Abstract
A 41-year-old woman had had slowly progressive loss of vision and exophthalmos of the left eye from the age of 35 years. Multiple café-au-lait spots and subcutaneous neurofibromatous masses were noted. Plain skull X-rays revealed a defect of the posterior wall of the left orbit and bulging of the left frontotemporal bone. There was also a small bony defect in the occipital bone adjacent to the left lambdoid suture. Computed tomography (CT) disclosed a marked deformity of the left middle cranial fossa and a bony defect in the posterior wall of the left orbit. Three-dimensional CT was extremely useful in the planning of the surgical repair. The posterior orbital wall defect, which was approached through a left frontotemporal craniotomy, was covered with a resin plate, and the exophthalmos was corrected under direct vision. The bulging frontotemporal bone was resected and replaced by a resin plate. Postoperatively, the exophthalmos was markedly reduced and the patient regained light perception in the left eye.
