The surgical management of orbital neurofibromatosis.
Orbitofacial neurofibromatosis may cause severe soft-tissue deformity along with pulsating exophthalmos. The orbit is enlarged, and the greater wing of the sphenoid is absent. The eye in some cases may be functionally useless, immobile, displaced, and blind. In these, orbital exenteration with soft-tissue excision and orbital reconstruction is recommended. External cover is provided by the thin eyelid skin, and a prosthesis ultimately allows a very satisfactory end result. Four such cases with a minimum of 2 years of follow-up are presented.
D000293:Adolescent; D000328:Adult; D001763:Blepharoptosis / Q000209:etiology; D002648:Child; D005094:Exophthalmos / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications / Q000601:surgery*; D009918:Orbital Neoplasms / Q000150:complications / Q000601:surgery*; D019736:Prostheses and Implants; D014786:Vision Disorders / Q000209:etiology