Pediatric Recurrent Respiratory Papillomatosis: Fighting the Battle of a Rare But Serious Disease

Pediatric recurrent respiratory papillomatosis (RRP) can be devastating for patients and their families. The disease is caused by two strains of the human papilloma virus, HPV-6 and HPV-11, and affects approximately 4 in 100,000 children. The disease course is highly variable, with some children requiring only one or two surgeries and others requiring a hundred. Research and new tools are helping to improve patient care, though many questions remain unanswered and the rarity of the disease makes finding some answers difficult.

HPV Type Influences Disease Course

Several recent studies show that HPV-11 is more frequently associated with aggressive RRP than is HPV-6, though both strains are associated with genital warts or condylomas in adults. In a 10-year prospective study of 73 pediatric RRP patients, Brian J. Wiatrak, MD, Chief of Pediatric Otolaryngology at The Children’s Hospital of Alabama in Birmingham, and colleagues found that patients infected with HPV-11 had a higher severity score at presentation, required more frequent surgeries, and more frequently required medical adjuvant therapies to control disease compared with patients infected with HPV-6.

HPV-16, which is known to cause cervical cancer in women, is infrequently found to infect the airway. When it does however, the likelihood that RRP will progress to cancer increases, said Dr. Wiatrak, and patients should be followed more closely.