Contents

Introduction to the BCSC xvii

Objectives ...... 1 Introduction 3 PART I Fundamentals and Diagnostic Approaches 5

1 Basic Anatomy ...... 7 The Vitreous 7 Neurosensory Ret­ina 9 Ret­i­nal Topography 9 Ret­i­nal Layers and Neurosensory Ele­ments 10 Ret­i­nal Vasculature and Oxygen Supply ...... 16 Ret­i­nal Pigment Epithelium 17 Bruch Membrane ...... 19 Choroid 19 Sclera 20

2 Diagnostic Approach to Ret­i­nal Disease 21 Ophthalmoscopy 21 Imaging Technologies 22 Fundus Camera Imaging 22 Scanning ­Laser Ophthalmoscopy 24 Optical Coherence Tomography ...... 25 Optical Coherence Tomography Angiography ...... 28 Fundus Autofluorescence ...... 29 Adaptive Optics Imaging ...... 33 Ret­i­nal Angiographic Techniques 33 Ultrasonography ...... 39

3 Ret­i­nal Physiology and Psychophysics 41 Electrophysiologic Testing 41 Electroretinography ...... 42 Full-­Field (Ganzfeld) ERG 42 Multifocal ERG 45 Pattern ERG 45 Clinical Considerations ...... 46 Electro-­oculography ...... 50 Visual Evoked Cortical Potentials 52 Psychophysical Testing ...... 53

vii viii  Contents

Color Vision 54 Contrast Sensitivity 56 Dark Adaptometry ...... 57

PART II Disorders of the Retina­ and Vitreous 59 4 Age-­Related Macular Degeneration and Other ­ Causes of Choroidal Neovascularization 61 Age-­Related Macular Degeneration 61 Ge­ne­tics and AMD 62 Nonneovascular AMD ...... 63 Neovascular AMD ...... 71 Other ­Causes of Choroidal Neovascularization 86 Ocular Histoplasmosis Syndrome 86 Angioid Streaks ...... 87 Pathologic Myopia ...... 89 Idiopathic CNV and Miscellaneous ­Causes of CNV 89

5 Ret­i­nal Vascular Disease: Diabetic Retinopathy . . . . . 91 Terminology and Classification ...... 91 Diabetes Terminology 91 Diabetic Retinopathy Terminology ...... 91 Epidemiology of Diabetic Retinopathy ...... 92 Pathogenesis of Diabetic Retinopathy 93 Recommended Diabetes Mellitus–­Related Ophthalmic Examinations 93 Systemic Medical Management of Diabetic Retinopathy 96 Abnormalities Associated With Vision Loss From Diabetic Retinopathy 98 Nonproliferative Diabetic Retinopathy ...... 99 Treatment of Nonproliferative Diabetic Retinopathy 101 Proliferative Diabetic Retinopathy ...... 102 Management of Proliferative Diabetic Retinopathy and Its Complications 103 Diabetic Macular Edema ...... 108 Classification of Diabetic Macular Edema ...... 109 Treatment of Diabetic Macular Edema 110 Cataract Surgery in Patients With Diabetes Mellitus 116 6 Ret­i­nal Vascular Diseases Associated With Cardiovascular Disease ...... 121 Systemic Arterial Hypertension ...... 121 Hypertensive Retinopathy 121 Hypertensive Choroidopathy 123 Hypertensive Optic Neuropathy ...... 123 Contents d ix

Ret­i­nal Vein Occlusion ...... 125 Branch Ret­i­nal Vein Occlusion 126 Central Ret­i­nal Vein Occlusion 130 Pharmacologic Management of Ret­i­nal Vein Occlusion ...... 135 Ocular Ischemic Syndrome and Retinopathy of Carotid Occlusive Disease ...... 138 Symptoms and Signs of Ocular Ischemic Syndrome 138 Etiology and Course of Ocular Ischemic Syndrome ...... 139 Treatment of Ocular Ischemic Syndrome 140 Arterial Occlusive Disease 140 Capillary Ret­i­nal Arteriole Obstruction (Cotton-­Wool Spots) 140 Branch Ret­i­nal Artery Occlusion ...... 141 Cilioret­i­nal Artery Occlusion 143 Paracentral Acute ­Middle Maculopathy ...... 143 Central Ret­i­nal Artery Occlusion 143 Ophthalmic Artery Occlusion ...... 146 Arterial Macroaneurysms 147

