Repair of a Complete Vascular Ring in a 15-Month-Old "Right Aortic Arch With Aberrant Retroesophageal LSCA and Persistent KD"

We present to you the surgical repair of a complete vascular ring consisting of a right aortic arch with a retroesophageal aberrant left subclavian artery and a persistent diverticulum of Kommerell. This is a 15-month-old girl who was diagnosed with a vascular ring prenatally. She was asymptomatic; however, the parents elected to proceed with elective repair considering the size of the Kommerell diverticulum and the presence of mechanical compression on the esophagus on computed tomography scan.

Chest x-ray shows a right aortic arch, and echocardiography did not demonstrate any structural heart defects. Computed tomography scan shows right aortic arch with retroesophageal left subclavian artery and persistence diverticulum of Kommerell that is causing mechanical compression on the esophagus.

After induction of general endotracheal anesthesia, a paravertebral catheter is placed for postoperative pain management, in addition to placement of the necessary monitoring lines including cerebral and somatic NIRS (near infrared spectroscopy), left radial and a femoral arterial lines, in other terms, all four extremities are routinely monitored. The patient is positioned in the right lateral decubitus position. The left chest is entered through the left third intercostal space. The left lung is retracted medially, and the mediastinal pleura is opened on top of the esophagus and left subclavian artery. We take considerable time to identify all components of the vascular ring including the left subclavian artery, diverticulum of Kommerell, the proximal descending aorta, left ligamentum arteriosum and the right aortic arch.

The ligamentum is then doubly ligated and divided. Heparin at 100 units/kg is administered systemically and a side-biting clamp is applied at the base of the diverticulum of Kommerell. Distal control is obtained on the left subclavian artery. The diverticulum is resected in its entirety and its site is secured with a double layers running 5/0 polypropylene suture. The left common carotid artery is then identified either anterior or posterior to the phrenic nerve. The left subclavian artery is then translocated to the left common carotid artery in an end-to-side fashion using running 7/0 polypropylene suture and flow is restored to the left arm.

We do not close the mediastinal pleura in these cases to avoid recurrence of scarring with possibility of development of symptoms. A single chest tube is placed and the incision was closed in the standard fashion.

The patient was extubated in the operating room, received no transfusions and the rest of the hospital course was uneventful. She was discharged on the fourth postoperative day.

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References

1. Backer CL. Vascular Rings With Tracheoesophageal Compression: Management Considerations. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2020;23:48-52
2. Backer CL, Mongé MC, Popescu AR, Eltayeb OM, Rastatter JC, Rigsby CK. Vascular rings. Semin Pediatr Surg. 2016 Jun;25(3):165-75
3. Backer CL, Russell HM, Wurlitzer KC, Rastatter JC, Rigsby CK. Primary resection of Kommerell diverticulum and left subclavian artery transfer. Ann Thorac Surg. 2012 Nov;94(5):1612-7

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