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Test Paper 6
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
Tracheobronchomegaly, known as Mounier–Kuhn disease, is a rare condition, usually affecting men in the fourth and fifth decade of life. It is characterised by marked dilatation of the trachea and bronchi. Patients typically present with recurrent respiratory infections and bronchitis. It is caused by atrophy and dysplasia of the tracheal wall and the tracheal diameter is usually more than 3 cm.
Mounier Kuhn syndrome presenting with recurrent atelectasis
Published in Acta Clinica Belgica, 2018
Christine Quentin, Nicolas Lefevre, Eddy Bodart, Laurence Hanssens
Enlargement of the proximal airways, mainly trachea and large bronchi, was first described in 1932 by Mounier Kuhn [1]. This tracheobronchomegaly, associated with recurrent airway infections, is due to a thinning of the muscular mucosa, as well as an atrophy of longitudinal muscles and elastic fibers [2]. Three different types are defined [3]: type 1, the most common, with symmetrical diffuse enlargement of both trachea and main bronchi; type 2 with eccentric enlargements, diverticula, and abrupt changes of the bronchial diameter; and type 3, the least frequent, with diverticula extending to the more distal bronchi. Etiology of these modifications is not yet known. Prevalence is estimated between 0.4 and 1.6% in patients with respiratory symptoms, and there is a strong male predominance with a ratio of 8–1. It is usually diagnosed in adulthood [2,4–6], and only a few cases have been described in childhood. We report a case of Mounier Kuhn syndrome in a young child with early respiratory symptoms. In addition, we would like to highlight the fact that large airway disease is usually overlooked initially at chest X-rays.