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SEVERE RETINAL ISCHEMIA IN INFANT WITH NEUROFIBROMATOSIS TYPE 1
Aditya S Rali1, J Clay Bavinger, Prethy Rao
1Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia.
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.
