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Double Aortic Arch with Atretic Left Segment

Imaging findings

CT angiography in a 3-month-old infant with DiGeorge syndrome (22q deletion) shows a right aortic arch with a left Kommerell diverticulum. A fibrous, atretic left arch segment completes a double aortic arch, causing severe narrowing of the trachea and esophagus.

Key takeaways

Double aortic arch is a vascular ring anomaly where both the right and left arches persist. If one segment is atretic (typically the left), it presents as a right aortic arch with an aberrant vessel, but functions as a tight vascular ring causing airway and esophageal obstruction.

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