Laparoscopic Management of Median Arcuate Ligament Syndrome in a Young Female with Right Renal Agenesis and Left-sided Nutcracker Phenomenon: A Rare Triple Vascular-Anatomical Association
Meet Suthar *
Department of Surgery, SMS Multispecialty Hospital, Ahmedabad, India.
Gunvant Rathod
Department of Surgery, SMS Multispecialty Hospital, Ahmedabad, India.
*Author to whom correspondence should be addressed.
Abstract
Background: Median Arcuate Ligament Syndrome (MALS), also referred to as celiac artery compression syndrome or Dunbar syndrome, is a rare vascular condition characterised by external compression of the proximal celiac artery by the median arcuate ligament of the diaphragm. While radiological evidence of celiac artery compression is observed in approximately 10–24% of individuals, only a small subset develop clinically significant symptoms. Patients with MALS commonly present with postprandial epigastric pain, nausea, vomiting, and unintentional weight loss, with a higher prevalence among young, slender females. The diagnosis of MALS is established by correlating characteristic clinical features with vascular imaging findings. Computed tomography (CT) angiography is particularly valuable, typically revealing focal narrowing of the proximal celiac artery with a distinctive hooked configuration and post-stenotic dilatation. The occurrence of MALS alongside other vascular compression syndromes or congenital vascular anomalies is exceedingly uncommon.
Case Presentation: We report the case of a 19-year-old female presenting with progressive postprandial abdominal pain, recurrent vomiting, and significant weight loss over a five-month period. Clinical examination was largely unremarkable except for a low body mass index (17.1 kg/m²). Contrast-enhanced CT angiography revealed focal narrowing of the celiac trunk with a characteristic hooked configuration consistent with MALS. Imaging also demonstrated an anatomical variant in which the common hepatic artery originated from the superior mesenteric artery, along with incidental findings of right renal agenesis and compression of the left renal vein between the aorta and superior mesenteric artery consistent with nutcracker phenomenon. The patient underwent successful laparoscopic median arcuate ligament release with circumferential decompression of the celiac artery and celiac plexus neurolysis.
Conclusion: This report highlights an extremely rare coexistence of MALS with unilateral renal agenesis and nutcracker phenomenon. Awareness of such vascular and congenital associations is important for accurate diagnosis and surgical planning. Laparoscopic median arcuate ligament release remains a safe and effective treatment for symptomatic MALS with favorable postoperative outcomes.
Keywords: Median arcuate ligament syndrome, celiac artery compression, nutcracker phenomenon, renal agenesis, laparoscopic decompression