Excision and "Transcutaneous" Lift in Patients With Neurofibromatosis of the Fronto-temporo-orbital and Auricular Regions

Abstract

Neurofibromatosis (NF) type 1 (von Recklinghausen disease) involves the face in up to 4% of the cases. Patients present with varying degrees of disfigurement according to the extent and location of the tumor. Many surgeons prefer multiple serial excisions, as it is almost impossible to remove the entire tumor without major extirpative surgery. However, because the "debulkings" are usually not accompanied by durable lift procedures, the skin sags again because of the gravitational effect on the remaining tumor. In a series of 7 patients with NF of the frontal, temporal, facial, and cervical areas and the auricle, a lift procedure was performed after functional debulking of the tumor. The lift sutures, which passed transcutaneously, supported and fixed the predetermined areas on the skin or the ear cartilage to the periosteum of the calvarium. All the patients previously had multiple debulking surgeries without any lift procedures, which resulted in resagging of the facial elements within 3 months of surgery. The transcutaneous lift was durable up to 1 year in preventing resagging. After 1 year, because of the presence of disease, there was a slow relapse in the amount of sagging in the temporal and frontal areas, but not in the auricle. In 3 patients, re-excision of the disease and repeat lift procedure using the same technique were performed after 1 year. All were satisfied with the results and stated that the period without sagging lasted longer compared with their prior surgeries. The patients indicated that they were able to socialize more often and without being overly concerned with their appearance. The patients with NF type 1 undergo many operations during their lifetime. Many of these procedures are debulking procedures to reduce the size of the diseased tissues. These procedures are not aimed to completely eliminate the disease, but rather to improve function and appearance. When these debulking procedures are combined with durable lifting procedures, the results are longer lasting with improved quality of life.

A Novel Technique Using Proline Mesh for Simultaneous Excision and Soft Tissue Suspension in the Management of Craniofacial Neurofibromatosis Type 1

“…Our patients according to the NAS score were rated average score 2.08 (±0.12), around categories II special in patients with both orbitotemporal and hemifacial lesions, while Lee et al, 2004 andNagata 2006 showed patients with NAS score to fall between category II and III [23,41,45] symmetry improvement showed average 8.90% (±) 4.89%, the mean percentage of improvement was lowest (7.9%) among patients with both orbitotemporal and hemifacial lesions, which may require reassessment for the possibility of surgical revisions.…”

“…The importance of tissue Suspension was revealed by the characteristics of NF-1 as the loss of skin elasticity, skin extensibility and tumor recurrence increases the tissue volume which will all act with the gravitational force adding to the deformity, making the long term results unpredictable [45,46]. Durable tissue suspension can theoretically slow the tumor progression, as it minimize the effects of gravity on tumor tissue expansion [45]. The proline mesh was chosen for its durability and multiple strips with different vectors are used according to the facial submit suspended.…”

Initial Exploration on Temporal Branch of Facial Nerve Function Preservation in Plexiform Neurofibroma Resection

“…Tumor resection is the only effective way of PNF treatment. 11 – 13 The aim of surgical intervention for facial disfigurement involves tumor debulking, function restoring, and cosmetic facial appearance reconstruction.…”