ObjectiveTo explore the plastic surgical treatment and the way to reduce hemorrhage for scalp and facial plexiform neurofibromas.
MethodsBetween July 2004 and July 2013, 20 patients with scalp and facial plexiform neurofibromas (17 cases of neurofibromatosis type Ⅰ and 3 cases of plexiform neurofibroma) were treated, and the clinical data were retrospectively analyzed. There were 9 males and 11 females with an average age of 37 years (range, 18-56 years). The disease duration ranged from 8 to 56 years (mean, 19 years). The scalp was involved in 6 cases, the face in 7 cases, and both the scalp and face in 7 cases. The extent of involvement ranged from 4 cm×3 cm to 15 cm×13 cm. Obvious pigmentation was seen in 2 cases. Endovascular embolization was performed before surgical intervention in 4 cases. Preliminary sutures around the lesion were carried out in 18 cases having an involved range over 5 cm×5 cm. One-stage excision was performed in 17 cases, and two-stage excision in 3 cases. Wound repair and facial orthopedic treatment were performed after tumor excision.
ResultsThe intraoperative blood loss was 100-500 mL (mean, 300 mL) for patients undergoing single operation. For 3 patients undergoing two-stage excision, the blood loss of the first operation was 500, 600, and 800 mL respectively, and the blood loss of the second operation was all 50 mL. Autologous blood transfusion of 200, 400, and 400 mL was performed in 3 cases respectively. The preliminary sutures were removed at 3-7 days (mean, 5 days) after operation. All the incisions healed primarily without secondary hemorrhage and hematoma, and the flap and skin graft survived totally. Fifteen patients were followed up 1 year to 7 years (mean, 2.5 years). All patients showed significant improvement in appearance. No significant progression, expanding, and sagging were observed.
ConclusionEndovascular embolization and preliminary sutures around the lesion can be used to reduce hemorrhage in resection of plexiform neurofibroma in the scalp and face. Personalized surgical plan of benign neurofibromatosis should be made to reduce the tumor mass, to improve function and appearance.
Neurofibromatosis type 1 (NF1), an autosomal dominant genetic disorder resulting from mutations in the NF1 gene, is frequently characterized by the development of plexiform neurofibromas (PNFs). A paradigm shift in the management of PNFs has occurred following the successful development of mitogen-activated protein kinase kinase inhibitors. In light of these advancements, the Expert Consensus for Full Course Management of Plexiform Neurofibroma (2025 Edition) was published by the Group of Neurofibromatosis, Plastic and Reconstructive Surgery Society of Chinese Medical Association in 2025. This consensus provides recommendations on the diagnosis, screening, surgical management, medical therapy, disease surveillance, as well as patient education and psychosocial support for NF1-associated PNFs. This article aims to interpret and discuss full course management of PNF in this consensus to provide references for clinical practice.
