OTHER VASCULAR DISORDERS / OCULAR VASCULAR DISORDERS

Retinal vein occlusion

Created 29/04/2022, last revision 01/05/2022

Content available only for logged-in subscribers (registration will be available soon)
  • 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, generally caused by macular edema (with or without concurrent ischemia)
    • branch RVO may present with peripheral visual-field defects or may be asymptomatic
  • prognosis is related to the extent of the initial visual defect
    • better for branch RVO than for central RVO
    • late complications may appear:  vitreous hemorrhages, retinal detachment, or glaucoma
  • the risk of recurrence is significant (in the same or 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 can compromise retinal vein outflow
    • the atherosclerotic retinal artery may compress the vein
  • endothelial damage
  • hypercoagulable states

Common risk factors:

  • age (usually patients older than 50 years of age
  • 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
  • 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

Content available only for logged-in subscribers (registration will be available soon)

Diagnostic evaluation

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

Differential diagnosis

Management

Ophthalmologic therapy

  • no fully effective medical treatment is available for the prevention or treatment of 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 venous anastomosis
    • radial optic neurotomy
    • pars plana vitrectomy

Antithrombotic and thrombolytic therapy

Content available only for logged-in subscribers (registration will be available soon)

Prognosis

  • prognosis correlates with initial visual impairment
    • In visual acuity < 20/200, improvement is improbable
    • 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, one-third stay the same, and one-third get worse
  • complications:
    • iris neovascularization (approx. 60% of cases) ⇒ neovascular glaucoma
    • retinal neovascularization
    • vitreous hemorrhage
icon-angle icon-bars icon-times