Unilateral Aggressive Ischemic Proliferative Retinopathy in a Child with Neurofibromatosis Type 1, A Case Report

Abstract

Background: Neurofibromatosis is one of the phacomatosis syndromes that affects different organs in the body and is divided into two types. Neurofibromatosis type 1 can cause eye involvement in various forms, including eyelid, conjunctiva, iris or optic nerve involvement. Involvement of retinal vessels in the form of retinal ischemia is rare in this disease and usually occurs in the second or third decades of life and is controlled by laser treatment or surgery. Case Report: In this report, we introduce a 3-year-old girl with neurofibromatosis type 1 who suffered severe retinal ischemia. This child suffered unilateral retinal involvement in the form of ischemic retinopathy with retinal and vitreous hemorrhage in her left eye, and despite two vitrectomy and retinal laser surgery in the left eye, the retinal involvement progressed and eventually the vision in the left eye was lost. Conclusion: This report shows for the first-time retinal involvement at a young age in a patient with neurofibromatosis type 1 and its resistant nature. © 2023 Isfahan University of Medical Sciences(IUMS). All rights reserved.