CTICasesTracheobronchomegaly (Mounier-Kuhn Syndrome)
Imaging findings
A 24-year-old woman presented with cough, dyspnea, and recurrent respiratory infections. CT shows predominantly central bronchiectasis sparing areas without superimposed infection, such as the right upper lobe, right middle lobe, and lingula, with the bronchiectasis largely stopping at the segmental to sub-segmental airway level. The trachea is markedly floppy and enlarged, measuring about 3.6 centimeters, with enlarged main-stem bronchi measuring about 2.6 centimeters, meeting the radiologic criteria for tracheobronchomegaly.
Key takeaways
The panel discussed distinguishing this from Williams-Campbell syndrome, in which the trachea is typically spared or near normal and bronchiectasis is most severe at the segmental and sub-segmental level due to deficient distal cartilage, versus this case with massively enlarged central trachea and bronchi more typical of Mounier-Kuhn from elastic tissue or cartilage deficiency. Two clinical phenotypes were discussed: younger patients with a more equal sex distribution, often associated with connective tissue disease such as Ehlers-Danlos, versus older male smokers with COPD who show an accordion appearance and diverticula, thought to relate to chronic obstruction and airway remodeling rather than a primary elastic deficiency. Because biopsy confirmation of elastic fiber or cartilage deficiency is essentially never obtained, these entities may represent a spectrum best described simply by the distribution and severity of bronchiectasis rather than forcing a specific eponymous diagnosis.
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