Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 7-18: Central & Branch Retinal Artery Occlusions + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Sudden monocular loss of vision No pain or redness Widespread or sectoral pale retinal 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) 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 Giant cell arteritis should be considered in cases of central retinal artery occlusion without visible emboli + Clinical Findings Download Section PDF Listen +++ +++ 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 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 Download Section PDF Listen +++ +++ 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 pale 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 coherence tomography The retinal swelling subsides over a period of 4–6 weeks, leaving a pale optic disk with thinning of the inner retina + Treatment Download Section PDF Listen +++ ++ If the patient is seen within a few hours after onset, emergency treatment may influence visual outcomes and includes Laying the patient flat Ocular massage High concentrations of inhaled oxygen Intravenous acetazolamide Anterior chamber paracentesis In central retinal artery occlusion not due to giant cell arteritis, early thrombolysis, particularly by local intra-arterial injection but also intravenously, may be beneficial Recommended initial empiric treatment for giant cell arteritis Perform a temporal artery biopsy promptly Institute a high-dose corticosteroid regimen immediately (possibly with low-dose aspirin (~81 mg/day orally) Intravenous methylprednisolone 0.5–1 g/day for 1–3 days All patients require subsequent long-term glucocorticoid therapy (eg, oral prednisone 1–1.5 mg/kg/day) and possibly low-dose aspirin (~81 mg/day orally) Whether oral methylprednisolone is similarly effective is unknown Monitor the patient closely to ensure that symptoms resolve and do not recur Carotid endarterectomy or angioplasty with stenting should be considered in patients in whom the following are present: Embolic retinal artery occlusion Ipsilateral carotid artery stenosis (70–99%) Possibly, ipsilateral carotid artery stenosis (50–69%) Surgical treatment of cardiac valvular disease and patent foramen ovale may be required + Outcome Download Section PDF Listen +++ +++ Prognosis ++ In giant cell arteritis, there is risk of involvement of the other eye without prompt treatment +++ When to Refer ++ Patients with retinal artery occlusions should be referred urgently to an emergency department to evaluate the patient for stroke manifestations and treatment Patients with central retinal artery occlusion should be referred emergently to an ophthalmologist Patients with branch retinal artery occlusion should be referred urgently to an ophthalmologist +++ When to Admit ++ Patients with visual loss due to giant cell arteritis may require emergency admission for high-dose corticosteroid therapy and close monitoring to ensure that treatment is adequate + References Download Section PDF Listen +++ + +Abel AS et al. Practice patterns after acute embolic retinal artery occlusion. Asia Pac J Ophthalmol (Phila). 2017 Jan–Feb;6(1):37–9. [PubMed: 28161924] + +Dumitrascu OM et al. Is intravenous thrombolysis safe and effective in central retinal artery occlusion? A critically appraised topic. Neurologist. 2017 Jul;22(4):153–6. [PubMed: 28644261] + +Hayreh SS et al. Ocular arterial occlusive disorders and carotid artery disease. Ophthalmol Retina. 2017 Jan–Feb;1(1):12–8. [PubMed: 28547004] + +Hocevar A et al. Do early diagnosis and glucocorticoid treatment decrease the risk of permanent visual loss and early relapses in giant cell arteritis: A prospective longitudinal study. Medicine (Baltimore). 2016 Apr;95(14):e3210. [PubMed: 27057850] + +Hong JH et al. Retinal artery occlusion and associated recurrent vascular risk with underlying etiologies. PLoS One. 2017 Jun 1;12(6):e0177663. [PubMed: 28570629] + +Mehta N et al. Central retinal artery occlusion: acute management and treatment. Curr Ophthalmol Rep. 2017 Jun;5(2):149–59. [PubMed: 29051845] + +Olsen TW et al. Retinal and ophthalmic artery occlusions Preferred Practice Pattern®. Ophthalmology. 2017 Feb;124(2):P120–43. [PubMed: 27742458] + +Préterre C et al. Management of acute central retinal artery occlusion: intravenous thrombolysis is feasible and safe. Int J Stroke. 2017 Oct;12(7):720–3. [PubMed: 28067616] + +Soriano A et al. Visual loss and other cranial ischaemic complications in giant cell arteritis. Nat Rev Rheumatol. 2017 Aug;13(8):476–84. [PubMed: 28680132] + +Vodopivec I et al. Management of transient monocular vision loss and retinal artery occlusions. Semin Ophthalmol. 2017;32(1):125–33. [PubMed: 27780399] + +Wu X et al. Oxygen therapy in patients with retinal artery occlusion: a meta-analysis. PLoS One 2018 Aug 29;13(8):e0202154. [PubMed: 30157206]