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Cervical Aortic Arch with Aberrant Subclavian Artery in DiGeorge Syndrome

Imaging findings

CT in a 20-year-old woman with a vagal nerve stimulator for refractory seizures shows an apparent left aortic arch on the scout with a high right paratracheal abnormality and leftward tracheal deviation; cross-sectional imaging reveals an extremely high right-sided cervical aortic arch that then descends on the left as a circumflex descending aorta, with the left subclavian artery arising anomalously from the descending aorta, forming a vascular ring.

Key takeaways

This is one of the most striking examples of a cervical aortic arch, occurring in a patient with 22q11 deletion (DiGeorge/velocardiofacial) syndrome, similar to a prior case presented from the same conference. Cervical arches can also show early carotid bifurcation with separate origins of the external and internal carotid arteries; despite the complex arch and vascular ring, surgery was deferred here given the patient's other comorbidities.

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