Median arcuate ligament syndrome often poses a diagnostic challenge: A literature review with a scope of our own experience

Abstract

The median arcuate ligament syndrome (MALS) is recognized as a rare clinical entity, characterized by chronic post-prandial abdominal pain, nausea, vomiting, and unintentional weight loss. Due to its vague symptomatology, it is mainly regarded as a diagnosis of exclusion. Patients can often be misdiagnosed for several years before a correct diagnosis is established, also due to a medical team’s clinical suspicion. We present a case series of two patients who suffered from MALS and were treated successfully. The first patient is a 32-year-old woman, presenting with post-prandial abdominal pain and weight loss that have lasted for the past ten years. The second patient, a 50-year-old woman, presented with similar symptomatology, with the symptoms lasting for the last five years. Both cases were treated by laparoscopic division of the median arcuate ligament fibers, which alleviated extrinsic pressure from the celiac artery. Previous cases of MALS were retrieved from PubMed, to assemble a better diagnostic algorithm and propose a treatment method of choice. The literature review suggests an angiography with a respiratory variation protocol as the diagnostic modality of choice, along with the laparoscopic division of the median arcuate ligament fibers as the proposed treatment of choice.

Keywords: Median arcuate ligament syndrome; Dunbar syndrome; Celiac trunk compression syndrome; Celiac artery compression syndrome; Case series; Review

Core Tip: Due to its rarity, reviews, meta-analyses, and guidelines regarding median arcuate ligament syndrome (MALS) are rare. Most data can be extracted by individual case reports and case series. Even though MALS has a low frequency among the general population, more and more studies continue to support the claim that an increasing percentage of people may be prone to present characteristics of the syndrome. Thus, using this case series of patients as an example, we explore the literature with an aim to propose an improved diagnostic algorithm and treatment of choice.