Clinical Retina
Edited By
David A. Quillen, MD
Barbara A. Blodi, MD
American Medical Association
Physicians dedicated to the health of America
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AMA Press
Vice President, Business Products: Anthony J. Frankos
Publisher: Michael Desposito
Editorial Director: Mary Lou White
Director, Production and Manufacturing: Jean Roberts
Senior Acquisitions Editor: Barry Bowlus
Developmental Editor: Katharine Dvorak
Copy Editor: Maxine Gere
Director, Marketing: J. D. Kinney
Marketing Manager: Reg Schmidt
Senior Production Coordinator: Rosalyn Carlton
Senior Print Coordinator: Ronnie Summers
© 2002 by the American Medical Association All rights reserved.
Printed in the United States of America.
Internet address: www.ama-assn.org
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 written permission of the publisher.
Additional copies of this book may be ordered by calling 800 621-8335. Mention product number OP856902.
ISBN 1-57947-284-2
The authors, editors, and publisher of this work have checked with sources believed to be reliable in their efforts to confirm the accuracy and completeness of the information presented herein and that the information is in accordance with the standard practices accepted at the time of publication. However, neither the authors, editors, and publisher, nor any party involved in the creation and publication of this work warrant that the information is in every respect accurate and complete, and they are not responsible for any errors or omissions or for any consequences from application of the information in this book.
In light of ongoing research and changes in clinical experience and in governmental regulations, readers are encouraged to confirm the information contained herein with additional sources, in particular as it pertains to drug dosage and usage. Readers are urged to check the package insert for each drug they plan to administer for any change in indications and dosage and for additional warnings and precautions, especially for new or infrequently used drugs.
Library of Congress Cataloging-in-Publication Data
Clinical retina / edited by David A. Quillen, Barbara A. Blodi. p. ; cm.
Includes bibliographical references and index. ISBN 1-57947-284-2
1. Retina--Diseases. 2. Retina--Diseases--Atlases.
[DNLM: 1. Retinal Diseases--physiopathology. 2. Retina--physiology. 3. Retinal Diseases--diagnosis. 4. Retinal Diseases--therapy. WW 270 C6146 2002] I. Quillen, David A., 1964II. Blodi, Barbara A.
RE551 .C575 2002 617.7'35—dc21
2002009372
BP90:02-P-052:10/02
To Betsy and our children, Erin, Matthew, and Jack. —DQ
To Justin, Jeremy, and Andrea. —BB
CONTENTS |
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|
chapter 1 |
Anatomy and Physiology of the Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.1 |
|
Pars Plana . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.2 |
|
Ora Serrata . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.2 |
|
Vortex Veins and Ciliary Nerves . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.2 |
|
Macula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.2 |
|
Fovea, Foveola, and Umbo . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.2 |
|
Neurosensory Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.4 |
|
Photoreceptors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.4 |
|
Retinal Pigment Epithelium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.4 |
|
Retinal Blood Flow . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.4 |
|
Bruch’s Membrane . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.6 |
|
Choroid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.6 |
|
Vitreous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.6 |
|
Normal Retinal Adhesion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.6 |
|
Blood-Retinal Barrier . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.6 |
|
Physiology of the Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.8 |
|
Clinical Correlation: Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
10 |
|
Clinical Correlation: Retinal Pigment Epithelium . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
11 |
|
Clinical Correlation: Bruch’s Membrane and Choroid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
12 |
|
Clinical Correlation: Vitreous, Retinal Adhesion, and Blood-retinal Barrier . . . . . . . . . . . . . . . . |
13 |
chapter 2 |
Ancillary Testing for Retinal and Choroidal Diseases . . . . . . . . . . . . . . . . . |
15 |
|
Fluorescein Angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
16 |
|
Fluorescein Angiography: Hyperfluorescence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
18 |
|
Fluorescein Angiography: Hypofluorescence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
22 |
|
Indocyanine Green Angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
26 |
|
Electroretinography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
28 |
|
Electro-oculography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
32 |
|
Echography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
34 |
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Scanning Laser Ophthalmoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
36 |
|
Optical Coherence Tomography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
38 |
chapter 3 |
Clinical Features of Retinal Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
41 |
|
Bull’s Eye Maculopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
42 |
|
Cherry Red Spot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
43 |
|
Chorioretinal Folds . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
44 |
v
vi |
C O N T E N T S |
|
Choroidal Neovascularization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.45 |
|
Cotton Wool Spot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.46 |
|
Cystoid Macular Edema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.47 |
|
Drusen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.48 |
|
Flecked Retina Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.49 |
|
Foveal Yellow Spot . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.50 |
|
Intraretinal Hemorrhages . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.51 |
|
Lipid Exudates . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.52 |
|
Macular Atrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.53 |
|
Optic Disc Edema with Macular Star . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.54 |
|
Peripheral Pigmentation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.55 |
|
Pigment Epithelial Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.56 |
|
Pigmented Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.57 |
|
Preretinal Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.58 |
