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Pediatric Hospital Medicine A High-Value Approach

Pediatric Hospital Medicine A High-Value Approach EDITORS Moises Auron, MD, FAAP, FACP, SFHM Professor of Medicine and Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Medicine & Pediatrics Pediatric Hospital Medicine Colleen Schelzig, MD, FAAP Division Chair, Pediatric Hospital Medicine Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatrics Pediatric Hospital Medicine

Sangeeta Krishna, MD, FAAP Fellowship Director, Pediatric Hospital Medicine Associate Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatrics Pediatric Hospital Medicine Anika Kumar, MD, FAAP, FHM Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatrics Pediatric Hospital Medicine

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This book is dedicated to our families, devoted colleagues and hospitalized children across the world

Foreword

T he journey to high-value consistent health care has been more than a half-century pursuit, but thanks to the Pediatric Hospital Medicine experts of Cleveland Clinic Children’s, this text enables all providers of inpatient pediatric care to provide the highest-value, state-of-the-art, care today. The journey started in 1970 when the Institute of Medicine (IOM) was created to provide evidence-based research and recommenda tions for public health and science policy. Among the initiatives the Institute has led, quality has been one of the most transformative for health care organizations and practicing providers. In 1998, the IOM categorized health care quality issues as underuse, misuse, or overuse, which started a dialogue about health care practice. In 2000, the IOM’s Committee on Quality of Health Care in America published “To Err Is Human: Building a Safer Health System,” which transformed thinking about health care errors, the culture of safety, and the role of processes in enabling individuals to do the right thing. Utilizing the methodol ogy for improvement developed by W. Edwards Deming (1900-1993), the Institute for Healthcare Improvement built on this published work, and the enhanced awareness regarding medical errors that it created, to advance the culture of health care improvement and evolution to high reliability (error-free performance and safety, in all activities, all of the time) in health care. Deming’s principles of management employ Plan-Do-Study-Act cycles to make small intentional changes, that are measurable, to move to a predetermined goal. In this journey of health care improvement, the need to standardize became apparent, and we now know that organizations that effectively manage in this way dem onstrate increases in the quality of care while simultaneously reducing cost, what has now been termed “value.” In 2012, the American Board of Internal Medicine Foundation com menced the Choosing Wisely ® campaign, which sought to decrease medical overuse via yearly recommendations to help standardize prac tice, thereby improving the “value” of care. Initially involving nine

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Foreword

national specialty societies with recommendations about 45 tests or treatments that were used in their fields without supporting evidence, the campaign has now highlighted the recommendations from more than 80 specialty societies. Despite increased awareness and targeted campaigns, medical care overuse continues to be a problem in the United States, increasing the risk of harm and cost of care, without the benefit of increased quality. As providers of frontline medical care in the inpatient setting, pediatric hospitalists are well positioned to lead change toward high-value pediat ric care, thereby decreasing medical waste, controlling costs, and, most importantly, providing the best evidence-based patient-centered care. While individual recommendations continue to be made for Choos ing Wisely and toward improving the value of care, this text provides the first compendium reference for clinicians worldwide who aspire to provide high-value, evidence-based, pediatric inpatient care. Orga nized by current and prior members of the world’s leading Pediatric Hospital Medicine team at Cleveland Clinic Children’s, the 55 plus ex perts in pediatric hospital medicine have developed this reference with the intent of supporting evidence-based, standardized, high-value care for the improved welfare of our patients, community, and health care system. With gratitude and admiration,

Karen F. Murray, MD Chair, Medical Subspecialties Vice Chair of Research Cleveland Clinic Children’s

■ REFERENCES Chassin MR, Galvin RW. The urgent need to improve health care quality. Institute of Medicine National Roundtable on Health Care Quality. JAMA . 1998;280(11):1000-1005. Rozich JD, Howard RJ, Justeson JM, Macken PD, Lindsay ME, Resar RK. Standardization as a mechanism to improve safety in health care. Jt Comm J Qual Saf . 2004;30(1):5-14. Tchou MJ, Schondelmeyer AC, Alvarez F, et al. Choosing wisely in pediat ric hospital medicine: 5 new recommendations to improve value. Hosp Pediatr. 2021;11(11):1179-1190.

Preface

T he extraordinary duty and privilege to care for hospitalized chil dren confers a tremendous responsibility. That responsibility includes providing evidence-based medical management and assurance that our patients receive the highest value health care. What does “high-value care” mean? To provide high-value care means to provide “the best care possible, efficiently using resources and providing optimal results for each patient.” 1 A reflection on the transformation of health care over the last 50 years inspired us to pub lish this book. As health care expenditure in the United States has significantly risen year over year, our diagnostic and therapeutic interventions must have the highest impact on our patients’ outcomes. This requires care ful consideration of procedures, treatment, and practice behaviors not proven to meaningfully affect patient outcomes. The conversations surrounding high-value intervention strategies with a focus on enhanced patient safety have increased over the past 23 years since the seminal “To Err Is Human” publication by the Insti tute of Medicine. 2 Many researchers have studied the value of routine patient interventions. Their studies have progressively found ways to deliver care in an efficient and effective way without pursuing unneces sary diagnostic tests or treatments. The field of Pediatric Hospital Medicine is privileged to have excep tional innovators in approaching patient care. These approaches have normalized the adoption of high-value care and avoidance of unneces sary diagnostic tests and treatments, both of which are strongly sup ported by over 20 years of robust research-based evidence. The authors and editors of this book are empowered stewards of high-value care. We have instilled high-value care into the curricula of our Pediatrics residency and Pediatric Hospital Medicine fellow ship programs. During patient rounds at our Children’s Hospital, the value of diagnostic tests and therapeutic interventions is deliberately and consistently discussed. The focus of these conversations is to

