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Vascular Ring with Unique Anatomical Variant Elucidated Using 3D Technology

Michael Hafertepe, BS, MS; Nhu-Nguyen Le, BS; Randy Richardson, MD; Randal Fortuna, MD


Vascular rings represent a rare subset of congenital cardiac anomalies that occur during the early development of the aorta. They are subdivided into two categories: complete rings that fully encircle the trachea and esophagus and incomplete rings that do not. Symptoms from vascular rings are generally related to compression of the airway and esophagus.[1]

Although the majority of patients with vascular rings are symptomatic in infancy and early childhood, it is not unusual for symptoms to manifest until later in life or not at all.[2] In symptomatic patients, prompt surgical treatment is of importance due to serious complications, such as major tracheal or bronchial damage that occurs or sudden death.[3]

FIGURE 1 Right aortic arch with aberrant left subclavian artery.

Many different forms of vascular rings exist due to the complex development of the aorta. However, two forms of complete vascular rings, the double aortic arch and the right aortic arch with an aberrant left subclavian artery (Figure 1), account for almost 90% of all cases.[4]

In this case, we present an asymptomatic pediatric patient with an extremely rare form of a complete vascular ring, a right aortic arch with normal subclavian architecture and ligamentum arteriosum completing the ring. We believe CT 3D reconstruction with inclusion of the esophagus provided detailed anatomical definition, which was crucial for accurate diagnosis and optimal treatment of the patient.

Case Report

A 4-year-old female patient, with history of recurrent emesis and reflux in infancy, presented with a chief complaint of fever. The initial diagnostic work-up, which included chest x-ray, revealed an incidental finding suspicious for a right aortic arch.

An initial CT chest angiogram with 3D rendering, which did not include the esophagus, was obtained for further evaluation. The images depicted a right aortic arch with mirror image branching and a Kommerell diverticulum with no aberrant left subclavian artery. The anatomy was not suggestive of a vascular ring and a diagnosis was not made at that time.

Due to clinical suspicion of a vascular ring, a repeat 3D CT reconstruction was obtained with inclusion of the esophagus and revealed an indentation on the esophagus (Figure 3). Together, these findings were consistent for diagnosis of a vascular ring encircling the esophagus completed by the ligamentum arteriosum (Figure 4). Maternal concern for long-term health effects of a complete vascular ring led to surgical correction of the anomaly.

Surgery confirmed that the ligamentum arteriosum connected from the thoracic aorta to the left pulmonary artery forming a complete vascular ring. The ligamentum arteriosum was divided. The patient tolerated the procedure well.

To read the full article, please go to the June 2016 Issue of CCT.