John M. Graham Glenis K. Scadding Peter D. Bull Editors Pediatric ENT # John M. Graham Glenis K. Scadding Peter D. Bull Editors Pediatric ENT With 355 Figures and 79 Tables 123 Mr. John M. Graham Royal National Throat, Nose and Ear Hospital 330 Gray’s Inn Road London WC1X 8DA United Kingdom Dr. Glenis K. Scadding Royal National Throat, Nose and Ear Hospital 330 Gray’s Inn Road London WC1X 8DA United Kingdom Mr. Peter D. Bull Sheffield Children’s Hospital Western Bank Sheffield S10 2TH United Kingdom Library of Congress Control Number: 2007923510 ISBN 978-3-540-33038-7 Springer Berlin Heidelberg New York This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilm or in any other way, and storage in data banks. 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Editor: Marion Philipp, Heidelberg, Germany Desk Editor: Irmela Bohn, Heidelberg, Germany Reproduction, typesetting and production: LE-TEX Jelonek, Schmidt & Vöckler GbR, Leipzig, Germany Cover design: Frido Steinen-Broo, EStudio, Calamar, Spain Printed on acid-free paper 24/3180/YL 5 4 3 2 1 0 Foreword Children are special! This book is special! with the contributions of intensive care specialists, pedia- Hospitals for sick children were founded in the first tricians and anesthetists have all contributed to raise the decade of the 20th century, and even before. Dedicated quality of patient care in pediatric otorhinolaryngology. nurses and doctors with an interest in children were pio- The biannual international congresses of the European neers in those child-friendly hospitals, although diagnos- Society of Pediatric Otorhinolaryngology (ESPO) have tic and therapeutic techniques were modest. provided a prominent worldwide forum, presenting the Currently, medical and surgical disciplines cooper- latest research and evaluation of new and current prac- ate with paramedical professionals and specially trained tice, and acting as a meeting point for all colleagues with nurses in the treatment of children. The contribution of an interest in treating children with otorhinolaryngol- professionals with an expertise in biochemistry and bio- ogy-related pathology. physics has also become essential, since routine proce- It is my great pleasure to welcome and recommend dures and research are dependent on advanced and ad- this book. We should be most grateful to John Graham, vancing technology. former president of the ESPO, Glenis Scadding, eminent The scope of pathologists has broadened to the cel- in the field of allergy, and Peter Bull a distinguished pe- lular and subcellular level. Molecular medicine is mak- diatric laryngologist, and appreciate their great efforts to ing progress. Information technology has contributed to present a state of the art account of our specialty. They the development of disciplines like epidemiology, clinical have brought together a group of eminent authors, pre- epidemiology and genetics. Modern imaging techniques senting recent advances in all aspects of pediatric oto- produce data pertinent to the anatomy and metabolism rhinolaryngology. You will certainly appreciate their up- of pathological processes. to-date information and critical evaluation of the field of Pediatric otorhinolaryngology has largely benefited pediatric otorhinolaryngology. from these new technologies and data produced by re- cent research. Moreover, interdisciplinary teamwork in Carel Verwoerd audiology, vestibular pathology, neuro-otology, speech Secretary General and hearing disorders, and allergy and genetics, together European Society of Pediatric Otorhinolaryngology (ESPO) Preface This book has been written to fill a need for a basic but clinics, able to accommodate the activities that children comprehensive text dealing with all aspects of paediat- usually indulge in, and not threatening to them. Wards ric ENT. It is aimed at trainees in our specialties who are staffed by nurses trained in childcare as well as in ENT developing an interest in the paediatric aspects of their nursing are now considered essential, and need to include work, at established ENT surgeons and audiologists who play areas and accommodation for parents. Many aspects have decided to specialise in this most enjoyable subspe- of the care of children with ENT problems involve several cialty, or who wish to know more about paediatric ENT, different disciplines: a multidisciplinary team. The rôles and as a work of reference for those who encounter par- of team members need both defining and acknowledging. ticular problems in their paediatric patients. The ENT doctor needs to accept and to encourage this. The scope of paediatric ENT has grown over recent The book includes some general chapters dealing with years to the extent that doctors may now subspecialise in these aspects of the specialty. paediatric otology, laryngology or rhinology rather than With contributors from many different countries, the dealing with all aspects of the field. It was therefore neces- use and the spelling of medical terms inevitably vary. In sary to cover a large number of different topics in consid- general we have accepted both standard US and standard erable depth. British spellings, trying to keep to one or the other within We have been truly fortunate in our authors, who the confines of separate chapters. Some medical terms come from many parts of Europe, the USA and South appear to be interchangeable: tracheotomy and tracheos- Africa. All share our great enthusiasm for our specialty, tomy are an example; some authors hold strong views on as we hope the reader will recognise. We are grateful to this subject and we have encouraged them