7 Other Ret­i­nal Vascular Diseases ...... 149 Sickle Cell Retinopathy ...... 149 Nonproliferative Sickle Cell Retinopathy 149 Proliferative Sickle Cell Retinopathy 150 Other Ocular Abnormalities in Sickle Cell Hemoglobinopathies 154 Management of Sickle Cell Retinopathy ...... 154 Vasculitis ...... 155 Cystoid Macular Edema 156 Etiologies of CME ...... 157 Incidence of CME ...... 157 Treatment of CME ...... 159 Coats Disease ...... 159 Macular Telangiectasia ...... 161 Macular Telangiectasia Type 1 ...... 161 Macular Telangiectasia Type 2 ...... 161 Macular Telangiectasia Type 3 ...... 164 Phakomatoses 165 Von Hippel–­Lindau Disease ...... 165 Wyburn-­Mason Syndrome 168 Ret­i­nal Cavernous Hemangioma ...... 168 Radiation Retinopathy ...... 170 Valsalva Retinopathy ...... 171 Purtscher Retinopathy and Purtscherlike Retinopathy 171 Terson Syndrome ...... 173 x  Contents

8 Retinopathy of Prematurity 175 Introduction 175 Epidemiology ...... 175 Terminology and Classification ...... 175 Pathophysiology of ROP ...... 180 Natu­ral Course 181 Associated Conditions and Late Sequelae ...... 181 Screening Recommendations ...... 182 Screening Criteria ...... 182 Screening Intervals ...... 183 Fundus Photographic Screening of ROP 184 Prevention and Risk ­Factors 184 Treatment 185 ­Laser and Cryoablation Surgery ...... 185 Anti-­VEGF Drugs ...... 186 Vitrectomy and Scleral Buckling Surgery 187

9 Choroidal Disease ...... 189 Central Serous Chorioretinopathy ...... 189 Demographics ...... 189 Imaging 191 Differential Diagnosis 193 Treatment 194 Choroidal Perfusion Abnormalities 194 Arteritic Disease ...... 195 Nonarteritic Disease ...... 195 Choriocapillaris Blood Flow Abnormalities 198 Increased Venous Pressure 198 Age-­Related Choroidal Atrophy ...... 200 Choroidal Folds ...... 200 Choroidal Hemangiomas ...... 202 Uveal Effusion Syndrome ...... 203 Bilateral Diffuse Uveal Melanocytic Proliferation 204

10 Myopia and Pathologic Myopia 207 Prevention ...... 207 The Ret­ina ...... 208 Bruch Membrane 210 Choroidal Neovascularization ...... 211 The Choroid in Pathologic Myopia ...... 212 The Sclera 216 The Optic Nerve 217 Contents d xi

11 Focal and Diffuse Choroidal and Ret­i­nal Inflammation 219 Noninfectious Ret­i­nal and Choroidal Inflammation 219 White Dot Syndromes 219 Chorioret­i­nal Autoimmune Conditions ...... 229 Sympathetic Ophthalmia ...... 233 Uveitis Masquerade: Intraocular Lymphoma ...... 233 Infectious Ret­i­nal and Choroidal Inflammation ...... 235 Cytomegalovirus Retinitis 235 Non-­CMV Necrotizing Herpetic Retinitis ...... 236 Endogenous Bacterial Endophthalmitis ...... 237 Fungal Endophthalmitis 239 Tuberculosis 240 Syphilitic Chorioretinitis 241 Cat-­Scratch Disease 242 Toxoplasmic Retinochoroiditis 243 Toxocariasis ...... 244 Lyme Disease ...... 245 Diffuse Unilateral Subacute Neuroretinitis 246 West Nile Virus Chorioretinitis 246 Zika Virus Chorioretinitis 247 Ebola Virus Panuveitis 247 Chikungunya Virus Retinitis 247

