Quick viewing(Text Mode)

Pathology of the Eye Dedicated to Hans Sautter and Lorenz E

Pathology of the Eye Dedicated to Hans Sautter and Lorenz E. Zimmerman G.O.H.Naumann D.J.Apple

Pathology of the Eye

With Contributions by D. von Domarus E.N.Hinzpeter R.M. Manthey L.R. Naumann K.W. Ruprecht H.E. Volcker

Translated by D.J. Apple

With 544 Illustrations in 1002 Parts

Springer-Verlag New York Berlin Heidelberg Tokyo G.O.H. NAUMANN, M.D. Chairman, Department of , University of Erlangen• Nlirnberg, 8520 Erlangen, Federal Republic of Germany

DJ. ApPLE, M.D. Professor of Ophthalmology and Pathology, University of Utah School of Medicine, University Health Sciences Center, Salt Lake City, Utah, 84132, U.S.A.

Library of Congress Cataloging in Publication Data Naumann, G.O.H. Pathology of the eye. "Translated and updated from the German edition: Pathologie des Auges"-T.p. verso. Includes bibliographies and index. 1. Eye-Diseases. I. Apple, David J., 1941- II. Title. [DNLM: 1. Eye-pathology. 2. Eye Diseases• pathology. WW 140 N311p] RE46.N3813 1985 617.17'1 85-14796

Translated and updated from the German edition: Pathologie des Auges. © by Springer-Verlag Berlin Heidelberg New York 1980.

© 1986 by Springer-Verlag New York, Inc. Softcover reprint of the hardcover 1st edition 1986 All rights reserved. No part of this book may be translated or reproduced in any form without written permission from Springer-Verlag, 175 Fifth Avenue, New York, New York 10010, U.S.A. The use of general descriptive names, trade names, trademarks, etc., in this publication, even if the former are not especially identified, is not to be taken as a sign that such names, as understood by the Trade Marks and Merchandise Marks Act, may accordingly be used freely by anyone. While the advice and information of this book is believed to be true 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 material contained herein.

Typeset by Kingsport Press, Tennessee.

9 8 765 4 3 2 I ISBN-13: 978-1-4613-8527-1 e-ISBN-13: 978-1-4613-8525-7 001-10.1007/978-1-4613-8525-7 Foreword

It is indeed a pleasure to write a foreword for this spectacular book which represents a landmark in the history of ocular pathology. Not since the three volumes of the Henke-Lubarsch handbook appeared forty years ago has anything like this come on the market. This book represents a compre• hensive, thorough and up-to-date clinically oriented textbook on ocular pathology. We are all indebted to the senior author, who is now professor of ophthal• mology at the University of Erlangen, who presents us here with the fruit of decades of industrious endeavors. We also have to be grateful to the American ophthalmic pathologist, Dr. David Apple, who, as one of the main collaborators of the German edition, has now provided us with the English translation. The book has a twofold purpose: First, it is meant to be a source of instruction for ophthalmologists and pathologists. For that purpose it is beautifully illustrated both by clinical pictures and by excellent photomicro• graphs and electromicroscopic pictures. Most valuable from a didactic point of view are the colored schematic drawings and the many tables. These two features are practically unique and should help any neophyte in grasp• ing the principles of ocular pathology. The second objective of this book is as a reference book for any type of investigation concerned with ocular pathology. The list of references (exceeding 5,000 citations) is indeed staggering and reflects the thorough• ness and scholarship of the authors. The references cover not only publica• tions in German and English, but also many works published in other languages. This gives the book a true cosmopolitan character. This book will become the yardstick against which any other future publi• cations in ocular pathology will have to be measured.

FREDERICK C. BLODI, M.D. Iowa City, Iowa Preface

We are pleased to introduce an English translation of this textbook of ocular pathology, which appeared with the title Pathologie des Auges in the original German edition in 1980. The original edition was the first compre• hensive textbook of ocular pathology that had appeared in the German language since the classic three volume work of Henke and Lubarsch, which was published between 1928 and 1937. In this English edition we have updated many sections and added numer• ous references that are current throughout 1985. Aside from the two-col• ored schematic line drawings and the differential diagnosis tables, one unique feature of this book is that it contains up-to-date English references from the literature, and references to much of the European literature of this century. This will be very useful for the scholar who wishes to research the background of many diseases. In addition to our colleagues and staff at the Universities of Hamburg, Tiibingen, Erlangen-Niirnberg, Illinois, and Utah, we would like to thank the Alexander von Humboldt Foundation, Bonn-Bad Godesberg, West Ger• many for support in the preparation of this translation and update. This work was done in part under the auspices of Professor Erich Weigelin of the University of Bonn, West Germany. We would also like to thank Dr. Randall J. Olson, Professor and Chairman of the Department of Ophthal• mology, University of Utah Health Science Center, for providing the finan• cial support and facilities that made this translation possible.

G.O.H. NAUMANN DAVID J. ApPLE Acknowledgments

We express our gratitude to Prof. H.C. Sautter, Director of the University Eye Hospital of Hamburg from 1953 through 1981. He allowed access to the files of clinical photographs and histopathological material, which represent a portion of the figures in this book. We also had the opportunity to utilize the collection of Prof. Wolfgang Stock, Director of the University Eye Hospital of Ttibingen from 1912 through 1953. The beautiful sections are still impressive and instructive today. We also express our gratitude to the staff of the University of Illinois Eye and Ear Infirmary and the Theobald Laboratory of Ocular Pathology for contributions of figures to this book. The source of figures is cited only if they have been previously published. The invaluable assistance of Miss Portwich and Mrs. Seibel in the prepa• ration of histological sections, and Miss Hadlok and Mr. Schneider in taking clinical photographs and providing photographs from the histological sec• tions, must be reemphasized for this edition. The graphic drawings were prepared by Mrs. Gay, Stuttgart, under the supervision of the senior author. At the University of Utah Health Sciences Center, Diane Rikkers and Lou H. Allred provided essential secretarial assistance, while Laura Lee Smith, Dolores K. Van Norman, and Steven M. Thiese furnished invaluable edito• rial assistance.

