TRANSCRANIAL CORRECTION OF ORBITAL NEUROFIBROMATOSIS

Neurofibromatosis is a systemic disease that often produces striking d isfigurement. Orbital manifestations are common and include sphenoid d ysplasia with or without infiltration of the periorbital soft tissues. The resultant deficiency of the posterolateral orbital wall may lead to protrusion of the temporal lobe into the orbit, displacement of the globe, and pulsatile exophthalmos. Treatment at our unit has consiste d of transcranial orbital reconstruction with bone grafts and periorbi tal soft-tissue correction. Observation of complete bone graft resorpt ion in one patient prompted an assessment of the Australian Craniofaci al Unit's experience with particular attention paid to the stability o f operative correction. Of 36 patients with head and neck neurofibroma tosis treated during the period from 1981 to 1995, 14 patients underwe nt transcranial correction of orbital deformities secondary to sphenoi d dysplasia. The treatment and outcomes of this transcranial group are reviewed. The most notable finding was that of recurrent globe pulsat ion in four patients following initial resolution. Computed tomography scans have documented partial to complete bone graft resorption in th ree of these patients. Titanium mesh is now being utilized to provide a more durable reconstruction.