Evaluation and Management of Cleft Lip and Palate A Developmental Perspective Evaluation and Management of Cleft Lip and Palate A Developmental Perspective David J. Zajac, PhD, CCC-SLP Linda D. Vallino, PhD, CCC-SLP/A 5521 Ruffin Road San Diego, CA 92123 e-mail: [email protected] Website: http://www.pluralpublishing.com Copyright © by Plural Publishing, Inc. 2017 Typeset in 10.5/13 Palatino by Flanagan’s Publishing Services, Inc. Printed in Korea by Four Colour Print Group All rights, including that of translation, 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, recording, or otherwise, including photocopying, recording, taping, Web distribution, or information storage and retrieval systems without the prior written consent of the publisher. For permission to use material from this text, contact us by Telephone: (866) 758-7251 Fax: (888) 758-7255 e-mail: [email protected] Every attempt has been made to contact the copyright holders for material originally printed in another source. If any have been inadvertently overlooked, the publishers will gladly make the necessary arrangements at the first opportunity. Names: Zajac, David J., author. | Vallino, Linda D., author. Title: Evaluation and management of cleft lip and palate : a developmental perspective / David J. Zajac, Linda D. Vallino. Description: San Diego : Plural, [2017] | Includes bibliographical references and index. Identifiers: LCCN 2015042352| ISBN 9781597565516 (alk. paper) | ISBN 1597565512 (alk. paper) Subjects: | MESH: Cleft Lip. | Cleft Palate. | Speech Disorders — prevention & control. | Velopharyngeal Insufficiency. Classification: LCC RD525 | NLM WV 440 | DDC 617.5/22 — dc23 LC record available at http://lccn.loc.gov/2015042352 Contents Preface vii Acknowledgments ix Contributors xi Part I. Fundamentals 1 1 Orofacial and Velopharyngeal Structure and Function 3 Jamie Perry and David J. Zajac 2 Clefts of the Lip and Palate 23 3 Syndromes and Associated Anomalies 49 Part II. Birth to Age Three 111 4 Feeding the Newborn 113 5 Presurgical and Surgical Management 129 6 Hearing and Otologic Management 151 7 Early Linguistic Development and Intervention 177 Nancy J. Scherer Part III. Early to Middle School Age 191 8 Speech and Resonance Characteristics 193 9 Assessment of Speech and Velopharyngeal Function 227 10 School-Based Intervention 281 Dennis M. Ruscello 11 Secondary Management of Velopharyngeal Inadequacy 319 12 Alveolar Cleft Repair 339 Joseph A. Napoli Part IV. Adolescents and Adults 355 13 Maxillary Advancement 357 Joseph A. Napoli and Linda D. Vallino 14 The Adult With Cleft Lip and Palate 379 Glossary of Terms 391 Index 401 v Preface This book is intended to be a concise, To help achieve these goals, the mate- practical, and evidence-based text on cleft rial in the book is presented in a devel- lip and palate and related craniofacial opmental framework that emphasizes the disorders for advanced undergraduate most critical needs of the individual from students, graduate students, and profes- birth to adulthood. This organizational sionals in speech-language pathology. approach has both practical and concep- Students and professionals in related dis- tual advantages. Practically, it allows the ciplines such as dentistry, medicine, psy- reader to access information more read- chology, and social work also may find ily according to the age and presenting this book useful in providing information condition of the individual (i.e., birth, lip on individuals with craniofacial condi- repair, palate repair, alveolar cleft repair tions. Cleft palate with or without cleft or bone grafting, maxillary advancement). lip is a congenital defect that varies both Conceptually, it chronicles the lifelong in its severity and impact on facial and impact of craniofacial birth defects on the oral structures and communication. Treat- individual and elucidates the timing and ment of individuals is typically a long and rationale of surgical, dental, and behav- often-complicated process, extending into ioral interventions. early adulthood and beyond, that is best Part I provides necessary funda- accomplished in a team setting. In addition mentals for the student and professional. to the speech-language pathologist, other Chapter 1 reviews anatomy and physiol- team members typically include a plastic ogy of the facial, oral, and velopharyn- surgeon, dentist, orthodontist, oral and geal structures that are affected by clefts. maxillofacial surgeon, otolaryngologist, Chapter 2 describes the types and causes audiologist, geneticist, social worker, and of clefts with an emphasis on embryologi- psychologist. Even though all members cal development and classification. The of the team contribute to the habilitation controversy regarding timing of palate of the individual througout his or her repair is discussed from the perspective life span, certain team members assume of speech and language development. critical roles at specific times of life. The Chapter 3 provides an overview of genet- purpose of the book is to provide the stu- ics and the terminology used to catego- dent and professional in speech-language rize and identify congenital anomalies. pathology the information needed to (a) A select group of craniofacial anomalies evaluate and treat communication disor- most likely to be encountered by the pro- ders associated with cleft palate regard- fessional is reviewed. less of their primary place of employment Part II focuses on evaluation and (i.e., craniofacial team, hospital, school, or management of the individual from birth private practice), and (b) understand the to 3 years of age. Chapter 4 describes nor- complex — and sometimes controversial — mal feeding physiology, feeding prob- surgical and dental management of indi- lems associated with cleft palate, and viduals across the life span. approaches to facilitate feeding prior to vii viii Evaluation and Management of Cleft Lip and Palate: A Developmental Perspective palate repair. Chapter 5 describes