Pediatric Airway Disorders

Recurrent Respiratory Papillomatosis (RPR)

Recurrent respiratory papillomatosis is an extremely rare condition caused by the growth of viral warts in the larynx. The growths typically cause the child to become hoarse but as the RRP get larger, the child may experience breathing difficulties. These benign growths are treatable but not curable.

What causes recurrent respiratory papillomatosis?

Image benign papilloma tumors in the airway.
Benign papilloma tumors in the airway.

Human papilloma virus (HPV) is believed to be the cause, or one of the causes of recurrent respiratory papillomatosis. HPV subtypes 6 and 11 are most commonly associated with RRP, but type 11 appears to be more aggressive and more associated with airway obstruction, and spread into the lungs.

Some children need a tracheostomy to bypass the obstruction because the RRP growth is so aggressive. Recurrent respiratory papillomatosis is the most common benign tumor of the larynx in children.

What are the signs of recurrent respiratory papillomatosis?

The most common symptoms of recurrent respiratory papillomatosis are:

Other symptoms of recurrent respiratory papillomatosis may be mistaken for conditions such as asthma, croup, allergy, bronchitis and vocal cord nodules. They include:

How is recurrent respiratory papillomatosis treated?

Recurrent respiratory papillomatosis is treated with surgery to debride (remove) the benign growths from the airway without damaging the underlying structures, such as the vocal folds. This is done in the operating room under anesthesia. Surgical removal must be done as often as necessary to provide the child with a safe, unobstructed airway. Surgery may be required anywhere from monthly, to once every year or two. The procedure is not painful and takes approximately 30 minutes. The debriding process may be done using a variety of techniques:

Your child's surgeon will select the method that best meets your child's needs.

Children who develop recurrent respiratory papillomatosis before the age of 3 usually have frequent recurrences and will require multiple surgical debridement procedures. In some children, the RRP may become latent (growth stops) after puberty.

Will my child have to take medications?

When recurrent respiratory papillomatosis continues to occur even after repeated surgical debridements, or when it spreads to the lungs, additional therapy with medications is needed. Drugs used include cidofovir, interferon alpha and other experimental chemotherapeutic regimens. Responses to the drugs tend to vary.

Prevention: HPV vaccine

The HPV vaccine is effective in preventing the virus that causes recurrent respiratory papillomas.

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