OCULAR FUNDUS IN NEUROFIBROMATOSIS TYPE-2

Several ocular findings have been associated with neurofibromatosis ty pe 2 (NF 2) since the identification of this disease as a distinct cli nical entity. juvenile cataracts were reported first, followed by comb ined pigment epithelial and retinal hamartomas. In a recent report, ep iretinal membranes were described in seven of nine patients. Moreover, an association between NF 2 and optic disc gliomas has been suggested based on earlier published reports. Six patients with a confirmed dia gnosis of NF 2 were examined. Four patients (six of 12 eyes) had epire tinal membranes and one had an optic disc glioma. In addition, one cas e of an optic disc glioma in a patient with NF 2 was tracked. It is co ncluded that epiretinal membranes are frequent in NF 2, and that optic disc glioma is a rare but specific sign of NF 2. Patients at risk for development of this disease should undergo careful examination of the ocular fundus.