Abstract
Median arcuate ligament syndrome (MALS) is a rare and often misdiagnosed vascular pathology. In this paper, we discuss a 51-year-old female with MALS presenting with hypotension due to retroperitoneal hemorrhage. Currently, there is no consensus regarding the optimal treatment approach for such patients. This case report demonstrates the utility of conventional mesenteric angiography, cone beam CT with 3D reconstruction, and selective mesenteric transarterial embolization as an effective treatment approach for patients with spontaneous aneurysm rupture in MALS.
Highlights
Median arcuate ligament syndrome (MALS), known as celiac artery compression syndrome, celiac axis syndrome, or Dunbar syndrome, is a rare and often misdiagnosed vascular pathology
The syndrome is more frequent in females in their 4th to 6th decades of life with chronic symptoms lasting from a couple of months to many years
The median arcuate ligament (MAL) is a ligament formed by tendinous fibers between the right and left diaphragmatic crura, which form the ventral arch of the aortic hiatus of which four variants have been reported
Summary
Median arcuate ligament syndrome (MALS), known as celiac artery compression syndrome, celiac axis syndrome, or Dunbar syndrome, is a rare and often misdiagnosed vascular pathology. Aneurysms of the pancreaticoduodenal arteries can occur, and the initial presentation may be sudden abdominal pain, anemia, and hypotension secondary to spontaneous retroperitoneal hemorrhage. Treatment guidelines in this situation are unclear, but given the complexity of the pathology, a multidisciplinary approach is often best. Transarterial embolization can be difficult given the complex collateral anatomy but has proved to be an effective initial treatment in the acute setting.
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