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Abstract #109678 Published in IGR 24-1
SEVERE RETINAL ISCHEMIA IN INFANT WITH NEUROFIBROMATOSIS TYPE 1
Rali AS; Bavinger JC; Rao P; Hubbard GBRetinal cases & brief reports 2023; 17: 471-473
PURPOSE: To report a case of severe retinal ischemia in an infant with neurofibromatosis type 1. METHODS: Chart review, analysis of imaging studies, and review of literature. RESULTS: A boy born at 37 weeks postmenstrual age with neurofibromatosis type 1 was noted to have a large plexiform neurofibroma with left-sided involvement of the cavernous sinus, internal carotid artery, orbit, and optic nerve. He was managed for left eye glaucoma with anti-hypertensive eye drops, and at 8 months of age, he was referred for retinal evaluation. Fluorescein angiography showed striking nonperfusion of the left retina with only a small area of perfused vessels in the posterior pole. A large frond of neovascularization extended anteriorly from the posterior pole. The right eye had a crescent of retinal nonperfusion in the far periphery but otherwise normal retinal vessels. CONCLUSION: This case demonstrates a severe form of retinal ischemia in the setting of a large neurofibroma because of neurofibromatosis type 1. We hypothesize that vascular compression from the tumor led to disruption of the neurovascular bundle with resultant severe nonperfusion, neovascularization, and retinal maldevelopment.
Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia.
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