|
Retinal Crystals . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.59 |
|
Retinal Neovascularization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.60 |
|
Retinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.61 |
|
Rubeosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.62 |
|
Subretinal Fluid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.63 |
|
Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.64 |
|
Vasculitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.65 |
|
Vitelliform Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.66 |
|
Vitreous Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.67 |
|
Vitreous Opacity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.68 |
|
White Dot Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.69 |
|
White-centered Retinal Hemorrhages . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.70 |
chapter 4 |
Macular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.85 |
|
Age-Related Macular Degeneration: Nonexudative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.86 |
|
Age-Related Macular Degeneration: Exudative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.90 |
|
Angioid Streaks . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.94 |
|
Central Serous Chorioretinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.96 |
|
Cystoid Macular Edema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.98 |
|
Epiretinal Membrane . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
100 |
|
Hypotony Maculopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
102 |
|
Macular Hole . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
104 |
C O N T E N T S |
vii |
Myopic Degeneration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .106
Pattern Dystrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .108
Photic Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .110
chapter 5 Retinal Vascular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .115
Branch Retinal Artery Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .116
Branch Retinal Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .118
Central Retinal Artery Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .120
Central Retinal Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .122
Coats’ Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .124
Diabetic Retinopathy: Nonproliferative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .126
Diabetic Retinopathy: Proliferative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .130
Eales’ Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .134
Hypertensive Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .136
Idiopathic Juxtafoveal Retinal Telangiectasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .138
Leukemic Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .140
Ocular Ischemic Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .142
Pregnancy-Related Retinal Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .144
Radiation Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .146
Retinal Arterial Macroaneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .148
Retinopathy of Prematurity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .150
Sickle Cell Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .152
chapter 6 Hereditary Retinal Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .157
Albinism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .158
Best’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .160
Choroideremia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .162
Cone Dystrophies/Cone-Rod Dystrophies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .164
Congenital Stationary Night Blindness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .166
Dominant Drusen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .168
Gyrate Atrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .170
Leber’s Congenital Amaurosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .172
North Carolina Macular Dystrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .174
Retinitis Pigmentosa (Rod-Cone Dystrophies) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .176
Sorsby’s Fundus Dystrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .178
Stargardt Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .180
viii |
C O N T E N T S |
|
X-Linked Juvenile Retinoschisis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.182 |
chapter 7 |
Drug Toxicities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
189 |
|
Aminoglycoside Toxicity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
19/0 |
|
Chloroquine/Hydroxychloroquine Toxicity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.192 |
|
Crystalline Retinopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.194 |
|
Cystoid Macular Edema-Associated Toxicities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.196 |
|
Iron Toxicity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.198 |
|
Phenothiazine Toxicity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.200 |
chapter 8 |
Intraocular Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
203 |
|
Choroidal Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.204 |
|
Choroidal Melanoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.206 |
|
Choroidal Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.208 |
|
Choroidal Nevus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.210 |
|
Choroidal Osteoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.212 |
|
Cogenital Hypertrophy of the Retinal Pigment Epithelium . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.214 |
|
Intraocular Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.216 |
|
Melanocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.218 |
|
Phakomatoses: Neurofibromatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.220 |
|
Phakomatoses: Sturge-Weber Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.222 |
|
Phakomatoses: Tuberous Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.224 |
|
Phakomatoses: von Hippel-Lindau Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.226 |
|
Phakomatoses: Wyburn-Mason Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.228 |
|
Retinoblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.230 |
chapter 9 |
Inflammatory Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
235 |
|
Acute Posterior Multifocal Placoid Pigment Epitheliopathy . . . . . . . . . . . . . . . . . . . . . . . . . . |
.236 |
|
Acute Retinal Necrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.238 |
|
Behçet’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.240 |
|
Birdshot Chorioretinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.242 |
|
