BACKGROUND: Neurofibromatosis type 2 (NF2) is a tumor suppressor syndrome defined by bilateral vestibular schwannomas. Facial paralysis, from either tumor growth or surgical intervention, is a devastating complication of this disorder and can contribute to disfigurement and corneal keratopathy. Historically, physicians have not attempted to treat facial paralysis in these patients. OBJECTIVE: To review our clinical experience with free gracilis muscle transfer for the purpose of facial reanimation in patients with NF2. METHODS: Five patients with NF2 and complete unilateral facial paralysis were referred to the facial nerve center at our institution. Charts and operative reports were reviewed; treatment details and functional outcomes are reported. RESULTS: Patients were treated between 2006 and 2009. Three patients were men and 2 were women. The age of presentation of debilitating facial paralysis ranged from 12 to 50 years. All patients were treated with a single-stage free gracilis muscle transfer for smile reanimation. Each obturator nerve of the gracilis was coapted to the masseteric branch of the trigeminal nerve. Measurement of oral commissure excursions at rest and with smile preoperatively and postoperatively revealed an improved and nearly symmetric smile in all cases. CONCLUSION: Management of facial paralysis is often times overlooked when defining a care plan for NF2 patients who typically have multiple brain and spine tumors. The paralyzed smile may be treated successfully with single-stage free gracilis muscle transfer in the motivated patient.
BACKGROUND:Neurofibromatosis type 2 (NF2) is a tumor suppressor syndrome defined by bilateral vestibular schwannomas. Facial paralysis, from either tumor growth or surgical intervention, is a devastating complication of this disorder and can contribute to disfigurement and corneal keratopathy. Historically, physicians have not attempted to treat facial paralysis in these patients. OBJECTIVE: To review our clinical experience with free gracilis muscle transfer for the purpose of facial reanimation in patients with NF2. METHODS: Five patients with NF2 and complete unilateral facial paralysis were referred to the facial nerve center at our institution. Charts and operative reports were reviewed; treatment details and functional outcomes are reported. RESULTS:Patients were treated between 2006 and 2009. Three patients were men and 2 were women. The age of presentation of debilitating facial paralysis ranged from 12 to 50 years. All patients were treated with a single-stage free gracilis muscle transfer for smile reanimation. Each obturator nerve of the gracilis was coapted to the masseteric branch of the trigeminal nerve. Measurement of oral commissure excursions at rest and with smile preoperatively and postoperatively revealed an improved and nearly symmetric smile in all cases. CONCLUSION: Management of facial paralysis is often times overlooked when defining a care plan for NF2patients who typically have multiple brain and spine tumors. The paralyzedsmile may be treated successfully with single-stage free gracilis muscle transfer in the motivated patient.
Authors: Scott R Plotkin; Simone L Ardern-Holmes; Fred G Barker; Jaishri O Blakeley; D Gareth Evans; Rosalie E Ferner; Tessa A Hadlock; Chris Halpin Journal: Neurology Date: 2013-11-19 Impact factor: 9.910