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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2589_Библиотеки_им_академика_М_И_Перельмана
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Contents
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8 Retinal Arteriolar Changes in Hypertension
and Arteriolosclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163
8.1 Structural Considerations of the Blood Vessels . . . . . . . . . . . . 163
8.1.1 Anatomical Considerations of the Central Retinal
Artery and the Blood Supply of the Retina . . . . . . . . . 164
8.1.2 Diameter of the Retinal Arterioles and
Their Fractal Dimensions . . . . . . . . . . . . . . . . . . . . . . . 165
8.2 Causes of Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
8.3 Measuring Blood Pressure . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
8.4 Arteriosclerosis and Arteriolosclerosis . . . . . . . . . . . . . . . . . . 166
8.5 Fundus Signs of Arteriolosclerosis and Hypertension . . . . . . . 167
8.5.1 Enhanced Central Light Reex . . . . . . . . . . . . . . . . . . 168
8.5.2 Arterial Wall-to-Lumen Ratio . . . . . . . . . . . . . . . . . . . 168
8.5.3 Arteriovenous Nicking (AVN) . . . . . . . . . . . . . . . . . . . 169
8.5.4 Focal and Diffuse Narrowing of the Retinal
Arterioles . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169
8.5.5 Retinal Haemorrhages, Cotton Wool Spots,
and Microaneurysms . . . . . . . . . . . . . . . . . . . . . . . . . . 170
8.5.6 Optic Neuropathy and Choroidopathy
in Malignant Hypertension . . . . . . . . . . . . . . . . . . . . . 171
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 175
9 Retinal Vascular Occlusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
9.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
9.2 Non-arteritic Central and Branch Retinal Artery
Occlusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182
9.3 Branch Retinal Vein Occlusion . . . . . . . . . . . . . . . . . . . . . . . . 185
9.3.1 Epidemiology of Branch Retinal Vein
Occlusion (BRVO) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185
9.3.2 Risk Factors for BRVO . . . . . . . . . . . . . . . . . . . . . . . . 185
9.3.3 Pathogenesis of BRVO . . . . . . . . . . . . . . . . . . . . . . . . . 185
9.3.4 Clinical Presentations of BRVO. . . . . . . . . . . . . . . . . . 185
9.3.5 Role of Fundus Fluorescein Angiography . . . . . . . . . . 187
9.3.6 Consequences and Complications of BRVO . . . . . . . . 188
9.4 Epidemiology of Central Retinal Vein Occlusion (CRVO) . . . 190
9.4.1 Risk Factors for CRVO . . . . . . . . . . . . . . . . . . . . . . . . 190
9.4.2 Pathogenesis of CRVO . . . . . . . . . . . . . . . . . . . . . . . . . 190
9.4.3 Clinical Presentation and Classication of CRVO. . . . 191
9.5 Inammatory Retinal Vein Occlusions . . . . . . . . . . . . . . . . . . 197
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199
10 Retinal and Choroidal Infections and Inflammation . . . . . . . . . 205
10.1 Anatomical Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . 205
10.2 Mediators of Inammation . . . . . . . . . . . . . . . . . . . . . . . . . . 206
10.3 Retinal and Choroidal Infections and Inammations:
Denition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206
10.4 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 207
10.5 Differentiating Retinitis from Choroiditis . . . . . . . . . . . . . . . 207
10.6 Infectious Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 209

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10.6.1 Toxoplasmic Retinochoroiditis . . . . . . . . . . . . . . . . 209
10.6.2 Infectious Herpes Simplex and Varicella Zoster
Virus Retinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 212
10.6.3 CMV Retinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215
