CTICasesHybrid CPAM with Bronchial Atresia
Imaging findings
A discrete area of rarefaction in the right upper lobe with expanded lobules resembling a focal patch of emphysema, and vessels spread apart with a paucity of normal distribution. A bubbly, mucus-filled bronchus is present that does not appear to connect with the lumen, though it contains air, suggesting either a pinhole connection or collateral air entry from the surrounding parenchyma. There is no scarring or other evidence of prior infection. On sagittal views the lesion lies against and slightly indents the fissure, with a discrete upper margin that does not fade into adjacent parenchyma. Only a few faint rim-like structures are visible rather than the distinct white cyst walls of a classic CPAM, and the tissue has a disorganized rather than purely emphysematous appearance.
Key takeaways
The differential lay between congenital lobar overinflation (now called lobar overinflation, and often segmental rather than lobar), congenital bronchial atresia, and CPAM. Congenital lobar overinflation is structurally normal lung that is simply over-expanded and would be expected to produce mass effect, which was absent here. The space-occupying, bubbly, disorganized appearance without normal intervening parenchyma favors a CPAM, and many CPAMs harbor bronchial atresia, making this a hybrid lesion. Distinct white-rimmed cyst walls support CPAM, though microcystic subtypes may show few. The presenters disagreed on the final label, and as they noted, the distinction does not change management.
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