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Ocular Pathology E-Book

Ocular Pathology E-Book

Myron Yanoff | Joseph W. Sassani

(2018)

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Book Details

Abstract

Bridge the gap between ophthalmology and pathology with the 8th Edition of this comprehensive, easy-to-understand reference from Drs. Myron Yanoff and Joseph W. Sassani. Designed to keep you up to date with every aspect of the field, from current imaging techniques to genetics and molecular biology to clinical pearls, Ocular Pathology provides the concise yet complete information you need for board exams and clinical practice.

  • Includes new coverage of genetics and molecular biology, complications in diabetes mellitus, and the role of new drugs and other treatments for macular degeneration.
  • Covers the latest imaging techniques, including optical coherence tomography (OCT), anterior segment OCT (AS-OCT) and OCT-angiography.
  • Contains new images throughout that provide updated correlations between pathological and clinical aspects of each disorder. Clinicopathological correlations are presented with side-by-side image comparisons to make clinical pearl boxes even more useful.
  • Features more than 1,900 illustrations from the collections of internationally renowned leaders in ocular pathology.
  • Presents information in a quick-reference outline format – ideal for today’s busy physician.

Table of Contents

Section Title Page Action Price
Front Cover cover
Inside Front Cover ifc1
Ocular Pathology i
Copyright Page iv
Table Of Contents v
Foreword vi
Forewords to the First Edition vii
Preface ix
Acknowledgments xi
Dedication xii
1 Basic Principles of Pathology 1
Inflammation 1
Definition 1
Causes 1
Phases of Inflammation 1
Staining Patterns of Inflammation 18
Immunobiology 18
Background 18
Cellular Immunity (Delayed Hypersensitivity) 23
Humoral Immunoglobulin (Antibody) 23
Autoimmunity and Autoinflammation 24
Immunohistochemistry 24
Immunodeficiency Diseases 25
Transplantation Terminology 29
Cellular and Tissue Reactions 29
Hypertrophy 29
Hyperplasia 29
Aplasia 29
Hypoplasia 29
Metaplasia 29
Atrophy 29
Dysplasia 29
Neoplasia 29
Degeneration and Dystrophy 30
Necrosis (Table 1.11) 30
Apoptosis 31
Calcification 32
Autolysis and Putrefaction 32
Pigmentation 32
Growth and Aging 33
Epigenetics and Ocular Disease 33
Modern Molecular Pathology Diagnostic Techniques 34
Concluding Comments 37
Bibliography 37.e1
Inflammation 37.e1
Immunobiology 37.e1
Cellular and Tissue Reactions 37.e2
Epigenetics and Ocular Disease 37.e2
Modern Molecular Pathology Diagnostic Techniques 37.e2
2 Congenital Anomalies 38
Phakomatoses (Disseminated Hereditary Hamartomas) 38
General Information 38
Angiomatosis Retinae (von Hippel’s Disease [VHL]) 38
Meningocutaneous Angiomatosis (Encephalotrigeminal Angiomatosis; Sturge–Weber Syndrome [SWS]) 38
Neurofibromatosis (Figs. 2.3–2.5) 40
Tuberous Sclerosis (Bourneville’s Disease; Pringle’s Disease) 43
Other Phakomatoses 45
Chromosomal Aberrations 46
Trisomy 8 47
Trisomy 13 (47,13+; Patau’s Syndrome) 47
Trisomy 18 (47,18+; Edwards’ Syndrome) 48
Trisomy 21 (47,21+; Down’s Syndrome) 48
Triploidy 48
Chromosome 4 Deletion Defect 49
Chromosome 5 Deletion Defect (46,5p−; Cri du Chat Syndrome) 49
Chromosome 11 Deletion Defect 49
Chromosome 13 Deletion Defect 49
Chromosome 17 Deletion (17p11.2; Smith–Magenis Syndrome) 49
Chromosome 18 Deletion Defect (46,18p−; 46,18q−; or 46,18r; Partial 18 Monosomy) (Fig. 2.11) 49
Chromosome 47 Deletion Defect 49
Mosaicism 50
Infectious Embryopathy 51
Congenital Rubella Syndrome (Gregg’s Syndrome) 51
Cytomegalic Inclusion Disease 53
Congenital Syphilis 53
Toxoplasmosis 53
Drug Embryopathy 53
Fetal Alcohol Syndrome (FAS) (Fig. 2.15) 53
Thalidomide 53
Lysergic Acid Diethylamide (LSD) (Fig. 2.16) 53
