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Congenital Pulmonary Airway Malformation

Imaging findings

Chest CT in a twenty-eight-year-old patient reveals an area of localized rarefaction in the right upper lobe. While the margins are not sharply demarcated and resemble localized emphysema, surgical resection of the lesion confirms a localized congenital pulmonary airway malformation with no evidence of adenocarcinoma.

Key takeaways

Congenital pulmonary airway malformations can occasionally present as localized, subtle areas of hyperlucency or parenchymal rarefaction mimicking focal emphysema rather than a clearly demarcated cystic mass. In young patients, congenital pulmonary airway malformations should remain on the differential for focal emphysematous changes, and resection can be performed to exclude an associated adenocarcinoma.

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