Ocular Angiographic Findings in Patients with Neurofibromatosis Type 1

摘要

Purpose: To describe the ocular angiographic findings in patients with Neurofibromatosis type 1 (NF-1). Methods: We examined 20 consecutive patients diagnosed with NF-1 by conventional ophthalmoscopy and fluores-cein 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 NF-1. RPE atrophy and the high frequency of optic disc abnormalities appear to be novel findings in patients with NF-1.