Preface

enhance patient outcomes without exposing patients to unnecessary interventions. The impact of high-value care reaches multiple care domains— patient safety, health care expenditure, patient satisfaction, patient out comes, and the progressive development of physicians’ awareness of best practices. This book offers a high-value perspective to common medical con ditions routinely seen in Pediatric Hospital Medicine, focusing primar ily on diagnosis and treatment. We hope readers find this approach useful, stimulating their desire to implement changes to their practices or reinforcing their current high-value care practice. Well-established, evidence-based high-value care can be replicated at institutions across the world. Changing behaviors may take significant time, but the most important date is the first day when change is considered and imple mentation begins. We wish our readers continued success in their practice. More im portantly, we wish all the children of the world healthy, happy, and suc cessful lives. If, for any reason, they are hospitalized, we hope this book will imparts their medical care team with knowledge and resources to provide effective, efficient, high-quality, and high-value patient care. With deepest gratitude, appreciation, and respect to all our col leagues and their patients.

Moises Auron, MD, FAAP, FACP, SFHM Sangeeta Krishna, MD, FAAP Anika Kumar, MD, FAAP, FHM Colleen Schelzig, MD, FAAP

■ REFERENCES 1. Razmaria AA. JAMA Patient Page. High-value care. JAMA . 2015;314(22):2462. 2. Institute of Medicine (US) Committee on Quality of Health Care in America, Kohn LT, Corrigan JM, Donaldson MS, eds. To Err Is Human: Building a Safer Health System . National Academies Press (US); 2000.

Contributors

Oloruntosin Adeyanju, MD, FAAP Assistant Professor of Pediatrics Pediatric Hospital Medicine Washington University School of Medicine St Louis, Missouri

Julie Cernanec, MD, FAAP Clinical Associate Professor of Pediatrics

Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Rena Chandra, MD, FAAP Clinical Assistant Professor of Pediatrics

Moises Auron, MD, FAAP, FACP, SFHM Professor of Medicine and Pediatrics

Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Divisions of Hospital Medicine and Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Jeremy Baker, DO, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Kara Ditlevson-Smith, DO, FAAP Clinical Instructor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Shanna Botos, DNP Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Dana M. Foradori, MD, MEd, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Associate Program Director, Pediatric Hospital Medicine Fellowship Cleveland Clinic Children’s Cleveland, Ohio

Catherine Brown, MD, FAAP Pediatric Hospital Medicine Fellow Cleveland Clinic Children’s Cleveland, Ohio

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Contributors

Amrit Gill, MD, FAAP Clinical Associate Professor of Pediatrics

Andrew Kern-Goldberger, MD, MSCE, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Sangeeta Krishna, MD, FAAP Fellowship Director, Pediatric Hospital Medicine Associate Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Marielle Kulling, DO, MPH, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Gloria Gordon-Ocejo, DNP Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Pediatric Hospital Medicine Akron Children’s Hospital Akron, Ohio Christine Halishak, MSN Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Tatiana Gurevich-Panigrahi, MD, FAAP, IBCLC Cory Henson, MD, FAAP Assistant Professor of Pediatrics Program Director, Pediatric Hospital Medicine Fellowship Baylor College of Medicine– CHRISTUS Children’s San Antonio, Texas Jane Im, MD, FAAP Assistant Professor of Pediatrics Pediatric Hospital Medicine University of Connecticut School of Medicine Frank H. Netter School of Medicine at Quinnipiac University

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Anika Kumar, MD, FAAP, FHM Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Suet Kam Lam, MD, MPH, MS, IBCLC, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Connecticut Children’s Hartford, Connecticut

Jennifer Kaczmarek, MD, MSc, FAAP Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

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Contributors

Stephanie Moss, MD, FAAP Divisions of Hospital Medicine and Pediatric Hospital Medicine Cleveland Clinic and Cleveland Clinic Children’s Cleveland, Ohio Chionye Ossai, MD, MS, FAAP Assistant Professor of Pediatrics Emory University School of Medicine Department of Pediatrics Atlanta, Georgia Rita M. Pappas, MD, FAAP, FHM Clinical Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Interim Chief, Interim Chair, Integrated Hospital Care, Children’s Institute Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Katie Pestak, DO, MEd, FAAP Clinical Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Michael Cory Lever, MD, FAAP Clinical Assistant Professor of Pediatrics Pediatric Hospital Medicine University of South Carolina School of Medicine - Columbia Columbia, South Carolina

