title: 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.
authors.affiliation: Department of Respiratory Medicine, University Hospital Maison Blanche, Reims, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Schmitt
authors.forename: Levent
authors.initials: L
authors.lastname: Dalar
authors.forename: Stéphane
authors.initials: S
authors.lastname: Jouneau
authors.forename: Bénédicte
authors.initials: B
authors.lastname: Toublanc
authors.forename: Juliette
authors.initials: J
authors.lastname: Camuset
authors.forename: Gérard
authors.initials: G
authors.lastname: Chatte
authors.forename: Laurent
authors.initials: L
authors.lastname: Cellerin
authors.forename: Hervé
authors.initials: H
authors.lastname: Dutau
authors.forename: Stéphane
authors.initials: S
authors.lastname: Sanchez
authors.forename: Maxime
authors.initials: M
authors.lastname: Sauvage
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Vergnon
authors.forename: Sandra
authors.initials: S
authors.lastname: Dury
authors.forename: Gaetan
authors.initials: G
authors.lastname: Deslée
authors.forename: François
authors.initials: F
authors.lastname: Lebargy
mesh_terms: D000328:Adult; D017668:Age of Onset; D000368:Aged; D001987:Bronchiectasis / Q000150:complications*; D001999:Bronchoscopy; D005260:Female; D005764:Gastroesophageal Reflux / Q000150:complications; D006801:Humans; D008297:Male; D008875:Middle Aged; D011656:Pulmonary Emphysema / Q000150:complications*; D012141:Respiratory Tract Infections / Q000209:etiology; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D055089:Tracheobronchomalacia / Q000150:complications*; D014137:Tracheobronchomegaly / Q000150:complications*