Late recurrence of tracheal papillomatosis in JORRP

Background Juvenile-onset recurrent respiratory papillomatosis (JORRP) is a rare disease caused by human papilloma virus (HPV) infection types 6 and 11 of the larynx which is diagnosed in children and juveniles, though median age at first appearance is 2-4y. 13-50 % of the patients are diagnosed with more than one anatomical side involved beneath the larynx.

JORRP has an unpredictable clinical course, though there are risk factors for an aggressive clinical course described such as infection of HPV 11 or young age at diagnosis.

Methods We report a case of a 59-year-old male patient whom was diagnosed with JORRP at the age of one. Due to massive tracheal spread tracheostomy had to be performed at the age of two, decannulation could be done at the age of six. In the following decades, more than 40 surgical reductions of laryngeal papilloma had to be performed.

Within another surgical reduction of laryngeal papilloma for the first time after childhood, 2019 tracheal spread could be diagnosed. A bronchoscopical removal of tracheal papilloma was done, HPV type 11 could be detected.

Results This patient suffered recurrence of tracheal papillomatosis after 50 years.

Discussion Due to young age at onset, early involvement of tracheal papillomatosis and infection of HPV type 11 this patient sustains a couple of known risk factors for an aggressive clinical course of JORRP.

It should be considered to keep patients with similar coincidental risk factors in clinical surveillance.

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Publication History

Article published online:
10 June 2020