- May present as nonproliferative or proliferative retinopathy.
- Among other systemic hemoglobinopathies, sickle cell disease constitutes the more common cause of peripheral proliferative retinopathy.
- Asymptomatic in the early stage of the disease
- Decreased vision secondary to vitreous hemorrhage, macular edema or capillary nonperfusion involving the macula
- 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
- 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.