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August 1994

Retinal Vascular Occlusive Disease in a Child With Neurofibromatosis

Author Affiliations

New York, NY

Arch Ophthalmol. 1994;112(8):1021-1023. doi:10.1001/archopht.1994.01090200023009

Systemic vascular ischemic manifestations of neurofibromatosis (NF) have been described in the aortic, cerebral, and renal vasculature.1 The occlusive manifestations include stenosis of small vessels, progressive capillary ischemia, and compensatory collateral circulation. Presented herein is a report of a case of a child with NF and unilateral, diffuse, retinal, vascular ischemia.

Report of a Case.  A 4-year-old Hispanic boy with a history of NF type I presented with an exodeviation and amblyopia involving the right eye. The best-corrected visual acuity was 20/40 OD and 20/20 OS.Diagnosis of NF type I was made on the basis of multiple café au lait spots, plexiform neurofibroma, and a paternal history of NF. There was no history of prematurity, low birth weight, or familial history of vitreoretinal disorders.Funduscopic examination of the right eye revealed a slightly pale, oval optic disc (Figure 1). The fovea was slightly ectopic superiorly and temporally. Diffuse

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