Initial results from the national registry for juvenile-onset recurrent respiratory papillomatosis

Objective: To characterize the spectrum of juvenile onset recurrent respira tory papillomatosis (RRP) in the United States and to obtain data about the natural course of the disease and its response to treatment. Setting: Twenty tertiary-care pediatric otolaryngology centers throughout t he United States. Patients: All patients with active RRP aged less than 18 years at the parti cipating sites. Main Outcome Measures: Number of surgical procedures performed per year, pr ogression of papillomas to previously nondiseased anatomical sites, drug in terventions and other adjuvant therapy, and need for tracheostomy. Results: Data were collected from 399 children enrolled from January 1, 199 7, through December 31, 1998. There were 51.9% male; 62.7% white, 28.3% bla ck, 9.0% other or unknown racial group; 10.8% Hispanic ethnicity. Mean age at diagnosis was 3.8 years (range, 0.1-16.3 years) and mean duration of dis ease was 4.4 years (range, 0.03-18.9 years). The mean number of surgical pr ocedures per child was 4.4 per year (range, 0.2-19.3 per year). Children wh ose RRP was diagnosed at younger ages (<3.0 years) were 3.6 times more like ly to have more than 4 surgical procedures per year (P =.001) and almost 2 times more likely to have 2 or more anatomical sites affected (P =.008) tha n were children whose RRP was diagnosed at later ages (greater than or equa l to 3.0 years), after adjusting for sex, race, and years of treatment. Conclusions: Children whose disease was diagnosed before age 3 years were m ore likely than children aged 3 years or older to have more severe disease as measured by the mean number of surgical procedures performed and by the number of anatomical sites affected. The registry will form the basis for f uture analysis on the outcome of disease, natural course of RRP under manag ement strategies, prevention strategies, and public health importance.