Other Congenital Anomalies 54
Cyclopia and Synophthalmos 54
Anencephaly 56
Anophthalmos (Fig. 2.18) 56
Microphthalmos 57
Noonan Syndrome (NS) 57
Walker–Warburg Syndrome 57
Oculocerebrorenal Syndrome of Miller 57
Subacute Necrotizing Encephalomyelopathy (Leigh’s Disease) 58
Meckel’s Syndrome (Dysencephalia Splanchnocystica; Gruber’s Syndrome) 59
Potter’s Syndrome 59
Menkes’ Kinky-Hair Disease 59
Aicardi’s Syndrome 60
Ectrodactyly–Ectodermal Dysplasia (EEC) 60
Trichothiodystrophy (TD) 61
Dwarfism 61
Other Syndromes 61
Bibliography 61.e1
Angiomatosis Retinae 61.e1
Meningocutaneous Angiomatosis 61.e1
Neurofibromatosis 61.e1
Tuberous Sclerosis 61.e2
Other Phakomatoses 61.e2
Chromosomal Trisomy Defects 61.e2
Triploidy and Chromosomal Deletion Abnormalities 61.e3
Mosaicism 61.e3
Infectious Embryopathy 61.e3
Drug Embryopathy 61.e3
Other Congenital Anomalies 61.e4
3 Nongranulomatous Inflammation 62
Definition 62
4 Granulomatous Inflammation 77
Introduction 77
Post-Traumatic 77
Sympathetic Uveitis (Sympathetic Ophthalmia [SO], Sympathetic Ophthalmitis) 77
Phacoanaphylactic (Phacoimmune, Phacoantigenic, or Phacogenic) Endophthalmitis 78
Foreign-Body Granulomas 80
Nontraumatic Infections 80
Viral 80
Bacterial 83
Fungal 88
Parasitic 91
Nontraumatic Noninfectious 97
Sarcoidosis (Figs. 4.22–4.27) 97
Granulomatous Scleritis 99
Chalazion 100
Xanthogranulomas (Juvenile Xanthogranuloma and Langerhans’ Granulomatoses; Histiocytosis X) 100
Granulomatous Reaction to Descemet’s Membrane 100
Chédiak–Higashi Syndrome 100
Allergic Granulomatosis and Midline Lethal Granuloma Syndrome 100
Weber–Christian Disease (Relapsing Febrile Nodular Nonsuppurative Panniculitis) 100
Vogt–Koyanagi–Harada Syndrome (Uveomeningoencephalitic Syndrome) 100
Familial Chronic Granulomatous Disease of Childhood 101
Bibliography 101.e1
Sympathetic Uveitis 101.e1
Phacoanaphylactic Endophthalmitis 101.e1
Foreign-Body Granulomas 101.e1
Viral 101.e1
Bacterial 101.e2
Fungal 101.e3
Parasitic 101.e4
Sarcoidosis 101.e5
Granulomatous Scleritis 101.e6
Granulomatous Reaction to Descemet’s Membrane 101.e6
Vogt–Koyanagi–Harada Syndrome 101.e6
Familial Chronic Granulomatous Disease of Childhood 101.e7
5 Surgical and Nonsurgical Trauma 102
Causes of Enucleation 102
Complications of Intraocular Surgery 102
Adult Cataract Surgery 102
Immediate 102
Postoperative 106
Congenital Cataract Surgery 114
Delayed 114
Complications of Neural Retinal Detachment and Vitreous Surgery Including Intravitreal Injections 123
Intravitreal Injections 123
Incisional Vitreoretinal Surgery 124
Neural Retinal Detachment 124
Immediate 124
Postoperative 125
Delayed 128
Vitreous Surgery 129
Complications of Corneal Surgery 129
Endothelial Transplant Procedures 129
Introduction 129
Penetrating Keratoplasty (Graft) 130
Other Refractive Keratoplasties 132
Complications of Glaucoma Surgery 134
Complications of Nonsurgical Trauma 135
Introduction 135
Contusion 137
Penetrating and Perforating Injuries 150
Intraocular Foreign Bodies 151
Chemical Injuries 154
Burns 156
Ocular Effects of Injuries to Other Parts of the Body 157
Radiation Injuries (Electromagnetic) 159
Bibliography 162.e1
Causes of Enucleation 162.e1
Complications of Intraocular Surgery 162.e1
Complications of Retinal Detachment and Vitreous Surgery Including Intraocular Injections 162.e1
Complications of Corneal Surgery 162.e3
Complications of Glaucoma Surgery 162.e4
Complications of Nonsurgical Trauma 162.e4
6 Skin and Lacrimal Drainage System 163
Skin 163
Normal Anatomy (Figs. 6.1 and 6.2) 163
Epidermis 163
Dermis 163
Subcutaneous Tissue 163
Terminology 163
Orthokeratosis and Parakeratosis 163
Acanthosis 163
Dyskeratosis 163
Acantholysis 163
Bulla 163
Atrophy 166
Atypical Cell 166
Leukoplakia 166
Polarity 166
Congenital Abnormalities 167
Dermoid and Epidermoid Cysts 167
Phakomatous Choristoma 167
