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Key Features

Essentials of Diagnosis

  • Sudden monocular loss of vision

  • No pain or redness

  • Widespread or sectoral retinal pallid swelling

General Considerations

  • In patients aged 50 years or older with central retinal artery occlusion, consider giant cell arteritis

  • 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)

  • Internal carotid artery dissection should be considered 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 is 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 visual field is the presenting complaint

    • Fundal 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

  • 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


Laboratory Tests

  • Test for diabetes (fasting serum glucose, hemoglobin A1C) and hyperlipidemia (fasting serum cholesterol, HDL cholesterol, LDL 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)

  • In younger patients, consider tests for congenital or acquired hypercoagulable states (thrombophilia)

    • Antiphospholipid antibody syndrome (lupus anticoagulant)

    • Activated protein C resistance/Factor V Leiden

    • Protein C, protein S deficiency

    • Antithrombin deficiency

    • Hyperprothrombinemia (G20210A prothrombin gene mutation)

    • Increased Factor VIII activity

    • Hyperhomocysteinemia

Imaging Studies

  • Obtain duplex ultrasonography of the carotid arteries, ECG, and echocardiography (with transesophageal echocardiography, if necessary), to identify carotid and cardiac sources of emboli

  • When indicated, obtain CT or MR angiography studies for internal carotid artery dissection

Diagnostic Studies

  • Central artery occlusion: Ophthalmoscopy

    • Reveals pallid swelling of the retina with a cherry-red spot at the fovea

    • The retinal arteries are attenuated, and "box-car" segmentation of blood may be seen in the retinal veins

    • Occasionally, emboli are seen in the central retinal artery or its branches

  • Optical ...

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