• Bing Sun and Hua-ping Dai.
• Beijing Institute of Respiratory Medicine, Department of Respiratory and Critical Care Medicine, Beijing, China.
• Zhonghua Jie He He Hu Xi Za Zhi. 2011 Aug 1; 34 (8): 600-3.

ObjectiveTo analyze the clinical, radiological, and pathological characteristics of tracheobronchomegaly (TBM, Mounier-Kuhn syndrome).MethodsThe clinical, radiological and pathological characteristics of 3 cases of TBM were analyzed, and the literatures were reviewed.ResultsAll 3 patients were men, between the age of 58 - 71 years old. From the onset to diagnosis, the shortest time was 2 months, and the longest 43 years. The most usual presentations included recurrent cough and sputum, and occasional haemoptysis. In the advanced stage of the disease, patients would present shortness of breath and the symptoms associated with respiratory failure because of the reduction in pulmonary function. All the diagnoses were confirmed by X-ray and CT of the chest finding that the trachea and the main bronchi dilated markedly. After anti-infection treatment, all patients recovered. Mounier-Kuhn syndrome was a rare congenital abnormality characterized by atrophy or absence of elastic fibers and thinning of smooth muscle layer in the trachea and main bronchi. These airways were thus flaccid and markedly dilated on inspiration and collapsed on expiration. The usual presentation was recurrent respiratory tract infections with a broad spectrum of functional impairment ranging from minimal disease with preservation of lung function to severe disease in the form of bronchiectasis, emphysema and pulmonary fibrosis, ultimately culminating in respiratory failure and death. Computed tomography scan of the chest was used for the diagnosis. Treatment was mainly supportive with chest physiotherapy and antibiotics.ConclusionsMounier-Kunh syndrome should be suspected in patient with recurrent respiratory infections and chronic sputum production. A careful analysis of the central airways at the chest radiograph of these patients is required.

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