Sickle cell disease presenting with extensive peri-macular arteriolar occlusions in a nine-year-old boy

PURPOSE: To report a dramatic occlusive event of the macula surrounding the foveal avascular zone, causing severe and permanent loss of vision in a ch ild with sickle cell disease. METHODS: Case report. A nine-year-old boy with SS hemoglobinopathy and ocul ocutaneous albinism developed acute unilateral loss of vision. RESULTS: Ophthalmoscopy revealed a pale, milky white, thickened retinal les ion centered on the fovea in the right eye as well as foveal hypoplasia in the left eye. The presence of macular malformation associated with oculocut aneous albinism precluded formation of a cherry-red spot. Fluorescein angio graphy of the right eye demonstrated extensive occlusions of the arterioles surrounding the foveal avascular zone. The presence of occlusions surround ing the fovea from multiple directions suggested the possibility of central retinal artery occlusion with migration of microemboli downstream. CONCLUSION: The patient, the youngest case reported, developed an irreversi ble macular infarction that was not improved by an exchange erythrocyte tra nsfusion. He was placed on a long-term monthly transfusion protocol to prot ect his unaffected eye. (Am J Ophthalmol 2001; 131:275-276. (C) 2001 by Els evier Science Inc. All rights reserved).