Créteil and Paris, France From the Plastic Surgery and Dermatology Departments, Henri Mondor Hospital, Université Paris 12, and the Neurofibromatosis Labeled Reference Center.
Plast Reconstr Surg. 2010 Apr;125(4):1197-1207. doi: 10.1097/PRS.0b013e3181d180e9.
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.
神经纤维瘤病 1 型是一种遗传的、普遍存在的疾病，估计每 3000 例活产儿中就有 1 例发病。神经纤维瘤被定义为各种良性肿瘤，是该病的标志。面部丛状或弥漫性神经纤维瘤通常单侧发生，并可导致面部半侧肥大。手术治疗包括部分切除，以防止牺牲非肿瘤组织，并旨在获得可接受的功能和美容效果，同时考虑到患者皮肤的过度伸展和缺乏弹性。
作者采用基于受累半侧面部美学单位与非受累侧面部对称性之间减法的切除模式，对面部弥漫性或丛状良性面部神经纤维瘤的 33 例神经纤维瘤病 1 型患者（男 15 例，女 18 例）实施了手术。采用肿胀浸润，然后使用双极电凝剪进行单块跨病变入路，系统结扎静脉汇合处，并喷洒纤维蛋白密封剂。无需术前血管造影、肿瘤动脉栓塞或自体输血。
每位患者的平均手术次数为 1.9 次。平均住院时间为 3.65 天。平均肿瘤大小为 11.13cm。平均随访时间为 5.89 年。仅 1 例患者需要输血。1 例双侧丛状神经纤维瘤患者超出了该技术的限制（14 次切除术），需要进行面部移植。
基于预先确定模式的面部美学单位重塑整块跨病变切除术技术具有可重复性，在功能和美容结果方面提供了更高的可预测性，并允许我们对广泛的半侧面部病变进行手术，降低了输血风险。
