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 Vein Occlusions + Key Features Download Section PDF Listen +++ +++ 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 Download Section PDF Listen +++ +++ 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 Download Section PDF Listen +++ ++ Check blood pressure Ask about tobacco use Ask women about estrogen therapy (including oral contraceptives) Patients should also be asked about a history of glaucoma; intraocular pressure should be measured to assess for signs of open- or narrow-angle glaucoma +++ 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 Download Section PDF Listen +++ +++ Therapeutic Procedures ++ Panretinal laser photocoagulation (prophylactically or as soon as there is evidence of neovascularization) can be 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], bevacizumab [Avastin], or aflibercept [VEGF Trap-Eye, Eylea]) is beneficial in patients with macular edema due to either branch or central retinal vein occlusion Intravitreal triamcinolone improves vision in chronic macular edema due to nonischemic central retinal vein occlusion Intravitreal implant containing dexamethasone Beneficial in both central and branch retinal vein occlusion However, carries the risk of glaucoma in 20–65% of patients Causes cataract in all patients who have not already had cataract surgery + Outcome Download Section PDF Listen +++ +++ Complications ++ Risk of neovascular (rubeotic) glaucoma is high if central retinal vein occlusion is associated with Widespread retinal ischemia, manifesting as poor visual acuity (20/200 [6/60] or worse) Florid retinal abnormalities Extensive areas of capillary closure on fluorescein angiography Branch retinal vein occlusion may be complicated by peripheral retinal neovascularization or chronic macular edema +++ Prognosis ++ In central retinal vein occlusion, severity of visual loss is a good guide to visual outcome Initial visual acuity of 20/60 (6/18) or better indicates a good prognosis Visual prognosis is poor for eyes with neovascular glaucoma In branch retinal vein occlusion, visual outcome is determined by the severity of glaucoma and macular damage from hemorrhage, ischemia, or edema +++ When to Refer ++ All patients with retinal vein occlusion should be referred urgently to an ophthalmologist + References Download Section PDF Listen +++ + +Bucciarelli P et al. Thrombophilic and cardiovascular risk factors for retinal vein occlusion. Eur J Intern Med. 2017 Oct;44:44–8. [PubMed: 28684050] + +Ehlers JP et al. Therapies for macular edema associated with branch retinal vein occlusion: a report by the American Academy of Ophthalmology. Ophthalmology. 2017 Sep;124(9):1412–23. [PubMed: 28551163] + +Hykin P et al; LEAVO Study Group. Clinical effectiveness of intravitreal therapy with ranibizumab vs aflibercept vs bevacizumab for macular edema secondary to central retinal vein occlusion: a randomized clinical trial. JAMA Ophthalmol. 2019 Aug 29;137(11):1256–64. [PubMed: 31465100] + +Li J et al. New developments in the classification, pathogenesis, risk factors, natural history, and treatment of branch retinal vein occlusion. J Ophthalmol. 2017;2017:4936924. [PubMed: 28386476] + +Pesin N et al. The role of occult hypertension in retinal vein occlusions and diabetic retinopathy. Can J Ophthalmol. 2017 Apr;52(2):225–8. [PubMed: 28457296] + +Pulido JS et al. Retinal vein occlusions Preferred Practice Pattern(®) Guidelines. Ophthalmology. 2016 Jan;123(1):P182–208. [PubMed: 26581559] + +Sinawat S et al. Systemic abnormalities associated with retinal vein occlusion in young patients. Clin Ophthalmol. 2017 Feb 23;11:441–7. [PubMed: 28260858] + +Wittström E. Central retinal vein occlusion in younger Swedish adults: case reports and review of the literature. Open Ophthalmol J. 2017 May 22;11:89–102. [PubMed: 28603574]