Pediatric Plastic and Reconstructive Surgery for Primary Care [1 ed.] 9781610023955, 9781610023948

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AUTHOR EDITORS American Academy of Pediatrics Peter J. Taub, MD, MS, FAAP, FACS, and Timothy W. King, MD, PhD, MSBE, FAAP, FACS Section on Plastic Surgery With contributions from the leading experts in the field, Pediatric Plastic and Reconstructive Surgery for Primary Care provides primary care pediatricians and other health practitioners who care for children essential support on caring for children who are faced with plastic and reconstructive surgery–related issues. Sixteen chapters with 200+ full-color photos provide superbly illustrated, authoritative guidance on when to treat and when to wait, the timing of corrective surgeries in pediatric patients, and surgical strategies and complications. Nonsurgical management and brief descriptions of how corrective procedures are performed are discussed, which is helpful to primary care pediatricians when counseling patients and their families and conducting long-term patient follow-up.

The book includes chapters on Cleft Lip and Palate Craniosynostosis Vascular Anomalies Congenital Ear Deformities Orthognathic Surgery Pediatric Facial Fractures Eyelid Anomalies Facial Paralysis

• • • • • • • •

Pediatric Neck Masses Congenital Hand Anomalies Pediatric Burn Injury Skin and Soft-Tissue Lesions Breast Anomalies Abdominal Wall Anomalies Posterior Trunk Anomalies Aesthetic Surgery in the Pediatric Patient

For other pediatric primary care resources, visit the American Academy of Pediatrics at shop.aap.org.

ISBN 978-1-61002-394-8

90000>

Peter J. Taub, MD, MS, FAAP, FACS Timothy W. King, MD, PhD, MSBE, FAAP, FACS

• • • • • • • •

Pediatric Plastic and Reconstructive Surgery for Primary Care

Pediatric Plastic and Reconstructive Surgery for Primary Care

Pediatric Plastic and Reconstructive Surgery for Primary Care

EDITORS

Peter J. Taub, MD, MS, FAAP, FACS Timothy W. King, MD, PhD, MSBE, FAAP, FACS

9 781610 023948

AAP

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Pediatric Plastic and Reconstructive Surgery for Primary Care

AUTHOR

American Academy of Pediatrics Section on Plastic Surgery EDITORS

Peter J. Taub, MD, MS, FAAP, FACS Timothy W. King, MD, PhD, MSBE, FAAP, FACS

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American Academy of Pediatrics Publishing Staff Mary Lou White, Chief Product and Services Officer/SVP, Membership, Marketing, and Publishing Mark Grimes, Vice President, Publishing Heather Babiar, MS, Senior Editor, Professional/Clinical Publishing Jason Crase, Senior Manager, Production and Editorial Services Theresa Wiener, Production Manager, Clinical and Professional Publications Linda Smessaert, Senior Marketing Manager, Professional Resources Published by the American Academy of Pediatrics 345 Park Blvd Itasca, IL 60143 Telephone: 630/626-6000 Facsimile: 847/434-8000 www.aap.org The American Academy of Pediatrics is an organization of 67,000 primary care pediatricians, pediatric medical subspecialists, and pediatric surgical specialists dedicated to the health, safety, and well-being of infants, children, adolescents, and young adults. The recommendations in this publication do not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into account individual circumstances, may be appropriate. Statements and opinions expressed are those of the authors and not necessarily those of the American Academy of Pediatrics. Any websites, brand names, products, or manufacturers are mentioned for informational and identification purposes only and do not imply an endorsement by the American Academy of Pediatrics (AAP). The AAP is not responsible for the content of external resources. Information was current at the time of publication. The persons whose photographs are depicted in this publication are professional models. They have no relation to the issues discussed. Any characters they are portraying are fictional. The publishers have made every effort to trace the copyright holders for borrowed materials. If they have inadvertently overlooked any, they will be pleased to make the necessary arrangements at the first opportunity. This publication has been developed by the American Academy of Pediatrics. The contributors are expert authorities in the field of pediatrics. No commercial involvement of any kind has been solicited or accepted in development of the content of this publication. Disclosures: [Populate in 2020 per MG]. Every effort has been made to ensure that the drug selection and dosages set forth in this text are in accordance with the current recommendations and practice at the time of publication. It is the responsibility of the health care professional to check the package insert of each drug for any change in indications or dosage and for added warnings and precautions. Every effort is made to keep Pediatric Plastic and Reconstructive Surgery for Primary Care consistent with the most recent advice and information available from the American Academy of Pediatrics. Special discounts are available for bulk purchases of this publication. Email Special Sales at [email protected] for more information. © 2020 American Academy of Pediatrics All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted in any form or by any means—electronic, mechanical, photocopying, recording, or otherwise—without prior permission from the publisher (locate title at http://ebooks.aappublications. org and click on © Get permissions; you may also fax the permissions editor at 847/434-8780 or email [email protected]). Printed in the United States of America 9-434/0520

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MA0962 ISBN: 978-1-61002-394-8 eISBN: 978-1-61002-395-5 Cover and publication design by LSD Design LLC Library of Congress Control Number: 2019944456

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American Academy of Pediatrics Reviewers Committee on Bioethics Council on Genetics Section on Anesthesiology and Pain Medicine Section on Breastfeeding Section on Cardiology and Cardiac Surgery Section on Infectious Diseases Section on Neonatal-Perinatal Medicine Section on Neurological Surgery Section on Ophthalmology Section on Oral Health Section on Orthopaedics Section on Otolaryngology–Head and Neck Surgery Section on Radiology

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Editors Peter J. Taub, MD, MS, FAAP, FACS Professor, Surgery, Pediatrics, Dentistry, and Medical Education Division of Plastic and Reconstructive Surgery Icahn School of Medicine at Mount Sinai New York, NY Timothy W. King, MD, PhD, MSBE, FAAP, FACS Associate Professor, Departments of Surgery and Biomedical Engineering Director of Research, Division of Plastic Surgery Associate Program Director, Plastic Surgery Residency University of Alabama at Birmingham Craniofacial and Pediatric Plastic Surgery Children’s of Alabama Chief, Plastic Surgery Section Birmingham VA Medical Center Birmingham, AL

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Contributors Stephen B. Baker, MD, DDS, FAAP, FACS Associate Professor Chief of Craniofacial Surgery Department of Plastic Surgery MedStar Georgetown University Hospital Washington, DC Codirector of Craniofacial Program Inova Children’s Hospital Falls Church, VA Chapter 5: Orthognathic Surgery Bruce S. Bauer, MD, FAAP, FACS Division of Plastic Surgery Department of Surgery The University of Chicago Chicago, IL Chapter 4: Congenital Ear Deformities Michael L. Bentz, MD, FAAP, FACS Chair, Division of Plastic and Reconstructive Surgery Vice Chair of Clinical Affairs, Department of Surgery Layton F. Rikkers, MD, Chair of Surgical Leadership Professor of Surgery, Pediatrics and Neurosurgery University of Wisconsin–Madison School of Medicine and Public Health Madison, WI Chapter 10: Congenital Hand Anomalies Lisa R. David, MD, FACS Department of Plastic and Reconstructive Surgery Wake Forest University Winston-Salem, NC Chapter 2: Craniosynostosis Claire Sanger Dillingham, DO Associate Professor Department of Plastic and Reconstructive Surgery Wake Forest School of Medicine Winston-Salem, NC Chapter 2: Craniosynostosis vii

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viii Contributors

Ravi K. Garg, MD Craniofacial Fellow Division of Plastic and Maxillofacial Surgery Children’s Hospital Los Angeles University of Southern California Los Angeles, CA Chapter 9: Pediatric Neck Masses Warren Garner, MD Director, LAC+USC Burn Center Professor of Surgery, Keck School of Medicine of USC University of Southern California Los Angeles, CA Chapter 11: Pediatric Burn Injury Jesse A. Goldstein, MD Assistant Professor, Department of Plastic Surgery Children’s Hospital of Pittsburgh University of Pittsburgh Medical Center Pittsburgh, PA Chapter 6: Pediatric Facial Fractures Arin K. Greene, MD, MMSc Associate Professor of Surgery Harvard Medical School Department of Plastic and Oral Surgery Vascular Anomalies Center Boston Children’s Hospital Boston, MA Chapter 3: Vascular Anomalies Jacqueline S. Israel, MD Integrated Plastic Surgery Resident Division of Plastic Surgery University of Wisconsin–Madison Department of Surgery Madison, WI Chapter 14: Abdominal Wall Anomalies

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ix Contributors

Timothy W. King, MD, PhD, MSBE, FAAP, FACS Associate Professor, Departments of Surgery and Biomedical Engineering Director of Research, Division of Plastic Surgery Associate Program Director, Plastic Surgery Residency University of Alabama at Birmingham Craniofacial and Pediatric Plastic Surgery Children’s of Alabama Chief, Plastic Surgery Section Birmingham VA Medical Center Birmingham, AL Chapter 4: Congenital Ear Deformities Chapter 14: Abdominal Wall Anomalies Brian I. Labow, MD, FAAP, FACS Associate Professor of Surgery, Harvard Medical School Department of Plastic and Oral Surgery Boston Children’s Hospital Boston, MA Chapter 13: Breast Anomalies Joseph E. Losee, MD, FAAP, FACS Ross H. Musgrave Endowed Chair in Pediatric Plastic Surgery Associate Dean for Faculty Affairs, University of Pittsburgh School of Medicine Professor and Executive Vice Chair, Department of Plastic Surgery, University of Pittsburgh Medical Center Division Chief, Pediatric Plastic Surgery, Children’s Hospital of Pittsburgh Pittsburgh, PA Chapter 6: Pediatric Facial Fractures Frederick Lukash, MD, FAAP, FACS Long Island Plastic Surgical Group New York, NY Chapter 16: Aesthetic Surgery in the Pediatric Patient Donald R. Mackay, MD, FAAP, FACS William P. Graham III Professor of Plastic Surgery Professor of Surgery and Pediatrics Vice Chair, Department of Surgery Penn State College of Medicine Hershey, PA Chapter 15: Posterior Trunk Anomalies

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x Contributors

Brad T. Morrow, MD Plastic Surgeon Marshfield Children’s Hospital Marshfield Clinic Health System Marshfield, WI Chapter 15: Posterior Trunk Anomalies Laura C. Nuzzi, BA Clinical Research Specialist Department of Plastic and Oral Surgery Boston Children’s Hospital Harvard Medical School Boston, MA Chapter 13: Breast Anomalies Jordan P. Steinberg, MD, PhD, FAAP, FACS Assistant Professor, Department of Plastic Surgery Johns Hopkins University School of Medicine Pediatric Plastic Surgery Johns Hopkins Children’s Center Baltimore, MD Chapter 12: Skin and Soft-Tissue Lesions Peter J. Taub, MD, MS, FAAP, FACS Professor, Surgery, Pediatrics, Dentistry, and Medical Education Division of Plastic and Reconstructive Surgery Icahn School of Medicine at Mount Sinai New York, NY Chapter 1: Cleft Lip and Palate Chapter 7: Eyelid Anomalies Mark M. Urata, MD, DDS Chair and Chief, Division of Plastic and Reconstructive Surgery Keck School of Medicine of USC Division Head, Plastic and Maxillofacial Surgery Children’s Hospital Los Angeles Chair, Division of Oral and Maxillofacial Surgery Herman Ostrow School of Dentistry of USC Los Angeles, CA Chapter 9: Pediatric Neck Masses

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xi Contributors

Haig Yenikomshian, MD Assistant Professor, Clinical Surgery Keck School of Medicine of USC University of Southern California Los Angeles, CA Chapter 11: Pediatric Burn Injury Ronald M. Zuker, MD, FRCSC, FACS, FRCSEd(Hon) Professor of Surgery University of Toronto Consultant Staff Surgeon The Hospital for Sick Children (SickKids) Toronto, Ontario, Canada Chapter 8: Facial Paralysis

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Contents

Acknowledgments ..................................................................... xv Preface ......................................................................................xvii

Chapter 1: Cleft Lip and Palate .....................................................................1 Peter J. Taub, MD, MS, FAAP, FACS Chapter 2: Craniosynostosis........................................................................ 17 Lisa R. David, MD, FACS, and Claire Sanger Dillingham, DO Chapter 3: Vascular Anomalies...................................................................39 Arin K. Greene, MD, MMSc Chapter 4: Congenital Ear Deformities....................................................... 51 Bruce S. Bauer, MD, FAAP, FACS, and Timothy W. King, MD, PhD, MSBE, FAAP, FACS Chapter 5: Orthognathic Surgery................................................................67 Stephen B. Baker, MD, DDS, FAAP, FACS Chapter 6: Pediatric Facial Fractures.......................................................... 81 Jesse A. Goldstein, MD, and Joseph E. Losee, MD, FAAP, FACS Chapter 7: Eyelid Anomalies...................................................................... 105 Peter J. Taub, MD, MS, FAAP, FACS Chapter 8: Facial Paralysis......................................................................... 113 Ronald M. Zuker, MD, FRCSC, FACS, FRCSEd(Hon) Chapter 9: Pediatric Neck Masses..............................................................129 Ravi K. Garg, MD, and Mark M. Urata, MD, DDS Chapter 10: Congenital Hand Anomalies................................................... 139 Michael L. Bentz, MD, FAAP, FACS Chapter 11: Pediatric Burn Injury............................................................... 153 Haig Yenikomshian, MD, and Warren Garner, MD Chapter 12: Skin and Soft-Tissue Lesions.................................................... 165 Jordan P. Steinberg, MD, PhD, FAAP, FACS

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xiv Contents

Chapter 13: Breast Anomalies...................................................................... 183 Laura C. Nuzzi, BA, and Brian I. Labow, MD, FAAP, FACS Chapter 14: Abdominal Wall Anomalies....................................................199 Jacqueline S. Israel, MD, and Timothy W. King, MD, PhD, MSBE, FAAP, FACS Chapter 15: Posterior Trunk Anomalies..................................................... 213 Brad T. Morrow, MD, and Donald R. Mackay, MD, FAAP, FACS Chapter 16: Aesthetic Surgery in the Pediatric Patient..............................229 Frederick Lukash, MD, FAAP, FACS

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Index..........................................................................................239

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Acknowledgments The authors would like to acknowledge the compassionate and intelligent collegiality they are fortunate to experience with colleagues who share a genuine interest in the health and welfare of pediatric patients. The ability to work with and learn from so many talented individuals has been truly extraordinary.

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Preface This text was created for pediatric primary care physicians and other health practitioners who care for pediatric patients, to help them understand, guide, and care for children who are faced with plastic and reconstructive surgery–related issues. We hope it will help provide insights into some of the more common conditions seen within the realm of pediatric plastic surgery. In most instances, pediatric plastic surgeons are not only skilled in basic plastic surgery, but they also receive subspecialty training to be able to manage complex conditions that are often seen exclusively in children (eg, vascular malformations, cleft and craniofacial anomalies, pediatric hand issues). In this way, these specialists are unique among the surgical disciplines in terms of the breadth and visibility of the work they undertake. No other specialty manages all tissue types, and in no other specialty is an appreciation for aesthetics so important. Many pediatric plastic surgeons are also involved in global surgical care, in addition to holding leadership positions at home. Most important of all, pediatric plastic surgeons continue to demonstrate a passion for their patients and the work that they do, and we hope this passion is reflected in the pages of this book. We sincerely hope you find this resource to be useful in guiding and supporting the children and families in your care. Peter J. Taub, MD, MS, FAAP, FACS Timothy W. King, MD, PhD, MSBE, FAAP, FACS

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CHAPTER

1

Cleft Lip and Palate PETER J. TAUB, MD, MS, FAAP, FACS

Introduction

Facial cleft is one of the most commonly recognized congenital anomalies. Its presence and attempts to repair it have been historically well described. Pediatric plastic surgeons usually treat infants with facial clefts as part of a multidisciplinary cleft team that is able to offer coordinated otolaryngological, dental, genetic, speech, audiological, and other services. Pediatricians of these infants should be aware of the association of cleft as a component of syndromic genetic disorders. These disorders need to be considered and ruled in or out before considering surgical correction. By definition, the primary palate is composed of structures anterior to the incisive foramen, while the posterior palate is composed of those structures posterior to it (Figure 1-1). The former would include the lip, the gum tissue (alveolar ridge), and the anterior portion of the hard palate. The latter would, thus, include the posterior hard palate and the soft palate. In reality, clinicians use the terms lip, alveolus, and palate more commonly. The whole palate is then separated into hard and soft portions, which are easy to

Figure 1-1. Intraoperative photograph of the roof of the mouth demonstrates the position of the incisive foramen, which separates the true primary and secondary palates. 1

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2 Pediatric Plastic and Reconstructive Surgery for Primary Care

delineate on palpation or at gross inspection. The soft, mobile portion is also referred to as the velum. The posterior soft palate contains the muscles that elevate and pull the palate posteriorly. This action seals off the nasal cavity from the pharynx, which is important for normal resonance in speech. Six muscles (ie, tensor veli palatini, levator veli palatini, palatoglossus, palatopharyngeus, superior constrictor, and uvulus) have some attachment to the palate, and all serve slightly different functions (Figure 1-2). In most cases of cleft palate, the tensor muscles, which normally originate laterally and insert in the midline, aberrantly insert along the posterior edge of the hard palate.1 The abnormal muscle position prevents adequate regulation of airflow and fluid flow through the distal eustachian tube, predisposing the patient to fluid buildup in the middle ear. Re-approximation of the muscle in the midline during palate repair serves to restore the normal anatomy of the palatal musculature. The upper jaw (maxilla) develops from 3 components that fuse at the incisive foramen. The midline 4 teeth (ie, paired central and lateral incisors) develop and erupt into the midline premaxilla, while the remaining teeth (ie, canines, premolars, and molars) erupt into the lateral palatal shelves. Typical clefts occur in the region of the lateral incisor and canine, either of which may or may not be present.2

Superior pharyngeal constrictor

Levator veli palatini

Figure 1-2. Schematic drawing of the muscles of the palate.

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3 Chapter 1: Cleft Lip and Palate

Epidemiology

Among ethnic groups, the incidence of cleft is highest in Native Americans, followed by the white population, Asians, and African Americans. Clefts of the lip, with or without a cleft of the palate, are now thought of as a separate entity from isolated clefts of the palate. The 2 differ significantly in terms of their epidemiological profiles. The incidence of clefts of the lip with or without a cleft palate is approximately 1 in 700 live births, while that of isolated clefts of the palate is closer to 1 in 2,000 live births. The isolated variety is also more commonly seen in girls, while clefts of the lip with or without a cleft palate are more commonly seen in boys.

Patient Presentation

The diagnosis of a facial cleft may be established early in pregnancy by a skilled ultrasonographer (Figure 1-3). In such cases, it is usually beneficial for the parents to visit with a pediatric plastic surgeon who is part of a multidisciplinary team to discuss the nature of the problem and associated management strategies in advance of the birth. Frequently, however, the cleft is noted at birth as a defect of the lip, nose, and/or roof of the mouth. A thorough history and physical examination should yield any history of cleft or congenital anomalies in family members and any prenatal problems that might be related to development of the cleft. Facial cleft may be seen as part of any number of sequences or syndromes that encompass other

Figure 1-3. Prenatal axial ultrasonographic image of a cleft. The arrow highlights the gap in the palate.

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4 Pediatric Plastic and Reconstructive Surgery for Primary Care

Figure 1-4. A patient with Pierre Robin sequence; this photo highlights the micrognathia.

anomalies. In patients with Pierre Robin sequence (Figure 1-4), the cleft is usually in the secondary (or soft) palate. Here, it is the result of the associated micrognathia, which does not allow the tongue to get out of the way of the descending palatal shelves and normally fuse in the midline before birth. In such patients, prone positioning, tongue-lip adhesion, tracheostomy, or early distraction of the mandible may be required before closure of the palate to stabilize the infant’s airway. More severe anomalies of which facial cleft is a component include Apert syndrome, hereditary progressive arthro-ophthalmopathy (Stickler syndrome), Treacher Collins syndrome, Van der Woude syndrome, and velocardiofacial syndrome.

Diagnosis

Clefts are generally described in terms of their laterality, their completeness, and the specific structures that are affected. The lip, alveolus, and palate may or may not be involved in the cleft. Common presentations include an isolated cleft lip (either complete or incomplete), an isolated cleft palate, and a complete cleft of the lip, alveolus, and palate (Figure 1-5). A rare combination may include a cleft of the lip, with an intervening intact alveolus, and a cleft palate. The simplest form of a cleft lip is a microform cleft in which the external skin and internal mucosa are intact but the underlying muscle is not joined across the defect (Figure 1-6). There may or may not be subtle involvement of the nose. The simplest form of a cleft palate is a bifid uvula, which occurs in roughly 2% of the population and may go undetected (Figure 1-7). The bifid

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5 Chapter 1: Cleft Lip and Palate

Figure 1-5. Complete cleft deformity.

Figure 1-6. Microform cleft lip.

Figure 1-7. Bifid uvula.

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6 Pediatric Plastic and Reconstructive Surgery for Primary Care

Figure 1-8. Zona pellucida, indicative of a submucous cleft palate.

uvula, however, may be a clue to the presence of an underlying submucous cleft palate. Here, the oral and nasal mucosal surfaces are intact, but the levator veli palatini muscle fibers are divergent. This may present in older children without a prior diagnosis of an overt cleft palate. Patients are referred with speech concerns, despite a period of therapy that has failed to provide adequate results. The specific findings that might accompany a patient’s hypernasal speech include a bifid uvula, a notch of the posterior nasal spine, and a zona pellucida, or clear area in the middle of the soft palate (Figure 1-8). Adolescents who present with an unrepaired cleft of the hard and/or soft palate are likely to have greater speech concerns than are younger patients. The adolescents develop errors of articulation as a means of compensation for the cleft. Depending on their access to health care, some may or may not have had years of speech therapy in an attempt to overcome the physiological problem of palatal incompetence. While closure is important to improve resonance and oral hygiene, it is usually difficult to achieve near-normal speech quality in these patients.

Management Infancy Newborns and infants with clefts should be evaluated and managed in the context of a multidisciplinary cleft team. Frequently, these are led by pediatric plastic surgeons who have an interest in cleft anomalies. Important team

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7 Chapter 1: Cleft Lip and Palate

members include pediatric otolaryngologists, pediatric dentists, feeding specialists, orthodontists, speech-language pathologists, audiologists, and social services personnel. The various members should evaluate the patient on a regular basis—the interval varies according to age and need of services. Early problems related to a cleft palate include difficulty with maintaining adequate nutrition, especially with breastfeeding, and regurgitation. Breastfeeding is often difficult because newborns and infants use suction to breastfeed successfully, and those with cleft palate have difficulty creating suction. A mother who wants to attempt breastfeeding and/or provide human (breast) milk to her baby who has a cleft palate should be seen by a certified lactation consultant for evaluation, feeding support, and assistance with procuring and using a breast pump. Pumping and feeding the baby expressed milk provides the baby with the benefits that breast milk offers. The situation is different for neonates who are born with cleft lip without a cleft palate. These newborns can typically form an adequate seal to generate adequate intraoral negative pressure to suck and transfer milk effectively and can often successfully breastfeed. Most newborns and infants do better with head-up positioning and the use of specially designed nipples that facilitate intake of milk or formula by diminishing resistance to flow or creating force to push fluid from the bottle in the presence of a weak suck. Later, sequelae of an open palate include fluid buildup in the middle ear, which can potentially lead to hearing loss, abnormal speech production and language development, and distortion of facial growth. The predisposition to acute otitis media may be offset by exclusive breastfeeding or the use of human milk; thus, despite difficulties in the establishment of breastfeeding, lactation, and transition to the breast for breastfeeding, the maintenance of the mother’s milk supply should be part of the overall management strategy.

Lip Repair Infants with an open lip and an intact palate usually feed with minimal difficulty. Parents usually want the lip closed as early as possible because of the social implications rather than any functional problems. As such, cleft lip repair is generally performed at about 3 months of age, a time chosen because of the relative safety of general anesthesia in an infant. Involvement of the palate, on the other hand, requires closure to improve oral and nasal hygiene and allow for the development of intelligible speech with normal resonance. For the infant with a wide cleft of the lip and palate, nasoalveolar molding of the palate before lip repair may be offered. This can be extremely helpful in the wide bilateral cleft, where the premaxilla is far anterior to the lateral segments. Molding will bring the 2 sides of the palate closer together, possibly elevate the flattened nose, and lessen any tension across the lip. Nasoalveolar molding requires obtaining an impression of the palate as soon as possible—often within the first few days after birth—from which an appliance may be fabricated (Figure 1-9). Frequent follow-up visits are

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8 Pediatric Plastic and Reconstructive Surgery for Primary Care

Figure 1-9. Intraoral appliance used for nasoalveolar molding.

necessary over the ensuing weeks to ensure correct and adequate movement of the arches in anticipation of lip repair. Modifications include attachment of a nasal bulb to facilitate recontouring of the alar rim. Prior to the advent of nasoalveolar molding, a wide cleft lip was closed in stages. An adhesion procedure was performed first by using soft tissues from the margins of the cleft that were not to be used for the later formal repair. After allowing the tension from the closure to mold the palatal arches, the lip was then repaired at a second setting. The adhesion facilitates not only lip repair but also the later palatal closure. Formal lip repair requires general anesthesia and, most often, an overnight stay in the hospital because of the young age of the patient and the proximity of the repair site to the airway. Most healthy infants without additional congenital or medical abnormalities should not have an increased risk of adverse effects from anesthesia. Preoperative discussion and evaluation with the anesthesiologist may help optimize the patient’s readiness for surgery and improve postoperative outcome. The lip is closed by designing and moving various small flaps of skin, muscle, and mucosa to fill the space of the cleft.3 The procedure generally takes 2 to 3 hours and results in minimal blood loss. Some surgeons use permanent skin sutures that are removed during the first postoperative week; others prefer buried absorbable or superficial dissolvable sutures. Soft arm restraints may be used to prevent the infant from inadvertently disrupting the repair. A regular diet may be resumed immediately. To rest the lip, feeding may be achieved with a large syringe and a red rubber catheter.

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9 Chapter 1: Cleft Lip and Palate

Palate Repair The timing of palate repair varies from team to team. Early palate closure creates normal anatomy for the development of proper speech development but may interfere with growth of the midface. It is widely accepted that repair before 18 months of age minimizes problems related to speech and language development, while not causing drastic effects on midfacial growth.4 Later repair, although ideal for minimizing scar formation during growth, leads to errors of articulation that become more difficult to reverse as children get older. Patients with cleft palate may have other congenital abnormalities and difficult airways. Whenever possible, an anesthesia evaluation is recommended. Some surgeons have advocated for repair of the palate in stages. The soft palate, which is more intimately involved with speech production, is repaired as early as 3 to 4 months of age, often at the time of lip repair. This is followed by later repair of the hard palate, delayed months to years, to minimize the detrimental effects on facial growth.5 A palatal obturator may be used in the interim to seal off the oral cavity and facilitate breastfeeding. Long-term evaluation, however, has failed to support any benefit of this approach on normal speech development.6,7 Palate repair is performed with general anesthesia, with the patient in a supine position. The procedure involves advancement of tissue from either side of the cleft toward the midline and generally takes 2 to 3 hours, with minimal blood loss (Figure 1-10). Infants should be admitted to the hospital overnight for observation because the procedure involves the airway. A large

Figure 1-10. Markings for cleft palate repair.

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suture is often placed through the tip of the tongue and taped to the cheek overnight, in the event of postoperative airway compromise. In infants and small children, the tongue may be difficult to grasp in an emergent setting, making the suture potentially lifesaving. The suture is easily removed on the first postoperative day with no adverse sequelae. Again, soft arm restraints may be used to prevent the infant from inadvertently disrupting the repair. Most children can recover in a regular hospital ward; others, such as those with associated micrognathia or a syndrome, may require oxygen saturation monitoring. Suction catheters, straws, and spoons should be avoided to minimize perforation of the repair. If suctioning is needed, it should be performed carefully and laterally, along each side of the tongue. Antibiotics are not required. Generally, the palate tolerates the normal colonization of bacterial flora within the oral cavity and does not become infected. Common floras include staphylococcal and streptococcal species, as well as Enterobacter, Neisseria, Bordetella, and Corynebacterium. However, infants and children with clefts often have fluid in the middle ear, which may harbor more virulent organisms and act as a source of contamination. Because repair is considered elective, deferring surgery until after any infection has subsided is often prudent. If the infant is able to tolerate a liquid diet and is otherwise stable, he or she may be discharged the following day. Different surgeons have different views on the postoperative diet. More conservative protocols have the patient ingest milk, formula, or pureed foods for 1 to 2 weeks to allow the palate to heal. Many submucous cleft palates go undiagnosed, and others require no treatment at all.8 Patients who present with hypernasal speech and are suspected of having a submucous cleft should undergo either nasopharyngoscopy and/or videofluoroscopy. The former involves passage of a small-caliber endoscope through the nose into the nasopharynx while the patient is awake. For this reason, infants and younger children may not tolerate the procedure as well as older ones, and the study may need to be deferred until the patient is more cooperative. The camera is positioned above the soft palate and is able to visualize closure of the velum during phonation (Figure 1-11). In the patient suspected of having a cleft palate, ridging of the lateral palatal elements may be indicative of incomplete muscle union in the midline. The soft palate may then fail to contact the posterior pharyngeal wall at the level of the Passavant (palatopharyngeal) ridge opposite the second cervical vertebra during phonation. Videofluoroscopy provides similar information but does so by imaging the nasopharynx with radiography. It provides detailed information about the movement of the velum with speech. However, a fluoroscopic image provides only a single, cross-sectional view of the palate, usually in the sagittal plane. It is not possible to record multiple planes simultaneously; therefore, it cannot be used to ascertain whether adequate velopharyngeal closure is possible.

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11 Chapter 1: Cleft Lip and Palate

Figure 1-11. Nasoendoscopic image of the velum and posterior wall of the pharynx demonstrates incomplete closure with phonation.

Nasal Repair The infant or child with a complete cleft of the lip and nose has a characteristic nasal deformity (Figure 1-12).9 The alar dome is flattened, and the alar base is generally more lateral and posterior on the face than it is on the normal side. Traditional teaching was that surgery on the nose at an early age would result in growth disturbance. Manipulation of the nasal tip cartilage is now commonly addressed at the time of lip repair. Some surgeons

Figure 1-12. Characteristic cleft nasal deformity.

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also address the septal deflection at this age. Fracture and repositioning of the nasal bones is generally deferred until adolescence.

Alveolar Repair The most anterior portion of the palate—the alveolar ridge—is generally not addressed during specific lip and palate repair (Figure 1-13) and is closed separately. The oronasal fistula in the region of the cleft is usually small and, despite problems with regurgitation, has little effect on speech production. Repair of the alveolar cleft requires placement of a small amount of bone graft under the mucosa to create a stable palatal arch, provide bone into which the erupting teeth may anchor, support the alar base of the nose, and provide a base for possible prosthetic dental restoration. The timing of alveolar closure has been described at various stages during the management of a cleft palate. Some authors advocate mucosal closure at the time of initial lip repair (if the mucosa can be easily closed over it), noting that the bony defect may then heal without the need for later grafting.10 Others advocate early bone grafting at the time of palatal repair.11 Still others prefer to delay grafting until anywhere from 2 to 12 years of age.12 A better gauge for timing repair may be based on dental eruption. In this protocol, grafting may be performed prior to eruption of the permanent incisor, after eruption of the incisor but before eruption of the cleft side canine, and after eruption of the cleft side canine. Bone is taken from the hip or tibia and placed into the open alveolus. The floor of the nose and gum tissue is repaired at the same time. The procedure can generally be performed on an outpatient basis because it is less invasive and the patient is older. Some surgeons are investigating the use of packaged bone matrices with or without bone morphogenetic protein as a substitute. While this minimizes the need for a donor site, it may increase cost and has yet to be shown to be an effective alternative. Additional procedures may be considered, if indicated. Certainly, revision of the lip may be performed if there are significantly noticeable differences from the other side.13 Dental issues may require orthodontia to maximize

Figure 1-13. Open alveolar defect.

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13 Chapter 1: Cleft Lip and Palate

Figure 1-14. Malocclusion accompanying maxillary hyperplasia due to cleft deformity.

occlusion. Future growth of the midface is also a concern after repair of the palate. It is thought that the production of scar tissue near the growth centers of the face interferes with normal skeletal development and leads to the development of a malocclusion (Figure 1-14). In this instance, advancement of the upper jaw (Le Fort osteotomy) may be required if orthodontia cannot address the discrepancy. To date, prospective, well-controlled studies are still lacking to establish the long-term effect of hard palate repair on facial growth.14

Complications

The complications of cleft repair are bleeding, airway compromise, and wound healing problems. Bleeding is a concern but is not commonly encountered. A solution of epinephrine and hypotensive anesthesia may be used during surgery to minimize blood loss. When the palate is closed, gentle compression over the bone until the patient emerges from anesthesia may be useful. Airway issues are more concerning in newborns and younger infants with smaller airways and in procedures that directly involve the airway. Postoperative airway compromise in the obligate nose breather may result from edema and blood present in an already small nasopharynx. It may also result from swelling caused by compression beneath the mouth prop used to expose the palate and subsequent edema of the tongue. While wound healing problems are infrequently seen after lip repair, palatal closure may be complicated by dehiscence or fistula formation. To combat the difficulty in creating a watertight seal, several authors have advocated the use of tissue sealants in cleft palate repair.15 Cyanoacrylate

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adhesive may be instilled beneath the mucoperiosteal flaps to fix the flaps in place and minimize dead space, or it may be placed as a topical sealant. It has yet to be shown whether such maneuvers minimize the risk of fistula formation. Often, some amount of tension is placed across the repair, and a fistula forms at the point of maximal tension (Figure 1-15). Fistulae as small as 5 mm2 may interfere with speech production.16 Rates of formation vary among published series but are approximately 5% to 10% (and higher for patients older than 2 years). Secondary closure of a palatal fistula is difficult because of the scarring present at the margins of the defect. Alloplastic material (acellular dermis), local flaps (adjacent palate), or distant flaps (tongue flap) may be used to cover the defect, and better results are obtained with a 2-layer closure. A speech-language pathologist with familiarity in treating infants and children with clefts should evaluate (and continue to follow up if necessary) patients undergoing cleft palate closure because the patients are at risk of problems related to velopharyngeal insufficiency. Speech impairments specific to velopharyngeal incompetence affect sounds that require pressure in the oral cavity, including the sibilant consonants, such as s, z, ch, sh, and zh, and the intraoral pressure consonants, such as b, d, k, p, and t. Reports of insufficiency range from 7% to 30%. Such problems have been shown to be more prevalent in patients with a complete cleft of the hard and soft palate or those with associated congenital anomalies.17 Frequently, adjunctive speech therapy is needed to correct errors of articulation that develop as a compensatory mechanism. Despite advances in the surgical management of cleft palate, children continue to require

Figure 1-15. Chronic fistula of the hard palate.

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speech therapy as an integral component of their global care. This can be conducted alone or in a group setting, in conjunction with a school program or independently. Hypernasal speech that does not improve with intensive therapy by a skilled speech-language pathologist may require a secondary procedure. These children should undergo nasoendoscopy or videofluoroscopy to better delineate the functional deficiency. If the soft palate is not able to seal off the nasal and oral cavities, surgery may be suggested. This may include lengthening the palate and/or rotating local muscles (sphincter pharyngoplasty) or local mucosa (pharyngeal flap) to the back of the pharynx to make the area smaller. Currently, modern interventions for patients with a cleft palate result in a good outcome, in terms of speech development and facial appearance, with a low risk of complications. Management should be directed by a multidisciplinary cleft team with experience in treating children with clefts of the lip and palate. Continued long-term follow-up by the complete team of specialists is important for monitoring improvement and identifying areas of concern. REFERENCES

1. Braithwaite F, Maurice DG. The importance of the levator palati muscle in cleft palate closure. Br J Plast Surg. 1968;21(1):60–62 PMID: 4868004 https://doi.org/10.1016/S0007-1226(68)80087-6 2. Vichi M, Franchi L. Eruption anomalies of the maxillary permanent cuspids in children with cleft lip and/or palate. J Clin Pediatr Dent. 1996;20(2):149–153 PMID: 8619976 3. Taub PJ, Collins M. Maintenance of certification: unilateral cleft lip repair. J Craniofac Surg. 2012;23(2):448–454 PMID: 22421837 https://doi.org/10.1097/SCS.0b013e318240ff1f 4. Witzel MA, Salyer KE, Ross RB. Delayed hard palate closure: the philosophy revisited. Cleft Palate J. 1984;21(4):263–269 PMID: 6595081 5. Schweckendiek H. Zur Frage der Fruh-und Spat-operationen der angeborenen Lippen-KieferGaumen-Spalten (mit Dem-onstrationen) [The problem of early and late surgery in congenital fissure of the lips and palate]. Z Laryngol Rhinol Otol. 1951;30:51 PMID: 14818332 6. Schweckendiek W, Doz P. Primary veloplasty: long-term results without maxillary deformity. a twenty-five year report. Cleft Palate J. 1978;15(3):268–274 PMID: 278679 7. Bardach J, Morris HL, Olin WH. Late results of primary veloplasty: the Marburg Project. Plast Reconstr Surg. 1984;73(2):207–218 PMID: 6695019 https://doi.org/10.1097/00006534198402000-00007 8. McWilliams BJ. Submucous clefts of the palate: how likely are they to be symptomatic? Cleft Palate Craniofac J. 1991;28(3):247–251 PMID: 1911811 https://doi.org/10.1597/ 1545-1569(1991)0282.3.CO;2 9. Henry C, Samson T, Mackay D. Evidence-based medicine: the cleft lip nasal deformity. Plast Reconstr Surg. 2014;133(5):1276–1288 PMID: 24776558 10. Santiago PE, Grayson BH, Cutting CB, Gianoutsos MP, Brecht LE, Kwon SM. Reduced need for alveolar bone grafting by presurgical orthopedics and primary gingivoperiosteoplasty. Cleft Palate Craniofac J. 1998;35(1):77–80 PMID: 9482227 https://doi.org/10.1597/1545-1569 (1998)0352.3.CO;2 11. Dado DV, Rosenstein SW, Alder ME, Kernahan DA. Long-term assessment of early alveolar bone grafts using three-dimensional computer-assisted tomography: a pilot study. Plast Reconstr Surg. 1997;99(7):1840–1845 PMID: 9180707 https://doi.org/10.1097/00006534-199706000-00006 12. Cohen M, Polley JW, Figueroa AA. Secondary (intermediate) alveolar bone grafting. Clin Plast Surg. 1993;20(4):691–705 PMID: 8275634 13. Monson LA, Khechoyan DY, Buchanan EP, Hollier LH Jr. Secondary lip and palate surgery. Clin Plast Surg. 2014;41(2):301–309 PMID: 24607196 https://doi.org/10.1016/j.cps.2013.12.008

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16 Pediatric Plastic and Reconstructive Surgery for Primary Care 14. Shi B, Losee JE. The impact of cleft lip and palate repair on maxillofacial growth. Int J Oral Sci. 2015;7(1):14–17 PMID: 25394591 https://doi.org/10.1038/ijos.2014.59 15. Turkaslan T, Ozcan H, Dayicioglu D, Ozsoy Z. Use of adhesives in cleft palate surgery: a new flap fixation technique. J Craniofac Surg. 2005;16(4):719–722 PMID: 16077326 https://doi. org/10.1097/01.SCS.0000159940.62875.95 16. Witt PD, D’Antonio LL. Velopharyngeal insufficiency and secondary palatal management. A new look at an old problem. Clin Plast Surg. 1993;20(4):707–721 PMID: 8275635 17. Persson C, Elander A, Lohmander-Agerskov A, Söderpalm E. Speech outcomes in isolated cleft palate: impact of cleft extent and additional malformations. Cleft Palate Craniofac J. 2002;39(4):397–408 PMID: 12071788 https://doi.org/10.1597/1545-1569(2002)0392.0.CO;2

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CHAPTER

2

Craniosynostosis LISA R. DAVID, MD, FACS, AND CLAIRE SANGER DILLINGHAM, DO

Introduction

The skull is made up of multiple bones, connected to each other by fibrous joints. This is called synarthrosis (Figure 2-1). These joints are referred to as sutures and are ideally open at birth to permit growth and elasticity. The growth of the skull is dependent on the growth of the brain. When there is an interruption in the growth of the skull for any reason, the growth of the brain will involuntarily be inhibited. This is true in reverse, as well. If the brain does not grow for any reason, neither will the skull. Keep in mind that most cranial growth occurs within the first year after birth, with the brain doubling in size during this year. This chapter focuses on factors that can affect skull development. Specifically, premature fusion of one or more of the cranial sutures is referred to as craniosynostosis. This condition occurs in around 3.5 of 10,000 live births worldwide.1–4 Craniosynostosis can involve one or several sutures. It can be Metopic suture

Anterior fontanelle

Coronal suture

Sagittal suture Posterior fontanelle Lambdoidal sutures

Figure 2-1. Illustration of the cranial sutures in an infant. 17

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associated with a syndrome (syndromic, occurring in 1 in 30,000 or more live births) or isolated from any other identified events or conditions (nonsyndromic, occurring in 1 in 2,500 live births).5 The most common presentation is nonsyndromic single-suture involvement.6 The phenotypic presentation of craniosynostosis is determined by the specific suture that is involved, and each will be described in detail. Nonsyndromic craniosynostosis usually involves one cranial suture. Single-suture nonsyndromic sagittal craniosynostosis is the most common presentation, and it affects approximately 1 in 2,500 infants worldwide.7 There has been debate on whether single-suture craniosynostosis has any detrimental effects on an infant. Now, data clearly show that these infants have a risk of developing increased intracranial pressure (ICP) with increasing frequency over time if surgical correction is not performed.8–10 They are also prone to developing amblyogenic levels of strabismus or refractive error—and, as such, they should be seen by a pediatric ophthalmologist.11 Syndromic craniosynostosis accounts for up to 40% of all occurrences of craniosynostosis.12,13 In addition to the cranial findings, patients may have other related congenital anomalies affecting other parts of the body. The published literature holds descriptions of hundreds of such syndromes associated with craniofacial anomalies. The Whitaker classification of craniofacial anomalies, although not all-inclusive, provides a framework to organize the different syndromes. Type I involves the different facial clefts. Type II includes synostoses subcategorized as symmetrical and asymmetrical. Type III involves the atrophy or hypoplasia of the skin, subcutaneous tissue, muscle, or bone. Type IV includes neoplasia such as hemangiomas and lymphangiomas. The final, type V, is the unclassified group and includes organ involvement.14 This classification system vividly describes how craniofacial syndromes can have clinical manifestations that affect systems or structures beyond the cranium and face. This supports the multidisciplinary approach to such patients so that the goal remains to provide treatment to all affected systems. Some of the more common syndromes are Apert, Crouzon, Pfeiffer, and Chotzen syndromes, which are considered acroceph­ alosyndactyly and are autosomal dominant inherited disorders. Some of these cause abnormalities, most commonly of the skull, face, hands, and feet. The details and differences will be further described so a differentiation can be made between them for diagnostic purposes and family counseling. Encouraging research was reported by Maliepaard et al, in which IQ scores were shown to be in the reference range for some patients with syndromic craniosynostosis. The less encouraging news was that these children had nearly 2-times higher risk for intellectual disability and higher rates of behavioral and emotional problems, when compared with the normative population.15 Apert and Crouzon syndromes fall into this category.

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Etiopathogenesis

For a half century, there were 2 main theories for the pathogenesis of craniosynostosis, as described by Virchow and Moss. Virchow held to the belief that the abnormality in growth occurred at the site of the cranial suture. On the other hand, Moss proposed that the abnormality was in the cranial base, resulting in a negative effect on the dura through ligamentous attachments.16 In the past 20 years, several researchers have shown that gene mutations in fibroblast growth factor receptors can lead to craniosynostosis, owing to the defects in signaling and tissue interactions.17–19 For example, the TWIST1 gene mutation is noted to cause Chotzen syndrome, one of the more common syndromic causes of craniosynostosis.

Patient Presentation

Clinical presentation is dependent on suture involvement. According to Virchow’s law, growth of the skull is restricted in the direction perpendicular to the fused suture and is increased in the direction that is parallel to the fused suture.20 An increase in ICP may be detected in a patient with premature fusion of one or more cranial sutures. It is more commonly seen with patients who have multiple craniosynostosis and syndromic craniosynostosis.21 This may be a result of a restriction on the growth of the brain and requires correction within the first year after birth. In infants with craniosynostosis, there is great concern that a postponement in surgical intervention can lead to developmental delay, visual loss, psychomotor impairment, Chiari malformations, and tonsillar herniation.22 This can be noninvasively detected during an eye examination by a pediatric ophthalmologist. An ophthalmologic examination may demonstrate papilledema, which is very sensitive in indicating an increase of ICP; however, the lack of papilledema does not exclude increased ICP. Papilledema can lead to optic nerve atrophy and irreversible visual deficits. The abnormal position of the orbit and muscles that occur with these infants can result in abnormal eye movements or strabismus.23 Infants with single-suture synostosis have up to 14% risk for developing increased ICP, and infants with multiple suture synostoses can have as high as a 67% risk of increased ICP.8,9 Prolongation of the condition can result in headaches, emesis, irritability, altered mental status, and permanent visual and cognitive impairment.24

Diagnostic Studies

Clinical examination is one of the most important diagnostic evaluations that can be conducted by the pediatrician. An abnormal examination finding or even a slight deviation from reference should not be minimized. If improvements are not seen in follow-up examinations performed within a

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month, further investigation and diagnostics are essential. An abnormal skull size, shape, or growth pattern is a strong indicator of a premature suture fusion. In the past decade, the clarity of this presentation has been complicated by the ongoing increase of infants and children with positional plagiocephaly. This is certainly one of the most common differential diagnoses. Positional plagiocephaly has increased dramatically since the “Back to Sleep” campaign was launched by the American Academy of Pediatrics in the early 1990s.25 It is caused by external forces affecting the skull for an extended period. It is estimated that 1 in 5 infants have experienced skull deformation caused by external pressure and positioning issues.26 The presentation of these patients has been confused with the presentation of patients with craniosynostosis. This can lead to significant delays in treatment of patients with craniosynostosis. For this reason, we discuss presentations and provide a reference for more details. Argenta et al provided a clinical classification of positional plagiocephaly from I to V.25 The severity can vary within each level, but the classification provides an organized means of evaluating patients and is most clearly noted by viewing the head from the top down. Type I involves only the back of the skull, without involvement of the ears, nose, or forehead. Type II involves the back of the skull and displacement of the ear on the same side (ipsilateral). Type III adds to the previous level, with the back of the skull, ear, and forehead all on the same side positioned in the anterior direction. This will create a parallelogram shape to the skull (Figure 2-2). Type IV adds to type III, with the addition of facial asymmetry that can involve soft tissue and even facial bones. Type V has deformity of the posterior skull, ear, and forehead, with facial asymmetry as with type IV, but it includes temporal bulging, with vertical growth of the posterior skull. Posterior brachycephaly presents with the entire back of the skull flat and can account for 15% of infants and children with cranial deformation.25 Plain radiography is able to show a loss of the visualization of the suture and fingerprinting or a copper-beaten appearance of the skull.27 The entire suture should be visualized because a partial closure can be present. In cases in which the probability of a partial closure is low, this examination can be helpful but is used as more of a screening tool than a standard-of-reference diagnostic modality. Plain radiographs will not be effective in the detection of early synostosis because of lack of bone mineralization in complex cases.28 It will also not be helpful in diagnosing intracranial anomalies or aiding in surgical planning. Ultrasonography is another screening tool for suture patency. It is inexpensive, does not expose the infant to radiation, and can be used in the prenatal setting or in infancy. The measurement of head circumference in relation to abdominal circumference can indicate an abnormality if it is lower than 1.07% or higher than 1.26%. If the cephalic index (the ratio of the biparietal diameter to the occipitofrontal diameter) is less than 75% or more

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21 Chapter 2: Craniosynostosis

A

B

Figure 2-2. Comparison of head shape differences with positional plagiocephaly. An external view (A) and an internal view (B) are shown. This illustrates a type III or IV plagiocephaly because the occiput, temporal bone, and frontal bone are involved. A type IV plagiocephaly would include changes in the facial structure, and type V would include temporal bulging and vertical growth of the posterior skull.

than 85%, there is a concern for dolichocephaly and brachycephaly, respectively.29 Unfortunately, the ossification centers do not become visible until the end of the first trimester, so for this purpose, ultrasonography is not useful until the second trimester.30 Computed tomography (CT) is effective in demonstrating bone ridge thickening, erosions of the bone, absence of opening between the sutures, and intracranial abnormalities such as hydrocephalus, hematoma, atrophy, and agenesis of the corpus callosum. The accuracy of 3-dimensional CT in the diagnosis of craniosynostosis is between 90% and 100%.28 Surgical reconstruction can be planned with CT, and it provides an accurate and reliable means of follow-up when indicated. While there is concern about the use of radiation in a growing infant, pediatric dosing can be used to minimize risk. Computed tomography protocols tailored to evaluate the calvarium only (sometimes referred to as “flash” CT) expose the patient to very low radiation dose levels. Magnetic resonance imaging is essential in the diagnosis of cerebral diseases, midline anomalies, parenchymal lesions, hydrocephalus, intracranial herniation, and dilation of the cerebral ventricles and in determining the severity of the condition.28 The advantage of this tool is the lack of radiation exposure, but one limitation is poor visualization of the bony structures. For the general pediatrician, a suspicion of craniosynostosis should warrant a baseline ultrasonographic examination (if the center has experience in that modality) and referral to a craniofacial surgeon.

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Nonsyndromic Craniosynostosis Sagittal Suture Synostosis Scaphocephaly, the premature fusion of the sagittal suture, is the most common type of single-suture synostosis. It occurs in 1 in 2,500 live births31 and has a 4:1 male to female predominance.32 The associated head shape is the most easily understood application of Virchow’s law, which states that the growth of the skull is restricted in the perpendicular direction to the fused suture and is increased in the parallel direction.20 The classic shape associated with sagittal synostosis is restricted growth in the side-to-side direction and expansion in the anterior-to-posterior direction (Figure 2-3). Specifically, it is characterized by a palpable sagittal ridge, narrowing in the temporal and parietal regions, frontal bossing, decreased posterior skull height, and occipital coning (Figure 2-4). Almost all these infants will have a large head circumference and a low cephalic index. Two other conditions with similar clinical findings must be distinguished from this, and these are

A

Normocephaly

C

B Scaphocephaly

Trigonocephaly

Fused suture

D

F

Unicoronal synostosis

E

Brachycephaly

Lambdoid synostosis

Figure 2-3. A–F, Skull shape changes associated with nonsyndromic single-suture craniosynostosis.

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23 Chapter 2: Craniosynostosis

A

C

B

D

Figure 2-4. Infant with sagittal craniosynostosis. A and B, sagittal and axial views, respectively, of the reconstructed 3-dimensional computed tomographic scan; C and D, clinical photos from the superior and posterior directions, respectively.

the secondary effects of preterm birth or ventriculoperitoneal shunting, as these do not usually warrant surgical intervention.

Metopic Suture Synostosis Trigonocephaly, the premature fusion of the metopic suture, classically manifests with a triad of symptoms, including a palpable metopic ridge, hypotelorism, and triangulation of the forehead (see Figure 2-3). It has become the second most common type of single-suture synostosis in recent years and occurs as frequently as 1 in every 10,000 live births.33 The metopic suture is the first cranial suture of significance to close and can close normally as early as 9 to 12 months of age.34 Additional clinical characteristics include depression of the superior lateral orbital rims and bitemporal narrowing of the skull, along with increased posterior width of the skull (Figure 2-5). This condition needs to be distinguished from isolated ridging, with none of the other findings or ridging caused by isolated brain-push problems, such as those seen with severe microcephaly. Unicoronal Suture Craniosynostosis Unicoronal craniosynostosis is the premature fusion of one of the 2 coronal sutures and is more common in boys than girls, at a ratio of 3:2 (see Figure 2-3).32

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Figure 2-5. Reconstructed 3-dimensional computed tomographic scan of patient with metopic craniosynostosis, viewed from the axial plane.

It is the third most common type of craniosynostosis, occurring in 1 in 15,000 live births. 33,35 Clinical findings are focused on the effects around the orbit, and as many as 90% of patients have subtle extraocular muscle irregularity that may require patching or surgical intervention. 36 Classic findings include flattening of the ipsilateral forehead and front parietal region, with compensatory bulging of the contralateral forehead. The eye contralateral to the unicoronal synostosis has a greater risk of amblyopia because of the higher amount and rate of ocular astigmatism. Elevation of the ipsilateral orbital rim is seen, as well as deviation of the nasal radix toward the side of the fused suture and the nasal tip, away toward the contralateral side (Figure 2-6). On plain radiographs, the orbital findings are described as a harlequin eye deformity (Figure 2-7). Severity of clinical presentation may be dependent on where suture fusion starts and whether there is involvement of the squamosal suture. 37 This explains why milder cases of premature fusion are difficult to diagnose and may manifest late.

Bicoronal Suture Craniosynostosis Bicoronal synostosis, the premature fusion of both coronal sutures, is the most common presentation for many of the syndromic craniosynostoses. Clinical characteristics include bilateral coronal ridging, shortening of the anteroposterior length of the skull, flattening of the occiput, skull widening, and increased height of the skull (Figure 2-8). In some cases, orbital proptosis is seen, and the eyes appear more prominent. Severity of presentation is dependent on the associated syndrome.

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25 Chapter 2: Craniosynostosis

A

B

C

Figure 2-6. Infant with unicoronal craniosynostosis. Reconstructed 3-dimensional computed tomographic scans viewed from the frontal (A) and superior (B) planes. C, Clinical photograph.

Lambdoid Suture Synostosis Lambdoid suture synostosis is the least common type of single-suture synostosis, occurring in 1 in 100,000 live births (see Figure 2-3).38 It rarely occurs bilaterally, and when it does, it is typically associated with the so-called Mercedes Benz deformity, involving the sagittal suture and both lambdoid sutures. Clinical characteristics include ridging of the lambdoid suture, ipsilateral parietal occipital flattening, prominent mastoid on the affected side, posterior displacement of the ipsilateral ear, and contralateral parietal occipital compensatory bulging (Figure 2-9). There are minimal forehead asymmetries seen with true lambdoid synostosis. This diagnosis is typically associated with scoliosis of the skull base and curvature of the cervical spine.39 This presentation is often confused with positional or deformational plagiocephaly.40,41 The easiest way to distinguish between the two is

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Figure 2-7. Harlequin eye deformity on a frontal skull radiograph.

Figure 2-8. Reconstructed 3-dimensional computed tomographic scan in a patient with bicoronal craniosynostosis. The skull is obliquely oriented.

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27 Chapter 2: Craniosynostosis

B

A

C

Figure 2-9. Infant with lambdoid craniosynostosis. A and B, Clinical photos. C, Reconstructed 3-dimensional computed tomographic scan viewed from the coronal plane.

the resulting shape. Deformational plagiocephaly results in a parallelogram shape, with ipsilateral anterior ear and forehead displacement and contralateral forehead recession and occipital bossing. Lambdoid suture synostosis results in a trapezoidal shape caused by the ear being pulled toward the fused suture (see Figure 2-2).

Multiple Suture Synostoses Multiple suture involvement is sometimes associated with a syndromic diagnosis, as seen in many bicoronal cases. The 2 most common presentations for bi-sutural involvement after bicoronal involvement are metopic combined with sagittal and unicoronal combined with sagittal. The most common tri-sutural involvement is the so-called Mercedes Benz deformity, consisting of fusion of the sagittal suture and both lambdoid sutures (see Figures 2-3 and 2-10). The least common 3-suture involvement is a Z pattern involving the unicoronal, sagittal, and contralateral lambdoid sutures.

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Figure 2-10. Reconstructed 3-dimensional computed tomographic scan of a child with so-called Mercedes Benz deformity—fusions of the sagittal and both lambdoid sutures. The posterior aspect of the skull is shown.

Multiple-suture fusion is more commonly related to increased ICP and is more likely to develop a Chiari malformation. Multiple-suture fusion does not appear to be caused by gene mutations.42 The increase in ICP causes erosive changes through the cranial bones (Figure 2-11). The most severe form of the multiple suture synostoses is the cloverleaf skull deformity, also known as the Kleeblattschädel skull deformity. This is associated with pansynostosis and has a very high associated morbidity and mortality.43

Syndromic Craniosynostosis Apert Syndrome Apert syndrome (acrocephalosyndactyly type I) was first described in 1906 by Eugene Apert. It occurs in 1 in 100,000 to 1 in 160,000 live births and is associated with increased paternal age (Figure 2-12).13,44–46 It is an autosomal dominant condition associated with an FGFR2 mutation, but one-half of the cases are believed to be spontaneous mutations.47 Apert syndrome is characterized by bicoronal craniosynostosis, a turribrachycephaly (or tower-shaped skull), midface hypoplasia, and complex syndactyly of the hands and feet. A high arched palate is seen with a longer soft palate that is often associated with clefts.48 This diagnosis is associated with variable degrees of developmental delay and an increased incidence of increased ICP

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29 Chapter 2: Craniosynostosis

A

B

C

Figure 2-11. An infant with multi-sutural synostosis causing erosion of the cranial bones. A, Clinical photograph. B, Axial computed tomographic (CT) scan and C, reconstructed 3-dimensional CT scan, as viewed from the sagittal plane.

in as many as 45% of cases.49 There is no absolute consensus on the optimal time for surgical intervention for the premature fusion of the coronal sutures in this setting. The range is from 4 to 15 months of age; however, the authors recommend the younger age (4–6 months) for optimal correction. This allows for more normal growth prior to 1 year of age, when the skull is better able to regenerate bone.

Crouzon Syndrome Crouzon syndrome (craniofacial dysostosis) was first described by French physician, Octave Crouzon, in 1912 and occurs in 1 in 65,000 live births.12,50 It is an autosomal dominant condition, with many of the cases showing spontaneous mutations. It is associated with an FGFR2 and FGFR3 gene mutation, which dictates the clinical presentation.47 The mutations on the FGFR2 gene have further been mapped to the 10q25-q26 chromosome location.51 Clinical presentation is a brachycephalic head shape caused by the coronal craniosynostosis, midface hypoplasia, and shallow orbits that result in prominent globes. Patients often present with proptosis caused by the shallow orbits, which can lead to conjunctivitis owing to an inability to close the eyes and resultant exposure keratopathy. The scarring of the

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30 Pediatric Plastic and Reconstructive Surgery for Primary Care

A

B

C

Figure 2-12. Clinical photos of a patient with Apert syndrome. Note the complex syndactyly of the hands.

cornea can cause blindness. Secondary conjunctivitis is a sign of the inflammation from the exposure keratopathy.52 It is reported that 9% to 26% of these patients have hydrocephalus and secondary problems with headaches and seizures.53,54

Pfeiffer Syndrome Pfeiffer syndrome is also autosomal dominant in inheritance and a result of either an FGFR1 or FGFR2 mutation.47 It was originally described by Rudolph Pfeiffer in 1964 and occurs in 1 in 100,000 live births.55 It is characterized by bicoronal craniosynostosis, turribrachycephaly, hypertelorism, midface hypoplasia, and extremity abnormalities, including broad thumbs and toes. In severe forms, patients can have intestinal malrotation and multiple sutures prematurely fused with a cloverleaf skull deformity (Kleeblattschädel skull deformity; see Figure 2-11).

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31 Chapter 2: Craniosynostosis

Muenke Syndrome All children with coronal synostosis should be tested for Muenke syndrome (Figure 2-13). It is an FGFR3 genetic mutation that is autosomal dominant and occurs in 1 in 30,000 live births.56 It more commonly manifests with bicoronal involvement than unicoronal suture fusion. It is associated with macrocephaly and mild to moderate midface hypoplasia with ocular hypertelorism.57 Up to one-third of these infants will have developmental delay.58 Additional functional problems include neurosensory hearing loss and strabismus, which are both common in this patient population.58,59 Chotzen Syndrome Chotzen syndrome is an autosomal dominant condition (acrocephalosyndactyly type III) that is associated with either a TWIST 1 or an FGFR2 gene mutation.47 It was originally described in 1931 by Saethre and Chotzen.60,61 It occurs in 1 in 25,000 to 1 in 50,000 live births and is the most common form of syndromic craniosynostosis.62 It commonly manifests with bicoronal craniosynostosis, hypertelorism, a low-set forehead hairline, ear abnormalities that include a small pinna and prominent superior crus, strabismus,

Figure 2-13. Clinical photographs of an infant with Muenke syndrome.

