The following tables can be used as a guideline in planning surgery. These numbers have been derived from Marshall Parks, with modifications from the surgical experience of Kenneth W. Wright. The numbers are only a guide and should be modified as necessary.

BINOCULAR SURGERY

Esotropia MROU LR OU ET Recession ET Resection* 1S~ 3.0mm 1S~ 3.Smm 20~ 3.Smm 20~ 4.Smm 2S~ 4.0mm 2S~ S.Smm 30~ 4.Smm 30~ 6.0mm 3S~ S.Omm 3S~ 6.Smm 40~ S.Smm 40~ 7.0mm so~ 6.0mm so~ 8.0mm 60~ 6.Smm 70~ 7.0mm

* When a lateral rectus resection is clone for residual esotropia after large medial rectus recession (6.00 mm or larger), these numbers should be lowered.

Exotropia LR OU MROU XT Recession XT Resection

1S~ 4.0mm 1S~ 3.0mm 20~ S.Omm 20~ 4.0mm 2S~ 6.0mm 2S~ S.Omm 30~ 7.0mm 30~ S.Smm 3S~ 7.Smm 3S~ 6.0mm 40~ 8.0mm 40~ 6.Smm so~ 9.0mm

MONOCULARSURGERY

Esotropia Exotropia MR LR LR MR ET Recession Resection XT Resection Resection 1S~ 3.0 mm ...... 3.S mm 1S~ 4.0 mm ...... 3.0 mm 20~ 3.S mm ...... 4.0 mm 20~ S.O mm ...... 4.0 mm 2S~ 4.0 mm ...... S.O mm 2S~ 6.0 mm ...... 4.S mm 30~ 4.S mm ...... S.S mm 30~ 6.S mm ...... S.O mm 3S~ S.O mm ...... 6.0 mm 3S~ 7.0 mm ...... S.S mm 40~ S.S mm ...... 6.S mm 40~ 7.S mm ...... 6.0 mm so~ 6.0 mm ...... 7.0 mm so~ 8.0 mm ...... 6.S mm 60~ 6.S mm ...... 7.S mm 70~ 7.0 mm ...... 8.0 mm THREE-MUSCLE SURGERY

For large horizontal deviations, surgery on three musdes may be planned for the primary operation. The amount of surgery can be judged from the above tables. This works especially well in adults, where one musde can be placed on an adjustable suture. The adjustable suture should be clone on the eye for which two musdes are being operated.

VERTICAL NUMBERS

A rule of thumb for vertical surgery is 3 prism diopters of vertical correction for every millimeter of recession. Inferior rectus reces• sions are notorious for late overcorrections; therefore, consider using nonabsorbable sutures or long-lasting absorbable sutures. Superior rectus recessions for dissociated vertical deviation (DVD) must be large, with the minimum recession of approximately 5 mm and a maximum of 9 mm (fixed-suture technique).

KESTENBAUM PROCEDURE FOR

Face-turn to the RIGHT To correct the right face-turn (eyes shifted to a left null point), move the eyes to primary position by moving both eyes to the right.

LEFT EYE RIGHT EYE

Degree of Recess Resect Recess Resect Face-Turn LR MR MR LR

Classic <20° 7mm 6mm 5mm 8mm Parks 30° 9mm 8mm 6.5mm 10mm Classic +40% 45° 10mm 8.5mm 7mm 11 mm Classic +60% 50° 11 mm 9.5mm 8mm 12.5 mm Pediatric and Strabismus Second Edition Springer Science+Business Media, LLC Pediatric Ophthalmology and Strabismus Second Edition

Editors

Kenneth W. Wright, MD Clinical Professor of Ophthalmology, University of Southern California-Keck School of Meclicine Director, Pediatric Ophthalmology Research and Education Cedars-Sinai Medical Center Los Angeles, California, USA

Peter H. Spiegel, MD Vision Professionals, Palm Springs, California Inland Eye Clinic, Hemet, California Attending Physician, Loma Linda University Medical Center Loma Linda, California, USA

With 838 IDustrations in 1197 Parts, 694 in Full Color

Illustrators Timothy C. Hengst, CMI Susan Gilbert, CMI Faith Cogswell

Springer Kenneth W. Wright, MD Peter H. Spiegel, MD Clinical Professor of Ophthalmology Vision Professionals University of Southern California• Palm Springs, CA Keck School of Medicine Inland Eye Clinic Director Hemet, CA Pediatric Ophthalmology Research and Education Attending Physician Cedars-Sinai Medical Center Loma Linda University Medical Center Los Angeles, CA, USA Loma Linda, CA, USA [email protected] [email protected]