10.6.4 Emerging Viral Infections and Retinitis . . . . . . . . . . 219
10.6.5 Bacterial Infectious Uveitis- Spirochetal
Infections . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 220
10.6.6 Other Bacterial Uveitis: Mycobacterium
tuberculosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223
10.7 Non-infectious Choroiditis and Retinitis . . . . . . . . . . . . . . . . 233
10.7.1 Systemic Immune-Mediated Disorders . . . . . . . . . . 233
10.7.2 Organ-Specic (Ocular) Immune-Mediated
Panuveitis with Multisystem Involvement
. . . . . . . . 243
10.7.3 Organ-Specic (Ocular) Immune-Mediated
Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 251
10.8 Multiple Evanescent White Dot Syndrome (MEWDS) . . . . . 253
10.9 Acute Posterior Multifocal Placoid Pigment
Epitheliopathy (APMPPE) . . . . . . . . . . . . . . . . . . . . . . . . . . . 255
10.10 Punctate Inner Choroidopathy and Idiopathic
Multifocal Choroidopathy (PIC and IMFC) . . . . . . . . . . . . . 256
10.11 Acute Zonal Occult Outer Retinopathy (AZOOR) . . . . . . . . 256
10.12 Birdshot Chorioretinopathy (BCR) . . . . . . . . . . . . . . . . . . . . 256
10.13 Acute Macular Neuroretinitis (AMN) . . . . . . . . . . . . . . . . . . 256
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 257
Contents
11 Macular Oedema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 271
11.1 Anatomical Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . 271
11.2 Homeostasis in the Retinal Microenvironment . . . . . . . . . . . 272
11.3 Examination of Retina and Documentation of Macular
Oedema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 272
11.3.1 Clinical Examination of the Macula . . . . . . . . . . . . 272
11.3.2 Documentation of Macular Oedema-Fundus
Photography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 273
11.3.3 Fundus Fluorescein Angiography . . . . . . . . . . . . . . 274
11.3.4 Optical Coherence Tomography . . . . . . . . . . . . . . . 274
11.3.5 Measuring the Central Subeld Thickness (CST)
with OCT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 275
11.4 Causes of Macular Oedema . . . . . . . . . . . . . . . . . . . . . . . . . . 276
11.4.1 Diabetic Macular Oedema . . . . . . . . . . . . . . . . . . . . 276
11.4.2 Macular Oedema in Retinal Vein Occlusions . . . . . 289
11.5 Macular Oedema in Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . 294
11.5.1 Diagnosis of Macular Oedema in Uveitis . . . . . . . . 295
11.5.2 OCT Biomarkers in Uveitis Macular Oedema . . . . . 296
11.5.3 Treatment of Uveitis Macular Oedema . . . . . . . . . . 296
11.5.4 Sustained-Release Corticosteroid Implants . . . . . . . 297
11.5.5 Suprachoroidal Injection of Triamcinolone
Acetonide Suspension . . . . . . . . . . . . . . . . . . . . . . . 298
11.5.6 Alternative Strategies to Treat UME . . . . . . . . . . . . 298

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11.6 Post-surgical Macular Oedema . . . . . . . . . . . . . . . . . . . . . . . 298
11.6.1 Historical Perspective: Role of Vitreous Traction . . 299
11.6.2 Clinical Picture and Fluorescein Angiography
in CME Following Cataract Surgery . . . . . . . . . . . . 300
11.6.3 Objective Measurement of Pseudophakic Cystoid
Macular Oedema (PCME) . . . . . . . . . . . . . . . . . . . . 300
11.6.4 Incidence and Risk Factor for Post-surgery
Macular Oedema . . . . . . . . . . . . . . . . . . . . . . . . . . . 300
11.6.5 Role of Inammatory Mediators and
Prostaglandins in Surgical Trauma . . . . . . . . . . . . . 301
11.6.6 Pathogenesis and Prevention of PCME . . . . . . . . . . 301
11.6.7 Treatment of PCME . . . . . . . . . . . . . . . . . . . . . . . . . 302
11.7 Macular Oedema in Vitreoretinal Traction . . . . . . . . . . . . . . 302
11.8 Macular Cystic Changes in Inherited Macular
Dystrophies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303
