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Recurrent respiratory papillomatosis
Other Resources UpToDate PubMed

Recurrent respiratory papillomatosis

Contributors: Sahil Patel, Paul C. Bryson MD, MBA
Other Resources UpToDate PubMed

Synopsis

Recurrent respiratory papillomatosis (also known as laryngeal papillomatosis [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. 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 from vertical transmission when a mother with HPV genital warts passes it onto her baby during 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 the 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

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Laryngeal carcinoma
  • Laryngeal dysplasia
  • Vocal cord leukoplakia – Typically presents as white plaques on the vocal folds; can be ruled out based on histopathology.
  • Vocal cord granuloma – Usually attributed to endotracheal intubation, vocal abuse, or gastroesophageal reflux disease. Typically seen in the vocal process region.

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Therapy

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References

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Last Reviewed:05/13/2021
Last Updated:06/07/2021
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Recurrent respiratory papillomatosis
A medical illustration showing key findings of Recurrent respiratory papillomatosis (Adult) : Choking sensation, Hoarseness, Non-productive cough, Dysphonia, Dyspnea, Wheezing, Stridor
Copyright © 2022 VisualDx®. All rights reserved.