Surgical intervention with tracheostomy from the side of descending aorta and division of the minor (smaller) arch and ligamentum arteriosum is indicated for symptomatic patients. In appropriate cases, surgical planning is directed toward transection of either the smallest arch (if both arches are patent) or an atretic arch segment.Congenital anomalies of the aortic arch are uncommon and may be associated with other congenital cardiovascular diseases. posterior indentation on lateral view produced by the encircling posterior arch (mostly right-sided aortic arch component)Ĭontrast-enhanced cross-sectional imaging is required to confirm the diagnosis and also to delineate details of anatomy in particular right or left arch dominance for surgical planning.classic "reverse S" indentation of the contrast column on frontal view produced by an upper indentation of the right-sided aortic arch and the lower indentation is by the left-sided aortic arch.Fluoroscopy: barium swallowĬontrast swallow studies are more helpful and demonstrate: Lack of air column in the thoracic portion of the trachea is sometimes seen. Right-sided aortic arch indenting the trachea and increase right paratracheal soft tissue thickness. It is rarely associated with congenital heart disease. When the minor arch is markedly hypoplastic or atretic ( incomplete double aortic arch), the affected segment tends to be almost always distal to the left subclavian artery. This anomaly is caused by persistence of the right and left embryonic fourth aortic arches, which results in formation of a vascular ring from the splitting of the ascending aorta into two limbs that pass to either side of the trachea and esophagus (both of which get encircled), which then join as a single descending aorta. Clinical presentationĭouble aortic arch is mostly diagnosed in childhood due to symptoms related to esophageal and/or tracheal obstruction. Respiratory symptoms can be more common in infancy or early childhood while adult patients complain of difficulties in swallowing rather than respiratory difficulties due to tracheal development. It may account for up to 50-60% of vascular rings. h Coronal volume-rendered image of the airway shows severe narrowing of the airway (arrow) by the double aortic archĭouble aortic arches are the most common symptomatic type of the aortic arch variant. g 3D volume-rendered image in another patient (same patient as e) demonstrates the double aortic arch, with similar sizes of the right arch (arrow) and the left arch (arrowhead), both of which join the descending thoracic aorta (*), which is located on the left. f 3D volume-rendered image exquisitely demonstrates the double aortic arch, with a dominant right arch (arrow) and a smaller left arch (arrowhead), both of which join the descending thoracic aorta (*), which is located on the left. e Coronal MIP CT image at the level of the trachea shows similar-sized double arches in another patient (arrows). d Coronal reconstructed CT image at the level of the trachea shows a larger right arch (long arrow) and a smaller left arch (smaller arrow) encircling the trachea (asterisk). The right aortic arch (arrow) is larger than the left aortic arch (arrowhead). c Axial MIP image shows bilateral aortic arches encircling the trachea and oesophagus. The first branch is the right subclavian (RSC) artery, the second is the right common carotid artery (RCC), the third is the left common carotid (LCC) artery and the fourth is the left subclavian (LSC) artery. b Axial CT scan shows the four-vessel sign. Both the trachea and oesophagus are completely encircled and compressed by the vascular ring. The right arch gives off the right subclavian (RSC) and right common carotid (RCC) branches, while the left arch gives off the left subclavian (LSC) and left common carotid (LCC) arteries. a Illustration showing the appearances of a double aortic arch, with a dominant right arch. Tomography in the evaluation of vascular rings and slings.
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