Ocular neurofibromatosis.

A 4 1/2-month-old girl with unilateral congenital glaucoma of the left eye subsequently underwent five surgical procedures that proved to be unsuccessful in controlling the intraocular pressure. Neurofibromatosis was diagnosed at the age of 5 1/2 years when she was found to have a plexiform neuroma of the left eyelids and multiple café au lait spots including in the left temple. Two years later the buphthalmic left globe was enucleated. Histopathologic findings included a diffuse neurofibroma of the ciliary body and choroid, peripheral anterior synechiae, total angle closure, and endothelialization of the iris, all of which probably contributed to the glaucoma. Other interesting features were melanocytic hamartomas of the iris, an anterior subcapsular cataract and ovoid bodies in the diffuse neurofibroma of the choroid.