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32 Pediatric Plastic and Reconstructive Surgery for Primary Care

ptosis, a depressed nasal dorsum, cleft palate, and incomplete simple syndactyly of the hands.60,61,63 Increased ICP is seen in up to 21% of cases and warrants a workup.64

Carpenter Syndrome Carpenter syndrome is an autosomal recessive condition associated with an RAB23 gene mutation.47 It was first described in 1909 by Carpenter, in a case in which a brother and 2 sisters were affected.65 Suture involvement has been described to include the coronal, sagittal, and lambdoid sutures and often manifests with more than one suture involved.66 Additional clinical characteristics include obesity, cardiac and renal defects, precocious puberty, mental delay, orbital defects, and hypoplastic and low-set ears.53,67

Surgical Management Informed Consent Regardless of the surgical approach, technique used, or age at surgery, there is a significant potential for surgical complications that must be addressed with the family. Blood loss is certainly among these, and owing to the size of the pediatric patient, blood replacement is often necessary in the perioperative period. Adjuncts such as erythropoietin, tranexamic acid, and the use of cell salvage to minimize the need for transfusion are available, but there are no definitive data to support the use of one over the other.68 The risk of infection is low because of the increased vascularity of the brain and scalp, but most advocate the use of perioperative antibiotics at a minimum. Increased postoperative temperatures are not uncommon but are usually caused by meningeal irritation or postoperative atelectasis rather than true infection. The potential for dural injury is also an issue, especially in patients with syndromic synostosis in whom increased ICP is more likely and copper-beaten skull deformities are seen. Scarring is most problematic in the temporal region of the incision. As such, numerous attempts to minimize this have been tried, and the use of a curved or zigzag incision seems to be helpful. Rarer complications include coagulopathy, which is usually seen in older pediatric patients who have highvolume blood loss and replacement; however, all patients should be evaluated with coagulation studies in the perioperative period. Postoperative concerns may include persistent bony defects, especially in children treated after 1 year of age, when the defects will not be re-ossified on their own and will likely require bone substitutes to be used at the time of surgery. The most frequent abnormalities seen in the late postoperative period are contour abnormalities that may require surgery when the patient is finished growing to address the asymmetry, such as temporal hollowing.

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33 Chapter 2: Craniosynostosis

Procedural Specifics The anesthesia team is a significant part of the success of any craniofacial surgery, regardless of the technique chosen. Preoperative planning and intraoperative care, such as intra-arterial monitoring, adequate intravenous access, and hyperventilation during the craniotomy portion of the procedure, as well as adequate fluid and blood replacement, are essential. Positioning for the procedure is dependent on suture involvement. The most challenging patients need the anterior and posterior skull addressed, such as those with turricephaly and severe scaphocephaly. In some cases, this may require a 2-stage approach to be able to access both areas safely. Incision choice is dependent on the procedure and the bicoronal approach. Either a curved or a zigzag incision is used for the more involved procedures, while the minimally invasive procedures have the advantage of a much smaller incision. Procedure choice is, of course, patient and surgeon specific. Volumes have been written on this topic, and there is still no consensus on which approach is likely to be most successful, owing to the fact that when a patient is in the right hands, there is more than one good option. The less severe deformities that manifest earlier are often good candidates for minimally invasive approaches, such as spring cranioplasty or strip craniectomy with postoperative banding therapy.69,70 This is most commonly used in single-suture deformities that manifest prior to 6 months of age, in patients who have good parental compliance. A more comprehensive open approach is needed in more complex cases, such as syndromic cases and cases in which the deformities manifest after 6 months of age. In these situations, the forehead is advanced and the skull expanded after an extensive craniectomy. Regardless of the technique used, the goals are the same and include release of the fused sutures and remodeling of the skull abnormalities that result from the fusion. In addition, it is important to minimize long-term secondary effects from the surgery, such as bony defects, contour abnormalities, or problems from the fixation techniques used on the bones. It has become apparent over the past decade that metal plating material should be avoided in infants and children, owing to the propensity to migrate intracranially over time. Alternative options include suture fixation in infants and younger children and absorbable plating material in older children. Postoperatively, these patients are monitored closely, and for the more invasive and longer procedures, this requires monitoring in the intensive care unit. These patients are watched closely for blood loss, neurological status, fluid resuscitation, and pain management. Fortunately, the perioperative morbidity and mortality are low. It is often many years before the true success of the surgery is known; for this reason, the importance of follow-up cannot be overstated.

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34 Pediatric Plastic and Reconstructive Surgery for Primary Care

Long-term Management Optimization

The importance of a craniofacial team in the treatment of children with craniosynostosis cannot be stressed enough. Ideally, this team would consist of an audiologist, dentist, orthodontist, geneticist, neurosurgeon, plastic surgeon, ophthalmologist, orthopedic surgeon, otolaryngologist, geneticist, pediatrician, developmental pediatrician, psychologist, nutritionist and/or feeding specialist, speech pathologist, occupational and physical therapist, and social worker. For a patient with single-suture synostosis, a single surgical procedure is often all that is needed, and these patients can be watched for changes with growth. For a patient with syndromic craniosynostosis, this is often not the case. These patients will often need multiple procedures for the associated deformities. Patients with syndromic craniosynostosis will often need surgery to correct their midface and jaw deformities after growth is complete. Occasionally, this will need to be done much earlier and prior to completion of growth, if issues of globe exposure or airway compromise need to be addressed. Timing and surgical planning require many of the subspecialists listed herein. Many of these children will face issues with their peers and self-esteem, and the help of the psychologist and social worker is often very important for these families. In addition, the geneticist is instrumental in family planning, not just for the parents of the affected child but also for the child when he or she is old enough to have children. Communication among team members is crucial for the optimization of each patient’s outcome.52 REFERENCES

1. Cohen MM, MacLean RE. Craniosynostosis. Diagnosis, evaluation, and management. 2nd ed. New York, NY: Oxford University Press; 2000 2. Alderman BW, Lammer EJ, Joshua SC, et al. An epidemiologic study of craniosynostosis: risk indicators for the occurrence of craniosynostosis in Colorado. Am J Epidemiol. 1988;128(2): 431–438 PMID: 3394707 https://doi.org/10.1093/oxfordjournals.aje.a114983 3. Kweldam CF, van der Vlugt JJ, van der Meulen JJNM. The incidence of craniosynostosis in the Netherlands, 1997-2007. J Plast Reconstr Aesthet Surg. 2011;64(5):583–588 PMID: 20888312 https://doi.org/10.1016/j.bjps.2010.08.026 4. Singer S, Bower C, Southall P, Goldblatt J. Craniosynostosis in Western Australia, 1980-1994: a population-based study. Am J Med Genet. 1999;83(5):382–387 PMID: 10232748 https://doi. org/10.1002/(SICI)1096-8628(19990423)83:53.0.CO;2-A 5. Di Rocco F, Arnaud E, Renier D. Evolution in the frequency of non-syndromic craniosynostosis. J Neurosurg Pediatr. 2009;4:21–25 6. Baranello G, Vasco G, Ricci D, Mercuri E. Visual function in nonsyndromic craniosynostosis: past, present, and future. Childs Nerv Syst. 2007;23(12):1461–1465 PMID: 17701186 https://doi. org/10.1007/s00381-007-0435-1 7. Kolar JC. An epidemiological study of nonsyndromal craniosynostoses. J Craniofac Surg. 2011; 22(1):47–49 PMID: 21187784 https://doi.org/10.1097/SCS.0b013e3181f6c2fb 8. Gault DT, Renier D, Marchac D, Jones BM. Intracranial pressure and intracranial volume in children with craniosynostosis. Plast Reconstr Surg. 1992;90(3):377–381 PMID: 1513883 https:// doi.org/10.1097/00006534-199209000-00003 9. Renier D, Sainte-Rose C, Marchac D, Hirsch JF. Intracranial pressure in craniostenosis. J Neurosurg. 1982;57(3):370–377 PMID: 7097333 https://doi.org/10.3171/jns.1982.57.3.0370 10. Seruya M, Oh AK, Boyajian MJ, Posnick JC, Keating RF. Treatment for delayed presentation of sagittal synostosis: challenges pertaining to occult intracranial hypertension. J Neurosurg Pediatr. 2011;8(1):40–48 PMID: 21721888 https://doi.org/10.3171/2011.4.PEDS1160

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35 Chapter 2: Craniosynostosis 11. Chung SA, Yun IS, Moon JW, Lee JB. Ophthalmic findings in children with nonsyndromic craniosynostosis treated by expansion cranioplasty. J Craniofac Surg. 2015;26(1):79–83 PMID: 25569390 https://doi.org/10.1097/SCS.0000000000001238 12. Cunningham ML, Seto ML, Ratisoontorn C, Heike CL, Hing AV. Syndromic craniosynostosis: from history to hydrogen bonds. Orthod Craniofac Res. 2007;10(2):67–81 PMID: 17552943 https://doi.org/10.1111/j.1601-6343.2007.00389.x 13. Lajeunie E, Heuertz S, El Ghouzzi V, et al. Mutation screening in patients with syndromic craniosynostoses indicates that a limited number of recurrent FGFR2 mutations accounts for severe forms of Pfeiffer syndrome. Eur J Hum Genet. 2006;14(3):289–298 PMID: 16418739 https://doi.org/10.1038/sj.ejhg.5201558 14. Whitaker LA, Pashayan H, Reichman J. A proposed new classification of craniofacial anomalies. Cleft Palate J. 1981;18(3):161–176 PMID: 6941862 15. Maliepaard M, Mathijssen IM, Oosterlaan J, Okkerse JM. Intellectual, behavioral, and emotional functioning in children with syndromic craniosynostosis. Pediatrics. 2014;133(6):e1608–e1615 PMID: 24864183 https://doi.org/10.1542/peds.2013-3077 16. Moss ML. The pathogenesis of premature cranial synostosis in man. Acta Anat (Basel). 1959; 37(4):351–370 PMID: 14424622 https://doi.org/10.1159/000141479 17. Malcolm S, Reardon W. Fibroblast growth factor receptor-2 mutations in craniosynostosis. Ann N Y Acad Sci. 1996;785(1):164–170 PMID: 8702123 https://doi.org/10.1111/j.1749-6632.1996. tb56255.x 18. Warren SM, Longaker MT. Re: regeneration of the sagittal suture by GTR and its impact on growth of the cranial vault. J Craniofac Surg. 2001;12(2):197–199 PMID: 11314633 https://doi. org/10.1097/00001665-200103000-00019 19. Slater BJ, Lenton KA, Kwan MD, Gupta DM, Wan DC, Longaker MT. Cranial sutures: a brief review. Plast Reconstr Surg. 2008;121(4):170e–178e PMID: 18349596 https://doi.org/10.1097/01. prs.0000304441.99483.97 20. Delashaw JB, Persing JA, Broaddus WC, Jane JA. Cranial vault growth in craniosynostosis. J Neurosurg. 1989;70(2):159–165 PMID: 2913214 https://doi.org/10.3171/jns.1989.70.2.0159 21. Thomas GP, Johnson D, Byren JC, et al. The incidence of raised intracranial pressure in nonsyndromic sagittal craniosynostosis following primary surgery. J Neurosurg Pediatr. 2015;15(4):350–360 PMID: 25559921 https://doi.org/10.3171/2014.11.PEDS1426 22. Pollack IF, Losken HW, Biglan AW. Incidence of increased intracranial pressure after early surgical treatment of syndromic craniosynostosis. Pediatr Neurosurg. 01996;24(4):202–209 PMID: 8873162 https://doi.org/10.1159/000121038 23. Baird LC, Gonda D, Cohen SR, et al. Craniofacial reconstruction as a treatment for elevated intracranial pressure. Childs Nerv Syst. 2012;28(3):411–418 PMID: 22068642 https://doi.org/ 10.1007/s00381-011-1615-6 24. Stavrou P, Sgouros S, Willshaw HE, Goldin JH, Hockley AD, Wake MJC. Visual failure caused by raised intracranial pressure in craniosynostosis. Childs Nerv Syst. 1997;13(2):64–67 PMID: 9105738 https://doi.org/10.1007/s003810050043 25. Argenta L, David L, Thompson J. Clinical classification of positional plagiocephaly. J Craniofac Surg. 2004;15(3):368–372 PMID: 15111792 https://doi.org/10.1097/00001665-200405000-00004 26. van Vlimmeren LA, van der Graaf Y, Boere-Boonekamp MM, L’Hoir MP, Helders PJ, Engelbert RH. Risk factors for deformational plagiocephaly at birth and at 7 weeks of age: a prospective cohort study. Pediatrics. 2007;119(2):e408–e418 PMID: 17272603 https://doi.org/10.1542/ peds.2006-2012 27. Benson ML, Oliverio PJ, Yue NC, Zinreich SJ. Primary craniosynostosis: imaging features. AJR Am J Roentgenol. 1996;166(3):697–703 PMID: 8623653 https://doi.org/10.2214/ajr.166. 3.8623653 28. Kotrikova B, Krempien R, Freier K, Mühling J. Diagnostic imaging in the management of craniosynostoses. Eur Radiol. 2007;17(8):1968–1978 PMID: 17151858 https://doi.org/10.1007/ s00330-006-0520-y 29. Jeanty P, Romero R. Obstetrical Ultrasound. Maidenhead, England: McGraw-Hill; 1984:91–99 30. Miller C, Losken HW, Towbin R, et al. Ultrasound diagnosis of craniosynostosis. Cleft Palate Craniofac J. 2002;39(1):73–80 PMID: 11772173 https://doi.org/10.1597/1545-1569(2002) 0392.0.CO;2

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36 Pediatric Plastic and Reconstructive Surgery for Primary Care 31. Cohen MM Jr. Sutural biology and the correlates of craniosynostosis. Am J Med Genet. 1993; 47(5):581–616 PMID: 8266985 https://doi.org/10.1002/ajmg.1320470507 32. Persing JA. MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis. Plast Reconstr Surg. 2008;121(4)(suppl):1–11 PMID: 18379381 https://doi. org/10.1097/01.prs.0000305929.40363.bf 33. van der Meulen J, van der Hulst R, van Adrichem L, et al. The increase of metopic synostosis: a pan-European observation. J Craniofac Surg. 2009;20(2):283–286 PMID: 19326483 https://doi. org/10.1097/SCS.0b013e31818436be 34. Weinzweig J, Kirschner RE, Farley A, et al. Metopic synostosis: Defining the temporal sequence of normal suture fusion and differentiating it from synostosis on the basis of computed tomography images. Plast Reconstr Surg. 2003;112(5):1211–1218 PMID: 14504503 https://doi. org/10.1097/01.PRS.0000080729.28749.A3 35. Selber J, Reid RR, Chike-Obi CJ, et al. The changing epidemiologic spectrum of single-suture synostoses. Plast Reconstr Surg. 2008;122(2):527–533 PMID: 18626371 https://doi.org/10.1097/ PRS.0b013e31817d548c 36. Newman SA. Ophthalmic features of craniosynostosis. Neurosurg Clin N Am. 1991;2(3):587–610 PMID: 1821306 https://doi.org/10.1016/S1042-3680(18)30721-6 37. Showalter BM, David LR, Argenta LC, Thompson JT. Influence of frontosphenoidal suture synostosis on skull dysmorphology in unicoronal suture synostosis. J Craniofac Surg. 2012;23(6):1709–1712 PMID: 23147332 https://doi.org/10.1097/SCS.0b013e31826beecc 38. Menard RM, David DJ. Regarding true lambdoid synostosis: long-term results of surgical and conservative therapy. Plast Reconstr Surg. 2008;122(2):673 PMID: 18626400 https://doi. org/10.1097/PRS.0b013e31817d62b3 39. Huang MH, Gruss JS, Clarren SK, et al. The differential diagnosis of posterior plagiocephaly: true lambdoid synostosis versus positional molding. Plast Reconstr Surg. 1996;98(5):765–774 PMID: 8823012 https://doi.org/10.1097/00006534-199610000-00001 40. Argenta LC, David LR, Wilson JA, Bell WO. An increase in infant cranial deformity with supine sleeping position. J Craniofac Surg. 1996;7(1):5–11 PMID: 9086895 https://doi.org/10.1097/ 00001665-199601000-00005 41. Argenta LC, David LR. Observations and thoughts on the changing constellation of cranial deformities. J Craniofac Surg. 1998;9(6):491–492 PMID: 10029760 https://doi.org/10.1097/ 00001665-199811000-00002 42. Czerwinski M, Kolar JC, Fearon JA. Complex craniosynostosis. Plast Reconstr Surg. 2011; 128(4):955–961 PMID: 21681124 https://doi.org/10.1097/PRS.0b013e3182268ca6 43. Comings DE. The Kleeblattschädel syndrome: a grotesque form of hydrocephalus. J Pediatr. 1965;67(1):126–129 PMID: 14302138 https://doi.org/10.1016/S0022-3476(65)80314-6 44. Fearon JA, Podner C. Apert syndrome: evaluation of a treatment algorithm. Plast Reconstr Surg. 2013;131(1):132–142 PMID: 23271523 https://doi.org/10.1097/PRS.0b013e3182729f42 45. Sisco M, Bauer BS. Craniofacial syndromes and sequences. In: Bentz ML, Bauer BS, Zucker R, eds. Principles and Practice of Pediatric Plastic Surgery. Vol 1. St Louis, MO: Quality Medical; 2008 46. Wilkie AO, Slaney SF, Oldridge M, et al. Apert syndrome results from localized mutations of FGFR2 and is allelic with Crouzon syndrome. Nat Genet. 1995;9(2):165–172 PMID: 7719344 https://doi.org/10.1038/ng0295-165 47. Jezela-Stanek A, Krajewska-Walasek M. Genetic causes of syndromic craniosynostoses. Eur J Paediatr Neurol. 2013;17(3):221–224 PMID: 23062756 https://doi.org/10.1016/j.ejpn.2012.09.009 48. Kreiborg S, Cohen MM Jr. The oral manifestations of Apert syndrome. J Craniofac Genet Dev Biol. 1992;12(1):41–48 PMID: 1572940 49. Renier D, Lajeunie E, Arnaud E, Marchac D. Management of craniosynostoses. Childs Nerv Syst. 2000;16(10-11):645–658 PMID: 11151714 https://doi.org/10.1007/s003810000320 50. Crouzon O. Dysostose cranio-faciale hereditaire. Bull Mem Soc Med Hop Paris. 1912;33:545–555 51. Reardon W, Winter RM, Rutland P, Pulleyn LJ, Jones BM, Malcolm S. Mutations in the fibroblast growth factor receptor 2 gene cause Crouzon syndrome. Nat Genet. 1994;8(1):98–103 PMID: 7987400 https://doi.org/10.1038/ng0994-98 52. Ganesh A, Edmond J, Forbes B, et al. An update of ophthalmic management in craniosynostosis. J AAPOS. 2019;23(2):66–76 PMID: 30928366 https://doi.org/10.1016/j.jaapos.2018.10.016

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37 Chapter 2: Craniosynostosis 53. Wilkie AO. Craniosynostosis: genes and mechanisms. Hum Mol Genet. 1997;6(10):1647–1656 PMID: 9300656 https://doi.org/10.1093/hmg/6.10.1647 54. Collmann H, Sörensen N, Krauss J. Hydrocephalus in craniosynostosis: a review. Childs Nerv Syst. 2005;21(10):902–912 PMID: 15864600 https://doi.org/10.1007/s00381-004-1116-y 55. Pfeiffer RA. Dominant hereditary acrocephalosyndactylia. Z Kinderheilkd. 1964;90(4):301–320 PMID: 14316612 https://doi.org/10.1007/BF00447500 56. Agochukwu NB, Doherty ES, Muenke M. Muenke syndrome. In: Pagon RA, Bird TD, Dolan CR, Stephens K, eds. GeneReviews. Seattle, WA: University of Washington; 1993 57. Reinhart E, Eulert S, Bill J, Würzler K, Phan The L, Reuther J. [Typical features of craniofacial growth of the FGFR3-associated coronal synostosis syndrome (so-called Muenke craniosynostosis)] [in German]. Mund Kiefer Gesichtschir. 2003;7(3):132–137 PMID: 12764678 https://doi. org/10.1007/s10006-002-0447-7 58. Muenke M, Gripp KW, McDonald-McGinn DM, et al. A unique point mutation in the fibroblast growth factor receptor 3 gene (FGFR3) defines a new craniosynostosis syndrome. Am J Hum Genet. 1997;60(3):555–564 PMID: 9042914 59. Doherty ES, Lacbawan F, Hadley DW, et al. Muenke syndrome (FGFR3-related craniosynostosis): expansion of the phenotype and review of the literature. Am J Med Genet A. 2007;143A(24): 3204–3215 PMID: 18000976 https://doi.org/10.1002/ajmg.a.32078 60. Saethre M. Ein beitrag zum turmschaedelproblem (pathogenese, erblichkeit und symptomatologie). Dtsch Z Nervenheilkd. 1931;119(1):533–555 https://doi.org/10.1007/BF01673869 61. Chotzen F. Eine eigenartige familiaere entwicklungsstoerung (akrocephalosyndaktylie, dysostosis craniofacialis und hyperteloismus). Mschr Kinderheilk. 1932;55:97–122 62. Gallagher ER, Ratisoontorn C, Cunningham ML. Saethre-Chotzen syndrome. In: Pagon RA, Bird TD, Dolan CR, Stephens K, eds. GeneReviews. Seattle, WA: University of Washington; 1993 63. Pantke OA, Cohen MM Jr, Witkop CJ Jr, et al. The Saethre-Chotzen syndrome. Birth Defects Orig Artic Ser. 1975;11(2):190–225 PMID: 1227525 64. de Jong T, Bannink N, Bredero-Boelhouwer HH, et al. Long-term functional outcome in 167 patients with syndromic craniosynostosis; defining a syndrome-specific risk profile. J Plast Reconstr Aesthet Surg. 2010;63(10):1635–1641 PMID: 19913472 https://doi.org/10.1016/ j.bjps.2009.10.029 65. Carpenter G. Acrocephaly, with other congenital malformations. Proc R Soc Med. 1909;2(Sect Study Dis Child):45–53 PMID: 19974019 66. Caouette-Laberge L, Bayet B, Larocque Y. The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities. Plast Reconstr Surg. 1994;93(5):934–942 PMID: 8134485 https://doi.org/10.1097/00006534-199404001-00006 67. Frias JL, Felman AH, Rosenbloom AL, et al. Normal intelligence in two children with Carpenter syndrome. Am J Med Genet. 1978;2(2):191–199 PMID: 263437 https://doi.org/10.1002/ajmg. 1320020210 68. Crantford JC, Wood BC, Claiborne JR, et al. Evaluating the safety and efficacy of tranexamic acid administration in pediatric cranial vault reconstruction. J Craniofac Surg. 2015;26(1):104–107 PMID: 25534062 https://doi.org/10.1097/SCS.0000000000001271 69. David LR, Plikaitis CM, Couture D, Glazier SS, Argenta LC. Outcome analysis of our first 75 spring-assisted surgeries for scaphocephaly. J Craniofac Surg. 2010;21(1):3–9 PMID: 20061981 https://doi.org/10.1097/SCS.0b013e3181c3469d 70. Jimenez DF, Barone CM. Endoscopic techniques for craniosynostosis. Atlas Oral Maxillofac Surg Clin North Am. 2010;18(2):93–107 PMID: 21036312 https://doi.org/10.1016/j.cxom.2010.08.004

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CHAPTER

3

Vascular Anomalies ARIN K. GREENE, MD, MMSc

Introduction

Vascular anomalies are disorders that affect capillaries, arteries, veins, or lymphatics. They are common, affecting approximately 5.5% of the population.1 The field is confusing because lesions look similar, and imprecise terminology is used.2 Vascular anomalies are classified biologically, on the basis of their clinical behavior and cellular characteristics (Table 3-1).3 By using the biological classification, at least 90% of lesions can be diagnosed on the basis of history and physical examination. There are 2 broad types of vascular anomalies: tumors and malformations.4 Tumors demonstrate endothelial proliferation and affect approximately 5% of the population. There are 4 major lesions: infantile hemangioma (IH), congenital hemangioma, kaposiform hemangioendothelioma, and pyogenic granuloma (Figure 3-1). Vascular malformations are errors in vascular development that have minimal endothelial turnover; they affect approximately 0.5% of the population. There are 4 major categories based on the anomalous vessel(s): capillary malformation, lymphatic malformation, venous malformation, and arteriovenous malformation (Figure 3-2).

Table 3-1. Classification of Vascular Anomalies Malformations Tumors

Slow Flow

Fast Flow

Overgrowth Syndrome

Infantile hemangioma

Capillary malformation

Arteriovenous malformation

CLOVES

Congenital hemangioma

Lymphatic malformation

Klippel-Trénaunay

Kaposiform hemangioendothelioma

Venous malformation

Parkes Weber

Pyogenic granuloma

Sturge-Weber

Abbreviation: CLOVES, congenital lipomatosis, overgrowth, vascular malformation, epidermal nevi, and scoliosis/skeletal/spinal anomalies.

39

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40 Pediatric Plastic and Reconstructive Surgery for Primary Care

A

B

C

D

Figure 3-1. Examples of the 4 major types of vascular tumors. A, Infantile hemangioma. B, Congenital hemangioma. C, Kaposiform hemangioendothelioma. D, Pyogenic granuloma.

Vascular Tumors Infantile Hemangioma Clinical Features Infantile hemangioma is the most common tumor of infancy, affecting 4% to 5% of newborns and infants.5 Infantile hemangioma is more frequent in preterm newborns and infants and in girls (female to male ratio of 4:1). The median age of appearance is 2 weeks; 50% of IH cases are noted at birth as a telangiectatic stain, pale spot, or ecchymotic area. Infantile hemangioma grows faster than the infant during the first 9 months after birth (“proliferating phase”); 80% of its size is achieved by 3.2 ± 1.7 months.6 Infantile hemangioma is red when it involves the superficial dermis. A lesion beneath the skin may not be observed until 3 to 4 months of age, when it has grown large enough to cause a visible mass; the overlying skin may appear bluish. By the age of 9 to 12 months, growth of IH reaches a plateau. After 12 months, the tumor begins to regress (“involuting phase”); the color fades, and the lesion flattens. Involution ceases in most children by the age of 4 years (“involuted phase”).7 After involution, one-half of patients will have residual telangiectasias, scarring, fibrofatty residuum, redundant skin, or remodeled anatomical structures. Most cases of

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41 Chapter 3: Vascular Anomalies

A

C

B

D

Figure 3-2. Examples of the 4 major types of vascular malformations. A, Capillary malformation. B, Lymphatic malformation. C, Venous malformation. D, Arteriovenous malformation.

IH are small, harmless lesions that can be monitored by the pediatrician. However, a minority of proliferating IHs can cause clinically significant deformity or complications, usually when located on the head or neck. Infants with 5 or more small (< 5 mm) tumors are at risk for hepatic lesions. Imaging for suspected IH is not routinely performed, except in cases in which the diagnosis is questionable, there are 5 or more cutaneous IHs, or there are suspected associated anatomical abnormalities. Most IHs can be diagnosed clinically. When imaging is indicated, ultrasonography (US) with Doppler imaging is often the initial study of choice. Ultrasonography can show a characteristic pattern for hemangiomas and document flow within the lesion without needing to sedate the patient or expose the patient to radiation, which makes it a good initial study of choice. Magnetic resonance (MR) imaging and MR angiography, which often require sedation, can often be used to better define the extent of the lesion and the feeding vessels. At MR imaging, hemangiomas are generally high-flow, solid lesions, often with tubular flow voids. With T1-weighted signaling, they have intermediate signal intensity, while with T2-weighted signaling, they have high signal intensity. They also enhance with the use of gadolinium-based contrast material. This is in distinction to venous malformations that are generally low-flow lesions that demonstrate low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.

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42 Pediatric Plastic and Reconstructive Surgery for Primary Care

PHACE association affects 2.3% of patients with IH and consists of a plaque-like IH in a regional distribution of the face, with at least one of the following anomalies: posterior fossa brain malformation, hemangioma, arterial cerebrovascular anomalies, coarctation of the aorta and cardiac defects, and eye/endocrine abnormalities.8 Because 8% of infants with PHACE association have a stroke in infancy, these patients should undergo MR imaging with appropriate vessel imaging techniques, such as MR angiography and sequences used to investigate the posterior fossa contents to investigate the brain cerebral vasculature in an optimal fashion. LUMBAR association (lower body IH, urogenital anomalies, myelopathy, bony deformities, anorectal malformations, and renal anomalies) is the lower-body equivalent of PHACE.9 The hemangioma typically affects the sacral area or lumbar region. Patients can have ventral-caudal malformations (ie, omphalocele, rectovaginal fistula, vaginal/uterine duplication, solitary/ duplex kidney, imperforate anus, tethered cord, and/or lipomyelomeningocele). Ultrasonography is performed to rule out associated anomalies in infants younger than 4 months. Magnetic resonance imaging by using techniques noted in the earlier discussion of PHACE is indicated in older infants or when US findings are equivocal.

Management Observation and Wound Care. Most IHs are simply observed because they are

typically small and localized and do not involve anatomically important areas. During the proliferative phase, lesions can ulcerate; the median age for this phase is 4 months. The lips, neck, and anogenital region are the most common locations. To protect against ulceration, IHs in these areas should be kept moist with hydrated petroleum to minimize desiccation and shearing of the skin. If ulceration develops, the wound should be washed gently with soap and water at least twice daily. Small, superficial areas are managed with the application of topical antibiotic ointment and occasionally with a petroleum gauze barrier. Large, deep ulcers require damp-to-dry dressing changes. Bleeding from an ulcerated IH is usually minor and is treated by applying direct pressure. Most ulcerations will heal with local wound care within 2 to 3 weeks.

Topical Pharmacotherapy. Topical timolol is effective for superficial

lesions that are treated within the first 2 to 3 months after birth.10 Usually, 1 drop is administered twice daily. Timolol is not effective for thick hemangiomas or those with a subcutaneous component because it cannot penetrate most of the tumor. Intralesional Corticosteroid. Problematic IHs that are too thick or deep to

be treated with topical timolol can be treated with intralesional corticosteroid. Tumors should be well localized and smaller than 3 cm in diameter. Triamcinolone (not to exceed 3 mg/kg) will stop the growth of the lesion; two-thirds of IHs will decrease in size.11 The corticosteroid lasts 2 to 3 weeks;

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thus, infants may require 2 to 3 injections during the proliferative phase. There is a potential risk of intralesional steroids affecting growth, as noted with oral steroids. Other rare complications include retinal artery occlusion if injected near eyelid lesions. Systemic Pharmacotherapy. Oral propranolol is the first-line agent for IHs that require systemic treatment. Propranolol is a nonselective antagonist of both β1- and β2-adrenergic receptors that has evolved to become the treatment of choice for IHs. The precise mechanism of action is unknown but may be related to vasoconstriction, angiogenesis inhibition, induction of apoptosis, inhibition of nitric oxide production, and regulation of the renin-angiotensin system.12 Oral propranolol hydrochloride (eg, Hemangeol) was approved by the U.S. Food and Drug Administration in March 2014 for use in proliferating IHs that require systemic therapy. This therapy has now replaced the previous standard-of-reference therapy for threatening IHs, systemic or intralesional corticosteroids.13 Propranolol dosing is typically 2 mg/kg/d.14 Approximately 90% of tumors will stop growing or regress. Risks (< 3%) include bronchospasm, bradycardia, hypotension, hypoglycemia, seizures, and hyperkalemia. Preterm newborns and infants younger than 3 months are more likely to experience adverse events. Patients usually have cardiology consultation and undergo electrocardiography, echocardiography, glucose and electrolyte measurements, and blood pressure, heart rate, and respiratory examinations. Inpatient initiation of treatment is used for preterm newborns or infants younger than 3 months.14 Potential contraindications include asthma, glucose abnormalities, heart disease, hypotension, bradycardia, and PHACE association. The drug should be discontinued if the newborn or infant is ill because reduced oral intake can increase the risk of hypoglycemia and seizures. Patients who have failed to improve with treatment or have a contraindication to propranolol can be treated with oral prednisolone, which has been used to treat IH for more than 40 years. Patients are given 3 mg/kg/d for 1 month; the drug is then tapered by 0.5 mL every 2 to 4 weeks until it is discontinued between 10 and 12 months of age, when the tumor is no longer proliferating.13 The drug is given once a day in the morning, and infants have monthly outpatient follow-up. By using this protocol, many tumors will stabilize in growth, and 88% will become smaller (accelerated regression).13 Potential side effects include cushingoid appearance and decreased growth, hypertension, hyperglycemia, immunosuppression, and adrenal suppression. Twenty percent of infants will develop a cushingoid appearance that resolves during tapering of therapy. Approximately 12% of infants treated after 3 months of age exhibit decreased gain in height but will return to their pretreatment growth curve by 24 months of age. Laser Therapy. There is little, if any, role for pulsed dye laser treatment of proliferating IH.15 The laser penetrates only 0.75 to 1.2 mm into the dermis and, thus, only affects the superficial portion of the tumor. Although

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lightening may occur, the mass is not affected. These patients have an increased risk of skin atrophy, ulceration, pain, bleeding, scarring, and hypopigmentation. Pulsed dye laser treatment is indicated during the involuted phase to fade residual telangiectasias. Resection. Resection of IH is generally not recommended during the early growth phase. The tumor is highly vascular during this period, and there is a risk of blood loss, iatrogenic injury, and an inferior outcome when compared to excising residual tissue after the tumor has regressed. It is preferable to intervene surgically between 3 and 4 years of age. During this period, the IH will no longer improve significantly, and the procedure is performed before the child’s long-term memory and self-esteem begin to form at about 4 years of age.

Congenital Hemangioma Clinical Features Congenital hemangiomas are fully grown at birth and do not have postnatal growth. They are red-violaceous with coarse telangiectasias, central pallor, and a peripheral pale halo. These lesions are more common in the extremities, have an equal male to female distribution, and are solitary, with a mean diameter of 5 cm.5 There are 2 forms: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). RICH involutes rapidly after birth, and 50% of lesions have completed regression by 7 months of age; the remaining tumors are fully involuted by 14 months of age. NICH, in contrast, does not regress; it remains unchanged with persistent fast flow. Management RICH usually does not require resection in infancy because it regresses so quickly. After involution, RICH may leave behind atrophic skin and subcutaneous tissue. NICH is rarely problematic in infancy and is observed until the diagnosis is clear; resection may be indicated to improve the appearance of the area. Kaposiform Hemangioendothelioma Clinical Features Kaposiform hemangioendothelioma is a rare vascular neoplasm (occurring in 1 in 100,000 children) that is locally aggressive but does not metastasize.5 It is present at birth in 50% of affected patients, has an equal male to female distribution, and affects the head/neck (40%), trunk (30%), or an extremity (30%). The tumor is often larger than 5 cm in diameter and appears as a flat, reddish-purple, edematous lesion. Seventy percent of patients with kaposiform hemangioendothelioma have Kasabach-Merritt syndrome (thrombocytopenia < 25,000/mm3, petechiae, bleeding). Kaposiform hemangioendothelioma partially regresses after 2 years of age, although it usually persists long-term and causes chronic pain and stiffness.

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Most lesions are extensive, involve multiple tissues, and occur well beyond the limits of resection. Oral sirolimus has replaced intravenous vincristine as first-line therapy. Thrombocytopenia is not significantly improved with platelet transfusion because the platelets are trapped in the tumor. Platelet transfusion also worsens swelling and should be avoided unless there is active bleeding or a surgical procedure is planned.

Pyogenic Granuloma Clinical Features Pyogenic granuloma is a solitary red papule that grows rapidly on a stalk.5 It is small, with a mean diameter of 6 mm; the mean age of onset is 6 years. Pyogenic granuloma is commonly complicated by bleeding and ulceration. It involves the skin or mucous membranes. It is distributed on the head or neck (62%), trunk (19%), upper extremity (13%), or lower extremity (5%). Management Pyogenic granulomas require intervention to control likely ulceration and bleeding. Numerous methods have been described, such as curettage, shave excision, laser therapy, and excision. Because the lesion extends into the reticular dermis, it may be out of the reach of the pulse dye laser, cautery, or shave excision. Consequently, these modalities have a recurrence rate of approximately 50%. Full-thickness excision is the definitive treatment.

Vascular Malformations Capillary Malformation Clinical Features Capillary malformation (previously referred to as port-wine stain) is the most common type of vascular malformation.16 The lesion is present at birth and can involve any area of the integument. The pink-purple skin discoloration can cause psychosocial distress. Over time, the lesion progresses as follows: (a) it darkens, (b) fibrovascular cobblestones can occur, (c) pyogenic granulomas may develop, and (d) soft-tissue and bony involvement may enlarge underneath the stain. The birthmark referred to as an “angel kiss” or “stork bite” is a fading capillary stain. It is present in one-half of white newborns and is located on the forehead, eyelids, nose, upper lip, or posterior neck. No treatment is necessary because it lightens over the first 2 years after birth. Management Management may be and should be observation in most cases, with laser treatment as an option, depending on the family’s preference. This modality improves the appearance of the lesion by lightening its color. Intervention

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during infancy or early childhood is recommended because superior lightening of the lesion is achieved, the risk of darkening and hypertrophy is reduced, and psychosocial morbidity is minimized. Pulse dye laser is less effective for capillary malformations that have progressed to a dark color with cutaneous thickening. Surgical procedures are indicated to correct overgrowth caused by the malformation. Small fibrovascular nodules or pyogenic granulomas can be excised. Trunk or extremity capillary malformation associated with increased subcutaneous adipose tissue can be improved with suction-assisted lipectomy.

Lymphatic Malformation Clinical Features Lymphatic malformation is defined by the size of its channels: macrocystic, microcystic, or combined.17 Lesions are usually noted at birth, although small or deep lymphatic malformations may not become evident until childhood or adolescence, after they have enlarged and/or become symptomatic. The most commonly affected sites are the neck and axilla. Lymphatic malformation causes 3 major problems: psychosocial morbidity, infection, and bleeding. Macrocystic lesions contain cysts large enough to be accessed by a needle (typically ≥ 5 mm) and are amenable to sclerotherapy. The most commonly affected sites are the neck and axilla. Microcystic lesions have cysts that are too small to be cannulated by a needle (usually < 5 mm) and, thus, cannot be treated with sclerotherapy. These lesions commonly affect the face and extremities and are often associated with cutaneous vesicles that can bleed and leak lymph fluid. Approximately one-half of lymphatic malformations are not purely macrocystic or microcystic; they contain both macrocysts and microcysts. The greater the macrocystic composition of the lesion, the better the prognosis, because the lesion can be treated with sclerotherapy. Ninety percent of lymphatic malformations are diagnosed by means of history and physical examination findings. Small, superficial lymphatic malformations do not require further diagnostic workup. Large or deep lesions are evaluated with MR imaging. Management Lymphatic malformation is benign; thus, intervention is not mandatory. Small or asymptomatic lesions may be observed. First-line treatment for a large or problematic macrocystic or combined lymphatic malformation is sclerotherapy. This technique involves aspiration of the cysts, followed by injection of an inflammatory substance that causes scarring of the cyst walls. Although sclerotherapy does not remove the lymphatic malformation, it effectively shrinks the lesion and alleviates symptoms. Sclerotherapy provides superior results and has a lower morbidity rate than resection. Resection of a macrocystic lymphatic malformation is generally not indicated unless the lesion is symptomatic and sclerotherapy is no longer

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possible because all of the macrocysts have been treated, or excision may be curative because the lesion is small and well localized. Resection is usually subtotal because lymphatic malformations typically involve multiple tissue planes and important structures. Nonproblematic microcystic malformations can be observed. Symptomatic lesions are managed by means of resection, bleomycin injection, carbon dioxide laser, and/or sirolimus.

Venous Malformation Clinical Features Although venous malformations are present at birth, they may not become evident until childhood or adolescence, when they have grown large enough to cause a visible deformity or symptoms.18 Lesions are blue, soft, and compressible. Hard calcified phleboliths may be palpable. The primary morbidity of venous malformation is psychosocial because most lesions affect the skin and cause a deformity. The second most common complication is pain secondary to thrombosis and phlebolith formation. Patients with venous malformations are not at risk for thromboembolism, unless a large phlebectatic vein is connected to the deep venous system. Venous malformations are diagnosed by means of history and physical examination findings. Dependent positioning will cause a lesion to enlarge. Small, superficial venous malformations do not require further diagnostic workup. Large or deep lesions are evaluated with MR imaging. Management Venous malformation is a benign condition, and nonproblematic lesions can be observed. Intervention is reserved for symptomatic lesions or asymptomatic phlebectatic areas at risk for thromboembolism. If possible, intervention should be postponed until after 12 months of age, when the risk of anesthesia is lowest. Therapy for lesions causing a visible deformity should be considered before the patient is 4 years of age to limit the risk of psychological morbidity. Sclerotherapy is typically first-line treatment of a problematic venous malformation and is generally safer and more effective than resection. Sclerotherapy reduces the size of the malformation and alleviates symptoms. Resection of a venous malformation should be considered for small, well-localized lesions that can be completely removed or persistent symptoms after completion of sclerotherapy. Almost all venous malformations should be treated with sclerotherapy prior to surgical intervention to facilitate resection. Arteriovenous Malformation Clinical Features Arteriovenous malformation has an absent capillary bed, which causes shunting of blood directly from the arterial to the venous circulation through a fistula (direct connection of an artery to a vein) or nidus (abnormal channels

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bridging the feeding artery to the draining veins).19 Although present at birth, an arteriovenous malformation may not become evident until childhood, after it has enlarged or become symptomatic. Lesions have a pink-red cutaneous stain, are warm, and can have palpable pulsations. Patients are at risk for pain, ulceration, bleeding, and congestive heart failure. Arteriovenous malformations can also cause disfigurement, destruction of tissues, and obstruction of vital structures. Ninety percent of arteriovenous malformations are diagnosed by means of history and physical examination findings. Handheld Doppler US shows fast flow. If the diagnosis is equivocal after history, physical examination, and handheld Doppler US are performed, US is the first-line study to confirm the diagnosis. Magnetic resonance imaging is usually performed to confirm the diagnosis and determine the extent of the lesion. Angiography is performed if the diagnosis remains unclear after US and MR imaging or if embolization is planned.

Management Arteriovenous malformation is not a malignancy, and intervention is not mandatory. Because the lesion is often diffuse and involves multiple tissue planes, cure is rare. An asymptomatic arteriovenous malformation should be observed, unless it can be removed for possible cure with minimal morbidity. Embolization is generally first-line therapy for a symptomatic lesion. It involves the delivery of a substance through an arterial catheter to occlude blood flow and/or fill a vascular space. Success requires that the embolic agent reaches the nidus of the arteriovenous malformation at the point of initial venous drainage. Embolization is not curative, and most arteriovenous malformations will re-expand after treatment. Indications for embolization include preoperative intervention to reduce blood loss during resection and definitive treatment to alleviate symptoms for lesions not amenable to resection. Resection of an arteriovenous malformation has a lower recurrence rate than embolization. Indications include a well-localized lesion, correction of a focal deformity, and a symptomatic arteriovenous malformation that has failed to resolve with embolization. When excision is planned, preoperative embolization will facilitate the procedure by minimizing blood loss.

Vascular Malformation Overgrowth Syndromes CLOVES Syndrome Patients with CLOVES (congenital lipomatosis, overgrowth, vascular malformation, epidermal nevi, and scoliosis/skeletal/spinal anomalies) syndrome have a truncal lipomatous mass, a slow-flow vascular malformation (most commonly capillary malformation), and hand and/or foot anomalies. Individuals also may have an arteriovenous malformation, neurological impairment, or scoliosis.

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Klippel-Trénaunay Syndrome Klippel-Trénaunay syndrome is a capillary-lymphatic-venous malformation of an extremity that causes overgrowth. The condition almost always affects the lower limb. A large, embryonic vein in the subcutaneous tissue is isolated in the lateral calf and/or thigh and communicates with the deep venous system. Complications include thrombophlebitis and pulmonary embolism. The lymphatic abnormalities are usually macrocystic in the pelvis and/or thigh and microcystic in the abdominal wall, buttock, and distal limb. Because embryonal veins can connect to the deep venous system, which causes thromboembolism, they are removed in early childhood with sclerotherapy or an endovascular laser. Parkes Weber Syndrome Parkes Weber syndrome consists of a diffuse arteriovenous malformation of an extremity (usually the leg), which causes soft-tissue and/or bony overgrowth. A capillary malformation involves the skin of the affected limb. Patients have subcutaneous and intramuscular microshunting and can develop congestive heart failure. Most children are observed until symptoms necessitate intervention. Embolization may reduce congestive heart failure, pain, or ulceration. Occasionally, amputation is necessary. Sturge-Weber Syndrome Sturge-Weber syndrome is defined by a capillary malformation in the V1 trigeminal nerve distribution with ocular abnormalities (eg, glaucoma, choroidal vascular anomalies) and/or a leptomeningeal vascular malformation. Extensive pial vascular lesions may cause refractory seizures, hemiplegia, and/or delayed cognitive development. Any infant or child with a capillary malformation in an upper trigeminal nerve distribution should be screened for Sturge-Weber syndrome. Magnetic resonance imaging is performed to rule out leptomeningeal vascular lesions. Patients undergo ophthalmology evaluation to assess whether choroidal anomalies and glaucoma are present. REFERENCES

1. Greene AK, Liu AS, Mulliken JB, Chalache K, Fishman SJ. Vascular anomalies in 5,621 patients: guidelines for referral. J Pediatr Surg. 2011;46(9):1784–1789 PMID: 21929990 https://doi. org/10.1016/j.jpedsurg.2011.05.006 2. Hassanein AH, Mulliken JB, Fishman SJ, Greene AK. Evaluation of terminology for vascular anomalies in current literature. Plast Reconstr Surg. 2011;127(1):347–351 PMID: 21200229 https://doi.org/10.1097/PRS.0b013e3181f95b83 3. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69(3):412–422 PMID: 7063565 https://doi.org/10.1097/00006534-198203000-00002 4. Wassef M, Blei F, Adams D, et al; ISSVA Board and Scientific Committee. Vascular anomalies classification: recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics. 2015;136(1):e203–e214 PMID: 26055853 https://doi.org/10.1542/ peds.2014-3673

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50 Pediatric Plastic and Reconstructive Surgery for Primary Care 5. Greene AK. Management of hemangiomas and other vascular tumors. Clin Plast Surg. 2011;38(1): 45–63 PMID: 21095471 https://doi.org/10.1016/j.cps.2010.08.001 6. Chang LC, Haggstrom AN, Drolet BA, et al; Hemangioma Investigator Group. Growth characteristics of infantile hemangiomas: implications for management. Pediatrics. 2008;122(2):360–367 PMID: 18676554 https://doi.org/10.1542/peds.2007-2767 7. Couto RA, Maclellan RA, Zurakowski D, Greene AK. Infantile hemangioma: clinical assessment of the involuting phase and implications for management. Plast Reconstr Surg. 2012;130(3): 619–624 PMID: 22575857 https://doi.org/10.1097/PRS.0b013e31825dc129 8. Hartemink DA, Chiu YE, Drolet BA, Kerschner JE. PHACES syndrome: a review. Int J Pediatr Otorhinolaryngol. 2009;73(2):181–187 PMID: 19101041 https://doi.org/10.1016/j.ijporl.2008.10.017 9. Iacobas I, Burrows PE, Frieden IJ, et al. LUMBAR: association between cutaneous infantile hemangiomas of the lower body and regional congenital anomalies. J Pediatr. 2010;157(5): 795–801.e1-7 PMID: 20598318 https://doi.org/10.1016/j.jpeds.2010.05.027 10. Püttgen K, Lucky A, Adams D, et al; Hemangioma Investigator Group. Topical Timolol Maleate Treatment of Infantile Hemangiomas. Pediatrics. 2016;138(3):e20160355. PMID:27527799 https://doi.org/10.1542/peds.2016-0355 11. Couto JA, Greene AK. Management of problematic infantile hemangioma using intralesional triamcinolone: efficacy and safety in 100 infants. J Plast Reconstr Aesthet Surg. 2014;67(11): 1469–1474 PMID: 25104131 https://doi.org/10.1016/j.bjps.2014.07.009 12. Storch CH, Hoeger PH. Propranolol for infantile haemangiomas: insights into the molecular mechanisms of action. Br J Dermatol. 2010;163(2):269–274 PMID: 20456345 https://doi.org/ 10.1111/j.1365-2133.2010.09848.x 13. Greene AK, Couto RA. Oral prednisolone for infantile hemangioma: efficacy and safety using a standardized treatment protocol. Plast Reconstr Surg. 2011;128(3):743–752 PMID: 21572374 https://doi.org/10.1097/PRS.0b013e3182221398 14. Drolet BA, Frommelt PC, Chamlin SL, et al. Initiation and use of propranolol for infantile hemangioma: report of a consensus conference. Pediatrics. 2013;131(1):128–140 PMID: 23266923 https://doi.org/10.1542/peds.2012-1691 15. Darrow DH, Greene AK, Mancini AJ, Nopper AJ; American Academy of Pediatrics Section on Dermatology, Section on Otolaryngology–Head and Neck Surgery, and Section on Plastic Surgery. Diagnosis and management of infantile hemangioma. Pediatrics. 2015;136(4):e1060–e1104 PMID: 26416931 https://doi.org/10.1542/peds.2015-2485 16. Maguiness SM, Liang MG. Management of capillary malformations. Clin Plast Surg. 2011;38(1): 65–73 PMID: 21095472 https://doi.org/10.1016/j.cps.2010.08.010 17. Greene AK, Perlyn CA, Alomari AI. Management of lymphatic malformations. Clin Plast Surg. 2011;38(1):75–82 PMID: 21095473 https://doi.org/10.1016/j.cps.2010.08.006 18. Greene AK, Alomari AI. Management of venous malformations. Clin Plast Surg. 2011;38(1): 83–93 PMID: 21095474 https://doi.org/10.1016/j.cps.2010.08.003 19. Greene AK, Orbach DB. Management of arteriovenous malformations. Clin Plast Surg. 2011; 38(1):95–106 PMID: 21095475 https://doi.org/10.1016/j.cps.2010.08.005

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CHAPTER

4

Congenital Ear Deformities BRUCE S. BAUER, MD, FAAP, FACS, AND TIMOTHY W. KING, MD, PhD, MSBE, FAAP, FACS

Introduction

Congenital ear deformities can range from small defects, such as crimping of the rim of the ear or notching of the earlobe, to major deformities of the ear, where all or most of the recognizable parts of the ear are missing (microtia) and the deformity is associated with clinically significant underdevelopment of the face (hemifacial microsomia). Within this spectrum of deformities are the most common, skin and/or cartilage tags in front of the ear, and common deformities in ear shape, size, and projection from the head. Asymmetries in ear size and shape are common. Even the most minor of ear deformities can represent a cause for marked peer ridicule and concerns about body image, and correction can greatly alleviate these issues for children and adults. The type of ear deformity will determine the timing and type of reconstruction necessary. Some types of deformities may be amenable to early, nonsurgical splinting techniques in the neonatal period; others may be treated surgically before a child reaches an age when negative comments can begin to affect his or her body image. Others may require a delay in reconstruction until a later age to be able to optimize the use of donor cartilage needed for the reconstruction. In this chapter, some of the more common congenital ear deformities and their treatments are discussed.

Epidemiology

The range of normal ear shape varies significantly, and what may be accepted as normal in some patient populations may be considered abnormal in others. Likewise, variation in size, shape, and projection between each individual’s 2 ears is extremely common and is often within the realm of normal variation, yet the asymmetry may become the source of concern. Before discussing the prevalence of each type of ear deformity, it is important to have a frame of reference on what is normal and what landmarks may be distorted during development (Figure 4-1).1,2 Equally important to the discussion is the less-well-recognized fact that ear positions and projections are intimately linked to the anatomy of the underlying craniofacial shape and symmetry. It may be obvious to the pediatrician that microtia is seen in the presence of the 51

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A

B

Otobasion superioris Fossa triangularis Root of helix Tragus Intertragic notch Otobasion inferioris

Helix Superior (posterior) crus Scapha Inferior (anterior) crus Cymba conchae Antihelix

15° – 20° .10 .5

8°

Cavum conchae Antitragus

.33

.43 .5

Lobule

37°

.23

Figure 4-1. A, The normal ear and its parts. B, The critical proportions of the ear. The ear height is measured on the long axis, and the width is measured at right angles to that. The long axis is the line that passes through the longest dimension of the ear. It is important is to recognize that the axis of the ear is rarely vertical. In the normal ear, the height ranges from 5.5 to 7.5 cm, and the width ranges from 3.0 to 4.5 cm (50%–65% of the height). The top of the ear should be even with the top of the brow, and the bottom of the ear should be even with the base of the nose. The ear is positioned approximately one ear length (5.5–7.5 cm) posterior to the lateral rim of the orbit. The protrusion of the normal ear is 1.5 to 2.0 cm from the scalp to the anterior surface of the superior pole of the helix.

spectrum of hemifacial microsomia, but it may be less obvious that otherwise normal ears may vary significantly in their position and projection in the presence of positional plagiocephaly. Minor deformities, such as preauricular branchial vestiges (the most common ear deformities), may be present in 1% of the population,3 while microtia may only occur in 1 in 8,000 births. The exact prevalence of this major deformity and more minor deformities, such as prominent ear, constricted ear, Stahl ear, and cryptotia, vary significantly in different races. While the developmental cause of some of these deformities may be genetically linked to specific syndromes (eg, Goldenhar, Treacher Collins), and ear prominence and atypical shape may be seen within generations of a given family, others may be deformational in origin, being influenced by head position during development (and compression of the developing ear, which can cause constriction, cryptotia, or minor helical rim deformities) and variations in head shape (with secondary variations in ear projection).

Patient Presentation and the Spectrum of Deformities

While this chapter covers surgical treatments of the more clinically significant deformities in some detail, thoughts on timing and treatment of more minor deformities and those amenable to nonsurgical treatment are covered in the

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initial description of their anatomy. The rationale and variable techniques used in nonsurgical neonatal splinting are also briefly discussed.

Preauricular Branchial Vestiges

These often small “tags” of skin and cartilage may be present alone or in combination with some syndromes (eg, Goldenhar syndrome) and are, not surprisingly, often the first ear deformities to be visualized at birth. They represent “displaced” segments of skin and cartilage that arise during development of the ear and facial structures. Most of these remnants are isolated deformities, and they represent the most common ear deformities. Opinion varies as to whether these should be treated in the immediate neonatal period, in the infant’s first year, or later in childhood. There may be less urgency in treating the smaller vestiges present in the immediate preauricular area than in treating larger remnants that manifest more medially on the cheek, along the line from the ear to the oral commissure. While more pedunculated vestiges may seem amenable to ligation of the stalk, it is rare that this will not leave a visible projection of the underlying cartilage and base of the vestige that will still be visible. Similar “incomplete” resection with need for subsequent treatment may also arise when the excision is performed on a visibly upset newborn or infant, despite anesthetizing the area appropriately. At least in the authors’ opinion, the optimal treatment may be delaying the excision until the patient requests the removal and is able to cooperate with the procedure with the use of local anesthesia. Alternatively, some parents may elect to have larger remnants excised surgically during a short procedure performed with general anesthesia.

Prominent Ear

The prominent ear represents a spectrum of ear deformities in which part of the ear or the whole ear projects from the head to an extent that it draws attention and appears abnormal. While much has been written about the “normal” ear shape and “ideal” projection, these measurements are far less significant than what our eye visualizes as “normal” and what is outside those boundaries. The compounding issue of what a child’s peers view as fair game to ridicule is the unfortunate link that is often made between an individual’s large or prominent ears and he or she being associated with immaturity and decreased intelligence. While parents may elect to have their child’s prominent ears corrected before they become the source of ridicule, others may prefer to delay treatment until the child is old enough to express a desire to have the deformity corrected. In a practice in which a broad spectrum of ear deformities are treated, it is not at all uncommon to have parents express that they had similar deformities that were not corrected until they had “suffered” substantially because of taunts from their schoolmates and having to accept a prolonged period of carrying a negative body image because their

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parents were not supportive of their having surgery or could not afford treatment. Of great significance is that even in cases in which these concerns and possibly negative feelings surrounding the deformity have persisted into adulthood, the correction can be just as beneficial. Prominent ear can arise from effacement of the antihelical fold, hypertrophy of the concha, or overall increase in ear size. As noted earlier, it may also arise from asymmetry of the cranial base secondary to uncorrected positional plagiocephaly, and this later cause is particularly significant in cases of unilateral prominence. It is not uncommon that the cause of prominence will vary from side to side. Its developmental cause is unknown but may be related to genetic tendencies, as well as deformational forces during development. When looking at the anatomy of a prominent ear, the excess projection is usually caused by underdevelopment of the antihelical fold, which causes the top of the ear to stand out, as well as hypertrophy (overgrowth) of the concha (the bowl-shaped portion of the ear), that causes the entire ear structure to be pushed away from the side of the head (Figure 4-2). In addition, the shape of the head and overall size of the ear can affect the appearance of the ear. A

B

C

D

Figure 4-2. Variations in anatomy and causes of ear prominence. A, Marked prominence resulting from near total effacement of the antihelical folds (with suggestion of macrotia). B, Prominence resulting from conchal hypertrophy. C, Prominence in association with hypoplasia of the underlying craniofacial skeleton. D, The critical angle of the cartilage between the conchal floor and the antihelix influences the prominence of the lobule.

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Macrotia is a variant of prominent ear in which any or all of these features may be present with an overall increase in ear size. This may be a variant or normal or secondary to causes such as hemangioma, vascular malformation, neurofibromatosis, or generalized facial hemihypertrophy.

Constricted Ear

Previously described by using a broad range of confusing terms, such as cup ear and lop ear, the terminology for this group of congenital ear deformities was simplified by Tanzer4 and Cosman,5 who classified this entire group of anomalies as constricted ear (Figure 4-3). Cosman described the following 4 fundamental aspects of the constricted ear deformity that vary, depending on its severity: • Lidding: Caused by helical overhang, arch shortening, and flattening of the antihelical crura. It may be further accentuated by a crimping of the rim with adhesion to the scapha. • Protrusion: Caused by an abnormally shaped conchal bowl and asymmetry of the cranial base. • Decreased ear size: Caused by previously described changes, in addition to a decreased skin envelope, conchal widening and angulation, and an actual decrease in the overall size of the cartilage. • Low ear position: Sometimes related to other associated skeletal deformities and syndrome-related anomalies. Tanzer4 divided constricted ear types on the basis of the degree of cartilage deformity and extent of skin deficiency, as follows: • Group I: Minor deformities involving only the helix, causing a lidded appearance to the ear.

A

B

C

D

Figure 4-3. Constricted ear deformities are grouped on the basis of the degree of lidding, decreased ear size as the radius of the curve of the helical rim decreases, and degree of skin shortage. A, Group I, with lidding alone. B, Group IIA, with combined lidding and increased constriction of the helical rim but without significant skin shortage. C, Group IIB, with greater constriction and skin shortage. D, Group III, with skin and cartilage deficiency comparable to a large conchal remnant microtia.

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• Group II: Moderate to severe deformities, involving the helix and scapha, which are further subdivided into – Group IIA: Without skin deficiency – Group IIB: With skin deficiency • Group III: Severe deformities with extreme cupping and a tubular shape to the ear, similar to a large chondral remnant–type microtia. Patients with this deformity also often have deformities of the external auditory canal and middle ear.

Cryptotia and Stahl Ear

A number of congenital ear deformities, in addition to those already described, involve varied deformation of the antihelix and upper pole of the auricle. Although these deformities are not common in the United States, they are seen with fairly high frequency in the Asian population (cryptotia occurs in approximately 1 in 400 to 500 Asian births).6 Because these deformities are relatively rare, most surgeons have limited experience treating them, and although an in-depth discussion of each is beyond the scope of this chapter, it is worthwhile to review their anatomy and make several key points concerning their evaluation and treatment. Each has an element of deformation or deficiency of cartilage, in part secondary to fibrous adhesions, and a varied degree of skin shortage.

Anatomy of Cryptotia Cryptotia is characterized by failure of the upper pole of the ear to stand out from the head (Figure 4-4). The 3 basic features of the deformity were described by Washio,6 with a fourth feature added by Bauer that may need to be addressed for optimum outcome. • Buried superior pole of the auricle beneath the scalp skin • Scaphal underdevelopment • Sharpening of the antihelical crura, particularly the superior crus • Helical rim crimping and adhesion Hirose et al7 believed that this deformity was the result of an anomaly of the intrinsic transverse and oblique auricular muscles and subcategorized cryptotia into the following: • Type I (transverse muscle type): The body of the antihelix and the superior crus are compressed together. • Type II (oblique muscle type): There is gross contraction of the body of the antihelix and an acutely bent inferior crus. Anatomy and Treatment of Stahl Ear Stahl ear, which is an unusual deformity sometimes referred to as Spock ear, has as its main feature a third crus, in combination with a flat helix and malformed scapha.8 The third crus typically projects from the antihelix at

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Figure 4-4. Cryptotia. While the anatomy varies in severity, all cryptotia cases involve an absence of the superior sulcus with a varying overhang of skin on the upper pole. Traction on the upper pole demonstrates the presence of the upper helical rim beneath the skinfold, with varying degrees of cartilage deformity.

almost a right angle outward toward the helical rim, and in some cases, there is only a displaced superior crus and not 3 crura. This deformity forces the helical rim up into a point at the junction of the middle and the upper poles of the auricle (Figure 4-5A). There is also a group of congenital ear deformities in which there is just a vertical lengthening of the superior crus A

B

Figure 4-5. A, Stahl ear deformity has as its main feature a third crus, in combination with a flat helix and a malformed scapha. B, A group of deformities are seen in which the upper pole is elongated by a long superior crus and an elongated scapha, which results in a pointed ear.