Library of Congress Cataloging-in-Publication Data

Pediatric ophthalmology and strabismus / editor, Kenneth W. Wright, Peter H. SpiegeL- 2nd ed. p.; cm. Previous ed. cataloged under: Wright, Kenneth W., (Kenneth Weston), 1950-. Includes bibliographical references and index. 1. Pediatric ophthalmology. 2. Strabismus. I. Wright, Kenneth W. (Kenneth Weston), 1950- . Pediatric ophthalmology and Strabismus. II. Wright, Kenneth W. (Kenneth Weston), 1950- . ill. Spiegel, Peter H. [DNLM: 1. Eye Diseases-Child. 2. Eye Diseases-Infant. 3. Strabismus-Child. 4. Strabismus-Infant. WW 600 P37141 2002] RE48.2C5 W747 2002 618.92'0977--dc21 2002020934

ISBN 978-1-4899-0511-6 ISBN 978-0-387-21753-6 (eBook) Printed on acid-free paper DOI 10.1007/978-0-387-21753-6

© Springer Science+Business Media New York 2003 Originally published by Springer-Verlag New York, Inc. in 2003 Softcover reprint of the bardeover 2nd edition 2003 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher Springer Science+Business Media, LLC, except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer sofrware, or by similar or dissimilar methodology now known or hereafter developed is forbidden.

The use in this publication of trade names, trademarks, service marks, and similar terms, even if the are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights.

While the advice and information in this book are believed to be tme and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein.

987654321 SPIN 10874558 www.springer-ny.com To my beautiful wife Donna and my children Jamie, Matthew, Lisa, Michael, and Andrew for their unselfish love that allows me to pursue my passion: pediatric ophthalmology and Strabismus and To my mother, Mary Jo, and my father, Harvey, for their steadfast support that gave me the opportunity to fulfill my dream.

Kenneth W. Wright, MD

To my wife, Lori, for her love and support.

Peter H. Spiegel, MD Foreward History of Pediatric Ophthalinology

he editors of this excellent balanced text of Pe• established in almost every metropolitan area of sig• T diatric Ophthalmology and Strabismus re• nificance. The new social demand for improved med• quested a foreward to the first revised edition ical care for children swept the struggling specialty of to inform the younger readership about the historical into prominence and acceptability. The re• details of pediatric ophthalmology and how strabis• sentment of general practitioners gradually disap• mus became joined to pediatric ophthalmology. I ac• peared and for good reason. Medicine, sooner or later, cepted the editors' challenging invitation with the un• inevitably smothers any trace of self-interest and derstanding that the reader should realize that history shortsightedness by invariably asking the question, is notorious for being recorded according to its author's What is in the best interest of the patient? This atti• memory, research, and point of view. This foreward is tude is the ingredient that makes medicine such a no exception to that observation. laudable profession. The answer was obvious, because Ophthalmology was one of the first specialties. time showed that pediatricians had raised the stan• Having evolved from surgery, it was welcomed as a dard of care for infants and children. specialty because ophthalmology was concerned with We should be proud that ophthalmology fulfilled other sciences and instrumentation unfamiliar to gen• the leadership role in the specialty movement by ini• eral surgeons. Refraction, assessment of the alignment tiating a certification process. Conceived in 1916, and motility of the eyes, and attempting to compre• this was the beginning of the American Board to cer• hend the complex neurology of vision were so far out• tify that candidates were adequately trained in their side the scope of knowledge expected of a general sur• specialty and had passed an examination given by geon that the development of ophthalmology as a peers and superiors that validated competence to specialty brought a sense of relief to its progenitors. practice that specialty. Before certification, the pub• In retrospect, as pediatric ophthalmologists today re• lic was victimized by self-proclaimed specialists in• flect an the evolution of ophthalmology from its be• adequately trained and incapable of rendering qual• ginning to the mid-20th century, they perceive the spe• ity care. Such a hold new order in the specialty cialty to have been primarily a geriatric one. Little in movement was met with resistance, not only by that formative period focused an the recognition and some ophthalmologists but also by the other spe• treatment of visual disorders in children. This per• cialties. Approximately 25 years passed before certi• ception is not a criticism; rather, it is an observation fication became accepted by all ophthalmologists and that the natural evolving process of ophthalmology 35 years before all specialties instituted their own preceded the evolution of pediatrics. boards. The American Board of Ophthalmology must In cantrast to ophthalmology, pediatrics evolved have considered itself vindicated by the fact that all from general practice with only a minimal difference boards of the more than two dozen specialties copied in the basic knowledge between practitioners of the the model it established. two fields, except for their experience with different Since the middle of the 20th century, subspecial• age groups. This difference proved to be inadequate to ization has become the second part of the specialties convince the medical community that pediatrics was movement. Subspecialization was the natural out• needed, so that it was difficult for pediatrics to gain growth of the specialty to cantend with its ever• the status of a specialty. However, the force that even• expanding base of knowledge and technology. Al• tually drove the development of pediatrics came from though subspecialization fragments the specialty, it a social thrust outside the me dical profession. By the will never be reversed because subspecialization is in beginning of the 19th century, children first became the best interest of the patient. All specialty boards recognized as the irreplaceable and essential treasure must cantend with the current subspecialization cer• of any nation wishing to ensure its success. In both tification problern they now face. Europe and North America, the new attitude encour• As the ophthalmology subspecialties were for• aged excellent education and medical care for all chil• mally introduced, pediatric ophthalmology was not dren. Within a few years, children's hospitals became included. The trend was to specialize by type of