The significant contributions of the following people to this work is ac• knowledged: D. VON DOMARUS, M.D., Professor, Department of Ophthalmology, Univer• sity of Hamburg, 2000 Hamburg 20, Federal Republic of Germany E.N. HINZPETER, M.D., Chief, Eye-Hospital, Allgemeines Krankenhaus Heid• berg, 2000 Hamburg 20, Federal Republic of Germany R.M. MANTHEY, M.D., Jules Stein Eye Institute (UCLA), Kelman Fellow, and External Disease Division, UCLA Medical Center, Los Ange• les, California, USA L.R. NAUMANN, M.D., Department of Ophthalmology, University Erlangen• Ntirnberg, 8520 Erlangen, Federal Republic of Germany K.W. RUPRECHT, M.D., Professor, Department of Ophthalmology, University Erlangen-Ntirnberg, 8520 Erlangen, Federal Republic of Germany H.E. VOLCKER, M.D., Professor, Department of Ophthalmology, University Erlangen-Ntirnberg, 8520 Erlangen, Federal Republic of Germany General References in Ophthalmic Pathology

Apple Dj, Rabb MF: Ocular Pathology-Clinical Applications and Self• Assessment, ed. 3. (Formerly Clinicopathologico Correlation of Ocular Disease: A Text and Stereoscopic Atlas), Mosby, St. Louis, 1985 Garner A, Klintworth GK (eds.): Pathobiology of Ocular Disease: A Dy• namic Process (2 Vols), Marcel Dekker, Inc., New York, 1982 Hogan Mj, Zimmerman LE: Ophthalmic Pathology-An Atlas and Text• book, 2nd edition. W. B. Saunders Co, London-Philadelphia, 1962 Spencer WH (ed.), Font RL, Green WR, Howes ELjr,jakobiec FA, Zimmer• man LE: Ophthalmic Pathology-An Atlas and Textbook (3 Vols), 3rd edition. W.B. Saunders Co, Philadelphia, 1985 T.N.M.-Classification of Ophthalmic Tumors International Union Against Cancer, Geneva 1985 YanoffM, Fine BS: Ocular Pathology. A Text and Atlas, 2nd edition, Harper & Row, New York, 1982 World Health Organization: International Histological Classification of Tumours, Geneva, 1969-1980 Zimmerman LE, Sobin L: International Histological Classification of Tu• mors, Nr. 24: Histological Typing of Tumours of the Eye and its Adnexa WHO, Geneva, 1980 Contents

Selected Table of Contents xv

General Anatomy and Development of the Eye: Techniques of Investigation DJ. ApPLE, G.O.H. NAUMANN

2 Microscopic Anatomy of the Eye DJ. ApPLE, G.O.H. NAUMANN, and R.M. MANTHEY 19

3 Malformations and Anomalies of the Eye DJ. ApPLE and G.O.H. NAUMANN 63

4 Intraocular Inflammations G.O.H. NAUMANN and L.R. NAUMANN. 99

5 Accidental and Surgical Trauma and Wound Healing of the Eye D. VON DOMARUS and G.O.H. NAUMANN. 185

6 H.E. VOLCKER and G.O.H. NAUMANN. 249

7 Cornea and E.N. HINZPETER and G.O.H. NAUMANN 317

8 G.O.H. NAUMANN. 413

9 G.O.H. NAUMANN. 509

10 Vitreous H.E. VOLCKER and G.O.H. NAUMANN. 557

11 DJ. ApPLE and G.O.H. NAUMANN 577 XIV Contents

12 General Pathology of the Retina: Correlation of the Ophthalmoscopic Appearance with Tissue Morphology G.O.H. NAUMANN and W. LULLWITZ ...... 697

13 G.O.H. NAUMANN and DJ. ApPLE .. 723

14 and Ocular Hypotony: Pathology of Abnormal Intraocular Pressure G.O.H. NAUMANN...... 771

15 Ocular Adnexae: and DJ. ApPLE and G.O.H. NAUMANN...... 849

16 The Eye and Systemic Disease K.W. RUPRECHT and G.O.H. NAUMANN ...... 873

17 Morphology of Drug-Induced Ocular Changes K.W. RUPRECHT and G.O.H. NAUMANN 957

Index 973 Selected Table of Contents

1 General Anatomy and Development of the Eye: Techniques of Investigation DJ. ApPLE and G.O.H. NAUMANN

Embryology and Development of the Eye Basic Ocular Structure . . . . . 1 Origin and Development of the Eye 2 Eye of the Newborn . . . . . 6 Growth and Aging Changes 9 Major Causes of 10 Gross Examination and Histologic Techniques 12 External Orientation ...... 12 Fixation, Sectioning, and Preparation of Histologic Sections 13 Important Histologic Stains in Ocular Pathology 13 Examination with Polarized Light 16 References 16

2 Microscopic Anatomy of the Eye DJ. ApPLE, G.O.H. NAUMANN, and R.M. MANTHEY 19

Conjunctiva ...... 19 Cornea and Sclera (Tunica Fibrosa) 20 Cornea 20 Sclera 23 Aqueous Filtration Structures 24 Lens .. .. 25 Embryology 25 Histology 29 Vitreous 30 Uvea 32 32 Embryology and Histology 32 ..... 36 ...... 37 Vascular Supply of the Uvea 38 XVI Selected Table of Contents

Retina ..... 38 Embryology 38 General Structure 39 Microscopic Structure 41 Internal Limiting Membrane 41 Nerve Fiber Layer 41 Ganglion Cell Layer 41 Inner Plexiform Layer 42 Inner Nuclear Layer 42 Outer Plexiform Layer 42 Outer Nuclear Layer . 43 External Limiting Membrane 43 Photoreceptors . . . . . 44 Retinal Pigment Epithelium 45 Bruch's Membrane 46 Macula Lutea 46 Peripheral Retina . 47 Vascular Supply of the Retina and Pigment Epithelium 48 Optic Nerve 50 Embryology 50 Biomicroscopy 51 Histology 51 Vascular Supply 54 Visual Pathways 55 Ocular Adnexa 55 Eyelids 55 Lacrimal Apparatus 57 58 References 58