11.9 Drug-Induced Macular Oedema . . . . . . . . . . . . . . . . . . . . . . 304
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305
12 Subretinal Fluid and Retinal Detachment . . . . . . . . . . . . . . . . . . 319
12.1 Developmental, Anatomical, and Physiological Aspects
in Brief . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 319
12.2 The Flow of Aqueous Fluid in the Eye . . . . . . . . . . . . . . . . . 319
12.3 Factors That Keep the Retina Attached to RPE
and the Accumulation of Subretinal Fluid (SRF) . . . . . . . . . 320
12.4 Causes of Subretinal Fluid . . . . . . . . . . . . . . . . . . . . . . . . . . . 321
Central Serous Chorioretinopathy . . . . . . . . . . . . . . . . . . . . 321
12.4.1 Subretinal Fluid in Choroidal Neovascular
Membranes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 327
12.4.2 Subretinal Fluid in Diabetic Macular Oedema . . . . 328
12.4.3 Subretinal Fluid in Retinal Vein Occlusions . . . . . . 329
12.4.4 Subretinal Fluid in Malignant Hypertension . . . . . . 330
12.4.5 Subretinal Fluid in Uveitis . . . . . . . . . . . . . . . . . . . . 331
12.4.6 Subretinal Fluid in Metastatic Cancer . . . . . . . . . . . 332
12.5 Consequence of Subretinal Fluid . . . . . . . . . . . . . . . . . . . . . . 334
12.5.1 Tractional Retinal Detachment . . . . . . . . . . . . . . . . 334
12.5.2 Myopic Tractional Maculopathy . . . . . . . . . . . . . . . 336
12.5.3 Treatment of Tractional Retinal Detachment . . . . . . 337
12.5.4 Rhegmatogenous Retinal Detachment . . . . . . . . . . . 337
12.5.5 Pathogenesis of Rhegmatogenous Retinal
Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 338
12.5.6 Risk Factors for Rhegmatogenous Retinal
Detachment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 339
12.5.7 Clinical Diagnosis of Rhegmatogenous
Retinal Detachment . . . . . . . . . . . . . . . . . . . . . . . . . 339
12.5.8 Development of Proliferative Vitreoretinopathy
in RRD . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 340
12.5.9 Treatment of RRD . . . . . . . . . . . . . . . . . . . . . . . . . . 340
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 341

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13 Macular Degeneration, Geographic Atrophy, and Inherited
Retinal Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351
13.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351
13.2 Anatomical Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . 351
13.3 Complement Pathway-Basics . . . . . . . . . . . . . . . . . . . . . . . . 352
13.4 Age-Related Changes in the Retina . . . . . . . . . . . . . . . . . . . . 353
13.5 Age-Related Macular Degeneration (AMD) . . . . . . . . . . . . . 354
13.5.1 Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354
13.5.2 Role of Complement Pathways in AMD . . . . . . . . . 354
13.5.3 Risk Factors for AMD and Its Pathogenesis . . . . . . 354
13.5.4 Epigenetic Factors in Dry AMD . . . . . . . . . . . . . . . 355
13.5.5 Clinical Diagnosis of AMD . . . . . . . . . . . . . . . . . . . 355
13.5.6 Classication of AMD . . . . . . . . . . . . . . . . . . . . . . . 356
13.6 Inherited Retinal Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . 361
13.6.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 361
13.6.2 Ancillary Lab Testing in Inherited Retinal
Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 363
13.6.3 Non-syndromic Retinitis Pigmentosa:
Clinical Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369
13.6.4 Leber Congenital Amaurosis . . . . . . . . . . . . . . . . . . 369
13.6.5 RPE 65-Associated LCA . . . . . . . . . . . . . . . . . . . . . 371
13.6.6 CEP290-Associated Retinal Dystrophy . . . . . . . . . . 372
13.6.7 Primary Ciliopathies and RP . . . . . . . . . . . . . . . . . . 372
13.6.8 Systemic Diseases Associated with Inherited