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(without a third crus) that creates a similar distortion of the helical rim and pointing of the ear; the authors consider this to be a variant of Stahl ear (Figure 4-5B).

Microtia

Microtia is the most significant of the ear deformities and represents a range from an ear that is undersized but relatively well formed (conchal remnant– type microtia) to near total absence of a recognizable ear structure with only a sausage-shaped vestige of skin and malformed cartilage (lobular-type microtia). The ear canal may or may not be present. It is reported to occur in about 1 in 6,000 to 12,000 births, is more common in boys, and usually only affects one ear. The actual cause is not fully known but is thought to be related to a blockage of blood flow in the area during gestation. In addition, several different inherited and environmental factors have also been found to be associated with microtia. Microtia can also be seen as part of a syndrome, such as Goldenhar or Treacher Collins syndrome, and is almost always seen with some underdevelopment of the facial structures on the affected side of the face (hemifacial microsomia). Children with microtia often lack an external auditory ear canal and have malformations of the bones of the middle ear. This being the case, there is typically a varying degree of hearing loss on the affected side (the air conduction of sound is affected, although bone conduction of sound is usually present). While patients with microtia on only one side often overcome the associated hearing loss, they should all be evaluated by an otolaryngologist and an audiologist and recommendations discussed to optimize hearing and to ensure proper speech development. The advent of the bone-anchored hearing aid (BAHA) has increased the interest and comfort levels of many patients with this means of improving hearing on the affected side, particularly when the placement can be incorporated in the second-stage reconstruction of the ear. The BAHA is affixed to the mastoid bone and held in place by an osseointegrated implant. This allows direct stimulation of the cochlea, which is normal in most patients with microtia. Unlike a canaloplasty procedure, this procedure does not depend on a functioning middle ear or a patent canal and is a much simpler and safer procedure to perform. Microtia manifests with varying degrees of ear cartilage absence, or malformation, and varying degrees of skin shortage. The treatment of microtia is focused on reconstructing a more normal external ear shape and position. The reconstruction requires borrowing cartilage from either the chest or the chest and the opposite ear and covering that cartilage with skin flaps from the area of the ear, in addition to skin grafts from other sites. The reconstruction is performed in a series of stages, depending on the deformity, the age of the patient, and the surgeon’s preference on optimal technique.

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Nonsurgical Treatment of Congenital Ear Deformities

Nonsurgical treatment of non-microtia ear deformities was first described by Matsuo and colleagues9 and then by Brown and colleagues.10 The approach is based on experimental evidence that auricular cartilage is soft and malleable during the neonatal period, while maternal estrogen levels are still high. Once these levels decrease (usually after 2 weeks of age), the cartilage becomes firmer and holds the shape into which it has been molded (Figure 4-6). While the technique described herein uses a simple splint of soft, malleable wire covered with a silicone sleeve and simple taping, the EarWell system (Becon Medical, Batavia, IL), recently popularized by Byrd, is being used with increasing frequency, and other systems are under design. This nonsurgical approach has been applied most successfully to Stahl ear, group I and II constrictions, and prominent ears when the primary feature is

A

B

D

C

F

G

H

E

I

Figure 4-6. Nonsurgical splinting of the ear in the neonatal period. A, A Stahl ear deformity. B, The splinting material of lead-free solder and silicone tubing used for protective covering. C, The splint fitted to shape the ear from the helical sulcus to the triangular fossa. D, Another variety of rubber-coated wire with Steri-Strips (3M, St Paul, MN) holding the splint in place. E, The corrected deformity. F and G, A more complex deformity in a 2-week-old with features of constricted and Stahl ear. H and I, Images obtained 6 weeks after 5 weeks of splinting show excellent correction.

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effacement of the antihelical fold. Although a variety of splinting materials and elaborate prefabricated splints have been used, we have found that leadfree solder with a covering silicone sleeve taped on with Steri-Strips (3M, St Paul, MN) is well tolerated and has never been associated with skin necrosis (a concern with harder splinting materials) and does not require purchase of costly materials. Although Yotsuyanagi11 has reported that splinting can be effective even in children 6 years and older with a thermoplastic splint applied for a relatively short period, this has not been substantiated by other investigators. It is the authors’ experience that the effective nonsurgical molding of the ear can be performed until about 3 months of age; after that age, it is rare for the infant to leave the splint in place continuously and for parents to persist in replacing it. Deformities that do not involve significant skin shortage, or sharp crimping of the cartilage, and that are associated with fibrous adhesions between cartilage surfaces are more amenable to nonsurgical treatment. A few final comments are appropriate before leaving this subject. First, when completing the splinting process described herein, it is important for parents to carefully observe the ear closely for 24 to 48 hours after completion of the process because some recurrence of the deformity may happen. If monitored closely, this recurrence can be corrected with an additional week or two of splinting. The EarWell system may require significantly more monitoring during the molding process, and in some deformities it may carry a higher risk of skin erosion than a simpler technique. However, it may have some benefits in partially correcting elements associated with some of the more non-microtia deformities, and the publications about its use and the internet presence of the technique have increased awareness of the nonsurgical treatment of many ear deformities in pediatricians and the public.

Timing of Surgery

There are 3 main considerations for determining the optimal timing for reconstructive procedures of the ear. • Psychological: Timing the reconstruction to avoid undue psychological trauma to the child because of the deformity • Anatomical: Taking into consideration the size of the contralateral ear in unilateral deformities and the strength of the available auricular donor cartilage in unilateral and bilateral surgeries • Growth: Both of the ears in general and the growth of the ear after reconstructive procedures Adamson et al12 reviewed the growth patterns of the external ear and concluded the following: • The ear reaches 85% of its adult size by the time a child is 3 years old. • Growth continues until adulthood, but little change in width or distance from the scalp occurs after 10 years of age.

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• For all practical purposes, the normal ear is almost fully developed by 6 years of age. Farkas13,14 differed some in his measurements, stating that the ear reaches 85% of full size by 8 years of age, 90% by 9 years, and 95% by 14 years. More recent work clearly demonstrates continued ear growth well into adulthood, although the conchal width does not change appreciably after childhood. This is something we intuitively recognize in viewing many elderly adults. Although these issues are important in timing surgical procedures for correction of the external ear, because most non-microtia deformities can be reconstructed by using auricular cartilage alone, while microtia and group III non-microtia deformities require the use of autogenous costal cartilage or synthetic substitute (eg, Medpor [Stryker, Kalamazoo, MI]), discussion herein of the treatment of non-microtia deformities is separated from treatment of microtia. Commonly, the term otoplasty is used to discuss the treatment of the former group of deformities, in which there are abnormalities in ear shape, size, and projection, while ear reconstruction is used to describe the latter. Correction of all of the varied external ear deformities can have profound benefits for the affected patient.

Timing of Otoplasty and Ear Reconstruction

The timing of treatment for congenital ear deformities can be divided into 3 groups. A number of the non-microtia deformities (including prominent ears for which the lack of antihelical fold is the primary defect), some constricted ears for which the degree of skin deficiency is limited, and upper-pole deformities such as Stahl ear can be treated nonsurgically with splinting during the neonatal period, as described earlier in this chapter. The non-microtia ear deformities in which correction of deformed cartilage requires surgical intervention with correction of abnormal cartilage shape or cartilage deficiencies, and change in the skin envelope, are usually delayed until at least 5 to 6 years of age, although some less complicated cases may benefit from earlier intervention. There is currently no consensus on the best age to perform reconstruction of microtia. Historically, this surgery has been performed around 5 years of age, and some practitioners still follow this guidance. However, using costal cartilage for the framework construction may be best delayed until the cartilage is larger and the chest wall growth is more complete, which requires waiting until at least 10 years of age before the reconstruction commences, with an effort to delay the procedure to an even later age if the patient is handling the deformity well. Thus, many surgeons have transitioned to performing this surgery beginning at 10 years and older. A closer look at each of these groups follows.

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Timing of Otoplasty and Surgical Treatment of Non-microtia Deformities

Few, if any, children express concern about the abnormal appearance of their ears before reaching the age of 5 or 6 years, even when a deformity is significant enough to elicit comments from their peers. The proportionally greater growth of the ear in these early years, in relation to the rest of the facial features, may, in fact, accentuate the ear prominence. Correction before this age, although quite possibly easing parental concerns about the child’s appearance, may complicate the postoperative course if the child, even if not intentionally, pulls off the bandages and potentially disrupts the repair. Having said this, the authors have seen children who were clearly aware of their appearance by 4 years of age and were bothered enough by it that they eagerly anticipated surgery and cooperated completely during the healing period. With regard to the more complex non-microtia deformities, such as group II and III constricted ears, Stahl ear, and some cryptotia, surgery may need to be delayed until the conchal cartilage used for “expanding” the auricular framework is sturdy enough to support the changed skin envelope. Certainly, this is the case by 5 to 6 years of age. While more of an issue in microtia cases that are associated with clinically significant displacement of the ear remnant because of underlying skeletal asymmetry, there are occasionally group II constricted ears in which unfolding the ear remnant, along with posterosuperior repositioning, may help with the hygiene of the canal and the masking of the more significant deformity until the age at which the greater part of the otoplasty or reconstruction can be accomplished.

Timing of Microtia Reconstruction

While advocates of reconstruction of microtia with Medpor or a similar non-autogenous framework prefer early reconstruction to be able to minimize the psychological stress on affected children, the authors’ experience would support most surgeons who are recognized as “masters” of autogenous reconstruction advocating for a child to be at least 10 years of age before beginning the reconstruction. Our experience over a long career of working with these patients demonstrates that they seem to handle their deformity with less psychological stress than those with the non-microtia deformities, and even at 10 years of age, they may be comfortable with waiting a few more years before starting the staged reconstruction. In reviewing cases of Bauer, Firmin, Nagata, Park, Wilkes, and others, probably the most important variable in obtaining the optimal outcome in the fewest stages of reconstruction is delaying the reconstruction until the costal cartilage is near fully grown and strong enough to allow for the carving of the finest detail and maintaining that detail after the elevation of the framework in the second stage of reconstruction.

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Having said this, there are surgeons who are comfortable with beginning the surgery at an earlier age (eg, 6–8 years), and they have their own opinions to support the benefits of that approach.

Surgical Treatment of Non-microtia Ear Deformities

While the specifics of the surgical techniques for each non-microtia ear deformity are beyond the scope of this chapter, the basics are relevant and can be separated into treatment of the straightforward prominent ear and the techniques for correction of constricted ears, cryptotia, and Stahl ears (upper-pole deformities).

Otoplasty The exact procedure to correct ear prominence will vary on the basis of the details of the deformity, and it is important that the procedure be individualized to avoid an unfavorable result. While much of the early otoplasty literature focused on the treatment of the effaced antihelical fold, conchal hypertrophy plays a far more important role in most ear prominence, and correction of the conchal component of the deformity minimizes the risks of many of the unfavorable outcomes associated with overcorrection and a “pinned back” appearance. Once the conchal support to the projecting antihelix is addressed, the antihelix can be readily reshaped with sutures alone, rather than any form of scoring or cartilage thinning, thereby avoiding the risk of irregular folds and an unnatural appearance. The critical part of treating any prominent ear is to fully analyze the deformity, recognizing that the deformity may vary from side to side, and addressing each element. The reduction of the conchal excess also provides conchal cartilage that can be used as a graft to help restructure the upper pole of the ear in the full spectrum of deformities, from constriction to cryptotia and Stahl ear. Treatment of Deformities of the Upper Pole of the Ear The key element of treatment for all upper-pole deformities is recognizing the common elements of compression and deformity of the cartilage and varying degrees of skin deficiency. A direct approach to the deformed cartilage allows the constricted upper ear to be enlarged and supported with a graft or reduced and reshaped in cases of Stahl ear. The direct approach allows for redraping of the constricted skin envelope to accommodate significant enlargement and expansion of the underlying cartilage support in the corrected ear. Postoperative Management Otoplasty can be performed as an outpatient, typically with the use of general anesthesia in children and more complex cases in adults. Postoperative management involves the use of a bulky supportive but protective dressing for 5 to 7 days, and in all cases we use permanent suture to restructure the cartilage and shape the ear. We also use absorbable sutures on the skin so there are

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no sutures to remove. Patients are typically given a broad-spectrum antibiotic for 4 to 5 days postoperatively, but this can vary from surgeon to surgeon. With long-acting regional blocks given at the completion of the surgery, it is rare for these patients to need more than ibuprofen or acetaminophen postoperatively, and patients with severe pain should be reexamined to ensure that there is not an untoward complication (usually a hematoma). Although hematoma and infection with progression to chondritis are well-recognized risks of otoplasty, these complications are extremely rare in the authors’ experience. Most cases of unfavorable outcomes of otoplasty are caused by failure to properly analyze the deformities before treatment or efforts to apply a single approach to all prominent ears, regardless of the underlying cause. While correction of unfavorable results requires special expertise in all but some cases of under-correction, in skilled hands, these deformities can almost always be corrected properly.

Reconstruction of Microtia: Basic Steps

Reconstruction of microtia remains one of the most demanding procedures in plastic surgery and requires dedicated training and years of perfecting. In the autogenous reconstruction, the first stage involves harvesting rib cartilage from the child, which is carved to match the shape of the opposite ear. A pocket is created underneath the hairless skin of the affected side, and any residual ear cartilage remnants are removed. The newly constructed ear framework is then placed into the pocket and secured into position. Usually, the earlobe remnant is moved into a better position during this surgery. The tragus is often reconstructed in the first surgery, as well. At the completion of this surgery the greater part of the ear shape is present, but there is insufficient skin present to have the ear separated from the side of the head. Excess cartilage is “banked” in a subcutaneous pocket at the site of the rib cartilage harvest. The second stage can be performed 4 to 6 months later after adequate healing of the area has occurred. During this surgery, an incision is made along the outer edge of the ear, and the ear is lifted out from the scalp to create the back side of the ear. A piece of rib cartilage that has been banked is removed from the chest and used behind the ear to prop it out and keep its projection. Local tissue from behind the ear is used to cover this cartilage, and then the entire back side of the ear is covered with a skin graft from either the scalp or the chest incision. If the scalp graft is used, a small area of hair will be shaved during surgery to allow harvest, which grows back normally as the area heals. No hair is transferred with the graft to the back of the ear. Close cooperation between the reconstructive surgery and the otologic surgeon is optimal in the management of all patients with microtia. In cases in which the patient is interested in surgery for hearing as well as external reconstruction, and in candidates for a BAHA implant, this can be placed easily in the second stage with a very limited extension of the incision used to elevate the ear. Typically, the first-stage reconstruction surgery takes 4 to 6 hours. The patient usually stays in the hospital overnight. The second stage is typically conducted as

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an outpatient. With rare exceptions, most patients can return to normal activities 3 weeks after each stage of the microtia reconstruction procedures. REFERENCES

1. Tolleth H. Artistic anatomy, dimensions, and proportions of the external ear. Clin Plast Surg. 1978;5(3):337–345 PMID: 699488 2. Tolleth H. A hierarchy of values in the design and construction of the ear. Clin Plast Surg. 1990;17(2):193–207 PMID: 2189636 3. Roth DA, Hildesheimer M, Bardenstein S, et al. Preauricular skin tags and ear pits are associated with permanent hearing impairment in newborns. Pediatrics. 2008;122(4):e884–e890 PMID: 18829787 https://doi.org/10.1542/peds.2008-0606 4. Tanzer RC. The constricted (cup and lop) ear. Plast Reconstr Surg. 1975;55(4):406–415 PMID: 1118500 https://doi.org/10.1097/00006534-197555040-00003 5. Cosman B. The constricted ear. Clin Plast Surg. 1978;5(3):389–400 PMID: 699490 6. Washio H. Cryptotia: pathology and repair. Plast Reconstr Surg. 1973;52(6):648–651 PMID: 4586231 https://doi.org/10.1097/00006534-197312000-00008 7. Hirose T, Tomono T, Matsuo K, et al. Cryptotia: our classification and treatment. Br J Plast Surg. 1985;38(3):352–360 PMID: 4016423 https://doi.org/10.1016/0007-1226(85)90241-3 8. Sugino H, Tsuzuki K, Bandoh Y, Tange I. Surgical correction of Stahl’s ear using the cartilage turnover and rotation method. Plast Reconstr Surg. 1989;83(1):160–164 PMID: 2909060 https:// doi.org/10.1097/00006534-198901000-00031 9. Matsuo K, Hirose T, Tomono T, et al. Nonsurgical correction of congenital auricular deformities in the early neonate: a preliminary report. Plast Reconstr Surg. 1984;73(1):38–51 PMID: 6691074 https://doi.org/10.1097/00006534-198401000-00009 10. Brown FE, Colen LB, Addante RR, Graham JM Jr. Correction of congenital auricular deformities by splinting in the neonatal period. Pediatrics. 1986;78(3):406–411 PMID: 3748674 11. Yotsuyanagi T. Nonsurgical correction of congenital auricular deformities in children older than early neonates. Plast Reconstr Surg. 2004;114(1):190–191 PMID: 15220591 https://doi. org/10.1097/01.PRS.0000128819.03187.5D 12. Adamson JE, Horton CE, Crawford HH. The growth pattern of the external ear. Plast Reconstr Surg. 1965;36(4):466–470 PMID: 5831865 https://doi.org/10.1097/00006534-196510000-00008 13. Farkas LG. Anthropometry of normal and anomalous ears. Clin Plast Surg. 1978;5(3):401–412 PMID: 699491 14. Farkas LG. Growth of normal and reconstructed auricles. In: Tanzer RC, Edgerton MT, eds. Symposium on Reconstruction of the Auricle. St Louis, MO: CV Mosby; 1974

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CHAPTER

5

Orthognathic Surgery STEPHEN B. BAKER, MD, DDS, FAAP, FACS

Introduction

Orthognathic surgery is the term used to describe surgical movement of the maxilla, mandible, or both jaws. The goal of orthognathic surgery is to establish ideal dental occlusion, with the jaws in a position that optimizes facial form and function. The patient’s occlusion (bite) can broadly be grouped into 3 categories: class I occlusion, a normal bite; class II occlusion, an overbite (ie, lower jaw and teeth too far behind the upper jaw and teeth); and class III occlusion, an underbite (ie, lower jaw and teeth too far forward, relative to the upper jaw and teeth) (Figure 5-1). The chance of a favorable surgical outcome is optimized if presurgical planning is performed with a dentist and orthodontist to optimize oral hygiene and align the teeth in preparation for surgery. The orthodontist’s role in the preoperative evaluation and management is critical. Prior to surgery, the patient requires a comprehensive workup that includes an analysis of the occlusion and the age of the facial skeleton. If orthognathic surgery is attempted before the facial skeleton reaches maturity, the need for revision surgery will increase because of unpredictable postoperative growth. Surgery should be delayed until growth has ceased. Skeletal growth is usually complete between the ages of 14 and 16 years in girls and between the ages of 16 and 18 years in boys. One can assess the maturity of the facial skeleton and the cessation of growth by using serial cephalometric radiographs or by looking for epiphyseal closure on hand radiographs. It is important to obtain a thorough medical and dental history from every patient. Systemic diseases such as juvenile rheumatoid arthritis, diabetes, and scleroderma may affect the treatment planning. Each patient should be questioned about symptoms of temporomandibular joint (TMJ) disorder or myofascial pain. Motivation and realistic expectations are extremely important to ensure an optimal outcome. It is important for the patient to have a clear understanding of the procedure, the recovery, and the anticipated result prior to surgery. Orthognathic surgery is a major undertaking, and the patient should be appropriately motivated to undergo all necessary perioperative orthodontic treatment and rehabilitation to achieve the desired result. 67

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Class I occlusion

Class II occlusion

Class III occlusion

Figure 5-1. Three general patterns of occlusion of the teeth (bite). Class I occlusion refers to normal occlusion; class II occlusion (overbite) refers to a position in which the upper jaw (maxilla) is anterior to the lower jaw (mandible); and class III occlusion (underbite) refers to a position where the upper jaw (maxilla) is posterior to the lower jaw (mandible).

Preparation for Surgery Physical Examination A complete physical examination should be performed on every patient prior to surgery. The frontal facial evaluation begins with the assessment of the vertical facial thirds. • Trichion (hairline) to glabella (point between the eyebrows) • Glabella to subnasale (point just under the nasal tip) • Subnasale to menton (point at the bottom of the chin) Each of these facial thirds should be about equal. If the lower two-thirds of the face is short, it can be increased by moving the maxilla inferiorly. In contrast, a long lower face may benefit from moving the upper jaw superiorly (“maxillary impaction”), which would have the opposite effect. An important factor in assessing the vertical height of the maxilla is the degree of frontal tooth (incisor) exposure, while the patient’s lips are in repose. A man should show at least 2 to 3 mm, whereas as much as 5 to 6 mm is considered attractive in a woman. If the patient shows the correct degree of incisor in repose (relaxed resting separation of the lips) but demonstrates excessive gingival show in full smile, the maxilla should not be

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affected. It is more important to have correct incisor show in repose than in full smile. The surgeon certainly would not want to bury the incisors in repose just to reduce the degree of gingival show in full smile. If lip incompetence or mentalis strain is present, it is usually an indicator of vertical maxillary excess. Asymmetries of the maxilla and mandible should be documented at physical examination, and the degree of deviation from the facial midline noted. The soft-tissue envelope of the upper face should also be evaluated for descent of the cheek fat pads, as well as the severity of the nasolabial creases. These changes are associated with aging; however, skeletal movements of the maxilla will also affect these areas. It is important for the surgeon to realize that skeletal expansion (ie, anterior or inferior repositioning) will improve the creases and folds, resulting in a more youthful appearance, whereas skeletal contraction (ie, posterior or superior repositioning) will accentuate these problems.1 The surgeon must avoid creating a patient who appears to have prematurely aged. However, the surgeon can frequently take advantage of skeletal expansion to reduce some of these soft-tissue creases, giving the patient a youthful appearance and reducing the signs of aging. In evaluating the chin, the physician should assess the labiomental angle. An acute angle may indicate a short or prominent chin, while an obtuse angle and effacement of the labiomental crease typically indicate excessive vertical length or insufficient anterior projection. The profile evaluation focuses on the projection of the forehead, malar region, maxilla and mandible, nose, chin, and neck. An experienced physician can usually determine whether the deformity is caused by the maxilla, the mandible, or both, simply by looking at the patient. This assessment is conducted clinically and verified at the time of cephalometric analysis via radiography of the facial bones. The intraoral examination should begin with an assessment of oral hygiene and periodontal health. Engagement with the patient’s dentist and orthodontist to confirm the absence or control of dental caries, gingivitis, periodontal disease, and hard- and soft-tissue oral pathology is helpful in confirming good oral health before surgery. These factors are critical for successful orthodontic treatment and surgery. Any retained deciduous teeth or unerupted adult teeth are noted. The occlusal classification is determined, and the degrees of incisor overlap and overjet (forward inclination) are quantified. The surgeon should assess the width of the maxilla because prior cleft palate repair will often result in transverse growth restriction. If the mandibular third molars are present, they must be extracted 6 months prior to surgery on the lower jaw. Any missing teeth or dental pathology should be noted, as should any signs or symptoms of TMJ disorder. These issues should be addressed prior to proceeding with orthognathic surgery. The term dental compensation is used to describe the tendency of teeth to tilt in a direction that minimizes dental malocclusion. For example, in a

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patient with an overbite (Angle class II malocclusion), the upper teeth tend to minimize the malocclusion by tipping inward (lingual retroclination), and the lower teeth tend to tip outward (labial proclination). The opposite occurs in a patient who has dental compensation for an underbite (Angle class III malocclusion). Thus, dental compensation will mask the true degree of skeletal discrepancy. Precise analysis of the dental compensation is conducted on the lateral cephalometric radiographs. If the patient is not interested in surgery, mild cases of malocclusion may be treated with further orthodontic compensation, which will camouflage the deformity and restore proper overjet and overlap. However, if the patient desires surgical correction of the deformity, orthodontia in the months before surgery will decompensate the occlusion, thereby exaggerating the malocclusion and allowing the surgeon to maximize skeletal movements. The importance of a commitment to surgery prior to orthodontics lies in the fact that dental movements for decompensation and compensation are in opposite directions, so this decision needs to be made prior to orthodontic therapy.2

Cephalometric Analysis and Models A cephalometric analysis (ie, radiography of the facial bones) and comparison with reference values can help the surgeon plan the degree of skeletal movement needed to achieve an optimal occlusion and an optimal aesthetic result. A lateral cephalometric radiograph is obtained under reproducible conditions so that serial images can be compared (Figure 5-2). This image is usually acquired at the orthodontist’s office by using a cephalostat, an apparatus specifically designed for this purpose, and a head frame to maintain

Figure 5-2. A cephalometric radiograph is used to examine the bones of the face. Both anteroposterior and lateral images are obtained.

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consistent head position. Once the normal structures are traced, several planes and angles are determined (Figure 5-3). The values obtained can be used to determine whether one or both jaws need to be moved superiorly, inferiorly, anteriorly, or posteriorly or rotated to one side or the other. The base of the skull, as determined by a line from the sella turcica to the nasion, is used as a reference that allows the physician to determine if one or both jaws contribute to the deformity. For example, a patient with an underbite could have a small upper jaw, a large lower jaw, or a combination of the two. All of these conditions yield a class III malocclusion, yet each requires a different treatment approach. The surgeon can delineate the true etiologic origin of the deformity by the fact that the upper jaw and the lower jaw can be independently quantified to a stable reference point—the cranial base. Cephalometric tracings give the surgeon an idea of how skeletal movements will affect one another, as well as the soft-tissue profile. They also allow the surgeon to determine the distances the bones will be moved to achieve the goals of specific procedures. Computer-aided cephalometric analysis can be used for isolated maxillary, isolated mandibular, or 2-jaw surgeries, which allows the surgeon to electronically position the maxilla and mandible on the cephalometric radiograph while recording the soft-tissue changes and measuring the degree of repositioning.

RO

SO

Sella

Nasion

Porion Orbitale A Point PNS

ANS

Basion

B Point

Figure 5-3. From the lateral cephalometric radiograph, points, angles, and dimensions can be traced on the face and skull to determine the relative positions of the upper and lower jaws relative to the cranial base, as well as plan the surgical steps. ANS, anterior nasal spine; PNS, posterior nasal spine; RO, roof of the orbitale; SO, supraorbitale.

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Figure 5-4. Plaster models of a patient’s teeth allow the surgeon to assess the occlusion that will result after surgery, determine whether further orthodontia is needed, plan the bone cuts needed to improve the occlusion, and fabricate acrylic splints used in surgery to move the jaws into their planned locations.

Complete dental records, including mounted dental casts, are needed to execute preoperative model surgery and fabricate surgical splints (Figure 5-4). Casts allow the surgeon to evaluate the occlusion before and after articulation into proper positions. Analysis of new occlusion gives the physician an idea of how intensive the presurgical orthodontic treatment plan will be. Casts also allow the physician to distinguish whether a transverse maxillary deficiency is present. Normally, the upper teeth overlap the lower teeth. With a transverse deficiency, the upper teeth lie inside the lower teeth (crossbite). This may need to be addressed by splitting the upper jaw into 2 pieces at the time of surgery. There are several commercially available computer-assisted treatment planning programs that can assist the surgeon with some or all of the preoperative patient preparation. Computed tomography (CT) of the patient’s face and head is performed. Although conventional helical CT scans with thin sections through the face work well, cone-beam CT offers a comparable image quality with considerably less cost and radiation exposure (50 µSv compared to 2,000 µSv). A cephalometric analysis can then be performed, as well as simulated movements of the jaws and chin in any dimension. Once the osteotomy movements are verified by the surgeon, the computer-aided design and/or manufacturing technology is used to fabricate surgical splints for the patient.3 If necessary, 3-dimensional (3D) models of the patient can

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Figure 5-5. Virtual planning may be done to plan the surgical steps and fabricate the splints.

be made to show the exact proposed movement (Figure 5-5). Some systems can actually “wrap” a 2-dimensional digital image around the soft-tissue envelope of the 3D CT image, thus replicating a 3D image of the patient’s actual face in color.

Overview of Treatment Up to Surgery The patient has multiple visits at the surgeon’s office and undergoes a thorough discussion of the surgical options. Having heard all options available to treat his or her condition, the patient and parents agree with the surgeon on the proposed plan. Good oral health has been achieved. The patient has undergone preoperative orthodontics assessment to level, align, and decompensate the occlusion. On the basis of the physical examination and radiographic findings, a treatment plan has been developed that will achieve a class I occlusion and optimize form and function. Cephalometric tracings have been used to determine the distances the jaws will have to move to achieve the desired result, and model surgery has been performed to develop surgical splints that will intraoperatively position the jaws into the position determined by the cephalometric tracing. If surgery on the lower jaw is planned, the lower third molars should be removed 6 months prior to surgery to reduce the chance of an unfavorable cut of the bone. The surgeon should verify that the splints fit and that good surgical lugs have been applied to the arch wire.

Surgical Technique General Principles Several principles have broad application to jaw surgery. Blood loss can be substantial in maxillofacial surgery, and even small volumes can have significant clinical implications in the pediatric population. A preoperative discussion with the anesthesiologist can often make a difference in improving patient experience and outcome. Standard techniques of head elevation, hypotensive anesthesia, blood donation, and administration of erythropoietin are useful adjuncts to reduce blood loss, especially in the

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younger population. Before incisions are made, an antimicrobial rinse is helpful to minimize the intraoral bacterial count. A topical steroid is applied to the lips to reduce pain and swelling associated with prolonged retraction. Intravenous steroids may also be useful to reduce postoperative edema. Elastic bands are useful postoperatively to control the bite. With rigid fixation, the elastics may not correct malposition in the jaws. They serve only to help the patient adapt to the new occlusion. Minor malocclusions can be corrected with postoperative orthodontic treatment. Certain skeletal movements are inherently more stable than others. Stable movements include mandibular advancement and superior positioning of the maxilla. Movements with intermediate stability include maxillary impaction combined with mandibular advancement, maxillary advancement combined with mandibular setback, and correction of mandibular asymmetry. The unstable movements include posterior positioning of the mandible and inferior positioning of the maxilla. The leaststable movement is transverse expansion of the maxilla. Long-term relapse with rigid fixation has not been demonstrated to be clearly superior to nonrigid fixation in single-jaw surgery. However, in 2-jaw surgery, rigid fixation results in less relapse. The judgment of the surgeon will dictate the extent to which the facial skeleton can be expanded without resulting in unacceptable relapse.

Surgically Assisted Rapid Palatal Expansion (Upper Jaw, Maxillary Surgery) Correction of transverse maxillary constriction can be addressed in adolescence, with nonsurgical orthodontic appliances. As the growth sutures begin to close during late adolescence, relapse rates increase. A multiple Le Fort I osteotomy (see the next section for details) can be performed to provide simultaneous maxillary expansion, but the degree of relapse is high. In the young adult, the preferred procedure is the surgically assisted rapid palatal expansion procedure. The orthodontist places a palatal expander prior to the procedure. A Le Fort I osteotomy is performed to completely mobilize the maxilla from the upper face. A small osteotome is used to make a thin cut between the roots of the central incisors, and a midline split is completed to the posterior nasal spine. Separation is verified by activating the device. The maxilla is widened until the gingival blanches and is then relaxed several turns to avoid ischemia. Surgically assisted rapid palatal expansion offers the best stability for maxillary expansion in the young adult and older patient. Le Fort I Osteotomy (Upper Jaw, Maxillary Surgery) The Le Fort I osteotomy is the name of the procedure that is used to move the upper jaw (maxilla) into a new position (Figure 5-6). The preoperative vertical position of the maxilla is recorded by using a large caliper to measure the distance between the medial canthus of the right eye and the

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Figure 5-6. The variations of Le Fort I osteotomy. The osteotomy can be made at various levels, depending on how much cheek projection is desired while moving the teeth into the desired occlusion.

right central incisal edge. An incision is then made inside the mouth, from first molar to first molar. The mucosa is reflected in a subperiosteal plane to expose the piriform aperture, the zygomatic buttress, and the posterolateral maxilla. The bone of the upper jaw is separated from the midface and mobilized, allowing it to be placed in its desired position. The surgical splint is then placed to orient the new position of the maxilla to the mandible. Wire loops are used to place the patient in maxillomandibular fixation. Titanium plates are used to secure the maxilla into its new position. Titanium is nonferromagnetic so that it does not set off metal detectors, and it will not pose a problem for any future magnetic resonance imaging. It is made of the same material that is used in dental implants, which have been used for more than half a century without any noted material-related complications.

Bilateral Sagittal Split Osteotomy (Lower Jaw, Mandibular Surgery) The bilateral sagittal split osteotomy is the procedure typically used to move the lower jaw (mandible) into a new position. The incisions are made inside the mouth, and the mandible is posteriorly exposed. An instrument is then used to make cuts in the bone on the right and left side of the lower jaw, so that the tooth-bearing segment of the lower jaw is mobilized from each side of the TMJ (Figure 5-7). Once the bone is cut, the tooth-bearing segment is

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Figure 5-7. The bilateral sagittal split osteotomy allows the teeth-bearing segment of the lower jaw to be moved anteriorly or posteriorly while maintaining overlapping bone. Titanium screws are placed through the overlapping portion of the osteotomy to maintain fixation while the bones heal in their desired position.

moved into the desired position, either with the final surgical splint or without a splint, if the desired occlusion can be achieved by placing the jaws into the desired occlusion. Titanium screws are then used to secure the bone segments together. Once both sides are completed, the intermaxillary fixation is released, and the final occlusion is verified.

Intraoral Vertical Ramus Osteotomy (Lower Jaw, Mandibular Surgery) Another technique for correcting mandibular prognathism or asymmetry is the intraoral vertical ramus osteotomy. The incision is the same as described earlier. A vertical cut is made in the bone of the posterior mandible. After both sides are complete, the distal segment is moved into occlusion, making sure that the proximal segments remain lateral to the distal segments posteriorly. Because rigid fixation is difficult to apply, a single wire or no fixation at all is used, and the patient requires having the jaws wired together for 4 to 6 weeks. This osteotomy can be performed from an external approach, but the incision results in a scar on the neck. Bimaxillary Osteotomies (2-Jaw Surgery) Moving the maxilla and the mandible in one procedure requires mobilizing both jaws and precisely securing them into the position determined in the treatment plan. If proper treatment planning, model surgery, and splint fabrication are performed, each jaw should be able to be placed into its desired position with precision in one procedure.

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Approaches to Commonly Encountered Problems

Once the clinical and radiographic data are obtained, the surgeon can determine which abnormalities the patient exhibits and the extent to which these features deviate from the norm. However, the treatment plan is the application of these data to give the patient the best functional and aesthetic result, while establishing a class I occlusion. The following sections outline basic treatment approaches to commonly encountered dentofacial deformities in patients undergoing orthognathic (jaw) surgery.

Skeletal Class III Occlusion (Underbite) A prominent lower jaw may be treated by advancing the maxilla, posteriorly positioning the mandible, or combining these 2 procedures. It is important to consider the contributions of the mandible and the chin separately, as each may require different treatments to achieve aesthetic goals. If some posterior positioning of the mandible is necessary, one may advance the maxilla to counteract the skeletal contraction produced from the mandible. Additionally, the patient may benefit from a chin procedure that can counteract any skeletal contraction that occurs from a mandibular setback. A minor malocclusion with minimal dental compensation may be corrected with orthodontic treatment alone. In contrast, a minor malocclusion with dental compensation may become a clinically significant malocclusion after dental decompensation, and the patient will be a good surgical candidate. Maxillary Constriction Patients can exhibit a maxilla that is narrow in a transverse dimension. Maxillary constriction may occur as an isolated finding or as one of multiple abnormalities. Up to about 15 years of age, the orthodontist can expand the maxilla nonsurgically with a palatal expander. If orthopedic expansion cannot be performed, a surgically assisted rapid palatal expansion can be conducted. If the maxilla requires movement in other dimensions, a 2-piece Le Fort I osteotomy can be performed to place the maxilla in its new position, while simultaneously achieving transverse expansion. Open Bite (Apertognathia) An anterior open bite is caused by premature contact of the posterior molars. The recommended treatment is a posterior impaction of the maxilla. By reducing the vertical height of the posterior maxilla, the mandible can come into occlusion with the remaining mandibular teeth. Posterior maxillary impaction does not necessarily result in incisor impaction; the posterior maxilla is simply rotated upward by using the incisal tip as the axis of rotation. Therefore, incisor show should not be affected. If a change in incisor show is also desired, the posterior impaction is performed, and then the whole maxilla can be inferiorly positioned or impacted to its new position.

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Vertical Maxillary Excess Vertical maxillary excess is typically associated with lip incompetence, mentalis strain, and an excessive degree of gingival show (“long face syndrome”). The treatment approach is to impact the maxilla to achieve the proper incisor show with the lips in repose. Impaction may result in skeletal contraction, however, so the surgeon must consider anterior repositioning of the jaws to neutralize the associate adverse soft-tissue effects. As the maxilla is impacted, the mandible rotates counterclockwise (with respect to a rightward-facing patient) to maintain occlusion. This rotation results in anterior positioning of the chin and is called “mandibular autorotation.” The opposite occurs if the maxilla is moved in an inferior direction. In this case, the chin point rotates in a clockwise direction, resulting in posterior positioning of the chin point. It is important to note that these affect the cephalometric tracing during treatment planning because surgery on the chin (genioplasty) may be required to reestablish proper chin position. Short Lower Face A short lower face is marked by insufficient incisor show and/or a short distance between the area beneath the nose (nasion) and the bottom of the chin (pogonion). Treatment is aimed at establishing a proper degree of incisor show. The facial skeleton should be expanded to the degree that provides optimal soft-tissue aesthetics. As the maxilla is inferiorly positioned, clockwise mandibular rotation leads to posterior positioning of the chin. The surgeon needs to assess the new chin position on the cephalometric tracing to determine if an advancement genioplasty is now necessary to counter the effects of mandibular clockwise rotation.

Complications

Improper positioning of the jaws is noted by poor occlusion or an obvious unaesthetic result. If the complication results from improper condyle position during fixation or improper indexing of the splint, fixation must be removed and reapplied. It is wise to verify splint fit prior to surgery. Meticulous treatment planning prior to surgery minimizes splint-related problems. Measures to reduce the chance of a bad split should always be used. Removal of mandibular third molars 6 months prior to the osteotomy allows time for the sockets to heal, which decreases the chance of a bad split. Bleeding may occur from any area, but most commonly, it occurs from the descending palatine artery in the maxilla. This can be stopped with packing or by placing a hemoclip on the artery. Bone wax is useful for bleeding bony edges. Nerve damage is rare but may occur. The nerves associated with these procedures are the infraorbital, the inferior alveolar, and the mental nerves. If a transection is witnessed, repair with fine suture is recommended, if

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possible. The patient should be informed that there is about a 25% chance of some paresthesia occurring immediately after surgery, but permanent changes are seen only in 1% to 2% of patients. The incidence of nonunion or malunion is rare after surgery. If a malunion occurs, the jaw may need to be osteotomized again to move it into the proper position. A nonunion would require secondary bone grafting to establish osseous continuity.

Conclusion

Orthognathic surgery is an effective treatment modality available to the craniofacial surgeon to correct dentofacial deformities. A review of the literature has shown that the procedure can be performed safely. The benefits of the surgery, such as enhanced aesthetics and optimal occlusion, must be balanced by the functional outcomes, such as a possible deterioration of speech patterns and a potential for other complications. The most important aspect in the management of these patients is that surgeons ultimately choose the techniques with which they feel comfortable, to get the best result for their patients. REFERENCES

1. Rosen HM. Facial skeletal expansion: treatment strategies and rationale. Plast Reconstr Surg. 1992;89(5):798–808 PMID: 1561250 https://doi.org/10.1097/00006534-199205000-00004 2. Tompach PC, Wheeler JJ, Fridrich KL. Orthodontic considerations in orthognathic surgery. Int J Adult Orthodon Orthognath Surg. 1995;10(2):97–107 PMID: 9082003 3. Baker SB, Goldstein JA, Seruya M. Outcomes in computer-assisted surgical simulation for orthognathic surgery. J Craniofac Surg. 2012;23(2):509–513 PMID: 22421859 https://doi. org/10.1097/SCS.0b013e31824cd46b

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CHAPTER

6

Pediatric Facial Fractures JESSE A. GOLDSTEIN, MD, AND JOSEPH E. LOSEE, MD, FAAP, FACS

Introduction

The American Academy of Pediatrics defines pediatric patients as those between birth and the age of 21 years. The wide variation in function, anatomy, and growth potential that occurs within this age range makes any single approach to facial fractures in pediatric patients challenging. Even though fractures of the craniofacial skeleton are uncommon in children when compared with adults, they are nonetheless frequently more difficult to treat because of clinically significant anatomical differences and the potential for long-term growth and development.1–6 Although the goals of treatment are the same as those for adults, the approaches to pediatric patients with facial trauma are often dramatically different.7–9 Even after years of advancement in craniofacial trauma treatment, many questions about pediatric patients remain unanswered. This chapter reviews the up-to-date literature, delineates the differences between adult and pediatric fracture patterns and management, and provides guidelines for the reconstruction of pediatric craniofacial fractures. Understanding the centers of growth and the development of the craniofacial skeleton is crucial to the ability to treat pediatric craniofacial trauma. At birth, the cranial to facial proportion is 8:1. This ratio becomes 4:1 at around 5 years of age and 2:1 in adulthood.10 The growth of the neurocranium is 25% complete at birth, 75% complete by 2 years of age, and 95% complete by 10 years of age.10–13 In contrast, facial growth demonstrates a drastically different pattern of discontinuous growth until puberty is complete. At 3 months of age, the facial dimensions are 40% of those in adults, and they become 70% by 4 years and 80% by 5 years of age.10 At approximately 5 years of age, growth slows dramatically until the beginning of puberty, at which point accelerated growth occurs, as mediated by increased hormonal input. At 17 years of age, facial growth slows down again and eventually halts. Upper facial skeletal growth occurs secondarily to cerebral and ocular growth. Lower facial growth also exhibits sequential growth. The primary growth centers of the mandible are thought to be the condyles, and they contribute to vertical growth, activated by muscular activities. These activities place stress on the periosteum, which, in turn, causes apposition and resorption of bone, creating mandibular growth in all other directions.9–11,13,14 81

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The risks of anesthetizing pediatric patients who have facial trauma should also be noted. Safety in securing a smaller pediatric airway is critical. Larger and more involved facial-type fractures that require more time in the operating room can be associated with significant blood loss and swelling. They may also require prolonged intubation and ventilation in an intensive care unit setting.

Epidemiology

Although the incidence of facial trauma is higher in children than in adults, the incidence of pediatric craniofacial fractures is much lower.15,16 Rowe2 reported that only 1% of all facial fractures occurred in children younger than 6 years. His findings have been echoed by McCoy et al,1 Panagopoulos,17 Kaban et al,18 and others. However, most of these reports were made before computed tomography (CT) was used to evaluate pediatric facial trauma, and, therefore, the investigators likely underestimated the incidence of pediatric facial fractures.19 Several factors contribute to the overall low incidence of pediatric craniofacial fractures. First, infants and young children usually live in a protected environment; therefore, the chance of trauma occurring with clinically significant effects is lower.10 As children grow older and begin to play independently and participate in sports, the risk of facial trauma increases. Second, unlike the adult population, children have a lower facial mass to cranium ratio and are, therefore, much more likely to sustain skull fractures and head injuries than facial fractures.10,20,21 Third, the pediatric facial skeleton is quite elastic because of immaturity, lack of sinuses, a higher percentage of cancellous bone, and the presence of cartilaginous sutures and growth centers. These factors allow pediatric facial bones to absorb more energy during impact without fracturing than adult facial bones can. The distribution of facial fractures varies in the pediatric population. For example, boys are twice as likely as girls to sustain facial fractures. In addition, the 6- to 12-year-old age group is more prone to facial fractures than any other age group. This is likely because the presence of mixed dentition and a higher tooth to bone ratio weakens bone.22 Other variations in fracture distribution with respect to age are likely associated with the decrease of the cranial to facial ratio that results in a more prominent face as the child grows.11,16,18 In addition, as the facial skeleton matures, it becomes increasingly mineralized and pneumatized and, therefore, more prone to fracture. As the pediatric population ages, there is a downward shift (superior-inferior) of the craniofacial trauma fracture pattern. Frontal skull and orbital fractures have a higher incidence rate in the newborn to 5-year-old age group, whereas midface and mandibular fractures have a higher incidence rate in the 6- to 16-year-old age group.23 The nose and mandible account for most reported facial fractures in older children, which is likely because of their prominence.19 McCoy et al1 and Bales et al7 found mandible fractures to be more common than nasal fractures, whereas Kaban et al18 reported the opposite. Midface fractures, including orbital,

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Figure 6-1. Computed tomography scans show a left-sided oblique craniofacial fracture pattern of the cranium (axial, left) and cranial base (axial, center). The oblique fracture is outlined in red (coronal, right). Reprinted with permission from Neligan PC, ed. Plastic Surgery. 4th ed. New York, NY: Elsevier; 2018.

zygomatic/malar, and maxillary fractures, are less common, as reported by both Kaban et al18 and McCoy et al.1 In an analysis of 772 fractures in patients who presented to the emergency department of a level 3 trauma center, Grunwaldt et al23 found the highest fracture rate in older pediatric patients, with 48% of fractures occurring in 12- to 18-year-olds. Additionally, younger patients were more likely to have frontal and orbital fractures caused by falls or motor vehicle collisions (MVCs), whereas older patients were more likely to sustain maxillary, nasal, and mandible fractures due to violence or assault, MVCs, and sports.23–25 When subjected to equal force, children develop incomplete greenstick fractures more often than adults.19,26,27 This is because the increased elasticity of the less mineralized and pneumatized pediatric skeleton and the greater cancellous to cortical bone ratio add much more flexibility to the mandible and maxilla. These factors also result in unique fracture patterns seen in children; for example, it has been reported that more oblique craniofacial fracture patterns are found in immature facial trauma (Figure 6-1).27 The classic Le Fort fracture patterns that are seen in mature craniofacial skeletons are rarely found in immature patients.

Patient Presentation

Pediatric patients with craniofacial fractures can present with typical or atypical signs of facial trauma. Malocclusion and orbital malposition may be absent because of incomplete greenstick fracture patterns, but inferior rectus entrapment is more common. Causative mechanisms of pediatric facial fractures include motor vehicle accidents (MVAs; 50%), falls (23%), and sports (15%).6 Assaults represent much less of a contributing factor4; however, violence-related facial fractures are more likely to occur in older male patients of lower socioeconomic status.24 There is a marked seasonal variation of pediatric facial

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fractures, with the peak being in July. All-terrain vehicles (ATVs) have become an increasingly common cause of pediatric facial fractures.22 Together, MVAs and ATV accidents cause more severe midface fractures than any other mechanism. This is especially evident in adolescents.22 Fortunately, child abuse is a rare cause of pediatric facial fractures, but it should be seriously considered in patients with associated unexplained long-bone fractures.

Diagnosis

Diagnosis of pediatric facial fractures is usually challenging. One must maintain a high degree of suspicion, especially in the presence of other major organ system injuries. It is not unusual to have a patient who is unable to cooperate, accompanied by anxious parents in the emergency department, which may necessitate sedated examination when safely possible. A history must be carefully obtained from the parents or caregivers and any witnesses of the incident, along with emergency medical support personnel. Preinjury photographs of the pediatric patient, dental records, and models from the patient’s dentist and orthodontist are often helpful when treating pediatric patients with facial fractures. Examination of a patient with craniofacial trauma should begin by observing the overall condition of the patient. Alertness and orientation should be carefully documented. The patient must be assessed for concussion symptoms. If any possible concussion signs or symptoms are noted, the patient should be referred to a concussion specialist for further management. Seventy-five percent of children with frontal sinus fractures have accompanying loss of consciousness,19 and close to one-half of children with facial fractures have accompanying neurological injuries. The airway should be thoroughly evaluated. Endotracheal intubation in these patients is uniquely challenging because cervical spine clearance is often not entirely possible and may be additionally complicated by mandible or midface fractures. Pediatric anesthesia support for nasal intubation over a flexible endoscope may be performed with limited cervical spine motion and may be the best way to establish a nonsurgical airway.28 If all else fails, tracheostomy or cricothyrotomy can be used. Physical examination begins with the head. Careful palpation of the skull should be performed after adequate cleansing of any scalp wounds, and this may require local anesthesia. Any scalp lacerations must be washed out and carefully examined for underlying skull fracture. Next, attention is given to the superior orbital ridge. Fractures in this region can often cause paresthesia of the forehead and scalp and may be associated with traumatic ptosis of the brow. It is important to realize that in young patients without a frontal sinus, forehead and supraorbital ridge fractures are essentially anterior cranial base fractures. Orbital examination is extremely important because there is a significantly higher percentage of ocular trauma and blinding injuries in this population than in adults. Studies have reported as high as a 3% blindness rate in children

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with orbital fractures.29 Consultation with a pediatric ophthalmologist should always be obtained when children present with periorbital trauma. Subconjunctival hematomas are pathognomonic for orbital fractures, because the conjunctiva is contiguous with the orbital periosteum; therefore, patients with these conditions must be assumed to have an orbital fracture until a CT scan is obtained. Bilateral vision should be carefully documented, and when possible, evidence of afferent papillary defects should be identified. Extraocular movements should be tested voluntarily in conscious patients, and forced duction testing should be performed in unconscious patients and in all patients undergoing surgery. If total ophthalmoplegia is found, along with upper eyelid ptosis and paresthesia in the V1 distribution, this indicates the presence of superior orbital fissure syndrome. If blindness is present in addition to these findings, orbital apex syndrome is diagnosed. Both of these conditions are ophthalmologic emergencies that may require urgent surgical decompression and high-dose steroid therapy. The medial canthal tendons should be tested in patients with periorbital trauma; this is done by using the bowstring test, which is performed by pinching the eyelids and distracting them laterally to check the stability or mobility of the medial canthus. Intercanthal distance should be measured and documented. Any periorbital lacerations medial to the lacrimal puncta indicate potential damage to the lacrimal drainage system. Examination of the nose begins with the nasal root and continues caudally. Deviation of nasal bones should be evaluated, as well as compressibility of the nasal dorsum. Any rhinorrhea or nasal discharge should raise suspicion for cerebrospinal fluid (CSF) leak, therefore prompting CT scanning and consultation with neurosurgical staff. A thorough intranasal examination should be performed by using a nasal speculum and an adequate light source, and any septal hematomas should be treated promptly. Examination of the midface begins with the inferior orbital rims. Fractures in this region are often associated with cheek paresthesia and a step deformity that can be felt with manual palpation. The entire zygoma should be palpated. Fractures of the zygoma often result in lateral canthal dystopia and loss of malar prominence. Midface stability should be tested by attempting manual distraction of the maxilla while stabilizing the head; any independent movement of the maxilla may indicate a midface fracture. However, if the patient has an obviously mobile midface, one should refrain from manipulating it more, because this may cause optic nerve trauma. Occlusion is also an important diagnostic tool when evaluating the midface, and abnormal occlusal findings should prompt further radiologic studies. While examining the midface, attention should also be given to the ears. Hemotympanum, as well as mastoid hematoma (Battle sign), may indicate a basilar skull fracture. External trauma, including subperichondrial hematomas, requires prompt treatment. Lower craniofacial skeleton examination begins with the mandible, by obtaining the patient’s own subjective assessment of occlusion whenever possible. Subjective malocclusion is a sensitive indicator of occlusal disorders. Objectively, malalignment of the dental arch and open bites are indicative of

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jaw fractures; however, patients with mixed dentition often have such preexisting malocclusions, which are normal for their development. The Angle classification should be used to document findings, and any evidence of malocclusion should prompt obtaining a panoramic radiograph (panorex) and/or a CT scan. Chin paresthesia is often found in patients with parasymphyseal fractures. Palpation of the mandible starts at the temporomandibular joint and ends at the symphysis. A high index of suspicion must always be maintained with pediatric patients because pain during palpation is often the only indicator of facial fractures. Diagnostic imaging for evaluating pediatric facial fractures has evolved over time. In modern practice, sophisticated CT algorithms have limited the role of plain radiography because the pediatric facial skeleton is immature and has a high cancellous to cortical bone ratio. Thin-section helical CT scans from skull to mandible should be used whenever possible. True coronal images are ideal, although if unable to clear the cervical spine, reconstructed coronal images based on the axial CT scan can often suffice. Three-dimensional reconstructions of thin-section CT scans are exceedingly useful for diagnosing fractures, as well as for treatment planning. Of note, the panorex is the single method of plain-film radiography that remains useful for evaluating pediatric patients who are able to cooperate for the study.

Management Skull and Forehead Children have larger cranium to face ratios than adults, and this is found in addition to the more elastic nature of pediatric bone and the lack of a developed frontal sinus,5 which begins its pneumatization around 5 years of age and completes the process around the age of 20 years.30 Because of this, children who sustain significant force to the skull often have fractures that extend to the cranial base.8 Mann et al31 reported that in 1,297 pediatric skull fractures, the incidence of associated intracranial injuries was directly related to age. Whereas children younger than 2 years with open skull fractures had a negligible chance of developing intracranial hematomas, children at 15 years of age had an incidence rate of intracranial hematoma equivalent to that of adults.31 Although children with head injuries generally have a better chance of survival than adults, the long-term sequelae are more significant. Skull fractures often occur in an oblique orientation in the pediatric population, with a fracture originating at the site of impact, extending to the supraorbital foramen, and progressing to involve the orbit and zygoma— resulting in an oblique craniofacial fracture.19 Oblique fracture patterns can be exceedingly difficult to treat, because a craniotomy is required to reduce the often depressed and impacted frontotemporal skull fracture that is contiguous with the facial fracture (see Figure 6-1). The most common mechanisms of pediatric skull fractures are MVAs, followed by falls and fights. It is important

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Figure 6-2. A, A 10-year-old with a growing skull fracture of the left forehead and orbit after trauma. Intraoperative view of the growing skull fracture. B, The reconstructed fracture is shown. Reprinted with permission from Neligan PC, ed. Plastic Surgery. 4th ed. New York, NY: Elsevier; 2018.

to remember that there is a 20% correlation between long-bone injuries and skull fracture in this population. Unique in pediatric craniofacial fractures is the growing skull fracture (Figure 6-2).32–34 A growing skull fracture is thought to occur when the fracture is associated with a dural tear. The dural diastasis allows brain pulsations to slowly separate the fracture edges, resulting in nonunion and “growth” of the fracture. One site of growing skull fractures is the frontal bone with extension into the supraorbital ridge, resulting in orbital changes such as bony orbital expansion, vertical orbital dystopia, and pulsatile exophthalmos.34 Contrary to previous reports,35 a series published by Losee et al demonstrated growing skull fractures in more than 4% of all pediatric orbital fractures and 15% of orbital roof fractures.36 For this reason, long-term follow-up is critical in this patient population. Frontal bone, frontal sinus, and cranial base fracture treatment should achieve 4 goals: isolation of intracranial structures (eg, separation of the brain from the nose), cessation of CSF leakage, prevention of posttraumatic infections (eg, pyocele, mucocele), and restoration of facial contour.19,37 Indications for surgical intervention include persistent CSF leak (longer than 4 days), intracranial/extracranial hematoma, clinically significant bony displacement, and deformed facial contour. Treatment of these fractures often requires the assistance of neurosurgical colleagues in a combined approach. Nondisplaced or minimally displaced frontal bone fractures are often treated nonsurgically. Displaced fractures are treated with a coronal incision approach, access by craniotomy, and fixation with either resorbable plating systems in young patients or wires or titanium plating systems in patients who are near adulthood (Figure 6-3). The incidence of meningitis after surgical treatment of frontal sinus fractures in pediatric patients is about 5%.38 Long-term follow-up of children with frontal bone or sinus fractures is required to ensure that their frontal sinuses

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Figure 6-3. A, A 13-year-old with frontal bone fracture after an all-terrain vehicle accident. B, The frontal bone pieces mapped out. C, Reconstruction is performed by using absorbable plates. D, A 6-month follow-up image obtained after reconstruction. Reprinted with permission from Neligan PC, ed. Plastic Surgery. 4th ed. New York, NY: Elsevier; 2018.

have adequate drainage as they grow. Routine imaging is often suggested for follow-up visits. Although routines may vary, it is reasonable to repeat CT or magnetic resonance imaging every 3 to 5 years to rule out mucocele formation. For pediatric patients with a cranial injury that involves bone loss, the task of reconstruction is difficult. The standard of reference for calvarial reconstruction remains split calvarial grafts (Figure 6-4); however, this option is unavailable to younger patients. The diploic space between the inner and outer tables of the calvarium begins to be evident at age 3 years and can be fully apparent by the age of 9 years. Therefore, when harvesting split calvarial bone grafts in children younger than 9 years, a full-thickness cranial bone graft should be obtained, and the split should be performed on the back table if possible (the sterile field in the operating room that is separated from the patient). After the age of 9 years, in situ harvesting becomes much safer. The ribs and ilium also provide a source of autogenous bone for calvarial reconstruction. Usually, multiple ribs are harvested and split. Each segment is then contoured with bone-bending forceps and applied to the defect. Rib grafts, however, have high rates of resorption.39 Although synthetic materials abound (eg, methyl methacrylate, titanium), it is best to use autogenous material when possible in a growing child.40 When using synthetic materials, the age and growth potential of the child must be considered. This is only significant for calvarial reconstruction in the 2- to 10-year-old age group, when children have lost their osteogenic potential and their diploic space is not developed enough for splitting. In the authors’ experience, a bilaminate construct composed of absorbable

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Figure 6-4. Left-sided orbital reconstruction performed by using split calvarial bone grafts. Reprinted with permission from Neligan PC, ed. Plastic Surgery. 4th ed. New York, NY: Elsevier; 2018.

mesh placed both intracranially and extracranially, interposed with a compact layer of demineralized bone matrix and chips or shavings of bone graft used in primary craniofacial surgery, has led to bony regeneration, providing a stable reconstruction in this age group. It is important to recognize that this technique is successful for primary calvarial defects with non-scarred or injured dura or scalp. In the compromised and/or scarred environment, this technique has demonstrated limited success.41 Additionally, the authors have increasingly used custom porous polyethylene implants for reconstructing large cranial defects where soft-tissue quality was uncompromised.42

Orbital Fractures Pediatric orbital trauma is rarely isolated and is often associated with zygomatic fractures.19 The orbit reaches adult size near the age of 7 years, and the small maxillary sinuses and flexible bone contribute to a lower incidence of blowout fractures in children. Tooth buds provide support to the orbit in very young children, particularly in the inferior medial region of the floor. The most common causes of pediatric orbital fractures are MVAs; the second most common cause is child abuse. The separation of the frontozygomatic suture in the lateral orbital wall is common in children, whereas it is uncommon in adults.19 In addition, the frontal sinus is absent before age 7 years. Therefore, traumatic energy is transmitted to the floor of the anterior cranial vault, which is the orbital roof, and causes isolated orbital roof fractures. It is important to note that when there is an isolated orbital floor fracture, children are prone to trapdoor-type fractures, in which the inferior rectus or inferior oblique muscle becomes trapped beneath the fractured segment, which has “snapped back” into position. This is one of the true surgical emergencies in pediatric facial trauma management (Figure 6-5). Immediate surgical exploration and release of the trapped muscle is critical, because the muscle has an increased risk of ischemic injury in a relatively

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Figure 6-5. A, A left-sided orbital floor trapdoor fracture with trapped muscle is visualized on a coronal computed tomographic scan. B, A patient with an entrapped left inferior rectus. He is being instructed to look up. Reprinted with permission from Neligan PC, ed. Plastic Surgery. 4th ed. New York, NY: Elsevier; 2018.

short period and can have permanent damage if not treated in a timely manner. Although orbital fractures are less common in children than in adults, there is a significantly higher incidence of blinding injuries in the pediatric population.29 Therefore, a higher index of suspicion for orbital injuries should be maintained for children with any evidence of periorbital trauma, and such patients should undergo thorough ophthalmologic evaluation. The presence of periorbital ecchymosis or subconjunctival hemorrhage should alert the surgeon to the possibility of orbital fracture. The infraorbital nerve is often contused or trapped in its course along the orbital floor, resulting in hypoesthesia in its distribution. Palpation of the orbital rims may elicit pain and reveal step-off deformities. Diplopia and restricted extraocular eye movements can result from entrapment of periorbital soft tissue and/or extraocular muscles. Computed tomographic examination of pediatric orbital fractures is crucial for confirming the physical findings and delineating the fracture pattern. The traditional absolute and relative indications for surgical management of orbital floor fractures commonly used in the adult patient do not apply to pediatric patients. The goals of treating pediatric orbital fractures are to restore globe position and correct visually handicapping diplopia. Treating should begin with pediatric ophthalmologic evaluation and coordination. With nondisplaced or minimally displaced fractures, a conservative approach can often have excellent results with careful weekly follow-ups. For trauma to produce posttraumatic enophthalmos or vertical orbital dystopia, there must be a composite injury to the orbital bone and supporting structures (ie, ligaments and periosteum). This composite injury allows the intraorbital volume to expand, creating changes in globe position. In our experience, pediatric patients are more likely to experience trauma that results in bony disruption that does not lead to enophthalmos, vertical orbital dystopia, or visually handicapping diplopia.36 This may be a result of greater resilience of the ocular supporting structures in the pediatric population. Therefore, it is recommended that orbital fractures in the absence of acute enophthalmos and

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vertical orbital dystopia initially be approached conservatively with close surgical and ophthalmologic follow-up, regardless of the bony fracture pattern. With malposition of the globe or persistent diplopia, the goals of surgical treatment should be restoration of orbital volume and release of all trapped soft tissue and muscle to correct globe position and diplopia. Early intervention is indicated when there is clinically significant alteration of orbital form and volume that results in vertical orbital dystopia and/or enophthalmos, because delayed repair is often difficult in children—fractures in children heal quickly. Diplopia and ocular movement abnormalities in the immediate postoperative period often resolve, provided there is adequate reduction and release of all entrapped tissue. Monitoring vision often necessitates overnight stays for children who are undergoing surgical treatment of orbital fractures. Steroids can be given if clinically significant intraorbital surgery is performed. Any ipsilateral eye pain that is unexpected or out of proportion, with or without change in vision, requires thorough examination and possible repeat surgical exploration.