3 Malformations and Anomalies of the Eye DJ. ApPLE and G.O.H. NAUMANN 63

Malformations of the Primary Optic Vesicle 63 Anomalies of Optic Vesicle Formation 63 Anophthalmia ...... 63 Cryptophthalmos ...... 64 Anomalies of the Rostral Forebrain 65 Cyclopia and Synophthalmia 65 Ethmocephaly, Cebocephaly, and Arrhinencephaly 65 Congenital Cystic Eye and Congenital Detachment of the Retina ...... 65 Colobomas ...... 66 Typical Colobomas (Anomalous Formation of the Optic Cup) 66 Iris Colobomas ...... 67 Colobomas of the Ciliary Body 67 Fundus Colobomas (Retina-Choroid) 67 Congenital Orbital Cyst . . . . 69 Colobomas of the and Optic Nerve 70 Selected Table of Contents XVII

Atypical Colobomas (Colobomas in an Atypical Direction) 73 Atypical Colobomas of the Iris 73 Optic Pit 73 "Macular Colobomas" 74

Anomalies Based on the Size of the Eye 75 Diminished Size (Microphthalmos) 75 Enlargement of the 76 Macrophthalmia 76 Congenital Glaucoma (Buphthalmos) (See Chapter 14) 76 76

Pigment Anomalies 80 Albinism 80 Chediak-Steinbrinck-Higashi Syndrome 81 Congenital Melanosis Oculi 81 Heterochromia Iridis and Iris Bicolor 82

Chromosomal Anomalies 84 Trisomy 21 85 Trisomy 13 and Chromosome 13 Defect 87 Trisomy 16-18 and 18 Deletion Defect 88 Chromosome-5 Defect (Cri du Chat Syndrome) 88 Sex Chromosome Anomalies 88 Klinefelter's Syndrome 88 Turner's and Noonan's Syndromes 90

References 90

4 Intraocular Inflammations G.O.H. NAUMANN and L.R. NAUMANN 99

Terminology 99 Localization of Tissue Changes 99 Histopathologic Tissue Changes 101 Granulomatous Inflammation 101 Nongranulomatous Inflammation 102 Portal of Entry 104 Course of the Disease 105 Acute Intraocular Inflammations 105 Subacute Intraocular Inflammations 105 Chronic Recurrent Intraocular Inflammations 105 Etiology 106 Pseudouveitis and Pseudoendophthalmitis 106

General Symptomatology 106 Exudation 106 Exudation into the Anterior Chamber 108 Exudation in the Vitreous 109 Exudation within the Anterior Uvea 109 Exudative 110 XVIII Selected Table of Contents

Retinal Vasculitis and Perivasculitis III Cystoid III Swelling of the Optic Disc III Destruction in the Acute Phase 112 Corneal Endothelium 112 Anterior Uvea 112 Vitreous 112 Choroid 112 Retina 113 Reactive Proliferation and Chronic Inflammatory Changes 116 Pigment Epithelium 116 Vascular Connective Tissue 119 119 Cyclitic Membrane . 121 Proliferative 121 Choriocapillaris Neovascularization 122 Glial Tissue 123

Complications of Intraocular Inflammation 123 Corneal Pannus and 123 Lens Changes 125 Secondary Glaucoma 125 Hypotony 126 Retinal Complications 126

Clinical Classification of Intraocular Inflammation 127 Infectious 127 Bacteria and Fungi 127 Virus 134 Protozoa 139 Parasites 145 Ophthalmomyiasis 147 Intraocular Inflammation Caused by Presumed Organisms 148 Beh~et's Disease 148 Whipple's Disease . 148 Reiter's Syndrome 148 Intraocular Inflammation without Known Organisms (N oninfectious) 148 Isolated Intraocular Inflammation without Known Organisms (No Systemic Manifestations) 149 Nonspecific, Nongranulomatous Diffuse Anterior 149 Autologous Reactions 150 Special Forms of Uveitis without Known Causative Organisms 155 without Known Causative Organisms 161 Intraocular Inflammation without Organisms Seen in General Diseases and Syndromes 162 Granulomatous Inflammations 162 N ongranulomatous Inflammations 163 Pseudouveitis and Pseudoendophthalmitis 166 Hypoxia 166 Selected Table of Contents XIX

Intraocular Neoplasms 167 Metabolic Diseases 170 References 170

5 Accidental and Surgical Trauma and Wound Healing of the Eye D. VON DOMARUS and G.O.H. NAUMANN 185

Mechanical Trauma 185 Perforating and Penetrating Wounds of the Globe 185 Paracentesis Effect 185 Prolapse of Intraocular Tissue 185 Hemorrhage and Its Sequelae 187 Posttraumatic Inflammation 189 Wound Dehiscence and Sequelae of Hypotony (See Chapter 14) 192 Traumatic 192 Intraocular Scar Formation (See Chapter 4) 193 Epithelial Ingrowth 193 Stromal Ingrowth and Retrocorneal Fibrous Membrane Formation 196 Atrophia Bulbi and Phthisis Bulbi 197 Intraocular Foreign Bodies and Metals 198 Siderosis Bulbi 199 Chalcosis Bulbi 201 Organic Material 201 Miscellaneous 201 Artificial Intraocular Lenses 202 Contusion of the Eye and Its Sequelae 203 Rupture of the Globe 203 Corneal-Scleral Injury 206 Uveal Injury 210 Lens Injury 211 Retinal-Vitreal Injury 212 Optic Nerve Injury 214 Contusion Effects on the Vitreous 215 Postcontusion Orbital Changes 216 Ocular Changes After Extraocular Trauma 216 Meningeal Hemorrhage . 216 Retinal Hemorrhages Associated with Acute Increases in Venous Pressure 216 Purtscher's Traumatic Retinopathy 217 Retinal Emboli 217 Surgical Trauma 217 Sequelae of Intraocular Operative Procedures 217 Retinal Detachment Operations 222 Complications Following Operations 225 Chemical and Thermal Burns 226 xx Selected Table of Contents