vii viii FOREWORD: HISTORY OF PEDIATRIC OPTHALMOLOGY ocular tissue and major pathology, such as cornea, This fact coincided with the pediatric ophthalmolo• retina, strabismus, glaucoma, oculoplastics, neuro• gists' realization that Strabismus was the predominant opththalmology, and ophthalmic pathology. Despite pathology. It became obvious that strabismologists this impressive list of subspecialties, general ophthal• needed to have pediatric skills and pediatric ophthal• mology prevailed as the dominant group delivering eye mologists needed to have Strabismus skills. The nat• care to children. Neither the generalists nor the sub• ural outcome was a marriage of the two subspecial• specialties initially welcomed pediatric ophthalmol• ties. The unacceptability of pediatric ophthalmology ogy into the recognized subspecialty group. The often• as a bona fide subspecialty within ophthalmology ter• heard comment was "we can do everything a pediatric minated with this marriage. ophthalmologist does," a repetition of the identical The overriding issue regarding ophthalmology be• comment by the general practitioners one century ear• ing originally perceived as a geriatric specialty changed lier when pediatrics was becoming a specialty. But dur• with the acceptance of pediatric ophthalmology as a ing the 1950s, ophthalmology consistently revealed its subspecialty. This change brought balance to the mis• unawareness of the social policy (not a medical pol• sion of ophthalmology. The original mission seemed icy) established a century earlier that children should to be saving vision from disorders that resulted from receive special care. The understanding that a special the ravages of aging. It then became obvious that oph• environmentwas needed to render the highest qual• thalmology's mission was actually twofold: (1) to fos• ity care resulted in the spread of children's hospitals ter the development of vision in the pediatric popula• through the world's developed countfies within just a tion and (2) to preserve and restore vision once it has few years. This policy decisively established the spe• developed, with a primary focus on the adult and ge• cialty of pediatrics and charged it with the responsi• riatric population. bility to control and develop the best care for infants The one outstanding exception in attempting to and children. For pediatrics to remain in control of the define the limits of pediatric ophthalmology is adult environment in which children are treated, pediatrics strabismus. It is practical for the entire subspecialty realized it needed to attract trained personnel from all of strabismus to be within the combined subspecialty other specialties willing to devote their professional of pediatric ophthalmology and strabismus. As stated lives to children's care. Almost all specialties re• previously, the pediatric ophthalmologist must also be sponded to this need, and soon there was pediatric sur• a trained strabismologist. As the knowledge base and gery, anesthesiology, cardiology, neurology, urology, the technology involved in investigating and treating otolaryngology, hematology, oncology, and so on. Oph• strabismus are practically the same for pediatric and thalmology, however, remained unresponsive. This adult patients, the need for an adult strabismus sub• unfortunate attitude left ophthalmology on the losing specialty is lacking, especially in light of the success• side of the issue, because the answer to the inevitable ful marriage of the two subspecialties. question, "What is in the best interest of the patient?" Pediatric ophthalmology and strabismus is now was obvious. firmly established as a single subspecialty. The clinical As subspecialties in ophthalmology were becom• society of the American Association of Pediatric Oph• ing recognized, strabismus, like glaucoma, easily fit thalmology and Strabismus is 25 years old and pub• the criteria of acceptance. Almost immediately, stra• lishes a journal. The specialty is well served by several bismologists became the strong proponents for pedi• excellent texts available in this field. With a rapidly ex• atric ophthalmology to be accepted as a subspecialty. panding trained personnel base distributed araund the In the 1950s it became clear that the predominant world, the subspecialty of pediatric ophthalmology and pathology of strabismus had its onset during infancy strabismus has become an essential component of oph• and early childhood. In addition, strabismologists be• thalmology. Its success is attributable to an obvious came aware that the best results of treatment were fact: it is in the best interest of the patient. obtained by the most minimal duration allowed between onset and initial treatment of the disorder. Marshall M. Parks, MD Tribute to Marshall M. Parl

