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PATIENT STORY

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A 22-year-old Caucasian woman presented to the vascular clinic with intense postprandial abdominal pain that occurred 20 to 30 minutes after eating. She also relayed a history of food fear along with occasional nausea and vomiting. She had experienced about a 25-lb weight loss over the last 6 months. Her past medical history was not significant for any chronic illnesses or conditions including absence of a psychiatric or drug abuse history. She stated that she had undergone an extensive gastrointestinal workup including upper and lower endoscopy and a right upper quadrant ultrasound, but no diagnosis had been established. On examination the patient appeared thin, in no acute distress. Her abdomen was soft, nontender, nondistended. On auscultation, an epigastric bruit that increased with expiration was found. A computed tomographic angiogram (CTA) was obtained that demonstrated extrinsic compression of the celiac axis by the median arcuate ligament of the diaphragm and poststenotic dilatation of the celiac artery (Figure 46-1).

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FIGURE 46-1

Computed tomographic angiogram (CTA) sagittal plane view of a patient with median arcuate ligament syndrome. Red arrow indicates median arcuate ligament causing compression of the celiac artery with poststenotic dilatation.

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This patient has the typical presentation of celiac axis compression syndrome (CACS), also known as median arcuate ligament syndrome.

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EPIDEMIOLOGY

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  • A significant number of individuals have narrowing of the celiac axis by the median arcuate ligament of the diaphragm, and full expiration seems to exacerbate this compression. It is still uncertain whether chronic intestinal ischemia can develop from compression of the celiac axis alone.

  • Usually occurs in young, thin females.1,2, and 3

  • Severe compression that persists during inspiration occurs in approximately 1% of patients.3

  • Between 13% and 50% of patients may have some degree of compression and experience no symptoms.3

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ETIOLOGY AND PATHOPHYSIOLOGY

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  • First described by Harjola in 1963.4

  • Also known as median arcuate ligament syndrome or celiac band syndrome.2

  • Median arcuate ligament is a tendinous group of fibers that form an arch between the diaphragmatic crura.3

  • Usually the ligament passes above the origin of the celiac axis, but in some individuals (10%-24%) it may cross the axis anteriorly leading to compression.3

  • Compression of the axis may cause obstruction of blood flow resulting in symptoms.3

  • The superior mesenteric artery (SMA), which is the main source of blood flow to the bowel, forms a rich collateral network with the celiac artery (CA).1

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DIAGNOSIS

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  • Usually a diagnosis of exclusion, both clinical and radiographic features should be present.1,3

  • Classic triad2

    1. Postprandial abdominal pain

    2. Epigastric bruit that increases with expiration (83%)

    3. Evidence of extrinsic compression of the celiac access by vascular imaging

  • Most frequently seen in young, thin females....

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