What is Celiac Artery Compression Syndrome?
Celiac artery compression syndrome, also known as Dunbar syndrome or median arcuate ligament syndrome, is a rare medical condition that often causes repeated abdominal pain. This syndrome happens when a fibrous band of the diaphragm, called the median arcuate ligament, squashes the celiac artery. Interestingly, Lipshutz first mentioned this kind of celiac artery squashing in 1917. The syndrome itself was first detailed as a distinct medical condition by Harolja in 1963. Then in 1965, Dunbar conducted the first clinical study on this syndrome.
What Causes Celiac Artery Compression Syndrome?
The true cause of celiac artery compression syndrome, a condition where the celiac artery is squeezed by the median arcuate ligament (a fibrous band connecting two parts of the diaphragm), isn’t fully known. This is mainly because some people with this type of compression don’t experience any symptoms. Not everyone with this anatomical pinch in the artery shows abnormal effects.
The places where the compression of the celiac artery typically happens are unusual: either the celiac artery starts higher than one would expect, or the diaphragm inserts lower than normal. Some people are perhaps born with these traits, which may explain why the compression occurs. This possibility is supported by a few studies conducted on families and identical twins.
It’s believed that the compression can cause temporary abdominal pain because it stops the flow of blood to the intestines (ischemia). However, this alone may not fully explain the condition because usually there is a strong network of backup blood vessels between the celiac artery and the superior mesenteric artery. It seems that a dysfunction in certain nerves (the celiac nerve plexus) may also play a role in causing this condition. If these nerves aren’t working properly, there may be an abnormal constriction in the digestive organs’ blood vessels, leading to ischemia.
Risk Factors and Frequency for Celiac Artery Compression Syndrome
Celiac artery compression syndrome is an uncommon problem seen mostly in young women between the ages of 30 to 50. Its occurrence is four times more common in females than in males. While it is mainly seen in adults, there have been cases in children as well. Although a number of people might show signs of celiac artery compression in medical images, only a small percentage actually have symptoms.
- The syndrome is not common, with only 2 cases per 100,000 people.
- The majority of patients are young females, between 30 to 50 years of age.
- Women are four times more likely to have this syndrome than men.
- Though it’s unusual, children can also get this syndrome.
- 10% to 24% of people might show signs of celiac artery compression in x-rays or other imaging, but only a small percentage will actually have symptoms of the syndrome.
Signs and Symptoms of Celiac Artery Compression Syndrome
When investigating a patient’s medical history, doctors will look into risk factors for hardening and narrowing of the arteries. They also check if the patient has been using non-steroidal anti-inflammatory drugs, which might explain other potential causes of their symptoms. It’s not uncommon for these patients to have tried medications like proton pump inhibitors, only to find they provide no relief.
Patients with celiac artery compression syndrome, a condition where the artery supplying blood to the stomach and other digestive organs is compressed, may report upper stomach pain, loss of appetite, and diarrhea. The pain usually starts after eating. Accompanying symptoms might include nausea and vomiting. To keep track of the disease progress, doctors will regularly check the patient’s weight, as a decrease might occur over time.
During a physical examination, doctors may find that the patient has a slight soreness when the upper abdomen area is pressed. They might also hear a bruit (`a whooshing sound`) when listening to the patient’s abdomen, suggesting abnormal blood flow in the artery.
Testing for Celiac Artery Compression Syndrome
Celiac artery compression syndrome is a condition that’s often determined by ruling out other possible causes. In diagnosing this condition, doctors may do a variety of simple tests to eliminate other possible concerns. This could include a colonoscopy, ultrasound checks of the liver, pancreas, and gallbladder, an upper gastrointestinal endoscopy, blood tests, liver function tests, and certain other specific tests like C-reactive protein and anti-smooth muscle antibody.
There are several ways doctors can diagnose celiac artery compression syndrome, which range from non-invasive methods to more invasive procedures.
More invasive procedures could include a particular type of x-ray test known as conventional visceral angiography. Non-invasive methods could involve Doppler ultrasound, magnetic resonance imaging (MRI), and computerized tomography angiography (CTA).
