Vascular ring describes an unusual formation of the aorta and the blood vessels around it. Children are diagnosed with vascular rings when their trachea (the tube that carries air to the lungs) and their esophagus (the tube that carries food to the stomach) are circled by a ring of blood vessels. Typically, the ring puts pressure on the trachea and esophagus, which can make it hard to breathe and eat.
While these rings are normal during fetal development, the body usually breaks them down before birth. Occasionally these blood vessels aren’t broken down and still exist when a child is born.
Vascular rings are rare and are present at birth. Symptoms usually begin appearing very early in life.
There are many kinds of vascular rings, but the two most common are double aortic arch and right aortic arch with aberrant subclavian and left ligamentum.
What Are the Symptoms of Vascular Rings?
Some children never develop symptoms. However, most children’s symptoms begin during infancy. The more pressure the rings apply to the trachea and esophagus, the more severe the symptoms will be.
Troubles with breathing may include:
- A high-pitched cough or wheezing
- Loud breathing
- Many respiratory infections or pneumonia
- Symptoms worsen when eating
Troubles with eating and digesting may include:
- Difficulty eating solid foods
- Swallowing difficulties
- Gastroesophageal reflux (GERD)
How Are Vascular Rings Diagnosed?
Most children have mild symptoms, and the condition can go undiagnosed for years, even into adulthood. However, children with more severe cases are often diagnosed a few weeks or months after being born. Occasionally, a vascular ring is diagnosed when a child is are being evaluated for a cardiac murmur, and the ring is present in association with other cardiac defects.
Physicians may use several tests to diagnose vascular rings, including:
- Medical imaging such as a chest x-ray, chest CT or a cardiac MRI
- An echocardiogram
- Trachea examination
- Gastrointestinal tests
How Are Vascular Rings Treated?
Vascular rings must be surgically corrected, though open-heart surgery isn’t required. Often surgeons make a surgical incision on the left side of the chest and go between ribs to enter the area around the heart. From there, the surgeon will split the vascular ring to relieve pressure on the esophagus and trachea, and then stitch the blood vessel closed.
Lurie Children’s surgeons have pioneered a number of procedures to advance the methods used to correct many different kinds of vascular rings. Our physicians were the first in the world to successfully repair a pulmonary artery sling, and continue to operate on more pulmonary artery slings than any other hospital in North America.
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