Abstract
Median arcuate ligament syndrome (MALS) is a rare but often underdiagnosed cause of chronic celiac occlusion. Flow-related inferior pancreaticoduodenal arcade (IPDA) aneurysms can often be challenging to manage. A retrospective review of a single-institution EMR from 1/1/2011 to 1/1/2016 was performed. The database included cases of pancreaticoduodenal arcade aneurysm, MALS, or idiopathic celiac stenosis. Eighty-seven cases met primary criteria. MALS was present in 21 cases. Of twenty-one (n = 21) patients with aortography-confirmed MALS, IPDA aneurysm was present in 6 (29%; mean age 58.7 years). Median aneurysm size was 2.1 cm (1.6–3.2 cm). All cases presented symptomatically (6/6): 4 with retroperitoneal hemorrhage and 2 with acute abdominal pain. Additional visceral aneurysms other than IPDA (splenic, right phrenic, and gastroepiploic) were identified in 3/6 cases in addition to IPDA aneurysm. Overall, 9/9 (100%) aneurysms were treated successfully (6 coil embolization, 2 coil/plug embolization, and 1 coil/stentgraft) and without procedure-related complications. All cases terminated with preserved collateral flow to the celiac axis. MALS with chronic abdominal pain was successfully managed in 7/21 [33%, mean age 58 (36–84) years] cases via surgical ligament release. Overall, 5/21 (24%) cases of MALS were incidentally identified post-hepatic transplant on routine surveillance imaging (sonography or MR angiography) [24%; mean age 57 (43–69) years] and confirmed with subsequent aortography. Overall, 0/5 cases demonstrated incidental visceral aneurysms. IPDA aneurysms seen in the setting of MALS can be treated using endovascular techniques with a high success rate. MALS is likely underdiagnosed and asymptomatic relative to true prevalence, as demonstrated by high relative incidence in orthotopic hepatic transplant surveillance imaging.
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