Circumferential parallel ligation for debulking of very large neurofibromas in patients with neurofibromatosis type 1.

Abstract

Neurofibromatosis Type 1 (NF1) is a genetic disorder characterized by the presence of neurofibromas, including diffuse cutaneous and plexiform variants. In large lesions, debulking surgery is often indicated, but presents challenges due to the hypervascularity of the tumor and the risk of hemorrhage. Circumferential parallel ligation (CPL), originally developed for the management of vascular malformations, may be an effective approach for controlling hemorrhage during debulking surgery. A retrospective review was conducted of 19 surgeries performed on 12 patients with NF1 and very large neurofibromas, between 2003 and 2024. All patients underwent debulking surgery using CPL. Data on tumor size, operative time, intra-operative blood loss, transfusion requirements, resection rate and post-operative complications were collected and analyzed. The mean tumor size was 26.6 cm, and the mean blood loss was 263.6 ml. Only two of the 19 surgeries required a blood transfusion. CPL was consistently effective in minimizing intra-operative hemorrhage without the use of specialized hemostatic devices. The mean resection rate was 71.9 %, indicating that significant tumor debulking was achieved in the majority of cases. Post-operative complications occurred in four surgeries but resolved with appropriate treatment. CPL is safe and effective for debulking large neurofibromas in patients with NF1, offering reliable hemostasis without requiring costly surgical devices.