Mounier-Kuhn syndrome

Case contributed by Senai Goitom Sereke
Diagnosis probable

Presentation

History of recurrent pneumonia

Patient Data

Age: 20 years
Gender: Male
ct

Marked dilatation of the trachea (reaching up to 4cm in diameter), the central and basal bronchi bilaterally with cystic bronchiectasis involving the right lower lobe most likely tracheobronchomegaly. Mild emphysema also seen.

Case Discussion

Tracheobronchomegaly aka Mounier-Kuhn syndrome is frequently seen in middle aged men below the age of 50. It can co-exist with COPD, as in this case with mild emphysema. It is due to an absence or marked atrophy of elastic fibers and smooth muscles from the walls of the tracheobronchial tree.

Asymmetry is unusual.

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