CTICasesBronchopulmonary Sequestration with Bronchial Atresia
Imaging findings
CT of the right lung base shows a bubbly lesion with a large component of emphysema, unusual bronchial-appearing elements in its center, and a systemic artery arising from the descending aorta that enhances on contrast-enhanced imaging and supplies the lesion. A coronal reformat highlights a distinctly bronchial branching pattern within the lesion that could not be traced back to a connection with the native airway. The lesion was resected and pathology described cysts lined by bronchial epithelium/endothelium, with surrounding emphysema centered on a denser nidus.
Key takeaways
This was an unusually well-formed example of pulmonary sequestration showing true bronchial branching rather than simple cysts, likely representing a hybrid lesion. There is a well-recognized association between sequestration, bronchial atresia, and congenital lobar overinflation because all three can arise from the same disrupted lung bud: airway remnants that never connect, vascular remnants that fail to regress, and parenchyma that develops abnormally.
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