Tracheotomy as a predictor of remission and demise for juvenile-onset recurrent respiratory papillomatosis

Juvenile-onset recurrent respiratory papillomatosis (JoRRP) is the most common benign neoplasm of the larynx in children, presenting with significant variation in clinical course and potential for progression to malignancy. Since JoRRP is driven by human papillomavirus (HPV), we evaluated viral factors in a prospective cohort to identify predictive factors of disease severity. Twenty children with JoRRP undergoing routine debridement of papillomas were recruited and followed for ≥1 year. Demographical features, clinical severity scores, and surgeries over time were tabulated. Biopsies were used to establish a tissue bank and primary cell cultures for HPV6 vs. HPV11 genotyping and evaluation of viral gene expression. We found that patients with HPV11+ disease had an earlier age at disease onset, higher frequency of surgeries, increased number of lifetime surgeries, and were more likely to progress to malignancy. However, the amplitude of viral E6/E7 gene expression did not account for increased disease severity in HPV11+ patients. Determination of HPV strain is not routinely performed in the standard of care for JoRRP patients; we demonstrate the utility and feasibility of HPV genotyping using RNA-ISH for screening of HPV11+ disease as a biomarker for disease severity and progression in JoRRP patients.

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Alpha, Beta, and Gamma HPV Infection in Juvenile Onset Recurrent Respiratory Papillomatosis

10.21203/rs.3.rs-552384/v1 ◽

2021 ◽

Author(s):

Martina Bertinazzi ◽

Tarik Gheit ◽

Jerry Polesel ◽

Sandrine McKay-Chopin ◽

Cesare Cutrone ◽

...

Keyword(s):

Small Sample Size ◽

Hpv Infection ◽

Recurrent Respiratory Papillomatosis ◽

Small Sample ◽

Clinical Severity ◽

Laser Vaporization ◽

Double Infection ◽

Juvenile Onset ◽

Hpv Dna ◽

Alpha Beta

Abstract Purpose The aim of our study is to evaluate the prevalence of different HPV genera – alpha, beta and gamma – in Juvenile onset Recurrent Respiratory Papillomatosis (JoRRP) and examine the association of type and genus-specific viral features with the clinical outcome of disease. Methods This retrospective observational study includes consecutive patients with JoRRP who were treated in a referral centre between October 2000 and October 2020. All patients underwent cold excision and laser vaporization of papillomatous lesions. Samples were analysed for the presence of 120 viral genotypes (22 alpha-HPV, 46 beta-HPV, 52 gamma-HPV) using a highly sensitive multiplex genotyping assay. Results Twenty patients with JoRRP, aged 0.3–11 years old, were included, with a median follow-up of 13.5 years. All samples were HPV DNA positive: 20 (100%) for alpha-HPV DNA; 7 (35%) for beta – HPV DNA; 0 for gamma-HPV DNA. Three groups were defined according to the number of infections: seven cases (35%) with HPV mono-infection; ten cases (50%) with HPV double-infection; three cases (15%) with ≥ 3 HPV infections. At diagnosis, patients with ≥ 3 HPV infections reported higher median Derkay’s score than those with mono-infection (21 vs 14, P = 0.018). Number of HPV infections was also associated with clinical outcomes, with an average of 0.5 surgical procedures/year in patients with mono-infection, 1.2 for double-infection, 2.6 for ≥ 3 infections (P = 0.006). Conclusion Despite the small sample size, these preliminary data support an association between the number of different alpha and beta HPV co-infections and the clinical severity of the disease.

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Distribution Characteristics of Juvenile-Onset Recurrent Respiratory Papillomatosis at First-Time Surgery

Ear Nose & Throat Journal ◽

10.1177/01455613211049845 ◽

2021 ◽

pp. 014556132110498

Author(s):

Xiaoli Qu ◽

Yang Xiao ◽

Lijing Ma ◽

Jun Wang

Keyword(s):

