Median arcuate ligament syndrome

Case contributed by Piyush P Siwach
Diagnosis almost certain

Presentation

Longstanding unexplained epigastric discomfort and pain.

Patient Data

Age: 60 years
Gender: Male

Contrast enhanced arterial phase CT scan of upper abdomen demonstrate

  1. Severe narrowing of proximal segment of celiac axis.
  2. Prominent inferior pancreaticuduodenal artery, the first branch of SMA.
  3. Opacification of celiac axis branches, suggesting collateral flow between inferior pancreaticoduodenal artery, a branch of superior mesenteric artery, and superior pancreaticoduodenal artery, a branch of common hepatic artery. 
  4. A saccular aneurysm with calcified walls in proximal segment of inferior pancreaticuduodenal artery.

Case Discussion

Anatomically, the appearance in this study is consistent with celiac artery compression by the medial arcuate ligament due to its high origin. However, clinical symptoms may not manifest proportionate to the severity of anatomical abnormality, as in this case, where the patient had relatively mild symptoms, mainly post-prandial, despite the complete obstruction of the celiac axis, because of well-developed collaterals between a superior mesenteric artery and the celiac axis around pancreas and second part of duodenum. This results in abnormally high volume flow through the inferior pancreaticoduodenal branch of the superior mesenteric artery, and in this case has resulted in an aneurysm of the artery, which is otherwise an uncommon location of such aneurysms.

A confirmed diagnosis of median arcuate ligament syndrome requires the anatomical abnormality detected on CT and confirmed by selective DSA if required, and relief of symptoms after decompression of the artery, which is obtained by open surgical division of the median arcuate ligament. The symptoms in this patient did not warrant a surgical treatment, but the patient was referred to a vascular surgeon regarding his aneurysm.

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