Miscellaneous Choristomas and Hamartomas 168
Cryptophthalmos (Ablepharon) 168
Microblepharon 169
Coloboma 169
Epicanthus 169
Ectopic Caruncle 170
Lid Margin Anomalies 170
Eyelash Anomalies 170
Ptosis 172
Ichthyosis Congenita 172
Xeroderma Pigmentosum 173
Aging 174
Atrophy 174
Senile Ectropion and Entropion 174
Dermatochalasis and Blepharochalasis 174
Herniation of Orbital Fat 175
Floppy Eyelid Syndrome 176
Inflammation 176
Terminology 176
Viral Diseases 179
Bacterial Diseases 181
Fungal and Parasitic Diseases 181
Lid Manifestations of Systemic Dermatoses or Disease 182
Ichthyosis Congenita 182
Xeroderma Pigmentosum 182
Pemphigus 182
Ehlers–Danlos Syndrome (“India-Rubber Man”) 182
Cutis Laxa 182
Pseudoxanthoma Elasticum 183
Erythema Multiforme 184
Epidermolysis Bullosa 186
Contact Dermatitis 187
Collagen Diseases 188
Granulomatous Vasculitis 194
Vasculitis-Like Disorders and Leukemia/Lymphoma 195
Xanthelasma 197
Necrobiotic Xanthogranuloma 198
Juvenile Xanthogranuloma (JXG) 199
Amyloidosis 200
Atrophic Papulosis (Köhlmeier–Degos Disease) (Benign and Malignant) 200
Calcinosis Cutis 200
Lipoid Proteinosis (Urbach–Wiethe Disease, Hyalinosis Cutis et Mucosae) 201
Idiopathic Hemochromatosis 202
Relapsing Febrile Nodular Nonsuppurative Panniculitis (Weber–Christian Disease) 203
Pigmentation 203
Cysts, Pseudoneoplasms, and Neoplasms 203
Benign Cystic Lesions 203
Benign Tumors of the Surface Epithelium 207
Precancerous Tumors of the Surface Epithelium 212
Cancerous Tumors of the Surface Epithelium 213
Tumors of the Epidermal Appendages (Adnexal Skin Structures) 217
Merkel Cell Carcinoma (Neuroendocrine Carcinoma, Trabecular Carcinoma) (Fig. 6.45) 228
Malacoplakia 229
Pigmented Tumors 229
Mesenchymal Tumors 229
Metastatic Tumors 229
Lacrimal Drainage System 229
Normal Anatomy (Fig. 6.46) 229
Congenital Abnormalities 230
Atresia of the Nasolacrimal Duct 230
Atresia of the Punctum 230
Congenital Fistula of Lacrimal Sac (Minimal Facial Fissure) 230
Inflammation—Dacryocystitis (Fig. 6.47) 230
Blockage of Tear Flow Into the Nose 230
Tumors 232
Epithelial 232
Melanotic 233
Mesenchymal 233
Miscellaneous 233
Bibliography 233.e1
Congenital Abnormalities 233.e1
Aging 233.e3
Inflammation 233.e3
Lid Manifestations of Systemic Dermatoses or Disease 233.e4
Cysts, Pseudoneoplasms, and Neoplasms 233.e7
Lacrimal Drainage System 233.e10
Tumors 233.e10
7 Conjunctiva 234
Normal Anatomy 234
Congenital Anomalies 235
Cryptophthalmos (Ablepharon) 235
Epitarsus 235
Hereditary Hemorrhagic Telangiectasia (Rendu–Osler–Weber Disease) 235
Ataxia–Telangiectasia (Louis–Bar Syndrome) 236
Congenital Conjunctival Lymphedema (Milroy’s Disease, Nonne–Milroy–Meige Disease) 236
Miscellaneous 236
Dermoids, Epidermoids, and Dermolipomas 236
Choristomas 236
Laryngo-Onycho-Cutaneous (LOC or Shabbir) Syndrome 236
Vascular Disorders 237
Sickle-Cell Anemia 237
Conjunctival Hemorrhage (Subconjunctival Hemorrhage) 238
Lymphangiectasia 238
Lymphangiectasia Hemorrhagica Conjunctivae 238
Ataxia–Telangiectasia 238
Diabetes Mellitus 238
Hemangioma and Lymphangioma 238
Inflammation 239
Basic Histologic Changes 239
Specific Inflammations 243
Infectious 243
Noninfectious 245
Injuries 247
Conjunctival Manifestations of Systemic Disease 247
Deposition of Metabolic Products 247
Deposition of Drug Derivatives 248
Vitamin A Deficiency: Bitot’s Spot 248
Sjögren’s Syndrome 248
Skin Diseases 248
Degenerations 249
Xerosis 249
Pterygium 249
Pinguecula 249
Lipid Deposits 250
Amyloidosis 250
Conjunctivochalasis 253
Cysts, Pseudoneoplasms, and Neoplasms 254
Choristomas 254
Hamartomas 254
Cysts 254
Pseudocancerous Lesions 255
Potentially Precancerous Epithelial Lesions 263
Cancerous Epithelial Lesions 263
Pigmented Lesions of the Conjunctiva 269
Stromal Neoplasms 269
Bibliography 271.e1
Normal Anatomy 271.e1
Congenital Anomalies 271.e1
Vascular Disorders 271.e1
