Mounier-Kuhn disease

Case contributed by Sarah AlJahdali
Diagnosis almost certain

Presentation

Immunocompromised patient, for screening

Patient Data

Age: 50 years
Gender: Male
x-ray

There is a large lucency projecting over the superior mediastinum on the right side on the frontal radiograph, causing mild displacement/widening of the right para-tracheal line. This correlates with likely dilation of the trachea, better appreciated on the lateral radiograph. The rest of the examination is otherwise unremarkable.

ct

The tracheal is significantly dilated (measuring >3 cm) and associated with an outpouching/diverticulum projecting posteriorly. There is also mild dilation of the central bronchi bilaterally. These findings are in keeping with tracheobronchomegaly.

Case Discussion

Tracheomalacia is defined as tracheomegaly (increased tracheal size > 3 cm) and increased compliance (tendency of the airway to collapse on expiration), best assessed on a biphasic CT with images of the trachea obtained during inspiration and expiration.

Tracheomalacia could be secondary to congenital causes, including Mounier-Kuhn syndrome, as in this case, or other acquired causes.

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