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Central retinal artery occlusion is blockage of the central retinal artery, usually due to an embolism. Its symptom is sudden, painless, unilateral blindness. Diagnosis is by history and characteristic retinal findings on funduscopy. Decreasing intraocular pressure can be attempted within the first 24 h of occlusion. If patients present within the first few hours of occlusion, some centers catheterize the carotid artery and selectively inject thrombolytic drugs.
Etiology
Retinal artery occlusion may be due to embolism or thrombosis.
Emboli may come from any of the following:
Giant cell arteritis (see Vasculitis: Giant Cell Arteritis) is another important cause of arterial occlusion.
Occlusion can affect a branch of the retinal artery as well as the central retinal artery.
Neovascularization (abnormal new vessel formation) of the retina or iris (rubeosis iridis) with secondary (neovascular) glaucoma can occur weeks to months after occlusion. Vitreous hemorrhage may result from retinal neovascularization.
Symptoms and Signs
Retinal artery occlusion causes sudden, painless blindness or visual field defect, usually unilaterally.
The pupil may respond poorly to direct light but constricts briskly when the other eye is illuminated (relative afferent pupillary defect). In acute cases, funduscopy discloses a pale, opaque fundus with a red fovea (cherry-red spot). Typically, the arteries are attenuated and may even appear bloodless. An embolic obstruction is sometimes visible. If a major branch is occluded rather than the entire artery, fundus abnormalities and vision loss are limited to that sector of the retina.
Patients who have giant cell arteritis often have headache, a tender and palpable temporal artery, jaw claudication, fatigue, or a combination.
Diagnosis
The diagnosis is suspected when a patient has acute, painless vision loss. Funduscopy is usually confirmatory. Fluorescein angiography is often done and shows obstruction clearly.
Once the diagnosis is made, carotid Doppler ultrasonography and echocardiography should be done to locate any embolic source so that further embolization can be prevented.
If giant cell arteritis is suspected, ESR, C-reactive protein, and platelet count are done.
Prognosis
Patients with a branch artery occlusion often maintain good to fair vision, but vision loss is often profound with central artery occlusion, even with treatment. Once retinal infarction occurs (possibly in < 2 h, almost always by 24 h), vision loss is permanent.
Treatment
Immediate treatment is indicated if occlusion occurred within 24 h of presentation. Reduction of intraocular pressure by ocular hypotensive drugs (eg, topical timolol 0.5%, acetazolamide 500 mg IV or po), intermittent digital massage over the closed eyelid, or anterior chamber paracentesis may dislodge an embolus and allow it to enter a smaller branch of the artery, thus reducing the area of retinal ischemia. Some centers have tried infusing thrombolytics into the carotid artery to dissolve the obstructing clot. Nonetheless, treatments for retinal artery occlusions rarely improve visual acuity. Surgical or laser-mediated embolectomy is available but not commonly done.
Patients with occlusion secondary to temporal arteritis should receive high-dose systemic corticosteroids.
Last full review/revision December 2008 by Sunir J. Garg, MD, FACS
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