Retinal Degeneration . . . . . . . . . . . . . . . . . . . . . . . . 372
13.7 Inherited Macular Dystrophies . . . . . . . . . . . . . . . . . . . . . . . 379
13.7.1 Stargardt’s Disease and Fundus
Flavimaculatus (ABCA4 Disorders) . . . . . . . . . . . . 379
13.7.2 Bestrophinopathies . . . . . . . . . . . . . . . . . . . . . . . . . . 383
13.8 Mitochondrial Retinal Dystrophies . . . . . . . . . . . . . . . . . . . . 385
13.9 Treatment of Inherited Retinal Disorders . . . . . . . . . . . . . . . 387
13.9.1 Gene Supplement Therapy . . . . . . . . . . . . . . . . . . . . 387
13.9.2 Antisense Oligonucleotide Treatment . . . . . . . . . . . 388
13.9.3 Optogenetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 388
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 388
Contents
14 Vascular Malformations, Childhood Cancer Predisposition
Syndromes and Their Systemic Associations . . . . . . . . . . . . . . . . 397
14.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397
14.2 Retinal Capillary Hemangioblastoma . . . . . . . . . . . . . . . . . . 398
14.2.1 Retinal Capillary Hemangioblastoma—Historical
Aspects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398
14.2.2 Retinal Capillary Hemangioblastoma—Genetic
Aspects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398
14.2.3 Retinal Capillary Hemangioblastoma—Systemic
Associations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398
14.2.4 Retinal Capillary Hemangioblastoma (Angiomas) . 400
14.2.5 Treatment of von Hippel- Lindau’s Disease . . . . . . . 403
14.2.6 Active Surveillance in VHL . . . . . . . . . . . . . . . . . . . 403

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14.3 Sturge-Weber Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . 404
14.3.1 Sturge-Weber Syndrome—Port-Wine Stain . . . . . . 404
14.3.2 Sturge-Weber Syndrome—Diffuse Choroidal
Hemangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405
14.3.3 Circumscribed Choroidal Hemangiomas
Are Not Associated with SWS . . . . . . . . . . . . . . . . . 406
14.3.4 Sturge-Weber Syndrome—Glaucoma . . . . . . . . . . . 406
14.3.5 Sturge-Weber Syndrome—Neurological
Associations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 407
14.4 Arteriovenous Malformation of the Retina (AVM)—
Wyburn-Mason Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . 408
14.5 Congenital Retinal Macrovessel . . . . . . . . . . . . . . . . . . . . . . 409
14.5.1 Retinal Cavernous Hemangioma—Clinical
Presentations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 409
14.5.2 Retinal Cavernous Hemangioma—Systemic
Associations and Genetics . . . . . . . . . . . . . . . . . . . . 410
14.5.3 Retinal Cavernous Hemangioma—Complications . 410
14.5.4 Retinal Cavernous Hemangioma—Treatment . . . . . 410
14.6 Retinal Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 410
14.6.1 Hamartomas-Tuberous Sclerosis
Complex—Introduction . . . . . . . . . . . . . . . . . . . . . . 410
14.6.2 Tuberous Sclerosis Complex—Genetics . . . . . . . . . 412
14.6.3 Retinal Astrocytoma—Clinical Signs . . . . . . . . . . . 412
14.6.4 Retinal Astrocytoma—Treatment . . . . . . . . . . . . . . 413
14.7 Glioma of the Optic Pathways and Neurobromatosis . . . . . 413
14.7.1 Retinoblastoma–Clinical Presentations . . . . . . . . . . 414
14.7.2 Retinoblastoma—Genetics in Brief and
Implications of Testing. . . . . . . . . . . . . . . . . . . . . . . 416
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 417
15 Optic Disc Signs—Cupping, Swelling, Inflammation,
and Pallor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 423
15.1 Anatomical Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . 423
15.2 Blood Supply of the Visual Pathways . . . . . . . . . . . . . . . . . . 425
15.2.1 Blood Supply of the Optic Chiasm . . . . . . . . . . . . . 425