presur- viduals affected in the United States is gical and surgical management of cleft lip relatively low. It is not unusual, therefore, and palate. Chapter 6 covers the almost for speech-language pathologists working universal occurrence of otitis media with in the schools to infrequently see children effusion and conductive hearing loss that with clefts. It is our hope that the materi- occurs in infants with cleft palate and cur- als in this book will be a valuable resource rent methods of management. Chapter 7 for school-based clinicians when they do reviews early linguistic development in encounter children with clefts. Some mate- infants with cleft palate and intervention rial traditionally covered in other texts is, strategies before and after palate repair. unfortunately, omitted in the book. Due to Part III focuses on evaluation and a goal to be concise and follow a develop- management of the individual from age mental framework, separate chapters on 3 throughout the middle school years. craniofacial team function and psycho- Chapter 8 describes the resonance, nasal social aspects of individuals with cranio- emission, articulation, voice, fluency, and facial anomalies are not included. These intelligibility characteristics of children are obviously important areas. We have, with repaired cleft palate. Chapter 9 pro- however, interweaved these materials in vides detailed coverage of perceptual various chapters throughout the book. assessment and an overview of instru- Maternal reactions to an infant born with mental assessment techniques. Chapter 10 a cleft are covered in Chapter 4, learning describes practical approaches to treat- disabilities of children with cleft palate ing children with articulation problems and treatment collaboration models with in the school setting. Chapter 11 presents teams are reviewed in Chapter 10, and the an overview of behavioral, surgical, and last chapter deals exclusively with quality prosthetic options to manage velopharyn- of life issues facing the adult. geal inadequacy that persists following Finally, the book has been written initial palate surgery. Chapter 12 describes with a goal to be concise and cite evidence- the orthodontic preparation and surgical based sources to support intervention correction of clefts of the alveolus. approaches. In some areas, there is little Part IV focuses on evaluation and man- objective evidence available to guide agement of adolescents and adults with cleft clinical decision making. In those areas, palate. Chapter 13 describes the rationale we note the lack of evidence and suggest and timing of maxillary advancement in directions for future research. It is our adolescents to improve facial aesthetics, hope that this book will not only inform dental occlusion, and articulation. Chapter but also challenge clinicians in speech- 14 discusses issues facing adult patients. language pathology to provide the best Although clefts of the lip and palate evidence-based evaluation and manage- are among the most frequently occurring ment of individuals with craniofacial birth defects, the actual number of indi- anomalies as possible. Acknowledgments I am indebted to many who have contrib- I strived to emulate relative to scholar- uted either directly or indirectly to this ship and research productivity. Others at book. While at the University of Pitts- the University of North Carolina at Cha- burgh, I was fortunate to have Betty Jane pel Hill who have been influential include McWilliams as an early mentor. Dr. McWil- Amelia Drake, Tim Turvey, Gerald Sloan, liams, the first and longtime director of Wolf Losken (also at Pittsburgh), and the Cleft Palate Center, was an inexhaust- John van Aalst. They were instrumental ible clinician and researcher who first and in helping me understand surgical treat- foremost put the welfare of the patient ment of patients. above all else. I have been truly influ- I am most appreciative of Linda Val- enced by her wisdom, knowledge, and lino, co-author of the book, and Jamie caring. Campbell C. Yates and Raymond Perry, Nancy Scherer, Dennis Ruscello, Linville also were mentors at Pittsburgh. and Joseph Napoli, contributors to the Professor Yates, chair and emeritus pro- book. They are outstanding clinicians fessor of mechanical engineering, volun- and researchers in their respective areas. teered his time to establish aerodynamic This book would not be what it is without assessment procedures at the center. Pro- their input. A special thanks is also due fessor Yates helped me to understand and to Marziye Eshghi, Jacqueline Dorry, and appreciate the nuances of fluid dynamics Ramona Hutton-Howe for their help with when applied to the complex geometry of figures, tables, and photography. the human upper airways. Raymond Lin- On a personal note, I am indebted to ville, a mentor and friend, was influential my family for their support and encour- in guiding my early research endeavors. agement. They graciously endured count- Many colleagues have been influen- less dinnertime book conversations — and tial during the past 20 years at the Uni- dinners without me — during the writing versity of North Carolina at Chapel Hill. of the book. I am grateful to my loving Donald W. Warren, the Craniofacial Cen- wife, Robin, children, Jared and Rachel, ter’s first director, was a prolific researcher daughter-in-law Elizabeth, son-in-law, who applied aerodynamic techniques Matt, and grandson, William, for their to the evaluation of nasal and palatal encouragement. The thought of William’s function. Don was a role model whom wonderful smile was the best inspiration. — David J. Zajac Chapel Hill, North Carolina This book would not have been possible ing and doctoral degree at the University without the encouragement, mentor- of Pittsburgh, under the direction of Dr. ship, and friendship of some very special Betty Jane McWilliams. Dr. McWilliams people. Like David, I received my train- was the quintessential teacher, researcher ix