Actinic Changes (Injury Due to Electromagnetic Waves) 227 Therapeutic Light Coagulation 228 Wound Healing 235 Cornea and Sclera 235 Uvea 238 Lens 239 Vitreous 240 Retina and Optic Nerve 240 References 240

6 Conjunctiva H.E. VOLCKER and G.O.H. NAUMANN 249

Congenital Malformations 250 Circulatory Defects and Vascular Changes 251 Conjunctival Inflammation 253 Acute Forms 253 Follicular 255 Chronic Conjunctivitis 256 Etiologic Classification of Conjunctivitis 261 Infectious Conjunctivitis 261 Noninfectious Conjunctivitis 266 Conjunctivitis Due to Tear Film Disturbances 270 Neoplastic Pseudoconjunctivitis 270 Conjunctiva and Systemic Disease 270 Metabolic Diseases 270 Conjunctival Diseases Dermatologic Disorders 273 Conjunctival Involvement Rheumatic and Collagen Diseases 275 Conjunctival Involvement Acute Graft-versus-Host Disease 275 Vitamin Deficiencies . 275 Degeneration, Aging Changes, and Deposition of Pharmacologic Agents 275 275 277 Amyloidosis 279 Deposition of Medicinal Agents in the Conjunctiva 279 Tumors of the Conjunctiva 280 Hamartomas and Choristomas 280 Epithelial Tumors 281 Superficial Epithelium 281 Carcinoma 287 Miscellaneous Epithelial Tumors 289 Basal Cell Carcinoma 290 Melanocytic Tumors 291 Benign Tumors 291 Precancerous Lesions: Acquired Intraepithelial Melanosis with Junctional Activity (Stage IB) 298 Malignant Melanoma of the Conjunctiva 298 Selected Table of Contents XXI

Stromal Tumors 299 Mesenchymal Tumors 299 Vascular Tumors 300 Neural Tumors 301 Tumors of Hematopoietic and Lymphatic Tissues 302 Inflammatory Pseudo tumors 304

References 305

7 Cornea and Sclera E.N. HINZPETER and G.O.H. NAUMANN 317

Cornea 317 Congenital and Infantile Diseases of the Cornea 317 Abnormal Diameter and Shape 317 Congenital Corneal Opacities (Leukomas) 319 Diffuse Opacification 319 Peripheral Leukomas 322 Central Leukomas 323 Staphyloma 325 Descemet's Folds and Ruptures After Forceps Birth Injury 326 Dermoids 327 Aging Changes and Degeneration 327 Aging and Environmental Factors 327 Corneal Degeneration Due to Other Ocular Diseases 329 Band Keratopathy 329 Pannus 330 Secondary Spheroidal Keratopathy 330 Salzmann's Nodular Degeneration 330 Lipid Keratopathy 331 Secondary Amyloidosis 331 Corneal Degeneration Caused by Deficiencies in the Tear ~lm 332 Degenerations Associated with Systemic Diseases 334 Hereditary Familial Corneal Dystrophies (Including Hereditary Systemic Metabolic Diseases Involving the Cornea) 338 Corneal Dystrophies Involving the Epithelium, Basement Membrane, and Bowman's Layer 338 Meesmann's Juvenile Hereditary Epithelial Dystrophy 339 Cogan's Microcystic Epithelial Dystrophy 339 Recurrent Nontraumatic Epithelial Erosions 339 Hereditary Anterior Membrane Dystrophy 339 Primary Hereditary Band Keratopathy 340 Superficial Ring Dystrophy of Reis-BUcklers 340 Vogt's Anterior Crocodile Shagrin (Mosaic Degeneration) 341 Cornea Verticillata (Fleischer-Gruber' s Disease) 341 Subepithelial Hereditary 341 341 XXII Selected Table of Contents

Stromal Dystrophies 343 Granular Dystrophy (Bticklers Type I or Groenouw Type I Dystrophy) 343 Lattice Dystrophy and Primary Corneal Amyloidosis 345 Macular Dystrophy (Bticklers Type II or Groenouw Type II Dystrophy) 348 Central Crystalline Corneal Dystrophy of Schnyder 349 Cornea Farinata ("Pre-Descemet's" Membrane Dystrophy) 350 Congenital Hereditary Stromal Dystrophy of the Cornea 350 Corneal Endothelial Dystrophy 350 Congenital Hereditary Corneal Dystrophy 350 Corneal Guttata 350 Fuchs' Combined Endothelial and Epithelial Dystrophy 352 Posterior Polymorphous Dystrophy of Schlichting 353 Chandler's Syndrome 356 Corneal-Endothelial Proliferation Syndromes 356 Corneal Dystrophies Associated with Familial Hereditary Metabolic Diseases 356 Mucopolysaccharidoses 356 Sphingolipidoses 356 Mucolipidoses 356 Disturbances in Protein and Amino Acid Metabolism 357 Lipidoses 357 Disturbances of Metal Metabolism 358 359 Nonulcerative Keratitis 359 Epithelial and Subepithelial Keratitis 359 Interstitial (Stromal) Keratitis 360 Ulcerative Keratitis Due to Known Organisms 362 Viral Keratitis 363 Bacterial Keratitis "Serpiginous Ulcer" 363 Mycotic Keratitis 366 Immune Ring of W essely and Ring Abscess 367 Ulcerative Keratitis without Known Organisms 367 Fuchs' Dellen 367 Phlyctenular Keratitis (Eczematous Keratitis) 367 Marginal Keratitis 367 "Ring Ulcers" 370 Fuchs' Marginal Degeneration (Terrien's Ulcer) 370 Pellucid Marginal Degeneration 370 Mooren's Ulcer (Rodent Ulcer) 370 Ulceration Associated with Tyrosine Aminotransferase Deficiency 371 371 Dendritic Keratitis 371 Geographic Epithelial Keratitis 374 "Disciform Keratitis" (Deep Stromal Keratitis without Ulceration) 374 Deep Keratitis with Ulceration (Metaherpetic Keratitis) 374 Granulomatous Reaction to Descemet's Membrane 374 Complications of Chronic Keratitis 374 Selected Table of Contents XXlI1

