Abstract
<title>Abstract</title> <p>Background: Circumflex aortic arch [CAA] is a rare congenital vascular anomaly in which the aortic arch [AA] crosses the midline posterior to the trachea and oesophagus and descends on the contralateral side. This anomaly, when symptomatic, is associated with a ligamentum arteriosum between the crossing AA and the contralateral pulmonary artery [PA]. Imaging plays a particular role in both the diagnosis of CAA and the active surveillance for the presence of the compressing ligamentum. In this article, we present two rare cases of CAA, highlighting the role of imaging in the preoperative evaluation of such a vascular ring. The tethered appearance of the distal AA towards the contralateral PA is a useful reminder Case presentation: We present two cases of CAA. One of them is a four-year-old male patient, and the other is a nine-year-old female patient, who both presented with progressive stridor. The CAA and compressing ductal ligament were accurately identified in both patients by preoperative cardiac computerised tomography [CCT]. Both patients underwent division of the ligamentum and aortopexy. Conclusions: Despite its rarity, a CAA should always be considered a cause of a symptomatic vascular ring. The active search for the signs of airway or oesophageal compression that suggest the presence of ligamentum arteriosum in preoperative imaging helps prevent the persistence of compression symptoms after surgical correction and the need to perform a revision operation.</p>