Experience with the surgical management of cranio-orbital neurofibroma
tosis 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.