Central Retinal Artery Occlusion and Branch Retinal Artery Occlusion

Retinal artery occlusion may be due to embolism or thrombosis.

Emboli may come from any of the following:

Thrombosis is a less common cause of retinal artery occlusion but can be seen with systemic vasculitis such as systemic lupus erythematosus (SLE) and giant cell arteritis Giant Cell Arteritis Giant cell arteritis involves predominantly the thoracic aorta, large arteries emerging from the aorta in the neck, and extracranial branches of the carotid arteries. Symptoms of polymyalgia... read more , which is an important cause of arterial occlusion that requires prompt diagnosis and treatment.

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 occurs in about 20% of patients within weeks to months after occlusion. Vitreous hemorrhage may result from retinal neovascularization.

Risk of stroke is increased after retinal artery occlusion, particularly in the first weeks.

Retinal artery occlusion causes sudden, painless, severe vision loss 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 shows a pale, opaque fundus with a red fovea (cherry-red spot). Typically, the arteries are attenuated and may even appear bloodless. An embolus (eg, a cholesterol embolus, called a Hollenhorst plaque) 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 are 55 or older and may have a headache, a tender and palpable temporal artery, jaw claudication, fatigue, or a combination.

The diagnosis is suspected when a patient has acute, painless, severe vision loss. Funduscopy is usually confirmatory. Fluorescein angiography is often done and shows absence of perfusion in the affected artery. However, if patients present with acute transient monocular vision loss thought to be due to a thromboembolic event, imaging tests take unnecessary time and should be deferred if the diagnosis is clear.

Once the diagnosis is made, carotid Doppler ultrasonography and echocardiography should be done to identify an embolic source so that further embolization can be prevented.

If giant cell arteritis Giant Cell Arteritis Giant cell arteritis involves predominantly the thoracic aorta, large arteries emerging from the aorta in the neck, and extracranial branches of the carotid arteries. Symptoms of polymyalgia... read more is suspected, erythrocyte sedimentation rate (ESR), C-reactive protein, and platelet count should be done immediately. These tests may not be necessary if an embolic plaque is visible in the central retinal artery.

Because risk of stroke is increased, some centers rapidly evaluate patients similarly to those who have had stroke or transient ischemic attack.

Patients with a branch artery occlusion may maintain good to fair vision, but with central artery occlusion, vision loss is often profound, even with treatment. Once retinal infarction occurs (as quickly as 90 minutes after the occlusion), vision loss is permanent.

If underlying giant cell arteritis is diagnosed and treated promptly, the vision in the uninvolved eye can often be protected and some vision may be recovered in the affected eye.

If acute thromboembolism is suspected, the patient should be referred immediately to a specialized stroke center because there is an increased risk of other cerebrovascular events.

Immediate treatment is indicated if occlusion occurred within 24 hours of presentation. Ocular massage with intermittent pressure on the closed eye or reduction of intraocular pressure with ocular hypotensive drugs (eg, topical timolol 0.5%, acetazolamide 500 mg IV or orally) may dislodge an embolus and allow it to enter a smaller branch of the artery, thus reducing the area of retinal ischemia. Anterior chamber paracentesis is sometimes used to reduce intraocular pressure and increase perfusion. Some centers have tried infusing thrombolytics into the carotid artery to dissolve the obstructing clot. Multiple case series have suggested hyperbaric oxygen may improve visual outcome in central retinal artery occlusion. Nonetheless, treatments for retinal artery occlusions rarely improve visual acuity. Surgical or laser-mediated embolectomy is available but not commonly done. These treatments are sometimes shown to be effective in small case series, but none have strong evidence to support efficacy.

Patients with occlusion secondary to giant cell arteritis should receive high-dose systemic corticosteroids.