Jodi Lohrey, MSN Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Michelle Marks, DO, FAAP, SFHM Clinical Associate Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatric Hospital Medicine Cleveland Clinic Children’s Hospital for Rehabilitation Cleveland, Ohio Raed Bou Matar, MD, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatric Nephrology Cleveland Clinic Children’s Cleveland, Ohio Marc R. Miller, MD, FAAP Clinical Associate Pediatric Hospital Medicine University of Chicago Medical Center Chicago, Illinois Melissa Iris Morse, MD, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Prabi Rajbhandari, MD, FAAP Associate Professor of Pediatrics Northeast Ohio Medical University Program Director, Pediatric Hospital Medicine Fellowship Akron Children’s Hospital Akron, Ohio

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

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Contributors

Reem Sarkis, MD, FAAP Pediatric Critical Care Physician Pediatric Emergency Medicine Department Akron Children’s Hospital Akron, Ohio Jennifer Scavone, MSN Pediatric Hospital Medicine Cleveland Clinic Children’s Hospital for Rehabilitation Cleveland, Ohio Colleen Schelzig, MD, FAAP Division Chair, Pediatric Hospital Medicine Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Lora G. Sowunmi, MD, FAAP, FHM Assistant Professor of Medicine and Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Divisions of Hospital Medicine and Pediatric Hospital Medicine Cleveland Clinic and Cleveland Clinic Children’s Cleveland, Ohio Rachana Sripathi, MD, FAAP Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio Corrie Stofcho, MD, FAAP Clinical Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Heidi Szugye, DO, IBCLC, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Primary Care Pediatrics Cleveland Clinic Children’s Cleveland, Ohio Christine Traul, MD, FAAP Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Director, Pediatric Palliative Care Cleveland Clinic Children’s Cleveland, Ohio

Megh M. Trivedi, MD, FAAP PGY-3 Neurosurgery

Cleveland Clinic Cleveland, Ohio

Larissa Wertalik, DO, FAAP Pediatrician Chesapeake, Virginia Martha Williams, MSN Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Arnaldo Zayas-Santiago, MD, FAAP

Assistant Professor of Pediatrics Cleveland Clinic Lerner College of Medicine of Case Western Reserve University

Pediatric Hospital Medicine Cleveland Clinic Children’s Cleveland, Ohio

Table of Contents

Foreword . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .xi PART I. Neurology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 Chapter 1 Headaches . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 Corrie Stofcho and Rachana Sripathi Chapter 2 Seizure............................... 9 Lora G Sowunmi and Christine Traul Chapter 3 Botulism . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 Christine Traul and Larissa Wertalik Chapter 4 Guillain-Barré Syndrome . . . . . . . . . . . . . . . 27 Christine Traul and Larissa Wertalik Chapter 5 Acute Disseminated Encephalomyelitis . . . 31 Christine Traul and Larissa Wertalik PART II. Ophthalmology . . . . . . . . . . . . . . . . . . . . . . . . . . 37 Chapter 6 Red Swollen Eyes . . . . . . . . . . . . . . . . . . . . . 39 Katie Pestak and Shanna Botos PART III. Ear, Nose, Throat . . . . . . . . . . . . . . . . . . . . . . . . . 45 Chapter 7 Pharyngitis . . . . . . . . . . . . . . . . . . . . . . . . . . 47 Melissa Iris Morse and Corrie Stofcho Chapter 8 Peritonsillar Abscess . . . . . . . . . . . . . . . . . . . 53 Melissa Iris Morse and Corrie Stofcho

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Table of Contents

Chapter 9 Retropharyngeal Abscess . . . . . . . . . . . . . . . 57 Melissa Iris Morse and Corrie Stofcho Chapter 10 Otitis Media . . . . . . . . . . . . . . . . . . . . . . . . . 61 Megh M Trivedi and Jeremy Baker Chapter 11 Mastoiditis........................... 65 Megh M Trivedi and Jeremy Baker PART IV. Cardiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69 Chapter 12 Syncope . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 71 Anika Kumar and Shanna Botos Chapter 13 Kawasaki Disease . . . . . . . . . . . . . . . . . . . . . 77 Arnaldo Zayas-Santiago and Catherine Brown Chapter 14 Multisystem Inflammatory Syndrome in Children . . . . . . . . . . . . . . . . . . . . . . . . . . . . 83 Dana M Foradori and Catherine Brown PART V. Pulmonology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 Chapter 15 Bronchiolitis . . . . . . . . . . . . . . . . . . . . . . . . . 91 Moises Auron Chapter 16 Acute Asthma . . . . . . . . . . . . . . . . . . . . . . . . 95 Michael Cory Lever and Lora G Sowunmi Chapter 17 Pneumonia . . . . . . . . . . . . . . . . . . . . . . . . . 103 Dana M Foradori and Martha Williams Chapter 18 Brief Resolved Unexplained Event . . . . . . . 111 Dana M Foradori and Martha Williams Chapter 19 Croup.............................. 117 Rita M Pappas PART VI. Allergy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123 Chapter 20 Anaphylaxis......................... 125 Kara Ditlevson-Smith and Rita M Pappas