would like to take this opportunity to pay tribute bringing tagether the many diverse factions interested I toMarshall M. Parks, MD. Over the past several in pediatric ophthalmology and strabismus to estab• decades, Marshall Parks has been the major driv• lish the American Association of Pediatric Ophthal• ing force that has led to the development and matu• mology and Strabismus. Appropriately, he was the ration of our specialty, pediatric ophthalmology and first president of this very important organization. strabismus. Same may be surprised to learn that many It is noteworthy that his monumental accom• of our previous leaders in plishments were achieved from solo private practice ophthalmology strongly in Washington, DC. During my fellowship, I had the resisted recognizing pedi• privilege to watch Dr. Parks administer his duties as atriC ophthalmology as a president of the American Academy of Ophthalmol• specialty. Using his ele• ogy, examine patients, and train fellows from a single gant power of persuasion exam lane office that was attached to his harne. and leading by example, Dr. Parks is highly respected by his colleagues for Dr. Parks won the battle his clinical and surgical acumen, but, even more im• agairrst those who felt portant, he is admired for his unyielding ethics and there was no need for this integrity. I know his professional modesty would not specialty. His scientific allow him to take the credit, but, without his lead• papers, books, innumer• ership, pediatric ophthalmology would not exist as it able lectures, and leader• is today. ship have been essential to Thank you, Dr. Parks, for all you have clone for our the field of ophthalmology recognizing that eye care specialty and our children. for children involves much more than just caring for little eyes. It was Dr. Parks who was instrumental in Kenneth W. Wright, MD

ix Preface to the Second Edition

here have been significant changes in pediatric Chapter 56 by Maya Eibschitz-Tsimhoni, MD, is a T ophthalmology and strabismus since the first wonderful contribution to the literature, as it reviews edition. Great effort has gone into incorporat• 235 important ocular disorders that have systemic ing recent advances into this second edition. Each manifestations, and it includes a detailed glossary of chapter in the book has been revised, and over half of terms. them have been completely rewritten. In addition to As with the first edition, our goal is to present a updating and revising the entire book, we have added comprehensive textbook of pediatric ophthalmology three new chapters: Chapter 7 on electrophysiology and strabismus written in a clear, reader-friendly style. and the eye, Chapter 1 7 on strabismus surgery, and Our hope is that the readerwill find the second edi• Chapter 56 on congenital syndromes with ocular man• tion of Pediatric Ophthalmology and Strabismus to ifestations. Chapter 17 is the definitive work on pedi• be scientifically informative, clinically useful, and en• atric ocular electrophysiology, bar none, and was fin• joyable to read. ished just weeks before the untimely death of its author, Dr. Tony Kriss (see tribute in Chapter 17). Kenneth W. Wright, MD Peter H. Spiegel, MD

xi Preface to the First Edition

ediatric ophthalmology and Strabismus has come as ocular embryology and postnatal ocular develop• P into its own as a bona fide subspecialty of oph• ment. Practical aspects of pediatric ophthalmology, thalmology. This evolution did not take place including examination techniques, ocular trauma, and without a struggle. In the 1960s and 1970s, there was communication techniques for "breaking the news," resistance to establishing a pediatric ophthalmology arealso covered. Since a major part of pediatric oph• specialty. Many Ieaders at that time felt that general thalmology involves the management of strabismus, ophthalmologists and adult subspecialists could and a section devoted to and strabismus in in• should manage children's eye disorders, so there was cluded. Many comprehensive pediatric ophthalmol• no need for "pediatric ophthalmology." Fortunately, ogy texts include a section on strabismus, but usually others saw the importance of establishing such a present an overview. Herein we provide a thorough specialty. Through gentle but firm persuasion, pio• Strabismus section, representing a text within a text. neers such as Frank Costenbader, Jack Crawford, and After sections on ocular development, practical as• Marshall Parks demonstrated how patient care, resi• pects, and strabismus, the book is organized by ana• dent training, and clinical research are enhanced by tomic and functional subspecialties within pediatric having a specialty devoted to pediatric ophthalmology ophthalmology. With this format the reader can either and Strabismus. Pediatric ophthalmology involves review a subspecialty topic, such as pediatric neuro• much more than just treating "small adult" eyes. The ophthalmology, pediatric retina, or pediatric anterior management of pediatric eye diseases requires inti• segment abnormalities, or work through the index to mate knowledge of embryology, eye development, look up a specific disease entity. genetics, neuro-visual development, amblyopia, and A major emphasis was placed on presenting a strabismus. Special skills are also required for exam• wealth of illustrations and clinical photographs to sup• ining and treating children. testing, vi• port the text. Clinical photographs are a vital part of sual field testing, slit lamp examination, and appla• the book because the diagnosis of many ophthalmic nation tonometry are easily abtairred in adults, but conditions depend on pattern recognition that is dif• require special expertise in children and infants. Now ficult to communicate with words alone. The con• that the specialty of pediatric ophthalmology and stra• tributors, section editors, and reviewers of this text• bismus is well established, its beneficial effects are ob• book, true experts in their fields, with extensive vious, the most important being improved eye care for clinical experience, have provided richness and depth children. to this work. I would like to personally thank all who This book was written to fulfill the need for a com• contributed to this fine work for their expertise, prehensive textbook on pediatric ophthalmology and knowledge, and passion. A variety of readers should strabismus. It is written in a style to be lucid and eas• find the text useful, including ophthalmology resi• ily understood by the initiate, yet at the same time dents, general ophthalmologists, pediatric ophthal• provide current detailed information, so that even the mologists, and pediatricians. The editors sincerely most accomplished pediatric ophthalmologist will find hope that the readerwill find this text complete, in• new and useful information. The book covers a broad formative, and enjoyable. spectrum of important pediatric eye disorders, but also presents chapters on fundamental core subjects such Kenneth W Wright, MD

