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What is a vascular ring?

In general, a vascular ring occurs when a child’s aorta — the body’s largest blood vessel — or its branches form abnormally, so that they encircle and constrict the trachea and usually the esophagus. This compression can cause breathing and swallowing problems.

There are several types of vascular rings. The most common are:

  • double aortic arch. In this type of ring there are two aortic arches. Typically, one of the arches is dominant and the other is small and underdeveloped, although they can be equal in size. The branches from these two aortic arches surround the trachea and esophagus.
  • right aortic arch with aberrant subclavian and left ligamentum. In this type of vascular ring, the child’s aortic arch curves right and the left subclavian artery, which branches from the aortic arch, passes behind their esophagus and airway instead of in front of the airway. The ligamentum arteriosum (a blood vessel remnant) passes between the left subclavian artery and left pulmonary artery, completing the ring.
  • left aortic arch with aberrant right subclavian artery. In this type, the right subclavian artery passes behind the trachea and esophagus, which become compressed.

How we care for vascular rings at Boston Children’s Hospital

The clinicians in Boston Children’s Esophageal and Airway Treatment Center and Benderson Family Heart Center work together to provide collaborative care and, when necessary, surgery for children with vascular rings and airway or esophageal compression. We are the only hospital in the U.S. to repair vascular rings with a combination of these procedures in one comprehensive surgical procedure. This helps ensure that the problem is treated thoroughly and prevents the need for future procedures. This depth and breadth of experience also allows us to revise incomplete or failed repairs performed elsewhere with recurrence of airway or esophageal symptoms.

Our areas of innovation for vascular rings

Surgery to divide the vascular ring was pioneered at Boston Children’s Hospital in 1945, with the first successful operation on a child with a double aortic arch. Today, we sometimes perform this procedure using robotic surgery equipment, allowing your child the benefits of minimally invasive surgery and improved surgical precision. We also offer the latest surgical techniques for treating vascular rings.

Vascular Ring | Symptoms & Causes

What are the symptoms of a vascular ring?

As their name suggests, vascular rings are caused by malformed blood vessels. But the vessels themselves aren’t really the issue, and some people without symptoms may live their entire lives not even realizing that they have a vascular ring. Instead, symptoms occur when a vascular ring puts pressure on a child’s esophagus, trachea, or both. Symptoms range widely depending on the severity of the compression and can include:

Children with vascular rings often also have an airway disorder called tracheomalacia. In this condition, the trachea narrows or collapses when your child exhales, which makes it feel hard to breathe and may lead to a vibrating noise or cough. Like vascular rings, tracheomalacia can be present at birth, or it can develop in response to vascular rings or other anomalies.

What causes a vascular ring?

Normally, the aorta develops from one in a series of symmetrical arches. By the end of the second month of fetal development, the other arches are naturally broken down or formed into arteries. When a vascular ring occurs, certain arches that should have disappeared still remain and form a ring structure.

Vascular Ring | Diagnosis & Treatments

How is a vascular ring diagnosed?

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

The gold standard for evaluating and identifying vascular rings is a computed tomography (CT) scan. This technique allows physicians to visualize your child’s vascular anatomy properly. Vascular rings can be diagnosed at any age, including while a child is still in the womb.

What are the treatment options for a vascular ring?

Children who with vascular rings but no symptoms usually don’t need surgical treatment. However, those who have symptoms from vascular rings typically require to surgery to relieve pressure on their airway 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

Boston Children’s is the only hospital to repair vascular rings with a combination of these procedures in one comprehensive surgical procedure.

Vascular Ring | Programs & Services