

Amethyst C. Kurbegov, MD, MPH [75] Assistant Professor of Pediatric Gastroenterology University of Colorado School of Medicine Colorado Springs, Colorado

Oren L. Koslowe, MD [77] Pediatric Gastroenterology and Nutrition Goryeb Children’s Hospital Morristown, New Jersey

Christopher P. Landrigan, MD, MPH [1] Research Director, Boston Children’s Hospital Inpatient Pediatrics Service Director, Sleep and Patient Safety Program Brigham and Women’s Hospital Associate Professor of Pediatrics and Medicine Harvard Medical School Boston Children’s Hospital Boston, Massachusetts

Christine Lauren, MD [61] Assistant Professor of Dermatology and Pediatrics Columbia University Medical Center New York, New York

Michael Levine, MD [178] Section of Medical Toxicology Department of Emergency Medicine University of Southern California Los Angeles, California

Phyllis Lewis [175] Assistant Professor of Pediatrics George Washington University College of Medicine Children’s National Medical Center Washington, DC

Marilyn G. Liang, MD [60] Associate Professor of Dermatology Boston Children’s Hospital Harvard Medical School Boston, Massachusetts

Daniel J. Licht, MD [10] Dir. June and Steve Wolfson Laboratory for Clinical and Biomedical Optics Associate Professor of Neurology The Children’s Hospital of Philadelphia and The University of Pennsylvania Philadelphia, Pennsylvania

Carrie B. Lind [22] Pediatric Hospitalist Division of Hospital Medicine Monroe Carell Jr. Children’s Hospital at Vanderbilt Assistant Professor of Clinical Pediatrics Vanderbilt University School of Medicine Nashville, Tennessee

Matthew T. Lister, MD [33] Division of Otolaryngology-Head and Neck Surgery UW Health Hospitals and Clinics

Clinical Associate Professor of Otolaryngology University of Wisconsin School of Medicine and Public Health Milwaukee, Wisconsin

Mindy S. Lo, MD, PhD [153] Division of Immunology Boston Children’s Hospital Instructor in Pediatrics Harvard Medical Schoo Boston, Massachusetts

Michelle A. Lopez, MD [112] Assistant Professor of Pediatrics Section of Pediatric Hospital Medicine Texas Children’s Hospital Houston, Texas

Katherine Lord, MD [69] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician Division of Endocrinology and Diabetes The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

M. Katherine Loudermilk, MD [130] Medical Director, Mother Baby Unit Good Samaritan Hospital Perinatal Institute Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Jeffrey P. Louie, MD [168,173] Assistant Professor Department of Pediatrics University of Minnesota

Masonic Children’s Hospital Minneapolis, Minnesota

Barry A. Love, MD [53] Director Congenital Cardiac Catheterization Laboratory Kravis Children’s Hospital at Mount Sinai Assistant Professor of Pediatrics and Medicine Icahn School of Medicine at Mount Sinai New York, New York

Ian MacLusky, MBBS, FRCP [143] Chief Division of Respirology Children’s Hospital of Eastern Ontario Associate Professor, University of Ottawa Ottawa, Ontario Canada

Mary Beth Madonna, MD [126] Attending Physician Department of Pediatric Surgery Ann & Robert H. Lurie Children’s Hospital of Chicago Assistant Professor of Surgery Feinberg School of Medicine Northwestern University Chicago, Illinois

Douglas Y. Mah, MD [52] Assistant Professor of Pediatrics Harvard Medical School Department of Cardiology Boston Children’s Hospital Boston, Massachusetts

Paul E. Manicone, MD [118] Pediatric Hospitalist Children’s National Health System Washington, DC

Jennifer Maniscalco, MD, MPH, FAAP [81] Associate Professor of Clinical Pediatrics University of Southern California Keck School of Medicine Director, Pediatric Hospital Medicine Fellowship Children’s Hospital Los Angeles Los Angeles, California

Jonathan M. Mansbach, MD [3] Clinical Research Director, Boston Children’s Hospital Inpatient Pediatrics Service Assistant Professor of Pediatrics Harvard Medical School Boston Children’s Hospital Boston, Massachusetts

Lauren E. Marlowe, MD [192] Pediatric Hospitalist, CHOP at Virtua Clinical Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Voorhees, New Jersey

Kimberly C. Martin, DO, MPH [98] Assistant Professor of Pediatrics Division of Pediatric Infectious Diseases University of Oklahoma School of Community Medicine Tulsa, Oklahoma

Peter Mattei, MD [156] General, Thoracic and Fetal Surgery Children’s Hospital of Philadelphia Associate Professor, Dept. of Surgery Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Sangeeta Mauskar, MD, MPH [180,181]

Director, Complex Care Service, Inpatient Program Boston Children’s Hospital Instructor, Harvard Medical School Boston, Massachusetts

Oscar H. Mayer, MD [10] Associate Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Pulmonologist Director, Pulmonary Function Laboratory Division of Pulmonary Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Pamela A. Mazzeo, MD [195] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician Division of General Pediatrics The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Sarah C. McBride, MD [37] Boston Children’s Hospital Department of Pediatrics Harvard Medical School Boston, Massachusetts

Zachary McClain, MD [44] Assistant Professor of Pediatrics Craig Dalsimer Division of Adolescent Medicine The Children’s Hospital of Philadelphia Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Russell McCulloh, MD [107]

Divisions of Infectious Diseases and Hospital Medicine Children’s Mercy Kansas City Assistant Professor of Pediatrics and Internal Medicine UMKC School of Medicine University of Kansas Medical Center Kansas City, Missouri

Lisa McLeod, MD, MSCE [2] Director, Pediatric Surgical Outcomes Research Center (PSORC), Center for Children’s Surgery Assistant Professor, Pediatric Hospital Medicine, Children’s Hospital Colorado/University of Colorado School of Medicine Aurora, Colorado

Suzanne Swanson Mendez, MD [32] Pediatric Hospitalist Santa Clara Valley Medical Center San Jose, California Clinical Instructor in Pediatrics Stanford University Palo Alto, California

Talene A. Metjian, PharmD [94] Antimicrobial Stewardship Program Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Manoj K. Mittal, MD, MRCP (UK), FAAP [198] Associate Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician Division of Emergency Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Vineeta Mittal, MD [7]

Associate Professor of Pediatrics Division of Hospital Medicine Department of Pediatrics UTSW Medical Center & Childrens Health System of Texas Dallas, Texas

Keith W. Morley, MD [106] Pediatric Preliminary Resident Geisinger Medical Center Danville, Pennsylvania

Martin J. Morrison III, MD [161] Department of Orthopaedic Surgery Loma Linda University School of Medicine Loma Linda Medical Center Loma Linda, California

Christelle Moufawad El Achkar, MD [118,119] Assistant in Neurology Boston Children’s Hospital Instructor of Neurology Harvard Medical School Boston, Massachusetts

Nancy Murphy, MD, FAAP, FAAPMR [179] Professor and Chief, Division Pediatric PM&R University of Utah Department of Pediatrics Salt Lake City, Utah

Frances M. Nadel, MD, MSCE [174,191] Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician Division of Emergency Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Joshua Nagler, MD, MPHEd [163] Division of Emergency Medicine Boston Children’s Hospital Assistant Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Sona Narula, MD [122] Assistant Professor of Clinical Neurology Division of Neurology The Children’s Hospital of Philadelphia Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Mark I. Neuman, MD, MPH [142] Division of Emergency Medicine Boston Children’s Hospital Associate Professor of Pediatrics and Emergency Medicine Harvard Medical School Boston, Massachusetts

Alice W. Newton, MD, FAAP [40] Medical Director, Child Protection Program Department of Pediatrics Massachusetts General Hospital Boston, Massachusetts

Alison E. Niebanck, MD [160] Assistant Professor of Pediatrics Mercer University School of Medicine, Savannah Willett Children’s Hospital at Memorial Health University Medical Center Savannah, Georgia

Lise E. Nigrovic [172] Associate Professor of Pediatrics and Emergency Medicine

Harvard Medical School Division of Emergency Medicine Boston Children’s Hospital Boston, Massachusetts

Richard J. Noel, MD, PhD [78] Associate Professor of Pediatrics Medical College of Wisconsin Milwaukee, Wisconsin

Kevin C. Osterhoudt, MD, MS [165,176] Attending Physician, Division of Emergency Medicine Medical Director, The Poison Control Center The Children’s Hospital of Philadelphia Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Mary C. Ottolini, MD, MPH, MEd [15] Professor of Pediatrics George Washington University Vice Chair for Medical Education Children’s National Medical Center Washington DC

Raj Padman, MD [195] Medical Director Pulmonology, Asthma and Sleep Disorders Lutheran Children’s Hospital Fort Wayne, Indiana

Horacio Padua, MD [185] Assistant Professor Harvard Medical School Attending in Pediatric Interventional Radiology Department of Radiology

Boston Children’s Hospital Boston, Massachusetts

Andrew A. Palladino, MD [68,69,72] Assistant Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician and Medical Director of Inpatient Endocrinology Division of Endocrinology and Diabetes The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Michelle W. Parker, MD [76] Cincinnati Children’s Hospital Medical Center Assistant Professor, Department of Pediatrics University of Cincinnati College of Medicine Cincinnati, Ohio

Sarah E.S. Parker, MD [24] Assistant Professor, Pediatrics Department of Pediatrics Division of General Pediatrics Monroe Carell Jr. Children’s Hospital at Vanderbilt Nashville, Tennessee

Vanda Passi, MD [195] Department of Pediatrics Division of Pediatric Pulmonology Nemours/Alfred I. DuPont Hospital for Children Wilmington, Delaware

Raina Paul, MD [36] Assistant Professor, Department of Pediatrics Division of Emergency Medicine Associate Medical Director, Center for Excellence, Ann and Robert H. Lurie Children’s Hospital of Chicago Feinberg School of Medicine at Northwestern University

Chicago, Illinois

Rebecka Peebles, MD [44] Co-Director of Eating Disorder Assessment and Treatment Program Craig-Dalsimer Division of Adolescent Medicine The Children’s Hospital of Philadelphia Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Jeremy Ryan Andrew Peña, MD, PhD [93] Director, Histocompatibility Laboratory Division of Laboratory and Transfusion Medicine Beth Israel Deaconess Medical Center Boston, Massachusetts

William H. Peranteau, MD [156] Attending Surgeon Division of Pediatric General, Thoracic and Fetal Surgery The Children’s Hospital of Philadelphia Assistant Professor of Surgery Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Jeannette M. Pérez-Rosselló, MD [41] Pediatric Radiologist Boston Children’s Hospital Assistant Professor of Radiology Harvard Medical School Boston, Massachusetts

Marissa J. Perman, MD [66] Assistant Professor of Pediatrics Department of Dermatology and Pediatrics Perelman School of Medicine at the University of Pennsylvania and The Children’s Hospital of Philadelphia

Philadelphia, Pennsylvania

Andrew E. Place, MD, PhD [133] Assistant Professor of Pediatrics Harvard Medical School Dana-Farber/Boston Children’s Cancer and Blood Disorders Center Boston, Massachusetts

Annapurna Poduri, MD, MPH [118,119] Associate Professor in Neurology Epilepsy Genetics Program Division of Epilepsy and Clinical Neurophysiology Boston Children’s Hospital Boston, Massachusetts

Jill C. Posner, MD, MSCE, MSEd [188,194] Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Director of Medical Education, Division of Emergency Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Sampath Prahalad, MD, MSc [150] Marcus Professor of Pediatric Rheumatology Chief, Division of Pediatric Rheumatology Professor of Pediatrics and Human Genetics Emory University School of Medicine Children’s Healthcare of Atlanta Atlanta, Georgia

Julianne Prasto, MD [193] The Children’s Hospital of Philadelphia University Medical Center of Princeton – Plainsboro The Children’s Hospital of Philadelphia Plainsboro, New Jersey

Genevieve Preer, MD [42] Pediatrician, Child Protection Team Boston Medical Center Assistant Professor of Pediatrics Boston University School of Medicine Boston, Massachusetts

Howard B. Pride, FAAD, MD [106] Pediatric Dermatology Geisinger Medical Center Danville, Pennsylvania

Vlad Calin Radulescu, MD [92] Assistant Professor University of Kentucky Lexington, Kentucky

Lindsay Burns Ragsdale, MD [10] Co-Director, Pediatric Advanced Care Team Associate Program Director, Pediatric Residency Program Kentucky Children’s Hospital Assistant Professor of Pediatrics University of Kentucky College of Medicine Lexington, Kentucky

Daniel A. Rauch, MD, FAAP, FHM [11,14,16] Visiting Associate Professor of Pediatrics Tufts University School of Medicine Chief of Pediatric Hospital Medicine at the Floating Hospital for Children at Tufts Medical Center Boston, Massachusetts

Robin Ray, MD [160] Vitreoretinal Service Georgia Eye Institute of the Southeast Savannah, Georgia

Rupa Redding-Lallinger, MD [88] Professor of Pediatrics and Internal Medicine Co-Director, UNC Comprehensive Sickle Cell Program Division of Pediatric Hematology and Oncology University of North Carolina Chapel Hill, North Carolina

Kris P. Rehm, MD [24] Associate Professor, Pediatrics Director, Division of Hospital Medicine Vanderbilt University School of Medicine Monroe Carell Jr Children’s Hospital at Vanderbilt Nashville, Tennessee

Daniel H. Reirden [44] Associate Professor of Medicine and Pediatrics Sections of Adolescent Medicine and Infectious Disease Aurora, Colorado

David Riester, MD [47,48] Northeast Allergy, Asthma and Immunology Leominster, Massachusetts

Jeffrey D. Roizen, MD, PhD [72] Assistant Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

José R. Romero, MD, FAAP, FDISA, FPIDS [98] Director, Section of Infectious Diseases Department of Pediatrics Horace C. Cabe Chair in Infectious Diseases University of Arkansas for Medical Sciences and Arkansas Children’s Hospital Little Rock, Arkansas

Colleen A. Ryan, MD [138] Medical Director, Inpatient Psychiatry Service Boston Children’s Hospital Instructor in Psychiatry Harvard Medical School Boston, Massachusetts

Julia S. Sammons, MD, MSCE [5] Medical Director, Infection Prevention and Control The Children’s Hospital of Philadelphia Assistant Professor of Clinical Pediatrics UPENN School of Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Esther Maria Sampayo, MD, MPH [190] Assistant Professor, Department of Pediatrics Section of Emergency Medicine Baylor College of Medicine Texas Children’s Hospital Houston, Texas

Gina Santucci, RN, MSM, APRN-BC [10] Nurse Practioner Pediatric Advanced Care Team The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Michele Saysana, MD [4] Associate Professor of Clinical Pediatrics Medical Director Quality and Safety Department of Pediatrics Division of Hospital Medicine Indiana University School of Medicine Riley Hospital for Children at Indiana University Health Indianapolis, Indiana

Julie V. Schaffer, MD [62] Associate Professor of Dermatology and Pediatrics New York University School of Medicine New York, New York

Karen E. Schetzina, MD, MPH, CLC, FAAP [29] Professor Director, Division of Community Pediatrics Research Department of Pediatrics Quillen College of Medicine East Tennessee State University Johnson City, Tennessee

Sandra Schwab, MD, MSCE [189] Hilbert Pediatric Emergency Department Peyton Manning Children’s Hospital at St. Vincent Indianapolis, Indiana

Donald F. Schwarz [44] Robert Wood Johnson Foundation Princeton, New Jersey

Jordan Scott, MD [47,48] President of Northeast Allergy Asthma and Immunology President of Allergy & Asthma Physicians Clinical Instructor Umass/Memorial Community Medicine Leominster, Massachusetts

Steven M. Selbst, MD [13] Vice-Chair for Education Director, Pediatric Residency Program Attending Physician, Division of Emergency Medicine Nemours/Alfred I. duPont Hospital for Children Wilmington, Delaware Professor of Pediatrics Sidney Kimmel Medical College at Thomas Jefferson University

Philadelphia, Pennsylvania

Kara N. Shah, MD, PhD [67] President, Kenwood Dermatology Cincinnati, Ohio

Rachana Shah, MD [70] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician Division of Endocrinology and Diabetes The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Samir S. Shah, MD, MSCE [95,96,99,101,102] Director, Division of Hospital Medicine James M. Ewell Endowed Chair Attending Physician in Hospital Medicine & Infectious Diseases Cincinnati Children’s Hospital Medical Center Professor, Department of Pediatrics University of Cincinnati College of Medicine Cincinnati, Ohio

Nader Shaikh, MD, MPH [104] Associate Professor of Pediatrics University of Pittsburgh Children’s Hospital of Pittsburgh Division of General Academic Pediatrics Pittsburgh, Pennsylvania

Adhi N. Sharma, MD [170] Medical Director Case Management South Nassau Communities Hospital Baldwin, New York

Akshay Sharma, MBBS [90] Pediatric Hematology/Oncology Fellow, PGY-4St. Jude Children’s Research Hospital Memphis, Tennessee

Eric Shelov, MD [6] Associate Chief Health Informatics Officer Clinical Assistant Professor of Pediatrics Perelman School of Medicine University of Pennsylvania Division of General Pediatrics The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Andrew Shriner, MD [100] Assistant Professor of Clinical Pediatrics Department of Pediatrics Division of Hospital Medicine Riley Hospital for Children at Indiana University Health Indianapolis, Indiana

Kelley Shultz, MD [123] Staff Physician Department of Neonatology and Pulmonary Biology Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Jeffrey Simmons, MD [4] Associate Professor, Department of Pediatrics University of Cincinnati College of Medicine Associate Director, Quality Division of Hospital Medicine Safety Officer, James M. Anderson Center for Health Services Excellence Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Tamara D. Simon, MD, MSPH [159] Associate Professor of Pediatrics Department of Pediatrics University of Washington School of Medicine Attending Physician Seattle Children’s Hospital Center for Clinical and Translational Research Seattle Children’s Research Institute Seattle, Washington

Steven R. Sloan, MD, PhD [93] Medical Director, Blood Bank Boston Children’s Hospital Boston, Massachusetts

Karen Smith, MD, MEd [15,85] Associate Professor of Pediatrics George Washington University Chief, Division of Hospital Medicine Children’s National Health System Washington, DC

Michael J.G. Somers, MD [26] Division of Nephrology Boston Children’s Hospital Department of Pediatrics Harvard Medical School Boston, Massachusetts

Philip R. Spandorfer, MD, MSCE [73] Pediatric Emergency Medicine Associates Children’s Healthcare of Atlanta at Scottish Rite Atlanta, Georgia

Lisa Santos Spector, MD [43] Division of Child Abuse and Neglect

Director, Beyond Adverse Childhood Experiences (ACEs) Program Medical Director, Sexual Assault Nurse Examiner Program Children’s Mercy Kansas City Associate Professor UMKC School of Medicine Kansas City, Kansas

Jonathan M. Spergel, MD, PhD [61,141] Chief, Allergy Section Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Division of Allergy and Immunology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

David A. Spiegel, MD [161] Associate Professor of Orthopaedic Surgery Division of Orthopaedic Surgery The Children’s Hospital of Philadelphia Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Mythili Srinivasan, MD, PhD [184] Associate Professor of Pediatrics Washington University School of Medicine St. Louis Children’s Hospital St. Louis, Missouri

Rajendu Srivastava, MD, MPH [1] Assistant Vice President of Research Intermountain Healthcare Salt Lake City, Utah

Angela M. Statile, MD, MEd [97,140] Assistant Professor, Department of Pediatrics University of Cincinnati College of Medicine Medical Director, Burnet Campus Division of Hospital Medicine

Assistant Program Director, Pediatric Residency Program Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Nicole E. St Clair, MD [33] Division of Pediatric Hospital Medicine UW Health, American Family Children’s Hospital Associate Professor of Pediatrics University of Wisconsin School of Medicine and Public Health Milwaukee, Wisconsin

Michael C. Stephens, MD [80] Assistant Professor of Pediatrics Mayo Clinic Rochester, Minnesota

Bryan L. Stone, MD, MS [28] Professor of Pediatrics University of Utah School of Medicine Pediatric Hospitalist Primary Children’s Hospital Salt Lake City, Utah

Ashley K. Crumby, MD (formerly Ashley K. Storrs) [137] Assistant Professor of Clinical Psychiatry Child and Adolescent Psychiatry Weill Cornell Medical College, New York, New York

Margaret J. Strieper, DO [55] Director, Pacing and Electrophysiology Associate Professor of Pediatrics Emory University Children’s Healthcare of Atlanta Atlanta, Georgia

Eric Sundel, MD [155] Pediatric Hospitalist Baltimore, Washington Medical Center University of Maryland Medical System Severna Park, Maryland

Robert Sundel, MD [30,147,148] Director of Rheumatology Boston Children’s Hospital Associate Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Nicole Sutton, MD [56] Associate Professor of Pediatrics Albert Einstein College of Medicine Children’s Hospital at Montefiore Bronx, New York

Sanjeev K. Swami, MD [105] Assistant Professor of Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Division of Infectious Diseases The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Khoon-Yen Tay, MD [174] Assistant Professor, Clinical Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician Division of Emergency Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Joanna Thomson, MD, MPH [107] Assistant Professor, Department of Pediatrics

University of Cincinnati College of Medicine Attending Physician Division of Hospital Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Avram Z. Traum, MD [26] Division of Nephrology Boston Children’s Hospital Department of Pediatrics Harvard Medical School Boston, Massachusetts

James R. Treat, MD [65] Associate Professor of Pediatrics and Dermatology Fellowship Director, Pediatric Dermatology The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Michael L. Trieu, MD [138] Child and Adolescent Psychiatry Inpatient Service Boston Children’s Hospital Harvard Medical School Boston, Massachusetts

Harsh K. Trivedi, MD, MBA [135] President and CEO Sheppard Pratt Health System Professor of Clinical Psychiatry University of Maryland Baltimore, Maryland

Venée N. Tubman, MD, MMSc [89] Assistant Professor of Pediatrics Baylor College of Medicine / Texas Children’s Cancer and Hematology Centers Houston

Houston, Texas

Michael Turmelle, MD [184] Associate Professor of Pediatrics Division Co-Chief Hospitalist Medicine Medical Director St. Louis Children’s Hospital Children’s Direct Washington University School of Medicine One Children’s Place St. Louis, Missouri

Lily Changchien Uihlein, MD, JD [60] Dermatology Program Boston Children’s Hospital Boston, Massachusetts

Levon Utidjian, MD, MBI [6] Clinical Instructor in Pediatrics Perelman School of Medicine at the University of Pennsylvania Attending Physician, Division of General Pediatrics The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Joyee G. Vachani, MD, MEd [115] Assistant Professor of Pediatrics Baylor College of Medicine Texas Children’s Hospital Houston, Texas

Charles P. Venditti, MD, PhD [87] Investigator National Human Genome Research Institute Genetics and Molecular Biology Branch National Institutes of Health Bethesda, Maryland

Menno Verhave, MD [82]

Clinical Director, Gastroenterology Boston Children’s Hospital Assistant Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Robert N. Vincent, MD, CM [55] Children’s Healthcare of Atlanta Professor of Pediatrics Emory University School of Medicine Atlanta, Georgia

Samuel Volchenboum, MD, PhD [91] Assistant Professor of Pediatrics Director, Center for Research Informatics University of Chicago Chicago, Illinois

Michael T. Vossmeyer, MD [7] Associate Professor, Department of Pediatrics, University of Cincinnati College of Medicine Attending Physician and Medical Director for Community Integration, Division of Hospital Medicine Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Robert M. Wachter, MD [Foreword] Professor and Associate Chairman Department of Medicine Marc and Lynne Benioff Endowed Chair Chief of the Division of Hospital Medicine University of California, San Francisco Chief of the Medical Service UCSF Medical Center San Francisco, California

Amy T. Waldman, MD, MSCE [122] Division of Neurology, Children’s Hospital of Philadelphia Assistant Professor of Neurology Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Sowdhamini Wallace, DO, FAAP [113] Assistant Professor, Department of Pediatrics Section of Pediatric Hospital Medicine Baylor College of Medicine and Texas Children’s Hospital Houston, Texas

Jennifer K Walter, MD, PhD, MS [12] Assistant Professor of Pediatrics, Medical Ethics and Health Policy University of Pennsylvania School of Medicine Attending Physician General Pediatrics and Pediatric Advanced Care Team The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Jolan E. Walter, MD, PhD [49] Department of Pediatrics Division of Allergy and Immunology Massachusetts General Hospital for Children Harvard Medical School Boston Massachusetts

George Sam Wang, MD [171] Assistant Professor Department of Pediatrics Section of Emergency Medicine University of Colorado School of Medicine Children’s Hospital Colorado Aurora, Colorado

Laura Placke Ward, MD, IBCLC [129]

Assistant Professor of Pediatrics Division of Neonatology Cincinnati Children’s Hospital Medical Center Cincinnati, Ohio

Kathy E. Wedig, MD [131] Associate Professor of Clinical Pediatrics UC College of Medicine Attending Neonatologist Perinatal Institute Cincinnati Children’s Hospital Cincinnati, Ohio

Kirstin Weerdenburg, MD, FRCPC [167] Pediatric Emergency Medicine The Hospital for Sick Children (SickKids) Toronto, Ontario, Canada

Daniel J. Weiner, MD [146] Associate Professor of Pediatrics University of Pittsburgh School of Medicine Medical Director, Pulmonary Function Laboratory Director, Antonio J. and Janet Palumbo Cystic Fibrosis Center Children’s Hospital of Pittsburgh of UPMC Pittsburgh, Pennsylvania

Michael Weinstein, MD, FRCPC [182] Associate Professor Department of Paediatrics Division of Paediatric Medicine University of Toronto The Hospital for Sick Children (SickKids) Toronto, Ontario, Canada

Derek J. Williams, MD, MPH [102] Assistant Professor of Pediatrics

Division of Hospital Medicine Monroe Carell, Jr. Children’s Hospital at Vanderbilt Vanderbilt University Medical Center Nashville, Tennessee

Celeste R. Wilson, MD, FAAP [39] Medical Director, Child Protection Program Boston Children’s Hospital Assistant Professor of Pediatrics Harvard Medical School Boston, Massachusetts

Stephen D. Wilson, MD, PhD [23] Professor of Pediatrics Department of Pediatrics University of California, San Francisco San Francisco, California

Sarah Wood, MD [45] Attending Physician Special Immunology Service The Children’s Hospital of Philadelphia Clinical Associate Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Albert C. Yan, MD, FAAP, FAAD [61] Chief, Section of Pediatric Dermatology Children’s Hospital of Philadelphia Professor, Pediatrics and Dermatology Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania

Sabrina W. Yum, MD [120] Assistant Professor of Clinical Neurology and Pediatrics Departments of Neurology and Pediatrics

Perelman School of Medicine of the University of Pennsylvania Director, CMTA Center of Excellence Co-director, Brachial Plexus Injury Program Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Elaine H. Zackai, MD [84] Director, Clinical Genetics Division of Human Genetics The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Andrea L. Zaenglein, MD [59] Professor of Dermatology and Pediatrics Penn State/Hershey Medical Center Hershey, Pennsylvania

David Zipes, MD FAAP, SFHM [11,14] Director, Henry County Pediatric Hospitalists Henry Community Health New Castle, Indiana Pediatric Hospitalists Peyton Manning Children’s Hospital at St. Vincent Indianapolis, Indiana

Michele L. Zucker, MD, MPH [46] Assistant Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania Craig Dalsimer Division of Adolescent Medicine The Children’s Hospital of Philadelphia Philadelphia, Pennsylvania

Foreword There was a time in the early days of the hospitalist field that my colleagues and I wondered whether the pediatric portion of the field was here to stay. At the first few gatherings of the Society of Hospital Medicine (SHM; then called the National Association of Inpatient Physicians), we organized a pediatric track, but only a few people showed up. One lonely pediatric hospitalist served on the SHM board—he constantly had to remind us to pay attention to the pediatric perspective, and frequently apologized for being a nag. While the early leaders of the hospitalist field were committed to integrating pediatrics, there were times when we wondered whether our efforts were a well-intended act of futility. Luckily, we persevered, and so did those early pediatric leaders. Today, the annual meeting of SHM has a robust pediatrics track, and so do the meetings of major pediatric specialty societies. There are thriving pediatric hospital medicine programs at virtually every major children’s hospital in the United States, and also programs at large and even some not-so-large general hospitals. At my own hospital, UCSF Medical Center, pediatric hospitalists have major leadership roles not only within our children’s hospital but also for the entire institution—in areas ranging from performance improvement to medical education to information technology. I am blessed to have several med-peds trained hospitalists in my own adult hospitalist group, and have been pleasantly surprised by the degree to which these individuals serve as bridges between the worlds of adult and pediatric care. While they have taken advances from the adult side to pediatrics, I have found that the arrow usually points in the other direction. For example, our pediatric hospitalists were early adopters of patient- and family-centered rounding, even scheduling appointments on rounds so that families could plan for the team’s arrival, and

nurses, case managers, and specialists could sometimes attend. We on the adult side have been awed by, and tried to learn from, this effort. As long as I’m on the subject of leadership, it’s worth noting that as of this writing, the top physician at the Centers for Medicare & Medicaid Services—the federal agency that funds more than 40% of US healthcare, to the tune of nearly $1 trillion per year—is Dr Patrick Conway, a pediatric hospitalist. Since I co-wrote the first article on pediatric hospitalists,1 the growth and success of the field has been astonishing. As in adult medicine, this growth has been fueled by residency duty hour limitations in teaching hospitals, which created a substantial need for physician coverage on services previously supported by trainees. Another driver has been new collaborative arrangements, in which hospitalists have branched out from simply practicing general pediatrics in the hospital to co-managing all sorts of surgical patients, running procedure services, staffing rapid response teams, and serving as leaders in systems improvement work. It is easy to look at this burgeoning and think that it all was preordained. But it was not. In the late 1990s and the early 2000s, hospitalists were seen by many as an untested innovation; many people were skeptical, even downright hostile, to the model. Given this, the growth in pediatric hospitalists (and leaders) is a testament to the quality and can-do spirit of the early entrants to the field. Former Disney CEO Michael Eisner once observed, “A brand is a living entity—and it is enriched or undermined cumulatively over time, the product of a thousand small gestures.” It is these gestures—making a tricky diagnosis, effectively leading a quality improvement initiative, being an enthusiastic participant in an interdisciplinary team, dealing sensitively with the family of a dying child— that have enhanced our field’s brand, and this brand has been the engine fueling our unprecedented growth. What’s in store for the next several years? In the United States, the Affordable Care Act and other payment and structural changes are beginning to transform healthcare. With fewer patients lacking insurance, access has improved (although it is certainly not assured). We are witnessing a greater emphasis on population health and care across the continuum, and experiencing a mandate to improve not only quality and safety but also patient experience. In addition, the pressure to “bend the cost curve” is just

beginning to accelerate. This pressure makes it essential that all hospitalists learn to practice in more cost-effective styles, and also assume leadership roles in helping their system identify and root out wasteful care. Federal incentives designed to move healthcare from an analog to a digital industry have been successful in driving the electronic health record adoption curve way up. All of us can expect to practice in a computerized environment for the rest of our careers. What does all of this mean for pediatric hospitalists? The intense cost pressures will inevitably lead to the closure of some hospitals, but patients in the ones that remain will be even sicker. There will be a premium on delivering demonstrably high quality, safe, and satisfying care at the lowest possible cost. The amount of money available to support pediatric hospitalist programs will be constrained, and so hospitalist programs will be asked to unequivocally demonstrate their value to justify ongoing support. Under the threat of readmission penalties or in the face of new payment models such as Accountable Care Organizations or bundled payments, hospitals will no longer be able to act as silos; there will be much greater emphasis on seamless transitions. Computerized tools will become essential to clinical practice, and good clinical delivery systems will learn to take full advantage of the power of these tools while mitigating some of the awkward workflows and even the harms that are emerging. Just as physicians in small practices are migrating to larger groups, individual hospitals are forging new relationships with others. As hospitalist groups become larger and span multiple institutions, new opportunities but also daunting management challenges will be created. The pace of change is unlike anything I have experienced in my 30 years of practice and administration. At a departmental meeting not too long ago, a department leader was discussing all of these changes: new regulations and payment systems, public reporting, information technology, and more. I could see the faculty—particularly my senior colleagues—squirming in their chairs. Finally one of our most respected cardiologists, an elder statesman in the department, grabbed the microphone. “You know,” he said, “this could be worse.” I found this a very surprising remark from a senior faculty member, particularly one who had clearly thrived under the old rules. But then he finished his thought.

“I could be younger.” While I still chuckle about this, I have to say that I completely disagree with the message. My own sentiments were well captured in an article by Don Berwick and Jonathan Finkelstein (both pediatricians) in 2010.2 They wrote, We think that the anxiety, demoralization, and sense of loss of control that afflict all too many healthcare professionals today comes not from finding themselves to be participants in systems of care, but rather from finding themselves lacking the skills and knowledge to thrive as effective, proud, and well-oriented agents of change in those systems…. A physician equipped to help improve healthcare will be not demoralized, but optimistic; not helpless in the face of complexity, but empowered; not frightened by measurement, but made curious and more interested; not forced by culture to wear the mask of the lonely hero, but armed with confidence to make a better contribution to the whole. This seems right to me. More importantly, when I find myself in a crowd of young pediatric hospitalists, I am quite certain that they see themselves— quite proudly—as leaders of a new, and improved, healthcare system. As well they should. Robert M. Wachter, MD References 1. Bellet PS, Wachter RM. The hospitalist movement and its implications for the care of hospitalized children. Pediatrics 1999;103:473-477. 2. Berwick DM, Finkelstein JA. Preparing medical students for the continual improvement of health and health care: Abraham Flexner and the new “public interest”. Acad Med 2010; 85(9 Suppl):S56-65.

Preface Come gather around people Wherever you roam And admit that the waters Around you have grown And accept it that soon You’ll be drenched to the bone And if your breath to you is worth saving Then you better start swimming or you’ll sink like a stone For the times they are a-changing Bob Dylan For pediatric hospitalists, the Nobel Laureate’s words could not ring more true. From that gathering of 130 or so of us in San Antonio in November 2003 to the announcement of subspecialty certification a mere 13 years later, the times are certainly a-changing. When we reread the preface of our first edition in 2007, we were tickled to find that we wrote “it would not be surprising if 10 years from now the field will have evolved dramatically.” Who knew we would be such excellent prognosticators? In our first edition, we were also quite proud of whom our book represented. In this edition, we endeavored to include a wide breadth of contributors as well. We have close to 300 contributing authors from over 50 different institutions represented—large and small children’s hospitals, pediatric departments in academic medical centers, community hospitals, private practice, and everything in between. The common thread that

connects all of these immensely talented individuals is the desire to present the best possible care to the hospitalized pediatric patient. And while our field has “grown up” so quickly, let us all recognize that there is still so much left to do. As hospitalists, we must continue to reduce unnecessary utilization of resources and eliminate unwarranted variation in care. There are still healthcare disparities and inequities in the system that need to be confronted and addressed. And while we may feel completely at home on the wards, hospitalization represents a vulnerable time for patients and families and we need to continue to work to improve that experience. We give our deepest and sincerest thanks to all of the individuals who made this second edition possible. In particular, we would like to recognize our associate editors Brian, Sanjay, and Samir, without whom this project would not have been possible. We are also most appreciative of the folks at McGraw-Hill who saw the need with us for a second edition and supported us through this effort. Lisa B. Zaoutis, MD and Vincent W. Chiang, MD

PART

Inpatient Pediatric Medicine

I

Part Editor Christopher P. Landrigan, MD, MPH

1 Understanding the Value of Pediatric Hospitalists: Studies of Efficiency, Education, Care Processes, and Quality of Care 2 Variation in Healthcare 3 Evidence-Based Medicine 4 Overview: Quality of Care 5 Infection Control for Pediatric Hospitalists 6 Electronic Health Records and Clinical Decision Support 7 Family-Centered Care 8 Medical Comanagement and Consultation 9 Child Development: Implications for Inpatient Medicine 10 Palliative Care 11 Communication and Discharge Planning 12 Ethical Issues in Pediatric Hospital Practice 13 Medicolegal Issues in Pediatric Hospital Medicine 14 Hospitalist Professional Organizations and Models of Care 15 Careers in Hospital Medicine

CHAPTER

1

Understanding the Value of Pediatric Hospitalists: Studies of Efficiency, Education, Care Processes, and Quality of Care Rajendu Srivastava and Christopher P. Landrigan

CURRENT STATE OF AFFAIRS The field of pediatric hospital medicine has grown rapidly over the past 18 years, since the term “hospitalist” was first coined. The number of hospitalists, including pediatric hospitalists, has increased exponentially and is expected to keep growing.1-4 Numerous pediatric hospital medicine fellowship programs have arisen, producing a new generation of clinicianquality improvement experts and clinician-investigators. Ten years after the first pediatric hospitalist conference, sponsored by the Ambulatory Pediatric Association, which drew more than 120 participants from the United States and Canada, the meeting is now an annual event. Sponsorship comes from the Academy of Pediatrics, the Society of Hospital Medicine, and the Academic Pediatric Association (formerly known as the Ambulatory Pediatric Association, the name change prompted in part by the organization’s recognition of the growth of pediatric hospital medicine). Pediatricians’ organizations have expanded to meet the needs of hospitalists, and hospitalists’ organizations have expanded to meet the needs of their pediatric contingent. The American Academy of Pediatrics Section on Hospital Medicine has one of the largest section memberships in the American Academy of Pediatrics (AAP) and an extremely active listserv; the Academic Pediatric Association Special Interest Group on Pediatric Hospital Medicine is also one of the largest interest groups and has a vital presence at

the yearly Pediatric Academic Societies meeting, and the Society of Hospital Medicine (the largest hospitalist organization) continues to foster a home for pediatric hospitalists—several key organizational leadership positions are held by pediatricians. Important research networks and collaboratives have arrived. The Pediatric Research in Inpatient Settings (PRIS; pronounced prize) network is focusing on a series of complementary national funded studies ultimately intended to provide the tools to measure and improve the quality of inpatient care. In addition, the Value in Inpatient Pediatrics (VIP) Network has partnered with the Quality Improvement Innovation Networks (QuINN) to conduct grassroots quality improvement studies engaging clinicians. Finally, pediatric hospitalists are continuing to expand their nonclinical responsibilities to include leadership roles in administration (as division chiefs, medical directors, quality improvement officers, and leaders in patient safety and informatics), research (leading research networks, developing health services research laboratories, and training junior investigators to be competitive in obtaining grants), and education (as clerkship and residency directors). A tremendous amount of knowledge has been gained from critical early work in the value of pediatric hospitalists in inpatient care delivery. This chapter outlines what we have learned from these early studies, as well as what we still need to learn, and proposes mechanisms to accomplish next steps.

STUDIES ON EFFICIENCY A substantial number of studies have been conducted on the efficiency of care delivered in pediatric hospitalist models. These studies have been largely single-center studies using before-and-after study designs or interrupted time series analyses. Most (but not all) have found 10–20% shorter length of stay (LOS) and reduced resource utilization (measured as charges or costs) in pediatric hospitalist systems.5-11 The types of pediatric hospitalist services evaluated across studies vary, but collectively the studies include academic and nonacademic pediatric hospitalist systems, managed care organization hospitalist systems, and attending-only hospitalist systems—one service that focused on medically complex cases, and another that focused on common conditions—with comparisons to community, general academic pediatric, and specialist attending physician traditional care systems. These findings, in

conjunction with adult hospitalist studies12-18 showing similar improvements in LOS and total costs and similar preservation of quality outcomes, indicate that the argument for the value of pediatric hospitalist systems is both strong and sound.12 Future research in this area should attempt to add new knowledge rather than replicate well-established findings. For example, future studies might help answer questions about the advantages and disadvantages of specific types of pediatric hospitalist models, why they work, and which patient populations are most or least likely to benefit from such systems.

EDUCATION A few studies have examined the experiences of medical students and house staff in hospitalist systems. Hospitalists have been rated highly as educators, compared with either traditional academic attending physicians or subspecialists.19-24 Initial concerns regarding a decrease in students’ exposure to subspecialists and community pediatricians in some medical centers have been mitigated by strategies to give medical students exposure to faculty other than hospitalists during inpatient rotations. However it will be important to continue to ensure that trainees have varied attending types to learn from. The professional development of hospitalist educators is an important area, and more research is needed on the educational impact of the hospitalist model.21

PHYSICIAN ATTITUDES Some studies have documented the experiences and attitudes of primary care physicians (those who refer patients to hospitalists) and subspecialists (those who serve as consultants to hospitalists) with regard to hospitalist models in adult medicine. These studies have found that some primary care physicians and subspecialists are concerned about the quality of care, teaching, and patient satisfaction provided by the hospitalist model,25,26 but others are more positive about the model. One pediatric study found that community physicians and residents rated the hospitalist system as excellent, whereas subspecialty physicians rated it as average.27 Another study of the initiation of a pediatric hospitalist service at a tertiary care children’s hospital found

that community physicians were more ambivalent than specialty physicians; community physicians’ specific concerns included impaired communication and maintenance of long-term relationships with their patients.28 As hospitalist models in pediatrics have become more prevalent and are now the dominant inpatient model of care in some hospitals, it appears that the acceptance of, and satisfaction with, hospitalist services have grown, while new challenges such as optimal communication and transitions of care are new areas of research.29-32

QUALITY While the body of research evaluating hospitalist systems has become far more robust, measuring quality of care remains a challenge for pediatric hospital medicine, as inpatient quality measures are still largely underdeveloped in pediatrics. The Institute of Medicine defines quality care as that which is effective, efficient, safe, patient centered, timely, and equitable13 (see Chapter 4 for further discussion). Evaluating quality involves measuring processes and outcomes of care.

PROCESS MEASURES Early studies of adults cared for by hospitalists focused on process measures and found that the quality of the care processes used by hospitalist and nonhospitalist systems is similar, but that hospitalist systems reduce LOS and costs, yielding more efficient care.14,15 Process measures are particularly challenging in inpatient pediatrics, because few diseases have well-defined quality measures or strong evidence to link particular processes of care with improved outcomes. A notable exception is asthma, for which evidence-based quality metrics have been developed (e.g. the percentage of asthmatic children discharged from the hospital with an inhaled steroid). To measure quality of care using such a process marker, a hospital would need to measure the frequency with which children are sent home with inhaled steroids, ideally controlling for severity of illness and potential confounders. Early work has been conducted to study this particular measure (along with other process and outcome measures) in hospitalized children. One study of 30 children’s hospitals, examining three

asthma process quality measures (use of relievers, use of systemic corticosteroids, and use of a written home asthma action plan), found no observed decrease in asthma readmissions within 30 days of being discharged.33 Another single children’s hospital study, however, found that after an intense period of dissemination and implementation of evidencebased best practices there was a reduction in pediatric readmissions, but it took 9 months to observe this change, and only if the readmission time period was extended to 6 months (and not 30 days).34 Studies can evaluate other disease-dependent process measures, such as the use of inhaled ipratropium bromide on wards or the use of chest radiographs to rule out pneumonia. Unlike the example of post-discharge inhaled steroids, however, which has a strong evidence base to support it, many process measures have a weaker evidence base (i.e. adherence to that process measure has not been strongly linked to improved patient outcomes) and hence are subject to greater disagreement. For example, the clinical criteria to determine which hospitalized children with fever and respiratory symptoms, as well as a preexisting condition of asthma, should have chest radiographs have not been well studied; thus, it is difficult to define objectively what constitutes optimal care. Research is needed to identify such process measures across a range of conditions. Because most pediatric conditions do not have well-defined disease-dependent process measures, quality-of-care studies are challenging, particularly those assessing the quality of care in patients with uncommon conditions or complex diseases. The development of disease-dependent measures has been extremely limited in pediatrics for a number of reasons. One reason is that a very large number of pediatric diseases lead to hospitalization, whereas a relatively small number of acute conditions in adults (which have been more thoroughly studied) lead to hospitalization. Disease-independent process measures are one method of addressing quality of care in the face of such a large range of infrequent conditions. To take the handoff process as an example (between a change of house staff teams on the wards), one could operationalize, test, and validate measures of quality unrelated to a particular condition, such as the content, mode, and quality of handoffs between house staff, incidence of medical errors on the wards, frequency of timely, accurate transmission of information from inpatient to outpatient providers before the follow-up visit, and timing and appropriateness of—and patient satisfaction

with—follow-up visits with specialists involved in inpatient care. Recent work has found that implementation of interventions to improve handoffs can lead to substantive improvements in this care process, which have been linked to improvements in outcomes.35,36

OUTCOME MEASURES Quality of care can also be measured by looking directly at outcomes. Two studies of hospitalist care for adults demonstrated decreased mortality in hospitalist systems.16,17 In pediatrics, death is a rare outcome and is therefore an insensitive measure of quality of care. More recently, pediatric readmissions have been proposed as a possible measure of pediatric inpatient care quality. One large study in 72 children’s hospitals of more than 568,000 index admissions found an overall 30-day readmission rate of 6.5% (far lower than common conditions studied in adults), but with some significant variation in certain populations.37 This measure is complex and requires further understanding of the factors influencing pediatric readmissions (including the social determinants) before using this in quality-of-care outcome studies. Mortality and readmission rates have been unchanged in most studies comparing pediatric hospitalist systems with traditional care systems. Bellet et al.’s study found that the 10-day readmission rate increased from 1% to 3% in a hospitalist system (P = .006), but no other study has found hospitalist systems to be associated with increased readmissions.5 One study comparing survival and LOS in a pediatric intensive care unit found that patients cared for by hospitalists had an odds ratio of 2.8 for survival and a 21.1-hour shorter LOS (both P = .13), when adjusted for severity of illness, compared with patients cared for by residents.18 Patient satisfaction and experience can also be measured. Studies of patients’ and parents’ experiences in hospitalist systems have found either no difference or occasionally improved satisfaction with care provided by hospitalists compared with nonhospitalist (general academic or community) attending physicians.6,10

CURRENT DIRECTIONS: VARIATION IN CARE,

COMPARATIVE EFFECTIVENESS, AND DISSEMINATION AND IMPLEMENTATION STUDIES In the past few years, a number of studies have emerged documenting variation in care across pediatric hospitals and beginning to compare the effects of alternative approaches to the management of common conditions. In addition, hospitalists have begun to get involved in studies seeking to disseminate evidence-based care approaches, evaluating the effectiveness of dissemination and implementation efforts.

VARIATION IN HOSPITAL CARE Several studies have now documented the large variation in resource utilization across a variety of pediatric conditions in children’s hospitals using existing databases. In addition to variation in disease-dependent therapies (e.g. surgery and medication regimens) and diagnostic testing (e.g. radiological studies), several studies have demonstrated large variation in complications and readmissions38-40 (see Chapter 2 for a full discussion of variation in care).

COMPARATIVE EFFECTIVENESS RESEARCH Comparative effectiveness studies seek to rigorously compare outcomes when alternative approaches are used in the management of a particular problem. In inpatient pediatrics, because there is wide variation in care, there are numerous opportunities to conduct comparative effectiveness research using data currently being collected. For example, many studies using the Children’s Hospital Association’s Pediatric Health Information System (PHIS) administrative database have been carried out in the past decade, both documenting variation in care and seeking to assess the comparative effectiveness of diverse management strategies. PHIS+, an ongoing study being run through the Pediatric Research in Inpatient Settings (PRIS) hospitalist research network, is currently building on this work, conducting four comparative effectiveness research (CER) studies to compare common treatment strategies for costly conditions (osteomyelitis, appendicitis, pneumonia, and gastroesophageal reflux disease);41 this database takes advantage of an infrastructure of shared clinical data (specifically laboratory,

radiology, and microbiology data) that have been overlaid on top of the PHIS dataset for six children’s hospitals. Other CER treatment studies are also being conducted, such as the Pediatric Intravenous Versus Oral Antibiotic Therapy (PIVVOT) study. This study aims to compare the effectiveness of oral versus intravenous antibiotics (via a peripherally inserted central catheter (PICC) line) in children who require prolonged home therapy after hospitalization for complicated pneumonia, ruptured appendicitis, or osteomyelitis. Similar research carried out across a range of conditions will unquestionably be of major value to the field of pediatric hospital medicine in the years to come.

DISSEMINATION AND IMPLEMENTATION RESEARCH Finding the answers to what works best is only the first step. Implementing the findings in real-world settings and testing adherence to best practices and their effectiveness in real-world settings is the next natural extension of this work. In one study, I-PASS, a series of handoff interventions, have been disseminated to nine hospitals across North America. Studies of the effectiveness of adoption and downstream outcomes are underway. Further studies evaluating the effects of implementing evidence-based practices in inpatient pediatrics—both for particular conditions and across conditions— are needed.

DIRECTIONS FOR THE FUTURE There is still much work to be done. Future studies should expand to include a new focus on how current inpatient care is being delivered (measuring the quality of inpatient pediatric care) and how this can be improved both for children with common conditions and for medically complex children who are frequent users of inpatient resources and highly susceptible to a fragmented delivery system. One method of overcoming the challenges of studying vulnerable populations with small numbers or rare conditions is to take advantage of multicenter research and improvement networks, an effort that is well underway in pediatric hospital medicine. Both the PRIS network and the Value in Inpatient Pediatrics (VIP) network have membership from large numbers of hospitals and hospitalists. Through networks, opportunities exist

to reduce unwarranted variation in inpatient pediatric care and spread best practices. Two critical steps in research must occur to improve the current system of inpatient care. First, measures of inpatient processes of pediatric care must be explicitly defined, validated, and tested. Second, pediatric hospitalists must be studied in their natural laboratory (i.e. the hospital) to understand both the limitations and the possibilities of translating research findings into clinical practice using a variety of quality improvement techniques. As both these steps occur, the care delivered to hospitalized children will undergo a paradigm shift. We will be able to measure routine care, compare it against high performers, and improve it so that we can deliver care that is effective, efficient, safe, patient centered, timely, and equitable.

REFERENCES 1. Srivastava R, Landrigan C, Gidwani P, et al. Pediatric hospitalists in Canada and the United States: A survey of pediatric academic department chairs. Ambul Pediatr. 2001;1:338-339. 2. Landrigan C, Srivastava R, Muret-Wagstaff S, et al. Pediatric hospitalists: What do we know, and where do we go from here? Ambul Pediatr. 2001;1:340-345. 3. Lurie JD, Miller DP, Lindenauer PK, et al. The potential size of the hospitalist workforce in the United States. Am J Med. 1999;106:441445. 4. Landrigan CP, Srivastava R. Pediatric hospitalists: Coming of age in 2012. Arch Pediatr Adolesc Med. 2012 166(8):696-699. 5. Bellet PS, Whitaker RC. Evaluation of a pediatric hospitalist service: Impact on length of stay and hospital charges. Pediatrics. 2000;105:478484. 6. Landrigan CP, Srivastava R, Muret-Wagstaff S, et al. Impact of a health maintenance organization hospitalist system in academic pediatrics. Pediatrics. 2002;110:720-728. 7. Dwight P, MacArthur C, Friedman J, Parkin PC. Evaluation of a staffonly hospitalist system in a tertiary care academic children’s hospital. Pediatrics. 2004;114:1545-1549.

8. Srivastava R, Muret-Wagstaff S, Young P, James BC. Hospitalist care of medically complex children. Pediatr Res. 2004;55:314A-315A. 9. Maggioni A, Rolla F. Comparison of hospitalist and pediatric subspecialist care on selected APR-DRGs: Length of stay and hospital charges. Pediatr Res. 2004;55:315A. 10. Wells RD, Dahl B, Wilson SD. Pediatric hospitalists: Quality care for the underserved? Am J Med Qual. 2001;16:174-180. 11. Seid M, Quinn K, Kuttin P. Hospital based and community pediatricians: Comparing outcomes for asthma and bronchiolitis. J Clin Outcomes Manage. 1997;4:21-24. 12. Wachter RM, Goldman L. The hospitalist movement 5 years later. JAMA. 2002;287:487-494. 13. Institute of Medicine (US) Committee on Quality of Health Care in America. Crossing the Quality Chasm: A New Health System for the 21st Century. Washington, DC: National Academy Press; 2001. 14. Lindenauer PK, Chehabeddine R, Pekow P, et al. Quality of care for patients hospitalized with heart failure: Assessing the impact of hospitalists. Arch Intern Med. 2002;162:1251-1256. 15. Rifkin WD, Conner D, Silver A, Eichorn A. Comparison of processes and outcomes of pneumonia care between hospitalists and communitybased primary care physicians. Mayo Clin Proc. 2002;77:1053-1058. 16. Auerbach AD, Wachter RM, Katz P, et al. Implementation of a voluntary hospitalist service at a community teaching hospital: Improved clinical efficiency and patient outcomes. Ann Intern Med. 2002;137:859865. 17. Meltzer D, Shah M, Morrison J. Decreased length of stay, costs and mortality in a randomized trial of academic hospitalists. J Gen Intern Med. 2001;16(Suppl):S208. 18. Tenner PA, Dibrell H, Taylor RP. Improved survival with hospitalists in a pediatric intensive care unit. Crit Care Med. 2003;31:847-852. 19. Hunter AJ, Desai SS, Harrison RA, Chan BK. Medical student evaluation of the quality of hospitalist and nonhospitalist teaching faculty on inpatient medicine rotations. Acad Med. 2004;79:78-82. 20. Kripalani S, Pope AC, Rask K, et al. Hospitalists as teachers. J Gen

Intern Med. 2004;19:8-15. 21. Hauer KE, Wachter RM. Implications of the hospitalist model for medical students’ education. Acad Med. 2001;76:324-330. 22. Landrigan CP, Muret-Wagstaff S, Chiang VW, et al. Effect of a pediatric hospitalist system on housestaff education and experience. Arch Pediatr Adolesc Med. 2002;156:877-883. 23. Landrigan CP, Muret-Wagstaff S, Chiang VW, et al. Senior resident autonomy in a pediatric hospitalist system. Arch Pediatr Adolesc Med. 2003;157:206-207. 24. Kemper AR, Freed GL. Hospitalists and residency medical education: Measured improvement. Arch Pediatr Adolesc Med. 2002;156:858-859. 25. Auerbach AD, Nelson EA, Lindenauer PK, et al. Physician attitudes toward and prevalence of the hospitalist model of care: Results of a national survey. Am J Med. 2000;109:648-653. 26. Auerbach AD, Davis RB, Phillips RS. Physician views on caring for hospitalized patients and the hospitalist model of inpatient care. J Gen Intern Med. 2001;16:116-119. 27. Ponitz K, Mortimer J, Berman B. Establishing a pediatric hospitalist program at an academic medical center. Clin Pediatr (Phila). 2000;39:221-227. 28. Srivastava R, Norlin C, James BC, et al. Community and hospital-based physicians’ attitudes regarding pediatric hospitalist systems. Pediatrics. 2005;115:34-38. 29. Auerbach AD, Aronson MD, Davis RB, Phillips RS. How physicians perceive hospitalist services after implementation: Anticipation vs. reality. Arch Intern Med. 2003;163:2330-2336. 30. Harlan G, Srivastava R, Harrison L, McBride G, Maloney C. Pediatric hospitalists and primary care providers: A communication needs assessment. J Hosp Med. 2009 Mar;4(3):187-193. 31. Snow V, Beck D, Budnitz T, et al. Transitions of Care Consensus policy statement: American College of Physicians, Society of General Internal Medicine, Society of Hospital Medicine, American Geriatrics Society, American College of Emergency Physicians, and Society for Academic Emergency Medicine. J Hosp Med. 2009;4(6):364-370.

32. Landrigan C, Stucky E, Chiang VW, Ottolini MC. Variation in inpatient management of common pediatric diseases: A study from the Pediatric Research in Inpatient Settings (PRIS) network. Pediatr Res. 2004;55:315A. 33. Morse RB, Hall M, Fieldston ES, et al. Hospital-level compliance with asthma care quality measures at children’s hospitals and subsequent asthma-related outcomes. JAMA. 2011;306(13):1454-1460. 34. Fassl BA, Nkoy FL, Stone BL, et al. The Joint Commission Children’s Asthma Care quality measures and asthma readmissions. Pediatrics. 2012;130(3):482-491. 35. Starmer AJ, Sectish TC, Simon DW, et al. Rates of medical errors and preventable adverse events among hospitalized children following implementation of a resident handoff bundle. JAMA. 2013;310:22622270. 36. Starmer AJ, Spector ND, Srivastava R, et al.; for the I-PASS Study Group. Changes in medical errors after implementation of a resident handoff program. New Engl J Med. 2014;371:1803-1812. 37. Berry JG, Toomey SL, Zaslavsky AM, et al. Pediatric readmission prevalence and variability across hospitals. JAMA. 2013;309(4):372-380 38. Rice-Townsend S, Barnes JN, Hall M, Baxter JL, Rangel SJ. Variation in practice and resource utilization associated with the diagnosis and management of appendicitis at freestanding children’s hospitals: Implications for value-based comparative analysis. Ann Surg. 2014;259(6):1228-1234. 39. Brogan TV, Hall M, Williams DJ, et al. Variability in processes of care and outcomes among children hospitalized with community-acquired pneumonia. Pediatr Infect Dis J. 2012;31(10):1036-1041. 40. Simon TD, Hall M, Riva-Cambrin J, et al. Hydrocephalus Clinical Research Network. Infection rates following initial cerebrospinal fluid shunt placement across pediatric hospitals in the United States. J Neurosurg Pediatr. 2009;4(2):156-165. 41. Narus S, Srivastava R, Gouripeddi R, et al. Federating clinical data from six pediatric hospitals: Process and initial results from the PHIS? Consortium. In: Improving Health: Informatics and IT Changing the World. Proceedings of the AMIA 2011 Annual Symposium, Washington,

DC, October 22–26, 2011; 994-1003. Epub 2011 October 22.

CHAPTER

2

Variation in Healthcare Lisa McLeod and Ron Keren

If all variation were bad, solutions would be easy. The difficulty is in reducing the bad variation, which reflects the limits of professional knowledge and failures in its application, while preserving the good variation that makes care patient centered. When we fail, we provide services to patients who don’t need or wouldn’t choose them while we withhold the same services from people who do or would, generally making far more costly errors of overuse than of underuse. Mulley AJ. Improving productivity in the NHS BMJ 2010

INTRODUCTION Variation in the structures, processes, and organization of healthcare services in the United States has been studied for decades. As the field of hospital medicine grows, contributions from hospitalists in the fields of health services research and quality improvement have been instrumental in advancing our understanding of the drivers of variation and the impact of this variation on the cost, quality, and outcomes of care delivered to hospitalized children.

HISTORICAL PERSPECTIVES ON VARIATION The first population-based study of variation in pediatric healthcare was published by British researcher J. Allison Glover in 1938.1 Starting early in the 20th century, rates of tonsillectomies among school children in England and Wales rose dramatically. By 1931, the surgery made up three-quarters of all procedures performed in children in the United Kingdom. Across boroughs, however, the proportion of children having their tonsils removed varied in some cases by tenfold, even among areas with similar populations. Seeking to understand why these rates were so variable, Glover closely examined the hospital and education health records from several neighboring boroughs and found significant disparities in the rates of tonsillectomy. For

example, “well-to-do” vs. poor children were more likely to enter secondary school without their tonsils. However, neither socioeconomic status nor any other health service area or population characteristics explained why a child living in one borough had a higher probability of getting their tonsils removed than a similar child in a different borough. Put simply, Glover concluded that the variation “defie[d] explanation.” In the 1960s, John Wennberg—then a researcher at the Dartmouth School of Medicine—would refer to this type of unexplained geographic variation as unwarranted, or variation in the utilization of health care services that cannot be explained by patient illness, patient preferences, or evidence, but rather indicates differences in health system performance. Taking advantage of newly implemented health data systems in Vermont and other areas of New England, Wennberg and his colleagues at Dartmouth and Johns Hopkins used advanced statistical methods to analyze patterns in the healthcare utilization among neighboring hospital service areas.2,3 In what was a highly influential series of publications, the Dartmouth group reported wide geographic variation in hospital admissions, diagnostic testing, and surgical procedures —including a tenfold difference in rates of tonsillectomies. All findings were still highly significant after case-mix adjustments, including prevalence of disease, patient demographics, socioeconomic factors, and medical practice differences.2 Stemming from these early studies, Wennberg and colleagues would go on to found the Dartmouth Atlas Project (http://www.dartmouthatlas.org), a publically available series of reports providing comprehensive information and analysis about how healthcare resources (and healthcare quality) vary, even within very small areas, across the United States. The Atlas publications have played a significant role in moving forward the agenda to standardize the care delivered and improve the overall value of healthcare. Perhaps the most significant Dartmouth Atlas finding has been that greater healthcare spending does not always correlate with higher quality of care, and in some cases may be associated with care that is suboptimal.4

VARIATION STUDIES IN PEDIATRICS In many ways, the Atlas has formed the foundation for our understanding of variation in healthcare, but until recently had remained largely limited to

analyses of Medicare data from the adult population. The Atlas Project recently investigated geographic variation in pediatric health care across Maine, New Hampshire, and Vermont using data from commercial insurers and Medicaid. The Dartmouth Atlas of Children’s Health Care in Northern New England5 mimicked the adult analyses, reporting rates of utilization and spending for primary care, inpatient care, emergency room care, advanced imaging, surgical care, and medication use across regions and hospitals. In parallel with the growth of the Atlas, pioneers in hospital medicine have utilized health data systems in Boston, Rochester, and other US states to uncover wide variation in the management of inpatient conditions such as asthma, gastroenteritis, and lower respiratory tract infections.6-8 More recently, the broader availability of patient data from sources such as the Health Care Utilization Project (HCUP) Kids’ Inpatient Database (KID), and the Pediatric Health Information Systems Database have provided researchers with the opportunity to examine variation from the perspective of nationally representative pediatric patient samples.9-12

CONCEPTUAL FRAMEWORKS FOR VARIATION: UNWARRANTED OR UNEXPLAINED? The challenge of much of the work surrounding practice variation lies in defining what constitutes “unwarranted” differences in care. Variation in practice can occur for many legitimate reasons, leading some to argue that variation is only unwarranted if it deviates from evidence-based standards of care or has a significantly negative impact on outcomes. By these definitions, much of the observed variation in healthcare could be considered unexplained as opposed to unwarranted. This is especially the case for variation in the care provided to children, since there are few conditions with indisputable standards of care. Regardless of whether it is considered unwarranted or unexplained, a general framework for understanding variation and generating hypotheses about its sources and significance has emerged from the literature, and involves grouping care into three major categories: (1) supply-sensitive, (2) preference-sensitive, and (3) effective. Supply-sensitive care refers to healthcare services that appear to be utilized at higher rates in areas where there is greater availability of certain resources. In Wennberg’s early studies, this was demonstrated by

observations that patients living in areas with greater bed capacity were more likely to be hospitalized, patients living in areas with more surgeons were more likely to have a surgical procedure, and patients living in areas with more radiology services were more likely to get x-rays or Computerized Tomography Scans (CTs).13 The primary driver of this type of variation is felt to be the subtle influence of service availability on clinician decisionmaking in cases where the tradeoffs are not as well defined and choices to admit or test are at the discretion of the physician. An obvious negative effect of supply-sensitive variation is that it leads to greater resource utilization and higher per capita healthcare expenditures. Unfortunately, this high utilization has not been shown to lead to better outcomes or patient satisfaction, and often represents waste of healthcare dollars and lost opportunity costs. Many believe that with rapid growth of the healthcare system, greater system capacity will lead to rampant overuse of diagnostic and therapeutic services and may in fact be causing significant and as of yet under-measured patient harm.14,15 Fee-for-service payment systems are felt to be largely responsible for what has been unchecked overuse of supply-sensitive care. Accordingly, proposed strategies for reducing variation and lowering these costs involve payment model reforms such as transitioning to bundled payment systems, pay-for-performance, and implementing penalties for high utilizers of discretionary services. Accountable care organizations (ACOs) also show great potential for reducing variation in the use of hospital services. In the ACO model, healthcare organizations contract to provide comprehensive care to a population of patients for a fixed per-member price, and payments for each hospitalization are shared across all members of the hospital system’s medical staff, including providers responsible for the outpatient care of the child. ACOs are paid on a capitated basis and/or for achieving certain quality/cost targets, and thus are incentivized to more carefully manage the quality and utilization of care. In their early stages of development, some ACOs appear to be successful at reducing cost while improving quality, but more needs to be learned about which organizational factors most contribute to a successful ACO model.16 Preference-sensitive care entails the management of conditions for which there are several justifiable options for diagnosis and treatment. In some cases, there may be no evidence to support any single option. In others,

evidence does exist, but each option may involve important tradeoffs that should be considered on a patient-by-patient basis. One similarity between supply-sensitive and preference-sensitive care is the hypothesis that variation in physician practice styles, as opposed to differences in patient preferences, are the major driver of variation in care. While patient preferences will produce some degree of variation in care, management decisions such as opting to perform surgery on adults with chronic lower back pain appear to be clustered within health systems, making it highly unlikely that patient preferences alone are driving variation.17 The unbalanced influence of the physician on clinical decision-making has also been demonstrated for elective procedures in pediatrics. In a recent study by Fox et al., the decision to proceed with a fundoplication procedure in children with severe reflux was rarely based on definitive testing, and most physicians felt that family decisions to proceed with surgery were more strongly influenced by the preferences of subspecialists than by counseling from their primary provider.18 Since there is little evidence to guide the treatment of severe reflux, these decisions were likely guided by anecdotal experiences and the physicians’ own interpretation of existing literature rather than informed family preferences. Public reporting of quality ratings and outcomes will theoretically allow patients to become better informed on the tradeoffs involved with their medical decisions. However, interventions that occur closer to the point of care such as telephone coaching and use of decision aids may more effectively promote shared decision-making. In one study examining decision-making and outcomes for adults with lower back pain, use of these tools was associated with lower rates of preference sensitive procedures and lower rates of readmission for patients who had elected to have back surgery.19 For now, families continue to feel poorly informed of the negative consequences of different management options, and physicians often overestimate their alignment with patient preferences. Fortunately, federal and state efforts including policies within the Affordable Care Act and Medicare’s Pioneer Accountable Care Organization Program, as well as other government-supported programs such as the Centers for Medicare & Medicaid Services (CMS) Partnership for Patients, are now lending stronger support to shared decision-making as a tool for reducing variation in

management of preference-sensitive conditions. Effective care involves the management of conditions for which there is strong scientific evidence to support best practices. Although many inpatient conditions in pediatrics have limited evidence to support care, conditions such as asthma, urinary tract infections (UTIs), and community-acquired pneumonia (CAP) have evidence-based guidelines to support clinical decision-making. Most providers can agree that adherence to these guidelines is of great clinical importance and value to their patients, yet a high proportion of children continue to receive improper care for these conditions. For example, in a 2009 study examining the management of febrile UTIs in young children, Conway et al. found that 83% of children >2 months of age were admitted to the hospital for treatment despite American Academy of Pediatrics (AAP) recommendations for outpatient management in this population.9 A second study by Neuman et al. found that only 24% of children hospitalized for CAP received the recommended first-line antibiotic.20 Though many factors can contribute to deviations in the management of common conditions in children, these findings are too large to be attributed to patient differences alone. The first hurdle in reducing variation in effective care is overcoming the gap between scientific discovery and clinical practice. For conditions with high variation in effective care such as febrile UTIs and CAP, clinical practice guidelines (CPGs) have been an important tool for educating providers on evidence-based practice, and use of CPGs has resulted in improvements in adherence to care, reduced lengths of stay, and lower resource utilization. Even with CPGs, however, variation in adherence to effective care persists, indicating that these interventions alone are not always enough to change physician behavior. Additional tools have been developed to support implementation of CPGs and reduce variation in evidence-based care in clinical settings. The most widely studied CPG support tools are practice protocols and clinical pathways. Unlike CPGs, which typically only describe best practice and reference-supporting evidence, protocols and pathways are designed to enhance coordination of care and reliability in the execution of evidencebased care. Protocols are most appropriate for systematically guiding individual providers through treatment decisions by outlining specific evidence-based criteria under which a physician would or would not choose a

certain course of treatment (e.g. phototherapy vs. exchange transfusion for neonates with hyperbilirubinemia). Alternatively, clinical pathways aim to decrease variation in effective care by promoting the coordination of the care team in executing evidence-based management. A strength of pathways is that they often contain a built-in component for measuring defined outcomes (cost, treatment success, adherence) and providing feedback to clinicians. The literature contains several examples of both successful and unsuccessful applications of CPGs, pathways, and protocols for reducing variation in effective care, and it is likely that there is no one-size-fits-all strategy. In order to optimize their generalizability and effectiveness across hospital systems, future research will need to explore organizational factors that may act as barriers or facilitators to the effective implementation of these tools. On a national policy level, hospitals are being encouraged to learn from variation in effective care by forming partnerships with neighboring health centers. Using what is called a positive deviance approach, the CMSsponsored Hospital Engagement Networks (HENs) have committed to exploring factors that may facilitate high quality of care. By sharing information on processes of care, quality improvement tools, and implementation strategies, HENs work to collectively identify and implement standardized practices that consistently produce better outcomes. To date, HEN collaborations have minimized variation in care across networks and led to dramatic reductions in adverse events (http://www.jcrinc.com/CMSHospital-Engagement-Network).

VARIATION AND COMPARATIVE EFFECTIVENESS RESEARCH Even in the case of effective care, it may be difficult to determine what degree of variation is truly unwarranted, emphasizing the need to continuously monitor both processes and outcomes of care. In addition, variation in care may actually be informative, revealing new knowledge about what works and what doesn’t work in our traditional management. In this way, existing variation creates new opportunities for comparative effectiveness research (CER). With new resources and tools such as nationally representative administrative datasets, clinical registries, and more

advanced statistical methods, CER can now address clinical questions that previously would have been too costly and infeasible to study. Hospitalists in particular are utilizing these tools to produce high impact research— measuring important indicators of how hospitalists deliver care and generating hypotheses about which strategies may facilitate the most effective and highest value care. As this work moves forward, it will be important to define priorities for comparative effectiveness research in pediatric hospital medicine. Due to growing funding constraints and other challenges inherent to pediatric research (e.g. small sample sizes, relatively rare outcomes), the research community has a great responsibility to focus CER efforts on conditions for which the opportunity to impact patient outcomes and/or resource utilization is greatest. Strategies for determining high priority conditions will require knowledge of current disease prevalence, burden of illness, cost, gaps in evidence, feasibility of study, and importance of outcomes to patients. Currently, however, some or all of these measures are unknown for many pediatric conditions, and methods for efficiently and accurately defining them are still needed. One such method for ranking the priority of inpatient conditions used the PHIS database and a standardized costing method to identify the 100 most costly and prevalent pediatric inpatient conditions.21 The investigators then quantified the degree of variation in resource utilization for each of these costly and common conditions. Not surprisingly, conditions such as asthma, pneumonia, and gastroenteritis were found to be among the highest priority conditions. Also identified were conditions for which variation and the potential for clinical impact may have been underappreciated, such as diabetic ketoacidosis, scoliosis surgery, and tonsillectomy procedures. Researchers have begun to explore the potential drivers of variation in effective care for these high priority conditions. In one study looking at 12,605 children hospitalized with acute gastroenteritis (a condition with high variation in effective care) admission to hospitals that cared for fewer children with the illness was associated with a higher probability of having non-recommended blood testing or receiving antibiotics.22 In another study evaluating variation in prophylactic antibiotics in scoliosis surgeries at PHIS hospitals, several centers used broader spectrum antibiotics only in patients with diagnoses indicating higher presurgical risk of infection, others used

them for all patients, and still others rarely used them at all.23 In each case, variation in effective care could not be fully explained by hospital or patient factors and is therefore likely unwarranted. This variation, however, can be informative and has provided the basis for future research comparing the effectiveness of tools such as care pathways and antibiotic stewardship programs designed to reduce overutilization of hospital services and promote the appropriate use of evidence-based care.

CONTRIBUTIONS FROM HOSPITAL MEDICINE Given their understanding of complex hospital systems, specialists in pediatric hospital medicine are well suited to study variation and best practices in the delivery of pediatric care and to design system level interventions to reduce unwarranted variation and improve the value of care in their health systems. Networks such as the Pediatric Research in Inpatient Settings (PRIS) Network and the AAP’s Value in Inpatient Pediatrics (VIP) Network have made significant progress in these domains. As the field advances as an academic specialty, hospitalists involved in research and operational efforts to reduce and study variation have several important challenges to address. First, providers and hospital leaders need to advocate for payment reforms that incentivize high value care and reduce unwarranted variation in supply-sensitive care. Second, given diminishing availability of support from federal funders, hospitalists need to prioritize the subjects of their CER efforts, focusing efforts on conditions for which innovations in care will have a large impact on value and outcomes. Third, networks of researchers and providers need to continue to develop reliable and transparent data sources with which to longitudinally monitor processes and outcomes across health systems. Such data will promote a collective sense of accountability as well as encourage collaborative efforts to disseminate guidelines and reduce variation in effective care. Fourth, the scope of comparative effectiveness research needs to be broadened to account for the diversity and complexity of healthcare systems and gain a deeper understanding of the unique organizational barriers and facilitators to implementing effective and more patient-centered care. Finally, CER conducted by hospitalists needs to be methodologically rigorous, utilizing advanced methods for risk adjustment

and subpopulation analyses of observational data, and rigorous systematic reviews, and meta-analyses on existing data from the literature.

SUMMARY It is well known that healthcare services, resource utilization, quality, and outcomes vary tremendously across the United States. In some cases, this variation may be the result of appropriate decision-making and consideration of differing patient preferences, but much of this variation is likely unwarranted and unexplained by patient preferences or evidence-based practice. The consequences of unwarranted variation are profound, and include uncontrolled healthcare spending and large disparities in quality of care. Payment system reform, better tools for shared decision-making, public reporting of outcomes, and implementation of CPGs, pathways, and protocols all require more research to determine the most effective way to reduce variation, control cost, and optimize quality. However, variation also provides opportunities to study how we can improve our care, and offers many opportunities for comparative effectiveness research. Understanding the driving factors and consequences of variation—warranted and unwarranted—will be essential to improving value and reducing disparities in the care of hospitalized children in the US healthcare system.

REFERENCES 1. Glover JA. The Incidence of Tonsillectomy in School Children (Section of Epidemiology and State Medicine). Proc R Soc Med. 1938;31(10):1219-1236. 2. McPherson K, Wennberg JE, Hovind OB, Clifford P. Small-area variations in the use of common surgical procedures: An international comparison of New England, England, and Norway. N Engl J Med. 1982;307(21):1310-1314. 3. Wennberg JE, Freeman JL, Shelton RM, Bubolz TA. Hospital use and mortality among Medicare beneficiaries in Boston and New Haven. N Engl J Med. 1989;321(17):1168-1173. 4. Yasaitis L, Fisher ES, Skinner JS, Chandra A. Hospital quality and

intensity of spending: Is there an association? Health Aff (Millwood). 2009;28(4):w566-572. 5. Goodman DC, Morden NE, Ralston SL, Chang C, Parker DM, and Weinstein BA. The Darmouth Atlas of Children’s Health Care in Northern New England. Trustees of Dartmouth College 2013. Available at: http://www.dartmouthatlas.org/downloads/atlases/NNE_Pediatric_ Atlas_121113.pdf. 6. Perrin JM, Homer CJ, Berwick DM, Woolf AD, Freeman JL, Wennberg JE. Variations in rates of hospitalization of children in three urban communities. N Engl J Med. 1989;320(18):1183-1187. 7. McConnochie KM, Russo MJ, McBride JT, Szilagyi PG, Brooks AM, Roghmann KJ. Socioeconomic variation in asthma hospitalization: Excess utilization or greater need? Pediatrics. 1999;103(6):e75. 8. Homer CJ, Szilagyi P, Rodewald L, Bloom SR, Greenspan P, Yazdgerdi S, Leventhal JM, Finkelstein D, Perrin JM. Does quality of care affect rates of hospitalization for childhood asthma? Pediatrics. 1996;98(1):18-23. 9. Conway PH, Keren R. Factors associated with variability in outcomes for children hospitalized with urinary tract infection. J Pediatr. 2009;154(6):789-796. 10. Brogan TV, Hall M, Williams DJ, Neuman MI, Grijalva CG, Farris RW, Shah SS. Variability in processes of care and outcomes among children hospitalized with community-acquired pneumonia. Pediatr Infect Dis J. 2012;31(10):1036-1041. 11. Pati S, Lorch SA, Lee GE, Sheffler-Collins S, Shah SS. Health insurance and length of stay for children hospitalized with community-acquired pneumonia. J Hosp Med. 2012;7(4):304-310. 12. Patrick SW, Schumacher RE, Davis MM. Variation in lumbar punctures for early onset neonatal sepsis: A nationally representative serial crosssectional analysis, 2003-2009. BMC Pediatr. 2012;12:134. 13. Wennberg J, Gittelsohn. Small area variations in health care delivery. Science. 1973;182(4117):1102-1108. 14. Fisher ES, Wennberg JE. Health care quality, geographic variations, and the challenge of supply-sensitive care. Perspect Biol Med.

2003;46(1):69-79. 15. Fisher ES, Welch HG. Avoiding the unintended consequences of growth in medical care: How might more be worse? JAMA. 1999;281(5):446453. 16. Fisher ES, Shortell SM, Kreindler SA, Van Citters AD, Larson BK. A framework for evaluating the formation, implementation, and performance of accountable care organizations. Health Aff (Millwood). 2012;31(11):2368-2378. 17. Birkmeyer JD, Sharp SM, Finlayson SR, Fisher ES, Wennberg JE. Variation profiles of common surgical procedures. Surgery. 1998;124(5):917-923. 18. Fox D, Barnard J, Campagna EJ, Dickinson LM, Bruny J, Kempe A. Fundoplication and the pediatric surgeon: Implications for shared decision-making and the medical home. Acad Pediatr. 2012;12(6):558566. 19. Veroff D, Marr A, Wennberg DE. Enhanced support for shared decision making reduced costs of care for patients with preference-sensitive conditions. Health Aff (Millwood). 2013;32(2):285-293. 20. Neuman MI, Hall M, Hersh AL, Brogan TV, Parikh K, Newland JG, Blaschke AJ, Williams DJ, Grijalva CG, Tyler A, Shah SS. Influence of hospital guidelines on management of children hospitalized with pneumonia. Pediatrics. 2012;130(5):e823-830. 21. Keren R, Luan X, Localio R, Hall M, McLeod L, Dai D, Srivastava R. Prioritization of comparative effectiveness research topics in hospital pediatrics. Arch Pediatr Adolesc Med. 2012;166(12):1155-1164. 22. McLeod L, French B, Dai D, Localio R, Keren R. Patient volume and quality of care for young children hospitalized with acute gastroenteritis. Arch Pediatr Adolesc Med. 2011;165(9):857-863. 23. McLeod LM, Keren R, Gerber J, French B, Song L, Sampson NR, Flynn J, Dormans JP. Perioperative antibiotic use for spinal surgeries in US children’s hospitals. Spine (Phila Pa 1976). 2013;38(7):609-616.

CHAPTER

3

Evidence-Based Medicine Jonathan M. Mansbach

INTRODUCTION Ideal clinical care integrates a health care professional’s clinical experience, individual patient preferences and values, and current best clinical evidence. Every hospitalist has his or her own set of clinical skills and experiences and each patient has his or her own beliefs. The current best clinical evidence, however, is universal. How clinicians apply and explain this evidence to individual patients and integrate the evidence into care plans is the art of practicing evidence-based medicine (EBM). This chapter provides practical guidance on formulating questions and uses clinical examples to discuss how to efficiently and effectively use and search the medical literature.

FORMULATING QUESTIONS Caring for patients frequently generates clinical questions. A 1985 study found that general practitioners in an office-based practice formulated two important clinical questions for every three patients examined.1 Since there are millions of research articles in the world’s literature, finding relevant articles and assessing their quality can be time consuming. If a wellformulated clinical question is posed, the process of finding answers is easier. There are four parts of a clinical question: the patient population, the intervention being considered, the comparison group, and the measurable outcome. Including these four parts to formulate questions will help focus literature searches.2 A 7-month-old with bronchiolitis was admitted to your service last night. In considering how best to care for her, you formulate the following:

1. Patient population (includes patient problem)—in children < 2 years with bronchiolitis 2. Intervention (diagnostic test or treatment), Prognostic factor, or Exposure —do bronchodilators 3. Comparison group—compared with placebo 4. Measurable outcome—reduce hospital length of stay? Often, developing one critical clinical question will suffice, but a series of questions is sometimes required, especially when inpatients have complex medical problems. Clinical questions should be focused but not overly narrow, since too narrow a question may not have an answer, or may have an answer that does not apply to the individual patient. In pediatrics, defining the patient population is important. Many hospitalists care for patients ranging in age from infants to young adults, and judgments must be made if studies that do not include your patient’s age will apply. Frequently, a study in adults has been performed and the hospitalist must decide if the study applies to a particular pediatric or adolescent patient. Sometimes the question may not involve an intervention, and there may therefore be no comparison group to consider. For example, during morning rounds you hear about a 16-year-old female with anorexia nervosa admitted for bradycardia. You ask the question: In a female adolescent with a restrictive eating disorder and heart rate of 35, what is the risk of sudden cardiac death? This question considers the prognosis of a specific population. The measurable outcome chosen reflects the current clinical concerns, values, and preferences of the clinician and patient. When a child is admitted with right lower quadrant abdominal pain the clinician may be most concerned about how to best evaluate for appendicitis, but the patient and parent may be most concerned about pain control.

SEARCHING FOR BACKGROUND INFORMATION A solid comprehension of the disease process or syndrome usually must precede asking specific questions. Background information about pathophysiology, clinical presentation, treatment, complications, and outcomes may be found in print textbooks, review articles, or electronic textbooks. Print textbooks are valuable resources, particularly for well-

established information. For example, if a patient presents with a parapneumonic effusion, reading a chapter in a textbook will be helpful in understanding the underlying disease process. A textbook chapter, however, may not contain up-to-date information on management from recently published articles. If you require further background information about parapneumonic effusions, accessing electronic texts may be most helpful. You begin your reading by reviewing the topic of parapneumonic effusions in children on UpToDate (www.uptodate.com), an easily accessible electronic textbook that is frequently revised and contains good references. If you enter “parapneumonic effusion” in the search line, there is a reference entitled, “Management and prognosis of parapneumonic effusion and empyema in children.” You click on the article and note when the review was most recently updated and when it is due to be revised. In reading the review you remind yourself about the pathogenesis of pleural effusions and the differences between uncomplicated effusions, complicated effusions, and empyemas. There are 82 references, and each reference can be viewed by clicking on the title. Review articles are another starting place, and Medline searches can be focused to locate review articles by using the “limit” function. For example, if you type parapneumonic effusions.tw (word in title or abstract—see below) and limit the search to review articles and English language, there are 32 hits. The articles include a consensus guideline published in Chest, and Cochrane reviews. Note that a separate search would need to be conducted to find articles that specifically address the definition and management of empyema.

SEARCHING MEDLINE USING OVID After reminding yourself of definitions and reviewing data, if you have more specific questions, Medline is a good place to begin searching. Medline is a National Library of Medicine database derived from more than 4500 journals and includes over 10 million citations. PubMed, frequently used to access Medline, contains additional basic science and life science articles. Ovid is another means of accessing Medline and is the software referred to in this chapter. Before you begin searching it is helpful to understand how articles are indexed in Medline. One method of limiting the number of articles you wade

through is by attaching suffixes to the keyword. These suffixes allow you to search one journal, search by author, look for words in a title or abstract, or restrict the search by year of publication. Some useful suffixes are listed in Table 3-1. For example, if you search for articles related to bronchiolitis and type bronchiolitis.tw (searches for bronchiolitis as a textword in the title or abstract) you will have 4000 hits. Typing bronchiolitis.ti generates 1900 hits. TABLE 3-1

Suffixes

.ab

Word in Abstract

.au

Author

.jn

Journal

.ti

Word in title

.tw

Word in title or abstract

.yr

Year of publication

Medline is also indexed using Medical Subject Headings (MeSH) terms. The keyword you type will be mapped automatically to one of the subject headings. If you search for the term bronchiolitis, it will automatically be mapped to the MeSH terms: bronchiolitis, bronchiolitis obliterans, bronchiolitis viral, and bronchiolitis obliterans organizing pneumonia. If you include bronchiolitis and viral bronchiolitis, there are 2100 hits and only 9 overlapping articles. To see the diagram of how bronchiolitis is mapped to the MeSH terms, type tree bronchiolitis. If you receive too many articles to review, you may limit the search by using subheadings. Some helpful subheadings are listed in Table 3-2. For example, if you want to limit your search to articles published in 2003–2004 related to bronchiolitis therapy, you would limit the publication years to 2003–2004 (using the suffix .yr) and type bronchiolitis/dt (generates 46 articles) and also viral bronchiolitis/dt (generates 21 articles). TABLE 3-2

Subheadings

/ae

Adverse effects

/co

Complications

/di

Diagnosis

/dt

Drug therapy

/ep

Epidemiology

/hi

History

/th

Therapy

WHAT ABOUT THE QUALITY? Once you find articles through Medline, you still have to assess the quality of the studies and validity of the conclusions. To bypass this time-consuming step, you can change the database searched using Ovid, from Medline to EBM reviews. The EBM reviews contain American College of Physicians (ACP) Journal Club, Database of Abstracts of Reviews of Effects (DARE), Cochrane Central Register of Controlled Trials (CCTR), and Cochrane Database of Systematic Reviews (CDSR). If you find articles on the topic of interest in one of these four databases, the quality of the article or articles will be assessed for you. Each of the four databases is described below. The editors of the ACP Journal Club collection identify clinically relevant, methodologically sound articles from top journals and provide a commentary on the value of the article for clinical practice. If you search for bronchiolitis in the ACP, one interesting article is found reviewing dexamethasone for bronchiolitis. DARE contains critical assessments of systematic reviews covering diagnosis, prevention, rehabilitation, screening, and treatment. DARE is produced by the National Health Services’ Centre for Reviews and Dissemination at the University of York, England. If you search for bronchiolitis in DARE, six articles are found. The CCTR (Cochrane Controlled Trials Register) is a database of definitive controlled trials in the literature. The database is an unbiased source of data and information. The Cochrane group, National Library of

Medicine in Washington, DC, and Reed Elsevier of the Netherlands collaborate to identify relevant studies for inclusion in the database. The word bronchiolitis generates 239 articles in the CCTR. The CDSR includes Cochrane Collaboration’s regularly updated systematic reviews about therapy, intervention, or prevention. The Cochrane Collaboration was developed in 1992 on the premise that interventions would be more effective if based on recent evidence. The reviews are presented as complete reviews or protocols yet to be finished. The completed reviews have summary statements and graphs. There are 39 systematic reviews related to the word bronchiolitis.

OTHER WEB RESOURCES Other resources on the web include The National Guideline Clearinghouse (www.guidelines.gov). This is a database of evidence-based clinical practice guidelines (CPG) and related documents originally developed by the Agency for Healthcare Research and Quality (AHRQ). A search for bronchiolitis will yield a CPG from Cincinnati Children’s Hospital Medical Center. Another site, from the University of Michigan (www.med.umich.edu/ pediatrics), has put together a collection of critically appraised topics (CAT). These are short summaries and reviews of interesting articles in the literature. Many of the CATs are applicable to inpatient medicine. Another good site is www.intensivecare.com. This site has many EBM links and also has a searchable pediatric critical care medicine evidence-based journal club. Although the reviews deal with critical care, many are relevant for hospitalists.

VALIDITY The above databases and web sites may not address all of the problems a hospitalist will encounter, so having a basic understanding of how to assess an article’s validity is helpful. In Table 3-3, basic items are presented to help evaluate an article investigating a prognostic factor, a treatment, or a diagnostic test.2-4 TABLE 3-3

Validity

Prognosis The patient population should be defined and patients should be enrolled at a similar stage of illness. The follow-up rate should be high and patients need to be followed for enough time to allow for development of the chosen outcome. Better outcomes are objective and easily measured. Treatment The most valid treatment trials are randomized and blinded. The patients in each randomized group should be similar. An “intention-to-treat” analysis should be used; once the patient is randomized they are included in the analysis. The chosen outcomes are relevant, objective, and measurable. Diagnosis

The test being studied is explained in detail and compared to the best available standard. The cohort of patients tested is explained in detail and includes some measure of severity of illness.

APPLICABILITY How valid information is integrated into the care of individual patients is the art of practicing evidence-based medicine. For example, a double-blind randomized controlled trial has determined that when medicine Z is given to patients with bronchiolitis, it reduces the length of stay in the hospital. You determine that the study is valid, but wonder if the new study will apply to your individual patient. Does the population in the trial sufficiently match the age, past medical history, severity of illness, and so forth of your patient? Is it possible that your patient with congenital heart disease is different enough from the previously healthy children in the trial that the risk of treatment increases? Does drug Z interact with other medications, altering the risk/benefit ratio? Are there other social factors (financial constraints, beliefs about medicine, etc.) that should be considered?

PUTTING IT ALL TOGETHER

During morning rounds you are told about a 3-year-old child who has a history of present illness, physical exam, and chest radiograph consistent with the diagnosis of pneumonia with an effusion. You begin considering antibiotic choices, are wondering about further diagnostic tests, and are thinking about the merits of thoracentesis, video-assisted thorascopic surgery (VATS), and fibrinolytics. You decide you need more information and also want to review the most recent literature before making any decisions. You review background information on UpToDate (discussed earlier). After reviewing this information, you pose the following two questions: In toddlers with parapneumonic effusions does surgical management compared to nonsurgical management improve long-term lung function; and does it shorten length of stay? Entering parapneumonic effusion into the EBM review database generated three articles. One CDSR topic review investigated surgical (VATS) versus nonsurgical (chest tube drainage with streptokinase) management of pleural empyema. This topic review included only one randomized adult trial of 20 patients.5 Although it is not ideal to make recommendations from one small trial, the results of the trial demonstrated that the VATS group compared to the nonsurgical group had a significantly shorter length of stay in the hospital. The study does not mention long-term lung function. The possible benefits of shorter length of stay need to be weighed against the risks of general anesthesia and the necessity for transient one-lung ventilation due to the procedure. Additional risks and possible complications may not be apparent from such a small study. In addition, the hospitalist must decide if this adult study should apply to a pediatric patient. The process described (using UpToDate and Medline restricted to EBM reviews) will most likely take less than 30 minutes. In this 30-minute search not only will you have a better understanding of background information, but you will have also reviewed some of the most recent literature on the topic of interest. One of the drawbacks of EBM reviews, however, is the limited number of pediatric studies that have large sample sizes, are well designed, and apply to general pediatric inpatient medicine. Furthermore, the outcomes studied are not always relevant for the individual patient. Despite these drawbacks, searching for the most recent evidence always has merit. Having the knowledge that there are no studies adequately addressing the topic or problem at hand is informative in itself and will hopefully guide future research.

TEACHING EBM One difficulty for hospitalists who round with medical students, residents, and fellows is teaching how to practice EBM. Modeling for learners your evidence-based approach to caring for patients is one method. Another interesting method presented by Dr. David Sackett and colleagues is having an “evidence cart” during rounds. In this study, having quick and easy access to evidence altered patient care decisions and increased the incorporation of evidence into patient care.6 Effectively teaching specific evidence to fatigued residents and fellows is also difficult. However, the results of an interesting ambulatory study suggest that the learner-focused “one-minute preceptor model” may be a quick, effective method of teaching.7,8 As a group, hospitalists should not only continue to incorporate EBM into their practice, but also teach EBM to students, residents, fellows, and colleagues.

SUGGESTED READINGS Guyatt G. Users’ Guides to the Medical Literature: A Manual for EvidenceBased Clinical Practice, Third Edition. McGraw-Hill Publication. 2015. Moyer VA, Elliott E eds. Evidence Based Pediatrics and Child Health. London, UK: BMJ Books Blackwell Publishing; 2005. Sackett D. Evidence Based Medicine: How to Practice and Teach EBM. Churchill Livingstone; 4th edition, (17 Dec. 2010).

REFERENCES 1. Covell DG, Uman GC, Manning PR. Information needs in office practice: Are they being met? Ann Intern Med. 1985;103(4):596-599. 2. Moyer V, Elliott E, Davis R, et al., eds. Evidence Based Pediatrics and Child Health. London: BMJ Books; 2000. 3. Richardson WS, Wilson MC, Guyatt GH, Cook DJ, Nishikawa J. Users’ guides to the medical literature: XV. How to use an article about disease probability for differential diagnosis. Evidence-Based Medicine Working Group. JAMA. 1999;281(13):1214-1219.

4. Richardson WS, Wilson MC, Williams JW, Jr., Moyer VA, Naylor CD. Users’ guides to the medical literature: XXIV. How to use an article on the clinical manifestations of disease. Evidence-Based Medicine Working Group. JAMA. 2000;284(7):869-875. 5. Wait MA, Sharma S, Hohn J, Dal Nogare A. A randomized trial of empyema therapy. Chest. 1997;111(6):1548-1551. 6. Sackett DL, Straus SE. Finding and applying evidence during clinical rounds: The “evidence cart”. JAMA. 1998;280(15):1336-1338. 7. Irby DM, Aagaard E, Teherani A. Teaching points identified by preceptors observing one-minute preceptor and traditional preceptor encounters. Acad Med. 2004;79(1):50-55. 8. Aagaard E, Teherani A, Irby DM. Effectiveness of the one-minute preceptor model for diagnosing the patient and the learner: Proof of concept. Acad Med. Jan 2004;79(1):42-49.

CHAPTER

Overview: Quality of Care

4

Jeffrey Simmons, Paul Hain, and Michele Saysana

INTRODUCTION If the value of a healthcare system is defined as the quality of the system divided by its cost, then the value of care in the United States is very low compared to other developed nations. The United States spent nearly 18% of GDP in 2012 on health care, while the next highest spending among developed nations was 12%.1 It is estimated that more than $700 billion of healthcare spending each year in the United States is waste.2 In the measure of outcomes, the US healthcare system ranks 37th globally.3 Low quality divided by high cost equals a system that has great potential for improvement. Inpatient hospital care accounts for nearly one quarter of the annual amount spent on child health,4 with that figure climbing even higher when emergency department visits are added. Therefore pediatric hospitalists are in a position to exert strong influence and leadership upon improving the value of that care by increasing the quality and decreasing the cost. Examples of quality projects in hospitals that have improved value include reduction of catheter-associated bloodstream infections (CLABSI),5 reduction in mortality through rapid response teams,6 reduction in codes outside of the ICU,7 increases in hand washing,8 decreases in contaminated blood cultures,9 and decreases in identification band errors.10,11 Payers have already begun to increase pressure on hospital systems to deliver value by implementing payment penalties for care that is perceived to be of low quality. The Center for Medicare and Medicaid Services (CMS) has issued instructions to stop paying for CLABSIs and pressure ulcers that occur during a hospital stay. Additionally, despite the ambiguity of evidence that unplanned readmissions

are either preventable or a marker of poor quality in pediatrics,12,13 many state Medicaid plans have begun to penalize hospitals for readmission rates that are perceived to be high. Historically, one of the barriers to improved quality was that the return on investment did not accrue to the hospital or the physicians. For example, decreasing a hospital’s CLABSI rate would lower charges and payments, thus lowering the revenue stream to the hospital and increasing profits for the payer. Over the previous decade, many individual quality measures had payment penalties associated with them as an incentive to the hospitals to improve. However, with the passage of the Patient Protection and Affordable Care Act (PPACA) in 2010, the potential for the incentives around quality improvement to align with both the payer and the hospital increased dramatically. One of the cornerstones of the PPACA was the creation of vehicles known as accountable care organizations (ACOs) that allow institutions to take financial risk for a group of patients.14 By taking on that risk, the increased value in the system that is created by quality improvement can accrue to the institution bearing the risk, instead of an insurance company.

EVIDENCE-BASED MEDICINE AND CARE PRACTICE GUIDELINES Using evidence-based medicine and the diffusion of new advances to change physician practice is challenging.15 Recently, however, it is becoming accepted that evidence-based medicine and care practice guidelines represent an excellent way to increase utilization of proven therapy processes, which commonly increase quality and decrease cost (as discussed further in Chapter 3)

QUALITY IMPROVEMENT TOOLS Walter Shewart and W. Edwards Deming are generally thought of as the fathers of the modern era of quality improvement, as they introduced new theories and techniques for process control and quality assurance.16 Many of their techniques are still in use today. What follows is a brief description of the most valuable of those techniques for improving hospital care.

