Clinical and sociodemographic characteristics associated with disease severity in juvenile recurrent respiratory papillomatosis: A study of 104 patients in a tertiary care pediatric hospital

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Abstract

Background

Juvenile recurrent respiratory papillomatosis (JRRP) is generally aggressive and with a high recurrence rate. Currently, there is no definite curative treatment for JRRP. Therefore, a greater understanding of the aspects that influence the severity and prognosis of the disease is required.

Objective

The aim of this study was to establish the clinical and socioeconomic characteristics of pediatric patients with JRRP and its relationship with the severity of the disease in a tertiary care pediatric hospital.

Results

A strong relationship was observed between the severity of the disease and the age at the time of diagnosis, and having a tracheostomy. A moderate association was found between the severity of the disease and the age at the time of the study, the area of origin and the recurrence rate. None of the socioeconomic statuses had a correlation with the severity of the JRRP.

Conclusions

JRRP is associated with multiple surgeries due to the recurrence and aggressiveness of the disease. The socioeconomic status does not seem to influence the severity of the disease, whereas younger patients and users of tracheostomy should receive a more strict follow-up given the increased risk of severe disease.

Introduction

Juvenile recurrent respiratory papillomatosis (JRRP) is the most common neoplastic laryngeal pathology in childhood [1]. Its etiological agent is human papillomavirus (HPV), and subtypes 6 and 11 are responsible for more than 90% of JRRP cases [2], with no relationship between HPV type and clinical severity [3].

The clinical course of JRRP is variable. However, most of the cases present progressive growth of the lesions, compromising the permeability of the airway.

The juvenile form develops in patients less than 20 years of age [4]. This form of the disease is generally aggressive and with a high recurrence rate. A variable prevalence has been reported depending on the age of presentation, country and socioeconomic status of the population studied. However, according to several series, the estimated prevalence is 1–4 per 100,000 children, with an approximate incidence of 4 per 100,000 [1,[4], [5], [6], [7], [8], [9]].

The JRRP has a high negative impact on the patient's quality of life [5], due to repeated hospital admissions, social stigma, and anxiety about facing new procedures or possible contagion to relatives. Additionally, quality of life of children with JRRP is worse in comparison to children with other laryngeal pathologies such as unilateral vocal fold paralysis and vocal fold nodules [6]. On the other hand, it has been estimated that the cost of recurrent respiratory papillomatosis care is 6 times higher in children than in adults [7], with an annual cost in the United States for a single case of JRRP of $ 57,996 [5].

Although JRRP is fundamentally benign, a malignant transformation may occur in less than 1% of cases [2]. Currently, there is no definite curative treatment for JRRP and its clinical behavior is markedly aggressive [10]. Therefore, a greater understanding of the aspects that influence the severity and prognosis of the disease is required.

The aim of this study was to establish the clinical and socioeconomic characteristics of pediatric patients with JRRP and its relationship with the severity of the disease in a tertiary care pediatric hospital.

Section snippets

Materials and methods

This is a retrospective cohort study.

Results

A total of 104 patients were included, all with JRRP confirmed histopathologically by biopsy of the lesions and surgically treated from January 1998 to May 2014.

Discussion

Juvenil recurrent respiratory papillomatosis (JRRP) is a complex pathology, due to its chronic and recurrent nature, as well as the lack of a gold standard treatment. Although theoretically universal vaccination against the human papillomavirus could effectively prevent the disease, the real situation in our environment is very far from this possibility [[8], [9], [10], [11]].

The present study constitutes the largest series of pediatric cases with an established diagnosis of JRRP treated in a

Conclusions

Recurrent respiratory papillomatosis has a more aggressive and recurrent presentation in children than in adults. Currently, there is no gold standard for its management; therefore, it is important to continue studying the factors that affect the severity and recurrence of the disease, such as vaccines, antibodies against HPV and adjuvant methods.

Lower age at the time of diagnosis, as well as the need for a tracheostomy are associated with the severity of the disease, followed by the area of

Conflicts of interest

None.

References (15)

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