Pediatric median arcuate ligament syndrome: first report of familial pattern and transperitoneal laparoscopic release

Abstract

Median arcuate ligament syndrome (MALS) is a rare disorder resulting from luminal narrowing of the celiac artery by the insertion of the diaphragmatic muscle fibers. Surgical management entails division of the median arcuate ligament with or without celiac artery reconstruction. We are presenting an interesting case of a 16-year-old girl with postprandial abdominal pain and weight loss. Her mother also had MALS treated via open celiotomy with complete median arcuate ligament division and patch angioplasty of the celiac artery owing to persistent stenosis at our institution. After a diagnosis of MALS was confirmed in our patient, a transperitoneal laparoscopic release of the median arcuate ligament with skeletonization of the celiac artery and branch vessels was performed. The postoperative course was uneventful, and she was dismissed on postoperative day 2. She remains asymptomatic at 12-months follow-up. This represents the first report of a transperitoneal laparoscopic approach to MALS in an adolescent and the first report of a familial/generational component to MALS.