Plexiform Neurofibroma of the Larynx: A Challenging Management Dilemma

Abstract

Introduction: Among the many causes responsible for stridor in the pediatric population, tumors of the larynx are relatively rare. Rarer still is the presence of endolaryngeal neurofibromas. Plexiform neurofibromas are associated with type I neurofibromatosis (NF1). Their typical characteristic is that they are diffuse tumors with indistinct margins, which makes complete resection a challenge and the chances of recurrences higher. Objective: To document our experience with endolaryngeal neurofibromas and to discuss the treatment options available for this rare condition. Methods: We present two cases of plexiform neurofibromas in pediatric patients. Both children presented with large supraglottic masses which interfered with breathing and swallowing. They also fulfilled the criteria for type I neurofibroma. Endolaryngeal approach with microdebrider and laser-assisted surgical excision was performed in both cases. Result: Following surgery, both patients had uneventful recovery and no subsequent breathing or swallowing difficulties. Conclusion: The dilemma in the management of endolaryngeal neurofibroma is the choice between endolaryngeal laser and aggressive external approach surgery. A short review of the limited existing literature shows that it is wiser to limit the surgery to as complete a resection as is possible endoscopically.