Recurrent Respiratory Papillomatosis

Overview

What is recurrent respiratory papillomatosis?

Recurrent respiratory papillomatosis (RRP) is a condition caused by the human papillomavirus (HPV). Another name for RRP is laryngeal papilloma.

People with RRP develop wart-like tumors (papillomas) in their respiratory tract, the airways traveling from their mouth and nose to their lungs. Tumors usually grow on and around vocal cords located in your voice box (larynx). Tumors can also occasionally spread downward to the other structures of your respiratory system, such as:

Though RRP is noncancerous (benign), the tumors can grow quickly and often require surgical removal. RRP treatment can be challenging because the tumors frequently come back (recur). Some people with RRP may require multiple surgeries per year.

Who does recurrent respiratory papillomatosis affect?

Recurrent respiratory papillomatosis affects both children and adults.

  • Juvenile-onset RRP is usually diagnosed before age 5. Compared with adults, RRP in children is often more aggressive and more likely to recur after treatment.
  • Adult-onset RRP can occur at any age — usually after age 14 — and is less likely to recur.

How common is recurrent respiratory papillomatosis?

Recurrent respiratory papillomatosis is a rare disease. Estimates of how often it occurs range from 1 in 100,000 people to 4 in 100,000 people.

HPV vaccination, which began in the U.S. in 2006, is effective against RRP. HPV vaccination is for teens starting at age 11 and up to age 26. Currently, more than 50% of teens in the U.S. are vaccinated. As vaccinated teens become adults, public health experts predict a decline in HPV-related cancers and conditions (including RRP).

Symptoms and Causes

What types of HPV cause recurrent respiratory papillomatosis?

HPV is a group of more than 100 types of related viruses. HPVs cause warts on your skin and moist tissues (mucous membranes) found in your mouth, throat, vagina and anus. Because HPV spreads easily through vaginal, anal and oral sex, it’s the most common sexually transmitted disease in the U.S.

Two types of HPV (HPV 6 and HPV 11) cause RRP. These are the same types that cause most cases of genital warts. HPV also causes several types of cancer, including cervical cancer and cancer of the larynx (laryngeal). On rare occasions, it can also cause lung cancer.

HPV infection is very common. Usually, the infection resolves on its own without causing any negative health effects. Healthcare providers aren’t sure why HPV causes disease in some people but not others.

How is HPV transmitted in recurrent respiratory papillomatosis?

Healthcare providers don’t fully understand HPV transmission in RRP. In children, HPV can be passed on during childbirth if the mother has active genital warts. But only a small percentage of babies exposed to genital warts during delivery develop RRP.

RRP transmission can also occur when your baby is born via cesarean section. This suggests that HPV can be transmitted before birth, while the baby is still in their mother’s uterus.

In adults, HPV may be transmitted through oral sex. Another theory is that adults with RRP were exposed to HPV during birth. The HPV then hid in healthy cells. Something activated this “hidden” HPV, which led to RRP during adulthood.

What are the symptoms of recurrent respiratory papillomatosis?

As the tumors grow on the vocal cords and in your respiratory tract, they can cause speaking, breathing and swallowing problems. The most common symptoms of RRP include:

Your healthcare provider will evaluate you or your child carefully to distinguish RRP from other similar conditions, including asthma and allergies.

Diagnosis and Tests

How is recurrent respiratory papillomatosis diagnosed?

Your healthcare provider assesses your or your child’s symptoms and does a physical examination. They may place a mirror and bright light at the back of your throat to look at your vocal cords. If your symptoms and exam suggest RRP, your healthcare provider will refer you to an ear, nose and throat (ENT) specialist.

What tests are used to diagnose recurrent respiratory papillomatosis?

Your ENT specialist may perform a laryngoscopy. This procedure uses an endoscope (a thin, flexible tube with a small camera and light) to view your throat and larynx. The ENT specialist:

  1. Numbs the inside of your or your child’s nose and throat.
  2. Threads the scope into your nose and down the back of your throat to your voice box.
  3. Examines your respiratory tract for tumors that look like bumps or tiny bunches of grapes.

To confirm an RRP diagnosis, an ENT specialist may need to do a biopsy to collect a small tissue sample for HPV testing. This procedure is similar to a laryngoscopy but happens in an operating room under general anesthesia.

The ENT specialist uses an endoscope with special tools to collect a tissue sample. A lab analyzes the tissue to confirm the presence and type of HPV.