Zygomaticomaxillary Fractures Isolated classic zygomatic fractures are uncommon in children. The incidence rate of zygomatic fractures in pediatric facial trauma is 4.7% when isolated, but it increases to 16.3% when associated with orbital fractures,1 and zygomatic fractures are more common with increasing age.23 This is likely because of the underdevelopment of the maxillary sinus in early childhood, which allows the zygomaticomaxillary buttress to provide rigid support. Significant force is required to cause a zygomatic fracture, because the immature bone is quite resilient. Fracture dislocation occurs through the zygomaticofrontal suture, downwardly displacing the zygoma and orbital floor and often resulting in an oblique craniofacial fracture pattern.19 The goal of treating pediatric zygomaticomaxillary fractures is restoration of preinjury facial appearance. Two functional deformities may result from significantly displaced zygomaticomaxillary fractures: orbital dysmorphology (enophthalmos and vertical orbital dystopia) and malocclusion. Nondisplaced or minimally displaced fractures are similarly treated with conservative care and close follow-up. For displaced zygomatic fractures, open-reduction internal fixation is required. Maxillary and Midface Fractures Isolated midface fractures in children are rare.43 This is an effect of the prominence of the cranium and mandible, which generally absorb most of the impact during trauma. Therefore, when midface fractures are seen in children, efforts should be made to identify other injuries, because the incidence of associated injuries ranges from 25% to 88%.1,19,44,45 Because the classic buttresses are underdeveloped, pure Le Fort fractures are uncommon and, as previously described, children often have oblique patterns of craniofacial fracture.27 The Le Fort I fracture is rare because the maxillary sinus is often

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not yet pneumatized and unerupted teeth act as internal support to the midface. Thus, the force is transferred to the alveolus, resulting in tooth avulsion and alveolar fractures.5 A midface fracture occurring at the Le Fort II level typically appears at presentation as a combined naso-orbital ethmoid and Le Fort I maxillary fracture and is often unilateral. Midface fractures occurring at the Le Fort III level are uncommon in children and classically appear at presentation as combined naso-orbital ethmoid and zygomaticomaxillary fractures. Bony injuries at this level appear at presentation as multipiece midface fractures. In contrast, because the palatal suture is incompletely ossified, midline palatal fracture separations are common in children.19 The diagnosis of a midface fracture is assigned according to the physical findings of maxillary mobility, bony step-offs, and malocclusion. Again, thin-section CT scans should be used to delineate the injury and prepare for surgery. Intracranial injury must be ruled out before surgical intervention begins because significant force is required to produce midface fractures in children. Typically, a conservative approach should be taken with infants and young children who have minimally displaced or greenstick maxillary fractures, and this usually includes a liquid or soft diet. A conservative approach is possible because pediatric patients are able to heal rapidly and they have the ability to remodel their fractures, which allows for the option of early orthodontic intervention to correct mild occlusal discrepancies. In older children and adolescents, a more classic approach with open reduction and internal rigid fixation (with or without wiring the teeth together) should be used (Figure 6-6).

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Figure 6-6. A, Piriform suspension wires used for maxillomandibular fixation. B, Maxillary “intermaxillary fixation (IMF) hooks,” similar to piriform suspension wires. C, Mandibular IMF hooks. D, Patient in maxillomandibular fixation. Reprinted with permission from Neligan PC, ed. Plastic Surgery. 4th ed. New York, NY: Elsevier; 2018.

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Cooperative interaction with pediatric dentists and orthodontists can often save a significant amount of effort when treating the patient, especially if presurgical impressions and models of the patient were obtained before the injury occurred and are available when developing corrective strategies. In infants and children, the duration of maxillomandibular fixation is often less than the 6 weeks used for adult patients. After a shortened course of rigid maxillomandibular fixation, dental elastics are used to maintain the desired occlusal relationship. Because there is substantial risk of growth disturbance, regardless of adequate treatment, parents of children with midface fractures must be informed of potential long-term growth effects and encouraged to pursue follow-up closely.

Nasal Fractures Nasal fractures are the second most common facial fracture in children and can often involve the nasal bones and cartilages, as well as the septum. Because of the relative abundance of cartilage in the pediatric nose, nasal fractures are often missed during examination in the pediatric population.46 The nasal bones in children can separate along an open midline suture, thus predisposing them to an “open book” type of fracture, which results in the nasal bones overriding the frontal process of the maxilla.10 Pediatric nasal fractures may have a significant effect on the growth and development of the facial skeleton, resulting in septal or nasal deviation, as well as midface hypoplasia. Even with early intervention and adequate reduction, cosmetic and functional deformities such as deviations, dorsal humps, saddlenose deformities, and airway obstruction can still occur. Therefore, it is important to discuss with parents the potential complications and the need for further corrective surgeries in the future. Unless clinically significant symptomatic nasal obstruction is present, secondary nasal surgery should be performed after skeletal maturity has been reached. It is often necessary for children to be given general anesthesia to be able to complete adequate nasal examination and treatment. A complete intranasal examination is routinely required to thoroughly evaluate the septum. As with other pediatric facial fractures, plain radiographic images are grossly inadequate for diagnosing nasal fractures in the pediatric population. Thin-section CT scans provide the necessary information for complete diagnosis and treatment. It is prudent to treat open fractures and septal hematomas acutely and without delay, whereas other types of nasal fractures often require a short delay to allow swelling to decrease and to reveal potential deformities. One unique injury found in pediatric nasal trauma is a hematoma between the upper lateral cartilages and the nasal bones.19,46 Because these 2 structures overlap and are loosely attached, the cartilages can easily detach, resulting in a hematoma between the cartilage and bone. This injury requires drainage through an intercartilaginous or subperiosteal incision.46 An untreated septal hematoma can lead to pressure necrosis and diminished blood supply to the septal cartilage. This can result in either thickening

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or destruction of the septum and classic saddlenose deformity. Adequate drainage of a septal hematoma requires an incision through the mucoperichondrium on the side of the hematoma at the most dependent portion. When present bilaterally, bilateral mucoperichondrial incisions or resection of a small piece of the inferior septum through a unilateral incision can be used for drainage. Silicone intranasal splints and/or nasal packing can be used for postoperative septal compression during the healing phase. Aggressive nasal surgery in infants and children is still thought to have a negative effect on the growing facial skeleton. Because of this concern, we believe that it is most appropriate to attempt closed reduction and external fixation of nasal fractures in the acute setting when there is obvious nasal bone deformity. This reduction of the nasal bones and septum should be attempted with the patient sedated or given general anesthesia. Intranasal packing is often required to maintain the reduction and is used in conjunction with an external nasal splint. When using intranasal packings, antibiotics should be given to prevent the potential risk of septic shock syndrome.47 Because facial fractures heal quickly in younger children, splints are rarely needed after 5 days.

Mandibular Fractures Mandibular fractures are the most common facial fractures reported in children, likely because the bony mandible is prominent. The reported incidence rate of mandible fractures is between 20% and 40% of all pediatric facial fractures.21,48 This high incidence rate may also be caused by the probable underreporting of nasal and dentoalveolar fractures. Mandibular fractures are uncommon in children younger than 6 years; therefore, the presence of these fractures should alert the physician to look for concomitant injuries. The incidence of pediatric mandibular fractures increases between 6 and 15 years of age.3 As children grow, the distribution of fractures in the various regions of the mandible also change. Condylar fractures are common during the first 6 years after birth (43.4%), but this decreases to 7% to 10% in adolescents aged 13 to 18 years.5,49 In adolescents older than 15 years, 76% of mandibular fractures occur in the regions of the angle and body of the mandible.49 The characteristics of pediatric mandibular fractures also differ from those in the adult population and include more greenstick fractures and long, irregular sagittal fractures.50 All these issues should be considered before undertaking management of pediatric mandibular fractures. Attention should be given to cervical spine injuries, because the force required to produce a fracture is significant. The most common symptoms of pediatric mandibular fracture are pain and malocclusion. Patients often complain that their teeth do not come together. Drooling and trismus, with a decreased maximal incisal opening, are often present. A dental step-off, with bleeding, swelling, and ecchymosis, is routinely found intraorally. In addition, classic patterns of malocclusion are easily noted. Although bilateral condylar fractures usually result in an anterior open bite, unilateral condylar fractures often manifest with contralateral

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posterior open bites. With condylar neck fractures, the condylar head is often medially displaced from the pull of the pterygoid muscles. The temporal mandibular joint can be assessed by placing fingers in the external auditory canal while the child opens and closes his or her mouth. Another useful technique when diagnosing pediatric mandible fracture is simply placing the heel of an examiner’s hand on the patient’ symphysis and asking the patient to push against it with his or her chin. If there is significant pain, mandible fracture should be suspected. Although a panorex is useful in older children, a CT scan is the most reliable way to delineate mandibular fractures in the pediatric population. To safely treat pediatric patients who have mandibular fractures, one must understand basic dental development. The deciduous mandibular incisors first appear at approximately 6 months of age, followed by eruption of the molars and then the cuspids. Even with their conical shape, primary tooth roots are strong enough for interdental wiring between 3 and 6 years of age, but care should be taken when circumdental wires are placed.51 Mixed dentition is present between 6 and 12 years of age, and the resorption of primary tooth roots, along with underdevelopment of permanent tooth roots, creates a unique challenge for securing arch bars in this age group. It is, therefore, helpful to perform a panorex study in these patients to delineate teeth with stable roots. The surgeon must be creative and well versed in all manner of interdental fixation, such as acrylic splints, circummandibular wiring, drop piriform aperture suspension wiring (Figure 6-7), and nasal spine suspension wiring. Even though teeth are available for placing circumdental wires and arch bars in younger patients (3–4 years of age), the inability of these patients to understand and cooperate can often result in disruption of intermaxillary fixation

Figure 6-7. Intraoperative view illustrates circummandibular and piriform drop wires for maxillomandibular fixation and a resorbable inferior border plate.

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(IMF) in the early postsurgical period because of their constant struggle against fixation. In this younger age group, we suggest that circummandibular and piriform suspension wires be placed in addition to arch bars for added maintenance of IMF.51 When treating jaw fractures, occlusal relationships are of the utmost importance. If available, every effort should be made to obtain up-to-date preinjury models to be able to make occlusal splints. Alternatively, presurgical impressions of the child’s injured jaws can be obtained, and these impressions can be used to create stone models that may be cut, manipulated, and mounted on an articulator. After establishing the ideal occlusion with the models, a surgical splint is made and used intraoperatively to guide the surgeon. Treatment of pediatric mandibular fractures should be tailored to address each region of the mandible specifically. The goals are the same as for adults: restore normal occlusion, ensure bony union, and avoid infection.19 Because the pediatric mandible is full of developing dental follicles, a conservative approach for treating fractures is always indicated.48 Minor malocclusions from fractures are often well tolerated in infants and young children, because they have a remarkable ability to remodel their fractures well, and an orthodontist may manipulate and correct minor occlusal discrepancies at a later date. Therefore, if a fracture creates minimal displacement and the occlusion is essentially normal, a conservative approach is warranted. This usually includes a soft diet, dental hygiene, and mandibular rest. For patients with displaced fractures and clinically significant malocclusion, surgical intervention is indicated. Many consider it appropriate to remove all non-resorbable fixation after fracture healing in growing children.

Panfacial Fractures Panfacial fractures, by definition, involve the upper, middle, and lower craniofacial skeleton. This fracture pattern is exceedingly rare in pediatric patients. When children experience panfacial fractures, the fractures are usually associated with multiorgan injuries that make management of the fractures challenging. It is important to develop a comprehensive treatment plan before surgery. This often includes coordination with the neurosurgery department for transcranial access, obtaining presurgical models for fabricating occlusal splints, and communicating with other surgical specialties and pediatric anesthesia personnel to maximize the resuscitation of the patient before the prolonged surgery he or she may need to undergo.

Postsurgical Care

The immediate postsurgical period may be exceedingly stressful for pediatric patients who undergo surgical treatment of facial fractures. This is especially true when IMF is involved. Parental presence and constant reassurance are an essential part of postsurgical care. Surgically, there are 2 areas that need

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special attention. First, the airway needs to be protected. This requires experience in the care of these patients to know when a surgical airway is required, and the decision should be made before fixation of the fractures. Second, in patients who have fractures involving the orbit, serial, thorough ophthalmologic examinations should be performed. Any signs of superior orbital fissure syndrome or orbital apex syndrome need to be addressed immediately. Antibiotics are not mandatory but should be used when specifically indicated. When bone grafts or hardware are used during the fixation of facial fractures, we advocate a short course (3–5 days) of antibiotics. Antibiotics are also indicated when there is salivary contamination of the fractures or when the frontal sinus and intracranial space are involved. Careful wound care and oral hygiene are also important. The authors’ protocol includes antibiotic mouthwash every 4 hours in the immediate postsurgical period and antibiotic ointment on all skin incisions. The role of parenteral steroids remains controversial. Their use is thought to be most beneficial for decreasing swelling in the airway and posterior orbit. Dexamethasone is usually administered intravenously as a single dose of 0.5 to 1.0 mg per kilogram of body weight.52 Repeating and/or tapering additional doses is often considered. Higher doses may be required for optic neuropathy, and the guidance of a pediatric ophthalmologist is recommended. The potential risks of steroid use include postsurgical infection, aseptic necrosis of the hip, and adrenal insufficiency, all of which are rare. Routine postsurgical CT scans are not always indicated; however, they are invaluable for evaluating reduction and fracture alignment and for documenting postsurgical treatment. These studies, in the authors’ opinion, remain the most valuable learning tool for surgeons who have limited experience with surgical management of pediatric facial fractures. All children who have sustained facial fractures require annual follow-up during the period of growth and development of their craniofacial skeleton. Annual physical examination and assessment by a craniofacial surgeon and orthodontist are suggested. Yearly cephalograms and panorex radiographs, along with dental impressions and standardized photographs, are obtained to document and track facial growth and development. We cannot stress enough the importance of serial dental and facial photographs obtained in standard settings. These annual photographs not only document the injury and wound healing but also show subtle soft tissue, skeletal, and dental changes that may or may not occur after fixation of facial fractures. A coordinated team approach is recommended for the care of pediatric patients with facial fracture, similar to the care of patients with cleft and craniofacial disorders. The team should include the craniofacial surgeon, pediatric neurosurgeons, pediatric orthodontists and dentists, a pediatric ophthalmologist, a social worker, and a pediatric developmental specialist/ psychologist who will focus on the developmental and psychological effects of the injury.

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Complications

Postsurgical complications that can occur after the treatment of pediatric facial fractures can be divided into 3 categories: those that occur as a result of the injury, those that occur as a result of the treatment, and those that occur over time with altered growth and development.53,54 The most drastic immediate complication is compromised vision. As previously noted, even though children experience fewer facial fractures in general, when they do, there is a higher incidence of blindness than in adult populations. It is imperative to have a pediatric ophthalmologist evaluate the patient presurgically to document a baseline examination and diagnose any injury. A postsurgical examination should also be completed. If vision deteriorates in the immediate postsurgical period, timely repeat surgical exploration is warranted. Infection can also occur in the immediate postsurgical period but is unlikely in healthy young patients. Infection is more concerning when hardware or bone grafts were used for reconstruction. Any early infection requires judicious use of culture-specific antibiotics, drainage, and irrigation. Infections in the oral cavity after open reduction and internal fixation with hardware exposure can often be treated with antibiotics and good oral hygiene, without hardware removal. True infections that occur elsewhere in the craniofacial skeleton are more concerning and may require washout and removal of hardware. Bone grafts rarely become infected, but if this should occur, they invariably require removal. This usually creates clinically significant defects that are cosmetically and functionally displeasing when the soft-tissue envelope collapses and scars. Often, these patients require extensive secondary reconstruction with autogenous split calvarial or rib grafts. It is prudent to perform such reconstruction in a delayed manner, allowing the infection to subside and preventing further graft loss. Infection can also occur in the subacute setting, which is more common when using non-autogenous materials such as those used for cranioplasty. When these infections occur, all of the offending material must be removed, and a delayed secondary autogenous reconstruction must be performed. Late postoperative complications in children with craniofacial fractures include bony contour deformities (eg, saddlenose), ophthalmologic disturbances (eg, enophthalmos, orbital dystopia), orthognathic deformities (eg, malocclusion), and the rare growing skull fracture. It is not uncommon to have minor contour discrepancies after the initial treatment of craniofacial fractures when unpredictable growth and development follow. Most often, these deformities do not require clinically significant surgical corrections. However, when major bony deformities develop, extensive reconstruction with autogenous calvarial bone or rib grafts is usually required. Postsurgical ophthalmologic disturbances include orbital volume‒related problems, such as enophthalmos and orbital dystopia, as well as soft-tissue and/or scarring problems that can include ectropion and extraocular muscle

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dysfunction. The goal of reconstruction is to restore pre-traumatic orbital volume and morphology. When this restoration is inadequate, or when bone grafts are absorbed and soft tissues atrophy, enophthalmos may occur. Characteristic areas of bony loss include the orbital floor and the posteromedial and lateral orbital wall. The basis of secondary orbital volume restoration is purely anatomical. This begins with alignment of the lateral orbital wall (greater wing of the sphenoid), establishing the key landmark for reduction of all other components of the orbital fracture. Often, the malaligned zygomaticomaxillary fracture needs to be re-osteotomized to mobilize and reduce the fracture segment. Additional osteotomies may be needed to restore natural anatomy. After these units are stabilized by miniplates, the floor and medial wall defects can be repaired. A coronal incision may be needed to approach the posterior aspect of the medial wall. Posttraumatic exophthalmos rarely occurs. It is most likely caused by a superior orbital roof fracture and herniation of the brain into the bony orbit. This generally requires a coronal incision and frontal craniotomy to reduce the herniated brain and place a bone graft and pericranial flap to cover the defect. Exophthalmos can also occur with excess correction and reduction of orbital volume. In the pediatric population, a growing skull fracture can also occur after an orbital roof fracture, and this may cause pulsatile exophthalmos. Secondary orthognathic deformities occur in recognized patterns after facial fracture, with or without treatment.55 An elongated face with an anterior open bite may occur after Le Fort fractures that are inadequately reduced and the posterior maxilla is inferiorly and posteriorly displaced.1 This pattern of malocclusion can also occur when posterior mandibular height is lost by means of improper fixation of mandibular fractures or when the condyles are not correctly seated into the fossa at the time of the repair. To correct an anterior open bite, the fracture pattern is recreated, and anatomical reduction is performed by using IMF. A posterior maxillary crossbite can occur if the ipsilateral mandibular ramus or angle fracture is inadequately reduced. This often requires orthodontic treatment to correct and, if severe enough, secondary orthognathic surgery. As discussed previously, the condyles in the pediatric mandible are growth centers. Therefore, condylar injuries have the potential to disrupt bony growth, causing asymmetry and malocclusion. This most commonly manifests as secondary asymmetrical mandibular retrognathism and a decreased maximal incisal opening, which gives the patient an ipsilateral occlusal cant and a deviated dental midline. To correct this deformity, distraction osteogenesis can be performed, or orthognathic surgery may be undertaken once skeletal growth has been completed. The typical orthognathic procedures used to correct this deformity are bilateral sagittal split osteotomies of the mandible with a possible combined Le Fort I osteotomy, with or without osseous genioplasty.

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Long-term Consequences

Despite timely and adequate treatment of pediatric facial fractures, long-term results remain unpredictable. Growth and developmental disturbances may occur from the fracture itself, an open approach and periosteal undermining, rigid fixation of the fracture, and disruption of growth centers within the pediatric facial skeleton. Numerous reports have documented unpredictable growth patterns and delayed deformities in the pediatric population after facial trauma. For example, growth disturbances associated with nasal fractures occur from a suspected premature ossification of the septovomerine suture,2 while some reports indicate that zygomatic fractures rarely incur any longterm consequences.10 Orbital fractures that occur before the age of 7 years have been noted to cause growth disturbances; however, orbital growth is often complete after 7 years of age, and fractures after this are rarely associated with abnormal development.10 Naso-orbital‒ethmoid fractures are not common in children; however, when they do occur, the disruption of facial growth centers can result in midface hypoplasia in the vertical and anteroposterior directions.2 The literature lacks a comprehensive long-term follow-up of pediatric patients with facial fractures. The authors are currently involved in establishing a multicenter pediatric facial trauma registry to document long-term consequences of facial fractures on the pediatric population and have published an early follow-up of a cohort of 177 patients with more than 1 year of follow-up.54 This study introduced a classification system for facial fracture–related adverse outcomes: type 1 adverse outcomes were those directly related to the fracture; type 2 adverse outcomes were directly related to the treatment; and type 3 adverse outcomes were related to the interaction between the fracture, the treatment, and patient growth. Factors associated with worse outcomes included multiple fractures and fractures that required surgical intervention. REFERENCES

1. McCoy FJ, Chandler RA, Crow ML. Facial fractures in children. Plast Reconstr Surg. 1966;37(3):209–215 PMID: 5932984 https://doi.org/10.1097/00006534-196603000-00005 2. Rowe NL. Fractures of the facial skeleton in children. J Oral Surg. 1968;26(8):505–515 PMID: 5243132 3. Rowe NL. Fractures of the jaws in children. J Oral Surg. 1969;27(7):497–507 PMID: 4893248 4. Carroll MJ, Hill CM, Mason DA. Facial fractures in children. Br Dent J. 1987;163(1):23–26 PMID: 3475087 https://doi.org/10.1038/sj.bdj.4806175 5. Bartlett SP, DeLozier JB III. Controversies in the management of pediatric facial fractures. Clin Plast Surg. 1992;19(1):245–258 PMID: 1537222 6. Posnick JC, Wells M, Pron GE. Pediatric facial fractures: evolving patterns of treatment. J Oral Maxillofac Surg. 1993;51(8):836–844 PMID: 8336220 https://doi.org/10.1016/S0278-2391 (10)80098-9 7. Bales CR, Randall P, Lehr HB. Fractures of the facial bones in children. J Trauma. 1972;12(1): 56–66 PMID: 5008863 https://doi.org/10.1097/00005373-197201000-00007 8. Gussack GS, Luterman A, Powell RW, Rodgers K, Ramenofsky ML. Pediatric maxillofacial trauma: unique features in diagnosis and treatment. Laryngoscope. 1987;97(8 Pt 1):925–930 PMID: 3613792 https://doi.org/10.1288/00005537-198708000-00008

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101 Chapter 6: Pediatric Facial Fractures 9. Enlow DH. Facial Growth. Philadelphia, PA: WB Saunders; 1990:25–130 10. Singh DJ, Bartlett SP. Pediatric craniofacial fractures: long-term consequences. Clin Plast Surg. 2004;31(3):499–518 PMID: 15219755 https://doi.org/10.1016/j.cps.2004.03.012 11. Haug RH, Foss J. Maxillofacial injuries in the pediatric patient. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2000;90(2):126–134 PMID: 10936829 https://doi.org/10.1067/moe.2000. 107974 12. Sperber GH, ed. Craniofacial Development. London, United Kingdom: BC Decker; 1991:67–111 13. Sticker M, Raphael B, Van der Meulen J. Craniofacial development and growth. In: Craniofacial Malformations. New York, NY: Churchill Livingstone; 1990:61–85 14. Fields HW. Craniofacial growth from infancy through adulthood. Background and clinical implications. Pediatr Clin North Am. 1991;38(5):1053–1088 PMID: 1886737 https://doi. org/10.1016/S0031-3955(16)38189-5 15. Posnick J. Discussion of “The effects of rigid fixation on craniofacial growth of rhesus monkeys” by Yaremchuk MJ, Fiala TG Barker F, et al. Plast Reconstr Surg. 1994;93(1):11–15 https://doi. org/10.1097/00006534-199401000-00002 16. Zerfowski M, Bremerich A. Facial trauma in children and adolescents. Clin Oral Investig. 1998;2(3):120–124 PMID: 9927912 https://doi.org/10.1007/s007840050056 17. Panagopoulos AP. Management of fractures of the jaws in children. J Int Coll Surg. 1957;28(6 Pt 1): 806–815 PMID: 13481421 18. Kaban LB, Mulliken JB, Murray JE. Facial fractures in children: an analysis of 122 fractures in 109 patients. Plast Reconstr Surg. 1977;59(1):15–20 PMID: 831236 https://doi.org/10.1097/ 00006534-197701000-00002 19. Shultz RC, Meilman J. Complications of facial fractures. In: Goldwyn RM, ed. The Unfavorable Result in Plastic Surgery. Boston, MA: Little Brown; 1984 20. Demas PN, Braun TW. Pediatric facial injuries associated with all-terrain vehicles. J Oral Maxillofac Surg. 1992;50(12):1280–1283 PMID: 1447606 https://doi.org/10.1016/0278-2391(92)90227-Q 21. Reedy BK, Bartlett SP. Pediatric facial fractures. In: Bentz ML, ed. Pediatric Plastic Surgery. Stamford, CT: Appleton & Lange; 1998:463–486 22. Iizuka T, Thorén H, Annino DJ Jr, Hallikainen D, Lindqvist C. Midfacial fractures in pediatric patients. Frequency, characteristics, and causes. Arch Otolaryngol Head Neck Surg. 1995; 121(12):1366–1371 PMID: 7488365 https://doi.org/10.1001/archotol.1995.01890120026005 23. Grunwaldt L, Smith DM, Zuckerbraun NS, et al. Pediatric facial fractures: demographics, injury patterns, and associated injuries in 772 consecutive patients. Plast Reconstr Surg. 2011;128(6):1263–1271 PMID: 21829142 https://doi.org/10.1097/PRS.0b013e318230c8cf 24. Mericli AF, DeCesare GE, Zuckerbraun NS, et al. Pediatric craniofacial fractures due to violence: comparing violent and nonviolent mechanisms of injury. J Craniofac Surg. 2011;22(4):1342–1347 PMID: 21772183 https://doi.org/10.1097/SCS.0b013e31821c944c 25. MacIsaac ZM, Berhane H, Cray J Jr, Zuckerbraun NS, Losee JE, Grunwaldt LJ. Nonfatal sport-related craniofacial fractures: characteristics, mechanisms, and demographic data in the pediatric population. Plast Reconstr Surg. 2013;131(6):1339–1347 PMID: 23714794 https://doi. org/10.1097/PRS.0b013e31828bd191 26. Messinger A, Radkowski MA, Greenwald MJ, Pensler JM. Orbital roof fractures in the pediatric population. Plast Reconstr Surg. 1989;84(2):213–216 PMID: 2748736 https://doi.org/10.1097/ 00006534-198908000-00003 27. Moore MH, David DJ, Cooter RD. Oblique craniofacial fractures in children. J Craniofac Surg. 1990;1(1):4–7 PMID: 2088563 https://doi.org/10.1097/00001665-199001000-00004 28. Davidson JS, Birdsell DC. Cervical spine injury in patients with facial skeletal trauma. J Trauma. 1989;29(9):1276–1278 PMID: 2769811 https://doi.org/10.1097/00005373-198909000-00016 29. Holt GR, Holt JE. Incidence of eye injuries in facial fractures: an analysis of 727 cases. Otolaryngol Head Neck Surg. 1983;91(3):276–279 PMID: 6410328 https://doi.org/10.1177/ 019459988309100313 30. Graney DO. Anatomy of paranasal sinues. In: Cummings CW, Fredrickson JM, Harker LA, eds. Otolaryngology. St Louis, MO: CV Mosby; 1986 31. Mann KS, Chan KH, Yue CP. Skull fractures in children: their assessment in relation to developmental skull changes and acute intracranial hematomas. Childs Nerv Syst. 1986;2(5): 258–261 PMID: 3791285

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102 Pediatric Plastic and Reconstructive Surgery for Primary Care 32. Lende RA, Erickson TC. Growing skull fractures of childhood. J Neurosurg. 1961;18:479–489 PMID: 13760791 https://doi.org/10.3171/jns.1961.18.4.0479 33. Thompson JB, Mason TH, Haines GL, Cassidy RJ. Surgical management of diastatic linear skull fractures in infants. J Neurosurg. 1973;39(4):493–497 PMID: 4730339 https://doi.org/10.3171/ jns.1973.39.4.0493 34. Havlik RJ, Sutton LN, Bartlett SP. Growing skull fractures and their craniofacial equivalents. J Craniofac Surg. 1995;6(2):103–110 PMID: 8601014 https://doi.org/10.1097/00001665199503000-00003 35. Menkü A, Koç RK, Tucer B, Kurtsoy A, Akdemir H. Growing skull fracture of the orbital roof: report of two cases and review of the literature. Neurosurg Rev. 2004;27(2):133–136 PMID: 14614595 https://doi.org/10.1007/s10143-003-0311-8 36. Losee JE, Afifi A, Jiang S, et al. Pediatric orbital fractures: classification, management, and early follow-up. Plast Reconstr Surg. 2008;122(3):886–897 PMID: 18766055 https://doi.org/10.1097/ PRS.0b013e3181811e48 37. Ioannides C, Freihofer HP, Vrieus J, Friens J [corrected to Vrieus J]. Fractures of the frontal sinus: a rationale of treatment. Br J Plast Surg. 1993;46(3):208–214 PMID: 8490699 https://doi. org/10.1016/0007-1226(93)90170-G 38. Wallis A, Donald PJ. Frontal sinus fractures: a review of 72 cases. Laryngoscope. 1988;98(6 Pt 1): 593–598 PMID: 3374232 https://doi.org/10.1288/00005537-198806000-00002 39. Goldstein JA, Paliga JT, Bartlett SP. Cranioplasty: indications and advances. Curr Opin Otolaryngol Head Neck Surg. 2013;21(4):400–409 PMID: 23770828 https://doi.org/10.1097/MOO. 0b013e328363003e 40. Koenig WJ, Donovan JM, Pensler JM. Cranial bone grafting in children. Plast Reconstr Surg. 1995;95(1):1–4 PMID: 7809219 https://doi.org/10.1097/00006534-199501000-00001 41. Chao MT, Jiang S, Smith D, et al. Demineralized bone matrix and resorbable mesh bilaminate cranioplasty: a novel method for reconstruction of large-scale defects in the pediatric calvaria. Plast Reconstr Surg. 2009;123(3):976–982 PMID: 19319063 https://doi.org/10.1097/ PRS.0b013e31819ba46f 42. Lin AY, Kinsella CR Jr, Rottgers SA, et al. Custom porous polyethylene implants for large-scale pediatric skull reconstruction: early outcomes. J Craniofac Surg. 2012;23(1):67–70 PMID: 22337376 https://doi.org/10.1097/SCS.0b013e318240c876 43. Enlow DH. Handbook of Facial Growth. 2nd ed. Philadelphia, PA: WB Saunders; 1982 44. MacLennan WD. Injuries involving the teeth and jaws in young children. Arch Dis Child. 1957; 32(166):492–494 PMID: 13498790 https://doi.org/10.1136/adc.32.166.492 45. McGraw BL, Cole RR. Pediatric maxillofacial trauma. Age-related variations in injury. Arch Otolaryngol Head Neck Surg. 1990;116(1):41–45 PMID: 2294939 https://doi.org/10.1001/ archotol.1990.01870010045014 46. Dufresne CR, Manson PN. Pediatric facial trauma. In: McCarthy JG, ed. Plastic Surgery. Vol 2. Philadelphia: WB Saunders; 1990 47. Jacobson JA, Kasworm EM. Toxic shock syndrome after nasal surgery. Case reports and analysis of risk factors. Arch Otolaryngol Head Neck Surg. 1986;112(3):329–332 PMID: 3942641 https:// doi.org/10.1001/archotol.1986.03780030093019 48. Smith DM, Bykowski MR, Cray JJ, et al. 215 mandible fractures in 120 children: demographics, treatment, outcomes, and early growth data. Plast Reconstr Surg. 2013;131(6):1348–1358 PMID: 23714795 https://doi.org/10.1097/PRS.0b013e31828bd503 49. Siegel MB, Wetmore RF, Potsic WP, Handler SD, Tom LW. Mandibular fractures in the pediatric patient. Arch Otolaryngol Head Neck Surg. 1991;117(5):533–536 PMID: 2021472 https://doi.org/ 10.1001/archotol.1991.01870170079017 50. Hall RK. Injuries of the face and jaws in children. Int J Oral Surg. 1972;1(2):65–75 PMID: 4199032 https://doi.org/10.1016/S0300-9785(72)80020-6 51. Naran S, Keating J, Natali M, et al. The safe and efficacious use of arch bars in patients during primary and mixed dentition: a challenge to conventional teaching. Plast Reconstr Surg. 2014;133(2):364–366 PMID: 24469169 https://doi.org/10.1097/01.prs.0000436842.07871.b6 52. Rubin PA, Bilyk JR, Shore JW. Management of orbital trauma: fractures, hemorrhage, and traumatic optic neuropathy. Focal Points: Clin Mod Ophthalmol. 1994;12(7)

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103 Chapter 6: Pediatric Facial Fractures 53. Chao MT, Losee JE. Complications in pediatric facial fractures. Craniomaxillofac Trauma Reconstr. 2009;2(2):103–112 PMID: 22110803 https://doi.org/10.1055/s-0029-1215873 54. Rottgers SA, Decesare G, Chao M, et al. Outcomes in pediatric facial fractures: early follow-up in 177 children and classification scheme. J Craniofac Surg. 2011;22(4):1260–1265 PMID: 21772202 https://doi.org/10.1097/SCS.0b013e31821c6ab7 55. Kawamoto HK. Correction of Established Traumatic Deformities of the Facial Skeleton Using Craniofacial Principles in Facial Injuries. St Louis, MO: Mosby-Year Book; 1988

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CHAPTER

7

Eyelid Anomalies PETER J. TAUB, MD, MS, FAAP, FACS

Introduction

There is perhaps no more upsetting facial anomaly to a parent than one that affects the periorbital structures. In addition to the obvious disfigurement, there may be functional and developmental concerns for vision. Preservation of normal visual development is critical. The globe needs to be adequately protected by the eyelids, and the visual axis must be free of obstruction. The treating physician needs to consider the eyes in the possibly larger setting of a syndromic disorder, and this should be identified before considering surgical correction. It is important to consider that the pediatrician will often be the initial driver of diagnostic evaluations and needs to be aware of all possibilities when confronted with these anomalies.

Visual Development

Visual stimulation of the brain is critically important for sight development. The brain also needs to process the visual cues taken in by the eyes to be able to understand its surroundings. Any obstruction to the visual axis results in errors of development. The occipital lobe in the posterior aspect of the brain houses the visual cortex. After birth, neurons in this region must be stimulated by visual stimuli from the eyes to be able to form directional columns. Direction columns are required to see lines, patterns, and movement. Newborns and infants learn to see over time, beginning in the neonatal phase. They have not yet developed the ability to easily tell the difference between 2 objects or move their eyes between the 2 images. Their primary focus is on objects 8 to 10 inches from their face. The ability to focus their eyes, move them accurately, and use them for stereoscopic vision must be learned. For the first 2 months after birth, an infant’s eyes are not well coordinated and may appear to wander or be crossed. This is usually normal. However, if an eye appears to turn in or out constantly, an evaluation is warranted. During the first months after birth, the eyes start working together, and vision rapidly improves. Eye-hand coordination begins to develop as infants start tracking moving objects with their eyes and reaching for them. By 8 weeks, infants begin to more easily focus their eyes on the face of a parent or other 105

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person near them. Infants should begin to follow moving objects with their eyes and reach for things at around 3 months of age. From 5 to 8 months of age, coordination skills continue to improve. This includes the control of eye movement and integration of the eyes with movement of other body parts. The ability to perceive depth and see objects in 3 dimensions—which is not present at birth—becomes possible. This helps infants to explore the environment around them as they begin to crawl at around 8 months of age. From 9 to 12 months of age, eye-hand coordination becomes more established. The infant can go from sitting to standing and grasp objects. This will progress to cruising and individual walking. By the end of the first year, infants should be able to judge distance.

Plastic Surgery Evaluation

When evaluating pediatric patients with eyelid deformities, the plastic surgeon should be aware that what the physician perceives as abnormal, such as epicanthal folds, may be considered normal to the family, as a parent or relative may have the same anomaly. Some family members may have undergone reconstructive surgery and may request the same for their child. Conversely, others may have not believed the anomaly was particularly unusual and may have left it alone. The anomalies that threaten vision are certainly important and require urgent intervention. However, those that affect the appearance of the child should not be minimized, because they may affect the child’s self-perception and psychosocial development.

Developmental Anomalies Epicanthal Folds Epicanthal folds are redundant skin creases in the area of the medial canthus. They are normal findings in Asian populations and are also found in patients with Down syndrome and blepharophimosis. Treatment involves eliminating the adhesions that contribute to the fold to produce a concave contour in the region of the medial canthus. Horizontally oriented flaps serve to break up the vertical contour. Telecanthus Telecanthus refers to an increase in the distance between the medial canthi (Figure 7-1). The intercanthal distance in a typically developing individual is approximately one-half the distance between the 2 pupils. In the case of telecanthus, the position of the orbits is normal, but the width of the soft tissue between the medial canthi is increased. In contradistinction, hypertelorism refers to an increase in the distance between the 2 bony orbits. Telecanthus can be associated with Waardenburg syndrome type 6 and is always part of

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Figure 7-1. A pediatric patient with congenital telecanthus caused by frontonasal dysplasia.

the congenital eyelid syndrome, blepharophimosis. Treatment of telecanthus involves pulling the medial canthi back toward the midline by placing one or more wires across the bridge of the nose. Prior to this, computed tomography should be performed to ensure that the cribriform plate is in a normal position.

Blepharophimosis Eyelid Syndrome The term blepharophimosis refers to vertical and horizontal shortening of the palpebral fissures. When this occurs with telecanthus, epicanthus inversus, and congenital ptosis, it is called congenital eyelid or blepharophimosis eyelid syndrome. Two types have been identified, as differentiated by the presence or absence of premature ovarian failure. Girls with blepharophimosis should undergo an appropriate evaluation of their ovaries with ultrasonography and endocrine testing. Blepharophimosis eyelid syndrome has been identified as autosomal dominant, and the FOXL2 gene on chromosome 3q23, which is a transcription factor, has been linked to the syndrome.1 Other syndromes in which blepharophimosis is observed include aniridia-Wilms tumor association, deletion 18p, FGFR3-associated coronal synostosis, Noonan syndrome, Chotzen syndrome, and cerebro-oculofacioskeletal syndromes.2 The timing of surgical repair depends on the severity of the patient’s visual acuity and the related extension posturing of the neck that allows the patient to see beneath the lid margin. Patients with unilateral upper eyelid ptosis have a greater risk of amblyopia as a preference for one eye over the other develops. Patching the dominant eye for 1 to 2 hours after 6 weeks of age assists with (but does not ensure) the prevention of amblyopia. Serial measurements of visual acuity are useful for monitoring visual development. Failure of improvement after conservative measures warrants surgical correction during the first 3 to 6 months after birth or even sooner for extremely severe degrees

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of ptosis that are sometimes seen with blepharophimosis eyelid syndrome. Surgical correction involves staged repair, beginning with the ptosis, between the ages of 9 and 12 months by using synthetic material. Repair of the epicanthal folds and telecanthus can then be performed at a late stage, although milder forms may be repaired simultaneously with the initial ptosis repair. A suspension of the eyelid to the frontalis muscle with autogenous fascia lata can be accomplished at around 4 to 5 years of age.

Euryblepharon Euryblepharon refers to an enlarged palpebral fissure. Children may present with symmetrical, horizontal enlargement of the eyelid; tight lower eyelid skin; ectropion laterally; poor apposition of the eyelid to the globe; and chronic eye irritation. A space between the outer aspect of the globe and the eyelid caused by an abnormal anterior and inferior insertion of the lateral canthal tendon may be noted. In addition, euryblepharon has been associated with ptosis, telecanthus, a laterally displaced punctum, and a double row of meibomian glands.3 Treatment should be directed toward the specific problems. Lid laxity may be managed with a lid-shortening procedure. Ectropion and corneal exposure can be managed with lateral tarsorrhaphies. Ptosis Ptosis of the eyelid is the most common eyelid malposition in children. Its causes include muscle and nerve problems, as well as scarring or trauma. Most cases of congenital ptosis are due to an idiopathic fibrosis and deficiency of striated muscle fibers within the levator muscle. It may also be transmitted through an autosomal dominant inheritance. There is no known racial or sex-based preference, and roughly 75% of cases are unilateral. Patients present with varying degrees of eyelid ptosis alone, without eyebrow or head positioning (Figure 7-2). Those with congenital ptosis with poor levator function have a poorly formed eyelid crease, and they exhibit

A

B

Figure 7-2. Mild (A) and severe (B) unilateral eyelid ptosis.

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lid lag on a downward gaze, which is indicative of a dystrophic muscle. Amblyopia must be ruled out via a complete ophthalmic examination, with particular attention to visual acuity. The degree of ptosis should be determined by the marginal reflex distance, the distance from the upper lid margin to a light reflex on the cornea (a normal distance is 4.0–4.5 mm). Function of the levator muscle is determined by having the patient look down and then up, with a finger over the eyebrow, to prevent the brow from lifting the eyelid. The distance the upper lid margin elevates in millimeters is the levator muscle function (normal excursion is roughly 15 mm). Congenital ptosis is classified as mild (2-mm ptosis), moderate (3-mm ptosis), or severe (4-mm ptosis). Levator function is classified as excellent (13–15 mm), very good (10–13 mm), good (8–10 mm), fair (5–7 mm) or poor (≤ 4 mm). Horner syndrome may be suggested by the pupillary size and a difference in the color of the iris muscle. The patient should be examined for the Bell phenomenon, the ability of the eye to roll upward to protect the cornea and prevent exposure keratopathy. Physical examination should be conducted to rule out any mass in the eyelid that can cause extra weight, such as a plexiform neuroma, vascular lesion, or rhabdomyosarcoma. Surgery is usually performed when the child is 4 to 5 years old and able to participate in postoperative care. Earlier intervention may be warranted if the visual axis is blocked, which can put the patient at risk for deprivation amblyopia. These 2 measurements are used to determine which surgical approach to take, with levator function being the more important of the two. Congenital ptosis can be corrected by 3 surgical procedures: plication of the levator muscle, resection of a portion of the levator, and suspension of the eyelid to the frontalis muscle.

Eyelid Retraction Eyelid retraction occurs when the upper (or lower) eyelid rests at a position superior (or inferior) to the corneal limbus. The upper eyelid normally lies 4 to 5 mm from the center of the cornea, producing a vertical opening of 9 to 10 mm. Congenital retraction is rare and can be associated with thyroid disease, hydrocephalus (setting sun sign), or Parinaud (dorsal midbrain) syndrome. Children with maxillary hypoplasia and resultant shallow orbits have greater show of the lateral scleral surface. A thorough workup is warranted in a child who has lid retraction to determine the cause and chronicity. Surgical approaches to upper-lid retraction may involve an internal approach via the conjunctiva or require an external incision. The surgery involves shortening the muscle and/or aponeurosis of the upper eyelid. Entropion Entropion refers to inward rotation of the eyelid margin, which causes the lashes to make contact with the globe (Figure 7-3). More commonly, it is seen in the lower eyelid. Entropion can cause corneal irritation, chronic scarring, and possibly visual loss. The mechanism of congenital entropion has been attributed

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Figure 7-3. A pediatric patient with congenital entropion that results in contact of the cornea with the eyelashes.

to a disinsertion of the lower lid retractors.4 This condition can be associated with microphthalmos, anophthalmia, and epiblepharon.5 Congenital entropion almost never resolves spontaneously and tends to worsen with age.6 The child may be followed up in the office with daily corneal lubrication if the corneal epithelium is intact. Definitive treatment is a procedure performed to evert the lid by removing an ellipse of skin and orbicularis and suturing the lower lid retractors to the tarsus.7

Epiblepharon Epiblepharon refers to vertically oriented eyelashes. Unlike entropion, this condition can improve with age as a child grows and the facial structures mature. Treatment depends on the severity and the presence of corneal damage caused by the constant rubbing of the lashes against the cornea. Mild cases can be managed with lubricating drops or ointment. Severe cases may require surgical removal of a small amount of excess skin and muscle below the margin of the lip to allow the lid to rotate outward. Ectropion Ectropion refers to an anterior position of the eyelid such that the lid margin does not rest against the globe. More commonly, it is seen in the lower eyelid. It is also more commonly encountered in elderly adults as a result of tissue laxity. The absence of lip apposition can cause tear film insufficiency, chronic

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dry eye, and corneal irritation. Similar to entropion, ectropion has been associated with blepharophimosis, Down syndrome, and ichthyosis and less commonly with microphthalmia, euryblepharon, and buphthalmos. Chronic lid ectropion can lead to keratinization of the palpebral conjunctiva, necessitating conjunctivoplasty. Treatment usually involves a lid-shortening procedure to invert the eyelid margin.

Ankyloblepharon Ankyloblepharon refers to congenital fusion of the upper and lower eyelids. Eyelid fold anomalies can range from a failure of normal lid formation to a variety of eyelid margin defects. The adhesions are easily separated surgically, but in cases in which the adhesion is located medially, the integrity of the medial canalicular system must be evaluated. The punctum can be intubated and protected. Coloboma A coloboma refers to incomplete formation of the eyelid. It has been associated with a number of causes, including amniotic bands, facial cleft, mandibulofacial dysostosis, and hemifacial microsomia. It is most commonly located in the upper eyelid, at the middle and inner third of the eyelid margin. In the lower lid, it is often located in the middle to lateral margin of the eyelid. True coloboma, in which there is a full-thickness defect, must be distinguished from pseudocoloboma, in which there is merely a bowing of the eyelid margin. The initial evaluation of a neonate with a coloboma must determine whether the cornea can be protected. If not, lubrication should be instituted until surgical repair is performed at around 6 months of age. If lubrication or a watertight dressing is unsuccessful, earlier repair is indicated to prevent amblyopia and permanent vision loss from corneal injury. Small defects less than one-quarter the width of the eyelid can usually be closed primarily. Larger defects may require advancement flap or tissue lateral to the eyelid; even larger defects, such as those greater than one-half the width of the eyelid, require borrowing tarsus and conjunctiva from the unaffected eyelid. Of course, if the visual axis is occluded for a period as part of the repair to permit adequate vascularization of the flap, there should be concern for the development of occlusion amblyopia. Colobomas can be seen in the constellation of CHARGE syndrome. CHARGE is an abbreviation that encompasses the various features of the disorder, including coloboma, heart defects, atresia of the choanae, growth retardation, genital anomalies, and ear anomalies. The spectrum of malformations differs among affected individuals. Many patients with CHARGE syndrome have several major characteristics or a combination of major and minor characteristics. The coloboma may affect one or both eyes and may impair the child’s vision, depending on the size and location. Some affected individuals also have abnormally small or underdeveloped eyes, called microphthalmia.

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Cryptophthalmos Cryptophthalmos refers to the rare condition of a hidden eye. The eyelids do not separate to produce a palpebral fissure, and the skin remains intact from the eyebrow to the cheek. When this happens, the deeper layers of the eyelid, including the tarsus, meibomian glands, lashes, and orbicularis muscle, also fail to develop. The underlying globe is usually microphthalmic, with corneal, iris, and lens abnormalities. In less severe forms of cryptophthalmos, a segment of eyelid is fused to the underlying globe, and the remainder of the eyelid may be otherwise normal. For patients with the severe form, testing of visual evoked potentials should be performed to determine whether reconstructive attempts are warranted, because surgical options may be challenging. For patients with less severe forms, there is a greater chance of improving visual function.

Anesthesia Considerations

The timing of surgical intervention in eyelid developmental anomalies is greatly affected by the need for general anesthesia. To date, it has been generally accepted that the risks of anesthesia decrease after 3 months of age, which is why cleft lip repair has traditionally been performed around this time. More recent animal experiments have led to this approach being questioned, so it is now recommended that patients wait until they are older than 2 years to undergo elective surgery. Some conditions, however, require urgent intervention to address corneal exposure or allow proper development of the visual axis. Other conditions may improve as the child ages and undergoes facial growth. If possible, anesthesiologists with fellowship training in pediatric anesthesia should be used. REFERENCES

1. Beysen D, De Jaegere S, Amor D, et al. Identification of 34 novel and 56 known FOXL2 mutations in patients with blepharophimosis syndrome. Hum Mutat. 2008;29(11):E205–E219 PMID: 18642388 https://doi.org/10.1002/humu.20819 2. Allen CE, Rubin PA. Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES): clinical manifestation and treatment. Int Ophthalmol Clin. 2008;48(2):15–23 PMID: 18427257 https://doi. org/10.1097/IIO.0b013e3181694eee 3. Betharia SM, Dayal Y, Nayar RC. Euryblepharon. Indian J Ophthalmol. 1984;32(1):41–43 PMID: 6500664 4. Tse DT, Anderson RL, Fratkin JD. Aponeurosis disinsertion in congenital entropion. Arch Ophthalmol. 1983;101(3):436–440 PMID: 6830498 https://doi.org/10.1001/archopht.1983. 01040010436020 5. Duke-Elder S. Congenital anomalies of the ocular adnexal. In: Duke-Elder S, ed. System of Ophthalmology. London, United Kingdom: Henry Kimpton; 1964 6. Collin JRO. A Manual of Systematic Eyelid Surgery. Philadelphia, PA: Elsevier; 2006:29 7. Maman DY, Taub PJ. Congenital entropion. Ann Plast Surg. 2011;66(4):351–353 PMID: 21301313 https://doi.org/10.1097/SAP.0b013e3181e56e69

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CHAPTER

8

Facial Paralysis RONALD M. ZUKER, MD, FRCSC, FACS, FRCSEd(Hon)

Introduction

Facial paralysis can lead to a number of functional, psychological, and aesthetic concerns. There is a wide range of presentations, from complete facial paralysis (Figure 8-1) to varying degrees of incomplete facial paralysis, with further subdivisions based on the section of the face that is involved. The most common form in children is unilateral congenital facial paralysis, sometimes called “developmental palsy” (Figure 8-2). This usually involves all components of the face but may be isolated to only the buccal segment or the mandibular segment (Figure 8-3). Facial paralysis can also be bilateral in children, with the most common form being that seen in Möbius syndrome (Figure 8-4). These clinical presentations are discussed in detail in this chapter, as well as preferred methods of treatment. The effects of facial paralysis involve orifice control for the eye, nose, and mouth, as well as facial expression. The lack of orifice control for the eye can lead to corneal exposure, keratopathy, and potential visual loss. The orifice control relating to the nose can cause difficulties breathing, with lack of a normal opening of the involved nostril. The lack of orifice control for the mouth can affect the symmetry of the face with drooping of the involved side, as well as problems related to speech, chewing, and oral competence that can lead to drooling. In some cases, the lack of dental protection can lead to dental decay. The mimetic function of the facial nerve, however, is critical for social interactions. Nonverbal communication is conveyed by facial expression and is essential for normal interpersonal interactions. A smile invokes a smile in others and conveys feelings that cannot be transmitted in any other way. Consequently, a spontaneous dynamic smile is critical for personal interactions (Figure 8-5). A smile consists of 2 components. One component is the physical movements of the corners of the mouth, which originates intracranially from the seventh nerve nucleus. The other component, however, is the emotional expression of joy that comes from the frontal lobe. It is a combination of these 2 components that lead to a normal, spontaneous smile. This is affected through the seventh cranial nerve and, for this reason, a seventh nerve–based reconstruction is preferred if possible. In unilateral congenital 113

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Figure 8-1. A patient with complete facial paralysis after surgery to remove a brain tumor.

Figure 8-2. A patient with incomplete unilateral congenital facial paralysis (“developmental palsy”).

facial paralysis (developmental palsy), this is the preferred method; the normal seventh nerve is used as the power source.

Epidemiology

A number of attempts have been made to classify facial paralysis. The differential diagnosis is long and extensive. Westin and Zuker have proposed a classification system for facial paralysis that may be more helpful for the

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Figure 8-3. A patient demonstrating mandibular branch paralysis, otherwise known as crying facies syndrome.

Figure 8-4. A patient with bilateral facial paralysis in Möbius syndrome.

clinical practitioner.1 It categorizes congenital facial paralysis versus acquired facial paralysis. The congenital form can be syndromic or nonsyndromic. The acquired form can be traumatic, a consequence of tumor excision, inflammatory, or the result of neuromuscular disease. The most common forms seen are congenital and nonsyndromic but are believed to be developmental in origin. Through the authors’ experience, when these facial sites are explored, there is no facial musculature. In other words, the muscle has not developed and is not a matter of early development followed by

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Figure 8-5. The lack of a symmetrical smile can make interpersonal communication awkward.

degeneration. These common, developmental, nonsyndromic forms are the result of an intrauterine insult early in development. The mildest form is that of mandibular branch paralysis or crying facies syndrome. Again, on the involved side, there is no musculature that has developed. The developmental form can also be bilateral and is most commonly seen in Möbius syndrome. This involves bilateral sixth and seventh cranial nerve involvement that may be complete or incomplete. It also often involves other cranial nerves, particularly the 12th nerve. The acquired form of facial paralysis is more severe and leads to more profound clinical symptomatology. The congenital form has a level of protection, particularly for the eye, that is not seen in the complete acquired facial paralyses. Usually, acquired facial paralysis is seen after trauma, either intracranial or extracranial. This can also involve tumor extirpation with resultant nonfunction of the seventh cranial nerve. In the adult population, Bell palsy is most common, particularly the incomplete recovery with synkinesis or hyperkinesis but minimal movement. This is seen in children but is unusual.

Patient Presentation

When a patient presents with facial paralysis, it is important to establish the likely cause. The anatomical pattern can be of assistance in making this decision and also leads to a treatment plan, particularly related to addressing corneal exposure or oral incompetence. Very often, the course of the disease will be helpful in making this determination. Bell palsy typically occurs rapidly or, as a slow-growing tumor in the cheek, leads to a slow onset of facial paralysis. When evaluating a baby born with facial paralysis, it is

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important to follow the course, as the clinical representation is identical for unilateral congenital facial paralysis (developmental palsy) versus acquired obstetric-forceps delivery-induced palsy. The former will not improve and will remain static during the first several months after birth. The latter, however, typically improves, often to a state where nothing further needs to be done. It is important to assess each specific branch of the facial nerve, as well. Varying patterns have been observed with paralysis of only the mandibular branch or paralysis of only the buccal branch. When it comes to treatment, it is important to take into consideration the timing of the paralysis and how long the musculature has been without innervation. .

Diagnosis

Once the diagnosis has been established and the particular type of facial paralysis has been identified, a treatment plan can be formulated. The diagnosis can be congenital or acquired, complete or incomplete, with specific branch identification and the clinical course that has been recorded. Once the patient has stabilized, it is appropriate to formulate a treatment plan and present this to the patient and family. In cases of complete facial paralysis, the family may want to proceed with treatment right away. However, in cases of incomplete facial paralysis, the family may elect to wait until the child is old enough to make his or her own decision with respect to surgical intervention. The following sections are a management program, with specific details on surgical reconstruction by using newer microsurgical techniques.

Management

There are a number of reanimation procedures available for the treatment of facial paralysis. First, nerve-based reconstruction by using direct nerve repair, nerve grafts, and transfers is helpful if timing permits. The muscle will unfortunately deteriorate after about 1 year of lack of innervation. The motor end plates lose their ability to function, and the muscle is rendered non-reinnervatable. However, prior to 1 year, a nerve-based reconstruction may be possible. If there is no usable musculature in the face, muscle transfers can be applied. Regional muscle transfers have been used with great success.2,3 However, they can lead to contour irregularities and limited excursion. This is particularly true with the masseter muscle transfer, which also has an inappropriate lateral direction of contraction. The authors’ preference is to replace the zygomaticus major and minor muscle complex activity with a free muscle transplant. These procedures are technically more difficult, but the transplanted muscles can be positioned where one wants without the limitation of the vascular or neural pedicle. Consequently, the vector of movement is more natural, and the extent of excursion approaches reference range.4

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A

B

Figure 8-6. Two patients with facial paralysis, before (A) and after (B) surgery. A, Preoperative photograph of bilateral facial paralysis (Möbius syndrome). B, Postoperative photograph after bilateral segmental gracilis muscle transplantation to the face, innervated by the motor nerve to the masseter muscle.

The goal of facial paralysis reconstruction is to produce a normal appearance. This involves symmetry at rest, with minimal distortions and active movement of the oral commissures in the appropriate direction and the appropriate excursion. Both form and function are essential (Figure 8-6). The keys to success in muscle transplantation will be discussed in detail but involve muscle positioning, reduction of bulk, strong motor input, and accurate tension.

Patient Selection

In the young patient, who has a minimal droop, adequate excursion can be achieved with a cross-face nerve graft, followed by a muscle transplant. However, in the older patient with excessive droop, it is preferable to do a muscle transplant powered by a stronger nerve, such as the motor nerve to the masseter muscle. When there is no seventh nerve bilaterally, muscle transplantation can also be performed, but it must be powered by a different motor. Again, in these situations, author preference is to use the motor nerve to the masseter muscle.

Cross-Face Nerve Graft and Gracilis Muscle Transplant Combination

In the younger patient with unilateral facial paralysis, a combination of cross-face nerve graft and muscle transplantation is preferred.5 In the cross-face nerve graft procedure, the normal side is explored, and an

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appropriate seventh nerve branch is identified (Figure 8-7). Equally important, an alternate nerve branch is preserved. This is done with a nerve stimulator, under direct vision. Once the appropriate nerve is selected, a tunnel is made across the upper lip to the buccal sulcus on the involved side. The proximal component of the sural nerve just distal to the knee is preferred as a nerve graft (Figure 8-8).6 The nerve is placed through the tunnel, and the end is marked with a polypropylene suture and a hemoclip so that it can be easily identified at the next surgery. The branch of the facial nerve that we had selected is divided; then, under high-powered magnification, it is coapted to the sural nerve graft.

Figure 8-7. Intraoperative photograph of a cross-face nerve graft, which includes exploration of the normal side, facial nerve mapping, and selection of an appropriate segment of facial nerve.

Figure 8-8. An intraoperative photograph demonstrating sural nerve procurement from the leg.

