Decision-making of orthopedic treatment in neurofibromatosis type 1
10.3760/cma.j.cn114453-20210603-00244
- VernacularTitle:1型神经纤维瘤病的整形外科治疗决策
- Author:
Jian ZHANG
1
;
Jinming ZHANG
;
Yun GE
;
Weiqiang LIANG
;
Jiaqi ZHANG
;
Xiaolian XIAO
;
Chen CHEN
Author Information
1. 中山大学孙逸仙纪念医院整形外科，广州 510000
- Keywords:
Neurofibromatosis 1;
Reconstructive surgical procedures;
Surgery, plastic
- From:
Chinese Journal of Plastic Surgery
2021;37(8):840-846
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To evaluate the feasibility and clinical effect of resection and repairing the neurofibromatosis type 1 (NF1).Methods:Retrospective analysis was performed with the data of patients diagnosed with NF1 in the Department of Plastic Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, from January 2000 to December 2020. All of these patients underwent resection and repair procedures with flap-transferringor skin-grafting.Results:A total of 34 patients with NF1 were included (16 patients with macrosomia NF1, including two patients with cephalic and facial macrosomia NF1 and 14 patients with extremities and somatic macrosomia NF1). There were 14 males and 20 females, aged from 16 to 46 years, with an average age of 30.4 years. The size of the tumor was 65-2 080 cm 2, with an average of 252 cm 2. Nine patients underwent preoperative interventional embolization of the main blood supply artery after consultation with the interventional department. All patients successfully resected the labeled tumor according to the preoperative plan, among which 33 patients underwent one-stage resection and repair. One female patient with large facial neurofibroma experienced uncontrollable bleeding after partial resection of the tumor during the first surgical resection, and the resection and repair were performed by staged surgery after pressurized bandaging for hemostasis. The bleeding during the operation of other patients was controllable. Fourteen cases were sutured directly after tumor resection. Among which, two cases appeared wound dehiscence within one week after the surgery but recovered well after resutured under local anesthesia. Sixteen cases were repaired by transferring skin flaps, of which five patients suffered from insufficient blood supply of the distal end of the flap and underwent debridement and suturing. Four patients were repaired by skin grafting, of which one patient underwent second skin grafting due to poor survival of part of the grafted skin and recovered well. All patients were followed up for 6 to 36 months (mean 22.6 months). Except for one patient with malignant change and one patient with evident tumor recurrence, no recurrence or malignant change occurred in the other patients, and the surgical effect was satisfactory. Conclusions:The resection and repair of NF1, especially giant NF1, should be based on the location and size of the tumor so as to achieve the one-stage resection and repair as far as possible under the premise of controlling bleeding. Different types of repair methods can be applied according to the location, size, and morphology of the lesion to achieve the purpose of removing the lesion and protecting the morphology and function.