Inflammation 271.e1
Conjunctival Manifestations of Systemic Diseases 271.e2
Degenerations 271.e2
Cysts, Pseudoneoplasms, and Neoplasms 271.e2
8 Cornea and Sclera 272
Cornea 272
Normal Anatomy 272
Congenital Defects 275
Absence of Cornea 275
Abnormalities of Size 275
Aberrations of Curvature 276
Congenital Corneal Opacities 276
Clinicopathologic Types—General 277
Clinicopathologic Types—Specific 277
Inflammations—Nonulcerative 285
Epithelial Erosions and Keratitis 285
Subepithelial Keratitis 286
Superior Limbic Keratoconjunctivitis 286
Stromal (Interstitial) Keratitis 286
Endothelial 288
Inflammations—Ulcerative 288
Peripheral 288
Central 289
Inflammations—Corneal Sequelae 292
Injuries 293
Degenerations 293
Epithelial 293
Stromal 295
Endothelial 305
Dystrophies and Simulating Disorders 305
Introduction 305
Classification of Dystrophies 305
True Corneal Dystrophies (Table 8.5) 307
Primary in the Corneal 307
Pigmentations (Table 8.14) 343
Melanin 343
Blood 344
Iron Lines 344
Kayser–Fleischer Ring 344
Tattoo 345
Drug-Induced 345
Infections 347
Crystals 347
Neoplasm 348
Sclera 349
Congenital Anomalies 349
Blue Sclera 349
Ochronosis (Alkaptonuria) 349
Inflammations 351
Episcleritis 351
Scleritis (Fig. 8.71) 352
Introduction 352
Tumors 355
Fibromas 355
Nodular Fasciitis 355
Hemangiomas 355
Neurofibromas 355
Contiguous Tumors 355
Conjunctival Tumors 355
Episcleral Osseous Choristoma and Episcleral Osseocartilaginous Choristoma 355
Ectopic Lacrimal Gland 356
Bibliography 356.e1
Normal Anatomy 356.e1
Congenital Defects 356.e1
Inflammations: Nonulcerative 356.e2
Inflammations: Ulcerative 356.e2
Degenerations: Epithelial 356.e2
Degenerations: Stromal 356.e2
Dystrophies: Introduction and Classification 356.e3
Dystrophies: Epithelial-Stromal Including TGFB1 Corneal Dystrophies 356.e3
Dystrophies: Stromal 356.e3
Dystrophies: Descemet’s Membrane and Endothelial 356.e4
Heredofamilial 356.e5
Nonheredofamial Dystrophy-Like Syndromes 356.e6
Crystals 356.e6
Congenital Anomalies 356.e7
Inflammations 356.e7
9 Uvea 357
Normal Anatomy 357
Congenital and Developmental Defects 357
Persistent Pupillary Membrane (PPM) 357
Persistent Tunica Vasculosa Lentis 357
Heterochromia Iridis and Iridum 359
Hematopoiesis 359
Ectopic Intraocular Lacrimal 359
Gland Tissue 359
Congenital and Developmental Defects of the Pigment Epithelium 359
Aniridia (Hypoplasia) of the Iris 359
Ectropion Uveae (Hyperplasia of Iris Pigment Border or Seam) 361
Peripheral Dysgenesis of the Cornea and Iris 361
Coloboma 361
Cysts of the Iris and Anterior Ciliary Body (Pars Plicata) 363
Cysts of the Posterior Ciliary Body (Pars Plana) 364
Inflammations 364
Injuries 364
Systemic Diseases 364
Diabetes Mellitus 364
Vascular Diseases 365
Cystinosis 365
Homocystinuria 365
Amyloidosis 365
Juvenile Xanthogranuloma (Nevoxanthoendothelioma) 365
Langerhans’ Granulomatoses (Histiocytosis X) 367
Collagen Diseases 367
Mucopolysaccharidoses 367
Atrophies and Degenerations 367
Iris Neovascularization (Rubeosis Iridis) 367
Choroidal Folds 368
Heterochromia 369
Macular Degeneration 369
Dystrophies 369
Iris Nevus Syndrome 369
Chandler’s Syndrome 369
Essential Iris Atrophy 369
Iridoschisis 369
Choroidal Dystrophies 369
Tumors 371
Epithelial 371
Muscular 373
Vascular 374
Osseous 375
Melanomatous 375
Leukemic and Lymphomatous (See Chapter 14) 375
Other Tumors 377
Secondary Neoplasms 378
Uveal Edema (Uveal Detachment; Uveal Hydrops) 379
Types 379
Bibliography 379.e1
Normal Anatomy 379.e1
Congenital and Developmental Defects 379.e1
Systemic Diseases 379.e2
Atrophies and Degenerations 379.e2
Dystrophies 379.e2
Tumors 379.e3
Uveal Edema 379.e5
10 Lens 380
Normal Anatomy 380
General Information 380
Congenital Anomalies 380
Introduction 380
Mittendorf’s Dot 380
Congenital Aphakia 380
Congenital Duplication of Lens 380
Fleck Cataract 380
Anterior Polar Cataract 381