Table of Contents xvii

PART VII. Gastroenterology . . . . . . . . . . . . . . . . . . . . . . . 131 Chapter 21 Abdominal Pain . . . . . . . . . . . . . . . . . . . . . 133 Andrew Kern-Goldberger Chapter 22 Constipation . . . . . . . . . . . . . . . . . . . . . . . . 141 Andrew Kern-Goldberger Chapter 23 Appendicitis . . . . . . . . . . . . . . . . . . . . . . . . 145 Julie Cernanec and Amrit Gill Chapter 24 Pancreatitis . . . . . . . . . . . . . . . . . . . . . . . . . 149 Amrit Gill and Julie Cernanec Chapter 25 Intussusception . . . . . . . . . . . . . . . . . . . . . . 153 Cory Henson Chapter 26 Gastroesophageal Reflux Disease . . . . . . . . 157 Cory Henson Chapter 27 Pyloric Stenosis . . . . . . . . . . . . . . . . . . . . . . 163 Cory Henson Chapter 28 Eating Disorders . . . . . . . . . . . . . . . . . . . . . 167 Chionye Ossai Chapter 29 Refeeding Syndrome . . . . . . . . . . . . . . . . . 173 Chionye Ossai Chapter 30 Electrolyte and Fluid Management . . . . . . 177 Michelle Marks and Christine Halishak Chapter 31 Gastroenteritis . . . . . . . . . . . . . . . . . . . . . . 183 Michelle Marks and Christine Halishak PART VIII. Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187 Chapter 32 Skin and Soft Tissue Infections . . . . . . . . . . 189 Katie Pestak and Jodi Lohrey Chapter 33 Bacteremia and Central Line–Associated Bloodstream Infections . . . . . . . . . . . . . . . 193 Christine Halishak and Marc R Miller Chapter 34 Systemic Inflammatory Response

Syndrome and Sepsis . . . . . . . . . . . . . . . . . 199 Rena Chandra, Marc R Miller, and Christine Halishak

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Chapter 35 Infectious Encephalitis/Meningitis . . . . . . . 205 Marc R Miller and Christine Halishak Chapter 36 Febrile Infant 0-60 Days of Life . . . . . . . . . 211 Prabi Rajbhandari Chapter 37 Pyelonephritis . . . . . . . . . . . . . . . . . . . . . . . 215 Rachana Sripathi and Raed Bou Matar PART IX. Nephrology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 219 Chapter 38 Acute Kidney Injury . . . . . . . . . . . . . . . . . . 221 Rachana Sripathi and Raed Bou Matar Chapter 39 Hypertension........................ 227 Rachana Sripathi and Raed Bou Matar Chapter 40 Hemolytic Uremic Syndrome . . . . . . . . . . . 233 Rachana Sripathi and Raed Bou Matar PART X. Endocrinology . . . . . . . . . . . . . . . . . . . . . . . . . . 239 Chapter 41 Diabetes Mellitus . . . . . . . . . . . . . . . . . . . . 241 Reem Sarkis and Gloria Gordon-Ocejo Chapter 42 Syndrome of Inappropriate Antidiuretic Hormone Secretion . . . . . . . . . . . . . . . . . . 249 Reem Sarkis and Gloria Gordon-Ocejo PART XI. Musculoskeletal . . . . . . . . . . . . . . . . . . . . . . . . 253 Chapter 43 Bone and Joint Infections . . . . . . . . . . . . . 255 Jennifer Kaczmarek and Jennifer Scavone Chapter 44 Transient Synovitis . . . . . . . . . . . . . . . . . . . 265 Jennifer Kaczmarek and Jennifer Scavone Chapter 45 Non-accidental Injury . . . . . . . . . . . . . . . . . 269 Jennifer Kaczmarek and Jennifer Scavone

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Table of Contents

PART XII. Hematology............................ 275 Chapter 46 Venous Thromboembolism Prophylaxis . . . . . . . . . . . . . . . . . . . . . . . . . 277 Stephanie Moss and Jennifer Scavone PART XIII. Gynecology . . . . . . . . . . . . . . . . . . . . . . . . . . . . 283 Chapter 47 Abnormal Uterine Bleeding . . . . . . . . . . . . 285 Stephanie Moss and Jennifer Scavone PART XIV. Toxicology/Psychiatry . . . . . . . . . . . . . . . . . . . . 291 Chapter 48 Medication Ingestion . . . . . . . . . . . . . . . . . 293 Marielle Kulling and Melissa Iris Morse Chapter 49 Safety Planning in Mental Health . . . . . . . 299 Marielle Kulling and Melissa Iris Morse Chapter 50 Somatic Symptom–Related Disorder . . . . . 307 Marielle Kulling and Melissa Iris Morse PART XV. Newborn Care . . . . . . . . . . . . . . . . . . . . . . . . . . 313 Chapter 51 Newborn Eye Anomalies . . . . . . . . . . . . . . 315 Jane Im, Oloruntosin Adeyanju, Katie Pestak, and Shanna Botos Chapter 52 Murmur/Critical Congenital Heart Disease . . 327 Jane Im and Oloruntosin Adeyanju Chapter 53 Neonatal Hearing Loss . . . . . . . . . . . . . . . . 331 Suet Kam Lam and Heidi Szugye Chapter 54 Breastfeeding . . . . . . . . . . . . . . . . . . . . . . . 335 Heidi Szugye and Suet Kam Lam Chapter 55 Neonatal Hypoglycemia . . . . . . . . . . . . . . . 341 Heidi Szugye and Suet Kam Lam

