Surgical treatment of vascular ring including right cervical aortic arch

Cervical aortic arch is a developmental entity consisting of persistence of the right or left third branchial arch and regression of the fourth branchial arches. In most cases, the aorta is redundant and crosses behind the esophagus to the opposite side. In the presence of an aberrant subclavian artery contralateral to the side of the aortic arch and a ligamentum arteriosum, a vascular ring is formed around the trachea and esophagus. Two young patients with right-sided cervical aortic arch, aberrant left subclavian artery, and ligamentum arteriosum presented with dysphagia and exertional dyspnea. In one patient, through a left thoracotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were dissected thoroughly above and below the level of the ring. In addition, the aberrant left subclavian artery was divided at its origin from a large diverticulum and implanted into the left common carotid artery; the aortic diverticulum was resected. In the other patient, who had associated 22q11 chromosomal deletion, in addition to left-sided compression of the trachea and esophagus, there was additional marked compression of the right anterolateral trachea by the redundant ascending aorta. Through a median sternotomy, the ligamentum arteriosum was divided, and the trachea and esophagus were widely mobilized; an additional aortopexy of the ascending aorta to the right of the sternum resulted in the absence of tracheal compression. The cases of the two reported patients illustrate the clinical variability of vascular ring, including a right cervical aortic arch and the consequently versatile surgical approach that is needed to successfully address this combination of vascular anomalies.