Sickle Cell Retinopathy: Goldberg Stages and Management

Last updated: November 2026

Sickle cell disease causes peripheral retinal ischaemia and proliferative retinopathy. It matters in India (central and western tribal belts, including parts of Gujarat) and in patients of African origin. HbSC disease causes more retinopathy than HbSS.

Signs

• Non-proliferative: salmon patch haemorrhages (fresh intraretinal), iridescent spots (resolved haemorrhage), black sunburst lesions (RPE hyperplasia), comma-shaped conjunctival vessels, angioid streaks.
• Proliferative: peripheral arteriolar occlusion, arteriovenous anastomoses, then sea-fan neovascularisation.

Goldberg staging of proliferative sickle retinopathy

Management

• Screen patients with HbSC and HbSS from around age 10 (yearly dilated examination, widefield imaging where available).
• Many sea fans auto-infarct; laser (scatter photocoagulation to the ischaemic periphery) for growing or bleeding neovascularisation; vitrectomy for non-clearing haemorrhage or detachment.
• Hyphaema in sickle trait or disease: sickled cells block the trabecular meshwork and the optic nerve is very vulnerable, so treat even modest IOP rises aggressively; avoid carbonic anhydrase inhibitors (acetazolamide) and osmotic agents where possible.

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Related: Traumatic Hyphema: Current Management, Diabetic Retinopathy: Classification and Management for Residents, Retinal Vein Occlusion: BRVO and CRVO Made Practical.

Key sources

• Goldberg MF. Classification and pathogenesis of proliferative sickle retinopathy. Am J Ophthalmol. 1971;71:649-665
• Elagouz M, et al. Sickle cell disease and the eye: old and new concepts. Surv Ophthalmol. 2010;55:359-377