Double aortic arch: an unusual congenital variation
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ORIGINAL ARTICLE
Double aortic arch: an unusual congenital variation K. S. Satyapal • L. Lazarus • D. Shama
Received: 12 June 2012 / Accepted: 3 October 2012 Ó Springer-Verlag France 2012
Abstract Double aortic arch is a rare variation of the aortic arch that may cause tracheal and esophageal compression. Two postnatal cases of double aortic arch and their outcomes are reported. Both patients presented with stridor, repeated respiratory infections and episodes of apnea. Computerized tomography angiographic threedimensional reconstruction revealed that in both patients, the right common carotid artery and right subclavian artery had separate origins from the right loop of the double aortic arch. The left subclavian artery and left common carotid artery arose separately from the left aortic arch. Both patients underwent corrective surgery and made an uneventful recovery. Keywords Double aortic arch Vascular ring Aortic arch variations Stridor
Introduction A double aortic arch refers to the congenital vascular variation characterized by the formation of an anatomically complete ring [37] around the trachea and esophagus due to the persistence of the right eighth segment of the dorsal aortic root during development of the branchial arteries [5].
K. S. Satyapal (&) L. Lazarus Department of Clinical Anatomy, School of Laboratory Medicine and Medical Sciences, College of Health Sciences, Westville Campus, University of KwaZulu-Natal, Private Bag X54001, Durban 4001, South Africa e-mail: [email protected] D. Shama St Augustine’s Hospital, Chelmsford Medical Centre, Durban, KwaZulu Natal, South Africa
This condition was first described by Hommel in 1737 as cited by Misser and Narsai [26]. Such variations are the result of abnormal regression of parts of the aortic arches that arise from the truncus arteriosus during fetal life and may result in complete or incomplete encirclement of the trachea, esophagus or both [6, 46]. Gross [13] introduced the term ‘‘vascular ring’’ to describe these mediastinal vascular variations. He reported a successful surgical division of a ‘‘vascular ring’’ due to a double aortic arch. Vascular rings are formed by double aortic arches with equal right and left components or with a smaller or atretic right or left component; the descending aorta may either be right or left sided, and there may be a right, left or bilateral patent ductus arteriosus or a ligamentum arteriosum [22, 29, 47]. In more than 75 % of cases, the right arch is dominant [38]. As mentioned above, the vascular ring formed by the double aortic arch may compress the trachea and esophagus and may cause serious life threatening symptoms such as dyspnea, stridor, cough, recurrent respiratory tract infections or dysphagia [44] in infancy and childhood that requires surgical intervention [2]. A detailed knowledge of such vascular variations is critical for image diagnosis and surgical intervention [28]. Two clinical cases in children that were diagnosed and treated with double aortic arch are reported.
Case reports C
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