Facial aesthetic unit remodeling procedure for neurofibromatosis type 1 hemifacial hypertrophy: report on 33 consecutive adult patients.

Abstract

Neurofibromatosis type 1 is a genetic and ubiquitous disease, with an estimated incidence of one in 3000 live births. Neurofibromas, defined as various benign tumors, are hallmarks of the disease. Facial plexiform or diffuse neurofibromas usually occur unilaterally and can induce facial hemihypertrophy. Surgical treatment consists of partial removal, to prevent sacrificing nontumoral tissues, and is aimed at acceptable functional and cosmetic results, considering the hyperextensibility and lack of elasticity of patients' skin. The authors operated on 33 neurofibromatosis type 1 patients (15 men and 18 women) suffering from diffuse or plexiform benign facial neurofibromas with a facial aesthetic unit remodeling surgical technique with a resection pattern based on the substraction between aesthetic units of the affected hemiface and the symmetry of the nonaffected one. A tumescent infiltration preceded a monobloc translesional approach using bipolar coagulation scissors, with systematic ligation of venous confluents and a fibrin sealant spray. No preoperative angiography, arterial embolization of the tumor, or autologous transfusion was required. The average number of procedures for each patient was 1.9. The mean length of stay was 3.65 days. The average tumor size was 11.13 cm. The average follow-up was 5.89 years. Only one patient received transfusion. One patient suffering from bilateral plexiform neurofibromas was beyond the limit of the technique (14 debulking procedures) and required a face transplantation. The facial aesthetic unit remodeling monobloc translesional resection technique on a preestablished pattern has been reproducible, offered increased predictability in functional and cosmetic results, and allowed us to operate on extensive hemifacial lesions with a lower transfusion risk.