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Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases

Abstract : Background: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections. Objective: This multicenter, retrospective study was carried out to describe respiratory conditions associated with tracheobronchomegaly. Methods: Nine institutions involved in the ‘Groupe d'Endoscopie de Langue Française' (GELF) participated in this study. A standard form was used to record patient characteristics, treatments and follow-up from medical charts. Results: Seventeen patients, 53% male, aged 58 ± 18 years at diagnosis were included. Recurrent infections revealed MKS in 88% of cases. Main comorbid conditions were diffuse bronchiectasis in 88% of patients and tracheobronchomalacia in 67% of cases. The exacerbation rate was 1.5 exacerbations/patient/year. The main non-respiratory morbid condition was gastroesophageal reflux disease in 29% of cases. Interventional bronchoscopy was performed in seven patients (41%), consisting of laser (n = 2) and tracheal stenting (n = 5). Complications related to stents were observed in 80% of cases with a mean stent duration of 8 months. Four deaths, including three due to respiratory causes, occurred during follow-up. Conclusions: This is the largest series of MKS reported in the literature, showing that bronchiectasis and tracheobronchomalacia are the main associated morbid conditions that constitute a challenge for treatment.
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https://hal-univ-rennes1.archives-ouvertes.fr/hal-01331182
Contributor : Laurent Jonchère <>
Submitted on : Monday, June 13, 2016 - 3:34:52 PM
Last modification on : Tuesday, February 25, 2020 - 10:10:56 AM

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Pierre Schmitt, Levent Dalar, Stéphane Jouneau, Bénédicte Toublanc, Juliette Camuset, et al.. Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases. Respiration, Karger, 2016, 91 (4), pp.281--287. ⟨10.1159/000445029⟩. ⟨hal-01331182⟩

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