15.2.2 Blood Supply of the Optic Nerve . . . . . . . . . . . . . . . 425
15.2.3 Blood Supply of the Optic Nerve Head . . . . . . . . . . 426
15.3 Evaluation of the Optic Disc . . . . . . . . . . . . . . . . . . . . . . . . . 427
15.3.1 Clinical Assessment of the Optic Disc Size . . . . . . . 427
15.3.2 Optic Disc Cup (ODC) and Glaucoma . . . . . . . . . . 427
15.3.3 Challenges of Evaluation of the Optic
Disc Cupping . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 429
15.3.4 OCT Evaluation of the Glaucomatous
Optic Neuropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . 430
15.3.5 Bruch’s Membrane Opening—Minimum
Rim Width in Glaucomatous Optic Neuropathy . . . 430
15.3.6 Macular OCT in Glaucoma . . . . . . . . . . . . . . . . . . . 431
15.3.7 Horizontal Raphe Hemield Test in Glaucoma . . . . 431

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15.3.8 OCT Angiography . . . . . . . . . . . . . . . . . . . . . . . . . . 431
15.3.9 Articial Intelligence for Automatic
Diagnosis of Glaucomatous Optic Neuropathy . . . . 431
15.3.10 Myopia and Glaucomatous Optic Neuropathy . . . . 431
15.4 Choosing a Tool for Fundus Examination in
Neuro-Ophthalmological Disorders . . . . . . . . . . . . . . . . . . . . 432
15.4.1 Optic Nerve Head Edema and
Papilledema-Clinical Evaluation . . . . . . . . . . . . . . . 435
15.4.2 Ocular Causes of ONH Swelling . . . . . . . . . . . . . . . 436
15.4.3 Signs of Papilledema . . . . . . . . . . . . . . . . . . . . . . . . 436
15.4.4 Role of OCT in Papilledema . . . . . . . . . . . . . . . . . . 440
15.4.5 Psudopapilledema Due to Optic Disc Drusen . . . . . 441
15.4.6 Papilledema in Idiopathic Intracranial
Hypertension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 442
15.5 Optic Neuritis—Inammation of the Optic Nerve . . . . . . . . 444
15.5.1 Epidemiology of Optic Neuritis . . . . . . . . . . . . . . . . 445
15.5.2 Evaluation of Optic Neuritis in the Clinic . . . . . . . . 446
15.5.3 Diagnosis of Optic Neuritis . . . . . . . . . . . . . . . . . . . 449
15.5.4 Multiple Sclerosis-Associated Optic
Neuritis (MS-ON) . . . . . . . . . . . . . . . . . . . . . . . . . . 449
15.5.5 Pathogenic Antibody-Associated
Optic Neuritis Syndromes . . . . . . . . . . . . . . . . . . . . 450
15.6 Optic Disc Pallor (Atrophy) . . . . . . . . . . . . . . . . . . . . . . . . . . 451
15.6.1 Inherited Optic Neuropathies . . . . . . . . . . . . . . . . . . 453
15.6.2 Chiasmal Compression Syndrome . . . . . . . . . . . . . . 456
15.6.3 Ischaemic Optic Neuropathies (ION) . . . . . . . . . . . 460
15.6.4 Posterior Ischaemic Optic Neuropathy (PION) . . . . 462
15.6.5 Arteritic Anterior Ischaemic Optic Neuropathy . . . . 463
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 465
Contents
Part II Extraocular Signs
16 Pupillary Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475
16.1 Anatomical Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . 475
16.1.1 The Sphincter Pupillae . . . . . . . . . . . . . . . . . . . . . . . 475
16.1.2 The Dilator Pupillae . . . . . . . . . . . . . . . . . . . . . . . . . 475
16.1.3 The Ciliary Ganglion . . . . . . . . . . . . . . . . . . . . . . . . 475
16.1.4 Pupil Size . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 476
16.2 Pathway for the Pupil Light Reex . . . . . . . . . . . . . . . . . . . . 476
16.2.1 Sympathetic Pathway . . . . . . . . . . . . . . . . . . . . . . . . 478
16.3 Testing for Pupillary Reexes . . . . . . . . . . . . . . . . . . . . . . . . 480
16.3.1 Swinging Flashlight Test . . . . . . . . . . . . . . . . . . . . . 481
16.3.2 Phases of Pupil Light Reex . . . . . . . . . . . . . . . . . . 481
16.3.3 Causes of RAPD . . . . . . . . . . . . . . . . . . . . . . . . . . . 481
16.4 Pupillary Changes in Lesions of
Parasympathetic Pathways . . . . . . . . . . . . . . . . . . . . . . . . . . . 481