Corneal and Vascularization 374 Corneal Edema 374 Vascularization 377 Penetrating Keratoplasty 381 Unsuccessful Keratoplasty 383 Pigmentation 386 Medicinal Agents and Other Chemicals 388 Tumors of the Cornea 390 Sclera 390 Congenital Malformations 390 Degenerations 390 Senile Hyaline Scleral Plaques 390 Staphylomas and Ectasias 391 "Spontaneous" Scleral Perforations 391 Inflammations 392 Tumors 393 References 393

8 Uvea G.O.H. NAUMANN 413

Anatomic Characteristics and Physiology 413 Optical Function 413 Extravascular Intraocular Circulation 413 Nutritive Functions of the Ciliary Body and Choroid 414 Localization of the Barriers Between the Vascular System and the Eye 414 Aging Changes 415 Malformations and Anomalies 416 Uvea as a Whole 416 Extramedullary Hematopoiesis in the Uvea 416 Congenital Ocular Melanosis 416 Albinism 416 Colobomas 419 Iris 419 Uveal Degenerations and Dystrophies 420 Systemic Diseases 420 Diabetes Mellitus 420 Marfan's Syndrome 421 Isolated Dystrophies and Degenerations 422 Iris and Ciliary Body 422 Choroidal Degenerations and Dystrophies 423 Vascular Diseases Affecting the Uvea 425 Hyperemia and Hemorrhage 426 Ischemic Infarcts of the Uvea 426 Ischemic Infarcts of the Iris and Ciliary Body 426 Choroidal Infarcts 427 Uvea in Systemic Vascular Diseases 427 Malignant Accelerated Arterial Hypertension 427 Arteriosclerosis 427 XXIV Selected Table of Contents

Uveal Neovascularization 427 Rubeosis Iridis 427 Cyclitic Membranes 428 Neovascularization Derived from the Choriocapillaris 428

Inflammation of the Uvea (See Chapter 4) 429 Trauma (See Chapter 5) 430 Tumors of the Uvea 430 Growths and Tumors Derived from Uveal Stromal Melanocytes 430 Benign Tumors 431 Congenital Melanosis Oculi 431 Nevus ofOta 431 Blue Nevus 431 Melanocytic Nevi of the Uvea 431 Ir~ 432 Posterior Uvea 434 Ciliary Body 437 Choroid 438 Optic Nerve 441

Malignant Melanoma of the Uvea 441 Epidemiology 441 Localization 443 Gross Appearance 443 Biopsy 443

Malignant Melanoma of the Iris 443 Malignant Melanoma of the Ciliary Body 446 Malignant Melanoma of the Choroid 447 Diagnosis 447 Gross Appearance 450 Routes of Invasion 451 Secondary Changes in Neighboring Tissues 454

Differential Diagnosis of Uveal Malignant Melanoma 456 Histopathologic Classification of Uveal Malignant Melanoma 459 Prognosis of Uveal Malignant Melanomas 464 Origin of Uveal Malignant Melanomas and Their Relationship to Other Melanocytic Tumors 468 Primary Nonmelanocytic Tumors of the Uveal Stroma 469 Hemangioma of the Uvea 469 Osteoma of the Choroid 469 Choristomas 470 Primary Rhabdomyosarcoma of the Iris 470 Leiomyoma 471 Neurofibroma 471 Histiocytic Tumors 472 Massive Reactive Lymphoid Hyperplasia of the Uvea 473 Secondary Tumors and Metastases to the Uvea 473 Local Invasion of Uveal Tissues from Other Malignant Ocular Tumors 473 Metastases to the Uvea 474 Selected Table of Contents xxv

Uveal Involvement in Diseases of the Hematopoietic System and Malignant Lymphomas 475 Reactive Growth and Tumors of the Pigment Epithelium of Iris, Ciliary Body, and Choroid 476 Tumorlike Growths and Proliferation of the Retinal Pigment Epithelium 477 Idiopathic 479 Extraocular Tumors Which Contain Ocular Pigment Epithelium 479 Tumors of the Nonpigmented Ciliary Body Epithelium 479 Cys~ 479 Reactive Hyperplasia and Pseudoadenomatous Hyperplasia 480 Embryonal and Adult Tumors Primarily Derived from the Nonpigmented Epithelium of the Ciliary Body (Neuroepithelial Tumors of the Ciliary Body) 480 Adenocarcinoma and Adenoma of the Nonpigmented Ciliary Epithelium 483 References 483

9 Lens G.O.H. NAUMANN 509

Anatomy and Physiology of the Lens 509 Aging Changes 512 General Pathology 512 Lens Epithelium and Fibers 513 Abnormal Proliferative Changes 513 Degenerative Changes 515 Capsule 516 Changes in Zonular Fibers: Luxation of the Lens 522 Isolated Dislocation 523 Systemic Diseases 523 Traumatic Luxation 525 Special Pathology 525 Primary Abnormalities of the Lens 525 Malformations of the Lens 525 Congenital and Juvenile Cataract 526 Total 526 Partial Congenital and Juvenile 526 Nuclear 526 Zonular 526 Crystalline 526 Anterior Polar 526 Posterior Polar 527 Lenticonus and Lentiglobus 527 Rubella 527 Congenital Cataracts Associated with Generalized Syndromes 528 Experimental Congenital Cataracts 528 Chromosomal Anomalies 528 XXVI Selected Table of Contents

Senile Cataracts 528 Consecutive Cataracts 530 Lens Changes Associated with Intraocular Diseases 530 Other Lens Changes 531 Exogenous I~ury to the Lens 531 Lens Opacities and Systemic Diseases 541

Lens-Induced Intraocular Diseases 542 Experimental Cataracts 546 References 546

10 Vitreous H.E. VOLCKER and G.O.H. NAUMANN

Embryonic Development 557 Microscopic Anatomy 558 Anomalies and Malformations 559 Persistent Primary Vitreous 559 Anterior Persistent Primary Vitreous: Mittendorf Dot 559 Posterior Persistent Primary Vitreous: Bergmeister's Papilla 559 Persistent Hyperplastic Primary Vitreous 560 Unilateral PHPV 560 Bilateral PHPV 561 Differential Diagnosis 561

