Specialty otolaryngology ICD-9-CM 212.1 | ICD-10 D14.1 | |
Laryngeal papillomatosis, also known as recurrent respiratory papillomatosis or glottal papillomatosis or associated with condyloma acuminata, is a rare medical condition (2 per 100,000 adults and 4.5 per 100,000 children), caused by a human papillomavirus (HPV) infection of the throat. Laryngeal papillomatosis causes assorted tumors or papillomas to develop over a period of time. 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. In addition antiangiogenic therapy shows promising results.
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Transmission
In general, physicians are not sure what causes certain people to develop laryngeal papillomatosis while others who have been exposed to HPV types 6 and 11 do not develop 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.
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.
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
Traditional surgery and carbon dioxide laser surgery, a "no touch" removal of affected tissue, are forms of treatment for laryngeal papillomatosis. Carbon dioxide laser removal is the most common removal method. The carbon dioxide laser must be used precisely to prevent scarring, fibrosis, and laryngeal web malformation. In children, carbon dioxide laser is effective for removing papillomas on the larynx. Photodynamic therapy controls tumors by using targeted dyes and bright light to illuminate tumors. In this procedure, a physician injects a light-sensitive dye that is only absorbed by the tumors. Then the physician activates the dye using a bright light, and the tumors are eliminated. This procedure has also been able to decrease the number of tumors that reoccur.
Another method is tracheotomy, which reroutes air around the affected area. An incision is made in the front of the patient's neck, and a breathing tube is inserted through a hole (stoma) into the windpipe. The patient is then able to breathe through the tube. Although this is usually temporary, some patients must use the tube indefinitely. This method should be avoided if at all possible, since insertion of a breathing tube may cause the tumors to form as far down as the lungs.
Many antiviral drugs like cidofovir have been used to treat laryngeal papillomatosis, but none completely stops the tumors from growing. Most antivirals are injected to control the frequency of tumor growth. The efficacy of the same is debated and subject to research. Some side effects of antivirals include dizziness, headaches, and body aches. Adjuvant chemotherapy with interferon may be used in very severe cases. Regardless of the treatment used, the tumors will recur. In severe cases, tumors may occur once or twice a month. In less severe cases, tumors may occur once or twice a year. In addition, speech therapy may be beneficial to assist with vocal hygiene and retraining of voice.
Antiangiogenic therapy: A strong mRNA expression of VEGF-A and its receptors,VEGFR-1 and -2 has been observed in tissue samples of RRP patients. The most promising adjuvant treatment approach seems to be the topical or systemic treatment with the anti-vascular endothelial growth factor (VEGF) antibody bevacizumab. In a small series of RRP patients the systemic antiangiogenic treatment showed rapid response and a significant reduction in the number of needed interventions.