Table of Contents

Chapter 56 Neonatal Jaundice . . . . . . . . . . . . . . . . . . . 349 Suet Kam Lam and Heidi Szugye Chapter 57 Apnea . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355 Tatiana Gurevich-Panigrahi and Larissa Wertalik Chapter 58 Neonatal Abstinence Syndrome/ Neonatal Opioid Withdrawal Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . 359 Tatiana Gurevich-Panigrahi and Larissa Wertalik Chapter 59 HipDysplasia........................ 365 Tatiana Gurevich-Panigrahi and Larissa Wertalik Chapter 60 Neonatal Infections . . . . . . . . . . . . . . . . . . 371 Jeremy Baker and Kara Ditlevson-Smith

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387

2 CHAPTER

Seizure

Lora G. Sowunmi and Christine Traul

■ INTRODUCTION Seizures can be defined as surges of abnormal and excessive electrical activity in any part of the brain that causes clinical symptoms ranging from brief lapses of awareness or muscle twitches to prolonged convul sions. 1 The terms seizures and epilepsy are often used interchangeably, but have different meanings. Epilepsy is defined as two or more recur rent, unprovoked seizures. Provoked seizures may occur with medical conditions including injury, electrolyte derangement, fever, or with drawal from drugs or alcohol. While the medical condition may have provoked one or more seizures, it is likely reversible and thus is not epilepsy. 1 Epilepsy has a wide spectrum of clinical characteristics due to vary ing seizure types. A very specific classification system has been im plemented to streamline the clinical descriptions of a seizure. These include onset, level of awareness, and whether movement is noted with the seizure. Classifications of seizure types can be found in Table 2.1.

TABLE 2.1 CLASSIFICATION OF SEIZURE TYPES ONSET AWARENESS

ABNORMAL MOVEMENTS

Focal

Aware or impaired awareness May be present

Generalized Impaired awareness

May be present May be present

Unknown

Impaired awareness

Data from Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy: position paper of the ILAE Commission for Classification and Terminology. Epilepsia . 2017;58(4):522-530.

■ EPIDEMIOLOGY AND RISK FACTORS An underlying cause for seizures, and ultimately the diagnosis of epi lepsy, may be identified in almost two-thirds of pediatric patients. See Table 2.2 for the etiologies.

Part I • Neurology

TABLE 2.2 ETIOLOGY OF EPILEPSY IN CHILDREN Genetic

• Sodium, potassium, or calcium channel gene alterations or deletions

■ CLINICAL MANIFESTATIONS Clinical manifestations vary depending on their individual character istics. For some, an aura may precede the seizure, such as a sensation or feeling. 2 A generalized seizure may be followed by a postictal state: a period of time when the child is sleepy, confused, disoriented, or exhibits weakness in a part of the body. Lasting seconds to hours, the postictal state ends when the child returns to baseline. 3 ■ DIAGNOSTIC STUDIES A seizure is a clinical diagnosis based on medical history focusing on age, duration, triggers, and state of awareness during the event. Past medical history, family history, exposures, and psychiatric history are also pertinent. See Table 2.3 for additional diagnostic studies to con sider after an initial unprovoked seizure. ■ MANAGEMENT Table 2.4 lists the high-value approach for management of a child with an unprovoked first seizure and considerations if an infectious cause is suspected. • Chromosomal abnormalities (eg, Wolf-Hirschhorn 4p-syndrome, Angelman syndrome [del 15q11-13]) Metabolic • Inborn errors of metabolism (eg, urea cycle disorders, phenylketonuria) • Lysosomal storage disorders (eg, Tay-Sachs, Gaucher disease) Structural • Cortical malformations during development • Stroke or trauma Immune • Anti– N -methyl- d -aspartate (NMDA) receptor encephalitis • Paraneoplastic due to underlying cancer Infectious • Bacterial central nervous system infections ( Neisseria menin gitidis , Streptococcus pneumoniae , gram-negative bacteria) • Viruses: Zika, HIV, and CMV • Other infectious agents: tuberculosis, neurocysticercosis, malaria, syphilis HIV, Human Immunodeficiency Virus; CMV, Cytomegalovirus. Data from Fine A, Wirrell EC. Seizures in children. Pediatr Rev . 2020;41(7):321-347.

