Foregut Anguish: A Rare Case of Median Arcuate Ligament Syndrome in Common Variable Immunodeficiency

Abstract

Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome (CACS), is a rare clinical entity that is characterized by extrinsic compression of the celiac artery by the median arcuate ligament of the diaphragm. It mostly presents as a triad of post-prandial abdominal pain, weight loss, and bruit in the epigastrium. Given its variable and atypical presentation, it is a diagnostic challenge for physicians. MALS is supposed to be a diagnosis of exclusion and, thus, many investigations need to be done before coining it as a definitive diagnosis. Herein, we present a case of a 17-year-old female patient, a known case of common variable immunodeficiency (CVID) who presented to our setup with progressive, excruciating epigastric pain and bilious vomiting after undergoing excision of CVID-associated gastric adenocarcinoma (CAGA). Multiple sets of tests were conducted to rule out possible cardiopulmonary, musculoskeletal, and abdominal etiology. The diagnosis was made on the basis of findings depicted on three-dimensional computed tomographic angiography (3D-CTA) and ultrasound Doppler studies of the celiac artery. She underwent laparoscopic dissection of the median arcuate ligament with a celiac plexus block, which yielded immediate relief in symptomatology and was later followed up with duplex scans and showed complete remission of symptoms.

Keywords: cacs; celiac artery stenosis; dunbar syndrome; mals; median arcuate ligament; mesenteric ischemia; postprandial pain; vascular stenosis.