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Recurrent respiratory papillomatosis
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Recurrent respiratory papillomatosis

Contributors: Paul C. Bryson MD
Other Resources UpToDate PubMed

Synopsis

Recurrent respiratory papillomatosis (also known as laryngeal papillomatosis, or LP) is a rare type of respiratory papillomatosis that presents with small, wart-like, noncancerous epithelial tumors of the laryngeal mucosa. These papillomas vary in size and growth rates. Recurrence is the rule. Recurrent respiratory papillomatosis has juvenile-onset and adult-onset forms. Childhood cases typically develop before age 12 and are more aggressive, as these cases are more likely to recur and require surgical treatment given the smaller pediatric airways. Although aggressive cases are less common in adults than in children, they can still occur.

Recurrent respiratory papillomatosis is caused by human papillomavirus (HPV) strains 6 and 11. HPV is spread through sexual contact or when a mother with HPV genital warts passes it onto her baby via childbirth.

Clinical signs and symptoms depend on the patient and the severity of recurrent respiratory papillomatosis. Recurrent respiratory papillomatosis most commonly causes a hoarse voice that may be low-pitched or sound strained. Patients may have labored breathing due to airway obstruction. Some patients may have difficulty speaking or may be unable to speak. Infants may have a weak cry, failure to thrive, and episodes of choking. Other clinical symptoms include chronic cough, difficulty swallowing, dyspnea, foreign body sensation, and choking episodes.

In some cases, the disease may go away on its own without requiring treatment or it may only require a few surgical procedures during a patient's lifetime. Complications may occur if left untreated, as airway may be compromised in life-threatening acute respiratory distress. Very rarely, recurrent respiratory papillomatosis may spread to the lungs and patients may experience recurrent pneumonia, chronic lung disease, and, in severe cases, progressive pulmonary failure. More rarely, papillomas become cancerous and develop into squamous cell carcinoma.

There is currently no cure for recurrent respiratory papillomatosis. Treatment usually involves removal of papillomas, reducing the spread of the disease, preventing airway obstruction, and if necessary, improving voice quality. Treatment procedures and interventions vary depending on specific location and size of papillomas, frequency of recurrence, and other factors. Treatment usually requires a multidisciplinary team to create a comprehensive plan individualized to the patient. A number of adjuvant therapies have been tried, all with varying success.

Codes

ICD10CM:
D14.1 – Benign neoplasm of larynx

SNOMEDCT:
232457008 – Laryngeal papillomatosis
472827002 – Recurrent respiratory papillomatosis

Differential Diagnosis & Pitfalls

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Last Updated:04/07/2021
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Recurrent respiratory papillomatosis
Recurrent respiratory papillomatosis (Adult) : Choking sensation, Hoarseness, Non-productive cough, Dysphonia, Dyspnea, Wheezing, Stridor
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