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Muscle Transplant Procedure

Approximately 9 months are allowed for the activity of the facial nerve to grow across the graft and for it to become ready to power the new muscle transplant. If both the nerve graft and the muscle transplant were performed at the same time, the muscle would atrophy excessively and not function as well. The preoperative planning for this procedure is essential, to be able to replicate the normal side of the face as well as possible (Figure 8-9). In the affected side, anchoring sutures are carefully placed into the oral commissure and upper lip. With traction placed on these sutures, the movement that is created is assessed, and the future location of the pull of the muscle transplant is identified (Figure 8-10). The facial artery and facial vein are also identified, as these will be used to revascularize the muscle transplant. An upper inner-thigh incision is made, and the gracilis muscle is procured (Figure 8-11). To secure the muscle appropriately into the commissure and upper lip, a line of mattress sutures going across the end of the muscle is used that will be inserted into the commissure and upper lip (Figure 8-12). The muscle is then revascularized through microvascular anastomoses to the facial artery and vein. The motor nerve to the gracilis is tunneled into the upper lip so that it lies next to the previously placed cross-face nerve graft. The nerve repair is performed intraorally with high magnification under the operating microscope. Now that the muscle is positioned into the oral commissure and upper lip, revascularized, and reinnervated, the proximal portion is secured to the temporal fascia. This puts the oral commissure even with the normal side

Figure 8-9. Preoperative planning prior to the muscle transplant procedure is shown, noting the vector, the point of pull, and the facial artery.

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Figure 8-10. Assessment of predicted excursion is conducted with traction placed on anchoring sutures.

Figure 8-11. An intraoperative photograph demonstrates segmental gracilis muscle procurement. Approximately one-third of the circumference of the muscle is used.

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Figure 8-12. An intraoperative photograph demonstrates insertion of the gracilis muscle with anchoring sutures locking behind row of mattress sutures.

and with only slight tension (Figure 8-13). It is not overcorrected, as this will distort the mouth; nor is it undercorrected, as it will not provide for the appropriate excursion. After the cross-face nerve graft muscle transplant combination procedures, muscle activity begins about 4 months after muscle transplant. Patients enter an exercise program with biofeedback. They try to smile in

Figure 8-13. An intraoperative photograph demonstrates the muscle transplant in place, revascularized and ready for securing sutures in the temporal fascia under appropriate tension.

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front of a mirror and observe the activity of the reconstructed oral commissure. They try to increase the excursion so that it is as close to normal as possible, and they also try to get it to become as spontaneous as possible. Because the seventh nerve is used, spontaneity is generally not a problem, and the muscle is activated at the same time as the normal side. Under close scrutiny, one can see a slight time delay as the neural activity moves across the cross-face nerve graft from the normal side and into the muscle on the reconstructed side (Figure 8-14).

A

B

C

D

Figure 8-14. A, A preoperative image of a patient with unilateral facial paralysis at rest. B, A preoperative image of the patient smiling. C, A postoperative image of the patient at rest. D, A postoperative image of the patient smiling.

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Muscle Transplant Innervated by the Motor Nerve to the Masseter Muscle

In bilateral facial paralysis, there is no facial nerve available; consequently, the cross-face nerve graft procedure is not possible. In these situations, the segmental gracilis muscle transplant is the most useful. However, it needs to be innervated by a different motor nerve. Various motor nerves have been tried, such as the hypoglossal nerve and the accessory spinal nerve, but the motor nerve to the masseter muscle functions the best (Figure 8-15).7 It is a powerful nerve that will provide excellent excursion to the muscle transplant. It is in a better position to allow for spontaneity through cortical plasticity (Figure 8-16).8 In bilateral cases, one side is corrected at a time, and at least 3 months is allowed to elapse between procedures. This creates time for the first side to become active so that the vector of pull and the location of the insertion can be seen. After about 10 weeks, the muscle is reinnervated when the motor nerve to the masseter muscle is used. Again, a rehabilitation program is instituted with smiling and biofeedback. Here, the biofeedback is extremely important to not only gain adequate excursion but also create a degree of spontaneity. With ongoing therapy in well-motivated patients, many do go on to have a spontaneous smile (Figure 8-17). However, this does not happen automatically and can only occur with a frequent exercise program in which biofeedback is used. When the motor nerve to the masseter muscle is used, initially patients have to bite down to activate the muscle. This is phase 1, and it occurs at about 10 to 12 weeks after surgery. Very quickly, however, they move on to phase 2, in which they are able to activate the muscle without moving the jaw. Then, with the rehabilitation described

Figure 8-15. An intraoperative photograph demonstrates the location of the motor nerve to the masseter muscle.

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Figure 8-16. An intraoperative photograph demonstrates the segmental gracilis muscle in position, revascularized and reinnervated by the motor nerve to the masseter muscle.

previously and the biofeedback, patients sometimes progress into phase 3, in which the activity becomes spontaneous when a smile is needed.

Complications

Surgical complications can involve hematoma formation or infection, but these are unusual in facial paralysis reconstruction. Excess bulk is noted at the site of muscle transplantation, and care must be taken to reduce this problem. Second, movement may not be adequate, and excursion of the oral commissure may be less than expected and particularly less than the normal side. Unfortunately, this is a problem that cannot be corrected with simple surgical intervention. If it is sufficiently severe, the only appropriate solution would be to redo the reconstruction and redo the muscle transplant. One other complication is that of slippage of the muscle, either proximally or distally. Again, this is a difficult problem to correct. Sometimes the muscle can be reinserted into the oral commissure or upper lip, but more often, the muscle has become firm, and its excursion is limited. Again, the only alternative is to redo the transplant. This is why such great care must be taken during the initial procedure to reduce these complications, as they are extremely difficult to correct after they have occurred. In conclusion, facial paralysis can have significant effects on the pediatric patient from functional, psychological, and aesthetic viewpoints. Reconstruction is possible. Muscle transplantation can yield excellent results, providing natural-appearing oral commissure movement and

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B

A

C

D

E

Figure 8-17. A, A preoperative image of a patient with bilateral facial paralysis (Möbius syndrome) at rest. B, A preoperative image of the patient attempting a smile. C, A postoperative image of the patient at rest. D, A postoperative image of a small smile. E, A postoperative image of a full smile.

excursion. The muscle can be powered by either a cross-face nerve graft in unilateral cases or the motor nerve to the masseter muscle in bilateral cases. This surgical intervention can lead to improved oral continence, clearer speech, improved facial symmetry, and, most important, a restored smile in acquired cases and a created smile in congenital cases. REFERENCES

1. Westin LM, Zuker R. A new classification system for facial paralysis in the clinical setting. J Craniofac Surg. 2003;14(5):672–679 PMID: 14501327 https://doi.org/10.1097/00001665200309000-00013 2. Labbé D, Huault M. Lengthening temporalis myoplasty and lip reanimation. Plast Reconstr Surg. 2000;105(4):1289–1297 PMID: 10744217 https://doi.org/10.1097/00006534-200004000-00005 3. Labbé D. Myoplastie d’allongement du temporal V.2. et réanimation des lèvres [Lengthening temporalis myoplasty V.2. and lip reanimation]. Ann Chir Plast Esthet. 2009;54(6):571–576 PMID: 19632753 https://doi.org/10.1016/j.anplas.2009.04.002

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127 Chapter 8: Facial Paralysis 4. Bae YC, Zuker RM, Manktelow RT, Wade S. A comparison of commissure excursion following gracilis muscle transplantation for facial paralysis using a cross-face nerve graft versus the motor nerve to the masseter nerve. Plast Reconstr Surg. 2006;117(7):2407–2413 PMID: 16772949 https://doi.org/10.1097/01.prs.0000218798.95027.21 5. Zuker RM, Gur E, Hussain G, Manktelow RT. Facial paralysis. In: Rodriguez ED, Losee JE, eds. Craniofacial, Head and Neck Surgery, and Pediatric Plastic Surgery. London, United Kingdom: Elsevier; 2018:329. Plastic Surgery; Neligan PC, ed; 4th ed; vol 3 6. Manktelow RT, Zuker RM. Cross-facial nerve graft—the long and short graft: the first stage for microneurovascular muscle transfer. Operative Techniques in Plastic and Reconstructive Surgery. 1999;6(3):174–179 https://doi.org/10.1016/S1071-0949(99)80030-4 7. Borschel GH, Kawamura DH, Kasukurthi R, Hunter DA, Zuker RM, Woo AS. The motor nerve to the masseter muscle: an anatomic and histomorphometric study to facilitate its use in facial reanimation. J Plast Reconstr Aesthet Surg. 2012;65(3):363–366 PMID: 21992936 https://doi. org/10.1016/j.bjps.2011.09.026 8. Zuker RM, Goldberg CS, Manktelow RT. Facial animation in children with Möbius syndrome after segmental gracilis muscle transplant. Plast Reconstr Surg. 2000;106(1):1–8 PMID: 10883605 https://doi.org/10.1097/00006534-200007000-00001

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CHAPTER

9

Pediatric Neck Masses RAVI K. GARG, MD, AND MARK M. URATA, MD, DDS

Introduction

Most pediatric neck masses are benign and either congenital or inflammatory in nature. They can be evaluated with a combination of a thorough medical history, physical examination that is attentive to the characteristics of the mass and the anatomical location in the neck, imaging, and/or biopsy. Most lesions can be treated with observation, physical therapy, or direct excision. There may be a role for sclerotherapy and electrocautery in the management of some lesions.

Types of Pediatric Neck Masses Thyroglossal Duct Cyst The thyroglossal duct represents the pathway along which the developing thyroid gland descends from the base of the tongue to its final position below the cricoid cartilage and anterior to the trachea (Figure 9-1). It forms during the third week of development from the first and second branchial pouches. By the fifth to eighth week of gestation, the thyroglossal duct obliterates, leaving behind the foramen cecum as its proximal remnant at the base of tongue and the pyramidal lobe distally at the superior pole of the thyroid gland.1 Any portion of the duct that fails to obliterate may form the nidus for a thyroglossal duct cyst. Branchial Cleft Cyst, Sinus, and Fistula Normally, the first branchial cleft develops into the external auditory meatus, and the remaining branchial clefts disintegrate. Residual branchial cleft tissue may form a cyst, sinus, or fistula. Cysts are fluid-filled masses that do not communicate with a body surface, while sinuses have a single connection to a body surface. Branchial fistulas are the least common and are characterized by a tract that connects 2 separate body surfaces. The anatomical course of these cysts, sinuses, and fistulas relates to the developmental anatomy of each branchial arch.

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Thyroglossal duct cyst

Figure 9-1. The thyroglossal duct originates from the base of the tongue and represents the path of descent of the developing thyroid gland to its final position anterior to the trachea. Failure of the duct to involute anywhere along this pathway may result in the development of a thyroglossal duct cyst.

Midline Cervical Cleft A midline cervical cleft may result from a fusion abnormality of the paired branchial arches during gestational weeks 3 and 4.2 There may be a sinus tract or band that connects the midline cutaneous lesion to the sternum or mandible. Plunging Ranula A ranula is a mucocele of the f loor of the mouth, usually involving the sublingual gland. It may occur secondary to trauma, blockage of salivary drainage by a sialolith, or prior surgery. 3 Rarely, the mass will descend into the submental neck through the interval between the mylohyoid and hyoglossus muscles or through a weakening in the mylohyoid muscle.

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Dermoid Cyst, Epidermoid Cyst, and Teratoma These lesions can be understood in terms of the germ layers from which they arise. Dermoid cysts are derived from ectodermal and mesodermal germ layers, unlike epidermal cysts, which are derived from the ectodermal germ layer only.4 Cyst content is sebaceous. Teratomas involve all 3 germ cell layers. Although rare, when they involve the head and neck, they can compress the upper airway and be life-threatening. Thymic Cyst The thymus is important for development of cell-mediated immunity during infancy. It grows to its maximum size at 2 to 4 years of age, prior to involuting. It derives from the third and fourth pharyngeal pouches during the sixth week of development, fuses in the midline, and attaches to the pericardium, subsequently descending into the mediastinum along with the pericardium. Solid or cystic ectopic tissue may be left along the path of migration if the superior attachment of the thymic primordium fails to regress.3 Thymic cysts are usually located anterior or deep to the sternocleidomastoid (SCM) muscle, and approximately 50% of these cysts extend into the mediastinum. Lymphatic Malformation Lymphatic malformations are characterized by clusters of fluid-filled aberrant lymphatic vessels.5 Often, lymphatic malformations of the neck have large cystic spaces, in what is also known as cystic hygroma or macrocystic lymphatic malformation. Microcystic lymphatic malformations have smaller cystic spaces and are more challenging to treat by using conservative techniques. Congenital Muscular Torticollis Congenital muscular torticollis is characterized by fibrosis of the SCM muscle with associated ipsilateral head tilt and contralateral chin deviation. Although debated, this may result from head positioning and injury to the muscle while in utero. Sometimes this fibrosis presents as a palpable mass, known as fibromatosis colli.6

Epidemiology

After cervical lymphadenopathy, thyroglossal duct cyst is the second most common pediatric neck mass and is the most common pediatric cystic mass. Thyroglossal duct remnants are estimated to occur in 7% of the population, but most of these are asymptomatic.1 Branchial cleft anomalies are the next most common, accounting for 30% of congenital neck masses. Second branchial cleft anomalies account for 90% of branchial cleft neck lesions, while first, third, and fourth branchial cleft anomalies are much less common.7

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The remaining neck masses that are discussed are all rare. Dermoid cysts, for example, are commonly found in the face and scalp but occur less commonly in the neck. Teratomas are exceptionally rare, occurring in an estimated 1 in 4,000 births.3

Patient Presentation Thyroglossal Duct Cyst This midline mass occurs in the vicinity of the hyoid bone in 60% of cases and may move with swallowing. It develops between the hyoid bone and tongue base in 24% of patients and between the hyoid bone and thyroid gland in the remaining 13% of cases.8 One-third of patients will have a history of previous infection. The cyst may manifest as a foul taste if there is drainage into the mouth from the foramen cecum. More rarely, an infant will experience sudden unexpected infant death or respiratory distress if the lesion involves the tongue base. Branchial Cleft Cyst, Sinus, and Fistula Second branchial cleft masses have a variety of presentations. There may be an external opening along the anterior border of the SCM muscle, usually inferior to the hyoid bone. There may also be a sinus along the supratonsillar fossa. The course of the lesion usually has 1 of 4 patterns: (a) passes anterior to the SCM and does not communicate with the carotid sheath; (b) courses deep to the SCM and either anterior or posterior to the carotid sheath; (c) travels between the internal and external carotid arteries; or (d) courses deep and medial to the carotid sheath, adjacent to the pharynx and tonsillar fossa (Figure 9-2). First branchial cleft anomalies may manifest with cervical, parotid, or auricular discomfort, or there may be otorrhea with mucopurulent drainage, a mass effect on the parotid, a parotid abscess, or a pitlike depression at the angle of the mandible.5 Third branchial cleft anomalies may manifest with acute thyroiditis, hypoglossal nerve palsy, or a foul taste in the mouth if there is infection at the level of the pyriform sinus.1 These symptoms can be understood by appreciating the course of third branchial cleft masses, which course along the lower SCM muscle, deep to the internal carotid artery, hypoglossal and glossopha­ ryngeal nerves, and above the superior laryngeal nerve before descending along the pharynx to enter the thyrohyoid membrane and piriform sinus. Fourth branchial cleft sinuses and fistulas also open into the piriform sinus. Unlike third branchial cleft sinuses and fistulas, they course inferior to the internal branch of the superior laryngeal nerve. Additionally, rightsided fourth branchial cleft anomalies course under the subclavian artery and deep to the internal carotid artery, while left-sided fourth branchial cleft anomalies course beneath the aortic arch before ascending to the piriform sinus.3

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Pharynx

Cyst

Cyst

Pharynx

Vein Vein

Artery

Muscle

Muscle

A

Artery

B

Pharynx

Cyst Pharynx

Cyst

Vein

Vein Muscle

C

Artery

Muscle

Artery

D

Figure 9-2. Second branchial cleft anomalies have 1 of 4 patterns. The mass either does not interface the carotid sheath as it remains superficial to the sternocleidomastoid muscle (A), interfaces with the carotid sheath (B), passes between the internal and external carotid arteries (C), or travels deep to the carotid sheath, adjacent to the pharynx (D).

Midline Cervical Cleft Midline cervical cleft may manifest as a skin ulceration with an associated skin or cartilaginous tag.3 Plunging Ranula A ranula usually manifests as a round, bluish mass in the floor of mouth. When it “plunges” or descends through the floor of mouth, there is palpable swelling in the submental neck. Dermoid Cyst, Epidermoid Cyst, and Teratoma A dermoid cyst may also manifest in the midline of the neck as a painless mass that grows over time as sebum accumulates within the cyst cavity. Unlike thyroglossal duct cysts, dermoid cysts usually do not move with tongue protrusion or swallowing. However, there may be fibrous attachments to the hyoid that result in movement with swallowing that can lead to diagnostic confusion. Epidermoid cysts have a similar presentation to dermoid cysts and tend to occur in acne-prone regions of the head and neck.

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Cervical teratomas may manifest with polyhydramnios during fetal gestation because of compression of the cervical esophagus by the mass. Dysphagia and respiratory distress may be life-threatening, presenting features of these masses.

Thymic Cyst Most cervical thymic cysts are asymptomatic. They may enlarge into a palpable cystic mass because of infection or hemorrhage, and they can expand with the Valsalva maneuver. Lymphatic Malformation The posterior triangle of the neck is the most common location of lymphatic malformations. Occasionally, these lesions will enlarge rapidly over the course of a few days, sometimes with no antecedent event and sometimes in the setting of an upper respiratory tract infection. Congenital Muscular Torticollis Patients with SCM fibrosis and congenital muscular torticollis present with a head tilt to the affected side and with the chin turned to the opposite side. It is important to note that this clinical finding of torticollis may also arise for other reasons, related to vision, hearing, or spinal anomalies.9 One-third of patients with congenital muscular torticollis will develop positional plagiocephaly in which there is an asymmetrical flattening of the head caused by unequal pressure on the skull.

Diagnosis

A thorough history and physical examination are critical first steps in the diagnosis of pediatric neck masses. Identification of whether the mass is on the midline and moves with swallowing may direct one toward a diagnosis of thyroglossal duct cyst. On the other hand, a mass anterior to the SCM that drains or becomes infected may be a branchial cleft cyst. Ultrasonography (US) is an extremely helpful imaging modality that does not expose the patient to radiation. It is useful for characterizing whether a mass is solid or cystic and whether it is homogeneous or heterogeneous in character. It can help define the septations and echogenicity of a macrocystic lymphatic malformation and the SCM fibrosis associated with congenital muscular torticollis.10 In the case of a thyroglossal duct cyst, US may be complemented by obtaining a screening thyroid-stimulating hormone level to identify whether the patient has hypothyroidism, because this mass represents the only functioning, ectopic thyroid tissue.8 A radionuclide study can also be useful in identifying whether the patient has additional thyroid tissue. Initial assessment of branchial cleft cysts may also begin with US. However, computed tomography or magnetic resonance imaging may be

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necessary to identify the anatomical course of the lesion, especially if surgical resection is planned. Flexible endoscopy plays a role in determining whether the tract opens into the tonsillar fossa or piriform sinus, while barium esophagography may help to identify the opening of the drainage tract into the piriform sinus for third and fourth branchial cleft sinuses. If there is diagnostic confusion between masses, such as a thyroglossal duct cyst and a dermoid cyst, fine-needle aspiration biopsy can be performed. This may also be helpful if the diagnosis of plunging ranula is questioned, in which case the fluid can be tested for amylase.

Management

Most pediatric neck masses are benign and can be treated with total excision. If a lesion is infected, it is best to treat it with antibiotics to allow the infection to resolve prior to undertaking elective removal. Antimicrobial coverage should target oral flora. Aspiration may be performed if cyst decompression is needed. It is critical with all masses to achieve complete removal to decrease the risk of recurrence. To accomplish complete removal of a thyroglossal duct cyst, a central portion of the hyoid bone is resected, along with the specimen, in what is known as the Sistrunk procedure.5 The thyroglossal duct is formed prior to development of the hyoid bone; therefore, it is feasible for ductal tissue to become trapped within the hyoid bone. During the procedure, dissection is performed caudal to the mass to identify whether there is a connection to the pyramidal lobe of the thyroid gland. It is also carried cephalad and involves resection of a core of tissue from the base of tongue to the foramen cecum. Fewer than 1% of thyroglossal duct cysts will have malignant tissue— usually papillary thyroid carcinoma.11 If there is no capsular involvement, cure is achieved with the Sistrunk procedure alone in 95% of cases, although some surgeons recommend completion thyroidectomy, regardless of whether there is capsular invasion. When capsular invasion is present, central neck dissection and radioactive iodine ablation may also be considered.3 In the scenario in which the central neck mass represents the patient’s only functioning thyroid tissue, there is controversy regarding management. Some authors prefer to suppress the ectopic thyroid tissue with exogenous thyroid hormone treatment. Others recommend resection of the tissue, which results in the lifetime need for thyroid hormone supplementation. Resection of branchial cleft cysts is sometimes performed early in life, while others can wait until age 2 to 3.12 Complete removal requires a thorough understanding of how the different branchial cleft cysts interface with the head and neck anatomy. First branchial cleft anomalies may communicate with the external auditory canal, middle ear, and/or parotid gland. Care must be taken to protect the facial nerve during dissection. If the external ear canal or middle ear is involved, a portion of these structures needs to be

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removed. An average of 2.4 procedures is necessary to achieve complete removal of first branchial cleft cysts.13 Second and third branchial cleft cysts have different patterns of relating to the carotid sheath as previously described, while fourth branchial cleft cysts also interface with the subclavian artery and aortic arch. To achieve complete removal, some surgeons have described using a stepladder approach, with multiple small incisions made in the neck to achieve complete removal, thereby enabling the surgeon to avoid making a single large incision. Critical structures to protect during removal of second, third, and fourth branchial cleft cysts include the great vessels of the neck and mediastinum, as well as hypoglossal, glossopharyngeal, vagus, and superior laryngeal nerves. Some authors have described endoscopic electrocauterization of third and fourth branchial cleft sinuses that have openings into the piriform sinus.14 One study demonstrated that 78% of patients had no recurrence, while 22% of patients experienced recurrence that required open resection of the tract.14 Certain neck masses may be amenable to injection with sclerosing agents. The management of lymphatic malformation may involve injection of steroids or sclerosing agents or surgical resection. Similarly, injection of sclerosing agents has been described for management of ranula, although in many cases, either transoral excision or transcervical resection is performed. Marsupialization of ranula into the floor of the mouth may also be considered, but it is associated with a 67% recurrence rate.15 Surgical excision is the standard of care for most other cervical masses. A midline cervical cleft may be excised with stairstep incisions or a series of Z-plasty procedures in the neck. Teratoma is often excised shortly after birth or as part of an ex utero, intrapartum procedure in which cesarean delivery is coordinated with intubation, tracheotomy, or extracorporeal membrane oxygenation prior to division of the umbilical cord.4 Children with thymic masses should first be assessed to ensure there is normal thymic tissue present, as this is critical to the development of cell-mediated immunity. If normal thymic tissue is present, the thymic cyst is completely excised. Congenital muscular torticollis secondary to SCM fibrosis often resolves in the first year after birth. Early physical therapy should be initiated for these babies and involves flexion, extension, lateral bending, and rotational exercises.9 In spite of bruising that results from tearing of the SCM muscle fibers with physical therapy, patients tolerate this well. Botox therapy and myotomy of the SCM have also been described. Myotomy, in which the muscle is disconnected, is rarely indicated and usually involves transecting the distal muscle.

Complications

The main risks with resection of any pediatric neck mass are recurrence of the mass and injury to surrounding anatomical structures. The risk of recurrence can be minimized by making sure to excise the mass in its entirety.

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Care must be taken to ensure that in the case of thyroglossal duct cysts, there is remaining thyroid tissue, and that in the case of thymic cysts, there is residual thymic tissue. Risks of injury to major vessels and nerves in the neck can be avoided by carefully understanding the neck embryology and anatomy through which these cysts and masses course. Most nerve injuries would be related to stretch injury and characterized by temporary weakness or numbness. Additionally, infection, bleeding, and adverse scarring are risks with any surgery and must be discussed with patients and families. REFERENCES

1. LaRiviere CA, Waldhausen JH. Congenital cervical cysts, sinuses, and fistulae in pediatric surgery. Surg Clin North Am. 2012;92(3):583–597, viii PMID: 22595710 https://doi.org/10.1016/j. suc.2012.03.015 2. Acierno SP, Waldhausen JH. Congenital cervical cysts, sinuses and fistulae. Otolaryngol Clin North Am. 2007;40(1):161–176, vii–viii PMID: 17346566 https://doi.org/10.1016/j.otc.2006.10.009 3. Rosa PA, Hirsch DL, Dierks EJ. Congenital neck masses. Oral Maxillofac Surg Clin North Am. 2008;20(3):339–352 PMID: 18603195 https://doi.org/10.1016/j.coms.2008.03.003 4. Goins MR, Beasley MS. Pediatric neck masses. Oral Maxillofac Surg Clin North Am. 2012;24(3): 457–468 PMID: 22857718 https://doi.org/10.1016/j.coms.2012.05.006 5. Gaddikeri S, Vattoth S, Gaddikeri RS, et al. Congenital cystic neck masses: embryology and imaging appearances, with clinicopathological correlation. Curr Probl Diagn Radiol. 2014; 43(2):55–67 PMID: 24629659 https://doi.org/10.1067/j.cpradiol.2013.12.001 6. Bredenkamp JK, Hoover LA, Berke GS, Shaw A. Congenital muscular torticollis. A spectrum of disease. Arch Otolaryngol Head Neck Surg. 1990;116(2):212–216 PMID: 2297419 https://doi. org/10.1001/archotol.1990.01870020088024 7. Valentino M, Quiligotti C, Carone L. Branchial cleft cyst. J Ultrasound. 2013;16(1):17–20 PMID: 24046795 https://doi.org/10.1007/s40477-013-0004-2 8. Foley DS, Fallat ME. Thyroglossal duct and other congenital midline cervical anomalies. Semin Pediatr Surg. 2006;15(2):70–75 PMID: 16616309 https://doi.org/10.1053/j.sempedsurg.2006.02.003 9. Do TT. Congenital muscular torticollis: current concepts and review of treatment. Curr Opin Pediatr. 2006;18(1):26–29 PMID: 16470158 10. Haque S, Bilal Shafi BB, Kaleem M. Imaging of torticollis in children. Radiographics. 2012;32(2): 557–571 PMID: 22411949 https://doi.org/10.1148/rg.322105143 11. El Bakkouri W, Racy E, Vereecke A, et al. [Squamous cell carcinoma in a thyroglossal duct cyst]. Ann Otolaryngol Chir Cervicofac. 2004;121(5):303–305 PMID: 15711485 https://doi.org/10.1016/ S0003-438X(04)95525-9 12. Waldhausen JH. Branchial cleft and arch anomalies in children. Semin Pediatr Surg. 2006;15(2): 64–69 PMID: 16616308 https://doi.org/10.1053/j.sempedsurg.2006.02.002 13. Ford GR, Balakrishnan A, Evans JN, Bailey CM. Branchial cleft and pouch anomalies. J Laryngol Otol. 1992;106(2):137–143 PMID: 1556487 https://doi.org/10.1017/S0022215100118900 14. Prosser JD, Myer CM III. Branchial cleft anomalies and thymic cysts. Otolaryngol Clin North Am. 2015;48(1):1–14 PMID: 25442127 https://doi.org/10.1016/j.otc.2014.09.002 15. Zhao Y-F, Jia J, Jia Y. Complications associated with surgical management of ranulas. J Oral Maxillofac Surg. 2005;63(1):51–54 PMID: 15635557 https://doi.org/10.1016/j.joms.2004.02.018

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CHAPTER

10

Congenital Hand Anomalies MICHAEL L. BENTZ, MD, FAAP, FACS

Introduction

Congenital hand and foot anomalies occur relatively commonly in the pediatric population, following intrauterine development that occurs between the third and eighth intrauterine week. At this time, many other key organ systems are forming, hence the syndromic association of congenital hand and foot anomalies with other systemic anomalies. While the etiologic origins of these are not clear, homeobox genes have been implicated as being important in the embryogenesis of limb anomalies, with a growing understanding of the formal genetic associations with these problems. Syndromic genetic disorders need to be considered and identified and, if present, surgical correction considered. It is important to note that the pediatrician will often be the initial driver of diagnostic evaluations and should, therefore, be aware of all of the possibilities when confronted by these anomalies. Congenital hand anomalies are classified by using the Oberg, Manske, and Tonkin classification of congenital anomalies of the hand and upper limb.1 It uses terms of dysmorphology that place conditions in 1 of 3 groups: (a) malformations, (b) deformations, or (c) dysplasias. The malformations group is further subdivided according to whether the whole of the limb is affected or the hand plate alone, and whether the primary insult involves 1 of the 3 axes of limb development and patterning or is non-axial. An earlier Swanson classification classified anomalies into failure of formation of parts, failure of differentiation, duplication, overgrowth, undergrowth, congenital constriction band syndrome, and generalized skeletal anomalies. While these classifications can be useful in academic exercises, they do not particularly affect clinical decision-making, and many children do not fit perfectly into a given classification heading.2

Malformations Failure of Axis Formation/Differentiation in the Entire Upper Limb These congenital anomalies represent a constellation of deformities that manifest with transverse tissue deficiencies that mimic an “amputation stump” or longitudinal deficiencies that manifest as an incomplete digit 139

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distribution. These represent roughly 15% of all upper-extremity anomalies. Their presentation is heterogeneous in that the level of transverse or longitudinal deficiency can occur anywhere from the shoulder distally to the fingertips. Because of this variable site of occurrence, treatment options need to be designed for each specific patient. Transverse arrests are usually unilateral, more commonly occurring on the left side, and not associated with other malformations. Depending on the level of transverse failure, corrective options can include no intervention, surgical optimization via web space deepening and reconstruction, or prosthetic training and use. For transverse failures proximal to the level of the elbow or just distal to it, prosthetic rehabilitation and training may be more successful. In the future, after immunosuppression is better controlled and the associated long-term risks of malignant neoplasm and infection are reduced or eliminated, hand and forearm transplantation may well become the treatment of choice.3 Longitudinal deficiencies occur somewhat less frequently than transverse deficiencies, with a reported incidence rate of 12.5%. Common presentations include a radial ray deficiency or radial ray hand, radial clubhand, radial aplasia, hypoplasia, or dysplasia being a possible portion of the presentation. The radial effect can occur at the level of the forearm and hand and commonly involves the thumb. While surgical decision-making about these deformities can be complicated on the basis of procedural choice and timing, it is particularly important to understand associated medical conditions, such as Holt-Oram syndrome in the cardiovascular system, bone marrow suppression such as Fanconi pancytopenia syndrome, or the VACTERL association of vertebral anomalies (ie, anal atresia, cardiac anomalies, tracheoesophageal fistula, renal, and limb anomalies). Centralization of the wrist is sometimes indicated prior to but in conjunction with thumb reconstruction. For absence of the thumb or for a substantially hypoplastic thumb, removal of that digit and pollicization of the index finger to create a thumb may be indicated (Figure 10-1). For less involved hypoplastic thumbs, local reconstruction may suffice.4

A

B

Figure 10-1. For absence of the thumb or for a substantially hypoplastic thumb (A), that digit may be removed, and pollicization of the index finger may be performed to create a thumb (B).

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Proximal-Distal Outgrowth Anomalies: Brachymelia With Brachydactyly, Symbrachydactyly, Transverse Deficiency, and Intersegmental Deficiency Brachydactyly describes disproportionately short fingers and toes. It is a common feature of associated syndromes. The absence of a phalanx and associated interphalangeal joint is a common presentation, but in and of itself, it does not require intervention. Because many of these patients also present with associated syndactyly, syndactyly release and reconstruction with proximal web space deepening can effectively lengthen the congenitally short digit, thus improving function. Like most congenital hand anomalies, because this is the hand that children are born with and use from birth, they optimize their functional outcome more than adults who experience a similar traumatic injury (Figure 10-2).5 Symbrachydactyly describes a unilateral malformation in which there is failure of formation of the fingers with only short rudimentary digits that include elements of the nail plate, bone, and cartilage. Typically, the hand lacks central digits with the border digits spared. Syndactyly between the digits may be present.6 The incidence of symbrachydactyly is roughly 0.6 in 10,000 live births. It is usually isolated but can be associated with Poland syndrome, in which hypoplasia or absence of the pectoralis major muscle occurs with additional variable abnormalities.7 The goals of treatment are to maximize function and improve appearance. Nonsurgical options include hand therapy, prostheses, and orthotics. Surgical options address the specific concerns. The syndactyly and web contracture may be treated to improve independent digital function, grasp span, and appearance. This generally involves local tissue rearrangement. Management of the brachydactyly and poor opposition may include lengthening of the existing metacarpal or transfer of a nonvascularized or vascularized portion of the great toe. These procedures are designed to improve the length and stability of one or more fingers to improve grasp and appearance.

Figure 10-2. Brachydactyly is a common presentation with associated syndromes. The absence of a phalanx and associated interphalangeal joint is common and does not require intervention. Many of these patients also present with associated syndactyly, so syndactyly release and reconstruction with proximal web space deepening can effectively lengthen the congenitally short digit and improve function.

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Radial-Ulnar (Anteroposterior) Axis Anomalies: Radial Longitudinal Deficiency, Ulnar Longitudinal Deficiency, Ulnar Dimelia, Radioulnar Synostosis, and Humeroradial Synostosis Radial longitudinal deficiency encompasses a host of dysplasia and hypoplasia that affect the upper limb, in which the hand has a perpendicular relationship between the forearm and the wrist. The thumb and forearm are usually deficient. It is bilateral in 50% to 72% of cases, and the reported incidence rate is 1 in 100,000. It likely related to an aberration of the SHH gene. Function of the limb is influenced by anomalies of the bone, muscles, nerves, vessels, and/or joints of the thumb and radius. Potential associated conditions include Fanconi anemia, Holt-Oram syndrome, and VACTERL association. As such, the workup should include renal ultrasonography, echocardiography, and a complete blood cell count. Nonsurgical treatment involves passive stretching that targets the tight radial-sided structures. Surgical intervention is performed at 6 to 12 months of age, if there is good elbow motion and the function of the biceps is intact. This involves centralization of the hand on the ulnar bone, followed by tendon transfers. Dorsal-Ventral Axis Anomalies: Nail-Patella Syndrome Nail-patella syndrome is a constellation of anomalies that affects the nails and the larger joints.8 The nails may be absent or underdeveloped and discolored, split, ridged, or pitted. The fingernails are affected more often than the toenails, and the thumbnails are usually the most severely affected of all the digits. The patellae may be hypoplastic, deformed, or absent. Some children may not be able to fully extend their arms or supinate their hands with the arms in extension. The eyes and kidneys may also be affected, which can lead to glaucoma or renal failure. Failure of Axis Formation/Differentiation in the Hand Plate Radial-Ulnar (Anteroposterior) Axis Anomalies: Radial Polydactyly, Triphalangeal Thumb, and Postaxial (Ulnar) Polydactyly Duplication implies polydactyly, manifesting as extra digits or digit parts. Duplication on the preaxial side of the hand or the radial side manifests most commonly as thumb duplication, with potentially associated syndromic issues in the heart (Holt-Oram syndrome) or bone marrow (Fanconi anemia). The most common preaxial thumb duplication presentation is classified as a Wassel type 4 duplication, seen as complete duplication of the distal and proximal phalanges (Figure 10-3). Wassel classifications type 1 through 6 are associated with the number of total skeletal parts in the digit, with even numbers occurring at joints and odd numbers occurring as skeletal bifurcations. Duplications that require surgical reconstruction most commonly involve digit oblation, articular surface tailoring to accommodate the smaller residual retained functional part, and osteoligamentous repair. Rarely are these simply performed as amputation procedures.9

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A

B

C

Figure 10-3. Duplication on the preaxial side of the hand, or the radial side, manifests most commonly as thumb duplication. There can also be potential associated syndromic issues in the heart (eg, Holt-Oram syndrome) or bone marrow (eg, Fanconi anemia). Wassel type 4 duplication is shown (A and B), which indicates complete duplication of the distal and proximal phalanges. C, A radiograph demonstrates the duplication on the right hand.

Duplication on the ulnar side has an incidence of 1.5 in 1,000 live births and may be associated with genitourinary and gastrointestinal anomalies but is usually idiopathic. This is otherwise called postaxial (ulnar) polydactyly. The surgical treatment of postaxial polydactyly is most commonly performed via excision of the duplicated part, with associated neurovascular dissection protecting and preserving the proper digital artery and nerve on that side of the digit. Occasionally, skeletal reconstruction or collateral ligament reconstruction is required. A properly performed suture ligation achieved in the delivery suite or the neonatal intensive care unit continues to be a treatment option. When performed correctly, this can prevent an unnecessary surgical procedure later in life. On the other hand, an inadequately performed ligation can result in persistent vascularity to the digit, infection, or evolution of a residual nubbin or neuroma.

Dorsal-Ventral Axis Anomalies: Dorsal Dimelia (Palmar Nail) and Hypoplastic/Aplastic Nail Dorsal and ventral dimelia refer to the appearance of dorsal (or ventral) structures on the palmar (or dorsal) aspect of the hand, respectively.10 They are rare congenital hand malformations that are caused by errors of the dorsoventral axis of development of the limb.

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Failure of Axis Formation/Differentiation, Unspecified Axis Soft-Tissue Anomalies: Syndactyly and Camptodactyly While there are multiple congenital hand anomalies that manifest within the classification of failure of differentiation, the most common one is syndactyly. Syndactyly is seen more commonly in boys than in girls by a 2:1 ratio, respectively, and most frequently involves the third web space, between the middle and ring fingers (Figure 10-4). This most commonly manifests as incomplete simple syndactyly. Simple syndactyly means there is no skeletal, cartilage, or nail bed fusion between the 2 digits involved in the syndactyly. Complex syndactyly involves fusion of the distal phalanx or nail bed, while complicated syndactyly involves additional anomalies, structures, or other processes. Incomplete syndactyly does not extend to the end of the finger, while complete syndactyly extends to the end of the digit. Description of syndactyly should always include whether it is complete or incomplete, as well as simple, complex, or complicated. While many congenital hand surgeries, including surgery for syndactyly, are performed at or after the age of 1 year to minimize anesthetic risks, border digit syndactyly, which means syndactyly occurring between the thumb and index finger or the ring and little finger, should be repaired at 6 months of age or as indicated by the amount of local deformity. Because the digit length discrepancy can be significant, syndactyly in these border digits can cause longerterm functional consequences, in contrast to the second and third web space involvement. To perform syndactyly reconstruction, the fingers are commonly separated by using some type of zigzag-patterned local flaps from the adjacent digits. Then, full-thickness skin grafts may be required from the abdomen to supplement the inadequate soft tissues that are present (Figure 10-5). While there are occasional situations in which a full-thickness skin graft is not required, most commonly, skin grafting is an inherent part of any successful syndactyly release, preventing longer-term growth complications associated with inadequate soft-tissue reconstruction at the first surgical procedure.11

Figure 10-4. Syndactyly is seen more commonly in boys than in girls and most frequently involves the third web space, between the middle and ring fingers.

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A

B

Figure 10-5. A, To perform syndactyly reconstruction, the fingers are commonly separated by using zigzag-patterned local flaps from the adjacent digits. B, Full-thickness skin grafts are then required from the abdomen to supplement the inadequate soft tissues.

Camptodactyly is also classified as failure of differentiation. This manifests with volar flexion of a digit commonly seen without a well-defined etiologic origin (Figure 10-6). This normally occurs at the proximal interphalangeal joint, with most cases occurring bilaterally. For significant camptodactyly, early splinting may be helpful. Surgical intervention has a fairly high complication rate and has less surgical success than many other congenital hand anomalies. Thus, one should consider whether to pursue surgical intervention with care.

Figure 10-6. Camptodactyly manifests with volar flexion of a digit and is commonly seen without a well-defined etiologic origin. It is classified as failure of differentiation.

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A

B

Figure 10-7. Clinodactyly manifests as either radial or ulnar deviation, most commonly of the little finger. It occurs in up to 15% of the pediatric population. Photograph (A) and associated radiograph (B) are shown.

Skeletal Deficiencies: Brachydactyly, Clinodactyly, Kirner Deformity, and Metacarpal and Carpal Synostoses Clinodactyly is a common deformity, seen in up to 15% of the pediatric population. This manifests as either radial or ulnar deviation, most commonly of the little finger (Figure 10-7). This deformity can respond to early splinting, with surgical intervention for clinodactyly less than 35° unlikely to be of functional significance. This would involve removal of a wedge-shaped portion of bone to straighten the bone. Assessment on a case-to-case basis is essential, as other associated skeletal anomalies can be present. Kirner deformity (dystelephalangy) is characterized by radial and volar deviation of the distal phalanx of the fifth finger.12 The condition is rare and often presents with painless swelling of the distal interphalangeal joint in the early teenage years. Girls are affected more than boys and the right hand is affected more than the left. Because the deformity does not improve with observation alone, treatment options may include splinting with or without osteotomy, distraction lengthening, hemiepiphysiodesis, and/or distal detachment of the FDP tendon.13 Complex Hand Anomalies: Cleft Hand, Synpolydactyly, and Apert Hand Cleft hand usually manifests as absence of the middle finger ray with associated syndactyly of the fourth web space and/or the first web space (Figure 10-8). Hypoplasia of associated digits can also be present. The right side is more commonly involved than the left, and this deformity can be familial, although most cleft hands are sporadic. Cleft hand is commonly associated with cleft foot deformity; therefore, tandem surgery may be indicated to optimize a child’s upper- and lower-extremity function. Clinically significant cleft hand is commonly treated with the Snow-Littler procedure, in which the index finger is transferred as a digital neurovascular island onto the middle finger metacarpal remnant, allowing creation of an adequate first web space and closing the cleft gap simultaneously.

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A

B

Figure 10-8. Cleft hand usually occurs as an absence of the middle finger ray, with associated syndactyly of the fourth web space and/or the first web space. Dorsal (A) and palmar (B) views are shown.

Symbrachydactyly, or atypical cleft hand, is diagnosed as a central deficiency that manifests as a more U-shaped defect in the midportion of the hand, in contrast with the V-shaped defect of the true cleft hand. In this U-shaped cleft are hypoplastic digits commonly referred to as nubbins. These can rarely be used to reconstruct digits and are most commonly amputated with attention given to the soft-tissue closure, facilitating creation of an optimal web space between the more commonly spared thumb and little finger. The incidence rate of this anomaly is roughly 1 in 40,000 births (Figure 10-9). Atypical symbrachydactyly can be associated with Poland syndrome, which commonly manifests with brachysyndactyly of the fingers and with shoulder girdle anomalies most commonly noted as an absence of the pectoralis major muscle, leading to a visible and palpable absence of the anterior axillary fold (Figure 10-10). Other shoulder girdle muscles can be missing, as can ribs. In girls, breast development is commonly abnormal and may necessitate formal surgical intervention during puberty or after puberty is complete.

A

B

Figure 10-9. Symbrachydactyly, or atypical cleft hand, is a central deficiency with a U-shaped defect in the mid hand. In this U-shaped cleft are hypoplastic digits that are most commonly amputated. Photograph (A) and associated radiograph (B) are shown.

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Figure 10-10. Atypical symbrachydactyly can be associated with Poland syndrome, which commonly manifests with brachysyndactyly of the fingers and shoulder-girdle anomalies most commonly noted as an absence of the pectoralis major muscle, leading to visible and palpable absence of the anterior axillary fold.

Synpolydactyly is a joint presentation of syndactyly and polydactyly.14 One to 4 limbs can be involved, and the severity of involvement ranges from partial skin syndactyly to complete duplication of a digit (phalanges and possibly the metacarpals/metatarsals). Treatment may involve a combination of resection and separation.

Deformations Constriction Ring Syndrome Constriction ring syndrome, or amniotic band syndrome, is a well-defined anomaly that affects only 1 in 10,000 births but can cause substantial dysfunction to the patients who manifest it. This syndrome can span simple bands without substantial vascular effects, up to the equivalent of intrauterine amputations and missing digits. Type 1 bands show a shallow constriction without vascular compromise, while type 2 bands show deeper constriction and the potential for evolving vascular embarrassment. Type 3 bands show distal fusion or threatened digit loss, and type 4 manifests where intrauterine amputations have occurred (Figure 10-11). Because of developmental timing, it can be associated with other congenital differences, including clubfeet, atypical facial clefts, cleft lip and palate, abdominal wall defects, and cutis aplasia. Early release of vascular-threatened digits via excision of the band (not incision) and Z-plasty closure of one-half of a digit in one surgical procedure is essential to allow neolymphatic and neovenous circulation to reestablish, thus preserving the digit. This staged excision is performed to prevent secondary vascular embarrassment in the inadvertent injury to

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Figure 10-11. Photograph of a patient with types 1, 2, and 3 constriction ring syndrome, indicated by shallow constriction without vascular compromise, deeper constriction and the potential for evolving vascular embarrassment, and distal fusion or threatened digit loss, respectively.

vascular inflow, as well as not compromising vascular outflow. For hands that manifest amniotic band syndrome as multiple digit amputations, web space deepening can effectively lengthen digits to improve function. Some of these deformities can be treated at the same time that other associated anomalies are cared for, including on the day of birth for patients who require treatment for acute abdominal wall defects.15

Arthrogryposis Arthrogryposis describes congenital, nonprogressive contractures that affect one or more areas of the body.16,17 Mutations in more than 400 genes have been ascribed to the different forms of arthrogryposis. Isolated congenital contractures affect approximately 1 in 500 individuals in the general population, with boys and girls affected equally. The most common form of an isolated, congenital contracture is clubfoot. When arthrogryposis affects 2 or more different areas of the body, it may be referred to as arthrogryposis multiplex congenita. The joints of the legs tend to be affected more often than the arms, and the muscles of affected limbs may be hypoplastic. Early physical therapy can improve joint motion and prevent muscle atrophy in the newborn period. Removable splints, and possibly serial casting, for the knees and ankles are also recommended. In some cases, tendon transfer may be required to improve muscle function. Trigger Digits Triggering of a digit occurs when one or more flexor tendons is unable to glide under the proximal pulley of the flexor sheath as the finger bends.18 A small nodule may be palpable on the volar surface of the tendon. When

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the nodule catches on the pulley, the patient has a sensation of painful catching or popping. The initial treatment for a trigger finger is usually nonsurgical and includes rest, splinting, gentle stretching, and nonsteroidal anti-inflammatory drugs, to alleviate pain and inflammation. Injection of corticosteroid into the tendon sheath may also resolve the triggering over a period of 1 day to several weeks. If symptoms do not improve with time, a second injection may be given. Failure of injection may be an indication for surgical intervention, which involves transecting the constrictive proximal flexor pulley.

Dysplasia: Macrodactyly

Overgrowth can occur at any point along the upper limb or lower limb, but it most commonly manifests as macrodactyly, an isolated, congenital, nonhereditary congenital digit enlargement (Figure 10-12). Macrodactyly can involve digits of the hand or foot and can be skeletal or soft tissue in nature, or a combination of deformities. While macrodactyly is rare, constituting 0.9% of all congenital hand anomalies, it is commonly obvious at visual inspection but can be associated with excellent function. Neurofibromatosis type 1 (von Recklinghausen disease) should be excluded in children who present with macrodactyly if the clinical picture is appropriate. Ninety percent of cases are unilateral, and 70% involve more than one digit, which are usually adjacent. Sixty percent of cases occur in the right hand, and the index finger is most commonly affected. In order of decreasing frequency, the long finger, thumb, ring finger, and small finger are also involved. Associated conditions include Proteus syndrome, Bannayan-Riley-Ruvalcaba disease, Maffucci syndrome, Ollier disease, and Milroy disease.

Figure 10-12. Overgrowth can occur at any point along the upper limb or lower limb, but most cases commonly present as “macrodactyly.” Note the large digits on the left hand of this patient.

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Treatment can involve observation, soft-tissue debulking, skeletal resection, growth plate destruction (epiphysiolysis), and, occasionally, amputation. The goal is always to optimize function, with initial conservative interventions moving to more aggressive ones as indicated. Reconstruction will not result in a normal digit but is well tolerated and may offer improvement in the appearance and function of the digit. While the cosmetic appearance of the hand should be optimized as part of this functional surgery approach, the functionality outcome is the primary consideration.19 REFERENCES

1. Tonkin MA, Oberg KC. The OMT classification of congenital anomalies of the hand and upper limb. Hand Surg. 2015;20(3):336–342 PMID: 26387992 https://doi.org/10.1142/ S0218810415400055 2. Mason C. Congenital hand anomalies: embryology and classification. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1075–1084 3. Borschel G, Ho E, Clarke H. Congenital hand anomalies: failure of formation. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1085–1126 4. Upton J, Coombs C. The hypoplastic and absent thumb. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1127–1190 5. Egerszegi P. Congenital hand anomalies: undergrowth. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1287–1332 6. Gupta A, Kay SP, Scheker LR. The Growing Hand: Diagnosis and Management of the Upper Extremity in Children. Maryland Heights, MO: Mosby; 2000 7. Ekblom AG, Laurell T, Arner M. Epidemiology of congenital upper limb anomalies in 562 children born in 1997 to 2007: a total population study from Stockholm, Sweden. J Hand Surg Am. 2010;35(11):1742–1754 PMID: 20961708 https://doi.org/10.1016/j.jhsa.2010.07.007 8. Sweeney E, Fryer A, Mountford R, Green A, McIntosh I. Nail patella syndrome: a review of the phenotype aided by developmental biology. J Med Genet. 2003;40(3):153–162 PMID: 12624132 https://doi.org/10.1136/jmg.40.3.153 9. Sood R, Cohen A. Polydactyly. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1249–1264 10. Al-Qattan MM. Classification of dorsal and ventral dimelia in humans. J Hand Surg Eur Vol. 2013;38(9):928–933 PMID: 23592534 https://doi.org/10.1177/1753193413484671 11. Havlik R. Failure of differentiation of the human hand. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1191–1231 12. Fairbank SM, Rozen WM, Coombs CJ. The pathogenesis of Kirner’s deformity: a clinical, radiological and histological study. J Hand Surg Eur Vol. 2015;40(6):633–637 PMID: 25274771 https://doi.org/10.1177/1753193414551911 13. Gamo K, Kuriyama K, Uesugi A, Nakase T, Hamada M, Kawai H. Percutaneous corrective osteotomy for Kirner’s deformity: a case report. J Pediatr Orthop B. 2014;23(3):277–281 PMID: 24590256 https://doi.org/10.1097/BPB.0000000000000042 14. Temtamy SA, McKusick VA. The genetics of hand malformations. Birth Defects Orig Artic Ser. 1978;14(3):i–xviii, 1–619 PMID: 215242 15. Dumanian G. Amniotic band sequence. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1333–1342 16. Hall JG. Arthrogryposis (multiple congenital contractures): diagnostic approach to etiology, classification, genetics, and general principles. Eur J Med Genet. 2014;57(8):464–472 PMID: 24704792 https://doi.org/10.1016/j.ejmg.2014.03.008

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152 Pediatric Plastic and Reconstructive Surgery for Primary Care 17. Bamshad M, Van Heest AE, Pleasure D. Arthrogryposis: a review and update. J Bone Joint Surg Am. 2009;91(suppl 4):40–46 PMID: 19571066 https://doi.org/10.2106/JBJS.I.00281 18. Saldana MJ. Trigger digits: diagnosis and treatment. J Am Acad Orthop Surg. 2001;9(4):246–252 PMID: 11476534 https://doi.org/10.5435/00124635-200107000-00004 19. Egerszegi P. Congenital hand anomalies: overgrowth. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing; 2008:1265–1286

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CHAPTER

11

Pediatric Burn Injury HAIG YENIKOMSHIAN, MD, AND WARREN GARNER, MD

Introduction

Burn injuries are a problem encountered by most practitioners who take care of children. As children begin to explore and ambulate, they are particularly at risk for injury. Most of these injuries are small, superficial burns that can be treated effectively by primary care practitioners with local wound care. In contrast, more extensive burns require a specialized burn unit with a multidisciplinary care team that is familiar with burn injuries. This would include a sophisticated intensive care unit (ICU), plastic or pediatric surgeons, and rehabilitative therapists. With the increase in the size and severity of the burn, morbidity and mortality rates grow exponentially, but best care can minimize both. This effective acute care has shifted the focus of burn treatment from survival to outcome and improved quality of life. Most children with modest burns recover completely. Those with serious childhood burns can still go on to have a good quality of life.1 These are the goals of burn treatment today.

Epidemiology

The number of pediatric burns is difficult to calculate because of the various sites where patients present for treatment. Approximately 121,000 pediatric burns are seen in emergency departments annually.2 There are 61,000 pediatric burns that require evaluation in a burn center yearly. Boys have a higher incidence of burns then girls in all age categories. Scald burns are of the highest incidence for patients younger than 5 years, while flame burns are the number one cause of injury for patients between the ages of 5 and 18 years. Most pediatric burns occur in the home.3 As such, any pediatric burn (or trauma) patient should be evaluated for abuse or neglect. The observed injuries should be evaluated in the context of the histories given by both the patient and the caregiver(s). Physicians have the responsibility for reporting suspicion of abuse to child protection agencies for investigation. They should also coordinate other professionals and community agencies to provide immediate and long-term treatment to such children. If needed, they should provide court testimony when necessary, as well as preventive care and advocacy for policies and programs that support families and protect children.4 153

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Patient Presentation

A pediatric patient with burns may present in different settings. Patients with simple burns often present in the pediatric office, while patients with more severe burns present in the emergency department. Treatment goals for the simple burn are local wound care and pain control, while severe burn requires further evaluation, workup, emergent management, and referral to a burn center. Scald injuries are the most common mechanism in children. Although usually small in area, scald injuries can encompass large surface areas and can be severe. Flame burns can happen in numerous manners, from house fires, campfires, electrical burns, or motor vehicle accidents. Serious electrical burns are uncommon in children, although toddlers will sometimes chew electric cords. If the burn looks atypical or the history seems to be inconsistent, the initial burn evaluator should always consider child abuse. Cigarette burns, stocking distribution of burn patterns, and burns occurring during negligence should all be thoughtfully considered. Figure 11-1 shows a stocking distribution scald burn to the bilateral lower extremities that is highly suspicious for child abuse. Reports have suggested as many as 16% of pediatric burn admissions are the result of abuse.5 If child abuse is remotely a consideration, the child should be admitted to the hospital, and protective services should be contacted for an evaluation. Serious burn injuries must be considered with a similar approach to other traumas—the ABC algorithm (airway, breathing, circulation) is essential. The airway should be evaluated first in a serious burn trauma. Inhalation of hot gas will induce local edema in the airway, quickly inducing loss of patency. Inhalation of aerosolized particles causes subsequent parenchymal lung damage. Inhalation injury is a serious complication of burns and must be identified early in treatment. Patients may initially present with no respiratory distress; however, inhalation of these toxins will cause subsequent soft-tissue

Figure 11-1. Stocking-distribution scald burn to the bilateral feet in a pediatric patient, which is highly suggestive of child abuse.

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edema hours after injury, causing a loss of airway patency. The mechanism of burn can also help guide the suspicion for inhalational injury. Enclosed spaces are of particularly high risk. At physical examination, factors such as carbonaceous sputum in the oropharynx, singed nasal hairs, and voice changes can be signs of inhalational injury.6 An arterial blood gas analysis can also be helpful in determining the carboxyhemoglobin level. Arterial oxygen saturation with a pulse oximeter is not a useful means of assessing for inhalational injury because values can be falsely increased. If it is difficult to assess whether patients have inhalational injury, they can undergo serial endoscopy by an experienced physician to assess whether there are changes of the soft tissue in the larynx. If edema is present, the patient’s airway should be splinted open by intubation. Other factors in the acute care setting are acquiring proper intravenous (IV) access and IV resuscitation. Large-bore IVs should be placed for significant burns, as patients will require large amounts of fluid volume in a short period. Central venous lines may be used but are not necessary. The amount of resuscitation needed is discussed later in this chapter. A tetanus vaccination should also be administered if the patient’s status is not up-to-date or is unknown. Systemic antibiotics are not recommended for acute burns because they will not provide adequate antimicrobial penetration to burned areas. If patients will be transferred to a burn unit, their wounds should be covered in dry, clean dressings only prior to transfer. Criteria for transfer to a burn unit for the pediatric population has been described by the American Burn Association and are shown in Box 11-1.

Box 11-1. Criteria for Transfer to a Burn Unit Burn Unit Referral Criteria A burn unit may treat adults, children, or both. Burn injuries that should be referred to a burn center include the following: • Partial-thickness burns of greater than 10 percent of the total body surface area. • Burns that involve the face, hands, feet, genitalia, perineum, or major joints. • Third-degree burns in any age group. • Electrical burns, including lightning injury. • Chemical burns. • Inhalation injury. • Burn injury in patients with preexisting medical disorders that could complicate management, prolong recovery, or affect mortality. • Burns and concomitant trauma (such as fractures) when the burn injury poses the greatest risk of morbidity or mortality. If the trauma poses the greater immediate risk, the patient’s condition be stabilized initially in a trauma center before transfer to a burn center. Physician judgment will be necessary in such situations and should be in concert with the regional medical control plan and triage protocols. • Burns in children; children with burns should be transferred to a burn center verified to treat children. In the absence of a regional pediatric burn center, an adult burn center may serve as a second option for the management of pediatric burns. • Burn injury in patients who will require special social, emotional, or rehabilitative intervention. Excerpted with permission from Committee on Trauma, American College of Surgeons. Resources for Optimal Care of the Injured Patient. Chicago, IL: American College of Surgeons; 2014:101.

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Diagnosis

Five factors are needed for complete diagnosis of a burn injury: the size of the burn (measured according to total body surface area [TBSA]), the depth of the burn, the anatomical part of the patient burned, any associated injuries, and other medical or psychosocial factors that will affect the patient’s response to the injury or the planned treatment. Assessing the severity of the burn, as well as TBSA, may be difficult in the acute setting. However, an accurate description of the degree of burns, the anatomical locations, and the TBSAs are integral in the triage and treatment of the pediatric burn. The terminology and identification of first-, second-, and third-degree burns are an important step in the evaluation. A first-degree burn penetrates only the epidermis. Presentation is usually erythematous skin without blisters. These can be very painful for the patient and, if the burns encompass a large area, might necessitate the use of large amounts of pain medication. Sunburns are typical examples of first-degree burns. Figure 11-2 shows first-, second-, and third-degree burns in the same patient. As shown, the first-degree burns are on the very border of the de-epithelized area and, in this case, are only mildly erythematous. A second-degree burn, or partial-thickness burn, is one that penetrates into the dermis. This can be subdivided into superficial partial-thickness and deep partial-thickness burns. Superficial partial-thickness burns are pink and painful. These burns tend to blanch after pressure is applied and removed. They often have overlying blisters, with clear serous fluid or blood.

1st

2nd

3rd

Figure 11-2. A scald burn that shows first-, second-, and third-degree burns.

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Deep partial-thickness burns are typically less painful and have a mottled pink and white appearance. They may still blanch and can be tender. In Figure 11-2, which shows a combination of the 3 types of burns, in the area that is marked “2nd,” the whiter areas are deep partial-thickness burns, while the pink, moist areas are more superficial burns. A third-degree burn is also known as a full-thickness burn. The thermal injury has penetrated through the entire epidermis and dermis. The appearance is pale, leathery, and dry. These burns are typically painless, as nerve endings have also experienced thermal injury. Third-degree burns require very careful monitoring with good wound care and are typically candidates for surgical treatment. Figure 11-2 shows a full-thickness burn with the typical dry, leathery appearance in the area marked “3rd.” Estimating the TBSA of a burn is important for the triage and treatment of the burn patient. The more extensive the burn, the higher the insensible losses and inflammatory responses. Fluid resuscitation is key to providing adequate perfusion to tissues and limiting the extent of the burn injury. Total body surface area is also useful in determining the severity of the burn, need for nutritional supplementation, degree of pain medication administered, and need for transfer to a burn center. When calculating the TBSA, only secondand third-degree burns are used in the calculation. Typically, the rule of 9s is an effective means for determining the burn size, as shown in Figure 11-3. For adolescents and adults, each upper extremity and the head are 9% of the TBSA apiece; each leg, the chest, and the back are 18% apiece; and the genitals constitute the remaining 1%. The pediatric patient’s percentage is different, with the head constituting a larger portion of the TBSA. The patient’s palm is also a quick reference to what 1% of the patient’s body area could be. Specific areas of the body require special attention in evaluation. If patients experience scalding or flash burns to the face, an ophthalmologic consultation may be needed to evaluate their eyes. At physical examination, patients may have singed eyelashes and injected conjunctiva. Any second- or third-degree burns should be evaluated by a burn surgeon and an ophthalmologist early, to prevent contractors and future ocular sequelae. Deep second-degree burns of the face and perioral tissues may scar and contract, limiting the mouth opening. Intense therapy is essential. Mouth burns from chewing on electrical cords usually heal reasonably well but can become problematic for the patient if not treated correctly. Parents must be warned about labial artery bleeding and instructed to apply pressure or seek medical attention immediately if bleeding occurs. Hand burns are frequently seen in the pediatric burn population, because children often reach for objects and subsequently experience burns. These burns require special attention in a burn unit, because maintaining motion by keeping the skin flexible is essential for joint and bone development. Any deep burns should be treated surgically, and patients must undergo aggressive therapy so that range of motion or function of the hand is not lost. One of the complications can be severe

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9

Front 18 9

Back 18

18

1

Front 16 10

9

10 Back 16

18

18

1 14

14

Figure 11-3. The rule of 9s. For adolescents and adults, each upper extremity and the head are 9% of the total body surface area (TBSA) apiece; each leg, the chest, and the back are 18% apiece; and the genitals constitute the remaining 1%. The pediatric patient’s percentage is different, with the head making up a larger portion of the TBSA. From the American Burn Association. Burn center referral criteria. http://ameriburn.org/wp-content/ uploads/2017/05/burncenterreferralcriteria.pdf. Accessed February 4, 2020.

contractures, as shown in Figure 11-4. Genital burns also require special care, and if the urethra is involved, a Foley catheter should be placed early, prior to swelling of the soft tissue, to prevent urinary retention. The catheter can be removed once soft-tissue swelling has decreased.

