double aortic arch

Angeborene
Anomalie des Aortenbogens mit Gabelung der aufsteigenden Aorta; Jeder der Äste verläuft über den jeweiligen Bronchus und sie vereinigen sich hinter der Speiseröhre zur absteigenden Brustaorta.
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Infant who is
choking with feedings. Lateral (right) image from an UGI exam shows a persistent posterior indentation on the mid-esophagus. There is associated narrowing of the trachea at the same level. The diagnosis was double aortic arch causing a complete vascular ring.
pediatricimaging.org • CC-by-nc-sa 4.0
Double Aortic
Arch: An Incidental or Sinister Finding. Frontal chest radiograph depicts widening of the superior mediastinum with prominence and calcification of the aortic knob. Lateral view reveals anterior displacement of the trachea with retrotracheal soft tissue density (red arrow)
www.eurorad.org • CC by-nc-sa-4.0
Double Aortic
Arch: An Incidental or Sinister Finding. Barium swallow, frontal and lateral views, show extrinsic impressions over upper thoracic oesophagus. Lateral view demonstrates impressions along the anterior and posterior walls of oesophagus.
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Double Aortic
Arch: An Incidental or Sinister Finding. Image A normal origin aorta Image B and C bifurcation of the ascending aorta. Image D double aortic arches Image E anomalous right arch merging with the left arch . Image F thoracic aorta.
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Computed
tomography in the evaluation of vascular rings and slings. Double aortic arch. a Illustration showing the appearances of a double aortic arch, with a dominant right arch. 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. Both the trachea and oesophagus are completely encircled and compressed by the vascular ring. b Axial CT scan shows the four-vessel sign. 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. c Axial MIP image shows bilateral aortic arches encircling the trachea and oesophagus. The right aortic arch (arrow) is larger than the left aortic arch (arrowhead). 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). e Coronal MIP CT image at the level of the trachea shows similar-sized double arches in another patient (arrows). 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. 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. h Coronal volume-rendered image of the airway shows severe narrowing of the airway (arrow) by the double aortic arch
insightsimaging.springeropen.com • Open Access
Computed
tomography in the evaluation of vascular rings and slings. Double aortic arch with an atretic left arch. a and b Axial CT scan images show bilateral aortic arches. The right aortic arch is dominant (arrow). The left aortic arch is atretic (arrowhead). c 3D volume-rendered image demonstrates the double aortic arch. The dominant right aortic arch (arrow) gives off the right subclavian and right common carotid branches, while the left atretic aortic arch gives off the left common carotid and left subclavian arteries proximal to the atretic region (arrowhead). Only the right aortic arch joins the descending aorta (*). d Virtual bronchoscopy demonstrates severe narrowing of the airway by the double aortic arch with atretic left arch
insightsimaging.springeropen.com • Open Access
Computed
tomography in the evaluation of vascular rings and slings. Post-surgical changes of double aortic arch repair. Postoperative sagittal 3D volume-rendered image demonstrates separation of the nondominant left aortic arch (arrow) from the descending aorta and surgical interruption of the complete vascular ring
insightsimaging.springeropen.com • Open Access
Preschooler
with wheezing. CXR (left) shows a right paratracheal mass. AP image from an UGI exam (middle) shows kinking of the upper esophagus while lateral (right) image shows a posterior indentation on the esophagus.The diagnosis was double aortic arch.
pediatricimaging.org • CC-by-nc-sa 4.0
Double Aortic
Arch: An Incidental or Sinister Finding. Axial MR image depicts two patent aortic arches completely encircling both trachea and oesophagus.
www.eurorad.org • CC by-nc-sa-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Double aortic arches are the most common symptomatic type of the aortic arch variant. It may account for up to 50-60% of vascular rings.

Clinical presentation

Double 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.

Pathology

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.

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 .

Subtypes
  • right dominant arch: ~75-80%
  • codominant arch: ~5%
  • left dominant arch: ~25%
Associations

It is rarely associated with congenital heart disease .

Radiographic features

Plain radiograph

Right-sided aortic arch indenting the trachea and increase right paratracheal soft tissue thickness. Lack of air column in the thoracic portion of the trachea is sometimes seen.

Fluoroscopy: barium swallow

Contrast swallow studies are more helpful and demonstrate:

  • 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
  • posterior indentation on lateral view produced by the encircling posterior arch (mostly right-sided aortic arch component)
CT and MRI

Contrast-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.

Treatment and prognosis

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.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu doppelter Aortenbogen:
Anomalien des
Aortenbogens
Wikipedia • CC BY-SA 4.0
doppelter
Aortenbogen mit atretischem linkem Bogen
insightsimaging.springeropen.com • Open Access