Pediatric Double Aortic Arch

Etiology: Vascular ring due to persistence of right and left aortic arches
Imaging UpperGI: 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
DDX:
Complications: Airway compression
Treatment:
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.

UGI of double aortic arch — 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.

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.