PLAN-DO-STUDY-ACT Also known as the PDSA cycle, Plan-Do-Study-Act describes the four phases of a quality improvement project. The “plan” phase is comprised of an analysis of the deficiencies of the system to be improved and the actions that will be taken to improve it. Once the plan is established, the “do” phase begins, and the plan is put into action. While those actions are taking place, predefined measurements are collected, and an analysis is undertaken to “study” how well the original plan is providing a remedy for the deficiencies in the system. After understanding where the original plan needs to be altered, those changes are “acted” upon, and the cycle continues.

STATISTICAL PROCESS CONTROL Statistical process control (SPC) is a statistical tool that allows for representation of data on a graph that helps users to understand what parts of processes are due to “natural” or “common cause” variation in systems, and what parts are due to changes that were made to a system, or “special cause.” With this method, variation in processes and systems can be understood in significantly less time than it would take to sample every single event, or to establish control groups,17 which is often an impossibility in a hospital. (Having a process run differently every other day just to study which way is better would be impossible for most processes in a modern hospital.) The fundamental tool in SPC is the control chart. The control chart takes a run chart (a series of measurements plotted in chronological order around a mean) and adds an upper control limit (UCL) and a lower control limit (LCL). These control limits are calculated by known formulas that rely on the measurement of variation inherent to the system. Points that fall outside of one of the control limits is considered to be a result of a “special cause”; something new in the system caused the change, as opposed to the normal random fluctuations one sees between the control limits.18 An example of an SPC chart for an improvement project in reducing identification bands defects in a children’s hospital is shown in Figure 4-1.

FIGURE 4-1. Statistical Process Control Chart of Defective ID Bands, November 07–May 08. (Reproduced with permission from Hain PD et al. An intervention to decrease patient identification band errors in a children’s hospital. Qual Saf Health Care. 2010;19(3):244-247. DOI: 10.1136/qshc.2008.030388. With permission from BMJ Publishing Group Ltd.) SPC charts are the simplest and most powerful tools in the quality improvement arsenal to track the effects of a PDSA cycle project. Although it is relatively simple to create an SPC chart using published formulas, many commercial programs are available that will create the appropriate charts when the user simply enters the collected data.

THEORY OF CONSTRAINTS The Theory of Constraints was introduced by Eli Goldratt in his novel The Goal,19 an allegory that tells the story of a manager whose plant is about to be closed. Through a series of encounters with a physicist, the manager creates rules that allow the system to reduce bottlenecks (constraints) and improve efficiency. In practical usage, this technique allows quality improvement efforts to be focused where they will return the most improvement.

Consider, for example, a desire to decrease IV drug delivery times during the night shift. A hypothetical pharmacy system might work like this (see Figure 4-2): The pharmacy printer which receives the order can print 10 orders per hour → the tech can mix 6 orders per hour → the labeling machine can label 20 orders per hour → the pharmacist can check 8 orders per hour → the delivery person can distribute 5 orders per hour.

FIGURE 4-2. Theory of Constraints algorithm. If a hospital were to start a quality improvement project without first knowing all of the steps and constraints, it might add another tech with the expectation that the process rate would move to 8 doses per hour, as that is what the pharmacist can check. However, since the delivery person can only deliver 5 doses per hour, no improvements in IV drug delivery times would actually be seen. Mr. Goldratt provides five specific steps for solving production problems in his novel, but they essentially boil down to the example above. One must find the tightest bottleneck (constraint) in the system and solve that one before solving any others, as solving any bottleneck other than the tightest one produces no value.

LEAN SIX SIGMA Lean and Six Sigma began as two separate methodologies that have merged into one way of thinking in its application in healthcare. Originally, Lean was concerned with the reduction of waste in the process steps of a production schema, while Six Sigma was invented by Motorola as a method of describing statistically how many defects per million were produced.20 That is to say, Lean makes a process more efficient, while Six Sigma makes the output of that process have fewer defects.

Clearly, both improved efficiency and fewer defects are laudable goals in delivering care in a hospital setting, and thus Lean Six Sigma has been combined into one overarching strategy. In general, the Lean part of a project focuses on what is known as a “value stream map,” which organizes all parts of the process into “value added” and “non-value added” steps. The steps that do not add value are considered waste, and there is an attempt to eliminate or minimize those steps. Lean itself has a standard group of solutions that are often effective to deploy in process redesign. The Six Sigma part of a project focuses on the rate defects in the product. (Six Sigma takes its name from a six standard deviation in a bell curve, which amounts to 3.4 defects per million opportunities.) Six Sigma is in many ways similar to the PDSA cycle, but with a control phase at the end. It relies on a routine of Define, Measure, Analyze, Improve and Control (DMAIC).21 Often the control phase is monitored by an SPC chart. An in-depth explanation of Six Sigma is beyond the scope of this text, but the understanding of how Lean and Six Sigma complement each other and can be used simultaneously is valuable to those pursuing quality improvement.

PATIENT SAFETY: A QUALITY OF CARE PROBLEM As defined by the Institute of Medicine (IOM), patient safety is “freedom from accidental injury” acquired from medical care leading to harm or death. The IOM has estimated that medical error contributes to 98,000 deaths annually, which placed medical error as the sixth leading cause of death.22,23 While the study that led to this estimate of deaths due to medical error has not been repeated, rates of medical error and patient harm have been evaluated and have not shown substantial improvement.24 Woods and colleagues estimate that about 70,000 children hospitalized annually experienced an adverse event and approximately 60% of these could be prevented.25 For many years after the IOM report, the focus for improving patient safety was on system redesign. The focus of decreasing patient harm and improving safety has shifted to balancing system factors with personal accountability.2628 Issues such as developmental level, reliance on adult caregivers, medical condition, and demographics make children more susceptible to medication errors and harm.29 Pediatric hospitalists understand the needs of children and the complexity of the healthcare system while also serving in leadership

positions, which puts them in the position to improve patient safety.

ORIGINS AND EPIDEMIOLOGY OF MEDICAL ERROR Understanding the cause and magnitude of adverse events is one of the first steps in developing improvements. Woods and colleagues conducted a study to determine the types of all adverse events and those that are preventable. Birth-related adverse events (32.2%), followed by diagnostic errors (30.4%) and medication-related adverse events (21.3), were the top three causes of preventable adverse events. Surgical causes only accounted for 3.5% of the preventable adverse events.25 Children with chronic conditions have an even higher rate of medical errors, found by Ahuja and colleagues in a 2006 study using the Kids’ Inpatient Database.30 While there have been improvements since the IOM report, these have largely focused on adults. Computerized physician order entry systems designed for adults have not been as effective in reducing medication errors in children because many errors in pediatric patients tend to be due to weightbased dosing errors,31 and because of other computerized system design problems. A systematic review conducted by Miller and colleagues found that most of the research conducted on medication error in children has been limited primarily to the domain of prescribing errors, and the authors put forth the challenge for future research in areas of dispensing, administering, and documenting of medication administration.32 Many factors may contribute to diagnostic errors. A survey by Singh and colleagues found that over half of the pediatricians who responded had made a diagnostic error at least once per month, and nearly half of the respondents had a diagnostic error that led to harm once or twice per year. The most common error made was diagnosing a viral illness as a bacterial illness.33 In a study by Zwaan, diagnostic errors leading to patient harm often transpired when deciding what laboratory testing was necessary. Physicians were often unaware that their decisions were incorrect.34 While medication errors and diagnostic errors are common causes of harm in pediatric patients, other hospital-acquired conditions also contribute to the overall scope of harm. Catheter-associated blood stream infections, catheter-associated urinary tract infections, surgical site infections, and ventilator-associated pneumonia are some of the hospital-acquired infections

which also lead to extended hospitalization along with increased morbidity and mortality.35-39 (See Chapter 5 for further information on infection control.) Falls, pressure ulcers, and venous thromboembolism acquired in the hospital are some other hospital-acquired conditions contributing to patient harm. Reason’s Swiss cheese model of error may also be helpful in understanding how risks persist even in the face of systems established to prevent harm. When an error occurs there are multiple protective barriers in place within the system to block the error from reaching the patient and causing harm. For example, if a physician writes an erroneous order, a nurse, clerk, the computer physician order entry system, pharmacist, or parent of a patient may interrupt it before it reaches the patient. None of these barriers is flawless and sometimes will allow an error to reach the patient and cause harm. At times systems also have what Reason described as latent conditions, in which weaknesses in the system result from decisions made for other reasons and may cause errors.40

IMPROVING PATIENT SAFETY Reducing harm and improving patient safety in pediatric patients is imperative for many reasons. Not only are external agencies such as CMS, the Joint Commission, state boards of health concerned with patient safety,41 parents are as well. When parents perceive issues with handoffs and transitions, they also have an increased need to be present and oversee their child’s medical care to prevent error.42 Some hospitals have conducted widescale cultural and system changes using quality improvement methodology to decrease serious harm events in children. Significant reductions in serious safety event rates have been demonstrated.43,44 This section discusses ways to improve patient safety and how hospitalists can play a role in these strategies. Systems to detect harm in individual hospitals are one of the first steps to improving patient safety. Voluntary reporting of incidents has not adequately reflected the actual number of medical errors including near misses and harm events. Hospitalists should continue to emphasize the need to report errors. To further quantify harm, some hospitals have used retrospective review with the Institute for Healthcare Improvement’s Global Trigger Tool or a pediatric version of this trigger tool. Trained reviewers such as nurses, pharmacists,

and physicians review charts to identify signs which prompt further review of the patient’s medical record to identify whether harm occurred.45,46 Further review of findings from trigger tools can help guide hospital’s efforts to reduce medication-related harm.45 In 2011, the American Academy of Pediatrics Steering Committee on Quality Improvement and Management and Committee on Hospital Care released a policy statement about patient safety and decreasing harm due to medical care. The committee recommended three main strategies. The first is to increase patient safety knowledge among pediatricians. Patient safety– focused sessions at conferences and learning collaborative at the local, regional, and national levels are ways to accomplish this. Hospitalists can participate in these and learn from one another, and commit to sharing this knowledge with their practice group and institutions in which they care for patients. Another recommendation is the use of “bundles” to decrease harm.41 The Institute for Healthcare Improvement (IHI) described bundle theory as a set of evidence-based interventions applied to a certain population of patients that when used together will result in better outcomes than if the interventions were applied separately.47 Some examples of the use of bundles have been applied to decrease central line–associated bloodstream infections and ventilator-associated pneumonia.49,37 Hospitalists have participated in bundle development in areas such as improving the discharge process.49,50 All of these projects have utilized Quality Improvement (QI) methodology to implement these bundles. Another way to view this recommendation is in terms of high reliability organization (HRO) theory. HROs are organizations such as aviation or the nuclear power industry which operate under high-risk conditions daily but achieve high safety records. Weick and Sutcliffe have found five characteristics that HROs share: sensitivity to operations, preoccupation with failure, deference to expertise, resilience, and reluctance to simplify.51 These organizations rely on all members of the organization to have a focus on safety by constantly looking for risk points and ways to mitigate risk. Baker and colleagues described how teamwork is a vital piece of HROs in healthcare settings.52 Hospitalists play a critical role in leading healthcare teams. Other examples of creating high reliability in hospitals include the use of checklists, standardized communication procedures, and time-outs prior to

procedures. The Joint Commission developed the Universal Protocol for Preventing Wrong Site, Wrong Procedure and Wrong Person Surgery and requires it be used by the their accredited institutions.53 Utilizing systems which decrease medication errors due to human factors, such as bar code scanning for medication administration, are another example of mitigating risk. Promoting patient safety with a focus on the unique risk for pediatric patients is the last strategy.41 By practicing family-centered care, hospitalist teams can partner with patients and families to empower them to speak up at any time. The Agency for Healthcare Research and Quality has resources to help patients and families partner with their physicians to improve patient safety.54 Hospitalists should also advocate for pediatric-specific safety interventions such as pediatric-specific drug catalogs and weight-based dosing for CPOE and electronic prescribing.55 Leadership scholars Kouzes and Posner stress that leadership is best accomplished when there is a shared vision and all team members realize they have leadership potential.56 Hospitalists at all levels need to realize they have leadership potential within their local institution and more broadly to advocate for safety for all children.

REFERENCES 1. Organisation for Economic Co-operation and Development (OECD). How Does the United States Compare? 2012. Available at: http:// www.oecd.org/unitedstates/BriefingNoteUSA2012.pdf. Accessed June 29, 2013. 2. Kelly R. Where Can $700 Billion in Waste Be Cut Annually from the US Health Care System? White Paper. Oct, 2009. Thompson Reuters. 3. WHO. World Health Report 2000—Health Systems: Improving Performance. ISBN 92 4 156198 X. 4. Health Care Cost Institute. July 2012. Trends in Children’s Health Care Costs and Utilization. Available at: http://www.healthcostinstitute.org/ files/HCCI_RB1.pdf. Accessed June 29, 2013. 5. Pronovost P, Needham D, Berenholtz S, et al. An intervention to decrease catheter-related bloodstream infections in the ICU. N Engl J

Med. 2006;355:2725-2732. 6. Sharek PJ, Parast LM, Leong K, et al. Effect of a rapid response team on hospital-wide mortality and code rates outside the ICU in a children’s hospital. JAMA. 2007;298(19):2267-2274. 7. Brilli RJ, Gibson R, Luria JW, et al. Implementation of a medical emergency team in a large pediatric teaching hospital prevents respiratory and cardiopulmonary arrests outside the intensive care unit. Pediatr Crit Care Med. 2007;8(3):236-246. 8. Zerr DM, Allpress AL, Heath J, Bornemann R, Bennett E. Decreasing hospital-associated rotavirus infection: a multidisciplinary hand hygiene campaign in a children’s hospital. Pediatr Infect Dis J. 2005;24(5):397403. 9. Hall RT, Domenico HJ, Self WH, Hain PD. Reducing the blood culture contamination rate in a pediatric emergency department and subsequent cost savings. Pediatrics. 2013;131(1):2012-1030. 10. Hain PD, Joers B, Rush M, et al. An intervention to decrease patient identification band errors in a children’s hospital. Qual Saf Health Care. 2010;19(3):244-247. 11. Phillips SC, Saysana M, Worley S, Hain PD. Reduction in pediatric identification band errors: A quality collaborative. Pediatrics. 2012;129(6):2011-1911. 12. Hain PD, Gay JC, Berutti TW, Whitney GM, Wang W, Saville BR. Preventability of early readmissions at a children’s hospital. Pediatrics. 2013;131(1):2012-0820. 13. Srivastava R, Keren R. Pediatric readmissions as a hospital quality measure. JAMA. 2013;309(4):396-398. 14. Integrated Healthcare Association. Accountable Care Organization Provisions in the Patient Protection and Affordable Care Act. Available at: http://www.iha.org/pdfs_documents/home/IHA_ PPACAACOSummary.pdf. Accessed on line June 29, 2013. 15. Lomas J, Anderson GM, Domnick-Pierre K, Vayda E, Enkin MW, Hannah WJ. Do practice guidelines guide practice? The effect of a consensus statement on the practice of physicians. N Engl J Med. 1989;321(19):1306-1311.

16. American Society for Quality. W. Edwards Deming. Available at: http:// asq.org/about-asq/who-we-are/bio_deming.html. Accessed June 29, 2013. 17. Wheeler DJ. Understanding Statistical Process Control. 2nd ed. Knoxville, TN: SPC Press; 1992. 18. Benneyan JC. Statistical process control as a tool for research and healthcare improvement. Qual Saf Health Care. 2003;12:458-464. 19. Goldratt EM. The Goal. 3rd rev. ed. Great Barrington, MA: The North River Press; 2004. 20. Chassin MR. Is health care ready for Six Sigma quality? Milbank Quart. 1998;76:565-591. 21. de Koning H, Verver J, van den Heuvel J, Bisgaard S, Does R. Lean six sigma in health care. J Healthc Qual. 2006;4-11. 22. Kohn LT, Corrigan J, Donaldson MS. To Err Is Human: Building a Safer Health System. Washington, DC: National Academy Press. 2000; xxi, 287 p. 23. Murphy SXJK, KD, Deaths: Final Data for 2010. Vital Stat Rep. 2013;61(4):1-168. 24. Landrigan CP, Parry GJ, Bones CB, Hackbarth AD, Goldmann DA, Sharek PJ. Temporal trends in rates of patient harm resulting from medical care. N Engl J Med. 2010;363(22):2124-2134. 25. Woods D, Thomas E, Holl J, Altman S, Brennan T. Adverse events and preventable adverse events in children. Pediatrics. 2005;115(1):155-160. 26. Wachter RM, Pronovost PJ. Balancing “no blame” with accountability in patient safety. N Engl J Med. 2009;361(14):1401-1406. 27. Goldmann D. System failure versus personal accountability—the case for clean hands. N Engl J Med. 2006;355(2):121-123. 28. Marx D. Patient Safety and the “Just Culture”: A Primer For Health Care Executives. New York: Columbia University; 2001. 29. Santell JP, Hicks R. Medication errors involving pediatric patients. Jt Comm J Qual Patient Saf. 2005;31(6):348-353. 30. Ahuja N, Zhao W, Xiang H. Medical errors in US pediatric inpatients with chronic conditions. Pediatrics. 2012;130(4):e786-793.

31. Han YY, Carcillo JA, Venkataraman ST. Unexpected increased mortality after implementation of a commercially sold computerized physician order entry system. Pediatrics. 2005;116(6):1506-1512. 32. Miller MR, Robinson KA, Lubomski LH, Rinke ML, Pronovost PJ. Medication errors in paediatric care: A systematic review of epidemiology and an evaluation of evidence supporting reduction strategy recommendations. Qual Saf Health Care. 2007;16(2):116-126. 33. Singh H, Thomas EJ, Wilson L, et al. Errors of diagnosis in pediatric practice: A multisite survey. Pediatrics. 2010;126(1):70-79. 34. Zwaan L, Thijs A, Wagner C, van der Wal G, Timmermans DR. Relating faults in diagnostic reasoning with diagnostic errors and patient harm. Acad Med. 2012;87(2):149-156. 35. Yogaraj JS, Elward AM, Fraser VJ. Rate, risk factors, and outcomes of nosocomial primary bloodstream infection in pediatric intensive care unit patients. Pediatrics. 2002;110(3):481-485. 36. Pittet D, Tarara D, Wenzel RP. Nosocomial bloodstream infection in critically ill patients. Excess length of stay, extra costs, and attributable mortality. JAMA. 1994;271(20):1598-1601. 37. Bigham MT, Amato R, Bondurrant P, et al. Ventilator-associated pneumonia in the pediatric intensive care unit: Characterizing the problem and implementing a sustainable solution. J Pediatr. 2009;154(4):582-587 e2. 38. Ryckman FC, Schoettker PJ, Hays KR, et al. Reducing surgical site infections at a pediatric academic medical center. Jt Comm J Qual Patient Saf. 2009;35(4):192-198. 39. Brindha SM, Jayashree M, Singhi S, Taneja N. Study of nosocomial urinary tract infections in a pediatric intensive care unit. J Trop Pediatr. 2011;57(5):357-362. 40. Reason J. Human error: Models and management. BMJ. 2000;320(7237):768-770. 41. Steering Committee on Quality Improvement and Management and Committee on Hospital Care. Policy statement—principles of pediatric patient safety: Reducing harm due to medical care. Pediatrics. 2011;127(6):1199-1210.

42. Cox ED, Carayon P, Hansen KW, et al. Parent perceptions of children’s hospital safety climate. BMJ Qual Saf. 2013;22(8):664-671. 43. Muething SE, Goudie A, Schoettker PJ, et al. Quality improvement initiative to reduce serious safety events and improve patient safety culture. Pediatrics. 2012;130(2):e423-431. 44. Peterson TH, Teman SF, Connors RH. A safety culture transformation: Its effects at a children’s hospital. J Patient Saf. 2012;8(3):125-130. 45. Takata GS, Mason W, Taketomo C, Logsdon T, Sharek PJ. Development, testing, and findings of a pediatric-focused trigger tool to identify medication-related harm in US children’s hospitals. Pediatrics. 2008;121(4):e927-e935. 46. Kirkendall ES, Kloppenborg E, Papp J, et al. Measuring adverse events and levels of harm in pediatric inpatients with the Global Trigger Tool. Pediatrics. 2012;130(5):e1206-e1214. 47. Resar RGF, Haraden C, Nolan TW. Using care bundles to improve health care quality. In: IHI Innovation Series White Paper. Cambridge, MA: Institute for Healthcare Improvement; 2012. 48. Miller MR, Griswold M, Harris JM, et al. Decreasing PICU catheterassociated bloodstream infections: NACHRI’s quality transformation efforts. Pediatrics. 2010;125(2):206-213. 49. Jack BW, Chetty VK, Anthony D, et al. A reengineered hospital discharge program to decrease rehospitalization: A randomized trial. Ann Intern Med. 2009;150(3):178-187. 50. Project BOOST Team. The Society of Hospital Medicine Care Transitions Implementation Guide. Project BOOST: Better Outcomes by Optimizing Safe Transitions. June 29, 2013. Available at: http:// www.hospitalmedicine.org. 51. Weick KE, Sutcliffe KM. Managing the Unexpected Resilient Performance in an Age of Uncertainty. San Francisco, CA: Jossey-Bass; 2007. 52. Baker DP, Day R, Salas E. Teamwork as an essential component of high-reliability organizations. Health Serv Res. 2006;41(4 Pt 2):15761598. 53. The Joint Commission. Facts about the Universal Protocol. Available

at: http://www.jointcommission.org/assets/1/18/Universal_Protocol_1_ 3_13.pdf. Accessed June 28, 2013. 54. AHRQ. 20 Tips to Help Prevent Medical Errors: Patient Fact Sheet. September 2011. Available at: https://archive.ahrq.gov/patientsconsumers/care-planning/errors/20tips/. Accessed April 27, 2017. 55. Johnson KB, Lehmann CU, Council on Clinical Information Technology of the American Academy of Pediatrics. Electronic prescribing in pediatrics: Toward safer and more effective medication management. Pediatrics. 2013;131(4):824-826. 56. Kouzes JM, Posner BZ. The Leadership Challenge: How to Make Extraordinary Things Happen in Organizations. 5th ed. San Francisco, CA: Jossey-Bass. 2012; xiii, 394 p.

CHAPTER

5

Infection Control for Pediatric Hospitalists Julia S. Sammons and Susan E. Coffin

INFECTION CONTROL: A PATIENT SAFETY ISSUE Nosocomial infections are the single most common adverse event experienced by hospitalized children and adults. A recent multicenter survey found that 6% of patients develop nosocomial infections during admission to an acute care hospital.1 A point prevalence study performed in 30 pediatric facilities in Canada found an 8.7% prevalence of healthcare-associated infections and the prevalence was greatest for patients in intensive care units and oncology wards.2 Healthcare-associated infections increase morbidity, extend hospital stays, and raise hospital charges, and they are also associated with substantial increases of in-hospital mortality. An analysis of discharge data from more than 5 million pediatric hospitalizations revealed that postoperative sepsis and infection as a result of medical care were common events among hospitalized children and had a remarkable impact on length of stay and hospital costs.3 These findings persisted even after adjustment for patient and hospital characteristics (Table 5-1). Additionally, patients with a healthcareassociated infection were more likely to require the use of isolation precautions and antimicrobial therapy.2 TABLE 5-1

Impact of Nosocomial Infections in Hospitalized Children Mean Increase in

Mean Increase in Hospital

Mean Increase in Hospital

Length Stay Charges (Days) (US$)

Mortality (OR)

30

121,010

2.2

Postoperative 26 sepsis

117,815

11

Infection as a result of medical care

Source: Data from Miller MR, Zhan C: Pediatric patient safety in hospitals: A national picture in 2000. Pediatrics. 2004;113:1741. OR, odds ratio.

Thus the risk of healthcare-associated infections is significant, and the consequences are great. It is critical that all members of a healthcare team remain vigilant in order to prevent their patients from acquiring nosocomial infections during hospitalization.

PREVENTING INFECTION To prevent nosocomial infections, healthcare providers must understand how organisms are transmitted, both between individuals and from the environment or an inanimate object to a patient, how and when colonizing organisms (often referred to as commensal organisms) can become pathogenic, and how host and environmental factors modify the risk of nosocomial infection.

HOW ORGANISMS ARE TRANSMITTED Three basic mechanisms explain how most microorganisms are transmitted from one person to another: contact, either direct or indirect; droplet transmission; and airborne spread. Contact is the most common route by which the vast majority of bacteria and viruses are spread among patients and healthcare workers. Viruses, such as respiratory syncytial virus (RSV), and bacteria, such as methicillinresistant Staphylococcus aureus (MRSA), are typically spread directly from one person to another, particularly when infected or colonized children play

together in hospital playrooms. Hands, particularly those of healthcare workers, are another critical method by which potentially pathogenic organisms spread between patients. Indirect contact or fomite transmission is yet another common way that organisms, especially those capable of surviving for long periods on inanimate objects, can spread within the hospital setting. Because many viruses such as RSV can survive in dried secretions for hours, commonly touched items, like bed rails, can become reservoirs for transmission in the absence of thorough disinfection between patients. Respiratory droplets are responsible for the transmission of many common pediatric pathogens, including influenza viruses. Large respiratory droplets that contain viral particles are expelled from the nose and mouth during coughing, sneezing, and talking or during procedures such as suctioning, bronchoscopy, and cough induction by chest physiotherapy. These droplets can travel 3 to 6 feet in the air before settling. Transmission typically occurs when droplets come into contact with mucous membranes. Thus, face-to-face encounters with infected individuals allow the transmission of many viral pathogens in the absence of direct physical contact. Inadvertent inoculation of the conjunctival mucosa has been shown to be an important route of transmission for viruses. Airborne transmission occurs by the dissemination of droplet nuclei (small particles ≤ 5 μm), which are evaporated droplets that contain infectious microorganisms. These droplet nuclei can remain suspended in the air for long periods, become airborne, and travel significant distances from their point of origin. Organisms such as Mycobacterium tuberculosis (MTB), varicella virus, and measles virus can survive desiccation and exist as droplet nuclei. The outbreak potential for these organisms is great.

HAND HYGIENE AND STANDARD PRECAUTIONS Hand hygiene is the most critical element in the prevention of nosocomial infections. Sadly, studies have repeatedly shown that healthcare workers frequently fail to clean their hands at the appropriate times while providing patient care.5 Thus, healthcare workers are one of the most common sources of transmission of infection among patients. Hands should be washed with soap and water before and after eating, after using the bathroom, and when

they are visibly soiled.4 At all other times, healthcare providers should use alcohol-based hand rubs. The World Health Organization specifies five opportunities for hand hygiene that may arise in clinical care: (1) before contact with the patient; (2) before an aseptic procedure is performed; (3) after blood and body fluid exposure; (4) after contact with the patient; and (5) after contact with the patient’s environment. One of the most common errors made by clinicians is failing to perform hand hygiene before donning and after removing gloves. Alcohol hand rubs are more effective than soap and water at reducing microbial colonization of the hands.6 In addition to performing hand hygiene before and after every patient contact, all healthcare workers should observe standard precautions with every patient. These are transmission-based precautions designed to protect healthcare workers from exposure to any known or unknown pathogens that might be transmitted by contact with blood or body fluids. Critical elements of standard precautions include: (1) hand hygiene; (2) use of gloves when touching blood, body fluids, mucous membranes, or nonintact skin; and (3) mask, gown, and eye protection during procedures that might result in sprays of blood or body fluids.

TRANSMISSION-BASED PRECAUTIONS AND THE USE OF PERSONAL PROTECTIVE EQUIPMENT In addition to standard precautions, transmission-based precautions markedly reduce the risk of spread of many common agents of healthcare-associated infections (Table 5-2).7 Because many community-acquired pediatric pathogens are easily spread in inpatient units, the use of transmission-based precautions is especially important for pediatric facilities. TABLE 5-2

Organism

Summary of Expanded Precautions for Selected Pathogens Precautions* Comments

Viruses Adenovirus

C+D

C only for patients with isolated

conjunctivitis or gastroenteritis Enterovirus

C

Influenza virus

D

Norovirus

C

Parainfluenza virus

C

Respiratory syncytial virus

C

Rotavirus

C

Rubeola virus (measles)

A

Varicella virus

C+A

Continue until all lesions are crusted; C only for immunocompetent patients with zoster

Bacteria Antibioticresistant organisms†

C

Bordetella pertussis

D

Continue for 5 days after initiation of appropriate therapy

Clostridium difficile

C

Continue until resolution of diarrhea

Mycobacterium A tuberculosis Mycoplasma

D

Only required for suspected cavitary, laryngeal, or miliary disease

pneumoniae Neisseria meningitidis

D

Continue for 24 h after initiation of appropriate therapy

*Specific recommendations for the proper use of personal protective equipment and patient placement can be found at http://www.cdc.gov/HAI/prevent/ppe.html. †Including

methicillin-resistant Staphylococcus aureus, vancomycin-resistant enterococci, and pan-resistant gram-negative organisms, including extended β-lactamase producers. A, airborne precautions; C, contact precautions; D, droplet precautions.

Some infections can be spread in multiple ways, necessitating the simultaneous application of multiple types of precautions (e.g. contact and airborne precautions for primary varicella infection). Special air handling and ventilation, as well as respiratory protection with a National Institute for Occupational Safety and Health–approved N-95 or higher respirator mask, are required to prevent airborne transmission of some microorganisms, such as measles, tuberculosis, and varicella. Healthcare workers must recognize the need for transmission-based precautions and be familiar with the appropriate use of personal protective equipment. For example, there is convincing evidence that the use of barrier precautions can reduce the transmission of MRSA. In an investigation that included weekly cultures of specimens from patients and personnel, molecular typing of isolates, and decolonization of organisms in some patients, investigators found that MRSA was spread to other patients at a rate of 0.14 transmission per day when patients who had been colonized or infected with MRSA were not cared for under contact isolation precautions. In contrast, when healthcare workers caring for patients with MRSA used gowns and gloves, the rate of MRSA transmission to other patients was 0.009 transmission per day. Thus, the risk of transmission was reduced nearly 16fold when MRSA patients were cared for under contact isolation precautions.8

MINIMIZING THE RISK ASSOCIATED WITH MEDICAL DEVICES Medical devices greatly increase the risk of nosocomial infection. Central venous catheters, urinary catheters, and endotracheal tubes all provide portals

of entry that permit organisms to migrate from the skin and mucous membranes to sterile body sites. Implanted devices can also disrupt host defenses and provide a site sequestered from the surveillance of the immune system in which bacteria can flourish. Thus, strict adherence to aseptic technique when placing or manipulating a medical device is crucial to prevent device-related infection. The following sections outline additional strategies to reduce the risk of healthcare-associated infections in patients who require advanced medical technologies.

CENTRAL VENOUS CATHETERS Catheter-associated infections include localized infection at the site of catheter entry, phlebitis, and bloodstream infections. The last is among the most common type of healthcare-associated infection in hospitalized children, and the majority of these infections occur in patients with central venous catheters. Although the risk of bloodstream infection is greatest among patients with nontunneled central venous catheters, all vascular catheters, including subcutaneous ports and peripheral intravenous catheters, are associated with an increased risk of infection. Practices associated with a reduced risk of catheter-associated infection can be grouped into two major categories. First, adherence to proper insertion technique is critical and includes (1) use of maximal sterile barrier precautions (e.g. cap, mask, sterile gown, sterile gloves, and large sterile drape) during catheter placement; (2) use of 2% to 3% chlorhexidine gluconate-70% isopropyl alcohol or other appropriate antiseptic agents to prepare the skin before placement; and (3) appropriate hand hygiene prior to placement. Recent studies have demonstrated that many catheter-associated bloodstream infections develop more than one week after catheter placement, suggesting that the techniques used to access and maintain catheters are also critical to the prevention of these infections. Maintenance practices associated with reduced risk of catheter-associated bloodstream infections include (1) appropriate hand hygiene prior to manipulating a catheter or changing a dressing; (2) aseptic technique whenever a catheter is accessed or opened (for medication administration or cap changes); (3) aseptic technique for dressing changes; and (4) maintenance of a secure dressing over the catheter entrance sites. Finally, prompt removal of catheters when they are no longer required has also been demonstrated to reduce the risk of catheter infections.9 Other

interventions being adopted more frequently in pediatric settings include the use of antimicrobial-impregnated catheters and daily baths with chlorhexidine gluconate.

URINARY CATHETERS Similar to vascular catheters, the use of urinary catheters is associated with an increased risk of urinary tract infection. Experts estimate that catheterassociated urinary tract infections are the most common device-associated infection among hospitalized patients, although the burden of disease is likely greater in adult than in pediatric patients. Inappropriate and prolonged use of urinary catheters has been noted in as many as 50% of patients who develop catheter-associated urinary tract infections. Guidelines have focused on several practices that can reduce the risk of these infections.10 First, catheters should be placed in a sterile fashion. Second, a closed system for urine collection should always be maintained and urine should never be allowed to reflux from the collecting system or reservoir bag back into the bladder. Finally, the use of urinary catheters should be minimized by prompt removal whenever possible.

VENTILATORS An endotracheal tube provides an ideal portal of entry for the numerous organisms that colonize the oropharynx, allowing their migration to the lower respiratory tract. An artificial airway also provides an ideal substrate for the formation of biofilm and inhibits host defenses, such as the gag reflex and cilia function. Pediatric patients appear to be at less risk of ventilatorassociated pneumonia than adults, likely because they have fewer comorbid conditions such as chronic heart or lung disease or immunosuppressive conditions. However, pediatric intensive care physicians have developed strategies that reduce the risk of ventilator-associated pneumonia, including (1) use of noninvasive ventilation, (2) avoidance of nasotracheal intubation, (3) use of in-line suctioning to prevent aspiration of pooled tracheal secretions, (4) elevation of the head of the bed 45 degrees from horizontal, and (5) regular oral care.11 Finally, strategies that shorten the duration of intubation, such as weaning protocols and daily assessment of readiness for

extubation, can also reduce the risk of ventilator-associated pneumonia.

MANAGING PATIENTS WITH PROBLEM PATHOGENS ANTIBIOTIC-RESISTANT BACTERIAL ORGANISMS Within 3 years of the introduction of penicillin, some strains of Staphylococcus aureus developed resistance to this drug. By the 1960s, some strains of S. aureus had become resistant to methicillin, a semisynthetic penicillin. Over the past decade the epidemiology of MRSA has changed from an infection of primarily hospitalized patients to a relatively common cause of infection among otherwise healthy children in the community. Community-associated MRSA strains are now endemic in many institutions, posing a challenge to infection control professionals. Hospital epidemiologists still debate whether all patients admitted to the hospital should be screened to identify and isolate those colonized with MRSA.12 Contact precautions are commonly used to prevent patient-to-patient transmission of specific antibiotic-resistant bacteria. Because colonization with these organisms often persists for many months—even in the absence of ongoing exposure to antibiotics—precautions may be continued indefinitely for patients known to harbor organisms such as vancomycin-resistant enterococci and multidrug-resistant gram negative bacilli.

CLOSTRIDIUM DIFFICILE INFECTION The incidence of C. difficile infection (CDI) has increased dramatically over the past decade in conjunction with the emergence of a hypervirulent strain of C. difficile, referred to as the NAP1 strain. C. difficile is the most common cause of healthcare-associated diarrhea among adults in the United States, but is also a growing problem among hospitalized children. Several studies using national data have shown a rise in CDI-related hospitalizations among pediatric patients.15 C. difficile is transmitted between patients through either direct or indirect contact, and can be found on the hands of healthcare workers as well as in the patient’s immediate environment. The ability of C. difficile to form spores enables the bacteria to survive on environmental surfaces for prolonged

periods. Spores are also relatively resistant to disinfectants, posing a unique challenge in preventing spread of infection within healthcare facilities. Contact precautions should be used while caring for patients with CDI and continued until diarrhea has resolved. Strict adherence to hand hygiene and vigorous environmental cleaning is also important. Of these measures, the use of gloves has been shown to be the most effective measure in preventing C. difficile transmission.16 The use of dilute bleach for room disinfection may be an important adjunct to routine environmental cleaning while managing patients with CDI, particularly in outbreak settings.

MYCOBACTERIUM TUBERCULOSIS Unlike adults, many children infected with MTB are not considered contagious. Several factors explain the low rate of communicability associated with pediatric MTB infection.13 First, most children infected with MTB have latent infections and harbor small numbers of organisms that are well sequestered in granulomas. Second, children with active MTB infection rarely have endobronchial or cavitary lesions that communicate with the lower airways. Finally, young children typically do not generate sufficient intrathoracic pressure during coughing to raise MTB organisms into the oropharynx. As a result, healthcare-associated tuberculosis in pediatric facilities occurs almost exclusively due to transmission from infected adults (i.e. parents, visitors, and healthcare workers). Although airborne precautions are not routinely used for pediatric patients with MTB infection in many institutions because of the factors mentioned above, they may be indicated to prevent possible transmission from infected family members and visitors until their diseases status is verified. Airborne precautions (including patient placement in a negative-pressure room and the use of N-95 respirator masks by healthcare providers) should be instituted for pediatric patients with suspected endobronchial or cavitary lesions or miliary disease. Precautions should be continued until the patient is demonstrated to have no acid-fast organisms visible on three consecutive induced sputum or gastric aspirates.

PERTUSSIS

Outbreaks of pertussis in pediatric hospitals are well documented, and have often been related to spread by healthcare workers. During 2012, there was a dramatic increase in the number of pertussis cases reported throughout the country, underscoring the importance of control measures to prevent spread within pediatric facilities.17 The prevention of healthcare-associated pertussis includes (1) appropriate isolation and use of droplet precautions for children in whom infection is suspected, (2) antimicrobial treatment of confirmed pertussis cases, (3) chemoprophylaxis of exposed individuals, and (4) vaccination of healthcare workers through booster doses of pertussis vaccine (Tdap). Hospital staff with symptoms suggestive of pertussis should be evaluated promptly, and infected individuals should be excluded from work until the first 5 days of appropriate therapy have been completed.

VIRAL PATHOGENS During seasonal outbreaks, common pediatric viral pathogens can pose a significant risk to hospitalized children.14 Transmission of organisms such as RSV, influenza virus, norovirus, or rotavirus is facilitated in inpatient pediatric units because of the relative concentration of susceptible subjects (i.e. the patients), ongoing introduction of virus from the community (by visitors, staff, and newly admitted patients), and environmental contamination with organisms that can live for hours on fomites. Hospital outbreaks of RSV and rotavirus have been associated with insufficient staffing, sub-optimal environmental cleaning, and illness among visitors and staff, as well as poor hand hygiene practices by healthcare workers. Contaminated environmental surfaces are a particularly important contributor to transmission of healthcare-associated norovirus and may require additional measures for appropriate disinfection during outbreak settings.18

CONCLUSION Healthcare-associated infections are common and serious complications among pediatric patients. However, many of these infections are preventable through the appropriate use of standard and transmission-based precautions, uniform policies and procedures related to the insertion and maintenance of medical devices, and scrupulous attention to hand hygiene before and after

patient care, between procedures, and when in contact with the patient’s immediate environment.

REFERENCES 1. Magill SS, Hellinger W, Cohen J, et al. Prevalence of healthcareassociated infections in acute care hospitals in Jacksonville, Florida. Infect Cont Hosp Epidemiol. 2012;33:283-291. 2. Rutledge-Taylor K, Matlow A, Gravel D, et al. A point prevalence survey of healthcare-associated infections in Canadian pediatric inpatients. Am J Infect Cont 2012;40:491-496. 3. Miller MR, Zhan C. Pediatric patient safety in hospitals: a national picture in 2000. Pediatrics. 2003;113:1741. 4. Boyce JM, Pittet D. Guideline for hand hygiene in health-care settings: recommendations of the Healthcare Infection Control Practices Advisory Committee. MMWR. 2002;71:1. 5. Erasmus V, Daha TJ, Brug H, et al. Systematic review of studies on compliance with hand hygiene guidelines in hospital care. Infect Cont Hosp Epidemiol. 2010;31:283-294. 6. Pittet, Didier, Allegranzi B, Boyce J. The World Health Organization guidelines on hand hygiene in health care and their consensus recommendations. World Health. 2009;30:611-622. 7. Siegel JD, Rhinehart E, Jackson M, Chiarello L, Healthcare Infection Control Practices Advisory Committee. Guideline for isolation precautions: preventing transmission of infectious agents in healthcare settings. 2007. Available at: http://www.cdc.gov/ncidod/dhqp/pdf/ isolation2007.pdf. 8. Jernigan JA, Titus MG, Groschel DH, et al. Effectiveness of contact isolation precautions during a hospital outbreak of methicillin-resistant Staphylococcus aureus. Am J Epidemiol. 1996;143:496. 9. O’Grady NP, Alexander M, Burns, LA, et al. Guidelines for the prevention of intravascular catheter-related infections. 2011. Available at: http://www.cdc.gov/hicpac/pdf/guidelines/bsi-guidelines-2011.pdf. 10. Wong ES. Guideline for prevention of catheter-associated urinary tract

infections. Available at: http://www.cdc.gov/ncidod/hip/GUIDE/ uritract.htm. 11. Tablan OC, Anderson U, Besser R, et al. Guidelines for preventing healthcare-associated pneumonia, 2003: recommendations of CDC and the Healthcare Infection Control Practices Advisory Committee. MMWR Recomm Rep/ 2004;53:1. 12. Muto CA, Jernigan JA, Ostrowsky BE, et al. SHEA guideline for preventing nosocomial transmission of multidrug-resistant strains of Staphylococcus aureus and Enterococcus. Infect Control Hosp Epidemiol. 2003;24:362. 13. Diagnostic standards and classification of tuberculosis in adults and children. Am J Respir Crit Care Med. 2000;161:1376. 14. Siegel JD. Controversies in isolation and general infection control practices in pediatrics. Semin Pediatr Infect Dis. 2002;13:48. 15. Schutze GE, Willoughby RE. Clostridium difficile infection in infants and children. Pediatrics. 2013;131(1):196-200. 16. Dubberke ER, Gerding DN, Classen D, et al. Strategies to prevent clostridium difficile infections in acute care hospitals. Infect Control Hosp Epidemiol. 2008;29(Suppl 1):S81-S92. 17. Centers for Disease Control. Pertussis epidemic—Washington, 2012. MMWR. 2012;61(28):517-22. 18. MacCannell T, Umscheid CA, Agarwal RK, et al. Guideline for the prevention and control of norovirus gastroenteritis outbreaks in healthcare settings. Infect Control Hosp Epidemiol. 2011;32:939-969.

CHAPTER

6

Electronic Health Records and Clinical Decision Support Levon Utidjian, Eric Shelov, Bimal R. Desai, and Christopher P. Bonafide

INTRODUCTION Over the last 50 years, the electronic health record (EHR) has emerged as a critical tool in the delivery of safe, efficient, quality healthcare. EHR systems have evolved from basic single-office databases into sophisticated applications capable of managing clinical documentation, laboratory results, images, and other patient data across care settings as well as providing decision support to promote safe patient care, reduce errors, and support adherence to practice guidelines. Data captured within the EHR are used to support nearly every aspect of patient care, including related billing and auditing activities. Increasingly, these data are also being used for quality improvement and research. Hospitalists and the patients whom we treat stand to benefit greatly from well-implemented EHRs that provide tools to review growth data and immunization histories, to identify when vital signs and laboratory values exceed normal parameters for age, and to deliver age-, weight-, and condition-appropriate decision support for medication dosing and management. However, most commercially available EHR systems were designed with adult patients in mind. Configuring these systems to care for children often requires additional customization, which can translate to the need for hospitalists to invest time working with EHR implementation teams to ensure that safe, efficient, quality pediatric care can be delivered. This is especially important for hospitalists who work in pediatric units within larger adult-centered hospitals. In this chapter, we provide a brief background of EHR systems, discuss the role of clinical decision support (CDS) tools in

delivering safe, efficient, quality pediatric hospital care, and review important patient safety considerations for hospitalists who may be asked to participate in the design, implementation, or optimization of an EHR.

ELECTRONIC HEALTH RECORDS HISTORY OF HOSPITAL MEDICAL RECORDS The practice of keeping medical records is one of the cornerstones of medicine. Hippocrates, the ancient Greek physician and father of Western medicine, famously kept and advocated for the use of medical records as early as the fifth century BC.1 These earliest medical records were chronological accounts of individual patient cases and served as important tools in the initial understanding of the natural history of diseases, patient outcomes, and sharing of knowledge among practitioners. Such physicianbased records dominated medical documentation for centuries and relied on the diligence of the conscientious physician to accurately document, maintain and preserve this information. In the mid-nineteenth century, the emergence of record keeping in hospitals represented an early attempt to organize patient information primarily for tracking cases and billing.2 Some saw the additional value of the aggregate information from the hospital population for improving patient care. In 1863, social reformer, nurse, and statistician Florence Nightingale described the challenges presented by the limitations of medical record keeping at that time: I am fain to sum up with an urgent appeal for adopting… some uniform system of publishing the statistical records of hospitals. There is a growing conviction that in all hospitals, even in those which are best conducted, there is a great and unnecessary waste of life… In attempting to arrive at the truth, I have applied everywhere for information, but in scarcely an instance have I been able to obtain hospital records fit for any purposes of comparison… If wisely used, these improved statistics would tell us more of the relative value of particular operations and modes of treatment than we have means of ascertaining at present.”3

The call for structuring and standardizing the medical record to improve its usefulness for healthcare and research grew stronger in the twentieth century, at the same time the computer was emerging as a tool for business and research. In the 1960s, physician Lawrence Weed proposed transitioning

to a problem-oriented medical record to improve the ability of clinicians to identify, manage, and treat a patient’s problems in the context of a shared record, as well as to measure and advance the quality of care delivered. He advocated for the use of the computer as a tool to provide better access to the medical record and help guide care delivery.4,5 Weed’s concepts helped guide the design of early EHRs, and some of these concepts are now standard components of medical documentation.

STRUCTURE AND COMPONENTS OF MODERN EHRS Modern EHRs continue to evolve from their origins as basic databases into sophisticated applications capable of managing clinical documentation, laboratory results, imaging, and other patient data across care settings. Although many use the terms EHR and electronic medical record (EMR) interchangeably, there is a distinction: EMR classically refers to the electronic version of the paper chart contained in a single office, while EHR refers to the patient-based record of care across multiple care settings and providers.6 In 2003, the Institute of Medicine (IOM) developed the following definition of the EHR: An EHR system includes (1) longitudinal collection of electronic health information for and about persons, where health information is defined as information pertaining to the health of an individual or health care provided to an individual; (2) immediate electronic access to person- and population-level information by authorized, and only authorized, users; (3) provision of knowledge and decision-support that enhance the quality, safety, and efficiency of patient care; and (4) support of efficient processes for health care delivery.”7

To further illustrate this definition, the IOM broadly outlined EHR core functions, shown in Table 6-1. TABLE 6-1

Core Functionalities for an Electronic Health Record System

• Health information and data • Results management • Order entry/management • Decision support

• Patient support • Administrative processes • Reporting and population health management

• Electronic communication and connectivity Source: Reprinted with permission from Institute of Medicine. Board on Health Care Services. Key Capabilities of an Electronic Health Record System: Letter Report. Washington, DC: National Academies Press; 2003.

A hospital’s EHR provides these functions by integrating numerous components.8 The Admission, Discharge and Transfer (ADT) component manages patient location and identifying information. The Computerized Physician Order Entry (CPOE) component enables the entry of orders that can then be communicated to different staff members or to specific hospital departments like the pharmacy, radiology, and laboratory. The Laboratory Information System (LIS) component handles laboratory result data transmission, and a Picture Archive and Communication System (PACS) component manages radiographic images and reports. Additional components manage non-clinical functions, including billing and accounting services.

PEDIATRIC-SPECIFIC EHR FUNCTIONS The care of children presents many unique challenges for EHR systems. The American Academy of Pediatrics’ (AAP) Council on Clinical Information Technology identified many key EHR features for pediatricians practicing in ambulatory and inpatient settings.9 These features include immunization support, growth tracking, medication dosing, and age-based data norms (Table 6-2). Among the most often cited concerns surrounding pediatric EHR adoption is the lack of robust functionality within commercial systems for providing pediatric care.10-13 Most pediatric-specific features are not automatically built-in when a hospital purchases an EHR, and those that are included often require detailed configuration by teams of clinicians, pharmacists, and the EHR vendor. During this process, special attention must be paid to the complexities of caring for patients whose care needs, normal reference ranges for vital sign and laboratory data, and recommended best screening and treatment practices can vary greatly and change rapidly with age, body weight, physiology, and developmental level. TABLE 6-2

Key Pediatric EHR Functions

Examples of EHR Functions Immunization management

Recording immunization data Linking to immunization registry systems Immunization decision support

Growth tracking

Graphical representation with growth curves Percentile calculations

Medication dosing

Dosing by weight Dose-range checking Rounding to safe and convenient doses Age-based dosing decision support

Patient identification

Newborn and prenatal identification Name changes Ambiguous sex

Norms for pediatric data

Numeric – laboratory reference ranges, vital signs Non-numeric – normal exam findings by age Complex normative relationships – BP tables Gestational age and corrected age

Privacy

Adolescent privacy Children in foster or custodial care Consent Adoption Guardianship

Pediatric-specific terminology

Physical exam findings Developmental milestones

Diagnoses Data precision

Age time scales (days, weeks, months, years) Weight to the nearest gram in neonates

Source: Data from Spooner SA, and the Council on Clinical Information Technology. Special requirements of electronic health record systems in pediatrics. Pediatrics. 2007;119(3):631637.

To further address the need for standards in the development of EHRs that could help meet the needs of children, the Agency for Healthcare Research and Quality (AHRQ) recently developed the Children’s EHR Format.14 The goal of the format was to provide the many key stakeholders, including EHR developers, EHR purchasers, and the end user care providers, with an understanding of the minimum requirements for data standards and EHR features to optimize pediatric healthcare. These lists of features by the AAP and the AHRQ can serve as a useful starting point for hospitalists evaluating potential new EHR systems or optimizing the features of an existing system.

CURRENT STATE OF EHR ADOPTION IN CHILDREN’S HOSPITALS Although the first EHR systems were implemented in the 1970s, a recent survey of children’s hospitals revealed that only 17.9% had a basic EHR system, and only 2.8% met criteria for having a comprehensive EHR.12 While over 95% of hospitals had radiology images and lab results available electronically, fewer had drug-allergy alerts (62%), age-based dosing support (44%), CPOE (34%), problem lists (24%), and physician notes (13%). Financial costs were identified as the largest barrier to EHR adoption or comprehensive feature implementation. Recent efforts by the United States government to provide financial incentives for EHR use may stimulate advancement in these low adoption rates.

FEDERAL INCENTIVES FOR EHR ADOPTION In an effort to spur adoption of health information technology, the federal

government allocated 155 billion dollars in the American Recovery and Reinvestment Act of 2009 (ARRA) to fund Title XIII, the Health Information Technology for Economic and Clinical Health (HITECH) Act. The HITECH Act allocates nearly 26 billion dollars to incentivize the adoption and “Meaningful Use” of certified EHR systems, as strictly defined by the Office of the National Coordinator for Healthcare Information Technology (ONCHIT). These incentives are in the form of Medicare and Medicaid payments to eligible professionals and hospitals.15,16 The three stages of Meaningful Use are designed to help organizations guide their incremental implementations of EHR systems.17,18 The goal of Stage 1 is to implement an EHR capable of data capture and sharing, followed by Stage 2’s requirement to demonstrate more advanced clinical processes and finally Stage 3’s requirement to show improved patient outcomes19 (Table 6-3). Hospitals must attest that their EHR meets certain quality measures for certification in order to advance to the next stage. To aid providers in undertaking EHR implementations, the HITECH act also describes funding for the creation of Regional Extension Centers (RECs) to provide information technology support, as well as other workforce training and research programs in information technology.17 TABLE 6-3

Stages of Meaningful Use

Stage 1: 2011–2012 Capture and share data Meaningful use criteria focus on:

Stage 2: 2014 Advance clinical processes Meaningful use criteria focus on:

Stage 3: 2016 Improve outcomes Meaningful use criteria focus on:

Electronically capture health information in a standardized

Perform more rigorous health information exchange

Improve quality, safety, and efficiency, leading to improved health outcomes

format Use that information to track key clinical conditions

Meet increased requirements for eprescribing and incorporating lab results

Develop decision support for national highpriority conditions

Communicate that information for care coordination processes

Demonstrate electronic transmission of patient care summaries across multiple settings

Give patients access to self-management tools

Initiate the reporting of clinical quality measures and public health information

Improve electronic communication with patients and their families

Provide access to comprehensive patient data through patientcentered health information exchanges

Use that information to engage patients and their families in their care

Improve population health

Source: Adapted from Meaningful Use. HealthIT.gov. Available at: http://www.healthit.gov/ policy-researchers-implementers/meaningful-use. Accessed February 20, 2013.

To qualify for financial incentives at each stage of Meaningful Use, eligible professionals and hospitals must attest to achieving a number of EHR utilization requirements such as capturing an electronic problem list and performing medication reconciliation electronically. Hospital EHRs must also collect and capture a number of clinical quality measures such as statistics on emergency room throughput and performance on quality measures for stroke and venous thromboembolism care. Achieving Meaningful Use criteria poses unique challenges in pediatrics.20 These clinical quality measures are

primarily applicable to adults, highlighting the current paucity of validated pediatric quality measures. Failure to achieve these Meaningful Use thresholds will lead to financial penalties in the form of lower Medicare payments starting in 2015. Fundamentally, HITECH and Meaningful Use represent the most ambitious effort to date to promote the use of EHRs in the United States, and they underscore the belief that EHRs are a vital part of the efforts to overhaul and improve the US healthcare system. As a result of these national efforts to promote safe, efficient, and quality care through the use of EHRs, hospitalists will increasingly find themselves in the valued position of leading implementation projects or participating in workflow design efforts.

CLINICAL DECISION SUPPORT Among the most anticipated benefits of the modern EHR is its potential not only to serve as a repository of clinical data and billing information, but also to assist in diagnostic and therapeutic decision-making. By translating complex medical knowledge into evidence-based rules to be applied in specific patient care situations, these systems can help physicians provide higher quality care. Tools that can help physicians prescribe the right doses of medications, avoid dangerous drug-drug interactions, and guide evidencebased treatment decisions are just a few examples of what is collectively known as clinical decision support (CDS).

DEFINITION AND FORMS CDS is “knowledge- and person-specific information, intelligently filtered or presented at appropriate times, to enhance health and health care.”21 Although CDS existed long before the EHR in the form of paper-based templates of common orders for specific conditions, clinical algorithms, drug references, and treatment guides, these all required a provider to actively seek out information. Ideally, an EHR’s CDS system is able to retrieve pertinent clinical information and offer it to the clinician in a clear and concise manner in order to maximize the efficiency and effectiveness of the decision-making process. Forms of CDS include reminders of routine screenings or vaccinations, alerts for critical situations, CPOE order sets, clinical

guidelines, and user-friendly displays of general or patient-specific diagnostic and therapeutic information.22 Any CDS intervention should be viewed in the context of the “CDS Five Rights” to ensure delivery of the right information to the right person, in the right format, through the right channel, at the right time23 (Table 6-4). These rights serve not only to maximize the effectiveness of CDS, but also to reduce the likelihood of unintended consequences of CDS that may be detrimental to patient care. A review of randomized controlled trials suggested that CDS systems improved clinical practice in 68% of studies, and the four independent predictors of improvement were the use of a computerbased system, the automatic provision of CDS, provision of recommendations rather than assessments, and the delivery of these interventions at the time and place of decision-making.24 Common forms of CDS are reviewed and summarized below, with specific reference to the benefits and challenges of medication alerting, order sets, and clinical practice guidelines in a hospital setting. TABLE 6-4

Clinical Decision Support Five Rights

1. The right information: evidence-based, suitable to guide action, pertinent to the circumstance 2. To the right person: considering all members of the care team, including clinicians, patients, and their caretakers 3. In the right CDS intervention format: such as an alert, order set, or reference information to answer a clinical question 4. Through the right channel: for example, a clinical information system (CIS) such as an electronic medical record (EMR), personal health record (PHR), or a more general channel such as the Internet or a mobile device 5. At the right time in workflow: for example, at time of decision/action/need Source: Reproduced with permission from Osheroff JA. Improving medication use and outcomes with clinical decision support: a step-by-step guide. Chicago, IL: Healthcare Information and Management Systems Society Mission; 2009.

EXAMPLES Medication Alerts With estimates of 44,000 to 98,000 deaths attributable to medical errors annually, a significant proportion of which are related to medications, a clear potential benefit of CDS is to reduce the number of preventable Adverse Drug Events (ADEs).25 This potential benefit has been studied in both adult and pediatric settings, and many analyses have demonstrated statistically significant reductions in prescription errors and some reductions in ADEs.26-30 CDS interventions for medication order completion can range from fairly simple allergy and drug-drug interaction checking to more advanced support such as formulary-based dosing shortcuts, maximum dose alerts, indication-based dosing, and integration of pertinent patient data such as lab results. Ideally a system has tools to prevent errors of commission, when the action taken is incorrect like medication overdose, as well as errors of omission, when a needed therapy is not ordered, such as serum medication levels in a patient with poor renal function.31 In pediatrics, age- and weight-based dosing are the primary methods for prescribing medications. Variations in these patient characteristics can be gradual and predictable as in the yearly well-child visits at the pediatrician’s office, or volatile as in the daily fluctuations seen in a neonatal intensive care unit. Performing dose calculations is perhaps the most fundamental of pediatric CDS functions. In addition to performing calculations, the EHR should also support the physician in ensuring that the weight used is the most accurate and up-to-date as possible. Weight accuracy can be supported by comparing the recorded weight to standard norms (Centers for Disease Control [CDC] growth curves) and by alerting the clinician of significant deviation, as well as alerting for implausible changes in weight over short periods of time. In addition to supporting calculation of an appropriate medication dose, a CDS system should alert a prescriber when an ordered dose is too low or too high for the patient. This should include both weight-based dose checking and absolute maximum dose checking. More advanced systems would also include support for appropriate dosing by indication (e.g. different antibiotic doses for pneumonia vs meningitis) or by co-morbidity (e.g. recommending a modified dose for patients with renal insufficiency) by either prompting the user to specify these conditions or querying the patient’s problem list. In

addition, the format of alerts can vary with escalating levels of interruption and override requirements based on the severity of the alert. Order Sets Order sets in CPOE systems group associated orders for specific clinical situations, conditions, or workflows for rapid ordering and can help guide the delivery of safe, efficient, quality care. Examples include orders grouped for specific presenting symptoms, such as fever in young infants, for conditions such as asthma, or for workflows such as general pediatric admissions. Order sets can also be used to suggest corollary orders (e.g. serum antibiotic levels to be ordered with aminoglycoside antibiotics, or urine pregnancy tests to be ordered prior to radiology studies for female patients). Order sets constitute a form of CDS in that they present the most appropriate therapeutic interventions and diagnostic testing for a given situation, reflecting the accepted standard of care of an institution.22 The benefits of using order sets include improving the consistency of care delivered, as well as helping to decrease the cognitive workload on providers.32,33 In addition, by guiding clinicians to the correct initial decisions, order sets can reduce the number of workflow interrupting alerts faced later in the ordering process. The ability to create order sets is a feature present in most EHRs, and there are many guidelines and reviews of best practices for their design and effective implementation.34-37 Although commercial EHRs may include prepackaged “pediatric” order sets, it is important to check that they are clinically appropriate and fit into the workflow of the providers who will use them. A well organized, evidence-based order set may be of little use to providers if it does not fit into their actual workflow, is not readily accessible at the right time in their workflow, or if they are not aware of its existence.22,38 Clinical Practice Guidelines A clinical practice guideline (CPG) represents an evidence-based, systematically developed set of recommendations for the management of a specific condition.39 The use of such guidelines is intended to improve healthcare quality and decrease variation in care delivery. A review of barriers to physician adherence to CPGs highlighted the lack of provider awareness of guidelines, as well as limited time to access them and absence of reminders to use them.40 Such barriers can be overcome by integrating CPGs into the EHR: translating the

CPGs into rule-based alerts to identify clinical situations where guidelines are applicable, and using order sets to organize recommended tests and treatments. Such guideline-related CDS tools have been demonstrated in modern EHR systems and were shown to increase guideline adherence and positively influence clinical practice.41-43 Multiple challenges to the successful implementation of CPGs in the her exist. Many CPGs are long, complex documents and not suitable for onscreen display in their entirety. Efforts at simplifying guideline information while still providing access to the details of the full guideline is important in improving physician adoption.34 CPGs can require multiple pieces of patient data to aid in decision-making. The more automated the data collection process, and the less the user must manually enter, the more likely they are to use the system. Guideline maintenance is also very important, as the standard of care changes with time and so they should be reviewed regularly. As the EHR is a centrally managed system, rapid deployment of updates is possible and does not require the replacement of prior paper guidelines, thus avoiding the costs and issues of version control. This approach helps reduce system variation and can be a powerful tool in helping avoid errors and improving quality of care.44

THE EHR, CDS, AND PATIENT SAFETY Although the benefits of EHR adoption include mechanisms to improve safety and quality in the healthcare system, the EHR itself is not without its own safety risks. As the technology behind the EHR grows more sophisticated and intertwined with the operations of a healthcare organization, it is increasingly difficult to anticipate potential issues between the EHR and providers.45 An EHR will do exactly what it is programmed to do, but flaws in system design or integration into provider workflow can carry unintended consequences and adversely affect patients.46 In the rush to implement EHRs to meet Meaningful Use criteria, healthcare organizations without EHR experience are at higher risk for such consequences. Sittig and Singh recently called attention to the safety challenges associated with EHR technology, including the adverse impacts of downtime and the unintended consequences of failing to use EHRs appropriately.47 These are outlined briefly below.

DOWNTIME As an EHR implementation steadily progresses towards the ideal of a fully electronic, paperless system, a healthcare organization’s operations will become more and more reliant on the EHR being up and running without interruptions due to unplanned crashes or routine maintenance. Downtimes expose weaknesses in the organization’s network and EHR technology infrastructure, while forcing clinicians to deliver medical care without the safety net of clinical decision support that they may rely upon.48,49 The need to resort to paper-based documentation and ordering processes can have a major impact on efficiency and patient safety, particularly as each successive generation of care providers will be less and less familiar with practicing without the aid of a computer. The effect of lengthy downtimes on an entire healthcare system can be profound, slowing care processes to the point that it is difficult to safely assume care of new patients and leading to considerable losses in revenue. Even the most secure and highly reliable system is vulnerable to an unplanned downtime, and every organization should be ready for the possibility. Steps to prepare for downtimes include establishing a downtime policy and contingency plans for extended downtime periods.50,51 Support materials like paper forms for documentation and ordering, drug and laboratory reference books, and backup communication systems should be in place to ensure care can continue without the EHR. Planning must include not only for the downtime itself but also the recovery process to return to the pre-downtime state as well as activities like the re-entry of orders and clinical data into the EHR to ensure completeness of the medical record. Such operational and recovery plans are only as effective as provider familiarity with the plans, so providers should be trained on downtime procedures, even practicing them as one might practice a fire drill or other disaster preparedness exercise.

UNINTENDED CONSEQUENCES How an organization chooses to implement and use its EHR system can have major impacts on patient safety and workplace efficiency. Underestimating the need for clinical process requirements gathering, redesign, and validation during an EHR implementation can have direct adverse effects on patients.

As an often-cited example, Han et al. demonstrated that implementation of a new CPOE system was associated with an unexpected increase in mortality.52 The authors described difficulties in placing multiple orders quickly and efficiently, the inability to pre-order medications for critically ill patients en route to the hospital due to registration workflows, and delays in obtaining medications in emergent situations as contributing factors. However, their report was met with stiff criticism, and can serve as a case study on the effects of failing to appropriately plan for EHR implementation.36,53 As an example, carefully designed ICU-specific order sets could have reduced the time required to order urgent medications. Consideration of a “preregistration” process could have allowed the organization to enter orders on patients who were en route to the intensive care unit. Furthermore, the authors also noted that elimination of a satellite medication dispenser located in the ICU led to delays in medication availability, an issue that could have been detected through EHR workflow simulations prior to go-live. Learning from this experience, subsequent implementations at other hospitals have described successful CPOE system implementation without adversely impacting mortality rates.53,54 Even the best intentions to leverage and maximize the capabilities of an EHR’s CDS systems can introduce and facilitate new errors. CPOE systems can easily check the list of active medications for possible drug-drug, drugallergy, drug-food, and drug-condition interactions. Given a streamlined and clinically validated set of possible interactions, these systems can help prevent administration of potentially dangerous medications. However, when used indiscriminately, these same tools can lead to excessive alerting and overwhelm providers.46,49 The provision of too many alerts that are frequently overridden by prescribers can lead to “alert fatigue” and lead prescribers to unintentionally ignore important alerts.55 At the heart of this problem is that standards and best practices for alert content are lacking. Although work is underway to identify priority drug-drug interactions for adult and pediatric populations, it will be some time before these standards are easily implemented into vendor EHR systems.56,57 A related example of an alerting tool’s unintended consequences is the use of the “hard stop” alert that cannot be overridden. Such an alert may be meant to prevent a rare drug-drug interaction, but this could be of less risk than the risk of not giving the two medications in some clinical situations.

Furthermore, in a time-critical situation, a “hard stop” may be inappropriate and lead to potentially dangerous delays in care.58 These and many other examples of unintended consequences of EHR implementation and usage are, unfortunately, common. In response, the AHRQ has created a report and website geared to both future and current EHR users to help them identify and address these issues.59,60

CONCLUSION The EHR is a powerful tool for improving clinical care and augmenting the abilities of hospitalists to deliver that care safely and efficiently. As the use of EHR systems continues to increase, these systems will become the standard form of medical record keeping and care management. Learning how to maximize their potential will be critical for all healthcare providers. Given that the volume of patient health data is expected to grow more rapidly with the emergence of genomic and personalized medicine, the EHR will take on even greater importance in helping healthcare providers organize and interpret this information in the coming years.

REFERENCES 1. Reiser SJ. The clinical record in medicine. Part 1: Learning from cases. Ann Intern Med. 1991;114(10):902-907. 2. Siegler EL. The evolving medical record. Ann Intern Med. 2010;153(10):671-677. 3. Nightingale F. Notes on hospitals. Longman, Green, Longman, Roberts, and Green; 1863. 4. Weed LL. Medical records that guide and teach. N Engl J Med. 1968;278(11):593-600. 5. Weed LL. Medical records that guide and teach. N Engl J Med. 1968;278(12):652-7. 6. Garrett P, Seidman J. EMR vs EHR – What is the difference? HealthITBuzz. 2011. Available at: http://www.healthit.gov/buzz-blog/ electronic-health-and-medical-records/emr-vs-ehr-difference/. Accessed

February 5, 2013. 7. Institute of Medicine. Board on Health Care Services. Key Capabilities of an Electronic Health Record System: Letter Report. Washington, DC: National Academies Press; 2003. 8. Carter JH. Electronic Health Records. Philadelphia, PA: ACP Press; 2008. 9. Spooner SA, and the Council on Clinical Information Technology. Special requirements of electronic health record systems in pediatrics. Pediatrics. 2007;119(3):631-637. 10. Johnson KB. Barriers that impede the adoption of pediatric information technology. Arch Pediatr Adolesc Med. 2001;155(12):1374-1379. 11. Kim GR, Lehmann CU, and the Council on Clinical Information Technology. Pediatric aspects of inpatient health information technology systems. Pediatrics. 2008;122(6):e1287-e1296. 12. Nakamura MM, Ferris TG, DesRoches CM, Jha AK. Electronic health record adoption by children’s hospitals in the United States. Arch Pediatr Adolesc Med. 2010;164(12):1145. 13. Leu MG, O’Connor KG, Marshall R, Price DT, Klein JD. Pediatricians’ use of health information technology: A national survey. Pediatrics. 2012;130(6):e1441-e1446. 14. Whitesides GM. Children’s Electronic Health Record Format Documentation and User Guide. AHRQ Publication No 13-0020-EF. 2012. Rockville, MD: Agency for Healthcare Research and Quality; 2012:1-23. 15. Blumenthal D, Tavenner M. The “meaningful use” regulation for electronic health records. N Engl J Med. 2010;363(6):501-504. 16. Blumenthal D. Wiring the health system—origins and provisions of a new federal program. N Engl J Med. 2011;365(24):2323-2329. 17. Blumenthal D. Launching HITECH. N Engl J Med. 2010;362(5):382385. 18. Blumenthal D. Implementation of the federal health information technology initiative. N Engl J Med. 2011;365(25):2426-2431. 19. Meaningful Use. Available at: http://www.healthit.gov/policyresearchers-implementers/meaningful-use. Accessed March 11, 2013.

20. Burton OM. Letter to office of the national coordinator for health information technology. AAP Advocacy Site. Available at: http:// www2.aap.org/informatics/pdfs/Advocacy/HITPC-MU-S2.pdf. Accessed March 17, 2013. 21. Osheroff JA, Teich JM, Middleton B, Steen EB, Wright A, Detmer DE. A roadmap for national action on clinical decision support. J Am Med Inform Assoc. 2007;14(2):141-145. 22. Horsky J, Schiff GD, Johnston D, Mercincavage L, Bell D, Middleton B. Interface design principles for usable decision support: A targeted review of best practices for clinical prescribing interventions. J Biomed Inform. 2012;45(6):1202-1216. 23. Osheroff JA, Healthcare Information and Management Systems Society. Improving Medication Use and Outcomes with Clinical Decision Support: A Step-By-Step Guide. Chicago, IL: Healthcare Information and Management Systems Society Mission; 2009. 24. Kawamoto K, Houlihan CA, Balas EA, Lobach DF. Improving clinical practice using clinical decision support systems: A systematic review of trials to identify features critical to success. BMJ. 2005;330(7494):765. 25. Institute of Medicine, Committee on Quality of Health Care in America. In: Kohn LT, Corrigan JM, Donaldson MS, eds. To Err Is Human: Building a Safer Health System. Washington, DC: National Academies Press; 2000. 26. Wang JK, Herzog NS, Kaushal R, Park C, Mochizuki C, Weingarten SR. Prevention of pediatric medication errors by hospital pharmacists and the potential benefit of computerized physician order entry. Pediatrics. 2007;119(1):e77-85. 27. Kaushal R, Shojania KG, Bates DW. Effects of computerized physician order entry and clinical decision support systems on medication safety: A systematic review. Arch Intern Med. 2003;163(12):1409-1416. 28. Kaushal R, Bates DW, Landrigan C, et al. Medication errors and adverse drug events in pediatric inpatients. JAMA. 2001;285(16):2114-2120. 29. Walsh KE, Wright A, Krist AH, et al. Effect of computer order entry on prevention of serious medication errors in hospitalized children. Pediatrics. 2008;121(3):e421-e427.

30. Potts AL, Barr FE, Gregory DF, Wright L, Patel NR. Computerized physician order entry and medication errors in a pediatric critical care unit. Pediatrics. 2004;113(1 Pt 1):59-63. 31. Osheroff JA. Improving Outcomes with Clinical Decision Support: An Implementer’s Guide. Chicago, IL: HIMSS; 2012. 32. Bobb AM, Payne TH, Gross PA. Viewpoint: Controversies surrounding use of order sets for clinical decision support in computerized provider order entry. J Am Med Inform Assoc. 2006;14(1):41-47. 33. Avansino J, Leu MG. Effects of CPOE on provider cognitive workload: A randomized crossover trial. Pediatrics. 2012;130(3):e547-e552. 34. Bates DW, Kuperman GJ, Wang S, et al. Ten commandments for effective clinical decision support: Making the practice of evidencebased medicine a reality. AMIA. 2003;10(6):523-530. 35. Miller RA, Waitman LR, Chen S, Rosenbloom ST. The anatomy of decision support during inpatient care provider order entry (CPOE): Empirical observations from a decade of CPOE experience at Vanderbilt. J Biomed Inform. 2005;38(6):469-485. 36. Sittig DF, Ash JS, Zhang J, Osheroff JA, Shabot MM. Lessons from “Unexpected increased mortality after implementation of a commercially sold computerized physician order entry system.” Pediatrics. 2006;118(2):797-801. 37. ISMP’s guidelines for standard order sets. Institute for Safe Medical Practices. Available at: http://www.ismp.org/tools/guidelines/ standardordersets.pdf. Accessed January 15, 2013. 38. McGreevey JD. Order sets in electronic health records - principles of good practice. Chest. 2013;143(1):228. 39. Institute of Medicine. In: Field MJ, Lohr KN, eds. Guidelines for Clinical Practice: From Development to Use. Washington, DC: National Academies Press; 1992. 40. Cabana MD, Rand CS, Powe NR, et al. Why don’t physicians follow clinical practice guidelines? JAMA. 1999;282(15):1458-1465. 41. Shiffman RN, Liaw Y, Brandt CA, Corb GJ. Computer-based guideline implementation systems: A systematic review of functionality and effectiveness. J Am Med Inform Assoc. 1999;6(2):104-114.

42. Damiani G, Pinnarelli L, Colosimo SC, et al. The effectiveness of computerized clinical guidelines in the process of care: A systematic review. BMC Health Serv Res. 2010;10(1):2. 43. Neuman MI, Hall M, Hersh AL, et al. Influence of hospital guidelines on management of children hospitalized with pneumonia. Pediatrics. 2012;130(5):e823-e830. 44. Reason J. Human error: Models and management. BMJ. 2000;320(7237):768-770. 45. Ash JS, Berg M, Coiera E. Some unintended consequences of information technology in health care: The nature of patient care information system-related errors. AMIA. 2004;11(2):104-112. 46. Koppel R, Metlay JP, Cohen A, et al. Role of computerized physician order entry systems in facilitating medication errors. JAMA. 2005;293(10):1197-1203. 47. Sittig DF, Singh H. Electronic health records and national patient-safety goals. N Engl J Med. 2012;367(19):1854-1860. 48. Kilbridge P. Computer crash—lessons from a system failure. N Engl J Med. 2003;348(10):881-882. 49. Campbell EM, Sittig DF, Ash JS, Guappone KP, Dykstra RH. Types of unintended consequences related to computerized provider order entry. J Am Med Inform Assoc. 2006;13(5):547-556. 50. Institute of Medicine. Health IT and Patient Safety: Building Safer Systems for Better Care. Washington, DC: National Academies Press; 2012. 51. Nelson NC. Downtime procedures for a clinical information system: A critical issue. J Crit Care. 2007;22(1):45-50. 52. Han YY, Carcillo JA, Venkataraman ST, et al. Unexpected increased mortality after implementation of a commercially sold computerized physician order entry system. Pediatrics. 2005;116(6):1506-1512. 53. Del Beccaro MA, Jeffries HE, Eisenberg MA, Harry ED. Computerized provider order entry implementation: No association with increased mortality rates in an intensive care unit. Pediatrics. 2006;118(1):290295. 54. Longhurst CA, Parast L, Sandborg CI, et al. Decrease in hospital-wide

mortality rate after implementation of a commercially sold computerized physician order entry system. Pediatrics. 2010;126(1):14-21. 55. Isaac T, Weissman JS, Davis RB, et al. Overrides of medication alerts in ambulatory care. Arch Intern Med. 2009;169(3):305-311. 56. Phansalkar S, van der Sijs H, Tucker AD, et al. Drug-drug interactions that should be non-interruptive in order to reduce alert fatigue in electronic health records. J Am Med Inform Assoc. 2012. 57. Phansalkar S, Desai AA, Bell D, et al. High-priority drug-drug interactions for use in electronic health records. J Am Med Inform Assoc. 2012;19(5):735-743. 58. Strom BL, Schinnar R, Aberra F, et al. Unintended effects of a computerized physician order entry nearly hard-stop alert to prevent a drug interaction: A randomized controlled trial. Arch Inter. Med. 2010;170(17):1578-1583. 59. Jones SS, Koppel R, Ridgely MS, Palen TE, Wu S. Guide to Reducing Unintended Consequences of Electronic Health Records. AHRQ Publication No. 11-0105-EF. Rockville, MD: Agency for Healthcare Research and Quality; 2011. 60. Guide to Reducing Unintended Consequences of Electronic Health Records. AHRQ Available at: http://www.ucguide.org/. Accessed February 10, 2013.

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Family-Centered Care Vineeta Mittal and Michael T. Vossmeyer

INTRODUCTION The approach to the hospitalized child and family has changed dramatically over the last decade. Physicians are rarely the sole decision makers when children are hospitalized. Patients and families expect to be actively involved in medical decision-making, and this expectation is strongly supported by leaders in health care and government. In 2001 the Institute of Medicine published a report outlining the six aims of a transformed health care delivery system in the United States. The report stated a health care system should be timely, effective, efficient, patientcentered, equitable, and safe. Inpatient care should be focused on achieving these aims. In recent years, healthcare reform in the United States has focused on cost-effective care and better patient outcomes. As many innovative models of healthcare emerge, new emphasis is on encouraging physicians, hospitals, and other healthcare providers to work more closely as a team and to better coordinate patient care through team-based approaches, therefore giving a greater role to patients in healthcare decision-making. Team-based care may help achieve improvement in health care at a reasonable cost. The health care teams may vary depending on setting (home, office, hospital), disease type (chronic care coordination vs. acute care or well checks), or personnel (care coordination, social worker, home health in a complex care setting vs. nursing, respiratory therapists, pharmacy in asthma centers). Healthcare teams may be large or small depending on the model of care provided. In high-functioning healthcare teams, patients are members of the team; they are the reason healthcare teams exist. “Nothing about me without me” conveys the powerful message of patients’ active involvement in care

decisions. This involves integrating patients, family, physicians, and other providers in healthcare teams.

HOW CAN HOSPITALISTS FACILITATE PATIENT- AND FAMILY-CENTERED CARE? Hospitalists play a crucial role in managing healthcare teams in the inpatient setting. They can incorporate the principles and values of family- and patientcentered care to improve quality as defined by outcomes, safety, and patient experience. A hospitalized child requires timely access to clinical services without delays attributable to system design. Variations in clinical care are frequently attributed to failures in clinical care. Systems that are well designed to ensure necessary levels of clinical reliability decrease variation and improve outcomes. Clinical care should be evidence based, and the implementation of evidence-based clinical guidelines decreases unnecessary variation in care and improves clinical outcomes. When there are no available guidelines, the original medical literature can be accessed and evaluated using generally accepted methods of critical appraisal. Equitable care must be available for all hospitalized children without regard to race, national origin, religious or cultural background, gender, or insurance status. Hospital systems must be designed to ensure patient safety and to dramatically decrease preventable sources of error. The use of computerized technologies, such as electronic medical records, computerized order entry systems, and automated safety triggers can increase the reliability of hospital systems. For successful transitions to occur, hospital cultures need to change from physician-centered to patient- and family-centered. Dimensions of care that patients and families value, including respect for preferences, involvement in decision making, access to care, coordination of care, information and education, physical comfort, emotional support, involvement of family and friends, and continuity in the transition from inpatient to outpatient or medical home setting should be the center of focus.

TRANSITION TO THE INPATIENT SETTING Hospitalization is a stressful event for both the child and family. Children and

families may experience feelings ranging from fear of the unknown to complete loss of control. These feelings complicate the clinical situation and detract from development of a healing environment. Physician and other healthcare professionals should address these stresses directly and provide an environment designed to preserve patient and family control. Whenever possible, the child and family should be prepared for the hospitalization; this allows them to be educated about the proposed care and can help clarify the expectations of the family and physicians. Such preparations also provide opportunity to initiate clear and open communication between physicians and prepare for safe handoffs of care and discharge planning. If possible, before a planned hospitalization, the child and family should be given an opportunity to tour the facility and meet the staff who will provide care. An active child life service can facilitate the prehospital activity. When hospitalization occurs urgently or emergently, the child life programs can provide support for children and families at the point of contact.

HOSPITAL CARE In the hospital setting, educating families about the roles of healthcare professionals improves satisfaction. Informing parents and families about daily ward routines including the timing of nursing shift change, the process of patient handoff, the timing of physician rounds, access to parking and meals, availability of interpreter services and guest relations, and role of medical emergency teams may alleviate parental anxiety associated with hospitalization. The amount of information given may be overwhelming, so multiple methods of communication including printed materials maybe helpful in educating patients and their families. Orientation to the hospital and the nursing unit with specific information about special services such as child life, social work, volunteers, and pastoral services is important. Patients and families will benefit from information about internet access, sport rooms, music, and access to movies for patients.

FAMILY-CENTERED ROUNDS Family-centered rounds (FCRs) are multidisciplinary rounds that take place in the patient’s room and involve active participation of the patient and

family in decision-making. FCRs involve medical decision-making, communication, teaching, discharge planning, and coordination of care. All healthcare professionals involved in the patient’s care, and the patient and families should participate in each of these processes. Involving patients and families as members of the healthcare teams is more than a shift in medical culture. It is crucial to incorporate values and principles of high-functioning healthcare teams to ensure successful culture change. Clear roles and responsibilities, mutual trust, effective communication, shared goals, honesty, discipline, and measurable processes and outcomes are important characteristics of high-functioning teams. Decision-making should be transparent to all involved. The family is often the best source of information about a child, but is frequently overlooked. Actively taking into account the family’s values, culture, race, and socioeconomic background as well as family members’ unique knowledge of the patient can assist in decision-making. All diagnostic and treatment decisions should be made with the full participation of the family. Healthcare teams members must be willing to work constructively with families even when their decisions do not coincide with the advice of the medical team. The use of evidence-based protocols can often facilitate a transparent decision-making process and establish a basis for the discussion of therapies with patients and families. Such protocols decrease variation among healthcare professionals that can confuse patients and families. Communication that recognizes the patient’s family as the driving force is central to their empowerment. A simple gesture such as asking patients or families about their preferences regarding rounds allows the family to regain a sense of control and involvement. Honest and open communication between medical professionals and families is vital to the success of the hospitalization. Total transparency in all aspects of hospitalization is necessary to maintain optimal lines of communication. FCRs present a venue for experienced physicians to teach aspects of core competencies to trainees that can only be taught at bedside. Trainees report improved physical examination and communication skills, and learn role modeling through attending physicians. Attending physician role modeling can help trainees learn about core aspects of compassion, accountability, and respect toward diverse populations. Communication skills cannot be taught as well in classrooms as observed and practiced in real time. Observing trainees

interact during rounds also allows for direct observation of trainees and realtime feedback, which is often under-reported and is highly valued by trainees. Trainees report that having a visual impression of a patient/disease condition and seeing how different physicians interact and respond to parental questions is a valuable learning experience. Unless families state otherwise, all critical discussions about medical care should occur in their presence. Morning rounds can and should be conducted with the family present. The patient is presented to the medical team in the presence of family, using language that can be understood by the patient and the family. Medical terminology should be minimized or explained in plain language. Interpreters should be present if there is a language barrier; it is not advisable to use family members as interpreters. FCRs help close the communication loop and bring all care givers including family to a common understanding. FCRs have been shown to improve parental satisfaction, parental understanding of discharge goals, discharge timeliness, and nursing satisfaction. Being present during the healthcare team’s discussion gives the family a great sense of control and dispels some fear of the unknown. Conducting FCRs can be challenging in certain situations. Hospitals with double rooms, patients on isolation precautions, certain unique circumstances like non-accidental injuries or teenagers with sexually transmitted diseases, teams with multiple consultants, and on healthcare teams where mid-level healthcare providers act as proxies for attending physicians pose unique challenges. Individual hospitals and units may need to develop their own rounding styles to ensure that core principles of patient- and family-centered care are carried out.

CHILD LIFE SERVICES The overall objectives of child life services are to recognize and address developmental issues related to healthcare experiences and to respond to fears and concerns of hospitalized children and their families. Child life professionals meet these objectives through a variety of activities, including creating opportunities for play and enhanced self-esteem, providing inpatient school work, assessing coping responses, minimizing stress, preparing children and families for healthcare experiences, facilitating family celebrations, and communicating these goals to the healthcare team.

The way a child plays often communicates his or her interpretation of medical care or illness in a manner that may be inaccessible to other healthcare professionals. Child life may correct misunderstandings that arise during play or relay critical developmental concerns to the family and other professionals on the child’s healthcare team. In addition, play is a method of coping in the foreign environment of the hospital. Therapeutic play can include dramatic play, artistic activity, pet therapy, sensory play (e.g. making dough), games, and attending special events such as musical performances. Medical play provides children with an open-ended opportunity to act, draw, or otherwise use creative media to explore medical equipment, situation, or themes. These activities familiarize children with their surroundings and help them better understand their own medical experiences. Child life specialists also provide educational resources and environments for family members to help them cope with a child’s illness. In-hospital libraries for families, educational video broadcasting in-hospital, and setting aside specific time for siblings in child life rooms can help alleviate a family’s anxiety during a child’s illness or hospitalization. Each child life specialist is a member of the comprehensive medical team caring for the patient and can serve as a valuable liaison between the family and child and healthcare professionals.

QUALITY AND OUTCOMES Hospitals strive to improve quality of care and better patient outcomes. Family/patient satisfaction correlates directly with physician and nursing communication. Involving patients and families in every aspect of hospitalization and having clear and open communication can help improve patient outcomes. Maintaining transparency in care and using lay language to improve family understanding of medical conditions may further help communication and parental satisfaction.

DISCHARGE PLANNING AND TRANSITION TO THE MEDICAL HOME An orderly transition from inpatient to ambulatory setting is important for continuity of clinical care. Discharge planning and discharge goals should be

established as soon as possible after admission. These goals should be reviewed and updated at least daily and discussed on rounds with the family and nurses. The more specific the discharge goals are, the more helpful they are to patients, families, and other team members. Once established, discharge goals facilitate ongoing care and discharge planning. Understanding discharge criteria and the role of care coordination is a valuable learning experience for trainees. Well-designed care systems are needed to facilitate discharges. The use of best-practice reminders embedded in the electronic medical record is helpful to remind healthcare professionals about discharge planning resources. Use of care coordinators or discharge planners may assist the primary medical team in accessing necessary outpatient services and proactively dealing with insurance-related issues. Communication with private/community physicians is also necessary before discharge and sets the stage for safe handoff of care and closes the communication loop. Gaining the support and cooperation of private physicians facilitates transition. Important items to discuss include discharge diagnosis, results of diagnostic testing, outstanding tests that need follow-up, discharge medications, follow-up appointments, and family concerns. This communication should supplement and not replace a written discharge summary and should optimally occur before the patient’s discharge from the hospital.

CONCLUSION A patient- and family-centered care approach to the hospitalized child uses dimensions of care that patients and families value. These dimensions of care include honoring patient’s and families’ preferences, showing respect, using effective communication techniques, developing shared goals, and being transparent about care processes and outcomes. Patient- and family-centered care includes evidence-based clinical care and addresses issues of timeliness, access, efficiency, and safety. Care that is system based and accounts for these aims will produce more satisfactory clinical outcomes. Pre-hospital preparation, orientation to hospital and the unit, and robust child life services can further enhance family comfort and the experience of hospitalization. Conducting daily FCRs to involve families in decision-making, improving communication, trainee education, and facilitating discharge timeliness is crucial. Attention must be paid to discharge planning and the transition from

inpatient to ambulatory settings. Good communication among healthcare providers supports a safe transition and handoff of care. Involving patients and their families at all levels of the care process can improve satisfaction with the healthcare delivery system and the quality of care delivered.

SUGGESTED READINGS Institute of Medicine. Crossing the Quality Chasm: A New Health Care System for the 21st Century. Washington, DC: National Academy Press; 2001. McLellan RK, et al. Optimizing health care delivery by integrating workplaces, homes, and communities: How occupational and environmental medicine can serve as a vital connecting link between accountable care organizations and the patient-centered medical home. J Occup Environ Med. 2012;54(4):504-512. Mittal V, et al. Pediatric resident perspectives on family-centered rounds: A qualitative study at two children’s hospitals. J Grad Med Educ. 2013;5(1):81-87. Moyer V, Williams K, Elliott EJ (eds.). Evidence Based Pediatrics and Child Health. London: BMJ; 2000. Wynia M, Kohorn I, Mitchell P. Challenges at the intersection of team-based and patient-centered health care. Insights from an IOM working group. JAMA. 2012;308(13):1327-1328.

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Medical Comanagement and Consultation Erin Stucky Fisher

INTRODUCTION Consultation and comanagement roles are still evolving for the pediatric hospitalist in the United States. Typically, hospitalists provide direct care for inpatients, seeking consultation from specialty colleagues as needed. For the past few years, however, hospitalists been called upon to provide consultation or comanage patients with another physician. The 2012 State of Hospital Medicine Report states that over 75% of pediatric hospitalists provide surgical or medical comanagement, with consultation roles less commonly (14% medical and 63% surgical, respectively).1 In the broader milieu of patient care, hospitalists practice comanagement daily. When a primary care provider asks a hospitalist to care for one of his or her inpatients, the hospitalist is in many respects comanaging the patient with the primary care provider. Rather than doing this simultaneously during the hospital stay (as is typically envisioned with comanagement), the patient is comanaged by the hospitalist and primary care provider sequentially. For the patient to receive optimal treatment, it is crucial that the two systems integrate. Viewing this relationship as comanagement rather than care transfer serves as a starting point for the concept of two physicians jointly managing patients, whether horizontally along a timeline, or vertically during a single episode of care. In other countries, hospital-based generalists have long been viewed as consultants to outpatient general practitioners. Studying the United Kingdom’s system of pediatrician hospitalists as consultants may aid in defining how hospitalists can serve as consultants in the United States. Specialist registrars in the United Kingdom who were asked to define key attributes of “the ideal hospital doctor” named the following eight areas as essential for the consultant: clinical knowledge and skills, clinically-related

non-clinical skills, self-directed learning and medical education, change management implementation, application of strategic and organizational skills, consultation (history and physical) skills, research, and key personal attributes.2 In the United States, pediatric hospitalists are more commonly now serving as clinical consultants and comanagers during a given hospital admission, working with other providers who serve as attending physicians of record. Pediatric hospitalists are called upon to provide a pediatrician’s view of global issues such as child development, pain management, and home health care, as well as to aid in the diagnosis and management of medically complex children.1 For hospitalists to provide optimal service to patients and providers in these consultant and comanagement roles, it is essential that definitions, expectations, and goals be clear. Consultation is defined as “a deliberation between physicians on a case or its treatment”3 to address a problem that has emerged.4 Management, on the other hand, is “to handle or direct with a degree of skill … the whole system of care and treatment of a disease or a sick individual”3 and is initiated at the onset of a care episode4 with provision of direct medical care in addition to advice.5 Comanagement implies that care is managed by more than one physician. To provide the “whole system of care” that patients deserve, physicians must work synergistically as a team. This chapter reviews consultation and comanagement in the current literature, defines goals of each, explores existing models, and examines future directions.

EVOLUTION OF HOSPITALISTS AS CONSULTANTS AND COMANAGERS: CURRENT LITERATURE Over the past 5 or 6 years, a number of studies have been published on the specific issues of hospitalist as consultant or comanaging physician. Though still limited in number, these studies address hospitalist and non-hospitalist physician experiences, opportunities and barriers, and impact on patient care outcomes. The literature regarding the use of hospitalists as consultants continues to be limited. One retrospective chart review of patients on which adult hospitalists were consulted by orthopedic surgeons for care of non-pathologic

hip fractures did not demonstrate significant improvements in osteoporosis treatment as defined by “addition of a medication for osteoporosis that strengthened treatment.”6 However, these patients had more comorbid illnesses and were significantly older than those receiving no consultation. One study of perioperative patients receiving a hospitalist medical consultation noted longer length of stay, higher cost, and no change in glycemic control and venous thromboembolism prophylaxis.7 However in this study only 9% of patients received consultation, and these patients more often had an American Society of Anesthesiologists score of 4 or higher, diabetes mellitus, vascular disease, or chronic renal failure. Moreover, consultations were requested due to a problem identified, which may have impacted the ability to anticipate and abate events. Literature on pediatric consultation is sparse.8 Canadian publications do note that consultation roles for inpatient (ward and interventional radiology)9 and ambulatory (hospital follow-up and general)10 settings exist, but no program or patient outcomes were reported. In addition to formal consults, hospitalists may be engaged in “curbside consultation,” defined as asking for advice, suggestions, or opinions about a patient’s care without asking the hospitalist to see the patient.11 In one recent study of 47 consultation events comparing curbside to formal consults performed by adult hospitalists on the same patients, curbside consults resulted in fewer questions asked and inaccurate or incomplete information received in 51% of cases.11 Formal consultation changed management in 60%, with the change deemed major in 36% of these cases. Although communication and leading a healthcare team are part of the pediatric hospital medicine core competencies,12 specific expectations for consultation are not highlighted. Implementing a formal curriculum may be a valuable consideration for hospitalists wishing to hone these skills.13 Results from comanagement studies are varied. Hospitalists and specialists in one model held different opinions regarding how the model should be implemented and whether the model improved patient care outcomes, but agreed on the need for “ownership of patients.”14,15 While reported impact on postoperative complications, mortality, and 6-month readmissions rates are conflicting,5,16,17 perceived improved communication by surveyed nurses and non-nursing staff has been noted.18 Pediatric

literature on comanagement is as limited as that for consultation. One small pediatric study of comanaged spinal fusion patients reported decreased length of stay and frequent information updates given to families as reported by hospitalists.19 Patient and family centeredness, medical errors, timeliness of care, and discharge coordination are other elements central to pediatrics yet not well studied.20,21

PEDIATRIC HOSPITALIST AS CONSULTANT: RECOMMENDED PRACTICES By definition, a pediatric hospitalist acting as consultant focuses on a specific question involving a given patient. Most consultations involve a single interaction. The goals should be to provide either diagnostic and therapeutic treatment options or a single comprehensive pediatric screening examination. Discussion of the findings and options with the requesting physician and communication with the family after that discussion are essential. When the pediatric hospitalist is consulted for a specific question but is interacting on a daily basis in a more broad capacity, the service requested is actually comanagement. The factor distinguishing consultation from comanagement is the lack of expectation for longitudinal care and coordination of all patient care needs. Pediatric hospitalist consultation almost always addresses one or more of the following questions: 1. Is this child stable for the planned procedure or intervention? This is answered by performing a global screening assessment and plan. 2. Are the evaluation and treatment appropriate? This requires a review of the plan of care. 3. What is the cause of and treatment for this problem? This requires a problem-focused assessment and plan. Each of these situations requires a thorough history and physical exam, but with a different emphasis. Even for the problem-focused question, however, the pediatric hospitalist should include relevant issues that are at the heart of general pediatrics and may not be thoroughly considered by consulting services, such as nutrition, pain management, psychosocial factors, and developmental needs. Any of these three key questions may be asked of a pediatric hospitalist

by any physician in the institution; however, certain trends have emerged. In particular, surgeons and specialists treating primarily adults may benefit from consistent, planned pediatric inpatient consultation for their patients.21 Expectations for facilities and personnel can also be a guide in determining the pediatric hospitalist’s level of involvement in acute care settings.22,23 Consultation may be requested on a case-by-case basis, or there may be an agreement that all patients in a particular group or setting will have a single initial consultation. Each pediatric hospitalist system must determine the best approach for their patients, community, and facility. Groups for whom routine consults might be considered include the following: Service specific: adult and pediatric surgeons, adult specialists, teaching service, family practitioners Acuity or site specific: newborn care (e.g. labor and delivery, newborn nursery, level II neonatal intensive care unit), pediatric intermediate care, non-pediatric emergency department Advice only: remote site, in hospital Although not as widespread, clinical consultation without chart review or patient examination is performed occasionally by hospitalists, both for patients on site or in remote locations. Academically based pediatric hospitalists are often asked diagnostic and treatment plan questions about patients not under their care. This often educational and uncompensated “curbside” consultation should be performed with acknowledgement of the risk for error.11,24 Remote consultation, usually done by phone, is of value for emergency department physicians as well as local community physicians. Clinical issues can vary from discussion of need for admission to highly complex emergency transport requirements. Pediatric hospitalists involved in coordinating and directing care teams for community wide emergency transport offer a unique and critical form of consultation. Regardless of the model used, guidelines should be developed for the hospitalist’s role as consultant to ensure that the requesting physician is satisfied, patient care is optimized, redundancies are removed, and communication is streamlined. When establishing guidelines, key issues to consider include determining whether consults are accessed by request only or through service agreements, defining who is responsible for communicating with the primary care physician, and clarifying whether the

consultant may write orders without approval of the primary attending physician. Basic tenets of effective consultation include determining the question, establishing the urgency, gathering data, generating succinct and specific recommendations that include solutions to anticipated problems, tactful communication, personal verbal or direct contact, and daily follow-up until the issue is not active.25

PEDIATRIC HOSPITALIST AS COMANAGER: RECOMMENDED PRACTICES Comanagement can be defined as a situation in which two or more physicians are involved in the ongoing care of a patient. As stated previously, comanagement routinely occurs between the hospitalist and primary care provider, with each physician filling a specific role. Additionally, just as primary care physicians’ office obligations preclude them from being available for inpatient care, surgeons’ operating room obligations may preclude them from being available for hospital management and timely discharge planning.4 Comanagement goals should be clearly laid out for each patient. In addition to addressing specific pediatric illness, the hospitalist may coordinate care, improve discharge planning, enhance communication with the primary care provider, and ensure care is family centered. Goals should include providing a comprehensive evaluation of all the patient’s needs, increasing team communication and patient engagement, decreasing errors and delays in decision-making, and assuring a sense of investment and accountability to the patient.26 Coordination of care is considered by many to be a significant strength of the pediatric hospitalist.20 The models for comanagement may have similarities to those described for hospitalist consultation and should be developed to fit individual community and hospital needs. As with consultation, guidelines must be established if comanagement is to succeed. Reason and sensitivity are required when treating physicians are in conflict over clinical management (including decisions to involve other consultants), or when there are conflicts with the family’s wishes. It is important to define which physician is the “captain of the ship.”26 Defining lines of authority is crucial, and these should be established upon assuming

care. Other expectations must also be addressed, including who will communicate with the primary care provider, the family, the patient, other consultants, nursing staff, and in academic settings, housestaff. Many models can be successful, as long as expectations are clear. For example, a 3-year-old child with cerebral palsy and a ventriculoperitoneal shunt presents with fever and renal failure. The hospitalist is the primary attending physician, comanaging with the patient’s neurologist and neurosurgeon. The decision to consult nephrology or infectious diseases is the hospitalist’s decision as the primary attending. In comparison, a 6-year-old child with known diabetes is admitted with fever and joint swelling. The patient’s endocrinologist manages the diabetes, and the hospitalist manages all other aspects of diagnostic and therapeutic evaluation, including the decision to consult orthopedics, if needed. In this scenario, the specialist manages specialty-related issues, leaving the hospitalist to manage all other aspects of care.