Posterior Polar Cataract 382
Anterior Lenticonus (Lentiglobus) 383
Posterior Lenticonus (Lentiglobus) 383
Other Congenital Cataracts 384
Capsule (Epithelial Basement Membrane) 384
General Reactions 384
Exfoliation of the Lens Capsule 386
Pseudoexfoliation Syndrome (Pseudoexfoliation of Lens Capsule, Exfoliation Syndrome, Basement Membrane Exfoliation Syndrome, Fibrillopathia Epitheliocapsularis) (Figs. 10.8–10.11) 387
Epithelium 391
Proliferation and Migration of Epithelium 391
Anterior Subcapsular Cataract (ASC) (Figs. 10.12–10.15) 391
Posterior Subcapsular Cataract (PSC) (Figs. 10.16 and 10.17; see Fig. 10.15) 392
Elschnig’s Pearls (see Fig. 5.15) 393
Degeneration and Atrophy of the Epithelium 393
Cortex and Nucleus (Lens Cells or “Fibers”) 393
Cortex (“Soft Cataract”) 393
Nucleus (“Hard Cataract”) 398
Age-Related (Senile) Cataracts 398
Secondary Cataracts 400
Intraocular Disease 400
Trauma 400
Toxic 400
Endocrine, Metabolic, and Others 400
Complications of Cataracts 400
Glaucoma 400
Phacoanaphylactic Endophthalmitis 402
Ectopic Lens 402
Congenital 402
Bibliography 406.e1
Normal Anatomy 406.e1
General Information 406.e1
Congenital Anomalies 406.e1
Capsule 406.e2
Epithelium 406.e3
Cortex and Nucleus 406.e3
Secondary Cataracts 406.e3
Complications of Cataracts 406.e4
Ectopic Lens 406.e4
11 Neural (Sensory) Retina 407
Normal Anatomy 407
Congenital Anomalies 407
Albinism (Fig. 11.4) 407
Grouped Pigmentation (Bear Tracks) 409
Coloboma 409
Retinal Dysplasia 410
Lange’s Fold 410
Congenital Nonattachment of the Retina 411
Neural Retinal Cysts 411
Myelinated (Medullated) Nerve Fibers 411
Oguchi’s Disease 412
Foveomacular Abnormalities 412
Leber’s Congenital Amaurosis 413
Inherited Retinal Arteriolar Tortuosity 413
Vascular Diseases 413
Definitions 413
Retinal Ischemia 413
Causes 413
Complications of Retinal Ischemia 415
Histology of Retinal Ischemia 416
Retinal Hemorrhagic Infarction (Fig. 11.12) 417
Causes and Risk Factors of Hemorrhagic Infarction 417
Types of Hemorrhagic Infarction 418
Complications of Hemorrhagic Infarction 420
Histology of Retinal Hemorrhagic Infarction (see Fig. 11.12) 420
Hypertensive and Arteriolosclerotic Retinopathy 422
Hemorrhagic Retinopathy 424
Exudative Retinopathy 424
Diabetes Mellitus 424
Coats’ Disease, Leber’s Miliary Aneurysms, and Retinal Telangiectasia 424
Idiopathic Macular Telangiectasia (Idiopathic Juxtafoveolar Retinal Telangiectasis) 424
Retinal Arterial and Arteriolar Macroaneurysms 424
Sickle-Cell Disease 424
Eales’ Disease (Primary Perivasculitis of the Retina) 426
Retinopathy of Prematurity 427
Hemangioma of the Retina 427
Hereditary Hemorrhagic Telangiectasia (Rendu–Osler–Weber Disease) 427
Disseminated Intravascular Coagulation 427
Inflammations 428
Nonspecific Retinal Inflammations 428
Specific Retinal Inflammations (see Chapters 2–4) 428
Injuries 430
Degenerations 430
Definitions 430
Microcystoid Degeneration 430
Degenerative Retinoschisis 431
Secondary Microcystoid Degeneration and Retinoschisis 434
Paving Stone (Cobblestone) Degeneration (Peripheral Chorioretinal Atrophy; Equatorial Choroiditis) 434
Peripheral Retinal Albinotic Spots 435
Myopic Retinopathy 435
Macular Degeneration 435
Idiopathic Serous Detachment of the RPE (Fig. 11.25) 435
Idiopathic Central Serous Choroidopathy (Central Serous Retinopathy; Central Angiospastic Retinopathy) (see Fig. 11.25) 436
Drusen 437
Dry Age-Related Macular Degeneration (Dry, Atrophic, or Senile Atrophic Macular Degeneration) 440
Age-Related Exudative Macular Degeneration (Exudative, Wet, or Senile Disciform Macular Degeneration; Kuhnt–Junius Macular Degeneration) 442
Congenital Hypotrichosis With Juvenile Macular Degeneration (CHWJMD) 445
Exudative Macular Degeneration Secondary to Focal Choroiditis (Juvenile Disciform Degeneration of the Macula) 445
Idiopathic Polypoidal Choroidal Vasculopathy 446