Cytomegalovirus Retinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.244 |
|
Diffuse Unilateral Subacute Neuroretinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.246 |
|
Endophthalmitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.248 |
|
Human Immunodeficiency Virus Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.250 |
|
Intermediate Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.252 |
|
Multifocal Choroiditis and Panuveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.254 |
C O N T E N T S |
ix |
|
Multiple Evanescent White Dot Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.256 |
|
Neuroretinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.258 |
|
Posterior Scleritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.260 |
|
Presumed Ocular Histoplasmosis Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.262 |
|
Sarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.264 |
|
Serpiginous Choroiditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.266 |
|
Syphilis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.268 |
|
Systemic Lupus Erythematosus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.270 |
|
Toxocariasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.272 |
|
Toxoplasmosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.274 |
|
Tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.276 |
|
Vogt-Koyanagi-Harada Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.278 |
chapter 10 |
Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
285 |
|
Choroidal Rupture . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.286 |
|
Commotio Retinae . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.288 |
|
Optic Nerve Evulsion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.290 |
|
Purtscher’s Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.292 |
|
Shaken Baby Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.294 |
|
Terson’s Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.296 |
|
Valsalva Retinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.298 |
chapter 11 |
Peripheral Retinal Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
301 |
|
Cystic Retinal Tufts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.302 |
|
Lattice Degeneration . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.304 |
|
Retinal Breaks . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.306 |
|
Retinal Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.308 |
|
Senile (Adult-Onset) Retinoschisis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.310 |
chapter 12 |
Diseases of the Vitreous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
313 |
|
Amyloidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.314 |
|
Asteroid Hyalosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.316 |
|
Idiopathic Vitritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.318 |
|
Persistent Hyperplastic Primary Vitreous . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.320 |
|
Posterior Vitreous Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.322 |
|
Proliferative Vitreoretinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.324 |
|
Vitreous Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.326 |
x |
C O N T E N T S |
chapter 13 |
Histopathology of Retinal Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.329 |
|
Macular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.330 |
|
Retinal Vascular Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.332 |
|
Intraoculor Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.336 |
|
Inflammatory Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.340 |
|
Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.342 |
|
Peripheral Retinal Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.343 |
chapter 14 |
Clinical Trials in Retina . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
345 |
|
The Diabetic Retinopathy Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.346 |
|
The Early Treatment Diabetic Retinopathy Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.347 |
|
The Diabetic Retinopathy Vitrectomy Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.349 |
|
The Diabetes Control and Complications Trial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.352 |
|
The Branch Vein Occlusion Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.354 |
|
The Central Vein Occlusion Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.355 |
|
The Multicenter Trial of Cryotherapy for Retinopathy of Prematurity . . . . . . . . . . . . . . . . . . |
.357 |
|
The Macular Photocoagulation Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.359 |
|
The Treatment of Age-Related Macular Degeneration with Photodynamic |
|
|
Therapy (TAP) Study . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.363 |
chapter 15 |
Laser Photocoagulation and Photodynamic Therapy for Retinal |
|
|
and Choroidal Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
367 |
|
Branch Retinal Vein Occlusion: Macular Edema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.368 |
|
Branch Retinal Vein Occlusion: Neovascularization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.370 |
|
Central Serous Chorioretinopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.372 |
|
Central Retinal Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.374 |
|
Choroidal Neovascularization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.376 |
|
Diabetic Retinopathy: Clinically Significant Macular Edema . . . . . . . . . . . . . . . . . . . . . . . . . |
.378 |
|
Diabetic Retinopathy: High-Risk Proliferative Diabetic Retinopathy . . . . . . . . . . . . . . . . . . . |
.380 |
|
Peripheral Retinal Neovascularization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.382 |
|
Retinal Arterial Macroaneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.384 |
|
Retinal Tears and Retinal Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.386 |
|
Retinal Telangiectasis and Retinal Angiomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.388 |
|
Photodynamic Therapy with Verteporfin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
.390 |
PREFACE
As we began our careers in ophthalmology, we were charged with the daunting task of teaching retina to ophthalmology residents. Clinical Retina evolved out of our efforts to provide residents the essential information about a given retinal condition in an organized, clinically relevant manner. Our goal was not to create another comprehensive, multi-volume reference book. There are several outstanding references currently available. Instead, we have attempted to develop a textbook-atlas that provides the reader a summary of the information on common and unusual retinal diseases. The use of highquality color figures was a must.