Aging Changes 561 Vitreous Liquefaction 561 Vitreous Detachment 562 Posterior Vitreous Detachment 562 Basal Vitreous Detachment 564 Anterior Vitreous Detachment 566

Pathologic Vitreous Opacities 566 Degenerative Changes 566 Vitreous Opacities in Myopia 566 Asteroid Hyalosis 567 Synchysis Scintillans (Cholesterolosis) 567 Amyloidosis of the Vitreous 568 Vitreoretinal Dystrophies 568 Juvenile 568 Wagner's Disease 570 Goldmann-Favre Disease 570 Other Vitreoretinal Dystrophies 570

Epiretinal and Vitreal Proliferations (Avascular Type) 571 Primary Epiretinal Gliosis (Not Associated with Complete Retinal Holes) 572 Secondary Periretinal Pigment Epithelial and Glial Proliferation (Associated with Complete Retinal Holes) 572

References 573 Selected Table of Contents XXVII

11 Retina DJ. ApPLE and G.O.H. NAUMANN 577

Congenital Anomalies and Hereditary Diseases 577 Hypoplasia and Dysplasia of the Retina 577 Myelinated (Medullated) Retinal Nerve Fibers 578 Congenital Retinal Folds and Congenital Falciform Folds 579

Vascular Diseases of the Retina 579 580 Retinal Vascular Occlusions 584 Central Artery Occlusion (Ischemic Retinopathy) 584 Retinal Venous Occlusion 586 Retinopathia Proliferans 589 Histopathology . 591 Retinopathia Proliferans with Fibroglial Proliferation 592 Recurrent Vitreous Hemorrhage and Organization 594 Macular Changes 595 Traction Retinal Detachment 595 Rubeosis Iridis and Cyclitic Membranes 595 Prognosis . 595 Eales' Disease 596 (See Chapter 16) 596 Sickle Cell Hemoglobinopathy (See Chapter 16) 596

Degenerations of the Peripheral Retina, Retinal Breaks, and Retinal Detachment 596 Degenerations 597 Peripheral Retinal Lesions that Rarely Produce Retinal Breaks 597 Sensory Retina 597 Retinal Pigment Epithelium 600 Chorioretinal Degeneration 600 Ora Serrata 601 Pars Plana Cysts and Cystic Excavations 602 Vitreoretinal Interface Changes 603 Retinal Pigment Epithelium and Sensory Retina 603 Lesions Predisposing to Retinal Breaks 603 Retinal Breaks 604 Retinal Detachment 606 Clinical Features 608 Histology 609

Dystrophic Lesions Affecting the Peripheral Retina (Differential Diagnosis of Retinopathia Pigmentosa) 611 Isolated Retinopathia Pigmentosa 612 Clinical Features 612 Histologic Features 612 Variations 613 Systemic Syndromes with Pigmentary Retinopathy 613 "Pseudoretinitis Pigmentosa" 615 Retinopathia Sclopeteria 615 XXVlll Selected Table of Contents

Postinftammatory and Postinfectious Pseudoretinitis Pigmentosa 616 Ischemic Infarcts of the Choroid and Retina 616 Toxins 616 Dystrophy of the Peripheral Choroid 616 Diseases of the Macula 616 Classifications 616 Anatomic Classification of Macular Diseases 619 Vitreoretinal Dystrophies 619 Sex-Linked Juvenile Retinoschisis 619 Goldmann-Favre Disease 620 Nerve Fiber Layer and Ganglion Cell Layer 620 Hereditary Cerebromacular Degeneration with a Cherry-Red Spot (Tay-Sachs Disease) 620 Hereditary Cerebromacular Diseases without a Cherry-Red Spot 620 Outer Plexiform Layer 622 Cystoid 622 Persisting Cystoid Maculopathy with Vitreous Incarceration (Hruby-Irvine-Gass Syndrome) 622 Stellate and Circinate Retinopathy 622 Photoreceptors, Pigment Epithelium, Bruch's Membrane, and Choroid 622 Dominant (Familial) Drusen 624 Fundus Flavimaculatus and Stargardt's Disease 625 Best's Vitelliform Dystrophy 625 Central Areolar Choroidal Dystrophy 625 Central (Inverse) 626 Miscellaneous Diseases of the Macula Affecting Retinal Pigment Epithelium and Bruch's Membrane . 626 Angioid Streaks 626 Acute Posterior Multifocal Placoid Pigment Epitheliopathy 626 Toxic Maculopathy 627 Diseases Causing Localized Detachment of the Retina and Pigment Epithelium 627 Idiopathic Central Serous Choroidopathy (Central Serous or Angiospastic Retinopathy) 627 Senile Macular Degeneration (Nondisciform and Disciform) 628 Tumors of the Retina and the Pigment Epithelium 631 Retinoblastoma 632 General Clinical Features 633 Pathology 641 Laboratory Diagnosis 643 Therapy 644 Course and Prognosis 645 Extraocular "Retinal Anlage Tumor" 647 Pseudoretinob1astoma (Pseudoglioma, , Amaurotic Cat's Eye) 647 Differential Diagnosis of Leukocoria 648 Persistent Hyperplasia of Embryonic Intraocular Vasculature 650 Selected Table of Contents XXIX

Persistent Hyperplastic Primary Vitreous (PHPV) 651 Toxic Retinopathy (Retinopathy of Prematurity) 655 Retinal Vascular Diseases with Lipid Exudation 658 Coats' Disease ...... 658 Leber's Military Aneurysm 659 Cavernous Hemangioma of the Retina 662 Inflammatory Conditions .... 662 Retinal Malformations .... 663 Prenatal and Infantile Trauma, Organizing Vitreous Hemorrhage, and Massive Retinal Gliosis 667 Miscellaneous Proliferative Lesions, Hamartomas, and Choristomas of the Fundus 668 References 669

12 General Pathology of the Retina: Correlation of the Ophthalmoscopic Appearance with Tissue Morphology G.O.H. NAUMANN and W. LULLWITZ ...... 697

