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Title: Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases
Authors: Akgedik, Recep
Karamanli, Harun
Kizilirmak, Deniz
Kurt, Ali Bekir
Ozturk, Hasan
Yildirim, Berna Botan
Cakir, Luetfullah
Ordu Üniversitesi
Keywords: bronchiectasis, computed tomography, Mounier-Kuhn syndrome, recurrent pulmonary infections, tracheal diverticula, tracheobronchomegaly
Issue Date: 2018
Citation: Akgedik, R., Karamanli, H., Kizilirmak, D., Kurt, AB., Öztürk, H., Yildirim, BB., Çakir, L. (2018). Mounier-Kuhn syndrome (tracheobronchomegaly): An analysis of eleven cases. Clin. Respir. J., 12(3), 885-889.
Abstract: Background and AimMounier-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. MethodsThe cases were obtained between September 2007 and November 2015. Computed tomography scans of the chest were used to diagnose tracheobronchomegaly. ResultsAll cases (a total of 11) were males with a mean age of 6313 (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. ConclusionsIn 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.
Description: WoS Categories: Respiratory System
Web of Science Index: Science Citation Index Expanded (SCI-EXPANDED)
Research Areas: Respiratory System
ISSN: 1752-6981
Appears in Collections:Dahili Tıp Bilimleri

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