Single stage near total resection of massive pediatric head and neck plexiform neurofibromas

Abstract

Objective: Plexiform neurofibromas of the head and neck in neurofibromatosis type 1 (NF 1) carry a significant morbidity with substantial loss of function as well as significant cosmetic problems. We describe our experience with early aggressive surgical intervention in such patients in order to avert these problems.

Methods: Retrospective review of four consecutive pediatric patients with massive head and neck plexiform neurofibromas who underwent single stage near total or sub-total tumor resections.

Results: All four patients were referred for obstructive airway symptoms. Each patient experienced complete relief of symptoms and return of function without additional neurological deficits. There were two minor complications and no major complications of surgical resection. There have been no recurrences to date, with follow-up ranging from 15 months to 5 years.

Conclusions: Early surgical intervention of NF 1 patients with plexiform neurofibromas of the head and neck with a goal of near total resection avoids the loss of function associated with these tumors, such as tracheostomy dependence, swallowing difficulty, and speech problems, and prevents the inexorable progression of substantial cosmetic deformity. Successful management of these complex lesions requires detailed preoperative planning, advanced surgical techniques, and vigilant postoperative care.