OTHER ISSUES Billing issues arise when the hospitalist serves as a consultant or comanages patients. Knowledge of initial consultation and follow-up codes will prevent billing confusion and delays. Only one physician per medical group can be the attending of record, requiring that other physicians bill as “consultants” (regardless of whether they are serving as consultants or comanagers of care, according to the definitions provided earlier). Contract, risk management, and Health Insurance Portability and Accountability Act issues should be considered by pediatric hospitalists performing “advice only” consulting. Pediatric hospitalists must acquire leadership skills to resolve differences of opinion regarding clinical care, particularly relevant in the arena of comanagement. These skills may also be used at an organizational level to institute consultation and comanagement policies and related changes, improve patient safety, streamline processes, advocate for new technology and services, and control costs.

FUTURE DIRECTIONS The pediatric hospitalist’s role as consultant and as partner in comanagement

is growing rapidly. Hospitalist comanagement and coordination of care vertically and horizontally are needed to ensure seamless care. Children with chronic complex conditions account for a growing percentage of pediatric admissions and over 25% of pediatric hospital days.27 These children stand to benefit most from a well-developed comanagement care model. However, such a model should also attend to the triple aim: improving population health, improving the patient experience of care (including quality and satisfaction), and reducing per capita costs of healthcare.28 In addition, the Accountable Care Organization model for healthcare challenges providers to bring coordinated care to the patient in a cost-conscious manner.29 Hospitalists should be accountable to these aims and challenges by consulting and comanaging with an acute and chronic view. The hospitalist can offer “anticipatory guidance” that includes avoidance of acute illness complications, incorporation of preventive care, and planning and execution of post-acute care. Studies are needed to demonstrate the variety and sources of consultations and comanagement, as well as effects on patients, specialist colleagues, resident trainees, hospital staff, and hospital administration. Methods should include surveys of those involved as well as studies of length of stay, cost, and clinical outcome data by disease type and acuity level. Multicenter data collection will be necessary to validate results in varied hospital settings. The impact on the hospitalist intensity of service, autonomy, schedules, and burnout should be studied. Training for current and future hospitalists should include lessons learned from these studies and from mature systems outside the United States. Current pediatric hospitalist task forces, with the leadership of the Joint Council of Pediatric Hospital Medicine and supporting groups—the Ambulatory Pediatric Association, American Academy of Pediatrics and the Society of Hospital Medicine, Pediatric Research in Inpatient Settings (PRIS) Research Network, and the Value in Inpatient Pediatrics (VIP) Quality Improvement30—are well positioned to study and refine expectations for the hospitalist as consultant and comanager in the ever-evolving healthcare system.

REFERENCES

1. Society of Hospital Medicine, Medical Group Management Association (MGMA). State of Hospital Medicine Report. Philadelphia; 2012. 2. Khera N, Stroobant J, Primhak RA, et al. Training the ideal hospital doctor: The specialist registrars’ perspective. Med Educ. 2001;35(10):957-966. 3. Merriam-Webster Medline Plus. Medical Dictionary. 2012. Available at: http://www.nlm.nih.gov/medlineplus/mplusdictionary.html. Accessed Jan 6, 2013. 4. Whinney C, Michota F. Surgical comanagement: A natural evolution of hospitalist practice. J Hosp Med. 2008;3(5):394-397. 5. Huddleston JM, Long KH, Naessens JM, et al. Medical and surgical comanagement after elective hip and knee arthroplasty: A randomized, controlled trial. Ann Intern Med. 2004;141(1):28-38. 6. Jachna CM, Whittle J, Lukert B, et al. Effect of hospitalist consultation on treatment of osteoporosis in hip fracture patients. Osteoporos Int. 2003;14(8):665-671. 7. Auerbach AD, Rasic MA, Sehgal N, et al. Opportunity missed: Medical consultation, resource use, and quality of care of patients undergoing major surgery. Arch Intern Med. 2007;167(21):2338-2344. 8. Vellody K, Zitelli BJ. Consultative pediatrics in the new millenium. J Hosp Med. 2010;5(1):E34-40. 9. Connolly B, Mahant S. The pediatric hospitalist and interventional radiologist: A model for clinical care in pediatric interventional radiology. J Vasc Intervention Radiol. 2006;17(11, Part 1):1733-1738. 10. Mahant S, Mekky M, Parkin P. Integrating pediatric hospitalists in the academic health science center: Practice and perceptions in a Canadian center. J Hosp Med. 2010;5(4):228-233. 11. Burden M, Sarcone E, Keniston A, et al. Prospective comparison of curbside versus formal consultations. J Hosp Med. 2013;8(1):31-35. 12. Stucky ER, Maniscalco J, Ottolini MC, et al. (eds.). The Pediatric Hospital Medicine Core Competencies Supplement: A Framework for Curriculum Development by the Society of Hospital Medicine with acknowledgement to pediatric hospitalists from the American Academy of Pediatrics and the Academic Pediatric Association. J Hosp Med.

2010;5(Suppl 2): i-xv, 1-114. 13. Wright R, Howell E, Landis R, et al. A case-based teaching module combined with audit and feedback to improve the quality of consultations. J Hosp Med. 2009;4(8):486-489. 14. Hinami K, Whelan C, Konetzka RT, et al. Effects of provider characteristics on care coordination under comanagement. J Hosp Med. 2010;5(9):508-513. 15. Hinami K, Feinglass J, Ferranti DE, et al. Potential role of comanagement in “rescue” of surgical patients. Am J Managed Care. 2011;17(9):e333-339. 16. Fisher AA, Davis MW, Rubenach SE, et al. Outcomes for older patients with hip fractures: The impact of orthopedic and geriatric medicine cocare. J Orthop Trauma. 2006;20(3):172-180. 17. Lucena JF, Alegre F, Rodil R et al. Results of a retrospective observational study of intermediate care staffed by hospitalists: Impact on mortality, co-management, and teaching. J Hosp Med. 2012;7(5):411415. 18. Auerbach A, Wachter RM, Cheng H, et al. Comanagement of surgical patients between neurosurgeons and hospitalists. Arch Intern Med. 2010;170(22):2004-2010. 19. Simon TD, Eilert R, Dickinson LM, et al. Pediatric hospitalist comanagement of spinal fusion surgery patients. J Hosp Med. 2007;2(1):23-30. 20. Rappaport DI, Pressel DM. Pediatric hospitalist comanagement of surgical patients: Challenges and opportunities. Clin Pediatr. 2008;47(2):114-121. 21. Lye PS, American Academy of Pediatrics Committee on Hospital Care and Section on Hospital Medicine. Clinical report—physicians’ roles in coordinating care of hospitalized children. Pediatrics. 2010;126(4):829832. 22. Jaimovich DG, Committee on Hospital Care, Section on Critical Care. Admission and discharge guidelines for the pediatric patient requiring intermediate care. Pediatrics. 2004;113(5):1430-1433. 23. Rosenberg DI, Moss MM, Committee on Hospital Care, Section on

Critical Care. Guidelines and levels of care for pediatric intensive care units. Pediatrics. 2004;114(4):1114-1125. 24. Cotton VR. Legal risks of “curbside” consults. Am J Managed Cardiol. 2010;106(1):135-138. 25. Salerno SM, Hurst FP, Halvorson S, et al. Principles of effective consultation: An update for the 21st-century consultant. Arch Intern Med. 2007;167(3):271-275. 26. Hinami K, Whelan CT, Konetzka RT, et al. Provider expectations and experiences of comanagement. J Hosp Med. 2011;6(7):401-404. 27. Simon TD, Berry J, Feudtner C et al. Children with complex chronic conditions in inpatient hospital settings in the United States. Pediatrics. 2010;126(4):647-655. 28. Berwick DM, Nolan TW, Whittington J. The Triple aim: Care, health, and cost. Health Affairs. 2008;27(3):759-769. 29. United States Congress and the White House. Healthcare Reform: Affordable Care Act. 2010. Available at: http://www.whitehouse.gov/ healthreform/healthcare-overview#healthcare-menu. Accessed Oct 20, 2012. 30. Fisher ES. Pediatric Hospital Medicine: Historical Perspectives, Inspired Future. Curr Prob Pediatr Adolesc Health Care. 2012;42(5):107-112.

CHAPTER

9

Child Development: Implications for Inpatient Medicine Deirdre A.L. Caplin

UNDERSTANDING DEVELOPMENT Understanding child development and its associated constructs sets the care of children apart from the care of adults.1 There is consensus on the need to provide developmentally appropriate health care to children and adolescents, especially in the context of disability and chronic illness.2 When provided with an appropriate venue children across developmental ages can develop mastery in skills necessary to adapt and cope with illness, hospitalization, and treatment. They begin to understand many aspects of illness and treatment. Over time they are adept participants in care and treatment planning, able to communicate their needs as active partners in physician-patient-parent communication triads.3

DEVELOPMENTAL STREAMS AND MILESTONES Human development is a highly dynamic process that involves an interaction between the genetic and neurologic makeup of a child with the environment in which he or she lives.4 Children are typically monitored in health supervision visits across dimensions or streams of development including physical, social and emotional, language, and cognitive growth.1 Milestones are the myriad of developmental achievements that occur in typical patterns over predictable sequences in time.4 Knowledge of these sequences aids the inpatient medicine physician in being able to explain diagnosis, care, and treatment to patients and families, aid parents in helping their children adapt to hospitalization, and promote health and well-being in a developmentally

appropriate manner. Table 9-1 outlines the specific milestones for various developmental streams, with special attention paid to when the absence of a skill becomes a red flag for problems. TABLE 9-1

Major Developmental Milestones Age at Which Absence of Skill Is a “Red Flag”

Average Developmental Age Physical Tasks 3 months

Pulls to sit, rolls over

5 months

6 months

Sits without support

7 months

7 months

Stands while holding on

9 months

12 months

Walks

14 months

17 months

Walks up stairs

21 months

24 months

Jumps with both feet

28 months

30 months

Stands on one foot momentarily

36 months

3.5 years

Hops

4 years

4.5 years

Walks a straight line back 5.5 years and forth, balances on one foot

5.5 years

Skips, rides a bike with training wheels

6 years

6.5 years

Repeats a sequence of movements

7 years

7.5 years

Hops, moves in rhythm, throws a ball >25 feet

8 years

9.5 years

Intercepts path of objects 11 years thrown from a distance, can wash and dry own hair Social and Emotional Tasks

Birth

Shows interest, disgust, and distress

Birth

4-6 weeks

Shows recognition of familiar people

2 months

6-8 weeks

Displays social smile

3 months

2 months

Stops crying with anticipation

4 months

3.5 months

Experiences anger, surprise, sadness

4 months

3 months

Engages in interactive “play,” laughing

7 months

6 months

Shows fear, shame, shyness

8 months

8 months

Distressed with caregiver, leaving/excited at return, wary of strangers

12 months

10 months

Tests parent responses to new behavior

15 months

18 months

Reappearance of stranger anxiety, shows need for independence

24 months

24 months

Spontaneously expresses affection

4 years

24 months

Imitates adults and playmates

36 months

30 months

Objects to changes in routine

4 years

3 years

Respond to people with wide range of emotions

5 years

3.5 years

Aware of own gender/sexuality

5 years

3.5 years

Negotiates solutions (makes deals)

4.5 years

4 years

Tells stories, lies; relates day’s events

5 years

6 years

Friendships based on common interests

8 years

7 years

Worries focus on school, health, personal harm

9 years

8 years

Focus on rule-oriented games, competition Worries about friends, acceptance

11 years

11 years

Preference for friends over 15 years family members

12 years

Social awareness is abstract Language Tasks

16 years

3 months

Vocalizes when hears speech

5 months

4 months

Babbling

6 months

7 months

Imitates syllables of language

9 months

7 months

Says “dada” or “baba”

10 months

8 months

Responds to his/her name

10 months

9 months

Points to indicate interest or desire

12 months

10 months

Repeats sounds/gestures for attention

12 months

12 months

Knows one word of meaning

14 months

14 months

Has at least three words of 16 months meaning

15 months

Attends to simple commands/gestures

18 months

20 months

Points to simple body parts

24 months

21 months

Uses two-word phrases

24 months

24 months

Asks for common objects, food by name

30 months

24 months

Uses at least one personal 30 months pronoun

3.5 years

All speech is intelligible

4 years

3.5 years

Understands prepositions

4 years

4.5 years

Follows three-step commands

5 years

4.5 years

Understands time sequences

5 years

4.8 years

Uses future, past, present tense correctly

5.3 years

5.5 years

Uses irregular nouns correctly

6 years

6 years

Can articulate things that have not yet happened

7.5 years

7 years

Appreciates multiple meanings of words, puns, metaphors

9 years

10 years

Can articulate logical sequence of abstract events (more effective arguing)

13 years

11 years

Understands irony and sarcasm

14 years

12 years

Refined grammatical structures such as the passive voice

15 years

Cognitive Tasks 2 months

Tracks moving objects with eyes

3 months

3 months

Brings objects to mouth

4 months

8 months

Searches for hidden object 12 months

9 months

Uses gestures to indicate thoughts

12 months

12 months

Imitates correct use of objects (cup, brush)

15 months

12 months

Explores new object uses (shake, throw, pull)

18 months

20 months

Can give you “one” of something if asked

24 months

24 months

Begins make-believe play

36 months

30 months

Knows own full name

5 years

36 months

Can follow two-step directions

4 years

4 years

Count sequentially from 1 to 10

5 years

4.5 years

Can fully dress self

5 years

5 years

Knows own birthday or address

6 years

5.5 years

Reads four or more words

7 years

6 years

Understands common jokes/humor

7 years

7 years

Able to provide directions

11 years

12 years

Able to use deductive reasoning

16 years

12 years

Uses abstraction as method of communication and reasoning

16 years

Physical Development Beginning with neonatal reflexes, the development of both gross and fine motor skills is a process of extreme variability, both in the rate of maturation and in the way that skills are achieved.4 Despite the variation, there are constants in motor development that are largely universal. For example, reflexive movement always precedes voluntary movement, proximal control always precedes distal control, and pronation always precedes supination.5 In addition, children develop the ability for a particular action before they learn to inhibit it; if they begin to run, it may take a few falls before they learn how to stop. Social and Emotional Development The development of adequate social and emotional skills is one of the most complex and critical tasks of childhood. Social development is interdependent on other streams of development as well as influenced by environment. Attachments formed in infancy are critical for emotional, social, and behavioral self-regulation and the formation of social relationships with others.4,6 In the medical setting, coping with hospitalization, separation, and overall adjustment to disease and illness, as well as family adaptation and adherence to regimens are correlated to the social and emotional abilities of a chronically ill child.3 Language Development Language is part of a broader set of communication skills that involve a combination of speech content and character (e.g. intonation), nonverbal gestures, attention, and comprehension skills. These skills are thought to be the building blocks for socialization, memory formation, achievement, and learning. In medicine, patient-provider communication involves understanding the level at which your patient can communicate effectively to you, and is vital to quality patient care.7 Eliciting symptoms from children, explaining illness, procedures, and regimens adequately necessitates a developmentally sensitive approach, which requires a fundamental understanding of the basic developmental milestones of expressive and receptive communication. Cognitive Development Cognitive development is a known determinant of child understanding of illness and pain.8-10 Historically, research has explored how cognition shapes a child’s attitudes, beliefs, and behavior, as well as overall adjustment to disease, injury, and illness.8-10 According to Piaget’s theory, children progress through stages of development, each with tasks necessary for cognitive progression to the next stage. Piagetian stages

have persisted as the foundation for how children formulate ideas about their own health and illness (Table 9-2). Although type of information presented and prior experience can enhance understanding, it has been shown that cognitive developmental status predicts childhood understanding of disease and medical procedures better than age or other variables do.6,8-10 TABLE 9-2

Piagetian Stages and Understanding of Illness

Piagetian Stage

Developmental Ages Tasks/Status

Understanding of Illness

Sensorimotor period

0–2 years

Status: Developing schema to integrate and organize motor and sensory input from the environment Task: Intentional goal-directed behavior

• No real understanding of illness • Experience is largely sensory and immediate

Preoperational 2–7 period years

Status: Concrete thinking, irreversibility of experiences, fantasy-reality confusion, egocentrism, poor generalizability Task: Generation of a internal cognitive structure based on permanence and conservation

• Illness is a sensory experience • Cause and cure are often magical and illogical • View is an egocentric experience (not generalizable)

Concrete operational period

7–11 years

Status: Temporal and spatial understanding, reversibility, conservation all present. This allows for distinction between fantasy and reality and ability for rule orientation Task: Ability for logical thinking but still in concrete and experiential context

• Inaccuracies result as complexity of understanding increases • Cause of illness is external and concrete and control is related to avoidance • Able to describe body processes • Appreciate the reversibility of illness (cure)

Formal operational period

11+ years

Status: Thinking reflects logical causality with the ability for both inductive and deductive reasoning Task: Understanding of hypothetical and abstract events

• Able to perceive multiple causes and cures • Understand that their actions influence the disease process • Able to differentiate between physical and psychological domains of self • Aware that thoughts and feelings can affect how the body functions

“ON-THE FLY” ASSESSMENT IN MEDICAL SETTINGS Formal developmental assessment is not reasonable in a busy inpatient setting and although a sensitivity to and understanding of development are

helpful they are not typically the focus of inpatient care. However, despite its complexity, development may be rapidly and rather easily screened as part of every examination. Parents are an excellent source of information about development because most know what their child can do if asked. In addition to parents, Dixon and Stein note that “children will [often] do their own developmental assessment” if given the opportunity.11 Presenting pediatric patients with a means of communication (i.e. a toy, a crayon, a tongue blade or stethoscope, a challenge) will facilitate children letting practitioners know what they are capable of and how they think. Observing the parent-child dyad lets you see how the child is developing socially, how well the parent can read a child’s signals, and where a child’s communication skills are. The key to success is often knowing what to look for and knowing how to interpret one’s observations. As one developmental expert stated, “Knowledge of child development allows us to be efficient and even downright lazy as we get the child and family to do all the work.”4

DEVELOPMENTAL IMPLICATIONS FOR THE HOSPITALIZED CHILD INFANCY Adaptation to Illness, Hospitalization, and Treatment Infants, even the very young, are intuitive and reactive to their environment. Contact— physical and verbal—is important to creating a safe and comfortable environment for the infant patient. The body of literature on attachment suggests that even at young ages the infant recognizes primary caregivers as distinguished from the strangers typically caring for them in the hospital, suggesting that parents are critical to the care team.5 Additionally, disruptions of attachment are uncommon but they increase risk for problems with nutritional status, irritability, and responsivity to parents.6 Changes in routine can also disrupt feeding, sleeping, and temperament.4 Few behavioral changes are noted with hospitalization at this age, and young infants generally reestablish patterns quickly after hospital discharge. From a cognitive development perspective, infants do not have the ability to understand the concept of illness. However, most of what they learn in the sensorimotor period they learn from integrating sensory input with motor

responses, making it important to provide opportunities for motor and sensory exploration.1,4 Participation in Care Physicians should not expect the infant to participate directly in care. It is important to teach parents to be aware of and responsive to infant cues regarding symptoms and responses to treatment. This interaction provides a rich opportunity for early learning in successful disease management strategies, especially for children with chronic illness.6,10,12 Physician-Patient-Parent Communication For many families, diagnosis is a time of guilt, worry, and negative emotions that could have a significant impact on mental and physical health over time.6 Communication with parents of hospitalized infants should have as its goal to maximize the parent’s ability to engage in a meaningful way in order to provide appropriate interventions.6 For infants with chronic illness, the hospital stay provides an opportunity to assist families in the development of effective routines around treatment tasks that take into account the infant’s developmental limitations and strengths.

EARLY CHILDHOOD Adaptation to Illness, Hospitalization, and Treatment In the toddler and preschool years, behavioral changes associated with hospitalization are more pronounced and more prolonged than in younger infants. A toddler is wary of strangers and anxious about the separation from parents.4 Tolerance for separation improves as the toddler ages into preschool years. Young children develop a broader cognitive repertoire and can grasp the idea that an absent parent will return. Young children have a highly developed memory and imagination which results in inaccuracies and inconsistencies regarding illness.6,8,10,11 Attribution of illness often applies to magical things, superstitions, a finding that persists despite some young children’s ability to provide sophisticated and detailed dialogues about their disease.6 Evidence suggests that children at this age are better at remembering words spoken by doctors and parents about their illness but fail to retain an accurate meaning.9 Subsequently, children often develop their own causal hypotheses for illness and their role in it: “I

was bad, so I got sick. If I am good, I will get better.” Participation in Care Hospitalization can be threatening and difficult for a young child, which could result in resistance to or refusal of certain aspects of care. The burden of treatment may also increase opportunities for resistance in the context of an increased desire for the developmentally expected independence that occurs in the toddler years.6 The very behaviors that parents report as the focus of battles for independence in well child care, such as sleeping, feeding, dressing, and elimination, provide a potential source of contention with medical restrictions or requirements for care.4 Conversely, taking advantage of the young child’s natural curiosity, desire for independence, and ability for self-control can be a great asset in engaging them actively in their own care.4,6,11 Physician-Patient-Parent Communication Developmentally accurate information should be targeted for simplicity and accuracy, as the number of details and complexity interfere with comprehension in the preoperational child.9 Preschoolers have a tendency to confuse cause and effect, and have a poor perception of time.8 It is important for clinicians to take extra time to help their preschool-aged patients understand that body state, germs, or accidents may be the cause of their current illness, rather than say the doctor or being in the hospital. In addition, engaging parents and children together in care through well-developed treatment plans has been shown to increase tolerance for treatment and reports of satisfaction with care from parents of young children.6

MIDDLE CHILDHOOD Adaptation to Illness, Hospitalization, and Treatment Fewer behavior problems are expected in school-aged children because they have acquired many cognitive skills that help them process the stress of hospitalization. They are capable of comprehending the cause of their illness.4,6,8,9 Emotionally, children at this stage have emerging internal control over their behavior, allowing for greater self-regulation of response and use of problem-focused coping strategies (seeking support, planning) rather than emotion-focused coping (avoidance, distraction). Secondary strategies, such as self-talk, and active distraction are a feature of increasing

cognitive maturity, all of which require the use of more subtle cognitive processes achieved in this stage.10 Emotionally, school-aged patients rely heavily on peer engagement, organized activity, and a global sense of initiative, typically fostered by school, sports, and social activities. Identity and competence are largely assessed by comparison to peers.7,13 Prolonged hospital stays, disease management regimens, activity restrictions, and other challenges associated with a sick child are all potential impediments to expected tasks of the school-aged child, and if not addressed can become a source of emotional reactivity and poor adaptation. For some children, illness may interfere with an otherwise active and busy lifestyle. For others, responding to peer questions, dealing with teasing, or visible reminders of an illness can be sources of emotional distress.6,10,13 Participation in Care Middle childhood is the time when children become more active partners in their medical care.6,7,13,14 Readiness for self-care is more accurately marked by cognitive maturity rather than chronological age, and self-care abilities increase with maturity.14 Children as young as 5 to 8 years are aware of changes in body sensations that help them identify symptoms and care needs. By middle childhood, most children are able to read, to communicate on their own behalf, to solve everyday problems with concrete thought.14 At middle school age, children typically have an accurate understanding of illness causality and reversibility, and grasp the relationships between symptoms and body systems.13 Parents and physicians are both aware of the need for child participation in some aspects of care. However, there are documented differences in how much self-care should be expected and at what age.14 Encouraging parents and clinicians to have developmentally appropriate expectations for tasks and behaviors will likely facilitate full engagement of the child as part of his or her care team.6 Physician-Patient-Parent Communication Children achieve greater flexibility and organization in their grasp of illness concepts over time, which makes them easier to communicate with.9,13,14 However, school-aged children maintain a great deal of variability in both knowledge and application of logic, which may result in the backfiring of proactive

messages, regardless of how much illness education they have received.12 Communication with school-aged patients is dependent on many other factors including the emotional maturity of the child, parent expectations, and the physician expectations. Children report having their own priorities in health, which are not always recognized by clinicians and parents.6 Familyphysician interactions can result in marginalizing children at this age if they perceive that communications are only between the parent and the clinician. Alternatively, parents who foster communications directly between their school-aged child and their child’s physician are more likely to have children with more independence and initiative in their care.14

ADOLESCENCE Adaptation to Illness, Hospitalization, and Treatment Most adolescents have at least a concrete understanding of their own disease, but few achieve a formal operational understanding, even up to age 17.12 Cognition continues to refine in adolescence, becoming more abstract through experiences. Typically developing adolescents possess the cognitive skills needed to understand the cause of their illness and the need for hospitalization and treatment.6 However, at a time when peer influences and the need for autonomy are at a peak, hospitalization and the external structure that it imposes can be distressful. The disruption of present and perhaps future life may cause much psychic distress. Prolonged absences from school, reliance on parents and others for care, the somatic invasion associated with treatment, or a heightened sense of a potentially foreshortened future may interfere with normal developmental requirements.6,15 Secondary to the increased incidence of many chronic conditions in adolescence, hospitalization may carry with it lifelong changes in the context of individuation, identity development, and socialization.13 Potential threats to optimal social development and emotional maturation increase with prolonged or chronic illness, often because of the impact it has on school attendance and performance, and inhibition of social experiences.13 Participation in Care The capacity for self-management depends on developmental age, but is also influenced by context, task, and emotional

factors.15 Development in adolescence can be at odds with the behaviors necessary for positive disease management. A positive correlation between cognitive maturity and negative changes in health-related behaviors and adherence to medical regimens has been noted in adolescents with chronic conditions.6 The extent to which this finding exists in those teens with acute illness is unknown. However, with any adolescent patient, an approach that balances their expressed needs with parent involvement and physician support and patience is likely to improve adolescent motivation and commitment to the care plan. Physician-Patient-Parent Communication Communication with the adolescent patient is often “practice” for their adoption of adult roles over time.2 There is a significant literature on medical communication in this transitional period, most of it addressing the role of the adolescent in decision-making, autonomy in care, determination of competence, and readiness for transition to adult care settings.2,3,6,15 Studies have indicated that there is a great deal of variability in interest and commitment to healthcare by adolescents, resulting in an uneven process of negotiating with adolescents and their parents over long periods of time.13 Successful communication with adolescents often requires the clinician to bring their teen patients’ priorities to light and address them within the context of appropriate care, patience, and respect.2

SUPPORTING A DEVELOPMENTAL APPROACH IN MEDICAL SETTINGS The goal of hospitalization is to promote healing of the body as well as rapid recovery from the physical, psychological, and social impacts of the admission. A developmental approach to healthcare optimizes the ability of children and adolescents to be active members of the healthcare team.2 Many children’s hospitals are equipped with resources that can facilitate a developmental approach, including behavioral health teams, child life specialists, and social work teams. There are successful programs in place to acquaint children with hospital environments, prepare them for procedures, and encourage age-appropriate medical play and role-playing. Hospitals allow and encourage parents to room-in, as well as to visit liberally. As the

leader of this team, the hospital pediatrician is charged with supporting his or her team in understanding this relationship and accurately assessing each child’s developmental level. Accurate developmental information facilitates children participating at an age-appropriate level in their own care, coping with hospitalization and illness, and developing mastery of an often stressful experience.11

SUGGESTED READINGS American Academy of Pediatrics: Bright Futures: Tool and Resource Kit. Elk GroveVillage, IL: AAP Publications; 2008. Dixon S, Stein M. Encounters with Children: Pediatric Behavior and Development. 4th ed. St. Louis: Mosby, 2006.

REFERENCES 1. Hagan J, Shaw J, Duncan P. Bright Futures: Guidelines for Health Supervision of Infants, Children, and Adolescents. 3rd ed. Elk GroveVillage, IL: AAP Publications; 2008. 2. Sawyer S. Developmentally appropriate healthcare for young people with chronic illness: Questions of philosophy, policy, and practice. Pediatr Pulmonol. 2003;36:363-5. 3. Leatherman S, McCarthy D. Quality of Health Care for Children and Adolescents: A Chartbook. Chapel Hill, NC: University of North Carolina Program on Health Outcomes, 2004. 4. DeCivita M, Dobkin PL. Pediatric adherence as a multidimensional and dynamic construct, involving a triadic partnership. J Pediatr Psychol. 2004;29(3):157-169. 5. Dosman C, Andrews D. Anticipatory guidance for cognitive and socialemotional development. Pediatr Child Health. 2012;17(2):75-80. 6. Touwen BC. The neurological development of prehension: A developmental neurologist’s view. Int J Psychophysiol. 1995;19:115127. 7. Ernst MM, Johnson MC, Stark LJ. Developmental and psychosocial

issues in CF. Child Adolesc Psychiatr Clinics North Am. 2010;19(2):263-viii. 8. Redpath C, Rogers C. Healthy young children’s concepts of hospitals, medical personnel, operations, and illness. J Pediatr Psychol. 1984;9(1):29-40. 9. Potter PC, Roberts MC. Children’s perceptions of chronic illness: The roles of disease symptoms, cognitive development, and information. J Pediatr Psychol. 1984;9(1):13-27. 10. Band EB. Children’s coping with diabetes: Understanding the role of cognitive development. J Pediatr Psychol. 1990;15(1):27-41. 11. Dixon S, Stein M. Encounters with Children: Pediatric Behavior and Development. 4th ed. St. Louis: CV Mosby, 2006. 12. Berry AL, Hayford JR, Ross CK, et al. Conceptions of illness by children with juvenile rheumatoid arthritis. J Pediatr Psychol. 1993;18(1):83-97. 13. Clark NM, Dodge JA, Thomas LJ, et al. Asthma in 10 to 13 year olds: Challenges at a time of transition. Clin Pediatr. 2010;49(10):931-937. 14. Buford TA. Transfer of asthma management responsibility from parents to their school-age children. J Pediatr Nursing. 2004;19(1):3-12. 15. Sawyer S, Drew S, Yeo M, Britto M. Adolescents with a chronic condition: Challenges living, challenges treating. Lancet. 2007;369:1481-9.

CHAPTER

10

Palliative Care Tammy Kang, Lindsay Burns Ragsdale, Daniel J. Licht, Oscar H. Mayer, Gina Santucci, Malinda Ann Hill, Jennifer Hwang, and Chris Feudtner

The field of pediatric palliative medicine has undergone significant growth in the last decade. Palliative care services were once only considered appropriate for children who were imminently dying, but are now recognized as vital to the care of those with complex potentially life-threatening conditions. Hospitals that care for the approximately 55,000 children who die each year in the United States have an obligation to provide excellent pediatric palliative care.1-3 Although palliative care is becoming standard of care in many hospitals, on average only 10% of pediatric patients who are eligible receive palliative care.1 A recent survey by the Center to Advance Palliative Care found that although 69% of hospitals caring for children had a pediatric palliative care program, the staffing and scope of programs varied widely.4 Healthcare professionals find this domain of practice extremely difficult and referral for services is sometimes delayed or omitted. This chapter addresses the core challenges of providing palliative care, supplying practical and, to the extent possible, evidence-based answers to these challenges.

GOALS OF PALLIATIVE CARE Palliative care seeks to maximize quality of life for patients and families through an interdisciplinary approach to minimize distressing or uncomfortable symptoms that patients experience and maximizing the quality of their remaining lives, while providing support for decision making concentrating on aligning treatments with family and patient goals of care. These services are provided across the care continuum and across care locations in conjunction with disease-directed therapies. Collaboration and

communication among patients, families, and healthcare professionals is essential.

IDENTIFYING PATIENTS WHO REQUIRE PALLIATIVE CARE Figuring out which patients and families would benefit from palliative care, and when, is challenging for two main reasons. First, the prevailing medical model presents palliative care as a mutually exclusive alternative to curative care. This is a false dichotomy: patients can simultaneously receive care that seeks to cure disease or extend life while also receiving complementary care that seeks to minimize bothersome symptoms and maximize the quality of life (Figure 10-1). Second, among the approximately 55,000 children who die each year in the United States,5 several trajectories of dying exist, including6:

FIGURE 10-1. Palliative care should complement other modes of care. 1. Children who die suddenly, before any diagnosis is made (e.g. due to trauma or conditions such as sudden infant death syndrome or occult cardiac arrhythmias). These families warrant bereavement care.

2. Children whose conditions are inevitably fatal. This group can be subdivided into the following groups: a. Patients who will inevitably die relatively quickly after diagnosis (e.g. nonviable prematurity, inoperable brain tumor). All of these patients warrant palliative care immediately. b. Patients who will inevitably die, but years to decades after diagnosis (e.g. many neurodegenerative disorders). These patients warrant palliative care, but when to institute such care is debatable. We believe that, along with life-extending care, complementary palliative care and advanced-care planning should be initiated at the time of diagnosis. 3. Children who have conditions that make them extremely frail and vulnerable (e.g. severe spastic quadriplegia with swallowing dysfunction and risk of aspiration pneumonia; cancer that requires debilitating chemotherapy; sepsis that makes the child critically ill). Because of their fragile health, such children have an increased risk of dying on any given day; however, the risk is still low enough that although death is likely at some point, it is not inevitable in the next year or even the next decade. For these patients, complementary palliative care and advanced-care planning are warranted as early as possible, with a transition toward a more exclusive focus on palliative care if the condition progresses to the point where the benefits of life-extending care are outweighed by the suffering such care imposes. A study evaluated demographic and clinical features of 24,342 children who died >=5 days after admission in a sample of children’s hospitals, comparing those who did and those who did not receive a palliative care consultation. About 4% of children received palliative care. Palliative care was more common in older patients and was associated with fewer hospitalization days prior to death. Children who received palliative care in this study were less likely to have invasive interventions, received fewer medications, and were less likely to die in an ICU.7 Another study provides a snapshot of 515 children receiving palliative care by six hospital-based pediatric palliative care teams in the United States and Canada. The two most common principal underlying diagnoses in this cohort of children were genetic or congenital disorders (40.8%) and neuromuscular disorders (39.2%). The study further highlighted that

palliative care for children is principally about how to help children and families live with serious illnesses, as 69.7% of the patients were still alive at the end of the 12-month follow-up.8

DISCUSSING PALLIATIVE CARE Talking about pediatric palliative care with families is one of the most demanding and difficult tasks of clinical medicine. Dividing the task into two major components—what the patient and family are up against, and what can and will be done—and having a distinct plan for each aspect of the conversation (which will likely occur over several interactions) can be helpful. The first component focuses on delivering the bad news itself, which may be a new diagnosis or the latest in a long series of illnesses and deteriorating health. This bad news threatens the goals, plans, and hopes of patients and parents, and the resulting emotional tumult can make even the most seasoned clinician feel overwhelmed and desperate. Preparation is therefore essential: have a precise plan of what information is to be conveyed to whom, allow ample time to hold this meeting, and have the discussion in a private environment with appropriate support persons (e.g. other family members, nursing staff, social workers) present. After briefly reviewing the clinical events, preface your remarks with a warning, such as, “I’m sad to say that the news I have is bad,” and then state the new information as simply as possible, such as, “The tests show that your child has cancer.” Then stop talking and let the family assimilate this information. Your quiet, compassionate presence will be perceived as supportive. Answer questions as they are posed, avoiding the temptation to downplay or sugar-coat the bad news. Given the turmoil that such news often creates in the minds of those who receive it, simply relaying the information and responding to initial questions may be all that can be accomplished at the first meeting. Before ending the meeting, however, propose some specific plans for follow-up conversations or actions. For example, say, “I will be back to check on you and your child in 2 hours, and I can answer any additional questions that may have occurred to you and start to discuss plans at that point.” The second component of palliative conversations focuses on possible therapeutic actions and deciding on which ones to pursue.9,10 After the family

has had adequate time to absorb the bad news (which, depending on its severity and unexpectedness, can take from minutes to weeks or even longer), the objective of this conversation is to help formulate new goals, plans, and hopes in light of the constraints imposed by the diagnosis. Instead of focusing on dying and death per se, this conversation focuses on what the therapeutic alliance among patient, family, and care team can strive to accomplish, despite the fact that death is likely to occur in the near future. A useful way to proceed is along the following lines: “Learning that your child has an incurable condition that will shorten her [or his] life has been incredibly upsetting and difficult. Given the problems that the condition creates, I am wondering how we can best care for your child. What are your major hopes for her?” At this juncture, family members often both mourn the loss of hope for a normal life and mention the desire to keep pain or other forms of suffering to a minimum or quality-of-life goals, including social or spiritual concerns. These hopes provide the basic goals of all subsequent palliative and end-of-life care plans, and they are the compass by which the rest of clinical care is oriented.

DISPELLING THE MYTH THAT PALLIATIVE CARE MEANS GIVING UP One of the most challenging aspects of providing palliative care to children and families has been overcoming the stigma that palliative care entails giving up; that to receive palliative care services, parents must forego disease-directed treatments. Historically this has been the case when hospice services are engaged. Since palliative care and hospice care are often linked, the bias that families feel toward hospice has extended to palliative care teams. However, this provision changed in 2010 for patients under the age of 21 with the passing of the Patient Protection and Affordable Health Care Act (PPACA). This act includes a provision entitled “Concurrent Care for Children” which requires that programs for children in state Medicaid or Children’s Health Insurance Programs (CHIP) allow children under the age of 21 to receive hospice care while still receiving curative treatments, as discussed in further detail later in this chapter.11

COMPLEX CHRONIC CARE

The evidence is clear that pediatric patient care has become more complex and an increasing proportion of hospitalizations are attributable to pediatric patients with complex chronic conditions (CCC). Many of these patients receive palliative care consults to help with symptom management, qualityof-life issues, or end-of-life care. Despite the overlap of palliative care and the care for pediatric patients with CCC, these patients still require robust, dedicated primary and specialty care services and care coordination. Palliative care can be an important adjunct to the treatment team; however, the patients require ongoing care coordination sometimes out of the scope and staffing abilities of a palliative care team.12-14 Many hospitals have developed specialized pediatric medical teams to help manage and coordinate care for this population.15 More pediatric patients are able to pursue parallel disease-directed care and palliative care at the same time due to the “Concurrent Care” provision of new federal regulations. Palliative care can start at diagnosis of a life-limiting illness and patients can benefit from the depth of services throughout the disease process. In the future, we hope that pediatric patients can continue to benefit from concurrent palliative care to maximize their quality of life.

MINIMIZING UNPLEASANT SYMPTOMS Children who are dying often suffer with seven main symptoms that are managed most effectively within a holistic framework that includes but extends well beyond pharmacotherapy.16

NAUSEA AND VOMITING Many children experience nausea and vomiting for a variety of reasons, including chemotherapy, other drugs, metabolic disturbances, central nervous system tumors, vestibular or middle ear pathology, gastrointestinal pathology, and anxiety or other conditioned responses. Treatment includes both pharmacologic and nonpharmacologic approaches. Medications commonly used include prochlorperazine, ondansetron, granisetron, scopolamine, dexamethasone, and benzodiazepines (use of metoclopramide has decreased since the black box warning about tardive dyskinesia in 2009 by the FDA). Unfortunately, scant data support the effectiveness of these drugs in the

pediatric palliative care setting. Initial therapy should target the primary cause of the nausea or vomiting (e.g. dexamethasone for vomiting due to raised intracranial pressure from a brain tumor, or a benzodiazepine for nausea caused by anxiety). These medications can then be titrated to a maximal dose, and if that is ineffective, therapeutic control with another agent can be attempted. Often, symptom relief can be achieved only with multiple agents. Some of the most effective nonpharmacologic methods are providing small, frequent meals; giving medications after meals if possible; and eliminating smells and tastes that exacerbate the symptoms.17

FATIGUE Fatigue is one of the most prevalent symptoms in children at the end of life. The definition of fatigue in the palliative care setting varies, but in general, it refers to the subjective feeling of being tired or lacking energy. Fatigue usually stems from several concomitant causes (Table 10-1). Although assessment tools exist for fatigue in children, the diagnosis is based on subjective reports by the patient or parent.18-20 Treatment begins by addressing any possible underlying causes. Maximizing the patient’s rest is important, and this may involve the use of sleep-promoting agents. Increasing wakefulness may also be beneficial; the potent psychostimulant amphetamine has produced consistent improvements in validated measures of fatigue in randomized, controlled trials in adult patients with cancer, human immunodeficiency virus (HIV), chronic fatigue syndrome, and multiple sclerosis. Less potent psychostimulants, such as methylphenidate, dextroamphetamine, or corticosteroids, may also improve fatigue.18-20 TABLE 10-1

Common Causes of Fatigue in Palliative Care Settings

Depression Anxiety Pain Poor nutrition Medication side effects Hypoxia

Infection Dehydration

PAIN Most children experience pain at some point during their illness. Pain is a subjective symptom that is physically and emotionally distressing for both the patient and caregivers. The type of pain may be somatic, visceral, or neuropathic and may be the result of a combination of disease-related, treatment-related, and psychological causes. It is important to make a detailed assessment of pain, including location, duration, and possible causes. It is also important to assess the patient’s ability to take oral medications and the family’s reaction to the use of pain medications. Many parents are afraid to permit treatment with opioids owing to the widespread negative cultural perception of opiates as drugs of abuse, and they need reassurance. Any treatment must take into account the developmental level of the child as well as the environment (home, hospital, or health care facility) in which the child lives.21,22 The World Health Organization’s (WHO) treatment ladder is a reliable tool for managing pain (Figure 10-2); it guides physicians to escalate therapy depending on whether the degree of pain is mild, moderate, or severe and whether the previous level of treatment intensity eliminated the pain. Treatment should begin with mild analgesics such as nonsteroidal antiinflammatory drugs and acetaminophen and, based on the patient’s response, can escalate to opioids. Two of the essential components of good palliative care are to assess pain frequently and treat it aggressively (see Chapter 197). In 2012, the WHO excluded codeine from the pain treatment ladder for persistent pain in children.23 Codeine, which is a weak opioid, has known wide variability in metabolism and efficacy in children and is not recommended for pain relief in children.

FIGURE 10-2. This “ladder” approach (based on the World Health Organization’s approach to cancer pain management) can be used to guide the escalation of pain management for dying children. PCA, patient-controlled analgesia.

ANOREXIA Loss of appetite is common and often results from other symptoms, such as nausea, vomiting, constipation, pain, depression, gastritis, and weakness. It is important to treat the underlying causes if possible. As with nausea and vomiting, nonpharmacologic measures may be helpful, including giving small, frequent meals, removing unpleasant odors, and providing a relaxing atmosphere for meals. Pharmacologic therapies include corticosteroids, megestrol acetate, and dronabinol.17,24,25 Each of these therapies has adverse effects (e.g. anxiety, dizziness, hypertension, hyperglycemia, adrenal insufficiency, fatigue) that can limit their usage.

DYSPNEA The sensation of breathlessness commonly occurs in conditions with respiratory insufficiency. Patients experiencing dyspnea typically breathe in a shallow, rapid pattern as they struggle with musculoskeletal or pleuritic pain (which restricts the range of chest wall excursion), respiratory muscle weakness (as occurs with myopathies or cachectic states), poorly compliant lung tissue (due to infection, heart failure, and other conditions), or lung lesions that compromise the airway (e.g. bronchiectasis, tumor), fill airspaces (e.g. atelectasis, edema, pneumonia), or occupy space in the chest cavity (e.g. pleural fluid). Dyspnea should first be treated by correcting the underlying cause. The accompanying pain also requires effective treatment. The discomfort or fatigue associated with hypoxemia can be ameliorated by

administering supplemental oxygen (recognizing, however, that for patients with long-standing respiratory insufficiency, supplemental oxygen may suppress the hypoxic drive to breathe and cause respiratory suppression). Fatigued respiratory muscles (which contribute to the sense of dyspnea) can often be supported using noninvasive ventilation through a nasal or oronasal interface (see Chapter 209). With proper fitting of the mask and adjustment of the device, continuous or biphasic airway pressure can be delivered either at home or in a health care institution. Finally, the sensation of dyspnea and the associated anxiety can be abated directly with narcotic agents (e.g. morphine) or with anxiolytics (e.g. benzodiazepines), thereby reducing discomfort and maximizing quality of life.

CONSTIPATION This remarkably common and often neglected complaint of dying children requires a daily assessment of stool output and pain with defecation to determine whether treatment with polyethylene glycol (MiraLax), Colace, or senna is adequate. Treatment failure most often occurs because the starting dose of laxative proves to be too small to produce a soft, painless, daily bowel movement or because the laxative is used only intermittently after constipation recurs. Better results ensue when constipation is assessed every day so that if bowel movements are infrequent or painful, doses can be increased quickly to maximal amounts. Anecdotally, the combination of stool-softening agents (polyethylene glycol and Colace) and a peristalsisstimulating agent (senna) may work best for patients with difficult-to-manage constipation. Although enemas, suppositories, or GoLYTELY clean-outs may be useful for patients with neurologic bowel dysfunction, such measures should be used sparingly in other patients, whose persistent constipation should be treated with increased maintenance doses of laxatives (see Chapter 94).

SEIZURES Convulsions may occur as patients approach the end of life, and they are often particularly distressing to patients and family members. Indeed, many parents who witness their child convulsing express the hope that they never have to see a similar event.26 In the context of end-of-life care, clinical

decisions regarding the treatment of seizures are guided by the acuity of presentation, the presence of other organ damage or malfunction, and, most important, the wish to preserve or cloud the patient’s consciousness. Benzodiazepines are the mainstay of treating seizures acutely, but they cause substantial sedation and may cause respiratory depression. Newer oral anticonvulsants such as levetiracetam (Keppra) and oxcarbazepine (Trileptal) are options when sedation is undesirable. Both medications are available only in oral formulations, but they achieve good serum concentrations after only two doses, with negligible sedation and good overall tolerance. Valproic acid (Depakote) also causes negligible sedation and is available in both oral and intravenous formulations, but it should not be used in patients younger than 2 years or in those who have bleeding diatheses (valproic acid interferes with platelet function) or liver dysfunction (the metabolism of valproate is dependent on hepatic function and may interfere with the hepatic metabolism of other drugs). For a child in hospice care who experiences a first seizure, a good treatment plan would be the following: rectal valium (Diastat) (0.5 mg/kg for children younger than 5 years, 0.2 mg/kg for older children and adults) to terminate a seizure in progress, and oxcarbazepine (8–10 mg/kg divided twice a day) started as a scheduled medication. The dose of oxcarbazepine can be increased as needed until dose-limiting toxicity is manifested by double vision, vertigo, or ataxia.

MAXIMIZING QUALITY OF LIFE To support children nearing the end of life, as well as their families, healthcare professionals need to encourage them to maintain their normal lifestyles to the best of their ability and ensure that their philosophy of life is respected. In any discussion about quality of life, the healthcare team needs to take into consideration the family’s cultural values and traditions. In conversations with families, it is important to emphasize improving the quality rather than the quantity of one’s days. There are many ways to maximize quality of life for dying children. Because time is a paramount consideration, the focus should be on making each day meaningful. Healthcare providers should encourage the child and family to make memories out of big and small moments and to celebrate milestones—first tooth, first words, birthdays, graduations, religious ceremonies—while the child is still able to participate. Activities such as keeping a journal, making a

photo album, videotaping special occasions, making imprints of the child’s hands or feet, sharing stories, and keeping locks of hair all help create memories for the whole family.

CHOOSING TO GO HOME The decision about whether to have a child die at home or in the hospital is one of the most difficult that parents face. Not all families are comfortable having a child die at home; the choice depends on many factors, including medical resources, the child’s condition, and the family’s cultural belief system. Even when families decide to take a child home, they must be reassured that they can always return to the hospital. Going home is often a stressful time, especially for long-term patients, because it represents the first step toward separating from the acute medical facility and team and often symbolizes the beginning of the end-of-life journey. To make the transition as seamless as possible, the healthcare team needs to have a well-organized plan that is communicated to everyone involved. The most important consideration in discharge planning is to honor the child’s and family’s wishes. Ideally, the conversation between the healthcare team and the family about going home should take place as early as possible so that referrals to hospice or home care services can be made and strategies for improving the quality of life can be implemented. When raising the possibility of involving a hospice agency, one should anticipate several common misperceptions and address them proactively (Table 10-2). TABLE 10-2

Common Misperceptions about Hospice Care

Misconception

Reality

Hospice is about dying and death

Hospice is about living with life-limiting conditions, making the most of one’s quality of life

Patients need to be “at death’s door” or have less than 6 months to live to

Patients with longer life expectancies can enroll in hospice, and physicians should consider hospice enrollment

enroll in hospice

whenever the goals of care are palliative

Enrollment in hospice means that the patient cannot return to the hospital or receive any lifeextending care

Hospice patients can re-enter the hospital at any time and usually can receive many forms of life-extending care

Hospice enrollment entails stopping all other forms of home nursing care

This is sometimes, but not always, true; direct conversations with the patient’s insurance case manager can often result in more flexible arrangements

Transitioning a dying patient to home or to a hospice facility is best achieved through a concentrated, multidisciplinary team approach. As an advocate for the child and family, the healthcare team can help identify appropriate services and providers, as well as clarify the treatment plan and the patient’s needs with third-party payers to ensure maximum utilization of benefits. The healthcare team can facilitate the discharge planning process by working with its institutional system (e.g. case management, social work) to access all the necessary information and medical orders and to arrange the desired hospice or home care services. Children and families choose to manage their end-of-life care in a variety of ways. Some continue with home care, others transition to home care with hospice services, and still others choose to end their life’s journey in an inpatient hospice or hospital facility. The most important thing to remember is that the journey belongs to the child and the family, and their wishes should be supported.

CONCURRENT CARE FOR CHILDREN Engaging families in palliative care has often been difficult due to the perception that in order to receive palliative care services, one must abandon disease-directed treatments. Although this has not been the case for hospital and community-based palliative care services, it has historically been true for

families wishing for hospice services. In 2010, this requirement changed with the passing of the PPACA. This act includes the “Concurrent Care for Children” provision which requires that programs for children in state Medicaid or CHIPs allow children under the age of 21 to receive hospice care while still receiving curative treatments.27 This provision has the potential to expand hospice services to children and families who may still be receiving disease-directed treatments including chemotherapy, dialysis, private duty nursing, and technology support in the home including ventilators.11 Implementation of the provision has been challenging in many states and utilization of the provision has yet to be fully documented. However, concurrent care for children may open the doors for children requiring technology support at home that requires in-home nursing care to receive the addition of hospice services as their condition progresses. It has also allowed many parents to be able to use hospice services to help maximize quality time at home while still pursuing potentially life-extending treatments.

BEREAVEMENT Families whose children have died need help to heal and find new ways to live. Each member of the family struggles with intense grief and may experience and express that pain differently. During the period of grief, the physical and emotional well-being of the bereaved individual may be threatened. Although rigorous evidence is lacking that health risks and psychological problems can be alleviated or avoided if proper support and help are made available,28 we believe that this absence of evidence should not deter efforts to provide grieving individuals with emotionally supportive interventions to prevent long-term problems. With advances in diagnostic and therapeutic capabilities, the period between the diagnosis of a terminal illness and the moment of death is becoming longer, and people often have time to consider death and grief before the loved one dies. Known as anticipatory grief, this form of grieving may help families prepare for the loss and lower the degree and intensity of grief at the time of a child’s death. Physicians and nurses who detect that parents or other family members are filled with anticipatory grief and acknowledge this emotion (with simple comments such as “I can see how sad

you are”) can help them cope. A comprehensive bereavement program helps families start on the painful journey of grieving. A program should be tailored to meet the unique needs of each family, which includes providing a safe place to sit with sadness, acknowledging the many facets of grief, and helping the family glue the pieces of life back together over time. When parents who have experienced the loss of a child meet together in a grief support group, they often report a sense of peace, healing, and comfort, knowing they are not alone in their grief. Although the journey is not easy, support, hope, and guidance should be offered to families who must begin the process of healing. Grieving families should be provided with a number of services and encouraged to choose which, if any, suit their particular needs. Bereavement follow-up services may include needs assessments and consultations, crisis intervention and referrals, individual counseling (in person or by telephone), support groups (for parents, siblings, and extended family), follow-up mailings and phone calls, written materials and resources, and memorial services. Little is known, however, about the effectiveness of these interventions in assisting the bereaved.

SPIRITUAL ISSUES Most Americans have religious or spiritual beliefs of some kind. When people are confronting grave illness or death, these beliefs and associated rituals or practices are often of paramount concern.29 Accordingly, when caring for a critically ill or dying child, healthcare professionals should always ask patients or family members whether their religious or spiritual beliefs should be considered in the provision of care, and they should offer to arrange a meeting with the hospital’s pastoral care service.

DO-NOT-ATTEMPT-RESUSCITATION ORDERS Do-not-attempt-resuscitation (DNAR) orders are considered at the end of this chapter because there is so much more involved in pediatric palliative care. Indeed, because DNAR orders require a discussion among the healthcare team and the patient and family members about values, preferences, and

views regarding the quality of life—and about whether the patient’s or family’s therapeutic goals are best achieved through cardiopulmonary resuscitation or other modes of care—hospitalists who participate in these discussions should thoroughly understand and be capable of providing the other forms of care outlined in this chapter. The conversation, in other words, should be as much about what will be done to promote comfort as about what will not be done. Ultimately, tailoring the DNAR order to fit the personal therapeutic goals set by the patient or family is an expression of the patientand family-centered philosophy of care that lies at the core of excellent palliative care.

SUGGESTED READINGS American Academy of Pediatrics. Section on Hospice and Palliative Medicine and Committee on Hospital Care, Feudtner C, Friebert S, Jewell J. Pediatric palliative care and hospice care: Committments, guidelines, and recommendations. Pediatrics. 2013;132. doi: 10.1542/peds.2013-2731. Carter BS, Levetown M, Friebert S. Palliative Care for Infants, Children, and Adolescents: A Practical Handbook. Baltimore, MD: Johns Hopkins University Press. 2011. Goldman A, Hain R, Liben S. Oxford Textbook of Palliative Care for Children, 2nd ed. Oxford: Oxford University Press; 2012. Wolfe J, Hinds PS, Sourkes BM. Textbook of Interdisciplinary Pediatric Palliative Care. Philadelphia, PA: Elsevier/Saunders; 2011.

REFERENCES 1. Feudtner C. Perspectives on quality at the end of life. Arch Pediatr Adolesc Med. 2004;158(5):415-418. 2. Behrman RE, Field MJ, eds. When Children Die: Improving Palliative and End-of-Life Care for Children and Their Families. Washington, DC: The National Academy of Sciences; 2003. Institute of Medicine of the National Academies. 3. American Academy of Pediatrics. Committee on Bioethics and Committee on Hospital Care. Palliative care for children. Pediatrics.

2000;106(2 Pt 1):351-357. 4. Remke S AR, Feudtner C, Friebert S, Sieger CE, Weissman D, Wolfe J. Pediatric palliative care programs in children’s hospitals: A crosssectional national survey. 2013. Pediatrics. 132(6):1063-1070. 5. Feudtner C, Hays RM, Haynes G, Geyer JR, Neff JM, Koepsell TD. Deaths attributed to pediatric complex chronic conditions: National trends and implications for supportive care services. Pediatrics. 2001;107(6):E99. 6. Wood F, Simpson S, Barnes E, Hain R. Disease trajectories and ACT/RCPCH categories in paediatric palliative care. Palliat Med. 2010;24(8):796-806. 7. Keele L, Keenan HT, Sheetz J, Bratton SL. Differences in characteristics of dying children who receive and do not receive palliative care. Pediatrics. 2013;132(1):72-78. 8. Feudtner C, Kang TI, Hexem KR, et al. Pediatric palliative care patients: A prospective multicenter cohort study. Pediatrics. 2011;127(6):10941101. 9. Levetown M ME, Gray D. Communication skills and relational abilities. In: Carter BS LM, Friebert S, eds. Palliative Care for Infants, Children and Adolescents. 2nd ed. Balitmore, MD: Johns Hopkins University Press; 2011:169-201. 10. Graham RJ LM, Comeau M. Decision making. In: Carter BS LM, Friebert S, eds. Palliative Care for Infants, Children and Adolescents. 2nd ed. Baltimore, MD: Johns Hopkins University Press; 2011:139-168. 11. Miller EG, Laragione G, Kang TI, Feudtner C. Concurrent care for the medically complex child: Lessons of implementation. J Palliat Med. 2012;15(11):1281-1283. 12. Morrison RS, Maroney-Galin C, Kralovec PD, Meier DE. The growth of palliative care programs in United States hospitals. J Palliat Med. 2005;8(6):1127-1134. 13. Klick JC, Hauer J. Pediatric palliative care. Curr Probl Pediatr Adolesc Health Care. 2010;40(6):120-151. 14. Johnston DL, Nagel K, Friedman DL, Meza JL, Hurwitz CA, Friebert S. Availability and use of palliative care and end-of-life services for

pediatric oncology patients. J Clin Oncol. 2008;26(28):4646-4650. 15. Bergstraesser E. Pediatric palliative care-when quality of life becomes the main focus of treatment. Eur J Pediatr. 2013;172(2):139-150. 16. Hain R, Zeltzer L, Hellstern M, Cohen SO, Orloff S, Gray D. Holistic management of symptoms. In: Carter BS LM, Friebert S, eds. Palliative Care for Infants Children and Adolescents. 2nd ed. Baltimore, MD: Johns Hopkins University Press; 2011:244-274. 17. Santucci G, Mack JW. Common gastrointestinal symptoms in pediatric palliative care: Nausea, vomiting, constipation, anorexia, cachexia. Pediatr Clin North Am. 2007;54(5):673-689, x. 18. Breitbart W, Rosenfeld B, Kaim M, Funesti-Esch J. A randomized, double-blind, placebo-controlled trial of psychostimulants for the treatment of fatigue in ambulatory patients with human immunodeficiency virus disease. Arch Intern Med. 2001;161(3):411420. 19. Sarhill N, Walsh D, Nelson KA, Homsi J, LeGrand S, Davis MP. Methylphenidate for fatigue in advanced cancer: A prospective openlabel pilot study. Am J Hosp Palliat Care. 2001;18(3):187-192. 20. Ullrich CK, Mayer OH. Assessment and management of fatigue and dyspnea in pediatric palliative care. Pediatr Clin North Am. 2007;54(5):735-756, xi. 21. Friedrichsdorf SJ. Pain management in children with advanced cancer and during end-of-life care. Pediatr Hematol Oncol. 2010;27(4):257261. 22. Friedrichsdorf SJ, Kang TI. The management of pain in children with life-limiting illnesses. Pediatr Clin North Am. 2007;54(5):645-672, x. 23. WHO Guidelines on the Pharmacological Treatment of Persisting Pain in Children with Medical Illnesses. Geneva. 2012. 24. Tomiska M, Tomiskova M, Salajka F, Adam Z, Vorlicek J. Palliative treatment of cancer anorexia with oral suspension of megestrol acetate. Neoplasma. 2003;50(3):227-233. 25. Strasser F, Bruera ED. Update on anorexia and cachexia. Hematol Oncol Clin North Am. 2002;16(3):589-617. 26. van Stuijvenberg M, de Vos S, Tjiang GC, Steyerberg EW, Derksen-

Lubsen G, Moll HA. Parents’ fear regarding fever and febrile seizures. Acta Paediatr. 1999;88(6):618-622. 27. The District of Columbia Pediatric Palliative Care Collaborative and the National Hospice and Palliative Care Organization. Concurrent Care for Children Requirement: Implementation Toolkit. 2012. 28. Forte AL, Hill M, Pazder R, Feudtner C. Bereavement care interventions: A systematic review. BMC Palliat Care. 2004;3(1):3. 29. Lanctot D MW, Koch KD, Feudtner C. Spritual Dimensions. In: Carter BS LM, Friebert S, eds. Palliative Care for Infants Children and Adolescents. 2nd ed. Baltimore, MD: Johns Hopkins University Press; 2011:227-243.

CHAPTER

11

Communication and Discharge Planning Daniel A. Rauch and David Zipes

INTRODUCTION In discussing hospitalist models, it is essential to keep in mind the relationships between hospitalists and the wider pediatric community. Communication is at the forefront of any hospitalist model and can be the Achilles’ heel of an otherwise high-quality program. Excellent communication to and from primary care physicians (PCPs) can help a program thrive and prosper. PCPs who are kept in the loop regarding their patients’ hospital courses will likely support and continue to utilize the hospitalist service. By communicating in an effective and timely manner, one of the major potential downsides (loss of valuable information) of a hospitalist service can be avoided. Communication can positively or negatively affect a variety of issues, including but not limited to quality of care, cost of care, patient and physician satisfaction, and liability. With excellent communication, the transition from inpatient to outpatient settings (including transitional care units, chronic care facilities, and rehabilitation hospitals) can be smooth, with minimal to no loss of information.

HANDING OFF CARE The concept of assuming care for other physicians’ patients and then returning their care to the PCPs at discharge is an integral component of all hospitalist models. This is generally accomplished via a formal handoff, which is one of the more controversial and variable aspects of hospital medicine. The intentional creation of discontinuity of care permits a physician (hospitalist) to be present in the hospital for an extended period to

manage inpatients throughout the day. This ongoing presence is one of the biggest advantages of the hospitalist model. As with most things, however, one must accept the good with the bad. As a result of the handoff, the PCP, with whom the patient has fostered a trusting relationship and who knows the patient’s medical history best, is not caring for the patient when he or she is most ill. This situation can result in a loss of essential information (“voltage drop”) from the outpatient to the inpatient setting and vice versa. Complex and expensive laboratory and radiology data, as well as vital information concerning possible medical allergies, medications, code status, and patient’s likes and dislikes, can be lost or poorly or miscommunicated during the transfer. This could result in a variety of negative outcomes—some relatively benign, and others potentially life threatening. With excellent communication between the PCP and the hospitalist, voltage drop can be minimized. In a study of 400 discharged patients, researchers found that 19% of patients suffered adverse events soon after discharge; about half of these events would have been preventable if communication had been adequate.1 The Value in Inpatient Pediatrics (VIP) network has sought to standardize the discharge communication process with their “Pediatric Hospitalists Collaborate to Improve Discharge Communication” project. Additionally, The Pediatric Research in Inpatient Settings (PRIS) network is working on handoffs in the academic setting with their I-PASS project. To date, no standard for communication between the hospitalist and the PCP has been set, and there is significant variation from practice to practice. Communication may occur in person, via telephone, fax, e-mail, text or internet, or sometimes not at all. Maintaining compliance with Health Insurance Portability and Accountability Act (HIPAA) standards adds another challenge to the process. A task force formed by the Society of Hospital Medicine and the Society of General Internal Medicine to address continuity-of-care issues found that more than a quarter of PCPs do not receive discharge summaries on their patients. Additionally, the task force discovered that more than half of discharged patients made contact with their PCPs before the PCPs had received any discharge information—many PCPs did not even know that their patients had been admitted to the hospital. Only 17% of those surveyed stated that hospitalists had notified them before their patients were discharged to home.

The growth of information technology and easy access to e-mail, the internet, faxes, wireless communications, and handheld devices have created many effective modes of communication. New computer and handheld device programs to address communication issues have been developing at a rapid pace, and many of them are quite useful. Template-driven discharge summaries can simplify the process and help ensure that essential information is communicated. The technology to create real-time communication exists, although many hospitalist programs have not made the leap because of cost (both financial and time), unwillingness to change, lack of administrative support, inadequate staffing, or a variety of other reasons. The information communicated, such as discharge summaries or laboratory reports, should be filtered appropriately to maximize the efficiency of communication and reduce the time commitment for PCPs. Some information, such as social issues and end-of-life care discussions, does not lend itself well to electronic communication, and in these situations, the value of the telephone or face-to-face communication should not be overlooked. “Social rounds” by the PCP, either by phone or in person, are an excellent patient satisfaction tool and can help erode the voltage-loss issue and increase the family’s confidence in the hospitalist. One effective technique is for the hospitalist to call the PCP while in the patient’s room so that the family can hear that everyone is on the same page. Similarly, including the PCP in complicated social and medical discussions, such as end-of-life care, code status, case conferences, and major medical or surgical decisions, can be useful to all involved. Telemedicine has not been routinely implemented to communicate with PCPs but with easy access to programs such as Google Talk, Facetime and Skype this technology may be promising. One needs to be cognizant that the PCP will be dealing with the aftermath of the hospitalization. By working as a team, the PCP and the hospitalist can maximize the advantages of hospital medicine while minimizing its disadvantages. The AMA recently published a white paper, There and Home Again, Safely,2 that addresses the PCP’s responsibilities in transitions of care in and out of the hospital setting. It makes clear that communication around patient care is a bi-directional dialogue so that it is reasonable for the hospitalist to have an expectation of communication from the PCP.

DISCHARGE PLANNING Discharge planning is an integral part of hospitalization and is an area in which hospitalists should have a significant impact. Many of the inherent benefits of a hospitalist service, such as improved quality of care, efficiency, and communication, all culminate in effectively transitioning the patient out of the hospital setting. Discharge planning must begin on admission to the hospital and continue throughout the hospital stay. It should include communication with the PCP and appropriate follow-up services. It must encompass coordination with social services, the payer, and, of course, the family. It must also provide for follow-up with regard to any issues that are still pending at discharge. As discussed in previous chapters, hospitalists can play a significant role in determining who gets admitted to the inpatient service and in facilitating intra-hospital transfers. This involvement may vary from program to program and from patient to patient. Even when the hospitalist plays no role in screening admissions, it is incumbent on the hospitalist to clearly define the goal of an inpatient stay; the discharge criteria should follow from this. Anticipating at the outset the durable medical equipment, medications, and services that will be required at discharge can greatly facilitate timely and efficient discharges, even on weekends.

COLLABORATION Discharge planning, just as inpatient management itself, requires a team approach (Table 11-1). Optimally, the inpatient service has a multidisciplinary approach that includes at least nursing, social services, and a coordinator familiar with local payer structures and other outpatient and community resources, such as home care agencies and alternative care facilities. TABLE 11-1

Discharge Planning Team

Participant

Role

Hospitalist

Acts as team leader

Other medical providers with knowledge of current inpatients

Provide medical information

Nursing staff

Provide additional medical information and insight into patient– family dynamics

Social workers

Provide information to patient and family about necessary resources for discharge

Discharge planner

Coordinates discharge needs with outpatient services in context of patient’s insurance

Other: therapists (occupational, physical, speech), nutrition, child life

Provide unique patient information that may impact discharge

Nursing input is vital because nurses interface with patients and families in a different way than physicians; this allows valuable insight into the relative strengths and weaknesses of each patient and their care providers— information that is critical for appropriate discharge planning. Social workers bring another viewpoint that can shed light on the patient’s and family’s response to illness and their ability to manage post-hospitalization care. This perspective should help guide the timing of discharge and what postdischarge services will be necessary. The discharge planner begins reviewing charts on admission, looking for medical documentation of discharge goals. This allows the planner to immediately start coordinating with payers on issues such as anticipated length of stay and available outpatient services. For adult patients, experience with nurse discharge planners and comprehensive discharge planning demonstrates reduced costs and lengths of stay.3,4 Other possible members of a discharge team include therapists, nutritionists, and child life specialists, all of whom may have unique information that impacts discharge planning. Additionally, a post-discharge coordinator can follow up on pending laboratory results and ensure that the family is able to keep or

schedule appointments, obtain prescribed medicines, and follow through with other discharge plans. A weekly (or more frequent) meeting of all parties involved in discharge planning to review ongoing cases and the availability of additional consultation is an effective technique used by many hospitalist groups. This type of multidisciplinary approach can identify significant issues that may affect discharge planning and may serve as a source of quality improvement projects. The hospitalist should be involved in developing mechanisms to ensure the timely review of patient charts by related services to identify discharge issues and improve hospital resource utilization. Most important, patients and families must be partners in the discharge process. Clear goals for admission, as well as discharge criteria and anticipated obstacles, make it easier for the patient and family to follow the course of the hospitalization and prepare for discharge. The hospitalist must be aware of the prevailing Patients’ Bill of Rights regarding necessary notification of discharge, as well as the mechanisms for patients to dispute discharge decisions. Communication with the PCP is essential for appropriate discharge planning,5 and the American Academy of Pediatrics has established the following guidelines for minimum communication with the PCP: communication on admission, for any significant events, and on discharge.6 Good relationships and ongoing communication with referring physicians can help define the best means of communication. It is also important to clarify the capacity of outpatient services to handle ongoing medical needs. Because many factors influence the PCP’s ability to handle various levels of acuity and necessary follow-up after discharge, these issues must be considered during discharge planning. Likewise, it is important to establish open lines of communication with alternative care facilities so that transfer procedures can be initiated as soon as a potential transfer is anticipated. All communication of patient information must be mindful of HIPAA regulations.7

CONTINUITY OF CARE Many successful hospitalist practices have the policy of calling PCPs with every discharge—regardless of the time. This avoids middle-of-the-night telephone calls from patients or families to uninformed PCPs. There are inherent issues with routine discharge phone calls including the time needed

to reach the PCP and potential dissatisfaction from the PCP who is interrupted from sleep or office duties. The median time from a patient’s discharge to his or her first visit to the PCP is 6 days; knowledge of events in the hospital is often critical to a successful office visit and helps assure the patient that his or her care has been adequately coordinated. In addition to a discharge phone call, many practices fax a summary of pertinent information, including dates of admission and discharge, discharge diagnosis, procedures, medicines, change in code status, pertinent laboratory results and pending tests, consultations, disposition, and follow-up appointments. As noted previously, the VIP network has made significant strides toward creating guidelines for discharge communication that incorporate both PCP and hospitalist preferences. A typed discharge summary can also be generated and sent (generally via fax) for each discharged patient, and many electronic medical records allow easy electronic communication with PCPs. Although this can be time consuming, the benefits of effective communication are worth the extra effort. Discharge from the inpatient service is usually not the end of care for a patient. Many patients reach their goals for admission and are successfully discharged with various tests still pending and therapies needed (e.g. antibiotics, respiratory therapy). An important element of discharge communication is conveying information regarding pending tests and studies. This can prevent the repetition of studies that have already been done and ensures that data from the studies are conveyed to the outpatient care providers. The person responsible for following up on pending results can vary from practice to practice, and it is unclear who is medicolegally responsible for following up on tests after discharge. Regardless of the legal implications, the hospitalist should either follow up on pending tests or make sure that the PCP does so. Many practices have implemented post-discharge follow-up clinics for one to two visits. The PCP should be notified of these visits, and the patient should be “returned” to the PCP as soon as possible. One must be aware of the potential political ramifications of delving into the outpatient arena. Along the same lines, a focused post-discharge phone call from the hospitalist practice can help ferret out problems with prescriptions, follow-up appointments, or the patient’s condition.

CONCLUSION Excellent communication is essential to any successful hospitalist service, and adequate discharge planning is essential to the smooth operation of a hospitalist system. The hallmark of effective discharge planning is that it never unnecessarily extends the inpatient stay. By anticipating patient needs, hospitalists can not only improve the care their patients receive but also maintain efficient throughput and maximize the beds available to the inpatient service and the hospital as a whole. PCPs may be concerned with many aspects of their interaction with a hospitalist service, and effective discharge planning can have an important impact on acceptance.8,9

REFERENCES 1. Forster AJ, Murff HJ, Peterson JF, et al. The incidence and severity of adverse events affecting patients after discharge from the hospital. Ann Intern Med. 2003;138:161-167. 2. Sokol PE, Wynia MK, writing for the AMA Expert Panel on Care Transitions. There and Home Again, Safely: Five Responsibilities of Ambulatory Practices in High Quality Care Transitions. Chicago IL: American Medical Association; 2013. 3. Palmer HC, Armistead NS, Elnicki DM, et al. The effect of a hospitalist service with nurse discharge planner on patient care in an academic teaching hospital. Am J Med. 2001;111:627-632. 4. Naylor MD, Brooten DA, Campbell RL, et al. Comprehensive discharge planning for the elderly: a randomized, controlled trial. J Am Geriatr Soc. 2004;52:675-684. 5. Goldman L, Patilat SZ, Whitcomb WF. Passing the clinical baton: 6 principles to guide the hospitalist. Am J Med. 2001;111:36S-39S. 6. Percelay JM, Committee on Hospital Care. Physicians’ roles in coordinating care of hospitalized children. Pediatrics. 2003;111:707709. 7. American Academy of Pediatrics. American Academy of Pediatrics Security Manual. Oct 2003.

8. Fernandez A, Grumbach K, Goitein L, et al. Friend or foe? How primary care physicians perceive hospitalists. Arch Intern Med. 2000;160:29022908. 9. Auerbach AD, Aronson MD, Davis RB, Phillips RS. How physicians perceive hospitalist services after implementation: anticipation vs reality. Arch Intern Med. 2003;163:2330-2336.

CHAPTER

12

Ethical Issues in Pediatric Hospital Practice Jennifer K. Walter, Sarah Hoehn, and Chris Feudtner

INTRODUCTION The ethical and moral dimensions of health and medicine pervade all aspects of clinical care. Providers of hospital care for children constantly encounter situations in which ethical considerations are vital, ranging from the routine task of obtaining parental permission for medical care to more rare quandaries regarding care deemed necessary (yet is refused) or futile (yet is requested). Competence in handling ethically problematic situations can be enhanced by expanding one’s perspective on ethical thinking, knowing what institutional resources are available to help resolve ethical problems, and learning how to approach specific common problems.

ETHICAL PERSPECTIVES RULES AND POLICIES There are many ways to approach ethical decision-making. An important practical starting point is the hospital’s rule or policy providing specific instructions regarding the issue at hand. For instance, hospitals typically provide guidance about when the general consent for care granted by a parent at the time of admission is inadequate and specific parental permission to perform certain diagnostic or therapeutic procedures must be obtained and documented. Such rules and policies can often be found in the medical staff bylaws or in national guidelines published by the American Academy of Pediatrics (AAP), American Medical Association, or other organizations. Ethically problematic situations are addressed by asking, “What rules and policies [or pertinent state or federal laws] govern our conduct and guide our

choices?”

DUTIES, RESPONSIBILITIES, AND COMMITMENTS Consideration of professionally defined duties and responsibilities can help guide decisions. A growing movement within medicine emphasizes the importance of professional duties, responsibilities, and commitments toward such ends as professional competence, just distribution of finite resources, the integrity and advancement of scientific knowledge, honesty with patients, patient confidentiality, maintenance of appropriate relations and boundaries with patients, improvements in quality of care and access to it, and maintenance of trust by managing conflicts of interest.1 To the degree that such commitments reflect professional consensus, they function as informal policies and guide our actions by telling us (less explicitly than policies) how to behave.

VIRTUES OR PERSONAL TRAITS An allied approach to ethics focuses on individual character traits such as honesty, compassion, competence, fortitude, temperance, fidelity, integrity, self-effacement, and wise and prudent decision-making. Here, however, the emphasis shifts away from what constitutes good behavior (which is the focus of policies or duties) and toward what constitutes a good person who will behave virtuously.2 When confronting ethically problematic situations, personal traits can be used to evaluate a proposed course of action: if I behave in this manner, will I be acting with honesty, fidelity, and fortitude? If not, why would I pursue this course of action? Can I chart another course with greater integrity and wisdom?

BIOETHICS PRINCIPLES To analyze what would be the “right” course of action, it may also be useful to consider the frequently cited core principles of bioethics as presented by Beauchamp and Childress (beneficence, non-maleficence, autonomy, and justice).3 These principles are described so that many different moral theories would endorse them. They offer a framework for how to approach an ethical

dilemma that speaks across different backgrounds because they are valued by many different moral traditions. Beneficence seeks to maximize the benefits caused by our actions, whereas nonmaleficence seeks to minimize the harm that our actions cause. Many therapeutic decisions involve some tradeoff between beneficence and nonmaleficence; simply making a list of the good and bad things that might happen, and how likely they are to happen, can help clarify some dilemmas. Autonomy seeks to enable an individual’s values to guide his or her medical care; respecting a patient’s autonomy is a central way we respect their dignity. Considerations of justice involve how the care of one person fits into the larger system of the care of all persons and can bring up issues of resource allocation and fair procedures to determine who gets what type of care. Understanding how these core principles may be in conflict with one another in specific situations can help elucidate why different individuals may view ethical dilemmas in different ways and may serve as a starting point for discussion and resolution of conflict.

MEDIATION OF HUMAN SOCIAL INTERACTIONS A final set of concepts focuses less on ethical theory and more on people’s understanding of a situation and their interactive behavior. Poor communication and misunderstandings lie at the heart of many conflicts that are perceived as ethical dilemmas. Patterns of interacting—ranging from hurried attempts to communicate complex information or bad news to vocal or body language expressions of anger or arrogance—can worsen misunderstanding and intensify noncooperation.4 If misunderstanding and mistrust are allowed to persist, the lines of the dispute can ossify into entrenched positions (e.g. “I will not permit such-and-such to be done under any circumstances”). Making progress in such cases takes time, patience, and a willingness to share control over the decision-making process. A mediation or negotiation model emphasizes, first and foremost, building mutual understanding and trust; only after these prerequisites have been created can one move on to problem solving.5

HOSPITAL RESOURCES When confronting ethically problematic situations, most pediatric physicians

can draw on valuable resources within their hospitals.

KEY PEOPLE To improve communication, build shared understanding, clarify goals, generate novel solutions, or mediate disputes, physicians may turn to professional translators or cultural mediators, social workers, nurses, pastoral care workers such as chaplains or rabbis, palliative care team members, legal counsel, or other physicians with particular skills in these areas.

ETHICS COMMITTEES Institutional ethics committees can develop or review hospital policies, perform clinical ethics consultations, mediate disputes or controversies, and educate healthcare professionals and patients.6,7 Seven principles guide the process of ethics consultation (Table 12-1).6 Overall, the quality of an ethics consultation rests on the committee’s preparation, including interviewing key stakeholders, gathering pertinent data, and reviewing pertinent literature,8 and its ability to provide an open forum for honest and confidential discussion. TABLE 12-1

Principles of Ethics Consultations

1. Any patient, parent or guardian, or family member can initiate an ethics consultation. 2. The patient and parent or guardian can refuse to participate in an ethics consultation. 3. The refusal of a patient or parent or guardian to permit an ethics consultation does not hinder the ability of an ethics committee to provide consultation services to physicians, nurses, and other concerned staff. 4. Any physician, nurse, or other healthcare provider involved in the care of the patient can request an ethics consultation without fear of reprisal. 5. The process of ethics consultation is open to all persons involved in the patient’s care but is conducted in a manner that respects

patient and family confidentiality. 6. Anonymous requests for ethics consultation are not accepted in the absence of an identified person who is willing to speak to the issue being raised. 7. The primary care pediatrician is invited to participate in the consultation to support existing physician–family relationships.

RESEARCH AND INSTITUTIONAL REVIEW BOARDS Children are allowed to participate in research only when the institutional review board determines that the risks are minimal, a minor increase over minimal risk, or that the pediatric subjects may benefit directly from participation in the research study.9 As always, fully informed permission or consent is a prerequisite for research conducted on human subjects (yet only a third of published pediatric studies include proper documentation of institutional review board approval and informed permission or consent).10

COMMON PEDIATRIC INPATIENT SITUATIONS Although rigorous estimates of the prevalence or incidence of ethically significant or problematic incidents in the inpatient setting are lacking, our experience suggests that hospital-based pediatricians will encounter most or all of the following situations.

OBTAINING PERMISSION, ASSENT, OR CONSENT In pediatric practice, there are three important processes by which parents or patients agree that the patient will receive certain medications, tests, or procedures.11 Informed permission is used when a pediatric patient lacks the necessary decision-making capacity and legal empowerment to make an autonomous decision, requiring the parent or designated surrogate caregiver to do so. Assent is sought when a child or adolescent has some decisionmaking capacity but is not yet legally empowered. Informed consent is used when an adolescent or young adult has legal authority to make decisions regarding his or her health care.

The processes of obtaining informed permission and informed consent include four key components: (1) appropriate disclosure of information, (2) adequate personal understanding of the information, (3) emotional capacity to make a decision, and (4) voluntariness or freedom from coercion.12 The process of seeking assent translates these elements into a developmental framework applicable to pediatric patients (Table 12-2). TABLE 12-2

Four Keys to Seeking Pediatric Patients’ Assent

1. Help the patient achieve a developmentally appropriate awareness of the nature of his or her condition. 2. Tell the patient what he or she can expect with regard to tests and treatments. 3. Make a clinical assessment of the patient’s understanding of the situation and the factors influencing how he or she is responding (including whether there is inappropriate pressure to accept testing or therapy). 4. Solicit an expression of the patient’s willingness to accept proposed care. Do not solicit a patient’s views without intending to weigh them seriously. Necessary urgent medical care should not be withheld because of a lack of consent.13 No evaluation of a life-threatening or emergency condition should be delayed because of a perceived problem with consent or payment authorization. A parent’s act of leaving his or her child with another custodian or the state represents implied consent when the parent is not immediately available for verbal consent and nonelective medical care is needed. These situations include relief of pain or suffering, suspected serious infectious disease, assessment and treatment of serious injuries and conditions that threaten life, limb, or central nervous system, and other potentially serious conditions.13

DETERMINING DECISION-MAKING CAPACITY AND COMPETENCE

When can an adolescent consent to medical care? The laws that govern this issue vary by state, so it is prudent to check with your hospital legal counsel. In general, however, persons are judged to be legally competent to consent to care when they are 18 years or older or when they have been emancipated from parental authority by circumstances such as being married, being pregnant or having borne a child, or living on their own. Adolescents seeking care for certain conditions (e.g. reproduction, emergencies, sexually transmitted diseases, drug and alcohol abuse) may also be deemed competent to consent to treatment without parental permission. Occasionally, pediatric hospitalists may be caring for persons 18 years or older who lack decision-making capacity (e.g. due to mental retardation). In such circumstances, the first step is to determine whether the patient has been determined by the legal system to be incompetent and, if so, who has been specified as the surrogate decision maker through either guardianship or durable power of attorney. Even more rarely, the decision-making capacity of a younger patient’s parent or guardian may be in doubt. In such an event, attending physicians should confer with other members of the healthcare team and perhaps hospital legal counsel before asking for a legal determination of incompetency and the specification of an authorized surrogate decision maker. Relevant criteria to be considered when determining decision-making capacity include the ability to communicate a choice for treatment, understanding the relevant information necessary for the decision, appreciating the situation and its consequences, and reasoning about the treatment options.14

BEING PURPOSEFULLY DECEPTIVE Although the use of deceptive practices by physicians and healthcare staff runs counter to the principle of truth-telling and the virtue of forthrightness, such practices still occur—in some cases, perhaps with justification—in the pediatric inpatient setting. Examples include the secretive use of placebos to determine whether a response to therapy is due to physiologic or psychological mechanisms, quasi-investigational feeding trials of a child with growth failure to determine whether the family is noncompliant with nutritional recommendations, and the use of covert surveillance video cameras to investigate potential cases of Munchausen syndrome by proxy. Recourse to these deceptive methods should be considered only after all other

possible nonsecretive means of addressing the situation have been exhausted, and then only if the potential benefits of deception greatly outweigh considerations of truth-telling, trustworthiness, and patient or parent autonomy, and only if the course of action has been evaluated by a sufficiently wide array of hospital staff (e.g. members of the ethics committee) to ensure that the two preceding conditions have been met.

MEDIATING DISPUTES REGARDING FUTILE OR ESSENTIAL CARE When parents want an increased level of technologic support for their child— often stated as wanting to “do everything”15—and health care providers disagree, the notion of futility is sometimes brought up.16—The Society for Critical Care Medicine defines futile treatments as those that will not accomplish their intended goal17 (although identifying clinical situations when we can predict with accuracy and assurance that interventions will have no chance of success is difficult18). Attempts have been made to better define futile treatments both quantitatively (e.g. treatment has been useless in the last 100 cases) and qualitatively (e.g. treatment will merely preserve permanent unconsciousness or fail to end total dependence on intensive medical care).19 Such definitions have been criticized, however, because of the imprecision of medical prognoses and because they are value laden and biased against technology-dependent children.20 Lacking a consensus definition of futility, many ethicists believe that parents’ wishes should be honored21 and that the treatment team’s attention should be focused on improving communication with the family, because poor communication often prompts such disputes.22,23 Failure to reach consensus after a team’s best efforts to improve communication is a good indication for an ethics consultation. In contrast to futile treatment disputes, parents sometimes refuse care that seems necessary. The AAP believes that all children deserve effective medical treatment that is likely to prevent substantial harm, suffering, or death.24 In addition, the AAP advocates that all legal interventions should apply equally whenever children are endangered or harmed, with no exemptions based on religious or spiritual beliefs—with the exception of

immunizations. The AAP does not support the application of medical neglect laws for failure to vaccinate unless there is substantial risk of serious harm, although it does endorse universal immunizations.25 In general, if the benefit of treatment is uncertain, the parents’ wishes should be honored. Some authorities also support pediatricians’ right to discontinue professional services due to vaccine refusal, but they still must comply with the applicable legal and ethical requirements to ensure that patients are not abandoned.26

DECIDING TO WITHHOLD OR WITHDRAW LIFESUSTAINING TREATMENT Within the medical ethics community, there is strong consensus that there is no salient ethical difference between withholding (i.e. never starting) and withdrawing (i.e. stopping) treatment. Simply stated, if the initiation or continuation of a potentially life-sustaining therapy adversely affects the likelihood of accomplishing therapeutic goals that the patient values above life (such as quality of life), therapy can be ethically withheld or stopped.27 Although most healthcare professionals are ethically comfortable withholding or withdrawing potentially life-sustaining high-technology therapy, many are uncomfortable with the practice of withholding or withdrawing fluids and nutrition administered through tubes, particularly for children who cannot consent for themselves.28 The AAP argues that intravenous infusions and tube feedings are no different from other forms of therapy that competent patients or their authorized surrogates have the right to refuse if the expected burden of the intervention to the patient exceeds the potential benefit.28 General consensus on these practices is lacking, however, and many states have ambiguous case law precedents that leave the legally sanctioned course of action unclear.

DISTINGUISHING BETWEEN TREATING SUFFERING AND PERFORMING EUTHANASIA Euthanasia is illegal throughout the United States,29 yet the proper management of a patient in severe pain or with some other form of intractable suffering sometimes requires doses of medications (especially opiates) that may hasten death and in other clinical circumstances would be considered

inappropriate. The goal of palliative care is to optimize the patient’s quality of life, and at times this may only be achieved by giving increasing doses of sedation that also may contribute to respiratory depression. If a patient or parent requests euthanasia, physicians should respond compassionately by looking to alleviate the sources of distress (e.g. concerns of abandonment, depression, loneliness, physical symptoms, or communication problems).30 Physicians committed to minimizing the suffering of dying patients can evaluate the ethical appropriateness of their actions by considering whether they can affirm the four rules of the doctrine of double effect (Table 12-3). TABLE 12-3

Four Rules Regarding the Doctrine of Double Effect

1. The intention when instituting treatment (typically, administering a drug) must be solely to relieve the patient’s suffering. 2. The treatment must be given in response to a sign or symptom of suffering (not a hypothetical potential for suffering to occur). 3. The potency of the treatment must be commensurate with the degree of suffering (not a massive overdose). 4. The treatment must not be given as a deliberate attempt to cause or hasten death.

ADVOCATING FOR PATIENTS AND ENHANCING QUALITY OF CARE Pediatrics as a profession has long recognized the need to advocate for children.31 Pediatric hospitalists have ethical obligations to improve how health care is delivered to their patients. Although these obligations are often most compelling on a case-by-case basis, hospitalists involved in the design of hospital practice guidelines or policies should also be mindful of how these practices can be evaluated from an ethical point of view. Enhancing access to care, ensuring that limited resources are allocated fairly, eliminating financial conflicts of interest, and improving the quality of care are all activities that can promote justice, equity, and beneficial outcomes in child

health care.

SOCIAL MEDIA AND RESPECTING PATIENT AND CLINICIAN BOUNDARIES The rapid expansion of online social networking sites has led to ethical challenges about protecting patient privacy and confidentiality, maintaining appropriate professional boundaries between physicians and patients, demonstrating respect for patients, and ensuring trust in physicians.32 While these technologies offer new opportunities for improving health care, they also present pitfalls that should be anticipated and preemptively avoided. This is a rapidly evolving area, and no clear consensus yet exists about appropriate versus inappropriate behavior. You should check with your hospital ethics committee to learn about your hospital’s policies regarding internet and social media use. If your hospital does not have a policy, you can learn more about the potential benefits, hazards, and recommendations around different online engagement in the American College of Physicians’ policy statement.32

REFERENCES 1. Medical professionalism in the new millennium: A physician charter. Ann Intern Med. 2002;136:243-246. 2. Pellegrino ED: Toward a virtue-based normative ethics for the health professions. Kennedy Inst Ethics J. 1995;5(3):253-277. 3. Beauchamp TL, Childress JF. Principles of Biomedical Ethics. 6th ed. Oxford: Oxford University Press; 2009. 4. Levetown M. American Academy of Pediatrics Committee on Bioethics: Communicating with children and families: from everyday interactions to skill in conveying distressing information. Pediatrics. 2008;121(5):e1441-1460. 5. Dubler NN, Liebman CB. Bioethics Mediation: A Guide to Shaping Shared Solutions. Revised and Expanded Edition. Nashville: Vanderbilt University Press; 2011. 6. American Academy of Pediatrics, Committee on Bioethics. Institutional

ethics committees. Pediatrics. 2001;107:205-209. [A statement of reaffirmation for this policy was published in Pediatrics 2009;123(5):1421]. 7. Spencer EM. A new role for institutional ethics committees: organizational ethics. J Clin Ethics. 1997;8:372-376. 8. Burns JP. From case to policy: institutional ethics at a children’s hospital. J Clin Ethics. 2000;11:175-181. 9. Shah S, Whittle A, Wilfond B, et al. How do institutional review boards apply the federal risk and benefit standards for pediatric research? JAMA. 2004;291:476-482. 10. Weil E, Nelson RM, Ross LF. Are research ethics standards satisfied in pediatric journal publications? Pediatrics. 2002;110:364-370. 11. American Academy of Pediatrics, Committee on Bioethics. Informed consent, parental permission, and assent in pediatric practice. Pediatrics. 1995;95:314-317. 12. Faden R, Beauchamp T. A History and Theory of Informed Consent. New York: Oxford University Press; 1986. 13. American Academy of Pediatrics, Committee on Pediatric Emergency Medicine. Consent for medical services for children and adolescents. Pediatrics. 1993;92:290-291. 14. Appelbaum PS. Assessment of patients’ competence to consent to treatment. N Engl J Med. 2007;357:1834-1840. 15. Feudtner C, Morrison W. The darkening veil of “do everything.” Arch Pediatr Adolesc Med. 2012;166(8):694-695. 16. Avery GB. Futility considerations in the neonatal intensive care unit. Semin Perinatol. 1998;22:216-222. 17. Consensus statement of the Society of Critical Care Medicine’s Ethics Committee regarding futile and other possibly inadvisable treatments. Crit Care Med. 1997;25:887-891. 18. Lantos J. When parents request seemingly futile treatment for their children. Mt Sinai J Med. 2006;73(3):587-589. 19. Schneiderman U, Jecker NS, Jonsen AR. Medical futility: response to critiques. Ann Intern Med. 1996;125:669-674.

20. Truog RD, Brett AS, Frader J. The problem with futility. N Engl J Med. 1992;326:1560-1564. 21. Nonbeneficial or futile medical treatment: conflict resolution guidelines for the San Francisco Bay area. Bay Area Network of Ethics Committees (BANEC) Nonbeneficial Treatment Working Group. West J Med. 1999;170:287-290. 22. Fins JJ, Solomon MZ. Communication in intensive care settings: the challenge of futility disputes. Crit Care Med. 2001;29(2 Suppl):N10N15. 23. Cogliano JF. The medical futility controversy: bioethical implications for the critical care nurse. Crit Care Nurs Q. 1999;22:81-88. 24. American Academy of Pediatrics, Committee on Bioethics. Religious objections to medical care. Pediatrics. 1997;99:279-281. 25. Diekema DS, American Academy of Pediatrics Committee on Bioethics. Responding to parental refusals of immunization of children. Pediatrics. 2005;115(5):1428-1431. 26. Gilmour J, Harrison C, Asadi L, Cohen MH, Vohra S. Childhood immunization: when physicians and parents disagree. Pediatrics. 2010;128(Suppl 4):S167-S174. 27. Casarett D, Kapo J, Caplan A. Appropriate use of artificial nutrition and hydration—fundamental principles and recommendations. N Engl J Med. 2005;353:2607-2612. 28. Diekema DS, Botkin JR, American Academy of Pediatrics Committee on Bioethics. Clinical Report—Forgoing medically provided nutrition and hydration in children. Pediatrics. 2009;124(2):813-822. 29. Feudtner C. Control of suffering on the slippery slope of care. Lancet. 2005;365:1284-1286. 30. American Academy of Pediatrics Committee on Bioethics and Committee on Hospital Care. Palliative care for children. Pediatrics. 2000;106(2):351-357. 31. American Academy of Pediatrics Committee on Bioethics. Professionalism in Pediatrics: Statement of Principles. Pediatrics. 2007;120(4):895-897. 32. Farnan JM, et al. Online Medical Professionalism: Patient and Public

Relationships: Policy Statement From the American College of Physicians and the Federation of State Medical Boards. Ann Intern Med. 2013;158:620-627.

