Abstract:

Mounier-Kuhn syndrome (MKS) is a rare entity characterised by an enlarged trachea and main bronchi. Despite the century-long delay between the first description in 1932, it is still under- diagnosed with only 300 cases reported in the literature. Histologically it is characterised by an atrophy of the smooth muscle and elastic fibres of the tracheobronchial tree resulting in impaired mucociliary clearance. The respiratory symptoms of MKS are non-specific and appear as a recurrent respiratory infection. Chest CT remains the gold standard for diagnosis treatment is non-specific and based on the control of associated respiratory diseases, physiotherapy and sometimes surgery. We report here the case of a 61-year-old man with a history ofchronic bronchitis and recurrent pneumonia who was admitted for acute dyspnea and in whom the diagnosis of MKS was retained. A better knowledge of the pathology will allow an early diagnosis and prevention of complications. This case illustrates the late diagnosis and management.

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