Sections

Sections Recurrent Respiratory Papillomatosis
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Medication
Follow-up
Media Gallery
References

Presentation

History

RRP is a rare disease, and adult patients may have symptoms for months or longer before the disease is recognized. Because the larynx is the most frequently affected site for both JORRP and AORRP, symptoms of upper airway obstruction predominate. Upper airway obstruction may be life threatening and may be the presenting symptom. Hoarseness is the most common presenting symptom. Other symptoms include the following:

Voice change
Choking episodes
Foreign body sensation in the throat
Cough
Dyspnea
Inspiratory wheeze
Stridor

Physical

Physical findings often are nonspecific. Voice change may be noted. Inspiratory wheezing, stridor, or both may develop over the trachea or the upper thorax.

Patients with JORRP commonly present with a weak cry, episodes of choking, hoarseness, or failure to thrive. Patients with AORRP present with hoarseness, choking spells, voice change, dyspnea, or a foreign body sensation in the throat.

Causes

HPV causes RRP. HPV-6 and HPV-11 are the most common types associated with RRP, but, rarely, affected tissues contain HPV-16 and HPV-18. HPV is the most common sexually transmitted disease in the United States; as many as 75% of women have genital HPV at some time in their lives. Thirty to 60% of mothers of children affected with JORRP have genital HPV, compared with 5% of mothers of unaffected children. A study using questionnaires of affected children or their parents (identified through the RRP Foundation) verified that the 3 risk factors for JORRP are (1) firstborn child, (2) vaginal delivery, and (3) mother younger than 20 years. The risk factors for JORRP do not apply to adult-onset cases. This suggests that adult disease does not represent reactivation of latent disease. The mode of transmission of HPV in AORRP is not known. Child-to-parent transmission by cough has never been documented. Sexual transmission is likely.

