Dysphagia lusoria is a rare congenital condition characterized by difficulty swallowing due to an artery compressing the esophagus. This condition is the result of a congenital defect of the artery, meaning that the person was born with the defect.
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What Is Dysphagia Lusoria?
Dysphagia lusoria (DL) is a congenital condition caused by an abnormality in the aortic arch that compresses the esophagus. The abnormal artery in the aortic arch is called the aberrant right subclavian artery (ARSA).
The ARSA typically comes off the right side of the aortic arch. But in dysphagia lusoria, it comes off the left side and loops around the esophagus, compressing it. This compression causes trouble swallowing.
What causes dysphagia lusoria?
This condition is caused by a congenital variation of the aberrant right subclavian artery. Patients with DL are born with their ARSA coming off the left side of the aortic arch rather than the right side.
Symptoms of DL may not be present until childhood or later in life due to the eventual hardening of the artery.
Dysphagia lusoria risk factors
Risk factors for dysphagia lusoria include:
- Age — Older people have less flexible esophaguses and more rigid blood vessels, contributing to dysphagia or difficulty swallowing.
- Esophageal dysmotility — When food has trouble moving through the esophagus, particularly in older people.
- Gender — The arterial defect is more common in women, who are at higher risk of vascular malformations.
- Other medical conditions — Certain syndromes carry a higher risk of vascular malformations, including Down, DiGeorge, and Edwards syndromes.
How common is dysphagia lusoria?
Dysphagia lusoria is a rare condition affecting up to 1% of Americans. Of all people with ARSA, nearly two-thirds have no symptoms. Of those who do experience symptoms, 90% have dysphagia or difficulty swallowing.
What Are the Signs and Symptoms of Dysphagia Lusoria?
Dysphagia lusoria may be diagnosed in childhood or later in life.
Symptoms of dysphagia lusoria in adults include:
- Acid reflux.
- Back pain or discomfort.
- Chest pain or discomfort.
- Cough.
- Difficulty breathing (dyspnea).
- Difficulty swallowing.
- Pain when swallowing (odynophagia).
- Regurgitation.
- Stridor – A raspy sound in the chest when breathing.
Symptoms of dysphagia lusoria in children include:
- Poor weight gain.
- Prolonged feeding or meal times.
- Refusing previously taken foods or liquids.
- Respiratory symptoms like dyspnea, pneumonia, stridor, and wheezing.
- Weight loss.
When should I see a doctor about my dysphagia lusoria symptoms?
While most people with ARSA don’t have symptoms, you should see your primary care provider if you have recurring bouts of dysphagia or swallowing difficulty.
How Do You Diagnose Dysphagia Lusoria?
Dysphagia lusoria is typically diagnosed through a series of tests, including a physical exam, barium swallow test, and CT scan.
For a barium swallow test, you swallow a chalky white substance known as barium to coat the inside of your upper gastrointestinal tract. The barium appears white on x-ray film and highlights your organs, their inner linings, and your swallowing motion. The barium test is used to detect compression of the esophagus.
A CT scan can help doctors visualize the aortic arch better and can confirm that the aberrant artery is causing compression of the esophagus.
How Do You Treat Dysphagia Lusoria?
Dysphagia lusoria can be treated through lifestyle changes and surgical intervention.
Lifestyle changes
At UPMC, your doctor will work with you to implement lifestyle changes to relieve symptoms of dysphagia lusoria. For mild symptoms, there are dietary changes that can help manage the condition, including:
- Chewing food fully.
- Eating food more slowly.
- Sipping liquids.
- Taking smaller bites.
More severe cases may require surgical intervention.
Surgical treatment
For patients who do not respond to lifestyle changes and have more severe cases, surgical intervention is the typical treatment approach.
For many cases, dilation or widening of the esophagus during endoscopy is an option. This procedure can provide long-term relief and can be repeated if symptoms return.
In very serious cases, surgery to remove the aberrant artery may be done. A vascular surgeon would first identify the specific anatomy of the person's arteries. They would then remove the aberrant artery and reconstruct the blood vessel in its appropriate position coming off the right side of the aortic arch.
Like any surgery, this procedure can have side effects. The most serious side effects are ischemia or restricted blood flow in the extremities and blood flow reversal through the vertebral artery.
Why Choose UPMC for Dysphagia Lusoria Care?
UPMC provides specialized care for a wide range of aortic conditions. Our center comprises experts in vascular surgery, cardiac surgery, and adult congenital heart disease. When you choose UPMC, you have access to:
- A multidisciplinary team of experts dedicated to providing a seamless continuum of care between specialties.
- Groundbreaking clinical trials and innovative treatment options for the full spectrum of aortic diseases.
- Support and resources through our partnerships with patient advocacy organizations like the Marfan Foundation.