CHAPTER

13

Medicolegal Issues in Pediatric Hospital Medicine Steven M. Selbst and Joel B. Korin

INTRODUCTION Legal risks are common in the care of hospitalized children. Pediatric hospitalists care for complex patients of all ages with a variety of medical conditions.1 Many are acutely ill with rapidly changing medical conditions. Hospitalists often find themselves juggling several ill patients simultaneously, and they must make critical decisions rapidly. The risk for error is high, and there is the possibility of a subsequent malpractice suit. In addition, certain hospital processes and situations pose a particular risk for lawsuits, including communication, informed consent, refusal of care, documentation, altering the medical record, use of consultants, treatment of incidental findings, patient confidentiality, and legal responsibility during discharge. The legal obligations surrounding each of these situations are described in this chapter. The hospitalist movement is relatively new, and little information has been generated regarding claims against these specialists. In general, pediatricians are less likely to be sued than other specialists, but they have the highest average indemnity payments ($521,000/claim) of all specialties.2 Evidence suggests that hospitalists reduce malpractice claims in the inpatient setting by improving quality of care and patient and parent satisfaction.3,4 In addition to improved quality of care, hospitalists have demonstrated enhanced efficiency.5 They often become team leaders in their hospitals, attempting to optimize quality and continuity while delivering evidencebased care for acutely ill patients.6,7 Hospitalists’ presence in the hospital enables the discovery and demonstration of best practices in detecting errors.5 Tenner’s retrospective study found that the quality of care of critically ill patients improved after

hours, when more experienced physicians provided care at the bedside; more specifically, patients cared for by pediatric hospitalists (general pediatricians who had completed training) had improved survival rates compared with patients cared for by pediatric residents who were supervised from a distance by intensivists.8 Thus, the use of hospitalists may lead to safe care, fewer adverse outcomes, and fewer malpractice suits. Like all physicians, hospitalists may face legal action when pediatric patients in their care have bad outcomes. When a lawsuit is instituted, the plaintiff must show that the physician had a duty to the patient, breached that duty, and did not meet the standard of care. The plaintiff must then show that this breach caused damage or injury to the patient.9

COMMUNICATION Good communication between the hospitalist and the patient and family is essential to prevent lawsuits. Poor communication can lead to angry feelings, omission or distortion of important information, and subsequent injury to the patient. Effective communication between the primary care physician (PCP) and the hospitalist is also crucial.10 One study showed a direct relationship between physicians’ enhanced communication skills and fewer malpractice suits.11 Another study of families that sued physicians (after infants suffered permanent injury or death) found that most were dissatisfied with physician-patient communication. They believed that the physicians would not listen to them (13%), would not talk openly (32%), attempted to mislead them (48%), or did not warn them about long-term neurodevelopmental problems (70%).12 The patient and family must perceive a caring attitude, openness, professional integrity, and standards of excellence; a sense of trust is also crucial. Patients are not always aware of the physician’s competence, but they are keenly aware of his or her manner. They remember how the physician looked, acted, spoke, and listened; whether he or she was neatly dressed; and whether the physician was pleasant or disinterested, serious or cavalier, haughty or condescending, polite or discourteous. Good eye contact and body language are important.13 No matter how tired, frustrated, or stressed the hospitalist feels, this should not be communicated to the family. Establishing good rapport with the family can sometimes be difficult, but it is essential.

The hospitalist should try to appear unhurried and ready to listen. An adverse outcome—even if inevitable—coupled with the parents’ feeling that the physician was rushed or was not sincerely interested in the child may provoke a lawsuit. Thus, when meeting with families, it is advisable to introduce oneself, shake hands, and sit down in the examination room. This gives the parents the message that, at least for the next few minutes, they have your undivided attention, no matter how many other children are in the hospital. Studies have shown that if the physician sits down in the presence of the patient, the perception of the time spent with the patient is doubled.14,15 The hospitalist must also establish rapport with the child. Avoid casual teasing, condescension, and talking about the child without attempting to include him or her in the discussion. Be careful not to violate modesty in school-age children. Speak at an age-appropriate level, and remember that even young children understand far more than they say. Try to be flexible, but do not offer choices when none exist.9 Keep the family informed about necessary procedures, suspected diagnoses, and the child’s overall progress. In addition, help the family understand the makeup of the health care team that is caring for the child. This is especially important in a teaching hospital, where residents, fellows, consulting physicians, and several nurses may participate in patient care. A recent American Academy of Pediatrics (AAP) policy statement recommends that providers conduct rounds in patients’ rooms, with family members present.16,17 This enhances communication and may reduce errors.17 At times, parents might insist on obtaining certain laboratory tests that the medical team believes to be unnecessary. The medical staff must then calmly and skillfully explain why such tests may not be needed. This need not become a point of contention if the parents recognize the physician’s sincere interest in the child. One study showed that patient satisfaction with care was related not to whether patients got antibiotics for respiratory infections but rather to their perception of the amount of time the physician spent explaining the illness and their understanding of the treatment.18 Discuss questions about pain management and convince the parents that everything possible is being done to relieve their child’s pain. Encourage parents to stay with their child for procedures whenever possible. Many parents can nurture the child through a painful procedure if they know what to expect.9,19 Take care when discussing the patient’s management with other staff

members in front of the family. Parents should not witness disagreements over plans for management, such as might occur if one staff member suggests ordering a specific laboratory test and the hospitalist disagrees. Likewise, it is not appropriate to reprimand or correct a nurse or physician-in-training in view of the family. This would undoubtedly create a feeling of uneasiness. In some cases, communication with a parent is particularly difficult. For example, parental anger can be predicted when they are informed that a report for suspected child abuse is being filed. Remain nonjudgmental in these circumstances. Tell the family that the physician is required by law to file such a report and that the medical staff is not accusing anyone of abuse.9 Finally, if a harmful medical error is made, explain to the family what has happened. It is best to be honest with parents about the mistake. If an error is merely suspected, do not admit negligence but rather explain that all will be done to investigate the situation and improve results.20 Lawsuits may be avoided if the physician is honest and forthright. As in politics, an attempted “cover-up” makes the situation worse. In all cases, follow hospital policies for disclosure of a medical error.

INFORMED DECISION-MAKING (INFORMED CONSENT) Parents have the right to be informed about their child’s medical care and to give consent for treatment. Parental rights are limited, however. For instance, adults can refuse treatment—even life-sustaining treatment—for themselves, but not for their children. Parents must act responsibly and in their child’s best interests. If they are neglectful or abusive, the courts can relieve them of decision-making and, if necessary, custody.21 Most states define informed consent as providing a description of the procedure or treatment and the risks and alternatives in such a way that a reasonably prudent person would be able to make an informed decision whether to undergo that procedure or treatment. Patients and parents are entitled to know the diagnosis, the nature and purpose of the proposed treatment or procedure, the risks, consequences, and side effects of the proposed procedure, reasonably available alternatives and their risks, and the anticipated prognosis without treatment. Be sure that the patient or parent understands the information given.21,22

In a true emergency, informed consent is not needed. When a hospitalist provides care in the emergency department, the legal guardian may not even be present. For an unconscious or severely injured child, no consent for treatment is necessary because it is assumed that a reasonable parent would want the physician to care for the child immediately. If it is not clear that a true emergency exists, it is generally best to treat the patient and get consent later.21 A physician is more likely to be sued for failure to treat without consent than for providing reasonable treatment without the guardian’s knowledge or approval. However, do not give a blood transfusion to a reasonably stable child or intravenous contrast material for a computed tomography scan if the risks and benefits have not been explained to the parents. It is conceivable that a patient could suffer a reaction to these procedures, and the guardian may sue the physician for failing to inform him or her of the risks involved.9 No patient or parent should be forced or influenced to make a specific decision; however, no decision by a parent is completely free of outside influences. Most parents need and want the physician’s opinion to help them decide what is best for their child. Finally, the physician has an ethical obligation to try to inform a minor child about the proposed treatment, to describe what he or she is likely to experience, and to get his or her assent to care, if possible.21,22 Consent forms are widely used at most hospitals and should be written at an appropriate level for most patients or parents (generally, a sixth- to eighthgrade reading level).21 Give parents the opportunity to ask questions after reading the form. Take language barriers into consideration. Parents usually sign a general consent form at the time of presentation to the hospital that allows for a general evaluation and treatment of the child. However, parental signing of such a form does not equate with informed consent. The patient or parent can still claim that the risks and benefits were not adequately explained.9,22 Patients and parents may not recall what was told to them when they were emotionally stressed. A signed consent form may provide some legal protection for the physician, because it documents that steps were taken to inform the parent about a procedure. However, a signed form is of little value unless a discussion about risks and benefits took place.22 Clear documentation in the record, noting what was explained to the parent, may be just as valuable as a signed consent form. Remember, informed consent is a process, not a paper. Table 13-1 summarizes procedures for assisting parents

with informed decision making. TABLE 13-1

Responsibilities for Informed DecisionMaking (Informed Consent)

Assess the patient’s or guardian’s decision-making capacity Provide the patient and guardian with appropriate information Diagnosis Nature of procedure or intervention Purpose of procedure or treatment Risks and benefits of procedure or treatment Alternative treatments and associated risks and benefits Prognosis with and without treatment Assess the patient’s or guardian’s comprehension of the discussion Ensure that the decision is made freely, not coerced After the discussion, obtain signed consent

REFUSAL OF CARE Despite all efforts, some patients or parents refuse treatment or leave the hospital against medical advice (AMA). This usually occurs when the patient or parent is angry, afraid, or disoriented or has certain religious beliefs. Emancipated minors and parents generally have the right to refuse treatment for themselves or their child. However, when a patient or family leaves the hospital without a full evaluation and complete treatment, everyone involved may suffer. The child may have persistent or worsening symptoms from a medical problem that has not been addressed. Likewise, the physician usually feels a sense of failure and frustration when advice and recommendations are not heeded. The hospital and physicians may be subjected to a lawsuit if the patient later suffers serious morbidity or dies, even if the family left voluntarily or signed out AMA. Therefore, the goal should always be to keep the child and family from leaving prematurely.9,23

UNDERSTANDING THE PATIENT

Some patients or parents wish to leave the hospital because they are angry about a long wait for medical treatment. Some may fear a prolonged or expensive hospitalization, or they anticipate unnecessary and painful procedures for their child. Others are afraid to learn of a serious diagnosis or are anxious about children left at home. Those who do not speak English may be especially frightened. Still others may fear teaching institutions in particular because they believe that students or residents “practice” on patients.9 Understandably, families may be less trusting of a hospitalist physician with whom they are not familiar.

PROVIDING TREATMENT If a true emergency exists, provide prompt medical intervention even over parental objections.21,24 It is extremely unlikely that the physician will be successfully sued for intervening and delivering care to a child in an emergency situation.22 In fact, the legal risk is much greater if emergency care is not given. Try to learn why the patient or family wishes to leave the hospital. If the patient or parent seems angry, allow him or her to express concerns without interruption. Remain courteous, concerned, and flexible in the treatment plan. It is never wise to challenge patients to sign out AMA, and they should not be threatened with a call to security officers. Call security only if necessary to maintain order.9 A social services worker may be extremely helpful in difficult cases. Further, a telephone call to a familiar PCP may allay the parent’s fears and convince the family to follow the proposed treatment. If a language barrier is a factor, provide a competent translator. Be sure the parents understand the risks and benefits of treatment and the risks of refusing treatment. Allow the patient and family to consider options in a low-pressure atmosphere so that they can make a rational decision.

DOCUMENTING EVENTS If a parent still wishes to leave the hospital despite all efforts to reach an agreement, ask the guardian or emancipated minor to sign a statement releasing the doctor and the hospital from all liability. Such statements may

have limited usefulness, because parents can later claim that they did not fully understand the risks involved in leaving AMA. However, a signed statement witnessed by one or two staff members may shift some responsibility to the parents in the event of an adverse outcome.9 Document in the hospital records exactly what was done for the child and what was told to the parents. Specifically, if the child was examined, record the findings and impression of the limited evaluation and what tests or treatment was contemplated. Also, document the parents’ reason for leaving AMA, as well as the risks of refusing treatment as they were explained to the parents. Tell parents (and document) that they can always return to the hospital for reevaluation if they change their minds or if the child’s condition worsens. It may be helpful to volunteer the names of alternative hospitals and doctors and to offer to arrange transportation, if feasible. This shows a sincere interest in the patient and may prevent litigation. Finally, if a parent refuses to officially sign out AMA before leaving the hospital, carefully document this, with a witness, in the medical record.9 In some situations, the patient cannot be permitted to leave the hospital under any circumstances. If the guardian is disoriented or intoxicated and cannot understand the risks and benefits of treatment or the consequences of refusing care, do not allow the patient to leave. A life-threatening medical problem also mandates immediate medical care. It is always better to win the cooperation of the parents, but if they refuse, the staff is justified in treating the child. Report the refusal to the proper authorities as medical neglect, and seek a court order while delivering emergency care. If it is unclear whether a life-threatening situation is present, err on the side of treatment.9,21,24 Similarly, in a case of suspected child abuse in which the perpetrator is unknown, do not release the child, despite the parents’ wishes or protests. In this situation, call the security staff to prevent the parents from removing the child from the hospital. The staff must contact the hospital lawyers, administrators, or a judge on emergency call to seek a verbal temporary restraining order. Record the conversation with the judge in the patient’s chart, including the specific actions authorized. The procedure to obtain court permission to treat a child may vary in different locales, and the hospitalist should be familiar with local and hospital policies.9

SPECIAL CASES HIV Testing Testing a pediatric patient for human immunodeficiency virus (HIV) is an important issue. HIV testing without a patient’s (or guardian’s) consent is illegal in almost every state. Occasionally, a staff member may unintentionally injure himself or herself with a needle stick and then request HIV testing of the patient. Follow the specific hospital policy for this event carefully. Lumbar Puncture Lumbar punctures arouse fear and concern among parents. Many parents have misperceptions about the procedure and an unwarranted fear of complications. Inform parents about the need to do this procedure—for instance, that a lumbar puncture is necessary to determine if a serious disease such as meningitis is present—and that there is no adequate alternative to establish the diagnosis. Inform them about the most common complications, such as local pain. Tell them that serious complications, such as apnea in a small infant who is curled for the procedure, are quite rare. There is no need to discuss possible herniation, because this is extremely rare when signs of increased intracranial pressure are absent. If such signs exist, withhold the procedure. If a parent refuses the lumbar puncture, the hospitalist may choose to treat the child for possible meningitis without doing the procedure. Tell parents of the inherent problems with this alternative plan, and pursue permission for the lumbar puncture after treatment is started.9 Blood Transfusions and Jehovah’s Witnesses If a parent refuses a blood transfusion because of religious beliefs, obtain legal counsel when time permits. A court order may be necessary. If the child’s life or health is in danger, nearly every state allows the physician to override the parents’ religious beliefs. Even for an older child or adolescent who holds the same beliefs, it is unlikely that the courts will uphold the minor’s right to refuse treatment if doing so means risking death.25 Still, it is best to avoid court action or coercing a teenager to accept treatment that is unwanted. Try to settle the affair with the patient and family whenever possible. Discuss the use of blood substitutes if time allows and if this is acceptable to the physician. If the patient is in shock and unable to consent, give blood as deemed necessary. A transfusion can also be authorized over a teenager’s objection if there is a question about the patient’s competency or if the state

can demonstrate an overriding interest.22

DOCUMENTATION—THE MEDICAL RECORD The importance of careful documentation cannot be overemphasized. Good documentation prevents lawsuits. The patient’s chart is almost always the first document reviewed by parents, attorneys, and their consulting physicians. A record that demonstrates a thorough examination and testing may convince the plaintiff’s attorney not to proceed further. A record that does not demonstrate conclusively whether an important test or portion of the examination was negative or positive may lead counsel to assume that it was not done. The record can also influence expert witnesses who will advise the attorneys whether the case has merit and should be pursued. Because one can never truly predict which patient will have a bad outcome and end up in litigation, prepare each chart carefully.9 Because of the extended statute of limitations for children, many years may pass before a physician is advised of malpractice litigation. The hospitalist often has little if any recollection of the treatment rendered without referring to the medical record. This document can prove to be the physician’s friend or foe, depending on how well it was prepared at the time of treatment. Many settlements have been offered even when good medical care was rendered but not substantiated in the medical records.9,26 The history of the present illness must be described completely but concisely. Even though there may be limited time for recording information, a note that is very brief and barely legible may convey a sense of carelessness and haste to a jury.26 Note any information that seems relevant to the chief complaint. Also record details about the child’s recent diet, level of activity, and medications given at home. Include a history of immunizations, allergies to any medications and underlying medical problems for every pediatric patient. Also, known exposure to infection may be important in many cases. For an adolescent girl, document the last menstrual period. For an injured child, describe how the injury occurred and whether the history is consistent with the physical examination. Note whether there is a concern about child abuse, as well as a decision to file a report with authorities.9 Record a thorough physical examination for each child, including a

complete, timed set of vital signs. Repeat abnormal vital sign. Make a note if the blood pressure or pulse is abnormal because the child is crying. Give particular emphasis to the child’s general appearance, state of hydration, and level of activity or playfulness. Be as descriptive as possible. For instance, “alert, interactive, playing with toys on mother’s lap” graphically depicts the general appearance of an infant. Rather than “irritable baby,” it is better to note “baby cries when approached but is easily consoled by mother.” For a febrile child or a complicated case, the chart should convincingly reflect that the child was well appearing at the time of discharge. In the emergency department, this may require that a second note, or progress note, be included in the chart. For an injured child, it may be best to use a picture or diagram to describe the trauma in detail.9,26 The medical record must reflect a timely process. All entries, including diagnostic or therapeutic orders, must be clearly written and the exact time of the entry noted. Likewise, date and time telephone conversations with consultants. For example, in the event of delayed surgery, recording the time when a consultant was called and when he or she arrived can prove useful years later when litigation begins. Document all laboratory test results or reports of procedures performed.26 The medical record must also include the physician’s clinical observations of the patient and the diagnostic impression formulated. Documenting one’s thought process can be helpful, especially if the case is complicated. For instance, if the physician initially orders laboratory tests and later cancels them, justify the reasons for this change in the management plan in the medical records (e.g. “child improved, drinking well, no need for spinal tap”).9,26 It is important that the hospitalist’s final disposition be reasonable and based on the history, physical examination, test results, and impression. For instance, if the final diagnosis is gastroenteritis, discharge the child only if the record reflects that he or she is well hydrated. The medical record should provide meaningful information to another practitioner if additional care is needed at a later time. It should also display a concerned, professional attitude toward the child and family. Although some charts use a checklist system in an effort to save time, always add pertinent positive and negative findings on the physical examination. For instance, in the evaluation of the skin of a febrile child, comments such as “no petechiae

or purpura” may be quite important. A chart in which the entire examination is merely checked off as “normal” will not convince a jury that a careful examination was performed. Do not include parts of the examination that were not done. For instance, the rectal examination should not be checked off as normal if no such examination was performed. This will damage the physician’s credibility and make a jury doubt that other parts of the examination were truly performed.9,26 The medical record will be made public if a lawsuit is filed. Therefore, only comments that one would be proud to read in front of a jury should be included. Illegible records can compromise the quality of care and make the hospitalist appear unprofessional. Fortunately, electronic medical records have reduced the likelihood of illegible records. Do not place insensitive terms in the record. A medical term such as “dysmorphic child” serves all parties better than “FLK” (funny looking kid). Avoid derogatory statements or descriptions of the parents as well. If the parents are angry during the evaluation, the note may reflect this; for example, “mother became upset during the exam and her concerns were addressed.” However, do not add any judgmental statements.9,23,26 If a patient returns to the hospital after a prior admission or emergency department visit, note the earlier treatment but avoid terminology that suggests that the initial care given was incomplete or careless. It is also advisable to avoid self-serving statements in the record that appear defensive. The purpose of the record is primarily to take care of the patient, and it should not look like it was written for legal defense purposes. The physician’s notes should be consistent with the nursing notes and those of any other disciplines. The hospitalist must read and acknowledge the nursing notes. If a physician disagrees with the nursing assessment (e.g. does not believe that a baby is lethargic), emphasize this in a non-combative manner in the record. It is important that nurses, physicians, consultants, and other members of the medical staff do not engage in intramural battles on the record. Settle disagreements before the patient goes home, and have the record reflect agreement among caretakers whenever possible. Do not write inflammatory remarks and omit extensive discussions about the frustrations of patient care from the record. When a child’s care is transferred to another physician, such as at a change of shift, the physician coming on duty can write a brief note to document the patient’s condition at that time. Document all procedures in a

detailed note, including how the child tolerated the procedure. Include brief descriptions of any conversations with the family and those with the PCP. Use abbreviations sparingly, and use only standard ones accepted by the medical records department of the hospital. Electronic health record systems are commonly used in hospitals across the United States. Electronic medical records may reduce medical errors and malpractice lawsuits. A study of office-based physicians showed that users of electronic health records were less likely to pay malpractice claims (over 10 years) than non-users (6.1% vs. 10.8%), but the difference was not statistically significant.27 However, electronic medical records are not without risk.28 Errors may occur when using drop-down lists in a hurry.29 Also, quickly clicking through a template without noticing that the language is inappropriate for a particular patient could compromise a physician’s defense in a malpractice suit.30 Furthermore, the temptation to copy and paste patient histories instead of taking new histories risks missing new information and perpetuates previous errors.28

ALTERING THE MEDICAL RECORD Correct written errors in a patient’s chart appropriately. Do not attempt to cover up mistakes by blacking out words or phrases, which tends to arouse suspicion. For hand-written notes, draw a single line drawn through the error, then initial and date it. Never attempt to “enhance” the medical record after the case is involved in litigation. It is likely that any change made to the record will be discovered and will be seen as a deliberate and dishonest cover-up. Especially for electronic medical records, it is easy to determine when a late entry into the chart was made. In general, it is easier to defend missing facts or a poor record than an altered one. Credibility is a key factor in any litigation, and an altered record frequently destroys the physician’s credibility.26 When there is a need to add clinical information after an untoward event occurs, it is best to do so in a confidential letter to the physician’s attorney rather than adding to the chart. Besides resulting in the loss of a lawsuit, tampering with the record can result in the award of “punitive damages,” intended to punish the physician for attempting to mislead the court. Some malpractice policies deny coverage

if it is determined that medical records were altered. In some states, altering medical records can be considered a criminal offense in certain circumstances. Finally, any attempt to destroy or “lose” the medical records will seriously hurt the physician’s ability to defend against the litigation.

CONSULTANTS No physician in the hospital works in isolation. There is often a need to consult with the PCP, critical care specialists, surgeons, and other physicians while caring for ill children. The hospitalist should call for help whenever he or she believes that the care required is beyond his or her expertise or when it appears that another opinion will be helpful. Failure to consult appropriately and in a timely manner can result in a lawsuit should there be a poor outcome.31 The hospitalist should generally complete a history and physical examination and obtain appropriate laboratory studies to ensure appropriate referral and avoid unnecessary consultation. However, if there is a true emergency, such as a surgical abdomen or testicular torsion, do not delay consultation by waiting for a urinalysis, Doppler studies, or other tests that may postpone definitive care.32 If the consultant does not arrive in a timely manner, consider calling the consultant’s supervisor, asking other sources for help, or transferring the child to another institution.31 Document the time the specialist was called, the time he or she arrived, and, if applicable, the time the consultant assumed care of the patient. The hospitalist is also responsible for making sure that the consultant is competent for the case in question. If appropriate consultation is not available, arrange safe transport of the child to another facility after discussion with the parents. If the hospitalist believes that consultation is warranted, do not be dissuaded by the PCP. Consider the opinion of the PCP advisory only. Further, if the family insists on consultation or a second opinion (e.g. a plastic surgeon to suture a facial laceration), it is wise to comply if the request is reasonable.33 Radiology consultation ensures proper reading of a film. The hospitalist may be found liable for misreading a child’s radiograph if a radiologist was available. The hospitalist will not be held to the same standard as a radiologist, but rather whatever expertise the jury believes the hospitalist

should possess.9,26

COMMUNICATION WITH THE CONSULTANT Good communication with the consultant is essential. Give appropriate information to the consultant and make certain that the specialist understands the reason for consultation or the question that needs to be addressed. The consultant should discuss his or her findings with the hospitalist, and the two should decide who will relay the information to the parents. Consultants should write complete notes and list clear reasons for their recommendations. For radiology consultations in particular, it is important to have a system in place so that reports of reread radiographs reach the hospitalist, especially any opinion that differs from that of the hospitalist. Telephone consultations without direct examination by the consultant pose a risk for both the consultant and the hospitalist. It is always possible that incomplete information will be provided to the consultant, resulting in incorrect advice. In general, the hospitalist should not allow the specialist to dictate care by phone or deem that consultation is unnecessary if the hospitalist is uncomfortable. If only phone advice is sought, record the gist of the conversation in the patient’s chart.32 Some hospitals allow consultants to write orders for the patient, whereas others request that specialists only give suggestions to the hospitalist. It should be clear to all parties who is placing the orders so that there is no confusion or omission of desired treatment. With most consultations, the hospitalist remains responsible for the patient. If care is to be formally transferred to a consultant who evaluates a child, document the time of transfer.

DISAGREEMENTS WITH CONSULTANTS Understandably, there are occasional disagreements about patient management. Do not take these lightly, because both physicians may be held responsible for a patient’s poor outcome. The hospitalist is not legally bound to accept the advice of a consultant. Blind acceptance of the consultant’s advice can leave the hospitalist liable if the child suffers.33 However, it is not advisable to reject the advice of a specialist without careful consideration of

the consequences. If there is a poor outcome, the consultant’s views as a specialist will be given great weight in court.31 If there are questions about care, discuss the case personally with the consultant. If the consultant’s recommendations are not followed, document why suggested studies were not obtained.31 Do not discuss disputes about care in front of the patient or family. Instead, try to resolve these disagreements amicably, away from the patient. If resolution cannot be reached, it may be appropriate to tell the family in a nonjudgmental way about the difference in opinion.32,33 If the family accepts the opinion of the consultant rather than the hospitalist, note this in the record, and transfer care to the consultant. Ideally, actions should be guided by hospital conflict resolution policies. Cordial, honest dialogue ensures a good outcome. If the hospitalist evaluates a child in the emergency department and believes that admission to the hospital is warranted, and the consultant disagrees, the specialist must come to the emergency department to evaluate the child. Responsibility for the disposition may still rest with the hospitalist, but there will probably be shared responsibility if both have evaluated the child.

TREATMENT OF INCIDENTAL FINDINGS Often a child is admitted to the hospital to manage a specific condition, and during the course of treatment another problem is identified. Whether the hospitalist is responsible for the workup of incidental findings (e.g. hypertension, scoliosis) is unclear.34,35 It depends somewhat on common practice in the particular area. Hospitalists may be held to the same standard as other pediatricians who discover an unexpected finding during an inpatient stay. It is in everyone’s best interest for the hospitalist to clarify who will manage the new incidental finding and whether it needs to be completed in an inpatient or ambulatory setting. If a patient or the family prefers an inpatient workup and it can be expeditiously and safely performed, it is reasonable for a hospitalist to pursue it. Regardless, inform the patient/parents of the findings and the need for follow-up. Document this conversation.

CONFIDENTIALITY

It is extremely important for the hospitalist to maintain patient confidentiality. Patients have filed many lawsuits because of breach of confidential information. Adolescent patients are particularly sensitive about confidentiality. Older adolescents (15–19 years old) might avoid health care in order to prevent parents from finding out confidential medical information.36 Patient privacy is governed by federal, state, and local laws, and the privacy of patient information has changed extensively with the implementation of the Health Insurance Portability and Accountability Act (HIPAA). Medical notes and patient-identifiable data are confidential and cannot be communicated to any third party without the patient’s or parent’s written consent. When conversing about a patient, pay attention to who is nearby and may overhear the conversation. Securely protect test results and other confidential information throughout the hospital. Be cautious when using a computer monitor that does not have a privacy screen.37 E-mail has become important in the practice of medicine. However, e-mail can result in a breach of privacy if the message is inadvertently sent to the wrong person or left unattended on a computer screen, where it can be viewed by another party.37 E-mail is discoverable in legal proceedings. Electronic medical records have the capacity to improve quality of care by compiling and centralizing all pertinent information related to the pediatric patient. The electronic medical records system must protect the privacy of patients’ health information by restricting access according to local and federal laws and policies.37 Electronic health record systems should also have the ability to protect adolescent privacy and prevent inadvertent disclosure through billing activities.38

DISCHARGE FROM THE HOSPITAL At the time of discharge from the hospital or from the emergency department, review the child’s problems and treatment and give directions for ongoing therapy. Write down the diagnosis, give written discharge instructions, and review these carefully with the guardian and the patient if he or she is old enough to understand.29 Nonspecific instructions to “give fluids” or “return as needed” are not helpful. Avoid abbreviations that may not be familiar to the parents or patient. Give clear instructions about medication regimens, use of inhalers, and how to taper steroids. Always include in the instructions a

few examples of worrisome signs to look for at home. Although it is not feasible to list every possible complication, the parents must have a general idea of when to visit the PCP and what warrants an immediate return to the emergency department. If no radiologist is available in the hospital during off-hours, tell the family that the hospitalist’s reading of the radiographs is preliminary and that the films will be reviewed by a radiologist and the family notified of any change in interpretation.9,29 This strategy may prevent anger or the feeling of poor care if they are called back for additional treatment or referral. Some authorities recommend that the parents read back the instructions to the physician to indicate that they can comprehend the treatment plan.29 Engel et al. found that many patients discharged from an emergency department do not understand their care or the discharge instructions.39 Of course, the physician must be sure that the parents understand English. Contact translators or translation services before the patient leaves the hospital whenever there is reason to believe that the family does not understand the instructions.9 Computer-generated discharge instructions, commonly used by many hospitals, are advantageous because they are legible and readable if designed at an appropriate reading level. Most can be personalized and edited. Many have the capability to produce the instructions in a foreign language or with large print for the visually impaired. They can be programmed to print disclaimers about final readings of radiographs and electrocardiograms, which many physicians may neglect to do if handwriting is required. An exit interview, during which important information is reviewed with the guardians, also improves their retention of discharge information. Return visits to the emergency department can be avoided with careful discharge instructions, including written material that is geared to the reading level of the patient.29 In all cases, ask the family to sign that they have received and understand the discharge instructions.26 A copy must become part of the medical record, because the physician will not remember what was discussed with the family years later. Good discharge instructions may prevent litigation or at least affect negotiations and damages in the event of a lawsuit.26,29 At the time of discharge, ask the guardians if they have any questions.

Likewise, investigate potential social problems, such as inability to obtain medications, need for transportation home, inability to be seen by the PCP, or other factors that may interfere with the care of the child. This must be done in a caring, non-obtrusive manner. The hospitalist has an obligation to protect the child if he or she is discharged on medications with potentially serious side effects. For example, if a teenager is discharged on a sedative medication or has an unusual condition that may predispose him or her to seizures or syncope, advise the patient not to drive.9 Table 13-2 lists responsibilities of the hospitalist at the time of patient discharge from the hospital. TABLE 13-2

Hospitalist Responsibilities when Discharging a Pediatric Patient

Give written instructions and review them verbally with the parent or guardian List medications and treatments clearly Ensure that the patient or parent understands discharge instructions and the importance of keeping appointments Have the parent sign a form indicating that he or she received the instructions and understands them Specify an interim plan if the patient becomes ill before the scheduled appointment with the PCP Be specific and clear about when to see the PCP and when to return to the emergency department or hospital Arrange appropriate follow-up care Communicate with the PCP Be certain that the discharge summary and important documents reach the PCP Develop a system to review and forward incoming results and reread radiographs after patient discharge PCP, primary care provider.

FOLLOW-UP CARE

One particular area of risk involves follow-up care for patients who were treated by the hospitalist. The duty to provide follow-up care is shared by the hospitalist and the PCP.34,35 The inpatient team is responsible for evaluating whether the outpatient treatment plan is feasible for the child’s family and modifying the plan if needed.34,35 The hospitalist has a duty to provide instructions to patients and parents regarding follow-up care. Some patients may have trouble getting medications prescribed or could develop new symptoms after discharge. In such cases, the patient needs to know who to call in the interim.35 If the patient calls the hospitalist about a change in condition, forward this information to the PCP. Counsel patients about the risks of failing to receive ongoing care, and ensure that the PCP has enough information about the hospitalization to provide high-quality care when the patient arrives for follow-up.34,35 Despite everyone’s best intentions, things can be overlooked during the transition from the hospital to the outpatient setting.35 Hospitalists may be held liable for failing to inform patients and the PCP of laboratory studies or test results that are received after discharge. The AAP has recently published guidelines for the role of each physician involved in the care of a hospitalized child. The AAP believes that if not directly involved in the hospital care, the PCP should be notified at the time of discharge.40 For a successful transition from the hospital to primary care, communication between hospitalists and PCPs must be consistent, timely, and informative.40,41 Ensure that relevant information, including the discharge summary, gets to the PCP. Timely dictation services are needed to forward complete and legible records. The PCP has a legal obligation to ensure follow-up once the hospitalist has communicated with him or her and an appointment has been scheduled. Be careful of weekends and holidays. For example, telling patients to follow up with the PCP in 2 days on December 23 would be worthless. There may be differences in expectations surrounding the timing and format of communication between inpatient and outpatient healthcare providers, who is responsible for managing clinical concerns immediately after discharge, and who should follow up any pending studies that remain at the time of discharge. These potential differences of opinion can lead to negative patient outcomes if they are not reconciled between healthcare providers.10

CONCLUSION Hospitalists have many medicolegal obligations in the care of hospitalized children. The risk of lawsuits can be greatly reduced through an understanding of these risks, good communication and documentation, and attention to the needs and wishes of patients and their families.

REFERENCES 1. Simon TD, Mahant S, Cohen E. Pediatric hospital medicine and children with medical complexity: Past, present, and future. Curr Probl Pediatr Adolesc Health Care. 2012;42(5):113-119. 2. Jenna AB, Seabury S, Lakdawalla D, et al. Malpractice risk according to physician specialty. N Engl J Med. 2011;365:629-636. 3. Narang AS, Ey J. The emerging role of the pediatric hospitalist. Clin Pediatr. 2003;42:295-297. 4. Wachter RM, Goldman L. The hospitalist movement 5 years later. JAMA. 2002;287:487-494. 5. Landrigan CP, Conway PH, Edwards S, Srivastava R. Pediatric hospitalists: A systematic review of the literature. Pediatrics. 2006;117(5):1736-1744. 6. Landrigan, CP Srivastava, R. Pediatric hospitalists: Coming of age in 2012. Arch Pediatr Adolesc Med. 2012;166(8):696-699. 7. Mussman GM, Conway PH. Pediatric hospitalist systems versus traditional models of care: Effect on quality and cost outcomes. J Hosp Med. 2012;7(4):350-357. 8. Tenner PA, Dibrell H, Taylor RP. Improved survival with hospitalists in a pediatric intensive care unit. Crit Care Med. 2003;31:847-852. 9. Selbst SM, Korin JB. Preventing Malpractice Lawsuits in Pediatric Emergency Medicine. Dallas, TX: American College of Emergency Physicians; 1999. 10. Ruth JL, Geskey JM, Shaffer ML, Bramley HP, Paul IM. Evaluating communication between pediatric primary care physicians and hospitalists. Clin Pediatr. 2011;50(10):923-928.

11. Adamson TE, Schann JM, Guillan DS, et al. Physician communication skills and malpractice claims: A complex relationship. West J Med. 1989;150:356-360. 12. Hickson GB, Clayton EW, Githens PB, et al. Factors that prompted families to file malpractice claims following perinatal injuries. JAMA. 1992;267:1359-1363. 13. Lester GW, Smith SG. Listening and talking to patients: A remedy for malpractice suits? West J Med. 1993;158:268-272. 14. Little NE. Image of the emergency physician. In Henry GL, Sullivan DJ eds. Emergency Medicine Risk Management: A Comprehensive Review. 2nd ed. Dallas, TX: American College of Emergency Physicians; 1997:9-13. 15. Barnett PB. Rapport and the hospitalist. Am J Med. 2001;111:31S-35S. 16. American Academy of Pediatrics, Committee on Hospital Care. Familycentered care and the pediatrician’s role. Pediatrics. 2003;112(3):691696. 17. Mittal VS, Sigrest T, Ottolini MC, Rauch D, Lin H, Kit B, Landrigan CP, Flores G. Family-centered rounds on pediatric wards: A PRIS network survey of US and Canadian hospitalists. Pediatrics. 2010;126(1):37-43. 18. Hamm RM. Antibiotics and respiratory infections: Are patients more satisfied when expectations are met? J Fam Pract. 1996;43:56. 19. Sachetti A, Guzzetta C, Harris R. Family member presence during resuscitation attempts and invasive procedures: Is there science behind the emotion? Clin Pediatr Emerg Med. 2004;4:292-296. 20. Loren DJ, Klein EJ, Garbutt J, et al. Medical error disclosure among pediatricians. Arch Pediatr Adolesc Med. 2008;163:922-927. 21. American Academy of Pediatrics, Committee on Pediatric Emergency Medicine and Committee on Bioethics. Consent for emergency services for children and adolescents. Pediatrics. 2011;128:427-433. 22. Kassutto Z, Vaught W. Informed decision making and refusal of treatment. Clin Pediatr Emerg Med. 2003;4:285-291. 23. McDonnell WM. Pediatric emergency medicine. In Donn SM, McAbee GN eds. Medicolegal Issues in Pediatrics. 7th ed. Elk Grove Village, IL:

American Academy of Pediatrics; 2012:141-152. 24. Diekema DS. Parental refusals of medical treatment: The harm principle as threshold for state intervention. Theor Med Bioth. 2004;25:243-264. 25. Sheldon M. Ethical issues in the forced transfusion of Jehovah’s Witness children. J Emerg Med. 1996;14:251-257. 26. Selbst SM, Korin JB. Malpractice and emergency care: Doing right by the patient—and yourself. Contemp Pediatr. 2000:17:88-106. 27. Virapongse A, Bates DW, Shi D, et al. Electronic health records and malpractice claims in office practice. Arch Intern Med. 2008;168:23622367. 28. Mangalmurti SS, Murtagh L, Mello MM. Medical malpractice liability in the age of electronic health records. N Engl J Med. 2010;363:20602067. 29. Yu KT, Green RA. Critical aspects of emergency department documentation and communication. Emerg Med Clin North Am. 2009;27:641-654. 30. AAP Department of Practice. Jury still out on whether EHRs reduce malpractice risk. AAP News. 2010;Feb:17. 31. Wilde JA, Pedroni AT. The do’s and don’ts of consultations. Contemp Pediatr. 1991;8:23-28. 32. Holliman CJ. The art of dealing with consultants. J Emerg Med. 1993;11:633-640. 33. O’Riordan WD. Consultations. In Henry GL, Sullivan DJ eds. Emergency Medicine Risk Management: A Comprehensive Review. 2nd ed. Dallas, TX: American College of Emergency Physicians; 1997;329334. 34. Alpers A. Key legal principles for hospitalists. Am J Med. 2001;111:5S9S. 35. Crane M. When a hospitalist discharges your patients. Med Econ. 2000;6:41-54. 36. Britto MT, Tivorsak TL, Slap GB. Adolescents’ needs for health care. Pediatrics. 2010;126:e1469-e1476. 37. McAbee GN, Brown JL. Legal issues related to e-mail, web sites and

telemedicine. In Donn SM, McAbee GN eds. Medicolegal Issues in Pediatrics. 7th ed. Elk Grove Village, IL: American Academy of Pediatrics. 2012:83-94. 38. American Academy of Pediatrics, Committee on Adolescence and Council on Clinical and Information Technology. Standards for health information technology to ensure adolescent privacy. Pediatrics. 2012;130:987-990. 39. Engel K, Heisler M, Smith D, et al. Patient comprehension of emergency department care and instructions: Are patients aware of when they do not understand. Ann Emerg Med. 2009;53:454-461. 40. Percelay CM, Strong GB, American Academy of Pediatrics Section on Hospital Medicine. Guiding principles for pediatric hospitalist programs. Pediatrics. 2005;115:1101-1102. 41. Lye P, American Academy of Pediatrics Committee on Hospital Care and Section on Hospital Medicine. Clinical report - Physicians’ roles in coordinating care of hospitalized children. Pediatrics. 2010;126:829832.

CHAPTER

14

Hospitalist Professional Organizations and Models of Care Daniel A. Rauch and David Zipes

PROFESSIONAL ORGANIZATIONS Since Wachter and Goldman coined the term hospitalist in 1996,1 the field has undergone tremendous growth and change and is now well established. The Academic Pediatric Association (APA), the American Academy of Pediatrics (AAP), and the Society of Hospital Medicine (SHM) are all strongly invested in pediatric hospital medicine. The three organizations each bring significant strengths to individual hospitalists and the field as a whole. All three organizations serve as sponsors of the annual Pediatric Hospital Medicine (PHM) conference, the largest national PHM gathering. The APA’s mission is to improve the health and well-being of all children and adolescents by promoting research, advancing a scholarly approach to education, developing innovations in healthcare delivery, advocating for an equitable child health agenda, and fostering leadership and career development of child health professionals. The APA sponsored the first national PHM conference in 2003 and provides PHM-specific content at the annual Pediatric Academic Societies conference through original science presentations and the Hospital Medicine Special Interest Group. Hospitalists are considered a core constituency and have been involved in development of the strategic plan, included as editors of the APA journal Academic Pediatrics, and have served as partial motivation for the change in name from the “Ambulatory” Pediatric Association to its current more inclusive Academic Pediatric Association. APA career development projects such as the Leadership in Academic Pediatrics conferences, the Educational Scholars Program, the Research Scholars Program, and the new Quality Scholars Program actively solicit participation of hospitalists.

The mission of the AAP is to attain optimal physical, mental, and social health and well-being for all infants, children, adolescents, and young adults. To advance that mission, the AAP has been a strong advocate of PHM. The Section on Hospital Medicine (SOHM) has been the fastest growing section in the AAP over the last 10 years and now counts almost 2000 members. The SOHM sponsored the development of the first journal dedicated to PHM, Hospital Pediatrics, as well as a forthcoming PHM PREP product. The SOHM created the national PHM Fellows Conference, sponsors a day-long program at the AAP National Conference and Exhibit, and supports grants for trainees and international hospitalists to the summer PHM conference. The SOHM was the first sponsor of the APEX (Advancing Pediatric Educator Excellence) Teaching program. The SOHM has seven subcommittees involving all aspects of PHM. The Committee on Hospital Care is a separate AAP entity from SOHM that includes hospitalists as well as other pediatricians involved in the care of hospitalized patients (surgeons, intensivists, etc.). It is charged with addressing AAP policy issues on all hospital care and so addresses many PHM activities. Many other AAP sections, committees and councils impact on care of the hospitalized child and so provide many opportunities for hospitalists to take on leadership roles within the AAP. The mission of the SHM is to promote the highest quality of care for all hospitalized patients and excellence in the practice of hospital medicine through education, advocacy, and research. Of the nation’s estimated 40,000 hospitalists, 12,000 are SHM members. SHM is a “big tent” organization that includes hospitalists from all disciplines (internal medicine, family medicine, pediatrics, and more recently general surgery, and obstetrics, as well as medical and surgical subspecialty fields) along with non-physicians such as physician assistants, nurse practitioners, and practice managers. SHM advocates for healthcare reform nationally, and many Medicare reform proposals are likely to impact Medicaid payment models in the future. Patient safety, quality improvement, leadership, and practice management efforts are directly parallel to issues pediatric hospitalists face, although clinical topics obviously vary. SHM sponsors an annual meeting that includes a pediatric track, publishes the Journal of Hospital Medicine, and has a specific Pediatric Committee that addresses pediatric-specific issues within and on behalf of SHM. SHM was the prime mover behind creation of recognition of focusedpractice in hospital medicine by the American Board of Internal Medicine

(ABIM). Under this model, ABIM-certified internists with 3 years of hospitalist experience may earn the above designation by completing hospital medicine–specific performance improvement and self-assessment modules and passing a secure hospital medicine–specific exam without any formal or informal fellowship training. More recently, several additional organizations have arisen that are also moving the field of PHM forward. The Pediatric Research in Inpatient Settings (PRIS) network is a research network focused on conducting multicentered studies in areas of inpatient care relevant to the clinician. PRIS has been successful in obtaining several large grants and has carried out several studies that promise to impact the practice of PHM. The Value in Inpatient Pediatrics (VIP) network is focused on process improvement projects with measurable outcomes. VIP has had several successful projects regarding bronchiolitis, patient identification, and discharge communication. VIP is part of the AAP Quality Improvement Innovations Networks. The Consortium of Pediatric Hospital Medicine (CPHM) includes representatives from the APA, AAP, and SHM, and helps coordinate activities between the organizations on behalf of PHM such as representation to the Council of Pediatric Subspecialties (CoPS) and the Pediatric Academic Societies’ Annual Meeting planning committee. The newly formed American Board of Pediatrics PHM sub-board is responsible for creating the certifying exam and MOC. The JCPHM includes representatives from the APA, AAP, SHM, and PRIS, as well as at-large members from the PHM community, including a dedicated spot for a community hospitalist. The JCPHM vision statement is “Pediatric hospitalists will transform the delivery of hospital care for children,” which will be attained through seven goals. The JCPHM convened the Strategic Planning Committee to thoughtfully examine the options for the field moving forward, ranging from American Board of Pediatrics (ABP) subspecialty recognition to maintaining the current status.

EMPLOYMENT AND COMPENSATION MODELS Multiple different employment and compensation models for hospitalists exist, and no one compensation model works for all situations. Each has different driving forces, advantages, and disadvantages. There are six basic employment categories: self-employed hospitalist-only groups, multistate hospitalist-only groups, hospital or hospital corporations, academic hospitals,

multispecialty groups, and insurance industry groups (managed care organizations). Certainly, there are permutations of and variations on these schemes, and as hospital medicine grows and evolves, other models may emerge. According to the SHM’s 2012 survey, most pediatric hospitalists are employed by hospitals or hospital corporations, with academic institutions running a distant second. The financial implications and limitations of each employment model are the driving forces behind their development and utilization. According to the SHM survey, when professional fees are compared with the costs of providing services, most groups are in the red. Most hospitalist programs therefore require external support to remain solvent. Although hospitalist systems yield cost savings to the system as a whole (usually more than accounting for their cost), largely owing to decreased lengths of stay and other benefits (e.g. system improvements, resident education), the decision of who should pay for these benefits varies considerably. Should it be the insurance companies, in the form of increased payments for inpatient care; the hospitals, in the form of bonuses, salaries, or subsidies; the PCPs; or the many other entities that benefit from a well-run hospitalist model? The SHM survey shows that currently hospitals (academic/university and community) employ almost 80% of all pediatric hospitalists. It is therefore not surprising that according to the PRIS survey, hospitals are also the most common source of external support for pediatric hospitalist programs.2 Cost-sharing models for the beneficiaries of hospitalist programs must be developed. If increased reimbursement for hospitalized patients can be achieved the employment models may shift, but for now, the trend of hospitals employing hospitalists will likely continue. The basic compensation models include salary only, productivity only (i.e. professional fees), and mixed productivity and compensation. Again, permutations and variations may exist, and new models may emerge in the future. According to the 2004 SHM productivity and compensation survey, more than 60% of all hospitalist programs receive money beyond what their professional fees generate; 80% of academic models receive supplementation. Among individual hospitalists, 38% are purely salaried, 8% earn productivity-based income, and 45% receive a combination of the two. Bonuses can be a variable in any of the three compensation models, and according to the survey, 61% of hospitalists receive some sort of bonus.

Although bonuses can certainly be an effective motivational tool, one needs to be cognizant of the potential legal ramifications of incentive systems. The Stark Law, for example, prohibits the referral of Medicare and Medicaid patients to an entity for the provision of certain health services if the physician or a member of the physician’s immediate family has a direct or indirect financial relationship with the entity. Bonuses can be interpreted as an indirect financial relationship. Consultation with legal counsel is strongly recommended during the development of incentive or bonus plans. Type of insurance (e.g. Medicaid, private, health maintenance organization, self-pay), reimbursement strategy (e.g. fee for service, capitated), inpatient volume, and type of hospital (e.g. community, academic) all have an impact on the type of employment and compensation models implemented. Much like Charles Darwin’s well-described Galapagos finches, the models that survive will be those that are well adapted to their particular environment. No one model works for every situation. Increased reimbursement for hospital care would likely affect compensation models as well as employment models.

SUBSPECIALTY HOSPITALIST MODELS A growing field that does not fit neatly into any of the previously described models is that of the subspecialty hospitalist. At present, neonatal (including well newborns and pediatric intensive care units) seem to be the largest employers of general pediatric hospitalists in positions traditionally filled by subspecialists. These hospitalists may work exclusively in the intensive care unit or both in that unit and on the general pediatric floor. Additionally, urgent care centers and emergency departments may employ hospitalists. The phenomenon of using hospitalists in other subspecialties is burgeoning in adult patient care but is just beginning to occur in the pediatric hospitalist community. Adult subspecialties that commonly use hospitalists include cardiology, hematology/oncology, neurology, obstetrics/gynecology, orthopedics, and pulmonology. These hospitalists can be trained in general pediatrics, internal medicine, or subspecialties. Any specialty with a large inpatient population can adopt this model. Freed et al.,3 in surveys sponsored by the ABP, showed that hospitalists are active wherever patients exist in the hospital. The relatively small inpatient volumes of most pediatric subspecialties (excluding neonatal and pediatric intensive care units) may

limit the cost-effectiveness of hiring hospitalists to care exclusively for patients of a particular subspecialty except in large academic centers, although models in which general pediatric hospitalists care for subspecialty patients as part of their job are emerging. The economic reality in smaller community hospitals has been a driving force for pediatric hospitalists to multitask and cover or cross-cover nurseries, NICUs, PICUs, emergency departments, and urgent care centers. Additionally, there are jobs focusing on single aspects or patient type within hospital medicine, such as care of the complex medical patient or sedation services.

SUGGESTED READINGS AHA survey, 2004, submitted for publication. AMA Physician Characteristics and Distribution in the US, 2004. Miller JA. The hospitalist movement: The quiet revolution in healthcare. HealthLeaders News. 2003;Jan 8. Pediatrician attitudes toward and experiences with pediatric hospitalists: A national survey. Society of Hospital Medicine Sixth Annual Meeting Abstracts, 2003. Shipman SA, Lurie JD, Goodman DC. The general pediatrician: Projecting future workforce supply and requirements. Pediatrics. 2004;113:435. Wachter RM. The hospitalist movement: Ten issues to consider. Hosp Pract (Minneapolis). 1999;34:95-98,104-106,111.

REFERENCES 1. Wachter RM, Goldman L. The emerging role of “hospitalists” in the American health care system. N Engl J Med. 1996;335:514. 2. Chiang VW, Landrigan CP, Stucky E, Ottolini MC. Financial health of pediatric hospitalist systems: A study from the Pediatric Research in Inpatient Settings (PRIS) network. Pediatr Res. 2004;55:1794. 3. Freed GL, Dunham KM; Research Advisory Committee of the American Board of Pediatrics. Pediatric hospitalists: training, current practice, and career goals. J Hosp Med. 2009;4(3):179186pmid:19301370

CHAPTER

15

Careers in Hospital Medicine Karen Smith and Mary C. Ottolini

BACKGROUND Adaptability is a core attribute for the successful Pediatric Hospitalist (PH). Perhaps it is because our specialty is so young that accepting and leading change seems to be woven into the PH’s DNA. Adaptability has served us well as our specialty has and continues to evolve to meet the changes in healthcare delivery models while striving to improve the care delivered to hospitalized children. In 1996 Dr. Robert Wachter defined a hospitalist as a physician dedicated to the delivery of comprehensive medical care to hospitalized patients, while engaging in clinical care, teaching, research, or leadership in the field of General Hospital Medicine. In addition, hospitalists work to enhance the performance of hospitals and healthcare systems.1 This definition for the hospitalist caring for adult patients also fits the pediatric hospitalist (PH), but in addition to providing expert care for infants and children, the PH must be proficient in providing family-centered patient care.2 Clinical expertise therefore, is another core attribute for PH. Pediatric Hospitalist Medicine (PHM) initially evolved to address the patient care needs of the hospitalized child. The PHM model of care diverged from the traditional model where pediatricians rounded on the ward and in the nursery at the beginning and end of the day, while spending the bulk of time caring for patients in the office setting. The traditional model worked because individual pediatricians generally had few inpatients with low acuity and relied on pediatric residents and subspecialists for hands-on management during office hours.3 Since the mid-1990’s care of the hospitalized child has become increasingly complex, both in terms of the nature of the underlying disease process, the complexity of the patient with respect to multisystem

chronic illness and survival based on medical technology.4 Increasingly, only the sickest of patients are hospitalized.5 The increased acuity and complexity of inpatient pediatrics coupled with external pressures focusing on patient safety and hospital utilization made the traditional model untenable for the majority of primary care pediatricians. What sets the PH apart from the primary care pediatrician however, is her/his focus on systems-based practice within the hospital setting. Essentially, everything that a PH does must be in the context of the impact on the healthcare system as well as that of the patient.6 Dr. Wachter stated during a recent American Public Media interview “…the doctor of the future has two sick patients. One is the patient they are taking care of. One is the system they are working in,”.7 This is especially true for PHs who are frequently the linchpin in effective patient management within the complex and expensive inpatient setting. In essence Wachter is saying that one of the keys to curing our healthcare system is preparing Hospitalists for effective careers. In this chapter we describe current career opportunities for pediatric hospitalists in a variety of settings. These include the tertiary care academic university/children’s hospital, the community hospital academically affiliated with a university program, and the non-affiliated community hospital with a smaller pediatric unit. We also describe possible career building experiences to achieve success in the various career roles for hospitalists in both community and academic settings. Finally, we look at building careers not only from the standpoint of the trainee or new hospitalist, but from the standpoint of the division chief who wants to develop the careers of the faculty within his or her division.

HOSPITAL SETTING TEACHING HOSPITAL Clinical Pediatric hospitalists fill a number of clinical roles in academic hospitals, and their careers can potentially focus on some or all of these roles. The clinical role of the PH is analogous to that of the utility infielder on a baseball team.8 The breadth of practice can range from caring for straightforward to medically complex patients,9 alone or as consultants or co-

managers with surgical subspecialists.10 Advances in pediatric medicine over the last generation have resulted in a population of medically complex patients, frequently with intellectual disabilities, who are growing into adulthood as disease processes that were once fatal have become treatable. The emerging field of complex care focuses on the holistic medical care of children with complex diseases.11 Many complex patients experience frequent hospitalizations as a result of exacerbations of chronic illness or technology malfunction.12 Their care requires general pediatric skills, expertise in coordination of care, and expertise in communication with patients’ families and other medical and nonmedical providers.13 PH are pivotal to providing the coordinated care needed care for these costly patients within accountable care organizations.14 PH are evolving models where they consult during pre-surgical outpatient visits to develop a plan of care that they later implement in the postoperative period.15 They are also critical to the smooth transition of the patient from the acute inpatient setting to home. Hospitalists increasingly staff follow-up clinics or transitional/rehabilitation facilities that serve to continue to provide care and coordinate the transition of responsibility back to the family and primary care provider and medical home.16 At the polar opposite of medically complex patients, PHs provide care for sick children increasingly classified as “observation patients”; previously healthy with an acute single-organ system disease process, such as asthma, necessitating brief hospital stays and rapid handoffs from the Emergency Department back to the Primary Medical Doctor (PMD).17 The PH must work with insurance payers and hospital utilization management departments to develop and continually refine evidence-based best processes to efficiently manage these patients at low cost in an inherently complex hospital environment. For both acute and complex patients, PHs often assume the role of proceduralists,18 performing conscious sedation, placing percutaneous catheters, etc.19 Some PHs also function as subspecialty PHs, extending the capability of subspecialists as an embedded member of the subspecialty inpatient team.20 Most subspecialty PHs obtain on-the-job training from subspecialists to be able to manage common problems that occur in that patient population. The PH remains present on inpatient units supervising

residents or ancillary staff and troubleshooting patient problems, while the subspecialist may be working in the outpatient clinic or doing procedures. Education In a typical Academic Children’s Hospital, the PH serves as the supervisor and team leader for pediatric residents medical students, and in some cases PH fellows. In this role they are responsible not only for day-today patient care decisions, but also for the education of trainees at a variety of levels.21 Numerous educational career opportunities exist for PHs. The role of the PH in medical education has increased dramatically over the past two decades. In a 1998 survey of academic pediatric department chairs in Canada and the United States, Srivastava et al. determined that 77% of respondents either had or were planning to institute pediatric hospitalists within their institutions.22 In those institutions with hospitals, two-thirds of hospitals were involved in teaching. By early 2008 approximately 75% of pediatric residents, program directors, and clerkship directors reported using PHs as teaching attendings.19 In a survey of a national sample of PHs, 94% of respondents reported teaching medical students and pediatric and housestaff, while 45% of respondents reported holding a leadership position in education.23 PH respondents to the 2007 PRIS (Pediatric Research in Inpatient Settings) network survey also reported intense involvement in medical education. Although most respondents reported teaching pediatric residents and medical students, up to 40% indicated that they were teaching other trainees, faculty members and community pediatricians.24 Hospitalists have been recognized by trainees as making exemplary teachers because of their enthusiasm, evidence-based practice, and ability to role-model core competencies such as interpersonal skills and professionalism through their daily interactions with parents/patients during family-centered rounds and interdisciplinary teams.25 These daily activities provide opportunities to also assess these skills in trainees, which is essential to collect data for the Milestones project.26 To be maximally effective in teaching activities, hospitalists must seek opportunities to further refine their teaching skills. Many residency programs now have RATS programs (Residents as Teachers)27 so that graduates pursuing hospitalist careers have an understanding of how to apply adult learning principles in their teaching strategies, and are effective at orienting

and providing feedback to learners. Hospitalists can learn teaching skills by attending workshops at regional and national conferences, such as the Pediatric Hospitalist Medicine Conference, the Society for Hospital Medicine annual conference, the Pediatric Academic Society Annual Meeting and the Pediatric Educational Excellence Across the Continuum (PEEAC) Conference.28 For more advanced study, there are several structured programs available through national organizations, such as the Academic Pediatric Association’s Educational Scholar Program (ESP),29 through institutions such as the Harvard Macy Foundation30 and formal universitybased Masters in Education certificate or degree programs (see Table 15-1). TABLE 15-1

Educational Resources

APA Educational Scholars Reference on Educator Portfolio Harvard Macy Foundation Michigan State Primary Care Faculty Development Program University of Chicago Master of health Professions Education The Master Teacher Leadership Development Course PEEAC Meeting Pediatric Academic Society Meeting SHM Academic Hospitalist Academy Association of Pediatric Program Directors Council on Medical Student Education in Pediatric Council of Pediatric Subspecialists Accreditation Council for Graduate Medical Education AAMC Member Communities Annual Meeting GRA- Group on Resident Affairs GEA- Group on Educational Affairs AAMC Group on Women in Medicine and Science Early Career Women Faculty Professional Development Mid Career Women Faculty Professional Development

Executive Leadership in Academic Medicine MedEdPORTAL To continually refine their teaching skills, hospitalists should reflect on feedback from their learners. They should encourage learners to provide qualitative feedback in addition to the standard numeric ratings.31 A safe learning climate is essential for this. In addition, peer feedback is invaluable to improving teaching techniques. Peer coaching serves several purposes in addition to providing feedback to the hospitalist being observed.32 By observing the practices of others, division members develop more uniformity in teaching and expectations for learners. Peer assessment makes teaching a more scholarly activity and raises its importance within the division and institution. Hospitalists are evolving the educational model in the inpatient setting. Traditional inpatient teaching has been carried out in teacher-led small groups using case-based didactics, where the attending described the epidemiology, clinical presentation, diagnostic testing, and management of a specific disease or syndrome. As PHs with advanced educational training assume leadership roles, they are incorporating best practices in adult learning theory to meet challenges posed by duty hour changes and make the most of opportunities afforded by technology. For example, PHs have developed and implemented a nighttime curriculum to enhance resident learning during the night shift.33 PHs lead simulation activities designed to provide deliberate practice in procedural skills and teamwork.34 Computer-based learning management systems provide PHs with a repository for e-learning modules—multimedia files and readings that can be delivered in a programmed sequence to learners so that they access and learn background information at their own pace and schedule.35 This “flipped classroom” approach allows small group teaching sessions to be learner-centered interactive discussions on how to apply background information to patients on the clinical service.36 Family-centered bedside rounds are an ideal setting for teaching and assessing communication, physical exam, diagnostic reasoning, and team leadership skills.37 Hospitalists are uniquely positioned to teach clinical reasoning skills, to emphasize the importance of good physical exam skills and judicious use of diagnostic testing. This is critical for reducing

unnecessary healthcare costs and to avoid diagnostic errors.38 PHs are in a unique position to advocate for optimal resident case experience and graduated autonomy, based upon entrustment decisions they can make through close working relationships with residents.39 PH educators can best position themselves to succeed in local leadership opportunities as the clerkship, residency, fellowship, or continuing medical education (CME) director by attending academic leadership conferences such as those sponsored by the Academic Pediatric Association or Society for Hospital Medicine. To advance academically as clinician-educators, PHs must produce scholarship that will advance the field. Scholarship in pediatric hospital medicine (PHM) need not be limited to published articles; it can include educational resources such as curricula and assessment tools that have been evaluated and posted on peer-reviewed repositories, such as Med Ed Portal.40 Research PH research has been rapidly advancing as the field as a whole has matured, and an increasing number of pediatric hospitalists are engaging in research as either the primary focus or as part of their professional careers. Initial hospitalist research focused on defining and characterizing the field. Much of the work was done through surveys of key stakeholders.41 The effectiveness and efficiency of hospitalist systems was also studied in initial hospitalist research.42,43 After these initial studies, PHM research began to focus on single- or multicenter retrospective studies that addressed critical PHM clinical issues.44 Large multicenter retrospective studies using administrative data such as the PHIS database have allowed PHs to compare variability in outcomes based upon treatment strategies.45 Variability of care across children’s hospitals has been described for a variety of diseases such as pneumonia and urinary tract infections.46 A problem with such studies is that it is difficult to definitively associate a specific treatment with an outcome. Prospective comparative effectiveness trials are needed to determine the impact of variability of different practices on patient outcomes in order to establish best evidence for practice. Once best practices are described, then quality improvement studies can help to implement and test the approach to determine the best process to adapt in a particular practice. Within PHM, several networks have evolved to enable research leaders across the country to collaborate in conducting clinical effectiveness and quality improvement

research. Two important networks focused on quality improvement and comparative effectiveness research are the PHM Value in Pediatrics Network,47 which merged with the American Academy of Pediatrics Quality Improvement Innovation Network (QuIIN),48 and PRIS.49,50 QuIIN is a network of practicing pediatricians and their staff that aims to improve care and outcomes for children and families through quality improvement (QI) projects. In 2010, PRIS partnered with the Child Health Corporation of America (CHCA) to create a national agenda for comparative effectiveness research QI studies conducted by PHs called the Prioritization Project.51 This project identifies inpatient medical and surgical pediatric conditions with the greatest opportunities to reduce patient costs and improve outcomes by decreasing variability in practice. Other PRIS studies such as the I-PASS study are designed to enhance patient safety by improving the handoff process through focused education and deliberate practice. The I-PASS project is an example of linking PH educational, QI, and leadership roles to effect rapid institutional change and measure the impact on patient outcomes, particularly medical errors. This is an example of a study looking at those processes that affects patient care as a whole, rather than for specific diseases.52 Working with electronic medical records and administrative data collected by CHCA, PRIS is trying to create a much more robust multicenter database that includes not only ICD-9 codes, but some clinical data— laboratory, radiology, and microbiology results. The success of PHM research in the future will require researchers with advanced training in study design, research methods, and data interpretation as well as the ability to reach out to colleagues to determine clinically meaningful research questions. Mentorship is needed to foster networking and success in competing for grant funding. This mentorship may occur informally within institutions and relationships developed at national meetings for PHs. However, to develop the critical mass of individuals needed to advance the specialty of PHM, a more formal approach will be needed through the rigorous training provided by PHM Fellowships focused on research training,53,54 and advanced degrees in clinical and translational science, Masters in Public Health or Quality Improvement/Informatics (see Table 15-2).

TABLE 15-2

Research Resources

ACADEMIC PEDIATRIC ASSOCIATION RESEARCH SCHOLARS PROGRAM PRIS APA YOUNG INVESTIGATOR AWARDS APA FELLOW RESEARCH AWARD NIH INTRODUCTION TO PRINCIPLES AND PRACTICE OF CLINICAL RESEARCH NIH CAREER DEVELOPMENT AWARDS (K AWARDS) CTSA- CLINICAL TRANSLATIONAL SCIENCE AWARDS Masters Degree Programs in Clinical and Translational Science

ADMINISTRATION AND LEADERSHIP Health care’s new leaders must organize doctors into teams, measure their performance not by how much they do but by how their patients fare, deftly apply financial and behavioral incentives, improve processes, and dismantle dysfunctional cultures.55 Hospital medicine is well suited to produce leaders in hospital administration. Hospitalists are caring for an increasing number of patients either as the primary attending, co-manager, or consultant. As a result, there are many opportunities for leadership. Often the path begins as a leader of a QI initiative or committee, which then progresses to unit director, assistant chief, chief, and so on. Even with this trajectory, however, many studies have shown that most physicians have little or no formal training in business or leadership.56 The heavy clinical load and changing schedule of a hospitalist adds to the difficulty in obtaining formal education. However, there are now many opportunities for in-person as well as online, asynchronous, or shortterm educational courses for a degree or certificate. Table 15-3 provides examples of educational options for administrative leadership training. TABLE 15-3

Career Preparation

Administration can be broken down into the following categories: regulatory requirements, financial performance, staff development, and management. Regulatory requirements involve both healthcare regulations and human resource requirements. Healthcare regulations vary slightly based on the type of institution and location (city/state). Common sources of regulation include the Joint Commission, Centers for Medicare and Medicaid Services (CMS), and the local department of health. Human resource requirements involve

employment law and regulations (federal, state, and city). These are often outlined in hospital policy and procedures. Financial performance is coming under increasing scrutiny. This includes both the cost of hospital medicine programs and the service utilization per patient by the individual hospitalist that will affect the hospital’s bottom line and reimbursement by insurance companies. Key areas of importance are return on investment, maintaining a positive margin, and developing a business plan, or “pro forma,” for new projects. As an administrator there are basic management duties essential to the performance of a department or division. This includes developing a schedule, obtaining resources (space, computers, and equipment) and maintaining a structure for the functioning of the division. Often overlooked, these functions can have a significant impact on staff satisfaction. Lastly, the hospital medicine leader needs to inspire and develop the members of the division. This is especially crucial with junior faculty who may have varying levels of training. Strategies for staff development are discussed later in this chapter. Opportunities in administration can come from several sources: the university, hospital, and medical staff of the hospital. Depending on the employment model, hospitalist programs can be employed by the university, hospital, or as an independent company. Leadership positions can include director of a department or unit, chief of a division, chair of a department, or chief medical officer. In addition to these roles, every hospital is required to have a separate organized medical staff. These leadership opportunities include chief of pediatrics, medical executive committee member, and president of the medical staff.

COMMUNITY HOSPITALS Community hospitals provide an excellent opportunity for leadership in hospital medicine. Compared to academic institutions, there are very few providers in a community hospital with pediatric expertise. Therefore, a community hospital-based PH is frequently identified as the expert in all things pediatric. This can be a blessing or a curse. However, there is great reward in establishing a safe, effective pediatric service for the community. Key areas of focus include medication safety, managing emergencies for both

the individual decompensating patient and community disasters, improving hospital efficiency through pathways and protocols, and educating allied health providers and other physicians on pediatric topics. The key to success in community hospitals is building relationships. By developing strong personal relationships with other departments, allied health staff, and community providers, a PH’s input and leadership will be regarded as a benefit rather than a threat.

STRATEGIES FOR STAFF DEVELOPMENT Leaders of hospital medicine divisions and departments are challenged with the professional growth of a relatively young workforce with varying degrees of training and experience.57 Barriers to growth are many, including limited numbers of senior mentors in pediatric hospital medicine within an institution and nationally, heavy clinical load, and limited formal training. However, many leadership opportunities are available within this new field of medicine. It is crucial that leaders in hospital medicine assist junior staff to take on leadership roles in their department, institution, and nationally. Key factors that promote leadership development include: Mentorship/Coaching Goal setting Career tracking/Reflection Early leadership opportunities Removal of barriers Promotion of success The remainder of this chapter focuses on two valuable tools that are underutilized: mentorship and coaching.57 Mentorship focuses on the individual and has a broad focus and a long-term commitment. Mentors can be within or outside a department. Due to the young age of the field of hospital medicine, available mentors for PHs are often more easily identified from other specialties. A leader can help members identify and connect with these types of mentors. An alternative and sometimes more effective option is peer mentorship.58 Peer mentorship capitalizes on relationships between colleagues as a valuable source of emotional support, feedback, advice, and skill development. The advantages of peer mentorship include shared

generational values, lack of power differential, and a higher abundance of mentors compared to the traditional model of mentorship. Most peer mentorship groups consist of four to five members. Some but not all programs have included senior leadership as facilitators. Several programs have described their peer mentorship program including structure and outcomes.59-61 In fact, Poloi and Knight found that peer mentorship demonstrated the most gain for the members and the most consistent attendance compared to traditional mentorship.58 For short, time-limited performance improvement, coaching is a viable option. Coaches do not need to be experts, but should be proficient in a particular task. Similar to peer mentorship, coaching allows members of the same department to assist their colleagues in performance improvement. Coaching sessions usually occur in a dyad and can focus on a variety of performance measures including communication, procedures, team leading, and so forth. Compared to mentorship, coaching relationships are usually for a short time period until the performance goal is met.62 Every leader will need to adapt their strategies and processes for faculty development to meet the needs of their members in relation to the institutional requirements for promotion and growth. Table 15-3 provides additional examples and opportunities for faculty development based on the interests of the individual faculty member. The information provided is not meant to be exhaustive, but rather to inspire creativity in developing one’s own career or that of a hospitalist group. Before you are a leader, success is all about growing yourself. When you become a leader, success is all about growing others. Jack Welch

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3. Seelbach EB, Ottolini MC. Primary care physicians and hospitalists: two branches of the same tree. Pediatr Ann. 2010;39(2):84-88. 4. Simon TD, Berry J, Feudtner C, et al. Children with complex chronic conditions in inpatient hospital settings in the United States. Pediatrics. 2010;126(4):647-655. 5. Burns KH, Casey PH, Lyle RE, Bird TM, Fusell JJ, Robbins JM. Increasing Prevalence of medically complex children in US hospitals. Pediatrics. 2010;126:638-646. 6. Teufel RJ 2nd, Garber M, Taylor RC. Pediatric hospitalist: a national and regional trend. J S C Med Assoc. 2007;103(5):126-129. 7. Gorenstien D. From ‘god-like’ to team huddle: training doctors for a new health care future Marketplace Morning Report, American Public Media. March 15, 2013. 8. Fisher ES. Pediatric hospital medicine: Historical perspectives, inspired future. Curr Probl Pediatr Adolesc Health Care. 2012;42(5):107-112. 9. Srivastava R, Stone BL, Murphy NA. Hospitalist care of the medically complex child. Pediatr Clin North Am. 2005;52:1165-1187. 10. Rappaport DL, Pressel DM. Pediatric hospitalist comanagement of surgical patients: Challenges and opportunities. Clin Pediatr. 2008;47:114-121. 11. Cohen E, Kuo DZ, Agrawal R, et al. Children with medical complexity: An emerging population for clinical and research initiatives. Pediatrics. 2011;127:528-538. 12. Berry JG, Hall DE, Kuo DZ, Cohen E, Agrawal R, Feudtner C, et al. Hospital utilization and characteristics of patients experiencing recurrent admissions within children’s hospitals. JAMA. 2011;305:682-690. 13. Simon TD, Mahant S, Cohen E. Pediatric hospital medicine and children with medical complexity: Past, present, and future. Curr Probl Pediatr Adolesc Health Care. 2012;42(5):113-119. 14. Ovretveit J. Evidence: Does Clinical Coordination Improve Quality and Save Money? London: Health Foundation. 2011. 15. Simon TD, Eilert R, Dickinson LM, Kempe A, Benefield E, Berman S. Pediatric hospitalist comanagement of spinal fusion surgery patients. J Hosp Med. 2007;2(1):23-30.

16. Misky GJ, Wald HL, Coleman EA. Post-hospitalization transitions: Examining the effects of timing of primary care provider follow-up. J Hosp Med. 2010;392-397. 17. Chadaga SR, Maher MP, Maller N, et al. Evolving practice of hospital medicine and its impact on hospital throughput and efficiencies. J Hosp Med. 2012;7(8):649-654. 18. Connolly B, Mahant S. The pediatric hospitalist and interventional radiologist: A model for clinical care in interventional radiology. J Vasc Interv Radiol. 2006;17:1733-1738. 19. Turmelle M, Moscoso LM, Hamlin KP, Daud YN, Carlson DW. Development of a pediatric hospitalist sedation service: Training and implementation. J Hosp Med. 2012;7(4):335-339. 20. Nash KB, Josephson SA, Sun K, Ferriero DM. Should there be pediatric neurohospitalists? Neurology. 2013;80(10):957-962. 21. Freed GL, Dunham KM, Lamarand KE. Hospitalists’ involvement in pediatrics training: Perspectives from pediatric residency program and clerkship directors. Acad Med. 2009;84:1617-1621. 22. Srivastava R, Landrigan C, Gidwani P, Harary OH, Muret-Wagstaff S, Homer CJ. Pediatric hospitalists in Canada and the United States: A survey of pediatric academic department chairs. Ambul Pediatr. 2001;1(6):338-339. 23. Freed GL, Dunahm KM. Research Advisory Committee of the American Board of Pediatrics. Pediatric hospitalists: Training, current practice and career goals. J Hosp Med. 2009;4:179-186. 24. Maniscalco J, Ottolini MC, Dhepysasuwan N, Landrigan CP, Stuckey E. Current roles and training needs of pediatric hospitalists: A study from the Pediatric Research in the Inpatient Setting (PRIS) Network. EPAS2008:6725.4. Available at: Pas-meeting.org/2012/Boston/abstract_ archives.asp. 25. Fromme HB, Bhansali P, Singhal G, Yudkowsky R, Humphrey H, Harris I. The qualities and skills of exemplary pediatric hospitalist educators: A qualitative study. Acad Med. 2010;85(12):1905-1913. 26. Hicks PJ, Schumacher DJ, Benson BJ, Burke AE, Englander R, Guralnick S, Ludwig S, Carraccio C. The pediatrics milestones:

Conceptual framework, guiding principles, and approach to development. J Grad Med Educ. 2010;2(3):410-418. 27. Keller JM, Blatt B, Plack M, Gaba ND, Greenberg L. Using a commercially available web-based evaluation system to enhance residents’ teaching. J Grad Med Educ. 2012;4(1):64-67. 28. Pediatric Educational Excellence Across the Continuum (PEEAC) Conference. Available at: peeac.org/meeting/2011/Program_2011_ preliminary_v1.6FINAL.pdf. 29. Academic Pediatric Association’s Educational Scholar Program (ESP). Available at: ambpeds.org/education/educational_scholars_program_ description.cfm. 30. Harvard Macy Foundation. Available at: harvardmacy.org/programs/ overview.aspx. 31. Leslie K, Baker L, Egan-Lee E, Esdaile M, Reeves S. Advancing faculty development in medical education: A systematic review. Acad Med. 2013. 32. Sullivan PB, Buckle A, Nicky G, Atkinson SH. Peer observation of teaching as a faculty development tool. BMC Med Educ. 2012;12:26. 33. Blankenburg R, Black N, Maniscalco J, Fromme B, Augustine E, Myers J, et al. The new era of nighttime resident education: The 2011 ACGME work hour changes. Presented at Pediatric Academic Societies Meeting, Denver CO. May 2011. 34. International Network for Simulation-based Pediatric Innovation, Research, & Education. Available at: inspiresim.com. 35. Shaw T, Long A, Chopra S, Kerfoot BP. Impact on clinical behavior of face-to-face continuing medical education blended with online spaced education: A randomized controlled trial. J Contin Educ Health Prof. 2011;31(2):103-108. 36. Pierce R, Fox J. Vodcasts and active-learning exercises in a “flipped classroom” model of a renal pharmacotherapy module. Am J Pharm Educ. 2012;76(10):196. 37. Mittal VS, Sigrest T, Ottolini MC, Rauch D, Lin H, et al. Familycentered rounds: A new approach to patient care and teaching. Pediatrics. 2007;119:829-832

38. Singh H, Naik AD, Rao R, Petersen LA. Reducing diagnostic errors through effective communication: Harnessing the power of information technology. J Gen Intern Med. 2008;23(4):489-494. 39. Landrigan CP. Senior resident autonomy in a pediatric hospitalist system. Arch Pediatr Adolesc Med. 2003;157:206-207. 40. Med Ed Portal. Available at: mededportal.org. 41. Srivastava R, Nrolin C, Muret-Wagstaff S, Young PC, Auerbach A. community and hospital-based physicians’ attitudes regarding pediatric hospitalist systems. Pediatrics. 2005;115:34-38. 42. Freed GL, Brzoznowski K, Neighbors K, Lakhani I. Characteristics of the pediatric hospitalist worksource: Its roles and work environment. Pediatrics. 2007;120:33-39. 43. Landrigan CP, Srivastava R, Muret-Wagstaff S, Soumerai SB, et al. Impact of a health maintenance organization hospitalist system in academic pediatrics. Pediatrics. 2002;110:720-728. 44. Landrigan CP, Conway PH, Edwards S, Srivastava R. Pediatric hospitalists: A systematic review of the literature. Pediatrics. 2006;117:1736-1744. 45. Zaoutis T, Localio AR, Leckerman K, Saddlemire S, Bertoch D, Keren R. Prolonged intravenous therapy versus early transition to oral antimicrobial therapy for acute osteomyelitis in children. Pediatrics. 2009;123:636-642. 46. Landrigan CP, Conway PH, Stucky ER, Chiang VW, Ottolini MC. Variation in pediatric hospitalists’ use of proven and unproven therapies: A study from the Pediatric Research in Inpatient Settings (PRIS) network. J Hosp Med. 2008;3(4):292-298. 47. Ralston S, Garber M, Narang S, et al. Decreasing unnecessary utilization in acute bronchiolitis care: Results from the value in inpatient pediatrics network. J Hosp Med. 2013;8(1):25-30. 48. American Academy of Pediatrics. Quality Improvement Innovation Network. Elk Grove Village, IL: American Academy of Pediatrics. Available at: aap.org/qualityimprovement/quiin. 49. Pediatric Research in Inpatient Settings (PRIS) Research Network. PRIS Network. Available at: prisnet.org.

50. Srivastava R, Landrigan CP. Development of the Pediatric Research in Inpatient Settings (PRIS) Network: Lessons learned. J Hosp Med. 2012;7(8):661-664. 51. Zimbric G, Srivastava R. Research in pediatric hospital medicine: How research will impact clinical care. Curr Probl Pediatr Adolesc Health Care. 2012;42(5):127-130. 52. Starmer AJ, Spector ND, Srivastava R, Allen AD, Landrigan CP, Sectish TC. I-PASS Study Group. I-pass, a mnemonic to standardize verbal handoffs. Pediatrics. 2012;129(2):201-204. 53. Freed GL, Dunham KM; Research Advisory Committee of the American Board of Pediatrics. Characteristics of pediatric hospital medicine fellowships and training programs. J Hosp Med. 2009;4(3):157-163. 54. Heydarian C, Maniscalco J. Pediatric hospitalists in medical education: Current roles and future directions. Curr Probl Pediatr Adolesc Health Care. 2012;42(5):120-126. 55. Lee, T. Turning doctors into leaders. Harvard Bus Rev. 2010. 56. Stockwell DC, et al. Leadership and management training of pediatric intensivists: How do we gain our skills? Pediatr Crit Care Med. 2005. 57. Reid MB, Misky GJ, Harrison RA, et al. Mentorship, productivity, and promotion among academic hospitalists. J Gen Intern Med. 2011;27(1):23-27. 58. Pololi L, Knight S. Mentoring faculty in academic medicine: A new paradigm? J Gen Intern Med. 2005;20:866-870. 59. Johnson KS, Hasting SN, Purser PT, Whitson HE. The junior faculty laboratory: An innovative model of peer mentoring. Acad Med. 2011;86:1577-1582. 60. Varkey P, Jatoi A, Williams A, et al. The positive impact of a facilitated peer mentoring program on academic skills of women faculty. BMC Med Educ. 2012;12:14. 61. Bussey-Jones J, Bernstein L, Higgins S, et al. Repaving the road to academic success: The IMeRGE approach to peer mentoring. Acad Med. 2006;81:674-679. 62. Kowalski K, Casper C. The coaching process: An effective tool for professional development. Nurs Admin Q. 2007;31(2):171-179.

PART

Common Presenting Signs and Symptoms Part Editors Amanda Growdon, MD Katherine A O’Donnell, MD

16 Abdominal Mass 17 Abdominal Pain 18 Acidosis 19 Altered Mental Status 20 Chest Pain 21 Cyanosis 22 Diarrhea 23 Failure to Thrive 24 Fever 25 Gastrointestinal Bleeding 26 Hypertension 27 Hypoglycemia 28 Hypoxemia 29 Irritability and Intractable Crying 30 Limp 31 Lymphadenitis

II

32 Oral Lesions and Oral Health 33 Neck Pain 34 Petechiae and Purpura 35 Respiratory Distress 36 Shock 37 Syncope 38 Vomiting

Abdominal Mass

CHAPTER

16

Daniel A. Rauch

BACKGROUND Although evaluation of an abdominal mass often occurs in an outpatient setting, pediatric hospitalists are often asked to expedite the initial assessment and coordinate the appropriate consultations. The diagnostic possibilities vary considerably, based on the patient’s age and associated symptoms. The most urgent considerations are acute surgical conditions and neoplasms. A careful history and physical examination should guide a directed laboratory and imaging evaluation, leading to the diagnosis.

PATHOPHYSIOLOGY There are many structures within the abdomen from which masses can arise (Table 16-1). Abdominal masses can represent abnormal tissue mass of a solid organ such as the liver, spleen, or kidney or abnormal filling of a viscous organ such as the bowel or bladder. The most common source of an abdominal mass besides constipation is kidney pathology. TABLE 16-1

Region

Possible Diagnoses of Abdominal Masses Organ or Site Diagnosis

Epigastrium Stomach

Flank

Distended stomach from pyloric stenosis, duplication

Pancreas

Pseudocyst

Kidney

Hydronephrosis, Wilms tumor,

dysplastic kidney, ureteral duplication Adrenal

Neuroblastoma, ganglioneuroblastoma, ganglioneuroma

Retroperitoneum Neuroblastoma, ganglioneuroblastoma, ganglioneuroma, teratoma Lower abdomen

Pelvic

Ovary

Dermoid, teratoma, ovarian tumor, torsion of ovary

Kidney

Pelvic kidney

Urachus

Urachal cyst

Omentum, mesentery

Omental, mesenteric, peritoneal cysts

Bladder, prostate

Obstructed bladder, rhabdomyosarcoma

Uterus, vagina

Hydrometrocolpos, hydrocolpos, rhabdomyosarcoma

Right upper Biliary tract quadrant Liver

Intestine

Cholecystitis, choledochal cyst Hepatomegaly from congestion, hepatitis, or tumor; mesenchymal hamartoma; hemangioendothelioma; hepatoblastoma; hepatocellular carcinoma; hepatic abscess; hydatid cyst Intussusception, duplication

Left upper quadrant

Spleen

Splenomegaly from congestion, infectious mononucleosis, leukemic infiltration, or lymphoma; splenic abscess; cyst

Right lower quadrant

Appendix lleum

Appendiceal abscess

Left lower quadrant

Meconium ileus, inflammatory mass (complication of Crohn disease), intestinal duplication Lymphatics

Lymphoma, lymphangioma

Colon Lymphatics

Fecal impaction Lymphoma, lymphangioma

Source: From Zitelli B, Davis H. Atlas of Pediatric Physical Diagnosis. 4th ed. Philadelphia: Elsevier; 2002.

HISTORY Abdominal masses present in two distinct ways: painless or with abdominal symptoms. A painless mass is the classic sign of abdominal malignancy, particularly Wilms tumor and neuroblastoma. However, painless masses may also be completely benign, such as a fecal mass in a constipated child, a horseshoe kidney, or a wandering spleen. Painless masses are usually identified incidentally, often by parents when bathing the child, and sometimes on routine physical examinations. Systemic symptoms such as weight loss, pallor, bruising, or bleeding are suggestive of a malignant process. Diarrhea can be a sign of a vasoactive secreting tumor, while hematuria strongly suggests renal involvement. Masses may cause or be the result of GI obstruction and can present with vomiting, abdominal pain, and constipation. Masses that are renal in origin can present with symptoms of urinary dysfunction and hematuria. Painful masses require an evaluation for possible urgent intervention for ischemic diseases like ovarian torsion or intussusception or the need for decompression of the bowel or bladder. Duration of the symptoms is an important consideration. Slowly growing masses are typical of some malignancies, while rapidly enlarging masses are

more typical of others. Chronic abdominal pain and abnormal bowel habits may point to constipation. Periodic pain may point to a recurrent process, either acutely, as in intussusception, or chronically, such as a volvulus. Age is an important factor to consider. Neonates can present with congenital anomalies such as cystic kidneys. Neuroblastomas and storage diseases typically present younger than 2 years old. Wilms tumors and other malignancies are more common in older children. Certain syndromes are at increased risk of Wilms tumors, such as Beckwith-Wiedemann.

PHYSICAL EXAMINATION A thorough physical examination will help determine the source of the mass. Tenderness will confirm the report of pain, and rebound tenderness indicates a more severe and acute process that has evolved into peritonitis. Localization of the mass aids the differential diagnosis. The abdomen is classically divided in four quadrants. The right upper quadrant contains the liver, gallbladder, and bowel. The liver is often palpated under the costal margin, and up to 2 to 3 cm below in infants. The liver may be displaced inferiorly with hyperaeration of the chest cavity. When the liver edge extends to the left of the midline, it is likely enlarged. Extreme hepatomegaly that extends to the right lower quadrant can be missed because the liver edge is not palpable anywhere in the right upper quadrant. The left upper quadrant contains the spleen, pancreas, stomach, and bowel. The spleen is a mobile structure so that palpation of a mildly enlarged spleen tip may move it beyond the tactile sensation of the following examiner. Both the right lower quadrant and the left lower quadrant contain bowel and the female reproductive organs. Aside from the liver, these intraperitoneal structures are not typically palpable. In young infants the bladder may be palpable in the suprapubic area, but this structure becomes intrapelvic in later infancy. Retroperitoneal structures, such as the aorta and kidneys, are not normally palpable. Normal structures may be felt in patients who are very thin and have relaxed abdominal musculature, especially the aorta, which can be appreciated as a pulsatile midline structure, and the tip of the spleen in the left upper quadrant. The female reproductive tract resides in the pelvis but can expand into the lower abdomen under certain circumstances. Ovarian cysts or, much less

commonly, neoplasms, can become large enough to be palpable by direct abdominal examination. An engorged uterus may be palpable in the suprapubic area with congenital vaginal atresia, and is seen with hydrometrocolpos. A gravid uterus extends beyond the pelvic brim at approximately 12 weeks’ gestation. Ectopic pregnancy within a fallopian tube is usually symptomatic before being palpable but should always be considered in patients with abdominal pain and a positive pregnancy test. An intravaginal-abdominal bimanual examination is appropriate for better palpation of the female reproductive organs. The bladder, which is a pelvic structure after infancy, can expand into the lower abdomen when enlarged. This may occur with urinary retention. Retroperitoneal structures such as the kidneys, adrenals, pancreas, and midline paravertebral nodes and vessels can be palpable through the abdomen when there is significant tissue bulk, as may be seen with neoplasms such as Wilms tumor, neuroblastoma, and lymphoma. Cystic kidney disease may present as an abdominal mass. Neonates may have a palpable right kidney, which may be completely normal. Mobile masses more commonly originate from the GI tract, while fixed masses are from the liver or retroperitoneal structures. Solid versus compressible can differentiate between solid and cystic masses. The classic extra-abdominal finding of neuroblastoma is “raccoon eyes,” and proptosis can also be observed. Aniridia is associated with Wilms tumors, as is hemihypertrophy.

DIFFERENTIAL DIAGNOSIS Focal areas of distention can present as a mass, such as in duplication, intussusception, volvulus, and constipation. GI duplications are rare and may present with obstruction or bloody diarrhea in addition to a palpable mass. Intussusception uncommonly presents with the classic triad of colicky abdominal pain, vomiting, and currant jelly stool. More typical is some combination of vomiting, lethargy, and abdominal pain, with a right upper quadrant sausage-shaped mass and an empty right lower quadrant (Dance sign) in an infant or toddler. Volvulus also presents with vomiting and pain associated with an acute obstruction. Constipation is the most common cause of a palpable abdominal mass in any of the quadrants; it is usually tubular,

mobile, and nontender. Hepatomegaly is a nonspecific finding that may represent primary liver pathology or myriad secondary processes. Enlargement may be due to an increase in the size or number of cells or structures in the liver, including the hepatocytes (e.g. glycogen storage diseases), biliary tree (cholelithiasis), and vascular spaces (e.g. congestive heart failure), or to an infiltrative process (e.g. neuroblastoma). A list of possible infectious causes is provided in Table 16-2. Other causes to consider are listed in Table 16-3. TABLE 16-2

Infectious Causes of Hepatomegaly

Bacteremia Infectious hepatitis Mononucleosis Syphilis Leptospirosis Histoplasmosis Brucellosis Toxoplasmosis Tuberculosis Ascariasis Amebiasis Pyogenic abscess Visceral larva migrans Q fever Gonococcal or chlamydial perihepatitis AIDS Neonatal enteroviral infection Gianottic-Crosti syndrome Cat-scratch disease (granuloma) Source: Reproduced with permission from Green M ed. The abdomen. In Pediatric Diagnosis: Interpretation of Symptoms and Signs in Children and Adolescents. 6th ed. Philadelphia: Saunders; 1998:97-98. Copyright © Elsevier.

TABLE 16-3

Noninfectious Causes of Hepatomegaly

Anemia (sickle cell disease) Passive vascular congestion (congestive heart failure) Metabolic disorders (mucopolysaccharidosis) Cholestatic disorders (biliary atresia) Neoplastic disease (Hodgkin disease) Cysts (congenital hepatic cyst) Fatty infiltration (idiopathic steatohepatitis) Trauma (subcapsular hematoma) Autoimmune (systemic lupus erythematosus) Hemosiderosis Poisoning (hypervitaminosis A) The spleen can enlarge in response to a wide range of insults. Many of the infectious, metabolic, neoplastic, and inflammatory disorders that can cause hepatomegaly can also cause splenomegaly, either in isolation or in combination. Hemolytic disorders can cause chronic splenomegaly or, as in the case of acute splenic sequestration of sickle cell disease, sudden splenic enlargement with profound anemia. Serum sickness and subacute bacterial endocarditis can cause splenomegaly, with deposition of immune complexes in the spleen. Chronic granulomatous disease can cause intrasplenic granuloma formation. Suprapubic masses can represent the bladder or the uterus. Enlarged bladders are typically painful and associated with urinary symptoms. Pregnancy should be considered in any pubertal girl. The abdominal wall is a rare source of abdominal masses, taking the form of incarcerated hernias or sarcomas.

DIAGNOSTIC EVALUATION Blood work should be directed toward the likely diagnosis. Initial tests can include a CBC looking for signs of cell line deficits, liver function tests,

inflammatory markers, and oncology markers for neuroblastoma. Hematuria can be found with Wilms tumors, and urine pregnancy tests should be done in pubertal girls. Imaging studies can help localize the mass, determine the origin, or identify the cause. Plain radiographs (flat plates) can identify some abnormalities (e.g. solid tumors with calcifications) but are more useful as part of an obstruction series. The additional views demonstrate the effect of gravity on the intra-abdominal structures and fluids, revealing patterns of bowel obstruction, abscess, or free air. Ultrasonography is a readily available, nonionizing imaging modality that usually does not require the patient to be sedated. It provides information regarding the consistency of the mass (e.g. solid vs. cystic), can readily identify an abscess, and can often identify the organ of origin. Cystic masses are usually from the GI or genitourinary tract and are less likely to be malignant than solid masses. Computed tomography and magnetic resonance imaging provide a more complete assessment and greater structural detail. Factors such as need for sedation, radiation exposure, availability, and likely yield are important considerations. In addition, when the diagnosis is likely to be a malignancy and additional body areas will need to be imaged for staging purposes, studies should be coordinated to the extent possible. Further discussion of imaging is provided in Chapter 185.

MANAGEMENT Treatment is entirely dependent on the ultimate cause of the abdominal mass and often involves coordination of care with nephrology, gastroenterology, infectious diseases, oncology, or genetics as appropriate.

SPECIAL CONSIDERATIONS Any evaluation that includes possible malignancy or other potentially fatal entities such as the storage diseases requires extreme sensitivity toward the patient and family. Families need to be engaged in the need for a prompt workup which necessitated the hospitalization and they should be kept abreast of the results of each study in a timely manner. Additionally, the

caregivers will direct how much information can be revealed to the child. A multidisciplinary approach including social work and child life can help families cope with the process and break any potential bad news to the child and other family members. The evaluation may require special testing that is not available at all hospitals, so prompt transfer to a tertiary care facility may be required to expedite certain tests. KEY POINTS The diagnostic possibilities for abdominal masses vary considerably, based on the patient’s age and associated symptoms. While the evaluation can often take place in an outpatient setting, pediatric hospitalists are often involved in the initial evaluation. Ultimate diagnosis may depend on testing that is not available at all centers, so appropriate referral may be required.

SUGGESTED READINGS Brodeur AE, Brodeur GM. Abdominal masses in children: neuroblastoma, Wilms tumor, and other considerations. Pediatr Rev. 1991;12:196-207. Golden CB, Feusner JH: Malignant abdominal masses in children: quick guide to evaluation and diagnosis. Pediatr Clin North Am. 2002;49:13691392. Green M ed. The abdomen. In Pediatric Diagnosis: Interpretation of Symptoms and Signs in Children and Adolescents. 6th ed. Philadelphia, PA: Saunders; 1998:97-98. Pearl RH, Irish MS, Caty MG, Glick PL. The approach to common abdominal diagnoses in infants and children Part II. Pediatr Clin North Am. 1998;45:1287-1326. Zitelli B, Davis H. Atlas of Pediatric Physical Diagnosis. 4th ed. Philadelphia, PA: Elsevier; 2002.

Abdominal Pain

CHAPTER

17

Timothy Gibson

INTRODUCTION Abdominal pain is one of the most common complaints encountered by pediatric hospitalists. Despite this, the evaluation is often exceedingly difficult, and in many cases no definitive diagnosis can be made. The most urgent matter for the hospitalist initially is to rule out a life-threatening surgical emergency. If an acute abdomen is suspected based on history and physical, prompt surgical consultation is imperative.

BACKGROUND Abdominal pain can be caused by inflammation of the abdominal organs themselves (or their visceral peritoneum) or by inflammation of the parietal peritoneum lying in proximity to the underlying inflammation. Irritation of the abdominal wall musculature can also lead to pain, and various extraabdominal processes have been associated with abdominal pain (e.g. diabetic ketoacidosis or lower lobe pneumonia). Pain due to a process in the small intestine is usually felt in the midline around the umbilicus initially; as the inflammation progresses, the parietal peritoneum in the area of inflammation becomes involved, allowing better localization of pain. The classic example is appendicitis, with a dull periumbilical ache early in the course followed by a progressive shift of pain to the right lower quadrant as the inflammation evolves. Certain elements of the history and physical examination findings may help identify the cause of abdominal pain (Table 17-1). TABLE 17-1

Using History and Physical Examination Findings to Diagnose Abdominal Pain

Findings

Possible Diagnosis

History of present illness Sudden onset

Ovarian or testicular torsion, intussusception, volvulus, trauma

Bilious emesis

Bowel obstruction

Bloody stools

IBD (older patients), intussusception (younger patients), infectious colitis

Recent weight loss IBD, malignancy Travel

Infectious colitis, parasitic infection

Fevers

Infectious colitis, IBD

Rash

Henoch-Schönlein purpura, IBD (erythema nodosum)

Menstrual history

Dysmenorrhea, pregnancy, mittelschmerz

Past medical history Pharyngitis

Mesenteric adenitis, EBV-associated splenic distention

Gastroenteritis

Intussusception, postinfectious gastroparesis

Abdominal surgery Obstruction from adhesions Family history IBD

IBD

Migraines

Abdominal migraines

Social history Pets, especially reptiles

Infectious colitis

Sexual history

Pelvic inflammatory disease, pregnancy, ectopic pregnancy

Physical examination Clubbing or pallor

IBD

Perianal skin tags

Crohn disease

Jaundice

Biliary disease

Bluish color of flank or umbilicus

Pancreatic disease, trauma

Hemorrhoids, caput medusae

Chronic liver disease

EBV, Epstein-Barr virus; IBD, inflammatory bowel disease.

