Sickle Cell Proliferative Retinopathy

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.