Soft tissue management of orbitotemporal neurofibromatosis

The aim of this study was to provide an overview of a single-institution, 30-year surgical experience with the soft tissue management of orbitotemporal neurofibromatosis. Lessons learned are highlighted in case presentations.

From 1981 to 2011, all patients who presented to the Chang Gung Memorial Hospital Craniofacial Center with craniofacial neurofibromatosis and orbitotemporal involvement were retrospectively reviewed. The medical records of those patients who underwent surgical correction were reviewed for age, extent of involvement, procedures performed, histologic confirmation, and acute complications. All patients were grouped according to the Jackson Classification. The electronic photobank was queried to evaluate results.

Thirty-five patients presented to our center with orbitotemporal neurofibromatosis during the study period. Thirty-one patients underwent surgical management of their disease. The average age was 25 years (range 4 to 57 years). Over half of our patients (n = 18) presented with concomitant disease of the cheek. The 2 most common procedures performed were lateral canthopexy (n = 24) and upper eyelid excision (n = 24). The only acute complication recorded was a postoperative hematoma on the fourth postoperative day following simultaneous lateral canthopexy and upper eyelid excision which required operative evacuation.

In orbitotemporal neurofibromatosis, tissue hyperextensibility and tumor weight adversely affect outcomes. Treatment of concomitant disease of the cheek should be prioritized in order to provide periorbital support prior to addressing the delicate structures of the eyelids. Preservation of the lateral canthal unit and levator muscle, despite neurofibroma infiltration, is critical to maximize outcomes following debulking procedures of the eyelid and orbit.