eMedicine Specialties > Pulmonology > Lung Tumors

Recurrent Respiratory Papillomatosis: Follow-up

Author: Eloise M Harman, MD, Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, University of Florida College of Medicine
Contributor Information and Disclosures

Updated: Mar 6, 2008

Follow-up

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.

Transfer

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.

Deterrence/Prevention

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.16

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.1

Complications

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.17 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.

Prognosis

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.

Patient Education

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, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Bronchoscopy.

Miscellaneous

Medicolegal Pitfalls

  • Failure to make the diagnosis: In JORRP, patients characteristically are symptomatic for 1 year prior to diagnosis, and this author's experience with adult patients is similar.
  • Failure to consider airway obstruction in patients with hoarseness, voice change, and wheezing: This may have dire consequences, including life-threatening airway obstruction.
  • Failure to consider direct laryngoscopy in adults and children with hoarseness
  • Failure to consider transfer to a tertiary center that has staff with experience in management of the disease

Special Concerns

Pregnancy in patients with RRP appears to increase the rate of growth of papillomas and may result in disease recurrence after a period of remission.

 


More on Recurrent Respiratory Papillomatosis

Overview: Recurrent Respiratory Papillomatosis
Differential Diagnoses & Workup: Recurrent Respiratory Papillomatosis
Treatment & Medication: Recurrent Respiratory Papillomatosis
Follow-up: Recurrent Respiratory Papillomatosis
Multimedia: Recurrent Respiratory Papillomatosis
References
[ CLOSE WINDOW ]

References

  1. Kosko JR, Derkay CS. Role of cesarean section in prevention of recurrent respiratory papillomatosis--is there one?. Int J Pediatr Otorhinolaryngol. Mar 1996;35(1):31-8. [Medline].

  2. Shah KV, Stern WF, Shah FK, Bishai D, Kashima HK. Risk factors for juvenile onset recurrent respiratory papillomatosis. Pediatr Infect Dis J. May 1998;17(5):372-6. [Medline].

  3. Armstrong LR, Derkay CS, Reeves WC. Initial results from the national registry for juvenile-onset recurrent respiratory papillomatosis. RRP Task Force. Arch Otolaryngol Head Neck Surg. Jul 1999;125(7):743-8. [Medline].

  4. Bishai D, Kashima H, Shah K. The cost of juvenile-onset recurrent respiratory papillomatosis. Arch Otolaryngol Head Neck Surg. Aug 2000;126(8):935-9. [Medline].

  5. Chhetri DK, Shapiro NL. A scheduled protocol for the treatment of juvenile recurrent respiratory papillomatosis with intralesional cidofovir. Arch Otolaryngol Head Neck Surg. Oct 2003;129(10):1081-5. [Medline].

  6. Derkay C; Multi-Disciplinary Task Force on Recurrent Respiratory Papillomas. Cidofovir for recurrent respiratory papillomatosis (RRP): a re-assessment of risks. Int J Pediatr Otorhinolaryngol. Nov 2005;69(11):1465-7. [Medline].

  7. Lee AS, Rosen CA. Efficacy of cidofovir injection for the treatment of recurrent respiratory papillomatosis. J Voice. Dec 2004;18(4):551-6. [Medline].

  8. Shehab N, Sweet BV, Hogikyan ND. Cidofovir for the treatment of recurrent respiratory papillomatosis: a review of the literature. Pharmacotherapy. Jul 2005;25(7):977-89. [Medline].

  9. Higdon JV, Delage B, Williams DE, Dashwood RH. Cruciferous vegetables and human cancer risk: epidemiologic evidence and mechanistic basis. Pharmacol Res. Mar 2007;55(3):224-36. [Medline].

  10. Rosen CA, Bryson PC. Indole-3-carbinol for recurrent respiratory papillomatosis: long term results. J of Voice. 2003;18:248-53.

  11. Healy GB, Gelber RD, Trowbridge AL, Grundfast KM, Ruben RJ, Price KN. Treatment of recurrent respiratory papillomatosis with human leukocyte interferon. Results of a multicenter randomized clinical trial. N Engl J Med. Aug 18 1988;319(7):401-7. [Medline].

  12. Leventhal BG, Kashima HK, Mounts P, Thurmond L, Chapman S, Buckley S, et al. Long-term response of recurrent respiratory papillomatosis to treatment with lymphoblastoid interferon alfa-N1. Papilloma Study Group. N Engl J Med. Aug 29 1991;325(9):613-7. [Medline].

  13. Shikowitz MJ, Abramson AL, Freeman K, Steinberg BM, Nouri M. Efficacy of DHE photodynamic therapy for respiratory papillomatosis: immediate and long-term results. Laryngoscope. Jul 1998;108(7):962-7. [Medline].

  14. Endres DR, Bauman NM, Burke D, Smith RJ. Acyclovir in the treatment of recurrent respiratory papillomatosis. A pilot study. Ann Otol Rhinol Laryngol. Apr 1994;103(4 Pt 1):301-5. [Medline].

  15. Lippman SM, Donovan DT, Frankenthaler RA, Weber RS, Earley CL, Hong WK, et al. 13-Cis-retinoic acid plus interferon-alpha 2a in recurrent respiratory papillomatosis. J Natl Cancer Inst. Jun 1 1994;86(11):859-61. [Medline].

  16. Freed GL, Derkay CS. Prevention of recurrent respiratory papillomatosis: role of HPV vaccination. Int J Pediatr Otorhinolaryngol. Oct 2006;70(10):1799-803. [Medline].

  17. Shapiro AM, Rimell FL, Shoemaker D, Pou A, Stool SE. Tracheotomy in children with juvenile-onset recurrent respiratory papillomatosis: the Children's Hospital of Pittsburgh experience. Ann Otol Rhinol Laryngol. Jan 1996;105(1):1-5. [Medline].

