Involvement of the eye and orbit in neurofibromatosis type 1.

ABSTRACT

11 individuals were diagnosed to have neurofibromatosis type 1 and were examined for evidence of any ophthalmic lesions. Lisch nodules were the commonest manifestation of the disease and were present in 73% of all the patients (88% of those aged 16 years or more). 55% of the cases showed presence of neurofibroma on the lids. Other findings were optic glioma, unilateral sphenoid dysplasia with enlarged orbit, medullated nerve fibers and prominent corneal nerves with an incidence of 9% each.