It is well known that mechanical irritation of the celiac plexus surrounding the celiac trunk by the median arcuate ligament, the lower border of the aortic hiatus, causes epigastric pain and numerous vegetative dysfunctions.

Despite the often severe complaints, this situation is not life-threatening because the compression of the celiac trunk, which is the hallmark of the disorder, is usually compensated by collaterals from the superior mesenteric artery via the pancreas.

Only rarely is the compression of the celiac trunk so severe that the superior mesenteric artery, which originates about 1 cm caudal to the celiac trunk from the midline of the aorta, is indirectly affected by the downward movement of the celiac trunk itself.

I report here a newly recognized vascular compression syndrome consisting of synchronous direct compression of both arteries by the median arcuate ligament.

Under normal anatomic circumstances, such a constellation is usually impossible because both the celiac trunk and the superior mesenteric artery branch off typically in the midline of the aorta. Thus, compression of the celiac trunk from above by the median arcuate ligament does not directly affect the more caudally branching SMA.

However, in this particular case, due to the deviation of the origin of the celiac trunk to the left of the aorta, the median arcuate ligament was able to compress both vessels simultaneously by rotating the entire aortic hiatus to the left, allowing the right crus (leg) of the diaphragm to run obliquely over the superior mesenteric artery.

This allowed the median arcuate ligament to compress the celiac trunk while the lateral portion of the median arcuate ligament compressed the superior mesenteric artery.

The pressure on the celiac trunk was higher, as evidenced by the exorbitant flow acceleration, which required special cardiologic techniques (continuous wave Doppler measurements) to precisely define the exact flow acceleration, which is a measure of the degree of compression.

By comparing the flow acceleration in both arteries, it is clear that the subtotal occlusion of the celiac trunk was accompanied by a high degree of compression of the SMA which, however, did not reach the severity of the celiac trunk compression.

However, the resulting clinical symptoms are striking.

The patient has intermittent, devastating upper abdominal pain that radiates throughout the abdomen and has necessitated several emergency consultations in which it has not been possible to define the exact source of the pain.

In contrast to a normal median arcuate ligament syndrome, the present situat ion could be dangerous because the simultaneous and prolonged compression of both arteries causes intestinal ischaemia which is not compensated by collaterals. Only in the case of progressive obstruction of both the celiac trunk and the superior mesenteric artery (e.g. in the case of arteriosclerosis) do collaterals develop from the inferior mesenteric artery along the mesenteric arch (the so-called Riolan’s anastomosis). However, the intermittent nature of the vascular compression does not induce sufficient collateral growth. Thus, sudden occlusion of both vessels can have life-threatening consequences due to intestinal ischaemia and subsequent peritonitis.