Cystoid Macular Edema (Irvine–Gass Syndrome) 446
Toxic Retinal Degenerations 446
Postirradiation Retinopathy 448
Bone Marrow Transplant Retinopathy 448
Cancer-Associated Retinopathy (Paraneoplastic Syndrome; Paraneoplastic Retinopathy; Paraneoplastic Photoreceptor Retinopathy; Melanoma-Associated Retinopathy) 448
Idiopathic Macular Holes 449
Light Energy Retinopathy 449
Traumatic Retinopathy 449
Hereditary Primary Retinal Dystrophies 450
Definitions 450
X-Linked Retinoschisis (Juvenile Retinoschisis, Vitreous Veils; Congenital Vascular Veils; Cystic Disease of the Retina; Congenital Retinal Detachment) 450
Choroidal Dystrophies 452
Stargardt’s Disease (Fundus Flavimaculatus) 452
Dominant Drusen of Bruch’s Membrane (Doyne’s Honeycomb Dystrophy; Malattia Lèventinese; Hutchinson–Tay Choroiditis; Guttate Choroiditis; Holthouse–Batten Superficial Choroiditis; Family Choroiditis; Crystalline Retinal Degeneration; Iridescent Crystals of the Macula; Hyaline Dystrophies) 454
Best Vitelliform Disease (Vitelliform Foveal Dystrophy; Vitelliform Macular Degeneration; Vitelliruptive Macular Degeneration; Exudative Central Detachment of the Retina—Macular Pseudocysts; Cystic Macular Degeneration; Exudative Foveal Dystrophy) 454
Dominant Progressive Foveal Dystrophy 455
Dominant Cystoid Macular Dystrophy (DCMD) 455
Fenestrated Sheen Macular Dystrophy 456
North Carolina Macular Dystrophy 456
Familial Internal Limiting Membrane Dystrophy 456
Central Pigmentary Sheen Dystrophy 456
Cone–Rod Dystrophy 456
Annular Macular Dystrophy (Benign Concentric Annular Macular Dystrophy) 457
Retinitis Punctata Albescens (Albipunctate Dystrophy; Fundus Albipunctatus; Panretinal Degeneration) 457
Central Retinitis Pigmentosa (Central Retinopathia Pigmentosa; Retinopathia Pigmentosa Inversa; Retinitis Pigmentosa Inversa; Pericentral Pigmentary Retinopathy) 457
Retinitis Pigmentosa (Retinopathia Pigmentosa; Pigmentary Degeneration of the Retina) 457
Clumped Pigmentary Retinal Dystrophy (Clumped Pigmentary Retinal Degeneration) 459
Hereditary Pigmented Paravenous Chorioretinal Atrophy 459
Pigment Epithelial Dystrophy 459
Central Areolar Pigment Epithelial Dystrophy 460
Patterned Dystrophies of the Retinal Pigment Epithelium (Reticular Dystrophy or Sjögren Dystrophia Reticularis Laminae Pigmentosae Retinae; Butterfly-Shaped Pigment Dystrophy of the Fovea; Macroreticular or Spider Dystrophy) 460
Bietti’s Crystalline Dystrophy (Bietti’s Tapetoretinal Degeneration With Marginal Corneal Dystrophy, Crystalline Retinopathy) 461
Sorsby Fundus Dystrophy (Sorsby’s Pseudoinflammatory Macular Dystrophy; Hereditary Macular Dystrophy) 462
Autosomal-Dominant Occult Macular Dystrophy 462
Unilateral Retinal Pigment Epithelial Dysgenesis (URPED) 462
Recessive Retinopathy Consequent on Mutant G-Protein β Subunit 3 (GNB3) 462
Martinique Crinkled Retinal Pigment Epitheliopathy (MCRPE) 462
Hereditary Secondary Retinal Dystrophies 462
Angioid Streaks 462
Sjögren–Larsson Syndrome (S-LS) 463
Mucopolysaccharidoses 463
Mucolipidoses 463
Sphingolipidoses 464
Other Lipidoses 465
Disorders of Carbohydrate Metabolism 466
Primary Hyperoxaluria (Primary Oxalosis; Fig. 11.43) 467
Osteopetrosis 467
Homocystinuria 467
Systemic Diseases Involving the Retina 467
Hereditary Secondary Retinal Dystrophies 467
Diabetes Mellitus 467
Hypertension and Arteriolosclerosis 467
Collagen Diseases 467
Blood Dyscrasias 468
Demyelinating Diseases 468
Tumors 468
Glia 468
Phakomatoses 473
Retinal Pigment Epithelium 473
Retinoblastoma and Pseudogliomas 473
Neural Retinal Metastases (Fig. 11.49) 473
Neural Retinal Detachment 473
Definitions 473
Major Causes 474
Classification of Neural Retinal Detachment 474
Predisposing Factors to Neural Retinal Detachment 476
Pathologic Changes After Neural Retinal Detachment 478
Pathologic Complications After Neural Retinal Detachment Surgery 480
