Long‐term Outcomes of Juvenile Onset Recurrent Respiratory Papillomatosis with Pulmonary Involvement

Abstract

Objective To investigate the clinical characteristics and long‐term outcomes of juvenile onset recurrent respiratory papillomatosis (JORRP) with or without pulmonary involvement. Methods A group of patients with JORRP who had clinical course over an extended period of time (at least 5 years) in the Department of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital were included in this retrospective study. Lung/bronchus involvement was revealed by lung imaging. Data on mortality rate, frequency of surgical interventions, and age of disease onset were collected and analyzed. Results The 192 patients (107 male and 85 female) included had a median [quartiles] age of JORRP onset of 2 [1, 4] years, and median follow‐up duration of 10 [7, 13] years; 17 patients (8.9%) had papilloma with bronchial and pulmonary involvement 7.0 [4.0, 12.5] years after the onset of the disease. Compared to patients without lung involvement, patients with lung involvement had a younger age of disease onset (P = .001), higher frequency of surgical interventions (P < .001), higher mortality rate (OR = 94.909), and an increased risk of tracheotomy that could not be decannulated (P < .001). They also had a younger age of disease onset, and a higher frequency of surgical interventions and mortality compared to patients with tracheotomy but free from lung involvement (P < .001). Conclusions Children with JORRP and with pulmonary involvement have a higher average number of operations per year than those without pulmonary involvement, and pulmonary involvement indicates a higher incidence of tracheotomy that cannot be decannulated. Level of Evidence 4 Laryngoscope, 131:E2277–E2283, 2021

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

“…Since tracheotomy is the primary cause of tracheal involvement, we and others have previously reported that the tracheal involvement rate in patients with tracheotomy ranges from 73.5% to 80%. 24,25 The tracheal involvement rate was low in this study because all patients had primary disease and did not undergo tracheotomy. Distal airway involvement has been used as a marker of aggressive disease because lesions in the distal airway often indicate more severe disease.…”

Pulmonary Involvement in Recurrent Respiratory Papillomatosis: A Systematic Review

“…The majority of the studies (10; 90.9%) had a retrospective/observational study design. The quality assessment showed a high risk of bias in the majority of the studies (8/10, 80%) [ 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 ], whereas only two had a moderate risk (2/10, 20%) [ 34 , 35 ] ( Table 1 ).…”

“…Studies were published between 1997 [ 30 ] and 2021 [ 25 , 34 ]. The recruitment period ranged from 1981 [ 30 ] to 2020 [ 25 ].…”

Transnasal Humidified Rapid Insufflation Ventilatory Exchange Augments Oxygenation in Children With Juvenile Onset Recurrent Respiratory Papillomatosis During Surgery: A Prospective Randomized Crossover Controlled Trial

“…20,21,[27][28][29] This study is clinically relevant: our results highlight the benefit of sustained oxygenation with THRIVE in allowing uninterrupted surgery, thereby reducing the overall procedure time, lessening the intraoperative repeated intubation frequency, and subsequently decreasing the lower airway spread risk since many children with JORRP must undergo multiple procedures throughout their childhood. [2][3][4] The prolonged apnea time without desaturation reduced the stress on both the trainee and supervisor, thus permitting an unrushed operation.…”

“…For those patients, repeated surgical interventions are required to maintain airway patency and function. To ensure the best surgical exposure for the resection of tumors in the posterior glottis and trachea [3][4][5] (Figure 1B), a tubeless airway is needed (Figure 1B), which poses a tremendous challenge to anesthetists. To date, there is no consensus on the optimal airway strategy for shared airway management.…”