Vascular Tumors and Malformations of the Ocular Fundus

1. Choroidal Hemangioma.- I. Pathogenesis.- II. Clinical forms.- II.1. Solitary choroidal hemangioma.- II.2. Sturge-Weber syndrome.- III. Symptoms.- III.1. Solitary choroidal hemangioma.- III.2. Sturge-Weber syndrome.- IV. Complications.- IV. 1. Retinal complications.- IV.2. Claucoma.- V. Diagnosis.- V.1. Ophthalmoscopy.- V.2. Diaphanoscopy.- V.3. Fluorescein angiography.- V.4. Infrared angiography.- V.5. Radiophosphorus uptake test.- V.6. Ultrasonography.- V.7. Radiography.- VI. Histopathology.- VII. Treatment.- VII.1. Photocoagulation.- VII.2. Retinal detachment surgery in diffuse hemangiomas.- VII.3. Radiotherapy.- 2. Retinal Angiomatosis — von Hippel-Lindau’s Disease.- I. Pathogenesis.- II. Incidence.- III. Heredity.- IV. Ocular findings.- IV.1. Retinal angiomatosis.- IV.2. Optic disc angiomas.- IV.3. Optic nerve hemangiomas.- IV.4. Ocular symptoms from intracranial manifestations of vHL diseases.- V. Associated neurological and visceral involvement or von Hippel-Lindau’s disease.- VI. Association of vHL disease with other diseases.- VI. 1. Association with other phakomatoses.- VI.2. Association with retinitis pigmentosa.- VII. Diagnosis.- VII. 1. Retinal angiomatosis.- VII.2. Von Hippel-Lindau’s disease.- VIII. Differential diagnosis.- IX. Pathology.- IX.1. The endothelial cells.- IX.2. The pericytes.- IX.3. Nestes of round to polyhedral cells.- X. Treatment.- X.1. Radiotherapy.- X.2. Transscleral diathermy.- X.3. Cryotherapy.- X.4. Photocoagulation.- X.5. Surgery.- 3. Capillary Hemangioma of the Optic Disc.- I. Classification of angiomatous tumors of the optic disc.- II. Incidence.- III. Clinical features.- IV. Relationship with von Hippel-Lindau’s disease.- V. Diagnosis.- VI. Differential diagnosis.- VI.1. Papiledema.- VI.2. Drusen of the optic disc.- VI.3. Peripapillary, choroidal neovascularization.- VI.4. Arterial macro-aneurysms.- VI.5. Inflammatory conditions.- VI.6. Congenital lesions.- VI.7. Tumors.- VII. Histopathology.- VIII. Treatment.- VIII.1. Radiotherapy.- VIII.2. Photocoagulation.- 4. Cavernous Hemangioma of the Retina and of the Optic Disc.- I. Incidence.- II. Clinical features.- II.1. Age at presentation.- II.2. Presenting symptoms.- II.3. Subjective signs.- II.4. Ophthalmological aspect.- II.5. Evolution.- III. Systemic manifestations — Gass’ syndrome.- IV. Differential diagnosis.- V. Histopathology.- VI. Treatment.- 5. Congenital Arteriovenous Communications in the Retina.- I. Pathogenesis.- II. Heredity.- III. Incidence.- IV. Ocular manifestations.- IV.1. Ophthalmoscopic aspects.- IV.2. Anterior segment.- IV.3. Orbit.- V. Wyburn-Mason syndrome.- V.1. Neurological signs.- V.2. Dermatological signs.- V.3. Muscosal involvement.- VI. Evolution of the retinal lesions.- VII. Histopathology.- VII.1. Extraocular AVM.- VII.2. Ocular lesions.- VIII. Diagnosis.- IX. Differential diagnosis.- IX.1. Other phakomatoses.- IX.2. Arteriovenous fistula.- IX.3. Secondary vascular retinal changes.- X. Treatment of the fundus lesions.- 6. Neurofibromatosis or von Recklinghausen’s Disease.- I. Pathogenesis.- II. Incidence and heredity.- III. Pathognomonic lesions.- III.1. Pigmentary changes of the skin.- III.2. Neurofibromas.- III.3. Lisch nodules of the iris.- IV. Fundus examinations in von Recklinghausen’Es disease.- IV.1. Choroidal lesions.- IV.2. Retina.- IV.3. Optic disk.- V. Other ophthalmological manifestations.- V.1. Orbit.- V.2. Tumors of the eye (except choroidal and retinal tumors).- V.3. Congenital glaucoma.- V.4. Cataract.- VI. Association with other phakomatoses.- VII. Treatment.- 7. TUBEROUS SCLEROSIS.- I. Incidence and heredity.- II. Diagnostic criteria.- III. Non-ocular manifestations of tuberous sclerosis.- III. 1. Cutaneous manifestations.- III.2. Neurological manifestations.- III.3. Visceral manifestations.- IV. Ocular manifestations of tuberous sclerosis.- IV.1. Clinical aspect.- IV.2. Histopathology.- V. Associations.- 8. Congenital Retinal Telangiectasis.- I. Pathogenesis.- II. Incidence.- III. Heredity.- IV. Clinical aspects.- IV.1. Presenting symptoms.- IV.2. Ophthalmoscopy.- IV.3. Fluorescein angiography.- IV.4. Natural history.- V. Associated conditions.- VI. Differential diagnosis.- VII. Histopathology.- VIII. Treatment.- 9. Idiopathic Juxtafoveolar Retinal Telangiectasis.- I. Incidence.- II. Heredity.- III. Clinical aspects.- III.1. Presenting symptoms.- III.2. Ophthalmoscopic appearance.- III.3. Prognosis.- IV. Differential diagnosis.- IV.1. Diabetic maculopathy.- IV.2. Retinal vein occlusion.- IV.3. Pseudovitelliform macular degeneration.- IV.4. Dominant cystoid macular edema.- IV.5. Other differential diagnosis.- V. Histopathology.- VI. Pathogenesis.- VII. Treatment.- 10. Coats’ Syndrome and Retinitis Pigmentosa.- I. Incidence.- II. Clinical characteristics.- III. Differential diagnosis.- IV. Histopathology.- V. Pathogenesis.- VI. Treatment.- 11. Presumed Acquired Retinal Angiomatosis (Adult Coats’ Disease).- I. Incidence.- II. Clinical aspects.- III. Differential diagnosis.- III.1. Congenital retinal telangiectasis.- III.2. Von Hippel’s disease.- III.3. Peripheral uveitis.- III.4. Malignant melanoma of the choroid.- III.5. Vascular changes in longstanding rhegmatogenous retinal detachment.- IV. Histopathology.- V. Pathogenesis.- VI. Treatment.- 12. Acquired Retinal Macroaneurysms.- I. Incidence.- II. Clinical manifestations.- II.1. Presenting symptoms.- II.2. Ophthalmoscopic findings.- II.3. Fluorescein angiographic findings.- II.4. Classification and evolution.- III. Associated diseases.- III.1 Associated retinal diseases.- III.2. General diseases.- IV. Histopathology.- V. Pathogenesis.- VI. Differential diagnosis.- VI.1. Retinal telangiectasis.- VI.2. Retinal angiomatosis.- VI.3. Retinal cavernous hemangiomas.- VI.4. Congenital vascular anomalies of the optic disc.- VI.5. Disciform macular degeneration.- VI.6. Malignant melanoma.- VI.7. Vitrous hemorrhages from other causes.- VII. Treatment.