Stage 3 sickle cell peripheral proliferative retinopathy with a "seafan" neovascularization and fibrovascular proliferation. Fluorescein angiogram demonstrating arteriolar-venular anastomoses at the junction of perfused and nonperfused retina and dye leakage.

May present as nonproliferative or proliferative retinopathy. Among other systemic hemoglobinopathies, sickle cell disease constitutes the more common cause of peripheral proliferative retinopathy. Clinical features: Symptoms: Asymptomatic in the early stage of the disease Decreased vision secondary to vitreous hemorrhage, macular edema or capillary nonperfusion involving the macula Signs: Nonproliferative Retinopahty; the following characteristics can be observed: comma-shaped vaso-occlusions of conjunctival vasculature, iris atrophy, "salmon patch" retinal hemorrhage, "black sunburst" spots, and various abnormalities of the retinal vasculature, macula, choroid, optic disc, and vitreoretinal interface Proliferative Retinopathy has been classified into 5 stages by Goldberg; Stage 1: Peripheral arteriolar occlusion or capillary nonperfusion Stage 2: Vascular remodeling at the boundary between perfused and nonperfused peripheral retina Stage 3: "Seafan" peripheral retinal neovascularization Stage 4: Vitreous hemorrhage Stage 5: Vitreous band, complicated with rhegmatogenous and tractional retinal detachment Management: Treatment of systemic condition. Indication for ocular treatment: bilateral, progressive proliferative disease, rapid neovascularization and spontaneous hemorrhages. Ocular treatment may include; laser photocoagulation, pars plana vitrectomy, and scleral buckling.

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