Chapter 2 • Seizure

TABLE 2.3 DIAGNOSTIC STUDIES PROCEDURE

TYPE OF TESTING PURPOSE OF TEST

CONSIDERATIONS

EEG

• 24 h • Sleep deprived • Intermittent photic stimulation

• Evaluate for epileptiform

• Normal EEG is not evidence for absence of seizures. • Skull defects

or background abnormalities.

can alter the patterns on EEG.

Neuroimaging Head CT

• Head CT may be performed in the ER setting; it may be helpful in cases of acute intracra nial processes. • Brain MRI may be helpful for some seizure types and in neonatal seizures. • Assess for acute and fixable abnormalities.

• Radiation

exposure and low yield

Brain MRI

• Depending on seizure type, low yield • Sedation may be indicated.

Laboratory testing

Glucose Electrolytes

• Often low yield

Metabolic testing (blood/urine amino acids, blood/urine organic acids)

• Metabolic

testing for those that present with extreme symptoms (multiorgan dysfunction, developmental concerns)

Genetic testing • Consider in children with epilepsy onset

before 3 y of age (in consultation with neurology).

Immune studies

• Consider in acute-onset seizures with

encephalopathy and/or other neurologic signs.

( continued )

Part I • Neurology

TABLE 2.3 DIAGNOSTIC STUDIES ( continued )

PROCEDURE

TYPE OF TESTING PURPOSE OF TEST

CONSIDERATIONS

• Consider in all children with new-onset seizures in the

• Neuroimaging to exclude a space occupying lesion should be performed prior to LP in children with seizure and new neurologic deficits.

setting of fever, especially before 6 mo of age. children with prolonged febrile seizure, new neurologic deficits.

• Perform in

CT, computed tomography; EEG, electroencephalogram; ER, emergency room; LP, lumbar puncture; MRI, magnetic resonance imaging.

TABLE 2.4 DO’S AND DON’TS FOR EVALUATION OF AN UNPROVOKED SEIZURE DIAGNOSTIC STUDIES TREATMENT DO DON’T DO DON’T • EEG for any child with a first-time un provoked seizure • Consider LP for a child with new-onset seizures in the set • Head CT • Brain MRI • Laboratory testing • LP in sim ple febrile seizure • Counseling for seizure safety to parents and families • Refer to Pedi atric Neurology for ongoing management.

• Empirically start anti epileptic

medications.

ting of a fever, espe cially those < 6 mo of age or a prolonged seizure.

CT, computed tomography; EEG, electroencephalogram; LP, lumbar puncture; MRI, magnetic resonance imaging.

■ REFERENCES 1. Kiriakopoulos E. Understanding seizures. Epilepsy Foundation. Published October 1, 2019. Accessed January 10, 2023. https://www.epilepsy.com/what-is-epilepsy/under standing-seizures#Is-this-epilepsy? 2. Asadi-Pooya AA, Farazdaghi M. Aura: epilepsy vs. functional (psychogenic) seizures. Seizure . 2021;88:53-55. 3. Wirrel E. A brief guide to diagnosis and management of new-onset, unprovoked seizures in children. Epilepsy Foundation. Reviewed March 19, 2014. Accessed January10,2023.https://www.epilepsy.com/stories/brief-g uide-d iagnosis-a nd-management- new-onset-unprovoked-seizures-children

Chapter 2 • Seizure

FEBRILE SEIZURE ■ INTRODUCTION

Febrile seizures are defined as a seizure accompanied by temperature ≥ 38.0 °C or 100.4 °F, without central nervous system infection. They occur most commonly in children 6 months to 5 years of age, with a predilection for children 12 to 18 months of age. Febrile seizures may be classified as simple or complex, based on their clinical features. Simple febrile seizures are a single generalized seizure lasting < 15 minutes. A febrile seizure that presents focally, occurs more than once during a con tiguous 24-hour period, or lasts > 15 minutes is considered complex. ■ EPIDEMIOLOGY AND RISK FACTORS Febrile seizures are the most common seizure type in childhood and occur in 2% to 5% of children. 1 A seasonal spike of febrile seizures is seen during winter due to greater febrile illnesses at this time. Febrile seizures do not have a gender predilection. See Table 2.5 for risk factors for febrile seizures. Unfortunately, febrile seizures recur in 30% to 50% of children who had a first febrile seizure. Table 2.6 shares the risk factors for recur rence of febrile seizures. TABLE 2.5 RISK FACTORS ASSOCIATED WITH FIRST FEBRILE SEIZURE Viral infections including influenza A and B, enterovirus, human herpes virus 6 (roseola), and respiratory syncytial virus Vaccinations that may induce fever, including measles, mumps, rubella Family history of febrile seizures or epilepsy Underlying neurodevelopmental deficits including cerebral palsy Low serum zinc or iron levels Children with two or more of the above-listed risk factors

Data from Sawires R, Buttery J, Fahey M. A review of febrile seizures: recent advances in understanding of febrile seizure pathophysiology and commonly implicated viral triggers. Front Pediatr. 2022;9:801321.

