It is not uncommon for children to present with visual complaints secondary to cataracts, retinal hamartomas, or optic nerve damage from a schwannoma. The most common eye findings are juvenile cataracts (up to 80% of patients), followed by epiretinal membranes in 12-40%, and retinal tumors in 6-22%. Lens opacities may be located in the posterior subcapsular region or as cortical wedge-shaped opacities in the periphery where they are easily missed if the pupil is not fully dilated. Translucent, grayish epiretinal membranes with white edges are present in a significant number of patients, including children. Elevated retinal hemartomas in the macula are often associated with pigmentary changes. Lisch nodules characteristic of type I neurofibromatosis (162200) are generally not found in type II. Corneal damage may occur as a complication of hypesthesia resulting from damage to the fifth cranial nerve.
CT scans reveal calcification along the optic nerve in a "tram-track" configuration which occurs in 20-30% of patients with NF type II.