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For further information, see CMDT Part 7-18: Central & Branch Retinal Artery Occlusions

KEY FEATURES

Essentials of Diagnosis

  • Sudden monocular loss of vision

  • No pain or redness

  • Widespread or sectoral pale retinal swelling

General Considerations

  • Acute retinal arterial ischemia, including central and branch retinal artery occlusion, is a true ocular and medical emergency

  • Consider giant cell arteritis in patients with central retinal artery occlusion and either

    • Age ≥ 50 years

    • No visible emboli

  • Carotid and cardiac sources of emboli must be sought so that treatment can be given to reduce the risk of stroke

  • In young patients, causes include

    • Migraine

    • Oral contraceptives

    • Systemic vasculitis

    • Congenital or acquired hypercoagulable states (thrombophilia) (see below)

    • Hyperhomocysteinemia

  • Consider internal carotid artery dissection when there is neck pain or a recent history of neck trauma

  • In all patients, consider

    • Diabetes mellitus

    • Hyperlipidemia

    • Systemic hypertension

CLINICAL FINDINGS

Symptoms and Signs

  • Central retinal artery occlusion

    • Presents as sudden profound monocular visual loss

    • Visual acuity is usually reduced to counting fingers or worse

    • Visual field may be restricted to an island of vision in the temporal field

  • Branch retinal artery occlusion

    • May also present with sudden loss of vision if the fovea is involved, but more commonly sudden loss of a discrete area in visual field in one eye is the presenting complaint

    • Fundus signs of retinal swelling and adjacent cotton-wool spots are limited to the area of retina supplied by the occluded artery

  • Identify risk factors for a cardiac source of emboli, including arrhythmia, particularly atrial fibrillation, and cardiac valvular disease; and check the blood pressure

  • Non-ocular clinical features of giant cell arteritis include

    • Jaw claudication (which is very specific)

    • Headache

    • Scalp tenderness

    • General malaise

    • Weight loss

    • Polymyalgia rheumatica: shoulder and hip girdle pain

    • Tenderness or thickening of, or absence of pulse in, the superficial temporal arteries

DIAGNOSIS

Laboratory Tests

  • Test for diabetes (fasting serum glucose, hemoglobin A1C) and hyperlipidemia (fasting serum cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, triglycerides) in all patients

  • Erythrocyte sedimentation rate and C-reactive protein are usually markedly elevated in giant cell arteritis but one or both may be normal

  • Consider testing for other types of vasculitis (eg, antinuclear antibody, rheumatoid factor, anticytoplasmic neutrophil antibody) (Table 22–11)

  • Particularly in younger patients, consider tests for

    • Antiphospholipid antibodies

    • Lupus anticoagulant

    • Inherited thrombophilia

    • Elevated plasma hyperhomocysteine

Table 22–11.Classification scheme of primary vasculitides according to size of predominant blood vessels involved.

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