Vascular Ring and Airway Program

The clinicians at Boston Children’s Vascular Ring and Airway Program and the Esophageal and Airway Treatment Center work together to provide collaborative care and, when necessary, surgery for children who have vascular rings and airway or esophageal compression. Our team is comprised of cardiac surgeons, general surgeons, cardiologists, pulmonologists, gastroenterologists, otolaryngologists and speech-language pathologists, orthopedic surgeons, and other specialists.

The breadth of our experience allows us to take a comprehensive approach to the diagnosis and management of vascular rings. This unique collaborative effort helps to ensure that the condition is treated thoroughly — with the goal of preventing the need for future operations. In addition to surgically repairing vascular rings, our skilled surgeons revise incomplete repairs and repairs that were complicated by recurrent symptoms. Our team also monitors for symptoms in children who are not designated for surgery, ensuring an appropriate treatment can be immediately pursued if surgery is eventually necessary and also to assess symptoms that are not directly related to the vascular ring.

When our team receives a second opinion request — whether for a prenatal case, a child, or an adult — our administrative clinical coordinators work to have sufficient patient records so that we can thoroughly review the case at our multidisciplinary Vascular Ring and Airway team conference. We review CT chest angiograms, chest MR angiograms, esophograms, modified barium swallows, bronchoscopies, endoscopies, direct laryngoscopies, echocardiograms, pulmonary function tests, and more.

If our team is scheduled to review your child’s case at our conference, our advanced practice providers, including nurse practitioners and physician assistants, will reach out to gather a thorough medical history and specific questions from your family. Once our team discusses your child’s case, we communicate directly with your family to review our recommended plan and answer any questions. The recommendations from our conference can include monitoring symptoms, obtaining additional testing, or pursuing a surgical repair of the vascular ring.

Additional testing may be recommended depending on a child’s symptoms and anatomy. For example, if your child feels that solid foods are getting stuck when trying to swallow, we might recommend an esophagram to objectively assess if they have esophageal compression. If surgery is recommended, we often recommend baseline studies. For instance, we often recommend, prior to surgery, a baseline vocal cord function evaluation and an echocardiogram to confirm a patient’s baseline heart function. If your child is recommended for symptom monitoring, our team will be available to you throughout scheduling and the follow-up process. Our administrative team coordinates visits in a way that works best for your family.

Boston Children’s has been at the forefront of treatment of vascular ring and airway compression for nearly a century. In 1945, Dr. Robert E. Gross pioneered a surgical procedure to treat double aortic arch, a common type of vascular ring, by dividing one of the two aortic arches. Today, our world-renowned team of specialists continue to develop innovative treatments and update conventional ones to create the most appropriate individualized care plans for children with vascular rings.

Surgery is pursued when our team and a family together agree that the benefits of an operation outweigh the risks. The exact surgical plan is determined based on a patient’s vascular ring anatomy and areas of compression to the esophagus and airway. Dr. Christopher Baird, a cardiac surgeon, and Dr. Benjamin Zendejas-Mummert, a general surgeon, are typically involved in each case. Additional surgeons may be involved depending on the recommended type of repair.

Our program and approach to these problems are unique. We focus on the patient as a whole, rather than the condition as an isolated problem. It is important to note that the final surgical recommendation is patient-specific and based on each child’s anatomy. Each patient is evaluated for not only fixing the vascular ring anatomy itself, but also the potential need for additional interventions of the airway and esophagus.

Here are some of the proven, cutting-edge surgical techniques we offer to treat vascular rings:

• Complete resection of the diverticulum of Kommerell
• Descending aortopexy
• Rotational esophagoplasty
• Posterior tracheobronchopexy
• Aortic arch reconstruction (aortic uncrossing and aortic relocation)

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

Learn about our approach to treatment.

Our team also closely monitors patients over the long term after surgery. It is critical to monitor symptoms after a surgical repair to ensure surgery is durable and that a patient has the best possible recovery.

After surgery, your child will be cared for in either our Cardiac Intensive Care Unit (CICU) or Post-anesthesia Care Unit (PACU) before to transferring our Acute Cardiac Care Unit (ACCU). Our team will follow your family throughout your post-operative stay and be a resource to support you and your child throughout recovery. Following discharge, we will closely follow your child through in-person or virtual visits, ensuring they are recovering well and so that we can be available to your family as needed. We plan for virtual post-surgery visits at seven to 10 days, one month, six months, and one year — and beyond. We especially monitor for pain control, incision healing, and symptoms. Our team is always available to answer any questions you or your child may have along the way.

While some adult patients’ surgeries can be performed at Boston Children's Hospital, some patients would benefit from having surgery at Brigham and Women’s Hospital. We work closely with Brigham and Women’s, including their cardiac surgery, gastrointestinal, pulmonary, and ear nose and throat providers, to ensure our adult patients receive comprehensive testing and evaluation. This same team also works together to coordinate surgical repairs at Brigham and Women’s, while still including our primary surgeons in the cases. Our team will follow each adult patient closely post-operatively and establish the same post-operative visits as noted above.