xiii Acl

We would like to thank the following sponsors for David L. Guyton, MD. Thank you, Dr. Parks, for the their support and ongoing commitment to furthering insightful Foreword you wrote on the history of pedi• education and research in pediatric ophthalmology atric ophthalmology and strabismus. I will always be and strabismus: grateful for the tremendous pediatric ophthalmology fellowship afforded me in Washington, DC, that, above Discovery Fund for Eye Research all, emphasized the importance of excellence. Also, I Gustavus and Louise Pfeiffer Research Foundation thank Dr. Guyton for his contribution to the Strabis• mus section. I am forever indebted to him for the stim• Henry L. Guenther Foundation ulating fellowship training in Strabismus at Johns• Wright Foundation for Pediatric Ophthalmology and Hopkins Hospitals and for encouraging me to think Strabismus "outside the box." Kenneth W. Wright, MD Finally, I sincerely thank Dr. Steven J. Ryan. Dr. Peter H. Spiegel, MD Ryan was my ophthalmology residency chairman and subsequently my faculty chairman at Doheny-USC would like to express my sincere gratitude to Tina Keck School of Medicine. His unselfish support made Kiss for her expert help in preparing the second my first book project possible and started me on the edition. Ms. Kiss was involved with virtually avocation of writing textbooks. every aspect of the project including editing manu• scripts, formatting references, communicating with Kenneth W. Wright, MD contributors (gentle persuasion), organizing art, and overseeing the overall book. Without her energy, en• thusiasm, and fortitude this book would never have been completed. We are grateful to the following talented and generous Also, a very special thanks to Paula Hong, MD, my individuals for their support and contributions to this Pediatric Ophthalmology and Strabismus Fellow at textbook: Theresa Kramer, MD, Julia Stevens, MD, Cedars-Sinai Medical Center during the final produc• Jonathan Song, MD, Timothy Stout, MD, PhD, David tion phases of this book. I appreciate all of her time V. Leaming, MD, Robert B. Guss, MD, Lari Wyman, S. and effort in reviewing hundreds of manuscript pages Maughan Parkinson, R. Michael Duffin, MD, Barratt required to publish this textbook. Phillips, MD, Robert Sorensen, MD, PhD, Christina I would like to acknowledge two mentors who Flaxel, MD, Elvin Rodriguez, and Rita Tindell. greatly influenced my career in pediatric ophthal• mology and Strabismus: Marshall M. Parks, MD, and Peter H. Spiegel, MD

XV Contents

Foreward by Marshall M. Parks, MD vii 11 Sensory Aspects of Strabismus 172 Tribute to Marshall M. Parks, MD ix Kenneth W Wright Preface to the Second Edition xi Preface to the First Edition xiii 12 The Ocular Motor Examination 189 Acknowledgments xv Kenneth W Wright

Section One Normal Development 13 Esodeviations 204 Kenneth W Wright 1 Embryology 3 Cynthia S. Cook, Kathleen K. Sulik, and Kenneth W Wright 14 Exotropia 224 Kenneth W Wright 2 Postnatal Development 39 H. Sprague Eustis and 15 Alphabet Patternsand M. Edward Gutbrie Oblique Museie Dysfunction 232 Kenneth W Wright Secdon Two Practical Aspects of Pediatric Ophthalmology 16 Camplex Strabismus: Restriction, Paresis, Dissodated Strabismus, and 3 Pediatric 57 Torticollis, 250 Ann U. Stout Kenneth W Wright

4 The Pediatric Low-Vision Patient 68 17 Strabismus Surgery 278 Anne Prances Walanker Kenneth W Wright and Pauline Hang 5 Breaking the News: The Role of the Physician 71 18 Optical Pearls and Pitfalls 292 Nancy Chernus-Manfield David L. Guyton, [oseph M. Miller, and Constance E. West 6 Pediatric Ocular Trauma 77 Kenneth W Wright Section Four Lids, Orbit, and Lacrima 7 Pediatric Visual Electrophysiology 90 Anthony Kriss and 19 Lid Malformations, Malpositions, Dorothy Thompson and Lesions 299 Parrest D. Ellis Secdon Three Strabismus and Amblyopia 20 Lacrimal System 313 8 Anatomy and Physiology of Parrest D. Ellis Eye Movements 125 Kenneth W Wright 21 Proptosis and Orbital Disease 321 Parrest D. Ellis 9 Binocular Vision and Introduction to Strabismus 144 Kenneth W Wright Secdon Five Anterior Segment Disease 10 Visual Development and Amblyopia 157 22 Pediatric 335 Kenneth W Wright Kenneth W Wright

xvii xviii CONTENTS 23 Pediatric Conjunctival Tumors 356 35 Retinablastoma and Malignant Matthew W Wilsan, Barrett G. Haik, Intraocular Tumors 584 Zeynel A. Karciaglu, Darryl J. Ainbinder, A. Linn Murphree and and Ernesta A. Calva Laurie E. Christensen

