Mosby is an affi liate of Elsevier Inc. © 2008, Elsevier Inc. All rights reserved. First published 2008 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 the prior permission of the Publishers. Permissions may be sought directly from Elsevier’s Health Sciences Rights Department, 1600 John F. Kennedy Boulevard, Suite 1800, Philadelphia, PA 19103-2899, USA: phone (+1) 215 239 3804, fax (+1) 215 239 3805, or e-mail [email protected]. You may also complete your request online via the Elsevier homepage (http://www.elsevier.com) by selecting ‘Support and Contact’ and then ‘Copyright and Permission’. ISBN 978-0-323-04443-1 British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging in Publication Data A catalog record for this book is available from the Library of Congress Notice Medical knowledge is constantly changing. Standard safety precautions must be followed, but as new research and clinical experience broaden our knowledge, changes in treatment and drug therapy may become necessary or appropriate. Readers are advised to check the most current product information provided by the manufacturer of each drug to be administered to verify the recommended dose, the method and duration of administration, and contraindications. It is the responsibility of the practitioner, relying on experience and knowledge of the patient, to determine dosages and the best treatment for each individual patient. Neither the Publisher nor the authors assume any liability for any injury and/or damage to persons or property arising from this publication. The Publisher The Working together to grow publisher’s libraries in developing countries policy is to use paper manufactured www.elsevier.com | www.bookaid.org | www.sabre.org from sustainable forests Printed in China Last digit is the print number: 9 8 7 6 5 4 3 2 1 FM-A04443.indd iv 9/13/2007 4:25:35 PM Given the complexity and quantity of clinical knowledge R S required to correctly identify and treat ocular disease, a a p quick reference text with high quality color images repre- i e d sents an invaluable resource to the busy clinician. Despite D r the availability of extensive resources online to clinicians, i i accessing these resources can be time consuming and a e g often requires fi ltering through unnecessary information. In n s the exam room, facing a patient with an unfamiliar presen- o tation or complicated medical problem, this series will be si an invaluable resource. s P in This handy pocket sized reference series puts the knowl- O r edge of world-renowned experts at your fi ngertips. The p e h standardized format provides the key element of each th f disease entity as your fi rst encounter. The additional infor- a a mation on the clinical presentation, ancillary testing, dif- lm ferential diagnosis and treatment, including the prognosis, o c lo allows the clinician to instantly diagnose and treat the most g e common diseases seen in a busy practice. Inclusion of y classical clinical color photos provides additional assur- ance in securing an accurate diagnosis and initiating management. Regardless of the area of the world in which the clinician practices, these handy references guides will provide the necessary resources to both diagnose and treat a wide variety of ophthalmic diseases in all ophthalmologic spe- cialties. The clinician who does not have easy access to sub-specialists in Anterior Segment, Glaucoma, Pedia- tric Ophthalmology, Strabismus, Neuro-ophthalmology, Retina, Oculoplastic and Reconstructive Surgery, and Uveitis will fi nd these texts provide an excellent substitute. World-wide recognized experts equip the clinician with the elements needed to accurately diagnose treat and manage these complicated diseases, with confi dence aided by the excellent color photos and knowledge of the prognosis. The fi eld of knowledge continues to expand for both the clinician in training and in practice. As a result we fi nd it a challenge to stay up to date in the diagnosis and manage- ment of every disease entity that we face in a busy clinical practice. This series is written by an international group of experts who provide a clear, structured format with excel- lent photos. It is our hope that with the aid of these six volumes, the clinician will be better equipped to diagnose and treat the diseases that affect their patients, and improve their lives. Marian S. Macsai and Jay S. Duker ix FM-A04443.indd ix 9/13/2007 4:25:35 PM e Anomalies and abnormalities of the crystalline lens and the variety of the glaucomas can be simultaneously fascinat- c ing, complex and overwhelming. The sections that follow a are designed to provide a rapid reference for a panoply of f clinical entities, many of which present daily in physicians e offi ces worldwide. r P We have included quick facts for diagnosis and treatment, as well as photographs of the entities of interest. This section