Initial Results From the National Registry for Juvenile-Onset Recurrent Respiratory Papillomatosis

Abstract

RECURRENT respiratory papillomatosis (RRP) is a chronic disease caused by human papillomavirus types 6 and 11. The most common site of lesions is the larynx, but papillomas can occur throughout the respiratory and upper gastrointestinal tracts. Juvenile-onset RRP (JORRP) presumably occurs by transmission of human papillomavirus from infected mothers to their infants, but the exact mechanism of transmission is unknown.1 Frequent recurrence of disease, despite surgical extirpation, results in airway compromise, permanent and transient voice changes, and substantial childhood morbidity. Surgical removal of laryngeal papillomas is often necessary every 1 to 2 months, placing extreme financial and psychological hardships on the patients and their families. Death occurs in RRP when papillomas obstruct the airway or spread to the lung parenchyma.2 Medical treatments, such as interferon alfa, acyclovir, retinoids, and indole-3-carbinol, have been used to treat RRP, with mixed success.3