75. Recurrent Respiratory Papillomatosis

Definition

Recurrent respiratory papillomatosis (RRP) is a manifestation of infection by the human papilloma virus (HPV) anywhere within the entire respiratory tract/system, from the nose into the lungs.

Incidence

Age distribution of RRP is bimodal. The most common presentations are in children younger than 5 years, juvenile-onset recurrent respiratory papillomatosis (JORRP), or in adults in the fourth decade of life, known as adult-onset recurrent respiratory papillomatosis (AORRP). Children 14 years old or younger average 4.3 cases per 100,000 of the population. Adolescents 15 years to adult average 1.8 cases per 100,000 of the population.

In JORRP, males and females are affected equally. In AORRP, males are affected more frequently in a ratio approaching 4:1. RRP also seems to appear more frequently in Caucasians than other races.

Etiology

RRP is caused by the human papilloma virus (HPV), usually HPV-6 or HPV-11 variants. The HPV-16 or HPV-18 variants are the causative agents in rare cases.

JORRP is typically transmitted from the infected mother during the perinatal period, most commonly during vaginal delivery. JORRP produces a more severe disease than AORRP.

AORRP’s mode of acquisition is not known with certainty, but it is strongly suspected to be via sexual transmission.

Signs and Symptoms

• Choking episodes

• Cough

• Dyspnea

• Failure to thrive

• Foreign body sensation

• Hoarseness

• Inspiratory wheeze

• Stridor

• Voice/vocal change

• Weak cry

Medical Management

Medical management of RRP consists almost entirely of surgical intervention. Pharmacotherapy is not a curative intervention but rather is intended to slow the growth of the HPV-produced papillomas and thereby extend the time between surgical debulking procedures. Pharmacologic agents with demonstrated effectiveness in increasing the intervals between resection or debulking include intralesional cidofovir, indole-3-carbinol, interferon, and photodynamic therapy. Other pharmacologic agents that have variable effects on RRP include cimetidine, acyclovir, and retinoic acid.

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