12 Congenital and Stationary Ret­i­nal Disease ...... 249 Color Vision (Cone System) Abnormalities ...... 249 Congenital Color Deficiency 249 Night Vision (Rod System) Abnormalities 251 Congenital Night-­Blinding Disorders With Normal Fundi ...... 251 Congenital Night-­Blinding Disorders With Fundus Abnormality . . . 252

13 Hereditary Ret­i­nal and Choroidal Dystrophies 255 Classification ...... 255 General Diagnostic Considerations 258 General Ge­ne­tic Considerations ...... 259 General Management Considerations 259 Diffuse Dystrophies ...... 261 Diffuse Photoreceptor Dystrophies ...... 261 Choroidal Dystrophies ...... 267 Macular Dystrophies 269 Stargardt Disease 269 Best Disease or Best Vitelliform Dystrophy 271 Adult-­Onset Vitelliform Lesions ...... 272 Early-­Onset “Drusenoid” Macular Dystrophies 273 Pattern Dystrophies 276 Aty­pi­cal and “Occult” Macular Dystrophies ...... 277 Inner Ret­i­nal Dystrophies 278 X-­Linked Retinoschisis ...... 278 xii  Contents

14 Ret­i­nal Degenerations Associated With Systemic Disease 281 Ret­i­nal Degeneration With Systemic Involvement ...... 281 Infantile-­Onset to Early Childhood–­Onset Syndromes 281 Bardet-­Biedl Syndrome ...... 281 Hearing Loss and Pigmentary Retinopathy: Usher Syndrome 284 Neuromuscular Disorders ...... 285 Other Organ System Disorders 285 Paraneoplastic and Autoimmune Retinopathies 286 Metabolic Diseases 288 Albinism ...... 288 Central Ner­vous System Metabolic Abnormalities 288 Amino Acid Disorders ...... 293 Mitochondrial Disorders ...... 293

15 Systemic Drug-­Induced Ret­i­nal Toxicity 295 Drugs Causing Abnormalities of the Ret­i­nal Pigment Epithelium/ Photoreceptor Complex ...... 295 Chloroquine Derivatives 295 Phenothiazines 297 Miscellaneous Medi­cations 298 Drugs Causing Occlusive Retinopathy or Microvasculopathy 300 Drugs Causing Ganglion Cell and Optic Nerve Toxicity 301 Drugs Causing Macular Edema ...... 301 Drugs Causing Crystalline Retinopathy 302 Drugs Causing Abnormalities in Color Vision and Electroretinography ...... 304 Miscellaneous Drugs Causing Ocular Toxicities ...... 305

16 Ret­i­nal Detachment and Predisposing Lesions . . . . . 307 Posterior Vitreous Detachment 307 Examination and Management of Posterior Vitreous Detachment 309 Lesions That Predispose Eyes to Ret­i­nal Detachment ...... 309 Lattice Degeneration ...... 309 Vitreoret­i­nal Tufts ...... 311 Meridional Folds, Enclosed Ora Bays, and Peripheral Ret­i­nal Excavations 312 Lesions That Do Not Predispose Eyes to Ret­i­nal Detachment 313 Paving-­Stone Degeneration ...... 313 Ret­i­nal Pigment Epithelial Hyperplasia ...... 313 Ret­i­nal Pigment Epithelial Hypertrophy 314 Peripheral Cystoid Degeneration ...... 314 Ret­i­nal Breaks 314 Traumatic Breaks 315 Trauma in Young Eyes 316 Contents d xiii

Prophylactic Treatment of Ret­i­nal Breaks ...... 317 Symptomatic Ret­i­nal Breaks ...... 318 Asymptomatic Ret­i­nal Breaks ...... 318 Lattice Degeneration ...... 319 Aphakia and Pseudophakia ...... 319 Fellow Eye in Patients With Ret­i­nal Detachment ...... 319 Subclinical Ret­i­nal Detachment 319 Ret­i­nal Detachment ...... 320 Rhegmatogenous Ret­i­nal Detachment 320 Tractional Ret­i­nal Detachment 325 Exudative Ret­i­nal Detachment 325 Differential Diagnosis of Ret­i­nal Detachment ...... 326 Retinoschisis 326 Differentiation of Retinoschisis From Rhegmatogenous Ret­i­nal Detachment ...... 327 Macular Lesions Associated With Ret­i­nal Detachment ...... 328 Optic Pit Maculopathy ...... 328 Macular Holes in High Myopia 330

