Recurrent respiratory papillomatosis
Benign wart-like growths caused by HPV, mostly on the vocal cords, that come back again and again and cause hoarseness and breathing problems.
Quick facts
- Key signs
- Progressive hoarseness · Weak cry in infants · Stridor
- Diagnosis
- Biopsy with histology
Red flags ↓
No doses: the clinician sets them by the current guideline.
What it is
Recurrent respiratory papillomatosis (laryngeal papillomatosis) is the growth of benign squamous papillomas in the airway, mostly in the larynx, caused by low-risk human papillomavirus types 6 and 11. It is the most common benign laryngeal tumour of children and tends to recur after removal.
Who gets it
Rare, with a bimodal age pattern: a juvenile form, usually diagnosed before 5 years, and an adult form peaking between 20 and 40 years. The juvenile form is usually more aggressive. Widespread HPV vaccination is expected to reduce cases, as seen in countries with high vaccine coverage.
Causes
- Infection with HPV types 6 and 11 (rarely higher-risk types)
- Juvenile form: transmission from mother to child during vaginal birth, especially with maternal genital warts
- Adult form: probably sexual transmission or reactivation of a latent infection
Risk factors
- Maternal genital warts during pregnancy
- First-born child and vaginal delivery (prolonged exposure in the birth canal)
- Young maternal age
- In adults: multiple sexual partners and oral sex
- Lack of HPV vaccination
Pathophysiology
HPV infects basal cells of the airway epithelium, especially where ciliated and squamous epithelium meet (the vocal folds, the laryngeal ventricle, the subglottis). The virus stays latent in normal-looking mucosa around the lesions, which explains recurrence after removal. Papillomas narrow the glottis and can spread to the trachea, bronchi and, rarely, the lungs.
Classification
- Juvenile-onset (aggressive, frequent surgeries) and adult-onset
- By extent: laryngeal only, or spreading to the trachea, bronchi or lungs
- Severity scores (such as the Derkay staging) record extent and guide follow-up
Symptoms and signs
- Progressive hoarseness, the main symptom
- Weak cry in infants
- Stridor, first inspiratory and then biphasic
- Chronic cough and shortness of breath
- Recurrent respiratory infections and, in severe cases, failure to thrive
Diagnosis
- Clinical
- Any persistent hoarseness in a child or adult deserves examination of the larynx. Flexible laryngoscopy shows exophytic, cauliflower-like lesions.
- Tests
- Direct laryngoscopy and bronchoscopy under anaesthesia map the extent; biopsy with histology confirms squamous papilloma and HPV typing identifies the virus. Pulmonary function tests may show upper airway obstruction.
- Imaging
- Chest CT when tracheal, bronchial or lung spread is suspected (nodules or cavities).
Differential diagnosis
- Vocal fold nodules, polyps and cysts
- Laryngeal cancer in adults
- Asthma and croup (misdiagnosis in children)
- Subglottic stenosis or haemangioma
- Vocal fold paralysis
Goal of treatment
Keep the airway open and the voice useful by removing papillomas while preserving normal tissue, reduce how often surgery is needed, and watch for malignant change.
Treatment
- Surgical
- Repeated endoscopic surgical removal (microdebrider, cold instruments or laser), aiming to debulk the lesions while protecting healthy tissue to avoid scarring and webs.
- Medical
- Adjuvant medical therapy for frequent recurrence or spread, such as intralesional antivirals or systemic anti-angiogenic agents (for example bevacizumab), decided in specialised centres; HPV vaccination may be offered as an adjuvant.
- Follow-up
- Long-term follow-up with laryngoscopy and voice assessment, and monitoring for lung spread or malignant transformation.
- Acute
- Urgent ENT evaluation for stridor or respiratory distress; tracheostomy is avoided when possible because it may favour spread down the airway.
Complications
- Airway obstruction
- Scarring, glottic webs and permanent voice changes from repeated surgery
- Spread to the trachea, bronchi and lungs
- Malignant transformation to squamous cell carcinoma (rare)
- Psychological and social burden of many operations
Prognosis
The course is unpredictable; some patients go into long remission, while others, especially young children, need many procedures. Mortality comes mainly from airway obstruction, lung spread or cancer. HPV vaccination is the main hope for prevention.
Prevention
- HPV vaccination of adolescents before sexual activity
- Diagnosis and treatment of genital warts
Test yourself
Think of the answer, then tap the question to see it.
Which HPV types cause recurrent respiratory papillomatosis?
Low-risk types 6 and 11.
How is the juvenile form usually acquired?
Vertical transmission from the mother during vaginal birth.
What is the main presenting symptom?
Progressive hoarseness (weak cry in infants).
Why do papillomas recur after removal?
HPV remains latent in the normal-appearing mucosa around the lesions.
Which public health measure is expected to reduce cases?
HPV vaccination.
Why it happens
In ENT
Connections
Educational information, not medical advice. For any symptom, see a health professional.
Sources: Laryngeal Papillomas (StatPearls (NCBI Bookshelf), 2023).
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Representative model, for educational purposes only.