A vascular ring is a congenital defect in which there is an abnormal formation of the

aorta The aorta ( ) is the main and largest artery in the human body, originating from the left ventricle of the heart and extending down to the abdomen, where it splits into two smaller arteries (the common iliac arteries). The aorta distributes ...

and/or its surrounding

blood vessel The blood vessels are the components of the circulatory system that transport blood throughout the human body. These vessels transport blood cells, nutrients, and oxygen to the tissues of the body. They also take waste and carbon dioxide away ...

s. The

trachea The trachea, also known as the windpipe, is a Cartilage, cartilaginous tube that connects the larynx to the bronchi of the lungs, allowing the passage of air, and so is present in almost all air-breathing animals with lungs. The trachea extends ...

and

esophagus The esophagus (American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the ...

are completely encircled and sometimes compressed by a "ring" formed by these vessels, which can lead to breathing and digestive difficulties. Most often this is because of persistence of the

double aortic arch Double aortic arch is a relatively rare congenital cardiovascular malformation. DAA is an :wikt:anomaly, anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the vertebrate trachea, trachea and/o ...

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 In humans, the circulatory system is different before and after birth. The fetal circulation is composed of the placenta, umbilical blood vessels encapsulated by the umbilical cord, heart and systemic blood vessels. A major difference between the ...

) that may connect between the aorta on the front and the left subclavian artery posteriorly going to the left arm.

Symptoms

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Diagnosis

Infants with vascular rings typically present before 12 months with respiratory or esophageal symptoms like stridor, wheezing, cough, dysphagia, or difficulty feeding. The stridor improves with neck extension, differentiating from

laryngomalacia Laryngomalacia (literally, "soft larynx") is the most common cause of chronic stridor in infancy, in which the soft, immature cartilage of the upper larynx collapses inward during inhalation, causing airway obstruction. It can also be seen in olde ...

which is relieved by prone or upright positioning, and will not be relieved with corticosteroids or epinephrine, unlike

croup Croup, also known as laryngotracheobronchitis, is a type of respiratory infection that is usually caused by a virus. The infection leads to swelling inside the trachea, which interferes with normal breathing and produces the classic symptoms o ...

. Diagnosis requires a high degree of clinical suspicion and can be confirmed with barium contrast esophagogram for those with esophageal symptoms, bronchoscopy, or CT or MRI.

Presentation

Symptoms

Diagnosis

Treatment

References

External links