24 Developmental Anomalies 36 Retinopathy of Prematurity 600 of the Anterior Segment and Richard R. Ober, Earl A. Palmer, Globe 369 Arlene V Drack, and Ken K. Nischal Kenneth W Wright

25 Corneal Abnormalities 391 3 7 Infectious, Inflammatory, and Ken K. Nischal Taxie Diseases of the Retina and Vitreous 629 26 Pediatric Iris Abnormalities 430 William L. Haynes, fase S. Pulida, Ken K. Nischal and Peter H. Spiegel 2 7 Lens Abnormalities 450 38 644 Kenneth W Wright Arlene V Drack

39 Patterns of Retinal Disease Section Six Glaucoma and Uveitis in Children 654 Arlene V Drack 28 Pediatric Glaucoma 483 fames D. Reynalds and Scatt E. Olitsky Section Eight Systemic Disease with Eye 29 Uveitis in Children 499 Involvement Narsing A. Raa 40 Chromosomal Abnormalities and the Eye 675 J. Bronwyn Bateman Section Seven Pediatric Retinal Disease 41 Craniofacial Syndromes 30 Heritable Disorders of RPE, and Malformations 705 Bruch's Membrance, and the Marilyn T. Millerand Anna Newlin Choriocapillaris 523 Arlene V Drack 42 Connective Tissue, Skin, and Bane Disorders 740 31 Retinitis Pigmentosa Elias I. Trabaulsi and Associated Disorders 539 Arlene V Drack and Alan E. Kimura 43 Neurocutaneous Syndromes 766 Maria A. Musarella 32 Disorders of the Vitreous and Vitreoretinal Interface 556 44 Metabolie Diseases 790 David M. Brawn and Thamas A. Weingeist Lais J. Martyn

33 Retinal Vascular Disorders 566 45 Selected Genetic Syndromes Richard M. Feist, with Ophthalmie Features 823 Christapher F. Bladi, and Natalie C. Kerr and Peter H. Spiegel Enikö Karman Pivnick

34 Nonvascular Harnartomas 576 46 Infectious Diseases 845 Chittaranjan V Reddy and R. Christapher W altan, Arlene V Drack Rager K. Gearge, and Alissa A. Craft CONTENTS xix Section Nine Neuro-Ophthalmology 53 Neurodegenerative Conditions of Ophthalmie Importanee 970 47 Pediatric Neuro-Ophthalmology Mark S. Borchert and Sarah Ying Examination 865 Edward G. Buckley 54 Neuroeranial Defeets with 48 Oeular Motility Disorders 876 Neuro-Ophthalmie Signifieanee 991 Mitra Maybodi, Richard W Hertle, Ronald M. Minzter and and Brian N. Bachynski Edward G. Buckley

49 Cognenital Optie Nerve 55 Management of Common Pediatrie Abnormalities 918 Neuro-Ophthalmology Problems 1004 Paul H. Phillips and James W McManaway III Michael C. Brodsky and Dean [. Bonsall 50 Cortieal Visual Impairment 936 Susan M. Garden and William V Good Section Ten Compendium of Inherited Diseases with Ocular Involvement 51 Brain Lesions with Ophthalmie Manifestations 940 56 Oeular Manifestations of Michael X. Repka Inherited Diseases 1021 52 Nystagmus and Oeular Oseillations Maya Eibschitz-Tsimhoni in Infaney and Childhood 954 Richard W Hertle Index 1059 Contributors

Darryl J. Ainbinder, MD, FACS Susan M. Carden, MBBS, FRACO, FRACS Chief, Ophthalmie Oncology/Pathology, Department Senior Lecturer, University of Melbourne; Royal of Ophthalmology, Madigan Army Medical Center, Children's Hospital and Royal Victorian Eye and Tacoma, WA, USA Ear Hospital, Melbourne, Australia

Brian N. Bachynski, MD Nancy Chernus-Mansfield, MA Director of Pediatric Ophthalmology, Henry Ford Executive Director, Institute for Families of Blind Health System, Detroit, MI, USA Children, Los Angeles, CA, USA

