The benefits of extrinsic ligament release for potentially hemodynamically unstable pancreaticoduodenal arcade aneurysm with median arcuate ligament syndrome: a case report

Abstract

Background: A pancreaticoduodenal artery aneurysm (PDAA) occurring in close association with median arcuate ligament syndrome (MALS) is rare. A surgical procedure, such as median arcuate ligament (MAL) release, should be considered in such cases, but the operative criteria remain unknown. In this study, we reported an extremely rare case of PDAA with periarteritis nodosa (PAN) and MALS.

Case presentation: A 60-year-old man was transferred to our department with sudden onset of abdominal pain. We initially diagnosed his condition as a PDAA rupture with MALS based on enhanced computed tomography (CT). We promptly performed transcatheter arterial embolization (TAE) of PDAA, and the angiogram showed stagnant contrast agent in the celiac trunk, indicating total celiac artery occlusion. Follow-up enhanced CT three weeks after the first TAE clearly demonstrated newly formed, multiple aneurysms in the pancreaticoduodenal arcade and the hepatic artery. These findings indicated a systemic disorder, such as PAN or segmental arterial mediolysis, as the underlying cause. Therefore, we started corticosteroid therapy and performed diagnostic angiography to clarify the celiac artery's patency. Contrary to the initial angiography, the second angiography showed sustained blood flow in the celiac artery. Nevertheless, we performed both extrinsic MAL release and consecutive TAE because of the risk of multiple aneurysms rupturing due to an uncontrolled systemic disorder and consequent hepatic ischemia. The patient had no episode of recurrence until one year of follow-up.

Conclusions: It is important to evaluate risk for hemodynamically unstable events to decide the best treatment strategy for MALS.

Keywords: Case report; Extrinsic median arcuate ligament release; Median arcuate ligament syndrome; Pancreaticoduodenal arcade; Pancreaticoduodenal artery aneurysm.

Fig. 1

Transcatheter arterial embolization for a ruptured pancreaticoduodenal artery aneurysm with median arcuate ligament syndrome. a The stagnant contrast agent in the celiac trunk indicates total occlusion of the CA. b Digital subtraction angiography of the SMA shows retrograde blood flow from the PIPDA to the CA. c Embolization of the PIPDA aneurysm is performed using both coil and N-butyl-2-cyanoacrylate. CA, celiac artery; PIPDA, posterior inferior pancreaticoduodenal artery; SMA, superior mesenteric artery

Fig. 2

Enhanced computed tomography and diagnostic angiography performed prior to the second transcatheter arterial embolization. a Multiplanar reformatted image of the follow-up enhanced computed tomography on day 21 shows transverse pancreatic artery aneurysm (arrow) and hepatic artery aneurysms (arrowheads). b Diagnostic angiography on day 24 demonstrates sustained blood flow in the CA hepatic artery aneurysms (arrow). c Sagittal view of the angiography demonstrates severe stenosis of the CA (arrow). CA, celiac artery

Fig. 3

Angiography performed on day 35. a There is total occlusion of the transverse pancreatic artery aneurysm (arrow). b The hepatic artery aneurysms resolved drastically after median arcuate ligament release (arrowheads)