 Vascular Ring
|
|
 |
|
|
|
A vascular ring is a congenital defect, which means it was present at birth. It occurs when there is an unusual formation of the aorta and/or its surrounding vessels. The improperly formed vessel configuration becomes problematic because the trachea and esophagus are completely encircled and compressed by a "ring" formed by these vessels, which leads to both breathing and digestive difficulties.
There are several types of vascular rings, each named for the erroneous configuration of vessels. The most common of these are:
|
|
|
- Double aortic arch: With this anomaly, there are two aortic arches on both the anterior left side of the heart and posterior right side of the heart. Typically, one of the arches is dominant and the other is small and underdeveloped (hypoplastic). Usually the right arch is largest. The trachea and esophagus are encircled by the two aortic arches.
- Right aortic arch with aberrant subclavian and left ligamentum: Normally, the arch of the aorta extends left from the heart has three main vessels that branch from it. These are the innominate artery, the left common carotid artery and left subclavian artery. In this kind of vascular ring, there is a right aortic arch that gives off the left common carotid, the right common carotid, the right subclavian and the left subclavian arteries. The left subclavian passes behind the esophagus. A ligament that normally occurs called the ligamentum arteriosum, passes between the left subclavian and left pulmonary artery to complete the ring.
|
|
|
|
For in-depth visual information on this condition, visit the Children's Hospital Boston Multimedia Library.
|
|
|
|
|
|
A vascular ring originates during gestation. Normally, the aorta develops as one in a series of symmetrical arches. By the end of the second month of fetal development, some of these arches are naturally broken down (resorbed) and others persist. The series of arches diminishes leaving the aorta with its three main branch arteries. When a vascular ring occurs, certain arches that should have diminished naturally continue to persist.
|
|
|
|
|
|
Since vascular rings encircle both the esophagus and trachea, your child may experience symptoms that result from obstruction of both. Symptoms range widely depending on how severe the compression is. Typically, if the condition is severe, symptoms occur during infancy, even as early as the first months of life. In some cases, vascular rings do not seem to compress the trachea and esophagus and causes no symptoms. In others, symptoms don't occur until later in childhood or even adulthood. The following are the most common symptoms of vascular rings:
|
|
|
- Stridor (noisy breathing)
- Respiratory distress
- Difficulty feeding when solids are introduced to the infant
- Swallowing difficulties in children (dysphagia)
- Gastroesophageal reflux
- Respiratory infections
|
|
|
|
|
|
If your child experiences symptoms of a vascular ring, particularly noisy breathing, or a weak pulse, your child will probably be referred to a specialist and may undergo some combination of the following tests:
|
|
|
- Chest X-ray: Your child will initially have a chest x-ray. This diagnostic tests uses invisible electromagnetic energy beams to produce images of the internal tissues, bone and organs onto film. With a vascular ring, your doctor may be able to pick up the abnormal formation of the aorta. However, this isn't always possible.
- Upper GI (gastrointestinal) series: This is a diagnostic test that examines the organs of the upper part of the digestive system: the esophagus, stomach, and duodenum (the first section of the small intestine). A fluid called barium (a metallic, chemical, chalky liquid) is swallowed. X-rays are then taken to evaluate the digestive organs. This test will show any compression of the esophagus, which will look like an indentation. The radiologist who reads this test may be able to determine what type of vascular ring is present, depending on the angle of compression.
- Echocardiogram (Echo): Your child may have this test to exclude or uncover any other potential cardiac anomalies that might coincide with the vascular ring. This procedure is used to evaluate the structure and function of the heart by using sound waves recorded on an electric sensor that produce a moving picture of the heart and heart valves.
- Cardiovascular MRI: MRI is a way to take pictures of the body using a large magnet, radio waves and a computer. It is used to further evaluate the structure and function of the heart and blood vessels.
|
|
|
|
|
|
An operation to divide the vascular ring is the treatment of choice. This kind of surgery was pioneered at Children's Hospital Boston in 1945 with the first successful operation on a child with a double aortic arch.
Today, surgeons at Children's use state-of-the-art robotic surgery equipment to divide vascular rings. This technology allows the procedure to be performed using minimally-invasive surgery techniques, which reduce pain, scarring, and recovery time.
Specific treatment for a vascular ring will be determined by your child's physician based on:
|
|
|
- Your child's age, overall health, and medical history
- The extent of the disease
- Your child's tolerance for specific medications, procedures or therapies
- How your child's doctor expects the disease to progress
- Your opinion or preference
|
|
If either respiratory or digestive symptoms are present, your child's doctor will probably recommend surgery to the divide the ring. This is typically the recommendation even when the symptoms are minor, because the risks of this surgery are very low.
If your child is experiencing none of the symptoms mentioned above, surgery can be deferred, but your child's doctor will want to watch your child closely over time for any emerging symptoms mentioned above.
Prior to surgery, your child's symptoms will be treated. If there are digestive problems, your child may need nutritional support. If your child is experiencing respiratory problems, appropriate respiratory therapy or treatment for infections will be administered.
The goal of surgery is to relieve compression on the trachea and esophagus. Typically, surgery involves an incision through the left side of the chest. If repairing a double aortic arch, the ring is divided on its smaller side, which is usually the left side of the ring, although your doctor will determine this for certain prior to surgery. The fibrous strands of the ligamentum arteriosum should also be divided because they may be contributing to pressure on the esophagus.
When repairing a right aortic arch with aberrant subclavian and left ligamentum arteriosum, the ligamentum arteriosum that is connecting the aorta to the pulmonary artery is divided.
|
|
|
|
|
|
The long-term outlook typically depends on how severe the compression of the vascular ring was. In a young infant with severe respiratory symptoms, it may take several months for stridor or other respiratory symptoms to resolve. Even in older children, the compression of the trachea results in a weakening in the cartilage that may take several months to recover. Thus, symptoms may persist even after the operation until that occurs.
|
|
|
|
|
|
|
