Sectoral retinal pigmentation in neurofibromatosis.

Sector retinitis pigmentosa of Bietti has not been studied histopathologically and has not been reported as occurring in a patient with neurofibromatosis. This report describes a patients with neurofibromatosis and biopsy-proven optic nerve glioma who was noted before death to have bilateral sectoral retinal pigmentary disturbances that appeared histopathologically to be areas of retinitis pigmentosa.