Differential Diagnoses

Verma H, Solanki P, James M. Acoustical and Perceptual Voice Profiling of Children With Recurrent Respiratory Papillomatosis. J Voice. 2015 Oct 13. [Medline].
Kosko JR, Derkay CS. Role of cesarean section in prevention of recurrent respiratory papillomatosis--is there one?. Int J Pediatr Otorhinolaryngol. 1996 Mar. 35(1):31-8. [Medline].
Shah KV, Stern WF, Shah FK, Bishai D, Kashima HK. Risk factors for juvenile onset recurrent respiratory papillomatosis. Pediatr Infect Dis J. 1998 May. 17(5):372-6. [Medline].
Karatayli-Ozgursoy S, Bishop JA, Hillel A, Akst L, Best SR. Risk Factors for Dysplasia in Recurrent Respiratory Papillomatosis in an Adult and Pediatric Population. Ann Otol Rhinol Laryngol. 2015 Oct 8. [Medline].
Kashima HK, Shah F, Lyles A, Glackin R, Muhammad N, Turner L. A comparison of risk factors in juvenile-onset and adult-onset recurrent respiratory papillomatosis. Laryngoscope. 1992 Jan. 102(1):9-13. [Medline].
Armstrong LR, Preston EJ, Reichert M, Phillips DL, Nisenbaum R, Todd NW. Incidence and prevalence of recurrent respiratory papillomatosis among children in Atlanta and Seattle. Clin Infect Dis. 2000 Jul. 31(1):107-9. [Medline].
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. 1999 Jul. 125(7):743-8. [Medline].
Bishai D, Kashima H, Shah K. The cost of juvenile-onset recurrent respiratory papillomatosis. Arch Otolaryngol Head Neck Surg. 2000 Aug. 126(8):935-9. [Medline].
San Giorgi M, van den Heuvel ER, Tjon Pian Gi RE, Brunings JW, Chirila M, Friedrich G, et al. Age of onset of Recurrent Respiratory Papillomatosis: a distribution analysis. Clin Otolaryngol. 2015 Oct 13. [Medline].
Derkay CS, Malis DJ, Zalzal G, Wiatrak BJ, Kashima HK, Coltrera MD. A staging system for assessing severity of disease and response to therapy in recurrent respiratory papillomatosis. Laryngoscope. 1998 Jun. 108(6):935-7. [Medline].
Derkay CS, Hester RP, Burke B, Carron J, Lawson L. Analysis of a staging assessment system for prediction of surgical interval in recurrent respiratory papillomatosis. Int J Pediatr Otorhinolaryngol. 2004 Dec. 68(12):1493-8. [Medline].
Chhetri DK, Shapiro NL. A scheduled protocol for the treatment of juvenile recurrent respiratory papillomatosis with intralesional cidofovir. Arch Otolaryngol Head Neck Surg. 2003 Oct. 129(10):1081-5. [Medline].
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. 2005 Nov. 69(11):1465-7. [Medline].
Lee AS, Rosen CA. Efficacy of cidofovir injection for the treatment of recurrent respiratory papillomatosis. J Voice. 2004 Dec. 18(4):551-6. [Medline].
Shehab N, Sweet BV, Hogikyan ND. Cidofovir for the treatment of recurrent respiratory papillomatosis: a review of the literature. Pharmacotherapy. 2005 Jul. 25(7):977-89. [Medline].
Higdon JV, Delage B, Williams DE, Dashwood RH. Cruciferous vegetables and human cancer risk: epidemiologic evidence and mechanistic basis. Pharmacol Res. 2007 Mar. 55(3):224-36. [Medline].
Rosen CA, Bryson PC. Indole-3-carbinol for recurrent respiratory papillomatosis: long term results. J of Voice. 2003. 18:248-53.
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. 1988 Aug 18. 319(7):401-7. [Medline].
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. 1991 Aug 29. 325(9):613-7. [Medline].
Gerein V, Rastorguev E, Gerein J, Jecker P, Pfister H. Use of interferon-alpha in recurrent respiratory papillomatosis: 20-year follow-up. Ann Otol Rhinol Laryngol. 2005 Jun. 114(6):463-71. [Medline].
Shikowitz MJ, Abramson AL, Freeman K, Steinberg BM, Nouri M. Efficacy of DHE photodynamic therapy for respiratory papillomatosis: immediate and long-term results. Laryngoscope. 1998 Jul. 108(7):962-7. [Medline].
Endres DR, Bauman NM, Burke D, Smith RJ. Acyclovir in the treatment of recurrent respiratory papillomatosis. A pilot study. Ann Otol Rhinol Laryngol. 1994 Apr. 103(4 Pt 1):301-5. [Medline].
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. 1994 Jun 1. 86(11):859-61. [Medline].
Derkay CS, Volsky PG, Rosen CA, Pransky SM, McMurray JS, Chadha NK. Current use of intralesional cidofovir for recurrent respiratory papillomatosis. Laryngoscope. 2013 Mar. 123(3):705-12. [Medline].
Freed GL, Derkay CS. Prevention of recurrent respiratory papillomatosis: role of HPV vaccination. Int J Pediatr Otorhinolaryngol. 2006 Oct. 70(10):1799-803. [Medline].
Fusconi M, Grasso M, Greco A, Gallo A, Campo F, Remacle M, et al. Recurrent respiratory papillomatosis by HPV: review of the literature and update on the use of cidofovir. Acta Otorhinolaryngol Ital. 2014 Dec. 34 (6):375-81. [Medline].
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. 1996 Jan. 105(1):1-5. [Medline].
Andrus JG, Shapshay SM. Contemporary management of laryngeal papilloma in adults and children. Otolaryngol Clin North Am. 2006 Feb. 39(1):135-58. [Medline].
Avidano MA, Singleton GT. Adjuvant drug strategies in the treatment of recurrent respiratory papillomatosis. Otolaryngol Head Neck Surg. 1995 Feb. 112(2):197-202. [Medline].
Baker GE, Tyring SK. Therapeutic approaches to papillomavirus infections. Dermatol Clin. 1997 Apr. 15(2):331-40. [Medline].
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. 2007 Aug. 117(8):1500-4. [Medline].
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. 2007 Jun. 136(6):863-9. [Medline].
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. 2005 Sep. 114(9):730-7. [Medline].
Green GE, Bauman NM, Smith RJ. Pathogenesis and treatment of juvenile onset recurrent respiratory papillomatosis. Otolaryngol Clin North Am. 2000 Feb. 33(1):187-207. [Medline].
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. 2008 Feb. 27(2):149-54. [Medline].
Kimberlin DW. Current status of antiviral therapy for juvenile-onset recurrent respiratory papillomatosis. Antiviral Res. 2004 Sep. 63(3):141-51. [Medline].
Kimberlin DW, Malis DJ. Juvenile onset recurrent respiratory papillomatosis: possibilities for successful antiviral therapy. Antiviral Res. 2000 Feb. 45(2):83-93. [Medline].
Mouadeb DA, Belafsky PC. In-office laryngeal surgery with the 585nm pulsed dye laser (PDL). Otolaryngol Head Neck Surg. 2007 Sep. 137(3):477-81. [Medline].
Naiman AN, Ceruse P, Coulombeau B, Froehlich P. Intralesional cidofovir and surgical excision for laryngeal papillomatosis. Laryngoscope. 2003 Dec. 113(12):2174-81. [Medline].
Pasquale K, Wiatrak B, Woolley A, Lewis L. Microdebrider versus CO2 laser removal of recurrent respiratory papillomas: a prospective analysis. Laryngoscope. 2003 Jan. 113(1):139-43. [Medline].
Pransky SM, Magit AE, Kearns DB, Kang DR, Duncan NO. Intralesional cidofovir for recurrent respiratory papillomatosis in children. Arch Otolaryngol Head Neck Surg. 1999 Oct. 125(10):1143-8. [Medline].
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. 2007 Nov. 127(11):1196-201. [Medline].
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. 1998 May. 108(5):735-40. [Medline].
Rimell FL, Shoemaker DL, Pou AM, Jordan JA, Post JC, Ehrlich GD. Pediatric respiratory papillomatosis: prognostic role of viral typing and cofactors. Laryngoscope. 1997 Jul. 107(7):915-8. [Medline].
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. 2004 Sep. 130(9):1039-42. [Medline].
Soma MA, Albert DM. Cidofovir: to use or not to use?. Curr Opin Otolaryngol Head Neck Surg. 2008 Feb. 16(1):86-90. [Medline].
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. 2007 Feb. 29(2):155-62. [Medline].
Wiatrak BJ. Overview of recurrent respiratory papillomatosis. Curr Opin Otolaryngol Head Neck Surg. 2003 Dec. 11(6):433-41. [Medline].

