Surgical Tips for Neurofibromatosis: Synopsis of Surgery with Technology -

Abstract

ABSTRACT Background: Plexiform neurofi bromas of the head and neck region inneurofi bromatosis-1 (NF-1) presentwith varying degrees of disfi gurement according to the extent andlocation of the tumor due to its growing continously. Objective: Plexiform neurofi bromas of the head and neck in NF-1 carry a signifi cant morbidity with substantial loss of function as well assignifi cant cosmetic problems. We describe our experience with early aggressivesurgical intervention in such patients in order to avert these problems. Methods: Retrospective review offi ve consecutive patients with head and neck plexiform neurofi bromas who underwent single stage near total or subtotaltumor resections. Results: One patient had local fl ap necrosis, which was covering the implant. Therefore free tissue transfer needed to be done. Otherwise, there were just two minor complications and no majör complications in rest of the operated patients. There have been no recurrences up to date, withfollow-up ranging from 15 months to 5 years. Conclusions: Early surgical intervention of NF-1 patients with plexiform neurofi bromasof the head and neck with a goal of near total resection avoids the loss of functionassociated with these tumors, such as tracheostomy dependence, swallowing diffi culty,and speech problems, and prevents the inexorable progression of substantialcosmetic deformity. Successful management of these complex lesions requiresdetailed preoperative planning, advanced surgical techniques, and vigilant postoperativecare. Custom-made CAD/CAM implants makes the skelotal reconstruction easier and more symmetrical.