Ophthalmoscopy and Histopathology ...... 699 Ophthalmoscopic Visibility of Retinal Diseases .... 699 "Bordering Structures" and their Manifestations in Retinal Disease ...... 701 Horizontal "Bordering Structures" 703 Vertical "Bordering Structures" 703 Double Vascular Supply 706 "Passive" Sensory Retina-"Reactive" Pigment Epithelium 706 Ophthalmoscopic Elements 706 Retinal "Folds" 706 Hemorrhage 707 Vascular Changes 708 Discoloration of the Fundus 710 Retinal Edema . . . . . 710 Retinal Defects . . . . . 711 Pigment Changes of the Retina 711 "Exaggerated Macular Reaction" Secondary to Intraocular Diseases ...... 712 Morphology and Functional Disturbances 716 References ...... 719

13 Optic Nerve G.O.H. NAUMANN and DJ. ApPLE 723

Malformations, Anomalies, and Hereditary Diseases 723 Aplasia and Hypoplasia .... 723 Anomalies of the Optic Nerve Head 724 Vascular Anomalies 724 Epipapillary Membranes 724 xxx Selected Table of Contents

Myelinated Nerve Fibers 725 Drusen of the Optic Nerve Head 725 Pseudoneuritis, Pseudopapilledema 727 Physiological Variations of the Optic Nerve Head 727 Anomalies in Shape 727 Anomalous Excavation 727 Congenital Conus or Crescent 728 Congenital and Hereditary Optic Atrophy 728 Leber's Optic Atrophy 728 Dominant Juvenile Optic Atrophy 729 Leukodystrophies 729 Schilder's Disease (Sudanophilic Leukodystrophy) 729 Krabbe's Disease 730 Metachromatic Leukodystrophy 730 Pelizaeus-Merzbacher Syndrome 730 Leigh's Necrotizing Encephalomyelopathy 730

Papilledema without Primary Axonal Damage ("Swollen Disc" or "Stauungspapille") 730 General Morphology 730 Clinical Features 731 Etiologic Classification 732 Prelaminar (Intraocular) Disease 732 Retrolaminar Disease 732 Venous Outflow Disturbances 735 Secondary to Increased CSF Pressure: "Stauungspapille" 735 Pseudopapilledema 736 Histopathology 736 Differential Diagnosis 736

Acute Optic Nerve Disease with Primary Damage to Axons 737 Papillitis and Neuroretinitis (Inflammation) 738 Clinical Presentation 738 Anatomic Classification 738 Etiologic Classification 738 True Inflammatory Processes 739 Demyelinating Diseases 740 Multiple Sclerosis 741 Acute Disseminated Encephalomyelitis 743 Neuromyelitis Optica of Delvic 743 Schilder's Disease 743 Vascular Disease 746 Anterior Ischemic Due to Arteriosclerosis 746 Anterior Ischemic Optic Neuropathy Due to Temporal Arteritis 748 Pulseless Disease 749 Radiation-Induced Ischemic Neuropathy 749 Degenerative and Toxic Optic Nerve Diseases 749 Exogenous Factors 749 Neoplastic Diseases 750 Endocrine Orbitopathy 750 Selected Table of Contents XXXI

Optic Atrophy: Chronic Optic Nerve Disease with Loss ofAxons 750 Histopathology ...... 750 Special Forms of Atrophy of the Optic Nerve Head 751 Classification of Acquired Optic Atrophy 753 Ophthalmoscopic and Gross Appearance 753 Pathogenetic Classification 753 Tumors of the Optic Nerve 754 Prelaminar Optic Nerve Tumors 754 Melanocytoma . . . . . 754 Malignant Melanoma of the Choroid Extending Onto the Optic Disc ...... 756 Pigment Epithelial Proliferation and Hyperplasia 757 Retrobulbar Optic Nerve Tumors 757 Glioma ...... 757 Meningioma ...... 760 Metastatic Tumors to the Optic Nerve 761 Zimmerman's Choristoma of Optic Nerve 761 References 761

14 Glaucoma and Ocular Hypotony: Pathology of Abnormal Intraocular Pressure G.O.H. NAUMANN . . . . 771

Physiology of Aqueous Circulation 772 Physiological Resistance 774 Aging Changes 781 Glaucoma 781 Pathogenesis of Increased Intraocular Pressure 781 Closed-Angle Glaucoma ...... 784 Angle-Closure Glaucoma with Pupillary Block 785 Angle-Closure Glaucoma Due to Ciliary Block 787 Angle-Closure Glaucoma without Pupillary or Ciliary Block 790 Open-Angle Glaucoma . . . . 793 Primary Open-Angle Glaucoma 797 Chronic Simple Glaucoma 797 Juvenile Glaucoma . . . . 798 Late Juvenile Glaucoma (Sautter) 798 Primary Chronic Open-Angle Glaucoma Associated with Myopia ...... 798 Pigmentary Glaucoma or Pigment Dispersion Syndrome 799 Chronic Open-Angle Glaucoma Associated with Diabetes Mellitus ...... 800 Corticosteriod-Induced and Glaucoma ...... 800 Pseudoexfoliation Syndrome (Glaucoma Capsulare) 801 Comment ...... 803 Secondary Open-Angle Glaucoma 803 Associated with Uveitis 804 a-Chymotrypsin-Induced Ocular Hypertension (Kirsch) 805 XXXII Selected Table of Contents

Secondary Open-Angle Glaucoma Induced by Blood in the Anterior Chamber ...... 806 Secondary Open-Angle Associated with Contusion Angle Deformity ...... 807 Macrophage-Induced Glaucomas ...... , 808 Pseudoexfoliation Syndrome ("Glaucoma Capsulare") 809 Open-Angle Glaucoma Induced by Intraocular Tumor 810 Epithelial Ingrowth into the Anterior Chamber 811 Secondary Open-Angle Glaucoma Caused by Orbital Diseases ...... 812 Secondary Open-Angle Glaucomas Associated with Dilated Episcleral Vessels ...... 812 Congenital and Infantile Glaucoma (Buphthalmos, Hydrophthalmia) ...... 812 Isolated Buphthalmos ...... 812 Buphthalmos Secondary to Other Intraocular Malformations and Diseases ...... 813 Buphthalmos Associated with Systemic Disease 815 Secondary Buphthalmos ..... 815 Critical Considerations of Classification of Secondary Glaucoma 816 Effects of Ocular Hypertension 817 Glaucomatous Damage in Adults 818 Changes in Acute Glaucoma 818 Changes in Chronic Glaucoma 822 Low-Tension Glaucoma 827 Pathologic Findings in Buphthalmos 828