16.5 Argyll Robertson Pupil . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 482
16.5.1 Automated Pupillometry . . . . . . . . . . . . . . . . . . . . . 483
16.5.2 Neurological Pupil Index . . . . . . . . . . . . . . . . . . . . . 484

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16.6 Effect of Head Injury on Pupillary Parameters . . . . . . . . . . . 485
16.6.1 Hutchinson Pupil . . . . . . . . . . . . . . . . . . . . . . . . . . . 485
16.6.2 Parinaud’ Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . 485
16.6.3 Horner’s Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . 486
16.6.4 Adie’s Tonic Pupil . . . . . . . . . . . . . . . . . . . . . . . . . . 487
16.7 Limitations of Automated Pupillometry . . . . . . . . . . . . . . . . 488
16.8 Effect of Drugs on Pupillary Parameters . . . . . . . . . . . . . . . . 488
16.9 Pupillometry in Psychological Evaluation . . . . . . . . . . . . . . . 488
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489
17 Red Eyes—Conjunctivitis, Corneal Ulcers,
Dry Eye Disease, and Acute Uveitis . . . . . . . . . . . . . . . . . . . . . . . . 493
17.1 Anatomy of the Conjunctiva . . . . . . . . . . . . . . . . . . . . . . . . . 493
17.1.1 Blood Supply of the Conjunctiva . . . . . . . . . . . . . . . 493
17.1.2 Nerve Supply of Conjunctiva . . . . . . . . . . . . . . . . . . 494
17.1.3 Lymphatic Drainage of the Conjunctiva . . . . . . . . . 494
17.2 Brief Anatomy of the Cornea . . . . . . . . . . . . . . . . . . . . . . . . . 495
17.3 Red Eyes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 495
17.3.1 Allergic Conjunctivitis—Clinical Features
and Pathogenetic Mechanisms in Brief . . . . . . . . . . 495
17.3.2 Vernal Keratoconjunctivitis . . . . . . . . . . . . . . . . . . . 496
17.4 Infectious Conjunctivitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 498
17.4.1 Viral Conjunctivitis . . . . . . . . . . . . . . . . . . . . . . . . . 498
17.4.2 Bacterial Conjunctivitis . . . . . . . . . . . . . . . . . . . . . . 500
17.5 Corneal Ulcers . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 502
17.5.1 Microbiological Prole of Corneal Ulcers . . . . . . . . 502
17.5.2 Predisposing Factors for Corneal Ulcers . . . . . . . . . 503
17.5.3 Clinical Features of Corneal Ulcers . . . . . . . . . . . . . 504
17.5.4 Parasitic Corneal Ulcers . . . . . . . . . . . . . . . . . . . . . . 509
17.6 Dry Eye Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 511
17.6.1 Physiological Considerations of Tears in Brief . . . . 511
17.6.2 Dry Eye Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . 512
17.6.3 Systemic Diseases and Other Factors
Associated with DED . . . . . . . . . . . . . . . . . . . . . . . . 512
17.6.4 Pathogenesis of Dry Eye Disorder (DED) . . . . . . . . 512
17.6.5 Diagnosis of Dry Eye Disease . . . . . . . . . . . . . . . . . 514
17.6.6 Management of dry eye Disorders . . . . . . . . . . . . . . 516
17.6.7 Newer Agents in DED . . . . . . . . . . . . . . . . . . . . . . . 516
17.7 Anterior Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517
17.7.1 Anatomical, Physiological, and Immunological
Considerations of the Anterior Segment . . . . . . . . . 517
17.7.2 Immunology of Uveitis in Brief . . . . . . . . . . . . . . . . 518
17.7.3 Mediators of Inammation . . . . . . . . . . . . . . . . . . . 518
17.7.4 Anterior Uveitis-Epidemiology . . . . . . . . . . . . . . . . 519
17.7.5 Tubulointerstitial Nephritis and Uveitis (TINU) . . . 524
17.7.6 Juvenile Idiopathic Arthritis (JIA) -Associated
Uveitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 526
17.7.7 Autoinammatory Syndromes and Uveitis . . . . . . . 528
17.7.8 Infective Anterior Uveitis—Viral Uveitis . . . . . . . . 530
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 532

