Incomplete double aortic arch

Case contributed by Zentout Sofiane
Diagnosis certain

Presentation

Dyspnea and dysphagia.

Patient Data

Age: 15 years
Gender: Male

An incomplete vascular ring encircles the trachea and esophagus which are displaced and compressed. The right arch is larger and higher and the left arch is atretric posteriorly.

The right common carotid artery and subclavian artery arise directly from the right arch and the left common carotid artery and subclavian artery arise directly from the left arch giving rise to the symmetrical four vessel sign.

The descending aorta arises from the right arch and is positioned more anteriorly than normal.

Case Discussion

This vascular ring is caused by a type 1 or 2 incomplete double aortic arch with an atretic posterior left arch distal to the subclavian artery. This atretic segment inserts into the right ductus diverticulum and can be surgically divided to relieve tracheal and esophageal compression.

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