Finding appropriate treatment to HPV infections is one of today’s scientific challenges. Amongst head and neck HPV-related pathologies, the Juvenile-Onset Recurrent Respiratory Papillomatosis (JORRP) might be the least understood of all. It is currently treated with repeated tumor-removal surgeries along with adjuvant therapies which can lead to severe side effects and a certain cost. Due to the scarcity of the JORRP, few studies are accurate in assessing its natural history. National registries have therefore been established in USA and Denmark to help understand JORRP population’s characteristics. The purpose of this study was to describe our pediatric serie of JORRP and to start a local registry.
Every patient diagnosed and treated for JORRP in the Head and Neck Pediatric Department of two tertiary-care hospitals was included in this study. 43 pediatric patients treated between 1980 and 2017 were thus included. Data regarding demographics, patients’ history, surgery, Derkay&Wiatrak score, HPV genotype, histology, and outcome were collected retrospectively in spring 2017.
In our serie, 23 patients were female and 20 were male. The mean age at diagnosis was 37,5 months-old (1-108 months). The average number of surgical procedures underwent by patient's during their follow up in the pediatric centers (up to 18 years-old) was 9,3. Seven cases of dysplasia (16,2%) were reported in this serie (6 low-grade dysplasia, 1 high-grade dysplasia). 5 patients presented a tracheal extent of the disease and 3 patients had pulmonary lesions. A safety tracheotomy had to be performed in one patient. Surgical after effects occurred in 7 patients.
In our experience, the JORRP was associated with a long-term treatment and follow-up including multiple surgeries. 16,2% of patients presented dysplasia. Further descriptive series, immunotherapy essays and therapeutic vaccination impact studies should lead to a better knowledge of this uncommon disease and how to treat it.