When using an ultrasound, doctors will look at the peak blood flow speed in the celiac artery during deep exhalation. They’ll typically look for a speed of more than 200 cm/s and a bend in the artery of more than 50 degrees. There might also be other findings like an unusually positioned celiac artery, reversed blood flow in the liver’s artery, and a drop in the celiac artery’s speed when the patient stands up.
During an angiography, doctors might find a narrowing to complete blockage of the celiac artery because of external pressure. Sometimes, this can lead to the artery enlarging after the blockage and blood filling into the artery in the opposite direction. During this process, another ultrasound can show a narrowing of the artery’s opening during exhalation.
A CTA, on the other hand, can indicate a compression in the celiac axis with a localized narrowing and enlargement after the obstruction. These tests can highlight the difference in blood supply to the celiac artery during inhaling and exhaling. Certain other tests can also be done to help diagnose celiac artery compression syndrome like gastric tonometry and percutaneous celiac ganglion block.
Treatment Options for Celiac Artery Compression Syndrome
Celiac artery compression syndrome is a condition where the celiac artery, one of the major arteries in the abdomen, is squeezed or compressed. Unfortunately, there’s no known medical treatment for this condition, so the focus is on relieving the compression. Treatment involves surgery that targets the median arcuate ligament – the structure causing the compression.
There are two main ways this surgery can be carried out: traditional open surgery or minimally invasive techniques. Open surgery can be performed via a retroperitoneal approach (from the back) or a transperitoneal approach (from the front). On the other hand, the minimally invasive approach involves either laparoscopy or the use of a robot. The advantage of minimally invasive techniques is that they lead to a shorter stay in the hospital, better control of pain, smaller scars, and lower risk of wound complications after the operation.
In this operation, not only is the ligament divided to relieve compression, but also the celiac nerve plexus, a network of nerves around the celiac artery, is often treated, which can contribute to better symptom relief.
For some patients, additional procedures might be necessary. These can include a bypass around the celiac artery, repositioning other major arteries in the abdomen, a type of treatment where a balloon is used to open up a narrowed or blocked artery (angioplasty), and blockade of a group of nerves (ganglion nerve blocks). If the first surgery does not provide symptom relief, other procedures such as angioplasty might be performed, with or without a stent (a small tube inserted into an artery).
What else can Celiac Artery Compression Syndrome be?
Celiac artery compression syndrome is a medical condition that can be tough to recognize because it can seem like various other health problems. Doctors could confuse it with conditions that cause discomfort after eating, like issues with the gallbladder, including a slowdown of normal gallbladder movements and inflammation of the gallbladder.
Other issues that involve the stomach and digestive tract could also appear to be celiac artery compression syndrome. These include appendicitis, colorectal cancer, liver inflammation (hepatitis), delayed emptying of the stomach (gastroparesis), and stomach ulcers or inflammation.
There’s also a condition called chronic mesenteric ischemia, which occurs due to hardening of the arteries, and its symptoms could seem like those of celiac artery compression syndrome.
Another consideration is that some of these conditions could happen at the same time.
If the doctor hears a whooshing sound in the abdomen (known as an abdominal bruit), it might suggest other conditions such as narrowing of the kidney artery or abnormal connections between arteries and veins. Thus, the process of diagnosing can be quite complicated and requires thorough examination.
What to expect with Celiac Artery Compression Syndrome
After undergoing surgery, around 60% to 70% of patients report experiencing relief from their symptoms. In certain cases, this relief is immediate, while in others, it may take several months for the pain to fully subside. Patients who previously reported post-exercise pain have been found to experience more relief after surgery.
However, those who experienced vomiting and unprovoked pain before the operation tend to report less successful outcomes after surgery. Patients who responded positively to a diagnostic procedure known as a celiac plexus block before the operation typically report better symptom relief after the surgery. Additionally, those with risk factors for hardening of the arteries may not experience as positive outcomes following the surgery.
