Vascular Ring | Diagnosis & Treatment

How is a vascular ring diagnosed?

If your child has any symptoms of a vascular ring — particularly noisy breathing or a weak pulse — your child’s doctor may refer you for testing. He or she may order one or more of the following tests to help diagnose this condition:

What are the treatment options for a vascular ring?

Children who don’t have symptoms may not need treatment.

Children who have symptoms usually need to surgery to divide the vascular ring and relieve pressure on the windpipe and esophagus. Surgeons may use a variety of techniques to accomplish this, including:

  • complete resection of the diverticulum of Kommerell, which is typically left as an out-pouching of the aorta and compresses the esophagus and airway from the back 
  • descending aortopexy, which moves the descending aorta to the side of the spine so it doesn’t compress the airway
  • rotation esophagoplasty, which moves the esophagus out of the airway so it doesn’t contribute to airway compression and can’t be compressed itself by the aorta and airway
  • posterior tracheobronchopexy, which keeps the airways open in children with vascular rings who also have tracheobronchomalacia below the narrow, compressed region of the airway. Occasionally, these children will also need anterior airway support to completely open the airways.
  • aortic uncrossing, which reroutes the aorta and can be combined with the airway procedure

What is the outlook for children with a vascular ring?

Children who have surgery for a vascular ring usually don’t need any other treatment or follow up. Those who don’t have surgery should see a cardiologist periodically to make sure the condition doesn’t get worse.