ASSESSMENT The differential diagnosis of abdominal pain is extensive (Table 17-2). In addition to the typical history and physical examination, epidemiologic factors are extremely helpful in narrowing the differential diagnosis, including age, gender, season, locale, and the like. As stated above, a patient with peritonitis requires timely surgical evaluation. With several conspicuous exceptions, most patients with signs of peritonitis on examination progress to that point gradually. On history, these patients, if old enough, may report that the bumps in the road on the trip to the emergency department caused pain. These patients prefer to lie motionless and do not want their abdomens palpated. The examiner can check for signs of peritonitis immediately by “inadvertently” bumping the bed; a wince of pain from the patient is a sign that any movement at all is irritating to the inflamed parietal peritoneum. Bowel sounds may be hypoactive or absent at this late stage, reflecting an ileus. The abdomen may be rigid, and the patient may have voluntary or involuntary guarding, a mechanism to prevent painful manipulation of the underlying enflamed peritoneum. Patients with peritonitis are likely to have rebound tenderness; where the pain that is felt when the examiner releases the abdomen is greater than that caused by the palpation itself. Patients of any

age who fit this classic description of an acute abdomen are highly likely to require surgical intervention and rarely need further evaluation such as laboratory tests or radiographic studies, except possibly to assess their surgical risk. TABLE 17-2

Differential Diagnosis of Abdominal Pain

Gastrointestinal Viral gastroenteritis Bacterial infectious colitis Parasitic infection Constipation Colic Functional abdominal pain Abdominal migraine Acute cholecystitis, cholelithiasis Ulcer disease (gastric, peptic) Pancreatitis Hepatitis Inflammatory bowel disease Gastroesophageal reflux Esophagitis Gastroparesis, ileus Lactose intolerance Toxic megacolon Meckel diverticulum Hirschsprung disease Urologic Urinary tract infection Urolithiasis Testicular torsion Gynecologic

Ovarian torsion, cyst Pregnancy, ectopic pregnancy Pelvic inflammatory disease Dysmenorrhea Endometriosis Hematocolpos Ovarian neoplasm Rheumatologic Henoch-Schönlein purpura Familial Mediterranean fever Surgical Appendicitis Intussusception Malrotation with midgut volvulus Incarcerated inguinal hernia Trauma with associated hematoma Postsurgical obstruction Psoas (or other) abscess Toxicologic Heavy metal poisoning Food (toxin-mediated) poisoning Infectious Tuberculosis Zoster Mesenteric adenitis Mononucleosis Sepsis Pulmonary Lower lobe pneumonia Meconium ileus equivalent Endocrinologic

Diabetic ketoacidosis Hematologic Vaso-occlusive crisis Porphyria Oncologic Tumor (e.g. Wilms) Renal Nephrosis Spontaneous bacterial peritonitis Hemolytic uremic syndrome Cardiovascular Abdominal aortic aneurysm Psychiatric Somatoform or conversion disorder Unfortunately for physicians, most cases of abdominal pain are not this clear cut. For each disease process in the differential diagnosis, there is a wide range of severity. For example, a patient with severe constipation may be in extremis, whereas a patient with early appendicitis may have so little pain (or such a high tolerance for pain) that he or she is sent home without having had further workup. A full history and physical examination are paramount to determine the cause of the pain. It must be kept in mind that common benign diagnoses (e.g. viral gastroenteritis, constipation) occur commonly, but depending on the presentation are often diagnoses of exclusion. It is the examiner’s job to triage these patients appropriately while recognizing the red flags that point to less common entities or those that need more urgent evaluation or intervention.

HISTORY The timing, location, and progression of the pain should be elicited, as well as its quality and severity. Did the pain begin in the umbilical area and progress to the right lower quadrant, as occurs with appendicitis? Is the pain

burning or boring, as in ulcer disease, or sharp and stabbing, as in peritonitis? Does it come in waves, suggesting a blockage of peristalsis in a hollow viscus, such as the small bowel, biliary tree, or ureter? Is the pain relieved by meals, as in some peptic ulcer diseases, or is the pain exacerbated by oral intake, as in biliary disease and pancreatitis? Is there vomiting before the pain, as in viral gastroenteritis, or after the pain, as in appendicitis? The pain in patients subsequently found to have appendicitis can be variable. If the appendix ruptures, the patient’s pain will often improve briefly, but will return in a gradual localized fashion, often accompanied by fever, as a phlegmon organizes at the site of rupture in the right lower quadrant. If the patient has bilious vomiting, the patient has a surgical emergency until proven otherwise, as this is suggestive of a bowel obstruction distal to the sphincter of Oddi. Younger children, classically 18–36 months, who have abdominal pain and bilious vomiting without other symptoms have intussusception until proven otherwise. The pain of intussusception can be intermittent and is occasionally associated with mental status changes or lethargy. Any toxic-appearing child at any age with bilious vomiting should be evaluated for malrotation with midgut volvulus. In a verbal child, questions about urinary complaints should be included, such as dysuria, frequency, or discolored urine, suggesting a urinary tract infection, nephrolithiasis, or the nephritis of Henoch-Schönlein purpura. What is the patient’s baseline stooling pattern? Stools that are hard or extremely large suggest constipation, perhaps the most common cause for severe (but generally not dangerous) abdominal pain. If there is diarrhea, is it bloody, as in inflammatory bowel disease (IBD) or a bacterial infection? Are the stools melanotic, as in a bleeding duodenal ulcer? Are there red flags—weight loss, fatigue, night sweats—for a prolonged systemic disease such as Crohn disease? Are there extraintestinal manifestations that are suspicious for IBD, such as rash, arthralgias or arthritis, aphthous stomatitis, or eye problems? Is there a family history of gastrointestinal disorders, especially heritable disorders such as IBD? Is the patient on any medications that could predispose to abdominal pain, especially nonsteroidial anti-inflammatory drugs (suspected ulcer disease) or recent antibiotics (possible Clostridium difficile infection)? Is there anything relevant in the social history, such as recent sexual activity predisposing to a sexually transmitted disease or pelvic inflammatory disease, or difficulties at school or at home causing stress, suggesting a psychosomatic illness?

PHYSICAL EXAMINATION The physical examination starts with an evaluation of the vital signs. True abdominal catastrophes with associated abdominal pain (volvulus, perforated duodenal ulcer, splenic rupture following Epstein-Barr virus [EBV] infection, severe trauma) quickly progress to frank shock, with accompanying changes in hemodynamics and vital signs. Fever is often found with inflammatory and infectious processes. The degree of fever is not necessarily directly related to the severity of the eventual diagnosis. Fever of 104°F to 105°F is often seen with viral gastroenteritis, whereas the fever of appendicitis starts much lower and spikes to 104°F to 105°F only if perforation occurs. The importance of the patient’s general examination on presentation cannot be overstated. A toxic, shocky child needs emergent evaluation and intervention after ensuring that circulation, airway, and breathing are maintained, whereas a patient with normal vital signs who smiles and jokes while complaining of 10 out of 10 abdominal pain likely does not warrant admission. Examination of the acute abdomen was addressed earlier, with an emphasis on recognizing the signs of peritonitis. After inspection of the abdomen (for distension, discoloration, visible masses, etc.) bowel sounds should be assessed, because deep palpation can alter their character. Are the bowel sounds present? Are they high pitched, as in a bowel obstruction? Percussion of the abdomen is invaluable. Is the abdomen distended and tympanitic, potentially suggestive of increased intraluminal air proximal to an obstruction? Is there a fixed area of dullness, signifying organomegaly or a mass (malignant or otherwise, including a firm stool which may present with a left lower quadrant mass)? Is there a shifting dullness or a fluid wave, suspicious for ascites? Palpation is also important in evaluating for masses and organomegaly and for eliciting pain, and should be performed after auscultation and percussion. One should always begin palpating as far away from the reported location of the patient’s pain as possible. Having the child flex the hips and bend the knees is helpful to relax the abdominal musculature. The importance of distraction during palpation, especially for younger patient, cannot be overstated. The practitioner’s eyes should be fixed on the patient’s face, looking for even subtle signs of tenderness. The obturator sign and the psoas sign are suggestive of inflammation in or near those muscles, often secondary to organic abdominal pathology (described in Chapter 143). The Rovsing sign is present when palpation in the left lower

quadrant produces pain in the right lower quadrant, suggesting appendicitis. Rebound is thought to be sensitive for peritonitis, although many examiners feel that this is an unnecessarily painful maneuver that often adds little to the final evaluation. All children should have a full examination in addition to the abdominal examination, with an emphasis on findings that could be causing or contributing to the abdominal pain. Controversy exists over which children need rectal examinations and which females need pelvic examinations. In selected cases, either of these examinations can be immensely informative, and the need for them is dictated by the history.

EVALUATION As stated earlier, a patient with an obvious surgical abdomen often needs no further evaluation before surgical exploration is undertaken. For other patients, the question is whether to perform radiologic studies and/or laboratory tests.

RADIOLOGY The simplest and most widely available initial study is a supine plain radiograph of the abdomen (also known as a kidney, ureter, and bladder [KUB] study). Most findings are nonspecific and merely confirm the findings elicited by physical examination. Exceptions include a renal or ureteral calculus, the finding of a fecalith in the right lower quadrant, or a large amount of stool suggesting constipation. A fecalith is a small, radiointense mass of hardened stool that obstructs the lumen of the appendix, preventing the outflow of intestinal bacteria and predisposing the patient to appendicitis. Many surgeons will operate on a patient with an equivocal history based on a radiographic finding of a fecalith. Distended loops of bowel are common and nonspecific, as is scoliosis toward the side of pain. The addition of a second abdominal view in the upright or left lateral decubitus allows for effect of gravity on air and fluid to be examined. Air–fluid levels may be visible within bowel secondary to ileus or obstruction. Free air may be visible on the upright film, but these patients often have peritoneal signs on examination. Patients subsequently diagnosed with intussusception often have a paucity of air in the ascending colon, as this is the most common site for an

intussusception to occur. The radiologic evaluation of patients with suspected appendicitis has changed over the years. Many centers now use the Pediatric Appendicitis Score to determine which patients need further workup such as blood work or imaging, and which can be observed (see Chapter 155). Ultrasonography is a preferred initial modality, owing to the lack of radiation and its specificity for appendicitis. An added advantage of ultrasonography is the ability to assess blood flow in real time, which is particularly helpful in female patients with right lower quadrant pain, for whom ovarian torsion is in the differential diagnosis. Ultrasound can also be used in the evaluation of suspected intussusception to identify a “donut” (the intussusceptum within the lumen of the intussuscipiens). Air-contrast enema has the advantage of being able to both diagnose and treat intussusception, but because there is a small risk of perforation, this procedure should be performed only in centers staffed by surgeons competent in the care of small children. Computed tomography, especially when done with effective rectal or oral contrast, has high specificity for appendicitis, and is often undertaken if an initial ultrasound is equivocal. The radiologist is able to see signs of inflammation in the right lower quadrant, including free fluid and stranding in the pericecal fat. With effective contrast, an appendix that does not fill is presumed to be obstructed, confirming the diagnosis of appendicitis. The study is quite operator and hospital dependent, however. Other imaging studies that may be encountered in the evaluation of abdominal pain include barium swallows (effective for the evaluation of malrotation with intermittent volvulus and for IBD) and, rarely, nuclear medicine studies for gastrointestinal bleeding with associated abdominal pain. Magnetic resonance enterography is a new modality that is effective at finding and monitoring the small bowel inflammation in patients with IBD.

LABORATORY STUDIES As with radiologic studies, the history and physical examination guide the practitioner regarding laboratory studies. The result of all laboratory testing needs to be taken in context and cannot supercede physical findings or discount the importance of serial physical examinations. In patients with abdominal pain, a complete blood count (CBC) with

differential is helpful in the evaluation for inflammation, infection, or anemia. In general, an elevated high white blood cell (WBC) count with a neutrophilic predominance is more suspicious for an acute infectious or inflammatory cause of the patient’s pain, including appendicitis. However, a normal WBC count does not rule out appendicitis, and an elevated WBC count does not confirm it. Anemia, if observed, can be very helpful in pointing the practitioner toward a chronic disease such as IBD, especially if accompanied by a relative reticulocytopenia. A low mean corpuscular volume (MCV) suggests iron deficiency, perhaps suggestive of chronic blood loss. Sudden, acute blood loss may present with evidence of hemodynamic compromise before there is a fall in the hemoglobin level on an initial CBC. A basic chemistry panel with serum electrolytes, glucose, creatinine, and blood can help evaluate hydration status, acid–base balance, and electrolyte derangements, especially if vomiting, diarrhea, or inadequate oral intake is part of the clinical picture. These findings are typically less helpful in identifying the etiology of abdominal pain but are often needed for appropriate management. Evaluation of serum hepatic enzymes and bilirubin levels, often referred to as liver function tests, are useful for patients with right upper quadrant pain or tenderness, or with jaundice. These tests usually include alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase, γ-glutamyl transferase (GGT), and levels of both the conjugated and unconjugated fractions of serum bilirubin (see Chapter 79 for further discussion of these tests). Concern for mononucleosis should prompt, testing for EBV with either a heterophil antibody test (“monospot”) for children over 4 years of age or EBV serology. Elevations of pancreatic enzymes, amylase, and lipase are quite specific for pancreatitis, and these studies would be warranted in patients with epigastric pain or tenderness or prominent vomiting (see Chapter 82). Markers of inflammation, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are usually elevated with chronic diseases like IBD, but again they are nonspecific and therefore can be elevated in many infectious, and inflammatory or malignant conditions. Newer serologic tests for IBD may be helpful in patients with a suggestive clinical picture, but definitive diagnosis still requires endoscopy with confirmatory tissue biopsy (see Chapter 80).

Urinalysis can provide information regarding hydration status (specific gravity) as well as indications of genitourinary involvement (e.g. red blood cells secondary to ureteral calculus or Henoch Schönlein purpura) or infection (e.g. nitrates and white blood cells secondary to pyelonephritis). Patients with appendicitis may have sterile pyuria if the inflamed appendix settles on the wall of the bladder. All stools for any child with abdominal pain should be tested for blood, and if there is concern for infectious gastroenteritis or colitis, appropriate samples should be sent to evaluate for the bacteria, toxins, parasites, or viruses of concern. Stool antigen for Helicobacter pylori can be sent if gastritis due to this entity is suspected (see Chapter 77).

TREATMENT The treatment of abdominal pain depends on what is discovered during the workup. Patients thought to have surgical pathology are given nothing by mouth and often need resuscitation with intravenous fluids before definitive surgical intervention. Particular attention should be paid to pain control, which often requires parenteral opioids. Surgery is undertaken when the patient is stable enough for anesthesia, or occasionally before this point if the patient is toxic and deteriorating. If bowel perforation is suspected, the patient is started on broad-spectrum antibiotics to cover enteric pathogens, including anaerobes. Patients who are admitted without a definitive diagnosis require close monitoring, and the importance of serial examinations cannot be overstated. Recent literature suggests that serial examinations by pediatric surgeons are as effective as radiologic evaluation in patients with suspected early appendicitis. Diligent monitoring of fluid intake and output, along with vital signs, can provide an early clue that a disease process is progressing. If there are stigmata of systemic gastrointestinal disease, consultation with a pediatric gastroenterologist, with an eye toward possible endoscopy, is appropriate. A number of treatments are available for constipation, and a child with viral gastroenteritis needs fluid resuscitation and tincture of time. As stated earlier, many patients who present with abdominal pain never receive a definitive diagnosis. When a symptomatic patient has been fully evaluated and organic disease has been ruled out, the goal of treatment is to control the patient’s symptoms and allow a return to the activities of daily living. A child must be able to tolerate enough by mouth to stay hydrated and

prevent weight loss. A school-age child must be able to attend school on most days. Above all, a patient with pain and a negative workup needs close follow-up, sometimes with a specialist, but always with the primary care physician. Literature increasingly suggests that patients with depression or anxiety may present with abdominal pain, and somatization disorder is a frequent cause of otherwise unexplained abdominal pain in the hospitalized patient. This clearly is a diagnosis of exclusion. In this case, a psychiatrist may need to be involved, both for the evaluation of nonorganic pain and for the teaching of coping strategies. For patients discharged to home, it should be stressed to parents that the workup is ongoing and will continue in the outpatient setting.

SUGGESTED READINGS Ashcraft KW. Consultation with the specialist: acute abdominal pain. Pediatr Rev. 2000;21:363-367. Boyle JT. Abdominal pain. In: Walker WA, Durie P, Hamilton J, et al., eds. Pediatric Gastrointestinal Disease. Hamilton, Ontario, BC: Dekker; 2000:129-149. Kliegman R. Acute and chronic abdominal pain. In: Kliegman R, ed. Practical Strategies in Pediatric Diagnosis and Therapy. Philadelphia, PA: WB Saunders; 2004:249-271. Kosloske AM, Love CL, Rohrer JE, et al. The diagnosis of appendicitis in children: outcomes of a strategy based on pediatric surgical evaluation. Pediatrics. 2004;113:29-34. Saito JM. Beyond appendicitis, evaluation and surgical treatment of pediatric acute abdominal pain. Curr Opin Pediatr. 2012;24:357-364.

CHAPTER

Acidosis

18

Chén Kenyon

BACKGROUND Acidosis is a common finding in many childhood disease states, ranging from self-resolving diarrheal illnesses to progressively degenerative mitochondrial disorders to acute septicemia and shock. Given the broad range of underlying illnesses and their disparate clinical consequences, identifying the specific etiology of acidosis is often crucial to a child’s hospital care. In circumstances where there is relative diagnostic certainty, appropriate identification and characterization of acidosis can contribute important clinical evidence for or against a prevailing diagnostic theory or management strategy. For patients whose illnesses have not been fully characterized, a rational approach to the interpretation of acidosis can help direct more specific diagnostic testing and further clinical evaluation. In this chapter, we discuss the pathophysiology, differential diagnoses, and workup strategies for acidosis, focusing primarily on metabolic acidosis. We include a section on respiratory acidosis in Special Considerations.

PATHOPHYSIOLOGY Acidosis is a metabolic state characterized by a relative excess of hydrogen ions (H+). This relative excess can result from a number of processes: (1) an increase in H+ generation, (2) a decrease in H+ elimination via the kidneys, (3) a decrease in basic buffer molecules, or (4) a combination of the three. These processes reflect imbalances in the various counter-regulatory systems involved with acid–base homeostasis. A growing child generates a net of 1–3 mEq/kg/day of acid through normal metabolic processes. To maintain the optimal conditions for

enzymatic activity (a pH between 7.35 and 7.45), the body must efficiently eliminate this and any additional acid load. Three primary mechanisms are responsible for maintaining this balance: the blood buffer system, respiratory ventilation, and renal excretion. Immediately upon exposure to excess acid, circulating buffers, including bicarbonate (HCO3−), bind excess H+, diminishing its concentration in the extracellular space. The excess H+ that binds to bicarbonate forms the “volatile” carbonic acid, which can then dissociate into carbon dioxide (CO2) and water. Assuming sufficient H+ excess and effective pulmonary ventilation, excess CO2 is then removed from the circulation through the pulmonary capillaries and alveoli. In response to persistently diminished pH, peripheral chemoreceptors and central respiratory centers increase minute ventilation to augment this process. Lastly, the proximal tubules in the kidney respond to excess H+ by increasing bicarbonate reabsorption and ammonia synthesis, the latter of which assists with renal hydrogen ion elimination in the form of ammonium ions (NH4+). Acidemia, defined as an arterial pH of less than 7.35, occurs when the body’s numerous compensatory mechanisms to address an acid load are overwhelmed or compromised. The compensatory mechanisms can be overwhelmed in the setting of excess “non-volatile” acid production, as occurs acutely in lactic acidosis, diabetic ketoacidosis (DKA), or more indolently in the organic acidopathies. The body’s buffer system can be compromised when there is a reduction in circulating bicarbonate from gastrointestinal losses (e.g. prolonged diarrheal illness) or renal losses (e.g. proximal renal tubular acidosis). Compromised ventilation from a number of etiologies diminishes the body’s ability to readily eliminate volatile acid in the form of CO2, leading to retained H+. Defects in the kidney’s ability to appropriately acidify urine, as occurs with renal tubular acidosis (RTA), comprise the last set of conditions that lead to imbalances of this intricate homeostatic system. DEFINITIONS Acidosis: A state of relative excess of hydrogen ions. Acidemia: Arterial pH less than 7.35.

Metabolic Acidosis: An acute or chronic state resulting from excess non-volatile H+ production, decreased H+ elimination via the kidneys, or diminished buffering capacity. Respiratory Acidosis: An acute or chronic state of excess H+ resulting from inadequate removal of volatile acid in the form of CO2 via the lungs.

PATIENT HISTORY Generally, the symptoms of acidosis are non-specific. History should be targeted toward identifying the source of the acidosis. Common features of metabolic acidosis are vomiting and tachypnea. Other symptoms may include fatigue, lethargy, seizures, and alterations in mental status. Children with diarrheal illness may have acidosis but this is not a symptom of the acidosis, rather the precipitant. Analogously, children with respiratory acidosis may present with decreased respiratory rate or respiratory failure, also conditions which led to the acidosis. History of ingestion is particularly important, as is history of medical conditions that may predispose the patient to acidosis. Among others, these include diabetes mellitus, prior diagnosis of metabolic disorder, seizure disorder on a ketogenic diet, and systemic lupus erythematosus (which can be associated with a distal RTA). A number of medications are also associated with acidosis, including acetazolamide and other medications that can inhibit carbonic anhydrase (i.e. some anti-epileptic medications) and amphotericin B or amiloride, that may mimic RTAs.

PHYSICAL EXAMINATION Physical examination findings for children with acidosis are similarly nonspecific. Signs of hypovolemia (tachycardia, hypotension, delayed capillary refill, etc.) and tachypnea are often present in children with a diarrheal cause of excess bicarbonate loss or in those with lactic acidosis or DKA. Children presenting with DKA may present with fruity smelling breath from increased acetone ventilation. Patients with severe acidosis may present with alterations in level of consciousness or obtundation.

DIFFERENTIAL DIAGNOSIS The differential diagnosis for acidosis is broad. As with other clinical scenarios, a basic understanding of epidemiology helps to generate an ordered differential diagnosis by assessing the prior probability of a particular cause with respect to the individual characteristics, symptoms, and signs of the child before you. Etiologies such as diarrheal illness and lactic acidosis are most probable from the general population perspective. However, certain groups require special consideration, including toddlers, where ingestions are particularly common, and newborns, where undiagnosed inherited disorders of metabolism are more prevalent. While Bayesian logic helps to order the possible diagnostic options, a number of well-established and relatively formulaic strategies to characterize a child’s acidosis are valuable in guiding further evaluation. One such strategy is detailed in Figure 18-1.

FIGURE 18-1. Step-wise approach to evaluation of metabolic acidosis. The initial step in categorization is to determine whether acidemia (pH 14 mEq/L provides the clinician with evidence of both the presence and quantity of unmeasured anions, although gaps of up to 16 mEq/L may be normal in newborns.1 These unmeasured anions are generated through the buffering action of HCO3− in the presence of excess acid, as depicted in the following equilibrium: H+anion− + NaHCO3 ⇔H2O + CO2 + Na+anion− In this equilibrium, H+anion− represents the excess acid, such as keto-

acids, lactic acid, and organic acids. Through this reaction, HCO3− is metabolized, and the anion salt of the excess acid remains and serves to maintain serum electrochemical neutrality. When HCO3− is metabolized at a greater rate than the acid salt is eliminated and new HCO3− generated, an unmeasured anion will be detectable when calculating the anion gap. Etiologies of anion gap and non–anion gap acidosis are presented in Table 18-1, and the diagnostic evaluation of these categories is discussed in the next section. Of note, the calculated anion gap may be inappropriately low in the presence of unmeasured cations, as with lithium toxicity and hypermagnesemia, or in the setting of hypoalbuminemia, which is a potent contributor to the normal anion gap. In the setting of low albumin, an adjusted AG can be calculated using the following formula: TABLE 18-1

Etiologies of Anion Gap and Non–Anion Gap Metabolic Acidosis

Anion Gap Acidosis Ketoacidosis Diabetic ketoacidosis Alcoholic ketoacidosis Starvation Lactic acidosis Tissue hypoxia Shock Dehydration Hypoxia Carbon monoxide poisoning Increased production or decreased clearance of lactate Leukemia, lymphoma, large tumors Liver or renal failure Renal failure (especially if GFR 6 suggests the presence of a mixed disorder, such as a concomitant respiratory alkalosis in salicylate toxicity. Exceptions to this rule include lactic acidosis, where the increase in the anion gap can be as much as 1.6–1.8 times the decrease in serum bicarbonate, and diabetic ketoacidosis, where it is often less than 1:1, as ketoacids are lost in the urine.

DIAGNOSTIC EVALUATION The most important initial labs in the evaluation of metabolic acidosis are a basic metabolic panel and an arterial, venous, or capillary blood gas. While the latter three have been demonstrated to be highly correlated on all parameters except pO2 in the ICU setting,2 an appropriately collected and processed sample is critical in making appropriate inductions regarding the nature of the acidosis. Using a systematic approach to interpretation of these

simple tests, such as the step-wise method described above, the clinician can determine whether further diagnostic evaluation is necessary, and if so, engage in a rational and targeted approach. Depending upon the acuity of the child’s illness and the suspected causes, this evaluation may be relatively extensive. At this point, involvement of the appropriate specialist, if available, may be warranted.

EVALUATION OF ANION GAP ACIDOSIS As described above, an anion gap of >14 usually suggests the presence of unmeasured anions and may warrant further investigation. An anion gap of 20 or greater is more than 4 standard deviations above the mean and in this scenario, a metabolic acidosis is likely to be present, regardless of whether the pH or serum HCO3− are within the normal range. In those with an anion gap greater than 20, a specific cause for the metabolic acidosis will likely be discerned from the usual screening tests2 (Table 18-2). TABLE 18-2

Initial Laboratory Evaluation for Metabolic Acidosis

Standard Arterial blood gas Electrolytes (Na+, K+, Cl−, HCO3−) Blood urea nitrogen, creatinine Glucose Urine pH Consider urine electrolytes Based on clinical suspicion Serum toxicology screen or measurement of specific drug if suspected Complete blood count Bacterial cultures Hepatic enzymes and albumin determinations Prothrombin time

Screening tests for suspected metabolic disorder (see Table 18-3) Electrocardiogram TABLE 18-3

Laboratory Studies for Suspected Inborn Error of Metabolism

Blood Complete blood count with differential Blood gas Serum electrolytes Glucose Ammonia, lactate, and pyruvate Amino acid quantification Acylcarnitine profile Free and total carnitine Urine Urinalysis, including ketones Organic acids Acylglycines Depending on clinical suspicions as to the cause of the acidosis, a number of studies should be considered. A complete blood count may be abnormal in the setting of lactic acidosis from distributive shock secondary to infection or systemic illness. Elevated liver enzymes and abnormal function test results might indicate hepatic failure/dysfunction. In an infant or toddler with a history of poor growth or delayed development, recurrent vomiting or lethargy, or acidosis or hypoglycemia with illness, a metabolic disorder should be considered, and appropriate screening studies should be performed in consultation with a metabolism specialist (Table 18-3). An electrocardiogram should be done if there is evidence of severe acidosis or other electrolyte abnormalities such as hyperkalemia. Serum or urine toxicology screening can be obtained if there is concern for ingestion or if interpretation of the initial labs reveals a second metabolic process.

In the setting of an elevated anion gap and concern for ingestion, calculating the osmolal gap can be useful. Serum osmolality is calculated according to the following equation: osmolalitycalc (mOsm/kg) = 2 × Na+ (mEq/L) + blood urea nitrogen (mg/dL)/2.8 + glucose (mg/dL)/18 The osmolal gap (the difference between measured osmolality and calculated osmolality) is normally 10–15 mOsm/kg. If the measured osmolality exceeds the calculated osmolality by more than 15–20 mOsm/kg, this indicates an accumulation of osmolytes other than the sodium salts, urea, and glucose, as seen with ingestion of ethylene glycol or methanol.

EVALUATION OF NON–ANION GAP ACIDOSIS The urinary anion gap (UAG) can be useful in differentiating between two common causes of non–anion gap acidosis: gastrointestinal losses and renal tubular acidosis (RTA). The UAG is calculated by obtaining spot measurements of urinary Na+, potassium (K+), and Cl− (all units mEq/L): UAG = (Na+ + K+)urine − (Cl−)urine The UAG is an indirect measure of urinary ammonium (NH4+), the precursor of which (ammonia = NH3) is generated in the proximal tubules in the setting of acidosis. NH4+ is assumed to be present if the sum of the major urinary cations (Na+ + K+) is less than the major anion (Cl−); because electrochemical neutrality must exist in the urine, some cation must be balancing the apparent surplus in measured Cl−. In a metabolic acidosis from chronic diarrhea, the functioning kidneys increase proximal tubule NH3 synthesis, thus providing more urinary buffer. In RTA, the urinary NH4+ is low because of the kidney’s intrinsic inability to compensate for acid loads. Thus, a negative UAG usually suggests an extra-renal cause of non–anion gap acidosis (but can be negative with a proximal RTA), whereas a positive

UAG suggests a renal cause (specifically distal RTA) of non–anion gap acidosis. The UAG should be interpreted with caution in the setting of hypovolemia, as adequate distal delivery of Na+ is necessary for appropriate distal acidification, and when urinary pH >6.5, as HCO3− is likely present in the urine. With specific regard to RTA, the acidosis in a distal RTA is usually more profound than with proximal RTA. A urine pH >5.5 in the setting of acidosis may indicate impaired acidification of the urine, pointing toward a distal RTA. The urine pH in proximal RTA is variable, depending on the threshold of bicarbonate reabsorption. In general, children with proximal RTA can appropriately acidify the urine to 5.3 if above the child’s bicarbonate reabsorptive threshold. If proximal RTA is suspected based on above evaluation, other features of proximal tubular dysfunction (Fanconi syndrome) may be present, including glucosuria, hypokalemia, hypophosphatemia, generalized aminoacioduria, and so forth.

MANAGEMENT Management of metabolic acidosis is directed to treatment of the underlying cause. In general, treating the causes of anion gap acidosis can regenerate bicarbonate within hours; however, non–anion gap acidosis can take days to resolve and may require exogenous bicarbonate therapy. Insulin, hydration, and electrolyte repletion will correct the acidosis in diabetic ketoacidosis. Lactic acidosis will resolve with increasing tissue oxygenation using crystalloid, blood products, inotropic agents, and oxygen. Renal failure may require dialysis. Ethylene glycol and methanol ingestion can be treated with fomepizole, ethanol, and hemodialysis. Management of salicylate ingestion may include repeated doses of activated charcoal, alkalization of urine to optimize excretion, correction of metabolic derangements such as hypoglycemia and hypokalemia, and hemodialysis for severe ingestion. For acidosis secondary to metabolic disorders, the basic principle is to reverse counter-regulation and tissue catabolism with appropriate energy substrate (see section Inborn Errors of Metabolism under Special Considerations for details). Diarrhea can be managed with fluid and electrolyte repletion. Bicarbonate supplementation and management of calcium and potassium

homeostasis are effective in managing RTA, regardless of type. The use of sodium bicarbonate in the treatment of severe metabolic acidosis is controversial. Once considered a mainstay of acute therapy for severe acute metabolic acidosis, studies have failed to demonstrate benefit of sodium bicarbonate in a number of scenarios, including severe diabetic ketoacidosis,3 neonatal resuscitation,4 and preterm metabolic acidosis.5 This has led some to question whether it has any role in acute metabolic acidosis therapy,6 especially given the list of potential side effects. Most notably, in the setting of suboptimal ventilation, bicarbonate infusion can lead to increase intracellular [pCO2], as HCO3− undergoes rapid conversion to carbonic acid and dissociation to H2O and CO2, which will diffuse into the tissue without adequate elimination via ventilation. Despite accumulating evidence for lack of clear benefit, sodium bicarbonate is frequently used in the setting of severe acidosis (pH 10 g/kg/day in children younger than 3 years and >200 g/day in children older than 3. Diarrheal episodes are typically classified into acute and chronic, based on duration. Acute diarrhea is defined as an episode of diarrhea that is acute in onset and lasts less than 14 days. Chronic diarrhea is defined as an episode that lasts equal to or longer than 14 days. This distinction has implications for etiology, management, and prognosis.

PATHOPHYSIOLOGY In the normally functioning gastrointestinal tract, nutrients are absorbed via

active, carrier-mediated transport across the intact mucosal lining. Electrolytes such as sodium, potassium, chloride, and bicarbonate are transported via both active and passive mechanisms, with sodium transport creating the most significant gradient. This sodium gradient is responsible for promoting the passive transport of water across the mucosal lining. Under normal circumstances, there is a balance between the absorptive and secretory functions, which are the opposing unidirectional electrolyte fluxes. This results in a net water absorption, with 90% of the absorption occurring in the small intestine. Altered pathophysiologic mechanisms resulting in diarrhea can be divided into four categories: osmotic, secretory, inflammatory, and motility disorders. Osmotic diarrhea occurs when excessive amounts of solutes are retained in the lumen of the intestine, resulting in decreased water absorption. Osmotic diarrhea will typically cease with fasting or withholding of the poorly absorbed solute. Secretory diarrhea occurs when secretion of water into the lumen of the intestine exceeds absorption. In most cases, the diarrhea is not affected by alterations in enteral intake. Inflammation can cause diarrhea when there is mucosal damage resulting in decreased water absorption and increased fluid secretion. Inflammatory diarrhea may be associated with mucus, blood, and protein losses in the stool. Lastly, motility disorders cause either increased or decreased transit time in the intestine, leading to altered water absorption. A decreased transit time due to increased motility results in diarrhea.

PATIENT HISTORY The frequency, duration, volume, and character of the diarrhea (including smell, color, and presence of mucus or blood) should be determined during history-taking. It is important to understand the patient’s normal bowel habits to determine the degree of change from baseline. A careful diet history may help identify inciting factors contributing to osmotic diarrhea, as well as identify foods associated with food- or water-borne pathogens (Salmonella, Shigella, Escherichia coli, Giardia). Daycare attendance, sick contacts, exposures to pets, and recent travel history should also be explored. Giardia and Salmonella are the most common parasitic and bacterial pathogens, respectively, causing diarrhea in daycare attendees. One should inquire about

exposure to animal vectors such as reptiles, which can be associated with Salmonella, as well as travel to areas with common endemic parasitic or bacterial infections. A history of recent antibiotic use should be obtained. Antibiotic-associated diarrhea typically resolves with cessation of medication, but Clostridium difficile can be associated with persistent diarrhea or blood in stool following recent antibiotic use. It is also important to elicit any accompanying systemic symptoms. Fever is often associated with viral and bacterial infections. Rash or wheezing may accompany allergy-mediated diarrhea. Seizure activity associated with diarrhea in an otherwise healthy child can be seen with Shigella infection. Growth history and nutritional status should be obtained, especially when considering the cause of chronic diarrhea. One of the most common causes of chronic diarrhea in toddler-age children is a disorder of small intestinal motility called nonspecific diarrhea of childhood (“toddler’s diarrhea”). Post-infectious carbohydrate intolerance, a form of acquired lactose intolerance, can result in osmotic diarrhea in an otherwise well patient with recent acute gastroenteritis. Both nonspecific diarrhea of childhood and postinfectious carbohydrate intolerance may be related to ingestion of excessive amounts of sugary fluids (juice, soda). Constipation with encopresis should also be considered in children with chronic diarrhea, which actually represents leakage of watery stool around a blockage in a dilated colon. Plotting a careful growth curve can assist in making a diagnosis in children with chronic diarrhea. Patients with cystic fibrosis often have poor growth beginning soon after birth. In contrast, children with celiac disease typically exhibit poor growth after 4 to 6 months of age, coincident with the introduction of gluten-containing solids to the diet.

PHYSICAL EXAMINATION The physical examination should first focus on the patient’s hemodynamic stability and hydration status to determine whether immediate fluid resuscitation is needed. Comparison of premorbid weight to the weight at presentation is the most reliable method of determining the degree of dehydration, but this is not always feasible. Mild dehydration occurs when the patient is 3% to 5% dehydrated. Clinical signs are usually evident at ~3%. Moderate dehydration results from 6% to 9% body weight lost. Severe

dehydration occurs when the patient is greater than or equal to 10% dehydrated, and can be fatal. Fluid status should also be assessed by examination of mental status, mucous membranes, anterior fontanelle, skin turgor, capillary refill, and urine output. Because chronic diarrhea may represent underlying systemic illness as well as lead to the loss of calories, protein and other minerals and vitamins (including zinc and vitamin B2), the physical examination should also look for evidence of these deficiencies (Table 22-1). TABLE 22-1

Selected Physical Findings Consistent with Evidence of Malnutrition or Systemic Illness

Area of Examination Finding

Nutritional Deficiency/Systemic Illness

General

Underweight

↓ Calories

Edematous

↓ Protein

Hair

Easily pluckable, sparse

↓ Protein, zinc

Skin

Generalized dermatitis

↓ Zinc

Erythema nodosum

Inflammatory bowel disease

Subcutaneous tissue

Decreased

↓ Calories

Muscles

Decreased mass

↓ Calories, protein

Mouth/tongue

Glossitis, angular stomatitis

↓ Vitamin B2

Oral ulcers

Inflammatory bowel disease

Extremities

Digital clubbing

Cystic fibrosis

Anus/rectum

Rectal prolapse

Cystic fibrosis

Perianal tags, fissures, fistulas

Inflammatory bowel disease

DIFFERENTIAL DIAGNOSIS The list of possible causes of diarrhea is extensive (Table 22-2). Distinguishing acute diarrhea from chronic diarrhea can be helpful in creating a differential. Most commonly, the cause of acute diarrhea is infectious. Viral infection (rotavirus, Norwalk virus, enteric adenovirus, calicivirus) account for most cases of acute diarrhea. Other infectious causes include bacterial pathogens (E. coli, Salmonella, Shigella, Campylobacter) and parasites (Giardia). TABLE 22-2

Causes of Diarrhea

Infectious Viruses Rotavirus Norovirus and other caliciviruses Enteric adenovirus Astrovirus Bacteria Salmonella Shigella Campylobacter jejuni Escherichia coli Staphylococcus aureus Clostridium difficile Aeromonas hydrophila Plesiomonas shigelloides

Vibrio cholera Vibrio parahaemolyticus Yersinia enterocolitica Parasites Giardia lamblia Entamoeba histolytica Cryptosporidium Congenital Congenital microvillus atrophy Autoimmune enteropathy Hirschsprung disease Congenital chloride-losing diarrhea Intractable diarrhea of infancy Short-bowel syndrome Malabsorptive Celiac disease Cystic fibrosis Inflammatory bowel disease Monosaccharide deficiencies Disaccharide deficiencies Post-infectious enteropathy Motility disorders Irritable bowel syndrome Allergic or intolerance Eosinophilic enteropathy Cow milk or soy protein intolerance Systemic Hemolytic uremic syndrome Hyperthyroidism α1-Antitrypsin deficiency

Immune deficiency Intestinal lymphangiectasia Acrodermatitis enteropathica Pancreatic insufficiency Hormone-secreting tumors Adrenal insufficiency α-lipoproteinemia Protein calorie malnutrition Miscellaneous Toddler’s diarrhea Constipation with encopresis Antibiotic associated Chronic diarrhea involves a broad list of possible causes. When evaluating chronic diarrhea, the age of the patient is helpful in narrowing the differential. In children under 1 year of age, intractable diarrhea of infancy is a common diagnosis. This entity is associated with diffuse mucosal injury resulting in persistent diarrhea, malabsorption, possible hematochezia, and malnutrition beginning before the age of 6 months. It is most commonly related to either cow milk or soy protein intolerance or prolonged postinfectious mucosal injury. Between the ages of 1 and 5 years, post-infectious enteritis, giardiasis, and celiac disease are more common diagnoses. In children older than 5 years, inflammatory bowel disease, constipation with encopresis, and acquired lactose intolerance are more likely. It is also important to remember that chronic diarrhea may be a presenting symptom of an immune deficiency, including HIV.

DIAGNOSTIC EVALUATION In most cases of viral gastroenteritis, laboratory evaluation is not necessary. The laboratory evaluation for patients presenting with diarrhea is individualized, with attention paid to the acute versus chronic nature of the diarrhea. For some patients, the search for a specific cause should be explored, including hospitalized patients with nosocomial exposure,

immunocompromised patients, and young infants, and in those presenting with high fever, toxic appearance, grossly bloody stools, and with recent antibiotic use. When a diagnosis is necessary, stool should be sent based on history for any or all of the following: occult blood, presence of fecal leukocytes, fecal fat, bacterial culture, ova and parasites, rotavirus antigen testing, C. difficile toxin, and Giardia antigen assay. If there is concern for a particular bacterial pathogen, this should be communicated to the laboratory so that the specimen can be plated on the appropriate media. If carbohydrate malabsorption is suspected, stool pH (41°C (105.8°F).

PATIENT HISTORY A thorough history and physical examination usually provide the most important clues in determining the cause of fever. Fever may be a sign of a benign, self-limited condition or may be indicative of a life-threatening illness. Important historical clues include the duration of fever, pattern of fever, and associated symptoms. A single spike of fever is typically not associated with an infectious disease, though it may be due to manipulation of catheters, drugs, and blood infusions. Viral infections typically are associated with a slow decline in fever over a week, whereas uncomplicated bacterial infections are associated with prompt resolution of fever within 24 to 48 hours of effective antibiotic administration.2 Table 24-1 describes common fever patterns and the associated diseases. Since the fever pattern is so important, a home diary of daily fevers is an extremely valuable tool when evaluating a patient with prolonged fever. The past medical history of a patient with fever should include any history of immunodeficiencies, previous major illnesses, and immunization status. Additional information that is important to elucidate are sick contacts (especially those with similar symptoms), attendance in daycare or school, medications currently used (including prescriptions, over-the-counter medications, and alternative remedies), travel and exposure history (pets, insects, undercooked foods).2 TABLE 24-1

Patterns of Fever Fever Type

Description

Intermittent Exaggerated circadian rhythm that includes normal temperatures on most days Septic

Wide fluctuation in temperatures

Sustained

Persistent fever that does not fluctuate by more than 0.5°C in 24 h

Remittent

Persistent fever that varies by more than 0.5°C in 24 h (e.g. tuberculosis, viral fever, many bacterial infections)

Relapsing

Febrile periods separated by intervals of normal temperature (e.g. Borrelia infection, syphilis, histoplasmosis, Behcet disease, systemic lupus erythematosus)

Tertian

Fever that occurs on the first and third days (malaria caused by Plasmodium vivax)

Quartan

Fever that occurs on the first and fourth days (malaria caused by Plasmodium malariae)

Biphasic

Illness with two distinct periods of fever over ≥1 wk (e.g. poliomyelitis, enteroviral infections)

Periodic

Recurring illnesses with some periodicity (e.g. cyclic neutropenia, Periodic fever aphthous stomatitis pharyngitis adenopathy (PFAPA), familial Mediterranean fever)

Double Quotidian

Fever that peaks twice in 24 h (classically associated with inflammatory arthritis)

PHYSICAL EXAMINATION A thorough physical examination is often crucial to determining the etiology of fever. The overall appearance of the patient must be assessed (i.e. does the patient appear toxic or well?) in addition to a complete examination with attention to possible signs of infection (pain, erythema, swelling, tenderness). The skin in particular should be examined closely, because a number of rashes with fever may be diagnostic. A full lymph node examination should also be performed. A complete set of vital signs including pulse oximetry, as always, is necessary for evaluation of a febrile child. The relationship between the patient’s pulse rate and fever may also aid diagnosis. It is normal for a certain amount of tachycardia to accompany fever, but relative tachycardia, in which the pulse rate is elevated out of proportion to temperature, is suggestive of a noninfectious process such as a toxin-mediated illness. Relative bradycardia, when the pulse rate is low despite the temperature elevation, suggests typhoid fever, brucellosis, leptospirosis, Legionnaires’ disease, drug fever, or a cardiac conduction defect that may occur with acute rheumatic fever, Lyme disease, viral myocarditis, or infective endocarditis.2 When a patient has a fever greater than 41°C, noninfectious conditions should be considered, including central fever resulting from central nervous system dysfunction, drug fever, malignant neuroleptic syndrome, heatstroke, and malignant hyperthermia.2

DIFFERENTIAL DIAGNOSIS The differential diagnosis of fever is broad and generally falls into one of four categories: infectious disease, rheumatic disease, malignancy, or miscellaneous (drug reaction, heat disorders, etc.). A complete history and physical will provide essential information needed to narrow the differential diagnoses being considered for each patient and dictate if further workup is necessary. For example, postoperative patients may develop fever from a number of causes, most commonly including atelectasis, wound infection, bacteremia, and deep venous thrombosis. Patients who have had urinary bladder catheters in place are at increased risk for urinary tract infection. Further, any procedure involving the surgical placement of a device such as a

catheter, shunt, rod, or other hardware carries a risk of infection (see Chapter 107).

DIAGNOSTIC EVALUATION Fever is a sign of a condition that incites cytokine production, so the focus of the evaluation is identifying that inciting condition. This evaluation is often dependent upon the specific characteristics and circumstances of the child presenting with fever. Most febrile episodes in a normal host can be diagnosed by a thorough history and physical and require few, if any, laboratory or radiographic tests. However, in children with fever and no apparent source, additional testing may be warranted on a case-by-case basis. Chapter 95 discusses fever in infants and toddlers and Chapter 96 discusses illnesses associated with prolonged fever (i.e. fever of unknown origin). Fever in an immunocompromised host is discussed in Chapter 109. Elevations in body temperature that occur without an inflammatory trigger, as seen in heat disorders, are discussed in Chapter 172.

MANAGEMENT Antipyresis is controversial among clinicians, scientists, caregivers, and patients. “Fever phobia,” or an unrealistic concern based on misperceptions of fever, is prevalent.3 Many caregivers believe that fever is a disease (rather than a symptom) and fear that fever itself may have life-threatening or braindamaging effects. There is no evidence that children with fever as a result of endogenous and exogenous pyrogens are at increased risk of adverse outcomes such as brain damage.5 Interestingly, over half of parents consider a temperature of less than 38°C (100.4°F) to be a fever, one quarter of parents would give antipyretics for temperatures less than 37.8°C (100°F), and 85% of parents reported awakening their child from sleep to give antipyretics. Treating a fever is not without risk, as up to one half of parents administer incorrect doses of antipyretics, with 15% of parents giving supratherapeutic dosing.3,5 The drawbacks to treating fever include the concerns that lowering a fever may impair the body’s ability to fight off infection, remove important diagnostic clues regarding the pattern of fever, and interfere with the

interpretation of the response to therapy for the inciting condition. There is limited data suggesting that fever actually helps the body recover more quickly from viral infections, although the fever may result in discomfort in children.4,5 The benefits of fever reduction include patient comfort, improved oral intake, decreased insensible fluid loss, and improved ability to assess the patient’s demeanor.5 However, the fever’s response to antipyretics is not diagnostic and does not provide information about its cause. There is some evidence that the same pyrogenic cytokines responsible for fever have potentially detrimental effects, including increased oxygen consumption, carbon dioxide production, and cardiac output, all of which can worsen the condition of children in shock or with chronic diseases such as cardiac insufficiency, sickle cell disease anemia, pulmonary insufficiency, diabetes mellitus, or inborn errors of metabolism.2 Of note, there is no evidence that antipyretic therapy decreases the recurrence of febrile seizures.5 Antipyresis is most commonly pursued pharmacologically. PGE2, which is central to the production of fever, is synthesized from arachidonic acid, mediated by the enzyme cyclooxygenase (COX). There are two isoforms of this enzyme, COX-1 and COX-2. COX-1 is expressed throughout the body constitutively and is thought to be important for a variety of “housekeeping” functions to maintain homeostasis. COX-2 is inducible by a number of the pyrogenic cytokines and is thought to be the key provider of PGE2 during the febrile and inflammatory response.1 Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen interfere with prostaglandin production through nonselective COX inhibition and down-regulation of the expression of COX enzymes. As a result, they have both antipyretic and anti-inflammatory effects. Selective COX-2 inhibitors were created to maintain the antipyretic and anti-inflammatory effects of the non-selective agents, but in theory cause fewer unwanted side effects of COX-1 inhibition, such as gastrointestinal bleeding. However, large clinical trials raised concerns that selective COX-2 inhibitors may increase the risk of cardiovascular and cerebrovascular events, leading to the removal of several of these drugs from the market in the United States and elsewhere.1 Surprisingly, the exact mechanism of action of acetaminophen, the most

commonly used antipyretic in pediatrics, remains unknown. It possesses excellent antipyretic but relatively weak anti-inflammatory effects. It is a weak inhibitor of COX, but its effect may be based on tissue-specific COX inhibitors. Acetaminophen penetrates the blood–brain barrier and causes a fall in central nervous system PGE2 production. Despite the controversies and uncertainties, virtually all parents expect their children to be treated for fever. It is of utmost importance that the goals of treating fever are discussed with families, emphasizing the therapeutic end-points of maximizing the child’s comfort and fluid intake rather than focusing on achieving normothermia. Families should be counseled to observe for signs of serious illness and discouraged from focusing on the fever alone. Acetaminophen (15 mg/kg every 4–6 hours, maximum of 5 doses in 24 hours) and/or ibuprofen (10 mg/kg every 6 hours in children over 6 months of age) are the mainstays of treatment. Aspirin should not be used due to the risk of Reye syndrome. Tepid sponge baths are not recommended to reduce fever because data have shown that they are no more effective than antipyretics alone and significantly increase patient discomfort scores. Alcohol baths are also discouraged because there have been reported adverse events associated with systemic absorption of alcohol.5 Some practitioners recommend using acetaminophen and ibuprofen in an alternating or combined fashion. While there are some studies that show lower temperatures, lower stress scores, and less time missed from child care in the combination treatment groups versus single-agent treatment groups, questions remain regarding the safety of this practice due to potential dosing confusion, whether it improves the comfort of the child, and whether this recommendation contributes to the fever phobia that already exists. Regardless of whether a single medication or alternating antipyretics are used, caregivers should be counseled on proper formulation, dosing (weightbased, not age-based), dosing intervals, and safe storage of these medications.5 Children should not receive cough and cold medications at the same time as antipyretics because there are often additional antipyretic medications in the cough and cold formulations. Families do not need to wake children for the purpose of administering antipyretic medications. It is often helpful for families, especially those with multiple caregivers, to keep a medication log to include the type of antipyretic, dose, and time given to

avoid inadvertent overdosing. Fever due to specific underlying etiologies resolves with appropriate treatment of the disease. Examples include administration of antibiotics for bacterial infections or administration of IVIG to treat Kawasaki disease. KEY POINTS Fever is a common presenting complaint prompting medical evaluation in children. Fever is a sign of an underlying pathology, not a disease itself. History and physical examination play an important role in determining diagnostic evaluation and management options. The differential diagnosis of fever is broad and generally falls into one of four categories: infectious disease, rheumatic disease, malignancy, or miscellaneous. Antipyresis using COX inhibition is the mainstay of treatment. The goal of treatment is patient comfort, not normothermia. Fever due to specific underlying etiologies resolves with the appropriate treatment of the disease.

REFERENCES 1. Kumar V, Abbas AK, Fausto N, eds. Acute and chronic inflammation. In Robbins and Cotran: Pathologic Basis of Disease. 8th ed. Philadelphia, PA: WB Saunders; 2010. 2. Nield LS and Kamat D. Fever. In: Kliegman RM, Stanton BF, St Gemell JW, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier; 2011:896e8-11. 3. Crocetti M, Moghbeli N, Serwint J. Fever phobia revisited: have parental misconceptions about fever changed in 20 years? Pediatrics. 2001;107:1241-1246. 4. Ward M. Pathophysiology and management of fever in infants and children. UpToDate. 2013. 5. Section on Clinical Pharmacology and Therapeutics, Committee on

Drugs, Sullivan JE, Farrar HC. Fever and the antipyretic use in children. Pediatrics. 2011;127(3):580-587.

CHAPTER

25

Gastrointestinal Bleeding April Buchanan

BACKGROUND Gastrointestinal (GI) bleeding produces alarm and anxiety in parents and physicians. Most causes of GI bleeding do not result in significant blood loss, and many cases of GI bleeding cease spontaneously. Larger volume bleeding can lead to hemodynamic compromise that requires aggressive resuscitation and intervention. A systematic approach to diagnosis is required. DEFINITIONS Hematemesis: Vomiting of either fresh or altered blood (such as coffee grounds emesis). Implies recent or continuing bleeding proximal to the ligament of Treitz. Hematochezia: Bright red blood per rectum or maroon-colored stools. Usually originates in the colon. Upper GI hemorrhage can also present with hematochezia secondary to decreased transit time in infants or with brisk bleeding. Blood-streaked stools suggest a bleeding source in the rectum or anal canal. Melena: Dark or black, tarry stools with a characteristic odor. Indicative of blood that has been in the GI tract for a long time, allowing the denaturation of hemoglobin by bowel flora. Melenic stools are typically from a hemorrhage originating proximal to the ileocecal valve. Occult blood: Presence of blood in the stool that is not visible but is confirmed by chemical testing (i.e. guaiac.)

PATIENT HISTORY The history can be helpful in identifying the cause and location of bleeding in the GI tract (Table 25-1). Quantifying the volume and acuity of blood loss is important in understanding the risk of hemodynamic compromise. The character of the blood may indicate a more likely location of bleeding, though brisk bleeding from an upper GI source can cause hematochezia. Fever, recent travel, or known sick contacts may implicate an infectious source, while longer-standing symptoms and associated weight loss may indicate a more serious underlying cause such as inflammatory bowel disease (IBD). A complete medication history is essential, including recent use of nonsteroidal anti-inflammatory drugs (NSAIDs) or antibiotics. Certain foods (e.g. beets) or medications can also cause a red discoloration of GI fluids and be mistaken for hematemesis or melena. TABLE 25-1

Focused History

Characteristics of Bleeding Quantity: volume of blood (few drops vs. a cup) Duration: intermittent bleeding, isolated episode, ongoing bleeding Character: bright red blood, coffee grounds emesis, melena, hematochezia Abdominal Complaints Bowel patterns: diarrhea (infectious) or constipation (fissures), change in stool color Abdominal pain: indicates inflammation or ischemia of bowel wall Painless bleeding: indicates Meckel diverticulum, duplication, vascular malformation, or polyps Abdominal distention: possible bowel obstruction Tenesmus or urgency to defecate: consider IBD or infectious colitis Dietary History Cow milk or soy formula: consider allergic colitis Breastfeeding: consider ingested maternal blood Ingestion of products mistaken for hematemesis: artificial food coloring, gelatin, artificial fruit drinks, certain antibiotics, and cough

syrups Ingestion of products mistaken for melena: beets, iron supplements, dark chocolate, bismuth, spinach, blueberries, grapes, licorice, others Review of Systems General: fever, weight loss or gain, anorexia Skin: rash, vascular malformations, edema, jaundice, lymphadenopathy, easy bruising Extremities: arthralgia, arthritis, clubbing Genitourinary: hematuria Abdomen: distention, pain, bowel patterns, vomiting Ears, nose, and throat: pharyngitis, epistaxis Past Medical History Previous GI bleeding Liver disease: indicates possible variceal bleeding or coagulopathy Previous or recent hospitalization: stress gastritis Medications or ingestions: NSAIDs, aspirin, steroids, anticoagulants, alcohol, toxins (rat poison) Umbilical artery catheterization: risk for portal vein thrombosis Coagulopathy Recent antibiotic exposure: pseudomembranous colitis Presence of gastrostomy or nasogastric tube Family History IBD, peptic ulcer disease, polyposis, bleeding diatheses Social History Immediate contacts with similar symptoms: may indicate infectious cause Travel, camping, or daycare: consider infectious causes GI, gastrointestinal; IBD, inflammatory bowel disease; NSAID, nonsteroidal anti-inflammatory drug.

PHYSICAL EXAMINATION The physical examination can be helpful in determining the etiology of GI bleeding (Table 25-2). The first priority is a quick assessment to identify patients with hemodynamic compromise or shock who need immediate resuscitation. Vital signs should be monitored closely. Patients may lose up to 15% of blood volume without evidence of hemodynamic compromise, and the blood pressure may be maintained until the patient has lost as much as 30% of the blood volume.1 Once the patient is determined to be stable, a comprehensive history and physical examination should be pursued. TABLE 25-2

Directed Physical Examination Findings and Associated Diseases

Vital Signs Fever: infectious causes or inflammatory diseases (IBD or HSP) Weight loss: chronic diseases (IBD, cystic fibrosis, liver disease) Tachypnea: hemodynamic compromise or acidosis Tachycardia: earliest sign of hemodynamic compromise Hypotension: present with significant blood volume loss General Distressed or toxic-appearing patient: hemodynamic compromise from significant hemorrhage or underlying process causing systemic illness (toxic colitis, intussusception, ischemic bowel, necrotizing enterocolitis) Well-appearing patient: less urgent causes of bleeding Failure to thrive: malnutrition from chronic diseases (IBD, cystic fibrosis, liver disease) Head, Eyes, Ears, Nose, and Throat Nose: evidence of epistaxis Eyes: scleral icterus (liver disease), iritis (IBD) Oropharyngeal: mucosal trauma or bleeding from posterior pharynx Cardiovascular Evidence of hemodynamic compromise: tachycardia, gallop rhythm,

delayed capillary refill, poor perfusion Abdominal Abdominal tenderness: nonspecific but indicates inflammation or ischemic injury of the bowel Abdominal mass: intussusception, intestinal duplication may result in right lower quadrant masses Evidence of portal hypertension: hepatosplenomegaly, ascites Rectal examination: stool specimen for hemoccult testing, palpable polyp Perineal and anal inspection: skin tags (IBD), fissures, superficial skin breakdown or inflammation (streptococcal cellulitis) Extremities Clubbing: chronic diseases (cystic fibrosis, IBD, liver disease) Arthritis: IBD Skin Vascular malformations: syndromes with associated Gl vascular malformations (e.g. Klippel-Trénaunay syndrome, Rendu-OslerWeber syndrome) Cutaneous or oral pigmentation: Peutz-Jeghers syndrome Purpura or petechiae: vasculitis (HSP) or bleeding diathesis Erythema nodosum: IBD GI, gastrointestinal; HSP, Henoch-Schönlein purpura; IBD inflammatory bowel disease.

DIFFERENTIAL DIAGNOSIS The differential diagnosis of GI hemorrhage can be divided into upper and lower GI bleeding. Although certain causes are more likely in certain ages, there is considerable overlap between age groups. Patients with complex medical issues have special diagnostic considerations in the evaluation of GI bleeding. The causes of upper and lower GI bleeding are summarized in Tables 25-3 and 25-4, respectively. TABLE 25-3

Causes of Upper Gastrointestinal

Bleeding Neonates and Young Infants

Older Infants, Children and Adolescents

Ingested maternal blood

Gastritis/ gastroduodenal ulceration

At delivery

Sepsis

During breastfeeding

Stress

Milk protein allergy

Medications

Trauma (nasogastric tube)

Ingestion

Gastritis

Burns

Overwhelming illness

Increased ICP

Medications

Ischemia

Idiopathic

Acidosis

Ischemia

Helicobacter pylori*

Acidosis

Esophagitis

Esophagitis

Reflux

Reflux

Infectious

Infectious

Ingestion

Necrotizing enterocolitis

Eosinophilic

Coagulopathy

Esophageal foreign body

Congenital malformations

Gastroesophageal varices

Duplication cyst

Cirrhosis

Vascular malformation

Extrahepatic portal vein thrombosis†

Budd-Chiari syndrome± Nasopharyngeal bleeding source Epistaxis Tonsils Tooth extraction Coagulopathy Hemobilia Hepatic injury Trauma to intestinal mucosa Nasogastric tube Gastrostomy tube Mallory-Weiss tear Prolapse gastropathy Vascular anomalies† Hemangioma Dieulafoy lesion Telangiectasia Other Henoch-Schönlein purpura Crohn disease Pulmonary hemorrhage Congenital malformations†

Duplication cyst *More common in older children and adolescents. †More

common in infants and young children.

± ICP, intracranial pressure.

TABLE 25-4

Causes of Lower Gastrointestinal Bleeding

Neonates and Infants Young Children

Older Children and Adolescents

Necrotizing enterocolitis

Anal fissure

inflammatory bowel disease

Anal fissure

Intussusception

Vasculitis

Allergic colitis

Juvenile polyps (>4 y)

Henoch-Schönlein purpura

Milk protein

Vascular lesions

Juvenile or inflammatory polyps

Soy protein

Hirschsprung enterocolitis

Colonic or rectal varices

Swallowed maternal blood

Intestinal duplication

Hemorrhoids

Meckel diverticulum

Infectious enterocolitis Anal or rectal fissure

Hirschsprung enterocolitis

Inflammatory bowel disease (>4 y)

Infectious enterocolitis

Infectious enterocolitis

Meckel’s diverticulum

Colonic or rectal ulceration

Malrotation or volvulus

Perianal streptococcal Bleeding diathesis cellulitis

Intestinal duplication

Henoch-Schönlein purpura

Eosinophilic gastroenteropathy

Bleeding diathesis

Hemolytic uremic syndrome

Rectal trauma

Typhlitis*

Typhlitis*

Eosinophilic gastroenteropathy Bleeding diathesis Colonic or rectal varices Lymphoid nodular hyperplasia Colonic or rectal ulceration Rectal trauma *lmmunocompromised patients.

UPPER GASTROINTESTINAL BLEEDING Some causes of upper GI bleeding are unique to neonates and young infants. Swallowed maternal blood from the birth process or from breastfeeding may cause hematemesis. To differentiate between maternal and fetal blood, an Apt test may be performed. Adult hemoglobin is reduced to a brown-yellow color, whereas fetal hemoglobin is resistant to reduction and remains pink. Esophagitis can lead to upper GI bleeding and may manifest with symptoms of dysphagia, regurgitation, cough, arching, or failure to thrive. It may be acid related, infectious, associated with a caustic ingestion, or allergic, as in the case of eosinophilic esophagitis. Children with neuromuscular diseases are more prone to severe gastroesophageal reflux disease that leads to esophagitis.

Esophageal varices can cause sudden, massive hematemesis, which can lead to hemodynamic compromise. Varices are associated with portal hypertension from both intrahepatic and extrahepatic causes. Portal vein thrombosis may be associated with umbilical artery catheterization or omphalitis, but it usually occurs in young, previously healthy children, and the cause is not identified.2,3 Thrombosis of the hepatic veins (Budd-Chiari syndrome) is usually associated with hypercoagulable states. Portal hypertension is most commonly caused by cirrhosis associated with chronic biliary diseases such as biliary atresia, cystic fibrosis, sclerosing cholangitis, and parenteral nutrition-induced cholestasis. Endoscopic evaluation of patients with known cirrhosis and esophageal varices is important, because the source of bleeding is commonly from gastric and duodenal ulcerations rather than varices.2 Patients with liver failure may develop GI bleeding from other mechanisms, such as associated coagulopathy. Forceful vomiting or retching may lead to a Mallory-Weiss tear or cause prolapse gastropathy, a phenomenon in which the gastric fundus is prolapsed into the esophagus, resulting in injury to the mucosa. Trauma to the esophageal or gastric mucosa may occur with nasopharyngeal suctioning or from nasogastric or gastrostomy tubes, which can lead to local irritation or laceration. Gastritis may be induced by many commonly used medications, including NSAIDs and steroids. Helicobacter pylori may cause gastritis and subsequent ulceration in children. Stress gastritis can occur in any age group and may be a complication of an overwhelming or life-threatening illness. Gastric or duodenal ulceration may also accompany head trauma, increased intracranial pressure (Cushing ulcer), or significant burns (Curling ulcer).

LOWER GASTROINTESTINAL BLEEDING Similar to upper GI bleeding, neonates and young infants have some unique causes of lower GI bleeding. In premature or young infants, necrotizing enterocolitis is a significant cause of morbidity and mortality and is an important diagnosis to exclude when evaluating hematochezia. Necrotizing enterocolitis typically presents with bloody stools, vomiting, abdominal distention, temperature instability, and discoloration or erythema of the abdominal wall. Volvulus is another diagnostic consideration when

evaluating lower GI bleeding in neonates and infants. Volvulus leads to ischemic injury of the bowel and can cause active bleeding; it is a surgical emergency. Hirschsprung colitis may also cause severe illness in young infants. A more common cause of bloody stools in well-appearing infants is allergic colitis, which is usually due to cow milk protein allergy. Soy protein intolerance is also common in these infants. Infectious enterocolitis can lead to bloody stools in infants and children. The classic pathogens include Salmonella, Shigella, Yersinia, Escherichia coli (enteroinvasive or enterohemorrhagic), Campylobacter jejuni, and Entamoeba histolytica. Viral pathogens such as rotavirus, Norwalk virus, and adenovirus 40/41 can also cause bloody stools. Antibiotic therapy puts patients at risk for the development of Clostridium difficile colitis; this should be considered in any patient who develops bloody stools with a history of recent antibiotic exposure. Meckel diverticulum is a remnant of the omphalomesenteric duct that can present with painless rectal bleeding. The rule of twos applies to Meckel diverticulum: it occurs in approximately 2% of the population, is located within 2 feet of the ileocecal junction, is around 2 inches long and 2 cm in diameter, has two types of ectopic mucosa (gastric and pancreatic), and has a 2:1 male-to-female ratio. In addition, Meckel’s presents before 2 years of age in 50% of patients. Bleeding is caused by ulceration of the ileal mucosa adjacent to the secretion-producing ectopic tissue. Duplications of the bowel may also contain ectopic mucosa and present with bleeding from a similar mechanism, or act as lead points for intussusception and subsequent ischemic injury. Painless, intermittent rectal bleeding may be caused by juvenile polyps, which are non-neoplastic inflammatory lesions found in the rectosigmoid region of school-age children. Bleeding diatheses are another cause of painless rectal bleeding in any age group. Henoch-Schönlein purpura (HSP) is a systemic vasculitis that affects primarily small vessels of the skin, joints, kidneys, and GI tract. The GI manifestations can precede the skin manifestations or occur days after. Bloody stools can result from diffuse mucosal hemorrhage or from intussusception resulting from HSP. Occasionally, HSP may result in massive hemorrhage. IBD is an important diagnostic consideration in older children and

adolescents with hematochezia. Ulcerative colitis is a diffuse mucosal inflammation limited to the colon. In contrast, Crohn disease may occur in any segment of the GI tract. Both may present with bloody mucopurulent diarrhea, abdominal pain, and weight loss, as well as extraintestinal manifestations.

DIAGNOSTIC EVALUATION LABORATORY STUDIES Laboratory studies are directed at determining both the extent of blood loss and the underlying cause. An initial complete blood count should be followed by serial hemoglobin measurements to assess ongoing bleeding. Thrombocytopenia can be associated with multiple systemic illnesses that cause GI bleeding, such as necrotizing enterocolitis, sepsis, idiopathic thrombocytopenic purpura, and hypersplenism. Eosinophilia may be present in allergic colitis or parasitic infections. Clotting studies should be performed to rule out a bleeding diathesis. Blood typing and cross-matching are necessary if there is any suggestion of profuse or ongoing bleeding or hemodynamic compromise. Routine chemistries, including renal and hepatic function, should be checked. Elevated blood urea nitrogen in the setting of a normal creatinine level may indicate a large amount of intraluminal blood in the small intestine with blood resorption. Azotemia may be present with hemolytic uremic syndrome or HSP. Elevated liver transaminases or bilirubin may indicate hepatic failure or cirrhosis, raising the suspicion for varices. In an ill-appearing neonate with bloody stools, a blood culture should be obtained. Inflammatory markers such as the erythrocyte sedimentation rate and C-reactive protein may be elevated in inflammatory diseases such as IBD or HSP. Infectious causes may also lead to increased inflammatory markers. Red dyes, fruit juices, tomato skins, and beets can cause the vomitus or stool to look reddish in color. In addition, grapes, spinach, blueberries, licorice, and iron can cause the stool to look dark. A stool guaiac test uses a leukodye that has an oxidative reaction in the presence of hemoglobin, producing a blue color. False positives can occur in the presence of certain ingested foods such as rare meat, turnips, tomatoes, horseradish, and cherries. False negatives can occur due to the consumption of vitamin C.4 The Hemoccult test is accurate on stool but is inactivated by acid; therefore,

gastric contents should be tested with the Gastroccult test. Stool studies should include fecal leukocytes, eosinophils, bacterial culture, ova and parasites, viral studies, and screening for C. difficile toxin A and B when appropriate.

IMAGING Imaging may be an important in the workup of GI hemorrhage to define the pathology or pinpoint the bleeding site. Some centers may offer interventional options as well. A barium study can detect abnormalities associated with infectious esophagitis, esophageal varices, or an esophageal foreign body. An upper GI study may demonstrate abnormalities associated with profound reflux, obstructive causes such as malrotation with midgut volvulus, or gastric or duodenal ulcers. Plain abdominal radiographs are helpful in the evaluation of GI bleeding to assess for an obstructive bowel gas pattern, intraperitoneal free air, and pneumatosis coli in necrotizing enterocolitis or typhlitis. However, computed tomography is the preferred imaging modality to confirm the diagnosis of typhlitis in immunocompromised children. A barium enema can reveal mucosal abnormalities associated with IBD and can demonstrate polyps. Extraluminal complications of Crohn disease, such as adhesions or abscess formation, are better evaluated with computed tomography or magnetic resonance imaging. An air-contrast enema can be diagnostic and therapeutic for intussusception. Nuclear medicine imaging using a tagged red blood cell scan can be helpful in pinpointing the location of bleeding. In one method, the patient’s blood is drawn, labeled with a marker, and then reinjected. The images are evaluated in 5-minute frames for up to 2 hours to localize a bleeding source. Another valuable nuclear medicine technique is the Meckel scan, which is designed to demonstrate the presence of ectopic gastric mucosa within the diverticulum. Technetium 99m pertechnetate is injected intravenously and concentrates in gastric mucosa. Serial images are then obtained in 5-minute intervals to determine the presence of ectopic mucosa within a Meckel diverticulum. In specialized centers, angiography of the celiac or superior mesenteric artery can be performed in cases of brisk bleeding that cannot be diagnosed with other approaches. Arteriography may allow for transcatheter therapy with vasopressin or embolization.

MANAGEMENT INITIAL STABILIZATION Patients with GI bleeding who present with evidence of hemodynamic compromise should have airway, breathing, and circulation (ABC’s) assessed (Figure 25-1). Once the airway and respiratory status are stabilized, the volume status should be addressed. Aggressive fluid resuscitation may be necessary, initially with crystalloid. Significant blood loss may require transfusion of blood products (red blood cells or fresh frozen plasma). In the case of any significant GI bleeding episode, a nasogastric tube should be placed and gastric lavage performed to assess for ongoing bleeding. Presence of blood in the nasogastric aspirate can confirm an ongoing upper GI bleed in patients with hematemesis or melena. The absence of blood in the nasogastric aspirate does not exclude upper GI bleeding from the postpyloric region in patients with hematochezia or melena with brisk bleeding. A gastroenterology consultation should be obtained for upper and/or lower endoscopy in cases of ongoing or severe bleeding or if a diagnosis cannot be established by other imaging modalities.

FIGURE 25-1. Diagnostic and treatment algorithm for GI bleeding.

PHARMACOLOGIC THERAPY Gastritis and peptic ulcer disease in children is effectively treated with H2antagonists and proton pump inhibitors, as well as bismuth preparations. Early use of acid-suppressive medications in critically ill patients or those on high-dose steroids or anti-inflammatory medications may be prudent. H. pylori infection in children is treated similarly to that in adults, with antibiotics, acid suppression, and bismuth preparation. C. difficile colitis requires antibiotic therapy. However, many bacterial pathogens causing bloody diarrhea do not require antibiotic treatment, and

appropriate therapy should be reviewed on a case-by-case basis. For esophageal varices, intravenous or intra-arterial continuous infusions of vasopressin, somatostatin, or octreotide may be administered. These therapies work by reducing splanchnic arterial blood flow, therefore reducing the amount of bleeding from the varices. There are many pharmacologic therapies available for the management of IBD. Use of these medications should be guided by a gastroenterologist (see Chapter 79).

ENDOSCOPY Upper GI endoscopy is the preferred procedure for the diagnosis and evaluation of acute, severe upper GI hemorrhage or persistent or recurrent upper GI bleeding. Endoscopy is sensitive and specific and may allow for immediate therapy. Endoscopic interventions are available for active bleeding ulcers and varices, and tissue specimens can be obtained by biopsy. Endoscopic sclerotherapy and elastic band therapy have been used successfully for esophageal varices in children. Lower GI bleeding that is persistent or recurrent can also be effectively evaluated and possibly intervened upon by endoscopy. Flexible sigmoidoscopy is usually sufficient to evaluate for colitis and polyps. Colonoscopy reaching the level of the terminal ileum may be needed to evaluate more proximal lesions. Lower endoscopy is a valuable diagnostic modality to detect vascular lesions. Polyps can frequently be removed with electrocautery through an endoscope.

SURGERY Surgical indications include bowel perforation, ischemic bowel from obstructive causes including irreducible intussusception, as well as ongoing or massive bleeding not amenable to control with endoscopy or interventional radiology. In cases of ulcerative colitis refractory to medical management, the disease can be cured with a total colectomy. Complications of Crohn disease, such as adhesions or fistula formation, may require surgical intervention. There are several surgical treatment options for portal hypertension and esophageal varices, including esophageal transection, portosystemic shunts,

and transjugular intrahepatic portosystemic shunts, which are most often used in children with extrahepatic portal vein obstruction. Candidates for these procedures should be determined by consultation with a gastroenterologist and a surgeon.

SPECIAL CONSIDERATIONS Immunocompromised patients may develop GI bleeding from some unique causes. They are at risk for esophagitis secondary to cytomegalovirus and fungi, as well as chemically induced mucositis from chemotherapeutic agents. Typhlitis is an important diagnostic consideration in immunocompromised patients with abdominal pain and lower GI bleeding. Immunocompromised patients with bloody diarrhea should be evaluated for opportunistic infections as well as cytomegalovirus colitis. KEY POINTS GI bleeding can present from infancy through adolescence, and the differential diagnosis changes with age. The most important initial step is hemodynamic stabilization, followed by a thorough evaluation of the likely location and source of bleeding. Evaluation and treatment can be guided by the age of the patient, the acuity of the patient, and presence or absence of continued bleeding. Treatment modalities include pharmacologic therapy, radiologic intervention, endoscopy, and surgical management.

SUGGESTED READING Boyle JT. Gastrointestinal bleeding in infants and children. Pediatr Rev. 2008;29:39-52.

REFERENCES

1. Rodgers B. Upper gastrointestinal hemorrhage. Pediatr Rev. 1999;20:171-174. 2. Karrer F, Narkewicz M. Esophageal varices: current management in children. Semin Pediatr Surg. 1999;8:193-201. 3. Lykavieris P, Gauthier F, Hadchouel P, et al. Risk of gastrointestinal bleeding during adolescence and early adulthood in children with portal vein obstruction. J Pediatr. 2000;136:805-808. 4. Liacouras CA, Piccoli DA, Bell LM, eds. Pediatric Gastroenterology: The Requisites in Pediatrics. 1st ed. Philadelphia, PA: Mosby; 2008:8797.

CHAPTER

26

Hypertension Avram Z. Traum and Michael J.G. Somers

BACKGROUND Although hypertension is a relatively uncommon disorder in pediatrics, its identified incidence is increasing, likely related to both heightened clinician awareness of the problem and increasing rates of obesity in children mediating earlier onset of high blood pressure.1-4 With adults, most hypertension is deemed to be primary or “essential” in etiology and often no diagnostic evaluation ensues. In children, on the other hand, primary hypertension has long been a diagnosis of exclusion and there has been consensus that hypertensive children need to undergo an evaluation to exclude any secondary causes to their hypertension, especially in the setting of significantly elevated blood pressures for a child’s age and body habitus. Regardless of its cause, depending on the degree of elevation and the duration of onset, hypertension can lead to both acute and chronic organ dysfunction. In ambulatory settings, otherwise healthy hypertensive children have been found to be more likely to have left ventricular hypertrophy, accelerated atherosclerosis, proteinuria, and decreased cognitive function. In hospitalized children, hypertension may complicate the management of coexisting clinical conditions and there is the additional burden of determining whether the high blood pressure is a primary problem or whether it stems from an ongoing condition or its treatment. Accordingly, a child who presents with hypertension often requires treatment while the diagnostic evaluation is ongoing. The approach to the evaluation and treatment of hypertension is often both more directed and more intensive in a hospitalized child than in an ambulatory setting. The extent of the blood pressure elevation and the degree of clinical concern for immediate harm to the child from the hypertension also guides the tempo of

diagnostic evaluation and therapeutic intervention.

ASSESSMENT AND DEFINITION MEASUREMENT OF BLOOD PRESSURE Blood Pressure Norms With adults, blood pressure standards are based on epidemiologic outcome measures related to chronic end-organ damage seen in patients followed longitudinally with high blood pressure. In contrast, hypertension standards in children are based on statistical population norms since end organ effects may take decades to manifest. Currently, the “Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents”5 is used widely in North America to define blood pressure norms in children, and is more comprehensive than previously published standards.6 In children, normal blood pressure is defined as blood pressure measurements consistently falling below the 90th percentile compared to a pediatric reference group of comparable gender, age, and height. Prehypertension (formerly known as high-normal blood pressure) is defined as blood pressure at the 90th percentile or higher but less than the 95th percentile. Stage One Hypertension is defined as blood pressure at the 95th percentile or greater, and Stage Two Hypertension—or what used to be called severe hypertension—is that exceeding the 99th percentile. Auscultation versus Oscillometry Accurate measurement of blood pressure is essential before any management decisions are made. Statistical blood pressure norms in pediatrics, such as those in the Fourth Report, are based on measurement by auscultation using a stethoscope. In spite of this, especially in hospitalized children and increasingly in the ambulatory office setting, oscillometric automated devices (e.g. Dynamap) are widely used to measure blood pressure because they are convenient and easy for any personnel to use. It is often not appreciated, however, that oscillometric measurements of blood pressure are typically at least 5 to 10 mmHg higher than those obtained by auscultation.7 This is true even in the hands of experienced clinical staff specifically trained to take blood pressures.8 As a result, blood pressure assessment by oscillometry is best used for screening, but any high blood

pressure measurements should be confirmed with auscultation to make sure oscillometric readings can be used to guide therapeutic intervention. Cuff Size and Location Cuff size is another important factor that impacts accurate blood pressure measurement. Cuffs that are too small tend to overestimate blood pressure, and cuffs that are too large may underestimate blood pressure, although not as significantly as the overestimation resulting from small cuffs. The most precise method for choosing an appropriate cuff size is controversial,9-11 but the recommendations of the National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents should be considered.5 Those guidelines delineate that the width of the cuff bladder should be at least 40% of the arm circumference measured midway between the olecranon and the acromion. This generally correlates with the bladder length covering 80% to 100% of the arm circumference. Appropriate-sized cuffs should be available from infant up to thigh-sized cuffs. In muscular or obese adolescents with large upper extremities, a thigh cuff may be necessary to cover the arm adequately. Cuff location can also significantly affect the blood pressure reading. Measurements on the upper arm should be made with the cuff at the level of the heart, whether the patient is in the upright or supine position. When the cuff is placed on the lower extremity, the patient should be supine for accurate measurement. The measurement will be elevated due to increased hydrostatic pressure if obtained on the lower extremity of a patient in a sitting or upright position. Auscultation Technique As noted earlier, auscultation is the preferred way to measure a child’s blood pressure and should be done to confirm hypertension. With the child at rest and acclimated to the local environment, the examiner places the diaphragm of the stethoscope over the brachial artery at the antecubital fossa. The right arm is used both for convenience and to allow the diagnosis of coarctation of the aorta; the left subclavian artery usually comes off the aorta after a thoracic coarctation and thus has normal blood pressure. The examiner inflates the cuff to a pressure above which a pulsatile sound can no longer be auscultated, at which point the cuff is deflated slowly. The systolic blood pressure is measured at the onset of the tapping, or what is termed the first Korotkoff sound; the diastolic blood pressure is measured at the disappearance of the Korotkoff sounds. In some children, the Korotkoff sounds may continue until the diastolic blood

pressure reaches zero. Although this is unlikely in the presence of significant hypertension, if it occurs, the diastolic blood pressure should be measured at the muffling or fourth Korotkoff sound. Ambulatory Blood Pressure Monitoring Over the past decade, there has been increasing use of ambulatory blood pressure monitoring to overcome the problems inherent in trying to diagnose hypertension from sporadic blood pressure measurements alone. These devices provide a 24hour log of blood pressures measured at regular intervals during normal activities. They also allow for assessment of diurnal variations in blood pressure for comparison with expected fluctuations, and provide a sense of the proportion of both systolic and diastolic blood pressures that are elevated, and the extent of these elevations. In the ambulatory setting, these devices can help distinguish between “white coat hypertension”—high blood pressure related to the medical office visit—and true sustained hypertension. In the hospitalized child, these devices have less applicability since it is easier to get multiple measurements by auscultation or oscillometry. Moreover, with the hospitalized patient, there is often more urgency in treating blood pressure elevations and erring on the side of overtreatment if there are concerns that blood pressure elevation may be clinically harmful.

PATHOPHYSIOLOGY As the incidence of obesity rises, so does the incidence of hypertension in children and the likelihood that children with hypertension will be cared for in the hospital.12 The etiology of hypertension in obese children is not clear, though many believe insulin resistance and subsequent vascular endothelial dysfunction play a key role. As a result, some argue that obesity-related hypertension is really a secondary form of hypertension and should not be lumped with primary hypertension in children. Regardless of body habitus, all children with evidence of sustained or recurrent hypertension should be evaluated for secondary hypertension in an individualized and stepwise fashion, guided by the findings on history, physical examination, and initial screening tests. Children with more significant and sustained elevations of blood pressure, as well as younger children, are more likely to have some specific cause of hypertension identified during the evaluation. The most common cause of secondary

hypertension in children is renal parenchymal disease,13 and the diagnostic evaluation should reflect this. An algorithm for the diagnostic evaluation of hypertension in hospitalized children is provided in Figure 26-1.

FIGURE 26-1. Algorithm for the evaluation and management of a hospitalized child with hypertension. BP, blood pressure; DMSA, dimercaptosuccinic acid.

EVALUATION HISTORY

In obtaining a history, the clinician should focus on disorders or conditions that predispose to hypertension and, in a hospitalized patient, should begin with the history of present illness leading to the child’s admission. For instance, in a postoperative patient who is otherwise healthy, pain or anxiety may play a role in hypertension. In a child hospitalized with severe reactive airway disease, steroid therapy or frequent administration of β-agonists may be problematic. In the absence of an obvious precipitating factor related to an acute illness or medical condition, the history should begin with the perinatal period. Prematurity is a risk factor for hypertension, because premature babies are born with fewer nephrons, have a reduced renal reserve, and may be more prone to hyperfiltration injury and glomerulosclerosis as both a child and adult. In addition, placement of umbilical catheters in the nursery or periods of hypotension early in life can mediate renal injury by thrombosis or hypoperfusion with increased risk for subsequent renal scarring. Especially in preschool children, febrile urinary tract infections commonly leave renal parenchymal scars that can then lead to hyperreninemic hypertension over time. These infections may have been misdiagnosed or even gone unappreciated, so any history of unexplained recurrent febrile illness should be elicited. Glomerulonephritis presents with edema, hematuria, and hypertension and may be isolated to the kidney or associated with systemic inflammatory disorders such as systemic lupus erythematosus. Relevant extra-renal findings include joint symptoms, rashes, and recurrent unexplained fevers or constitutional symptoms. Recent systemic infections may also lead to postinfectious glomerulonephritis and may be associated with gross hematuria or tea-colored urine in addition to high blood pressure. The family history focuses on relatives with pediatric or early-onset hypertension and inherited diseases that affect the kidneys, such as the polycystic kidney disease complexes, tuberous sclerosis, and neurofibromatosis. A strong family history of cardiovascular disease, such as coronary artery disease, stroke, and hyperlipidemia, suggests similar risk to a hypertensive child as adulthood is reached. Certain medications are known to cause hypertension, including oral contraceptives, corticosteroids, stimulants, decongestants, and the calcineurin inhibitors (cyclosporine, tacrolimus) that are the mainstay of

immunosuppression in transplant patients. Recreational drugs with stimulant effects, such as cocaine, nicotine, and ephedra, can also raise blood pressure, as can withdrawal from the effects of central nervous system depressants such as ethanol or narcotic analgesics. The review of systems should evaluate for symptoms such as headache, chest pain, visual changes, or mental status changes, because these may be related to severe hypertension or end-organ damage. Sweating, palpitations, and flushing are associated with states of catecholamine excess such as occurs with a pheochromocytoma.

PHYSICAL EXAMINATION The most essential part of the physical assessment of a child with suspected hypertension is accurate measurement of the blood pressure. Blood pressure should be measured in all four extremities. When measured at the same encounter with the same equipment, the blood pressure in the lower extremities is typically 10 to 20 mmHg higher than in the right arm. If lower extremity blood pressure is not higher, this suggests a coarctation or other narrowing of the aorta and is often associated with diminished femoral pulses. Height, weight, and body mass index should be measured to assess for growth anomalies or to document obesity. The physical examination should look for signs of end-organ damage from hypertension. This involves funduscopy and cardiovascular, pulmonary, and neurologic examinations. Although the finding is uncommon, the abdomen should be auscultated for abdominal bruits, which can be seen in renovascular hypertension. The physical examination should also look for findings of genetic syndromes that may explain the elevated blood pressure. These include neurofibromatosis (café au lait spots, axillary freckling, Lisch nodules), tuberous sclerosis (ash-leaf macules, shagreen patches, adenoma sebaceum, peri- or subungual fibromas, retinal hamartomas), Turner syndrome (short stature, shield chest, upturned mouth, webbed neck), and Williams syndrome (overfriendly personality, cognitive impairment, prominent ears).

LABORATORY AND RADIOLOGIC EVALUATION

A urinalysis should be preformed on a freshly voided urine sample, and if the dipstick is positive for blood or protein should include microscopy. Initial screening blood work should include electrolytes, blood urea nitrogen, and creatinine. This effectively evaluates renal function and screens for states of mineralocorticoid excess that lead to hypokalemia and alkalosis. All children with hypertension should undergo renal ultrasonography. An ultrasound study will uncover differential renal size, hydronephrosis, changes in parenchymal echogenicity, or diffuse cystic changes. More extensive areas of renal scarring will often be appreciated on ultrasound as thinning of the renal cortex or loss of parenchyma. Subtler scarring may not be seen on ultrasonography, but a size discrepancy of greater than 1 cm between the two kidneys is unusual and may reflect scarring in the smaller kidney. Since there is little yield in routinely doing other laboratory work or imaging studies with initial screening, each individual’s history and physical examination findings should guide further evaluation. For example, tachycardia suggests hyperthyroidism or high-catecholamine states and should trigger blood thyroid function tests and quantification of catecholamines. A history of urinary tract infections with unexplained fevers or positive urine cultures should precipitate a dimercaptosuccinic acid (DMSA) scan to determine the presence of cortical scars. Plasma renin activity and aldosterone levels are helpful only if the results are unequivocally low or very high. They are most useful when a diagnosis of mineralocorticoid excess is suspected, because plasma renin activity is typically suppressed. Aldosterone levels may be low or high, depending on the specific cause of the mineralocorticoid excess. The diagnosis of renal artery stenosis continues to be challenging in children. The gold standard study for diagnosis remains angiography. It allows for both diagnostic and therapeutic interventions, although the invasive nature of angiography limits its use as first-line approach. Doppler studies of renal vasculature can show changes in flow rates through areas of measurement and may suggest the presence of renovascular hypertension. A normal Doppler study does not, however, rule out renal artery stenosis, especially stenosis in the smaller segmental arteries that are not well visualized by Doppler. Computed tomographic angiography (CTA) and magnetic resonance angiography (MRA) have been studied as alternate imaging modalities. MRA lacks the spatial resolution of CTA but avoids the

radiation exposure that comes with CT.14 One study in approximately 400 adults demonstrated that compared to conventional angiography, CTA had a sensitivity of 64% and sensitivity of 92%, and MRA had slightly lower values of 62% and 84%.15 No such comparison studies have been performed in children, and consultation with a pediatric radiologist is always recommended to decide which imaging study may be best, always considering that angiography may ultimately still be necessary if there is a high level of suspicion for renal artery stenosis. Echocardiography is necessary in any child suspected of having a structural lesion causing hypertension. In addition, echocardiography may be a sensitive tool to measure cardiac changes such as left ventricular hypertrophy that accompanies sustained hypertension, and can be used over time to document the effect of antihypertensive therapy on end-organ damage. Similarly, in some children with hypertension, formal ophthalmology examinations may provide initial information about the chronicity of a child’s hypertensive state and help document amelioration of vascular changes with therapy.

TREATMENT Acute Management Because hospitalization is an anxiety-producing experience for most children, if the blood pressure is elevated but lower than the 99th percentile, it is often best to monitor it with frequent readings to document the trend of measurements. As the child recovers and acclimates to the hospital, blood pressure often falls to normal ranges. If there is concern that the blood pressure may adversely affect a clinical condition or is directly related to a primary disease process (e.g. glomerulonephritis), more immediate measures may be necessary. If blood pressure readings are still consistently between the 90th and 99th percentiles by the time of discharge, it is prudent to arrange follow-up by the child’s primary care provider and further evaluation as an outpatient. In a child with sustained blood pressure exceeding the 99th percentile, diagnostic evaluation and therapy must be carefully considered. The tempo and urgency of the intervention are guided by the individual child’s clinical presentation and course.

Children with sustained blood pressure readings more than 30% above the 99th percentile are at particular risk of developing acute sequelae; even if they are asymptomatic, blood pressure control must begin immediately to prevent progression to a hypertensive urgency (severely elevated blood pressure that is potentially harmful but without evidence of end-organ damage or dysfunction) or emergency (severely elevated blood pressure associated with evidence of secondary end-organ dysfunction, such as hypertensive encephalopathy). Schemata outlining sequential approach to management of these conditions are found in Figures 26-2 and 26-3.

FIGURE 26-2. Algorithm for therapeutic intervention in a hypertensive emergency. See Table 26-1 for drug dosing. HTN, hypertension; BP, blood pressure; IV, intravenous.

FIGURE 26-3. Algorithm for therapeutic intervention in a hypertensive urgency. See Table 26-1 for drug dosing. BP, blood pressure; HTN, hypertension; IV, intravenous. Severe hypertension with actual acute end-organ dysfunction or impending end-organ dysfunction should be treated with short-acting intravenous antihypertensive medications. The medications most commonly used in a hypertensive crisis are outlined in Table 26-1. The blood pressure should be lowered by 20% to 30% in the first 2 to 3 hours. Once the blood pressure falls into a range that is not acutely dangerous for the patient, it should be lowered more gradually over the next few days, aiming eventually for pressures less than the 95th percentile for a patient’s age, gender, and height. TABLE 26-1

Medications for Pediatric Hypertensive Urgency or Emergency

Drug

Mechanism Dose

Onset

Duration

Hydralazine

Arteriolar dilator

IV: 0.1–0.4 5–15 mg/kg to max min dose of 20 mg

3–8 h

Labetalol

α- and (βadrenergic antagonist

Initial IV 5 min bolus: 0.25 mg/kg; repeat q15min at increasing doses up to 1 mg/kg until effective or to total dose of 4 mg/kg

2–6 h

Maintenance IV drip: 1–3 mg/kg/h Nitroprusside Venodilator and arteriolar dilator

IV: start at 0.5 1–2 min μg/kg/min

3–5 min

Nifedipine

Calciumchannel antagonist

PO: 0.25–0.5 mg/kg

10–20 min

3–6 h

Nicardipine

Calciumchannel antagonist

IV: 0.5–5 μg/kg/min

10 min

2–6 h

Esmolol

β-adrenergic antagonist

Loading dose: Seconds 10–20 min 500 μg/kg over 2 min Maintenance IV drip: 50– 250 μg/kg/min

Enalaprilat

ACE inhibitor

IV: 5–10 μg/kg q 8–24 h

0.5–4 h

6h

ACE, angiotensin-converting enzyme.

Hydralazine is a commonly used vasodilator with a rapid onset that can often be titrated to achieve good blood pressure control. It is well tolerated and can be given readily in a non–intensive care unit setting. Nifedipine is another short-acting agent and has the advantage of oral administration. Its use in adults is rare owing to reports of myocardial infarction.16 The use of short-acting nifedipine in children is safe, although a higher incidence of adverse events was seen in patients with preexisting central nervous system disease.17 The sublingual route is somewhat controversial because accurate dosing is problematic since it requires aspiration of liquid from within a capsule; moreover, its absorption is erratic. Nicardipine and isradipine can be used for vasodilation as intravenous calcium channel blocker in cases where oral nifedipine or intravenous hydralazine cannot be considered. Labetalol and esmolol can also be used as intermittent intravenous medications if necessary. Severe hypertension refractory to intermittent therapy may require continuous infusions to reduce blood pressure to safe levels. Historically, nitroprusside and labetalol have been used most commonly in children. Both agents are shorter acting than hydralazine, and continuous infusions may be titrated to achieve specific target blood pressures. These medications require an intensive care unit setting. Labetalol can be administered via both continuous infusion and scheduled intravenous dosing. It is the only drug that is both an α- and β-adrenergic blocker. Nitroprussside is a vasodilator with a wide dosage range. The drug is photosensitive and must be protected from light. Its use is limited by the toxicity of its metabolites; nitroprusside is converted to cyanide by tissue sulfhydryl groups, and cyanide is converted to thiocyanate in the liver. In patients with liver disease, cyanide levels should be followed. Thiocyanate levels should be monitored in patients on nitroprusside for more than 72 hours and in patients with renal insufficiency. Thiocyanate toxicity manifests primarily as neurotoxicity and includes psychosis, blurred vision, confusion, weakness, tinnitus, and seizures. An early sign of cyanide toxicity includes metabolic acidosis; other signs of

toxicity include tachycardia, pink skin, decreased pulse, decreased reflexes, altered consciousness, coma, almond smell on breath, methemoglobinemia, and dilated pupils. Continuous infusions of the angiotensin-converting enzyme (ACE) inhibitor enalaprilat, the calcium-channel blocker nicardipine, or the βblocker esmolol have also been used in hypertensive children, and according to local experience may be used more routinely when continuous infusions are needed for hypertensive children. In most children who will require continuous infusions for a limited time, there is no specific advantage to using any of these therapies versus older therapies. Long-Acting Medications As blood pressure is controlled by intravenous medication, it is also important to initiate longer-acting oral antihypertensives to transition off infusions. Table 26-2 reviews the oral drugs used most often for antihypertensive therapy in pediatrics. The choice of antihypertensive agent should address the presumed underlying pathophysiology of the blood pressure perturbation. For instance, children with fluid overload related to glomerulonephritis should be treated with diuretics and vasodilators. Hyperreninemic hypertension due to renal parenchymal scarring should be treated with ACE inhibitors or angiotensin receptor blockers. TABLE 26-2

Drug

Long-Acting Antihypertensive Agents in Children Initial Dose (mg/kg/ day)

Maximal Dose (mg/ kg/day)

Dosing Frequency

Calcium channel antagonist Nifedipine

0.25

3

XL or SR forms, 2 times/day

Amlodipine

0.1

0.4

1–2 times/day

Isradipine

0.15

0.8, up to 20

1–2 times/day

mg/day

as SR

ACE inhibitor Captopril (neonate)

0.03–0.15

2

2–3 times/day

Captopril (child)

1.5

6

2–3 times/day

Enalapril

0.08

0.6, up to 40 mg/day

1–2 times/day

Lisinopril

0.07, up to 5 mg/day

0.6, up to 40 mg/day

Once a day

Benazepril

0.2, up to 10 mg/day

0.6, up to 40 mg/day

Once a day

Angiotension receptor blockers Losartan

0.7, up to 50 mg/day

1.4, up to 100 mg/day

Once a day

Irbesartan

6–12 years: 75 mg/day

150 mg/day

Once a day

≥13 years: 150 mg/day

300 mg/day

Once a day

1–1% drop with an upper respiratory infection.27-36 The limited data available in children living at moderate altitudes suggest they may experience chronic borderline hypoxemia and even more significant intermittent desaturations.37 There is inconclusive evidence with regard to adverse long-term consequence

of transient mild hypoxemia or intermittent SpO2 desaturation events in otherwise normal infants and children.38-42

SPECIAL CONSIDERATIONS Special cases of hypoxemia include variant hemoglobins and the dyshemoglobinemias, methemoglobinemia, and carboxyhemoglobinemia. Several hemoglobin variants have been identified that either have altered peak light absorption for oxyhemoglobin or altered oxyhemoglobin affinity resulting in falsely low SpO2 readings when tissue O2 delivery is normal. Examples include hemoglobins Bonn, Venusburg, Titusville, M, Lansing, Rothschild, Lake Tapawingo, and others. Unnecessary evaluations are common in undiagnosed patients. Anomalous hemoglobin should be considered in a child without clinical evidence of hypoxemia or hypoxia, but an unexpectedly low SpO2. An arterial blood gas can assist in the differential. Diagnostic evaluation may require hemoglobin electrophoresis, high performance liquid chromatography, hemoglobin gene sequencing, and spectral photometry.43,44 Methemoglobin is hemoglobin with the iron in the ferric state, which is unable to bind O2 and shifts the oxyhemoglobin dissociation curve to the left in the remaining hemoglobin molecules in the tetramer. Only about 1.5 g/dL methemoglobin in the circulation results in clinical cyanosis. A small amount of methemoglobin (1%) is normal, and there are cellular mechanisms to reduce iron back to the ferrous state, although these can be genetically absent (e.g. cytochrome b5 reductase deficiency). Exogenous agents such as nitrites, phenacetin, sulfonamides, lidocaine (including topical absorption45), and aniline dyes are known to cause methemoglobinemia. Pulse oximetry is not adequate to recognize methemoglobinemia; a blood gas sample (arterial, capillary, or venous) will confirm the diagnosis. In most cases, discontinuation of the offending agent is adequate treatment. Intravenous methylene blue acts as an electron acceptor in patients who are not glucose-6phosphate dehydrogenase (G6PD)-deficient (pretreatment screening is indicated in populations likely to be G6PD-deficient), and ascorbic acid may be useful in G6PD-deficient patients. In severe cases of methemoglobinemia, transfusion and hyperbaric O2 have been used.

