CTICasesVACTERL Syndrome with Circumflex Aortic Arch and Dysphagia Lusoria
Imaging findings
CT demonstrates a left aortic arch that crosses the midline and descends on the right side, consistent with a circumflex aortic arch. An aberrant right subclavian artery is also present but the main compressive effect on the esophagus is from the crossing arch itself, which wraps posteriorly and to the right of the esophagus. The patient has dysphagia with esophageal compression at the mid-esophageal level. A hemivertebra is visible from prior VACTERL-associated vertebral anomaly. Post-operative CT shows a right aortic arch configuration after surgical aortic uncrossing procedure in which the descending aorta was divided and reconnected to the ascending aorta via a graft, effectively re-routing the aorta to the right side.
Key takeaways
A circumflex aortic arch is defined by the aorta crossing the midline so that the arch and descending aorta are on opposite sides. In this case with a left arch and right descending aorta, the arch crosses posteriorly around the esophagus and causes dysphagia lusoria from extrinsic esophageal compression. Surgical correction with an aortic uncrossing procedure re-routes the descending aorta to the same side as the arch via graft interposition. VACTERL syndrome (vertebral, anal atresia, cardiac, tracheoesophageal fistula, renal, limb) is associated with aortic arch anomalies and complex airway and lung abnormalities as illustrated by this case.
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