to use the term them all for working to a short timescale. This has allowed they prefer. us to put together a fairly large multi-author book in a We end by acknowledging the support we have re- relatively short time, with up to date contributions. Our ceived from our publishers and the staff at Springer Ver- authors are active in international academic work and lag. We have also received considerable help from friends meet each other regularly at the international gatherings and colleagues, including those authors of chapters who of the European Society of Pediatric Otorhinolaryngol- have taken the time and trouble to review other chapters ogy, American Society of Pediatric Otolaryngology and and offer advice. Kevin Gibbin and Will Hellier in partic- Society for Ear, Nose and Throat Advances in Children, ular have given much time and sound advice. Emily Gra- as well as at the many national paediatric ENT societies ham has contributed great skill and some hours of work that have been formed in the last 25 years. in manipulating tables, figures and all kinds of illustration Paediatric ENT has developed, during the careers of to fit the requirements of several chapters. the editors, from being considered a relatively simple and Every text of this kind inevitably includes a sincere minor part of our professional life into a broad, complex vote of heartfelt thanks to the spouses and family of the and often difficult subject. It no longer deals primarily editors. We now understand why. Sharing one’s marriage with tonsillectomy, adenoidectomy and the insertion of with a book is not necessarily something to be recom- ventilation tubes. However, these common procedures mended. We, too, would like to thank Sandy Graham, remain important and this book contains detailed analy- Hilary Bull and Adam Pearson for their patience, forbear- ses of these well-known aspects of paediatric ENT life, ance, much sound advice and constructive criticism. whose rôles are still being defined. Letting a book loose on the public could be thought There are some variations in the depth of cover among an irresponsible act. We hope this is not the case and that the 47 chapters of this book. Some surgical topics are you, our readers, find its contents informative, useful and dealt with clearly but fairly briefly. In contrast, fields such absorbing. as immunology, allergy and genetics, in which there con- It is inevitable in a book of this nature that errors will tinues to be quite rapid scientific progress, needed more have crept in. For these we apologise and crave your in- detail. dulgence. It is a commonplace understanding that children are not small adults. Similarly, much of paediatric ENT is John M. Graham significantly different from the practice of ENT in adults. Glenis K. Scadding Children benefit from being looked after in dedicated Peter D. Bull Introduction Each patient is a part of a society: successful care given its gross domestic product from the economic sequelae of to that patient adds value to that society. Pediatric oto- communication disorders. laryngology encompasses the traditional purposes of A second substantial impact related to pediatric otolar- medicine in the prevention and curing of illness. It also yngology is in the relationship between communication focuses especially on hearing, speech, gustation and the disorders and juvenile crime. The prevalence of commu- sense of smell, swallowing and respiration. Children able nication disorders is many times greater in populations to express themselves will say “Thank you, I can hear bet- of juvenile delinquents than in the general population. ter, smell and taste better, breathe better, sleep better, look Communication disorders appear to act synergistically better…”. Pediatric otolaryngology has particular impor- with diminished economic and social resources in lead- tance because it deals with communication by language, ing to violent behavior and crime. through the vehicles of hearing, voice and speech. The pediatric otolaryngologist’s role in the prevention, This is critically important in two ways. The first relates cure and care of communication disorders is therefore to the economic basis of our society – the way in which one of the most important fields of medical care for the we make our livelihoods; this underwent fundamental 21st century – the age of communication. change during the last half of the 20th century (Ruben 2000). In earlier periods we depended largely on manual Robert J. Ruben, MD, FACS, FAAP labor. Today we depend upon communication skills, me- Distinguished University Professor diated through hearing, voice, speech and language, and Albert Einstein College of Medicine directly linked to literacy. Pediatric otolaryngology con- Department of Otolaryngology tributes to the economic basis of society by facilitating the Montefiore Medical Center development of the skill of communication in individual children. Comparing three different countries (Ruben 2003): one very highly dependent upon communica- tion skills (the Netherlands), one highly dependent upon 1. Ruben RJ (2000) Redefining the survival of the fittest: com- communication skills (the USA), one a developing nation munication disorders in the 21st century. Laryngoscope less dependent upon communication skills (the Philip- 110:241–245 pines), reveals that all three nations are adversely affected 2. Ruben RJ (2003) Valedictory – why pediatric otorhinolar- economically and socially by communication disorders. yngology is important. Int J Pediatr Otorhinolaryngol 67: It is estimated that the USA loses between 2.5 and 3% of S53–61 Contents 1 A Paediatric Overview of Children 3 Anaesthesia for Paediatric Seen in the ENT Outpatient Department 1 ENT Surgery ............................. 