Operative management of juvenile-onset recurrent respiratory papillomatosis

Abstract

Juvenile-onset recurrent respiratory papillomatosis is a devastating illness of children who develop obstruction of the airway from neoplastic growths of infectious origin. The disease is caused by human papillomavirus (HPVs), types 6 and 11, incorporating itself into the genome of the respiratory mucosa. The consequence of this genetic piracy is a morphologic change from an airway lining that is flat and smooth to an irregular "mulberry like" hyperplasia. The growths seem to have a predilection for the glottic mucous membranes but can involve any part of the aerodigestive tract. The most common symptom is hoarseness, and children can have an alarming degree of obstruction with this as their only symptom. When hoarseness progresses to stridor, respiratory distress is impending. The dynamics of disease recurrence vary widely, with the more severe, rapidly recurring disease requiring frequent operative excisions to maintain airway patency. Surgery has been the mainstay of management but is not curative. Adjuvant therapies are elusively effective, unpredictably decreasing the disease activity and increasing the duration between surgeries. The quadrivalent HPV vaccine (Gardisil; Merck, NJ) is promising to reduce the HPV infection rate in young women, with the hope that by eliminating this reservoir of disease, the incidence of HPV-related illnesses, juvenile-onset including recurrent respiratory papillomatosis, will dramatically drop.