17 Diseases of the Vitreous and Vitreoret­i­nal Interface 331 Posterior Vitreous Detachment 331 Epiret­i­nal Membranes 332 Vitreomacular Traction Diseases ...... 336 Idiopathic Macular Holes ...... 337 Developmental Abnormalities ...... 340 Tunica Vasculosa Lentis 340 Prepapillary Vascular Loops ...... 341 Per­sis­tent Fetal Vasculature ...... 341 Hereditary Hyaloideoretinopathies With Optically Empty Vitreous: Wagner and Stickler Syndromes 342 Familial Exudative Vitreoretinopathy 343 Vitreous Opacities ...... 344 Vitreous Degeneration and Detachment Associated Opacities (“Floaters”) ...... 344 Asteroid Hyalosis 346 Vitreous Hemorrhage 347 Pigment Granules 348 Cholesterolosis 348 Amyloidosis 348 Vitreous Abnormalities Secondary to Surgery ...... 349

18 Posterior Segment Manifestations of Trauma 351 Evaluation of the Patient ­After Ocular Trauma 351 Blunt Trauma Without Break in Eye Wall ...... 353 Commotio Ret­i­nae ...... 353 Choroidal Rupture ...... 354 Posttraumatic Macular Hole ...... 355 xiv  Contents

Vitreous Hemorrhage 357 Traumatic Chorioret­i­nal Disruption (Ret­i­nal Sclopetaria) ...... 357 Open-­Globe Injuries ...... 358 Scleral Rupture 358 Lacerating and Penetrating Injuries 358 Perforating Injuries 358 Surgical Management 359 Intraocular Foreign Bodies 360 Posttraumatic Endophthalmitis ...... 362 Prognostication of Globe Injuries 363 Sympathetic Ophthalmia ...... 364 Avulsion of the Optic Nerve Head ...... 365 Abusive Head Trauma ...... 365 Photic Damage 367 Solar Retinopathy 367 Phototoxicity From Ophthalmic Instrumentation 368 Occupational Light Toxicity ...... 369 Handheld Laser-­Pointer Injury 369

PART III Selected Therapeutic Topics ...... 371

19 ­Laser Therapy for Posterior Segment Diseases . . . . . 373 Basic Princi­ples of Photocoagulation ...... 373 Choice of ­Laser Wavelength ...... 373 Practical Aspects of ­Laser Photocoagulation ...... 375 Complications of Photocoagulation 378 Transpupillary Thermotherapy 380 Photodynamic Therapy 380 Complications of Photodynamic Therapy ...... 380

20 Vitreoret­i­nal Surgery and Intravitreal Injections 381 Pars Plana Vitrectomy ...... 381 Vitrectomy for Selected Macular Diseases 382 Macular Epiret­i­nal Membranes 382 Vitreomacular Traction Diseases ...... 382 Submacular Hemorrhage ...... 385 Vitrectomy for Vitreous Opacities ...... 386 Vitrectomy for Complications of Diabetic Retinopathy ...... 386 Vitreous Hemorrhage 386 Diabetic Tractional Ret­i­nal Detachment 387 Diabetic Macular Edema 388 Vitrectomy for Posterior Segment Complications of Anterior Segment Surgery 388 Postoperative Endophthalmitis 388 Retained Lens Fragments ­After Phacoemulsification 391 Posteriorly Dislocated Intraocular Lenses ...... 393 Cystoid Macular Edema 393 Contents d xv

Suprachoroidal Hemorrhage 394 Needle Penetration of the Globe ...... 395 Rhegmatogenous Ret­i­nal Detachment Surgery 396 Techniques for Surgical Repair of Ret­i­nal Detachments ...... 397 Outcomes Following Ret­i­nal Reattachment Surgery 401 Complications of Pars Plana Vitrectomy 402 Intravitreal Injections 403

Basic Texts ...... 405 Related Acad­emy Materials ...... 407 Requesting Continuing Medical Education Credit ...... 409 Study Questions ...... 411 Answer Sheet for Section 12 Study Questions ...... 421 Answers ...... 423 Index 431