Case 33 :
History of dyspnoea. What is the diagnosis?

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Laryngeal neurofibromatosis


Neurogenic tumors including neurofibromas of the larynx are uncommon. Some of these have been associated with VRD but the majority of reports do not mention associated features of von Recklinghausen's disease (VRD). The cases of laryngeal plexiform neurofibromas are less common than solitary neurofibromas and some reports do not identify whether or not the neurofibromas are of the plexiform variety. Plexiform neurofibroma exists with and without associated VRD. Extensive involvement of many structures with these tumors is the usual situation. Nearly all of the solitary tumors are supraglottic with a predilection for the aryepiglottic fold or false vocal cord. Based on this anatomic association, Nanson has speculated that they most commonly arise from the superior laryngeal nerve and its terminal ramifications. Schwannomas and neurofibromas have been reported to arise throughout the larynx, involving the false vocal cord, epiglottis, and subglottis. Management of these tumors may be as simple as endoscopic removal or may involve extensive radical surgery to restore function. Plexiform tumors infiltrate widely and complete removal is usually not a realistic goal.

The management of these patients is highly individualized. The variable but always progressive nature of this disease necessitates a cautious, conservative approach whenever possible.

Dr. Bhujang Pai, Hinduja Hospital, Mumbai