Management

The treatment of all burn injuries requires local, topical wound treatment; provision of sufficient nutrition for the injury to heal; and pain control. Full-thickness burns require excision of necrotic skin and skin grafting. Burns that cover a larger surface area induce a systemic inflammatory state, which can require intense medical care or support in the ICU. Burn management can be subdivided into local and systemic management, which is usually reflected in small and large burns. A small pediatric

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Figure 11-4. Contracture in a 3-year-old from a flame burn injury that was left untreated.

burn is one that is less than 10% of the patient’s TBSA. Generally speaking, these burns are treated with local wound care (different options for which will be discussed later in this section). These can be treated on an outpatient basis if the patient and family meet the following criteria: First, the patient must have good oral intake. Thermal injury increases the metabolic state, and proper nutrition is paramount for proper wound healing. In the authors’ burn unit, if it is not believed that the patient will have adequate oral intake, a Dobhoff feeding tube is placed and enteral nutrition is begun. Second, if the patient’s responses suggest that he or she is going to require large amounts of pain medicine or IV pain medication, admission to the hospital is considered. Third, proper wound care, including dressing changes, are important for burn healing. If the dressing change is too large or too complicated for the family to perform, the patient may require hospital admission. Finally, family support for the patient is assessed. If, for any reason, the social situation at home is not optimal, a pediatric patient should be admitted for proper care. When the patient does not clearly meet these expectations, the patient is admitted to the burn center for supportive care. Patients with larger burns—10% to 20% of TBSA—are generally admitted to the burn ward. Patients with burns larger than 20% of TBSA are admitted to the ICU for monitoring. Clinically significant burn injuries cause an inflammatory response that can affect the entire body. If this response becomes excessive, the patient may experience systemic inflammatory response syndrome, which is an inflammatory response that affects the whole body. Large and deep burns in the pediatric population may cause this response; close monitoring and support in the ICU are necessary to prevent multisystem organ failure. Morbidity and mortality in these situations are high and require coordination between burn surgeons and pediatric ICU specialists.

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The immediate concern for patients with larger injuries is the replacement of fluids that predictably transudate into tissues after major injuries. Resuscitation for this systemic response is necessary to compensate for the insensible losses of the burns and to keep the remaining tissues and organs well perfused. The Parkland formula is the classic method for estimating fluid resuscitation. The formula is as follows: 4 × percentage TBSA of second- and third-degree burns × weight in kilograms. The first half of this number is given in the first 8 hours after injury, and the rest over the next 16 hours.7 For children who are younger than 14 years or weigh less than 40 kg, the Parkland formula changes to the following: 3 × body weight in kilograms × TBSA of second- and third-degree burns. Patients weighing less than 10 kg should be given dextrose 5% in lactated Ringer injection.8 If presentation is delayed, fluid requirements may be higher. The goal for this resuscitation is maintaining perfusion, so fluid rates must be adjusted on the basis of the response. We use urine output as an outcome monitor, with a goal of 1.0 to 1.5 mL/kg/h. Narcotics for pain control and a sedative should be delivered via infusion until oral absorption is ensured. If the patient requires high doses of narcotics, intubation may be necessary. The goals of wound dressing are to prevent infection and provide a microenvironment supportive of healing. Choosing the best wound dressing is a critical decision and must be individualized. One must consider the ease and frequency of dressing changes, as well as the pain from dressing changes. Generally, for healthy wounds and healthy patients, antimicrobial occlusive dressings are used. For more serious injuries, the type of wound dressing becomes more challenging. While there are many different treatment options, there have been few good prospective trials that show one treatment to be superior to another.9 Silver sulfadiazine is the most commonly used topical agent, and it is used with gauze burn dressings. Silver sulfadiazine is manufactured as a 1% topical cream. The silver ions have excellent antimicrobial properties and penetration. The problem is that the ions become oxidized and need to be changed daily, which causes pain and is inconvenient for families. Additionally, the oxidization process causes the cream to turn yellow, and many families and inexperienced health care professionals mistake this for pus. Mupirocin comes in ointment form and provides antimicrobial coverage for staphylococcus, streptococcus, and methicillin-resistant Staphylococcus aureus. This ointment adheres well, so it can be used without a dressing on the face and ears, but it can also be used for small injuries elsewhere. It should be changed twice a day. Silver-releasing dressings are a newer form of burn dressings that are ideal for patients who do not need daily evaluation of the burns and have healthy, noninfected wounds.10 The advantage of these dressings is that they do not require frequent dressing changes. In the authors’ unit, an antimicrobial foam dressing (Mepilex AG; Mölnlycke Health Care, Norcross, GA), which is a silver-impregnated foam that can be left in place for up to 7 days,

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is used most often. The foam may be cut and stuck onto different shapes and has been used effectively on small areas, such as the hand, as well as burns with large surface areas. Figure 11-5 illustrates the versatile use of the antimicrobial foam dressing, which is tailored to fit the hand. After 5 days, the foam dressing peels off easily, and there is minimal trauma for the child. Silver sheeting is also an effective way of delivering antimicrobial dressings. There are more than 20 other silver-releasing dressings, with individual benefits and disadvantages. The choice of which product to be used is based on practitioner familiarity, cost, and availability. There are countless types of burn dressings, some of which are of historical interest only, and many of which have not proven to be as effective as the materials listed herein. The recommendation to treat the burn surgically can be difficult for parents and children to hear. For life-threatening burns, families are acceptant. For smaller injuries, parents often ask if surgery is necessary. It is important to explain that slow, prolonged healing results in more severe scarring, more pain, and a prolongation of the time the child must go through until returning to a normal life. Typically, third-degree burns and deep seconddegree burns should be excised and skin grafting performed. Deep burns that are not surgically removed may become infected and cause burn sepsis. Also, deep burns can cause contractions that may be more debilitating than surgery. The typical surgery for this is tangential excision, which means excising down to healthy bleeding tissue. Small burns can sometimes be excised and closed primarily or through local tissue rearrangement. Larger burns will require skin grafting. Donor sites for skin grafts are ideally taken from areas that will be covered by clothes in the future. Figure 11-6 demonstrates a primary A

B

C

Figure 11-5. An antimicrobial foam dressing (Mepilex AG; Mölnlycke Health Care, Norcross, GA) to the hand. A, Scald wound to the palmar surface of the hand. B, The foam dressing being placed, which will be cut to fit. C, Wrapping of the foam with the outer hand dressing.

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Figure 11-6. Deep partial-thickness burns to the thigh and pubic area. Primary excision of the pubic area and a split-thickness skin graft to the right thigh were performed.

closure in the pubic area and a split-thickness skin graft to the areas of the thigh. Dressings are taken down on postoperative day 2 for sheet grafts, and underlying seromas are drained with an 18-gauge needle. Meshed skin grafts can be bolstered down for a total of 5 to 7 days before dressing takedown. The risks of surgery in the pediatric burn population are minimal, with the obvious exception of a skin graft donor site. Postoperative management of burns is as critical as the surgery itself. Once discharged, patients have close weekly or biweekly follow-up visits until the burns are well healed. They continue to undergo occupational and physical therapy regularly. Healing happens over months as the area of grafting becomes incorporated into the wound bed and the donor site re-epithelizes. Maintaining motion over the joints is important, and occupational and physical therapists are integral parts of any burn team to ensure early and aggressive treatment. Joints that are not actively moved in the early postoperative period will become stiff and form contractures, which are much more difficult to manage in the future. Psychological support and a stable social situation are essential to the child and family for best recovery. Burn center teams include child life specialists, social workers, and access to pediatric psychological and psychiatric support. For children with smaller injuries, it is important for the pediatrician to notice obsessive concern for scarring and to reassure parents when indicated. Burns are a common traumatic injury, and it is important to assess the patient for posttraumatic stress disorder, as well as other psychological needs. Identifying and treating these problems early will help the child recover from the overall trauma of the burns.

Complications and Outcomes

Most pediatric burn patients survive and have few serous long-term medical sequelae, with the exception of changes in functional mobility and appearance. Scarring and contractures are common in areas of deep second-degree burn

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injury, when meshed grafts are needed to perform lifesaving wound closure or when children were not treated optimally. Healing by wound contraction, the exuberant healing of children, and the limited growth potential of scar tissue are the cause of the beneficial outcomes. Figure 11-4 shows an example of a burn scar that was left to contract without proper medical treatment. Scars and contractures form in areas not adequately covered by skin grafts or deep second-degree burns that were allowed to heal without surgery. Areas with movement, such as antecubital fossa or axilla, commonly form contractures. Another typical area is the neck; Figure 11-7 shows a patient who underwent a contracture release and rotational flap. Nonsurgical treatments of this can involve steroid injections, silicone sheeting, or compression garments. Surgical treatment performed by experienced plastic surgeons and therapists can restore normal function and improve the appearance in most children who have experienced thermal injury. These deformities may be excised; contractures may be released by means of Z-plasty (ie, a plastic surgery technique used to improve the functional and cosmetic appearance of scars) or a rotational flap to bring in healthy tissue to lengthen the scar (see Figure 11-7). Tissue expansion is also commonly used, which is especially useful in the hair-bearing portion of the scalp. Figure 11-8 shows a tissue expander placed in the occipital region of the scalp. The expanded skin shown in the middle image was then used to excise a significant portion of the burned skin. A

B

C

Figure 11-7. A, Neck contracture after a nonsurgical treatment for a deep second-degree burn that was allowed to heal without surgery. The patient experienced significant scarring and alteration of normal neck motion. B, A first-stage reconstruction, with tissue advancement and rotation from the right neck skin, with partial resection of hypertrophic scar. C, The 6-month postoperative release of contracture with recurrent scar. The patient will undergo the next stage of reconstruction 1 year postoperatively. The motion of the patient’s neck has significantly improved.

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A

B

C

Figure 11-8. Tissue expansion for scalp burn. A, Preoperative scalp burn with a split-thickness skin graft. B, The tissue expander in place with maximal expansion. C, The postoperative rotation of expanded skin.

Summary

Pediatric burns are a common problem that require close evaluation and even closer follow-up. For the child to recover fully, treatment involves the coordination of multiple disciplinary teams, including the burn unit and/or plastic surgeon, pediatrician, occupational and physical therapists, and the social worker. REFERENCES

1. Herndon DN, LeMaster J, Beard S, et al. The quality of life after major thermal injury in children: an analysis of 12 survivors with greater than or equal to 80% total body, 70% third-degree burns. J Trauma. 1986;26(7):609–619 PMID: 3723636 https://doi.org/10.1097/00005373-19860700000004 2. D’Souza AL, Nelson NG, McKenzie LB. Pediatric burn injuries treated in US emergency departments between 1990 and 2006. Pediatrics. 2009;124(5):1424–1430 PMID: 19805456 https://doi.org/10.1542/peds.2008-2802 3. Bessey PQ, Phillips BD, Lentz CW, et al. Synopsis of the 2013 annual report of the national burn repository. J Burn Care Res. 2014;35(suppl 2):S218–S234 PMID: 24642761 https://doi. org/10.1097/BCR.0000000000000080 4. Christian CW; American Academy of Pediatrics Committee on Child Abuse and Neglect. The evaluation of suspected child physical abuse. Pediatrics. 2015;135(5):e1337–e1354 PMID: 25917988 https://doi.org/10.1542/peds.2015-0356 5. Hight DW, Bakalar HR, Lloyd JR. Inflicted burns in children. Recognition and treatment. JAMA. 1979;242(6):517–520 PMID: 448982 https://doi.org/10.1001/jama.1979.03300060019019 6. Grunwald TB, Garner WL. Acute burns. Plast Reconstr Surg. 2008;121(5):311e–319e PMID: 18453944 https://doi.org/10.1097/PRS.0b013e318172ae1f 7. Baxter CR, Shires T. Physiologic response to crystalloid resuscitation of severe burns. Ann N Y Acad Sci. 1968;150(3 Early Treatme):874–894 PMID: 4973463 https://doi.org/10.1111/j.1749-6632.1968. tb14738.x 8. American Burn Association. Advanced Burn Life Support Guidelines. 2010. 9. Wasiak J, Cleland H, Campbell F, Spinks A. Dressings for superficial and partial thickness burns. Cochrane Database Syst Rev. 2013;3(3):CD002106 PMID: 23543513 https://doi.org/10.1002/ 14651858.CD002106.pub4 10. Tang H, Lv G, Fu J, et al. An open, parallel, randomized, comparative, multicenter investigation evaluating the efficacy and tolerability of Mepilex Ag versus silver sulfadiazine in the treatment of deep partial-thickness burn injuries. J Trauma Acute Care Surg. 2015;78(5):1000–1007 PMID: 25909422 https://doi.org/10.1097/TA.0000000000000620

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12

Skin and Soft-Tissue Lesions JORDAN P. STEINBERG, MD, PhD, FAAP, FACS

Introduction

Skin and soft-tissue lesions are commonly encountered by the pediatrician. Often, such lesions are first noted by parents and are brought to the attention of the pediatrician. The pediatrician may then refer parents to pediatric dermatologists or plastic surgeons accordingly. Concern about potential malignant degeneration of skin lesions, as well as unsightliness and teasing by the child’s peers, may drive initial inquiries from parents. While the surgical excision of lesions can substantially reduce these risks in many cases, other attendant risks of intervention need to be considered carefully in the pediatric population. Such risks include psychological distress to the child and the trade-off for a scar that may actually be more visible than the presenting lesion itself. Decisions about surgery should be made by carefully weighing risks and benefits. The nature of the lesion, age of the child, and cognitive and verbal abilities of the child, which may enable the child to participate in the decision-making process, are all factors that need to be taken into account when managing skin and soft-tissue lesions. This chapter will focus on common melanocytic lesions, common non-melanocytic lesions, common types of cutaneous trauma, and cutaneous scars and scar management.

Melanocytic Lesions Congenital Melanocytic Nevi Congenital melanocytic nevi (CMN) are pigmented lesions that are typically apparent at birth and are commonly labeled as birthmarks. They are present in approximately 1 in 100 newborns and vary in color and size. With respect to color, CMN vary from tan to blue-black. Their surfaces may be irregular in texture, and coarse terminal hairs may be present. These characteristics may evolve over time, with hyperkeratosis, verrucous changes, and coarse hair growth becoming more common near puberty. In terms of size, CMN are classified as small (diameter of ≤ 1.5 cm), medium (diameter of 1.5–19.9 cm), or large and/or giant 165

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(diameter > 20 cm), where the respective size criteria represent the sizes in adulthood. Histologically, CMN differ from standard or acquired nevi because of the presence of nevus cells in the lower two-thirds of the dermis and subcutaneous tissue; extension of nevus cells into adnexal skin structures, including sweat glands; and infiltration of nevus cells in a perivascular and perifollicular distribution, which simulates an inflammatory reaction. Congenital melanocytic nevi occur most commonly on the trunk, followed by the extremities and the head and neck. Giant CMN are frequently accompanied by satellite nevi that may be dispersed over regions that are distant from the central lesion itself. A clinically significant concern with CMN is the risk of malignant transformation. The rates appear to range from 0% to 5% for small to medium CMN to 4.5% to 10.0% for giant CMN. It has been noted that 50% of cases of melanoma with giant CMN arise in the first 3 years after birth, and 70% of cases arise by age 13 years. These findings have reinforced the decision to begin surgical excision and reconstruction of larger lesions during early childhood. Malignant change in larger lesions may be difficult to diagnose clinically but can be signaled by focal growth, ulceration, tenderness, dark pigmentation, pruritus, pain, or bleeding. Microscopically, dysplasia of nevus cells, pagetoid nevus cell spread, proliferative dermal nodules, high mitotic rate, and highgrade atypia may be found with malignant transformation. The term pagetoid refers to cells that demonstrate an upward spreading of abnormal cells in the epidermis (ie, from the basement membrane to the surface). It is uncommon and a possible indication of a premalignant or malignant condition. Independent of malignant transformation, CMN may be associated with central nervous system involvement in what has been referred to as neurocutaneous melanosis. This appears to result from an embryological error in which dysregulated proliferation and migration of melanoblasts leads to a collection of ectopic melanocytes in the brain or spinal cord. Risk factors of neurocutaneous melanosis include multiple satellite nevi and CMN of the trunk and skull in a midline location. Patients may present in infancy with increased intracranial pressure, hydrocephalus, or developmental delay. Alternatively, patients may present in the second or third decades with increased intracranial pressure or spinal cord compression. Because some patients may have no clinical symptoms of neurocutaneous melanosis, baseline contrast-enhanced magnetic resonance imaging (MRI) of the entire central nervous system, to include the head as well as the cervical, thoracic, and lumbar spine, should be performed when risk factors are present. Such patients can be followed up serially with MRI to rule out malignant changes. Malignant potential, as well as symptoms such as pruritus and ulceration, make CMN candidates for surgical excision in early childhood.

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Because non-excisional methods such as lasers and peels can leave deep nevus cells in place and make it difficult to monitor them for malignant change, surgery is the preferred modality of treatment. Small lesions can be excised in a single stage or in multiple stages. Medium-sized lesions can be excised with local tissue rearrangement or with grafting, as needed. Giant CMN (Figure 12-1) may pose great reconstructive challenges, and patients are best referred early to pediatric plastic surgeons for counseling and treatment planning. Tissue expansion with subsequent expanded full-thickness graft coverage, expanded local flap coverage, or expanded free-flap coverage has proven successful for larger CMN of the trunk, extremities, scalp, and face. Staged reconstruction is often commenced as early as 6 months of age.

Café au lait Spots Café au lait spots are well-circumscribed pigmented macules or patches that range in color from light brown (the color of “coffee with milk”) to dark brown in color. Café au lait spots can be anywhere from 2 mm to 20 cm in greatest diameter. While solitary spots are commonly recognized as birthmarks in approximately 2% to 3% of newborns, multiple café au lait spots are less common, particularly in the white population. Although most café au lait spots are present at the time of birth, some may become apparent later, within the first few years after birth. Most occur on the torso, buttocks, and lower extremities, although they may be

Figure 12-1. Giant congenital melanocytic nevi of the left flank/buttocks region requiring tissue expansion and flap reconstruction.

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found anywhere on the body. Histologically, café au lait spots show an increase in the melanin content of melanocytes and basal keratinocytes. Giant melanosomes (macromelanosomes) may also be seen. No significant risk of malignant melanoma has been described. The presence of multiple café au lait spots in a child should raise suspicion for a possible syndromic association. Neurofibromatosis 1 (NF1) is the most common and widely recognized syndrome associated with café au lait spots. In contrast to sporadic café au lait spots, those found in patients with NF1 may develop later in childhood and adulthood and do not tend to fade with time. Six or more must be present to serve as a diagnostic criterion. Macules in NF1 have been described as having a smooth “coast of California” appearance, which is different from the jagged “coast of Maine” appearance that may be seen with another commonly associated syndrome, Albright syndrome. In general, café au lait spots require no treatment. Laser treatment may be beneficial for cosmetically sensitive areas, such as the face. Large and deeply pigmented lesions may occasionally warrant surgical excision and reconstruction in a similar fashion to giant CMN.

Nevus Spilus (Speckled Lentiginous Nevus) This lesion appears similar to a café au lait spot but contains darkly pigmented areas within it that resemble CMN. Nevus spilus is, therefore, considered to be part of the spectrum of CMN. These lesions are common, with an overall prevalence of 1% to 2% in the neonatal period. Speckled areas may continue to evolve during childhood. While these lesions generally occur in isolation, they can, in rare cases, be associated with syndromes. Speckled lentiginous nevus syndrome refers to a clinical entity characterized by a large nevus spilus, hyperhidrosis, and sensory and motor neurological abnormalities. Malignant transformation has been noted in nevus spilus, but the risk of this appears to be very low. Suspicious areas should be biopsied. Lesions may otherwise be excised in a fashion similar to CMN when symptomatic or when aesthetic concerns predominate. With respect to the latter, darker areas may be specifically targeted, while lighter areas are left untreated. Slate Gray Nevus (Mongolian Spot) Slate gray nevi are blue-gray patches typically located over the lumbosacral region that are present at birth or manifest within the first few weeks after birth. They tend to be most prominent at 1 year of age and regress thereafter, with most disappearing by 3 to 4 years of age. Slate gray nevi result from an increased number of melanocytes in the deep dermis and are most commonly seen in babies of Asian and African descent. Malignant transformation has not been reported, and no surgical treatment is required for these lesions.

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Nevi of Ota and Ito Nevi of Ota and Ito lesions consist of blue-gray to brown macules in the distribution of the first and second branches of the trigeminal nerve (fifth cranial nerve) (nevus of Ota) or along the shoulder region (nevus of Ito). Pigmentation results from aberrant migration of melanocytes during embryological development. The nevi are predominantly found in those of Asian descent and are typically unilateral, although bilateral presentations may be seen in a minority of cases. Although present at birth, the lesions may darken with development and do not resolve on their own. Laser therapy has been most commonly used for treatment. Spitz Nevus (Spindle Cell Nevus) The Spitz nevus lesion is named after the American pathologist Sophie Spitz. Spitz nevi typically present as rapidly growing, well-circumscribed nodules that range from pink to dark brown in color. They are found most commonly on the head and neck in children and range in size from just a few millimeters to 2 cm (Figure 12-2). Histologically, the lesions are composed of melanocytes with patterns of atypia that can often be difficult to distinguish from melanoma. Complete surgical excision is indicated. In cases in which melanoma cannot be ruled out, wide local excision with possible sentinel lymph node biopsy may be pursued as for melanoma treatment. Acanthosis Nigricans Acanthosis nigricans describes areas of dark discoloration and velvety consistency of the skin within the groin and/or axilla. It typically occurs in children with obesity or diabetes. Those who develop the condition are at higher risk of developing type 2 diabetes. Rarely, it can be a harbinger

Figure 12-2. A Spitz nevus lesion on the right cheek.

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of a malignant tumor elsewhere in the body, such as the stomach or liver. Confirmation of the diagnosis can be achieved with a skin biopsy. No specific treatment exists; however, some modalities, such as laser therapy, may restore some of the normal color and texture to affected areas.

Non-melanocytic Lesions Nevus Sebaceus Nevus sebaceus, first described in 1895 by Jadassohn, is a common, well-circumscribed round or oval pink-yellow to orange smooth plaque that is most often located on the scalp, face, or neck (Figures 12-3 and 12-4). The scalp is involved in 90% of cases and appears as a patch of alopecia. Histologically, nevus sebaceus is a hamartoma of skin adnexal structures, including sebaceous and apocrine glands. During puberty, these lesions can become thicker and more verrucous. Although most lesions are isolated, in 7% of cases, the nevus sebaceus may be more extensive and associated with neurological, ocular, and skeletal defects (sebaceous nevus [Schimmelpenning] syndrome). Secondary neoplasms may develop in as many as 25% of nevus sebaceus lesions. Most are benign, including trichoblastoma and syringocystadenoma papilliferum. The basaloid proliferation seen with benign trichoblastoma may be difficult to distinguish from basal cell carcinoma and may account

Figure 12-3. A nevus sebaceus lesion on the scalp.

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Figure 12-4. A nevus sebaceus lesion on the scalp.

for the high (10%) rate of association with basal cell carcinoma in early reports. Current literature suggests that the true incidence of basal cell carcinoma arising from nevus sebaceus is closer to 1%. Nonetheless, surgical excision remains the standard treatment. The goals of surgery are not only to minimize neoplastic risk but also to obviate any irritation associated with the verrucous nature of the lesion and to improve overall cosmesis.

Epidermal Nevus As with the nevus sebaceus, epidermal nevi result from somatic mutations during neuroectodermal patterning in embryological development. Unlike the nevus sebaceus, however, keratinocyte epidermal nevi are less often found on the head and neck as a solitary plaque and are not associated with neoplasia. Variants of epidermal nevi include the verrucous epidermal nevus, inflammatory linear verrucous epidermal nevus (ILVEN), nevus comedonicus, and Becker nevus. Like nevus sebaceus, these lesions may also be associated with systemic syndromes characterized by neurological, musculoskeletal, cardiovascular, renal, and endocrine abnormalities. Aesthetic concerns, as well as irritation, often prompt families to seek treatment for children with epidermal nevi. Pruritus, in particular, can be a debilitating symptom with ILVEN, which is often located on the lower extremities, buttocks, and perineum. Laser therapies, cryotherapies, and

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surgical excision have all been used for treatment. Extensive symptomatic lesions may require excision and reconstruction with techniques similar to those commonly used for CMN.

Pilomatricoma Pilomatricoma is the most common acquired cyst in children. It typically manifests as a firm mass within the skin of the face, often with an overlying bluish or reddish hue. Periorbital, lateral cheek, and preauricular sites are most common for this benign lesion derived from the hair follicle (Figure 12-5). Children may present with superinfected lesions. Ultrasonography (US) is an excellent first-line imaging study that can help differentiate pilomatricoma from other skin lesions. Excision is recommended, given the low likelihood of complete spontaneous regression and the potential for further infectious complications. Dermoid Cyst Dermoid cysts are commonly encountered lesions in the pediatric population that arise from the trapping of ectodermal elements along embryonic lines of fusion. Histologically, they are seen as hamartomas of ectodermal elements that may include skin, hair follicles, sweat glands, and sebaceous glands, all contained within a cyst lined by squamous epithelium. Although congenital dermoid inclusion cysts may be seen in the midline over the spine and within the abdomen, they are more commonly observed in the head and neck. In the brow region, dermoid cysts are commonly found fixed to the orbital rim periosteum at the frontozygomatic suture (so-called external angular dermoids) (Figure 12-6). Magnetic resonance imaging is best for identifying dermoid cysts on the basis of imaging characteristics. These lesions rarely

Figure 12-5. Pilomatricoma.

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Figure 12-6. A dermoid cyst under the brow, on the orbital rim.

have deep extensions and can be excised via aesthetically pleasing incisions in an upper eyelid crease or immediately under the eyebrow. Similar approaches can be used for other dermoid cysts of the orbital region, which likewise rarely have deep extensions. Dermoids of the frontonasal or glabellar region pose a significantly greater challenge with respect to management. These lesions, which constitute approximately 4% to 12% of dermoids of the head and neck, are at the highest risk for intracranial and intradural extension. Intracranial extension may be associated with clinically significant neurological sequelae, including papilledema, seizures, and infection. Frontonasal dermoids are typically seen as a mass or punctum within the first 2 months after birth. Fine hair growth and the release of sebaceous material may also be apparent. Ultrasonography is suggested as a first-line imaging study. The appearance of dermoid or epidermoid cysts at US is slightly variable on the basis of internal content; however, US can allow easy differentiation of these lesions from other pathological findings. Isolated glabellar masses at or above the frontonasal suture tend to not have deep extension and may be addressed via direct local excision. On the other hand, lesions that manifest on the nasal dorsum or tip as puncta, with or without an associated mass, have a risk of intracranial extension as high as 45%. As a consequence, any nasal lesions in the midline with features of a dermoid should be imaged with contrastenhanced MRI of the face and brain (Figure 12-7). Lesions with intracranial extension typically course through a patent foramen cecum and a bifid crista galli toward the falx cerebri and anterior cranial base. Complete resection often requires a combined external nasal approach in conjunction with bifrontal craniotomy. Intraoperative frozen-section biopsy may be used for sinus tracts that extend through the foramen cecum to determine the

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Figure 12-7. A nasal dermoid cyst (arrow) depicted on an axial magnetic resonance image of the brain.

presence of epithelial elements; simple fibrous remnants may be left, while deeply rooted cysts require craniotomy to prevent recurrence. Dermoid cysts of the frontonasal region are part of a wider spectrum of anomalies that involve heterotopic neuroectodermal tissue that also encompasses nasal gliomas and encephaloceles. This further reinforces the recommendation for preoperative imaging for suspicious midline nasal masses in infancy. Gliomas represent retained glial tissue and may manifest as intranasal or extra-nasal masses. Encephaloceles represent herniated meningeal and/or parenchymal tissue, in addition to glial tissue. Both lesions require neurosurgical consultation and, typically, craniotomy, followed by lesion excision and bony reconstruction.

Neurofibroma Neurofibromas are benign nerve sheath tumors that are composed of Schwann cells, fibroblasts, mast cells, nerve axons, and perineural cells. Although not specific for NF1, they are a cardinal feature of the disease. Cutaneous neurofibromas are characterized by rubbery, exophytic papules that typically appear during adolescence and increase in number with age. Subcutaneous neurofibromas, which are well assessed with contrast-enhanced MRI of the affected region when necessary, are palpable as rubbery masses beneath the skin and may be painful. Malignant transformation of cutaneous or subcutaneous lesions is uncommon. Plexiform neurofibromas are larger nodular tumors that develop along nerves and have a “bag of worms” texture. These lesions are present in up to 25% of patients with NF1 and may cause significant pain and disfigurement. Moreover, they are associated with the development of malignant peripheral nerve sheath tumors, which have a

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poor prognosis. Simple surgical excision can be performed for cutaneous and subcutaneous neurofibromas, whereas plexiform lesions tend to be more problematic. Intimate involvement of plexiform lesions with major nerve trunks can make resection challenging and morbid. The main goals of resection are to restore function, relieve pain, and improve cosmesis. Newer molecular targeted therapies for plexiform neurofibromas are being explored to avoid disfiguring and highly morbid surgical procedures.

Tuberous Sclerosis Complex Tuberous sclerosis complex is an uncommon genetic disorder that produces benign tumors throughout the body—notably, the brain, eyes, kidneys, heart, lungs, and skin. About two-thirds of patients have a new mutation in the TSC1 or TSC2 gene. The remaining one-third have inherited an altered version of either gene. Most patients demonstrate patches of light-colored skin or small areas of thickened, smooth skin or reddish bumps under or around the nails. Facial growths that begin in childhood and resemble acne are also common. Concomitant issues include seizures, behavioral or cognitive problems, kidney problems, heart problems, lung problems, and eye abnormalities. In addition to genetic tests, imaging studies such as MRI, computed tomography, and/or US may be indicated to evaluate one or more organ systems. Management may include one or more complimentary modalities, including medicine, surgery, and/or behavioral management. Antiseizure medications may be indicated to control seizure activity, while anti-arrhythmic medications may be indicated for cardiac problems. Brain lesions not amenable to resection may respond to everolimus (trade names Afinitor [Novartis, Deerfield, IL] or Zortress [Novartis]). The topical ointment form sirolimus may help treat acne-like skin growths. Surgical resection is indicated if a particular lesion affects the ability of an organ to function. Dermabrasion or laser therapy may improve the appearance of skin lesions. The latter can be treated by a dermatologist by using topical molecular targeted therapies, thereby avoiding surgical procedures that can lead to scarring. Noonan Syndrome Noonan syndrome is an autosomally inherited genetic disorder that causes abnormal development in various parts of the body, including the skin. The skin may appear thin and transparent with age. The signs and symptoms of Noonan syndrome vary greatly among individuals and may be mild to severe. Characteristics may be related to the specific gene that contains the mutation. The appearance of the face is characteristic of Noonan syndrome. The forehead may be prominent, with a low posterior hairline. The eyes tend to be wide set and down slanting, with droopy lids. The irises are pale blue or green. The ears are similarly low set and rotated posteriorly. The nose tends to have a wide base, a bulbous tip, and a flattened dorsum. These

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features may be more pronounced in infants and young children but change with age. More importantly, children with Noonan syndrome have cardiac anomalies such as valvular defects, hypertrophic myopathy, and/or septal defects and arrhythmias. Patients have a mild but wide range of mental, emotional, behavioral, and learning issues. Treatment largely addresses the cardiac and learning issues.

Cutaneous Trauma Lacerations As with any laceration in the adult trauma patient, soft-tissue injuries in the child should be documented in the context of a standard trauma assessment that includes attention to the ABCs (airway, breathing, circulation) as a matter of first priority. In children, lacerations are frequently found on the head and neck, owing to their disproportionately large size during periods of rapid brain growth. Underlying bony fractures are less common than in adults because of the relatively elastic nature of the developing facial bones; therefore, skin and soft-tissue injuries predominate. Although the principles of soft-tissue repair do not differ fundamentally from those in adult patients, special consideration must be given to 2 issues in children: appropriate sedation and anesthesia, and future growth and scar development. Regarding anesthesia and sedation, it should be recognized that soft-tissue injuries that would otherwise require simple repair in the emergency department for adult trauma patients may demand considerable efforts for the pediatric patient. Complex lacerations, particularly those of the periorbital region or those for which patient cooperation is paramount, will likely require more than simple infiltration with local anesthetic. Popular options for anesthesia and sedation in the pediatric emergency department can be categorized according to the level of invasiveness and include the following: • Simple local anesthetic injection (eg, lidocaine 0.5%–1.0% or bupivacaine 0.25% with 1:100,000–1:200,000 epinephrine), with or without preapplication of topical skin anesthetic creams (eg, lidocaine-epinephrine-tetracaine or lidocaine-prilocaine) • Intranasal or oral midazolam for anxiolysis, in addition to local anesthetic injection • Intranasal or oral midazolam for anxiolysis, insertion of an intravenous (IV) line, and IV ketamine administered as a sedative with appropriate cardiorespiratory monitoring In general, school-aged children (6–12 years of age) with low-complexity lacerations are treated by using the first option of simple local anesthesia, whereas neonates and infants (from birth to 1 year) and preschoolers (2–5 years of age) are treated by using the second or third paradigms, depending on the complexity of the repair. Intranasal midazolam has become a popular option in many cases, as this obviates placement of an IV line and can often quell patient

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anxiety to a sufficient degree to enable irrigation, tetanus prophylaxis, and suture repair of most lacerations. It should be emphasized that soft-tissue injuries that require detailed exploration and major repair, including facial nerve injuries, parotid duct injuries, and lacrimal system injuries, should be addressed in the operating room with general anesthesia, where conditions are optimal. An additional important concern in children with soft-tissue injuries of the head and neck is the issue of potential growth disturbances and scar formation, resulting from the trauma itself or from attempts at repair. On the scalp, for example, care must be taken to minimize disturbance to the hair growth pattern. For the ear, attempts at primary layered closure or closure with local advancement flaps may produce shortening of the auricle, and parents must be counseled about the possibility of persistent visible asymmetries in size. With regard to scar formation, it should be noted that children often heal with exuberant hypertrophic scars that continue to fade and flatten over time (see the next section for more details). Parents should be counseled about the inevitability of scarring, despite meticulous attempts at soft-tissue repair. The possibility of reduced inflammation and scarring with the use of nonabsorbable sutures must be balanced with the need for subsequent suture removal, which may be difficult or impossible to achieve with young children in the office setting. Octyl cyanoacrylate glues or fast-absorbing catgut sutures for the skin layer are popular alternatives that have not been shown to produce inferior scars in the laceration literature.

Bite Wounds It has been estimated that 50% of people in the United States will, at some point during their lifetimes, experience an animal or human bite wound. Dogs are, by far, responsible for most bite wounds and account for an estimated 80% to 90% of bites that require medical attention. Children have been estimated to sustain serious dog bites that require medical attention 3 times more often than adults. The prevalence of facial dog bites in infants and toddlers predominantly reflects their stature, as their faces are often at eye level with the dog and are easily accessible on provocation. Bites are most common in a central target area that includes the nose, cheeks, and lips (Figure 12-8). Biting dogs are typically family or neighborhood pets that are known to the victims. Pit bulls, rottweilers, and German shepherds are the breeds responsible for most documented bites. In addition to dog bites, cat and human bites may result in soft-tissue trauma to the head and neck. Cat bites are the second most common bites, accounting for approximately 5% to 15% of animal bite wounds. When compared with dog bites, cat bites are believed to carry a higher risk of wound infection, on the order of 30% to 50%. This may stem from the long, slender teeth of cats, which allow deep inoculation into the tissues, or from the unique oral flora in cats. Human bites are less common than animal bites, but they may have the highest rates of infection of all bite wounds.

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Figure 12-8. Bite wounds on the central target area of the face in a pediatric patient, which includes the nose, cheeks, and lips. Dog bites commonly occur in this area because a small child’s face is often at eye level with dogs.

Management of bite wounds involves the same principles discussed earlier for cutaneous trauma, albeit with an increased vigilance for postoperative infection owing to the contaminated nature of the wounds. The risk of infection may be overestimated, however, for bites to the head and neck where vascularity is excellent. Although prospective data are lacking, general consensus suggests that bite wounds to the head and neck are appropriately managed with primary closure within 24 hours of injury, provided there are no signs of acute infection such as cellulitis, purulence, or fever. Wounds should be thoroughly irrigated with sterile saline under pressure and then repaired with as few deep sutures as necessary to reduce tension, followed by fine, simple, interrupted sutures for the skin to allow for potential drainage. The use of prophylactic antibiotics for bite wounds is controversial but may be considered for deep-tissue injuries with exposed structures, such as cartilage, wounds closed after 6 to 12 hours, cat and human bites, and patients with immunosuppressive comorbidities. Prophylactic antibiotics or empirical antibiotics administered in the case of wound infection should be directed at the common causative organisms, which include Pasteurella, Streptococcus, Staphylococcus, and anaerobes for cat and dog bites and Eikenella, Streptococcus, Staphylococcus, and anaer­ obes for human bites. Amoxicillin/clavulanate (or IV ampicillin/sulbactam) is the most suitable single agent to cover these pathogens. In children who are allergic to penicillin, clindamycin with trimethoprim/sulfamethoxazole is an alternative. Treatment duration is generally 5 to 7 days for prophylaxis and 10 to 14 days for empirical therapy. Tetanus prophylaxis is recommended if the patient has had fewer than 3 doses of tetanus toxoid or if more than 5 years

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have elapsed since the last vaccination. In the case of dog bites, rabies prophylaxis is administered if the offending dog was seen to manifest signs of disease at the time of the bite or if any dog placed into a 10-day quarantine period begins to manifest such signs. For bites from stray dogs for which vaccination status and/or behavior is unknown, the local or state public health department should be consulted.

Cutaneous Scars and Scar Management Hypertrophic Scars and Keloids Hypertrophic scars are exuberant scars that remain within the boundaries of the original wound, whereas keloids classically extend beyond these boundaries. Both occur with greater frequency in children and may form in response to traumatic skin injuries or surgery. Hypertrophic scars tend to regress with time in children, and counseling of parents is indicated before discussing any plans for revisional surgery. Six to 12 months should typically be allowed for red, raised hypertrophic scars to remodel. Different from hypertrophic scars, keloids are characterized by their increased fibro­ blastic activity and overproduction of collagen. These lesions occur more frequently in individuals with darkly pigmented skin (Figure 12-9). They may be symptomatic, with irritating burning and itching that may stem from high histamine content.

Figure 12-9. A keloid lesion is shown. Keloids occur more frequently in patients with darkly pigmented skin.

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Prevention and Treatment In general, rapid wound closure, adequate debridement of any devitalized tissues, and provision of a moist environment promote optimal healing and help to minimize scar hypertrophy. Reduction of wound tension is also paramount, given the signal transduction capabilities of fibroblasts and the resultant stimulatory effect on collagen production. Recent work has shown that epithelial hydration is an additionally important factor in minimizing activation of dermal fibroblasts. The ability of silicone products to reduce transepidermal water loss is thought to be a key mechanism underlying their reported effectiveness in minimizing and/or treating hypertrophic scarring. New silicone gels have been shown to have effectiveness equivalent to that of more traditional silicone sheets and have the advantage of easy application for pediatric patients. A variety of such products are currently available. These are typically applied several times daily to newly healed incisions, and this is continued for 3 months (or, in the case of existing hypertrophic scars, until improvement is observed). Silicone appears to be the only topical agent to date with evidence-based support for its effectiveness in scar modulation. Other commonly recommended creams and ointments (many of which contain onion extract, such as Mederma [Merz North America, Raleigh, NC], or vitamin E derivatives) have not been conclusively shown to reduce postoperative scars. Scars that have already begun to hypertrophy, as well as incipient keloids, may be treated with other modalities. Triamcinolone acetonide is the most commonly used corticosteroid. In children, this must be administered with caution owing to the potential for adrenal suppression and depigmentation and atrophy of the surrounding skin. Furthermore, injections are painful and may require sedation in the operating room. Manual massage is commonly recommended for scar treatment, but there is little clinical evidence to support its widespread use. Radiation therapy is often suggested to halt the progression of keloids, but the side effects in growing children generally make for an unacceptable risk-benefit ratio. Finally, surgical re-excision of scars can be considered after full maturation at 6 to 12 months. Because surgery alone is unlikely to be of benefit for keloids, given the high recurrence rate, re-excision should be combined with other modalities of treatment, including the application of pressurized garments and/or intralesional injection. SELECTED REFERENCES

1. Ambro BT, Wright RJ, Heffelfinger RN. Management of bite wounds in the head and neck. Facial Plast Surg. 2010;26(6):456–463 PMID: 21086232 https://doi.org/10.1055/s-0030-1267720 2. Arneja JS, Gosain AK. Giant congenital melanocytic nevi. Plast Reconstr Surg. 2009;124(1) (suppl):1e–13e PMID: 19568135 https://doi.org/10.1097/PRS.0b013e3181ab11be 3. Aslam A, Salam A, Griffiths CE, McGrath JA. Naevus sebaceus: a mosaic RASopathy. Clin Exp Dermatol. 2014;39(1):1–6 PMID: 24341474 https://doi.org/10.1111/ced.12209 4. Bartlett SP, Lin KY, Grossman R, Katowitz J. The surgical management of orbitofacial dermoids in the pediatric patient. Plast Reconstr Surg. 1993;91(7):1208–1215 PMID: 8497520 https://doi. org/10.1097/00006534-199306000-00005

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181 Chapter 12: Skin and Soft-Tissue Lesions 5. Benjamin LT. Birthmarks of medical significance in the neonate. Semin Perinatol. 2013;37(1): 16–19 PMID: 23419758 https://doi.org/10.1053/j.semperi.2012.11.007 6. Burstein FD, Bauer BS. Congenital and acquired deformities of the nose. In: Bentz ML, Bauer BS, Zuker RM, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing, Inc; 2008:897–944 7. Canavese F, Krajbich JI. Resection of plexiform neurofibromas in children with neurofibromatosis type 1. J Pediatr Orthop. 2011;31(3):303–311 PMID: 21415691 https://doi.org/10.1097/BPO. 0b013e31820cad77 8. Cohen IK. Pediatric and fetal wound healing. In: Bentz ML, Bauer BS, Zuker RM, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing, Inc; 2008:73–82 9. Corcoran J, Bauer BS. Cutaneous lesions in children. In: Bentz ML, Bauer BS, Zuker RM, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: Quality Medical Publishing, Inc; 2008:83–104 10. Dombi E, Baldwin A, Marcus LJ, et al. Activity of selumetinib in neurofibromatosis type 1-related plexiform neurofibromas. N Engl J Med. 2016;375(26):2550–2650 PMID: 28029918 https://doi.org/ 10.1056/NEJMoa1605943 11. Gupta D, Thappa DM. Mongolian spots—a prospective study. Pediatr Dermatol. 2013;30(6): 683–688 PMID: 23834326 https://doi.org/10.1111/pde.12191 12. Guy RJ, Zook EG. Successful treatment of acute head and neck dog bite wounds without antibiotics. Ann Plast Surg. 1986;17(1):45–48 PMID: 3078618 https://doi.org/10.1097/00000637198607000-00009 13. Hogg NJ. Primary and secondary management of pediatric soft tissue injuries. Oral Maxillofac Surg Clin North Am. 2012;24(3):365–375 PMID: 22695255 https://doi.org/10.1016/j.coms.2012. 04.007 14. Holger JS, Wandersee SC, Hale DB. Cosmetic outcomes of facial lacerations repaired with tissueadhesive, absorbable, and nonabsorbable sutures. Am J Emerg Med. 2004;22(4):254–257 PMID: 15258862 https://doi.org/10.1016/j.ajem.2004.02.009 15. Kaye AE, Belz JM, Kirschner RE. Pediatric dog bite injuries: a 5-year review of the experience at the Children’s Hospital of Philadelphia. Plast Reconstr Surg. 2009;124(2):551–558 PMID: 19644273 https://doi.org/10.1097/PRS.0b013e3181addad9 16. Leducq S, Giraudeau B, Tavernier E, Maruani A. Topical use of mammalian target of rapamycin inhibitors in dermatology: a systematic review with meta-analysis. J Am Acad Dermatol. 2019; 80(3):735–742 PMID: 30744877 https://doi.org/10.1016/j.jaad.2018.10.070 17. Meaume S, Le Pillouer-Prost A, Richert B, Roseeuw D, Vadoud J. Management of scars: updated practical guidelines and use of silicones. Eur J Dermatol. 2014;24(4):435–443 PMID: 25141160 https://doi.org/10.1684/ejd.2014.2356 18. Morganroth P, Wilmot AC, Miller C. JAAD online. Over-the-counter scar products for postsurgical patients: disparities between online advertised benefits and evidence regarding efficacy. J Am Acad Dermatol. 2009;61(6):e31–e47 PMID: 19846237 https://doi.org/10.1016/j. jaad.2009.02.046 19. Narayan D. Benign and malignant tumors of the skin. In: Guyuron B, Eriksson E, Persing J, eds. Plastic Surgery: Indications and Practice. Philadelphia, PA: W.B. Saunders; 2008 20. Price HN, Zaenglein AL. Diagnosis and management of benign lumps and bumps in childhood. Curr Opin Pediatr. 2007;19(4):420–424 PMID: 17630606 https://doi.org/10.1097/MOP. 0b013e328224b8ee 21. Shah KN. The diagnostic and clinical significance of café-au-lait macules. Pediatr Clin North Am. 2010;57(5):1131–1153 PMID: 20888463 https://doi.org/10.1016/j.pcl.2010.07.002 22. Vasconez HC, Buseman JL, Cunningham LL. Management of facial soft tissue injuries in children. J Craniofac Surg. 2011;22(4):1320–1326 PMID: 21772187 https://doi.org/10.1097/ SCS.0b013e31821c9377

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CHAPTER

13

Breast Anomalies LAURA C. NUZZI, BA, AND BRIAN I. LABOW, MD, FAAP, FACS

Introduction

Benign breast conditions are common in both sexes, arising predominantly during adolescence. They span a spectrum of normal to disordered, with distinctions often blurred and patient specific. Breast concerns during adolescence are prevalent. One study showed that 25% of healthy adolescent girls were unsatisfied with their breast shape, size, or appearance.1 Thus, the pediatrician must be skilled in the nuances of breast presentation and diagnosis, parsing typical findings from abnormal variants. Pediatric breast disorders encompass congenital and acquired conditions. They can be deformational; hyperplastic, which results in excessive tissue; or hypoplastic, owing to breast insufficiency. Disorders may also present unilaterally or bilaterally. Although many adolescent breast conditions self-resolve, they may cause considerable physical and psychological distress.1–3 As such, early diagnosis and management of these conditions are crucial. In this chapter, we highlight the breast anomalies frequently encountered by the pediatrician: macromastia, gynecomastia, breast asymmetry, and fibroadenoma. Although these conditions vary in presentation, diagnosis, and management, all may benefit from early referral and evaluation by a plastic surgeon with expertise in the adolescent breast.

Macromastia Introduction Macromastia, the benign overgrowth of the breast, including the glandular and adipose portions of one or both breasts, is the most common hyperplastic breast anomaly that affects adolescent girls.2 The physical burden of macromastia is well established, with demonstrated breast, back, shoulder, and neck pain in most affected adolescents. Patients often indicate impairment with exercise and participating in sports, as well as with finding properly fitted clothing and bras. Macromastia is also known for its negative effect on psychosocial well-being, specifically diminished mental health, social functioning, emotional well-being, and self-esteem, as well as an increase in disordered eating behaviors.2 183

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Epidemiology Treatment of macromastia remains one of the most common breast procedures performed by plastic surgeons in young women. Roughly 80% of adults with the breast condition were symptomatic during adolescence.4 The incidence of macromastia continues to increase and is hypothesized to be caused in part by increasing childhood obesity trends, precocious puberty, and xenoestrogens. Patient Presentation Patients with macromastia have breasts that are disproportionately large for their body (Figure 13-1). The breasts may appear ptotic (with the nipple position below the inframammary fold) with striae due to rapid growth. Patients may also present to the clinic with painful bra strap grooving of the shoulders, musculoskeletal pain, and inframammary fold intertrigo (Figure 13-2). Macromastia is also strongly associated with obesity, with an estimated two-thirds of affected adolescents having overweight or obesity.2 Diagnosis Patients suspected of having macromastia should undergo physical examination, and a complete history should be compiled. Patient-reported musculoskeletal pain and degree of physical impairment should be documented. Mental health, self-esteem, and disordered eating thoughts and behaviors should be assessed and addressed, if necessary. The physician should also evaluate the patient’s skeletal and psychological maturity before considering treatment. A formal diagnosis of macromastia can also be established by using the Schnur sliding scale,5 which is used by many insurers to determine A

B

Figure 13-1. A, Severe bilateral adolescent macromastia in an 18-yearold with an H cup size. The patient underwent reduction mammaplasty, with 4,530 total grams removed. B, Two-and-a-half years after reduction mammaplasty, the same patient has a D cup size.

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Figure 13-2. Intertrigo in the inframammary fold, with Candida infection.

the minimum amount of breast tissue necessary for resection during reduction surgery, by using the patient’s calculated body surface area.

Management Currently, no standardized treatment guidelines exist for adolescent macromastia. Many plastic surgeons opt to delay reduction mammaplasty owing to concerns of breast regrowth and repeat surgery, as well as the emotional effect of breast surgery on an adolescent. Many surgeons and some thirdparty payers require weight loss or physical therapy before covering the costs of surgical intervention. However, a growing body of data suggests that early surgical intervention may improve the associated psychosocial impairments in adolescents and that age and weight status at surgery may not negatively affect positive surgical outcomes.2,4 Associated obesity and psychosocial impairment may require nutritional and mental health support. Reduction mammaplasty is a safe procedure that requires a general anesthetic and may be performed on an outpatient basis or with a single-night hospital stay. Redundant skin and excess glandular and adipose tissue are resected, with the breast reshaped and the nipple-areola complex repositioned and resized. Patients are out of school or work for roughly 1 week and are allowed to resume full activity at 6 weeks. The musculoskeletal symptoms are improved almost immediately, with breast appearance continuing to improve for several months. Lateral breast sensation normalizes around 6 months, and scar maturation may take 1 to 2 years to achieve optimal appearance. Complications Early surgical complications are frequent but minor. Major complications, such as hematoma that necessitates surgical evacuation, nipple and skin necrosis, or wound infection that necessitates oral antibiotics and drainage,

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are extremely rare. However, minor wound complications, such as small wound separations, exposed suture, and minor skin irritation, are more common. Late complications, such as hypertrophic scarring or keloids, areola or breast asymmetry, or altered breast and nipple sensation, can also occur.6 Reduction mammaplasty during adolescence may hinder some women’s ability to lactate or produce sufficient milk volume in the future.7 It is important that the adolescent and parent or guardian have a sufficient understanding of the potential effect of this surgery on future function of the breast, including lactation. A recent systematic review demonstrated that surgical techniques that preserve the column of subareolar parenchyma appear to have a greater likelihood of successful breastfeeding. Preservation increased the success of breastfeeding from 4% to 75% for techniques with partial preservation and 100% for techniques with full preservation. Although many postoperative patients have had success with breastfeeding, outcomes data are largely limited and inconclusive. One frequently quoted source suggests a 65% success rate.8

Gynecomastia Introduction Adolescent gynecomastia is the excess of glandular breast tissue in boys. Gynecomastia is extremely common, particularly during early puberty. By age 14 years, up to 69% of boys will have some degree of gynecomastia, with a complete resolution of symptoms within 3 years after first onset in 92% of affected patients.9 The range of severity is broad, encompassing a puffyappearing nipple-areola complex to marked breast enlargement and ptosis. Similar to macromastia, gynecomastia has been found to cause significant psychosocial distress in young men. Boys with gynecomastia have been found to have diminished mental health and self-esteem, regardless of severity.3 Epidemiology Gynecomastia is highly associated with obesity. An estimated two-thirds of boys with gynecomastia are overweight or obese.3 Analogous to macromastia, the incidence of gynecomastia is thought to be increasing because of increasing obesity rates and xenoestrogens. Gynecomastia is heritable, with approximately one-fifth of patients having a positive family history.10 Around 20% of gynecomastia is caused by medications or exogenous chemicals. Common agents include those that bind to the estrogen receptor (eg, digitalis and marijuana), those that stimulate estrogen synthesis (eg, growth hormone), estrogen precursors (eg, androstenedione), those that cause testicular damage (eg, ethanol), those that block testosterone synthesis (eg, spironolactone), and those that block androgen action (eg, finasteride, cimetidine, and ranitidine). Numerous others act to produce gynecomastia via uncertain mechanisms.11

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Patient Presentation True gynecomastia refers to glandular hypertrophy, as opposed to pseudogynecomastia, where most of the breast mound is secondary to excessive adipose tissue. Both elements are present in all patients to varying degrees, and additional peripheral adipose tissue and aromatase can stimulate glandular hypertrophy in patients who have overweight and obesity.8 Regardless, it is usually the size of the breast mound, rather than its relative composition, that is concerning to patients. Gynecomastia is bilateral in most cases, although asymmetry can be seen in roughly one-third of patients.8,10 Diminished self-esteem and mental health also commonly accompany gynecomastia.3 Gynecomastia is described by using a graded scale (Figure 13-3).12 Grade I gynecomastia is the mildest, with minimal breast hypertrophy without ptosis, while grade II is defined by moderate hypertrophy without ptosis. Grade III gynecomastia involves severe hypertrophy with skin excess and is marked by mild ptosis. Grade IV is the most severe, with considerable hypertrophy, skin excess, and moderate or severe ptosis. Diagnosis Patients who present with gynecomastia should undergo a complete history and physical examination. Pubertal physiological gynecomastia, which is absent of any risk factors or findings at physical examination (eg, a testicular mass), can be followed without additional studies. In particular, environmental risk factors should be specifically ruled out. Historically, marijuana and alcohol have been associated with gynecomastia. However, the use of over-the-counter medications such as H2 blockers, as well as functional foods (nutraceuticals) to improve strength or athletic performance, should also be discussed. Last, a long list of prescription medications has been associated with gynecomastia, as well.9,10 Clinicians can also adversely influence breast growth by iatrogenic prescription of medications associated with gynecomastia. If there is no obvious metabolic, endocrine, neoplastic, or druginduced cause, and if gynecomastia persists or progresses over time or arises later in pubertal development, additional studies are warranted. Screening laboratory studies may include a free testosterone, luteinizing hormone, follicle-stimulating hormone, estradiol, and b-human chorionic gonadotropin level. Low testosterone levels, with or without increased gonadotropin levels, may be indicative of Klinefelter syndrome (46,XXY).13 A karyotype should be obtained in cases in which testicular atrophy or laboratory values are suggestive of hypogonadism. Imaging of the breast is rarely performed, except when a suspicious mass is noted at examination or if required to document glandular excess. Male breast cancer is rare, and idiopathic gynecomastia is not associated with an increased risk.13

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I

II

III

IV

Figure 13-3. Grades of gynecomastia. Grade I, Mild breast hypertrophy without ptosis. Grade II, Moderate breast hypertrophy without ptosis. Grade III, Moderate breast hypertrophy with mild ptosis and skin excess. Grade IV, Severe breast hypertrophy with considerable ptosis and skin excess.

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Management Because gynecomastia is largely self-resolving, most idiopathic cases may be managed with support and reassurance. Patients suspected of having congenital and acquired hormonal causes should be referred to an endocrinologist who has expertise in adolescent patients. Medical treatment of endocrine-related gynecomastia may include antiestrogens or aromatase inhibitors, with androgen use falling out of favor.9,10,13 Nonsurgical management of gynecomastia may also include compression shirts, which are worn under clothing to minimize the appearance and shape of the chest and provide support. Because gynecomastia is associated with obesity and poor mental health and self-esteem, treatment should include nutrition and mental health services, if necessary.3 Patients should be advised that for moderate to severe gynecomastia, weight loss later in puberty may be beneficial for a variety of health reasons, but it will not resolve excess gland. For persistent, progressive, or severe gynecomastia that results in considerable distress, surgical intervention may be considered (Figure 13-4). Management is highly dependent on the grade of gynecomastia and the degree of glandular, adipose, and skin involvement. Mild to moderate gynecomastia may be treated with simple excision, ultrasonography-assisted lipectomy, or a combination of liposuction and mastectomy. In cases involving considerable skin excess, the excision of skin may be advantageous. Except for very mild cases, surgery requires a brief general anesthetic but is an outpatient

A

B

Figure 13-4. A, A 17-year-old patient with grade III adolescent gynecomastia. B, The same patient is shown 3 years after suction lipectomy, bilateral simple mastectomies, and concentric mastopexies.

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procedure. Generally, a compression vest and possibly drains are used for a short postoperative period. Discomfort is mild to moderate, lasting 3 to 5 days after surgery, with most patients resuming full activity in 4 to 6 weeks.

Complications Complications generally concern wound healing. Seroma, hematoma, and infection are much rarer than small areas of wound dehiscence or hypertrophic scarring and minor asymmetry.

Breast Asymmetry Introduction Breast asymmetry is an extremely common finding in young women. An estimated 90% of women will experience differences in breast position, size, and shape over their lifetime. Asymmetry is prevalent during early puberty, typically improving or resolving over the course of adolescence.14 Similar to macromastia and gynecomastia, breast asymmetry can result in considerable psychological distress and diminished self-esteem. A 2014 study showed that even mild breast asymmetry of one cup-size difference can result in lowered psychological well-being and mental health.1 Although most cases of idiopathic breast asymmetry can be managed with sympathetic reassurance, surgical or nonsurgical treatment may be helpful when breast asymmetry persists or causes considerable psychological distress. Breast asymmetry is not a formal diagnosis; it is an umbrella term that encompasses normal and abnormal breast differences (Figure 13-5). Hyperplastic conditions, such as unilateral macromastia, and hypoplastic conditions, such as Poland syndrome, breast hypoplasia, amazia (Figure 13-6), A

B

D

C

E

Figure 13-5. Examples of breast asymmetry. A, Mild breast asymmetry, without deformity (this patient has a 250-mL or 2-cup-size difference). B, Moderate breast asymmetry that meets the Schnur criteria for unilateral macromastia (this patient has a 395-mL or 2-cup-size difference). C, Severe breast asymmetry that meets the Schnur criteria for unilateral macromastia (this patient has a 1,250-mL or 4-cup-size difference). D, Tuberous breast deformity. E, Poland syndrome.

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Figure 13-6. A 19-year-old with amazia, characterized by absence of breast gland with the presence of nipple-areola complex.

amastia (Figure 13-7), or tuberous breast deformity, can result in asymmetry. Patients can also acquire breast asymmetry secondary to breast masses (Figure 13-8) or prior breast surgery or trauma.1,14,15

Epidemiology Most cases of breast asymmetry are idiopathic. Hypoplastic conditions that result in breast asymmetry are extremely rare.16 Poland syndrome, for example, has an incidence of roughly 1 in 100,000 births, with a 2:1 male to female ratio.17 Breast asymmetry is associated with obesity, and a 2014 study

Figure 13-7. Amastia, characterized by the absence of breast gland and nipple-areola complex.

