Orbitotemporal neurofibromatosis: classification and treatment

In localized orbitotemporal neurofibromatosis, the treatment depends very much on the type and severity of the orbital involvement and on the functional state of the eye. The condition can be conveniently divided into three groups, each requiring different treatment: (1) orbital soft-tissue involvement with a seeing eye, (2) orbital soft-tissue and significant bony involvement with a seeing eye, and (3) orbital soft-tissue and significant bony involvement with a blind or absent eye. A total of 24 patients with a maximum of 12 years recurrence-free follow-up are presented.