Carboxyhemoglobin is hemoglobin with the iron bound to carbon monoxide (CO). CO binds with an affinity about 240 times stronger than O2, rendering the molecule unavailable to carry O2 until the complex dissociates. Carboxyhemoglobin also shifts the oxyhemoglobin dissociation curve leftward. Levels of 2% to 5% are normal, and levels of 5% to 10% are commonly observed in smokers. There are many sources of CO poisoning, including virtually any combustion process without proper venting, smoke inhalation, tobacco smoke exposure, and automobile exhaust. Carboxyhemoglobin is not differentiated from oxyhemoglobin by pulse oximetry. A blood gas sample (arterial, capillary, or venous) to measure SaO2 is required to determine the carboxyhemoglobin level. Treatment is O2, which competes with CO and reduces the binding half-life to about 90 minutes. Severe cases may need hyperbaric O2 treatment (see Chapter 184). KEY POINTS Hypoxemia is a low arterial O2 level and is one of four major categories of hypoxia. Clinically, pulmonary infection, chronic lung disease, or both are the most common causes of hypoxemia, with V/Q mismatch the most common pathophysiology. V/Q mismatch responds to O2 administration, which is the mainstay of treatment. The nuances of O2 delivery techniques need to be understood. Pulse oximetry can detect and monitor hypoxemia in most situations, and is adequate for titrating O2 therapy but has limitations that must be understood by the practitioner. Overreliance on and misunderstanding of pulse oximetry may lead to misdiagnosis, inappropriate evaluation and treatment, excess admissions, and delay in discharge. History, physical examination, and blood gas studies can add significantly to the understanding of a clinical presentation of hypoxemia.

SUGGESTED READINGS Silverman M, O’Callaghan CL eds. Practical Paediatric Respiratory Medicine. London: Arnold Publishing; 2001. West JB. Pulmonary Pathophysiology: The Essentials. Philadelphia, PA: Lippincott Williams & Wilkins; 2003. West JB. Respiratory Physiology. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2000.

REFERENCES 1. Mason. Murray and Nadel’s Textbook of Respiratory Medicine. 4th ed. Saunders; 2005. 2. Miller R. Oxygen therapy. In: Anesthesia. 5th ed. New York: Churchill Livingstone 2000:715-718. 3. Weiskopf RB, Toy P, Hopf HW, et al. Acute isovolemic anemia impairs central processing as determined by P300 latency. Clin Neurophysiol. 2005;116:1028-1032. 4. Weiskopf RB, Aminoff MJ, Hopf HW, et al. Acute isovolemic anemia does not impair peripheral or central nerve conduction. Anesthesiology. 2003;99:546-551. 5. Lieberman JA, Weiskopf RB, Kelley SD, et al. Critical oxygen delivery in conscious humans is less than 7.3 ml O2 x kg(-1) x min(-1). Anesthesiology. 2000;92:407-413. 6. Buchta RM, Bickerton R. A case of profound iron deficiency anemia owing to corrosive esophagitis in a 20-year-old developmentally delayed male. J Adolesc Health. 1994;15:592-594. 7. Snider HL. Cyanosis. In: Walker HK, Hall WD, Hurst JW, eds. Clinical Methods: The History, Physical, and Laboratory Examinations. Boston, MA: Butterworth; 1990. 8. Snider HL, Roy TM. Deoxyhaemoglobin concentrations in the detection of central cyanosis. Thorax. 1988;43:801. 9. Goss GA, Hayes JA, Burdon JG. Deoxyhaemoglobin concentrations in the detection of central cyanosis. Thorax. 1988;43:212-213.

10. Scrase E, Laverty A, Gavlak JC, et al. The Young Everest Study: effects of hypoxia at high altitude on cardiorespiratory function and general well-being in healthy children. Arch Dis Child. 2009;94:621-626. 11. Wilson WC, Shapiro B. Perioperative hypoxia. The clinical spectrum and current oxygen monitoring methodology. Anesthesiol Clin North America. 2001;19:769-812. 12. Zhang L, Mendoza-Sassi R, Santos JC, Lau J. Accuracy of symptoms and signs in predicting hypoxaemia among young children with acute respiratory infection: a meta-analysis. Int J Tuberc Lung Dis. 2011;15:317-325. 13. Rojas MX, Granados Rugeles C, Charry-Anzola LP. Oxygen therapy for lower respiratory tract infections in children between 3 months and 15 years of age. Cochrane Database of Syst Rev (Online). 2009:CD005975. 14. Mower WS, Sachs C, Nicklin EL, Baraff LJ. Pulse oximetry as a fifth pediatric vital sign. Pediatrics. 1997;99 681-686. 15. Sandweiss DR, Corneli HM, Kadish HA. Barriers to discharge from a 24-hour observation unit for children with bronchiolitis. Pediatr Emerg Care. 2010;26:892-896. 16. Schroeder AR, Marmor AK, Pantell RH, Newman TB. Impact of pulse oximetry and oxygen therapy on length of stay in bronchiolitis hospitalizations. Arch Pediatr Adolesc Med. 2004;158:527-530. 17. Cunningham S, McMurray A. Observational study of two oxygen saturation targets for discharge in bronchiolitis. Arch Dis Child. 2012;97:361-363. 18. Corneli HM, Zorc JJ, Holubkov R, et al. Bronchiolitis: clinical characteristics associated with hospitalization and length of stay. Pediatr Emerg Care. 2012;28:99-103. 19. Shay DK, Holman RC, Roosevelt GE, Clarke MJ, Anderson LJ. Bronchiolitis-associated mortality and estimates of respiratory syncytial virus-associated deaths among US children, 1979-1997. J Infect Dis. 2001;183:16-22. 20. Shay DK, Holman RC, Newman RD, Liu LL, Stout JW, Anderson LJ. Bronchiolitis-associated hospitalizations among US children, 1980-1996. JAMA. 1999;282:1440-1446.

21. Gerstmann D, Berg R, Haskell R, et al. Operational evaluation of pulse oximetry in NICU patients with arterial access. J Perinatol. 2003;23:378-383. 22. Langhan ML, Chen L, Marshall C, Santucci KA. Detection of hypoventilation by capnography and its association with hypoxia in children undergoing sedation with ketamine. Pediatr Emerg Care. 2011;27:394-397. 23. Kannikeswaran N, Chen X, Sethuraman U. Utility of endtidal carbon dioxide monitoring in detection of hypoxia during sedation for brain magnetic resonance imaging in children with developmental disabilities. Paediatr Anaesth. 2011;21:1241-1246. 24. Deitch K, Miner J, Chudnofsky CR, Dominici P, Latta D. Does end tidal CO2 monitoring during emergency department procedural sedation and analgesia with propofol decrease the incidence of hypoxic events? A randomized, controlled trial. Ann Emerg Med. 2010;55:258-264. 25. Myers T. AARC Clinical Practice Guideline: selection of an oxygen delivery device for neonatal and pediatric patients—2002 revision & update. Respir Care. 2002;47:707-716. 26. Gibson RC, PB, Beckham RW, McGraw CP. Actual tracheal oxygen concentrations with commonly used oxygen equipment. Anesthesiology. 1976;44:71-73. 27. Hunt CE, Corwin MJ, Weese-Mayer DE, et al. Longitudinal assessment of hemoglobin oxygen saturation in preterm and term infants in the first six months of life. J Pediatr. 2011;159:377-383 e1. 28. Beresford MW, Parry H, Shaw NJ. Twelve-month prospective study of oxygen saturation measurements among term and preterm infants. J Perinato.l 2005;25:30-32. 29. O’Brien LM, Stebbens VA, Poets CF, Heycock EG, Southall DP. Oxygen saturation during the first 24 hours of life. Arch Dis Child Fetal Neonatal Ed. 2000;83:F35-38. 30. Hunt CE, Corwin MJ, Lister G, et al. Longitudinal assessment of hemoglobin oxygen saturation in healthy infants during the first 6 months of age. Collaborative Home Infant Monitoring Evaluation (CHIME) Study Group. J Pediatr. 1999;135:580-586.

31. Poets CF, Stebbens VA, Lang JA, O’Brien LM, Boon AW, Southall DP. Arterial oxygen saturation in healthy term neonates. Eur J Pediatr. 1996;155:219-223. 32. Hunt CE, Hufford DR, Bourguignon C, Oess MA. Home documented monitoring of cardiorespiratory pattern and oxygen saturation in healthy infants. Pediatr Res. 1996;39:216-222. 33. Masters IB, Goes AM, Healy L, O’Neil M, Stephens D, Harris MA. Age-related changes in oxygen saturation over the first year of life: a longitudinal study. J Paediatr Child Health. 1994;30:423-8. 34. Poets CF, Stebbens VA, Samuels MP, Southall DP. Oxygen saturation and breathing patterns in children. Pediatrics. 1993;92:686-690. 35. Stebbens VA, Poets CF, Alexander JR, Arrowsmith WA, Southall DP. Oxygen saturation and breathing patterns in infancy. 1: Full term infants in the second month of life. Arch Dis Child. 1991;66:569-573. 36. Mok JY, McLaughlin FJ, Pintar M, Hak H, Amaro-Galvez R, Levison H. Transcutaneous monitoring of oxygenation: what is normal? J Pediatr. 1986;108:365-371. 37. Thilo EH, Park-Moore B, Berman ER, Carson BS. Oxygen saturation by pulse oximetry in healthy infants at an altitude of 1610 m (5280 ft). What is normal? Am J Dis Child. 1991;145:1137-1140. 38. Ibuki K, Watanabe K, Yoshimura N, et al. The improvement of hypoxia correlates with neuroanatomic and developmental outcomes: comparison of midterm outcomes in infants with transposition of the great arteries or single-ventricle physiology. J Thoracic Cardiovasc Surg. 2012;143:1077-1085. 39. Zhang J, Liu H, Yan X, Weng X. Minimal effects on human memory following long-term living at moderate altitude. High Alt Med Biol. 2011;12:37-43. 40. Urschitz MS, Eitner S, Guenther A, et al. Habitual snoring, intermittent hypoxia, and impaired behavior in primary school children. Pediatrics. 2004;114:1041-1048. 41. Bass JL, Corwin M, Gozal D, et al. The effect of chronic or intermittent hypoxia on cognition in childhood: a review of the evidence. Pediatrics. 2004;114:805-816.

42. Oates RK, Simpson JM, Cartmill TB, Turnbull JA. Intellectual function and age of repair in cyanotic congenital heart disease. Arch Dis Child. 1995;72:298-301. 43. Zur B, Bagci S, Ludwig M, Stoffel-Wagner B. Oxygen saturation in pulse oximetry in hemoglobin anomalies. Klin Padiatr. 2012;224:259265. 44. Mounts J, Clingenpeel J, White N, Villella A. Apparent desaturation on pulse oximetry because of hemoglobinopathy. Pediatr Emerg Care. 2010;26:748-749. 45. Dahshan A, Donovan GK. Severe methemoglobinemia complicating topical benzocaine use during endoscopy in a toddler: a case report and review of the literature. Pediatrics. 2006;117:e806-809.

CHAPTER

29

Irritability and Intractable Crying Karen E. Schetzina

BACKGROUND Irritability and intractable crying may be the presenting complaint for a wide range of medical problems in infants and children, some of which are potentially serious. Recently it has been estimated that 5% of infants presenting to the emergency department with crying have a serious underlying illness and two-thirds of these cases may be identified through careful history and physical examination.1 Symptoms may also begin after hospitalization. Hospital providers must be able to differentiate significant irritability and intractable crying from developmentally appropriate crying. They must be familiar with the common causes of irritability and intractable crying as well as the more unusual causes and a stepwise approach to evaluation. Irritability is a state of increased sensitivity to stimuli; it may also be described by parents as fussiness, whining, or increased crying. Crying is the primary way that infants and young children express hunger, thirst, fear, fatigue, desire for attention, and discomfort or pain. When caregivers have taken the usual measures to address these common needs, such as feeding and holding the child and changing the diaper, yet the child continues to cry, the child is said to be inconsolable or to have intractable crying. The quantity as well as the quality of crying behavior should be considered. What qualifies as excessive crying varies based on the age and developmental level of the child, as well as the clinical scenario. Normal infants cry most during the first 3 months of life; during this period, serious illness may present with few or only subtle signs and symptoms, making evaluation in this age group particularly challenging. Crying should also be evaluated to determine whether it is appropriate for

the clinical scenario. For example, a febrile infant with a viral upper respiratory infection is likely to be irritable and may cry more than usual. However, crying with movement of the child’s lower extremities should lead to suspicion of an alternative cause, such as meningitis or a septic hip joint. Stranger anxiety—which appears at around 8 to 9 months of age, peaks at 12 to 15 months, and decreases thereafter—may manifest as inconsolable crying during examination; however, an otherwise healthy child should be comforted and calmed in the arms of a caregiver. A change in the character of a child’s cry may also be significant: louder, higher pitch, or more urgent tone or a weak, stridulous, or hoarse cry may suggest the presence of illness.

PATHOPHYSIOLOGY Irritability may result from pain, discomfort, or fatigue, direct neurologic insult, or altered metabolic or endocrine status. Crying in infants and young children is an involuntary action that serves physiologic and protective purposes. A newborn’s first cries enable essential changes in the cardiorespiratory system during the transition to postnatal life. Crying increases to almost 3 hours per day, on average, by 6 weeks of life and decreases thereafter. During this period of greatest crying, infants cry most during the late afternoon and evening hours, perhaps to release tension accumulated throughout the day from internal and external stimuli and maintain homeostasis. Crying is also a way for infants and children to express their emotional needs. DEFINITIONS Irritability: A state of increased sensitivity to stimuli. Intractable crying: Crying that persists despite usual efforts to comfort.

PATIENT HISTORY It is important to obtain a detailed description of the irritability and crying, as

delineated in Table 29-1. Note history of associated symptoms, including fever, malaise, feeding difficulty, respiratory symptoms, vomiting or bilious vomiting, stool patterns and composition, parents’ perception of the source of pain, limping or joint irritability, skin irritation or redness, poor weight gain, and developmental delay or regression. TABLE 29-1

Elements of the History Relevant to Intractable Crying

Normal or baseline crying behavior Duration of presenting crying episode Quality of crying Intensity Character Pitch Characteristics of crying episodes Duration of previous crying episodes Time of day Circumstances or triggers Efforts of caregivers to console child Conditions that make crying better or worse Associated symptoms Exposures, including recent immunizations Caregivers’ ideas regarding causes Normal and recent feeding patterns Normal and recent sleep patterns Growth pattern Birth history, including gestational age and any history of in-utero drug exposure Developmental history Past medical and surgical history Medications

Allergies

PHYSICAL EXAMINATION When examining the child, it is often helpful to observe respiration, movement, and behavior from across the room at first. This provides useful information and helps eliminate the confounding influences of stranger anxiety. With a frightened toddler, the parents can assist in the physical examination by exposing areas of skin and moving the extremities to check for tenderness while the examiner stands back several feet and observes. Use of distraction techniques can facilitate a complete physical examination. It is essential to examine the child from head to toe, fully exposed (removing clothes, shoes, socks, bracelets, barrettes, and so forth). Growth parameters, including head circumference in infants, should be assessed. Because of the extensive differential diagnosis, a stepwise approach is essential. The presence of fever makes an infectious cause more likely; other inflammatory processes and endocrine disorders must also be considered. It is important to remember that serious bacterial infections can be present in the absence of fever, particularly in neonates, and may also coexist with more benign conditions such as viral upper respiratory tract infections. Most common causes of irritability and intractable crying in afebrile patients are apparent after a careful history and physical examination. A period of observation may also be helpful. Bruising in perambulatory infants, particularly over the ears, facial injuries, limb swelling, or other signs of unexplained injury should raise concern for abuse. Evaluation for the presence of rib bruising or crepitus, a bulging fontanelle, and retinal hemorrhages should be included. Care must be taken not to routinely attribute irritability and crying to otitis media. In older infants with intact tympanic membranes, determining whether otitis media observed on physical examination is the cause of the symptoms can be accomplished by placing anesthetic drops in the affected ear and observing the patient’s response. If the irritability does not dramatically improve, another cause should be sought. Scrotal swelling that may or may not be tender may be seen with incarcerated inguinal hernia. Parents may not notice the presence of a hair tourniquet around an appendage such as a digit, penis, or clitoris, which can

cause swelling, ischemia, and pain. The band of hair may not be visible if it is buried beneath a fold of edematous tissue. In some cases, the hair may be so tightly wound that it is difficult to release, making it necessary to remove the hair. Applying a hair removal cream, such as Nair, to the affected area for about 10 minutes may dissolve the hair and relieve the constriction. Ocular abnormalities, including corneal abrasion and acute glaucoma, are difficult to discern by history or physical examination, because tearing and conjunctival injection are difficult to appreciate in the setting of excessive crying. If suspected, consider a topical anesthetic, fluorescein stain, ultraviolet light examination of both eyes, and lid eversion to rule out corneal abrasion or foreign body. Tonometry with evidence of increased intraocular pressure indicates acute glaucoma.

DIFFERENTIAL DIAGNOSIS If a child continues to cry inconsolably during a period of observation after the initial examination and no reasonable cause has been identified, admission for further evaluation and observation should be considered, because a serious occult condition may be present. At this point it is essential for the clinician to entertain an extensive differential diagnosis, focusing first on potentially serious or life-threatening conditions. The presence of tachypnea may suggest metabolic acidosis, respiratory tract infection, or cardiac dysfunction. Foreign body aspiration or other airway obstruction may produce symptoms of respiratory distress such as cyanosis, increased work of breathing, diaphoresis, drooling, stridor, wheezing, or rales. Evidence of recent trauma ascertained either by history or on physical examination should be investigated further. Evidence of injury in the absence of a history supporting a reasonable mechanism of injury is a red flag. An assessment of the family environment and social stressors and the observation of interactions among family members are also important to identify possible non-accidental trauma, such as shaken baby syndrome (see Chapter 40). Symptoms of altered sensorium, such as increased somnolence, lethargy, confusion, or agitation, may suggest central nervous system infection or injury, a metabolic disorder, drug ingestion or withdrawal, toxin exposure, or

cardiopulmonary failure. It is important to inquire specifically about toxins and medications (prescription, nonprescription, and traditional or homeopathic) that the child may have ingested or been exposed to. Loss of developmental milestones is suggestive of a metabolic or neurodegenerative disorder. Constipation is a common problem that can cause irritability. Further investigation of symptoms of gastrointestinal obstruction should be done expeditiously to evaluate for ischemia-inducing gastrointestinal disorders. Screaming attacks are a common presenting sign of intussusception, and are particularly concerning if accompanied by vomiting and/or lethargy. Crying associated with bilious emesis in the young infant is midgut volvulus, a surgical emergency, until proven otherwise Table 29-2 provides a list of causes to consider in the evaluation of prolonged irritability or intractable crying. Table 29-3 provides a list of conditions associated with recurrent irritability or crying episodes. TABLE 29-2

Causes of Irritability and Intractable Crying

General Infection Dehydration Anemia Leukemia Kawasaki syndrome Eyes, ears, nose, and throat Corneal abrasion Ocular foreign body Glaucoma Otitis media Thrush, herpangina, herpes stomatitis Teething Nasal, oropharyngeal, or otic foreign body Cardiovascular and respiratory

Supraventricular tachycardia Myocardial ischemia or infarction Congestive heart failure or congenital heart defect Respiratory distress or failure Gastrointestinal Incarcerated hernia Spontaneous perforation of bile duct Appendicitis Malrotation, volvulus, intussusception Henoch-Schönlein purpura Bowel perforation Constipation Anal fissure Genitourinary Testicular torsion Urinary tract obstruction Urethritis Meatal ulcer Sexual abuse Endocrine and metabolic Congenital adrenal hyperplasia Hyperthyroidism Syndrome of inappropriate antidiuretic hormone Hypoglycemia Electrolyte abnormalities (sodium, calcium, potassium) Hyperammonemia Metabolic acidosis Pheochromocytoma Reye syndrome Neurologic Central nervous system injury, hemorrhage, tumor, shaken baby

syndrome Meningitis, encephalitis, brain abscess Pseudotumor cerebri or hydrocephalus Migraine Spontaneous epidural hematoma Drugs and toxins Neonatal abstinence or other withdrawal syndromes Fetal alcohol syndrome Poisoning (e.g. lead, mercury, iron, carbon monoxide) Monosodium glutamate reaction Drugs (e.g. antihistamines, atropinics, phenothiazines, pseudoephedrine, albuterol, theophylline or other methylxanthines, metoclopramide, promethazine, barbiturates, tricyclic antidepressants, vitamin A, vitamin D, salicylates, amphetamines, cocaine, phencyclidine) Vaccine reaction Skin Hair tourniquet Insect, arachnid, or arthropod bite or sting Burn Herpes dermatitis (pain may precede vesicles) Paronychia Atopic dermatitis, acrodermatitis enteropathica, or other pruritic conditions Orthopedic Fracture Acute arthritis (infectious, postinfectious, inflammatory) Nursemaid’s elbow or joint dislocation Diskitis Osteomyelitis

TABLE 29-3

Syndromes and Conditions Associated with Chronic or Recurrent Irritability or Intractable Crying

Anomalous origin of the coronary artery Congenital heart disease (e.g. “tet” spells of tetralogy of Fallot) Gastroesophageal reflux, Sandifer syndrome Colic Milk protein intolerance Lactose intolerance Nutritional deficiencies (e.g. iron, zinc, vitamin C) Diabetes insipidus Sickle cell disease Metabolic and neurodegenerative disorders (e.g. glutaric aciduria type I, acute intermittent porphyria, Pompe disease, pyridoxine deficiency, tryptophan malabsorption, hypoglycinemia, arginemia, tyrosinemia, pyruvate carboxylase deficiency, biotin deficiency, argininosuccinate lyase deficiency, Krabbe disease, lipogranulomatosis) Genetic syndromes (e.g. Smith-Lemli-Opitz, Williams, de Lange) Unrecognized deafness Behavioral problems (e.g. attention-deficit hyperactivity disorder) Parent–child interactions, problems, or stress Mental illness (e.g. depression, bipolar disorder) Rheumatologic disorders (e.g. juvenile rheumatoid arthritis) Infantile cortical hyperostosis (Caffey disease)

DIAGNOSTIC EVALUATION There are no standard screening laboratory tests, with the possible exception of urinalysis and culture in females and uncircumcised males younger than 12 months and circumcised males younger than 6 months. Laboratory and radiographic evaluation should be guided by findings from the history and

physical examination.

MANAGEMENT A multidisciplinary, family-centered approach to care is recommended given how stressful irritability and intractable crying in infants and children can be to families. The opportunity to educate and advise parents on the care of their child should not be disregarded. Achieving a careful balance of not dismissing concerns as a benign condition without proper evaluation yet avoiding unnecessary testing or medical intervention can be challenging.

SPECIAL CONSIDERATIONS One in five infants may struggle with excessive crying or regulatory problems and these infants may be at increased risk of child abuse and future behavior problems in childhood.2,3 Mothers of these infants are at increased risk of postpartum depression and early breastfeeding cessation.4,5

COLIC, GASTROINTESTINAL DISTURBANCES, AND FEEDING PROBLEMS Clinicians should be familiar with the clinical features of colic—a common cause of excessive and intractable crying in infants—so that they can differentiate it from other conditions. Colic is characterized by episodes of inconsolable crying in an otherwise healthy infant and is a diagnosis of exclusion. The timing of colic parallels the normal peak of infant crying, usually beginning during the third week of life and resolving by 3 or 4 months of age. Colic is commonly defined by the “rule of threes”: crying for more than 3 hours per day, on more than 3 days per week, in an infant 3 months of age or younger. The crying episodes tend to occur suddenly during the late afternoon and evening hours and are often described as more intense than the infant’s usual crying. The episodes may be associated with facial flushing, abdominal distention, and increased tone. Colic likely stems from different causes in different infants. Proposed causes include immaturity of the nervous system, impaired intestinal absorption or motility, or a reflection of temperament or parent–child

interactions. Parental education, reassurance, and support are important aspects of management, and efforts to soothe the infant and limit air swallowing, improve burping, and minimize stimulation may be helpful. Medications such as simethicone may be used by parents, but use is not supported in trials. Colic can be extremely stressful for families and may have long-term effects on the parent–child relationship. Therefore, it requires individualized management and close outpatient follow-up. Excessive crying beyond 3 months of age might raise suspicion for a gastrointestinal disorder. While crying in infants is frequently attributed to gastroesophageal reflux disease (GERD) by both parents and clinicians, objective evidence that GERD underlies symptoms or that acid-suppressive medications are beneficial in this group are lacking. Food allergies, primarily cow milk protein allergy in infants, may present in both formula-fed and breastfed infants with irritability, eczema, bloody stools, and less commonly, vomiting, diarrhea, feeding difficulties, or failure to thrive. Protocols for allergen-eliminated diets in breastfeeding mothers have been published, and some evidence suggests that use of hypoallergenic formula may reduce crying in formula-fed infants.6,7 Congenital lactase deficiency is extremely rare, although secondary lactose intolerance may result from damage to intestinal villa, such as from gastroenteritis. Probiotics or use of lactose-free formula in non-breastfed infants may have a role in managing symptoms. Functional lactose overload in breastfed infants may produce excessive crying accompanied by poor satiety and frothy or explosive stools as a result of low milk fat content and rapid gastrointestinal transit, such as in instances of oversupply. Lactation consultation may be helpful in such instances.

NEW IRRITABILITY OR EXCESSIVE CRYING IN A HOSPITALIZED PATIENT Although the approach to irritability or inconsolability that develops in a hospitalized patient is similar, additional causes must be considered. Dietary, environmental, and sleep–wake schedule changes should be examined. For example, restricting oral intake before procedures could result in crying due to hunger. Patients with prolonged exposure during examinations and monitoring may become too cold, or infants under warmers may become

overheated. Intravenous catheter sites should be inspected for any evidence of thrombophlebitis, and the locations of other catheters, tubes, and probes should be examined for skin irritation, excessive traction, and infection. Adverse effects and interactions of medications started in the hospital should also be considered as possible contributors. Appropriate prevention and treatment of procedural pain must also be considered, including use of breastfeeding/breast milk, sucrose, and non-nutritive sucking in infants.8 KEY POINTS Crying that is concerning to parents is prevalent in infancy and a frequent reason for seeking care. A careful history and physical examination should identify most serious underlying causes of irritability and crying. Diagnostic examinations should be directed based on results of the history and physical.

REFERENCES 1. Freedman SB, Al-Harthy N, Thull-Freedman J. The crying infant: diagnostic testing and frequency of serious underlying disease. Pediatrics. 2009;123:841. 2. Reijneveld SA. Can der Wal MF, Brugman E, et al. Infant crying and abuse. Lancet. 2004;364(9442):1340-1342. 3. Hemmi MH, Wolke D, Schneider S. Associations between problems with crying, sleeping, and/or feeding in infancy and long-term behavioral outcomes in childhood: a meta-analysis. Arch Dis Child. 2011:96(7):622-629. 4. Howard CR, Lanphear N, Lanphear BP, et al. Parental responses to infant crying and colic: the effect on breastfeeding duration. Breastfeed Med. 2006;1:146-155. 5. Vik T, Grote V, Escribano J, et al. Infantile colic, prolonged crying, and maternal postnatal depression. Acta Paediatr. 2009;98:1344-1348.

6. Academy of Breastfeeding Medicine. ABM clinical protocol # 24: allergic proctolitis in the exclusively breastfed infant. Breastfeed Med. 2011;6(6):435-440. 7. Douglas PS, Hiscock H. The unsettled baby: crying out for an integrated, multidisciplinary primary care approach. MJA. 2010;193(9):533-536. 8. Shah PS, Herbozo C, Aliwalas LL, et al. Breastfeeding or breast milk for procedural pain in neonates (Review). Cochrane Database Syst Rev. 2012;12:1-24.

CHAPTER

30

Limp Robert Sundel

BACKGROUND Musculoskeletal complaints account for a significant number of outpatient visits to the pediatrician—up to 10% of non–well-child appointments in some studies. Only a minority of these visits result in hospitalization, but in many cases, even these admissions could have been avoided if a logical, stepwise approach to evaluation and management had been used. This chapter focuses on the entities to consider in a limping child and the appropriate approaches to take with regard to the history, physical examination, and diagnosis.

PATHOPHYSIOLOGY The normal gait is the most efficient and stable means for humans to walk upright on two legs. For this gait to be altered, strong countervailing forces must be applied. These may be anatomic (e.g. broken bone, muscular weakness), neurologic (disrupted proprioception or balance), or nocioceptive in nature. Pain is the most common cause of an abnormal gait in children. Recognizing specific gait aberrations can facilitate the localization and identification of musculoskeletal pathology. For example, a psoas abscess is often difficult to diagnose because of poor localization of discomfort within the pelvis; pain may be perceived as occurring anywhere from knee to the diaphragm. Trying to walk with a psoas abscess, however, results in a characteristically altered gait, as contraction of the psoas muscle is avoided to minimize discomfort. This causes leaning to the involved side and using the bones of the pelvis and upper leg to substitute for the support usually provided by the psoas muscle. Further, the contralateral hemipelvis dips to keep the psoas muscle relaxed and thus avoid pain. The result is the

Trendelenburg gait, which can be caused only by a pathologic condition involving the proximal femur or muscles of the pelvis (Figure 30-1). Analogous effects of other sites of pathology on gait are listed in Table 30-1.

FIGURE 30-1. Trendelenburg gait, with pathology of the right proximal femur or adjacent muscles causing characteristic dipping of the contralateral hemipelvis.

TABLE 30-1

Characteristic Alterations in Gait Based on Location of Pathology

Location Effect on Gait Example

Result

Hip

Refusal to bear weight

Decreased or Avascular eliminated swing necrosis of phase femoral head Inguinal tendinitis

Circumduction or dragging of involved side

Knee

Decreased extension > flexion

Lyme arthritis

Stiff-kneed gait

Ankle

Decreased dorsiflexion

Chondrolysis

Ginger gait, like walking on coals

PATIENT HISTORY The key elements of the medical history that help identify the cause of a limp include the timing of the symptoms, the nature of the pain with regard to alleviating and exacerbating factors, particularly response to activity (Table 30-2), and the character of the pain, such as dull, sharp, radiating, or burning. TABLE 30-2

Characteristics of Musculoskeletal Pain

Pain Type

Morning Afternoon Nighttime Activity

Inflammatory (e.g. arthritis)

+++

+

–

Improves

Mechanical (e.g. overuse syndromes)

+/–

++

–

Worsens

Bone (e.g. tumors)

++

Neuropathic (e.g. + neuritis)

++

++

No change

++

+++

No change

While pain is the most common cause of a limp in children, it is not the only cause. When children do complain of pain, it may originate in sensory nerves of the skin, soft tissues, muscles, bone, or nerves. Joint nocioceptors found in the joint capsule and adjacent connective tissue are activated only by extreme mechanical or chemical irritation. This high threshold for sensing joint pain likely accounts for the fact that up to 80% of children with arthritis do not report pain. For example, an Oklahoma study found that children with arthritis typically presented with stiffness, avoidance of activities, or regression of skills, but only 13 of 226 children referred to a rheumatology clinic with joint pain actually had arthritis.1

INFLAMMATORY SYMPTOMS The most characteristic feature of discomfort related to inflammatory processes is the classic morning stiffness of arthritis. Difficulty may also be reported after other periods of inactivity, such as long car rides or sitting in school. Inflamed synovial joint tissue produces less hyaluronic acid, which causes gelling of joint fluid and decreased lubrication. This can be reversed by warming of the joint, returning the synovial fluid to the liquid state and once again permitting efficient, low-friction movement. Thus children with arthritis typically feel better after a warm bath or after several minutes of activity. These children may suffer joint stiffness in the morning but be quite comfortable exercising strenuously later in the day. Cold, damp weather or swimming in cool water also tends to disturb children with arthritis. Nighttime awakening is unusual with arthritis. Any atypical symptoms— especially nighttime pain or discomfort with activity—should raise the suspicion of an alternative diagnosis, even in the setting of otherwise typical signs of joint inflammation.

MECHANICAL SYMPTOMS

The timing of symptoms caused by mechanical factors is the mirror image of manifestations of inflammation (Figure 30-2). Children typically feel well in the morning, but become more uncomfortable with increasing activity. Like inflammation, however, mechanical pain generally does not awaken children from sleep. Rest and ice tend to alleviate mechanical symptoms. The precise type of overuse syndrome or injury causing a child’s symptoms can generally be determined from a careful history (e.g. Osgood-Schlatter syndrome in an adolescent male athlete, iliotibial band syndrome in an adolescent female runner) and physical examination.

FIGURE 30-2. Limp: diagnostic and treatment algorithm. ANA, antinuclear antibody; CBC, complete blood count; CF, cystic fibrosis; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; IBD, inflammatory bowel disease; NSAID, nonsteroidal anti-inflammatory drug; JIA, juvenile idiopathic (rheumatoid) arthritis; SLE, systemic lupus erythematosis; U/S, ultrasound.

BONE PAIN Pain originating in the osseous compartment tends to be constant and does not change significantly with activity. Bone pain raises concern for infection, trauma, and malignancy. This type of pain may awaken a child at night, particularly when it is related to leukemia or a tumor. Cytopenias are typically seen with leukemia, although a normal complete blood count does not exclude the possibility. Other tumors, such as sarcoma or metastatic neuroblastoma, are far less common but must be considered in children with significant nighttime pain. Osteomyelitis should be considered for patients with new onset of fever, localized pain, and decreased mobility; many patients will have a history of preceding trauma (see Chapter 70).

NEUROPATHIC PAIN Nerve pain tends to be worst at bedtime, when the usual distractions of daily activities abate and children are left to focus on their discomfort. In patients old enough to describe the sensation, neuropathic pain typically has a sharp, burning, or shooting character. It is also commonly associated with allodynia (a sense of discomfort from stimuli that normally do not cause pain) and hyperesthesia (a decreased threshold for discomfort in overlying tissues). Although joints may be involved, neuropathic pain generally encompasses extra-articular areas as well and can follow a dermatomal distribution. Activity does not have a significant effect on neuropathic pain. When nerve pathology due to severe trauma, tumor, or vasculitis cannot be identified, pain syndromes such as fibromyalgia or complex regional pain syndrome should be considered.

PHYSICAL EXAMINATION The four cardinal signs of inflammation (dolor, tumor, rubor, and calor) are the hallmarks of arthritis. The musculoskeletal examination should begin with careful observation of the extremities in a child who has removed his clothing and is robed in a gown. Whether assessing pain, swelling, changes in color, or variations in temperature, the most sensitive aspect of the examination is comparing the two sides of the body. Decreased extension of the right knee compared to the left, or an identifiably warmer or more

sensitive left ankle leads to rapid identification of likely pathology. Careful examination of all the joints is mandatory, even when the patient complains about a single joint, as the potential causes of a monoarticular process differ significantly from those of polyarticular conditions. Warmth and swelling are most characteristic of inflammatory arthropathies, while overlying erythema is more indicative of septic arthritis. An inflamed knee may exhibit a ballotable effusion, meaning that applying pressure directly to the patella forces it downward, displacing synovial fluid and causing a bounce against the femur. The characteristic springiness is not noted when fluid does not intervene between the patella and femur, as in healthy children. The joint may also be swollen from synovial proliferation, which has a boggier consistency than the free fluid of a joint effusion. Osteomyelitis most typically causes point tenderness on palpation of the involved bone, though in younger children precise localization of the discomfort can be more difficult. Careful examination of an inflamed joint may allow an estimation of the duration of the arthritis. Synovitis is characterized by increased blood flow, typically more pronounced in the portion of the joint compartment subjected to maximal force. In the knee, this is the medial aspect, where hyperemia leads to increased delivery of nutrients and accelerated growth. This may manifest initially as prominence of the medial femoral condyle, and later as genu valgus. Ultimately, the leg with the inflamed knee grows more rapidly, and a leg length discrepancy develops. The lower leg may bow to compensate for the greater length of the upper leg. At the same time, the knee loses extension and develops a flexion contracture, with resultant atrophy of the vastus medialis and wasting of the quadriceps muscle. In contrast, significant inflammation in the hip or the temporomandibular joint of the jaw often damages the growth plate and leads to shortening of the involved leg or hemimandible. Inflammation in other joints generally causes little demonstrable discrepancy in size.

DIFFERENTIAL DIAGNOSIS When a child presents with a complaint referable to the lower extremities, the list of possible causes is long and varied (Table 30-3). In the absence of an obvious explanation such as known trauma, it is helpful to start the evaluation by categorizing the type of pain or discomfort according to the nature of its

onset (acute vs. chronic), the number of joints involved, and whether extraarticular signs or symptoms such as fever or rash are present. Most normally active children have some history of trauma during the preceding 24 hours; however, unless the trauma is significant (e.g. football injury, automobile accident, bicycle fall), it is more likely to have unmasked a preexisting pathologic condition than to have caused damage to the child’s resilient tissues. TABLE 30-3

Common Causes of Arthritis

Monoarticular Acute onset Septic arthritis Reactive arthritis Trauma Hemophilia Lyme disease Chronic Juvenile rheumatoid arthritis Lyme disease Tuberculosis (rare without pulmonary disease) Tumor (pigmented villonodular synovitis most common, but rare) Polyarticular Juvenile rheumatoid arthritis Spondyloarthropathies Systemic autoimmune diseases Systemic lupus erythematosus Vasculitis Arthritis associated with inflammatory bowel disease Viral arthritis Reactive arthritis Serum sickness Rheumatic fever (migratory arthritis)

Malignancies Periodic fever syndromes

MONOARTHRITIS The potential causes of a monoarticular process can be narrowed by considering the acuity and duration of symptoms. Acute Onset When pain and swelling of a single joint start acutely, traumatic injury must always be considered. Documentation of antecedent trauma is helpful, but this may be difficult to elicit in young children who are unable to verbalize the specifics of the history. Routine daily activities may cause hemarthrosis in patients with an underlying bleeding disorder, but minor injuries are unlikely to lead to a recognizable gait disturbance in otherwise healthy children. Bacterial infection also must be considered in children with sudden onset of a red, swollen, painful, or hot joint. When accompanied by fever, arthrocentesis for cell count and culture is generally indicated. Treatment of septic arthritis must not be postponed (in contrast, for most types of inflammatory arthritis, a delay in the diagnosis by days or weeks has few long-term implications). Postinfectious or “reactive” arthritis may involve one or many joints, but it characteristically causes less inflammation than that seen with acute infection. Nonetheless, while postinfectious arthritis usually does not cause erythema overlying the joint, it may cause significant discomfort or even excruciating pain. Reactive arthritis generally responds well to nonsteroidal anti-inflammatory agents and is typically transient. Lyme disease may also be difficult to distinguish clinically from septic arthritis, although it more commonly causes indolent symptoms. Subacute Onset The diagnostic considerations in cases of an isolated, chronically swollen joint differ from those related to an acute arthritis. Bacterial infection is far less likely, but low-grade infections, especially Lyme disease in endemic areas, must be excluded. Chronic monoarthritis also may be caused by Mycobacterium tuberculosis, particularly in immunocompromised children. This category also includes chronic forms of juvenile arthritis, especially pauciarticular juvenile idiopathic (rheumatoid) arthritis (JIA), psoriatic arthritis, and juvenile spondyloarthritides. Less

prevalent inflammatory arthropathies, such as arthritis due to sarcoidosis, may also cause monoarthritis. Tumors of the cartilage and synovium, though extremely rare, also typically present in an indolent manner. The most common of these, pigmented villonodular synovitis, generally causes a chronically painful and swollen knee. A nontraumatic arthrocentesis that yields bloody fluid suggests the possibility of an articular tumor.

POLYARTHRITIS When several joints have objective evidence of inflammation, rheumatologic conditions rise to the top of the differential diagnosis. Most common among these is polyarticular JIA, although other autoimmune diseases, such as systemic lupus erythematosus and vasculitis, typically involve multiple joints as well. Infection is progressively less common as more joints are involved, with the exception of gonococcal arthritis in sexually active or abused children and salmonella arthritis in immunocompromised patients. Arthritis associated with systemic conditions, such as inflammatory bowel disease or cystic fibrosis, must also be considered. Usually, extra-articular involvement (e.g. a new murmur in rheumatic fever, hives in serum sickness) offers a clue to these conditions. The pattern of joint involvement may also be suggestive: rheumatic fever, vasculitis, and serum sickness characteristically cause a migratory polyarthritis, whereas most other conditions cause additive or fixed involvement of multiple joints. Children with polyarticular arthritis are particularly likely to benefit from consultation with a pediatric rheumatologist.

DIAGNOSTIC EVALUATION LABORATORY STUDIES Laboratory studies may be helpful in excluding infectious and malignant causes of a swollen joint, but they cannot confirm a diagnosis of arthritis; a child may have JIA despite uniformly normal laboratory studies. Even when laboratory studies support a diagnosis of JIA, they are nonspecific. Ultimately, a diagnosis of JIA rests on the history and physical examination. The laboratory studies most characteristic of arthritis are those that reflect systemic inflammation: elevated erythrocyte sedimentation rate, C-reactive

protein, and platelet count. In general, the elevation of acute-phase reactants is proportional to the number of joints involved. In a case of monoarticular arthritis, therefore, normal laboratory studies are the rule. Leukocytosis may be present in JIA, particularly in children with systemic-onset JIA; a mild to moderate anemia may also be seen in the setting of chronic inflammation. The serum antinuclear antibody (ANA) level is usually measured when a child is being evaluated for possible arthritis. This is rarely diagnostic, although an ANA at a titer of 1:1024 or higher is strongly suggestive of an autoimmune condition. At lower titers, a positive ANA result is nonspecific, occurring in conditions from arthritis and lupus to viral illnesses. Thus up to 2% of children have a positive ANA at any point in time (typically low titer), and most are healthy. A positive ANA assay in a child with known arthritis is a marker for an increased risk of developing anterior uveitis. One of the most overused tests in children with swollen joints is the rheumatoid factor (RF), an autoantibody directed against the Fc portion of the immunoglobulin-G (IgG) molecule. Unlike adult rheumatoid arthritis, in which 80% of cases are associated with a positive RF result, only 2% of more than 400 children seen at a rheumatology clinic in Philadelphia had a positive RF result, and many of these were considered to be false positives.2 The only setting in which RF is helpful in children is in cases of polyarticular JIA; those with a positive RF are more likely to have a severe, erosive course, warranting more aggressive management.

RADIOGRAPHIC STUDIES Imaging studies are essential for evaluating children with musculoskeletal pathology. They may be particularly helpful in confirming a clinical impression of arthritis by excluding other causes of joint pathology and by showing the characteristic changes caused by longstanding joint inflammation. Plain radiographs are usually the first imaging modality used to evaluate a child with joint complaints. In the initial stages of arthritis, the radiographs are generally normal or may show nonspecific findings such as soft tissue swelling, joint effusion, periarticular osteopenia, or periosteal new bone formation.3 Radiographs may help rule out fractures or foreign bodies. It is important to remember, however, that bone demineralization or callus

formation takes up to 10 days to become visible on x-rays. In longer-standing arthritis, more severe changes are evident on plain radiographs. Joint space narrowing reflects cartilage destruction, and it may be accompanied by other signs of inflammatory damage, including bony erosions and subchondral cysts. Such changes may be evident after several weeks in the case of untreated articular infections, or after months to years in inflammatory conditions. Epiphyseal maturation also tends to be accelerated in JIA, leading to asymmetric growth. Thus bilateral films to compare symptomatic areas with those on the uninvolved side may be particularly helpful. Plain radiographs provide limited information on the soft tissues of joints, restricting their utility in the evaluation of inflammatory synovitis and soft tissue infections. Accordingly, other imaging modalities may be preferable, particularly for detecting and assessing early disease. Magnetic resonance imaging (MRI) is able to demonstrate soft tissue masses, cartilage thinning, meniscal changes, joint effusions, and popliteal cysts. The sensitivity of MRI increases significantly when performed with gadolinium, which accumulates in tissues with increased vascularity, including inflamed synovium.4 Use of MRI is limited primarily by its cost and by the need to sedate young children. Ultrasonography is also used to assess arthritic joints, and in the hands of experienced operators may be useful for detecting joint effusions, popliteal cysts, lymph nodes, and to some degree, changes in articular cartilage. Although MRI is more sensitive than ultrasonography for most purposes, the latter is less expensive, and ultrasound images can be acquired rapidly and without sedation.5

MANAGEMENT Treatment of a limping child depends on the cause. If a child is unable to ambulate and initial outpatient or emergency department screening does not confirm a diagnosis, admission is generally appropriate. In general, the goal of therapy is to exclude acutely dangerous conditions, prevent irreversible sequelae, and restore full functioning. This is particularly important in a growing child, in whom inflammation may result in permanent derangements in joint development and function.6 Conversely, a growing child is also far better able to heal damage if the pathologic process is completely suppressed,

increasing the incentive to rapidly control the underlying disease. Indeed, evidence suggests that the longer an autoimmune condition persists, the more resistant to therapy it becomes.7 Treatments for extremity abnormalities can be divided into those that relieve symptoms but do not prevent joint damage, and specific therapies that alter the biology of the process. Although symptomatic relief may or may not be necessary, depending on a child’s level of discomfort, disease control is essential for preventing chronic joint changes due to ongoing inflammation.8

SYMPTOMATIC THERAPIES First-line agents for relief of joint pain are acetaminophen and nonsteroidal anti-inflammatory drugs (NSAIDs) (Table 30-4). Although dozens of medications in this category are available, only a handful have been approved by the Food and Drug Administration for use in children. Ibuprofen is available over the counter as a suspension, so it is often prescribed for children. It should be given every 6 hours to optimize anti-inflammatory effects. Naproxen is more convenient because of its twice-daily dosing. Although all NSAIDs have potential gastrointestinal, hepatic, and renal toxicities, naproxen has an additional predilection for causing pseudoporphyria, especially in fair-skinned children. Up to 12% of pediatric patients develop this potentially scarring photosensitive eruption, so physicians should stress the need to use sunscreen. Parenteral NSAIDs such as ketorolac are significantly more expensive than oral anti-inflammatory agents, and offer no advantage for children who can take medications by mouth or nasogastric tube. TABLE 30-4

Dosages and Adverse Effects of Medications Used to Treat Juvenile Rheumatoid Arthritis

Drug Class

Typical Dosing

Side Effects

Nonsteroidal anti-inflammatory drugs Ibuprofen (Advil, Motrin)

10 mg/kg/dose every 6h

Gastric irritation, hepatotoxicity,

nephrotoxicity, headache, rash Naproxen (Aleve, Naprosyn)

10 mg/kg/dose every 12 h

Gastric irritation, hepatotoxicity, nephrotoxicity, headache, rash, pseudoporphyria

Diclofenac gel (Voltaren)

4 g of 1% gel to lower extremities, or 2 g to upper extremities to affected joint 4 times per day

Localized burning

Disease-modifying antirheumatic drugs Sulfasalazine (Azulfidine)

40–70 mg/kg/day divided into 2–3 doses

Gastrointestinal upset, aplastic anemia, photosensitive eruptions, StevensJohnson syndrome Contraindicated in children aged 2 cm), quality (matted or firm), and duration of swelling increase the possibility of malignancy. Focal oncologic processes such as soft tissue neoplasms may also mimic lymph node swelling. The location of the process plays an important role in determining whether congenital malformations and anatomic abnormalities are possible causes. In the head and neck region, midline thyroglossal duct cysts, lateral branchial cleft cysts, and dermoid cysts in a variety of locations may present with swelling, with or without superinfection; parotid or submental salivary gland dysfunction or infection may also be present. In the inguinal area, hydroceles and undescended testes or testicular torsion should be added to the differential diagnosis in males, whereas in females an entrapped ovary may present in the setting of a hernia. In either sex, entrapped intestine or omentum may be present in a hernia.5

DIAGNOSTIC EVALUATION

Laboratory testing is indicated if there is suspicion of a disease other than localized Staphylococcus or Streptococcus infection, thereby affecting treatment decisions. Bacteremia can occur in pediatric patients—particularly young patients with high fevers—with significant focal bacterial lymph node infections,1 so obtaining blood cultures may help identify a bacterial organism as well as its sensitivities. A complete blood count with leukocyte differential may be helpful to determine leukocytosis and indicate possible infectious mononucleosis or oncologic processes. Infectious mononucleosis is caused by EBV and commonly presents with atypical lymphocytosis, lymphadenopathy, and fever as well as exudative pharyngitis and hepatosplenomegaly.3 Initial testing for EBV may include heterophil antibody testing—most sensitive in patients older than 4 years—as well as EBV serum titers for immunoglobulins M and G; it is important to keep in mind that these tests are most sensitive after 2 to 3 weeks of illness.3 Cytomegalovirus infections can present similarly to EBV infections, so serum cytomegalovirus titers may be helpful. Depending on the differential diagnosis for a given patient, serologies for more unusual organisms may also be indicated. HIV testing should be performed if there is any history of HIV exposure or if the clinical history leads to suspicion of an underlying immune deficiency. In the setting of recurrent infections, an immunology consultation may be helpful in recommending further testing.1 If there is a history of travel to developing countries or other possible exposure to tuberculosis (TB), a Mantoux tuberculin purified protein derivative (PPD) test should be done. A negative PPD test should not be considered definitive in the setting of high clinical suspicion for TB, because 10% of immunocompetent children with culture-documented TB may have a negative PPD; this number is higher in immunocompromised patients and in those with disseminated TB.3 Atypical mycobacterial disease can sometimes cause a positive PPD.3 In the setting of a positive PPD test or supraclavicular adenopathy on examination, a chest radiograph can screen for pulmonary TB or other diseases, particularly oncologic processes in the chest, whose lymphatics may drain to a sentinel supraclavicular node. To visualize the presenting lesion itself—particularly if it is rapidly progressive, fluctuant, or close to vital structures—ultrasonography often provides distinct images of a superficial process without exposing the patient to radiation. If there is concern for a deeper or more widespread process, a

computed tomography scan may be more helpful. If the infection has a loculated component or an organized abscess, drainage may be therapeutic as well as diagnostic. Fine-needle aspiration, under ultrasound guidance if necessary, is a useful method of obtaining a fluid sample for bacterial, mycobacterial, and fungal stains and cultures. It is important to note that many organisms that cause lymphadenitis, especially mycobacteria, are fastidious and may not grow in culture.3 Incision for drainage can lead to a chronically draining sinus tract, particularly in the setting of a mycobacterial infection; in these cases, excisional biopsy is the preferred approach. If there is little concern about rare or resistant organisms and if a good response to antibiotic treatment alone is expected in a given patient, treatment is often initiated without obtaining microbiologic studies from the site of infection. When wound cultures cannot be obtained and concerns exist for resistant organisms, swabs from the nares, axilla, and/or rectum to detect for methicillin-resistant S. aureus (MRSA) colonization may be performed, though concordance of nasal carriage and infectious agent is not assured.6,7

MANAGEMENT After the initial assessment, which often occurs in a primary care setting, an important issue is whether a patient requires hospitalization. Possible criteria for hospital admission include toxic appearance, rapid progression of infection, airway involvement of a neck process, inability to tolerate enteral antibiotics or lack of clinical improvement on a prior trial of outpatient antibiotics, comorbid conditions, suspicion of an underlying secondary diagnosis, and possible bacteremia, particularly in a febrile infant or young child. If a case of likely bacterial lymphadenitis warrants hospital admission, intravenous (IV) antibiotics are usually the initial treatment. Antibiotic choice should be directed by the likely bacteria, mycobacteria, or fungi responsible for the process, as well as by patient allergies and recent antibiotic exposures. For lymphadenitis due to S. aureus or Streptococcus species, a βlactamase–resistant β-lactam antibiotic such as oxacillin or nafcillin is indicated; a combination of the β-lactam ampicillin and the β-lactamase inhibitor sulbactam sodium is also an option.3 Clindamycin may be another

good option for empirical treatment of these common pathogens as well as MRSA. However, susceptibility to MRSA varies widely by region and empirical therapy should be guided by local patterns of antibiotic resistance. If there is suspected or documented MRSA, IV vancomycin should be considered; for serious infections, vancomycin should be combined with a βlactamase–resistant β-lactam antibiotic.3 For lymphadenitis due to other bacterial, mycobacterial, or fungal causes, medication choices should be based on the likely organisms involved and their susceptibility profiles in the geographic region. The treatment course can be adjusted based on culture results and tested susceptibilities when those are available. Lack of clinical improvement typically dictates further therapeutic interventions or changes. An initial consideration is whether an unusual organism may be responsible for the process or whether the organism involved may demonstrate a degree of antibiotic resistance. In these cases, identifying the organism via stained and cultured tissue samples can be invaluable; consultation with an infectious disease specialist is also helpful. The possibility of immunocompromise in a patient who is not responding to conventional therapy should be reconsidered. The lymphadenitis lesion can be imaged or reimaged if it is not regressing. One possible reason for failed treatment response is low antibiotic penetrance into an organized, circumscribed abscess; penetrance into a walled-off loculation may be low despite relatively high vascular flow to inflamed surrounding tissues. For an identified abscess, drainage is often the most appropriate course of treatment. This can be performed via needle aspiration rather than open incision and drainage to reduce the risk of chronic drainage and scar formation.1 Excision is indicated for lesions with chronic drainage (as can occur with M. tuberculosis lymphadenitis) and for atypical mycobacteria, which are often multidrug resistant.1 Depending on the site in question, consultation with a general surgeon or otorhinolaryngologist is recommended. Discharge criteria for pediatric patients admitted with lymphadenitis include demonstration of significant clinical improvement, ability to receive enteral antibiotics for the remainder of the required course, and family support for treatment and follow-up. The choice of discharge antibiotics for likely or identified bacterial processes depends largely on the organisms

involved and the IV antibiotics used during the hospital stay. Common conversions to oral (PO) medications for pediatric lymphadenitis patients include IV oxacillin to PO dicloxacillin, IV ampicillin-sulbactam sodium to PO amoxicillin-clavulanate, IV to PO clindamycin, and IV cefazolin to PO cephalexin. KEY POINTS The history and physical examination are key to understanding the likely etiology of lymphadenitis and formulating the treatment plan. Concerning findings in a patient with lymphadenopathy include generalized lymphadenopathy, larger, matted or firm lymph nodes, or systemic symptoms. The most common causative organisms include Staphylococcus aureus and Streptococcal species; treatment is often started empirically directed toward these organisms. If there is inadequate response to the initial antibiotic regimen, consider antibiotic resistance (e.g. MRSA), unusual organisms, or development of an abscess.

SUGGESTED READINGS Camitta BM. The lymphatic system. In: Behrman RE, Kliegman RM, Jenson HB eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PA: WB Saunders, 2004:1677-1678. Davis HW, Michaels MG. Infectious disease. In: Zitelli BJ, Davis HW, eds. Atlas of Pediatric Physical Diagnosis. 4th ed. Philadelphia, PA: Mosby; 2002:396-454. Friedmann AM. Evaluation and management of lymphadenopathy in children. Peds Rev. 2008;29:53-60. Liu JH, Myer CM. Evaluation of head and neck masses. In: Rudolph CD, Rudolph AM, Hostetter MK, et al., eds. Rudolph’s Pediatrics. 21st ed. New York: McGraw-Hill, 2003:1279-1281.

REFERENCES 1. Davis HW, Michaels MG. Infectious disease. In: Zitelli BJ, Davis HW eds. Atlas of Pediatric Physical Diagnosis. 4th ed. Philadelphia, PA: Mosby, 2002:396-454. 2. LeBlond RF, DeGowin RL, Brown DD. Non-regional systems and diseases. In: LeBlond RF, DeGowin RL, Brown DD, eds. DeGowin’s Diagnostic Examination. 9th ed. New York: McGraw-Hill; 2009. Available at: accessmedicine.com/content.aspx?aID=3659310. Accessed April 26, 2013. 3. Pickering LK ed. Red Book: 2003 Report of the Committee on Infectious Diseases. 26th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2003:189-692. 4. Liu JH, Myer CM. Evaluation of head and neck masses. In: Rudolph CD, Rudolph AM, Hostetter MK, et al., eds. Rudolph’s Pediatrics. 21st ed. New York: McGraw-Hill, 2003:1279-1281. 5. Fink DL, Serwint JR. The knotty problem in an infant girl’s groin. Contemp Pediatr. 2005;22:24. 6. Schleyer AM, Jarman KM, Chan JD, Dellit TH. Role of nasal methicillin-resistant Staphylococcus aureus screening in the management of skin and soft tissue infections. Am J Infect Control. 2010;38:657-659. 7. Chen AE, Cantey JB, Carroll KC, Ross T, Speser S, Siberry GK. Discordance between Staphylococcus aureus nasal colonization and skin infections in children. PIDJ. 2009;28(3):244-246.

CHAPTER

32

Oral Lesions and Oral Health Suzanne Swanson Mendez

BACKGROUND Pediatric dental disease is the most common chronic illness of school-aged children.1 It is five times more common than asthma and has lifelong health implications. Severe tooth decay can lead to bacterial infections within the tissues of the mouth, the bones surrounding the oral cavity, and the sinuses. Pediatric dental disease can lead to malnourishment and pain, and adult dental disease is associated with bacterial pneumonia, diabetes, heart disease, stroke, and poor pregnancy outcomes, including premature labor.1 It is important to include a close examination of the oral cavity on admission, as many children have not seen a dentist in the past and have undiagnosed pathology. In a study of 120 children on a pediatric ward, more than 40% had unmet oral health needs, as determined by a dental assessment.2 Dental caries lead to more cumulative missed school hours than any other chronic disease and can lead to difficulties with eating, drinking, speaking, and paying attention.1 Children with special healthcare needs are at particularly high risk of severe tooth decay due to their underlying medical issues and barriers to accessing dental care. Dental care remains the most frequently cited unmet health need for this population of children.3 Pediatric hospital medicine physicians can help fill this gap by addressing oral health concerns on admission and facilitating proper dental care after discharge from the hospital. Intraoral disease may be a primary indication for admission to the hospital (e.g. dental abscess) or may be a secondary finding on an inpatient examination (e.g. aphthous ulcers in lupus [Table 32-1] or delayed tooth eruption due to a genetic disorder [Table 32-2]). Oral lesions, commonly

caused by viruses (see Table 32-3) or candida may also lead to such significant difficulty with drinking and swallowing that infants and children may require admission for pain control and treatment of dehydration. See Chapter 70 for a review of causes of stomatitis. TABLE 32-1

Conditions with Oral Manifestations

Condition

Oral Signs or Symptoms

Cyclic neutropenia

Oral ulcers, early loss of primary teeth

Leukocyte adhesion deficiency disorder

Severe gingival inflammation around primary teeth, increased tooth mobility, early tooth loss

Systemic lupus erythematosus

Oral or nasal mucocutaneous ulcerations

Crohn disease

Recurrent oral abscesses, dry mouth, redness and scaling around the lips, angular chelitis

Scarlet fever

Red “strawberry” tongue

Kawasaki syndrome

Dry, fissured lips and “strawberry tongue”

Stevens-Johnson syndrome

Fragile mucosal bullae, shallow oral ulcers with a gray or white membrane

Peutz-Jeghers syndrome

Melanotic spots on lips and buccal mucosa

Iron-deficiency anemia

Burning sensation of the tongue, pallor of oral mucosa, atrophy of papillae on tongue

Osler-WeberRendu disease

Telangiectasia of oral mucosa

Acrodermatitis enteropathica

Angular chelitis, glossitis

Porphyria

Possibly reddish brown discoloration of teeth

Acute myelogenous leukemia

Subtypes can present with gingival hyperplasia, oral ulcers

Primary HIV infection

Oral ulcers, pharyngitis, cervical lymphadenopathy

HIV, human immunodeficiency virus.

TABLE 32-2

Syndromes Associated with Delayed Tooth Eruption

Albright hereditary osteodystrophy Apert syndrome Cornelia de Lange syndrome Hunter syndrome Incontinentia pigmenti Miller-Dieker syndrome Osteogenesis imperfecta type I Progeria syndrome TABLE 32-3

Cause

Viral Stomatitis Lesion

Site and Distribution

Inflamed gingiva and mucosa, followed by vesicles that promptly rupture to reveal characteristic irregular, painful, superficial ulcers

Anterior: gingival, labial, lingual, and buccal mucosa; floor of mouth; extension to perioral skin involvement and “drop” lesions

HSV Primary (herpes stomatitis)

Posterior: hard palate and tonsils Recurrent

Single or small clusters of vesicles

Mucocutaneous junction of lips

Varicellazoster HHV-6

Shallow, non-painful ulcers

Palate

Erythematous papules

Soft palate and base of uvula

Rubeola (measles)

Mottled erythema in prodromal phase; grayish white granular lesions on pronounced erythematous mucosa (Koplik spots) on about day 10

Prodromal findings: palate Koplik spots— initially, buccal mucosa adjacent to lower molars; subsequently extends throughout oral mucosa

Enteroviruses

Rapidly-ulcerating vesicles, painful

Lingual and buccal surfaces; soft palate

Several coxsackie- and echoviruses Coxsackievirus A16 (handfoot-andmouth disease) HHV-6, human herpesvirus 6; HSV, herpes simplex virus.

Traumatic mucosal injuries are the most common type of oral injury in infants and young children and may be caused by burns, either chemical (e.g. alkali) or thermal (e.g. hot drinks); by sucking on a pacifier or finger; by sharp objects inserted into the mouth, resulting in abrasions or lacerations; or

by blunt trauma.4 Many children presenting with mucosal trauma also have injuries involving the teeth.

PATHOPHYSIOLOGY Dental abscesses generally result from untreated tooth decay, which leads to a deep cavity within a tooth, penetrating through the enamel and dentin into the central pulp. Abscesses can also develop after trauma causes a deep crack in a tooth. Once the pulp is exposed, bacteria can invade, causing the pulp to necrose and resulting in pockets of pus at the base of the tooth (see Figure 32-1 for tooth anatomy).

FIGURE 32-1. Tooth anatomy. DEFINITIONS Enamel: Hard mineralized external layer covering the crown of the tooth (above the gumline) Cementum: Calcified substance covering the root of the tooth and assisting in tooth support in the bone, along with the

periodontal membrane Dentin: An inner, second layer of calcified material, located deep to the enamel of the tooth (above the gum line) or cementum (below the gum line) Pulp: The central inner section of the tooth, composed of connective tissue, blood vessels and nerves Apical: Describes a direction toward the root tip(s) of the tooth Coronal: Direction toward the crown of the tooth or something related to the crown itself Root canal: A canal extending from the central pulp to the root, or apex, of the tooth Root canal treatment: Dental procedure which removes the infected or necrosed pulp of the tooth in order to save the tooth from possible extraction

PATIENT HISTORY Children with dental abscesses may present with excruciating pain and swelling within the mouth. Other symptoms may include fever, pain with chewing, sensitivity of the tooth to hot or cold, bad breath, a bitter taste in the mouth, general discomfort, or feeling unwell. The child may also have nausea, vomiting, diarrhea, chills, or sweats. If the abscess has spread, a child may present with complaints of facial swelling, trismus, symptoms of maxillary sinusitis, or difficulty swallowing or speaking.5 Of note, a child with a dental abscess may also be asymptomatic, with the infection only noted on thorough examination, particularly if an abscess is draining spontaneously through a sinus tract. Past medical history may reveal a history of dental caries or trauma to the teeth. Children with a history of osteogenesis imperfecta and other conditions resulting in abnormal dentin are at higher risk of developing dental abscesses from dental caries. Spontaneous dental abscesses are also frequently noted in familial hypophosphatemia or vitamin D–resistant rickets, due to hypomineralization of the dentin and enlargement of the pulp. Children with diabetes and boys are also at higher risk.6 Soft tissue injuries within the mouth are generally quite painful and are

noted shortly after the trauma occurs. Cuts of the lower lip or tongue are usually caused by biting down during a fall. A child who falls with an object in his mouth such as a pencil or toothbrush may have serious injuries to the posterior oral cavity or pharynx.

PHYSICAL EXAMINATION On physical examination, the child with a dental abscess may have fever or tachycardia, and depending on the extent of illness, the child may appear sick or uncomfortable. On focused examination of the oral cavity, the gums surrounding an infected tooth are often reddened and swollen, and one may be able to express pus from the base of the tooth. Pain is often elicited on palpation of the tooth, and the tooth may feel loose. Swelling may extend along the base of several teeth. If the infection tracks into the soft tissues of the neck, it can lead to airway compromise, and drooling or a muffled voice may be noted. The abscess also may cause trismus due to pain and swelling.5 If the abscess has spread to involve the soft tissues of the face, the child may have notable unilateral swelling of either the jaw or the upper face, depending on whether the lower teeth or upper teeth are involved. The facial skin may be erythematous and warm, particularly when compared to the opposite side of the face. The maxillary sinus can become involved with a dental abscess of the upper teeth, and unilateral maxillary sinus tenderness may be noted on percussion. There may also be unilateral cervical lymphadenopathy, and this may be the only external sign in early dental infections. Children with thermal or chemical burns may have areas of mucosal erythema or sloughing, and as healing proceeds an adherent white material appears. The distribution may include the palate, the lips, or the peripheral areas of the tongue. These lesions may be painful but generally heal within 2 weeks. Ulcers from sucking are usually found on the hard palate.

DIFFERENTIAL DIAGNOSIS The differential diagnosis for dental abscesses includes inflammation or infection of the surrounding tissues (Table 32-4). For children with intraoral injuries, one must consider the possibility of inflicted trauma. Child abuse should be considered with any signs of facial or oral trauma, particularly with

lacerations of the oral frenulae or bruising of the labial sulcus (lower border between the lip and gums) in a child who is not yet walking. The head, face, and neck are the most common sites affected by physical abuse and may be the only manifestation on physical examination. Other orofacial injuries suggestive of child abuse include swollen lips with underlying ecchymoses and tooth fractures without an adequate history. Sexual abuse may present with oral findings, such as bruising of the hard and soft palates, tears of the lingual frenulum, or oral lesions associated with a sexually transmitted disease.4 Neglect may be suspected in a child with severe, untreated dental caries and oral infection, but this can be difficult to differentiate from parental ignorance of proper dental hygiene or difficulty with obtaining a dental care provider. TABLE 32-4

Differential Diagnosis for Dental Abscess

Acute inflammation of the salivary glands (e.g. parotitis, mumps) Periorbital cellulitis Peritonsillar abscess Sinusitis Burkitt’s lymphoma Unerupted tooth or impaired eruption of tooth

DIAGNOSTIC EVALUATION The diagnosis of a dental abscess is mostly clinical (Figure 32-2). However, if there is concern for involvement of the upper airway or the spread into the lateral pharyngeal space, a CT of the neck may be helpful to determine if a procedure is needed. If a dentist is involved with the child’s care while in the hospital, x-rays of the teeth and surrounding structures may be requested. Routine laboratory studies are not indicated. A blood culture may be considered in cases of dental abscess complicated by osteomyelitis or with signs of septicemia or endocarditis.

FIGURE 32-2. Suspected dental abscess: diagnostic and treatment algorithm.

If the abscess is surgically drained or is spontaneously draining through a sinus tract, the material may be sent for gram stain and culture. However, with intraoral drainage, the culture can be contaminated by normal oral flora if not properly obtained, so the utility of the results may be limited. An acute dental abscess usually is polymicrobial in origin, comprised of both facultative anaerobes (e.g. viridans group streptococci) and strict anaerobes (e.g. Prevotella and Fusobacterium) as well as possible aerobes.7,8 For intraoral injuries, clinical examination should reveal the extent of damage but radiographs of the teeth and surrounding bones may be indicated. Head or neck CT or CTA may be needed for possible retained foreign bodies or to evaluate for associated arterial injury. There is a small risk (0.6% incidence in recent studies) of internal carotid artery damage or thrombosis with lateral soft palate or peritonsillar injury.9,10 If child abuse is suspected, radiographs may reveal multiple healed fractures of the teeth and jaw. Unexplained oral bleeding in an infant warrants investigation with a complete blood count and coagulation studies. If sexual abuse is suspected, testing for sexually transmitted infections such as herpes, gonorrhea, or syphilis may be indicated. (See Chapter 40 for more on child abuse injuries.)

MANAGEMENT DENTAL ABSCESS Treatment of an uncomplicated dental abscess without facial swelling or severe pain may be with a dental procedure alone. For an infected primary tooth, the treatment is extraction. For an infected permanent tooth, the infected pulp material is removed and root canal treatment is performed instead of extraction. No antibiotics are needed if the infection is limited to the tooth and its root(s).8,11 If facial swelling or severe pain is present, oral antibiotics are given first to reduce the inflammation, followed by a dental examination in 1 week. Amoxicillin or penicillin should cover the flora in most dental abscesses. However, if local epidemiologic patterns reveal a high resistance rate to amoxicillin alone, or if the child is at higher risk of resistant organisms, alternative regimens include amoxicillin with clavulanic acid, clindamycin, or

a combination of metronidazole plus amoxicillin or a macrolide.8 For penicillin-allergic children, oral clindamycin is the recommended alternative agent. Once the swelling and severe pain have resolved, treatment can proceed with either extraction or root canal therapy as above. If the swelling or severe pain do not resolve or worsen after 1 week of oral antibiotics, the child may need a different oral antibiotic or admission for an intravenous (IV) antibiotic. Recommended regimens include ampicillin plus sulbactam or clindamycin or metronidazole plus ampicillin/sulbactam, depending on which antibiotics have been utilized as an outpatient and the extent of the infection. For infections involving the deep neck tissues, coverage of Staphylococcus aureus and Group A Streptococcus organisms is particularly important. Inpatients also often require IV fluids to treat dehydration, and most will require pain control with either oral or IV medications. The pain may improve if the abscess begins to spontaneously drain, but the abscess still requires treatment. Warm saltwater rinses of the mouth may also help with pain and discomfort. If a dentist is available for pediatric inpatient consultation, he/she should be contacted upon the child’s admission to the hospital. An otolaryngology (ENT) consult may be needed if there is extension of the abscess into the soft tissues of the neck or pharynx. Additional complications of dental abscesses are listed in Table 32-5. TABLE 32-5

Possible Complications from Dental Abscess

Dental cyst

Septicemia

Facial cellulitis

Endocarditis

Sinusitis

Pneumonia

Osteomyelitis

Brain abscess, meningitis

Airway compromise

Cavernous sinus thrombosis

Septic thrombophlebitis (Lemierre syndrome)

Sublingual or submandibular cellulitis (Ludwig angina)

Discharge criteria include improvement in any facial swelling or erythema, an ability to take enteral fluids and antibiotics, adequate pain control on oral medications, and adequate treatment of any complications such as osteomyelitis. Fortunately, the risk of recurrence of a dental abscess is relatively slim and most patients can wait 1 to 2 weeks to see their dentist, but close follow-up must be assured prior to discharge.11 More than 20 million children lack dental insurance, and for those on Medicaid, it can be difficult to find a dental provider.1 Thus, a dentist must be identified for follow-up prior to discharging the patient home. Many children with dental abscesses suffer from poor oral health in general. Prevention is key in order to avoid future dental problems. (See Box, Prevention of Dental Abscesses.) PREVENTION OF DENTAL ABSCESSES Most, if not all, dental abscesses are preventable with good dental hygiene and prompt attention to any dental trauma. Children should have their first visit to the dentist by age 3 and have a dental home as well as a medical home. If the family’s tap water is not fluoridated sufficiently, the child should be on daily oral fluoride supplementation (see American Academy of Pediatrics recommendations for dosages). The risk of dental caries is increased with a diet high in sugars and with the intake of sodas and sports drinks.

ORAL TRAUMA Treatment for children with mucosal injuries depends on the etiology but is largely supportive, and most children who seek care at the hospital are discharged from the emergency department. Bleeding from the oral mucosa often initially appears to be more significant due to mixing with saliva but should resolve within 10 minutes with direct pressure. Lacerations of the lower lip do not usually penetrate the entire lip and do not need suturing unless the outer cut is gaping or involves the vermillion border. Tongue lacerations also tend to heal on their own, provided any gaping resolves when

the tongue is relaxed. Lacerations of the hard or soft palate or involving the tonsils may need surgical repair, although observation may be an option for smaller lacerations provided no foreign body is retained. Children with dental injuries are also typically cared for in the outpatient setting. Injuries to primary teeth involve different strategies than injuries to permanent teeth, as damaged or missing permanent teeth can have significant negative functional, cosmetic, economic, and psychological effects.12 A severe dental injury (e.g. an avulsed permanent tooth), requires emergent dental evaluation, whereas a loose permanent tooth or fractured permanent tooth without exposure of the pulp should be evaluated by a dentist within 24 hours. Less significant injuries such a non-painful fracture to a primary or permanent tooth may be followed up on a less urgent basis. If a primary tooth is slightly loose after trauma, it should be left in place, as many of these injuries will heal on their own. A soft diet is recommended for 1 to 2 weeks and good oral hygiene is important to reduce the chance of infection.4 However, if the primary tooth is extremely loose, it may need to be removed to reduce the risk of aspiration of the tooth. A primary tooth that is completely avulsed out of the socket should not be replaced, as this can damage the permanent tooth beneath the gums. An avulsed permanent tooth is a true dental emergency, and a dentist should be contacted immediately if the tooth is located and the child is stable. The avulsed tooth should be held by the crown, gently rinsed in saline or tap water, and then placed back into the socket and held there by the child by biting down on gauze or with finger pressure. Alternatively, a temporary splint comprised of aluminum foil placed over the tooth and surrounding teeth can be used until dental intervention is available.13 Ideally, the tooth should be replaced in the socket within 15 minutes to an hour, but the tooth may remain viable for longer if placed in a container of cold milk or tooth storage media. Displaced permanent teeth due to luxation injuries will have the best possible outcome with urgent repositioning by a dentist within a few hours. Subluxed teeth, or loose teeth in normal position, must be followed closely and seen by a dentist as soon as possible for stabilization, ideally within 24 hours.13 Permanent teeth that are fractured with exposure of the pulp should be seen by a dentist within 24 hours. Tooth fragments should be located and

saved in tooth storage media or tap water. (Cold milk is not necessary, as the fragments do not contain live fibroblasts, but the tooth fragments will become discolored if not kept wet.14) If the fracture does not involve the pulp of the tooth, the fracture is not usually painful or at high risk for infection, so dental evaluation can wait up to 7 days.

SPECIAL CONSIDERATIONS During hospitalization, good oral hygiene should be taught and emphasized for all patients, with a particular focus on children with special healthcare needs. Teeth should be brushed twice daily with a small amount of water, and for children over age 2 years, a pea-sized amount of fluoridated toothpaste should be added. The teeth do not need to be rinsed after brushing, as the fluoride in the toothpaste has a beneficial topical effect. Children with tight spaces between primary teeth or with permanent teeth should also floss at least once a day.15 Children who are NPO or exclusively tube-fed should receive the same oral care as other children and are at higher risk for alterations in oral pH. Bruxism in children with cerebral palsy can erode the enamel and lead to a higher chance of tooth decay. Also, children with gastroenteritis, gastroesophageal reflux disease, or bulimia need to have the stomach acids removed from their teeth to prevent decalcification.15 Pediatric oncology patients are at higher risk of mucositis if oral hygiene is not maintained, and saliva provides an important barrier of defense against infection. For children at risk of gum bleeding, brushing should be done gently and the use of mouthwashes encouraged. Medications may also increase the risk of oral health problems. Some medications may decrease salivary flow, increasing the risk of dental decay (see Table 32-6), while others such as phenytoin may cause gingival hyperplasia, making plaque more difficult to remove.15 Also, many oral medications for children are given in sugar-based syrups, which lead to a higher risk of tooth decay. Children receiving these types of medications on a frequent or chronic basis may need to brush more often or use antibacterial mouthwashes. TABLE 32-6

Medication Classes Associated with

Decreased Salivary Flow Anticholinergics

Benzodiazepines

Antidepressants

Cytotoxic drugs

Antihistamines

Diuretics

Antihypertensives

Opiates

ADHD medications

Proton pump inhibitors

KEY POINTS A periapical abscess can be dangerous and may cause both local and systemic complications. Indications for admission include dehydration, worsening facial or neck swelling, inability to handle oral secretions, concern for airway compromise, systemic involvement, or failure of outpatient therapy. Antibiotics should cover both anaerobes and strict aerobes. Include Group A Strep and Staph aureus coverage for neck involvement.

ACKNOWLEDGMENT Special thanks to Dr. Rex Kido and Dr. Charles Klass for their assistance with this chapter and its preparation.

REFERENCES 1. US Department of Health and Human Services. Oral Health in America: A Report of the Surgeon General–Executive Summary. Rockville, MD: US Department of Health and Human Services, National Institute of Dental and Craniofacial Research, National Institutes of Health; 2000. 2. Nicopoulos M, Brennan MT, Kent ML. Oral health needs and barriers to

dental care in hospitalized children. Spec Care Dentist. 2007;27(5):206211. 3. Lewis CW. Dental care and children with special health care needs: a population-based perspective. Acad Pediatr. 2009;9(6):420-426. 4. American Academy of Pediatric Dentistry. Guideline on management of acute dental trauma. 2001, 2004, 2007, 2010, 2011. Available at: aapd.org/media/Policies_Guidelines/G_trauma.pdf. 5. Walsh LJ. Serious complications of endodontic infections: some cautionary tales. Aust Dent J. 1997;42(3):156-159. 6. Seow WK. Review: diagnosis and management of unusual dental abscesses in children. Aust Dent J. 2003;48:(3):156-168. 7. Robertson D, Smith AJ. The microbiology of the acute dental abscess. J Med Microbiol. 2009;58(2):155-162. 8. Brook I. Microbiology and management of endodontic infections in children. J Clin Pediatr Dent. 2003;28(1):13-7. 9. Randall DA, Kang DR. Current management of penetrating injuries of the soft palate. Otolaryngol Head Neck Surg. 2006;135(3):356-360. 10. Soose RJ, Simons JP, Mandell DL. Evaluation and management of pediatric oropharyngeal trauma. Arch Otolaryngol Head Neck Surg. 2006;132(4):446-451. 11. Kido R. Personal communication. February 2012. 12. Lee J, Divaris K. Hidden consequences of dental trauma: the social and psychological effects. Pediatr Dent. 2009;31(2):96-101. 13. Emerich K, Wyszkowski J. Clinical practice: dental trauma. Eur J Pediatr. 2010;169(9):1045-1050. 14. Macedo GV, Diaz PI, De O Fernandes CA, Ritter AV. Reattachment of anterior teeth fragments: a conservative approach. J Esthet Restor Dent. 2008;20(1):5-18; discussion 19-20. 15. Blevins JY. Oral health care for hospitalized children. Pediatr Nurs. 2011;37(5):229-235.

CHAPTER

33

Neck Pain Matthew T. Lister and Nicole E. St Clair

BACKGROUND Although neck pain is a common presenting symptom, it is rarely a discharge diagnosis. Estimates of the incidence of neck pain necessitating admission to a hospital do not exist, but data from a regional children’s hospital with 40,000 emergency department visits per year suggest that less than 10% of patients seen with a complaint of neck pain are admitted. This chapter emphasizes the most common diagnoses associated with neck pain that result in hospital admissions for children. DEFINITIONS A variety of terms deserve attention in the description and management of neck pain symptoms. Neck stiffness refers to an abnormal preferred position of the neck or a normal position with restricted range of motion. Meningismus indicates neck stiffness related to meningeal irritation or inflammation. Torticollis (Latin for “twisted neck”) refers to neck stiffness associated with the child holding his or her head to the side with the chin rotated in the opposite direction. Trismus refers not to neck stiffness, but stiffness and limited opening of the jaw. Various neck spaces or potential spaces deserve description (Figure 33-1), as infection of a certain space often indicates likely pathophysiology, determines the symptomatology and examination presentation, and dictates treatment of the disorder. The sublingual space (supramylohyoid space, a subdivision of the submandibular space) is that space beneath the tongue, medial to

the body of the mandible and superior to the myelohyoid muscle. The peritonsillar space is a potential space between the capsule of the pharyngeal or palatine tonsil and the superior constrictor muscle of the pharynx. The danger space is a potential space that is bound superiorly by the skull base, anteriorly by the alar fascia, and posteriorly by the prevertebral fascia, extending down to the diaphragm. The retropharyngeal space is anterior to the danger space and posterior to the visceral space containing the esophagus and trachea. It potentially communicates laterally with the parapharyngeal and danger spaces.1 The parapharyngeal space (also pharyngomaxillary space or lateral pharyngeal space) is best described as an inverted pyramid with the base at the skull base and the apex at the greater cornu of the hyoid bone, lateral to the superior pharyngeal constrictors, medial to the parotid gland, mandible, and lateral pterygoid muscles, anterior to the prevertebral fascia, and posterior to the pterygomandibular raphe. General definitions applicable to a description of infections anywhere in the body include cellulitis, phlegmon, and abscess. Cellulitis is a superficial infection, usually with signs of induration, erythema, and warmth. A phlegmon is an infection deeper in tissue with signs including induration, edema of surrounding tissues on imaging, possible necrosis of tissues within a given area, but no clearly-defined capsule or enhancing rim on contrast imaging. It may or may not be possible to obtain fluid from a phlegmon using needle or open techniques. An abscess is a walled-off collection of necrotic tissue and purulent material which typically exhibits an enhancing rim on imaging.

FIGURE 33-1. Lateral pharyngeal, retropharyngeal, danger, and prevertebral spaces and their relationship with each other. (A) Midsagittal section of the head and neck. (B) Coronal section in the suprahyoid region of the left side of the neck. (C) Cross-section of the neck at the level of the thyroid isthmus. a, artery; m, muscle; v, vein; 1, superficial space; 2, pretracheal space; 3, retropharyngeal space; 4, danger space; 5, prevertebral space. (From Chow AW. Lifethreatening infections of the head and neck. Clin Infect Dis. 1992;14:992; with permission from Oxford University Press and Dr. Anthony Chow.)

PATHOPHYSIOLOGY Neck pain can be caused by various pathologies. Because of the complicated anatomy and diverse structure in the neck, diseases of widely different etiologies may present with similar symptoms and can include infectious,

vascular, inflammatory, neoplastic, iatrogenic, congenital, autoimmune, traumatic, neurologic, and idiopathic etiologies (Table 33-1). TABLE 33-1

Differential Diagnosis of Neck Pain in Children

Diagnostic Category Diagnosis Infectious

Cervical lymphadenitis Peritonsillar abscess Retropharyngeal abscess Parapharyngeal abscess Jugular septic thrombophlebitis (Lemierre syndrome) Dental abscess Submandibular space infection (Ludwig angina) Sialadenitis Meningitis Cervical osteomyelitis Suppurative thyroiditis Infected branchial cleft cyst Infected thyroglossal duct cyst

Vascular

Vertebral artery dissection Stroke

Inflammatory/Idiopathic Atlantoaxial instability

Cervical spine stenosis Acute cervical disk calcification Relapsing polychondritis Neoplastic

Posterior fossa, spinal canal, and neck tumors Osteoid osteoma

Iatrogenic

Non-traumatic atlantoaxial rotatory subluxation Conditions associated with surgical positioning

Congenital/CNS

Chiari I malformation

Autoimmune

Arthritis and spondyloarthropathy

Traumatic

Vertebral fracture Spinal epidural hematoma Esophageal injury Laryngotracheal injury

CNS, central nervous system.

EVALUATION The diagnostic evaluation of neck pain is guided by findings from the history and physical examination. The need for and direction of more urgent evaluation is often evident on initial assessment. In cases involving stridor or respiratory distress (posturing, drooling, accessory muscle use, retractions), specialty evaluation by otolaryngology, airway films, or fluoroscopy may be warranted. With fever, lethargy, and/or meningismus, lumbar puncture with or without preceding head computed tomography (CT) to identify cases of increased intracranial pressure related to meningitis should be considered. In

cases of trauma, existing protocols, mechanism of injury, and examination findings dictate the initial steps taken. If there is altered mental status and/or cranial neuropathy, gait abnormality, or hemiparesis, then urgent CT or MR angiography or other modality may be warranted to rule out stroke or vessel dissection. Additional laboratory studies including blood cultures, complete blood count, erythrocyte sedimentation rate, and C-reactive protein may be helpful for suspected infectious or inflammatory problems. If indicated by examination, rapid strep antigen test, throat culture, or culture of purulent drainage from a lymph node, ear, or other non-oral source may be helpful prior to initiation of antibiotic therapy. Radiologic studies are often helpful in the diagnosis of neck pain. Depending on the history and physical examination findings, a cervical spine series can identify any fracture, dislocation, or instability. A CT scan with contrast is useful for evaluating the soft tissue structures of the neck and can suggest a diagnosis of phlegmon or abscess. Lateral neck films can also serve as an initial screening tool when retropharyngeal abscess or even epiglottitis/supraglottitis is suspected, as they may reveal widening of the retropharyngeal space, or a thumbprint sign from a thickened, edematous epiglottis. Widening can also be seen in normal patients with inadequate neck extension or inspiration, so the finding should be interpreted in the proper clinical context. Following trauma, in addition to identifying fractures or dislocations and evaluating for soft tissue injury, a CT scan can identify or raise suspicion of injury to the trachea or esophagus although crepitus, respiratory distress, and other history and examination findings are at least as important in alerting the practitioner to these entities. MRI is indicated if cervical osteomyelitis is suspected and for the evaluation of certain tumors.

HISTORY A thorough history and physical examination are warranted in all children presenting with neck pain. A few pieces of information from the history can quickly guide the examiner. Specifically, the presence or absence of fever and related complaints such as a sore throat, ear pain, dental pain, headache, or vomiting might point one toward an infectious or inflammatory cause. Important information in the history of present illness includes the temporal onset of pain, whether it is progressive or static, location of pain, radiation of

pain, and presence of associated neurologic symptoms, including bowel or bladder dysfunction, gait disturbance, or mental status change. Often a history of trauma is obvious or known, but eliciting a history of more subtle trauma is important. Systemic findings such as weight loss, fatigue, or night sweats can be suggestive of oncologic problems. Family history can be helpful in some cases if the cause is rheumatologic or vascular. Because of the known associations among some conditions or syndromes, it is important to obtain a thorough past medical and surgical history, including medication use, genetic syndromes, bleeding disorders, and immunodeficiency.

PHYSICAL EXAMINATION On inspection, it is important to note the general appearance of the patient, including his or her position of comfort. Signs of respiratory distress could include difficulty managing saliva and other secretions, stridor, posturing such as tripod posture with trunk leaning forward and neck extended, retractions, and paradoxical movement of the abdomen. Asymmetry in the neck, face, or any paired structures should be noted and investigated. Eye examination should include taking note of pupils, conjunctivae, extraocular movements, and gross visual acuity when possible. Oral examination includes assessment of jaw opening, teeth and gums, the floor of examination, tonsils, posterior oropharynx, uvula and palate. External ear (including position with notation of proptosis or mastoid tenderness), ear canal, and middle ear assessment should be completed. Inspection of the nasal tissues should be performed with an otoscope. Neck examination should assess for decreased mobility, torticollis, head tilt, stiffness, meningeal irritation, masses, lymphadenopathy, crepitus, fluctuance, tenderness, bruits, sterdor or stridor, and skin lesions. Finally, a complete neurologic examination is important to rule out mental status changes, nerve palsy, motor weakness, sensory deficit, and gait abnormality.

DIFFERENTIAL DIAGNOSIS AND TREATMENT When neck pain is a presenting symptom, the underlying cause must be determined to guide diagnostic studies and appropriate consultation or

treatment (Table 33-1). Diagnostic considerations recommendations can include the following.

and

treatment

INFECTIOUS CAUSES Cervical Lymphadenitis The most common infection associated with neck pain is cervical lymphadenitis. Often these infections resolve with antibiotics alone, but they may require surgical or needle drainage if an abscess forms. Children typically present with a unilateral enlarged, tender lymph node or nodes with overlying erythema (compared to children with generalized reactive lymphadenopathy, which is non-erythematous and occasionally tender); most have fever. The most common causative bacteria are Staphylococcus aureus and group A β-hemolytic streptococcus (GABHS). Cervical lymphadenitis can also be caused by viruses, mycobacteria, and other atypical organisms such as Bartonella henselae (the cause of cat scratch disease). The diagnosis is often made based on clinical presentation, but a CT scan or ultrasound may be indicated to identify other causes of a neck mass or abscess formation. Successful treatment most commonly involves empiric anti-staphylococcal agents or other antibiotics dictated by culture results, if available. Drainage of associated lymph node abscess may also be necessary. Deep Neck Space Infections Peritonsillar abscess presents in patients of all ages, more commonly in older children and adolescents. There may be an antecedent tonsillar or upper respiratory infection (URI), but symptoms typically progress rapidly once unilateral throat pain begins. Symptoms include severe unilateral tonsillar pain, otalgia, odynophagia, and sometimes fevers. Signs include trismus, drooling, tender cervical adenopathy, asymmetry of tonsils with edema and intense erythema of the soft palate, and uvular deviation. Treatment includes specialty consultation with otolaryngology for localization and drainage of the abscess and possible tonsillectomy followed by IV or oral antibiotic therapy. Peritonsillar “cellulitis” or phlegmon may have a similar presentation, but may respond to oral antibiotics and pain medication without the need for drainage. A CT scan is not always indicated, but may be helpful in cases with atypical presentation or in younger children (Figure 33-2).

FIGURE 33-2. Computed tomography of a peritonsillar abscess. (Used with permission from Children’s Hospital of Wisconsin Imaging Department, David Gregg, MD.) Retropharyngeal phlegmons and abscesses occur most commonly in preschool children. They are usually the result of a suppurative lymph node infection in the retropharyngeal space. There is often an antecedent history of URI, and occasionally with the presence of neck stiffness and pain. Hallmarks include high fever, drooling, torticollis, dysphagia, odynophagia, edema or mass of the posterior pharyngeal wall, and airway distress. Diagnostic studies include lateral neck films, which are abnormal 90% of the time, and for more detail, CT scans (Figures 33-3 and 33-4). Typical pathogens include β-hemolytic streptococci, respiratory anaerobes, and S. aureus.1 Children with retropharyngeal infections should be hospitalized, started on empiric broad-spectrum antibiotics, and managed in consultation with an otolaryngologist. Patients with airway compromise should undergo immediate surgical drainage. Patients with early retropharyngeal disease without associated abscess (retropharyngeal cellulitis or phlegmon) may respond to antibiotics alone and not require surgical drainage. For stable

patients with small or immature retropharyngeal abscesses, there is growing evidence to support initial management with antimicrobial therapy and close observation, and surgical drainage if there is not a clinical response within 24 to 48 hours; those with mature retropharyngeal abscesses, particularly if >2 cm, generally require both drainage and antimicrobial therapy.2,3

FIGURE 33-3. Enlarged retropharyngeal space secondary to soft tissue swelling anterior to the upper cervical bodies seen in a retropharyngeal abscess. (Used with permission from Children’s Hospital of Wisconsin Imaging Department, David Gregg, MD.)

FIGURE 33-4. Computed tomography of a retropharyngeal abscess. (Used with permission from Children’s Hospital of Wisconsin Imaging Department, David Gregg, MD.) Suppurative infections can also occur in the parapharyngeal space. Patients tend to be older than those with retropharyngeal abcesses. Dysphagia and trismus are common; patients may also develop a Horner syndrome on the affected side and other unilateral cranial nerve deficits.1 An associated complication is septic jugular vein thrombophlebitis (Lemierre syndrome). The most useful imaging study is a CT scan with contrast to determine whether there is abscess formation. Treatment involves empirical coverage with broad-spectrum intravenous antibiotics. If there is no response to medical therapy within 24 to 72 hours, or an abscess is clearly identified, incision and drainage may be warranted. Jugular Septic Thrombophlebitis (Lemierre Syndrome) Patients with Lemierre syndrome present with systemic findings concurrent with or following pharyngotonsillitis, dental infection, deep neck infection, mastoiditis, or sinusitis. The classic presentation is associated with a dental

infection and foul-smelling breath. Most patients with jugular septic thrombophlebitis have unilateral neck pain below the mandibular angle along the sternocleidomastoid muscle with severe tenderness, but no discrete mass or other associated findings to account for the tenderness. Patients often present with shaking chills, spiking fevers, and possibly dyspnea. The most common organism identified is Fusobacterium necrophorum, although this infection is usually polymicrobial. The diagnosis is suggested if F. necrophorum grows from a blood culture, and it can be confirmed by contrast CT scan of the neck. Treatment is a 4- to 6-week course of broad-spectrum antibiotics to cover aerobic and anaerobic bacteria, and supportive care. Anticoagulation is recommended only if there is evidence of continued propagation of the thrombus. Dental Abscess Dental abscess is typically caused by mouth flora in the setting of dental caries. It typically presents with face or neck swelling, pain, and fever. A fluctuant area near the affected tooth or gum is sometimes palpable. The acute treatment is intravenous or oral antibiotics to cover aerobic and anaerobic organisms, followed by definitive dental treatment. Submandibular Space Infection (Ludwig Angina) Ludwig angina is a cellulitis of the connective tissue, fascia, and muscles of the submandibular space. The source is usually a dental infection. In its most severe and lifethreatening form, induration of the submandibular tissues can force the tongue base to occlude the airway. It occurs most often in older children and adolescents. The clinical presentation includes a toxic-appearing patient with fever, foul breath, drooling, odynophagia; frequently the voice is affected. Physical examination characteristics include an erythematous, tender, and indurated floor of mouth, swollen and tender neck, and an elevated tongue. This is a rapidly progressive illness and represents a true airway emergency. Nearly all children require emergent intubation or tracheotomy. Treatment involves controlling the airway and administering broad-spectrum intravenous antibiotics; abscess is usually not identified, and imaging is not usually helpful in the initial assessment and management. Sialadenitis Inflammation or infection of the major salivary glands may be a cause of neck pain. The most commonly affected are the parotid glands and submandibular glands. Edema, induration, tenderness, and occasionally warmth and erythema may be present and are usually unilateral. Causes of infection or inflammation include viral illnesses, ductal obstruction due to

stones, dehydration leading to thickening of secretions and poor salivary flow with secondary infection, thickened secretions due to medications such as antihistamines and anticholinergics, autoimmune conditions associated with Sjogren syndrome, prior treatment with radiation or chemotherapy, and trauma to ducts. The most common pathogens are Staphylococcus species or other oral flora. Treatment includes aggressive oral hydration when feasible, IV hydration when necessary, IV or PO antibiotic therapy, heat over the affected gland, pressure/massage to encourage drainage of infected saliva, removal of obstructing debris or stone by an otolaryngologist if needed, and the use of sialogogues such as sour candy or lemons. Meningitis The classic clinical presentation of meningitis is fever, meningismus, irritability, and vomiting in a toxic-appearing patient. It is usually associated with viral or bacterial infections. Diagnosis is made by evaluation of cerebral spinal fluid (CSF) from a lumbar puncture; CSF pleocytosis is present in most cases. Bacterial culture and viral studies can provide a definitive diagnosis. Appropriate treatment includes supportive care and empiric or targeted antimicrobial treatment. Please refer to Chapter 97 for further details pertaining to the evaluation and management of meningitis. Cervical Osteomyelitis The clinical presentation of osteomyelitis of the cervical spine includes focal tenderness, limited range of motion, and fever. This infection occurs most often in children older than 8 years. The most common organism identified is S. aureus. Evidence of infection is supported by magnetic resonance imaging (MRI) or bone scan. Cultures of the blood or bone obtained by biopsy are useful in choosing antibiotics, but in their absence, treatment should be a prolonged course of antibiotics covering S. aureus and GABHS, with consideration of Kingella kingae for younger children (see Chapter 105). Surgical debridement is indicated if the patient does not demonstrate clinical improvement after appropriate treatment. Suppurative Thyroiditis This rare disease typically occurs after an upper respiratory infection and presents with the acute onset of fever, chills, and a tender, enlarged thyroid gland. Infection is most often caused by S. aureus, Streptococcus, and Enterobacter. Diagnosis is made by ultrasound-guided needle aspiration or biopsy with culture. Thyroid function tests are rarely abnormal. Many cases may turn out to be related to a persistent thyroglossal duct, though some children have a pyriform sinus fistula.1 After treatment of acute infection, endoscopy, CT, or a barium swallow is recommended to

identify a communication with the pyriform sinus. Initial treatment is empiric IV antibiotics to cover S. aureus, Streptococcus pneumoniae, and Enterobacter. Treatment can be narrowed based on culture results. Other Infections Infections in congenital abnormalities such as a branchial cleft cyst/sinus or a thyroglossal duct cyst are not uncommon. Commonly, there is a history of antecedent upper respiratory illness, followed by swelling of a midline neck cyst (in the case of a thyroglossal duct cyst) or a cyst along the sternocleidomastoid anterior border (in the case of a second branchial cleft cyst), which may then become painful, tender, and fluctuant. There may be overlying skin erythema, and drainage from a sinus tract in the case of a branchial cleft abnormality. Treatment is with needle aspiration or drainage procedures along with oral antibiotics covering Staphylococcus species for less severe infections.