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Figure 13-8. Breast asymmetry secondary to giant fibroadenoma excision (arrow) 4 years prior.

showed that 66% of affected adolescents with varying etiologic origins had overweight or obesity.1

Patient Presentation The presentation of breast asymmetry varies widely. Most affected patients will have normally developed breasts of unequal sizes that range from less than 1 cup-size difference to 4 or more cup-sizes difference. Patients with unilateral macromastia may present with ipsilateral musculoskeletal pain, bra strap grooving, and inframammary intertrigo. Breast asymmetry secondary to hypoplastic breast conditions present with varying degrees of breast insufficiency. Breast hypoplasia and amazia are defined, respectively, by an insufficiency or complete absence of breast tissue, with the presence of the pectoralis muscle and nipple-areola complex.16 Tuberous breast deformity is a developmental anomaly that results in a narrow, constricted breast; a high inframammary fold; and pseudo-herniation of the breast gland through the areola.18 The differences seen in Poland syndrome include partial unilateral absence of the pectoralis muscle, usually with some degree of breast hypoplasia, which ranges from severe to subtle.17 Breast asymmetry due to hypoplasia of one or both sides is often associated with lactation insufficiency or lactation failure of the affected side, and women with this condition should be made aware of this. Diagnosis The diagnosis for patients with a specific etiologic origin (eg, Poland syndrome) can be established at birth via physical examination. In most cases, the cause is idiopathic, and a specific diagnosis may not be established. Less severe forms of asymmetry can manifest during adolescence as the breast develops. Because some degree of asymmetry is common during early thelarche, it is generally advisable to observe these patients over time to determine whether the asymmetry resolves.

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Management Although surgery should be deferred until skeletal and emotional maturity is reached, intervention may help offset the negative psychosocial effects of breast asymmetry. In very young patients or in those who have mild asymmetry, support and reassurance from a primary care physician or a pediatric plastic surgeon can be helpful. When the patient is older or the difference is more severe, patients should be encouraged to take advantage of nonsurgical options, such as bra inserts or breast prostheses. Persistent and psychologically distressing asymmetry can be surgically corrected once the patient is skeletally and emotionally mature. Most patients who seek surgical correction for breast asymmetry opt for augmentation. While placement of a breast implant is a straightforward procedure, patients should be informed of the risks and limitations of implants and the possibility of additional procedures needed later in life. Patients with unilateral macromastia undergo reduction mammaplasty of the affected breast. Correction of tuberous breasts (Figure 13-9) or amastia associated with Poland syndrome may require complex reconstruction that involves the use of tissue expanders or regional or distant flap transfers.14,15,17,18 A

B

Figure 13-9. Tuberous breast deformity shown preoperatively (A) and postoperatively (B).

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Complications Complications vary according to the type of surgical procedure performed. When one breast is reduced in size, the complications are as described earlier. Complications with breast augmentation include bleeding, infection (which may necessitate implant removal), and implant exposure. Other implant-related complications include capsular contracture, visible wrinkling or rippling, displacement, and failure that can necessitate an implant replacement. Patients should be advised that secondary procedures due to augmentation may be required as patients age. At mammography, additional views will be required because the implant can obscure the breast tissue.19 In rare instances, implant-associated lymphomas have been reported, and some federal recommendations have been made for interval radiographic evaluation of the implant over time.20

Fibroadenoma Introduction Palpable masses in the developing breast are common and are typically self-resolving. Although the occurrence of breast malignancy in adolescents is rare (< 0.1% of all breast cancers), the mass may be painful or worrisome to the patient and parent.21 Fibroadenoma, which is defined as a proliferative lesion without atypia,22 is the most common benign breast mass that occurs during adolescence, accounting for 44% to 94% of all biopsies. Although 40% of adolescent occurrences self-resolve, surgical management may be appropriate for large or painful lesions.23 Epidemiology Fibroadenoma typically develops during late adolescence and young adulthood. Between 10% and 25% of affected patients will develop recurrent or multiple fibroadenoma over their lifetime.23 Patients and families should be reassured that the presence of one or more simple fibroadenomas does not increase the risk of future breast malignancy. Albeit small, women with complex fibroadenoma carry a 1.5% to 2.0% increased risk of breast cancer.22 Patient Presentation Fibroadenoma is a rubbery, mobile, and circumscribed breast mass of stromal and glandular tissue (Figure 13-10). It typically measures 2 to 3 cm in diameter but can grow to more than 10 cm. The upper outer quadrant of the breast is most commonly affected. Although fibroadenoma is largely painless, symptomatic adolescents may present with breast pain and tenderness around menses, changes in breast skin appearance, and breast asymmetry secondary to larger or multiple fibroadenoma.22,23 These masses are classified as simple or complex, with the latter exhibiting at least one of the following: calcification, sclerosing adenosis, cystic change, or papillary apocrine hyperplasia.22

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A

C

B

D

E

Figure 13-10. A, Enlarging fibroadenoma of the left breast and giant fibroadenoma of the right breast. B, Right-side giant fibroadenoma measuring 5.7 × 5.8 × 5.0 cm. Note the solid, multinodular, well-circumscribed features. C, Intraoperative incision planning by the breast surgeon and plastic surgeon. Immediate breast reconstruction was planned because of the large size of the right breast mass and the anticipated breast asymmetry after excision. D, Right breast defect post-resection of giant fibroadenoma, involving the upper outer quadrant. E, Postoperative result at 1 year.

Diagnosis Because most cases of palpable fibroadenoma are asymptomatic, masses are often discovered during routine breast self-examinations or by a health care professional. Cases of suspected fibroadenoma should be observed over the duration of at least one menstrual cycle. Persistent masses are confirmed with imaging studies, frequently ultrasonography. Magnetic resonance imaging and mammography are discouraged in adolescents because of increased breast density.23 Management Small and asymptomatic masses can be managed with reassurance and continued observation for changes in size, tenderness, and appearance. Fine-needle aspiration and core-needle biopsy may be used for smaller masses, but caution should be exercised because treatment may injure the developing breast. For large, painful, or growing fibroadenoma, excision with total enucleation performed with local or general anesthesia may be warranted. Giant fibroadenoma may be excised to conserve maximum functionality of the breast architecture for later lactation through an inframammary excision, minimizing scar visibility (Figure 13-11).22,23 After excision, patients may be asked to refrain from heavy physical activity for up to 6 weeks. Patients should be reexamined after the first postoperative month and again every 3 months for the first year. Patients should undergo annual breast examinations to assess recurrent and incident masses.

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A

B

Figure 13-11. A, Giant fibroadenoma of the right breast that resulted in asymmetry and distortion. B, Gross appearance of the giant fibroadenoma during excision.

Complications Fibroadenoma excision in the developing breast may result in visible scarring or deformation. Secondary asymmetry—particularly after the excision of giant fibroadenoma—has also been reported, with the affected breast being larger or smaller after completion of breast development.24 For this reason, and because of breast plasticity during adolescence, immediate reconstructive surgery after excision should be avoided. After the first postoperative year and once the patient has reached maturity, secondary asymmetry may be addressed. REFERENCES

1. Nuzzi LC, Cerrato FE, Webb ML, et al. Psychological impact of breast asymmetry on adolescents: a prospective cohort study. Plast Reconstr Surg. 2014;134(6):1116–1123 PMID: 25415081 https:// doi.org/10.1097/PRS.0000000000000736 2. Cerrato F, Webb ML, Rosen H, et al. The impact of macromastia on adolescents: a cross-sectional study. Pediatrics. 2012;130(2):e339–e346 PMID: 22802601 https://doi.org/10.1542/peds.2011-3869 3. Nuzzi LC, Cerrato FE, Erickson CR, et al. Psychosocial impact of adolescent gynecomastia: a prospective case-control study. Plast Reconstr Surg. 2013;131(4):890–896 PMID: 23542261 https://doi.org/10.1097/PRS.0b013e3182818ea8 4. Nguyen JT, Palladino H, Sonnema AJ, Petty PM. Long-term satisfaction of reduction mammaplasty for bilateral symptomatic macromastia in younger patients. J Adolesc Health. 2013;53(1):112–117 PMID: 23523309 https://doi.org/10.1016/j.jadohealth.2013.01.025 5. Schnur PL. Reduction mammaplasty-the Schnur sliding scale revisited. Ann Plast Surg. 1999;42(1):107–108 PMID: 9972729 https://doi.org/10.1097/00000637-199901000-00020

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197 Chapter 13: Breast Anomalies 6. Xue AS, Wolfswinkel EM, Weathers WM, Chike-Obi C, Heller L. Breast reduction in adolescents: indication, timing, and a review of the literature. J Pediatr Adolesc Gynecol. 2013;26(4):228–233 PMID: 23889919 https://doi.org/10.1016/j.jpag.2013.03.005 7. Kraut RY, Brown E, Korownyk C, et al. The impact of breast reduction surgery on breastfeeding: systematic review of observational studies. PLoS One. 2017;12(10):e0186591 PMID: 29049351 https://doi.org/10.1371/journal.pone.0186591 8. Cruz NI, Korchin L. Lactational performance after breast reduction with different pedicles. Plast Reconstr Surg. 2007;120(1):35–40 PMID: 17572542 https://doi.org/10.1097/01. prs.0000263371.37596.49 9. Lemaine V, Cayci C, Simmons PS, Petty P. Gynecomastia in adolescent males. Semin Plast Surg. 2013;27(1):56–61 PMID: 24872741 https://doi.org/10.1055/s-0033-1347166 10. Braunstein GD, Anawalt BD. Clinical features, diagnosis, and evaluation of gynecomastia in adults. UpToDate website. https://www.uptodate.com/contents/clinical-features-diagnosis-andevaluation-of-gynecomastia-in-adults. Reviewed January 2020. Accessed February 5, 2020 11. Bowman JD, Kim H, Bustamante JJ. Drug-induced gynecomastia. Pharmacotherapy. 2012; 32(12):1123–1140 PMID: 23165798 https://doi.org/10.1002/phar.1138 12. Rohrich RJ, Ha RY, Kenkel JM, Adams WP Jr. Classification and management of gynecomastia: defining the role of ultrasound-assisted liposuction. Plast Reconstr Surg. 2003;111(2):909–923 PMID: 12560721 https://doi.org/10.1097/01.PRS.0000042146.40379.25 13. Johnson RE, Murad MH. Gynecomastia: pathophysiology, evaluation, and management. Mayo Clin Proc. 2009;84(11):1010–1015 PMID: 19880691 https://doi.org/10.1016/S0025-6196(11)60671-X 14. Banikarim C, De Silva NK. Breast disorders in children and adolescents. UpToDate website. https://www.uptodate.com/contents/breast-disorders-in-children-and-adolescents. Reviewed January 2020. Accessed February 5, 2020 15. Novakovi_ M, Lukac M, Kozarski J, et al. Principles of surgical treatment of congenital, developmental and acquired female breast asymmetries. Vojnosanit Pregl. 2010;67(4):313–320 PMID: 20465160 https://doi.org/10.2298/VSP1004313N 16. Winocour S, Lemaine V. Hypoplastic breast anomalies in the female adolescent breast. Semin Plast Surg. 2013;27(1):42–48 PMID: 24872739 https://doi.org/10.1055/s-0033-1343996 17. Slezak R, Sasiadek M. [Poland’s syndrome]. Pol Merkur Lekarski. 2000;9(50):568–571 PMID: 11081328 18. Chan W, Mathur B, Slade-Sharman D, Ramakrishnan V. Developmental breast asymmetry. Breast J. 2011;17(4):391–398 PMID: 21645170 https://doi.org/10.1111/j.1524-4741.2011.01104.x 19. Pelosi MA III, Pelosi MA II. Breast augmentation. Obstet Gynecol Clin North Am. 2010;37(4): 533–546, viii PMID: 21093748 https://doi.org/10.1016/j.ogc.2010.09.003 20. Alobeid B, Sevilla DW, El-Tamer MB, Murty VV, Savage DG, Bhagat G. Aggressive presentation of breast implant-associated ALK-1 negative anaplastic large cell lymphoma with bilateral axillary lymph node involvement. Leuk Lymphoma. 2009;50(5):831–833 PMID: 19330656 https://doi.org/10.1080/10428190902795527 21. Shannon C, Smith IE. Breast cancer in adolescents and young women. Eur J Cancer. 2003;39(18): 2632–2642 PMID: 14642925 https://doi.org/10.1016/S0959-8049(03)00669-5 22. Sabel MS. Overview of benign breast disease. UpToDate website. https://www.uptodate.com/ contents/overview-of-benign-breast-disease. Reviewed January 2020. Accessed February 5, 2020 23. Cerrato F, Labow BI. Diagnosis and management of fibroadenomas in the adolescent breast. Semin Plast Surg. 2013;27(1):23–25 PMID: 24872735 https://doi.org/10.1055/s-0033-1343992 24. Cerrato FE, Pruthi S, Boughey JC, et al. Intermediate and long-term outcomes of giant fibroadenoma excision in adolescent and young adult patients. Breast J. 2015;21(3):254–259 PMID: 25772491 https://doi.org/10.1111/tbj.12394

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CHAPTER

14

Abdominal Wall Anomalies JACQUELINE S. ISRAEL, MD,  AND TIMOTHY W. KING, MD, PhD, MSBE, FAAP, FACS

Introduction

Abdominal wall anomalies can be challenging to manage and are best addressed by a multidisciplinary team. Pediatricians, neonatologists, and pediatric intensivists should have at least a basic knowledge of the most common abdominal wall defects. Anomalies that may be encountered by this multidisciplinary pediatric care team include gastroschisis, omphalocele, prune-belly syndrome (EagleBarrett syndrome), cloacal exstrophy, bladder exstrophy, ectopia cordis, and patent urachus or omphalomesenteric duct. While these defects may share common attributes, they are best thought of as separate conditions, approached on an individual patient basis.1 Gastroschisis is a full-thickness lateral abdominal wall defect that results in intestinal herniation. There is no membrane overlying the herniated bowel, and associated anomalies are relatively uncommon.1,2 Omphalocele, in contrast, is a midline fascial defect that involves the umbilicus, and its herniated contents may include small and large bowel, liver, stomach, and other viscera, covered with a thin membrane or sac.1,2 Additional associated congenital anomalies are more common with omphalocele than with gastroschisis. Prune-belly syndrome is a collection of anomalies characterized by 3 main features: abdominal wall laxity, cryptorchidism, and genitourinary abnormalities.3,4 In cloacal exstrophy, there is abnormal development of the hindgut and pelvis. Herniation of the entire pelvic contents is possible, and bowel may protrude through a split bladder. Bladder exstrophy is caused by a defect in the caudal abdominal wall, whereby the genitourinary tract is improperly formed and a bladder mass protrudes through the skin below the umbilicus. Finally, ectopia cordis results from a cranial abdominal wall defect, causing the heart to be located outside of the thoracic cavity, and may, along with omphalocele, be associated with pentalogy of Cantrell (eg, thoracoabdominal syndrome). This chapter will focus primarily on gastroschisis and omphalocele, the 2 most commonly encountered abdominal wall anomalies. Both may be associated with significant morbidity but can often be successfully managed throughout infancy and childhood. 199

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Embryology

A competent abdominal wall is essential for several vital physiological processes. The cardiopulmonary and gastrointestinal systems require working abdominal musculature for functions such as venous return and cardiac output, respiratory mechanics, and bowel motility. In addition, the abdominal wall is important for posture and cosmesis. Normal embryological development of the abdominal wall occurs during the first trimester.1,2,5 Early in gestation, before the fourth week, a primitive abdominal wall is formed when the cranial, caudal, and 2 lateral embryonic folds coalesce near the ventral midline, surrounding the centrally located umbilical cord.1,5 This occurs around the same time as midgut closure.2 The embryonic folds form what is called an abdominal somatopleure, consisting of thin layers of ectoderm and mesoderm without accompanying muscles, nerves, or vessels.2 Starting around weeks 4 and 5, intestinal growth occurs more rapidly than that of the abdominal wall, and the midgut contents herniate through the umbilical cord.2 Around week 6, mesoderm grows into the somatopleure, forming the paired midline rectus muscles and the individual layers of the lateral wall. For this reason, prenatal ultrasonography prior to 12 weeks’ gestation is unreliable for the diagnosis of gastroschisis. Between weeks 10 and 12, the intestines return to the abdominal cavity, rotating approximately 270° in a counterclockwise fashion.1 Proposed etiologic origins for gastroschisis include failure of migration and formation of mesoderm, improper folding, amnion rupture near the umbilical ring, and bowel wall ischemia due to a vascular insult.1,6 Omphalocele is thought to be caused by failure of the midgut to return to the abdominal cavity after herniation, although others have proposed that it relates to a wide umbilical ring after failed migration of the rectus musculature.1,2 Almost all cases of omphalocele and gastroschisis are associated with some degree of intestinal malrotation.

Epidemiology

The incidence of any abdominal wall defect is approximately 4 to 5 out of 10,000 births.2 Male and female neonates are equally affected.2 Gastroschisis occurs in 2 to 5 of 10,000 live births,1,5,6 although an increased incidence has been observed in recent years for reasons that are unclear.2 Several authors have noted that young maternal age is associated with increased incidence of gastroschisis, although the defect is thought generally to occur sporadically.1,6 Omphalocele is slightly less common, occurring in 1.5 to 3 of 10,000 births.1 Prune-belly syndrome, bladder exstrophy, and cloacal exstrophy are all much less common; prune-belly syndrome is found in 1 of 35,000 to 50,0000 births3,4 and is observed primarily in male neonates. 3

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Clinical Presentation

Pediatricians may be the first to examine a newborn with an abdominal wall anomaly. Each of the congenital abdominal wall defects should be considered separate entities. Routine follow-up is imperative, as well as understanding the warning signs of various complications.

Gastroschisis and Omphalocele Gastroschisis presents in a neonate as a full-thickness lateral abdominal wall defect that is typically located to the right of midline (Figure 14-1). There are reports of a less common “mirror image” or left-sided gastroschisis.5 The umbilicus is not involved, and there is typically a bridge of skin between the umbilicus and the defect.2 There is no membrane or sac overlying the herniated intraabdominal contents. The small bowel is most often herniated through the defect, although occasionally there is large bowel, as well. Likely owing to the lack of overlying membrane, the intestines are often inflamed and edematous and are found to have a filmy, gelatinous-like covering.2 This bowel inflammation, serositis, is thought to be attributable to direct exposure to amnion while in utero.2 Bowel function may be impaired.2 Associated congenital anomalies may be present but are relatively uncommon. Urgent intervention is often required, as the bowel is directly exposed (without an overlying membrane), which increases the risk for sepsis, hypothermia, bowel injury, and dehydration.6 Omphalocele, or exomphalos, manifests as a midline abdominal wall defect through which the bowel and other viscera (eg, liver, spleen, stomach) herniate (Figure 14-2). The intra-abdominal contents are covered with a sac or membrane, which consists of an inner layer of peritoneum, an outer layer

Figure 14-1. Gastroschisis.

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Figure 14-2. Omphalocele. Courtesy of Robert Russell, MD.

of amnion, and a middle layer of Wharton jelly.1,5,6 The defect involves the umbilicus and the umbilical vessels1,2; it may be located in the upper, middle, or lower abdomen; and its size varies from smaller than 4 cm to larger than 12 cm.7 The classification of omphalocele by size is somewhat controversial, although “giant omphalocele” typically refers to a defect that is larger than 6 cm.8 Other authors9 have classified omphalocele into 2 types, whereby type 1 reflects a defect that is smaller than 4 cm with an accompanying sac smaller than 8 cm, and with only intestinal herniation. In contrast, type 2 omphalocele may be characterized by a defect larger than 4 cm, a sac larger than 8 cm, and/or a sac that contains liver or other viscera in addition to intestine.9 Regardless of how large the defect may be, the rectus muscles are not abnormal.7 The neonate’s overall prognosis is related to the severity of the associated anomalies.1 Because the herniated contents are relatively protected by an overlying sac, therapeutic intervention is less urgent.6 Table 14-1 demonstrates the key differences between gastroschisis and omphalocele.

Other Abdominal Wall Anomalies The abdominal wall anomaly associated with prune-belly syndrome is severe hypoplasia of the rectus musculature that results in laxity and impaired bowel and cardiopulmonary function (eg, constipation, poor coughing). Additional key characteristics include genitourinary anomalies such as hydroureteronephrosis, vesicoureteral reflux, and bilateral cryptorchidism.3,4 Ultrasonography is warranted to assess the location of the testes and degree of urinary tract dilation after birth. Most patients also require evaluation for vesicoureteral reflux with either traditional voiding cystourethrography or

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203 Chapter 14: Abdominal Wall Anomalies Table 14-1. Key Differences Between Gastroschisis and Omphalocele Gastroschisis

Omphalocele

Is the viscera covered?

No membrane/sac

Membrane/sac

Location

Lateral; most often to right of midline

Midline (upper, middle, or lower abdomen)

Umbilicus

Umbilical ring and umbilical cord unaffected

Involves umbilical ring and umbilical cord; cord inserts onto membrane

Frequency of associated anomalies

More common in isolation

Multiple associated anomalies/syndromes

Examples of associated anomalies

Cryptorchidism, intestinal atresia

Cardiac, pulmonary, gastrointestinal, genitourinary; pentalogy of Cantrell; Beckwith-Wiedemann syndrome; trisomies

Risk factors

Preterm birth, young maternal age

Advanced maternal age

Bowel quality

Perivisceritis, serositis, dysmotility, poor absorption

Typically unaffected

Herniated contents

Small bowel, ± large bowel

Small bowel, ± liver, stomach, large bowel, spleen

Urgency of reduction after birth

Urgent, as bowel is exposed

Less urgent

Prognosis

Associated with degree of bowel injury

Dependent on prognosis of associated anomalies

Derived from multiple references.1,2,5,6

the newer technique of contrast-enhanced voiding urosonography. Prunebelly syndrome may be caused by oligohydramnios.3 Most children undergo abdominal wall reconstruction, although the timing of repair is often determined by the timing of genitourinary surgical procedures.4 Patients with bladder exstrophy present with a soft mass that herniates through the caudal abdominal wall, below the umbilicus. Its etiologic origin is unknown, and there is wide variation in clinical presentation. Other defects, such as cloacal exstrophy, ectopia cordis, patent urachus, and omphalomesenteric duct malformation, are exceedingly rare. Regardless of the defect, some general considerations are universal: Clinicians should assess whether there is an overlying membrane or sac (eg, Is the viscera exposed?), as well as the presence or absence of associated anomalies (eg, Are there associated life-threatening conditions? Is additional workup warranted?).

Associated Anomalies An abdominal wall anomaly that does not occur in isolation is most often found in the setting of omphalocele. The term complex abdominal wall defect is often used when referring to an abdominal wall defect that is associated

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with additional anomalies.10 Koivusalo et al provide a list of associated anomalies that have been observed. • Cardiovascular defects: coarctation of the aorta, ventricular septal defect, atrial septal defect, dextroposition of the aortic arch • Gastrointestinal defects: malrotation, esophageal atresia, small intestinal atresia, colonic atresia, Meckel diverticulum, volvulus, intestinal duplication, multicystic liver, diaphragmatic hernia, imperforate anus, aganglionosis11 • Genitourinary defects: cryptorchidism, testicular aplasia, vesicoureteral reflux, kidney hypoplasia, hypospadias • Musculoskeletal defects: syndactyly, digital hypoplasia, pes equinovarus, pectus carinatum • Other: Beckwith-Wiedemann syndrome, chromosomal abnormalities (eg, trisomies) Omphalocele is more commonly associated with congenital anomalies, with an incidence rate that may be higher than 75%.1,6,11 Anomalies can be further characterized by whether they are associated with a syndrome (approximately 40% of cases), a chromosomal abnormality (30% of cases; most often trisomies [eg, trisomy 13, 14, 15, 18, or 21]), or nonsyndromic (30% of cases).1,6,11 Examples of associated syndromes include Beckwith-Wiedemann, Donnai-Barrow, pentalogy of Cantrell, and Gershoni-Baruch.1,5,6 BeckwithWiedemann is the most commonly associated syndrome (seen in approximately 10% of cases) and is characterized by macroglossia (large tongue), organomegaly, hypoglycemia, and increased risk for Wilms tumor, hepatoblastoma, or neuroblastoma.1 Pentalogy of Cantrell refers to the presence of omphalocele, diaphragmatic hernia, sternal cleft, pericardial defect, and cardiac defect, such as ventricular septal defect.1,5 Other severe defects may occur, such as holoprosencephaly or anencephalus.6 Gastroschisis may be associated with additional congenital anomalies in 10% to 20% of neonates with a right-sided defect1,11 and in 40% of infants with a right-sided defect.11 Intestinal atresia is the most common associated anomaly and is observed in 10% to 15% of cases.1,2

Diagnosis

Gastroschisis and omphalocele are often diagnosed prior to birth via ultrasonography. Ultrasonography has a sensitivity rate of approximately 83% in the diagnosis of gastroschisis and 75% in the diagnosis of omphalocele,5 while its specificity for both diagnoses approaches 95%.1 Serum markers may also assist in the diagnosis of gastroschisis and omphalocele.1,5 Maternal serum α-fetoprotein level is increased in both conditions, although it is more consistently increased in the setting of gastroschisis.1 Acetylcholinesterase level may also be increased.5

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Management Prenatal Management After ultrasonographic diagnosis of gastroschisis or omphalocele, amniocentesis may be performed for karyotyping and additional testing. Diagnostic evaluation for associated anomalies is warranted and may include fetal echocardiography, genetic karyotyping, and renal ultrasonography.1 Prenatal care should be transitioned to a high-risk obstetrics specialist at a specialized care center. Referral to a pediatric surgeon, geneticist, and perinatologist is also indicated. A prenatal conversation with a pediatric and/or reconstructive surgeon can facilitate further discussion of the condition, treatment options, and expectations.1 In addition, consultation with specialists allows for the family to be informed of the risk of complications, such as intrauterine growth restriction (in as many as 70% of neonates), preterm birth, intestinal abnormality and dysfunction, and even fetal death.1 Principles and Goals The primary goals for management include resuscitation, followed by adequate reduction of the herniated intra-abdominal contents and eventual closure of the fascial defect.1 This restores the visual and functional integrity of the abdominal wall. Reduction and closure may be performed in a single stage or in multiple stages, either primarily or with the use of prosthetics.1,5 Regarding repair of the defect, the primary goal is to correct the mismatch between the space available in the abdominal cavity, or domain, and the total volume that needs to be returned to the abdominal cavity. This is referred to as “abdominal visceral disproportion.”8 Ideally, providing complete muscle coverage is optimal, as the abdominal wall is most functional when the muscles are able to contract.12 Potential complications include dehiscence, evisceration, bowel obstruction, and infection. Medical Management Postnatally, newborns should be immediately assessed for abnormalities of the ABCs (ie, airway, breathing, circulation).1,6 The abdominal wall defect will be visually evident, and the herniated viscera, whether it is accompanied by an overlying membrane or not, should be covered with saline-moistened gauze, as well as a plastic bag or plastic wrap.2,6 This will minimize heat and fluid losses and protect the exposed bowel from mechanical injury. Owing to the risk of dehydration attributable to fluid losses, intravenous access should be obtained and resuscitative and maintenance fluids begun. The neonate should be positioned with his or her right side down, to avoid vascular compromise caused by mechanical compression of the umbilical cord or bowel.6 Broad-spectrum intravenous antibiotics (eg, ampicillin/gentamycin) should be started early because of the risk of infection attributable to exposed bowel.2 The patient should be transferred to a neonatal intensive care unit and monitored for respiratory compromise, hemodynamic instability, and

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hypoglycemia. The patient will require access for and preparation of parental nutrition. The inpatient pediatric surgery team should be consulted immediately after birth. Relevant preoperative considerations include protecting the exposed viscera prior to attempted reduction and repair and medically optimizing the neonate to undergo surgical treatment. The risk of volvulus and bowel ischemia should be minimized with nasogastric tube placement for bowel decompression, positioning, and close monitoring.6,11 Nonadherent dressings should be maintained continuously and changed when necessary. Chest radiography and echocardiography are typically indicated. The neonate may not be physiologically cleared to undergo general anesthesia because of the presence of cardiopulmonary abnormalities.13

Reduction: General Considerations Once the patient has been medically optimized, the next step in management is reduction of the herniated contents. Reduction can be performed immediately after birth, in a delayed fashion, in 1 stage, or in multiple stages. Timing for and complexity of reduction is dependent on the size of the fascial defect, the volume of exposed viscera, the physiological fitness of the patient, the presence of additional anomalies, and the location of the defect on the abdomen.1,14 Immediate reduction with primary repair can typically only be performed when the defect is small and the viscera appear viable and minimally inflamed. There has been considerable debate about timing of and setting for hernia reduction.1,11 Marven and Owen11 offer 4 different strategies for reduction1: primary reduction in the operating room, 2 primary reduction at the bedside, 3 staged (multiple-step) reduction by using a silo or prosthetic patch in the operating room, and staged reduction at the bedside.4 A spring-loaded silo is likely the most common technique for reduction of the hernia contents for gastroschisis and omphalocele. There are several modifications available for each of these options, including surgically enlarging the defect to accommodate the silo device and using “gravity-based reduction,” whereby a silo is hung from the patient’s bed or incubator and is sequentially tied off over several days (Figure 14-3).1 When the viscera are covered with a membrane (as in omphalocele), surgical intervention is not urgent.1 The optimal technique for fascial closure in patients with omphalocele is related to size of the defect.5 Some authors promote early closure (eg, within the first 1 to 2 weeks after birth) to minimize time that the bowel is exposed and thus reduce the risk for sepsis, the most frequent cause of mortality.9 Because associated anomalies are common in omphalocele, immediate or early repair may not be feasible. It is possible to facilitate epithelialization of the overlying sac and perform reduction and repair in a delayed fashion.5

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Figure 14-3. The use of a silo for hernia reduction. Courtesy of Robert Russell, MD.

One agent that facilitates epithelialization, or “escharotic treatment,” is topical silver sulfadiazine (Silvadene; Pfizer, New York, NY).1,6 This also promotes contraction of the membrane, and repair can be delayed until the neonate is medically stabilized. Other topical agents include povidoneiodine and neomycin-bacitracin.5 The relatively high incidence of intestinal atresia or stenosis in gastroschisis may preclude reduction prior to surgical repair. In these instances, urgent surgical exploration is performed, and patients often have ostomies and/or bowel in discontinuity for a period. If there is no appreciable bowel atresia or stenosis, reduction and closure may be performed in 1 stage if possible. Alternatively, a staged reduction can be performed by using a silo, followed by eventual repair of the defect.5,11 As noted by Marven and Owen, “The safety of reduction and closure techniques is related to the level of intra-abdominal pressure and therefore the degree of viscera-abdominal disproportion.”11 Mortellaro and colleagues cite “grade C evidence” that immediate reduction and staged reduction with delayed closure have equivalent survival rates.5

Surgical Repair of the Abdominal Wall Clinicians should apply the principle of the reconstructive ladder when considering repair of abdominal wall defects.15 The general concept of the reconstructive ladder is that there are often several options for management, ranging from least complex to most complex, and a primary option should

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be selected that best suits the individual characteristics of the defect for that particular patient.15 Options include, but are not limited to14,15 • Primary closure with or without the use of temporary or permanent mesh • Secondary closure (assisted by dressing changes, vacuum-assisted closure devices) • Skin grafting with or without the use of relaxing incisions • Local or locoregional soft-tissue flaps • Free tissue transfer The particular location of the defect is also relevant, because abdominal wall laxity and rotational capacity vary by location.14,16 There is substantial variability in the timing and method of surgical repair. Regardless of the selected technique, surgeons should be cautious when manipulating the midline sac in omphalocele, as the hepatic vasculature and bladder are often in close proximity and are susceptible to inadvertent injury.6 Primary fascial closure may be successful for small defects.6 Authors have described either a linear or a purse-string closure technique, or a method referred to as a plastic closure, whereby the umbilical cord is incorporated into the initial defect.6 Reportedly, the latter method facilitates improved cosmesis.6 The fascial defect may also be repaired by using mesh or a patch. Mesh, whether prosthetic, biological, or bioprosthetic, can be used as either a fascial substitute or to reinforce the repair.16 In addition, mesh or a patch may be used in a temporary or permanent fashion; when used temporarily, a silicone/silastic sheet inlay may prevent evisceration of the intra-abdominal contents and may ultimately be removed after several days to definitively repair the fascia and overlying skin.6 Examples of prosthetic mesh materials include polypropylene, polytetrafluoroethylene (Gore-Tex; WL Gore & Associates Inc, Newark, DE), and polyglactin (Vicryl; Ethicon, Blue Ash, OH). Acellular bioprosthetic materials, such as porcine small intestinal mucosa (Biodesign; Cook Medical, Bloomington, IN), contain collagen, are infection resistant, and allow for vascular ingrowth.6,11,17 Other bioprosthetic materials, such as AlloDerm (Allergan, Madison, NJ) and Integra (Integra LifeSciences, Plainsboro Township, NJ), may be used, but more evidence of their safety and effectiveness in the pediatric population is warranted.5 Mesh is often used in combination with other reconstructive procedures, described as follows. Skin grafting may be performed over granulated fascia. Notably, neonates and infants have far less surface area for skin graft donor sites, so this option may be limited. Zaccara et al describe a technique for skin closure for which bilateral skin flaps are elevated and advanced over closed fascia or an omphalocele sac, facilitated by the use of bilateral relaxing incisions that are then skin grafted.9,18 Abdominoplasty has been described as a method for reconstructing the abdominal wall laxity observed in prune-belly syndrome; this technique has been shown to improve strength, function, and cosmesis.3,4

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Delayed repair of fascial defects may be performed months or years after reduction and skin closure. Intraperitoneal tissue expanders allow for increased volume within the intra-abdominal cavity.8,12 This method for increasing abdominal domain may be optimal in children, as it involves the use of native tissue that will grow with the child and preserve muscular contractility.12 All health care professionals involved in the care of patients with intraperitoneal tissue expanders should be aware of the risks and signs of abdominal compartment syndrome (detailed as follows). Component separation is a technique initially described by Ramirez et al for reconstruction of abdominal wall defects in adults.19 This technique involves releasing the fascia of the external oblique muscle and advancing bipedicle muscle flaps to meet in the midline.7,19,20 Valerio et al reported a case of primary fascial closure by using component separation in a 1-yearold who sustained penetrating and blunt abdominal trauma.20 Full-thickness abdominal wall defects may be repaired by using a combination of mesh and tissue flaps. Flaps may be local (eg, connected to their native blood supply) or free (eg, disconnected from their native blood supply and connected to a new blood supply at the recipient site). They may contain any combination of skin, subcutaneous fat, fascia, and muscle. Options for flap selection in the pediatric population are similar to those in adults and include rectus abdominis, tensor muscle of fascia lata, anterolateral thigh, latissimus dorsi, omentum, and rectus femoris.

Adjunctive Therapy Negative-pressure wound therapy, or vacuum-assisted wound closure, has been used in many clinical scenarios to achieve secondary wound closure, promote granulation, and minimize the risk of infection.21 In abdominal wall defects, it may be used as a primary or temporizing therapy. DeFranzo et al reported on a series of 100 adults who underwent negative-pressure wound therapy prior to definitive abdominal reconstruction and concluded that this therapy “frequently shortened time to abdominal wall reconstruction and simplified the method of reconstruction.”14 While more extensively studied in adults, negative-pressure wound therapy is thought to be safe for use in neonates and children, although lower settings are recommended.21,22

Outcomes Immediate Postoperative Considerations When reduction and/or repair of the fascial defect is performed soon after birth, postoperative care in an intensive care unit is often indicated. A multidisciplinary care team is essential. Patients are continued on parenteral nutrition until enteral feeding can be initiated and advanced. If a silo is in place for staged reduction, it may become inadvertently detached or cause infection if it

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remains in place for too long. Adverse effects related to parenteral nutrition, such as cholestasis and liver failure, may occur.2

Complications Major complications of reduction and repair of gastroschisis and omphalocele include bowel obstruction, volvulus and associated bowel ischemia, fascial dehiscence, wound dehiscence, prosthetic-related infection, graft loss, flap loss, prolonged hospitalization, recurrent hospitalizations, and chronic bowel dysmotility.11 Reduction and closure may also result in abdominal compartment syndrome, as diagnosed by an intra-abdominal pressure higher than 20 mm Hg, and can result in hypoventilation, poor cardiac output, acidosis, renal failure, sepsis, bowel ischemia, necrotizing enterocolitis, wound breakdown, and even death.11 After any reduction and/or closure technique, intra-abdominal pressure may be monitored via serial bladder pressures. Peak inspiratory pressures can also be obtained as a surrogate for intra-abdominal pressure. Clinicians should have a low threshold for returning to the operating room for suspected abdominal compartment syndrome. Suboptimal cosmesis may occur as the result of multiple surgical procedures or infectious complications. Scar revisions can be performed later in life, and umbilical revisions performed by using an ellipse flap or reverse fan-shaped flap have been described.23,24 Prognosis Overall prognosis of isolated gastroschisis and omphalocele is favorable. Survival in gastroschisis is 90% to 95%, with deaths primarily attributable to bowel necrosis and its associated complications.1,6 In contrast, overall prognosis and survival in omphalocele relate primarily to the presence and type of associated anomalies.1,6 A survey of 57 adult patients with a history of gastroschisis and omphalocele reported similar quality-of-life scores in this patient population relative to the general population.10 Morbidity seemed primarily attributable to scar appearance and relatively mild gastrointestinal conditions.10

Future Research

Ongoing developments in stem cell research may facilitate new methods for abdominal wall reconstruction. Composite tissue allotransplantation, in the form of intestinal and abdominal wall transplantation, is another field that offers promise for neonates with profound loss of abdominal domain.25 Composite tissue allotransplantation offers a single-stage surgical procedure and may be optimal when considering the multiple procedures and high complication rate of current reconstructive options. Further research in immunogenic tolerance and immunosuppressive therapies may enable

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application of abdominal composite tissue allotransplantation in the pediatric population.

Conclusion

The management of the most common abdominal wall anomalies, gastroschisis and omphalocele, is a collaborative effort that involves pediatricians, pediatric specialists, the patient, and the patient’s support system. These defects are often diagnosed prenatally, offering families and health care professionals the opportunity to learn more about the diagnosis and anticipate the treatment course. Goals of repair include restoration of function, optimization of aesthetic outcome, and avoidance of complications. A basic understanding of the defect, selected reconstructive technique, and associated sequelae is essential when caring for these patients throughout childhood and adolescence. REFERENCES

1. Ledbetter DJ. Congenital abdominal wall defects and reconstruction in pediatric surgery: gastroschisis and omphalocele. Surg Clin North Am. 2012;92(3):713–727, x PMID: 22595717 https://doi.org/10.1016/j.suc.2012.03.010 2. Yang J, Lund D. Congenital defects of the abdominal wall. In: Bentz M, Bauer B, Zuker R, eds. Principles and Practice of Pediatric Plastic Surgery. St Louis, MO: CRC Press Quality Medical Publishing; 2008:1545 3. Lesavoy MA, Chang EI, Suliman A, Taylor J, Kim SE, Ehrlich RM. Long-term follow-up of total abdominal wall reconstruction for prune belly syndrome. Plast Reconstr Surg. 2012;129(1): 104e–109e PMID: 22186524 https://doi.org/10.1097/PRS.0b013e3182362091 4. Lopes RI, Tavares A, Srougi M, Dénes FT. 27 years of experience with the comprehensive surgical treatment of prune belly syndrome. J Pediatr Urol. 2015;11(5):276.e1–276.e7 PMID: 26143487 https://doi.org/10.1016/j.jpurol.2015.05.018 5. Mortellaro VE, St Peter SD, Fike FB, Islam S. Review of the evidence on the closure of abdominal wall defects. Pediatr Surg Int. 2011;27(4):391–397 PMID: 21161242 https://doi.org/10.1007/ s00383-010-2803-2 6. Christison-Lagay ER, Kelleher CM, Langer JC. Neonatal abdominal wall defects. Semin Fetal Neonatal Med. 2011;16(3):164–172 PMID: 21474399 https://doi.org/10.1016/j.siny.2011.02.003 7. Wijnen RM, van Eijck F, van der Staak FH, Bleichrodt RP. Secondary closure of a giant omphalocele by translation of the muscular layers: a new method. Pediatr Surg Int. 2005;21(5):373–376 PMID: 15803336 https://doi.org/10.1007/s00383-005-1387-8 8. Tenenbaum MJ, Foglia RP, Becker DB, Kane AA. Treatment of giant omphalocele with intraabdominal tissue expansion. Plast Reconstr Surg. 2007;120(6):1564–1567 PMID: 18040189 https://doi.org/10.1097/ 01.prs.0000282091.40103.6b 9. Zama M, Gallo S, Santecchia L, et al. Early reconstruction of the abdominal wall in giant omphalocele. Br J Plast Surg. 2004;57(8):749–753 PMID: 15544772 https://doi.org/10.1016/j. bjps.2004.05.021 10. Koivusalo A, Lindahl H, Rintala RJ. Morbidity and quality of life in adult patients with a congenital abdominal wall defect: a questionnaire survey. J Pediatr Surg. 2002;37(11):1594–1601 PMID: 12407546 https://doi.org/10.1053/jpsu.2002.36191 11. Marven S, Owen A. Contemporary postnatal surgical management strategies for congenital abdominal wall defects. Semin Pediatr Surg. 2008;17(4):222–235 PMID: 19019291 https://doi. org/10.1053/j.sempedsurg.2008.07.002 12. Clifton MS, Heiss KF, Keating JJ, Mackay G, Ricketts RR. Use of tissue expanders in the repair of complex abdominal wall defects. J Pediatr Surg. 2011;46(2):372–377 PMID: 21292090 https://doi. org/10.1016/j.jpedsurg.2010.11.020

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212 Pediatric Plastic and Reconstructive Surgery for Primary Care 13. Cauchi J, Parikh DH, Samuel M, Gornall P. Does gastroschisis reduction require general anesthesia? A comparative analysis. J Pediatr Surg. 2006;41(7):1294–1297 PMID: 16818066 https://doi.org/10.1016/j.jpedsurg.2006.03.010 14. DeFranzo AJ, Pitzer K, Molnar JA, et al. Vacuum-assisted closure for defects of the abdominal wall. Plast Reconstr Surg. 2008;121(3):832–839 PMID: 18317132 https://doi.org/10.1097/01. prs.0000299268.51008.47 15. Janis JE, Kwon RK, Attinger CE. The new reconstructive ladder: modifications to the traditional model. Plast Reconstr Surg. 2011;127(suppl 1):205S–212S PMID: 21200292 https://doi.org/10.1097/ PRS.0b013e318201271c 16. Mathes SJ, Steinwald PM, Foster RD, Hoffman WY, Anthony JP. Complex abdominal wall reconstruction: a comparison of flap and mesh closure. Ann Surg. 2000;232(4):586–596 PMID: 10998657 https://doi.org/10.1097/00000658-200010000-00014 17. Dasgupta R, Wales PW, Zuker RM, Fisher DM, Langer JC. The use of Surgisis for abdominal wall reconstruction in the separation of omphalopagus conjoined twins. Pediatr Surg Int. 2007;23(9):923–926 PMID: 17437118 https://doi.org/10.1007/s00383-007-1909-7 18. Zaccara A, Zama M, Trucchi A, Nahom A, De Stefano F, Bagolan P. Bipedicled skin flaps for reconstruction of the abdominal wall in newborn omphalocele. J Pediatr Surg. 2003;38(4): 613–615 PMID: 12677577 https://doi.org/10.1053/jpsu.2003.50133 19. Ramirez OM, Ruas E, Dellon AL. “Components separation” method for closure of abdominalwall defects: an anatomic and clinical study. Plast Reconstr Surg. 1990;86(3):519–526 PMID: 2143588 https://doi.org/10.1097/00006534-199009000-00023 20. Valerio I, Sabino J, Kumar A. A case of pediatric abdominal wall reconstruction: components separation within the austere war environment. Plast Reconstr Surg Glob Open. 2014;2(7):e180 PMID: 25426363 https://doi.org/10.1097/GOX.0000000000000120 21. Baharestani M, Amjad I, Bookout K, et al. V.A.C. therapy in the management of paediatric wounds: clinical review and experience. Int Wound J. 2009;6(suppl 1):1–26 PMID: 19614789 https://doi.org/10.1111/j.1742-481X.2009.00607.x 22. Byrne-Bowens P, Mieczyslawa F. Negative pressure wound therapy in a neonate with a complex abdominal wound. Wounds. 2013;25(1):E1–E4 23. Masuda R, Takeda A, Sugimoto T, Ishiguro M, Uchinuma E. Reconstruction of the umbilicus using a reverse fan-shaped flap. Aesthetic Plast Surg. 2003;27(5):349–353 PMID: 14691608 https://doi.org/10.1007/s00266-003-3032-z 24. Park S, Hata Y, Ito O, Tokioka K, Kagawa K. Umbilical reconstruction after repair of omphalocele and gastroschisis. Plast Reconstr Surg. 1999;104(1):204–207 PMID: 10597697 https://doi. org/10.1097/00006534-199907000-00032 25. Quigley MA, Fletcher DR, Zhang W, Nguyen VT. Development of a reliable model of total abdominal wall transplantation. Plast Reconstr Surg. 2013;132(4):988–994 PMID: 24076687 https://doi.org/10.1097/PRS.0b013e31829f4bd3

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CHAPTER

15

Posterior Trunk Anomalies BRAD T. MORROW, MD, AND DONALD R. MACKAY, MD, FAAP, FACS

Introduction

Posterior trunk anomalies can encompass a broad spectrum of congenital defects, ranging from the integument to the thoracic wall; however, most cases involve the spine. Spinal anomalies include neural tube defects, teratomas, dermal sinuses, lipomas, and postanal pits. Myelomeningoceles represent the most challenging aspect of spinal dysraphism. The principles of myelomeningocele reconstruction are applicable to most posterior trunk anomalies.

Classification and Presentation of Spinal Dysraphism

Spinal dysraphism encompasses a spectrum of congenital anomalies that result from a failure in differentiation or fusion of dorsal midline structures. The classification of spinal dysraphism can be divided into open and closed defects (Figure 15-1). In open defects, neural tissue is exposed to the environment through a congenital posterior vertebral defect. Conversely, in closed defects, a posterior vertebral defect is present, but the neural tissue is covered by skin and not exposed.1 However, the overlying skin may be thin, irregular, and atrophic or associated with a subcutaneous mass, hemangioma, or dimple.2 Open spinal dysraphism includes myelomeningoceles, myeloceles, and myeloschisis. A myelomeningocele is formed by the herniation of meninges and spinal cord through the posterior vertebral arch defect and skin, but the contents are covered by a membranous sac (Figure 15-2). A myelocele is similar, but the defect is flush with the surrounding skin. In myeloschisis, the herniated tissue has no overlying membrane and is exposed to the outside environment. The extent of the neurological deficit depends on the level of the lesion. Lesions at or below L2 will have a flaccid paralysis of the affected muscle groups, sensory loss in the cephalad dermatome, and bladder and bowel incontinence. Lesions below S3 will have bladder and bowel paralysis and saddle anesthesia, but lower-extremity motor deficits will be absent. Most patients with a myelomeningocele will also have hydrocephalus and a type II Chiari malformation (Arnold-Chiari malformation), which is a downward displacement of the cerebellum and medulla through the foramen magnum. 213

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Spinal dysraphism

Open

Myelomeningocele

Closed

Myeloschisis

Subcutaneous mass

• Meningocele • Myelocystocele • Lipoma with a dural defect

No subcutaneous mass

• Posterior spina bifida • Dermal sinus • Diastematomyelia

Figure 15-1. Classification of spinal dysraphism.

Figure 15-2. Myelomeningocele. From Luciano MG, Elbabaa SK. Myelomeningocele and associated anomalies. In: Benzel EC, ed. Spine Surgery: Techniques, Complication Avoidance, and Management. 3rd ed. Philadelphia, PA: Elsevier Saunders; 2012:1155–1162.

Closed spinal dysraphism includes a heterogeneous group of anomalies that may or may not be associated with a subcutaneous mass. Those with a subcutaneous mass include meningoceles, myelomeningoceles, and a lipoma with a dural defect. A meningocele is formed by the herniation of meninges

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Figure 15-3. Midline lumbosacral hypertrichosis and hyperpigmentation in posterior spina bifida. From Spine and spinal cord: developmental disorders. In: Schapira AHV, ed. Neurology and Clinical Neuroscience. Philadelphia, PA: Mosby Elsevier; 2007.

through a defect in the posterior vertebral arch or anterior sacrum, but the spinal cord remains unaffected in an anatomical position. As such, patients will typically have normal neurological examination findings; however, spinal cord tethering, syringomyelia, or diastematomyelia may be associated. A meningocele may be covered by intact skin; however, it can be thin, atrophic, and prone to rupture. Closed spinal dysraphism without a subcutaneous mass includes posterior spina bifida, a dermal sinus, and diastematomyelia. Posterior spina bifida, previously referred to as spina bifida occulta, consists of a midline posterior vertebral body fusion defect without herniation of the spinal cord or meninges and coverage with intact skin. External manifestations are often subtle but can include midline lumbosacral cutaneous lesions, such as hypertrichosis or hyperpigmentation (Figure 15-3). Most patients are asymptomatic, but an index of suspicion must remain because neurological sequela can develop insidiously in late childhood or early adulthood owing to spinal cord tethering. The term spina bifida cystica has previously been used to refer to a meningocele or myelomeningocele (Figure 15-4).

Evaluation

Recent advancements in maternal screening and prenatal imaging with ultrasonography and magnetic resonance imaging have led to an increased rate of myelomeningocele detection in utero. On identification, the family can be referred to a comprehensive, multidisciplinary team owing to the potential complexity of these patients. The assembled team should consist of

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Sac containing: Meninges + CSF Spinal cord + menings + CSF

Spinal cord

Skin

Vertebrae

Hairs

Vertebrae

Vertebral arches missing

Skin Meningocele

Viscera

Meningom

Figure 15-4. Sagittal view of spina bifida malformations. Abbreviation: CSF, cerebrospinal fluid. From Haines DE, ed. Fundamental Neuroscience for Basic and Clinical Applications. 3rd ed. Philadelphia, PA: Churchill Livingstone; 2006.

a neonatologist, neurosurgeon, orthopedic surgeon, urologist, plastic surgeon, pediatrician, social worker, physical therapist, and geneticist. Prenatal counseling can facilitate postnatal care by providing the family with emotional support and education to allow for informed decisionmaking.3 With the use of prenatal diagnostic modalities, some of these patients may be candidates for antenatal repair.4

Prognosis

The multidisciplinary team approach, coupled with early meningocele closure, the introduction of cerebrospinal fluid (CSF) shunts, and neurogenic bladder management, has drastically improved mortality rates. In the 1950s, prior to these techniques, the mortality rate was greater than 80% by 24 months of age.5 The bleak prognosis led some to propose selective management by actively withholding treatment in the presence of gross macrocephaly or myelomeningoceles at or above L3.6–8 In the modern era, survival rates have significantly increased, with 75% of patients reaching adulthood and 60% reaching 35 years of age.9,10

Indications

The indications for surgical management of closed spinal dysraphism will depend on the extent and location of the lesion. An asymptomatic meningocele covered by intact, full-thickness skin may be electively repaired or deferred. However, if a CSF leak or atrophic skin is present, the meningocele should be urgently repaired to prevent meningitis.

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In open spinal dysraphism, the neural tube must be reconstructed and covered with well-vascularized tissue to prevent the development of meningitis, preserve the remaining function of the spinal cord, and seal CSF leaks. The associated bony defects do not typically require reconstruction, except in pronounced kyphosis, when prominent laminectomies may be performed to decrease tension on the soft-tissue closure.

Preferred Technique

Immediately after birth, open spinal dysraphisms must be kept moist to prevent desiccation and further neurological deterioration. While there are conflicting results in the literature for the optimal timing of myelomeningocele closure, repair of defects within the first 72 hours after birth has drastically reduced associated morbidity and mortality.3,11–13 In most myelomeningocele defects, the dural repair can be immediately covered with local fascial turnover flaps, with or without bilateral para­ spinous muscle flaps and wide undermining for primary midline skin closure. The extent of the closure will be determined by the location of the myelomeningocele. If the defect is at the level of the paraspinous muscles, the paraspinous fascia is elevated laterally to medially on the basis of paravertebral perforating arteries, turned over, and sutured to each other in the midline. The bilateral paraspinous muscles are then elevated as a bipedicle flap and advanced to the midline. If the defect is caudal to the paraspinous muscles, the gluteal fascia is elevated in continuity with the paraspinous fascia and turned over into the midline (Figure 15-5). At this location, additional muscle will not be incorporated into the repair. A liner midline skin closure can be achieved by widely undermining, often to the anterior axillary fold. Drains should be placed to prevent the development of a seroma. Postoperatively, prone positioning may be used to decrease pressure on the repair. There are several advantages to this technique, including a layered closure with vascularized soft tissue in between the dural repair and skin. Reinforcement of the dural repair with vascularized paraspinous fascia creates a tension-free, watertight closure that minimizes the risk of a CSF leak that could potentially develop into meningitis. Paraspinous fascia has been previously used after myelomeningocele repair and tethered cord correction. In a study by Zide et al, the use of paraspinous fascia in closures reduced the rate of pseudo-meningocele formation from 43% (26 of 60) to 3% (2 of 70) and reduced the rate of CSF leak from 13% (8 of 60) to 0% (0 of 70).14 Depending on the amount of fascial mobilization, the turnover fascial flaps may be overlapped in a “pants-over-vest” fashion.15,16 The addition of bilateral paraspinous muscle flaps provides bulk that can obliterate any dead space and further separates the dural repair from the integument. In the event of wound dehiscence or necrosis, the dural repair is protected, and the wound may be allowed to heal by secondary intention.

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Figure 15-5. Myelomeningocele repair at or caudal to the paraspinous muscles. From Patel KB, Taghinia AH, Proctor MR, Warf BC, Greene AK. Extradural myelomeningocele reconstruction using local turnover fascial flaps and midline linear skin closure. J Plast Reconstr Aesthet Surg. 2012;65(11):1569–1572.

Furthermore, regional muscles are not violated, which limits functional deficits in patients who are already neurologically compromised. Dissection and advancement of gluteal or latissimus muscle flaps can compromise future ambulation, use of crutches, or transfers to wheelchairs. Myelomeningocele repairs must take into account the eventual need for additional procedures owing to spinal cord tethering or scoliosis.9 A linear midline closure will minimize scarring, enhance exposure, and prevent the potential devascularization of tissue caused by multiple incisions during subsequent procedures. Patel et al reported on this technique in 3 myelomeningocele closures, with defects ranging from 4.5 to 8.5 cm in width (Figure 15-6).17 There were no reported complications such as CSF leak, hematoma, infection, skin flap necrosis, or wound breakdown.

Additional Techniques

Myelomeningoceles have a marked heterogeneity in their presentation. As such, a single technique may not be applicable to all defects, and the reconstructive surgeon must be capable of tailoring the repair to the specifics of the defect. While the size of the defect is an important guide in selecting the most appropriate technique, the ultimate determination will be based on the quantity and quality of the surrounding tissues.

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Figure 15-6. Closure of an 8.5 × 8.0-cm lumbosacral myelomeningocele in a 2-day-old boy with local fascial turnover flaps, paraspinous muscle flaps, and linear midline closure. From Patel KB, Taghinia AH, Proctor MR, Warf BC, Greene AK. Extradural myelomeningocele reconstruction using local turnover fascial flaps and midline linear skin closure. J Plast Reconstr Aesthet Surg. 2012;65(11):1569–1572.

Historically, thoracolumbar myelomeningocele defects were closed with bilateral latissimus dorsi musculocutaneous flaps pedicled on the dominant thoracodorsal artery. In lumbosacral defects, the external oblique muscle was included and advanced as a composite musculocutaneous unit.18 As the external oblique was the limiting factor in advancement, the technique was further modified to incorporate thoracolumbar and gluteal fascia. However, for adequate advancement, bilateral relaxing incisions were required that created bipedicle skin flaps that necessitated a donor site skin graft.19,20 In an effort to avoid relaxing incisions, Ramirez et al proposed en bloc advancement of bilateral latissimus dorsi and gluteus maximus muscles interconnected by thoracolumbar fascia (Figure 15-7).21 After the dura is repaired, the thoracolumbar fascia is incised over the paraspinous muscles, and dissection proceeds on the undersurface of the latissimus dorsi to free the lateral attachments. The dorsal intercostal perforating vessels, which consist of the minor segmental blood supply to the latissimus dorsi, are cauterized and divided to allow for adequate medial advancement of the flaps. The intervening lumbar skin connecting the latissimus dorsi and gluteus maximus is preserved, provided that there is minimal subcutaneous undermining. The gluteus maximus is released from its origin on the iliac crest and sacrum, and dissection continues in the plane between the gluteus maximus and medius, ensuring that the gluteal perforators are preserved. The bilateral flaps are mobilized to the midline and closed in layers.

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Latissimus dorsi

A

B

Gluteus maximus

Latissimus dorsi

Gluteus maximus C

Figure 15-7. Closure of (A) a large lumbosacral meningomyelocele defect is shown with (B, C) en bloc advancement of bilateral latissimus dorsi and gluteus maximus muscle flaps. Based on Ramirez OM, Ramasastry SS, Granick MS, Pang D, Futrell JW. A new surgical approach to closure of large lumbosacral meningomyelocele defects. Plast Reconstr Surg. 1987;80(6):799–809.

Variations on the latissimus dorsi flap have been described in the literature by splitting or reversing the muscle. In the split technique, bilateral skin paddles are raised in continuity with the medial aspect of the latissimus dorsi muscle and on the basis of thoracic and lumbar intercostal perforating arteries. The latissimus dorsi muscle is then split to preserve the lateral aspect, which is supplied by the thoracodorsal artery. The composite skin paddle and muscle is then advanced in an inferomedial direction and closed in a V-Y procedure.22 The reverse latissimus dorsi flap is harvested by disinserting the humeral tendon and dividing the dominant thoracodorsal vessels, thereby perfusing the flap on segmental pedicles from the 9th, 10th, and 11th dorsal intercostal vessels.23,24 The unilateral or bilateral flap can be inset as a transposition of a skin paddle or in a turnover fashion to close myelomeningocele and myelo-rachischisis defects (Figure 15-8).25–30 The proposed benefits include avoiding a suture line over the dural repair and well-vascularized tissue bulk to fill the defect. The primary disadvantage of the reverse technique is

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Figure 15-8. The reverse turnover latissimus dorsi muscle based on the 9th, 10th, and 11th dorsal intercostal perforators. Flap inset with a split-thickness skin graft. From Zakaria Y, Hasan EA. Reversed turnover latissimus dorsi muscle flap for closure of large myelomeningocele defects. J Plast Reconstr Aesthet Surg. 2010;63(9):1513–1518.

devitalizing the latissimus dorsi muscle with added morbidity in the turnover technique, due to the requirement of a skin graft to cover the muscle. The latissimus dorsi muscle is innervated by the thoracodorsal nerve, derived from C6-C8, and contributes to arm adduction, internal rotation, and extension. While there are extensive data in the literature regarding the relatively low morbidity of harvesting the latissimus dorsi muscle in adults, specifically for breast reconstruction, there remains a paucity of data on the long-term effects of using the muscle for myelomeningocele closure. 31,32 Multiple authors have raised concerns about sacrificing functional muscles that could impair ambulation, use of crutches, or transfers from wheelchairs in patients who are already neurologically compromised.16,17,21,33,34 To date, there are 2 studies in the literature on the examination of the use of the latissimus dorsi muscle for reconstruction in paraplegics. Hui et al published a case report of 2 patients with paraplegia—an 8-year-old with spina bifida and a 55-year-old with T10 paraplegia—who underwent respective free-tissue transfer of the latissimus dorsi muscle for a chronic lumbar wound and an excision of a Marjolin ulcer secondary to a sacral pressure ulcer.35 Both patients reported minor subjective deficits but no significant change in activities of daily living postoperatively. Osinga et al reported on 3 patients with thoracolumbar myelomeningoceles that were closed within 5 days after birth with reverse, turnover latissimus dorsi muscle flaps (2 unilateral, 1 bilateral).27 The patients with unilateral flaps required relaxing incisions (1 unilateral, 1 bilateral) that were covered with a synthetic wound dressing and subsequent delayed primary closure. Two patients (one with unilateral flaps and one with bilateral flaps) required split-thickness skin grafts to cover the latissimus flap. The mean age at follow-up was 11.7 years (range, 8–16 years). Shoulder function was assessed by using the modified Constant score, which consists of a subjective questionnaire and an objective appraisal of shoulder movement and

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abduction strength. While not included in the modified Constant score, dorsal extension and adduction strength were also measured. The results showed a slight decrease in dorsal extension, internal rotation, and adduction strength, but the authors noted that all patients were able to independently use their wheelchairs without restriction. On the basis of these observations, they concluded that harvesting the latissimus dorsi muscle in newborns does not significantly affect future activities of daily living. However, the conclusions derived from poorly designed and powered level IV and V evidence should be interpreted with caution. Notably, the modified Constant score was not designed or validated to be used to assess shoulder function in those younger than 20 years.33 Additionally, the modified Constant score cannot be used to assess arm adduction, the main function of the latissimus dorsi muscle. While Osinga et al did include arm adduction strength in the analysis with the contralateral arm that did not undergo surgery as a control,27 there are no established reference ranges for comparison, and a statistical analysis could not be performed because of the small sample size. Numerous random, geometric-pattern fasciocutaneous flaps have been described in the literature, including rotation, transposition, and advancement techniques.36–38 Most of these techniques have been based on modifications of V-Y, Z-plasty, bilobed, or rhomboid designs. However, without a well-defined axial vascular supply, these designs may be prone to ischemia and wound breakdown. With a thorough knowledge of the vascular anatomy of the back, flaps may be designed to incorporate the dorsal intercostal, lumbar, or superior gluteal arteries, as well as the musculocutaneous perforators of the latissimus dorsi to decrease the risk of ischemia.39–41 Advancements in the understanding of angiosomes and vascular perforators have also greatly increased the possibility of flap designs while minimizing donor site morbidity by preserving the underlying muscles. Since its initial description, the keystone perforator island flap has been used in all facets of reconstructive surgery.42 Gutman et al first applied this technique to close lumbosacral myelomeningoceles by designing the flap to capture dorsal intercostal and lumbar perforators.43 The longitudinal orientation of the flaps takes advantage of the increasing skin laxity toward the posterior axillary line.44 Horizontal movement is gained by releasing the lumbosacral fascia and V-Y closure of the right-angle limbs of the flap (Figure 15-9). Duffy et al reported on 6 patients treated with a pedicled superior gluteal artery perforator flap for closure of a myelomeningocele, with a mean defect size of 4.8 × 6.5 cm.45 After dural closure, Doppler ultrasonography was used to identify and mark the superior gluteal artery perforating vessels. The flap was elevated on the dominant perforator, which was dissected intramuscularly to its origin from the source vessel. Thorough dissection allowed the flap to be rotated and tunneled to the recipient site (Figure 15-10). The authors reported one case of moderate epidermolysis that healed without tissue necrosis and no major complications, such as dehiscence, infection, or need

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Figure 15-9. The bilateral keystone perforator island flap for closure of a myelomeningocele. From Park HS, Morrison E, Lo C, Leong J. An application of keystone perforator island flap for closure of lumbosacral myelomeningocele defects. Ann Plastic Surg. 2016;77(3):332–336.