On rare occasions, a lung specialist may need to look inside of your windpipe in a similar fashion to collect a sample.

Management and Treatment

Can recurrent respiratory papillomatosis be cured?

RRP has no cure. The goal of treatment is to remove the growths and keep them from coming back. You or your child may need a combination of surgery and medication.

How is surgery used to treat recurrent respiratory papillomatosis?

The primary treatment for recurrent respiratory papillomatosis is surgery. Your healthcare provider removes as many of the growths as possible without harming healthy tissue. Techniques for surgical removal include:

  • Laser surgery: This treatment uses beams of light to shrink or destroy tumors.
  • Microdebrider: This advanced tool uses gentle suction to pull the tumor toward a specialized, precise blade, which quickly and efficiently removes it.
  • Cryoablation: During cryoablation, healthcare providers apply freezing temperatures to kill tumor cells.

Most RRP surgeries, especially in children, occur under general anesthesia in the operating room. You’re asleep during the surgery.

Office-based laser surgery is an option for some adults. In-office procedures are faster and eliminate the need for general anesthesia. You receive local anesthesia to keep you comfortable, but are awake during the procedure.

How often will my child or I need surgery for RRP?

After surgery, RRP tumors often return. Some people need retreatment every few weeks. Others may go a year or more between treatments. Recurrence is more common in children.

How do medications treat recurrent respiratory papillomatosis?

Medications can treat RRP in people who have:

  • More than four RRP surgeries per year.
  • Rapid tumor regrowth.
  • Difficulty breathing due to tumors blocking their airway.
  • Tumors that have spread to multiple locations.

These medications are called adjuvant therapies because they’re used after the primary treatment to help prevent the return of tumors. About 20% of people with RRP (1 in 5) require adjuvant therapy.

RRP adjuvant medications use different processes to reduce tumor growth:

Can the HPV vaccine help treat recurrent respiratory papillomatosis?

Researchers have found that the HPV vaccine may also be an effective adjuvant treatment option. Early studies show that HPV vaccination slows tumor growth and decreases the frequency of surgeries.

What are the complications of recurrent respiratory papillomatosis?

In severe RRP, your airway can become blocked. To provide air to the lungs, you or your child may need a tracheostomy, which is a hole through your neck into your trachea. A tracheostomy affects your ability to speak. Your healthcare team will help you care for the tracheostomy and learn how to use your voice.

Cancer is another complication that can happen. Though rare, RRP can transform from a benign disease to (malignant) cancer. Transformation can occur many years after diagnosis and is more likely if the tumors have spread to lower parts of your respiratory tract. Healthcare providers aren’t sure why RRP becomes cancerous.

Prevention

How can I prevent recurrent respiratory papillomatosis?

The HPV vaccine is effective at preventing RRP. The Centers for Disease Control and Prevention (CDC) recommends HPV vaccination for all children at age 11 or 12. People who weren’t vaccinated as teens can receive the vaccine up to age 26.

Condoms can also prevent RRP by lowering your risk of getting HPV through sex. But condoms don’t protect against HPV if the genital warts are on areas where condoms can’t cover.

If you have genital warts and are pregnant or thinking about becoming pregnant, talk to your healthcare provider. Overall, the risk of transmission from you to your baby is low. If you and your healthcare provider decide to treat genital warts during pregnancy, several safe treatment options are available.

Outlook / Prognosis

What is the prognosis for people with recurrent respiratory papillomatosis?

The outlook for most children and adults with RRP is good. Surgery and medication therapy can effectively manage tumors, so they don’t interfere with daily activities. Rarely, RRP will require a tracheostomy or become cancerous.

Living With

How do I live with recurrent respiratory papillomatosis?

If you or your child has RRP, your healthcare provider will monitor you closely and develop a treatment plan. Follow their instructions and attend your appointments. By doing so, you can manage the condition and prevent a life-threatening airway blockage. If you’re having trouble breathing, call your provider or 911.

A note from Cleveland Clinic

Recurrent respiratory papillomatosis (RRP) is a disease caused by the human papillomavirus (HPV). Healthcare providers manage RRP with surgery, adding medication therapy for severe RRP. Frequent surgeries can be physically and emotionally challenging, especially for children. If you need support, talk to your healthcare provider. They can connect you with resources for people living with chronic illnesses.

Resources

Last reviewed by a Cleveland Clinic medical professional on 04/25/2022.

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