CTICasesMounier-Kuhn Syndrome (Tracheobronchomegaly)
Imaging findings
Chest CT shows a dilated, abnormally corrugated, and scalloped trachea and main bronchi associated with severe cystic bronchiectasis. Coronal reformats demonstrate marked tracheobronchial enlargement and tracheal scalloping, which is characteristic of the condition.
Key takeaways
Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare condition characterized by marked dilatation of the trachea and main bronchi due to atrophy of the elastic fibers and smooth muscle. It often coexists with tracheomalacia, bronchiectasis, and recurrent respiratory infections. Whether it represents a distinct congenital entity or is part of a spectrum of chronic airway injury and bronchiectasis remains an academic discussion.
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