Contributing authors from the United States, Canada, and England have joined together to produce Clinical Retina. The textbook is organized using the traditional classification of retinal diseases. Within each chapter, conditions are presented in alphabetic order using a two-page format: text on the left page, figures on the right. Therefore, in two pages, the reader can review the essential material about a given retinal condition. The text is organized into sections: general information; symptoms; clinical features; ancillary testing; pathology/pathogenesis; treatment/prognosis; and systemic evaluation. A list of selected references is provided for those readers who desire more information.
Chapter 1 provides a review of the retinal anatomy and physiology and highlights important structures using ample clinical examples. Chapter 2 reviews commonly used ancillary studies including fluorescein angiography, electrophysiology, and ultrasonography.
Chapter 3, Clinical Features of Retinal Disease, describes a variety of ophthalmoscopic findings and provides a differential diagnosis of the most common conditions likely to manifest a particular feature. Photographic
examples are provided. This chapter provides a more “problem-based” approach to learning retina. If readers do not immediately recognize the diagnosis, they can identify significant clinical features and review the most common associations. Then, they can refer to the specific condition in the body of the textbook for more detailed information.
Chapters 4 through 12 cover the spectrum of retinal disease: Macular Diseases; Retinal Vascular Diseases; Hereditary Retinal Disorders; Drug Toxicities; Intraocular Tumors; Inflammatory Diseases; Trauma; Peripheral Retinal Diseases; and Diseases of the Vitreous. Chapter 13 reviews the histopathology of selected retinal diseases.
The major clinical trials for retinal diseases are summarized in Chapter 14, Clinical Trials in Retina. The reviews highlight the study objectives, treatment groups and trial design, summary of results, and implications for clinical practice. Chapter 15, Laser Photocoagulation and Photodynamic Therapy for Retinal and Choroidal Disease, provides information on the indications, treatment techniques, complications, and follow-up considerations of laser photocoagulation for a variety of conditions and photodynamic therapy for age-related macular degeneration.
Clinical Retina contains over 800 color and black and white photographs, one of the largest collections of retinal images in any ophthalmology textbook. The CDROM contains all of the figures displayed in the book as well as 50 additional images provided in a self-assess- ment program. Every attempt was made to ensure that the images used were of high quality and representative of the condition featured.
xi
ACKNOWLEDGMENTS
I am thankful to God for the opportunity to prepare this book. The entire process has been a tremendous life experience and I am grateful for the chance to participate. I appreciate the opportunity to collaborate with Barbara Blodi and all of the contributing authors during the preparation of Clinical Retina.
I would like to thank my wife, Betsy, for everything she has done for me over the past 20 years since we met in WAWA. She has been my teacher in so many ways and I am thankful to have her in my life.
I would like to thank my parents, Joan and Bob Quillen, and my brothers, Greg and Bobby Q, for their enthusiasm and support.
Throughout my life I have been blessed with special teachers. Mrs Schracht, Mrs Marrone, Mr Lange, Mr Mazziota, Mr Harris, Fr Burch, Cheston Berlin, George Blankenship, Tom Gardner, Ali Aminlari, Bill Cantore, Joe Sassani, James McManaway, George Rosenwasser, Don Gass, and numerous others have had a lasting impact on my life. They continue to inspire me to this day.
A special thank you to Tim Bennett, CRA, for his assistance in preparing this book. Photographs taken from Tim’s collection are scattered throughout this book and CD-ROM package. I appreciate the opportunity to share his gift with you.
Both Barb and I as well as many of the contributing authors have had the good fortune to work with Dr Gass as fellows. Dr Gass is an inspiration to us all. For me,
the opportunity to work with Dr Gass in Miami was life changing. He is a remarkable man in every way: honest, kind, thoughtful, insightful. His love of ophthalmology and humility regarding his accomplishments are examples for us all.
I would like to thank George Blankenship for his support and guidance. From the first day I decided to pursue a life in ophthalmology, George has been a friend and mentor.
I would like to thank my partners at the Penn State Department of Ophthalmology for their support over the past ten years. I am proud to be a member of the Penn State Ophthalmology family.
