Reconstruction of the sphenoid wing in a case of neurofibromatosis type 1 and complex unilateral orbital dysplasia with pulsating exophthalmos.

Sphenoid wing dysplasia is a defining feature of neurofibromatosis type 1 (NF1). This defect of the skull base can be associated with pulsating exophthalmos. This report describes the successful reconstruction of a dysplastic sphenoid wing in an NF1 patient using lateral orbitotomy as a scarcely visible and sufficiently extendable approach. An intraoperative computed cone-beam computed tomography system (3D C-arm system) was used as a prompt and feasible technique to check the positioning of the titanium mesh in an anatomic region that is sensitive to mechanical stress.