Ocular Hypotony . . 828 Pathogenesis 828 Hyposecretion 828 Hypotony Due to Increased Outflow of Aqueous Fluid 828 Sequelae of Hypotony ...... 829 Hypotony Associated with Atrophia and Phthisis Bulbi 830

References 832

15 Ocular Adnexae: Eyelids and Lacrimal Apparatus DJ. ApPLE and G.O.H. NAUMANN ...... " 849

Eyelids ...... 849 Inflammatory Diseases and "Pseudotumors" 849 .... 849 Molluscum Contagiosum 852 Benign Lid Tumors 852 852 Squamous Papilloma 852 Seborrheic Keratosis 852 Cysts 853 Hemangioma and Lymphagioma 854 Melanocytic Nevi . . . . . 854 Selected Table of Contents XXXlll

Phakomatous Choristomas of the Lid (Zimmerman's Tumor) 856 Pseudo epitheliomatous Hyperplasia 856 Malignant Tumors of the Eyelid 856 Basal Cell Carcinoma 856 Squamous Cell Carcinoma 858 Sebaceous Gland Tumors 860 Metastatic Eyelid Tumors 863 Lacrimal System 864 Tumors of the Lacrimal Gland 864 Inflammatory Pseudo tumors 864 Pathology of the Lacrimal Outflow Channels and Lacrimal Sac 864 Orbit 865 Graves' Disease (Endocrine Orbitopathy, Basedow's Disease) 865 Tumors and Pseudo tumors of the Orbit 865 Differential Diagnosis of Primary Orbital Tumors 865 Orbital Tumors of the Adults 865 References 866

16 The Eye and Systemic Disease K.W. RUPRECHT and G.O.H. NAUMANN . 873

Factors Determining the Differential Localization of Ocular Changes Associated with Systemic Diseases 873 Blood-Ocular Barriers 873 Vascular Supply to the Eye 873 Tissue Elements in the Eye 873 In traocular "Bordering Structures" 875 Environmental Exposure 875 Diabetes Mellitus 875 Extraocular Manifestations 875 Intraocular Manifestations 877 Cornea 877 Anterior Chamber and Aqueous 877 Primary Open-Angle Glaucoma 877 Iris 877 Ciliary Body 879 Lens 880 Vitreous 880 Diabetic Retinopathy 880 Optic Nerve 886 Lipoatrophic Diabetes (Laurence Syndrome) 886 Metabolic Diseases 886 Disturbances in Lipid Metabolism 886 Hyperlipoproteinemias 886 Hypolipoproteinemias 888 Dyslipoproteinemias 888 XXXIV Selected Table of Contents

Lipid Storage Diseases 888 Mucolipidoses 891 Ceroid Lipofuscinoses (Batten-Mayou-Vogt-Stock Syndrome) 892 Disturbances in Carbohydrate Metabolism 893 H ypergalactosemia 893 Hyperoxaluria (Oxalosis) 893 Glucose Phosphate Dehydrogenase Deficiency (Favism) 893 Carbohydrate Storage Diseases (Glycogenoses and Mucopolysaccharidoses) 893 Disturbances in Protein Metabolism 895 Albinism 895 Amino Acid Disturbances 896 Hyperuricemia (Gout) 899 Familial Dysautonomia (Riley-Day Syndrome) 899 Miscellaneous Disturbances of Metabolism 899 Disorders of Copper Metabolism 899 Hemochromatosis 900 Disorders of Calcium and Phosphate Metabolism 900 Porphyria . 900 Nutritional Deficiencies 900

Endocrine Diseases 902 Endocrine Ophthalmopathy (Thyroid Orbitopathy, Graves' Disease) 902 Noninfiltrative Stage (Class 1) 902 Infiltrative Stage (Classes 2-6) 902 Pituitary Gland, Adrenal Gland, and Pancreas 904

Diseases of the Circulatory System 904 Embolization 904 Blood Vessel Wall Changes and Their Effects 905 Arteriosclerosis and Atherosclerosis 905 Arterial Hypertension 907 Arterial Hypotension and Ischemic Ophthalmopathy 907

Immunologic Diseases 911 Rheumatoid Diseases 911 Collagen Diseases 912 Sarcoidosis (Besnier-Boeck-Schaumann Disease) 915 Beh~et's Syndrome 917 Amyloidosis 917 Special Immune Reactions of the Eye 920

Diseases of the Hematopoietic System 920 Disturbances of Erythropoiesis 920 Disturbances of Granulopoiesis 923 Disease of the Lymphatic System and Malignant Lymphoma 923 Multiple Myeloma 924 Diseases of the Reticuloendothelial System 924 Disturbances in Thrombocyte Formation 925 Hemorrhagic Diatheses and Hyperviscosity Syndromes 926 Selected Table of Contents xxxv

Metastatic Tumors to the Eye and Orbit 926 Phakomatoses ...... 928 Diseases of the Mesenchyme and Skeletal System 933 Stomach and Intestinal Diseases 936 Renal Diseases 937 References 938

17 Morphology of Drug-Induced Ocular Changes K.W. RUPRECHT and G.O.H. NAUMANN 957

Eyelids and Lacrimal Apparatus 957 Conjunctiva 958 Systemic Therapy 958 Local Therapy . 958 Cornea ..... 958 Systemic Therapy 959 Local Therapy . 960 Lens 961 Anterior Subcapsular Opacities 961 Posterior Subcapsular Opacities 961 Retina and Vitreous ...... 962 Uvea ...... 964 Intraocular Hypertension and Hypotony 965 Optic Nerve and Visual Pathways 966 Orbit ...... 967 Intrauterine Injury to the Entire Eye 967 References ...... 968

Index ...... 973

Top View