A special thank you to Barry Bowlus and Katharine Dvorak, and the rest of the staff at AMA Press who contributed to the development of Clinical Retina. To Barry, we appreciate your enthusiasm for this project and your unyielding commitment to excellence. To Katie, thank you for making this project your own. Your participation has greatly enhanced the quality of this book. —DQ
I am indebted to my parents, Frederick and Ottilie, who instilled in me a love of books, language, and learning. I am also grateful for my brother, Christopher, who has been a welcome mentor throughout my education and my professional career. My husband, Justin, has been a steady, calm, and encouraging influence on me; I thank him for his love, friendship, and constant support during this project. —BB
xii
ABOUT THE EDITORS
David A. Quillen, MD is currently Associate Professor of Ophthalmology and Residency Program Director at the Penn State College of Medicine in Hershey, Pennsylvania. He specializes in medical retina diseases.
He performed his ophthalmology residency training at the Penn State Ophthalmology Residency Program. He completed his Medical Retina fellowship at the Bascom Palmer Eye Institute, University of Miami under the direction of J. Donald M. Gass, MD. Dr Quillen has a strong interest in ophthalmology education and participates in a variety of local and national educational programs.
Dr Quillen is married to Betsy and has three children: Erin, Matthew, and Jack. In addition to ophthalmology, Dr Quillen enjoys dinner and a movie with Betsy, book shopping with Erin, playing and watching sports with Matt, and going to Chocolate World with Jack.
Barbara A. Blodi, MD is Associate Professor at the University of Wisconsin-Madison Medical School in the Department of Ophthalmology and Visual Sciences. She specializes in medical retina diseases.
Dr Blodi graduated from the University of Iowa School of Medicine in 1987 and completed her residency in ophthalmology at Bascom Palmer Eye Institute, University of Miami in 1991. She remained at Bascom Palmer for a fellowship in medical retina with J. Donald M. Gass, MD. This was followed by a year of surgical retina fellowship at the Kellogg Eye Center in Ann Arbor, Michigan, where Dr Blodi continued as a faculty member for two years.
Dr Blodi joined the faculty of the University of Wisconsin in 1997 and became Associate Professor in 2001. She is currently Co-director of the Fundus Photograph Reading Center and collaborates on the design, conduct, and analysis of clinical trials in ophthalmology, specifically in the areas of age-related macular degeneration and diabetic retinopathy.
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CONTRIBUTORS
G. William Aylward, MD |
Nancy M. Holekamp, MD |
Moorsfields Eye Hospital |
Barnes Retina Institute |
London, England |
St. Louis, Missouri |
Alistair J. Barber, PhD |
Michael S. Ip, MD |
Penn State College of Medicine |
University of Wisconsin Hospital and Clinics |
Hershey, Pennsylvania |
Madison |
Timothy J. Bennett, CRA |
Mark W. Johnson, MD |
Penn State College of Medicine |
Kellogg Eye Center |
Hershey, Pennsylvania |
Ann Arbor, Michigan |
Barbara A. Blodi, MD |
T. Mark Johnson, MD |
University of Wisconsin |
Bert M. Glaser National Retina Institute |
Madison |
Chevy Chase, Maryland |
Dean J. Bonsall, MD, FACS |
Peter J. Kertes, MD |
Penn State College of Medicine |
University of Ottawa Eye Institute |
Hershey, Pennsylvania |
Ontario, Canada |
Roy D. Brod, MD |
Kimberly A. Neely, MD, PhD |
Penn State College of Medicine |
Penn State College of Medicine |
Hershey, Pennsylvania |
Hershey, Pennsylvania |
Norman E. Byer, MD |
Timothy W. Olsen, MD |
University of California |
University of Minnesota |
Los Angeles |
Minneapolis |
David G. Callanan, MD |
Robert E. Parnes, MD |
University of Texas, Southwestern |
Cumberland Valley Retina Consultants |
Arlington |
Hagerstown, Maryland |
Vanessa Cruz-Villegas, MD |
David A. Quillen, MD |
Bascom Palmer Eye Institute |
Penn State College of Medicine |
Miami, Florida |
Hershey, Pennsylvania |
Thomas W. Gardner, MD, MS |
Robert H. Rosa, Jr, MD |
Penn State College of Medicine |
Scott and White Clinic |
Hershey, Pennsylvania |
Temple, Texas |
Justin L. Gottlieb, MD |
Philip J. Rosenfeld, MD, PhD |
University of Wisconsin |
Bascom Palmer Eye Institute |
Madison |
Miami, Florida |
J. William Harbour, MD |
|
Washington University |
|
St. Louis, Missouri |
|
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