Media Gallery

A 48-year-old woman presents with inspiratory stridor, dyspnea, and hoarseness. On direct laryngoscopy, extensive respiratory papillomatosis were diagnosed as the cause of her symptoms. Courtesy of Sat Sharma, MD, and L. Garber, MD.
This adult patient was seen for hoarseness, with a history of several prior procedures for recurrent respiratory papillomatosis. A papillomatous lesion is seen along the left true vocal fold with associated reactive edema. On pathologic analysis, moderate squamous dysplasia was seen within the papilloma. Video courtesy of Vijay R Ramakrishnan, MD.

of 2

Author

Eloise M Harman, MD Staff Physician and MICU Director, Pulmonary Division, Gainesville Veterans Affairs Medical Center

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, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Harold L Manning, MD 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, American Thoracic Society

Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, FACP, FCCP Geri and Richard Brawerman Chair in Pulmonary and Critical Care Medicine, Professor and Executive Vice Chairman, Department of Medicine, Medical Director, Women's Guild Lung Institute, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

Zab Mosenifar, MD, FACP, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

Sat Sharma, MD, FRCPC Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba Faculty of Medicine; Site Director, Respiratory Medicine, St Boniface General Hospital, Canada

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, World Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Medscape Reference thanks Vijay R Ramakrishnan, MD, Assistant Professor, Department of Otolaryngology, University of Colorado School of Medicine, for assistance with the video contribution to this article.

Sections Recurrent Respiratory Papillomatosis
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Medication
Follow-up
Media Gallery
References

Recurrent Respiratory Papillomatosis Clinical Presentation

History

Physical

Causes

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

Recurrent Respiratory Papillomatosis Clinical Presentation

History

Physical

Causes

References

Tables

Contributor Information and Disclosures

What would you like to print?

Find Us On

About

Membership

Apps

WebMD Network

Editions

What to Read Next on Medscape

Related Conditions and Diseases

Medscape Consult

News & Perspective

Tools

Slideshow

Most Popular Articles

Recommended