Laryngeal papillomatosis overview
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Differentiating Laryngeal papillomatosis from other Diseases |
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Diagnosis |
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Treatment |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Laryngeal papillomatosis, also known as recurrent respiratory papillomatosis, is a rare medical condition (2 per 100,000 adults and 4.5 per 100,000 children), caused by an HPV infection of the throat. It causes assorted tumors, papillomas, to develop in the air passages leading from the nose and mouth into the lungs (respiratory tract) over a period of time. The tumors may vary in size and grow very quickly. They often grow back even when removed. Without treatment it is potentially fatal as uncontrolled growths could obstruct the airway. Laryngeal papillomatosis is caused by HPV types 6 and 11, in which benign tumors form on the larynx or other areas of the respiratory tract. These tumors can recur frequently, may require repetitive surgery, and may interfere with breathing. The disease can be treated with surgery and antivirals.
Pathophysiology
In general, doctors are not sure what causes some people to contract laryngeal papillomatosis while others who have been exposed to HPV types 6 and 11 do not contract the disease. Since the disease is most commonly found in children the disease may be caused by an infant contracting HPV from the mother during vaginal child birth. There is no evidence that it is transmitted through oral sex, and it is not considered a sexually transmitted disease. Laryngeal papillomatosis affects adults as well as infants and small children who may have contracted the virus during childbirth.
Causes
Laryngeal papillomatosis is caused by two types of human papilloma virus (HPV), called HPV 6 and HPV 11. There are more than 150 types of HPV and they do not all have the same symptoms.
Differentiating Laryngeal papillomatosis from other Diseases
Laryngeal papillomatosis is most often misdiagnosed as asthma, croup, or chronic bronchitis.
Epidemiology and Demographics
According to the Centers for Disease Control and Prevention, the incidence of laryngeal papillomatosis is rare. Fewer than 2,000 children get laryngeal papillomatosis each year. According to the RRP (Recurrent Respiratory Papillomatosis) Foundation, there are roughly 20,000 cases in the United States. Among children, the incidence of RRP is approximately 4.3 per 100,000; among adults, it’s about 1.8 per 100,000.
Natural History, Complications and Prognosis
The complications of laryngeal papillomatosis may be serious, as papillomas are at least partially obstructing the airway to cause these symptoms and should be removed immediately. Regardless of the treatment used, the tumors will reoccur. In severe cases, tumors may occur once or twice a month. In less severe cases, tumors may occur once or twice a year. Some children experience some relief or remission of the disease when they begin puberty.
Diagnosis
History and Symptoms
Normally, vocal sounds are produced when air from the lungs is pushed past two side-by-side elastic muscles—called vocal folds or vocal cords—with sufficient pressure to cause them to vibrate. When the tumors interfere with the normal vibrations of the vocal folds, it causes hoarseness, which is the most common symptom of laryngeal papillomatosis. Eventually, the tumors may block the airway passage and cause difficulty breathing.
Treatment
Medical Therapy
Adjuvant therapies (therapies that are used in addition to surgery) have been used to treat more severe cases of laryngeal papillomatosis. 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. Some side effects of antivirals include dizziness, headaches, and body aches.
Future or Investigational Therapies
Scientists and clinicians are working to discover more about laryngeal papillomatosis. While HPV 6 and HPV 11 are known causes, millions of people are exposed to these two viruses without developing the disease. It is not known why some people are more at risk than others or why some cases are much more serious than others.
Researchers funded by the National Institute on Deafness and Other Communication Disorders (NIDCD) are exploring how our genes and immune system may contribute to our risk for contracting the disease as well as the severity of its symptoms. Researchers are also studying other possible therapies for laryngeal papillomatosis. In one clinical trial, researchers are investigating whether a common anti-inflammatory drug can inhibit an enzyme that promotes tumor growth in laryngeal papillomatosis, thus reducing the recurrence of tumors in children and adults with laryngeal papillomatosis. Researchers are also investigating the effectiveness of a pulsed dye laser, a laser that delivers short intense pulses of light, to determine if it is more effective at preserving the surrounding tissue while removing tumors from vocal folds. In one NIDCD- sponsored clinical investigation, researchers are studying whether a certain dietary supplement can work with the pulsed dye laser to decrease the rate of recurrence of tumors in children with laryngeal papillomatosis. To read about these and other clinical trials, go to Pulsed Dye Laser for Juvenile Onset Recurrent Respiratory Papillomatosis.