J. Bronwyn Bateman, MD Laurie E. Christensen, MD Professor and Chair, Department of Ophthalmology, Assistant Professor, Pediatric Ophthalmology and University of Colorado School of Medicine; Strabismus, Oregon Elks Children's Eye Clinic, Director, Rocky Mountain Lions Eye Institute, Casey Eye Institute, Oregon Health & Seiences Denver, CO, USA University, Portland, OR, USA

Christopher F. Blodi, MD Cynthia S. Cook, DVM, PhD Iowa Retinal Consultants, Inc., West Des Moines, American College of Veterinary Ophthalmologists, IA, USA Veterinary Vision-Animal Eye Specialists, San Mateo, CA, USA Dean J. Bonsall, MD Alissa A. Craft, MD Assistant Professor of Ophthalmology and Department of Ophthalmology, University of Pediatrics, Pennsylvania State University College of Tennessee College of Medicine, Memphis, Medicine, Hershey, PA, USA TN,USA

Mark S. Borchert, MD Arlene V. Drack, MD Head, Division of Ophthalmology, Childrens Associate Professor of Ophthalmology, Emory Eye Hospital Los Angeles; Associate Professor of Center, Atlanta, GA, USA Clinical Ophthalmology and Neurology, University of Southern California School of Medicine, Los Maya Eibschitz-Tsimhoni, MD Angeles, CA, USA Resident, Department of Ophthalmology and Visual Sciences, University of Michigan, Kellogg Eye Michael C. Brodsky, MD Center, Ann Arbor, MI, USA Professor of Ophthalmology, Pediatric Ophthalmology and Strabismus, and Neuro• Forrest D. Ellis, MD ophthalmology, University of Arkansas Medical Emeritus Professor of Ophthalmology, Indiana Sciences, Jones' Eye Institute, and Arkansas University School of Medicine, Midwest Eye Children's Hospital, Little Rock, AR, USA Institute, Indianapolis, IN, USA

David M. Brown, MD H. Sprague Eustis, MD Vitreoretinal Consultants, Houston, TX, USA Residency Program Director, Department of Ophthalmology, Ochsner Clinic Edward G. Buckley, MD and Altan Ochsner Medical Foundation, Professor of Ophthalmology and Pediatrics, Duke New Orleans, LA, USA University Medical Center, Durham, NC, USA Richard M. Feist, MD Ernesto A. Calvo, MD, BC, FAAD Assistant Professor, Depatment of Ophthalmology, Clinica de Ojos Orillac-Calvo, Panama City, Panama University of Alabama, Birmingham, AL, USA

xxi xxi i CONTRIBUTORS Roger K. George, MD Lois J. Martyn, MD Department of Ophthalmology, Madigan Army Associate Professor of Ophthalmology; Associate Medical Center, Tacoma, WA, USA Professor of Pediatrics; Temple University School of Medicine, Philadelphia, PA, USA William V. Good, MD Professor of Ophthalmology, Smith-Kettlewell Eye Mitra Maybodi, MD Research Institute, San Francisco, CA, USA Department of Ophthalmology, Children's National Medical Center, Washington, DC, USA M. Edward Guthrie, MD Department of Surgery, Broward General Hospital, James W. McManaway 111, MD Fort Lauderdale, FL, USA Professor of Ophthalmology and Pediatrics, Pennsylvania State University College David L. Guyton, MD of Medicine, Hershey, PA, USA Krieger Professor of Ophthalmology, Department of Ophthalmology, The Johns Hopkins University Joseph M. Miller, MD School of Medicine; Director of Wilmer Children's Professor, Department of Ophthalmology and Eye Center, Baltimore, MD, USA Optical Sciences, University of Arizona, Tucson, AZ, USA Barrett G. Haik, MD, FACS Rarnilton Professor and Chair, Department of Marilyn T. Miller, MD Ophthalmology, College of Medicine, University of Professor, Department of Ophthalmology and Visual Tennessee at Memphis, Memphis, TN, USA Sciences, University of Illinois at Chicago, Chicago, IL, USA William L. Haynes, MD Partner, Asheville Eye Associates, Asheville, NC; Ronald M. Minzter, MD Consulting Assistant Professor of Ophthalmology, Freehold, NJ, USA Duke University School of Medicine, Durham, NC, USA A. Linn Murphree, MD Professor of Ophthalmology and Pediatrics; Director, Richard W. Herde, MD, FACS Ocular Oncology Service, University of Southern Associate Professor of Ophthalmology, Co-Chief, California Keck School of Medicine; Director, Pediatric Ophthalmology; Director of Pediatric Clayton Foundation Center for Ocular Oncology, Ophthalmology Fellowship, Columbus Children's Childrens Hospital Los Angeles, Los Angeles, CA, Hospital, Ohio State University, Columbus, OH, USA USA Maria A. Musarella, MD, Pauline Hong, MD Associate Professor of Ophthalmology and Sierra Eye Associates, Reno, NV, USA Pediatrics, State University of New York Downstate, Brooklyn, NY, USA Zeynel A. Karcioglu, MD Professor of Ophthalmology and Pathology, Anna Newlin, MS, CGC Department of Ophthalmology, Tulane University Center for Medical Genetics, Evanston Hospital, Medical Center, New Orleans, LA, USA Evanston, IL, USA