Vocal Cord ◽

Disease Severity ◽

Recurrent Respiratory Papillomatosis ◽

Age At Diagnosis ◽

Juvenile Onset ◽

Vocal Cords ◽

Younger Age ◽

False Vocal ◽

The Impact ◽

First Time

Objectives The lesion distribution of juvenile-onset recurrent respiratory papillomatosis (JORRP) during first-time surgery has been rarely reported. The purpose of this study was to describe the anatomical distribution of papilloma across 25 Derkay sites during initial surgery and to assess the impact of the lesion distribution on disease severity. Methods Surgical videos and medical records of 106 patients with JORRP (27 aggressive and 79 nonaggressive cases) were retrospectively reviewed. Lesion locations were recorded using Derkay anatomical sites. Logistic regression was used to analyze the effect of the lesion distribution on disease severity. Results Among the 106 patients, the true vocal cords (90.6% left, 84.0% right) were the most frequently involved site, followed by the false vocal cords (39.6% left, 35.8% right) and the anterior commissure (26.4%). Two patients (1.9%) had tracheal involvement. Patients with false vocal cord involvement (odds ratio [OR] = 3.425, 95% confidence interval [CI] [1.285, 9.132], P = .014) and a younger age at diagnosis (OR = .698, 95% CI [.539, .905], P = .007) were more likely to require more than 4 procedures in the year following first-time surgery. Conclusions Lesions were most common on the true vocal cords. False vocal cord involvement and a younger age at diagnosis were risk factors for disease severity.

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Juvenile Onset Recurrent Respiratory Papillomatosis and the Voice

Sataloff's Comprehensive Textbook of Otolaryngology: Head and Neck Surgery (Pediatric Otolaryngology) - Volume 6 ◽

10.5005/jp/books/12712_87 ◽

2016 ◽

pp. 1031-1031

Author(s):

Seth Pransky

Keyword(s):

Recurrent Respiratory Papillomatosis ◽

Juvenile Onset ◽

The Voice

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Juvenile-Onset Recurrent Respiratory Papillomatosis

Advances in Oto-Rhino-Laryngology - Pediatric Airway Surgery ◽

10.1159/000334457 ◽

2012 ◽

pp. 105-108

Author(s):

Stephen C. Maturo ◽

Christopher J. Hartnick

Keyword(s):

Recurrent Respiratory Papillomatosis ◽

Juvenile Onset

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Experimental research on the therapeutic effect of MMR vaccine to juvenile-onset recurrent respiratory papillomatosis

European Archives of Oto-Rhino-Laryngology ◽

10.1007/s00405-019-05351-6 ◽

2019 ◽

Vol 276 (3) ◽

pp. 801-803 ◽

Cited By ~ 1

Author(s):

Yu Wang ◽

Pei-Dong Dai ◽

Tian-Yu Zhang

Keyword(s):

Therapeutic Effect ◽

Experimental Research ◽

Recurrent Respiratory Papillomatosis ◽

Mmr Vaccine ◽

Juvenile Onset

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Challenges in Managing Sars-Cov-2 Positive Child with Juvenile Onset Recurrent Respiratory Papillomatosis: A Case Report with Review of Literature

Tribhuvan University Journal ◽

10.3126/tuj.v34i0.31538 ◽

2020 ◽

pp. 51-58

Author(s):

Heempali Das Dutta ◽

Bibhu Pradhan

Keyword(s):

Airway Obstruction ◽

Surgical Intervention ◽

Severe Form ◽

Recurrent Respiratory Papillomatosis ◽

Juvenile Onset ◽

Aerosol Generation ◽

Laryngeal Surgery ◽

Urgent Surgery ◽

Acute Airway Obstruction ◽

Challenging Situation

Juvenile-Onset Recurrent Respiratory Papillomatosis (JORRP) is a viral infection caused by Human papilloma virus 6, 11 which presents as hoarseness, shortness of breath and in severe form may present with acute airway obstruction because of papillomatous growth obstructing the larynx. Urgent surgical intervention may be needed to secure the airway. In the present situation of Coronavirus disease-2019 (COVID-19) pandemic, the case of JORRP with COVID-19 positive state is a challenging situation because any laryngeal or airway surgery is associated with high amount of aerosol generation. Air borne transmission is one of the major routes of transmission for SARS-Cov-2 virus which may result in spread of COVID-19 among to the health care provider. In this situation, it is better to avoid delay laryngeal surgery if the patient is not in acute airway obstruction requiring urgent surgical intervention. Otherwise, if the urgent surgery has to be done, then it should be done properly by following institutional guidelines to avoid the spread of the disease and to be done with all precautionary biosafety measures like use of personal protective equipment (PPE), N95 mask and proper donning and doffing.

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