Specialty medical genetics ICD-9-CM 747.21 eMedicine med/2981 | ICD-10 Q25.4 MedlinePlus 007318 | |
A vascular ring is a congenital defect in which there is an abnormal formation of the aorta and/or its surrounding blood vessels. The trachea and esophagus are completely encircled and sometimes compressed by a "ring" formed by these vessels, which can lead to breathing and digestive difficulties.
Contents
Most often this is because of persistence of the double aortic arch after the second month of fetal life.
Presentation
The two arches surround the esophagus and trachea which, if sufficiently constrictive, may cause breathing or swallowing difficulties despite medical therapies.
A less common ring is present with a right aortic arch instead of the usual left-sided aortic arch. This compresses the esophagus and trachea because of the persistence of a ductal ligament (from fetal circulation) that may connect between the aorta on the front and the left subclavian artery posteriorly going to the left arm.
Treatment
It is sometimes treated with surgery.