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Right Isomerism with Bilateral Tracheal Bronchi

Imaging findings

Chest CT shows right isomerism (asplenia syndrome) with bilateral tracheal bronchi (upper lobe bronchi arising directly from the trachea), bilateral morphologically right lungs, right aortic arch, retroaortic brachiocephalic vein, and abdominal situs inversus with malrotation and asplenia.

Key takeaways

Right isomerism (asplenia syndrome) is characterized by bilateral right-sidedness (bilateral trilobed lungs, asplenia). The presence of bilateral tracheal bronchi is a rare manifestation of this syndrome, which is typically associated with severe congenital heart disease.

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