Sickle Cell Retinopathy

Sickle cell retinopathy is retinal vascular disease from sickling in retinal vessels.

• More frequent proliferative disease in HbSC and sickle thalassaemia (up to 40%) than HbSS (about 10%).
• Nonproliferative: salmon patch haemorrhages, iridescent spots, black sunbursts, venous tortuosity, vascular occlusion.
• Proliferative (Goldberg stages I to V): peripheral arteriolar occlusion, arteriovenous anastomoses, sea fan neovascularisation, vitreous haemorrhage, tractional retinal detachment.
• Onset in second decade.

• Asymptomatic in early disease.
• Floaters, sudden vision loss from vitreous haemorrhage, field defects.
• Hyphaema with acute IOP rise in sickle patients.

• Dilated fundus examination annually from age 10; widefield imaging and OCT angiography.
• Fluorescein angiography (caution) to delineate peripheral nonperfusion and neovascularisation.
• OCT for macular thinning.

• Nonproliferative: observation and annual review.
• Proliferative: scatter laser photocoagulation to ischaemic peripheral retina, anti VEGF injection (bevacizumab 1.25 mg, aflibercept 2 mg) adjunct for sea fans and vitreous haemorrhage.
• Vitrectomy for non clearing vitreous haemorrhage, tractional detachment; careful anaesthesia (maintain oxygenation, avoid hypovolaemia), consider transfusion preoperatively.
• Hyphaema: carbonic anhydrase inhibitors avoided (acidosis promotes sickling); use topical beta blockers; early washout for IOP above 24 mmHg.
• Hydroxyurea and systemic management; avoid gas tamponade pressure rises.
• Encourage regular screening.