Mounier-Kuhn syndrome

• Published in: Postgraduate medical journal Vol. 93; no. 1104; p. 642
• Main Authors: Simkins, Aron, Maiti, Abhishek, Cherian, Sujith V, Trujillo, Daniel O, Estrada-Y-Martin, Rosa M
• Format: Journal Article
• Language: English
• Published: England Oxford University Press 01.10.2017
• Subjects: Infections; Pneumonia; Tomography; Mounier-Kuhn syndrome; tracheobronchomegaly
• ISSN: 0032-5473; 1469-0756
• DOI: 10.1136/postgradmedj-2017-134958

A 95-year-old man with hypertension presented with altered mental status and progressive weight loss for 8months. Tracheobronchomegaly or Mounier-Kuhn syndrome is a rare condition characterised by a defect in the cartilaginous rings causing dilation and atrophy of the muscular and elastic tissues in the trachea and bronchial walls. 1-3 It was first described in 1932 by Mounier-Kuhn after the association of tracheal dilation and recurrent respiratory tract infections. 2 Most cases are congenital, however, acquired cases have been reported in patients with connective tissue diseases like Ehlers-Danlos or Marfan syndrome. 2 3 The enlarged airways and ineffective cough cause deficiency in mucociliary activity and poor secretion clearance ultimately leading to recurrent pulmonary infections. 2 3 Pneumothorax and haemoptysis have been associated with tracheobronchomegaly, however to our knowledge there have been no reports of spontaneous pneumomediastinum. 3 Diagnosis is often made with chest radiographs or CT distinguishing a trachea larger than 30mm3.