Children with vascular rings and vascular compression of the airway and/or esophagus need care from a team with experience treating these rare, complex congenital conditions. Traditionally, patients have often experienced recurrence of vascular rings and continued vascular compression even after treatment and have had to live with the breathing and swallowing challenges.

Our team in the Esophageal and Airway Treatment Program at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida, has developed innovative, comprehensive diagnostic and treatment techniques to provide patients with vascular rings and vascular compression with the care needed to effectively repair the defect and optimize airway and esophageal function.

A vascular ring occurs when the aorta — the main artery of the body, originating from the heart’s left ventricle (or pumping chamber) — or other blood vessels form abnormally during fetal development and wrap around the trachea (the windpipe) and the esophagus. This causes compression of the trachea and esophagus that leads to breathing and swallowing problems.

Sometimes the blood vessels form a complete ring, and often they form an incomplete ring but still lean on or compress the airway and/or esophagus. The details matter and understanding the nuanced details of each patient’s condition is often the difference between unsuccessful and successful treatment. Our team provides this expertise in diagnosis and treatment.

Why Choose Johns Hopkins All Children’s

Expert Multidisciplinary Team

Our program is a collaboration between our pediatric thoracic surgeons who have deep experience in the surgical treatment of rare, complex esophageal, airway and thoracic conditions, and our pediatric cardiothoracic surgeons who provide advanced specialized care for vascular and cardiac repairs.

Our team includes pediatric anesthesiologists who are experienced in meeting the unique anesthesia needs of children needing surgery for airway conditions, and we also work closely with pediatric gastroenterology specialists to address esophageal and swallowing function in our patients. The hospital’s radiology specialists and nutrition teams also provide additional expertise in caring for these patients. Learn more about our team.

Advanced Diagnostic Techniques

Understanding the full scope of a child’s airway condition is essential to providing them with the best treatment. Our team developed a complete dynamic three-phase airway exam that allows us to perform a full assessment of your child’s airway before surgery. Using various methods, we examine the structure and function of the vocal cords, larynx (or voice box), trachea, esophagus and into the stomach if needed, to establish a complete picture of your child’s current condition. This thorough testing allows us to understand the problems and carefully plan your child’s treatment, and identify additional issues that may be associated with the vascular rings so that your child receives all the treatment they need.

Innovative Care

Our team has developed unique strategies to treat vascular rings. During surgery we monitor the nerves of the vocal cords. Historically, vocal cord injury is a risk of vascular ring surgery and monitoring the vocal cord nerves during surgery is uncommon, but this monitoring is part of our protocols to minimize the risk of vocal cord injury during surgery.

We also prioritize fixing the compression of the airway in a way that minimizes the risk of recurrence. The constant beating of the blood vessel against the esophagus in a vascular ring causes scar tissue, and it is often an abnormal bulge in the aorta called Kommerell’s diverticulum that causes the actual compression. We divide the scar tissue and remove the diverticulum to alleviate pressure on the esophagus and protect against recurrence.

Additional tracheal anomalies may also result from the compression of the trachea — we address these as well during the same surgery. We have also developed minimally invasive techniques to fix some forms of blood vessel compression without a large incision in the chest, allowing for a faster recovery.

Recurrence can occur as a complication from a previous surgery to correct vascular rings, particularly if the diverticulum or scar tissue were not adequately addressed. Our team has the expertise to address these concerns and effectively manage the underlying issues of your child’s condition, providing the best chance for lasting results and improved quality of life.

Our Approach to Treatment

Proper treatment begins with an accurate diagnosis of all the problems. We’ve developed a comprehensive three-phase exam that allows us to get a complete picture of your child’s airway. Our goal is to understand your child’s airway and the full scope of their conditions so we can create a treatment plan to address their individual needs.

Testing includes:

Flexible laryngoscopy: A thin, flexible camera is used to examine the nasal passages, throat, and vocal cords, allowing us to look at the structure of the vocal cords and how they work when your child is breathing normally.

Bronchoscopy: Done with anesthesia under the close care of our expert anesthesia team, a thin tube with a camera is used to examine the trachea, bronchi (the passages between the trachea and the lungs), and smaller airways. We can examine things like the anatomy of the trachea and the shape of the cartilage of the trachea and identify secretions that can be sent to the lab for testing, all of which may indicate the extent of the compression and help uncover related issues.

Positive pressure testing: Under anesthesia, we use positive pressure in the airway to open the posterior membrane. This allows us to see things like holes or fistulas to the esophagus, connections to abnormal lung tissue, strictures and narrowing of the airway, and aids us in identifying any rare anomalies that may be contributing to your child’s airway issues before surgery.

Additional testing includes CT scan with IV contrast so we can evaluate the structures in the chest and better plan for surgery, as well as a complete esophageal evaluation, and fluoroscopy exam if needed (fluoroscopy uses X-ray to provide a view of the internal organs working in real time).

What to Expect

During your child’s first appointment with us, we will typically listen to their lungs, evaluate their coughing, and get a full medical history. We will discuss our findings with you and what kind of testing we recommend. We will typically schedule a CT scan with IV contrast, as well as endoscopic evaluation of airway and esophagus, to understand the blood vessels and their impact on the esophagus and the airway, and as an aid to planning the treatment – like a road map for us.

Our program is family- and patient-centered, and we work as much as possible to make your experience in our program comfortable and streamlined. We schedule your child’s complete three-phase evaluation and CT scan for the same day to minimize the number of appointments your child needs.

We follow all our patients long-term after surgery. Typically, we’ll have a phone follow up with you about a week after your child’s discharge to home, and then they will have an appointment with us in our clinic about a month after the surgery. We understand the challenges faced by our patients from other states and other countries, and will work with you and your home physicians. We’ll determine appropriate follow up from there depending on your child’s condition.