Ocular Angiographic Findings in Patients with Neurofibromatosis Type 1

Abstract

Purpose: To describe the ocular angiographic findings in patients with Neurofibromatosis type 1 (NF-1). Methods: We examined 20 consecutive patients diagnosed with NF1 by conventional ophthalmoscopy and fluorescein angiography (FA), indocyanine green angiograms (ICGA) were also obtained from 11 of the patients. Forty FAs and 25 ICGAs were also obtained from age-matched controls. Results: Fluorescein angiography revealed patchy hyperfluorescence in 30%, retinal pigment epithelial (RPE) atrophy in 30%, “corkscrew” vessels in 50%, optic disc abnormalities in 25% and choroidal nevi in 30% of the patients. All the patients investigated by ICGA (n = 11) demonstrated hypofluorescent lesions corresponding to the patchy hyperfluorescence on fluorescein angiography. None of the control patients had any of the findings described neither on FA nor on ICGA. Conclusion: Because of their high frequency (100%), the hypofluorescent lesions on ICGA should be added as a new diagnostic criterion in NF1. RPE atrophy and the high frequency of optic disc abnormalities appear to be novel findings in patients with NF-1.