Recurrent respiratory papillomatosis (RRP), also known as laryngeal papilloma, is a rare but benign disease that is caused by the human papilloma virus (HPV) and characterized by the growth of tumors in the respiratory tract, most commonly within the larynx around the vocal cords. The majority of RRP is caused by HPV type 6 and 11 and has tendency to recur, thus the term “recurrent.” Occasionally growths may be found in the lower airways such as the trachea, bronchi and lungs. RRP often appears “cauliflower-like” and can be attached to the tissue by a small stalk or spread over the mucosa at several areas of the larynx.
RRP can be diagnosed in children as well as adults. The incidence of RRP in children is approximately 4.3 per 100,000 and is transmitted either in utero or at the time of birth by mother to infant, although presence of HPV in the mother does not always lead to RRP in children. Diagnosis in children usually occurs before the age of 5 and is often more severe than adult onset RRP. Adult RRP is rare, with incidence of approximately 1.8 per 100,000 and is transmitted sexually. HPV cannot be passed to other persons by drinking from the same cup, kissing or coughing on someone. Transformation of RRP to cancer is extremely rare (1-5%) and seen mostly in adults.
RRP usually presents in adults and children as hoarseness, noisy breathing and often trouble breathing. Visualization of the airway is usually performed in the clinic with a small flexible telescope that is passed through the nostrils or sometimes using a rigid telescope that sits on the tongue.
Treatment of RRP is mostly surgical, with lesions removed using different microscopic instruments as well as laser. In cases where RRP is found beyond the larynx, a pulmonologist may be involved in treatment as well. Patients with recurrent disease despite multiple procedures may consider additional therapies including injection of antiviral medications as well as medications to control reflux.
A vaccine for HPV 6,11, as well as 16 and 18 (associated with cervical cancer) was introduced in 2006 with hopes that administration to children will lead to decreased HPV infections and thus decreased RRP. Research on HPV vaccines in patients with RRP is ongoing. Although there is no cure available at this time, RRP can be managed effectively and patients can maintain a good quality of life when regularly followed by an otolaryngologist.
For more information regarding RRP, check out the Recurrent Respiratory Papillomatosis Foundation at RRPF.org.