should be a ready reference for the busy clinician grappling with either familiar or unfamiliar fi ndings. We hope that this information will be of benefi t on both sides of the slit lamp. Joel S. Schuman Viki Christopoulos Deepinder K. Dhaliwal Malik Y. Kahook Robert J. Noecker x FM-A04443.indd x 9/13/2007 4:25:35 PM We thank Diane Curtain for her tremendous photographic A and artistic expertise, as well as her enthusiasm for educa- c tion and her passion for our patients. k n To our best teachers: our patients and our students. o w To my mother Evelpia who is my angel and my children Constantine, Evely, and Thea who enchant me on a daily l e basis. VC d To my parents, Gurmeet and Amrik, and to my husband, g Sanjiv, for their continued love and support. To my darling daughter, Diya, who is the light of my life. DKD m e n t / D e d i c a t i o n s xi FM-A04443.indd xi 9/13/2007 4:25:35 PM We thank Diane Curtain for her tremendous photographic A and artistic expertise, as well as her enthusiasm for educa- c tion and her passion for our patients. k n To our best teachers: our patients and our students. o w To my mother Evelpia who is my angel and my children Constantine, Evely, and Thea who enchant me on a daily l e basis. VC d To my parents, Gurmeet and Amrik, and to my husband, g Sanjiv, for their continued love and support. To my darling daughter, Diya, who is the light of my life. DKD m e n t / D e d i c a t i o n s xi FM-A04443.indd xi 9/13/2007 4:25:35 PM Section 1 Congenital Abnormalities Lenticonus and Lentiglobus 2 Lens Coloboma 4 Mittendorf Dot 6 Ch001-A04443.indd 1 9/13/2007 4:26:26 PM Lenticonus and Lentiglobus Key Facts • Localized bulge (lenticonus) or generalized protrusion (lentiglobus) of anterior or posterior lens capsule • Presumably due to lens capsular thinning • Rare (1–4/100 000) • Congenital or acquired • Unilateral or bilateral • Anterior rarer than posterior types • Anterior lenticonus associated with Alport syndrome (90%) • Posterior lenticonus usually idiopathic Clinical Findings • May present as progressive myopia, high astigmatism (irregular) • Oil droplet refl ex on retinoscopy • Adjacent cortical opacifi cation • Uncommonly, spontaneous capsular rupture S E C Ancillary Testing T IO • None N 1 • Differential Diagnosis C o • Galactosemia and galactokinase defi ciency (oil droplet cataracts) ng • Posterior polar cataract en • Persistent hyaloid remnants ita l Treatment A b • Cataract surgery if: n o • associated lens opacity • high astigmatism • anisometropia r m a Prognosis litie • Favorable on removal of associated cataract s • Vigilant postoperative occlusion to avoid amblyopia in children 2 Ch001-A04443.indd 2 9/13/2007 4:26:26 PM Fig. 1.1 A dramatic view of anterior lenticonus in a patient with idiopathic lenticonus. L e n t ic o n u s a n d L e n t ig lo b Fig. 1.2 The same patient also has posterior lenticonus. u Although not as dramatic as his anterior lenticonus, the s out-pouching of the posterior lens is still obvious. Fig. 1.3 The classic oil droplet refl ex of lenticonus is best seen on retroillumination. 3 Ch001-A04443.indd 3 9/13/2007 4:26:26 PM Lens Coloboma Key Facts • Equatorial lens fl attening or notch due to a focal absence of lens zonules • Underlying colobomatous ciliary body • Usually sporadic (occasionally dominant) • Unilateral or bilateral • Isolated fi nding or associated with other ocular colobomas • Systemic associations: • Patau syndrome (trisomy 13) • cat eye syndrome (trisomy 22) • coloboma, heart defects, choanal atresia, retarded development, genital and ear anomalies (CHARGE) • basal encephaloceles or cysts • Goldenhar syndrome • other syndromes Clinical Findings • Usually inferonasal quadrant • Associated sectoral cataract • Phacodonesis S E • Often associated with colobomas of iris, ciliary body, retina, and choroid C T Ancillary Testing I O N • None 1 • C Differential Diagnosis o n • Lens-dislocating diseases (e.g. Marfan syndrome and homocystinuria) g e • Adjacent pars plicata or ciliary body tumor (e.g. melanocytoma) n ita • Contusion or intraoperative trauma (acquired zonular defects) l • Normal variant (minor indentations between inserting zonules in young lenses) A b n Treatment o r m • Intervention usually not necessary alitie • cWonhseind ecraetdaract surgery is indicated, a capsular tension ring should be s Prognosis • Excellent when isolated Fig. 1.4 A myopic patient with primary lens coloboma. 4 Ch001-A04443.indd 4 9/13/2007 4:26:31 PM Fig. 1.5 Note the scalloped nasal edge of this 12-year old’s lens coincident with absent lens zonules. Fig. 1.6 Left eye of the same 12-year- L e old boy, taken at a n different angle, s showing a ciliary C body cyst (seen o inferonasally) as the lo cause of this b secondary lens o m coloboma. a Fig. 1.7 Another case of secondary lens coloboma due to a ciliary body tumor. 5 Ch001-A04443.indd 5 9/13/2007 4:26:33 PM