The surgical management of cranio-orbital neurofibromatosis.

Abstract

Experience with the surgical management of cranio-orbital neurofibromatosis in 14 patients is reported (age range, 6-40 years). The skeletal abnormality of the orbit that occurs in a small proportion (less than 1%) of patients with neurofibromatosis is, in essence, the absence of the membranous portions of the sphenoid and the adjacent bone forming the boundaries between the cranium and the orbit. The goal of surgery is tumor resection and reconstruction of the posterior bony defect by bone graft. Two additional procedures are also described that better enhance aesthetically-the mask lift and facial tissue expansion.