Recurrent respiratory papillomatosis

Other Names: RRP; Juvenile laryngeal papilloma; Laryngeal papilloma, recurrent; Juvenile-onset recurrent respiratory papillomatosis (type); JORRP (type); Adult-onset recurrent respiratory papillomatosis (type); AORRP (type); Respiratory papillomatosis, recurrent

Recurrent respiratory papillomatosis (RRP) 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, they most commonly grow in the larynx (voice box)—a condition called laryngeal papillomatosis. The papillomas may vary in size and grow very quickly. They often grow back after they have been removed.

Epidemiology

It is estimated that approximately 4 in 100,000 children and 2 in 100,000 adults develop recurrent respiratory papillomatosis in the United States. The incidence of this condition may be decreasing with increased use of the HPV vaccine.

Forms

There are two types, a juvenile-onset form and an adult-onset form.

Cause

• RRP is caused by two types of human papilloma virus (HPV): HPV 6 and HPV 11. There are more than 150 types of HPV, and they do not all have the same symptoms.
• Most people who encounter HPV never develop a related illness. However, in a small number of people exposed to the HPV 6 or 11 virus, respiratory tract papillomas and genital warts can form. Although scientists do not fully understand why some people develop the disease and others do not, the virus is thought to be spread through sexual contact or when a mother with genital warts passes the HPV 6 or 11 virus to her baby during childbirth.

Signs and symptoms

The following list includes the most common signs and symptoms in people with recurrent respiratory papillomatosis (RRP). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list also does not include every symptom or feature that has been described in this condition.

Symptoms may include:

• Growth of benign (non-cancerous) tumors in the airway
• Voice hoarseness (dysphonia)
• Shortness of breath (dyspnea)
• Chronic cough

Recurrent respiratory papillomatosis (RRP) is a chronic disease, meaning it is a long-term or persistent condition. It can occur in childhood, before age 12, or in adulthood, usually between ages 20-40. Earlier age at onset is associated with more severe symptoms. RRP is very unpredictable. In some people, RRP goes away on its own, while in others, it causes severe, recurring disease. Very rarely, the tumors in RRP can become cancerous and spread.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Hoarse voice(Hoarseness)

30%-79% of people have these symptoms

• Dysphonia(Inability to produce voice sounds)
• Respiratory distress(Breathing difficulties)

5%-29% of people have these symptoms

• Dysphagia(Poor swallowing)
• Failure to thrive(Faltering weight)
• Hemoptysis(Coughing up blood)
• Nonproductive cough(Dry cough)
• Recurrent pneumonia
• Recurrent upper respiratory tract infections(Recurrent colds)
• Respiratory insufficiency(Respiratory impairment)
• Stridor
• Tachypnea(Increased respiratory rate or depth of breathing)
• Upper airway obstruction
• Wheezing

1%-4% of people have these symptoms

• Atelectasis(Partial or complete collapse of part or entire lung)
• Choking episodes
• Fever
• Squamous cell carcinoma
• Syncope(Fainting spell)
• Tracheomalacia(Floppy windpipe)

Diagnosis

Health professionals use two routine tests for RRP: indirect and direct laryngoscopy. In an indirect laryngoscopy, an otolaryngologist—a doctor who specializes in diseases of the ear, nose, throat, head, and neck—or speech-language pathologist will typically insert a fiberoptic telescope, called an endoscope, into a patient’s nose or mouth and then view the larynx on a monitor. Some medical professionals use a video camera attached to this endoscope to view and record the exam. An older, less common method is for the otolaryngologist to place a small mirror in the back of the throat and angle the mirror down toward the larynx to inspect it for papillomas.

A direct laryngoscopy is conducted in the operating room with the use of general anesthesia. This method allows the otolaryngologist to view the vocal folds and other parts of the larynx under high magnification. This procedure is usually used to minimize discomfort, especially with children, or to enable the doctor to biopsy tissue samples from the larynx or other parts of the throat to obtain a diagnosis of RRP.

Treatment

• Vaccination with the HPV vaccine could prevent the development of RRP. The CDC currently recommends that all children (both boys and girls) receive the HPV vaccine at age 11 or 12. Ask your child’s doctor whether the type of HPV vaccine your child will receive will protect against HPV 6 and 11. As more young people receive the vaccine, future research will reveal its effectiveness in preventing HPV-associated diseases such as RRP.
• Once RRP develops, there is currently no cure. Surgery is the primary method for removing tumors from the larynx or airway. Because traditional surgery can cause problems due to scarring of the larynx tissue, many surgeons now use laser surgery. Carbon dioxide (CO2) or potassium titanyl phosphate (KTP) lasers are frequently used for this purpose. Surgeons also commonly use a device called a microdebrider, which uses suction to hold the tumor in place while a small internal rotary blade removes the growth.
• Once the tumors have been removed, they can still return. It is common for patients to require multiple surgeries. With some patients, surgery may be required every few weeks in order to keep the breathing passage open, while others may require surgery only once a year or even less frequently.
• In the most extreme cases of aggressive tumor growth, a tracheotomy may be performed. A tracheotomy is a surgical procedure in which an incision is made in the front of the patient’s neck and a breathing tube (trach tube) is inserted through an opening, called a stoma, into the trachea (windpipe). Rather than breathing through the nose and mouth, the patient will now breathe through the trach tube. Although the trach tube keeps the breathing passage open, doctors try to remove it as soon as possible.
• Some patients may be required to keep a trach tube indefinitely in order to keep the breathing passage open. Because the trach tube re-routes all or some of the exhaled air away from the vocal folds, the patient may find it difficult to use his or her voice. With the help of a voice specialist or speech-language pathologist who specializes in voice, the patient can learn to use his or her voice with the use of a speaking valve.
• In severe cases of RRP, therapies in addition to surgery may be used. Drug treatments may include antivirals such as interferon and cidofovir, which block the virus from making copies of itself; indole-3-carbinol, a cancer-fighting compound found in cruciferous vegetables such as broccoli and brussels sprouts; or bevacizumab, which targets the blood vessel growth of papilloma. To date, the results of these and other nonsurgical therapies have been mixed or not yet fully proven.

NIH genetic and rare disease info

• NIH info on Recurrent respiratory papillomatosis

Recurrent respiratory papillomatosis is a rare disease.

Recurrent respiratory papillomatosis Resources
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