Retinal Vein Occlusion

BRANCH RETINAL VEIN OCCLUSION:

Main risk factors:

  • HTN

  • Hyperlipidaemia

  • DM

  • OCP

  • Raised IOP

  • Smoking


What to consider in under 50?

  • Myeloproliferative disease – polycythaemia, abnromal plasma protein (myeloma)

  • Acquired hypercoagulable states – raised homocysteine levels, lupus anticoagulant, antiphospholipid antibodies

  • Inhired hypercoagulable states – Factor V Leiden mutation, protein C or S deficiency, antithrombin deficiency, Factor VII deficiency

  • Inflammation – Behcet syndrome, sarcoidosis, Wegener’s granulomatosis, Goodpasture syndrome

  • Others – CRF, secondary HTN (Cushing syndrome), secondary hyperlipidaemia (hypothyroidism), orbital disease, dehydration.

Main investigations:

  • BP

  • FBC / U+E / ESR / PV / TFT / glucose / cholesterol

  • Plasma protein electrophoresis

  • ECG

  • Other test to consider:

  • CXR

  • CRP

  • Thrombophilia screen – thrombin time, prothrombin time, activated partial thromboplastin time, protein C & S, factor V Leiden mutation, prothrombin mutation, anticardiolipin antibody (IgG and IgM), lupus anticoagulant

  • Autoantibodies – rheumatoid factor, ANA, anti-DNA antibody

  • ACE

  • Syphilis serology

  • Carotid dopplers

Clinical presentation:

  • Variable VA presentation.

  • Flame-shaped +/- dot blot haemorrhages with retinal oedema and cotton wool spots

Investigations: OCT, FFA, OCTA

Long-term prognosis:

  • 6 months – 50% VA 6/12 or better.

  • 25% less than 6/60 vision.

Complications of RVO:

  • Chronic macular oedema

  • Macular ischaemia

  • Neovascularisation – 40% of eyes > 5 disc diameter of non-perfusion.


Treatment:

  • Medical treatment – treat systemic risk factor or hypercoagulable state (anticoagulant)

  • Laser treatment – BVOS: macular laser after 3-6 months observation, good macular perfusion and vision 6/12 or worse. PRP if signs of neovascularisation.

  • Steroid treatment – SCORE: only use IVTA in refractory and pseudophakic cases; GENEVA: Ozurdex for macular oedema (last 3 months)

  • Anti-VEGF treatment – BRVO (ranibizumab) study – ranibizumab is superior to laser for macular oedema ; BRIGHTER study – ranibizumab + laser made no difference to macular oedema ; BERVOLT study – bevacizumab is safe for macular oedema associated BRVO ; VIBRANT study – aflibercept is superior to laser for macular oedema

  • Surgical treatment – PPV for BRVO related vitreous haemorrhage or tractional retinal detachment.

Pathway for treatment:

  • Observe for 2-3 months for VA progression and neovascularisation

  • Macular oedema present – anti VEGF for first 3 months then see response.

  • If not improving macular oedema – consider steroid treatment

  • If still not response – consider grid macular laser.

  • If signs of neovascularisation – PRP

CENTRAL RETINAL VEIN OCCLUSION:

  • Demographics: Usually unilateral; M=F; >65 years old; Annual risk of 1% in fellow eye.

Clinical features:

  • Sudden painless loss of vision

  • Check for NVI and NVA (NVA appears in 12% on first presentation)

  • Raised IOP – risk NVG – ‘100 day glaucoma – neovascularisation within 3 months of CRVO‘

  • RAPD

  • Flame shaped & Dot/blot haemorrhages in all 4 quadrants of retina

  • Other changes in retina – macular oedema, cotton wool spots, optic disc oedema, vitreous haemorrhage or NVD/NVE.

  • Chronic changes (6-12 months) – collaterals, venous sheathing & sclerosis at site of obstruction, RPE changes or ERM.

Investigations: FFA; OCT

Central retinal vein occlusion study (CVOS):

  • Visual acuity 6/12 or better – maintained their vision.

  • Poor visual acuity presentation (6/60 or worse) – 20% improvement chance.

  • NVA without NVI in 12% of cases.

  • Perfused (non-ischaemic) 80% cases vs. Non-perfused (ischaemic) 20% cases.


Treatment:

  • Primary treatment – Systemic treatment (BP, DM); hypercoagulable states.

  • Macular oedema treatment – observation (first few weeks); SCORE study (IVTA useful – this study led to to the use of Ozurdex); CRUISE study (ranibizumab use – RETAIN study shows ranibizumab use maintain condition in < 1/2 patients at 4 years); COPERNICUS & GALILEO study (aflibercept use maintain vision long-term when used early); Bevacizumab (off license use)

  • Neovascularisation treatment: PRP for signs of NVI/NVA/NVD/NVE; topical/systemic anti-glaucoma agents; cycloplegic agents; surgery if IOP uncontrolled; anti-VEGF prior to PRP as adjunct (note: PRP best perform within a week after anti-VEGF).

  • Non-clearing vitreous haemorrhage: PPV + endolaser PRP.

 
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