Atretic Double Aortic Arch: Imaging Appearance of a Rare Anomaly and Differentiation From Its Mimics

A double aortic arch (DAA) with atresia is an uncommon cause of a symptomatic vascular ring resulting in trachea-esophageal compression. An atretic double aortic arch can resemble the right aortic arch with a mirror image branching pattern or the right arch with an aberrant left subclavian artery depending upon the level of atresia. The double aortic arch with atresia is difficult to detect on pre-surgical computed tomography angiography or magnetic resonance angiography due to a lack of contrast in the obliterated arch segment. Differentiation of a double arch with atresia from the right aortic arch is vital as an atretic double arch is a form of the complete vascular ring while the right aortic arch may or may not be symptomatic. Knowledge of some key imaging features can help distinguish these entities. In this case report, we discuss an uncommon case of a double aortic arch with atresia between the left common carotid and left subclavian artery. We also describe its close mimics, their embryological basis, and ways to differentiate it from the right aortic arch.