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18 Episcleritis, Scleritis, and Peripheral Corneal Ulceration . . . . . 543
18.1 Sclera and Episclera- Anatomical Considerations . . . . . . . . . 543
18.2 Episcleritis and Scleritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 543
18.2.1 Epidemiology of Episcleritis and Scleritis . . . . . . . . 544
18.2.2 Etiology of Non-infectious Scleritis . . . . . . . . . . . . 544
18.2.3 Etiology of Infectious Scleritis . . . . . . . . . . . . . . . . 545
18.2.4 Clinical Characteristics of Episcleritis . . . . . . . . . . . 546
18.2.5 Clinical Characteristics of Scleritis . . . . . . . . . . . . . 548
18.2.6 Surgically Induced Necrotizing Scleritis . . . . . . . . . 554
18.2.7 Complications of Episcleritis and Scleritis . . . . . . . 554
18.2.8 Anterior Segment Optical Coherence
Tomography to Differentiate Episcleritis
from Scleritis
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 555
18.2.9 Differentiating Infectious from Autoimmune
Scleritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 555
18.2.10 Pathogenesis of Scleritis . . . . . . . . . . . . . . . . . . . . . 556
18.2.11 Pathology of Scleritis . . . . . . . . . . . . . . . . . . . . . . . . 556
18.2.12 Treatment of Episcleritis and Scleritis . . . . . . . . . . . 556
18.3 Cornea-Anatomical Considerations . . . . . . . . . . . . . . . . . . . . 558
18.3.1 Epidemiology and Etiology of Peripheral
Ulcerative Keratitis . . . . . . . . . . . . . . . . . . . . . . . . . 558
18.3.2 Systemic Associations of Peripheral
Ulcerative Keratitis . . . . . . . . . . . . . . . . . . . . . . . . . 558
18.3.3 Peripheral Stromal Keratitis and Peripheral
Ulcerative Keratitis . . . . . . . . . . . . . . . . . . . . . . . . . 559
18.3.4 Para Central Corneal Melt-Pathogenesis . . . . . . . . . 561
18.3.5 Mooren’s Ulcer . . . . . . . . . . . . . . . . . . . . . . . . . . . . 561
18.3.6 Pathology of PUK . . . . . . . . . . . . . . . . . . . . . . . . . . 562
18.3.7 Systemic Implications for PUK in Systemic
Collagen Vascular Diseases . . . . . . . . . . . . . . . . . . . 562
18.3.8 Pathogenetic Mechanism for Peripheral
Ulcerative Keratitis . . . . . . . . . . . . . . . . . . . . . . . . . 562
18.3.9 Treatment of PUK . . . . . . . . . . . . . . . . . . . . . . . . . . 563
18.3.10 Adjunctive Local Treatment to Restore
the Integrity of the Globe . . . . . . . . . . . . . . . . . . . . . 565
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 565
Contents
19 Lid Signs, Paralytic Squint, and Ocular Movement
Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 573
19.1 Lids: Anatomical Considerations . . . . . . . . . . . . . . . . . . . . . . 573
19.2 Anthropometry of Lids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 574
19.3 Lid Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 575
19.3.1 Ptosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 575
19.3.2 Lid Retraction of the Upper Eyelid . . . . . . . . . . . . . 580
19.3.3 Lid Lag . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 581
19.3.4 Spontaneous Blinking . . . . . . . . . . . . . . . . . . . . . . . 584
19.3.5 Blepharospasm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 586

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19.4 Extraocular Muscles- Anatomical Considerations . . . . . . . . . 586
19.5 Cranial Nerve Palsies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 588
19.5.1 Oculomotor Nerve Paresis . . . . . . . . . . . . . . . . . . . . 588
19.5.2 Recurrent Painful Ophthalmoplegic
Neuropathy (Previously Labelled)
Ophthalmoplegic Migraine) . . . . . . . . . . . . . . . . . . . 589
19.5.3 Tolosa-Hunt Syndrome . . . . . . . . . . . . . . . . . . . . . . 590
19.5.4 Generalized Myasthenia Gravis . . . . . . . . . . . . . . . . 591
19.5.5 Ocular Myasthenia Gravis . . . . . . . . . . . . . . . . . . . . 593
