Recurrent Respiratory Papillomatosis
Presenter:Dr. Jesca Lyimo., Resident 3
Supervisor:Dr. Kenneth Mlay.,ENT Specialist
Department of Otorhinolaryngology
Kilimanjaro Christian Medical University College
Kilimanjaro, Tanzania
OBJECTIVES
• Introduction
• Epidemiology
• Etiology
• Classification
• Transmission
• Clinical presentation
• Histology
• Diagnosis
• Treatment
• Complication
• Prevention
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Introduction
• A rare disorder characterized by the development of small, wart-like growths
(papillomas) in the respiratory tract.
• Most common benign disease of the upper aero-digestive tract, larynx is the most
frequently affected site.
• RRP has potentially fatal consequences and often difficult to treat because of its
tendency to recur and spread throughout the respiratory tract.
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Anatomy
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Epidemiology
• Incidence JoRRP between 0.12 and 4.5 per100,000/year and AoRRP
1.8/100,0000/year in USA.
• More than 10,000 surgical procedures annually for children with RRP in
US in which 75% of children diagnosis was made before the 5yrs of age.
(RY Seedat,& FG Dikkers.(2022)
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Epidermiology
• The incidence in Europe is 3.62 in JORRP and AORRP is 3.94 per 100,000
Population.
• The incidence in SA is 1.34 per 100,000 in JORRP per year and AORRP is 3.88
per 100,000 population.
(Jacob J. Benedict MD 2020 et al)
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Epidemiology Cont...
• Tanzania; (KCMC), Prevalence 0.09%, 37.3% under 5 and 58% had repeated
surgeries.(2005-2015)
Clinical triad of risk factors for RRP
• First born
• Born by teenage mother
• Spontaneous vaginal delivery
(Marco James et al. (2018).
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Etiology
• Human papilloma virus (HPV), non-enveloped icosahedral double stranded
DNA virus.
• Two subtypes identified according to their genome as low risk and high risk.
- Type 6 and 11 mostly identified
- Type 16 & 18 high risk and most malignant potential
- Types 31, 33, 35, 39,45, 52, 58 also been identified
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Classification Of RRP
JUVENILE ONSET RRP{JoRRP}=/<12 years
• Aggressive form with higher recurrence rates.
• More aggressive and more anatomical site are affected.
ADULT ONSET RRP{AoRRP} >12 years Peaks between 20-40yrs .
• Less aggressive, more in immunocompromised patients.
• 50% patient need <5 procedures over their lifetime.
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Transmission
• JoRRP Acquired vertical transmission that occurs during birth from mother’s
infected genitals tract.
• AoRRP sexually transmitted.
• How ever the exactly mode of transmission is still unknown
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Clinical Presentation
• Hoarseness of voice
• Progressive dysphonia
• Difficult in breathing, respiratory distress and stridor
• Recurrent RTI, episodes of chocking
• Failure to thrive
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Histology
• Multiple finger like projection with central
Microscopically hyper vascular core covered by SSE
• keratinization and basal cell hyperplasia
• Cauliflower/grapelike projections
Macroscopically • Sessile/pedunculated, pinkish to white in color
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Macroscopic picture
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Histology cont…
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Disease Staging
• Determine the appropriate therapy. Staging includes an assessment of clinical
features and a structural assessment
Derkay scoring system;
• Severity of hoarseness.
• Degree of airway obstruction.
• Anatomical assessment for the extent of disease.
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Diagnosis
• Proper history taking and physical examination
• Flexible fiberoptic laryngoscopy
• Direct laryngoscopy+ biopsy
• DLTB
• Chest X-ray
• PCR
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Treatment
• RRP remains a challenge to treat with high recurrence rate, risk of airway
obstruction, and significant reduction in quality of life.
• No cure for RRP and no single modality has consistently been shown to be
effective in its eradication
• Aim to maintain airway and improve voice
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Treatment Cont…
MAIN STAY : SURGICAL EXCISION
• Microlaryngeal surgery
• Laser –Co2, NdYAG, PDL,KTP
• Coblation
• Microdebrider
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Treatment cont…
Microlaryngeal surgery (phonomicrosurgery)
Microscopic surgery of vocal folds or other parts of laryngeal framework.
