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Recurrent respiratory papillomatosis (RRP)
RRP is a disease of benign warts in the airway caused by HPV types 6 and 11; it recurs again and again after removal, so treatment is repeated surgical removal that spares normal tissue, with adjuvant treatments in severe disease — and the aim is an open airway and a useful voice, not a "cure" in one operation.
Why it matters in the exam
There are two forms: in children (juvenile onset) — transmission from the mother at birth, commoner in a first-born child, with vaginal delivery and a young mother; and in adults (adult onset). The first symptom in a child is progressive hoarseness, then stridor and breathlessness — sometimes misdiagnosed as asthma or croup. HPV-11 is linked to more aggressive disease. Lesions tend to appear where ciliated epithelium meets squamous epithelium: at the glottis, the laryngeal ventricle and below the vocal folds.
Surgical treatment: removal with a microdebrider (preferred in children), CO2 laser or an angiolytic laser (KTP), sparing normal mucosa to prevent scarring and a web at the anterior commissure. Tracheostomy is avoided where possible, because it can spread the disease into the trachea. Adjuvant treatments when frequent operations are needed or the trachea or lungs are involved: systemic bevacizumab (anti-VEGF), which has become the main adjuvant; intralesional cidofovir is used less and less. HPV vaccination prevents the disease (vaccinating the mother before pregnancy) and probably also reduces recurrences when given as an adjuvant.
Malignant change to squamous cell carcinoma is rare (about 1% to 3%) and commoner with HPV-11, smoking and lung involvement. Pulmonary involvement shows as nodules and cavities on chest CT and indicates a poor prognosis. Severity is scored with the Derkay system, used for follow-up and comparison between operations. In a child with persistent hoarseness the larynx is always examined — "asthma with hoarseness" is not a diagnosis.
Key points
- HPV-6 and 11; 11 more aggressive.
- Children: hoarseness, then stridor.
- Repeated removal that spares mucosa.
- Bevacizumab and HPV vaccine as adjuvants.
- Malignancy rare, mainly with lungs and smoking.
Summary table
| Aspect | Detail | Meaning |
|---|---|---|
| Cause | HPV-6 and HPV-11 | HPV-11 more aggressive |
| Juvenile form | Hoarseness, then stridor | Transmission at birth |
| Site | Junction of ciliated and squamous epithelium | Glottis, ventricle, subglottis |
| Surgery | Microdebrider, CO2 or KTP laser | Spare normal mucosa, avoid tracheostomy |
| Adjuvant | Bevacizumab, HPV vaccine | In severe or pulmonary disease |
| Malignancy | About 1% to 3% | HPV-11, smoking, lungs |
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Sources
- Cummings Otolaryngology: Head and Neck Surgery, 7th edition (2021) — Recurrent respiratory papillomatosis
- RRP Task Force / international consensus on adjuvant therapy in recurrent respiratory papillomatosis, 2023 — Bevacizumab and HPV vaccination
- Derkay CS et al. A staging system for assessing severity of disease and response to therapy in RRP, 1998 — Staging score
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.