19.6 Paralytic Squint (Tropia) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 594
19.6.1 Testing for the Paretic Muscle . . . . . . . . . . . . . . . . . 595
19.6.2 Parks-Bielschowsky 3-Step Test-Diagnosing
Paresis of a Cyclovertical Muscle
. . . . . . . . . . . . . . 595
19.6.3 Diplopia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 596
19.6.4 Hess/Lees Screen/KM Digital Screen . . . . . . . . . . . 596
19.7 Brief Physiological Considerations of Eye Movements . . . . 597
19.7.1 Movements of the Eye-Ductions . . . . . . . . . . . . . . . 599
19.7.2 Conjugate Movements-Versions . . . . . . . . . . . . . . . 599
19.7.3 Vergence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 600
19.8 Supranuclear Control of Conjugate Ocular
Movements (Gaze) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 601
19.8.1 Saccades . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 601
19.8.2 Smooth Pursuit Movements . . . . . . . . . . . . . . . . . . . 603
19.8.3 Vestibulo-Ocular Reexes . . . . . . . . . . . . . . . . . . . . 604
19.8.4 Optokinetic Movement Reex . . . . . . . . . . . . . . . . . 605
19.9 Nystagmus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 605
19.10 Disorders of the Ocular Movements . . . . . . . . . . . . . . . . . . . 606
19.10.1 Internuclear Ophthalmoplegia . . . . . . . . . . . . . . . . . 606
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 608
20 Orbital Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 617
20.1 Anatomical Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . 617
20.1.1 Superior Orbital Fissure . . . . . . . . . . . . . . . . . . . . . . 618
20.1.2 Inferior Orbital Fissure . . . . . . . . . . . . . . . . . . . . . . 619
20.1.3 Contents of the Orbit . . . . . . . . . . . . . . . . . . . . . . . . 619
20.1.4 Spaces of the Orbit . . . . . . . . . . . . . . . . . . . . . . . . . . 619
20.1.5 Spaces of the Orbit and Orbital Pathologies . . . . . . 619
20.2 Measurement of the Proptosis . . . . . . . . . . . . . . . . . . . . . . . . 620
20.3 Orbital Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 620
20.4 Thyroid Eye Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 620
20.4.1 Pathogenesis of Thyroid- Associated Orbitopathy . . 620
20.4.2 Ophthalmic Signs of Thyroid Eye Disease . . . . . . . 622
20.4.3 Evaluation of the Patient with Thyroid
Orbitopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 624
20.4.4 Laboratory Evaluation of Thyroid-Associated
Orbitopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 626
20.4.5 Prevention of Thyroid- Associated Orbitopathy . . . . 626
20.4.6 Treatment of Thyroid- Associated Orbitopathy . . . . 626

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20.5 Inltrative Lesions of Extraocular Muscles . . . . . . . . . . . . . . 628
20.6 Causes of Extraocular Muscles Enlargement. . . . . . . . . . . . . 628
20.6.1 Orbital Myositis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 628
20.6.2 IgG4-Related Ophthalmic Disease (IgG4-ROD) . . . 628
20.7 Orbital Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 629
20.8 Orbital Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 631
20.9 Lacrimal Gland Granulomas and Tumours . . . . . . . . . . . . . . 632
20.10 Orbital Granulomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 634
20.11 Tuberculosis of the Orbit . . . . . . . . . . . . . . . . . . . . . . . . . . . . 635
20.12 Orbital Granulomatosis with Polyangiitis . . . . . . . . . . . . . . . 636
20.13 Vascular Malformations of the Orbit . . . . . . . . . . . . . . . . . . . 636
20.13.1 Carotid-Cavernous Fistula . . . . . . . . . . . . . . . . . . . . 636
20.13.2 Other Orbital Malformations . . . . . . . . . . . . . . . . . 636
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 642
Contents
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