11 Helen Bantock Ian Barker Introduction: Paediatric Consultation .... 1 Introduction ........................... 11 Development in the First Five Years of Life 2 General Considerations ................ 12 Children with Special Needs .............. 2 Anaesthesia for Children ............. 12 Speech and Language Delay or Disorder 2 Anaesthesia for the Newborn ......... 12 Autistic Spectrum ...................... 2 Hypoxia ........................... 12 Global Delay ........................... 3 Hypothermia ....................... 12 Specific Learning Difficulties ........... 3 Hypoglycaemia ..................... 12 Syndromes ............................... 3 Intravenous Fluid Therapy Down Syndrome ...................... 3 for Children ......................... 12 Behaviour Difficulties .................. 3 Specific Conditions .................... 13 Attention Deficit Hyperactivity Post-Tonsillectomy Bleeding ......... 13 Disorder ............................. 3 Assessment ......................... 13 Social Difficulties ...................... 3 Resuscitation ....................... 13 Types of Abuse ....................... 3 Anaesthesia ........................ 13 Child Abuse in the Context Airway Endoscopy ................... 13 of an ENT Clinic ..................... 4 Indications ......................... 13 Alerting Signs of Non-Accidental Induction .......................... 14 Injury and Abuse in a Clinic Setting ... 4 Maintenance ......................... 14 What to do if Abuse is Suspected ....... 4 Laser Surgery .......................... 14 Links with Local Services ................. 4 Obstructive Sleep Apnoea .............. 15 Primary Care Team .................... 4 Anaesthetic Equipment ................. 15 The Child Development Team .......... 4 Endotracheal Tubes .................. 15 The Education Service ................. 4 Laryngeal Mask Airway .............. 16 Child and Adolescent Mental Health Pulse Oximeter ...................... 16 Services ................................ 5 Limitations ........................ 16 Social Services ......................... 5 Carbon Dioxide Monitor ............. 16 Non-Statutory Organisations ........... 5 4 The Evolution of Speech 2 Nursing Aspects of Paediatric ENT ...... 7 and Language .......................... 19 Rosalind Wilson and Judith Barton John F. Stein Introduction ............................. 7 Introduction ........................... 19 Communication ......................... 7 Is Language Innate or Learnt? .......... 20 Consent for Surgery ...................... 8 Human Evolution ...................... 20 Staffing .................................. 8 Left-Sided Specialisation ............. 20 The Physical Environment ................ 8 Emotional Vocalisations .............. 21 Tracheostomy ............................ 8 Mirror Neurones ..................... 21 Parents .................................. 9 Bipedalism and the Aquatic Ape Day Surgery .............................. 9 Hypothesis ........................... 22 Pain Control ........................... 10 Larynx Descent ...................... 22 XII Contents Brain Enlargement ................... 23 7 Genetics of Non-Syndromic Deafness 47 Right-Handedness ................... 23 Maria Bitner-Glindzicz Selective Advantages of Speech ....... 23 Gesture and Speech .................. 24 Introduction ........................... 47 Writing .............................. 24 Investigation of the Aetiology Conclusion ............................ 24 of Hearing Loss ........................ 48 Reasons .............................. 48 Protocol ............................. 48 5 Evidence-Based Management History .............................. 48 of Speech and Language Delays ....... 27 Examination ......................... 48 Amy McConkey Robbins Investigation ......................... 48 Genetic Hearing Loss ................ 49 Introduction ........................... 27 Recessive Genes: Common/ Milestones for Communication Recognizable Types of Development .......................... 28 Non-Syndromic Genetic Hearing Loss 50 Normal Variation in Language GJB2 (Connexin26) ................. 50 Learning ............................. 28 SLC26A4 (Pendred and Non- Normal Variation versus Syndromic Enlarged Vestibular Communication Delay ............... 28 Aqueduct) ......................... 52 Communication Milestones OTOF (Auditory Neuropathy) ....... 53 in the First Year of Life ............... 29 Dominant Genes: Common/ Clinical Use of Table 5.1 by Physicians 31 Recognizable Types of Non- Speech and Language Intervention Syndromic Genetic Hearing Loss ..... 53 is Correlated with Young Age WFS1 (Dominant Low-Frequency at Identification ........................ 32 Sensorineural Hearing Loss) ........ 53 Hearing Loss can be Identified COCH (Menière-Like Symptoms) ... 54 and Treated as Early as the Newborn Period ................................. 32 Differential Diagnosis of Several 8 ENT-Related Syndromes ................ 57 Communication Disorders ............. 33 David Albert and Fiona Connell Association Between Some Speech/ Language Characteristics and Reading Definition ............................. 58 Disability .............................. 34 Introduction ........................... 58 Evidence-Based Approach Resources .............................. 59 to the Management of Children’s Speech Syndromes of Particular Relevance and Language Development ............ 