CTICasesMounier-Kuhn Syndrome
Imaging findings
Chest radiograph and CT demonstrate marked dilatation of the trachea and main bronchi, consistent with tracheobronchomegaly. Multiple bilateral cystic bronchiectatic spaces are present in the lung parenchyma, some containing air-fluid levels. In addition, there is evidence of prior bronchial artery embolization.
Key takeaways
Mounier-Kuhn syndrome (tracheobronchomegaly) is a rare congenital disorder characterized by marked dilatation of the trachea and main bronchi. It is associated with atrophy or absence of elastic fibers and smooth muscle cells within the airway wall. This leads to redundant wall tissue, pooling of secretions, recurrent lower respiratory tract infections, progressive bronchiectasis, and occasionally life-threatening hemoptysis.
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