title: Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases.
abstract: OBJECTIVE
  Mounier-Kuhn syndrome (MKS) is a congenital disorder characterized by tracheobronchomegaly resulting from the absence of elastic fibers in the trachea and main bronchi or atrophy and thinning of the smooth muscle layer. In this syndrome, dead space associated with tracheobronchomegaly increases and discharge of secretions decreases because of ineffective coughing. The most common complications are recurrent lower respiratory tract infections and bronchiectasis. We examined the clinical characteristics, radiological features, and related complications of patients with MKS.
  
  
  METHODS
  The cases were obtained between September 2007 and November 2015. Computed tomography scans of the chest were used to diagnose tracheobronchomegaly.
  
  
  RESULTS
  All cases (a total of 11) were males with a mean age of 63 ± 13 (range, 38-80) years. The mean diameter of the trachea was 31.53 ± 2.99 mm; the mean transverse diameter was 31.69 ± 3.10 mm and the mean sagittal diameter was 31.36 ± 3.01 mm. Complaints at the time of presentation included chronic cough, purulent sputum, dyspnea, and hemoptysis. There were recurrent pulmonary infections in seven cases, bronchiectasis in six, and tracheal diverticulum in four at the time of diagnosis.
  
  
  CONCLUSIONS
  In this article, 11 cases with various rarely seen complications are presented and evaluated in the light of current literature. We recommend that if chronic cough, recurrent pulmonary infections, and bronchiectasis seen in a patient, MKS should be kept in mind.
authors.affiliation: Department of Chest Diseases, Faculty of Medicine, Ordu University, Ordu, Turkey.
authors.forename: Recep
authors.initials: R
authors.lastname: Akgedik
authors.affiliation: Department of Chest Diseases, Atatürk Chest Diseases and Chest Surgery Education and Research Hospital, Ankara, Turkey.
authors.forename: Harun
authors.initials: H
authors.lastname: Karamanli
authors.affiliation: Department of Chest Diseases, Hakkari state Hospital, Hakkari, Turkey.
authors.forename: Deniz
authors.initials: D
authors.lastname: Kizilirmak
authors.affiliation: Department of Radiology, Faculty of Medicine, Ordu University, Ordu, Turkey.
authors.forename: Ali Bekir
authors.initials: AB
authors.lastname: Kurt
authors.affiliation: Department of Radiology, Faculty of Medicine, Ordu University, Ordu, Turkey.
authors.forename: Hasan
authors.initials: H
authors.lastname: Öztürk
authors.affiliation: Department of Chest Diseases, Faculty of Medicine, Ordu University, Ordu, Turkey.
authors.forename: Berna Botan
authors.initials: BB
authors.lastname: Yildirim
authors.affiliation: Department of Family Medicine, Faculty of Medicine, Ordu University, Ordu, Turkey.
authors.forename: Lütfullah
authors.initials: L
authors.lastname: Çakir
mesh_terms: D000368:Aged; D001980:Bronchi / Q000000981:diagnostic imaging; D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000209:etiology*; D001999:Bronchoscopy / Q000379:methods; D003371:Cough / Q000175:diagnosis; D004240:Diverticulum / Q000473:pathology; D004417:Dyspnea / Q000175:diagnosis; D006469:Hemoptysis / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000175:diagnosis / Q000209:etiology*; D013183:Sputum / Q000382:microbiology; D014057:Tomography, X-Ray Computed / Q000379:methods; D014132:Trachea / Q000033:anatomy & histology / Q000000981:diagnostic imaging / Q000473:pathology*; D014134:Tracheal Neoplasms / Q000473:pathology; D014137:Tracheobronchomegaly / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology*
keywords: Mounier-Kuhn syndrome; bronchiectasis; computed tomography; recurrent pulmonary infections; tracheal diverticula; tracheobronchomegaly