Pediatric Double Aortic Arch

  • Etiology: vascular ring due to persistence of right and left aortic arches
  • UGI: focal anterior indentation of trachea and focal posterior indentation of esophagus
  • Imaging: arch location is bilateral, right arch is higher and larger, left arch is smaller and lower, 70% are right sided dominant, branching is 4 vessels, descending aorta is typically on the opposite side of the dominant arch, Kommerell diverticulum is not present
  • Complications: airway compression
  • Clinical: Most common vascular ring, rarely associated with congenital heart disease

Radiology Cases of Double Aortic Arch

Upper GI of double aortic arch
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.
Angiogram of double aortic arch
The upper GI (not provided) showed a focal posterior indentation of the esophagus. AP and lateral images from an angiogram shows the aortic arch to have left and right components.

Surgical Cases of Double Aortic Arch

Surgical image of double aortic arch
Surgical image through a left posterolateral thoracotomy shows the red vessel loops around the double aortic arch and the blue vessel loops around the ligamentum arteriosum. The posterior aortic arch was divided, causing an impressive release of the ring with the 2 ends of the posterior arch separating by 2 to 3 centimeters. The ligamentum arteriosum was then divided.