What are the other Names for this Condition? (Also known as/Synonyms)

• Celiac Access Syndrome
• Dunbar Syndrome
• Median Arcuate Ligament Syndrome (MALS)

What is Celiac Artery Compression Syndrome? (Definition/Background Information)

• Celiac Artery Compression Syndrome is a rare disorder characterized by chronic, recurrent abdominal pain related to compression of the celiac artery (which supplies blood to the upper abdominal organs) by the median arcuate ligament (a muscular fibrous band of the diaphragm)
• It usually presents with symptoms of abdominal pain, weight loss, and an abdominal bruit (abnormal sound of a blood vessel when blocked or narrowed)
• The consequence of chronic celiac trunk compression is unknown. While no known correlation currently exists, future studies may find other gastrointestinal disorders in patients diagnosed with this syndrome
• The cause is not fully understood; however, it is suspected that there could be a combination of vascular (blood supply) and neurogenic (neurological) components involved
• Diagnosis is usually confirmed with imaging such as CT angiography, MRI, ultrasound, and arteriography
• Surgery is currently the only treatment option for Celiac Artery Compression Syndrome and involves releasing the ligament

(Source: Celiac Artery Compression Syndrome; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Who gets Celiac Artery Compression Syndrome? (Age and Sex Distribution)

• Celiac Artery Compression Syndrome is a rare disorder. The presentation of symptoms may occur between the ages of 20-40 years
• Although both genders are susceptible to the disorder, it is more common in middle aged females
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Celiac Artery Compression Syndrome? (Predisposing Factors)

The risk factors for Celiac Artery Compression Syndrome may include:

• Female gender
• Low body weight (being thin)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Celiac Artery Compression Syndrome? (Etiology)

• The cause of Celiac Artery Compression Syndrome is disputed
• While it was initially thought to be caused by a restriction of blood supply secondary to compression of the celiac artery(supplies blood to the upper abdominal organs) by the median arcuate ligament (a muscular fibrous band of the diaphragm), other factors have been proposed
• It has been suggested that nerve dysfunction might additionally be involved, which could explain some of the associated symptoms such as pain and delayed gastric emptying

(Source: Celiac Artery Compression Syndrome; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

What are the Signs and Symptoms of Celiac Artery Compression Syndrome?

The signs and symptoms of Celiac Artery Compression Syndrome may vary among affected individuals in type and severity, and may include:

• Classical of symptoms, namely
  • Abdominal pain after eating
  • Weight loss (usually >20 pounds)
  • Abdominal bruit (abnormal sound of a blood vessel when blocked or narrowed)
• Other symptoms, such as the following, may be noted:
  • Nausea
  • Diarrhea
  • Vomiting 
  • Delayed gastric emptying

One review found that abdominal pain is the most common symptom, found to be present in approximately 80% of individuals, while weight loss was found in approximately 48% and abdominal bruit was appreciated in approximately 35%.

(Source: Celiac Artery Compression Syndrome; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How is Celiac Artery Compression Syndrome Diagnosed?

• A diagnosis of Celiac Artery Compression Syndrome might be suspected in middle aged (40-60 years) female patients with a triad of symptoms including abdominal pain after eating, weight loss, and abdominal bruit (abnormal sound of a blood vessel when blocked or narrowed)
• Abdominal imaging is used to confirm the diagnosis and rule out other similarly presenting disorders. Imaging methodologies might include: CT angiography, MRI, ultrasound, and arteriography

(Source: Celiac Artery Compression Syndrome; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Celiac Artery Compression Syndrome?

The complications of Celiac Artery Compression Syndrome may include:

• Chronic compression of the celiac artery, leading to sustained weight loss
• Aneurysm of the superior and inferior pancreaticoduodenal arteries
• Gastrointestinal bleeding, if rupture of aneurysm occurs

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Celiac Artery Compression Syndrome Treated?

Surgery is currently the only treatment option for Celiac Artery Compression Syndrome.

• Surgery typically involves decompression of the celiac artery by dividing the fibers of the median arcuate ligament and celiac plexus (network of nerves in the abdomen)
• Surgical decompression might additionally be combined with stent placement, angioplasty, or vascular reconstruction of the celiac artery

(Source: Celiac Artery Compression Syndrome; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How can Celiac Artery Compression Syndrome be Prevented?

• The cause of Celiac Artery Compression Syndrome is not clearly known, and hence, there are no guidelines or methods available for its prevention at the present time
• Seeking prompt medical attention for abdominal pain and unexplained weight loss may help avoid complications from the condition
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Celiac Artery Compression Syndrome
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Celiac Artery Compression Syndrome? (Outcomes/Resolutions)

• The prognosis for Celiac Artery Compression Syndrome varies. While long-term relief of symptoms has been described, many individuals unfortunately do not experience clinical benefits from surgery
• In a large study of 51 individuals treated for celiac artery compression, the following factors were found to be associated with a favorable outcome after surgery:
  • Pain after eating (81% cured)
  • Age between 40 and 60 (77% cured)
  • Weight loss of 20 pounds or more (67% cured)
• Less success was associated with the following factors:
  • Atypical pain patterns with periods of remission (43% cured)
  • Age greater than 60 (40% cured)
  • A history of psychiatric disorder or alcohol abuse (40% cured)
  • Weight loss less than 20 pounds (53% cured)

(Source: Celiac Artery Compression Syndrome; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

Additional and Relevant Useful Information for Celiac Artery Compression Syndrome:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/