VASCULAR CAUSES Spontaneous Arterial Dissection Dissection of the cervical and intracranial portions of the carotid and vertebrobasilar arteries may occur spontaneously or in association with trauma or other underlying risk factors. Classically, patients present with head and neck pain in association with ataxia, vomiting, or other focal neurologic signs. Dissection is a known cause of stroke in children. In one study of vertebral artery dissection in children, half of those who had cervical radiographs had associated cervical anomalies.4 Causes of dissection include trauma and vasculopathy; in some cases, the cause is unknown. Dissection can occur at any age; a review of 68 cases by Hasan and colleagues showed a 6.6:1 male predominance and a median age of 9 years.4 The diagnosis is made most accurately by angiography; magnetic resonance angiography may also be useful. Limited data are available regarding the most effective medical treatment of dissection in children and young adults. Most physicians consider antiplatelet and/or anticoagulation therapy. Interventional neuroradiology may offer an approach in difficult cases.

INFLAMMATORY/IDIOPATHIC Upper Cervical Spine Instability Atlantoaxial instability is typically

caused by congenital abnormalities of the odontoid process or ligamentous hyperlaxity, and it affects as many as 15% of children with Down syndrome. It can also be caused by inflammatory conditions such as juvenile idiopathic arthritis (JIA). Patients with cervical spine instability may be totally asymptomatic or they may have severe neck pain and neurologic deficits. The diagnosis is made using plain films of the cervical spine or three-dimensional CT reconstruction. Treatment is surgical fusion; however, the decision to treat should be individualized, because many children with radiographic evidence of instability do not exhibit symptoms. As many as 15% of children with Down syndrome have cervical spine instability, but most experts agree that operative fusion should be performed only on those who are symptomatic or have neurologic symptoms. Cervical Stenosis In children, cervical stenosis is most often associated with achondroplasia. All children with this condition have abnormal growth of the pedicles with associated spinal stenosis. One third of patients with achondroplasia have symptomatic stenosis by age 15 years. Patients present with pain in the lower back and legs that is exacerbated by activity. MRI is diagnostic and can identify the extent of the stenosis. The treatment is surgical decompression. Acute Cervical Disk Calcification Disk calcification is thought to be due to a nonspecific inflammatory process. The child usually presents with fever and a painful, stiff neck. Examination reveals tenderness over the affected disk, most commonly in the cervical area. Radiographs show calcification of the intervertebral disk space 1 to 3 weeks after the onset of symptoms. Treatment is symptomatic, with a cervical collar and antiinflammatory medication. Most cases resolve within 2 to 3 weeks. Relapsing Polychondritis Relapsing polychondritis is a rare condition involving inflammatory changes in cartilage and other connective tissue, and can occur in both children and adults. Episodes occur with rapid onset of inflammatory symptoms and signs over cartilage in the head and neck as well as in other joints, causing pain, erythema, warmth, and tissue swelling usually involving the auricular cartilage. The inflammation may last 1 to 2 weeks and is occasionally treated with systemic steroids. Diagnosis is clinical, with laboratory testing notable only for nonspecific signs of inflammation.1

NEOPLASTIC Posterior Fossa, Spinal Canal, and Neck Tumors Tumors causing neck pain can occur in a variety of locations, and the signs and symptoms vary with location. In general, pain is a prominent symptom. Patients often have associated neurologic deficits involving the cranial nerves or motor function; sensory deficits are uncommon. Up to 25% of patients with neck or spinal tumors present with secondary scoliosis; torticollis can be seen with posterior fossa tumors. Diagnosis is usually made by CT scan or MRI. Treatment is dependent on the cause of the tumor. Osteoid Osteoma Osteoid osteomas are benign bone tumors characterized clinically by localized pain over the affected region. They most commonly occur in the lower extremities but may occur in the spine as well, in which case a painful secondary scoliosis may be evident. The cause of these lesions is unclear. They most commonly occur in older children and adolescents, with a 2:1 male predominance. The diagnosis can be made by plain films; a bone scan or CT provides more detail if needed. Most osteoid osteomas are self-limited and gradually calcify and blend into surrounding bone. Treatment with nonsteroidal anti-inflammatory drugs is sufficient in most cases, with some requiring surgical excision of the nidus.

IATROGENIC Non-Traumatic Atlantoaxial Rotatory Subluxation (Grisel Syndrome) Grisel syndrome is rare, and presents with painful torticollis, most commonly after otolaryngologic procedures such as adenoidectomy. The cause is thought to be hyperemia of the tissues, leading to laxity of the atlantoaxial joint. The diagnosis is made by CT scan with three-dimensional reconstruction. Treatment consists of a cervical collar and anti-inflammatory medications until symptoms resolve. If reduction does not occur, cervical traction may be necessary. Conditions Associated with Surgical Positioning In the appropriate clinical setting, disorders of the neck musculature and joints including the temporomandibular joint can present after surgery. These may be simply related to muscle strain or spasm from a period of neck rotation, flexion, or extension during a procedure or may relate to intubation or use of jaw-

opening devices and retractors during surgery in the mouth or throat. Management is through the use of anti-inflammatory medications, heat, and possibly physical therapy in the case of muscle strain/spasm, with more urgent intervention if jaw dislocation is suspected.

CONGENITAL In addition to infectious processes involving congenital cysts, some conditions may present with neck pain as a symptom of other pathology affecting the central nervous system (CNS) and spine. Chiari I Malformation Type I Chiari malformation is defined as descent of the cerebellar tonsils more than 5 mm below the foramen magnum. The cause is unknown. Classically, it presents with occipital headache or posterior neck pain lasting seconds to minutes, which is worsened by Valsalva maneuvers. Although associated neurologic deficits are common, many patients with this malformation are asymptomatic. Up to 50% of individuals with type I Chiari malformation have an associated syrinx, or a collection of fluid caused by obstruction of CSF flow. The incidence of Chiari malformation has dramatically increased with the advent of MRI scanning, the diagnostic test of choice. Treatment, if indicated, is surgical decompression.

AUTOIMMUNE Arthritis and Spondyloarthropathy Although uncommon, neck stiffness can be the presenting sign of diagnoses such as JIA and psoriatic arthritis. The affected joints have limited motion and are warm, tender, and swollen. The cause of most inflammatory joint diseases is unknown. With the spondyloarthropathies, involvement of the cervical spine increases with age and disease duration. The diagnosis is made by history and physical examination findings. Laboratory tests such as sedimentation rate, HLA typing, or specific antibodies may help support the diagnosis (see Chapter 150).

TRAUMATIC CAUSES Discussion of traumatic causes of neck pain is beyond the scope of this

chapter. Most significant trauma in children is managed by a trauma team. Less obvious forms of trauma, such as an unwitnessed fall from a bed or a hyperextension injury from gymnastics, are important considerations when a child presents with neck pain that does not appear to have an infectious cause. In nonverbal children, it is important to consider non-accidental trauma as a possible issue. When neck trauma is suspected, priority should be placed on cervical spine immobilization with appropriate imaging and evaluation. KEY POINTS Etiologies of neck pain in children can vary widely, but for hospitalized children the diagnoses often require prompt evaluation and management. For young children, neck pain can be subtle and often the only indication of pain is the presence of limited neck movement. Careful head, oropharyngeal, neck, and neurologic examinations are indicated, and the presence of fever should warrant exclusion of meningitis and deep neck space infections.

ACKNOWLEDGMENT This chapter has been revised from the original version, which was authored by Nicole Frei, MD.

SUGGESTED READINGS American Academy of Pediatrics Committee on Sports Medicine and Fitness. Atlantoaxial instability in Down syndrome: subject review. Pediatrics. 1995;96:151-154. Bliss SJ, Flanders SA, Saint S. Clinical problem-solving: a pain in the neck. N Engl J Med. 2004;350:1037-1042. Coticchia JM, Getnick GS, Yun RD, Arnold JE. Age-, site-, and time-specific differences in pediatric deep neck abscesses. Arch Otolaryngol Head Neck Surg. 2004;130:201.

Craig FW, Schunk JE. Retropharyngeal abscess in children: clinical presentation, utility of imaging, and current management. Pediatrics. 2003;111:1394-1398. Greenlee JD, Donovan KA, Hasan DM, Menezes AH. Chiari I malformation in the very young child: the spectrum of presentations and experience in 31 children under age 6 years. Pediatrics. 2002;110:1212-1219. Nicklaus PJ, Kelley PE. Management of deep neck infection. Pediatr Clin North Am. 1996;43:1277-1296. Peters TR, Edwards KM. Cervical lymphadenopathy and adenitis. Pediatr Rev. 2000:21;399-405. Pharisa C, Lutz N, Roback MG, Gehri M. Neck complaints in the pediatric emergency department: a consecutive case series of 170 children. Pediatr Emerg Care. 2009;25:823. Sia KJ, Tang IP, Kong CK, Nasriah A. Grisel’s syndrome: a rare complication of tonsillectomy. J Laryngol Otol. 2012;126:529. Silverboard G, Tart R. Cerebrovascular arterial dissection in children and young adults. Semin Pediatr Neurol. 2000;7:289-300. Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. Spine. 1998;23:2174-2179. Yassari R, Frim D. Evaluation and management of the Chiari malformation type 1 for the primary care pediatrician. Pediatr Clin North Am. 2004;51:477-490.

REFERENCES 1. Lee KJ. Essential Otolaryngology Head and Neck Surgery. 8th ed. New York, NY: McGraw Hill; 2003:426-436, 489, 628, 828, 1035. 2. Page NC, Bauer EM, Lieu JE. Clinical features and treatment of retropharyngeal abscess in children. Otolaryngol Head Neck Surg. 2008;138:300. 3. Wong DK, Brown C, Mills N, Spielmann P, Neeff M. To drain or not to drain - management of pediatric deep neck abscesses: a case-control study. Int J Pediatr Otorhinolaryngol. 2012;76(12):1810-1813. 4. Hasan I, Wapnick S, Tenner MS, Couldwell WT. Vertebral artery dissection in children: a comprehensive review. Pediatr Neurosurg.

2002;37:168-177.

CHAPTER

34

Petechiae and Purpura Susan H. Frangiskakis

INTRODUCTION Petechial and purpuric rashes can cause even seasoned clinicians to become very concerned because they can signify dangerous disease such as meningococcemia, as well as more benign disorders such as viral infections. It is important for the pediatric hospitalist to have an approach to the evaluation and management of patients with petechiae or purpura. A more complete discussion of the individual disease entities associated with thrombocytopenia and coagulation disorders are provided in Chapters 9 and 92. Petechiae and purpura are hemorrhages in skin or mucous membranes that are less than 2 mm in diameter or greater than 2 mm in diameter, respectively. These lesions do not blanch under diascopy. Ecchymoses are subcutaneous hemorrhages that are greater than 1 cm. Purpura fulminans is a progressive condition in which cutaneous infarctions occur and result in extensive skin necrosis.

PATHOPHYSIOLOGY Petechiae and purpura can occur from a variety of pathophysiologic mechanisms that interfere with the complex process of hemostasis. Platelets, von Willebrand factor, and the coagulation cascade are essential for hemostasis. Thrombocytopenia, abnormal platelet function, von Willebrand factor defects, and clotting factor deficits can result in petechiae and purpura. Disruption of normal vascular integrity, such as occurs with endothelial injury as a result of infection or inflammation, can cause petechiae and purpura. Mechanical causes, such as trauma or increased intravascular

pressure from coughing or vomiting, can cause petechiae and purpura by this mechanism as well. Intrinsically abnormal vascular components, such as collagen defects in connective tissue disorders, can result in these lesions. Ecchymoses are most often caused by trauma. Purpura fulminans typically occurs in the setting of bacterial sepsis and disseminated intravascular coagulopathy (DIC).

DIFFERENTIAL DIAGNOSIS Because a number of different pathophysiologic mechanisms can cause petechiae or purpura, the differential diagnosis is extensive (Table 34-1). The clinical manifestations of bleeding disorders vary according to the underlying defect. Thrombocytopenia and abnormal platelet function typically result in petechiae, ecchymoses, and persistent bleeding from superficial cuts and mucosal membranes. Infection and vasculitis can cause petechiae or purpura, which can be palpable. Clotting factor deficits and other coagulation disorders typically cause hemarthroses, soft tissue bleeding, and prolonged bleeding, but they can also cause petechiae or purpura. Von Willebrand disease is usually manifested as mucosal membrane bleeding, postsurgical bleeding, and menorrhagia, but it can result in petechiae. Disorders of vascular fragility can give rise to ecchymoses, as well as petechiae and purpura. TABLE 34-1

General Mechanism

Causes of Petechiae and Purpura Categories Examples

Disrupted vascular Infection integrity

Bacterial (meningococcemia, pneumococcal or Haemophilus influenzae sepsis, group A streptococcal infections) Viral (adenovirus, enteroviruses, influenza A virus, Epstein-Barr virus, parvovirus B19)

Rickettsial (Rocky Mountain spotted fever) Vasculitis

Henoch-Schönlein purpura, Kawasaki disease, systemic lupus erythematosus

Mechanical

Trauma (accidental, nonaccidental, or birth related), coughing, vomiting

Abnormal vascular components

Collagen vascular disorders (Ehlers-Danlos syndrome), vitamin C deficiency (scurvy)

Thrombocytopenia Decreased platelet production Increased platelet destruction

Leukemia, aplastic anemia, medications (sulfonamides, carbamazepine, valproic acid), vitamin B12 or folate deficiency Idiopathic thrombocytopenic purpura, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura

Splenic Hypersplenism, consumptive sequestration hemangioma (Kasabach-Merritt syndrome) Platelet dysfunction

Coagulation defects

Hereditary

Bernard-Soulier syndrome, Glanzmann thrombasthenia, storage pool disease

Acquired

Medications (aspirin, nonsteroidal anti-inflammatory drugs), uremia

Hereditary

Factor deficiencies, factors VIII and IX most commonly

(hemophilia A and B); von Willebrand disease; fibrinogen disorders Acquired

Liver disease, vitamin K deficiency, medications (heparin, warfarin), disseminated intravascular coagulopathy, clotting factor inhibitors

Note: Some causes may result in petechiae or purpura by more than one mechanism.

HISTORY AND PHYSICAL EXAMINATION Some key information from the history and physical examination can help determine the most likely cause of the petechiae or purpura and help direct the appropriate steps in evaluation of the patient. See Table 34-2 for focused history questions and Table 34-3 for directed physical examination items. TABLE 34-2

Focused History

Characteristic Questions Onset and duration of lesions

Acute? Rapidly progressing? Recurrent or chronic?

Location of the lesions

Only above the nipple line? Only on the buttocks and lower extremities? In a stocking-glove distribution? Generalized? Involving the mucous membranes?

Predisposing factors

Coughing? Vomiting? Recent trauma? Recent illness such as an upper respiratory infection? Bloody diarrhea? Medications?

Systemic signs or symptoms

Fever? Irritability? Lethargy?

Review of systems

Headache? Stiff neck? Bleeding from other sites such as epistaxis or per rectum? Darkened urine? Joint pain or swelling? Abdominal pain? Bone pain? Myalgias?

Past medical history

Immunization status? History of prolonged bleeding?

Birth history for neonates

Was the mother ill? Did the mother have any medical conditions such as preeclampsia or lupus? Was the mother taking any medications?

Family history

Bleeding disorders? Siblings with conditions such as neonatal thrombocytopenia?

TABLE 34-3

Directed Physical Examination

System

Physical Examination Findings

Vital signs

Febrile? Hypotensive?

General

Well or toxic appearing? Irritable? Lethargic?

HEENT

Bulging fontanelle? Pupils equal and reactive? Photophobia? Palatal petechiae? Erythematous oropharynx? Tonsillar exudates?

Neck

Nuchal rigidity? Kernig or Brudzinski signs?

Cardiovascular

New murmur? Capillary refill and extremity perfusion?

Abdomen

Hepatomegaly or splenomegaly? Tenderness?

Musculoskeletal Joint swelling? Joint pain on movement? Neurologic

Mental status? Focal findings?

Skin

Location of the lesions (e.g. at the site of the blood

pressure cuff? Above the nipple line only? On the buttocks and lower extremities primarily? In a stocking-glove distribution or generalized?) Characteristics of the lesions (e.g. are the lesions palpable?). Quantity of lesions present? HEENT, head, ears, eyes, nose, and throat.

The anatomic location of the skin lesions is suggestive but not definitive of certain disease processes. In general, petechiae solely above the nipple line suggest a benign cause such as vomiting or coughing. These Valsalva-like maneuvers cause spikes in intravascular pressure in the venous system of the head and neck. Lesions primarily on the buttocks and lower extremities are suggestive of Henoch-Schönlein purpura. Lesions in a stocking-glove or generalized distribution are more concerning for bacterial disease, although viruses can also give rise to this pattern; for instance, papularpruritic gloveand-stocking syndrome caused by parvovirus B19.

EVALUATION Children presenting with petechiae and purpura often warrant a complete blood count (CBC), including a platelet count and white blood cell (WBC) differential, peripheral blood smear, partial thromboplastin time (PTT), and prothrombin time (PT) performed to direct the remainder of the evaluation. Children who present with fever and petechiae or purpura warrant immediate attention because this can be a medical emergency (see Chapter 58). Approximately 0.5% to 11% of children presenting with fever and petechiae or purpura have a serious bacterial infection. In addition to the initial screening laboratory tests mentioned, a blood culture and cerebrospinal fluid (CSF) studies, including cell counts, glucose, protein, Gram stain, and culture, should be obtained. If infection by group A β-hemolytic streptococcus is suspected, a rapid streptococcal test or culture of the oropharynx, or both, are indicated. Testing for viruses, such as adenovirus and influenza, may be performed, depending on the level of clinical suspicion. Other studies such as enteroviral polymerase chain reaction assay on CSF or Rocky Mountain spotted fever antibody titers may also be helpful in some settings. If DIC is suspected, flbrinogen, fibrin degradation product,

and D-dimer studies of blood should be obtained. Children with an ill appearance, hypotension, decreased perfusion, or mental status changes, including irritability or lethargy, are more likely to have a serious bacterial infection. Laboratory evidence supporting a potential serious bacterial cause includes an abnormal peripheral WBC count, elevated C-reactive protein, abnormal PT, and abnormal CSF studies. If the child does not have a fever or does not appear acutely ill and infection is not likely, the evaluation should be directed by the type of bleeding and clinical scenario. See Table 34-4 for suggested studies to work up various causes. Some conditions such as viral infections, idiopathic thrombocytopenic purpura, and Henoch-Schönlein purpura are more common causes of petechiae and purpura in children. If the child is otherwise well and recently had a viral infection, idiopathic thrombocytopenic purpura would be likely. The child would be expected to have a decreased platelet count with a normal WBC count and hemoglobin. If this patient has an abnormal WBC count or is anemic, in addition to having thrombocytopenia, evaluation of a peripheral blood smear and assessment by a hematologist for conditions such as leukemia are indicated. If the child has abdominal pain, arthritis, and hematuria, Henoch-Schönlein purpura should be considered, provided that the CBC, peripheral smear, PT, and PTT are normal (see Chapter 58). TABLE 34-4

Condition Suspected

Evaluation of Petechiae and Purpura Recommended Studies

Acute lymphocystic leukemia

CBC with platelet count and WBC differential, peripheral blood smear, PT, and PTT

Infection

Blood culture, CSF studies, CRP; consider a rapid streptococcal antigen test or oropharyngeal culture, respiratory viral cultures, specific viral antibody titers, RMSF antibody titers

Vasculitis

ESR, CRP, ANA, anti-double-stranded DNA

antibody, complement levels (C3, C4, CH50); consider other specific autoantibody titers Mechanical

Imaging studies for trauma, including head CT scan and skeletal survey for suspected nonaccidental trauma

Abnormal vascular Skin biopsy for Ehlers-Danlos syndrome or components other collagen vascular disorders, ascorbic acid level Thrombocytopenia Consider bone marrow biopsy; urinalysis, BUN, creatinine Platelet dysfunction

Bleeding time, platelet aggregation studies, clot retraction assay, BUN, creatinine

Coagulation defect

Specific clotting factor assays, thrombin time, fibrinogen level; bleeding time, factor VIII activity, von Willebrand factor antigen level, and ristocetin cofactor activity for von Willebrand disease; liver function tests, correction of PT and PTT after administration of vitamin K, mixing studies to detect factor inhibitors

ANA, antinuclear antibody; BUN, blood urea nitrogen; CBC, complete blood count; CH50, 50% hemolyzing dose of complement; CRP, C-reactive protein; CSF, cerebrospinal fluid; CT, computed tomography; ESR, erythrocyte sedimentation rate; PT, prothrombin time; PTT, partial thromboplastin time; RMSF, Rocky Mountain spotted fever; WBC, white blood cell.

TREATMENT Treatment of petechiae and purpura is based on the underlying cause. Aggressive management with the administration of antibiotics and supportive care is warranted in cases concerning possible serious infectious disease (see Chapter 58). For example, meningococcemia can progress very rapidly because of the release of a potent endotoxin, with the development of shock, purpura fulminans, and respiratory failure, and the patient can potentially die within hours of presenting. It is imperative that the administration of

antibiotics not be delayed if there is difficulty obtaining the screening tests or cultures. Admission to a pediatric intensive care unit in cases of suspected bacterial sepsis is warranted for supportive care and frequent monitoring of vital signs, mental status, and the development of shock, respiratory failure, or DIC. Hemorrhage from other sites needs to be evaluated in these patients. In some circumstances patients may require transfusions with red blood cells, platelets, fresh frozen plasma, or cryoprecipitate (see Chapters 92 and 93).

SPECIAL CONSIDERATIONS NEONATAL PETECHIAE OR PURPURA Petechiae and purpura in a neonate can be due to a number of unique causes in addition to those listed earlier, including birth trauma, TORCH (toxoplasmosis, other infection, rubella, cytomegalovirus, herpes simplex virus), other infections such as syphilis or human immunodeficiency virus infection, neonatal alloimmune thrombocytopenic purpura, congenital thrombocytopenic syndromes such as thrombocytopenia-absent radius syndrome, and congenital abnormalities of platelet function. It is critical to determine whether the neonate is well or ill in order to direct the investigation. If the neonate is ill, sepsis and DIC must be strongly considered and addressed appropriately. If the neonate is well, one can proceed with the basic screening laboratory tests as stated earlier. Depending on these results, further investigation under the guidance of a hematologist or neonatologist may be helpful. KEY POINTS Children who present with fever and petechiae or purpura warrant immediate attention because this can be a medical emergency. Treatment of petechiae and purpura is based on the underlying cause.

SUGGESTED READINGS Brogan PA, Raffles A. The management of fever and petechiae: Making sense of rash decisions. Arch Dis Child. 2000;83:506-507. Mandl KD, Stack AM, Fleisher GR. Incidence of bacteremia in infants and children with fever and petechiae. J Pediatr. 1997;131:398-404. Nielsen HE, Anderson EA, Anderson J, et al. Diagnostic assessment of haemorrhagic rash and fever. Arch Dis Child. 2001;85:160-165. Wells LC, Smith JC, Weston VC, et al. How likely is meningococcal disease in a child with a non-blanching rash: A prospective cohort study. Arch Dis Child. 2001;84(Suppl 1):A10-A68.

CHAPTER

35

Respiratory Distress Juliann Lipps Kim

BACKGROUND Respiratory distress is one of the most common reasons for a child to present to the emergency department or a practitioner’s office. Respiratory distress can result from disorders in the respiratory system or in organ systems that control or influence respiration. Young children have an increased risk for respiratory distress because of their anatomy and physiology. Nearly 20% of all emergency department visits for children younger than 2 years are for respiratory disease.1 The causes of respiratory distress are vast, and practitioners caring for children should have a systematic approach to its diagnosis and management. Cardiopulmonary arrest in children is largely due to respiratory failure (in adults, cardiac causes are most common). Rapid evaluation and management of severe pediatric respiratory disease may be necessary to prevent respiratory failure.

PATHOPHYSIOLOGY Understanding respiratory physiology can aid the practitioner in diagnosing the cause of respiratory symptoms. The main goals of respiration are oxygen uptake and elimination of carbon dioxide. Secondary goals include acid–base buffering, hormonal regulation, and host defense. To achieve the goals of respiration, three main functional components of the respiratory system are used: (1) mechanical structures (including chest wall, respiratory muscles, and pulmonary circulation), (2) membrane gas exchanger (interface between airspace and pulmonary circulation), and (3) regulatory system (network of chemical and mechanical sensors throughout the circulatory and respiratory systems). All three components are tightly integrated, and dysfunction of one

can lead to respiratory distress or failure. Respiratory function is tightly controlled by a complex network of central and peripheral chemoreceptors and mechanoreceptors responding to information from the body about the status of the respiratory system. This network modulates the neural output to the respiratory muscles, affecting the timing and force of respiratory effort. Central chemoreceptors in the ventral reticular nuclei of the medulla are sensitive to changes in pH and partial pressure of carbon dioxide (PCO2) of cerebrospinal fluid. The intrinsic brainstem function of the dorsal and ventral respiratory centers of the medulla controls inspiration and expiration, respectively. The apneustic center in the pons increases the depth and duration of inspiration, whereas the pneumotaxic center decreases depth and duration. The cerebellum, hypothalamus, motor cerebral cortex, and limbic system also play a role in mediating respiration. Carotid and aortic bodies are peripheral chemoreceptors sensitive to the partial pressure of oxygen (PO2), PCO2, and pH in arterial blood. Mechanoreceptors (stretch, juxtacapillary, and irritant reflex) distributed along the airways, lung parenchyma, and chest wall respond to lung volume, changes in pulmonary microvasculature, chest wall muscle activity, and environmental irritants. Information from central and peripheral receptors is integrated in the brainstem, and efferent impulses are transmitted to alter respiratory function and maintain homeostasis. Even slight alterations in arterial pH, PO2, or PCO2 stimulate the respiratory centers to modify the respiratory pattern. Increased arterial PCO2 and decreased arterial PO2 lead to an increase in respiratory drive and increased neural output to respiratory muscles. The resulting increase in ventilation can be achieved by increases in tidal volume or respiratory rate, because the product of these two factors determines the minute ventilation. Respiratory insufficiency or the more severe entity, respiratory failure, occurs when regulatory systems or the effector organs (lungs, respiratory muscles) are impaired or overwhelmed. This results in diminished oxygenation (decreased arterial PO2), retention of carbon dioxide (increased arterial PCO2), and acidosis (decreased arterial pH). Disruption of the mechanics of the lung or chest wall results in the majority of respiratory disease in children.2 Obstructive or restrictive disease leads to increased work of breathing and increased energy demands on the

respiratory muscles to meet the body’s needs. This increased work manifests itself clinically as respiratory distress, evidenced by increased work of breathing. When demand exceeds capability, the result is respiratory insufficiency, which can progress to respiratory failure. In respiratory muscle dysfunction, neural output is sent to the respiratory muscles but they are unable to respond adequately to increase respiratory effort. The physical signs of respiratory failure in this setting are more subtle, and the signs of increased work of breathing may not be present. When the difficulty involves the control of breathing, there is an inadequate neural response to hypoxemia or hypercarbia. Arterial hypoxemia or hypercarbia without an increase in respiratory effort should lead one to suspect an anomaly in the neural control of breathing caused by central nervous system injury, drug-induced inhibition, or dysfunction of spinal motor neurons or nerve fibers innervating the respiratory muscles.

INITIAL ASSESSMENT The first priority in evaluating a child with respiratory distress is to assess the airway, breathing, and circulation (ABC’s). Rapid assessment and intervention can prevent the progression from respiratory compromise to respiratory failure. To assess the airway, determine whether it is patent, stable, and maintainable. If not, such an airway must be established (e.g. repositioning, instrumentation). Once these three criteria are met, the breathing process is evaluated by determining the respiratory rate, oxygen saturation, adequacy of breath sounds, and quality of respiratory effort (e.g. labored, use of accessory muscles). Determination of arterial blood gas levels may be needed if there is evidence of respiratory insufficiency or failure. If spontaneous ventilation is inadequate, assisted breathing with positive pressure, initially via a bag-mask device, is indicated. Assessment of the patient’s circulation and mental status are other key aspects of the initial evaluation. Based on this rapid assessment, the clinician obtains information about the severity of the patient’s condition and how rapidly interventions must be performed. Providing supplemental oxygen is an important first step for a child presenting in distress, especially with hypoxemia demonstrated by oximetry. Oxygen can be delivered by multiple devices, and the choice depends on the child’s clinical status and oxygen needs (see Chapter 195). Some of these

devices can agitate a child further, worsening respiratory compromise and increasing the metabolic demand for oxygen. Allow the child to remain with the parent as much as possible, and use the least noxious form of oxygen delivery necessary. In a small number of children, airway adjuncts such as a nasopharyngeal airway, oropharyngeal airway, or assisted ventilation are needed. An oropharyngeal airway holds the tongue forward to prevent airway obstruction, so it will not be tolerated in a conscious or semiconscious patient with an intact gag reflex, and it may induce vomiting. A nasopharyngeal airway can be used in a conscious or unconscious patient but should be avoided in those with facial trauma. Continuous positive airway pressure may assist inspiratory efforts as well as provide positive end-expiratory pressure (PEEP). PEEP provides resistance to expiration, which may help expand atelectatic portions of the lung and thereby reduce ventilation-perfusion mismatch. Bilevel positive airway pressure provides inspiratory and expiratory pressure via a firmly fitting facemask, which may be uncomfortable and not well tolerated in infants or young children (see Chapter 195). Careful reevaluation after every intervention is critical to the care of a child with respiratory compromise. This confirms proper application of the intervention and monitors the patient’s response.

PATIENT HISTORY Therapeutic interventions in an ill child should not be delayed to perform a detailed history. A focused history includes an assessment of respiratory and systemic symptoms (Box 35-1). The history will guide the practitioner toward the diagnostic possibilities and appropriately focus the management. For children with special needs or chronic respiratory problems, ask about the child’s baseline respiratory status, how this episode is different, and what interventions have been successful in the past. Box 35-1 Components of a Focused History Onset, duration, chronicity, character of symptoms Alleviating and provoking factors Treatment to date

Activity level Respiratory symptoms Cold symptoms Cough (wet, dry, time of day) Trouble breathing (rapid breathing, retractions, abdominal muscle use, “seesaw” respirations, positional distress) Color change (pale, cyanotic) Altered pattern of breathing (periodic, shallow or deep, bradypnea, apnea) Inability to clear upper respiratory tract because of weakness or poor gag Systemic symptoms Fever Poor feeding, fluid intake Urine output Weight loss or failure to gain weight Emesis or diarrhea Past medical history Special healthcare needs or preexisting medical conditions (complex heart disease, chronic infections, prematurity) Respiratory disease (asthma, cystic fibrosis) Family history of atopy, cardiac or respiratory disease Medications (respiratory treatments, oral medications, herbal supplements) Allergies (medications, foods, environment) Immunization status Last oral intake (in case airway management becomes necessary)

PHYSICAL EXAMINATION

Certain features of the physical examination are particularly important in determining the severity of respiratory distress and localizing the primary cause (Box 35-2). Vital signs help determine cardiopulmonary status but may be influenced by various factors. Fever increases the respiratory rate by approximately three to seven breaths per minute for each degree Celsius above normal.3,4 Tachycardia commonly occurs as a response to the increased sympathetic tone in a child with respiratory distress; sympathetic tone is also increased with fever. Bradycardia is a late and ominous sign of severe hypoxia. Pulsus paradoxus (>10 mmHg decrease in blood pressure during inspiration) can occur with severe pulmonary disease. Cyanosis is not evident until more than 5 g of hemoglobin are desaturated, which correlates with an oxygen saturation of less than 70% to 75%. Central cyanosis is evidenced by blue, gray, or dark purple discoloration of mucous membranes, most reliably assessed at the lips and tongue. Perioral cyanosis is not indicative of central cyanosis and likely represents local venous congestion. It is often seen with Valsalva-like maneuvers (e.g. crying) in fair-skinned infants. Acrocyanosis is often present in healthy newborns; in older children, it may be more suggestive of cardiovascular status (e.g. peripheral vasoconstriction secondary to hypotension). Pulse oximetry is a useful, noninvasive means of assessing hypoxia, but it has some limitations. It can be unreliable with low blood pressure, movement, or arterial PO2 less than 50 mmHg, if hemoglobin is bound to molecules other than oxygen (e.g. carbon monoxide), or if significant methemoglobinemia is present. Box 35-2 Directed Physical Examination General Level of consciousness (alertness, responsiveness) Preferred position of child Color Failure to thrive Signs of prematurity or trauma Vital signs Respiratory

Airway Nasal flaring Inspiratory noises: seal-like barking, sonorous (nasal or pharyngeal), harsh (tracheal), high pitched (intrathoracic), stertor (gurgling) Symmetry of palate and tonsils Prominence of posterior pharynx Drooling, dysphagia Inspection Depth and quality of respirations Retractions (subcostal, supraclavicular, intercostal) Accessory muscle use Symmetry of chest wall movement Shape of chest (scoliosis, pectus excavatum or carinatum) Auscultation Air entry Inspiration-to-expiration ratio Rales or crackles Wheezing (inspiratory or expiratory) Symmetry of breath sounds Presence of pleural rub Cardiac Murmur Rhythm Heart sounds Thrill, rub, gallop Quality of pulses Capillary refill time Color Abdomen

Hepatomegaly Splenomegaly Mass Ascites Extremities Clubbing Deformities Neurologic Focal deficit Cranial nerves Muscle tone and strength Evidence of increased intracranial pressure Restlessness or anxiety is associated with early hypoxia (“air hunger”), but the mental status can progress to somnolence and lethargy with hypercarbia and severe hypoxemia. The position of comfort can provide information about the location of dysfunction. Children with upper airway obstruction often sit upright with the neck extended to open the airway. Infants with laryngomalacia have more pronounced stridor in the supine position compared with the prone position. Inspiratory and expiratory noises can help localize the underlying process and may suggest cause. Stridor is a predominantly inspiratory monophasic noise indicating an extrathoracic airway obstruction. Upper airway obstruction can lead to acute deterioration and severe morbidity and mortality. Expiratory wheezing or stridor usually reflects intrathoracic obstruction; however, biphasic stridor may suggest a fixed lesion at any point along the airway. Grunting represents a child’s attempt to increase the PEEP and raise the functional residual capacity by closing the glottis during expiration. Auscultation along the entire airway, including the nose, mouth, neck, and chest, may provide localizing information. The presence of rales, wheezing, or asymmetrical breath sounds and the duration of inspiration versus expiration are particularly noteworthy. The ratio of inspiration to expiration (I/E) is normally 1:1. Extrathoracic obstruction tends to prolong

the inspiratory phase (e.g. I/E ratio of 3:1), whereas lower airway obstructive pathology, such as asthma or bronchiolitis, produces a prolonged expiratory phase (e.g. I/E ratio of 1:3). Respiratory distress may be caused by nonrespiratory pathology or may have an impact on other organ systems. Detailed cardiac, abdominal, musculoskeletal, skin, and neurologic examinations should be performed.

DIFFERENTIAL DIAGNOSIS Respiratory difficulties may result from abnormalities within the respiratory system or from the dysfunction of organ systems influencing the respiratory system. Within the respiratory system, problems can arise from upper airway obstruction, lower airway obstruction, changes in gas diffusion from the alveolus to the capillary, abnormal pulmonary blood flow, or alterations in nerves and muscles that control breathing. Disease in other organ systems can compromise respiratory function directly or may induce compensatory respiratory mechanisms (Tables 35-1 and 35-2). TABLE 35-1

Differential Diagnosis of Respiratory Distress

Respiratory System

Other Organ Systems

Upper airway (nasopharynx, oropharynx, larynx, trachea, bronchi)

Central nervous system

Anatomic: craniofacial abnormalities, choanal atresia, tonsillar hypertrophy, macroglossia, midface hypoplasia, micrognathia, laryngomalacia, tracheomalacia, hemangioma, webs, cysts, laryngotracheal cleft, papilloma, subglottic stenosis, vocal cord paralysis,

Structural abnormality: agenesis, hydrocephalus, mass, arteriovenous malformation Infectious: meningitis, encephalitis, abscess, poliomyelitis Dysfunction or immaturity:

tracheal stenosis, fistula, bronchomalacia, bronchogenic cyst

apnea, hypoventilation, hyperventilation

Infectious: nasal congestion, peritonsillar abscess, tonsillitis, croup, epiglottitis, retropharyngeal abscess, tracheitis, bronchitis

Inherited degenerative disease: spinal muscular atrophy

Environmental or traumatic: chemical or thermal burn, aspiration of foreign body

Intoxication or toxins: alcohol, barbiturates, benzodiazepines, opiates, tetanus

Mass, including malignancy Inflammatory: angioneurotic edema, anaphylaxis

Seizure Trauma: hemorrhage, birth asphyxia, spinal cord injury, anoxic encephalopathy

Lower airway (bronchioles, acini, interstitium) Inflammatory: asthma, allergy, angioneurotic edema, meconium aspiration, near drowning, gastroesophageal reflux, aspiration

Other: transverse myelitis, acute paralysis, myopathy

Infectious: bronchiolitis, pneumonia (bacterial, viral, atypical bacterial, Chlamydia, Pertussis, fungal, Pneumocystis), abscess, empyema

Peripheral nervous system

Congenital malformation:

Phrenic nerve injury

congenital emphysema; cystic adenomatoid malformation; sequestration; pulmonary agenesis, aplasia, or hypoplasia; pulmonary cyst

Environmental or toxins: tick paralysis, heavy metal poisoning, organophosphates, botulism, snakebite

Environmental or traumatic: thermal or chemical burn, smoke, carbon monoxide, hydrocarbon, drug-induced pulmonary fibrosis, bronchopulmonary traumatic disruption, pulmonary contusion

Metabolic: inborn errors of metabolism, carnitine deficiency, porphyria

Other: bronchiectasis, interstitial lung disease, bronchopulmonary dysplasia, cystic fibrosis, pulmonary edema, hemorrhage, embolism, atelectasis, mass

Other: Guillain-Barré syndrome, multiple sclerosis, myasthenia gravis, muscular or myotonic dystrophy, muscle fatigue

Chest wall and intrathoracic Pneumothorax, tension pneumothorax Pneumomediastinum Pleural effusion Empyema Chylothorax Hemothorax

Inflammatory: dermatomyositis, polymyositis

Cardiovascular Structural: congenital heart disease, pericardial effusion, pericardial tamponade, aortic dissection or rupture, mass, coronary artery dilation or aneurysm, pneumopericardium, great vessel anomalies Other: arrhythmia, myocarditis, myocardial ischemia or infarction, congestive heart failure

Diaphragmatic hernia Cyst, mass Spinal deformity (kyphoscoliosis)

Gastrointestinal

Pectus excavatum or carinatum

Appendicitis

Rib fracture, flail chest

Necrotizing enterocolitis Mass (including hepatomegaly, splenomegaly) Ascites Obstruction Perforation, laceration Hematoma Contusion Esophageal foreign body Abdominal pain Metabolic and endocrine Acidosis: fever, hypothermia, dehydration, sepsis, shock, inborn errors of metabolism, liver disease, renal disease, diabetic ketoacidosis, salicylates

TABLE 35-2

Common Causes of Respiratory Distress

Neonate

Infant or Child

Transient tachypnea of the newborn

Bronchiolitis

Sepsis

Pneumonia

Respiratory distress syndrome

Asthma

Pneumonia

Croup

Nasal obstruction or airway anomalies

Foreign body aspiration

Congenital heart disease

Fever Sepsis Metabolic derangements Allergy

DIAGNOSTIC EVALUATION Further workup may be indicated to evaluate the severity of illness or its cause and should be guided by the clinical assessment. In children with chronic disease, compare laboratory studies and radiographs to previous studies, if available. Pulse oximetry can detect hypoxemia but does not provide direct information about the adequacy of ventilation. Exhaled carbon dioxide levels can be measured with noninvasive devices, but this determination is used most often in the setting of endotracheal intubation. Arterial blood gas analysis can assess oxygenation (arterial PO2) and ventilation (arterial PCO2) and detect metabolic derangements of pH, bicarbonate, and base excess. Venous blood samples can provide some information about ventilation but are less useful for assessing oxygenation. The utility of obtaining other laboratory studies depends on the clinical setting and the diagnostic considerations. Radiographs may be an important adjunct in the evaluation of a child

with respiratory disease. Chest radiographs (anteroposterior and lateral views) are performed upright and on full inspiration whenever possible. Quality images can assist in the identification of airspace disease, hyperinflation, effusion, mass, foreign body, chest wall deformity or trauma, or cardiac abnormality. If an effusion is suspected, decubitus films may be helpful to determine whether the fluid shifts with gravity (“layers out”). Inspiratory and forced expiratory or bilateral decubitus chest films are useful to evaluate for the presence of a foreign body or other localized lower airway obstruction. An obstructing lesion can cause air trapping distal to the site. On an expiratory film, the unobstructed lung tissue decreases in volume and appears more dense, whereas the obstructed region is relatively hyperlucent. With decubitus films, the dependent (inferiorly positioned) lung should decrease in volume and appear denser, except for an obstructed region that remains inflated due to air trapping. When the patient’s position is switched, the affected lung is superior and appears fully aerated, making the obstructed portion difficult to appreciate. If the obstruction is long-standing, the obstructed portion may become atelectatic or fluid filled and appear as a persistent density in the various views. Lateral and anteroposterior radiographs of the neck are useful for the evaluation of upper airway obstruction, allowing the visualization of retropharyngeal, supraglottic, and subglottic spaces. These films may be useful to diagnose retropharyngeal abscess, epiglottitis, foreign body aspiration, or tracheitis. Ill children with suspected upper airway obstruction should not be sent to the radiology department unattended or should have films deferred until they are stabilized. Other imaging modalities to consider are computed tomography (CT), magnetic resonance imaging (MRI), airway fluoroscopy, and barium swallow studies. CT evaluates cervical and thoracic structures in greater detail and may help identify areas of intrinsic or extrinsic compression. Mediastinal, pleural, and pulmonary parenchymal abnormalities are well identified with CT. MRI is particularly helpful to delineate hilar and vascular anatomy. Airway fluoroscopy provides dynamic pictures of the respiratory system, including the airways and diaphragm. Barium swallow studies are helpful in the evaluation of patients with recurrent pneumonia, persistent cough, stridor, or wheezing. The anatomy of the gastrointestinal tract from the mouth to the stomach is revealed, and compression along the esophagus may suggest a lesion that is compressing the airway as well. Contrast material is mixed with

substances of different consistencies and textures, ranging from thin liquids to thick liquids to solids. Barium swallow studies evaluate swallowing mechanics and the presence of vascular rings, tracheoesophageal fistulas, and gastroesophageal reflux.

MANAGEMENT Acute interventions should not be delayed in a child with respiratory distress, regardless of the underlying cause. Restoration of oxygenation and ventilation is the main priority. Airway patency should be established and maintained, followed by a rapid assessment of breathing and circulation. Oxygen delivery should be initiated using the least noxious form possible that provides an adequate fraction of inspired oxygen (FIO2), and the child should be placed on cardiopulmonary monitors, including pulse oximetry. Airway adjuncts may be necessary to maintain patency. If these are unsuccessful, or if oxygenation and ventilation are inadequate, endotracheal intubation may be indicated. Breathing treatments (albuterol, racemic epinephrine, ipratropium bromide) may be useful in certain clinical situations, including asthma, bronchiolitis, or croup. Further therapies are determined by the underlying cause of respiratory distress. KEY POINTS Do not delay the management of a compromised child to perform detailed history and physical examination. Clinical history and physical examination are critical to determining likely etiologies of respiratory compromise. Respiratory difficulties may arise from causes other than abnormalities within the respiratory system.

SUGGESTED READINGS Barren JM, Zorc JJ. Contemporary approach to the emergency department management of pediatric asthma. Emerg Med Clin North Am. 2002;20:1. Haddad GG, Palazzo RM. Diagnostic approach to respiratory disease. In:

Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. Philadelphia: WB Saunders; 2004:1363-1370. Infosino A. Pediatric upper airway and congenital anomalies. Anesthesiol Clin North Am. 2003;20:4. Ruddy RM. Evaluation of respiratory emergencies in infants and children. Clin Pediatr Emerg Med. 2002;3:156-162. Stevenson MD, Gonzalez del Rey JA. Upper airway obstruction: infectious cases. Clin Pediatr Emerg Med. 2002;3:163-172.

REFERENCES 1. Weiner DL. Respiratory distress. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. Philadelphia, PA: Lippincott Williams & Wilkins; 2000:553-564. 2. Perez Fontan JJ, Haddad GG. Respiratory pathophysiology. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. Philadelphia: WB Saunders; 2004:1376-1379. 3. O’Dempsey TJ, Laurence BE, McArdle TF Todd JE, Lamont AC, Greenwood BM. The effect of temperature reduction on respiratory rate in febrile illnesses. Arch Dis Child. 1993;68(4):492. 4. Gadomski AM, Permutt T, Stanton B. Correcting respiratory rate for the presence of fever. J Clin Epidemiol. 1994;47(9):1043.

Shock

CHAPTER

36

Raina Paul and Andrew M. Fine

BACKGROUND Shock is a clinical condition that occurs when there is inadequate delivery of oxygen and other nutrients to meet the metabolic demands of the tissues. If left untreated, shock results in irreversible cell and organ damage and death. The clinician must be able to recognize shock early, initiate therapy rapidly, and arrange safe transport of the patient to an intensive care facility. These lifesaving tasks require that the clinician have a fundamental knowledge of the causes, presentations, therapies, and complications of shock. Physiologically, shock can be classified as compensated, when the patient is able to maintain a normal blood pressure for age, or decompensated, when deterioration has led to hypotension. In general, compensated shock progresses to decompensated shock if left untreated, which emphasizes the importance of early recognition and intervention. Children can generally maintain a normal blood pressure until advanced stages of shock; therefore hypotension (see Table 36-1 for age-based definitions of hypotension) in a pediatric patient is an ominous sign of impending circulatory collapse. When measuring blood pressure with a sphygmomanometer, it is important to select the smallest cuff that covers two-thirds of the upper arm or leg. TABLE 36-1

Hypotension Parameters

Age

Minimum Systolic Blood Pressure (mmHg)

Term neonate (0–28 days)

60

Infant (1–12 mo)

70

Child (1–10 y)

70 + (2 × age in years)

Older than 10 y

90

PATHOPHYSIOLOGY AND DEFINITIONS Shock can also be classified by cause, with the main types being hypovolemic, cardiogenic, and distributive shock, which includes septic shock. The mechanisms may differ, but inadequate tissue perfusion is the common final pathway.

HYPOVOLEMIC SHOCK Hypovolemic shock, by far the most common type of shock in children, occurs when a decrease in intravascular volume leads to decreased venous return and subsequently, decreased preload. Decreased preload results in decreased stroke volume. An increase in heart rate often maintains cardiac output initially, but when this compensatory response is inadequate, cardiac output diminishes. The formula that defines this relationship is as follows: Cardiac output = Heart rate × Stroke volume Decreased cardiac output results in decreased delivery of oxygen and other substrates to the tissues. The two main categories of hypovolemic shock are hemorrhagic and non-hemorrhagic; examples are provided in Table 362. TABLE 36-2

Causes of Hypovolemic Shock

Nonhemorrhagic

Hemorrhagic

Vomiting or diarrhea

Trauma

Diabetes insipidus, diabetes mellitus

Gastrointestinal bleeding

Heat stroke

Postsurgical bleeding

Burns

Sequestration crisis

Intestinal obstruction

Splenic rupture

Water deprivation Adrenal insufficiency Hypothyroidism In the early stage of hypovolemic shock, autoregulatory mechanisms shunt blood flow preferentially to the brain, heart, and adrenal system thus preserving blood pressure. Because flow is diverted from less critical organs, patients may present initially with cool or mottled extremities and decreased urine output. Other signs may include dry mucous membranes, absence of tears, and abnormal skin turgor. Hemorrhagic shock due to known trauma is typically diagnosed at the initial presentation; however, hemorrhagic shock can present during hospitalization, especially in postoperative patients. Victims of child abuse are also at risk for delayed diagnosis of hemorrhagic shock because the initial history may be incomplete, inaccurate, or misleading, and symptoms may progress over time. Nonhemorrhagic shock may present in patients with ongoing fluid losses (e.g. vomiting, diarrhea, gastric suctioning, burns), especially if there is inadequate replacement.

DISTRIBUTIVE SHOCK Distributive shock is caused by a decrease in systemic vascular resistance. Abnormalities in vasomotor tone cause peripheral pooling of blood, which leads to a diminished effective preload, decreased cardiac output, and inadequate tissue perfusion. This process can occur without frank fluid loss. Causes of distributive shock are listed in Table 36-3. TABLE 36-3 Anaphylaxis Medications Foods

Causes of Distributive Shock

Envenomations or stings Blood products Latex Neurologic Head injury Spinal shock Septic shock Drugs Septic Shock Sepsis is a clinical syndrome that results from overwhelming infection creating a cascade of endogenous inflammatory mediators resulting in widespread tissue injury. Shock occurring during sepsis begins with volume maldistribution due to leaky vessels, resulting in intravascular volume depletion, decreased flow to vital organs including the heart, and ultimately metabolic derangements at the cellular level. It is important to consider septic shock early, because a seemingly stable patient with minimal findings of infection can quickly progress to sepsis. In the early (compensated) phase, often designated as “warm shock,” septic shock may present with a hyperdynamic state including decreased vascular resistance, widened pulse pressure, increased cardiac output, tachycardia, tachypnea, warm extremities, and normal urine output. As it progresses to the decompensated phase, patients develop intravascular volume depletion, myocardial depression, cool extremities, thready pulses, central nervous system changes, respiratory distress, and decreased cardiac output, often denoted as “cold shock.” Although anyone can develop septic shock, risk factors include young age, chronic medical condition, presence of central intravenous (IV) catheters, immunocompromise, burns, and malnutrition. Septic shock can result from bacterial, viral, or fungal infection. It is important to note that septic shock can occur when cultures or other diagnostic tests do not yield a definitive organism. Several studies have demonstrated that an organism is recovered only 50% of the time in the pediatric population. Shock can also develop from localized bacterial infections that produce toxins; this is referred to as toxic shock. This form of shock is most commonly associated with toxin-producing strains of group A Streptococci and Staphylococcus

aureus. Other Forms of Distributive Shock Anaphylactic shock occurs when an exogenous stimulus causes an allergic systemic immunoglobulin E–mediated response that triggers the release of histamine and other vasoactive factors from mast cells, with resulting vasodilation (see Chapter 47). Neurogenic shock may occur with spinal cord transection above the first thoracic level, with severe injuries to the brainstem or with isolated intracranial injuries. An injury to the cervical cord may result in unopposed parasympathetic tone and subsequent vasodilation. Some drugs can cause severe vasodilation, resulting in shock; these drugs include those that cause anaphylaxis and those that cause severe hypotension (e.g. β-antagonists and calcium channel antagonists).

CARDIOGENIC SHOCK Cardiogenic shock occurs when there is decreased cardiac output caused by pump failure. The main causes are listed in Table 36-4. Excluding patients with congenital heart disease, cardiogenic shock is much less common in children than in adults because of the relatively low incidence of coronary artery disease and congestive heart failure in the pediatric population. Cardiogenic shock should be strongly considered in the following clinical scenarios: no history of fluid losses, physical examination reveals hepatomegaly or rales, chest radiograph demonstrates cardiomegaly, and when there is no clinical improvement despite oxygenation and volume expansion. Worsening symptoms, including persistent tachycardia or shortness of breath, during volume resuscitation should also heighten one’s suspicion for cardiogenic shock. TABLE 36-4

Causes of Cardiogenic Shock

Cardiomyopathies Familial Infectious Infiltrative Idiopathic Arrhythmias

Ventricular fibrillation Supraventricular tachycardia Bradycardia Complete heart block Mechanical defects Congenital heart disease Coarctation of the aorta Cardiac tumor Obstructive disorders Pulmonary embolism Tension pneumothorax Constrictive pericarditis Pericardial tamponade Pulmonary hypertension Ischemia or infarction Anomalous coronary artery Kawasaki disease Management of cardiogenic shock should focus on correcting arrhythmias if present, improving preload and cardiac contractility, and reducing afterload. The stress on the heart can be minimized by decreasing metabolic demand including achieving normothermia, correction of anemia if present, and sedation, intubation, and mechanical ventilation if necessary. Although cardiogenic shock is uncommon as the primary cause of shock in children, it may be a late manifestation of other forms of shock.

PROGRESSION OF SHOCK In early, or compensated, shock, pediatric patients are able to maintain cardiac output and blood pressure by increasing the heart rate and peripheral vascular resistance. With further compromise of cardiac function, the compensatory tachycardia is insufficient to maintain cardiac output. Decreased perfusion affects many systems, causing metabolic acidosis,

oliguria, elevated transaminases from hepatocellular injury, cool extremities, mottling of the skin, and ileus. Tachypnea develops to increase the elimination of carbon dioxide, which helps compensate for the metabolic acidosis. In the late stages of shock, end-organ failure may manifest as central nervous system depression (ranging from confusion and agitation to coma), anuria, uremia, hyperkalemia, acute respiratory distress syndrome, and coagulopathies. Specific organ dysfunctions that are the result of shock are outlined in Table 36-5. TABLE 36-5

Organ Dysfunction in Shock

Cardiovascular • Decreased BP 50% FiO2 needed for sat >92% • CO2 >65 mm Hg on blood gas • PaO2/FiO2 2 × upper limit of normal or 2-fold increase in baseline Hepatic • bilirubin ≥4 mg/dL (not for newborns)

• ALT 2 × upper limit of normal

PATIENT HISTORY Early recognition of shock is important. Table 36-6 identifies patients at increased risk for developing shock. The history should elicit whether the patient falls into one of these high-risk categories. Additional questions should be directed at recent exposures through travel, trauma or infectious agents, recent therapies such as medications, fluids, and procedures, and allergies to medications, foods, and environmental chemicals such as latex. TABLE 36-6

Patients at Increased Risk for Shock

Febrile infants Immunocompromised patients Neonates Patients with congenital heart disease Patients with excessive fluid losses Patients with central venous catheter

PHYSICAL EXAMINATION The physical examination (Table 36-7) should focus on aspects that will help the clinician distinguish among the different causes of shock. TABLE 36-7

Directed Physical Examination

Vital signs Skin Rash Petechiae Ecchymosis Erythroderma Mottling

Head, eyes, ears, nose, and throat Evidence of head trauma Pupil size and reactivity Conjunctival pallor Central cyanosis (lips, tongue) Neck Meningismus Cervical spine tenderness Lungs Abnormal breathing pattern (e.g. Kussmaul) Retractions Wheezes or rales (focal or bilateral) Diminished breath sounds or dullness to percussion Heart Hyperdynamic Murmur Rub Gallop Abdomen Tenderness, rebound, or guarding Distention or ascites Mass or organomegaly Extremities Temperature Peripheral pulses Clubbing Neurologic Mental status Food deficit

DIFFERENTIAL DIAGNOSIS Shock, once recognized, should be treated immediately and attention to specific underlying causes should be pursued only after the basic resuscitation needs are met. Shock in a neonate may be the result of any of the previously mentioned causes, but the differential diagnosis of neonatal shock should also include those listed in Table 36-8. Likewise, the diagnosis of shock may be more challenging in medically complicated patients as they can have abnormal vasomotor responses resulting in widely variable blood pressures and heart rates. These patients may also be unable to mount an adequate immune response, or possess internal hardware that could harbor infection. TABLE 36-8

Special Considerations in Neonatal Shock

Congenital adrenal hyperplasia Inborn errors of metabolism Left-sided cardiac obstructive lesions Aortic stenosis Hypoplastic left heart syndrome Critical coarctation of the aorta Interrupted aortic arch Blood loss at delivery Perinatal infection Abdominal catastrophe (midgut volvulus)

DIAGNOSTIC EVALUATION Diagnostic tests should focus on the aforementioned organ dysfunctions and tests that may aid identify the inciting cause of shock (Table 36-9). A lactate should always be checked, though elevation often occurs only late in disease course. Many pediatric patients in shock will have a normal lactate and thus the clinician should not be falsely reassured. TABLE 36-9

Diagnostic Tests Considered for Patients

in Shock Complete blood count with differential Serum chemistries Electrolytes Blood urea nitrogen Creatinine Glucose* Liver enzymes including bilirubin Pancreatic enzymes Cardiac enzymes Lactic acid Coagulation studies Prothrombin time Partial thromboplastin time Fibrinogen Fibrin split (degradation) products Arterial blood gas Type and crossmatch Cultures Blood Urine Cerebrospinal fluid Pleural fluid Abscess drainage Toxicology Urine Serum Urinalysis Imaging Chest radiograph

Abdominal radiograph or obstruction series Electrocardiogram *Consider a bedside rapid blood glucose test (Dextrostix).

MANAGEMENT A pediatric patient in shock represents a medical emergency. For this reason, identification of the precise cause of shock may be delayed while resuscitation and stabilization proceed. Attention to the ABCs (airway, breathing, and circulation) remains a basic principle in the initial assessment and management. In general, increasing oxygen delivery to the tissues and decreasing oxygen demand are key. General time goals for resuscitation are delineated in Figure 36-1. It is imperative that the entire care team is aware of the timing of the resuscitation interventions. Use of a visible countdown clock is often helpful in keeping these time constraints at the forefront of resuscitation efforts. Adherence to timely fluid and algorithm goals has been shown to decrease hospital and intensive care length of stay as well as decrease mortality in the community setting. Using a standardized protocol is useful in ensuring all algorithm components are met. If there is suspicion of trauma, cervical spine immobilization should be maintained until the spine can be evaluated. The primary survey of a pediatric patient in shock is directed toward the rapid identification of immediately life-threatening conditions that should be addressed before a more extensive evaluation.

FIGURE 36-1. Resuscitation goals in shock. (Data from Pediatric Advanced Life Support Manual. American Heart Association; 2010 and Brierley J, et al. Clinical practice parameters for hemodynamic support of pediatric and neonatal shock. Crit Care Med. 2009;37(2):666-88.) A stable airway should be established and maintained, and supplemental oxygen should be provided to keep arterial oxygen saturation greater than 95%. Hypoxemia should be corrected with generous oxygen supplementation, but all patients should receive oxygen even if the saturation is normal. Evaluating peripheral perfusion and pulses provides a rapid assessment of the circulation. Continuous cardiorespiratory monitoring and frequent blood pressure measurement are important to assess the response to intervention. The standard of two large-bore IV lines may not be feasible, especially in very young pediatric patients in shock, but the goal is to maintain reliable intravascular access through which vigorous resuscitation can proceed. If peripheral IV access is not immediately obtained, intraosseous access should be considered. (see Chapter 192). Unless the patient has myocardial or known chronic renal disease, the initial therapy should be 20 mL/kg of isotonic fluid (normal saline or lactated Ringer’s) administered intravenously as rapidly as possible. This may be repeated two more times, to a total of 60 mL/kg, with close clinical assessment of vital signs, perfusion, urine output, and mental status for improvement. A fourth bolus for a total of

80 mL/kg is often needed. In the case of hemorrhagic shock, blood products should be used as soon as possible. Until appropriate blood products are available, isotonic IV fluids are used as a temporizing measure to restore intravascular volume. If the patient remains hypotensive and poorly perfused despite 60 mL/kg of IV fluids, termed fluid-refractory shock, inotropic support should be strongly considered. Dopamine IV infusion is commonly initiated at a rate of 10 μg/kg per minute and then titrated to improve and maintain blood pressure, peripheral perfusion, and urine output. In patients who do not respond to dopamine, IV epinephrine, norepinephrine, or both may be considered. These three vasoactive agents can be initiated peripherally for up to 4 hours until central access is obtained. Hydrocortisone (1–2 mg/kg IV) should also be considered for patients who show resistance to vasoactive agent or have adrenal suppression, usually from chronic medications such as steroids. A chest radiograph may be helpful to assess the presence of cardiomegaly, fluid overload, pulmonary edema, pneumonia, pleural effusion, or pneumothorax. Central venous pressure is also helpful, if obtainable, so that the degree of volume repletion can be assessed. Antipyretics should be administered, when feasible, to a febrile patient to decrease metabolic demand. Conversely, warming should be instituted in a hypothermic patient (see Chapter 173). Broad-spectrum antibiotics should be given intravenously or intraosseously unless it is known that infection is not the cause of the patient’s condition. The choice of antibiotic is driven by the likely pathogens (Table 36-10) and the age of the patient (Table 36-11). TABLE 36-10

Pathogens in Septic Shock

Neonate and Young Infant

Infant and Young Child

Group B streptococci

Neisseria meningitides

Escherichia coli and other gram-negative bacilli

Streptococcus pneumoniae

Enterococci

Haemophilus influenzae type B

Herpes simplex virus

TABLE 36-11

Group A streptococci Staphylococcus aureus Rickettsieae

Antimicrobials in Shock

Age

Antimicrobial Agent

Initial IV Dose (mg/kg)

0–4 wk

Ampicillin

50–100*

plus Gentamicin

2.5

or

>4 wk

Cefotaxime

50

Ceftriaxone

50–100*

Consider adding vancomycin

10–15*

Immunocompromised Cefepime

25–75*

or Piperacillin/tazobactam 100+ Consider vancomycin

10–15*

Possible rickettsial infection

Add doxycycline

2.2

Allergic to cephalosporins

Clindamycin and

25–40

Gentamicin

2.5–7.5

*Higher dosing recommended for possible meningitis. †Based

on piperacillin component.

In a neonate with shock in whom a duct-dependent cardiac lesion is a possible cause, prostaglandin E1 should be considered to maintain patency of the ductus arteriosus. Preparation should be made for the possibility of intubation, because prostaglandin E1 may cause apnea. The decision to administer prostaglandin E1 is usually made in consultation with a neonatologist or pediatric cardiologist. Anaphylactic shock management should include administration of intramuscular epinephrine, antihistamines such as diphenhydramine, and corticosteroids in addition to supportive measures listed above. Continuing monitoring is essential, as development of a biphasic reaction can occur (see Chapter 47).

SPECIAL CONSIDERATIONS TRANSPORT OF THE PATIENT IN SHOCK Early initiation of transport can be life- and limb-saving for patients in shock. The child should be hospitalized in an intensive care unit capable of invasive monitoring and airway intervention and with personnel who are experienced in managing critically ill children. If transfer to another institution is necessary, the clinician has the challenging task of weighing the risks and benefits of the different modes of transportation (see Chapter 11). In every case, whether the patient is transferred to another facility or from one unit to another within the same facility, it is essential that the team transporting the patient have the appropriate personnel and equipment to do so safely. In addition, the receiving facility or unit must be ready for the patient’s arrival. Communication of the patient’s condition and the interventions performed is key, and medical records, imaging studies, and laboratory results should accompany the patient whenever possible. KEY POINTS Shock can be classified as hypovolemic, cariogenic, or distributive. Hypotension is often a late finding in pediatric shock. Early recognition of shock and rapid intervention is crucial.

SUGGESTED READINGS Carcillo JA, Fields AI. Clinical practice parameters for hemodynamic support of pediatric and neonatal patients in septic shock. Crit Care Med. 2002;30:1365-1378. Cruz AT, Perry AM, Williams EA, et al. Implementation of goal-directed therapy for children with suspected sepsis in the emergency department. Pediatrics. 2011;127:e758-766. Dellinger RP, Levy MM, Rhodes A, et al. Surviving Sepsis Campaign guidelines for management of severe sepsis and septic shock. Intensive Care Med. 2013;39:165-228. Larsen GY, Mecham N, Greenberg R, et al. An emergency department septic shock protocol and care guideline for children initiated at triage. Pediatrics. 2011;127:e1585-e1592. Pediatric Advanced Life Support Provider Manual. Dallas, TX: American Heart Association; 2010. Paul R, Neuman MI, Monuteaux M, Melendez E. Adherence to PALS sepsis guidelines and hospital length of stay. Pediatrics. 2012;130:e273-e280.

CHAPTER

37

Syncope Sarah C. McBride

BACKGROUND Syncope, also described as “fainting” or “blackouts,” is defined as a transient period of unconsciousness that is brief and abrupt in onset and resolves spontaneously to a restored level of function. It is a symptom rather than a diagnosis and does not necessarily indicate disease. The causes of syncope in children are heterogeneous and are most often benign in nature. Nevertheless, syncopal events can cause significant distress among patients, families, school personnel, and physicians. Since it is a relatively common occurrence in childhood, hospitalists should have a practical approach to the evaluation of pediatric syncope, focusing on identification of those rare individuals who are at risk for serious underlying disease.

PATHOPHYSIOLOGY Patients experience syncope when inadequate cerebral perfusion results in a transient loss of consciousness and postural tone. Presyncope describes a similar event without full loss of consciousness. Girls present more commonly for the evaluation of syncope than boys, with a peak incidence between 13 and 19 years of age.1 Most syncope in pediatric patients is autonomic in origin, isolated, and benign. In fact, the risk of sudden death among pediatric patients with prior syncopal events has been found to be equivalent to the risk of sudden death in the general population.2

PATIENT HISTORY History and physical examination can provide valuable clues to the probable

cause of a syncopal episode and may curtail or circumvent the need for further diagnostic evaluation. Important historical features of a patient presenting with syncope are included in Table 37-1. Recent circumstances and those immediately preceding the syncopal event should be thoroughly explored. Because patients are often unable to provide details of an episode, witnesses to the event are important. Past medical history, especially with regard to previous syncopal episodes, cardiac or neurologic conditions, and medications, should always be obtained. Specific inquiries about family history should include sudden death, syncope, sudden infant death syndrome, congenital heart disease, seizures, and congenital deafness (associated with a genetic disorder that includes Long-QT). A patient’s baseline level of health should also be ascertained, including any overall changes in energy level, exercise tolerance, nutritional status, or the development of new respiratory symptoms, chest pain, or tightness and any emotional stressors. TABLE 37-1

Directed Patient History for Syncope

Environmental factors/triggers (posture, temperature, hydration status, fatigue, illness) Frequency and duration of syncope Level of activity just prior to syncopal episode (exertion*) Elements of history from any witnesses (cyanosis*) Medication history (potential for lengthening QTc or arryhthmogenic*) Baseline level of health Generalized symptoms of concern (fatigue, cough, weight loss, shortness of breath, chest pain*) Personal or family history of any of the following conditions* congenital heart disease sudden cardiac death Long-QT syndrome sensorineural hearing loss other familial heart diseases *Increased concern for cardiac causes

PHYSICAL EXAMINATION Although the findings are often normal, a thorough physical examination should be performed. Global mental status should be evaluated and compared to the patient’s baseline level of function. Blood pressure measurements and evaluation of distal pulses in both the supine and upright positions and from both upper extremities and a lower extremity should be obtained. Cardiac examination may reveal a gallop, murmur, or click and there may be hepatomegaly or crackles on lung examination. Any of these examination findings raises the concern for cardiac disease. In addition, a complete neurologic examination is indicated. In young children and infants, a thorough skin and musculoskeletal examination should be performed to assess for any signs of trauma.

DIFFERENTIAL DIAGNOSIS The differential diagnosis of pediatric syncope is listed in Table 37-2. The etiologies of childhood syncope can be divided into three major categories: (1) autonomic, (2) cardiac, and (3) metabolic. Regardless of the cause, syncope is the end result of inadequate cerebral perfusion, oxygen, or glucose delivery to the brain. TABLE 37-2

Differential Diagnosis of Syncope

Cardiac Obstruction to blood flow Hypertrophic cardiomyopathy Valvular aortic stenosis Primary pulmonary hypertension Eisenmenger syndrome Myocardial dysfunction Dilated cardiomyopathy Arrhythmogenic right ventricular dysplagia/cardiomyopathy (ARVD/C) Mitral valve prolapse (with severe mitral valve regurgitation)

Neuromuscular disorders Inflammatory disease (e.g. Kawasaki disease, viral myocarditis) Ischemia (e.g. anomalous coronary artery) Arrhythmias Long QT syndrome (congenital or acquired) Supraventricular tachycardia Sinus node dysfunction Atrioventricular block Arrhythmogenic right ventricular dysplasia Brugada syndrome Autonomic Reflex (neurocardiogenic, situational) Dysautonomic (familial dysautonomia, drugs/toxins, immunemediated) Metabolic Hypoglycemia Electrolyte derangement Endocrine disorders Toxins and drugs

AUTONOMIC SYNCOPE Autonomic syncope is the most common cause of syncope in pediatrics and can be further described as either reflex or dysautonomic. Of these two types, reflex syncope is more common. Many triggers are known to precipitate autonomic syncope. Although their exact mechanisms of action are not clearly understood, physical stressors such as anemia, dehydration, hunger, illness, heat, and physical exhaustion can predispose patients to autonomic syncopal episodes. Orthostatic hypotensive syncope occurs during an excessive or prolonged decrease in blood pressure when an individual suddenly assumes an upright position or after prolonged standing. Patients with high basal vagal tone are at higher risk for autonomic syncope.

Neurocardiogenic syncope (often referred to as vasovagal or vasodepressor syncope) is the most common type of reflex syncope and can usually be diagnosed by gathering a careful and complete history, performing a thorough physical examination, and with minimal use of diagnostic testing. Postural changes and variation in cardiac output or blood volume are triggers for pressure sensors that send signals to the medulla by vagal C fibers. This signaling causes compensatory changes in heart rate, ventricular contractility, and blood vessel compliance by neurohormonal transmitters as a way to maintain cerebral perfusion. A common clinical presentation begins with a prodrome lasting seconds to minutes, followed by a brief period of unconsciousness, after which the patient returns to a previous level of alertness. The premonitory symptoms may include lightheadedness, dizziness, nausea, pallor, shortness of breath, diaphoresis, and visual changes. Figure 37-1 depicts one hypothesized mechanism for neurocardiogenic syncope, called the hypotensive cardioinhibitory response (Figure 37-2).

FIGURE 37-1. Autonomic syncope cascade. LV, left ventricular.

FIGURE 37-2. Hyypotensive cardioinhibitory response algorithm. a: exertional syncope, history of cardiac surgery, palpitations, chest pain, or shortness of breath; b: first-degree relative or multiple family members with sudden death before age 50 years, cardiomyopathy, pulmonary hypertension, or familial arrhythmia; c: pathological murmur, gallup, rub, loud or single S2, toxic appearance, marked tachycardia, tachypnea, peripheral edema, crackles on lung examination, hepatomegaly, or irregular heart rhythm. Dysautonomic syncope is caused by dysfunction of the autonomic nervous system and can occasionally lead to syncope in children. Pediatric causes for autonomic failure include familial dysautonomia, toxin exposure, and infectious or inflammatory disease. Syncope caused by a more variable dysfunction of the autonomic nervous system beyond what is typically seen in more straightforward neurocardiogenic syncope but without serious underlying disease, has been termed “postural orthostatic tachycardia syndrome” (POTS) or “chronic orthostatic intolerance.” Children and adolescents with more constant symptoms and disability related to orthostatic

intolerance fall into this category. Although the complete pathophysiology is not understood, a hallmark finding is tachycardia during upright posture.

CARDIAC SYNCOPE Syncope may occur when there is obstruction to cardiac outflow, myocardial dysfunction, or a cardiac arrhythmia. Patients who have a prior history of surgery for congenital cardiac lesions are often at increased risk for acquired and residual structural lesions, myocardial dysfunction, and supraventricular or ventricular arrhythmias. Therefore, a cardiologist should evaluate any patient with a prior cardiac history who presents with syncope. Cardiac syncope that is caused by an obstruction to blood flow usually occurs in association with exertion or exercise. It is often related to a fixed or dynamic obstruction to cardiac output due to a structural lesion or related to pulmonary vascular disease. Myocardial dysfunction may cause syncope due to an associated cardiac arrhythmia. However, when the myocardial dysfunction is severe enough to lessen stroke volume and cardiac output is inadequate to meet increased physical demands (e.g. exertion) syncope can result even in the absence of an arrhythmia. Bradycardia and tachycardia may also lead to syncope if effective stroke volume is detrimentally affected. Syncope due to isolated bradycardia in pediatrics is uncommon and is usually due to a medication effect, cardiac manifestations of severe anorexia, or central nervous system trauma. Sinus node pauses and first- and second-degree heart block do not commonly cause syncope. Tachycardia, originating from the sinus node or from an ectopic supraventricular focus, may lead to syncope and is often associated with chest pain, palpitations, lightheadedness, pallor, or nausea. Ventricular arrhythmias, such as Long-QT syndrome and Brugada syndrome, can occur in the absence of structural heart disease. Long-QT syndrome is characterized by a prolonged QT interval due to either a genetic defect in cardiac potassium or sodium channels with a resultant delay in repolarization. Syncope among patients with Long-QT syndrome is an ominous finding and is presumed to relate to an episode of torsades de pointes that terminates spontaneously. Brugada syndrome is caused by a genetic defect in cardiac sodium channels, which can lead to polymorphic ventricular tachycardia. ST segment elevation may be seen in the anterior precordial leads by electrocardiogram (ECG) in Brugada syndrome, but these

findings may also be intermittent. Even though mitral valve prolapse is generally asymptomatic in the pediatric population, it is worth mentioning because of its prevalence. It is now believed that when syncope occurs in patients with mitral valve prolapse, the causes are multifactorial and may involve either autonomic dysfunction or arrhythmia, depending on the nature and severity of mitral valve dysfunction.3

METABOLIC SYNCOPE Metabolic derangements that can lead to syncope include hypoglycemia, hypoxia, and hyperventilation. In general, these conditions produce more persistent symptoms that require medical intervention and are usually unaffected by position or activity. Presentations may include a depressed level or loss of consciousness or seizure activity. Diaphoresis, tremor, and altered mental status may precede an episode of hypoglycemic syncope. Hypoxia leading to syncope may initially present with agitation and increasing somnolence as the hypoxemic state continues. Hyperventilation may lead to a loss of consciousness if cerebral vasoconstriction occurs due to resultant hypocapnia. Drugs and toxin exposure can cause syncope or altered consciousness due to metabolic derangement. Certain drugs may also induce cardiac arrhythmias, which can present as a syncopal episode. Breath-holding spells are common among young children. They typically start with crying, followed by apnea (often at end-expiration), then cyanosis or pallor, and ultimately a brief syncopal event. Patients promptly return to a normal level of consciousness following the event. Hypoxia and hypocapnia are thought to be significant factors that lead to loss of consciousness in these instances. A number of conditions that can mimic true syncope are listed in Table 37-3. For example, a seizure may mimic a syncopal episode but generally lasts longer than several seconds and may include a preceding aura, abnormal movements, and a postictal period. Atypical migraines can present with syncope-like spells and may include a preceding aura or severe headache. Psychogenic episodes such as panic attacks or hyperventilation can mimic true syncope and occur most often among adolescent patients. Breath holding in a younger child may lead to syncope and self-resolves as soon as the child resumes normal breathing. A detailed history of the event usually supports

the diagnosis and may include a preceding period of stress or hyperventilation while in the presence of others. Patients who have a full recollection of the event are less likely to have suffered a full loss of consciousness. Although uncommon, malingering, Munchausen syndrome, or Munchausen syndrome by proxy may present with a history of a syncope-like episode. Features that support these diagnoses include the patient’s or parent’s strong desire for extensive or invasive evaluation or therapy. TABLE 37-3

Conditions That Mimic Syncope

Neurologic Seizure Migraine Psychogenic Panic attacks Breath-holding Conversion disorder Malingering Munchausen syndrome Munchausen syndrome by proxy

DIAGNOSTIC EVALUATION Diagnostic evaluation of the patient with syncope is aimed at detecting those few individuals with serious underlying cardiac or non-cardiac disease. The diagnosis of myocardial dysfunction can usually be achieved by clinical examination, ECG, and echocardiography. An ECG should be performed as part of all initial evaluations for syncope, with special attention paid to the QT interval corrected for heart rate (QTc) interval and T-wave morphology for evidence of Long-QT syndrome. In addition, voltage criteria for ventricular hypertrophy suggestive of an obstructive lesion, the pre-excitation of Wolff-Parkinson-White syndrome, and ectopy and conduction disturbances should be ruled out during ECG interpretation. In a patient with a normal ECG but a history suspicious for cardiac arrhythmia, a 24-hour ambulatory Holter monitor may aid in the

diagnosis if the episodes are frequent. In a patient whose episodes are isolated or less frequent, an event monitor that can be turned on to record during symptomatic events may be more appropriate. In general, a more extensive cardiovascular evaluation is indicated if syncope occurs with exertion and in cases in which there is chest pain preceding the loss of consciousness, recurrent syncope, or an abnormal cardiac examination. It is important to realize that cardiac causes of syncope may be accompanied by seizures that result from global cerebral hypoperfusion. Supraventricular arrhythmias generally cause palpitations rather than syncope. However, atrioventricular nodal bypass tracts, which occur in Wolff-Parkinson-White syndrome, may cause rapid conduction of atrial flutter or fibrillation to the ventricles, resulting in syncope or sudden death. Ventricular arrhythmias occur more frequently in postoperative patients with congenital heart disease but may also be seen in patients with structurally normal hearts. Long-QT syndrome predisposes patients to ventricular arrhythmias by its characteristic prolongation of the QTc, which places patients at risk for a polymorphic ventricular tachycardia known as torsades de pointes. If prolonged, torsades de pointes can lead to cardiac arrest or death as it degenerates into ventricular fibrillation. In some patients, an ECG demonstrating borderline QTc prolongation may be consistent with the upper range of normal but should be put into context with other features of the syncopal event and past medical history. Some ECG abnormalities may become more exaggerated with an increased heart rate as the QTc fails to shorten appropriately with the R-R interval. Brief and self-resolving periods of rhythm disturbances causing syncope may be precipitated by exercise or a startling experience. Genetic causes of Long-QT syndrome are due to either a defect in cardiac potassium or sodium channels. Acquired causes of Long-QT include electrolyte disturbances (e.g. hypokalemia, hypocalcemia, hypomagnesemia), increased intracranial pressure, and medications (e.g. tricyclic antidepressants, phenothiazines, antiarrhythmics) and should be considered based on laboratory data, neurologic examination, or by medication history. Laboratory testing for metabolic or endocrine derangements associated with syncope is easily performed and should include a blood glucose level, electrolyte panel, and complete blood count. If a toxic ingestion is suspected, a serum and urine toxicology screen should be obtained. Anemia can predispose to syncope when it is severe or more acute in onset. Because

hypoxia due to carbon monoxide poisoning can lead to syncope, co-oximetry with a carboxyhemoglobin level should be considered when indicated by circumstances. A pregnancy test is also indicated in teenage girls with syncope. An electroencephalogram or imaging of the central nervous system is often part of an evaluation when epilepsy or other neurologic causes for syncope are being considered. The use of upright tilt table testing has not been shown to be reliable in improving diagnostic accuracy during the general evaluation of syncope, or in guiding management or prognosis.6

MANAGEMENT Therapeutic intervention to prevent or treat recurrent syncope depends on the pathogenic cause and the frequency and severity of the episodes. Standard resuscitative interventions should be instituted for acute syncopal events. Patients who have known structural heart disease or other cardiac disease should be admitted to the hospital and evaluated by a cardiologist. The treatment of cardiogenic syncope is generally overseen by a cardiologist and is based on a careful and thorough diagnostic evaluation to determine the underlying abnormality. Suppression of symptomatic supraventricular arrhythmias is often managed with medication, but treatment varies based on the inciting or propagating feature of the disorder. Patients with Long-QT syndrome may benefit from β-adrenergic antagonists to prevent exerciseinduced ventricular arrhythmia, but pacemakers or implanted cardiac defibrillators are necessary in some cases. Surgical intervention may be needed for structural abnormalities, and intensive care interventions are required for those with progressive or unstable conditions. Autonomic syncope is considered a benign illness, and in most cases, prophylaxis against presyncope or “near syncope” is sufficient intervention. Educating patients and families generally results in earlier awareness of symptoms and the ability to abort episodes by assuming a recumbent posture. Additional measures for prophylaxis include the avoidance of dehydration, a salt-enriched diet, and in some cases the use of mineralocorticoids to induce salt and water retention. When simple prevention strategies fail, treatment with β-adrenergic receptor blockade is often used to decrease stimulation of

the cardiac mechanoreceptors. Disopyramide, an anticholinergic, negative inotropic, and vasoconstrictive agent, is effective in some patients. Finally, direct α-adrenergic receptor stimulation and stimulation of peripheral norepinephrine release can be tried. It is important to remember that no single pharmacologic agent has been consistently efficacious in clinical trials and often a combination of therapeutic strategies is necessary to manage symptoms effectively.4-6 Atrioventricular pacing has been used in some patients with frequent episodes of symptomatic bradycardia, but this is reserved for refractory cases when pharmacologic agents are unsuccessful. The treatment of metabolic or other systemic causes of syncope focuses on the underlying process, both in the acute resuscitation phase and in the prevention stage. Identification of a causative entity often allows patients and families to prevent future syncopal events or to manage such events safely if and when they reoccur.

SPECIAL CONSIDERATIONS The diagnosis of heritable conditions during an evaluation for syncope necessitates genetic counseling for family members. When underlying heart disease is identified as a cause for syncope, evaluation and management should address both the underlying disease condition itself and the potential for an associated life-threatening arrhythmia. Cerebrovascular events are rare in children but should be considered in patients with underlying disease affecting the peripheral vasculature who present with syncope. KEY POINTS Syncope in children is most often benign and self-limited. History and physical examination are the most important initial diagnostic tools in the evaluation of syncope. Evaluation of a patient presenting with syncope is geared toward identifying those few patients who are at high risk for serious underlying disease.

REFERENCES 1. Anderson JB, Czosek RJ, Cnota J, et al. Pediatric syncope: National Hospital Ambulatory Medical Care Survey results. J Emerg Med. 2012;43:575-583. 2. Driscoll DJ, Jacobsen SJ, Porter CJ, Wollan PC. Syncope in children and adolescents. J Am Coll Cardiol. 1997;29:1039-1045. 3. Boudoulas H, Wooley CF. The floppy mitral valve, mitral valve prolapse, and mitral valvular regurgitation. In: Allen HD, Gutgesell HP, Clark EB, Driscoll DJ, eds. Moss and Adam’s Heart Disease in Infants, Children, and Adolescents. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:964. 4. Massin MM. Neurocardiogenic syncope in children: current concepts in diagnosis and management. Pediatr Drugs. 2003;5:327-334. 5. Strickberger SA, Benson DW, Biaggioni I, et al. AHA/ACCF Scientific Statement on the evaluation of syncope: from the American Heart Association Councils on Clinical Cardiology, Cardiovascular Nursing, Cardiovascular Disease in the Young, and Stroke, and the Quality of Care and Outcomes Research Interdisciplinary Working Group; and the American College of Cardiology Foundation: in collaboration with the Heart Rhythm Society: endorsed by the American Autonomic Society. Circulation. 2006;113:316. 6. Sapin SO. Autonomic syncope in pediatrics: A practice-oriented approach to classification, pathophysiology, diagnosis, and management. Clin Pediatr. 2004;43:17-23.

CHAPTER

38

Vomiting Anne K. Beasley

BACKGROUND Vomiting is a common chief complaint for hospitalized pediatric patients. It is more often a symptom than a diagnosis and can be a presenting sign of illness in nearly every organ system. Investigation into the etiology of vomiting can help prevent metabolic-, nutritional-, and trauma-related complications as well as diagnose potentially life-threatening but treatable conditions.

PATHOPHYSIOLOGY Vomiting is a highly coordinated, centrally mediated reflex. It occurs when the contents of the stomach are forcefully expelled out of the mouth. Descent of the diaphragm and constriction of abdominal musculature occurs simultaneously with the relaxation of the gastric cardia, forcing the contents of the stomach retrograde into the esophagus. The vomiting centers of the brain, which reside in the reticular formation of the medulla, receive sensory input from a number of sources that trigger vomiting. These include afferent signals from the gastrointestinal (GI) tract arising from the vagus nerve and other sympathetic nerves, afferent signals from outside the GI tract originating from organs located in the thorax and abdomen, as well as sensory input received from the vestibular nucleus. Additionally, the chemoreceptor zone in the brainstem detects chemical abnormalities in the body, such as uremia or ketoacidosis. In the setting of cerebral trauma, extramedullary centers in the brain receive afferent signals as a result of signals from other areas of the brain. By understanding the pathophysiology of vomiting, one can appreciate that vomiting can be a

manifestation of disease not only in the GI tract but in multiple organ systems.

PATIENT HISTORY A thorough history should be conducted in all children presenting with vomiting. The examiner can be guided by a few key elements of the history. What is the age of the patient? What is the character of the emesis? Bilious or nonbilious? Bloody or nonbloody? What is the nature of the emesis? Is it projectile? What was the onset? What is the timing? Is it associated with eating? Does it only occur in the morning? Are the vomiting episodes cyclical in nature? How have the symptoms progressed? Are there associated complaints within the GI tract? Are there associated systemic signs and symptoms such as fever or weight loss? Additionally, completing a comprehensive review of systems is a helpful adjunct in guiding the examiner toward an appropriate workup and ultimately in yielding a diagnosis.

PHYSICAL EXAMINATION The physical examination in the evaluation of the vomiting child begins with an overall assessment. Is the patient ill or toxic appearing? Next, a careful review of the vital signs with attention to age-appropriate normal ranges can help to guide the assessment of hydration status, acuity of the illness, and overall cardiovascular stability. A complete head-to-toe physical examination must be performed including a careful examination of the abdomen to determine presence of abdominal pain and distension. Assessing the overall patient and doing a thorough examination will help the examiner to narrow a very broad differential diagnosis for what is a common and often nonspecific presenting sign and symptom.

DIFFERENTIAL DIAGNOSIS1-3 It is helpful to think through the differential diagnosis of vomiting in terms of gastrointestinal versus non-gastrointestinal pathologies as well as by age of the patient. (Tables 38-1 to 38-4).

TABLE 38-1

Causes of Vomiting by Organ System

Gastrointestinal Esophageal Stricture Web Ring Atresia Tracheoesophageal fistula Achalasia Foreign body Stomach Pyloric stenosis Web Duplication Ulcer Gastroesophageal reflux disease Small Intestine Duodenal atresia Malrotation with midgut volvulus Duplication Intussusception Foreign body Bezoar Pseudo-obstruction Necrotizing enterocolitis Colon Hirschsprung disease Meconium plug or ileus Microcolon Imperforate anus

Bezoar Foreign body Helicobacter Pylori Celiac disease Milk/soy protein allergy Food allergies Inflammatory bowel disease Pancreatitis Hepatitis Cholecystitis, cholelithiasis, choledocholithiasis Abdominal trauma Neurologic Intracranial mass lesion Hydrocephalus Intracranial bleed Cerebral edema Pseudotumor cerebri Migraine headache Cyclic vomiting syndrome Abdominal migraine Seizure Meningitis Kernicterus Vestibular system disruption Genitourinary Obstructive uropathy Pyelonephritis Renal insufficiency Renal tubular acidosis Glomerulonephritis Metabolic

Hypercalcemia Hypokalemia Hyperammonemia Urea cycle defects Amino acidopathy Organic acidopathy Glycogen storage disease Fatty acid oxidation defects Galactosemia Lysosomal storage diseases Peroxisomal disorders Endocrine Diabetic ketoacidosis Congenital adrenal hyperplasia Adrenal insufficiency Infectious Viral gastroenteritis Bacterial gastroenteritis Post-viral gastroparesis Appendicitis Pneumonia Bronchiolitis Pertussis Sinusitis Pharyngitis Meningitis Encephalitis Sepsis Cardiac Arrhythmias

Heart failure Miscellaneous Psychiatric Anorexia nervosa Bulimia Hyperventilation Anxiety Ingestions Drug toxicities Child abuse TABLE 38-2

Common Causes of Vomiting in Newborns and Infants

Benign Overfeeding Aerophagia Innocent spitting Excessive handling Parental anxiety Neurologic Hydrocephalus Subdural bleeding Cerebral edema Kernicterus Genitourinary Obstructive uropathy Pyelonephritis Pulmonary Bronchiolitis Pertussis

Reactive airway disease Pneumonia Metabolic or endocrine Urea acid cycle defects Aminoacidopathies Organic acidopathies Hypercalcemia Glycogen storage disease Fatty acid oxidation defects—MCAD deficiency Galactosemia Congenital adrenal hyperplasia Gastrointestinal Gastroesophageal reflux Milk protein intolerance Allergy Eosinophilic enteritis Lactobezoar Gastritis Intestinal atresia, stenosis, or webs Intestinal duplication Hiatal or diaphragmatic hernia Pyloric stenosis Malrotation with midgut volvulus Meconium plug or ileus Annular pancreas Hirschsprung disease Hepatitis Pancreatitis, necrotizing enterocolitis, imperforate anus, microcolon Other infections Meningitis

Gastroenteritis Other Medications Child Abuse MCAD, Medium-chain acyl-CoA dehydrogenase.

TABLE 38-3

Common Causes of Vomiting in Infants and Toddlers

Neurologic Hydrocephalus Subdural bleeding Cerebral edema Kernicterus Benign paroxysmal vertigo Migraine Cyclic vomiting Intracranial mass lesion Genitourinary Obstructive uropathy Pyelonephritis Renal insufficiency Glomerulonephritis Renal tubular acidosis Ovarian or testicular torsion Metabolic or endocrine Urea acid cycle defects Aminoacidopathies Hypercalcemia Glycogen storage disease Congenital adrenal hyperplasia

Fatty acid oxidation defects Gastrointestinal Gastroesophageal reflux Milk/soy protein intolerance Lactose intolerance Food allergy Eosinophilic enteritis Celiac disease Bezoar Foreign body Gastritis Intestinal stenosis or adhesions Intestinal duplication Hiatal hernia Malrotation with midgut volvulus Annular pancreas Hepatitis Pancreatitis Intussusception Incarcerated hernia Meckel diverticulum Infections Meningitis Gastroenteritis Appendicitis Otitis Sinusitis Postviral gastroparesis Bronchiolitis Pertussis Pneumonia

Other Medications Toxic ingestions Child abuse Psychogenic TABLE 38-4

Common Causes of Vomiting in Children and Adolescents

Neurologic Intracranial bleeding Cerebral edema Benign paroxysmal vertigo Migraine Cyclic vomiting Intracranial mass lesion Concussion Genitourinary Obstructive uropathy Pyelonephritis Renal insufficiency Ovarian or testicular torsion Pregnancy Metabolic or endocrine Inborn errors of metabolism Diabetic ketoacidosis Adrenal insufficiency Gastrointestinal Gastroesophageal reflux Lactose intolerance Allergy

Eosinophilic enteritis Celiac disease Inflammatory bowel disease Bezoar Foreign body Gastritis Gastric or duodenal ulcer Intestinal stenosis or adhesions Hiatal hernia Malrotation Volvulus Annular pancreas Hepatitis Pancreatitis Cholecystitis, cholelithiasis Intussusception Incarcerated hernia Meckel diverticulum Other infections Meningitis Encephalitis Gastroenteritis Appendicitis Otitis Sinusitis Helicobacter pylori Postviral gastroparesis Pertussis Pneumonia Rheumatologic or immunologic Scleroderma

Chronic granulomatous disease Other Medications Toxic ingestions Child abuse Psychogenic Anorexia nervosa Bulimia

GASTROINTESTINAL CAUSES The causes of vomiting within the GI tract are numerous, and can be divided by age and by obstructive versus non-obstructive causes. Obstructive Vomiting in the first few days of life can be a sign of serious pathology. Nonbilious emesis is a sign that the obstruction is proximal to the ampulla of Vater. Esophageal atresia presents with nonbilious emesis in an infant where a nasogastric tube cannot be passed into the stomach and where an abdominal film shows a paucity of bowel gas. The caveat to these x-ray findings would be if there is the presence of a tracheoesophageal fistula in addition to the esophageal atresia. In this setting, air could be delivered into the stomach due to a connection distal to the atretic esophagus. Duodenal atresia can present with nonbilious emesis depending on the level of atresia. It usually presents within hours of birth and rarely is associated with abdominal distension due to the proximal nature of the obstruction. The abdominal film would show the classic “double bubble” appearance due to air within the stomach as well in the first portion of the duodenum proximal to the atresia. Bilious emesis is indicative of more distal congenital obstructive GI malformations such as duodenal or jejunal atresias, intestinal duplication, malrotation with midgut volvulus, meconium plug or ileus, colonic atresia or stenosis, imperforate anus, microcolon, or Hirschsprung disease. The more distal jejunal obstructions usually present within the first 24 hours of life and are associated with abdominal distension. Malrotation with midgut volvulus usually presents within the first week after birth with bilious emesis. A

meconium plug or ileus can cause bilious emesis that either resolves as the meconium passes or may require surgical intervention. This diagnosis should always raise clinical suspicion for cystic fibrosis. Hirschsprung disease should be considered in cases of colonic obstruction where there is a history of failure to pass meconium. Abdominal radiographs showing marked dilation throughout the bowel would accompany bilious emesis. Beyond the immediate newborn period, nonbilious emesis can be a sign of pyloric stenosis. The classic history includes that of projectile vomiting in a hungry infant that continues to desire to feed despite persistent emesis. A palpable “olive” on examination in the epigastric region due to pyloric hypertrophy is often a late finding. Lactobezoars, a conglomeration of milk and mucous, can be a rare cause of gastric outlet obstruction and nonbilious emesis in milk-fed infants. Bilious emesis should always raise the suspicion for intestinal obstruction and prompt urgent evaluation into the etiology. Bowel strangulation and ischemia can occur outside the newborn period in the setting of malrotation due to increased risk of volvulus secondary to lack of mesenteric anchoring to the retroperitoneum. In addition to bilious emesis, infants would present with abdominal distension, electrolyte abnormalities, irritability, lethargy, and even shock due to bowel ischemia and acidosis. Obstructive pathologies beyond infancy can include intussusception, achalasia, cricopharyngeal incoordination, hiatal and diaphragmatic hernias, duodenal webs, duodenal hematomas, intestinal strictures, and vascular rings and slings. Stridor can be present in the setting of a vascular ring, as the aberrant vessel compresses the trachea in addition to the esophagus. Additionally, superior mesenteric artery syndrome should be considered in a child who has had rapid weight loss resulting in the loss of the fat pad that normally is situated between the superior mesenteric artery and the duodenum, causing compression of the duodenum and eventual obstruction and emesis. Intussusception should be suspected in patients presenting with paroxysms of severe pain, vomiting, and lethargy. Currant jelly stools are a late finding indicative of bowel ischemia and necrosis. A torsed Meckel diverticulum, incarcerated hernias, and adhesions in the setting of prior abdominal surgeries should be on the differential of vomiting due to obstruction beyond infancy. A history of abdominal trauma should raise suspicion for a duodenal hematoma or other bowel trauma that could lead to an obstructive process. Lastly, it is important to remember that the ingestion of foreign bodies can obstruct the pylorus or esophagus in toddlers. This can

include coins or other nonfood items, as well as bezoars that build up over time due to chronic ingestion of hair, carpet, or other unusual items. Non-Obstructive Non-obstructive causes of vomiting within the GI tract in the immediate newborn and infant period include those ranging from necrotizing enterocolitis and inborn errors of metabolism to milk/soy protein allergy, gastroesophageal reflux, overfeeding, and improper mixing of formula. Necrotizing enterocolitis can present with bilious or nonbilious emesis and should be suspected in a toxic-appearing infant with abdominal distension and x-ray evidence of pneumatosis intestinalis and, after perforation, hepatic portal venous gas. Inborn errors of metabolism are a rare cause of vomiting but should be suspected in the vomiting, acidotic, or hypoglycemic infant. In infants and beyond into the early childhood period, dietary protein intolerance can be an etiology of emesis, diarrhea, bloody stools, irritability, and failure to thrive. The most commonly implicated proteins include milk, soy, and egg. Gastroesophageal reflux can affect children of any age. A history of emesis, back arching, crying with feeds, or symptoms that intensify with supine positioning should raise the suspicion for gastroesophageal reflux disease. If undiagnosed or untreated, reflux can lead to apnea, aspiration, and Sandifer syndrome. Food allergies should be considered as a source of emesis, especially if accompanied by urticaria, respiratory distress, edema, abdominal pain, and anaphylaxis. Gastritis and gastric or duodenal ulcers can occur secondary to stress, allergies, medications, or Helicobacter pylori infections. Eosinophilic enteritis can present with emesis, abdominal pain, reflux, and food refusal. It can affect any portion of the GI tract and is often associated with food or other allergies. Celiac disease should be considered in an infant or child with failure to thrive, vomiting, and diarrhea. Gluten sensitivity leads to villous atrophy and malabsorption that resolves with a gluten elimination diet. Inflammatory bowel disease such as Crohn disease or ulcerative colitis can also cause emesis along with a constellation of other symptoms including hematochezia, weight loss, and abdominal pain. Additional non-obstructive causes of vomiting in the older child include cholecystitis, cholelithiasis, and choledocholithiasis. Biliary pathologies tend to present with right upper quadrant abdominal pain that is colicky in nature and associated with meals. This should be suspected in children with rapid red blood cell turnover, those with prolonged parenteral nutrition, or it can be

medication induced. Pancreatitis can occur in the pediatric population secondary to trauma, viral infection, or may be medication induced. It also may be due to structural causes, or can be genetic in etiology. In many cases, however, it may be idiopathic in nature. The hallmark of pancreatitis is nausea, emesis, and severe epigastric abdominal pain. Hepatitis secondary to viruses, toxins, drugs, and metabolic or autoimmune causes can also present with emesis.