■ CLINICAL MANIFESTATIONS The mechanism of febrile seizures is unknown. They may occur as a result of underlying irregular brain reaction to higher temperatures, and both environmental and genetic factors are presumed to play a

Part I • Neurology

role in causing the event. 2 Table 2.7 describes the three types of febrile seizures. Data from Sawires R, Buttery J, Fahey M. A review of febrile seizures: recent advances in understanding of febrile seizure pathophysiology and commonly implicated viral triggers. Front Pediatr. 2022;9:801321. TABLE 2.6 RISK FACTORS FOR RECURRENCE OF FEBRILE SEIZURES Age < 12 mo for first febrile seizure Personal history of febrile seizures—each seizure increases the risk for recurrence. Initial seizure is a complex febrile seizure or febrile status epilepticus. Family history of febrile seizures

■ DIAGNOSTIC STUDIES See Table 2.8, based on the American Academy of Pediatrics guidelines. 1

■ MANAGEMENT A simple febrile seizure rarely requires hospitalization as most are self-limited and without sequelae. Table 2.9 reflects when to consider a hospital admission following a febrile seizure. Hospital discharge should occur when the child is at baseline and infection is managed.

TABLE 2.7 TYPES OF FEBRILE SEIZURES LENGTH

CLINICAL FEATURE RECOVERY AFTER SEIZURE

< 15 min

Simple febrile seizure

Generalized

Immediate recovery

≥ 15 min

Complex febrile seizure

• Focal • Generalized

May have some abnormali ties including an occurrence of temporary paralysis following a seizure (Todd’s paralysis)

and recurrent

• Generalized

and prolonged

> 30 min of multiple sei zures without recovery of consciousness in between 2

Febrile status epilepti cus

• Dependent on duration,

• Rarely stops

spontaneously • Often needs multiple medications, including

• Focal or

antiepileptics and benzodiazepines 3

generalized

Data from Mewasingh LD, Chin RFM, Scott RC. Current understanding of febrile seizures and their long-term outcomes. Dev Med Child Neurol. 2020;62(11):1245-1249.

Chapter 2 • Seizure

TABLE 2.8 HIGH-VALUE APPROACH TO SIMPLE FEBRILE SEIZURES DIAGNOSTIC STUDIES TREATMENT DO DON’T DO DON’T Lumbar puncture • If meningeal signs and • Initiate

• Electroencepha logram in a neurologically healthy child • Labs • Neuroimaging

antiepileptic medications 4

symptoms or exam sugges tive of a central nervous system infection

• Consider for:

• Age 6-12 mo with

deficient Haemophilus influenzae type b (Hib) or Streptococcus pneu moniae immunizations • Immunization status cannot be determined • A child who presents with a seizure and fever after receiving antibiotics

Data from Patel AD, Vidaurre J. Complex febrile seizures: a practical guide to evaluation and treatment. J Child Neurol . 2013;28(6):762-767.

TABLE 2.9 HOSPITALIZATION FOLLOWING A FEBRILE SEIZURE

• If seizure lasts > 5 min and needs a benzodiazepine to stop the seizure, suggesting a seizure may be less likely to stop on its own; benzodiazepines may lead to respiratory depression, requiring monitoring. • Abnormal examination or concerns for a central nervous system infection requiring further observation • If follow-up is not assured

Data from Smith DK, Sadler KP, Benedum M. Febrile seizures: risks, evaluation, and prognosis. Am Fam Physician . 2019;99(7):445-450.

■ REFERENCES 1. Subcommittee on Febrile Seizures; American Academy of Pediatrics. Neurodiagnostic evaluation of the child with a simple febrile seizure. Pediatrics . 2011;127(2):389-394. 2. Mewasingh LD, Chin RFM, Scott RC. Current understanding of febrile seizures and their long-term outcomes. Dev Med Child Neurol. 2020;62(11):1245-1249. 3. Loddenkemper T. Febrile status epilepticus: time is of the essence. Epilepsy Curr . 2014;14(6):345-347. 4. Patel AD, Vidaurre J. Complex febrile seizures: a practical guide to evaluation and treat ment. J Child Neurol . 2013;28(6):762-767.

Part I • Neurology

FIRST UNPROVOKED SEIZURE ■ INTRODUCTION

The first presentation of seizures represents a frightening experience for patients and their families. Clinicians must stabilize the patient, identify inciting factors, and determine if the event is epileptic or non epileptic in origin. ■ EPIDEMIOLOGY AND RISK FACTORS Estimates of recurrence of seizure in pediatric patients with first-time unprovoked seizures, also called afebrile or nonfebrile seizures, vary greatly from 23% to 71%. 1 Provoking factors for seizures include fe ver, central nervous system infection, neurologic disease, genetic syn dromes, brain trauma, and medications such as tramadol, bupropion, and tricyclic antidepressants. Risk factors for recurrent seizures are as follows 2,3 : • Intellectual disability • Two or more afebrile seizures within 24 hours • History of earlier unrecognized event • Abnormal baseline neurologic exam • Epileptiform abnormalities on electroencephalogram (EEG) • Nocturnal seizures • Significant abnormalities on brain imaging ■ CLINICAL MANIFESTATIONS Semiology of first-time seizures includes generalized tonic-clonic, tonic, and simple or partial complex seizures with or without general ization. First-time seizures are one or more seizures occurring within a 24-hour period with recovery of consciousness in the interictal period. Epilepsy is traditionally defined as two or more unprovoked seizures occurring > 24 hours apart; thus, these patients are in a distinct clini cal category. Neonatal and febrile seizures, posttraumatic seizures, and status epilepticus are defined by specific clinical presentation and di agnostic testing. 4

■ DIAGNOSTIC STUDIES See Table 2.10.

