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Key Features

Essentials of Diagnosis

  • Sudden monocular loss of vision

  • No pain or redness

  • Widespread or sectoral retinal hemorrhages

General Considerations

  • Common causes of acute vision loss

  • Branch retinal vein occlusion is four times more common than central retinal vein occlusion

  • The major predisposing factors are the etiologic factors in arteriosclerosis (diabetes mellitus, systemic hypertension, hyperlipidemia) and glaucoma

  • In younger patients, consider estrogen therapy (including oral contraceptives), and inherited or acquired hypercoagulable states (thrombophilia) (see below)

  • In bilateral retinal vein occlusions, consider hyperviscosity syndromes related to myeloproliferative disorders(eg, Waldenström macroglobulinemia)(rare)

Clinical Findings

Symptoms and Signs

  • Central vein occlusion

    • Ophthalmoscopic signs include

      • Widespread retinal hemorrhages

      • Retinal venous dilation and tortuosity

      • Retinal cotton-wool spots

      • Optic disk swelling

    • Branch retinal vein occlusions may present in a variety of ways

      • Sudden loss of vision may occur at the time of occlusion if the fovea is involved or some time afterward from vitreous hemorrhage due to retinal new vessels (neovascularization)

      • More gradual visual loss may occur with development of macular edema

      • In acute cases, the retinal abnormalities (hemorrhages, venous dilation and tortuosity, and cotton-wool spots) are confined to the area drained by the obstructed vein

Diagnosis

  • Check blood pressure

  • Ask about tobacco use

  • Ask women about estrogen therapy (including oralcontraceptives)

Laboratory Tests

  • Obtain screening studies for diabetes mellitus, hyperlipidemia, and hyperviscosity

  • 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

Treatment

Therapeutic Procedures

  • Panretinal laser photocoagulation (prophylactically or as soon as there is evidence of neovascularization) can used to treat eyes at risk for neovascular glaucoma following ischemic central retinal vein occlusion

  • Intravitreal injections of bevacizumab, an inhibitor of vascular endothelial growth factor (VEGF), achieve regression of iris neovascularization

  • Retinal laser photocoagulation

    • May be indicated in chronic macular edema due to branch but not central retinal vein occlusion

    • Should be done when branch retinal vein occlusion is complicated by retinal neovascularization

  • Intravitreal injection of a vascular endothelial growth factor (VEGF) inhibitor(such as ranibizumab [Lucentis], pegaptanib [Macugen], bevacizumab [Avastin], or aflibercept [VEGF Trap-Eye, Eylea])is beneficial in chronic macular edema due to either branch or nonischemic central retinal vein occlusion

  • Intravitreal triamcinolone improves vision in chronic macular edema due to nonischemic central retinal vein occlusion

  • Intravitreal implant containing dexamethasone is beneficial in both central and branch retinal vein occlusion

  • Improvement in vision has also been reported after

    • Vitrectomy with direct injection of tissue plasminogen activator into the retinal venous system or incision of the sclera at the edge of the optic disk (radial optic neurotomy)

    • Isovolemic hemodilution in central retinal vein occlusion

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