A prospective study of the incidence of juvenile-onset recurrent respiratory papillomatosis after implementation of a national HPV vaccination program

Recurrent respiratory papillomatosis (RRP) is a condition characterized by the repeated growth of benign exophytic papillomata in the larynx. The juvenile-onset form of recurrent respiratory papillomatosis (JORRP) usually develops in early childhood with a mean age of diagnosis between 3 and 4 years [1, 2]. Although rare, affected children often require multiple surgical procedures to debulk recurrent lesions, in order to maintain voice function and airway patency. Distal airway spread can occur in up to a quarter of cases, tracheostomy may be required in more severe cases, and deaths have been reported [2].

RRP is caused by human papillomavirus (HPV) infection, with over 85% thought to be related to HPV-6 or 11 genotypes. HPV-11 appears to be associated with more aggressive clinical disease, especially in children [3, 4]. Perinatal transmission of HPV in JORRP is thought to occur from infected mother to child intrapartum [5]. Maternal genital warts during pregnancy and delivery is the strongest risk factor for development of JORRP [6]. Vaginal delivery, firstborn child, prolonged labor, premature rupture of membranes, and maternal age <30 are also thought to be potential risk factors [5, 6]. The mechanisms of infection and disease development are not fully understood as JORRP is still rare in neonates born to HPV-positive mothers [7, 8].

The introduction of Australia’s national HPV vaccination program in 2007, during which at least half of Australia’s female population aged 12–26 years were fully vaccinated [9, 10], has raised the possibility of prevention of JORRP. The quadrivalent vaccine Gardasil® confers protection against high-risk HPV types 16 and 18, which are responsible for about 70% of cervical cancer worldwide [11], and it also offers protection against HPV types 6 and 11, which cause the majority of genital warts and RRP [12,13].