Management of Recurrent Respiratory Papillomatosis
Surgery is necessary in the management of recurrent respiratory papillomatosis, with surgical removal using contemporary laryngeal instruments including laser and microdebrider to prevent airway obstruction and reduce dysphonia symptoms. 1
Disease Overview
Recurrent respiratory papillomatosis (RRP) is a benign but aggressive neoplasm of the upper airway characterized by multiple papillomas that cause obstructive symptoms such as shortness of breath, hoarseness, or stridor. The disease is caused by human papillomavirus (HPV) infection, with genotypes 6 and 11 present in 90-95% of cases 1.
RRP is classified into two forms:
- Juvenile-onset RRP (JORRP): onset before age 18 years, believed to result from vertical transmission of HPV from mother to baby during delivery
- Adult-onset RRP: less well characterized than the juvenile form
The incidence of both forms is estimated at 3-7 per 100,000 individuals 1. JORRP is associated with extensive morbidity, requiring a median of 13 lifetime surgeries to maintain an open airway 1.
Management Algorithm
First-line Treatment: Surgical Management
- Surgical removal is the primary treatment modality 1, 2
- Contemporary surgical techniques include:
- Laser therapy (photoangiolytic lasers)
- Microdebrider excision
- Can be performed in office setting in some cases 2
Important Surgical Considerations
- Every effort must be made to avoid injury to the underlying vibratory layers of the vocal folds to prevent long-term dysphonia related to scar formation 1
- Surgery is unlikely to be curative since HPV is present in adjacent normal-appearing mucosa 1
- The goal is to maintain airway patency and improve voice quality
Adjuvant Therapies
When surgical management alone is insufficient (aggressive disease), adjuvant therapies may be considered:
Immunomodulatory Agents:
Antiviral Medications:
- Cidofovir
- Acyclovir
- Ribavirin 4
Other Pharmacologic Options:
Prevention Strategies
HPV Vaccination
- HPV vaccination is strongly recommended for patients with RRP aged 9-26 years 1
- Quadrivalent or nonavalent vaccine is preferred given the relationship of RRP to HPV types 6 and 11 1
- Preliminary data suggest HPV vaccines may prolong time to recurrence in RRP patients 2
- Compassionate use may be considered in juvenile patients under the indicated age in the product characteristics 1
Prevention of Vertical Transmission
- Cesarean delivery is NOT recommended solely to prevent HPV transmission 6
- Cesarean delivery is indicated only when genital warts obstruct the pelvic outlet or when vaginal delivery would result in excessive bleeding 6
- Pregnant women with genital warts should be counseled about the low risk of laryngeal papillomatosis in their infants 6
Clinical Course and Follow-up
- RRP is a chronic disease with an unpredictable course
- Disease may become more aggressive in the setting of immune suppression 2
- Regular follow-up with laryngoscopy is essential to monitor for recurrence
- Frequency of follow-up depends on disease aggressiveness
- Monitor for rare but serious complications:
- Airway obstruction
- Pulmonary spread
- Malignant transformation
Future Directions
- Widespread HPV vaccination could potentially reduce RRP incidence by preventing vertical HPV transmission to newborns 2
- Research into immunomodulatory approaches is ongoing 2, 7
- Novel surgical instrumentation is being developed to minimize long-term stenotic complications 7
The management of RRP remains challenging due to its recurrent nature, but a combination of surgical intervention with appropriate adjuvant therapy when indicated, along with preventive HPV vaccination, offers the best approach to control this disease and improve quality of life.