Bilateral macular infaction in SS disease.

A 10-year-old Afro-American female with SS disease presented with a history of painless loss of vision (RE) of 26 hours duration. Sequential fluorescein angiography revealed bilateral parafoveal occlusions and evidence of prior vaso-occlusions in each eye. Initial hydration therapy with dextrose 5% and 1/4 normal saline was followed within eight hours by transfusion with two units of packed red blood cells. Patency was restored to the occluded arterioles with return of normal acuity in LE, though the vision remains at hand motions in the RE. Sudden loss of central acuity in sickle hemoglobinopathies may result from vaso-occlusion in terminal arterioles near the foveal avascular zone. Therefore a reduced number of permanently irreversibly sickled red blood cells may prevent these macular infarcts.