Natalie C. Kerr, MD, FACS, FAAP Ken K. Nischal, FRCOphth Associate Professor, Ophthalmology and Pediatrics, Consultant Ophthalmie Surgeon, Great Ormond St University of Tennessee Health Science Center, Hospital for Children; Hon. Senior Lecturer, Memphis, TN, USA Institute of Child Health, London, UK

Alan E. Kimura, MD Richard R. Ober, MD Clinical Associate Professor, Department of Professor of Ophthalmology, University of Arizona, Ophthalmology, University of Colorado Health Tucson, AZ, USA Seiences Center, Denver, CO, USA Scott E. Olitsky, MD Anthony Kriss, PhD (Posthumous) Associate Professor of Ophthalmology, Pediatric The Tony Kriss Visual Electrophysiology Unit, Ophthalmology and Adult Strabismus, State Great Ormond Street Hospital for Siek Children, University of New York at Buffalo, Children's London, UK Hospital of Buffalo, Buffalo, NY, USA CONTRIBUTORS xxiii Earl A. Palmer, MD Kathleen K. Sulik, PhD Professor, Department of Ophthalmology and Division of Cell Biology and Anatomy, University of Pediatrics; Chief, Strabismus Service; Director, North Carolina, Chapel Hill, NC, USA Oregon Elks Children's Eye Clinic, Casey Eye Institute, Oregon Health &. Seiences University, Dorothy Thompson, PhD Portland, OR, USA The Tony Kriss Visual Electrophysiology Unit, Great Ormond Street Paul H. Phillips, MD Hospital for Siek Children, London, Associate Professor of Ophthalmology, Pediatric UK Ophthalmology and Strabismus, and Neuro• ophthalmology, Jones Eye Institute, University of Elias I. Traboulsi, MD Arkansas Medical Center, and Arkansas Children's Head, Pediatric Ophthalmology and Strabismus; Hospital, Little Rock, AR, USA Director, The Center for Genetic Eye Diseases, Cole Eye Institute, Cleveland Clinic Foundation, Enikö Karman Pivnick, MD Cleveland, OH, USA Associate Pediatric Geneticist, Department of Pediatrics, Division of Clinical Genetics, University Anne Frances Walonker, CO of Tennessee Health Science Center, Memphis, Chief Orthoptist, TN,USA Department of Ophthalmology, University of Southern California School of Jose S. Pulido, MD Medicine, Doheny Eye Institute, Los Angeles, CA Professor and Head, Ophthalmology and Visual USA Sciences, University of Illinois at Chicago, Chicago, IL, USA R. Christopher Walton, MD Associate Professor, Department of Ophthalmology, Narsing A. Rao, MD University of Tennessee College of Medicine, Professor of Ophthalmology and Pathology, Memphis, TN, USA University of Southern California Keck School of Medicine, Director of Ophthalmie Pathology Laboratories, Doheny Eye Institute, Los Angeles, Thomas A. Weingeist, MD, PhD CA, USA Professor and Head, Department of Ophthalmology, University of Iowa Hospitalsand Clinics, Chittaranjan V. Reddy, MD Iowa City, lA, USA Retina Consultants of Central Illinois, Peoria, IL, USA Constance E. West, MD Director, Abrahamsan Pediatric Eye Institute, Michael X. Repka, MD Division of Pediatric Ophthalmology, Children's Professor of Ophthalmology and Pediatrics, Hospital Medical Center, Cincinnati, OH, USA The Wilmer Eye Institute of The Johns Hopkins University Hospital, Baltimore, MD, USA Mathew W. Wilson, MD, FACS James D. Reynolds, MD Assistant Professor, Department of Ophthalmology, Professor, State University of New York at Buffalo; University of Tennessee Health Seiences Center, Chief of Pediatric Ophthalmology, Children's Memphis, TN, USA Hospital of Buffalo, Buffalo, NY, USA Kenneth W. Wright, MD Peter H. Spiegel, MD Clinical Professor of Ophthalmology, University of Vision Professionals, Palm Springs, CA; Inland Eye Southern California Keck School of Medicine; Clinic, Hemet California; Attending Physician, Pediatric Ophthalmology Director, Research Loma Linda University Medical Center, Loma and Education, Cedars-Sinai Linda, CA, USA Medical Center, Los Angeles, CA, USA Ann U. Stout, MD Assistant Professor of Ophthalmology, Oregon Elks Sarah Ying, MD Children's Eye Clinic, Casey Eye Institute, Oregon Department of Neurology, Johns Hopkins Health &. zSciences University, Portland, OR, USA University, Baltimore, MD, USA