Median arcuate ligament syndrome is a rare anatomic disorder leading to symptoms of foregut ischemia that can be complicated by aneurysm formation and rupture. This syndrome is difficult to diagnose because there is no direct study to evaluate it, and it is often discovered incidentally on imaging or following complications. Aneurysm rupture presents a life-threatening complication due to the volume of potential hemorrhage secondary to increased back pressure. A 46-year-old male presented with an acute atraumatic onset of severe abdominal pain and was found to have a massive intra-abdominal hemorrhage due to gastroduodenal artery aneurysm rupture. Median arcuate ligament syndrome was diagnosed during operating room re-exploration. Following ligament resection and vessel ligation, the patient was stabilized and had an uneventful post-operative course. Median arcuate ligament syndrome is a rare condition that is a diagnosis of exclusion during the workup of chronic mesenteric ischemia. Complications of this syndrome are even more rare, with a gastroduodenal artery (GDA) aneurysm presenting as an even more rare but potentially more dangerous complication given the increased size of this proximal vessel. While rare, this case presents a very important reminder when assessing patients with long-standing vague abdominal symptoms, concern for chronic mesenteric ischemia, and the potential critical course resulting from this disease process, necessitating prompt intervention.
Shellman et al. (Sun,) studied this question.