  18. Andrus JG, Shapshay SM. Contemporary management of laryngeal papilloma in adults and children. Otolaryngol Clin North Am. Feb 2006;39(1):135-58. [Medline].

  19. Avidano MA, Singleton GT. Adjuvant drug strategies in the treatment of recurrent respiratory papillomatosis. Otolaryngol Head Neck Surg. Feb 1995;112(2):197-202. [Medline].

  20. Baker GE, Tyring SK. Therapeutic approaches to papillomavirus infections. Dermatol Clin. Apr 1997;15(2):331-40. [Medline].

  21. Burns JA, Zeitels SM, Akst LM, Broadhurst MS, Hillman RE, Anderson R. 532 nm pulsed potassium-titanyl-phosphate laser treatment of laryngeal papillomatosis under general anesthesia. Laryngoscope. Aug 2007;117(8):1500-4. [Medline].

  22. Chadha NK, James AL. Antiviral agents for the treatment of recurrent respiratory papillomatosis: a systematic review of the English-language literature. Otolaryngol Head Neck Surg. Jun 2007;136(6):863-9. [Medline].

  23. Derkay CS, Smith RJ, McClay J, van Burik JA, Wiatrak BJ, Arnold J, et al. HspE7 treatment of pediatric recurrent respiratory papillomatosis: final results of an open-label trial. Ann Otol Rhinol Laryngol. Sep 2005;114(9):730-7. [Medline].

  24. Green GE, Bauman NM, Smith RJ. Pathogenesis and treatment of juvenile onset recurrent respiratory papillomatosis. Otolaryngol Clin North Am. Feb 2000;33(1):187-207. [Medline].

  25. Hawkes M, Campisi P, Zafar R, Punthakee X, Dupuis A, Forte V, et al. Time course of juvenile onset recurrent respiratory papillomatosis caused by human papillomavirus. Pediatr Infect Dis J. Feb 2008;27(2):149-54. [Medline].

  26. Kimberlin DW. Current status of antiviral therapy for juvenile-onset recurrent respiratory papillomatosis. Antiviral Res. Sep 2004;63(3):141-51. [Medline].

  27. Kimberlin DW, Malis DJ. Juvenile onset recurrent respiratory papillomatosis: possibilities for successful antiviral therapy. Antiviral Res. Feb 2000;45(2):83-93. [Medline].

  28. Mouadeb DA, Belafsky PC. In-office laryngeal surgery with the 585nm pulsed dye laser (PDL). Otolaryngol Head Neck Surg. Sep 2007;137(3):477-81. [Medline].

  29. Naiman AN, Ceruse P, Coulombeau B, Froehlich P. Intralesional cidofovir and surgical excision for laryngeal papillomatosis. Laryngoscope. Dec 2003;113(12):2174-81. [Medline].

  30. Pasquale K, Wiatrak B, Woolley A, Lewis L. Microdebrider versus CO2 laser removal of recurrent respiratory papillomas: a prospective analysis. Laryngoscope. Jan 2003;113(1):139-43. [Medline].

  31. Pransky SM, Magit AE, Kearns DB, Kang DR, Duncan NO. Intralesional cidofovir for recurrent respiratory papillomatosis in children. Arch Otolaryngol Head Neck Surg. Oct 1999;125(10):1143-8. [Medline].

  32. Preuss SF, Klussmann JP, Jungehulsing M, Eckel HE, Guntinas-Lichius O, Damm M. Long-term results of surgical treatment for recurrent respiratory papillomatosis. Acta Otolaryngol. Nov 2007;127(11):1196-201. [Medline].

  33. Rady PL, Schnadig VJ, Weiss RL, Hughes TK, Tyring SK. Malignant transformation of recurrent respiratory papillomatosis associated with integrated human papillomavirus type 11 DNA and mutation of p53. Laryngoscope. May 1998;108(5):735-40. [Medline].

  34. Rimell FL, Shoemaker DL, Pou AM, Jordan JA, Post JC, Ehrlich GD. Pediatric respiratory papillomatosis: prognostic role of viral typing and cofactors. Laryngoscope. Jul 1997;107(7):915-8. [Medline].

  35. Schraff S, Derkay CS, Burke B, Lawson L. American Society of Pediatric Otolaryngology members' experience with recurrent respiratory papillomatosis and the use of adjuvant therapy. Arch Otolaryngol Head Neck Surg. Sep 2004;130(9):1039-42. [Medline].

  36. Soma MA, Albert DM. Cidofovir: to use or not to use?. Curr Opin Otolaryngol Head Neck Surg. Feb 2008;16(1):86-90. [Medline].

  37. Stamataki S, Nikolopoulos TP, Korres S, Felekis D, Tzangaroulakis A, Ferekidis E. Juvenile recurrent respiratory papillomatosis: still a mystery disease with difficult management. Head Neck. Feb 2007;29(2):155-62. [Medline].

  38. Wiatrak BJ. Overview of recurrent respiratory papillomatosis. Curr Opin Otolaryngol Head Neck Surg. Dec 2003;11(6):433-41. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

RRP, juvenile-onset recurrent respiratory papillomatosis, JORRP, adult-onset recurrent respiratory papillomatosis, AORRP, human papilloma virus, HPV, human papillomavirus, airway obstruction, voice change, warts, sexually transmitted diseases, STDs

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Eloise M Harman, MD, Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, University of Florida College of Medicine
Eloise M Harman, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Chest Physicians, American Medical Women's Association, American Thoracic Society, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Harold L Manning, MD, Associate Professor, Departments of Medicine, Anesthesiology and Physiology, Section of Pulmonary and Critical Care Medicine, Dartmouth Medical School
Harold L Manning, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.