Chapter 2 • Seizure

TABLE 2.10 HIGH-VALUE APPROACH TO FIRST-TIME UNPROVOKED SEIZURE DIAGNOSTIC STUDIES TREATMENT DO DON’T DO DON’T • Detailed history and physical • EEG within 24 h to de • Lumbar • Supportive care • Rule out

• Initiate anti

puncture (unless specific indications exist)

epileptic drugs. 1,5

termine if seizure is epi leptic and classify seizure type 4

seizure mimics. • Follow course longitudinally and consider risks of ac tivities such as driving and swim ming, and potential ter atogenicity of medications.

• Labs

• Serum

• CBC • CMP, including

prolactin • Creatine kinase 4

magnesium, phosphorus • Urine toxicology screen • MRI brain if focal neuro logic exam or prolonged loss of consciousness • Evaluation by pedi atric neurologist or epileptologist 2,4

CMP, complete metabolic panel; CBC, complete blood count; EEG, electroencephalogram; LP, lumbar puncture; MRI, magnetic resonance imaging.

■ MANAGEMENT Management of first-time unprovoked seizure is largely supportive and aimed at determining whether the event is epileptic in origin. Antiepilep tic drugs are not recommended as they have not been shown to decrease recurrence of seizure, affect prognosis or diagnosis of epilepsy, or de crease seizure-related death. Furthermore, only 10% of those with first time seizure develop epilepsy regardless of treatment. Pediatric patients with first-time unprovoked seizure should be evaluated by a specialist with training in pediatric epilepsy to enhance diagnostic accuracy. 5 ■ REFERENCES 1. Stroink H, Brouwer OF, Arts WF, Geerts AT, Peters AC, van Donselaar CA. The first un provoked, untreated seizure in childhood: a hospital based study of the accuracy of the diagnosis, rate of recurrence, and long term outcome after recurrence. Dutch study of epilepsy in childhood. J Neurol Neurosurg Psychiatry . 1998;64(5):595-600. 2. Hamiwka LD, Singh N, Niosi J, Wirrell EC. Diagnostic inaccuracy in children referred with “first seizure”: role for a first seizure clinic. Epilepsia . 2007;48(6):1062-1066.

Part I • Neurology

3. Bergey GK. Management of a first seizure. Continuum (Minneap Minn) . 2016;22 (1 Epilepsy):38-50. 4. Hirtz D, Ashwal S, Berg A, et al. Practice parameter: evaluating a first nonfebrile seizure in children: report of the quality standards subcommittee of the American Academy of Neurology, The Child Neurology Society, and The American Epilepsy Society. Neurology . 2000;55(5):616-623. 5. Hirtz D, Berg A, Bettis D, et al. Practice parameter: treatment of the child with a first unprovoked seizure: report of the quality standards subcommittee of the American Acad emy of Neurology and the Practice Committee of the Child Neurology Society. Neurol ogy . 2003;60(2):166-175.

PSYCHOGENIC NONEPILEPTIC SEIZURES ■ INTRODUCTION

Psychogenic nonepileptic seizures (PNES) are paroxysmal events char acterized by changes in behavior without corresponding EEG abnor malities and are not due to cerebral dysfunction. 1 PNES are observable, have rapid onset, and are thought to have a psychogenic origin. 2 ■ EPIDEMIOLOGY AND RISK FACTORS PNES have been studied extensively in adults, and true incidence and prevalence in the pediatric population are unknown. School-age chil dren and more commonly adolescents tend to be affected, and the prev alence is greater in females. Patients are often misdiagnosed as having epilepsy and incorrectly treated with antiepileptic drugs. 1-3 Risk factors include the following: • School-related problems • Family discordance • Interpersonal conflicts • Physical and sexual abuse • Psychiatric comorbidities, such as, anxiety, depression, eating disor ders, posttraumatic stress disorder, and somatization • Neurologic and medical comorbidities, such as history of head trauma, intellectual disability, headaches, and chronic illnesses • Concurrent seizure disorder 1 ■ CLINICAL MANIFESTATIONS PNES are repeated paroxysmal events that are refractory to appropri ate medical therapy, initiated in response to specific stressors or trig gers, and uniquely occur only when spectators are around. 1,3 Close observation of the event frequently leads to the correct diagnosis. See Table 2.11 for other characteristic clinical features.

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