A compressive vascular ring: A case report and review of literature

Introduction

Double aortic arch (DAA) is one of the classes of congenital anomalies of the aortic arch system called vascular ring.

Abnormal circular formation of blood vessels are incircled around the trachea and oesophagus resulting in airway compression.

Objective

We report a case of a 2-month-old infant that was born at full term and had no medical or surgical history who presented with progressive stridor and dyspnea reported by the parents.

Methods

On examination, we noticed a stridor, with suprasternal and intercostal recessions.

Oxygen saturations on room air was 93% and chest auscultation revealed bilateral transmitted stridor. A chest X-ray was normal. Echocardiography was performed and revealed a left-right shunting atrial septal defect with dilated right chambers, in addition to a persistent ductus arteriosus with a doubt on double aortic arch.

Results/Expected results

Thoracic computed tomography (CT) was performed and a vascular ring, consisting of a double aortic arch, was found compressing and narrowing the trachea (Figure 1).

The CT allowed confirmation of the anomaly, its location and the severity of airway and oesophageal compression.

Three-dimensional reconstruction helped to plan surgical intervention.

The patient was then referred to the cardiothoracic surgery.

The intervention consisted of resecting the minor arch freeing the trachea.

Conclusion/Perspectives

DAA is a rare issue mostly diagnosed in childhood due to symptoms related to oesophageal and/or tracheal compression and obstruction.

It can be associated to cardiac malformations including ventricular septal defect and Fallot's tetralogy.

Left untreated, it may lead to significant morbidity and mortality from airway obstruction.

Surgical repair remains the mainstay of treatment and is indicated for patients with symptoms of airway or digestive compression or as a supplementary procedure in patients undergoing other cardiac surgery.

The principle of surgery is to relieve the vascular compression on the trachea and/or esophagus by the division of the lesser arch.