34 to the ENT Clinician ................... 59 Down Syndrome ..................... 59 Genetics ........................... 59 6 Paediatric Voice ......................... 37 ENT Features ...................... 59 Mark E. Boseley General Features ................... 59 and Christopher J. Hartnick Pierre Robin Sequence ............... 59 Genetics ........................... 59 Introduction ........................... 37 ENT Features ...................... 60 Anatomy ............................... 38 Treacher Collins Syndrome ........... 60 Gross ................................ 38 Genetics ........................... 60 Histology ............................ 38 ENT Features ...................... 60 Diagnostic Techniques ................. 39 General Features ................... 60 Physical Examination ................ 39 Goldenhar Syndrome ................ 60 Voice Outcome Surveys .............. 39 CHARGE Syndrome ................. 60 Common Voice Pathologies ............ 39 Genetics ........................... 60 Vocal Fold Nodules .................. 39 ENT Features ...................... 60 Voice Results Following General Features ................... 61 Laryngotracheal Reconstruction ...... 42 Genetics ........................... 61 Recurrent Respiratory Papillomatosis 43 ENT Features ...................... 61 Vocal Fold Paralysis .................. 43 General Features ................... 61 Contents XIII Branchio-oto-renal Syndrome ........ 62 ENT Features ...................... 68 Genetics ........................... 62 General Features ................... 68 ENT Features ...................... 62 Waardenburg Syndrome .............. 68 General Features ................... 62 Genetics ........................... 68 22q11.2 Deletion Syndrome .......... 62 ENT Features ...................... 68 Genetics ........................... 62 General Features ................... 68 ENT Features ...................... 62 Jervell and Lange-Nielsen Syndrome .. 68 General Features ................... 62 Genetics ........................... 68 Craniosynostosis Syndromes ......... 63 ENT Features ...................... 69 Genetics ........................... 63 General Features ................... 69 ENT Features ...................... 63 Gorlin Syndrome .................... 69 General Features ................... 63 Genetics ........................... 69 Alport Syndrome .................... 63 ENT Features ...................... 69 Genetics ........................... 63 General Features ................... 69 ENT Features ...................... 63 Holoprosencephaly .................. 69 General Features ................... 63 Genetics ........................... 69 Pendred Syndrome ................... 63 ENT Features ...................... 69 Usher Syndrome ..................... 63 General Features ................... 69 Genetics ........................... 63 Mucopolysaccharidoses .............. 69 Alstrom Syndrome ................... 65 Genetics ........................... 69 Genetics ........................... 65 ENT Features ...................... 69 ENT Features ...................... 65 General Features ................... 69 General Features ................... 65 Foetal Alcohol Syndrome ............. 70 Syndromes Less Commonly Seen Genetics ........................... 70 by ENT Surgeons ....................... 65 ENT Features ...................... 70 Achondroplasia ...................... 65 General Features ................... 70 Genetics ........................... 65 Foetal Cytomegalovirus Syndrome .... 70 ENT Features ...................... 65 Features ........................... 70 General Features ................... 65 Congenital rubella syndrome ......... 70 Beckwith-Wiedemann Syndrome ..... 65 Features ........................... 70 Genetics ........................... 65 Acknowledgements .................. 70 ENT Features ...................... 65 General Features ................... 65 Neurofibromatosis Type 2 ............ 66 9 EXIT – Antenatal (Pre-natal) Genetics ........................... 66 Diagnoses and Management ........... 73 ENT Features ...................... 66 Gavin Morrison General Features ................... 66 Noonan Syndrome ................... 67 Introduction ........................... 73 Genetics ........................... 67 Pre-natal Diagnosis .................... 74 ENT Features ...................... 67 Diagnostic Ultrasound Features ...... 74 General Features ................... 67 Congenital High Airway Obstruction Osteogenesis Imperfecta ............. 67 Syndrome ............................ 75 Genetics ........................... 67 Foetal MRI Scanning ................. 75 ENT Features (Type 1) .............. 67 The EXIT Procedure ................... 76 General Features (Type 1) ........... 67 Indications for the EXIT Procedure ... 76 Prader-Willi Syndrome ............... 67 Techniques of the EXIT Procedure .... 76 Genetics ........................... 67 Tips and Pitfalls of EXIT ............. 77 ENT Features ...................... 67 Spontaneous Onset of Labour ....... 77 General Features ................... 67 Cystic Hygroma .................... 78 Stickler Syndrome .................... 68 Maintaining Placento-Foetal Genetics ........................... 68 Circulation ......................... 78 ENT Features ...................... 68 Loss of Placento-Foetal Circulation .. 78 General Features ................... 68 Risks to Mother .................... 78 Turner Syndrome .................... 68 Outcomes of EXIT ................... 78 Genetics ........................... 68 Counselling and Ethical Issues .......... 78