Myelomeningocele

SGAP flap Cutaneous defect after elevated closure of the neural tube

SGAP flap with perforating vessels dopplered and marked

SGAP flap sutured into position Donor site closed

Figure 15-10. Pedicled superior gluteal artery perforator (SGAP) flap closure of a myelomeningocele. Based on Duffy FJ, Weprin BE, Swift DM. A new approach to closure of large lumbosacral myelomeningoceles: the superior gluteal artery perforator flap. Plast Reconstr Surg. 2004;114(7):1864–1870.

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for further revisions. The purported advantages include thick, well-vascularized padding and an offsetting suture line over the dural repair. However, this technique should be approached with caution, as the intramuscular dissection of a 1- to 2-mm perforator bundle is technically challenging. The propeller concept has also been applied to large thoracolumbar myelomeningoceles. Çöloğlu et al designed elliptical fasciocutaneous propeller flaps based on dorsal intercostal and lumbar artery perforators to close thoracolumbar defects (Figure 15-11). The flaps were inset with a single-layer closure, and the donor sites were closed primarily. In a series of 7 patients with a mean defect of 89.3 cm2 and mean follow-up of 10 months, transient venous congestion that resolved without intervention was noted, but there were no long-term complications.34

Outcomes

Myelomeningocele closure is a challenge to reconstructive surgeons and can be fraught with complications, including CSF leak, meningitis, wound infection, seroma, skin flap necrosis, and dehiscence. Seidel et al retrospectively investigated the complication rate in 65 myelomeningoceles repaired with primary skin re-approximation

Dorsal intercostal artery perforator propeller flap

Dorsal intercostal artery perforator propeller flap

A

Lumbar artery perforator propeller flap

B

Lumbar artery perforator propeller flap

Figure 15-11. Dorsal intercostal and lumbar artery perforator propeller flap closure of a thoracolumbar myelomeningocele. The design (A) and the closure patterns of the bilateral propeller flaps (B) are shown. Based on Çöloğlu H, Ozkan B, Uysal AC, Çöloğlu O, Borman H. Bilateral propeller flap closure of large meningomyelocele defects. Ann Plast Surg. 2014;73(1):68–73.

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(n = 48, 73.8%) or flap closure (n = 17, 26.2%).46 In their study, primary skin re-approximation resulted in 5 of 5 major complications and 9 of 13 minor complications. Major complications consisted of complete wound dehiscence that required revision (n = 2), a midline dehiscence with a CSF leak and meningitis (n = 1), and a case of cellulitis (n = 1) and a midline dehiscence with cellulitis (n = 1). All major complications occurred in defects larger than 18 cm2. Minor complications consisted of wound dehiscence, CSF leak, or subcutaneous fluid collections that resolved with nonsurgical management. On the basis of the prior study, Lien et al examined the effects of a multilayered closure in myelomeningocele defects to improve outcomes.47 A retrospective review was conducted of 45 consecutive patients who underwent myelomeningocele repair with a variety of multilayered closure techniques, with staggered, nonoverlapping suture lines. In their series, 15 of 45 patients (33%) experienced complications. Skin flap separation of less than 1 cm was the most common complication involving 9 of 45 patients (20%) and only required local wound care. Cellulitis occurred in 3 of 45 patients (7%), and it responded to antibiotics. Skin flap necrosis of less than 1 cm was noted in 1 patient (2%), which resulted in surgical debridement and revision. No patients developed a CSF leak, pseudo-meningocele, meningitis, seroma, hematoma, or major flap loss. The authors attributed their decreased complication rate, as compared with that of Seidel et al, to the use of a multilayered, staggered closure that would protect and cover the dural repair if dehiscence or necrosis occurred. Kobraei at al retrospectively reviewed 32 consecutive patients who underwent myelomeningocele closure by means of primary skin closure (n = 3), fasciocutaneous flaps (n = 13), or musculocutaneous flaps (n = 16) within 48 hours of birth.48 Primary skin closure was only used for defects smaller than 20 cm2, and fasciocutaneous and musculocutaneous flaps were used equally for defects larger than 20 cm2. With a mean of 27.3 months of follow-up (range, 6–88 months), 6 patients (18%) experienced complications. There were no complications in the primary closure group. In the fasciocutaneous group, 4 complications (30%) were noted to include a wound dehiscence, 2 occurrences of skin breakdown and necrosis, and a chronic nonhealing that required repeat surgery. In the musculocutaneous group, 2 complications (12%) were noted to include a gibbus deformity and a pseudo-meningocele. While there was a trend toward an association of fasciocutaneous flaps with complications, there was no statistically significant difference in complication rates between all groups. While the literature is replete with case series of techniques to close myelomeningoceles, there have been few high-quality studies to compare outcomes of different methods. As such, there is limited evidence to support the superiority of a singular technique. More studies are necessary to identify methods with the least complexity, morbidity, complications, and duration of hospitalization.

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226 Pediatric Plastic and Reconstructive Surgery for Primary Care REFERENCES

1. Tortori-Donati P, Rossi A, Cama A. Spinal dysraphism: a review of neuroradiological features with embryological correlations and proposal for a new classification. Neuroradiology. 2000;42(7):471–491 PMID: 10952179 https://doi.org/10.1007/s002340000325 2. Drolet B. Birthmarks to worry about. Cutaneous markers of dysraphism. Dermatol Clin. 1998;16(3):447–453 PMID: 9704204 https://doi.org/10.1016/S0733-8635(05)70245-X 3. Charney EB, Weller SC, Sutton LN, Bruce DA, Schut LB. Management of the newborn with myelomeningocele: time for a decision-making process. Pediatrics. 1985;75(1):58–64 PMID: 2578222 4. Kabagambe SK, Jensen GW, Chen YJ, Vanover MA, Farmer DL. Fetal surgery for myelomeningocele: a systematic review and meta-analysis of outcomes in fetoscopic versus open repair. Fetal Diagn Ther. 2018;43(3):161–174 PMID: 28910784 https://doi.org/10.1159/000479505 5. Laurence KM. The survival of untreated spina bifida cystica. Dev Med Child Neurol. 1966; 8(suppl 11):10–19 PMID: 5333891 https://doi.org/10.1111/j.1469-8749.1966.tb02172.x 6. Lorber J. Early results of selective treatment of spina bifida cystica. BMJ. 1973;4(5886):201–204 PMID: 4586035 https://doi.org/10.1136/bmj.4.5886.201 7. Lorber J. Selective treatment of myelomeningocele: to treat or not to treat? Pediatrics. 1974; 53(3):307–308 PMID: 4592677 8. Lorber J, Salfield SA. Results of selective treatment of spina bifida cystica. Arch Dis Child. 1981; 56(11):822–830 PMID: 6458248 https://doi.org/10.1136/adc.56.11.822 9. Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg. 2001;34(3):114–120 PMID: 11359098 https://doi.org/10.1159/000056005 10. Oakeshott P, Hunt GM, Poulton A, Reid F. Expectation of life and unexpected death in open spina bifida: a 40-year complete, non-selective, longitudinal cohort study. Dev Med Child Neurol. 2010;52(8):749–753 PMID: 20015251 https://doi.org/10.1111/j.1469-8749.2009.03543.x 11. McLone DG. Care of the neonate with a myelomeningocele. Neurosurg Clin N Am. 1998;9(1): 111–120 PMID: 9405769 https://doi.org/10.1016/S1042-3680(18)30284-5 12. Gamache FW Jr. Treatment of hydrocephalus in patients with meningomyelocele or encephalocele: a recent series. Childs Nerv Syst. 1995;11(8):487–488 PMID: 7585688 https://doi.org/10.1007/BF00334972 13. Pinto FC, Matushita H, Furlan AL, et al. Surgical treatment of myelomeningocele carried out at ‘time zero’ immediately after birth. Pediatr Neurosurg. 2009;45(2):114–118 PMID: 19307745 https://doi.org/10.1159/000209285 14. Zide BM, Epstein FJ, Wisoff J. Optimal wound closure after tethered cord correction. Technical note. J Neurosurg. 1991;74(4):673–676 PMID: 2002386 https://doi.org/10.3171/jns.1991.74.4.0673 15. Fiala TG, Buchman SR, Muraszko KM. Use of lumbar periosteal turnover flaps in myelomeningocele closure. Neurosurgery. 1996;39(3):522–525 PMID: 8875482 https://doi.org/10.1097/00006123199609000-00017 16. Arad E, Barnea Y, Gur E, et al. Paravertebral turnover flaps for closure of large spinal defects following tethered cord repair. Ann Plast Surg. 2006;57(6):642–645 PMID: 17122550 https://doi. org/10.1097/01.sap.0000235424.26158.e5 17. Patel KB, Taghinia AH, Proctor MR, Warf BC, Greene AK. Extradural myelomeningocele reconstruction using local turnover fascial flaps and midline linear skin closure. J Plast Reconstr Aesthet Surg. 2012;65(11):1569–1572 PMID: 22503313 https://doi.org/10.1016/j.bjps.2012.03.029 18. McCraw JB, Penix JO, Baker JW. Repair of major defects of the chest wall and spine with the latissimus dorsi myocutaneous flap. Plast Reconstr Surg. 1978;62(2):197–206 PMID: 353843 https://doi.org/10.1097/00006534-197808000-00007 19. Moore TS, Dreyer TM, Bevin AG. Closure of large spina bifida cystica defects with bilateral bipedicled musculocutaneous flaps. Plast Reconstr Surg. 1984;73(2):288–292 PMID: 6364193 https://doi.org/10.1097/00006534-198402000-00026 20. McDevitt NB, Gillespie RP, Woosley RE, Whitt JJ, Bevin AG. Closure of thoracic and lumbar dysgraphic defects using bilateral latissimus dorsi myocutaneous flap transfer with extended gluteal fasciocutaneous flaps. Childs Brain. 1982;9(6):394–399 PMID: 6756806 https://doi. org/10.1159/000120079 21. Ramirez OM, Ramasastry SS, Granick MS, Pang D, Futrell JW. A new surgical approach to closure of large lumbosacral meningomyelocele defects. Plast Reconstr Surg. 1987;80(6):799–809 PMID: 3685183 https://doi.org/10.1097/00006534-198712000-00007

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227 Chapter 15: Posterior Trunk Anomalies 22. Sarifakioglu N, Bingül F, Terzioglu A, Ates L, Aslan G. Bilateral split latissimus dorsi V-Y flaps for closure of large thoracolumbar meningomyelocele defects. Br J Plast Surg. 2003;56(3): 303–306 PMID: 12859933 https://doi.org/10.1016/S0007-1226(03)00115-2 23. Stevenson TR, Rohrich RJ, Pollock RA, Dingman RO, Bostwick J III. More experience with the “reverse” latissimus dorsi musculocutaneous flap: precise location of blood supply. Plast Reconstr Surg. 1984;74(2):237–243 PMID: 6463148 https://doi.org/10.1097/00006534-198408000-00011 24. Bostwick J III, Scheflan M, Nahai F, Jurkiewicz MJ. The “reverse” latissimus dorsi muscle and musculocutaneous flap: anatomical and clinical considerations. Plast Reconstr Surg. 1980;65(4):395–399 PMID: 7360805 https://doi.org/10.1097/00006534-198004000-00001 25. de Fontaine S, Gaede F, Berthe JV. The reverse turnover latissimus dorsi flap for closure of midline lumbar defects. J Plast Reconstr Aesthet Surg. 2008;61(8):917–924 PMID: 17884743 https://doi.org/10.1016/j.bjps.2007.05.005 26. Clark DH, Walsh JW, Luce EA. Closure of myelorachischisis defects with reverse latissimus dorsi myocutaneous flaps. Neurosurgery. 1982;11(3):423–425 PMID: 7133360 https://doi. org/10.1227/00006123-198209000-00015 27. Osinga R, Mazzone L, Meuli M, Meuli-Simmen C, von Campe A. Assessment of long-term donor-site morbidity after harvesting the latissimus dorsi flap for neonatal myelomeningocele repair. J Plast Reconstr Aesthet Surg. 2014;67(8):1070–1075 PMID: 24865618 https://doi. org/10.1016/j.bjps.2014.04.018 28. Zakaria Y, Hasan EA. Reversed turnover latissimus dorsi muscle flap for closure of large myelomeningocele defects. J Plast Reconstr Aesthet Surg. 2010;63(9):1513–1518 PMID: 19726259 https://doi.org/10.1016/j.bjps.2009.08.001 29. Scheflan M, Mehrhof AI Jr, Ward JD. Meningomyelocele closure with distally based latissimus dorsi flap. Plast Reconstr Surg. 1984;73(6):956–959 PMID: 6374709 https://doi. org/10.1097/00006534-198406000-00019 30. VanderKolk CA, Adson MH, Stevenson TR. The reverse latissimus dorsi muscle flap for closure of meningomyelocele. Plast Reconstr Surg. 1988;81(3):454–456 PMID: 3340683 https://doi. org/10.1097/00006534-198803000-00025 31. Yang JD, Huh JS, Min YS, Kim HJ, Park HY, Jung TD. Physical and functional ability recovery patterns and quality of life after immediate autologous latissimus dorsi breast reconstruction: a 1-year prospective observational study. Plast Reconstr Surg. 2015;136(6):1146–1154 PMID: 26267396 https://doi.org/10.1097/PRS.0000000000001769 32. Lee KT, Mun GH. A systematic review of functional donor-site morbidity after latissimus dorsi muscle transfer. Plast Reconstr Surg. 2014;134(2):303–314 PMID: 24732650 https://doi. org/10.1097/PRS.0000000000000365 33. Touil L, Fisher DM, Fattah AY. Re: ‘Assessment of long-term donor-site morbidity after harvesting the latissimus dorsi flap for neonatal myelomeningocele repair’. J Plast Reconstr Aesthet Surg. 2015;68(5):747–749 PMID: 25601426 https://doi.org/10.1016/j.bjps.2014.12.037 34. Çöloğlu H, Ozkan B, Uysal AC, Çöloğlu O, Borman H. Bilateral propeller flap closure of large meningomyelocele defects. Ann Plast Surg. 2014;73(1):68–73 PMID: 24918736 https://doi. org/10.1097/SAP.0b013e31826caf5a 35. Hui KC, Zhang F, Sutkin H, Wu P, Tingley S, Lineaweaver WC. Functional assessment of the shoulder following latissimus dorsi muscle donation in the handicapped. J Reconstr Microsurg. 1999;15(2):101–103 PMID: 10088919 https://doi.org/10.1055/s-2007-1000077 36. Davies D, Adendorff DJ. A large rotation flap raised across the midline to close lumbosacral meningomyelocoeles. Br J Plast Surg. 1977;30(2):166–168 PMID: 322779 https://doi. org/10.1016/0007-1226(77)90016-9 37. Ohtsuka H, Shioya N, Yada K. Modified Limberg flap for lumbosacral meningomyelocele defects. Ann Plast Surg. 1979;3(2):114–117 PMID: 543641 https://doi.org/10.1097/00000637197908000-00004 38. Kankaya Y, Sungur N, Aslan ÖÇ, et al. Alternative method for the reconstruction of meningomyelocele defects: V-Y rotation and advancement flap. J Neurosurg Pediatr. 2015; 15(5):467–474 PMID: 25679381 https://doi.org/10.3171/2014.12.PEDS14133 39. Minabe T, Harii K. Dorsal intercostal artery perforator flap: anatomical study and clinical applications. Plast Reconstr Surg. 2007;120(3):681–689 PMID: 17700119 https://doi. org/10.1097/01.prs.0000270309.33069.e5

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228 Pediatric Plastic and Reconstructive Surgery for Primary Care 40. Kiil BJ, Rozen WM, Pan WR, et al. The lumbar artery perforators: a cadaveric and clinical anatomical study. Plast Reconstr Surg. 2009;123(4):1229–1238 PMID: 19337091 https://doi. org/10.1097/PRS.0b013e31819f299e 41. Iacobucci JJ, Marks MW, Argenta LC. Anatomic studies and clinical experience with fasciocutaneous flap closure of large myelomeningoceles. Plast Reconstr Surg. 1996;97(7): 1400–1408 PMID: 8643723 https://doi.org/10.1097/00006534-199606000-00012 42. Behan FC. The Keystone Design Perforator Island Flap in reconstructive surgery. ANZ J Surg. 2003;73(3):112–120 PMID: 12608972 https://doi.org/10.1046/j.1445-2197.2003.02638.x 43. Gutman MJ, Goldschlager T, Fahardieh RD, Ying D, Xenos C, Danks RA. Keystone design perforator island flap for closure of myelomeningocele. Childs Nerv Syst. 2011;27(9):1459–1463 PMID: 21523390 https://doi.org/10.1007/s00381-011-1448-3 44. Park HS, Morrison E, Lo C, Leong J. An application of Keystone perforator island flap for closure of lumbosacral myelomeningocele defects. Ann Plast Surg. 2016;77(3):332–336 PMID: 26418773 https://doi.org/10.1097/SAP.0000000000000600 45. Duffy FJ Jr, Weprin BE, Swift DM. A new approach to closure of large lumbosacral myelomeningoceles: the superior gluteal artery perforator flap. Plast Reconstr Surg. 2004;114(7):1864–1868 PMID: 15577360 https://doi.org/10.1097/01.PRS.0000142741.11963.10 46. Seidel SB, Gardner PM, Howard PS. Soft-tissue coverage of the neural elements after myelomeningocele repair. Ann Plast Surg. 1996;37(3):310–316 PMID: 8883731 https://doi. org/10.1097/00000637-199609000-00013 47. Lien SC, Maher CO, Garton HJ, Kasten SJ, Muraszko KM, Buchman SR. Local and regional flap closure in myelomeningocele repair: a 15-year review. Childs Nerv Syst. 2010;26(8):1091–1095 PMID: 20195618 https://doi.org/10.1007/s00381-010-1099-9 48. Kobraei EM, Ricci JA, Vasconez HC, Rinker BD. A comparison of techniques for myelomeningocele defect closure in the neonatal period. Childs Nerv Syst. 2014;30(9):1535–1541 PMID: 24802545 https://doi.org/10.1007/s00381-014-2430-7

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CHAPTER

16

Aesthetic Surgery in the Pediatric Patient FREDERICK LUKASH, MD, FAAP, FACS

Introduction

It is not uncommon for adolescents to agonize over their appearance. By 17 years of age, teens have been exposed to more than 250,000 media bytes about beauty,1 and they find themselves under significant societal pressure to appear a certain way. While adults may seek plastic surgery to stand out, teens and adolescents have a strong desire to fit in. The emotions surrounding this desire are not dictated by age; being young does not negate a child’s feelings about his or her appearance. For an adolescent, standing out may not be easy, as it may lead to ridicule, insecurity, unwanted behaviors, and other ramifications. The pediatrician who has a longitudinal trusting relationship with the family is often able to be of assistance to the patient and family, in terms of navigating ethical and medical issues and providing support and follow-up. Plastic surgery in the pediatric population is, for the most part, structural in nature, and the rejuvenating value systems of adults do not apply. It can be easy to accept the concept of reconstructive surgery on the young, but any terms relating to “cosmetic” or “aesthetic” procedures can be considered frivolous, or even abhorrent. A high burden of responsibility can be placed on the pediatric plastic surgeon and pediatrician to sort out the shades of gray between purely reconstructive correction and what might be construed by some as cosmetic—when, in fact, the physical issue being corrected can have a significant effect on a child’s quality of life. This chapter provides insight into these concerns and illuminates the problems and dilemmas that face children in the high-pressure, socially driven, “beauty is success,” quick-fix world they have been thrust into. It will also address the ethical concerns surrounding pediatric corrective procedures. Data on outcomes of aesthetic surgery in the pediatric population, addressing the functional and psychological health of patients, are lacking. More research in this area would be valuable.

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When Is Plastic Surgery Recommended?

Children can be lured into believing that the unrealistic standards they are exposed to, whether it be in the media (especially social media), magazines, or stores, are the same standards they must live up to in order to be accepted and fit in. For some, it is a passing phase; for others, it becomes an obsession. Plastic surgeons are keenly aware of the pressures these children face as they make their way in this environment. Thus, from the outset, responsible pediatric plastic surgeons are not about promoting procedures or products. They serve to solve problems. In children, plastic surgery can be used to correct structural body and facial imbalances that may be affecting a child’s self-esteem or self-image. Children may notice that they look different from their peers in some substantial way, or they may even be taunted or bullied because of their appearance. It has been noted that “Once a child is aware of disfigurement, it predisposes [the child] to psychological disturbances, and correction restores psychic balance.”2 In terms of correcting physically noticeable imbalances, it has been stated, “Plastic surgery became a way to change rather than cope, and to alter rather than endure.”3 Many studies have documented the correlation among body image, the developing child, and self-esteem. In one study,4 it was noted that as early as nursery school and kindergarten, teachers and school staff gave more attention to the more attractive children than they did the unattractive children, and they were more tolerant of the attractive children’s transgressions. Others have commented that “attractive people are assumed to be smart, capable, personable, and better in the art of persuasion.”5 While “Intellects claim that beauty explains nothing, teaches nothing, solves nothing…outside this theoretical realm, beauty and appearance do rule.”6 In an article detailing the art of young children who underwent reconstructive surgery, it was noted that the children expressed their perceptions of themselves before and after intervention through nonverbal communication—their artwork.7

Risks, Benefits, and Outcomes

A grid for addressing the physical and emotional issues that result from medical concerns has been created, in concert with a psychologist, to aid in determining the indications and contraindications of plastic surgery procedures (Figure 16-1).8 From left to right, primary physical: secondary physical represents physical problems that cause further physical problems. One example is a deviated nasal septum that leads to breathing disorders, such as snoring and sleep apnea. Another example is macromastia, or mammary hypertrophy, accompanied by back pain, shoulder grooving from having to wear support bras all the time, and associated intertriginous rashes and dermatitis. The decision to proceed with surgery in such cases is

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Physical

Emotional

Primary

Secondary

Primary Physical

Secondary Physical

Primary Emotional

Secondary Emotional

Figure 16-1. The physical and emotional grid.

reasonably concrete, if the patient is experiencing physical effects of correctible issues. Primary physical: primary emotional defines physical problems that directly link to emotional issues. Situations that fall into this category are typically obvious to others, such as prominent ears, gynecomastia (ie, male patients with breasts), and breast asymmetries, as well as sagging skin that can result from massive weight loss. Although there are no consequences to one’s physical health from conditions such as these, the emotional devastation that can result from peer influences can be substantial. Surgical correction in such situations can be key to supporting a child’s emotional well-being. Psychological support may also be required, not only to help the pediatric patient cope with the initial problem but also to help the child adjust to the corrective surgery, recovery, and potential responses he or she may encounter from peers afterward. Emotional situations can have physical consequences, as indicated by the primary emotional: secondary physical category. Social isolation can cause adolescents to see themselves negatively, resulting in behaviors that have harmful physical effects. If teens perceive that they are overweight, a starvation diet may ensue, which can result not only in illness but also in substantial alterations in body image. A female patient who has large breasts but loses a great deal of weight may notice that her breasts have shrunk, and she may seek a breast augmentation or a spot liposuction treatment. The root cause for surgical treatment in these cases is often emotional, and providing psychological support to the patient is essential. The most critical category for any physician who deals with adolescents who are discussing or researching plastic surgery is primary emotional: secondary emotional. For emotional reasons, patients may seek out surgical treatment that may further exacerbate their emotional challenges. Depression, eating disorders, obsessive-compulsive behavior patterns, and the most dreaded trap, body dysmorphic disorder (BDD), a mental

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232 Pediatric Plastic and Reconstructive Surgery for Primary Care

illness that creates an obsessive focus on a perceived flaw in the patient’s appearance, may lead patients to seek out plastic surgery de novo or to “correct” an already surgically treated body part that has been successfully normalized previously. It is important to recognize aberrant psychological behavior patterns that now, in the world of the internet, can include obsessive searching for celebrity body parts. Counseling, not surgery, should be the driving force in treating this scenario. Patients like these should be referred to psychiatrists who treat adolescents if their behaviors are severe or worrisome or if there is suspicion for self-harm surrounding the issue.

Shades of Gray: Variations to the Grid There can be many variations on the physical and emotional grid, which can be described as “shades of gray.” In the author’s experience, a teenaged girl with macromastia who does not have family support to undergo a breast reduction may resort to severe weight loss to reduce her breast size, believing that is the only option within her control. Here, her family’s denial of a correctable and reasonable solution can lead to an emotional crisis that can result in another physical problem—anorexia or bulimia. Similarly, also in the author’s experience, a child with prominent ears may use ethyl cyanoacrylate to pin the ears back to his or her head to feel “normal” and damage the skin and cartilage by doing so. Not all teens with emotional angst over a body part have true BDD; it occurs in 1% of the population and is an often overused term. True BDD must be diagnosed by a psychiatrist. In the Diagnostic and Statistical Manual of Mental Disorders,9 BDD is defined as a preoccupation with a physical part that causes significant stress or impairment in social functioning and cannot be attributed to another diagnosis, such as anxiety disorder (which 10% of the general population has) or obsessive-compulsive disorder (which 3% of the general population has). There are situations in which a physical flaw is real, but it has been so internalized by the patient that it interferes with functioning. This does not represent true BDD, for which a multidisciplinary team approach is strongly advised and can be very beneficial. Management The famed diagnostician Sir William Osler once said, “Listen to your patient—he is trying to tell you what is wrong.” Not all adolescents who experience angst over their bodies have psychological issues, and they should not be dismissed by adults as being too immature to understand their own emotions. For some teens, their physical and emotional challenges can be very real, and reconstructive surgery may be the most reasonable option available. If pediatricians are willing to consult with responsible pediatric plastic surgeons about their patients with these issues, under the right circumstances, a beneficial quality-of-life– enhancing surgery can be considered.

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233 Chapter 16: Aesthetic Surgery in the Pediatric Patient

Ethical Concerns

The timing of surgery for the pediatric and adolescent population must be balanced among physical, emotional, and financial components. Parental consent is needed for patients who are minors. Therefore, all discussions about the need for surgery and timing are family discussions. The goal is to find the right time for surgery, once growth of the area of concern is complete; the ears are grown by age 6 years, noses are often done growing by 16 years of age, and breasts are done growing once puberty is complete. This is the physical component. Emotionally, the patient needs to understand the risks of surgery and the outcomes, especially during the healing process. Occasionally, the emotional angst is such that earlier decisions to undergo surgery are made knowing that, in time, revisions may be needed. In these situations, the emotional toll of waiting can outweigh the physical ramifications of future revisions. Certain corrective surgical procedures, such as a deviated nasal septum that affects breathing or very large breasts that restrict activities of daily living, can often be covered to the limits of individual insurance policies. Cosmetic components are never covered. Unfortunately, some structural issues, such as prominent ears, asymmetrical breasts, and gynecomastia, are rarely covered. Even though these conditions can cause an emotional toll, insurance companies reject coverage because there is no functional deficit.

Sample Clinical Scenarios

Here are some clinical scenarios that can serve as metaphors for approaching evaluation and referral to pediatric plastic surgeons who are adept in dealing with body image issues. Please note that plastic surgery should not be considered as a quick fix for immature adolescents who are not yet in control of their emotions. Rather, for patients who are dealing with very real physical challenges that a corrective procedure may alleviate, it can serve as a possible solution to a problem that is affecting that patient’s self-image.

Scenario 1: Prominent Ears A 10-year-old boy with prominent ears is failing academically and experiencing difficulties socially (Figure 16-2). His mother states that every day is an exercise in survival, once he’s outside the safety and security of his home. Repeated bullying comments have led the boy to resort to reclusive and sometimes aggressive behavior. This is a classic primary physical: primary emotional situation, where surgery (an otoplasty) will be the most beneficial solution. Additionally, supportive psychological care may be needed to help the boy overcome previous emotional scars and adjust to his new body image. Scenario 2: Mammary Hypertrophy A 16-year-old girl has mammary hypertrophy (Figure 16-3), wherein the breasts grow rapidly during puberty. Not only is the condition interfering

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234 Pediatric Plastic and Reconstructive Surgery for Primary Care

A

B

Figure 16-2. A 10-year-old boy with prominent ears (A) who underwent corrective otoplasty (B).

A

B

Figure 16-3. A 16-year-old girl with mammary hypertrophy (A) who underwent bilateral reduction mammoplasty (B).

with her athletics, but it is causing significant social problems. Shopping for clothes is a struggle, and she overhears her peers making salacious comments at school. She has begun dieting to shrink her breast size. This is a primary physical: primary emotional: secondary physical situation, in which an unaddressed physical problem is leading to an emotional crisis, which initiates another physical set of problems. Here, consideration of a bilateral reduction mammoplasty can be used to intercede and ameliorate an impending health-related catastrophe.

Scenario 3: Gynecomastia A 15-year-old boy has gynecomastia (Figure 16-4), but he is too embarrassed to mention it to his pediatrician, who does not address the condition because it is not causing physical harm. The boy’s parents, who are unaware of his angst about his appearance, think of him as a problem child because he

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A

B

Figure 16-4. A 15-year-old boy with gynecomastia (A) who underwent surgical correction (B).

refuses to go to summer camp or on outings, such as to the beach. The boy will not take his shirt off to change for gym class at school, and he is rapidly becoming the class bully so that other kids will leave him alone and not taunt him. This is a primary physical: primary emotional case, where the emotional ramifications of the condition can easily be reversed by bringing together patient, family, and pediatrician for awareness and insight.

Scenario 4: Breast Asymmetry A 17-year-old girl has breast asymmetry—one breast is large and saggy and the other is small and tubular (Figure 16-5). No bra will fit her, and cruising the mall with her friends to try on clothes is out of the question. She is maturing into advanced social situations with college on the horizon, and depression is setting in because her breasts do not look “normal.” Insurance companies will not cover a corrective procedure because they consider it to be cosmetic in nature, so her parents are hesitant to pursue a surgical solution. This is a primary physical: secondary physical problem (physical problems that cause further physical problems) that may lead to an emotional issue.

A

B

Figure 16-5. A, A 17-year-old girl with breast asymmetry. One breast is large and saggy and the other is small and tubular. B, The same patient after corrective surgery.

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236 Pediatric Plastic and Reconstructive Surgery for Primary Care

A

B

Figure 16-6. A 14-year-old girl with a nose “bump” and a drooping tip before (A) and after (B) corrective surgery.

Scenario 5: Large Nose A 14-year-old girl is small in stature but has a large nose (Figure 16-6). Embarrassed by her nose, she feels like a “freak” and looks for excuses to stay home from school. She wants to look like her classmates, who seem to be enjoying a healthy social life. Her concerned parents took her to see a psychologist, but working with the psychologist did not alleviate her social angst. This situation is a classic primary physical: primary emotional problem. Scenario 6: Underdeveloped Breasts An 18-year-old high school senior has finished puberty but has underdeveloped breasts (Figure 16-7). She feels masculine and, as college is approaching, is having anxiety issues that are manifesting in an eating disorder. As well as recommending psychological counseling, consideration for a breast augmentation procedure may be warranted in the transitional period between high school and college. This scenario is a primary physical: primary emotional situation that may become a secondary physical issue. She is finished with puberty. This transitional period after high school is the social time between new endeavors. It is a fresh start, so new acquaintances can see her with her current appearance, and not her previous one.

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A

B

Figure 16-7. A, An 18-year-old woman has completed puberty but has not developed breasts. B, The same patient is shown after a breast augmentation procedure. REFERENCES

1. Ojeida A. Teen Decisions: Body Image. San Diego, CA: Greenhaven Press; 2002 2. Maliniac J. Sculpture in the Living: Rebuilding the Face and Form by Plastic Surgery. New York, NY: Lancet Press; 1934 3. Cooke MacGregor FM. Transformation and Identity: The Face and Plastic Surgery. New York, NY: Quadrangle Press; 1974 4. Dion KK, Berscheid E. Physical attractiveness and peer perception among children. Sociometry. 1974;37(1):1–12 https://doi.org/10.2307/2786463 5. Jeffes S. Appearance is Everything: The Hidden Truth Regarding Your Appearance and Discrimination. Pittsburgh, PA: Sterling House; 1998 6. Etcoff N. Survival of the Prettiest: The Science of Beauty. New York, NY: Doubleday; 1999 7. Lukash FN. Children’s art as a helpful index of anxiety and self-esteem with plastic surgery. Plast Reconstr Surg. 2002;109(6):1777–1786 PMID: 11994573 https://doi.org/10.1097/00006534200205000-00001 8. Gerstein M. Physical and emotional grid. In: Lukash F, ed. The Safe and Sane Guide to Teenage Plastic Surgery. Dallas, TX: BenBella Books; 2010 9. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. 5th ed. Arlington, VA: American Psychiatric Publishing; 2013

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Index Page numbers followed by f indicate a figure. Page numbers followed by t indicate a table.

A

B

Abdominal wall anomalies associated anomalies with, 203–204 clinical presentation of, 201–204 diagnosis of, 204 embryology of, 200 epidemiology of, 200 future research on, 210–211 introduction to, 199 management of adjunctive therapy, 209 medical, 205–206 prenatal, 205 principles and goals in, 205, 211 reduction, 206–207 surgical, 207–209 surgical outcomes for, 209–210 Abdominoplasty, 208 Acanthosis nigricans, 169–170 Aesthetic surgery in pediatric patients ethical concerns with, 233 introduction to, 229 risks, benefits, and outcomes of, 230–232 management of, 232 shades of gray in, 232 sample clinical scenarios in, 233–237 when to recommend, 230 African Americans, cleft lip and palate in, 3 Airway compromise after cleft repair, 13 Albright syndrome, 168 All-terrain vehicle (ATV) accidents, 84 Alveolar repair, 12–13 Amblyopia, 24, 107, 109, 111 American Academy of Pediatrics, 81 Animal bites, 177–179 Ankyloblepharon, 111 Apert hand, 146–148 Apertognathia, 77 Apert syndrome, 4, 18 Arnold-Chiari malformation, 213 Arthrogryposis, 149 Arthrogryposis multiplex congenita, 149 Asians cleft lip and palate in, 3 slate gray nevi in, 168

“Back to Sleep” campaign, 20 Bannayan-Riley-Ruvalcaba disease, 150 Becker nevus, 171 Beckwith-Wiedemann syndrome, 204 Bell palsy, 116 Bicoronal suture craniosynostosis, 24, 26f Bicoronal synostosis, 24, 26f Bifid uvula, 4, 5f Bilateral sagittal split osteotomy, 75–76 Bimaxillary osteotomies, 76 Bite wounds, 177–179 Bladder exstrophy, 199, 200, 203 Bleeding after cleft lip and palate repair, 13 from orthognathic surgery, 78 Blepharophimosis eyelid syndrome, 106–108, 111 Border digit syndactyly, 144 Brachydactyly, 141, 146 Brachymelia with brachydactyly, 141 Branchial cleft cyst, sinus, and fistula, 129 management of, 135–136 patient presentation in, 132, 133f Breast anomalies breast asymmetry, 190–194, 235 fibroadenoma, 194–196 gynecomastia, 186–190, 234–235 introduction to, 183 macromastia, 183–186, 233–234 mammary hypertrophy, 233–234 underdeveloped breasts, 236, 237f Breast asymmetry case scenario, 235 diagnosis of, 192 epidemiology of, 191–192 introduction to, 190–191 management of, 193 patient presentation in, 192 postsurgical complications of, 194 Burn injuries complications and outcomes of, 162–164 diagnosis of, 156–158 epidemiology of, 153 introduction to, 153 management of, 158–162 patient presentation in, 154–155

239

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240 Index

C Café au lait spots, 167–168 Camptodactyly, 144–145 Capillary malformation, 39t, 45–46 Cardiovascular defects and abdominal wall anomalies, 204 Carpal synostoses, 146 Carpenter syndrome, 32 Cat bites, 177–178 Cephalometric analysis and model, 70–73 Cerebrospinal fluid (CSF) leak, 85, 87, 216–217 CHARGE syndrome, 111 Chotzen syndrome, 18, 19, 31–32, 107 Cleft hand, 146–148 Cleft lip and palate, 1–15 complications after repair of, 13–15 diagnosis of, 4–6 epidemiology of, 3 introduction to, 1–2 management of alveolar repair, 12–13 in infancy, 6–7 lip repair, 7–8 nasal repair, 11–12 palate repair, 9–10, 11f patient presentation in, 3–4 Clinodactyly, 146 Cloacal exstrophy, 199, 203 CLOVES syndrome, 48 Coloboma, 111 Complete syndactyly, 144 Complex abdominal wall defect, 203–204 Complex hand anomalies, 146–148 Complex syndactyly, 144 Complicated syndactyly, 144 Computed tomography (CT) in cephalometric analysis and modeling, 72–73 for craniosynostosis, 21 for facial fractures, 82, 85–86, 92, 93 Condylar fractures, 94–95 Congenital hemangioma, 39, 40f, 44 Congenital melanocytic nevi (CMN), 165–167 Congenital muscular torticollis, 131 management of, 136 patient presentation in, 134 Constricted ear, 52, 55–56, 61–63 Constriction ring syndrome, 148 Corticosteroids facial fractures and, 97 infantile hemangioma and, 42–43 Cosmetic procedures. See Aesthetic surgery in pediatric patients Craniofacial fractures. See Facial fractures

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Craniosynostosis diagnostic studies of, 19–21 etiopathogenesis of, 19 introduction to, 17–18 long-term management optimization in, 34 nonsyndromic, 18, 22–28 patient presentation in, 19 surgical management of, 32–33 syndromic, 18, 28–32 Cross-face nerve graft, 118–119 Crouzon syndrome, 18, 29–30 Cryotherapy, epidermal nevus, 171 Cryptophthalmos, 112 Cryptotia, 56–58 Cup ear. See Constricted ear Cutaneous scars and scar management, 179–180 Cutaneous trauma bite wounds, 177–179 lacerations, 176–177

D Decreased ear size, 55 Deep partial-thickness burns, 157 Dental compensation, 69–70 Dermoid cyst, 131, 172–174 patient presentation in, 133–134 Diabetes, orthognathic surgery and, 67 Diplopia, 90–91 Dog bites, 177–179 Donnai-Barrow syndrome, 204 Dorsal dimelia, 143 Dorsal-ventral axis anomalies dorsal dimelia and hypoplastic/aplastic nail, 143 nail-patella syndrome, 142 Dysplasia, 150–151

E Ear deformities, congenital constricted ear, 52, 55–56, 61–63 cryptotia and Stahl ear, 52, 56–59, 61–63 epidemiology of, 51–52 introduction to, 51 microtia, 58 reconstruction of, 64–65 timing of reconstruction in, 62–63 non-microtia, surgical treatment of, 62–64 nonsurgical treatment of, 59–60 otoplasty timing of ear reconstruction and, 61 timing of surgical treatment of non-microtia and, 62

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241 Index patient presentation and spectrum of deformities in, 52–53 preauricular branchial vestiges, 53 prominent ear, 53–55, 233 timing of surgery for, 60–61 Ear reconstruction, 61 Ectopia cordis, 199, 203 Ectropion, 108, 110–111 Embryology of abdominal wall anomalies, 200 Emotional issues with aesthetic surgery, 230–232 Entropion, 109–110 Epiblepharon, 110 Epicanthal folds, 106 Epidermal nevus, 171–172 Epidermoid cyst, 131 patient presentation in, 133–134 Ethical concerns with aesthetic surgery, 233 Euryblepharon, 108, 111 Eyelid anomalies anesthesia considerations for, 112 ankyloblepharon, 111 blepharophimosis eyelid syndrome, 107–108, 111 coloboma, 111 cryptophthalmos, 112 developmental, 106–112 ectropion, 108, 110–111 entropion, 109–110 epiblepharon, 110 epicanthal folds, 106 euryblepharon, 108 eyelid retraction, 109 introduction to, 105 plastic surgery evaluation for, 106 ptosis, 108–109 telecanthus, 106–107 visual development and, 105–106 Eyelid retraction, 109

F Facial cleft. See Cleft lip and palate Facial fractures complications of long-term, 100 postsurgical, 98–99 diagnosis of, 84–86 epidemiology of, 82–83 introduction to, 81–82 management of mandibular, 94–96 maxillary and midface, 91–93 nasal, 93–94 orbital, 89–91 panfacial, 96 skull and forehead, 86–89 zygomaticomaxillary, 91

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patient presentation in, 83–84 postsurgical care for, 96–97 Facial paralysis diagnosis of, 117 epidemiology of, 114–116 introduction to, 113–114 management, 117–118 patient presentation in, 116–117 surgical procedures complications of, 125–126 cross-face nerve graft and gracilis muscle transplant combination for, 118–119 muscle transplant, 120–123 muscle transplant innervated by motor nerve to masseter muscle, 124–125 patient selection for, 118 Failure of axis formation/differentiation in the entire upper limb, 139–142 dorsal-ventral axis anomalies, 142 proximal-distal outgrowth anomalies, 141 radial-ulnar axis anomalies, 142 in the hand plate, 142–143 unspecified axis, 144–148 complex hand anomalies, 146–148 skeletal deficiencies, 146 soft-tissue anomalies, 144–145 FGFR2 and FGFR3 genes, 28–31 Fibroadenoma of breast, 192f, 194–196 First-degree burns, 156 Forehead fractures, 86–89 FOXL2 gene, 107 Fractures, facial. See Facial fractures Full-thickness burns, 157

G Gastrointestinal defects and abdominal wall anomalies, 204 Gastroschisis, 199, 201–202, 203t, 207, 210 Gender, cleft lip and palate and, 3 Genitourinary defects and abdominal wall anomalies, 204 Gershoni-Baruch syndrome, 204 Gliomas, 174 Gracilis muscle transplant, 118 Growing skull fracture, 87 Gynecomastia case scenario, 234–235 diagnosis of, 187 epidemiology of, 186 introduction to, 186 management of, 189–190 patient presentation in, 187 postsurgical complications of, 190

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242 Index

H

L

Hand anomalies, congenital deformations arthrogryposis, 149 constriction ring syndrome, 148 trigger digits, 149–150 dysplasia, 150–151 introduction to, 139 malformation failure of axis formation/differentiation, unspecified axis, 144–148 failure of axis formation/differentiation in the entire upper limb, 139–142 failure of axis formation/differentiation in the hand plate, 142–143 Harlequin eye deformity, 24, 26f Hemangioma congenital, 39, 40f, 44 infantile, 39, 40f, 40–44 Hematoma, septal, 93–94 Hernia reduction, 206 Horner syndrome, 109 Humeroradial synostosis, 142 Hypertelorism, 106 Hypertrophic scars and keloids, 179 Hypoplastic/aplastic nail, 143

Lacerations, 176–177 Lambdoid suture synostosis, 25, 27, 28f, 32 Laser therapy acanthosis nigricans, 170 café au lait spots, 168 capillary malformation, 45–46 epidermal nevus, 171 infantile hemangioma, 43–44 tuberous sclerosis complex, 175 Le Fort fractures, 91–92 Le Fort I osteotomy, 74–75, 75f, 77, 99 Lidding, ear, 55 Lip repair, 7–8 Lop ear. See Constricted ear Low ear position, 55 LUMBAR association, infantile hemangioma, 42 Lymphatic malformation, 46–47, 131 patient presentation in, 134

I Incomplete syndactyly, 144 Infantile hemangioma, 39, 40f, 40–44 Infection, postsurgical, facial fractures, 98 Inflammatory linear verrucous epidermal nevus (ILVEN), 171 Informed consent, 32 Intermaxillary fixation (IMF), 95–96 Intersegmental deficiency of hand, 141 Intracranial pressure (ICP), in craniosynostosis, 19 Intraoral vertical ramus osteotomy, 76 Isolated cleft lip, 4, 5f

J Jaw surgery. See Orthognathic surgery Juvenile rheumatoid arthritis, orthognathic surgery and, 67

K Kaposiform hemangioendothelioma, 39, 40f, 44–45 Kasabach-Merritt syndrome, 44 Keloids, 179 Kirner deformity, 146 Klinefelter syndrome, 187 Klippel-Trénaunay syndrome, 39, 49

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M Macrodactyly, 150–151 Macromastia case scenario, 233–234 diagnosis of, 184–185 epidemiology of, 184 introduction to, 183 management of, 185 patient presentation in, 184 postsurgical complications of, 185–186 Maffucci syndrome, 150 Magnetic resonance imaging (MRI) congenital melanocytic nevi (CMN), 166 craniosynostosis, 21 dermoid cyst, 172 infantile hemangioma, 41 Malformations arteriovenous, 47–48 hand failure of axis formation/differentiation, unspecified axis, 144–148 failure of axis formation/differentiation in the entire upper limb, 139–142 failure of axis formation/differentiation in the hand plate, 142–143 lymphatic, 46–47, 131, 134 vascular capillary, 39t, 45–46 lymphatic, 46–47 venous, 47 Mammary hypertrophy, 233–234 Mandibular fractures, 94–96 Mandibular surgery. See Orthognathic surgery Marginal reflex distance, 109

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243 Index Maxillary constriction, 77 Maxillary fractures, 91–93 Melanocytic lesions acanthosis nigricans, 169–170 café au lait spots, 167–168 congenital melanocytic nevi (CMN), 165–167 nevi of Ota and Ito, 169 nevus spilus, 168 slate gray nevus, 168 Spitz nevus, 169 Meningitis, 87–88 Meningocele, 214–215 Mercedes Benz deformity, 25 Metacarpal synostoses, 146 Metopic suture synostosis, 23 Microform cleft lip, 4, 5f Microtia, 58 basic steps in reconstruction of, 64–65 timing of reconstruction for, 62–63 Midface fractures, 91–93 Midline cervical cleft, 130 management of, 136 patient presentation in, 133 Milroy disease, 150 Möbius syndrome, 116 Mongolian spot, 168 Motor vehicle accidents (MVAs), 83–84, 89 Motor vehicle collisions (MVCs), 83 Muenke syndrome, 31, 31f Multiple suture synostoses, 27–28 Muscle transplant, facial, 120–123 innervated by motor nerve to masseter muscle, 124–125 Musculoskeletal defects and abdominal wall anomalies, 204 Myelomeningocele, 214–215 surgical repair of, 217–224 Myofascial pain, 67

lymphatic malformation, 46–47, 131, 134 management of, 135–136 midline cervical cleft, 130, 133 patient presentation in, 132–134 plunging ranula, 130, 133 thymic cyst, 131, 134 thyroglossal duct cyst, 129, 130f, 132 types of, 129–131 Nerve damage after orthognathic surgery, 78–79 Neurofibroma, 174–175 Neurofibromatosis 1, 168, 174 Nevi of Ota and Ito, 169 Nevus comedonicus, 171 Nevus sebaceus, 170–171 Nevus spilus, 168 Noninvoluting congenital hemangioma (NICH), 44 Non-melanocytic lesions dermoid cyst, 131, 133–134, 172–174 epidermal nevus, 171–172 neurofibroma, 174–175 nevus sebaceus, 170–171 Noonan syndrome, 107, 175–176 pilomatricoma, 172 tuberous sclerosis complex, 175 Non-microtia ear deformities, 62–64 Nonsurgical treatment, of congenital ear deformities, 59–60 Nonsyndromic craniosynostosis bicoronal suture craniosynostosis, 24, 26f defined, 18 lambdoid suture synostosis, 25, 27 metopic suture synostosis, 23 multiple suture synostoses, 27–28 sagittal suture synostosis, 22–23 unicoronal suture craniosynostosis, 23–24, 25f, 26f Noonan syndrome, 107, 175–176

N

O

Nail-patella syndrome, 142 Nasal fractures, 93–94 Nasal reconstruction, 236 Nasal repair, 11–12 Native Americans, cleft lip and palate in, 3 Neck masses branchial cleft cyst, sinus, and fistula, 129, 132, 133f complications of, 136–137 congenital muscular torticollis, 131, 134 dermoid cyst, epidermoid cyst, teratoma, 131, 133–134 diagnosis of, 134–135 epidemiology of, 131–132 introduction to, 129

Ollier disease, 150 Omphalocele, 199, 201–202, 203t, 206–207, 210 Omphalomesenteric duct malformation, 203 Open bite, 77, 85, 94–95, 99 Orbital examination, 84–85 Orbital fractures, 89–91 long-term complications of, 100 postsurgical complications of, 98–99 Orthognathic surgery approaches to commonly encountered problems in, 77–78 bilateral sagittal split osteotomy, 75–76 bimaxillary osteotomies, 76 complications of, 78–79 effectiveness of, 79

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244 Index Orthognathic surgery (continued) intraoral vertical ramus osteotomy, 76 introduction to, 67, 68f Le Fort I osteotomy, 74–75, 75f, 77, 99 preparation for cephalometric analysis and models in, 70–73 overview of, 73 physical examination in, 68–70 surgically assisted rapid palatal expansion, 74 surgical technique in, 73–76 Otoplasty, 61–62 postoperative management after, 63–64

P Palate repair, 9–10, 11f Panfacial fractures, 96 Papilledema in craniosynostosis, 19 with intracranial extension of dermoids, 173 Paralysis, facial. See Facial paralysis Parkes Weber syndrome, 39t, 49 Patent urachus, 199, 203 Pediatric patients, AAP definition of, 81 Pentalogy of Cantrell, 199, 203t, 204 Pfeiffer syndrome, 18, 30 PHACE association, infantile hemangioma, 42–43 Pharmacotherapy facial fractures, 97 infantile hemangioma, 42–43 kaposiform hemangioendothelioma, 45 scar management, 180 tuberous sclerosis complex, 175 Phleboliths, 47 Physical examination for cleft lip and palate, 3–4 for facial fractures, 84–86 for gynecomastia, 187 for macromastia, 184–185 for orthognathic surgery, 68–70 Physical issues with aesthetic surgery, 230–232 Pierre Robin sequence, 4 Pilomatricoma, 172 Plastic closure of abdominal wall anomalies, 208 Plunging ranula, 130, 135 patient presentation in, 133 Polydactyly, radial and postaxial, 142–143 Port-wine stain. See Capillary malformation Positional plagiocephaly, 20 Postaxial polydactyly, 142–143 Posterior palate, 1–2 Posterior trunk anomalies classification and presentation of spinal dysraphism, 213–215

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evaluation of, 215–216 indications for surgical management of, 216–217 introduction to, 213 outcomes, 224–225 prognosis in, 216 surgical technique for additional, 218–224 preferred, 217–218 Preauricular branchial vestiges, 52–53 Primary palate, 1 Prominent ear, 53–55, 233 Propranolol, infantile hemangioma and, 43 Proteus syndrome, 150 Protrusion, ear, 55 Proximal-distal outgrowth anomalies, 141 Prune-belly syndrome, 199, 200, 202–203 Ptosis, 32, 84–85, 107–109, 186–188, 188f Pulmonary embolism, Klippel-Trénaunay syndrome and, 49 Pyogenic granuloma, 39, 40f, 45

R RAB23 gene, 32 Radial longitudinal deficiency, 142 Radial polydactyly, 142–143 Radial-ulnar axis anomalies radial longitudinal deficiency, ulnar longitudinal deficiency, ulnar dimelia, radioulnar synostosis, and humeroradial synostosis, 142 radial polydactyly, triphalangeal thumb, and postaxial polydactyly, 142–143 Radiography cephalometric analysis and modeling, 70–73 craniosynostosis, 20 Radioulnar synostosis, 142 Rapidly involuting congenital hemangioma (RICH), 44 Resection, infantile hemangioma, 44 Rule of 9s, 157, 158f

S Sagittal suture synostosis, 22–23 Scaphocephaly, 22–23 Scar management, 179–180 Scleroderma, orthognathic surgery and, 67 Sclerosing agents for neck masses, 136 Sclerotherapy lymphatic malformation, 46 venous malformation, 47 Second-degree burns, 156 Septal hematoma, 93–94 Septic shock syndrome, 94 Serum markers in abdominal wall anomalies, 204

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245 Index Short lower face, 78 Sight development, 105–106 Simple syndactyly, 144 Sistrunk procedure, 135 Skeletal class III occlusion, 77 Skeletal deficiencies, hand, 146 Skin and soft-tissue lesions cutaneous scars and scar management, 179–180 cutaneous trauma, 176–179 introduction to, 165 melanocytic acanthosis nigricans, 169–170 café au lait spots, 167–168 congenital melanocytic nevi (CMN), 165–167 nevi of Ota and Ito, 169 nevus spilus, 168 slate gray nevus, 168 Spitz nevus, 169 non-melanocytic dermoid cyst, 131, 133–134, 172–174 epidermal nevus, 171–172 neurofibroma, 174–175 nevus sebaceus, 170–171 Noonan syndrome, 107, 175–176 pilomatricoma, 172 tuberous sclerosis complex, 175 Skin grafting for abdominal wall anomalies, 208 Skull fractures, 86–89 Slate gray nevus, 168 Soft-tissue anomalies, hand, 144–145 Speckled lentiginous nevus, 168 Speech therapy after cleft repair, 14–15 Spina bifida cystica, 215 Spina bifida occulta, 215 Spinal dysraphism, 213–215 Spindle cell nevus, 169 Spitz nevus, 169 Stahl ear, 52, 56–59, 61–63 Sturge-Weber syndrome, 39t, 49 Superficial partial-thickness burns, 156 Surgically assisted rapid palatal expansion, 74 Sutures, skull, 17 Symbrachydactyly, 141, 147, 148f Synarthrosis, 17 Syndactyly, 144–145 Syndromic craniosynostosis Apert syndrome, 4, 18, 28–29 Carpenter syndrome, 32 Chotzen syndrome, 18, 19, 31–32 Crouzon syndrome, 18, 29–30 defined, 18 Muenke syndrome, 31 Pfeiffer syndrome, 18, 30 Synpolydactyly, 146–148

PedPS_Index_239-246.indd 245

T Tags, skin, 53 Telecanthus, 106–107 Temporomandibular joint (TMJ) disorder, 67, 69 Teratoma, 131 management of, 136 patient presentation in, 133–134 Third-degree burns, 157 Thrombocytopenia, kaposiform hemangioendothelioma and, 45 Thrombophlebitis, Klippel-Trénaunay syndrome and, 49 Thymic cyst, 131, 137 management of, 136 patient presentation in, 134 Thyroglossal duct cyst, 129, 130f, 137 management of, 135 patient presentation in, 132 Timolol, 42 Total body surface area (TBSA) in burns, 156–157, 159–160 Transverse deficiency of hand, 141 Trauma burn injuries. See Burn injuries cutaneous, 176–179 facial paralysis with, 116 fractures and. See Facial fractures Trigger digits, 149–150 Trigonocephaly, 23 Triphalangeal thumb, 142–143 TSC1 and TSC2 genes, 175 Tuberous sclerosis complex, 175 Tumors, vascular congenital hemangioma, 39, 40f, 44 infantile hemangioma, 39, 40f, 40–44 kaposiform hemangioendothelioma, 39, 40f, 44–45 pyogenic granuloma, 39, 40f, 45 TWIST 1 gene, 31

U Ulnar dimelia, 142 Ulnar longitudinal deficiency, 142 Ultrasonography abdominal wall anomalies, 202, 204 craniosynostosis, 20–21 dermoid cyst, 173 infantile hemangioma, 41 neck masses, 134–135 pilomatricoma, 172 Underbite, 77 Unicoronal suture craniosynostosis, 23–24, 25f Upper pole of the ear, treatment of, 63

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246 Index

V Vascular anomalies arteriovenous malformation, 47–48 capillary malformation, 39t, 45–46 CLOVES syndrome, 39t, 48 congenital hemangioma, 39, 39t, 40f, 44 infantile hemangioma, 39, 39t, 40f, 40–44 introduction to, 39, 40f kaposiform hemangioendothelioma, 39, 39t, 40f, 44–45 Klippel-Trénaunay syndrome, 39t, 49 lymphatic malformation, 39t, 46–47 Parkes Weber syndrome, 39t, 49 pyogenic granuloma, 39, 39t, 40f, 45 Sturge-Weber syndrome, 39t, 49 venous malformations, 39t, 47 Vascular malformation overgrowth syndromes CLOVES syndrome, 39t, 48 Klippel-Trénaunay syndrome, 39t, 49 Parkes Weber syndrome, 39t, 49 Sturge-Weber syndrome, 39t, 49 Venous malformations, 47 Verrucous epidermal nevus, 171 Vertical maxillary excess, 78

PedPS_Index_239-246.indd 246

Virchow’s law, 19 Visual development, 105–106 Voiding cystourethrography, 202 Voiding urosonography, 203

W Whitaker classification of craniofacial anomalies, 18 White population, cleft lip and palate in, 3 Wound care abdominal wall anomalies, 209 bite wounds, 177–179 burn, 159–162 cleft repair, 13–14 facial fractures, 97 infantile hemangioma, 42 lacerations, 176–177 scar management and, 179–180

Z Zona pellucida, 6 Z-plasty procedures, 136 Zygomaticomaxillary fractures, 91

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AUTHOR EDITORS American Academy of Pediatrics Peter J. Taub, MD, MS, FAAP, FACS, and Timothy W. King, MD, PhD, MSBE, FAAP, FACS Section on Plastic Surgery With contributions from the leading experts in the field, Pediatric Plastic and Reconstructive Surgery for Primary Care provides primary care pediatricians and other health practitioners who care for children essential support on caring for children who are faced with plastic and reconstructive surgery–related issues. Sixteen chapters with 200+ full-color photos provide superbly illustrated, authoritative guidance on when to treat and when to wait, the timing of corrective surgeries in pediatric patients, and surgical strategies and complications. Nonsurgical management and brief descriptions of how corrective procedures are performed are discussed, which is helpful to primary care pediatricians when counseling patients and their families and conducting long-term patient follow-up.

The book includes chapters on Cleft Lip and Palate Craniosynostosis Vascular Anomalies Congenital Ear Deformities Orthognathic Surgery Pediatric Facial Fractures Eyelid Anomalies Facial Paralysis

• • • • • • • •

Pediatric Neck Masses Congenital Hand Anomalies Pediatric Burn Injury Skin and Soft-Tissue Lesions Breast Anomalies Abdominal Wall Anomalies Posterior Trunk Anomalies Aesthetic Surgery in the Pediatric Patient

For other pediatric primary care resources, visit the American Academy of Pediatrics at shop.aap.org.

ISBN 978-1-61002-394-8

90000>

Peter J. Taub, MD, MS, FAAP, FACS Timothy W. King, MD, PhD, MSBE, FAAP, FACS

• • • • • • • •

Pediatric Plastic and Reconstructive Surgery for Primary Care

Pediatric Plastic and Reconstructive Surgery for Primary Care

Pediatric Plastic and Reconstructive Surgery for Primary Care

EDITORS

Peter J. Taub, MD, MS, FAAP, FACS Timothy W. King, MD, PhD, MSBE, FAAP, FACS

9 781610 023948

AAP

PPARSFPC - COVER SPREAD.indd All Pages

3/12/20 11:29 AM