THE OCULAR PRESENTATION OF NEUROFIBROMATOSIS-2

Neurofibromatosis 2 (NF2) is an inherited disorder characterised prima rily by bilateral vestibular schwannomas and other central nervous sys tem tumours, Individuals with NF2 also have early onset cortical and p osterior subcapsular or capsular cataract and other ocular abnormaliti es, such as retinal hamartomas. Although their diagnostic significance is rarely appreciated, the ocular manifestations are often the first sign of disease. We describe 5 cases that illustrate the diverse ocula r manifestations of NF2.