Ocular ischemic syndrome in neurofibromatosis type 1 treated with corticosteroids.

ABSTRACT

Neurofibromatosis type 1 (NF-1) is an autosomal dominant condition characterized by café-au-lait spots, neurofibromas, and multisystem 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 syndrome in a patient with NF1 who had remarkable improvement in retinal perfusion and visual acuity following high-dose corticosteroid treatment.