Bibliography 480.e1
Normal Anatomy 480.e1
Congenital Anomalies 480.e1
Vascular Disease 480.e1
Inflammation 480.e2
Degenerations 480.e3
Hereditary Primary Retinal Dystrophies 480.e7
Hereditary Secondary Retinal Dystrophies 480.e10
Systemic Diseases Involving the Retina 480.e11
Tumors 480.e11
Retinal Detachment 480.e11
12 Vitreous 481
Normal Anatomy 481
Congenital Anomalies 481
Persistent Primary Vitreous 481
Persistent Fetal Vasculature (PFV; Persistent Hyperplastic Primary Vitreous [PHPV]) 481
Inflammation 481
Acute 481
Chronic 481
Vitreous Adhesions 481
Post Nonsurgical and Surgical Trauma 481
Postinflammation 484
Idiopathic 484
Vitreous Opacities 484
Hyaloid Vessel Remnants 484
Acquired Vitreous Strands and Floaters 484
Inflammatory Cells 484
Red Blood Cells 484
Iridescent Particles 484
Tumor Cells 486
Pigment Dust 486
Cysts 486
Retinal Fragments 486
Traumatic Avulsion of Vitreous Base 486
Vitreous Detachment 486
Proteinaceous Deposits 487
Amyloid 488
Familial Exudative Vitreoretinopathy (FEVR) 490
Autosomal-Dominant Vitreoretinochoroidopathy (ADVIRC; Peripheral Annular Pigmentary Dystrophy of the Retina) 490
Autosomal-Dominant Neovascular Inflammatory Vitreoretinopathy (ADNIV) 491
Erosive Vitreoretinopathy 491
Knobloch Syndrome 491
Vitreous Hemorrhage 491
Definitions 491
Causes 491
Complications 491
Bibliography 493.e1
Normal Anatomy 493.e1
Congenital Anomalies 493.e1
Vitreous Adhesions 493.e1
Vitreous Opacities 493.e1
Vitreous Hemorrhage 493.e2
13 Optic Nerve 494
Normal Anatomy 494
Congenital Defects and Anatomic Variations 494
Aplasia 494
Hypoplasia 494
Dysplasia 497
Anomalous Shape of Optic Disc and Cup 497
Congenital Crescent or Conus 497
Congenital (Familial) Optic Atrophies 497
Coloboma (Table 13.1) 498
Myopia 501
Optic Disc Edema 501
General Information (Fig. 13.7; see Fig. 13.22) 501
Causes 501
Pseudopapilledema 502
Histology of Optic Disc Edema 502
Optic Neuritis 503
Causes 503
Histology of Optic Neuritis 509
Optic Atrophy 509
Causes 509
Histology of Optic Atrophy 510
Injuries 510
Tumors 510
Primary 510
Secondary 518
Bibliography 518.e1
Normal Anatomy 518.e1
Congenital Defects and Anatomic Variations 518.e1
Optic Disc Edema 518.e2
Optic Neuritis 518.e2
Optic Atrophy 518.e4
Tumors 518.e4
14 Orbit 519
Normal Anatomy 519
Exophthalmos 519
Developmental Abnormalities 519
Developmental Abnormalities of Bony Orbit 519
Microphthalmos With Cyst 519
Cephaloceles 521
Congenital Alacrima 521
Orbital Inflammation 522
Acute 522
Chronic 522
Injuries 524
Penetrating Wounds 524
Nonpenetrating Wounds 525
Vascular Disease 525
Primary 525
Part of Systemic Disease 525
Ocular Muscle Involvement in Systemic Disease 525
Graves’ Disease (Fig. 14.10) 525
Myasthenia Gravis (MG) 527
Myotonic Dystrophy (Myotonia Dystrophica; Steinert’s Disease) 527
Myotonia Congenita (Thomsen’s Disease) 527
Mitochondrial Myopathies 527
Dermatomyositis 528
Neoplasms and Other Tumors 529
Primary Orbital Tumors 529
Secondary Orbital Tumors 581
Bibliography 582.e1
Normal Anatomy 582.e1
Exophthalmos 582.e1
Developmental Abnormalities 582.e1
Orbital Inflammation 582.e1
Injuries 582.e2
Vascular Disease 582.e2
Ocular Muscle Involvement in Systemic Disease 582.e2
Tumors: Choristoma 582.e3
Tumors: Hamartomas 582.e3
Tumors: Mesenchymal–Vascular 582.e3
Tumors: Mesenchymal–Fatty 582.e4
Tumors: Mesenchymal–Fibrous–Histiocytic 582.e4
Tumors: Mesenchymal–Muscle 582.e4
Tumors: Mesenchymal–Cartilage 582.e5
Tumors: Mesenchymal–Bone 582.e5
Tumors: Neural 582.e5
Tumors: Miscellaneous 582.e6
Tumors: Epithelial of Lacrimal Gland 582.e6
Tumors: Reticuloendothelial System 582.e7
Tumors: Inflammatory Pseudotumor 582.e7
Tumors: Malignant Lymphoma 582.e7
Tumors Leukemia 582.e9
Tumors: Monoclonal and Polyclonal Gammopathies 582.e10
Secondary Tumors 582.e10
15 Diabetes Mellitus 583
Natural History 583
