Reversal of paracentral occlusive retinopathy in a case of sickle cell disease using exchange transfusion.

ABSTRACT

Paracentral occlusive retinopathy is an uncommon manifestation of sickle cell disease. If macular ischemia is not reversed, permanent vision loss can result. The authors report the successful use of exchange transfusion to treat unilateral paracentral occlusive retinopathy secondary to sickle cell disease in a 23-year-old man with hemoglobin SS disease. Initial presentation demonstrated arteriolar occlusion, perivenous hemorrhages, vessel tortuosity, and areas of retinal ischemia. Visual acuity was count fingers, and the patient noted a paracentral scotoma. Following transfusion, there was restoration of arteriolar flow as documented with fluorescein angiogram, and visual acuity returned to 20/20.