Recurrent Respiratory Papillomatosis Follow-up
- Author: Eloise M Harman, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP more...
Further Outpatient Care
RRP is devastating to individuals and families. Children with JORRP may require repeated surgery and may be ill for a long time. The RRP Foundation may provide group support for individuals and families.
Inpatient & Outpatient Medications
The primary treatment involves surgery and intralesional therapy at the time of surgery. Six months of subcutaneous interferon therapy frequently is beneficial.
Because RRP is a rare disease, consider transfer to a center with personnel experienced in its management. Patients may receive the best care in a tertiary center.
In 2006, a vaccine was released that protects against HPV types 6, 11 (associated with venereal warts), 16, and 18 (associated with cervical cancer). Administration to girls before they become sexually active is expected to be highly effective in preventing HPV infection and resultant venereal warts and would be expected to lead to a future reduction in JORRP incidence.
The role of cesarean delivery in the prevention of JORRP is controversial because the disease is quite uncommon, despite the frequency of genital HPV infection. Consider cesarean delivery in a young woman with visible condylomata who is giving birth to her first child.
Complications of this disease include airway obstruction and malignant transformation.
With regard to tracheostomy, older literature suggests that in RRP patients, it may promote distal airway spread (ie, distal to the larynx) of papillomas. A more recent review suggests that patients who require tracheostomy tend to present at a younger age with more severe disease that already involves the more distal airway. Distal spread after a tracheostomy most commonly involves the tracheostomy site. After laser and antiviral treatment over a period of years, the tracheostomy often can be removed successfully; therefore, tracheostomy is a reasonable option if required due to significant airway obstruction. The need for tracheostomy probably is a marker of more severe disease rather than an independent cause of distal spread.
Malignant degeneration of papillomatous lesions to squamous cell carcinoma occurs in 3-5% of patients with RRP. Distal airway spread of papillomas often is a forewarning of malignant degeneration. The site of malignancy in JORRP usually is the bronchial or pulmonary parenchyma, whereas the larynx is the usual site in AORRP. Malignant degeneration is more common with disease caused by HPV-11 and HPV-16. Cigarette smoking, bleomycin therapy, and radiation treatment of involved areas also increase the risk of malignant degeneration in RRP.
Children with RRP frequently experience remission after several years, which may be related to puberty. By this time, the patient may have undergone more than 20 surgical procedures. Disease in adults tends to be milder.
As noted previously, 3-5% of patients develop squamous cell carcinoma. The prognosis for squamous cell carcinoma in the context of RRP is grave. Cure is uncommon.
RRP usually is a pediatric disease. The main problem is recurrent airway obstruction. Teach parents to recognize potential warning signs, including a weak cry, hoarseness, stridor, wheezing, cyanosis, and decreased exercise tolerance. Airway obstruction may recur as soon as 2-4 weeks after laser procedures, and recognizing its development before critical, life-threatening obstruction develops is important.
For excellent patient education resources, see eMedicineHealth's patient education article Bronchoscopy.
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