Disadvantages
• More bleeding , post operative pain
• Teeth, other soft tissue injuries and numbness of the tongue
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Treatment Cont…
Laser Surgery ( CO2, NdYAG, PDLs, KTN)
This is the surgery that uses special light beams for surgical procedures
Advantages
• Vaporize lesions with minimal bleeding
• Decrease the interval surgical procedure.
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Treatment Cont…
Disadvantages
• tracheal injuries and scaring to surrounding laryngeal structures
• tracheal esophageal fistula formation
• Exposes staff to an infective plume
• airway burns.
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Treatment Cont…
Coblation
Surgical procedure that uses a bipolar plasma devices to gently dissolve or shrink
tissue.
Advantages
• Less bleeding and less injury to surrounding tissue
• Preserve quality of voice
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Treatment cont…
Microdebrider
This is a rotary shaving device that precisely resects tissue
Advantage
• Faster and cheaper
• Decrease the interval surgical procedure
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Treatment cont…
• Lower risk of thermal injury
• Good for anterior commissure
Disadvantages
• More bleeding
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Treatment cont…
Adjuvant Therapy
• Cidofovir
• mitomycin C
• Systemic Bevacizumab(Avastin)
• Alpha-Interferon: adjuvant IFN alpha-2b
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Post Operative Care
• Analgesics, steroids +/- antibiotics
• Steam inhalation
• Sniffing exercises
• Voice rest
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Complications
• vocal cord scarring
• Laryngeal webs.
• Decrease in Phonatory qualities of the vocal cords.
• Spread of disease to adjacent tissues
• Malignant transformation,1–2%
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Prev cont…
HPV therapeutic vaccination
• PGRN-2012
• INO-3107
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Prev cont…
HPV vaccination
• Gardasil vaccine (6,11,16,18)
• Cevarix vaccine (6,11)
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Prev cont…
Empower them with knowledge
• Patient directed intervention
• Expectations
• Support group
• Sex education
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References
• Cummings Otolaryngology: Head and Neck Surgery, 3-Volume Set 7th Edition
• Lee MY, Metenou S, Allen C. Preclinical study of a novel therapeutic vaccine for recurrent respiratory papillomatosis.
NPJ vaccines.2021,6:86. https://www.nature.com/articles/s41541-021-00348-x
• James, M. et al. (2018) ‘Prevalence, clinical presentations, associated risk factors and recurrence of laryngeal
papillomatosis among inpatients attended at a Tertiary Hospital in Northern zone Tanzania’, Pan African Medical
Journal, 30, pp. 1–7. Available at: https://doi.org/10.11604/pamj.2018.30.209.11211.
• December 11, 2020. Seedat RY, Schall R. Age of diagnosis, incidence and prevalence of recurrent respiratory
papillomatosis-A South African perspective. 2018. Clin Otolaryngolo. 2018;43(2):533–537.
https://doi.org/10.1111/coa.13016
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References
• 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. 2016 Mar. 125(3):235-41.
• Derkay CS. Recurrent respiratory papillomatosis. National Organization for Rare Disorders. Available at
https://rarediseases.org/rare-diseases/recurrent-respiratory-papillomatosis/. 2019; Accessed:
• Ballenger’s Otorhinolaryngology Head and Neck Surgery16th Edition
• Verma H, Solanki P, James M. Acoustical and Perceptual Voice Profiling of Children With Recurrent Respiratory
Papillomatosis. J Voice. 2015 Oct 13. [Medline].
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References
• Norberg SM, Bai K, Sievers C, Robbins Y, Friedman J, Yang X, Kenyon M, Ward E, Schlom J, Gulley J, Lankford A,
Semnani R, Sabzevari H, Brough DE, Allen CT. The tumor microenvironment state associates with response to HPV
therapeutic vaccination in patients with respiratory papillomatosis. Sci Transl Med. 2023 Oct 25;15(719):eadj0740.
doi: 10.1126/scitranslmed.adj0740. Epub 2023 Oct 25. PMID: 37878675; PMCID: PMC11279704.
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