Long Term Outcome Of Juvenile Onset Recurrent Respiratory Papillomatosis In Universiti Kebangsaan Malaysia Medical Centre

Abstract

Objective: To review the long term outcome of juvenile onset recurrent respiratory papillomatosis (JORRP) in University Kebangsaan Medical Centre (UKMMC). Methods:A sixteen-year retrospective study -from January 2002 to June 2019 -of patients who presented with JORRP at our hospital was carried out.Results: Nineteen cases were identified with the youngest age of presentation being eight months old. The mean follow-up time was 4.8 years. All patients had glottic papilloma, ten had multi-site involvement, namely at the subglottic, trachea, oropharynx and one case of distal spread to the lungs. The majority (71.4%) of patients with tracheostomy had extralaryngeal spread and shorter surgical intervals (a mean of 3.2 procedures per year). All our patients were treated surgically. Two patients with aggressive disease received a trial of the adjuvant therapies Gardasil and Cidofovir. However, no improvement was observed. Conclusion:The worst prognosis is seen in youngeronset JORRP. Surgery is the mainstay of treatment and patients with aggressive disease may need adjuvant therapy. Unfortunately, there is a lack of a standard protocol for adjuvant therapies. Tracheostomy increases the risk of extralaryngeal spread and aggressive disease, and thus should be reserved for patients with severe airway compromise.

A Case Report of Juvenile-Onset Recurrent Respiratory Papillomatosis

“…Referrals to otorhinolaryngologists for FNPLS should be made for patients who exhibit persistent hoarseness of voice in primary care, as this procedure is paramount in making the clinical diagnosis, which typically reveals exophytic and pedunculated lesions with punctate hemorrhage. The largest case series in our country by Goh et al showed 68.4% of patients had extralaryngeal involvement of RRP [3]. Thus, it is important to examine the entire airway thoroughly to exclude extralaryngeal involvement of RRP.…”

“…The triad of JORRP is first-born, delivered vaginally, and child of a young mother [4]. In this case, despite being atypical as the patient is not the first child, the pregnancy-related condyloma is likely to be the primary risk factor for vertical HPV transmission during vaginal delivery and the acquisition of JORRP [3].…”

“…RRP is characterized by the formation of exophytic proliferative lesions of connective tissue covered by epithelium anywhere in the respiratory tract, with predilection towards the squamocolumnar junction primarily involving the larynx, especially vocal cords. There is a bimodal age distribution: juvenile-onset RRP (JORPP) occurs in children below 12 years and adult-onset RRP (AORRP) occurs after 12 years of age [3]. Human papillomavirus (HPV) is the established etiology for JORRP, with HPV types 6 and 11 being the most frequent strains.…”

“…The results show 75% of JORRP patients' complete remission after intralesional cidofovir following surgical excision [1]. However, the experience by Goh et al using Gardasil and intralesional cidofovir was the opposite, and the patient developed recurrence after completing adjuvant therapies [3].…”