NON-GASTROINTESTINAL CAUSES Neurologic Vomiting may be the presenting sign of serious neurologic sequelae, including increased intracranial pressure (ICP). Increased ICP may also be associated with headache, vision changes, and, in signs of impending herniation, cranial nerve palsies and vital sign changes including hypertension, bradycardia, and abnormal respirations. Increased ICP may be due to a mass lesion, hydrocephalus, vascular malformations, or hematomas. An expanding mass lesion may lead to intractable vomiting, headaches that awaken a child from sleep, or one that worsens with Valsalva maneuvers. Idiopathic intracranial hypertension can stimulate nausea and vomiting as well as a positional headache and visual field deficits. Vomiting can also occur in association with seizure activity or may be indicative of an intracranial infection such as meningitis or encephalitis. Migraine headaches are associated with nausea and emesis. Cyclic vomiting syndrome and abdominal migraines are thought to share pathophysiologic mechanisms. The former is characterized by recurrent episodes of nausea and vomiting where no organic cause is identified. There must be at least three episodes of recurrent vomiting, intervals of normal health, episodes stereotypic for rapid onset and duration of hours to days, and lack of evidence to support an alternative diagnosis. Abdominal migraines involve episodic abdominal pain and emesis associated with nausea, anorexia, photophobia, headache, and pallor, and usually occur in the setting of a family history of migraines. A history of intervals of normal health between episodes also exists. Disruption of the vestibular system as in otitis media or in cases of benign paroxysmal positional vertigo can also cause vomiting.

Genitourinary Pyelonephritis, nephrolithiasis, ureteropelvic junction obstruction, hydronephrosis, glomerulonephritis, renal tubular acidosis, and renal insufficiency can all present with emesis. Torsion of the ovary or testes can present with severe abdominal pain and emesis. Adolescent females presenting with vomiting should undergo routine pregnancy testing prior to any radiographic imaging to diagnose an etiology of emesis. Metabolic An underlying metabolic or mitochondrial process should be considered in an infant or young child with persistent emesis and electrolyte abnormalities. Hypercalcemia, hypokalemia, hypoglycemia, and hyperammonemia can all cause vomiting. Urea acid cycle defects such as ornithine transcarbamylase deficiency generally present in newborns with emesis, lethargy, coma, and death if not recognized and treated aggressively. Even those with partial defects may have more exaggerated vomiting illnesses in childhood. Aminoacidopathies such as tyrosinemia can present with vomiting in children aged 2 weeks to 1 year. Organic acidopathies such as maple syrup urine disease, isovaleric acidemia, mevalonic acidemia, propionic acidemia, and methylmalonic acidemia present with vomiting in infancy. Lactic acidosis, fatty acid oxidation defects, particularly mediumchain acyl-CoA dehydrogenase deficiency, glycogen storage disease, and galactosemia have vomiting as a primary presenting feature as well. Porphyria can present with periods of vomiting, change in mental status, or rash, depending on the subtype. Mitochondrial diseases are often associated with intestinal dysmotility or pseudo-obstruction. These children often have a long history of gastroesophageal reflux, feeding intolerance, abdominal pain, constipation or diarrhea, and failure to thrive. Leigh disease, also known as subacute necrotizing encephalomyelopathy, usually presents in infancy with feeding or swallowing difficulties, failure to thrive, and vomiting. Endocrine Diabetic ketoacidosis can lead to intractable vomiting in the setting of worsening ketosis and acidosis. Salt-wasting congenital adrenal hyperplasia should be suspected in a vomiting female infant with any signs of virilization. Male infants usually have normal genitalia, leading often to a delayed diagnosis. Adrenal insufficiency can present with emesis during a crisis. Hypothyroidism and associated constipation may also lead to vomiting. Infectious Infectious etiologies of vomiting are numerous. The majority of cases are caused by viral infections that are self-limited in nature. Bacterial

causes of emesis should be considered in a history consistent with certain food exposures. Examples include Staphylococcus aureus, Bacillus cereus, Shigella, Salmonella, Escherichia coli, Yersinia enterocolitica, and Campylobacter jejuni. Parasitic infections such as ascariasis or giardiasis can lead to emesis, diarrhea, and obstructive pathology causing emesis. Appendicitis should be considered in the child presenting with fever, anorexia, nausea, emesis, and periumbilical pain that migrates to the right lower quadrant. Viral or bacterial meningitis can present with vomiting in addition to headache and vision changes, as noted above. A child presenting with emesis in the setting of a preceding viral illness may have post-viral gastroparesis leading to delayed gastric emptying and emesis. This process can take days to months to resolve. Sinusitis or pharyngitis can also present with nausea and emesis, as can any infection causing excessive coughing leading to post-tussive emesis such as pertussis or bronchiolitis. It is important not to forget pneumonia as a cause of emesis and not to overlook a lower-lobe pneumonia as an etiology of abdominal pain and emesis in the setting of fever, tachypnea, and cough. Generalized sepsis syndromes can also cause vomiting. Cardiac Cardiovascular etiologies of vomiting generally involve arrhythmias such as supraventricular tachycardia in the irritable infant, or can occur in the setting of heart failure where an infant may have emesis and feeding intolerance in addition to poor weight gain. Myocarditis may also present with vomiting.

MISCELLANEOUS Psychiatric etiologies of emesis should not be overlooked when the history supports a diagnosis such as anorexia nervosa, bulimia, hyperventilation, or severe anxiety. Additional causes of vomiting include intentional or unintentional ingestions, drug toxicities, and child abuse.

MANAGEMENT The management for vomiting4-6 is dependent on the etiology and pathophysiology of the organ system involved (Figures 38-1 and 38-2). A thorough history and physical examination will help to guide a focused

diagnostic evaluation of the vomiting infant or child. If clinical concern for obstruction exists, a nasogastric tube should be placed immediately, followed by abdominal radiographs and appropriate contrast imaging to help pinpoint a diagnosis. Urgent surgical consultation should be obtained early in the diagnostic workup if a bowel obstruction is suspected. An upper GI series is diagnostic in the setting of malrotation with midgut volvulus, although abdominal ultrasound maybe used to reveal an abnormal relationship of the superior mesenteric vessels. Urgent surgical intervention is key to preventing continued bowel ischemia necessitating intestinal resection and resultant short gut syndrome.

FIGURE 38-1. Diagnosis of vomiting.

FIGURE 38-2. Treatment of vomiting. In general, after life-threatening etiologies are ruled out and initial diagnostic evaluation yields a diagnosis, management of the vomiting child should focus on rehydration and correction of any electrolyte abnormalities. The World Health Organization and Centers for Disease Control advocate oral rehydration therapy in children with mild to moderate dehydration due to gastroenteritis with the ability to tolerate oral fluids. In the critically ill child, fluid resuscitation with isotonic fluids should be the mainstay of initial rehydration. The use of antiemetic therapies such as ondansetron (being cognizant of the potential risk for QTc prolongation) have been shown to reduce vomiting, improve tolerance of oral fluids, and reduce hospital admissions and length of stay in children with gastroenteritis. Additional medication regimens may be considered in the setting of refractory vomiting or specific diagnoses (i.e. cyclic vomiting syndrome). Several studies exist showing acupuncture to be as effective as antiemetic medications in the treatment of vomiting related to medication side effects such as anesthesia and chemotherapy. KEY POINTS

Vomiting is a common presenting sign and symptom in the pediatric population and can be an indicator of pathology in nearly every organ system. A thorough history and physical examination should be conducted in every child presenting with vomiting to help narrow what is a very broad differential diagnosis and help guide diagnostic evaluation. A history of bilious emesis can be a sign of a surgical emergency and should prompt urgent evaluation and treatment. Management of vomiting, after initial stabilization of the critically ill child, should focus on rehydration and correction of electrolyte abnormalities. Peer-reviewed literature supports the use of antiemetic medications in the acute setting once the etiology of emesis is determined to be due to gastroenteritis.

REFERENCES 1. Kleigman RM, Behrman RE, Jenson HB, Stanton BF. Major symptoms and signs of digestive tract disorders. In: Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders; 2007:1523-1524. 2. Zitelli BJ, Davis HW. Surgery: gastrointestinal obstruction. In: Atlas of Pediatric Physical Diagnosis. 5th ed. Philadelphia, PA: Mosby; 2007:632-641. 3. Chandran L, Chitkara M. Vomiting in children: reassurance, red flag, or referral? Pediatr Rev. 2008;29(6):183-192. 4. King CK, Glass R, Bresee JS, Duggan C. Managing acute gastroenteritis among children: oral hydration maintenance and nutritional therapy. MMWR Morbid Mortal Weekly Report. 2003;52(RR16):1-16. 5. Fedorowicz Z, Jagannath VA, Carter B. Antiemetics for reducing vomiting related to acute gastroenteritis in children and adolescents. Cochrane Database. 2011;9:CD005506. 6. Anders EF, Findeisen A, Lode HN, Usichenko TI: Acupuncture for treatment of acute vomiting in children with gastroenteritis and

pneumonia. Klin Padiatr. 2012;224(2):72-75.

PART

Systems Approach

III

Section A Abuse and Neglect 39 Cutaneous Injuries of Concern for Nonaccidental Trauma 40 Abusive Head Trauma 41 Imaging of Child Abuse 42 Medical Child Abuse: Münchausen Syndrome by Proxy and Other Manifestations 43 Legal Issues Section B Adolescent Medicine 44 Eating Disorders 45 Sexually Transmitted Infections in Adolescents and Young Adults 46 Abnormal Uterine Bleeding Section C Allergy and Immunology 47 Anaphylaxis 48 Drug Allergy 49 Primary Immunodeficiency Diseases 50 Intravenous Immunoglobulin Section D Cardiology 51 The Cardiac Examination 52 Electrocardiogram Interpretation

53 Congenital Heart Disease 54 Infective Endocarditis 55 Myocarditis and Cardiomyopathy 56 Pericarditis 57 Acute Rheumatic Fever Section E Dermatology 58 Purpura 59 Vesicles and Bullae 60 Vascular Anomalies 61 Atopic Dermatitis 62 Ecthyma Gangrenosum 63 Drug-Associated Rashes 64 Erythema Multiforme 65 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis 66 Skin Disease in Immunosuppressed Hosts 67 Epidermolysis Bullosa Section F Endocrinology 68 Diabetes Mellitus and Hyperglycemia 69 Disorders of Thyroid Hormone 70 Disorders of Pituitary Function 71 Disorders of Calcium Metabolism 72 Disorders of the Adrenal Gland Section G Fluids and Electrolytes 73 Dehydration 74 Fluid and Electrolyte Therapy Section H Gastroenterology and Nutrition

75 Biliary Disease 76 Constipation 77 Dyspepsia 78 Disorders of Gastric Emptying 79 Liver Failure 80 Inflammatory Bowel Disease 81 Malnutrition 82 Pancreatitis 83 Feeding Issues Section I Genetics and Metabolism 84 Genetic Syndromes Caused by Chromosomal Abnormalities 85 Hyperammonemia 86 Hypoglycemia 87 Metabolic Acidosis Section J Hematology 88 Anemia 89 Management of Sickle Cell Disease 90 Neutropenia and Bone Marrow Failure 91 Thrombocytopenia 92 Disorders of Coagulation and Thrombosis 93 Transfusion Medicine Section K Infectious Diseases 94 Empirical Treatment of Bacterial Infections 95 Fever 96 Prolonged Fever and Fever of Unknown Origin 97 Fever and Rash 98 Central Nervous System Infections

99 Complications of Acute Otitis Media and Sinusitis 100 Neck and Oral Cavity Infections 101 Middle Respiratory Tract Infections and Bronchiolitis 102 Lower Respiratory Tract Infections 103 Gastrointestinal Infections 104 Urinary Tract Infections in Childhood 105 Bone and Joint Infections 106 Skin and Soft Tissue Infections 107 Device-Related Infections 108 Human Immunodeficiency Virus 109 Infections in Special Hosts Section L Nephrology 110 Acute Renal Failure 111 Chronic Renal Failure 112 Glomerulonephritis 113 Hemolytic Uremic Syndrome 114 Interstitial Nephritis 115 Nephrotic Syndrome 116 Renal Tubular Acidosis 117 Renal Venous Thrombosis Section M Neurology 118 Seizures 119 Headache 120 Hypotonia and Weakness 121 Stroke, Arteriopathy, and Vascular Malformations 122 Demyelinating Disease Section N Newborn Medicine

123 Delivery Room Medicine 124 The Well Newborn 125 Birth Injury 126 Congenital Anomalies 127 Transient Tachypnea of the Newborn and Persistent Pulmonary Hypertension 128 Congenital and Perinatal Infections 129 Hypoglycemia and Infants of Diabetic Mothers 130 Neonatal Hyperbilirubinemia 131 Neonatal Abstinence Syndrome Section O Oncology 132 Childhood Cancer 133 Oncologic Emergencies 134 Hematopoietic Stem Cell Transplant Section P Psychiatry 135 Depression and Physical Illness 136 Assessment and Management of the Suicidal Patient 137 Conversion and Pain Disorders 138 Agitation 139 New-Onset Psychosis Section Q Pulmonology 140 Apparent Life-Threatening Event, Infant Apnea, and Pediatric Obstructive Sleep Apnea Syndrome 141 Asthma 142 Aspiration 143 Bronchopulmonary Dysplasia and Chronic Lung Disease of Infancy 144 Cystic Fibrosis

145 Choking and Foreign Body Aspiration 146 Pulmonary Function Testing Section R Rheumatology 147 Kawasaki Disease 148 Henoch-Schönlein Purpura 149 Juvenile Dermatomyositis 150 Juvenile Idiopathic Arthritis 151 Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome 152 Infection-Associated Arthritis 153 Systemic Lupus Erythematosus Section S Surgical Issues 154 Gastrointestinal Obstruction: Pyloric Stenosis, Malrotation and Volvulus, and Intussusception 155 Appendicitis 156 Hernias 157 General Trauma 158 Ear, Nose, and Throat 159 Neurosurgical Issues 160 Ophthalmology 161 Orthopedics 162 Burns and Other Skin Injuries 163 Pneumothorax and Pneumomediastinum 164 Urology Section T Toxins, Substance Abuse, and Environmental Exposures 165 Stabilization and Hospitalization of the Poisoned Child 166 Toxicity of Over-the-Counter Medications and Oral Hypoglycemic Agents

167 Hazardous Household Chemicals: Hydrocarbons, Alcohols, and Caustics 168 Lead, Other Metals, and Chelation Therapy 169 Drugs of Abuse 170 Withdrawal Syndromes 171 Fire-Related Inhalational Injury 172 Heat Disorders 173 Hypothermia and Cold-Related Injuries 174 Drowning 175 Human and Animal Bites 176 Envenomation 177 Infant Botulism 178 Anticoagulants and Antithrombotics Section U Care of the Child with Medical Complexity 179 Introduction to the Child with Medical Complexity 180 Acute Care of the Child with Medical Complexity 181 Managing Comorbidities in Children with Severe Neurologic Impairment 182 Technologic Devices in the Child with Medical Complexity 183 Do-Not-Attempt-Resuscitation Orders

SECTION A Abuse and Neglect

CHAPTER

39

Cutaneous Injuries of Concern for Nonaccidental Trauma Celeste R. Wilson

BACKGROUND The topic of child abuse and neglect is often a source of anxiety and discomfort for even the most seasoned of pediatric medical providers. While disheartening to even consider the possibility of a child being intentionally injured by a trusted caretaker, pediatric providers are uniquely positioned to identify situations concerning for child maltreatment and intervene accordingly. Each year, nearly one million children in the United States are abused or neglected.1 The majority of these children are victims of neglect, with the remaining being physically or sexually abused. For the purpose of this section, we will concentrate on the various aspects of child physical abuse (Chapters 38-41), as well as the legal issues which are inherent in all cases of child maltreatment (Chapter 42). Cutaneous injuries are the most noticeable telltale sign suggesting that a child has been physically abused.2 They should be documented and carefully considered in the context of the child’s overall history and presentation. Although alone not specific for nonaccidental trauma, the presence and particular characteristics of skin findings such as bruises, lacerations, abrasions, burns/thermal injuries, and bite marks can raise suspicion for an abusive etiology.

CLINICAL PRESENTATION The clinical presentation of the child with cutaneous lesions can vary widely.

Regardless of whether the child is presenting with a stated concern for a skin injury or one is detected as an incidental finding, the medical provider should be prepared to seek additional information if the child’s injury is not consistent with the history provided.

BRUISES Bruises are a common cutaneous finding in the ambulatory child seeking to explore his/her environment. However, when present in the nonambulatory child, bruises should raise concern for possible physical abuse or an underlying medical condition. In a population of 973 children less than 36 months of age attending well-child care visits, Sugar and colleagues found that “those who don’t cruise rarely bruise.”3 That is, bruising was more common among those children who were cruising (17.8%) and walking (51.9%). Bruises were rare (2.2%) in those who were not yet walking with support (cruising). Moreover, the location of the bruises in the ambulatory children was noteworthy. Bruises typically occurred over anterior surfaces or bony prominences. The most common sites of bruising were the anterior tibia or knee, forehead, scalp, and upper leg. It was far less common for children to have bruising over posterior surfaces, chest, face (except for forehead), buttocks, or hands.3 Hence bruising in these areas as well as protected areas such as the abdomen, genitalia, and ears in infants and toddlers is extremely worrisome for the possibility of inflicted trauma.3,4 In addition to correlating the bruise with the developmental stage of the child, providers should pay careful attention to patterned features of bruises that may belie the device or implement used to cause the injury. Children struck with linear objects (e.g. rods, rulers, belts) may present with linear configured scars. Likewise, flexible implements which are doubled over (e.g. ropes, cords, chains) can leave a loop-configured bruise, abrasion, or scar at the site of contact. Slap marks may appear as a negative image such that an outline of the handprint is created on the skin as a result of blood extravasating from vessels into the surrounding interstitial space. Binding devices (e.g. wires, ropes, cords) may manifest as circumferentially configured bruises, lacerations, or abrasions involving the neck, wrists, ankles, or oral commissure. The combined presence of patterned cutaneous findings appearing over unusual locations (e.g. posterior surfaces, soft

tissues, genitalia) should raise grave concern for child abuse.5

BURNS No matter the etiology, burns of any type are particularly concerning for abuse and/or neglect. Of children who are physically abused, it has been estimated that burns account for nearly 25% of cutaneous injuries.6 The mere presence of a burn does not distinguish between an accidental or intentional event; rather, it is necessary to examine the features of the injury and correlate the injury with the explanation provided. In the pediatric population, the scald burn resulting from contact with a hot liquid is the most frequently encountered burn injury.7 In young children, the scald injury can inadvertently occur when the child overturns a hot beverage onto himself or bumps into another person carrying a hot beverage. In those situations, the most significant area of skin involvement is located at the site of initial contact with less skin involvement distally, as a result of the liquid cooling while traveling away from the central point of contact. Of course, it is entirely plausible that the caretaker intentionally threw the hot substance onto the child, as this would result in a similar pattern of injury. The immersion burn is most closely associated with inflicted trauma. This type of burn results from a child being placed, thrown, or held in a hot substance. Characteristically, the burn will have a sharp demarcation between the burned and unburned skin (i.e. water level) and uniformity of burn severity. If the extremities were immersed into the hot liquid, the child can present with a patterned and localized stocking (i.e. feet/leg) or glove (i.e. hand/arm) distribution of burned skin. Immersion of the child in a flexed position can result in skip areas, whereby the creased skin is protected relative to the surrounding exposed areas. If the buttocks comes into contact with the cooler surface (e.g. bathtub bottom), there may be a region of spared skin over the buttocks as a result. Kicking of the extremities and other physical efforts to resist the immersion event may result in splashing of the hot substance and subsequent “splash marks” (i.e. round areas of burned skin). Similar to bruises, burns in a patterned configuration are strong indicators of abuse. Cigarettes, automobile cigarette lighters, steam irons, radiator grids, and blow dryer gratings are only a few commonly recognized burn patterns.

Children burned by cigarettes typically present with a 6- to 8-mm round burn, with the center of the burn being most heavily affected due to the hot embers. Burns from automobile cigarette lighters produce a characteristic round burn containing concentric circles. Visualization of a burn pattern depicting round holes and a triangular tip is strongly suggestive of a steam iron burn. Similarly, the parallel and perpendicular pattern created by contact with a hot radiator grid or inner blow dryer grating is easily identifiable. Other types of burns less frequently encountered include flame, electrical, and chemical burns. Obtaining an appropriate history can be most helpful in discerning their etiology.

BITE MARKS The identification of bite marks on a child warrants special consideration. These lesions typically appear as an interrupted ovoid-shaped lesion. There may be surrounding edema or ecchymosis. Adult bite marks can be distinguished from those made by a child by measuring the maxillary intercanine distance. In adults, the standard maxillary intercanine distance ranges from 3 to 4 cm, whereas in children it is 98th percentile

S wave in V1 >98th percentile

Upright T wave in V1† Supplemental

Supplemental

Q wave in V1

Deep Q wave in V6

Right axis deviation

Left axis deviation

Source: From Gunn V, Nechyba C, eds. Harriet Lane Handbook. 16th ed. St Louis: Mosby; 2002. *At least one criterion must be present to entertain the diagnosis. †In

patients between 3 days and 10 years old.

Abnormal depolarization within the ventricles results in widening of the QRS complex. In complete right or left bundle branch block the QRS duration exceeds 0.12 seconds. As seen in Figure 52-6A, a right bundle branch block has a small R wave in V1 followed by a tall, slurred R’ reflecting late right ventricular depolarization. Figure 52-6B shows a left bundle branch block in which V6 has a tall, notched R wave and no septal q wave. Incomplete right bundle branch block, also called right ventricular conduction delay, has a similar pattern to right bundle branch block, though the QRS duration measures within normal limits. This may be a normal variant in children. Of note, neither the presence of ventricular hypertrophy nor the QT interval can be reliably assessed in the context of a bundle branch block. Bundle Branch Block

FIGURE 52-6. (A) Complete right bundle branch block. (continued)

FIGURE 52-6. (Continued) (B) Complete left bundle branch block.

Q WAVE With normal ventricular activation, the septum is the first to depolarize. This gives a q wave in the left precordial and inferior leads. Enlargement of the left ventricle, either through hypertrophy or dilation, can alter this q wave. Q Wave Abnormalities Small or absent q waves may be due to concentric left ventricular hypertrophy. Deep q waves (>5 mm) can result from septal hypertrophy or left ventricular dilation. Wide q waves (>40 ms) may represent an infarction.

ST SEGMENT This is isoelectric on a normal ECG. A key component of the ST segment is the J-point, or the point at which the S wave stops and meets the isoelectric segment. The J-point should not deviate more than 1 mm from the baseline. ST Segment Abnormalities Changes in the ST segment may reflect electrolyte imbalance, myocardial injury or ischemia, pericarditis, central nervous system disease, drug effect (eg, digoxin), ventricular hypertrophy, metabolic perturbations, hypothermia, or ventricular hypertrophy. Clinical

correlation is required to interpret ST segment irregularities (Figure 52-7).

FIGURE 52-7. ECG demonstrating diminished voltages, ST depression with downward T waves in V2 and V3, and flattened T waves in many leads in this patient with myopericarditis. (Used with permission from Dr. Michael Saalouke.) Frequently seen in adolescents, early repolarization results in a J-point elevation of 2 to 4 mm in the lateral and inferior leads. This can be a normal finding, especially in males and athletes. Early Repolarization

Diffuse ST elevation can result from pericardial inflammation. If the inflammation continues, this can be followed by normalization of the ST segment, followed by T-wave flattening and then inversion. Pericarditis

T WAVE The T wave direction changes by age in the precordial plane. In the right precordium (V1–V2), the T wave is positive from birth to approximately 7 days of age. After this, the T wave inverts until adolescence, after which it becomes positive again. In general, by 6 months of age, the QRS and T wave axis in the frontal plane are similar, differing by at most 60 degrees. Tall, peaked T waves may signify hyperkalemia, or accompany tall R waves in ventricular hypertrophy (Figure 52-8).

FIGURE 52-8. ECG demonstrating peaked T waves in a patient with hyperkalemia. There is also gradual prolongation of the PR interval, with a nonconducted P wave after the sixth QRS complex, consistent with Wenckebach second–degree heart block. (Used with permission from Dr. Michael Saalouke.)

QT INTERVAL Accurate measurement of the QT interval is important in determining if a patient has important depolarization abnormalities. The QT is longest in leads II and V5, and should always be measured in these leads. A tangent should be drawn from the steepest downslope of the T wave to the baseline. The QT interval is measured from the onset of the q wave to where the tangent and baseline intersect.3 This should then be corrected for heart rate by dividing it by the square root of the preceding RR interval (Bazett’s formula). QT Abnormalities Normal values for the corrected QT interval (QTc) vary by age and gender. In children less than 12 years of age, the QTc should be ≤450 ms. In adults, gender plays a more significant role, with women having longer corrected QT intervals than pre-adolescent girls. Males should continue to have QTc 2 mm in more than one right precordial lead (Figure 52-9). Clinical manifestations and/or family history are needed in addition to the ECG pattern to properly diagnose this syndrome.5

FIGURE 52-9. Patient with Brugada pattern on ECG. This is a type 1 pattern with a coved appearance to the ST segment elevation in leads V1 and V2.

ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a familial disorder that results in fibrofatty replacement of the ventricular myocardium, predominantly in the right ventricle, but can involve the left ventricle as well. This can lead to ventricular tachycardia and sudden death. Although there are recessive forms, ARVC is usually autosomal dominant with variable penetrance. Diagnostic criteria have been published,6 with classic ECG findings including right ventricular conduction delay and inverted T waves in the right precordial leads (V1–V3). An epsilon wave (high frequency signal in the ST segment) may also be seen.

CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR TACHYCARDIA Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) have a normal ECG at baseline but during periods of stress or exercise develop an array of arrhythmias, both atrial and ventricular, that can lead to polymorphic ventricular tachycardia and sudden death. It is caused by a mutation in either the ryanodine receptor or calsequestrin protein within the heart, both responsible for myocardial calcium regulation. A distinctive tachycardia seen in CPVT is bidirectional ventricular tachycardia, in which the QRS axis flips with every other beat. This pattern can also be seen in Andersen’s syndrome (Long QT type 7) and digoxin toxicity. KEY POINTS The electrocardiogram can be a powerful diagnostic tool. Using a systematic approach to interpret ECGs is imperative for accurate diagnosis and management. Caution should be used when reading ECGs in isolation, as a good history and physical examination can shed light on the significance of potential abnormalities. Since the ECG changes with age, pediatric standards should always be used when interpreting ECGs in the young.

REFERENCES 1. Rijnbeek PR, Witsenburg M, Schrama E, Hess J, Kors JA. New normal limits for the paediatric electrocardiogram. Eur Heart J. 2001;22(8):702-711. 2. Walsh EP, Berul C, Triedman JK. Cardiac Arrhythmias. In: Keane JF, Lock J, Fyler DC, eds. Nadas’ Pediatric Cardiology. 2nd ed. Philadelphia, PA: Saunders Elsevier; 2006:477-524. 3. Postema PG, De Jong JS, Van der Bilt IA, Wilde AA. Accurate electrocardiographic assessment of the QT interval: teach the tangent. Heart Rhythm. 2008;5(7):1015-1018.

4. Ackerman MJ, Priori SG, Willems S, et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm. 2011;8(8):1308-1339. 5. Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005;111(5):659-670. 6. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121(13):1533-1541.

CHAPTER

53

Congenital Heart Disease Barry A. Love

BACKGROUND Excluding patent ductus arteriosus of the premature infant, congenital heart disease is estimated to occur in about 0.8% of all children.1 Fortunately, most of these problems are minor or self-limited and do not require intervention. Congenital heart disease requiring intervention is less common—about 3 to 4/1000.2 For this important group of patients with heart disease, the past 70 years have witnessed a remarkable advance in diagnostic and therapeutic techniques such that many of these children with previously lethal defects may now lead relatively normal lives. Congenital heart disease ranks ninth in cause of death of infants 200 Torr almost always excludes cyanotic congenital heart disease. A value lower than that or an infant who has poor perfusion or remains critically ill despite the usual treatment measures for these other conditions should have a complete echocardiogram performed and interpreted by a physician qualified to diagnose complex congenital cardiac disease. In those infants suspected of having critical heart disease, empiric therapy with intravenous prostaglandin E-1 should be commenced immediately at a dose of between 0.01 μg/kg/min and 0.05 μg/kg/min. Prostaglandin E-1 will reopen a closing ductus arteriosus and restore pulmonary or systemic flow in critical heart disease, temporizing the situation and allowing time for diagnosis and planned intervention. Side effects of prostaglandin include apnea, hypotension, tachycardia, and fever. Two points are important to make at this juncture: 1. A “normal” fetal ultrasound or even an echocardiogram should not be reason to exclude the consideration of serious congenital heart disease. The fetal diagnosis of congenital heart disease is a very operatordependent test. There are many instances where infants with hearts diagnosed as “normal” in-utero turn out to have critical congenital heart disease. 2. An echocardiogram done on a critically ill newborn is a very difficult test to perform and interpret accurately. The infant may be ventilated on an oscillator, be very labile with blood pressure or saturation to even brief imaging, and a whole other host of hemodynamic factors may make the acquisition and interpretation difficult. In addition, some of the most difficult diagnoses to make by echocardiogram (such as obstructed total anomalous pulmonary venous return) typically present in this fashion. While more neonatologists, radiologists and hospitalists are acquiring advanced training in pediatric echocardiography, it is imperative that the physician caring for the critically ill infant does not overstep his/her degree of expertise when assessing for potential critical heart disease.6

DIAGNOSTIC EVALUATION FOR CONGENITAL HEART DISEASE

The recognition of congenital heart disease rests on the foundation of a good clinical history and physical examination. The electrocardiogram may add supplemental information to the assessment of cardiac anatomy, and is necessary for diagnosis of rhythm problems. The diagnosis of congenital heart disease has been revolutionized by two-dimensional echocardiography. Echocardiography provides a detailed anatomic and physiologic diagnosis of congenital heart disease, is noninvasive, and is performed at the bedside. Coupled with the Doppler technique, blood velocity may also be assessed, and this can be used to estimate chamber pressures and presence of heart or great vessel obstruction or valve regurgitation. Because almost all the ultrasound waves are reflected at tissue–air interfaces, echocardiography is limited to views where the lungs do not obstruct the path between the ultrasound beam and the heart. Factors that limit the usefulness of transthoracic echocardiography include obesity, obstructive lung disease, and certain chest wall deformities. Three-dimensional echocardiography is in the early stages of clinical application and may provide additional understanding of the anatomic information Magnetic resonance imaging (MRI) is increasingly being used for diagnostic evaluation of congenital heart disease. Advantages of cardiac MRI include excellent anatomic detail that is not limited by lung tissue, and MRI is therefore an especially good modality for imaging the great vessels. A major disadvantage is the requirement for sedation and the relatively long post-processing and interpretation time. Functional assessment of blood flow by MRI is possible and in many instances may be more accurate than echocardiography. Implanted metal such as surgical clips or embolization coils render bothersome imaging artifacts in many patients when performing cardiac/thoracic MRIs. MRI is generally contraindicated in patients with pacemakers and defibrillators. Cardiac catheterization with angiography was once considered the gold standard for anatomic diagnosis. However, echocardiography and MRI have now almost completely supplanted cardiac catheterization for anatomic diagnosis. Cardiac catheterization remains the gold standard for hemodynamic assessment, and increasingly cardiac catheterization is being used as an interventional rather than diagnostic tool.

MANAGEMENT OF CONGENITAL HEART DISEASE Definitive treatment of congenital heart disease is complex due to the wide variety of anatomy, the physiologic changes that occur over time (e.g. fall in pulmonary vascular resistance), and growth. Straightforward heart lesions are approached with surgical or transcatheter techniques that address a single problem (e.g. atrial septal defect). More complex forms of congenital heart disease are approached by cardiologists and cardiovascular surgeons by first attempting to decide if there are enough required cardiac “parts” to immediately or eventually repair the heart in such a way that it is physiologically, if not anatomically, correct. A “complete” or “two-ventricle” repair entails having separate systemic and pulmonary circuits, each with its own ventricle and atrioventricular valve able to pump deoxygenated blood to the lungs and oxygenated blood to the body. Although this type of repair is referred to as a “complete,” the resulting cardiac anatomy is often very different from normal. Where possible, complete repair is generally the preferred option. If there are insufficient “parts” to perform a complete repair (e.g. hypoplastic left heart syndrome, tricuspid atresia) then a single ventricle pathway must be pursued. The single ventricle pathway typically follows as a series of palliative surgeries that culminate with a Fontan procedure. This staged cardiac surgery ultimately results in a circulation where the venous blood return is passively routed to the lungs without passing though a ventricle, and then returns to a functionally single ventricle that pumps to the body. For this type of repair to succeed, the patient must have a relatively competent atrioventricular valve, good systolic ventricular function, unobstructed outflow from the heart, widely patent pulmonary arteries, and low pulmonary vascular resistance. The vascular resistance of the neonatal pulmonary vascular bed is too high to accept the passive pulmonary flow of a Fontan, and so a series of operations is necessary to ultimately achieve this type of palliation.

STAGE I: SINGLE VENTRICLE PATHWAY (Figure 53-1) The first stage of the single ventricle pathway provides for adequate pulmonary blood flow and unobstructed systemic flow in the newborn period. If the original cardiac anatomy is such that there is insufficient pulmonary

flow, a shunt is surgically placed to provide flow from the systemic circulation to the pulmonary circulation taking the place of the ductus arteriosus. One of the more common types of shunt is the modified BlalockTaussig shunt, which is a synthetic tube, placed from the subclavian artery to the ipsilateral pulmonary artery. There are many types of shunts and modifications of techniques, which depend on the individual anatomy and operator preference.

FIGURE 53-1. Stage I single ventricle pathway for hypoplastic left heart syndrome (see Figure 53-8 for unrepaired anatomy). 1. Right modified Blalock-Taussig shunt. 2. Large (surgically created) atrial septal defect. 3. Main pulmonary artery has been detached form branch pulmonary arteries. 4. Unobstructed egress of blood from the heart to the body has been achieved by using the main pulmonary artery and native aorta with additional patch material. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http:// www.pediatriccardiacinquest.mb.ca/pdf/index.html. Accessed March 21, 2017.)

The infant with an “ideal” physiology following a shunt has a systemic oxygen saturation of about 80%. Empirically this saturation has been found to be the best balance between systemic oxygen delivery and pulmonary overcirculation. Because blood circulating to the lungs is already partially saturated, shunt physiology is inefficient, and even with this “ideal” saturation of 80%, there is still about twice as much blood being circulated to the lungs as to the body. If the original cardiac anatomy dictates the single ventricle pathway but there is too much pulmonary flow such that the pulmonary pressures are high and/or the infant is in congestive heart failure, then the first stage in palliation is a pulmonary artery band (PAB). A PAB is a surgical “noose” placed around the pulmonary artery that creates an artificial stenosis and limits pulmonary flow. Of great importance in the single ventricle pathway is the provision for an unobstructed pathway for systemic blood flow. The proximal pulmonary artery and aorta are often used together to recreate a single unobstructed outflow from the heart in this first stage.

STAGE II: BIDIRECTIONAL GLENN (Figure 53-2) At about 4 to 8 months of age, the pulmonary vascular resistance has typically decreased sufficiently to accept a portion of the venous return in a passive manner. The second step of cardiac palliation for children proceeding down the single ventricle pathway is the “bidirectional Glenn,” or stage II. This involves connecting the superior vena cava directly to the pulmonary arteries and eliminating any previous shunted source of pulmonary blood flow. The arterial saturations are usually about the same before and after the procedure (~80%); however, this type of circulation is preferable because it is more efficient. In the shunted scenario, the single ventricle pumps blood to both the body and the lungs, and the blood pumped to the lungs is partially saturated—an inefficient type of arrangement. After the stage II repair, the heart is only pumping blood to the body, and only desaturated blood is flowing to the lungs, thereby increasing the efficiency.

FIGURE 53-2. Stage II single ventricle pathway for hypoplastic left heart syndrome. 1, 2. The right modified Blalock-Taussig shunt that was created at the stage 1 repair has been removed. The superior vena cava is divided and the proximal portion is attached directly to the right pulmonary artery, allowing the blood from the superior vena cava (SVC) to flow to both lungs (“bidirectional Glenn”). “Bidirectional” refers to the SVC blood flowing to both the right and left lungs. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http:// www.pediatriccardiacinquest.mb.ca/pdf/index.html. Accessed March 21, 2017.)

STAGE III: FONTAN (Figure 53-3) The third stage (Fontan completion) consists of redirecting the blood from the inferior vena cava to the lungs, thereby separating the pulmonary and systemic circuits. Directing the inferior vena cava blood to the lungs may be accomplished by use of a synthetic tube lateral to the heart (external conduit)

or using part of the right atrium as a wall (lateral tunnel). Often a small communication is left in the tube (fenestration) that may later be closed in the cardiac catheterization lab. After closure of the fenestration, these patients have normal oxygen saturations. Stage III is usually performed between ages 2 to 5 years, depending on center preference and technique.

FIGURE 53-3. Stage III –single ventricle pathway for hypoplastic left heart syndrome. 1. A tube has been placed within the right atrium to direct the blood from the inferior vena cava to the superior vena cava. The superior vena cava is reattached to the undersurface of the pulmonary artery. 2. Fenestration: a small hole is left in the tube to allow some of the venous blood to bypass the lungs. The fenestration keeps the venous pressure from rising too high after the Fontan operation, but results in some desaturation. The fenestration may be optional in some patients, but if desired can be easily closed in the cardiac catheterization laboratory afterward. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http:// www.pediatriccardiacinquest.mb.ca/pdf/index.html. Accessed March

21, 2017.) Postoperative problems encountered by patients with Fontan-type repairs include early problems such as prolonged pleural effusions, and late problems such as increased risk for stroke and thrombus formation in the Fontan, arrhythmia development (especially atrial flutter), and protein-losing enteropathy.

CRITICAL NEWBORN HEART DISEASE CRITICAL NEWBORN HEART DISEASE: PATENT DUCTUS ARTERIOSUS AUGMENTS Pulmonary Blood Flow In this set of lesions, a portion of the normal anatomy supporting pulmonary blood flow (tricuspid valve, right ventricle, pulmonary valve, and pulmonary arteries) is small or absent. The ductus arteriosus provides pulmonary blood flow by shunting systemic blood flow to the lungs. While d-transposition of the great arteries (d-TGA) is also included in this section, the physiology of this lesion is somewhat different, as explained below. Tricuspid Atresia (Figure 53-4) The tricuspid valve is atretic in this lesion and the right ventricle is typically severely hypoplastic. The pulmonary artery arises from the right ventricle, and there may be a ventricular septal defect. When the ventricular septal defect and/or pulmonary artery are hypoplastic, pulmonary blood flow is inadequate and the ductus arteriosus is required to support pulmonary blood flow. In some instances, there may be adequate pulmonary flow through the ventricular septal defect, and in other cases the ventricular septal defect and pulmonary artery may be so large that the infant has too much pulmonary blood flow.

FIGURE 53-4. Tricuspid atresia (normal great arteries). 1. Patent foramen ovale with flow right to left. 2. Atretic tricuspid valve. 3. Ventricular septal defect (VSD). Size of VSD and amount of restriction is variable. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http://www.pediatriccardiacinquest.mb.ca/pdf/index.html. Accessed March 21, 2017.) If the pulmonary flow is inadequate, a systemic-to-pulmonary artery shunt is required. If there is too much pulmonary flow, the infant may require a pulmonary artery band to reduce the amount of pulmonary flow. In some instances, nature provides just enough pulmonary stenosis so that the child is not in congestive heart failure with adequate systemic saturations. In any case, the infant with tricuspid atresia also follows a single ventricle pathway. In some instances, tricuspid atresia is also found with transposition of the great arteries. These infants will require a repair similar to hypoplastic left heart syndrome, as the aortic valve and arch are typically hypoplastic when the great arteries are transposed. Tetralogy of Fallot (Figure 53-5) This condition arises when the

ventricular septal muscle between the aorta and the pulmonary artery is deviated anteriorly. This causes the pulmonary outflow area to be stenotic, a special type of ventricular septal defect to be present under the aorta (anterior malalignment), and the aorta to then be aligned over the ventricular septal defect and crest of the ventricular septum (overriding aorta). There is usually accompanying right ventricular hypertrophy, which completes the four parts of the tetralogy. While simple in its description, there is a huge variation in the severity of this disease that is largely dependent upon the pulmonary artery architecture. When there are well-developed pulmonary arteries and mild or moderate pulmonary stenosis, this lesion is not critical. Systemic oxygen saturations from 80% to 95% are possible, and infants may undergo elective complete repair between 3 and 12 months of age. Surgical repair in these cases involves closing the ventricular septal defect and relieving the pulmonary obstruction by patching the right ventricular outflow tract, pulmonary valve, and main pulmonary artery as needed.7

FIGURE 53-5. Tetralogy of Fallot. 1. Pulmonary valve and subvalvar stenosis. 2. Hypertrophied right ventricle. 3. Overriding aorta. 4. Ventricular septal defect. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994.

2000. Available at http://www.pediatriccardiacinquest.mb.ca/pdf/ index.html. Accessed March 21, 2017.) More severe obstruction at the pulmonary valve and right ventricular outflow tract may lead to more profound cyanosis and ductal dependency. These infants may require a shunt to increase pulmonary flow in anticipation of a complete repair at an older age, or they may undergo complete repair as infants. At the extreme, there may be complete atresia of the pulmonary valve, and only a thread-like main pulmonary artery. Associated with this more severe obstruction are downstream architectural abnormalities of the pulmonary arteries, with stenosis or absence of branches of the pulmonary arteries and the presence of collateral vessels from the aorta to the pulmonary arteries that may supply multiple segments of the lung. Presence of these aorto-pulmonary collateral vessels and small, stenotic pulmonary arteries makes definitive surgical repair a challenge, and is individualized for each patient’s anatomy, usually requiring a series of staged operations and cardiac catheterizations for dilation of stenotic pulmonary arteries.8 Important pediatric concerns of infants with tetralogy of Fallot, especially when associated with pulmonary atresia, is the association with the 22q deletion/velocardiofacial/DiGeorge syndrome complex. Infants with this heart disease should have genetic testing (fluorescence in situ hybridization (FISH)) done for the 22q deletion, and calcium levels should be monitored in the newborn period. If positive, immunologic workup and genetic and anticipatory guidance should be provided to patients and families. Another important issue for patients with tetralogy of Fallot is the possibility of experiencing “tet-spells,” which are sudden episodes of profound desaturation. The classic explanation of the pathophysiology of this condition is “spasm” of the muscle under the pulmonary valve leading to decreased pulmonary flow and increased cyanosis, though this is certainly an oversimplified mechanism. Tet-spells can be treated by increasing the systemic resistance to force more blood across the pulmonary circuit. This can be done mechanically by putting the patient in a knee-to-chest position, or pharmacologically by administering intravenous phenylephrine. Supplemental oxygen may be of some benefit as is the administration of subcutaneous or intravenous morphine as a sedative. Ketamine is an especially useful drug in managing tet-spells, as it provides both sedation and

increased systemic vascular resistance, and it can be administered intravenously or intramuscularly. Patients who have had one or more tetspells should be considered for surgical palliation or repair, as these spells, if prolonged, can be associated with morbidity and mortality. d-Transposition of the Great Arteries (Figure 53-6) In dtransposition of the great arteries (d-TGA), the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. This leads to systemic blood returning from the body, recirculating to the body, and pulmonary blood recirculating to the lungs (parallel circulations), and profound cyanosis. Survival depends on mixing of the two circulations at the atrial, ventricular, or great vessel levels. Prostaglandin E-1 temporizes this condition by increasing the amount of blood in the pulmonary system, which encourages mixing at the atrial or ventricular level. For prostaglandin to work, there must be an atrial or ventricular septal defect. If no septal defect is present, or if mixing is inadequate as evidenced by oxygen saturation less than 80%, an atrial septal defect may need to be created, or an existing one enlarged, to stabilize the infant in anticipation of corrective surgery. This procedure (balloon atrial septostomy) is accomplished by passing a balloon catheter through the atrial septum, inflating the balloon, and pulling it back across the atrial septum to tear a hole in the atrial septum, thereby creating an atrial septal defect. This procedure can be performed at the bedside under echocardiographic guidance alone, or in the cardiac catheterization laboratory under fluoroscopic guidance.9

FIGURE 53-6. d-Transposition of the great arteries. 1. Patent foramen ovale with left-to-right flow. 2. Transposed great arteries. Aorta arises from right ventricle. 3. Ductus arteriosus with flow from aorta to pulmonary artery. 4. Transposed great arteries. Pulmonary artery arises from left ventricle. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http://www.pediatriccardiacinquest.mb.ca/pdf/ index.html. Accessed March 21, 2017.) Definitive therapy for d-TGA is surgical. Since the mid-1980s, the procedure of choice for this lesion is the “arterial switch,” where the aorta and pulmonary arteries are transected above the valves and reattached to their physiologically correct ventricle. The Achilles-heel of this procedure is transfer of the coronary arteries, which must be removed separately and reattached to the aorta. The coronary artery anatomy is variable and makes preoperative definition of this anatomy important and appropriate surgical planning imperative.10 Associated lesions are usually corrected at the same surgery, which may include ventricular septal defect and coarctation of the aorta. The prognosis for children with d-TGA is generally excellent.

Postoperative problems related to coronary artery obstruction can occur, as can residual lesions related to the surgical correction.

CRITICAL NEWBORN HEART DISEASE: PATENT DUCTUS ARTERIOSUS AUGMENTS SYSTEMIC BLOOD FLOW In this set of lesions, a portion of the normal anatomy supporting systemic blood flow (mitral valve, left ventricle, aortic valve, and aorta) is narrowed, small, or absent. The ductus arteriosus provides systemic blood flow by shunting some of the pulmonary blood through the ductus arteriosus to the aorta. Infants with these lesions often present with shock or decreased femoral pulses as the ductus arteriosus closes. Critical aortic stenosis, interrupted aortic arch, and critical coarctation of the aorta are all examples of critical left heart disease that require prostaglandin to stabilize and temporize, but can all have repairs that result in a complete repair. Hypoplastic left heart syndrome is an extreme form of left heart obstructive disease that requires a single ventricle surgical approach.

CRITICAL AORTIC STENOSIS In this lesion, the aortic valve opening is narrowed by partial fusion of the leaflets and there is severe obstruction to outflow from the left ventricle. Before repair, most of the blood returning from the left atrium passes across the foramen ovale to the right atrium and subsequently to the right ventricle and main pulmonary artery, where a portion of the blood passes across the ductus arteriosus to the body and a portion passes to the lungs. For this lesion to be defined as critical aortic stenosis rather than hypoplastic left heart syndrome, the left ventricle, and mitral valve need to be of normal size, though the left ventricular function is often severely depressed before intervention. Treatment of this condition is usually transcatheter balloon dilation of the aortic valve. This procedure relieves the narrowing at the valve sufficiently to permit recovery of left ventricular function, allowing the left ventricle to pump all the systemic blood. Prostaglandin can be discontinued after the dilation once the left ventricle has recovered adequately, but this may take

several days. There is usually residual aortic stenosis of a mild to moderate degree after neonatal dilation, and these patients usually require further interventions for the aortic valve later in infancy or childhood.11

COARCTATION OF THE AORTA AND INTERRUPTED AORTIC ARCH In coarctation of the aorta, the aorta is severely narrowed at or just beyond the origin of the left subclavian artery. In interrupted aortic arch, there is a discontinuity of the aorta at a point between one of the head and neck vessels (usually between the left carotid and left subclavian arteries). Both lesions typically present with shock and/or decreased femoral pulses as the ductus arteriosus closes and are temporized by prostaglandin E-1, which opens the ductus arteriosus, supplementing systemic blood flow. The treatment of either lesion is surgical repair in the neonatal period. The surgical repair for critical coarctation usually involves removing the narrowed segment and rejoining the descending aorta to the aortic arch in an unobstructed fashion.12 The repair is usually done without the need for cardiopulmonary bypass. The repair of interrupted aortic arch is a much more complex arch reconstruction requiring the use of cardiopulmonary bypass. A ventricular septal defect—associated with the most common type of interrupted aortic arch—is repaired at the same surgery. Interrupted aortic arch (type B) is also associated with the 22q deletion/DiGeorge complex and as with tetralogy of Fallot, these patients should have appropriate calcium monitoring, and genetic and immunologic workup.

HYPOPLASTIC LEFT HEART SYNDROME (Figure 53-7) When the left-sided heart structures (mitral valve, left ventricle, aortic valve, and aortic arch) are too small to support the cardiac output needed for the body, the condition is referred to as hypoplastic left heart syndrome. The mitral or aortic valves may be so small as to be atretic. In the unrepaired state, the right ventricle pumps the combined venous return from the body and from the lungs to the pulmonary artery, where a portion of the blood passes to the lungs and a portion passes through the ductus arteriosus to supply the body. The degree of cyanosis depends on the proportion of

pulmonary to systemic blood flow. As the ductus arteriosus closes, more blood is directed to the lungs and less to the body, leading to higher systemic saturations but signs and symptoms of low cardiac output and ultimately shock. Prostaglandin E-1 opens the ductus arteriosus and temporizes the situation.

FIGURE 53-7. Hypoplastic left heart syndrome. 1. Atrial septal defect with left-to-right flow. 2. Coarctation of the aorta. 3. Patent ductus arteriosus with flow from pulmonary artery to aorta. 4. Hypoplastic ascending aorta. 5. Small mitral valve—sometimes atretic. 6. Small Aortic valve—sometimes atretic. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http://www.pediatriccardiacinquest.mb.ca/pdf/ index.html. Accessed March 21, 2017.) Surgical repair of this condition follows the principles of the single ventricle pathway outlined in a subsequent section and involves three stages. The first stage is more involved than most other types of first-stage single ventricle palliations because the main pulmonary artery must be used along with the native (small) aorta and prosthetic patch material to refashion the

aortic arch. Pulmonary blood flow is provided by placing a tube graft from either a subclavian artery to pulmonary artery (modified Blalock Taussig shunt) or from the right ventricular outflow tract to the pulmonary artery (Sano modification13). The initial palliation strategy for hypoplastic left heart syndrome is technically difficult and even in the best centers has one of the higher mortalities for congenital heart disease surgery. As a result, the past decade has seen the development and refinement of an alternative strategy called a “hybrid stage I” (Figure 53-8). This technique is a hybrid surgical and transcatheter technique where a surgical noose (band) is placed on each pulmonary artery to limit pulmonary blood flow, and a stent is placed in the ductus arteriosus to maintain systemic perfusion. The atrial septum is also enlarged if needed using catheter balloon and/or stent techniques. This hybrid stage I approach results in a much more stable infant post-procedure. The infant is then adequately palliated until he/she is about 4 to 6 months of age and then undergoes a “comprehensive stage II” procedure where the ductal stent and pulmonary artery bands are removed, and then the previously described first-stage operation is performed, with the exception that a Glenn shunt (superior vena cava to pulmonary artery) is used rather than a systemicpulmonary artery shunt. This makes for a more complex stage II procedure, but at a time when the infant is much bigger, making the procedure easier. In addition, the Glenn shunt is a more stable source of pulmonary blood flow than is a systemic-pulmonary artery shunt. The infant then continues down the single ventricle pathway to arrive at a Fontan procedure typically between 3 to 5 years of age. These two techniques in multicenter series appear to have similar outcomes, but some centers prefer one technique over the other depending on their center-specific outcomes for both approaches.

FIGURE 53-8. Hypoplastic left heart syndrome after hybrid stage I palliation. 1. Stent in atrial septum (sometimes required). 2. Pulmonary artery bands on right and left pulmonary arteries. 3. Stent across ductus arteriosus. Note that stent often covers the origin of the aortic arch. Blood traverses through the struts of the stent to reach the head and perfuse the coronary arteries. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http://www.pediatriccardiacinquest.mb.ca/pdf/ index.html. Accessed March 21, 2017.) Another strategy for managing patients with hypoplastic left heart syndrome is infant heart transplantation. Despite significant advances in immunosuppressive regimens, success of the heart transplantation strategy for hypoplastic left heart syndrome has been limited by a lack of donor organs and late graft failure, which occurs over decades in virtually all transplanted hearts.14

CRITICAL NEWBORN HEART DISEASE: PATENT

DUCTUS ARTERIOSUS NOT REQUIRED Total anomalous pulmonary venous return (TAPVR) with obstruction is the classic example of newborn heart disease that is not temporized by prostaglandin E-1. In this lesion, the pulmonary veins do not return to the left atrium, but communicate with the systemic veins by means of a supplemental venous channel. This channel may be obstructed, leading to progressive pulmonary edema, “whiteout” on chest x-ray, and an inability to ventilate or oxygenate. This lesion is the one most often mistaken for pulmonary disease in the newborn. If a newborn has progressive pulmonary disease and is failing conventional treatment, the diagnosis of congenital heart disease— especially obstructed TAPVR—should be entertained and an echocardiogram obtained. Repair consists of reattaching the pulmonary venous chamber to the left atrium.

LESIONS PRESENTING WITH CONGESTIVE HEART FAILURE OR MURMUR IN INFANCY As the pulmonary vascular resistance falls in the first weeks of life, lesions that permit the transmission of high flow or pressure to the lungs become manifest. These lesions include ventricular septal defect (VSD), atrioventricular septal defect (AVSD), and patent ductus arteriosus (PDA).

VENTRICULAR SEPTAL DEFECT (Figure 53-9) The ventricular septum is a complex anatomical structure dividing the left and right ventricles. Near the aortic valve, the septum is a thin membrane (membranous septum) whereas it is a thick muscle in other parts. A defect in the wall is called a ventricular septal defect (VSD). The most common defects occur in the membranous septum, but they can occur anywhere in the wall, and may be multiple. The presentation of VSD is dependent on two factors—the size of the ventricular septal defect and the pulmonary vascular resistance.

FIGURE 53-9. Ventricular septal defect. 1. Ventricular septal defect (VSD) with flow from left ventricle to right ventricle. Position of VSD in ventricular septum determines type. More common type located just underneath the aortic valve (membranous). (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http:// www.pediatriccardiacinquest.mb.ca/pdf/index.html. Accessed March 21, 2017.) Small VSD A small muscular ventricular septal defect presents in the first days to weeks of life with a high-pitched, asymptomatic murmur. When the resistance in the pulmonary circulation falls, so too does the right ventricular pressure, creating a large pressure difference between the left and right ventricles. A small hole between the ventricles creates a turbulent left-to-right flow jet, and a loud murmur is heard. In this scenario, the total amount of blood recirculated through the lungs and back to the left ventricle is minimal. Moderate VSD If the VSD is somewhat larger, as the pulmonary resistance falls, the VSD may still be small enough to limit the pressure transmitted to the right ventricle but large enough to admit more blood flow. In this

circumstance, the amount of extra blood flow to the lungs recirculating back to the left heart may be significant, and the infant may have respiratory symptoms and perhaps some degree of congestive heart failure. A loud murmur will be present as the pressure gradient and flow are high. Large VSD At a certain point, the VSD is so large as to be unable to limit the pressure transmitted by the left ventricle. As the pulmonary resistance falls, there is more and more blood recirculating to the lungs, but the pressure in the right ventricle and pulmonary artery remains at systemic levels (socalled “unrestrictive” defects). The intensity of the systolic murmur is proportional to the degree of left-to-right shunting; however, there is often only a relatively soft murmur because there is little turbulence across the large VSD. With excessive pulmonary blood flow, infants typically display failure to thrive. If left uncorrected for a prolonged period of time, these infants run the risk of developing irreversible changes to the pulmonary vasculature that progresses even after the defect is corrected. Pulmonary blood vessels when exposed to high pressure for too long develop irreversible changes called pulmonary vascular obstructive disease (PVOD). PVOD is averted by limiting the pressure to which the lung vasculature is exposed by either closing the VSD or applying downstream resistance to flow by placing a pulmonary artery “band.” Irreversible PVOD does not usually develop until after age 1 year, although the time course after this point is remarkably variable. Patients with Down syndrome seem to be at higher risk at an earlier age and intervention is recommended before age 9 months in this subgroup.15 Although most infants with large VSDs with left-to-right shunt present with congestive heart failure as the pulmonary resistance falls, there is a small group of patients with unrestrictive VSDs in whom the pulmonary resistance falls very little or not at all. These infants will not develop congestive heart failure, and if the pulmonary resistance is very high, may not have much additional pulmonary flow. There is little if any heart murmur as there is no restriction to flow at the ventricular level and not enough increase in pulmonary flow to produce an appreciable flow murmur. The only cardiac sign may be relatively subtle—a single, loud second heart sound. These children often have frequent respiratory illness, which may further limit the ability of the pediatrician to clearly auscultate the heart. With no loud murmur demanding attention, and the more subtle signs often masked by respiratory noise, these children may go undiagnosed for a prolonged period

and are therefore at high risk of developing PVOD. The pediatric hospitalist should keep this infrequent presentation of congenital heart disease in mind when assessing and treating infants and young children with frequent respiratory problems. Surgical closure of a VSD is indicated in infancy (less than 1 year of age) if there is persistent congestive heart failure, or if the pulmonary pressures remain high. As some VSDs get smaller with time and even close, medical management with anticongestive medications (lasix, digoxin, angiotensinconverting enzyme inhibitors) may be indicated for a period in some infants with moderate and large VSDs. More complex decision-making is required for VSDs that are multiple—especially those located at the apex of the heart. This location is typically difficult to approach surgically. In this instance, a pulmonary artery band may be placed to limit pulmonary flow and pressure until the child is larger. Transcatheter closure devices may be another option in selected children–, sometimes combined with a surgical approach.16 Moderate ventricular septal defects are usually followed through infancy and early childhood as many become small and not require intervention. Closure is recommended for VSDs that remain moderate with a pulmonary to systemic flow ratio of more than about 2:1. Transcatheter closure devices for membranous VSDs are in clinical trials. These devices need special design and delivery systems to avoid interfering with the aortic valve, which forms the superior border of the membranous septum.17 Atrioventricular Septal Defect (Figure 53-10) The atrioventricular septal defect (AVSD) is a more complex form of heart disease than simply a combination of an atrial and ventricular septal defect. It involves an incomplete separation of the embryonic common atrioventricular valve into separate tricuspid and mitral components. In addition, there is both a ventricular and atrial septal defect in the portion of the septa that are immediately adjacent to the atrioventricular valve. This lesion is often associated with Down syndrome. Presentation is similar to those patients with moderate or large ventricular septal defects. The septal defects do not close spontaneously and so all patients with AVSD will require surgical repair to partition the atrioventricular valve and close the atrial and ventricular septal defects. Partitioning the atrioventricular valve is surgically challenging so as not to produce mitral stenosis or regurgitation. Most centers perform

complete repairs on these children between 3 and 6 months when the infants are somewhat larger, but soon enough to prevent the development of pulmonary vascular disease.18

FIGURE 53-10. Atrioventricular septal defect. 1. Atrial septal defect (ostium primum type). 2. Right side of common atrioventricular valve (incompletely formed tricuspid valve). 3. Left side of common atrioventricular valve (incompletely formed mitral valve). 4. Ventricular septal defect (of “inlet” ventricular septal type). (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http:// www.pediatriccardiacinquest.mb.ca/pdf/index.html. Accessed March 21, 2017.) Patent Ductus Arteriosus (Figure 53-11) The ductus arteriosus is the conduit for fetal blood to bypass the lungs in utero. After birth, the ductus usually closes in the first days of life. If it does not close, as pulmonary vascular resistance falls, blood shunts from the aorta into the pulmonary artery. Depending on the size of the ductus arteriosus and the pulmonary vascular resistance, the ductus will shunt a variable amount of blood similar

to the physiology of a ventricular septal defect, and this lesion may present with congestive heart failure if large, or a murmur if the ductus is smaller.

FIGURE 53-11. Patent ductus arteriosus. 1. Ductus arteriosus with flow from aorta into pulmonary artery. (Reproduced from Sinclair CM. The Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http://www.pediatriccardiacinquest.mb.ca/pdf/ index.html. Accessed March 21, 2017.) The treatment of a patent ductus arteriosus is surgical or transcatheter closure. Transcatheter closure is now routine in children and infants older than 6 months.19 Surgical closure is indicated for refractory heart failure in smaller infants, premature infants, or in those where there is other accompanying heart disease that requires surgical intervention. A small patent ductus arteriosus associated with a murmur—even in the absence of significant shunting—is closed in the catheterization laboratory to prevent the lifelong risk of endarteritis of the ductus, which is estimated at ~0.45%/year.20

The general consensus is that a small ductus arteriosus found by echocardiography without an associated murmur should not be closed, as the risk of endarteritis does not appear to be higher than that of the general population. Coarctation of the Aorta When severe, this lesion presents in the newborn period as a critical newborn heart lesion with shock or cyanosis. If milder, this lesion may present with a murmur, or more commonly, with the astute pediatrician noting decreased intensity of the femoral pulses. Repair of this lesion is usually done surgically. Diagnosis of coarctation later in childhood is sometimes made by the finding of upper extremity hypertension. Therapeutic options later in childhood and through adulthood include surgery or transcatheter balloon dilation with stent placement. Later diagnosis and repair of this lesion may lead to persistent hypertension even after repair.

LESIONS PRESENTING WITH A MURMUR IN CHILDHOOD Less severe obstructions and shunts typically present with a murmur in childhood. Stenosis of the semilunar valves (aortic stenosis or pulmonary stenosis) as well as atrial septal defects may present in this time frame.

PULMONARY STENOSIS AND AORTIC STENOSIS These lesions, when severe, present as critical heart disease in the newborn, as previously discussed. When milder, they present as a murmur. Treatment is undertaken for pulmonary stenosis when the narrowing is moderate or severe, and consists of transcatheter balloon dilation of the valve. Such intervention is highly effective and typically does not need to be repeated.21 Treatment is undertaken for aortic stenosis when the gradient is moderate to severe. The first approach is usually balloon dilation of the valve. The success of aortic balloon dilation may be good, but relief of stenosis in many patients is often accompanied by creation of aortic regurgitation. Surgical valve repair, replacing the aortic valve with the patient’s own pulmonary valve (Ross procedure), or prosthetic valve replacement are all options. Prosthetic valve replacement is undesirable in small children because of the limited lifespan of these valves, and growth considerations.

ATRIAL SEPTAL DEFECT (Figure 53-12) The atrial septum divides the right and left atria. A defect in the wall is called an atrial septal defect (ASD). Blood returning to the left atrium passes from left to right across the ASD and recirculates to the lungs, placing a volume load on the right ventricle, pulmonary vascular bed, and atria. The amount of excess blood flow (or “shunt”) is determined by both the size of the defect as well as the balance between the diastolic compliance of the left and right heart. In infancy, the right ventricle is relatively noncompliant, and there may be minimal flow across even a large ASD. During childhood the right ventricle becomes more compliant while the left ventricular compliance decreases, thereby increasing the amount of shunting. The consequences of an unrepaired ASD include right ventricular dysfunction and atrial arrhythmias from chronic volume load, the potentially devastating complication of pulmonary hypertension and pulmonary vascular disease— which may develop in up to 5% to 10% of these patients during adult life— and risk of paradoxical embolus.

FIGURE 53-12. Atrial septal defect. 1. Atrial septal defect with flow from left atrium into right atrium. (Reproduced from Sinclair CM. The

Report of the Manitoba Pediatric Cardiac Surgery Inquest: An Inquiry Into Twelve Deaths at the Winnipeg Health Sciences Center in 1994. 2000. Available at http://www.pediatriccardiacinquest.mb.ca/pdf/ index.html. Accessed March 21, 2017.) If there is evidence of right ventricular volume overload by echocardiography, then the lesion should be closed. Usually this decision is made after age 2 years, as some ASDs will get smaller over the first years of life. If the defect is of the secundum type it may be closed in the cardiac catheterization laboratory with specially designed closure devices.22 If the defect is large or is located in other anatomic regions of the atrium, then surgery is required for closure.

ADMISSION AND DISCHARGE CRITERIA Patients who present critically ill obviously require admission. Infants with congestive heart failure may require admission for initiation or optimization of medical management that may include diuretics, digoxin, and supplemental caloric addition and/or nasogastic feeding to assist with growth. Patients with congenital heart disease may require admission for intercurrent illness such as respiratory syncytial virus and other viral or bacterial infections. Infants with congenital heart disease may have a more severe course with a higher risk of complications than other children. Discharge of patients after congenital heart disease surgery has been evolving. There has been a greater move to “fast-track” patients with congenital heart disease after surgery with a view toward early extubation, early removal of central lines and chest tubes, and ultimately early discharge. The average length of stay in some centers for simple congenital heart disease surgery is now 1 day for atrial septal defect and 3 days for VSD repair.23 When properly implemented, this has been shown to not increase mortality or readmission rates. Infants and children are discharged postoperatively once all the acute surgical issues are resolved, they are able to be fed, pain is well controlled, and they are on an oral medical regimen. Early discharge, however, also mandates close early follow-up. For infants with shunt-dependent pulmonary blood flow or those with hypoplastic left heart syndrome after conventional stage I or hybrid stage I, a

significant “interstage” mortality has been recognized. Some of these children will die at home or present in extremis to the emergency department for anatomic problems that can arise insidiously. For this reason, many centers have taken the approach that a very close monitoring program will lead to decrease in interstage mortality if these children can be monitored very closely at home and appropriate intervention undertaken preemptively. These conditions include development of residual arch obstruction, shunt narrowing, atrial septal narrowing, and other issues. Preliminary research seems to support this hypothesis.24 Many centers have established intensive home monitoring programs where parents are provided with an oxygen saturation monitor and a scale to monitor daily oxygen saturations and at least biweekly weights. Some programs use visiting nurse checks one a week or more to ensure medication compliance, verify oxygen saturation and weight checks, and communicate with the cardiology and pediatric team.

CONSULTATION Close collaboration between the pediatric cardiologist and the pediatrician is essential for successful outcomes in patients with complex congenital heart disease. Other teams that are frequently consulted include: Genetics: In many children, there may be an underlying genetic syndrome or genetic etiology that is important for prognosis, anticipatory guidance and reproductive counseling. Neurology: Congenital heart disease may be associated with neurologic deficits or these may develop as a result of neurologic injury during interventions, primarily cardiac surgical. Developmental and behavioral pediatrics: Many children with complex congenital heart disease will require assessment for developmental and behavioral needs so they can be provided with the needed services. Social work: The social work team is invaluable in assessing the needs of the patient and family with regard to access to care, insurance, social supports, ability of parents and caregivers to care for a child with additional needs, etc. Other subspecialty consultations may be needed as indicated.

SPECIAL CONSIDERATIONS FOR THE PEDIATRIC HOSPITALIST CARING FOR PATIENTS WITH CONGENITAL HEART DISEASE ENDOCARDITIS PROPHYLAXIS Most congenital heart lesions put patients at risk for the development of endocarditis. Children with congenital heart disease should have regular dental care and maintain good oral hygiene. It used to be recommended that antibiotics be given prior to dental and other gastrointestinal procedures for most patients with congenital heart disease. These recommendations were recently revised and have generally been lifted except for patients with unrepaired cyanotic congenital heart disease and others with turbulence adjacent to prosthetic material (see Chapter 54, Infective endocarditis).

RESPIRATORY SYNCYTIAL VIRUS Respiratory syncytial virus (RSV) may produce severe disease in children less than 2 years of age with cyanotic heart disease, single ventricle anatomy pre or post surgery, dilated or hypertrophic cardiomyopathy, pulmonary hypertension, or significant left-to-right shunts requiring medication to control pulmonary vascular congestion. In such patients the American Academy of Pediatrics recommends monthly passive immunization during the RSV season with palivizumab.25 Steps should be taken to minimize the risk from this potentially life-threatening illness. If a congenital heart lesion needs repair, consideration should be given to timing the repair before the RSV season which typically extends from November through March. Appropriate reverse isolation of hospitalized children with heart disease at high risk should be undertaken to prevent nosocomial transmission.

PACEMAKERS AND IMPLANTABLE DEFIBRILLATORS Children with congenital heart disease may have congenital or iatrogenic surgical conduction defects that necessitate pacemaker placement. Pacemakers work by emitting an electric current that stimulates cardiac depolarization. Though pacemakers are very reliable, the interface between the pacemaker and the heart (the leads) are prone to a variety of problems.

Extreme physiologic changes (e.g. acidosis) in the patient may also increase the amount of energy needed to depolarize the heart above that set by the pacemaker. In-hospital monitoring of children with pacemakers using a cardiac monitor is insufficient, as these monitors will usually detect the pacemaker impulse even when not followed by a cardiac depolarization. A physiologic monitor such as a pulse oximeter or arterial waveform should be used to confirm heat rate in paced patients. Similarly, during resuscitation, one should not mistake the pacemaker impulse as a cardiac depolarization, and physiologic parameters (e.g. palpation of a pulse) should be used to determine the cardiac rate. Patients with pacemakers or implantable defibrillators requiring non– cardiac surgery may need the device reprogrammed prior to surgery to avoid oversensing, and electrocautery should be avoided. Patients with these devices may not undergo MRI examinations with the exception of the Medtronic Revo dual chamber pacemaker system with both atrial and ventricular leads in place and the pacemaker verified and programmed for MRI use.

CENTRAL ACCESS AND PARADOXICAL EMBOLIZATION Central access may be difficult in patients with congenital heart disease because of vein occlusion owing to previous long-term access lines or cardiac catheterization access. Central access in the internal jugular or subclavian veins is usually discouraged in patients who are progressing down the single ventricle pathway because of the risk of possible superior vena cava obstruction making the Fontan palliation problematic. In children with any right-to-left shunting, bubbles or clots introduced in the venous system risk traveling to the cerebral circulation, causing strokes. Intravenous lines should be meticulously cleared of air prior to infusion to prevent this potential problem, and indwelling lines should be heparinized.

FEEDING/GROWTH ISSUES Infants with congenital heart disease and pulmonary overcirculation have significantly increased caloric needs. Infants may require more than 160

Kcal/kg/day to grow with these increased metabolic demands. In addition, infants may be less able to tolerate normal feeding volumes due to tachypnea. Increased caloric density of feeds is usually required and tube feeding is sometimes required to assist these infants.

PREVENTION There have been studies showing a decrease in the incidence of congenital heart defects in infants of mothers taking multivitamins with folic acid during the periconceptual period compared to a similar group of mothers who did not take supplements. A case-control study in Atlanta showed a 24% decrease in the incidence of congenital heart disease,26 and a study in California had similar results. These studies are not definitive, but folate appears to be a reasonable preconceptual regimen, and the additional riskreduction of neural tube defects appears to make this recommendation compelling. Other than folate, there have been no other preventative measures identified that may decrease the risk of congenital heart disease. Fetal diagnosis may provide the option of termination for those defects with poor prognosis if the parents so choose. KEY POINTS The incidence of congenital heart disease is 0.8%, while the incidence of congenital heart disease requiring intervention is lower—about 3 to 4/1000. Nonetheless, congenital heart disease remains in the top 10 causes of death for children 2 positive cultures of blood samples drawn >12 h apart, or All of 3 or a majority of >4 separate blood cultures (with first and last samples drawn >1 h apart) Evidence of endocardial involvement Positive echocardiogram for IE defined as: Oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation, or Abscess, or New partial dehiscence of prosthetic valve New valvular regurgitation (worsening or changing of preexisting murmur not sufficient) Minor Criteria Predisposition: predisposing heart condition or IV drug use Fever: temperature >38°C

Vascular phenomena: major arterial emboli, septic pulmonary infarct, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway lesions Immunologic phenomena: glomerulonephritis, Osler nodes, Roth spots, and rheumatoid factor Microbiologic evidence: positive blood culture that does not meet major criteria (see above) or serologic evidence of active infection with organism consistent with IE Echocardiographic findings: consistent with IE but do not meet major criteria (see above) Source: Reproduced with permission from Durack OT, Lukes AS, Bright DK. New criteria for diagnosis of infective endocarditis: utilization of specific echocardiographic findings. Am J Med. 1994;96:200-209. Copyright © Elsevier. *Excludes single positive cultures for coagulase-negative staphylococci and organisms that do not cause endocarditis. HACEK, Haemophilus species, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella species, and Kingella kingae.

TABLE 54-3

Clinical Criteria for the Diagnosis of Infective Endocarditis (IE)

Definite IE Pathologic criteria Microorganisms: demonstrated by culture or histology in a vegetation that has embolized or in an intracardiac abscess, or Pathologic lesions: vegetation or intracardiac abscess present, confirmed by histology showing active endocarditis Clinical criteria (as defined in Table 54-2) 2 major criteria, or 1 major criterion and 3 minor criteria, or 5 minor criteria Possible IE Findings consistent with IE that fall short of “definite” but not “rejected” Rejected

Firm alternative diagnosis for manifestations of endocarditis, or Resolution of manifestations of endocarditis with antibiotic therapy for >4 days, or No pathologic evidence of IE at surgery or autopsy, after antibiotic therapy for >4 days Source: Reproduced with permission from Durack OT, Lukes AS, Bright DK. New criteria for diagnosis of infective endocarditis: utilization of specific echocardiographic findings. Am J Med. 1994;96:200-209. Copyright © Elsevier.

IMAGING Echocardiography with Doppler color flow mapping is the standard diagnostic modality in patients with suspected infective endocarditis. It can identify valvular vegetations (Figure 54-1) as well as impaired cardiac performance, prosthetic valve dehiscence, disturbed conduit flow, and abscess. Moreover, echocardiography can define the presence of an occult structural abnormality, such as a bicuspid aortic valve, thus identifying a clinically silent risk factor. It has been well established that echocardiography can detect cardiac involvement in patients with otherwise occult infective endocarditis,12 and this has been confirmed in children.13

FIGURE 54-1. Two-dimensional echocardiogram from a child with tricuspid valve vegetation (outlined). LA, left atrium; LV, left ventricle; MV, mitral valve; RA, right atrium; RV, right ventricle. For

most

infants

and

children,

unlike

adults,

transthoracic

echocardiography is usually sufficient to establish a diagnosis. In certain circumstances, however, transesophageal imaging is required, particularly in children with previous cardiothoracic surgical procedures, pulmonary problems that confound imaging windows, or substantial obesity. A transesophageal study may be superior, however, to detect paravalvar leakage or dehiscence, aortic root abscess development, and prosthetic valve endocarditis.14 Serial echocardiographic Doppler studies are also useful to gauge the response to therapy and assess for potential complications. For example, vegetation size and mobility may be an indicator of risk for embolization.14,15 Importantly, in a patient undergoing treatment for infective endocarditis, the diagnosis of myocardial abscess causing a new arrhythmia or progressive aortic root enlargement could suggest the need for a shift in treatment, including surgical intervention.

LABORATORY STUDIES Large-volume blood cultures are mandatory in all patients with suspected infective endocarditis. This includes any patient with a history of fever without an obvious explanation who has known heart disease or who has had endocarditis previously. Multiple cultures (three to five) are best, with 1- to 3-mL/culture in infants and 5- to 7-mL/culture in older children usually being sufficient. In children who have been on antibiotics, greater numbers of cultures over an extended period may be required. It is useful to notify the laboratory that infective endocarditis is suspected so that prolonged (>2 weeks) incubation of culture specimens will be done. Gram-negative rods (the HACEK [Haemophilus species, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella species, and Kingella kingae] organisms), for example, are typically slowly growing organisms. In organisms successfully cultured, testing for antibiotic susceptibility is extremely important for clinical management. Measurement of the minimum inhibitory concentration of the particular antibiotic chosen is the standard guide to therapeutic efficacy. While the minimum bactericidal concentration is not usually employed, it may be of use in particular situations, based on the advice of an infectious disease specialist. Use of antibiotics may be delayed in selected patients who are not acutely ill, in order to obtain additional blood cultures if the initial set remain negative after preliminary incubation.2

Other laboratory markers of infection and inflammation can be useful, serially, as guides to therapy. Acute-phase reactants (erythrocyte sedimentation rate and C-reactive protein) are usually elevated, and hypergammaglobulinemia may be noted. Rheumatoid factor may initially be elevated and can be followed as confirmation of response to therapy. Although the presence of leukocytosis (white blood cell count >15,000) is variable, increased segmented neutrophils or other immature forms (bands) are typical. Thrombocytopenia and mild anemia can be present. Abnormal liver function tests are less common without heart failure or hepatic emboli, but abnormalities in urinary sediment are frequently present. Hematuria may represent either an embolic process or glomerular nephritis. Signs of an immune complex nephritis can include proteinuria, red cell casts, and abnormal renal function measures, including creatinine and blood urea nitrogen. Low serum complement levels support the diagnosis of glomerular nephritis, but this is not definitive.

TREATMENT ANTIBIOTIC REGIMENS Owing to the tenacious nature of these infections, a prolonged course of parenteral antibiotics is required. Several factors contribute to the difficulty of eradicating the source of the infection. As described earlier, the microbial organisms are layered within elements of the vegetation, which sequesters them from the immune system and antibiotic exposure. Organisms with limited antibiotic susceptibilities may also require prolonged treatment for eradication. Tables 54-4 to 54-6 outline antibiotic regimens for childhood infective endocarditis in a variety of settings.2,16 TABLE 54-4

Treatment Regimens for Native Valve Infective Endocarditis Caused by Viridans Streptococci, Streptococcus bovis, or Enterococci Dosage (per

Organism Penicillinsusceptible streptococci (MIC ≤0.1 μg/mL)

Antimicrobial kg/24 Agent h)*

Frequency of Duration Administration (wk)

Penicillin G†

200,000 U IV

q4–6h

4

Ceftriaxone

100 mg IV

q24h

4

Penicillin G†

200,000 U IV

q4–6h

2

100 mg IV

q24h

2

Gentamicin

3 mg IM or IV

q8h‡

2

Penicillin G†

300,000 U IV

q4–6h

4

100 mg IV

q24h

4

3 mg IM or IV

q8h‡

2

300,000 U IV

q4–6h

4–6¶¶

or

or Ceftriaxone plus

Streptococci relatively resistant to penicillin (MIC>0.1– 0.5 μg/mL)

or Ceftriaxone plus Gentamicin

Enterococci,§ Penicillin G† nutritionally variant

viridans streptococci, or high-level penicillinresistant streptococci (MIC >0.5 μg/mL)

plus Gentamicin

3 mg IM or IV

q8h¶

4–6¶¶

Source: Reproduced with permission from Ferrieri P, Gewitz MH, Gerber MA, et al. Unique features of infective endocarditis in childhood. Circulation. 2002;105:2115-2127. © 2002 American Heart Association, Inc. *Dosages are for patients with normal renal and hepatic function. Maximum dosages per 24 h are as follows: penicillin, 18 million U; ampicillin, 12 g; ceftriaxone, 4 g; gentamicin, 240 mg. The 2-wk regimens are not recommended for patients with symptoms of infection lasting >3 mo, an extracardiac focus of infection, myocardial abscess, mycotic aneurysm, or infection with nutritionally variant viridans streptococci (Abiotrophia species). †Ampicillin

300 mg/kg per 24 h in 4–6 divided dosages may be used as alternative to

penicillin. ‡Studies

in adults suggest that the gentamicin dosage may be administered in single daily dose. If gentamicin is administered in 3 equally divided doses over 24 h, adjust the dosage to achieve peak and trough concentrations in serum of ≈3 and 1 g)

Source: Adapted with permission from Gewitz MH, Taubert KA. Infective endocarditis. In: Moller JH, Hoffman JIE, eds. Pediatric Cardiovascular Medicine. 2nd ed. Chichester, UK: Wiley-Blackwell; 2012. Copyright © 2012 Blackwell Publishing Ltd. *Single dose 30–60 min before procedure.

KEY POINTS The incidence of endocarditis is steady or increasing owing to increases in populations susceptible to the disease. Gram-positive bacteria represent the largest subgroup of causative organisms in infective endocarditis, although infections with gram-negative bacteria and fungi occur, especially in specific subgroups. Blood cultures and echocardiography with Doppler color flow mapping are the standard diagnostic modalities in patients with

suspected infective endocarditis; echocardiography is also used to assess the effectiveness of therapy. Complications of infective endocarditis can be severe and include mechanical obstruction by vegetations or emboli, cardiac dysfunction (including arrhythmias and heart block), and systemic infection.

ACKNOWLEDGMENTS The author appreciates the assistance of Ms. Patty Libby in the preparation of this chapter.

SUGGESTED READINGS Baddour LM, Wilson WR, Bayer AS, et al. Infective endocarditis: diagnosis, antimicrobial therapy, and management of complications. Circulation. 2005;111:e394-e434. Day MD, Gauvreau K, Shulman S, et al. Characteristics of children hospitalized with infective endocarditis. Circulation. 2009;119:865-887. Gewitz MH. Infective endocarditis. In: Moller M, Hoffman JH, JIE, eds. Pediatric Cardiovascular Medicine. 3nd ed. Chichester, UK: WileyBlackwell; 2011. Wilson W, Taubert KA, Gewitz MH, Lockhart P, et al. Prevention of infective endocarditis. Guidelines the American Heart Association. Circulation. 2007:116:1736-1754.

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3. Morris CD, Reller MD, Menashe VD. Thirty year incidence of infective endocarditis after surgery for congenital heart defect. JAMA. 1998;279:599-603. 4. Stockheim JA, Chadwick EG, Kessler S, et al. Are the Duke criteria superior to Beth Israel criteria for diagnosis of infective endocarditis in children? Clin Infect Dis. 1998;27:1451-1456. 5. Tunkel AR, Scheld WM. Experimental models of endocarditis. In: Kaye D, ed. Infective Endocarditis. 2nd ed. New York, NY: Raven Press; 1992:3756. 6. Saiman L, Prince A, Gersony WM. Pediatric infective endocarditis in the modern era. J Pediatr. 1993;122:847-853. 7. Baddour LM, Bettmann MA, Bolger AF, et al. Nonvalvular cardiovascular device-related infections. Circulation. 2003;108:20152031. 8. Opie GF, Fraser SH, Drew JH, Drew S. Bacterial endocarditis in neonatal intensive care. J Paedtr Child Health. 1999;35:545-548. 9. Durack OT, Lukes AS, Bright DK. New criteria for diagnosis of infective endocarditis: utilization of specific echocardiographic findings. Am J Med. 1994;96:200-209. 10. Li JS, Sexton OJ, Mick N, et al. Proposed modifications to the Duke criteria for the diagnosis of infective endocarditis. Clin Infect Dis. 2000;30:633-638. 11. Del Pont JM, DeCicco LT, Vartalis C, et al. Infective endocarditis in children: clinical analysis and evaluation of two diagnostic criteria. Pediatr Infect Dis J. 1995;14:1079-1086. 12. Fowler VG, Li JS, Corey GR, et al. Role of echocardiography in evaluation of patients with Staphylococcus aureus bacteremia. J Am Coll Cardiol. 1997;30:1072-1078. 13. Valente AM, Jain R, Scheurer M, et al. Frequency of infective endocarditis among infants and children with Staphylococcus aureus bacteremia. Pediatrics. 2005;115:e15-e19. 14. Barbour SI, Louoie ED, O’Keefe IP. Penetration of the atrioventricular septum by spread of infection from aortic valve endocarditis: early diagnosis by transesophageal echocardiography. Am Heart J.

1996;132:1287-1289. 15. DiSalvo G, Habib G, Pergola V, et al. Echocardiography predicts embolic events in infective endocarditis. J Am Coll Cardiol. 2001;37:1069-1076. 16. Ferrieri P, Gewitz MH, Gerber MA, et al. Unique features of infective endocarditis in childhood. Circulation. 2002;105:2115-2127. 17. Berkowitz FE. Infective endocarditis. In: Nichols DG, Cameron DE, Greeley WJ, et al., eds. Critical Heart Disease in Infants and Children. St Louis, MO: Mosby-Year Book; 1995;961-986. 18. Cobell CH, Lerakis S, Selton-Sutty C, et al. Cardiovascular risk factors and outcomes in patients with definite endocarditis: findings from 1024 patients in the ICE prospective cohort study [abstract]. Circulation. 2003;108:IV-432. 19. Stinson EB. Surgical treatment of infective endocarditis. Prog Cardiovasc Dis. 1979;22:145-168. 20. Shamszad P, Kahn MS, Rossano JW, et al. Early surgical therapy of infective endocarditis in children. J Thorac Cardiovasc Surg. 2013;146:1-6. 21. Strom BL, Abrutyn E, Berlin JA, et al. Risk factors for infective endocarditis: oral hygiene and nondental exposures. Circulation. 2000;102:2842-2848. 22. Wilson W, Taubert KA, Gewitz MH. Prevention of infective endocarditis. Circulation. 2007;116:1736-1754. 23. Gould FK, Elliott TSJ, Foweraker J, et al. Guidelines for the prevention of endocarditis: report of the Working Party of the British Society for Antimicrobial Chemotherapy. J Antimicrob Chemother. 2006;10:10931121.

CHAPTER

55

Myocarditis and Cardiomyopathy Robert N. Vincent, Margaret J. Strieper, and Kenneth J. Dooley

BACKGROUND Myocarditis is a pathologic process characterized by inflammation of the myocardium leading to cellular necrosis and myocardial dysfunction. Although often thought of as a viral or post-viral process, causes of myocarditis are numerous and include infectious (viral, bacterial, fungi, yeast, rickettsial, protozoal, and parasitic) as well as non-infectious (toxins, drugs, autoimmune diseases, and Kawasaki disease) etiologies. Cardiomyopathy is a general term referring to diseases of the myocardium. Clinically and pathologically, cardiomyopathy can be divided into dilated, hypertrophic, and restrictive types. As myocarditis is a precursor to and one of the causes of dilated cardiomyopathy, the two will be considered together.

DILATED CARDIOMYOPATHY CLINICAL PRESENTATION The presentation of a child with dilated cardiomyopathy (DCM) depends on the age of the patient and the acuteness of the illness. Aggressive acute viral myocarditis may present as cardiovascular collapse and cardiac arrest over a very short period of time. Other causes of DCM in general, and occasionally myocarditis as well, may have a more progressive subacute to chronic course. Unless the disease process progresses to the point of causing significant myocyte necrosis and myocardial dysfunction, clinical symptoms may be absent. A slow decrease in ventricular function over many months or years may go unnoticed, whereas a rapid drop in ventricular function over several

days may result in severe heart failure and cardiovascular collapse, as compensatory mechanisms have not set in. However, the chronic stable patient has little cardiac reserve, and therefore an unrelated illness such as an upper respiratory infection may result in cardiac decompensation. The history of an antecedent viral infection in the child presenting with a new-onset DCM may not herald the initiation of cardiac disease, as it is possible that the viral illness merely unmasked the chronic compensated form of DCM. The usual presentation of DCM is that of congestive heart failure and low cardiac output. Signs and symptoms of heart failure and low cardiac output in newborns and infants include fussiness, poor appetite, poor feeding, fever, listlessness, diaphoresis, and respiratory distress. Physical examination generally demonstrates a patient with tachypnea, tachycardia, cardiomegaly, and hepatomegaly with pallor or an ashen appearance. The older child and adolescent will generally have a history of a viral illness 7 to 14 days prior to presentation with myocarditis. Findings of poor appetite and abdominal pain due to hepatic congestion, lethargy, exercise intolerance, malaise, and fever may also be present. Findings of heart failure including jugular venous distention and rales as well as low cardiac output may be present on physical examination in an older child, whereas the findings of rales and jugular venous distention are often absent in the newborn and infant.

DIFFERENTIAL DIAGNOSIS The differential diagnosis of DCM based on age is seen in Table 55-1. All attempts should be made to diagnose the treatable causes of cardiomyopathy such as structural heart disease, underlying arrhythmias, and vitamin deficiencies. Although structural heart defects and arrhythmias are not truly diseases of the myocardium, they are listed, as they may present similar to a DCM and must be considered in the evaluation of these children. TABLE 55-1

Differental Diagnosis of Dilated Cardiomyopathy Based on Age

Fetal AV malformation (vein of Galen) Myocarditis

Severe outflow obstruction Severe valve insufficiency Tachyarrhythmias Severe anemia (immune hydrops) Bradyarrhythmias (congenital complete heart block) Newborn–1 year old Myocarditis Endocardial fibroelastosis (EFE) Barth syndrome Carnitine deficiency Selenium deficiency Anomalous left coronary artery from pulmonary artery (ALCA) Kawasaki disease (KD) Critical aortic stenosis (AS) Supraventricular tachycardias (SVT) Arterio-venous malformation (especially vein of Galen) Calcium deficiency Hypoglycemia Left ventricular non-compaction Mitochondrial cardiomyopathy Nemaline myopathy Minicore-multicore Myopathy Myotubular myopathy 1–10 years old Familial dilated cardiomyopathy (FDCM) Barth syndrome Myocarditis Arrhythmogenic right ventricular dysplasia Endocardial fibroelastosis (EFE) Carnitine deficiency

Selenium deficiency Anomalous left coronary artery from pulmonary artery (ALCA) Kawasaki disease (KD) Supraventricular tachycardias (SVT) Toxic (adriamycin) β-ketothiolase deficiency Ipecac toxicity Systemic lupus erythematosus Polyarteritis nodosa Hemolytic–uremic syndrome Mitochondrial cardiomyopathy Nemaline myopathy Minicore-multicore myopathy Myotubular myopathy >10 years of age Familial dilated cardiomyopathy X-linked dilated cardiomyopathy (XLCM) Myocarditis Supraventricular tachycardia (SVT) Congenital heart disease (Ebstein’s, etc.) (CHD) Postoperative congenital heart disease (P/O CHD) Mitochondrial cardiomyopathy Chagas disease Arrhythmogenic right ventricular dysplasia (ARVD) Eosinophilic cardiomyopathy Adriamycin toxicity Pheochromocytoma Duchenne muscular dystrophy/Becker muscular dystrophy (DMB/BMD) Emery-Dreifuss muscular dystrophy (EDMD) Hemochromatosis

Limb-girdle muscular dystrophy Myotonic dystrophy Peripartum cardiomyopathy Alcoholic cardiomyopathy

DIAGNOSTIC EVALUATION Table 55-2 is a list of studies that may be performed in the evaluation of a patient with newly diagnosed DCM. The extent of evaluation will depend on initial findings of some of the more specific tests and history (e.g. a history of adriamycin administration or cocaine abuse likely point to the etiology). When identifiable causes are excluded or point to a familial or inherited disorder, family screening becomes important. TABLE 55-2

Evaluation of Newly Diagnosed Dilated Cardiomyopathy

• Chest x-ray • ECG • Echocardiogram (including relatives and tissue Doppler imaging) • Urine Organic acids including 3-methylglutaconic acid UA Amino acids • Blood Lactic acid Pyruvate SMA-7 Glucose Ca2+ Mg2+ Selenium CBC with diff

CPK (MM, MB, total) Troponin T or I Liver function studies Carnitine Acylcarnitine profile Cholesterol Thyroid function studies Plasma for amino acids ESR Viral serologies including adenovirus Cytogenetics • Skeletal muscle biopsy Histology EM Mitochondrial respiratory chain analysis, acyl CoA DH analysis • Endomyocardial biopsy (with hemodynamic catheterization and angiographic evaluation of structural lesions) Histology EM PCR for vital genome Mitochondrial respiratory chain analysis • Blood for cell lines Genetics consultation to include comprehensive metabolic/enzymatic evaluation Strong consideration of drawing all transplant labs particularly before administration of IV gamma globulin Chest X-Ray A chest x-ray is often the first test done and is often performed for reasons other than suspicion of cardiomyopathy. Radiography demonstrates cardiomegaly and increased pulmonary venous markings with or without pulmonary edema or pleural effusion. The chest x-ray is often ordered because the child presents with some degree of respiratory distress.

Electrocardiogram The electrocardiogram (ECG) is usually nonspecific but may show decreased QRS voltages and low voltage or inverted T waves in myocarditis. Q waves in lead I and aVL due to myocardial infarct are very suspicious of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Ectopic beats should raise the suspicion of poorly controlled supraventricular tachycardia or ventricular arrhythmias, although in severe cardiac dysfunction, ventricular arrhythmias may occur secondary to the myocardial disease. Echocardiogram The echocardiogram establishes the diagnosis of a dilated poorly contracting left ventricle (Figure 55-1A, 1B), while at the same time should exclude all structural causes of left ventricular (LV) dysfunction (LV outflow tract obstruction and anomalous origin of the left coronary artery). Structural and treatable anomalies that cannot be excluded with certainty, such as ALCAPA, require further diagnostic testing. Although ventricular dysfunction is usually in the form of global hypokinesis, segmental wall motion abnormalities may be present. The evaluation should also search for the presence of a thrombus in the left atrium or ventricle (Figure 55-1C).

FIGURE 55-1. A. Two-dimensional echocardiogram and color Doppler of a patient with DCM, demonstrating a dilated LV cavity and

mitral regurgitation. B. M-mode echocardiogram demonstrating a dilated LV cavity and poor systolic function (shortening fraction 15%, ejection fraction 28%). C. Two-dimensional echocardiogram demonstrating a large thrombus in the apex of the left ventricle. Pericardial and pleural effusions are not uncommon, and Doppler cardiography will often demonstrate mitral as well as tricuspid insufficiency. Right ventricular (RV) size and function may be spared early on but the RV may also dilate and demonstrate poor contractility as well. There may be secondary pulmonary hypertension arising from LV dysfunction. Doppler interrogation of the tricuspid regurgitation jet