Retinal Vein Occlusion Definition: Vein occlusion occurs as a result of circulatory dysfunction in the central vein or one of its

branches. Epidemiology. Retinal vein occlusion is the second most frequent vascular retinal disorder after diabetic retinopathy. The most frequent underlying systemic disorders are arterial hypertension and diabetes mellitus; the most frequent underlying ocular disorder is glaucoma. Frequent underlying systemic disorders of retinal vein occlusion include arterial hypertension and diabetes mellitus. Frequent underlying ocular disorders include glaucoma and retinal vasculitis. Etiology. Occlusion of the central vein of the retina or its branches is frequently due to local thrombosis at sites where sclerotic arteries compress the veins. In central retinal vein occlusion, the thrombus lies at the level of the lamina cribrosa; in branch retinal vein occlusion, it is frequently at an arteriovenous crossing. Symptoms. Patients only notice a loss of visual acuity if the macula or optic disc are involved. Diagnostic considerations and findings. Central retinal vein occlusion can be diagnosed where linear or punctiform hemorrhages are seen to occur in all four quadrants of the retina (Fig. 12.20a). Often one will find distended and increasingly meandering veins. In branch retinal vein occlusion, intraretinal hemorrhages will occur in the area of vascular supply; this bleeding may occur in only one quadrant or in two quadrants (hemispheric vein occlusion) (Fig. 12.20b). Cotton-wool spots and retinal or optic-disc edema may also be present (simultaneous retinal and optic-disc edema is also possible). Chronic occlusions may also be accompanied by lipid deposits. One differentiates between nonischemic and ischemic occlusion depending on the extent of capillary occlusion. Ischemic occlusion is diagnosed with the aid of fluorescein angiography (central retinal vein occlusion_10 disc areas of nonperfusion, branch retinal vein occlusion_5 disc areas of nonperfusion). Differential diagnosis. Other forms of vascular retinal disease have to be excluded, especially diabetic retinopathy. An internist should be consulted to verify or exclude the possible presence of an underlying disorder. Treatment. In the acute stage of vein occlusion, hematocrit should be reduced to 3538% by hemodilution. The underlying disorders have to be treated. The patients should have follow-up examinations every 4weeks for 6 months, and thereafter every 3 months. In central retinal vein occlusion, iris neovascularization most often develops within 3 months. Laser treatment is performed in ischemic occlusion that progresses to neovascularization or rubeosis iridis. Focal laser treatment is performed in branch retinal vein occlusion with macular edema when visual acuity is reduced to 20/40 or less within 3 months of occlusion. Prophylaxis. Early diagnosis and prompt treatment of underlying systemic and ocular disorders is important. Clinical course and prognosis. Visual acuity improves in approximately onethird of all patients, remains unchanged in one-third, and deteriorates in onethird despite therapy. Complications include preretinal neovascularization, retinal detachment, and rubeosis iridis with angle closure glaucoma. Retinal Arterial Occlusion Definition: Retinal infarction due to occlusion of an artery in the lamina cribrosa or a branch retinal artery occlusion. Epidemiology. Retinal artery occlusions occur significantly less often than vein occlusions. Etiology. Emboli (Table 12.4) are frequently the cause of central retinal artery and branch retinal artery occlusions. Less frequent causes include inflammatory processes such as temporal arteritis (Hortons arteritis). Hortons arteritis should be excluded where retinal artery occlusion is accompanied by headache. Symptoms. In central retinal artery occlusion, the patient generally reports sudden, painless unilateral blindness. In branch retinal artery occlusion, the patient will notice a loss of visual acuity or visual field defects. Diagnostic considerations. The diagnosis is made by ophthalmoscopy. In the acute stage of central retinal artery occlusion, the retina appears grayishwhite due to edema of the layer of optic nerve fibers and is no longer transparent. Only the fovea centralis, which does not contain any nerve fibers, remains visible as a cherry-red spot because the red of the choroid shows through at this site (Fig. 12.21 a). The column of blood will be seen to be interrupted. Rarely, one will observe an embolus. Patients with a cilioretinal artery (artery originating from the ciliary arteries instead of the central retinal artery) will exhibit normal perfusion in the area of vascular supply, and their loss of visual acuity will be less (Fig. 12.21b). An afferent pupillary defect is found. Perimetry reveals a severe defect in the visual field. Atrophy of the optic nerve will develop in the chronic stage of central retinal artery occlusion. In the acute stage of central retinal artery occlusion, the fovea centralis appears as a cherry-red spot on ophthalmoscopy. There is no edema of the layer of optic nerve fibers in this area because the fovea contains no nerve fibers. In branch retinal artery occlusion, a retinal edema will be found in the affected area of vascular supply (Fig. 12.21c) in the acute stage.

Perimetry (visual field testing) will reveal a total visual field defect in central retinal artery occlusion and a partial defect in branch occlusion corresponding to the area of occlusion. In the chronic stage the vessel is either narrowed or occluded. Differential diagnosis. Lipid-storage diseases that can also create a cherry-red spot, such as TaySachs disease, NiemannPick disease, or Gaucher disease, should be excluded. These diseases can be clearly identified on the basis of their numerous additional symptoms and the fact that they afflict younger patients. Treatment. Emergency treatment is often unsuccessful even when initiated immediately. Ocular massage, medications that reduce intraocular pressure, or paracentesis are applied in an attempt to drain the embolus in a peripheral retinal vessel. Calcium antagonists or hemodilution are applied in an attempt to improve vascular supply. Systemic lysis therapy is no longer performed due to the poor prognosis (it is not able to prevent blindness) and the risk to vital tissue involved. Local lysis is being investigated. Work-up to identify the source of the embolus is important in order to treat the underlying disease and prevent another embolization (such as stroke). Prophylaxis. Excluding or initiating prompt therapy of predisposing underlying systemic disorders is crucial Clinical course and prognosis. The prognosis is poor because irreparable damage to the inner layers of the retina occurs within 1 hour. Blindness usually cannot be prevented in central retinal artery occlusion. The prognosis is better when only a branch of the artery is occluded, unless a macular branch is affected.