What are vascular rings / tracheal stenosis?
Vascular ring is used to describe several anomalies that result from abnormal development of the aortic arch complex and cause compression of the trachea, esophagus or both.
Different kinds of vascular rings include:
- Double aortic arch
- Right aortic arch/left ligamentum
- Innominate artery compression
- Pulmonary artery sling
- Complete tracheal rings
When an infant is born with tracheal stenosis, there is no normal membranous trachea, and at the site of the complete rings the tracheal lumen is very small, often only 2 to 3 millimeters.
How are vascular rings/tracheal stenosis diagnosed?
Most infants with vascular rings present with symptoms in the first few months of life, and require surgery. Some of these anomalies are anatomically complete rings or “true” vascular rings while others are anatomically incomplete or partial vascular rings but still produce similar symptoms because of the tracheoesophageal compression. The symptoms include:
- Respiratory distress
- Stridor (noisy breathing)
- Barky cough
- Apnea (pauses in breathing)
- Dysphagia (trouble swallowing)
- Recurrent respiratory tract infections
Some older children will present with symptoms of dysphagia or slow feeding. Infants may tend to hold their head hyper-extended to lessen the obstruction therefore improving their breathing. Apnea or cyanosis (blue spells) may be precipitated by swallowing a bolus of solid food that presses on the soft posterior trachea within the restrictive confines of a ring. When infants progress from formula to solid foods the symptoms become more evident.
The diagnosis of vascular rings begins with a chest x-ray. There are many other diagnostic tests, such as a barium esophagram, bronchoscopy, tracheograms, CT scan, MRI, ECHO and cardiac catheterization. Often, patients begin with a chest x-ray and ECHO. If there is suspicion of a vascular ring, it can be confirmed with a CT scan. All patients undergo a bronchoscopy at the time of surgery.
Infants born with complete tracheal rings often present with life-threatening respiratory distress in infancy.
How are vascular rings/tracheal stenosis treated?
Our Heart Center has made many significant historic contributions to the understanding and treatment of children with vascular rings, including:
- 502 patients in 63 years, the largest reported series in North America
- First PA sling repair in 1953
- First pericardial tracheoplasty in 1982
- First tracheal autograft in 1996
An operation is indicated in all symptomatic patients who have a vascular ring. Initiation of early surgical intervention is important to avoid serious complications that may arise after hypoxic or apneic spells.
For those patients with double aortic arch and right aortic arch/left ligamentum, the surgical approach is through a left thoracotomy incision with division of the lesser of the two arches (in double aortic arch), and division of the ligamentum in cases of right aortic arch.
Innominate artery compression is repaired via a small right thoracotomy incision through which the innominate artery is secured with sutures to the posterior sternum simultaneously pulling the anterior tracheal wall open.
Tracheal stenosis and pulmonary sling often are associated together. Both are surgically repaired via mediansternotomy with open heart surgery. The pulmonary artery sling (vascular anomaly) is repaired by transecting the left pulmonary artery and anastomosing it to the main pulmonary artery. The tracheal stenosis is repaired either by resecting the abnormal area or performing a slide tracheoplasty.
What is the long-term outlook for a patient with vascular rings/tracheal stenosis?
Complete relief of symptoms may not be noted immediately after surgery, but most patients are significantly improved. Several months to one year may be required before disappearance of the noisy respirations caused by tracheomalacia. In general terms, the long-term outlook is very good.
Learn more about the specialized teams caring for these patients: