An unusual case of Juvenile-onset Recurrent Respiratory Papillomatosis with lower airway involvement in a 15-month-old boy

Recurrent Respiratory Papillomatosis (RRP) is a rare disease caused by human papillomavirus (HPV) infection, especially with types 6 and 11. It is characterised by the presence of multiple airway papillomas located mainly in the larynx; involvement of the distal airways and lungs may also occur. There are two clinical forms of the disease depending on the age of onset, Juvenile-onset RRP (JoRRP) and Adult-onset RRP (AoRRP). JoRRP is the most common clinical form and usually affects children younger than 5 years of age. It is generally more aggressive with a high recurrence rate and is acquired by vertical transmission during vaginal delivery of infected mothers. There is currently no effective treatment for RRP and surgery remains the main treatment option. However, systemic treatment with bevacizumab, a recombinant humanized monoclonal antibody that binds to vascular endothelial growth factor-A (anti-VEGF-A) and prevents angiogenesis, has been proposed as adjuvant therapy in advanced RRP cases. We present an unusual and aggressive case of JoRRP with distal airway involvement in a 15-month-old boy who showed a complete response to systemic bevacizumab.
Bevacizumab is a promising adjuvant therapy for recurrent respiratory papillomatosis, particularly in cases involving the distal airways that are difficult to treat with standard surgical procedures.

Received: Nov 27, 2023
Accepted: Jan 18, 2024

Table of Contents: Online first

Remember that the download is free only for personal use. If you want to utilize articles for large distribution, please contact us at