Retinal vein occlusion (RVO)

Created 29/04/2022, last revision 06/04/2023

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  • retinal vein occlusion (RVO) is the second most common retinal vascular disease after diabetic retinopathy
  • it may affect the central retinal vein or its branches (CRVO, HCRVO, BRVO)
  • the most common clinical presentation is sudden, unilateral, and painless loss of vision, usually caused by macular edema (with or without concurrent ischemia)
    • branch RVO may present with peripheral visual-field defects or may be asymptomatic
  • prognosis depends on the extent of the initial visual defect
    • better for branch RVO than for central RVO
    • late complications may occur:  vitreous hemorrhage, retinal detachment, or glaucoma
  • risk of recurrence is significant (in the same eye or in the fellow eye)

Etiology and pathophysiology

The three main factors contribute to retinal vein thrombosis:

  • reduced venous outflow with venous stasis
    • increased intraocular pressure in glaucoma may compromise retinal vein outflow
    • atherosclerotic retinal artery may compress the vein
  • endothelial damage
  • hypercoagulable states

Common risk factors:

  • age (usually patients older than 50 years
  • arterial hypertension
  • open-angle glaucoma
  • diabetes mellitus
  • hyperlipidemia

Other associated conditions:

  • smoking
  • optic disc drusen or edema
  • hypercoagulable state
    • polycythemia, multiple myeloma, cryoglobulinemia, Waldenstrom macroglobulinemia, antiphospholipid syndrome, Leiden factor V, APC resistance, hyperhomocysteinemia, protein C and S deficiency, antithrombin III mutation, prothrombin mutation
  • infection
    • syphilis
    • HIV infection
  • sarcoidosis
  • sickle cell disease
  • vasculitis
  • drugs (oral contraceptives, diuretics)
  • abnormal platelet function
  • orbital diseases
  • vein thrombosis provokes increased production of vascular endothelial growth factor (VEGF)
  • this leads to swelling and neovascularization with bleeding
    • treatment involves intravitreal injections of an anti-VEGF drug to reduce the new blood vessel growth and swelling

Clinical presentation

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Diagnostic evaluation

  • extensive laboratory workup is needed to determine the cause
  • standard laboratory tests include:
    • complete blood count (CBC)
    • erythrocyte sedimentation rate (ESR), C-reactive protein (CRP)
    • blood glucose
    • total cholesterol, LDL, and HDL
    • plasma protein electrophoresis (to exclude multiple myeloma)
    • BUN and creatinine
    • electrolytes
    • thyroid function tests
  • extended laboratory tests (patients < 50 years of age, those with bilateral retinal vein occlusion, history of the previous thrombosis, or a family history of thrombosis)
    • chest x-ray
    • thrombophilia screening
    • autoantibodies (rheumatoid factor, antinuclear antibodies, anti-DNA antibodies)
    • serum angiotensin-converting enzyme (ACE) level
    • plasma homocysteine level
  • ECG (left ventricular hypertrophy secondary to hypertension)
  • check blood pressure
  • Duplex carotid ultrasound (exclusion of ocular ischemic syndrome)

Differential diagnosis


Ophthalmologic therapy

  • no fully effective medical treatment is available to prevent or treat central retinal vein occlusion
  • medical treatments (with varying efficiency):
    • antithrombotic therapy
      • antiplatelet therapy (aspirin)
      • anticoagulant therapy
    • anti-inflammatory agents
    • isovolemic hemodilution
    • plasmapheresis
    • fibrinolytic agents
    • systemic corticosteroids
    • intravitreal injection of
      • alteplase
      • ranibizumab
      • triamcinolone
      •  bevacizumab
  • surgical interventions:
    • laser photocoagulation
    • chorioretinal vein anastomosis
    • radial optic neurotomy
    • pars plana vitrectomy

Antithrombotic and thrombolytic therapy

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  • prognosis correlates with initial visual impairment
    • In visual acuity < 20/200, improvement is unlikely
    • If visual acuity is > 20/60, it is likely to remain the same
  • chronic macular edema and macular ischemia are the main causes of poor vision
  • better prognosis can be expected in younger patients and those without ischemia
    • 1/3 of older patients improve without treatment, 1/3 stay the same, and 1/3 get worse
  • complications:
    • iris neovascularization (approx. 60% of cases) ⇒ neovascular glaucoma
    • retinal neovascularization
    • vitreous hemorrhage
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Retinal vein occlusion (RVO)
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