Ocular Surface Disease 585
Intraocular Changes 589
Lens 589
Iris 591
Ciliary Body and Choroid 592
Retinal Vasculature in Normal Subjects and Diabetic Patients 596
Neural Retina 596
Vitreous 613
Optic Nerve 615
Bibliography 616.e1
Natural History 616.e1
Ocular Surface Disease 616.e1
Lens 616.e3
Ciliary Body and Choroid 616.e4
Retina 616.e4
Vitreous 616.e5
Optic Nerve 616.e6
16 Glaucoma 617
Normal Anatomy (Figs. 16.1–16.3) 617
Introduction 620
Normal Outflow 624
Hypersecretion 624
Impaired Outflow 624
Congenital Glaucoma (Table 16.3) 624
Primary Glaucoma (Closed- and Open-Angle) 628
Secondary Angle-Closure Glaucoma 635
Causes 635
Secondary Open-Angle Glaucoma 642
Tissue Changes Caused by Elevated Intraocular Pressure 651
Cornea (Figs. 16.26–16.28; See Also Fig. 8.49A,B) 651
Anterior-Chamber Angle 652
Iris 652
Ciliary Body 653
Lens 654
Sclera 654
Neural Retina (Fig. 16.31) 654
Optic Nerve 655
Bibliography 657.e1
Normal Anatomy 657.e1
Introduction 657.e1
Impaired Outflow: Congenital Glaucoma 657.e1
Impaired Outflow: Primary Closed-Angle 657.e2
Impaired Outflow: Primary Open-Angle 657.e2
Impaired Outflow: Secondary Closed-Angle 657.e2
Impaired Outflow-Secondary Open-Angle 657.e2
Tissue Changes Caused by Elevated Intraocular Pressure 657.e3
17 Ocular Melanocytic Tumors 658
Normal Anatomy 658
Ocular Melanocytes 658
Melanotic Tumors of Eyelids 658
Ephelis (Freckle) 658
Lentigo 658
Nevus 660
Malignant Melanoma 664
Melanotic Tumors of Conjunctiva 666
Ephelis (Freckle) 666
Lentigo 666
Nevus 667
Primary Acquired Melanosis (PAM; Figs. 17.15 and 17.16; see also Table 17.2) 671
Primary Malignant Melanoma of Conjunctiva (Fig. 17.17; see also Fig. 17.16) 674
Lesions That May Simulate Primary Conjunctival Nevus or Malignant Melanoma 678
Melanotic Tumors of Pigment Epithelium of Iris, Ciliary Body, and Retina 678
Reactive Tumors 678
Nonreactive Tumors 683
Acquired Neoplasms 686
Melanotic Tumors of the Uvea 688
Iris 688
Ciliary Body and Choroid 694
Melanotic Tumors of the Optic Disc and Optic Nerve 721
Melanocytoma (Magnocellular Nevus of the Nerve Head) 721
Malignant Melanoma 722
Melanotic Tumors of the Orbit 723
Bibliography 725.e1
Melanocytic Tumors of the Eyelids 725.e1
Melanocytic Tumors of Conjunctiva 725.e1
Melanocytic Tumors of Pigment Epithelium of Iris, Ciliary Body, and Retina 725.e1
Melanocytic Tumors of the Uvea: Iris 725.e1
Melanocytic Tumors of the Uvea: Ciliary Body and Choroid 725.e1
Melanocytic Tumors of the Orbit 725.e3
18 Retinoblastoma and Simulating Lesions 726
Retinoblastoma 726
General Information 726
Heredity 729
Clinical Features 731
Histology 734
Prognosis 742
Overview 742
Lesions Simulating Retinoblastoma (Pseudoglioma) 744
General Information 744
Leukokoria (Box 18.1) 745
Discrete Retinal or Chorioretinal Lesions 759
Bibliography 759.e1
Retinoblastoma—General Information 759.e1
Retinoblastoma—Heredity 759.e1
Retinoblastoma—Clinical Features 759.e2
Retinoblastoma—Histology 759.e2
Retinoblastoma—Prognosis 759.e2
Lesions Simulating Retinoblastoma (Pseudoglioma)—General Information 759.e3
Lesions Simulating Retinoblastoma (Pseudoglioma)—Leukokoria: Introduction 759.e3
Lesions Simulating Retinoblastoma (Pseudoglioma)—Leukokoria: Persistent Fetal Vasculature 759.e3
Lesions Simulating Retinoblastoma (Pseudoglioma)—Leukokoria: Retinal Dysplasia 759.e4
Lesions Simulating Retinoblastoma (Pseudoglioma)—Leukokoria: Retinopathy of Prematurity 759.e4
Lesions Simulating Retinoblastoma (Pseudoglioma)—Leukokoria: Coats’ Disease 759.e4
Lesions Simulating Retinoblastoma (Pseudoglioma)—Leukokoria: Incontinentia Pigmenti 759.e5
Lesions Simulating Retinoblastoma (Pseudoglioma)—Leukokoria: Other Causes 759.e5
Index 760
A 760
B 761
C 762
D 765
E 766
F 767
G 768
H 769
I 770
J 771
K 771
L 771
M 773
N 774
O 775
P 776
R 778
S 779
T 781
U 782
V 782
W 783
X 783
Z 783
Inside Back Cover ibc1