ickle cell dieae preenting with extenive peri-macular arteriolar occluion in a nine-year-old boy

PURPOE: To report a dramatic occluive event of the macula urrounding the foveal avacular zone, cauing evere and permanent lo of viion in a child with ickle cell dieae. METHOD: Cae report. A nine-year-old boy with hemoglobinopathy and oculocutaneou albinim developed acute unilateral lo of viion. REULT: Ophthalmocopy revealed a pale, milky white, thickened retinal leion centered on the fovea in the right eye a well a foveal hypoplaia in the left eye. The preence of macular malformation aociated with oculocutaneou albinim precluded formation of a cherry-red pot. Fluorecein angiography of the right eye demontrated extenive occluion of the arteriole urrounding the foveal avacular zone. The preence of occluion urrounding the fovea from multiple direction uggeted the poibility of central retinal artery occluion with migration of microemboli downtream. CONCLUION: The patient, the younget cae reported, developed an irreverible macular infarction that wa not improved by an exchange erythrocyte tranfuion. He wa placed on a long-term monthly tranfuion protocol to protect hi unaffected eye.