Median arcuate ligament syndrome

Case contributed by Dr Werner Harmse

Presentation

Vague chronic upper abdominal pain for years. Often worse after eating.

Patient Data

Age: 50
Gender: Female
CT

CT upper abdomen arterial phase

Upper abdominal solid organs are unremarkable.

Midline sagittal images show focal smooth narrowing of the proximal coeliac artery. Features suggest external compression.

There is also prominent vessels seen in the pancreas head region suggesting collateral flow.

DSA (angiography)

Initial aortogram confirms smooth stenosis of the proximal coeiliac artery.

Selective superior mesenteric artery injection demonstrates a replaced right hepatic artery. There is however also filling of the left hepatic artery via the pancreatico-duodenal arcade. Retrograde flow is also observed into the common hepatic artery with filling of the splenic artery and coeliac on the late images.

Selective injection of the coeliac artery during inspiration again shows the tight smooth stenosis well. This injection is repeated during expiration where complete occlusion is seen with no flow into the distal portion of the coeliac artery.

Case Discussion

This patient had a chronic history of vague upper abdominal symptoms.

Initial CT had the unexpected finding of coeliac artery stenosis. This is a smooth stenosis with the appearance of external compression. CT findings were compatible with median arcuate ligament syndrome, where the median arcuate ligament compresses the proximal coeliac artery (also known as Dunbar syndrome). This characteristic appearance of the coeliac artery compression may however be seen in normal subjects, especially when imaged during expiration. CT however had some secondary signs supporting the diagnosis including post stenotic dilatation and suspected pancreatico-duodenal collaterals. 

The patient then underwent conventional angiography. Selective coeliac injections were done in both inspiration and expiration. During inspiration the appearance was similar to the CT with smooth, fairly tight stenosis and post stenotic dilatation. During expiration there was however no flow through the stenosis which was completely compressed. This together with the retrograde filling of the coeliac branches, when injecting the superior mesenteric artery confirmed the significance of the compression in the patient. Angiography findings further supported the diagnosis of Median Arcuate Ligament Syndrome.

Patient underwent surgery 2 days later to decompress the coeliac artery.

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Case information

rID: 43372
Case created: 7th Mar 2016
Last edited: 26th Mar 2017
Inclusion in quiz mode: Included

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