Recurrent respiratory papillomatosis

December 31, 2014

Synonyms

Overview

Recurrent respiratory papillomatosis is a rare viral disease where tumors (papillomas) grow in the air passages leading from the nose and mouth into the lungs (respiratory tract). Although the tumors can grow anywhere in the respiratory tract, their presence in the larynx (voice box) causes the most frequent problems, a condition called laryngeal papillomatosis. The tumors may vary in size and grow very quickly. They often grow back even when removed. RRP is caused by two types of human papillomavirus (HPV), called HPV 6 and HPV 11. These tumors can recur frequently, may require repetitive surgery, and may interfere with breathing. The disease can be treated with surgery and antivirals.

Symptoms

Symptoms usually begin with hoarseness and/or a change in the voice. Some people may experience difficulty breathing (dyspnea) and/or experience other life-threatening complications if the papillomas block the airway. The tumors may vary in size and grow very quickly. They often grow back even when removed.

Adults:
In adults, the symptoms of laryngeal papillomatosis are hoarseness, or a strained or breathy voice. Size and placement of the tumors dictate the change in the person's voice. Breathing difficulties may occur but more commonly are found in children.

Children:
In babies and small children, the signs and symptoms include a weak cry, trouble swallowing, noisy breathing, and chronic cough. Noisy breathing may be a stridor, which can sound like a whistle or a snore, and is a sign that the laryngeal or tracheal parts of the airway are narrowing.

Symptoms:

Breathing difficulty
Respiratory obstruction
Swallowing difficulty
Hoarseness
Chronic coughing
Dysphagia
Voice changes
Rapid breathing

Causes

Laryngeal papillomatosis is caused by two types of human papilloma virus (HPV), called HPV 6 and HPV 11.

Diagnosis

A physician may diagnose laryngeal papillomatosis by placing a mirror into a patient's mouth to reflect light onto the vocal cords and examining the larynx. More often, a doctor or a trained speech-language pathologist diagnoses laryngeal papillomatosis by an indirect laryngoscopy in the office. This procedure involves the placement of a flexible, fiber optic camera through the patient's nose to view the vocal folds in the throat or the use of a straight, rigid camera placed through the mouth to view the vocal folds.

The most accurate way to diagnose laryngeal papillomatosis is for a biopsy to be conducted and for the lesion to be tested for HPV. This procedure takes place in an operating room with the patient under general anesthesia. This is sometimes the best option for small children. This disease is most often misdiagnosed as asthma, croup, or chronic bronchitis. The consequences may be serious, as papillomas are at least partially obstructing the airway to cause these symptoms and should be removed immediately.

Treatment

There is no cure for recurrent respiratory papillomatosis. Surgery is the primary method for removing tumors from the larynx or airway. Because traditional surgery can result in problems due to scarring of the larynx tissue, many surgeons are now using laser surgery, which uses an intense laser light as the surgical tool. Carbon dioxide lasers—which pass electricity through a tube containing carbon dioxide and other gases to generate light—are currently the most popular type used for this purpose. In the past 10 years, surgeons have begun using a device called a microdebrider, which uses suction to hold the tumor while a small internal rotary blade removes the growth.

Once the tumors have been removed, they have a tendency to return. It is common for affected individuals to require repeat surgery. In the most extreme cases where tumor growth is aggressive, a tracheostomy may be performed.

Adjuvant therapies—therapies that are used in addition to surgery—have been used to treat more severe cases of RRP. Drug treatments may include antivirals such as interferon and cidofovir, which block the virus from making copies of itself, and indole-3-carbinol, a cancer-fighting compound found in cruciferous vegetables, such as broccoli and Brussels sprouts. To date, the results of these and other adjuvant therapies have been mixed or not yet fully proven.