Ocular ischemic syndrome in neurofibromatosis type 1 treated with corticosteroids.

Neurofibromatosis type 1 (NF-1) is an autosomal dominant condi-tion characterized by cafe ?-au-lait spots, neurofibromas, and multi -system involvement, including vasculopathy that may lead to ischemic or hemorrhagic events. Cases of vascular occlusions involving the retinal or ophthalmic circulation have also been described. The majority of cases with reported outcomes describe poor visual acuity following resolution. We report a case of retinal and ophthalmic artery occlusion resulting in ocular ischemic syn-drome in a patient with NF1 who had remarkable improvement in retinal perfusion and visual acuity following high-dose corticoste-roid treatment.