Exploring the Association Between Median Arcuate Ligament Syndrome and Hypermobile Ehlers-Danlos Syndrome: A Case...

Median arcuate ligament syndrome (MALS) is a rare vascular compression disorder resulting from external compression of the celiac artery and surrounding neural plexus by the median arcuate ligament of the diaphragm. It often presents with non-specific symptoms such as postprandial abdominal pain, weight loss, and gastrointestinal distress, making diagnosis challenging. Recent literature suggests a potential association between MALS and connective tissue disorders, particularly hypermobile Ehlers-Danlos syndrome (hEDS), due to increased ligamentous laxity and vascular vulnerability. We present the case of a young adult female with a confirmed diagnosis of hEDS who developed MALS, characterized by chronic epigastric pain worsened by meals, significant weight loss, and an extensive negative gastrointestinal workup. Initial imaging was inconclusive, but subsequent evaluation by a vascular surgeon revealed a low-riding median arcuate ligament with the characteristic "hook-sign" deformity of the celiac artery. A diagnostic celiac plexus block provided temporary relief, supporting the diagnosis, and was followed by robotic-assisted ligament release with bilateral celiac ganglionectomy. Postoperatively, the patient reported substantial improvement in symptoms, though partial recurrence occurred months later, raising concern for post-surgical fibrosis or recurrent vascular compression. This case highlights the diagnostic complexity of MALS, particularly when it overlaps with connective tissue disorders such as hEDS. It emphasizes the utility of celiac plexus blocks as both a diagnostic and prognostic tool and underscores the need for high clinical suspicion in patients with hEDS presenting with chronic, unexplained abdominal pain. Furthermore, it reflects the psychological burden of delayed diagnosis and the impact of receiving definitive treatment. This case is unique in describing the co-occurrence of MALS and hEDS with the associated treatment difficulties, illustrating the complex interplay of vascular compression and connective tissue laxity. It contributes to a limited body of literature exploring how overlapping syndromes complicate diagnosis, surgical decision-making, and long-term outcomes. This case adds to the growing body of evidence linking connective tissue disorders to vascular compression syndromes and supports the need for further research to better define this relationship and improve long-term management strategies.