Recurrent Respiratory Papillomatosis

Abstract

Recurrent respiratory papillomatosis (RRP) is the most common benign, often multifocal neoplasm of the larynx and trachea. Despite its benign histology, RRP is a potentially fatal manifestation of human papillomavirus infection. RRP is rare; the annual incidence rate is estimated at 0.35 per 100,000 persons with a prevalence of 4 in 100,000 children. RRP is characterized by the growth of wart-like lesions in the aerodigestive tract, often having a cauliflower-like appearance. They are either pedunculated or sessile and show a tremendous predilection for the larynx. Hoarseness is the leading symptom of RRP, although some children do not present until they develop life-threatening airway obstruction. Affected patients usually require multiple interventions and in severe cases even tracheotomy is needed. Surgical excision is the mainstay of therapy, but it often only alleviates symptoms of the disease. The clinical course of RRP is characterized by adamant recurrences even after seemingly complete surgical excision. The frequency of recurrence, the severity of disease and age of spontaneous remission are variable and unpredictable. To date, there is insufficient evidence to support effectiveness of adjuvant medical treatment in the management of RRP. The clinical course including patient’s history, physical examination, airway endoscopy, surgical and nonsurgical treatment modalities are discussed. Additionally, the cause of RRP, mode of transmission, pathogenesis, and the outcome are presented in this article.