CTICasesMounier-Kuhn Syndrome (Tracheobronchomegaly) in a Young Female
[...]. Mounier-Kuhn Syndrome (Tracheobronchomegaly) in a Young Female. CTICases. Published March 2, 2017. https://str-case-explorer.pages.dev/c/my6x3
Imaging findings
Chest CT reveals massive dilation of the trachea and mainstem bronchi in a young female patient. The tracheal diameter exceeds 3 cm, and the main bronchi are also abnormally widened, with extremely thin, nearly imperceptible bronchial walls. There is associated peripheral bronchiectasis and tree-in-bud nodular opacities from recurrent infections, along with dynamic tracheobronchomalacia.
Key takeaways
Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare congenital disorder characterized by marked dilation of the trachea and main bronchi due to atrophy of the elastic fibers and smooth muscle. Although classically described with a strong male predominance, it can occasionally present in females. Patients suffer from impaired mucociliary clearance, leading to recurrent respiratory infections, bronchiectasis, and tracheobronchomalacia.
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