Management of Neurofibromatosis Type 1 of Mid and Lower Face

Abstract

Neurofibromatosis type I (von-Recklinghausen's disease, NF1) is a common autosomal dominant hereditary disease. Patients are characterized by multiple cafe-au lait spots and neurofibromas. Plexiform neurofibroma, especially those in the head and neck, frequently elicits major cosmetic and functional impairment. We present two cases of plexiform neurofibroma of face who underwent serial tumor ablation and reconstructive surgeries. Because the infiltrating nature of plexiform neurofibroma makes complete excision difficult, our surgical strategy was to preserve the remaining neuromuscular function and remove as much of the tumor as possible to restore the normal appearance. When using surgical techniques, we recommend the following: (1) Surgery is performed under hypotensive anesthesia and liberal suture ligation to decrease intraoperative bleeding. (2) Performing serial excisions shortens the operation time and decrease blood loss. The plan of serial excisions is mainly based on ”aesthetic unit”concept. (3) Applying technique of rhytidectomy smoothes the facial contours. (4) Preserving the neuromuscular tissue improves the function; and restores normal anatomic position and profile of the involved mouth, nose and ear.