Lisch nodules in neurofibromatosis.

Abstract

Neurofibromatosis is an autosomal dominant disease with multiple systemic and ocular signs and features. The ocular manifestations of neurofibromatosis include a high incidence of iris hamartomas (Lisch nodules), choroidal hamartomas, and eye lid neurofibromas. Other ocular features may include conjunctival neurofibromas, prominent corneal nerves, glial tissue overlying the optic nerve, and bilateral optic atrophy due to optic glioma. Once neurofibromatosis is diagnosed, patients must have long-term ophthalmic evaluations throughout their lifetime, as this is a progressive systemic and ocular disease.