Median arcuate ligament syndrome: A case report of a rare disease

Median arcuate ligament syndrome (MALS) is a rare condition caused by the compression of the celiac trunk by the median arcuate ligament, which may trigger a typical symptom triad: postprandial abdominal pain, weight loss, nausea and vomiting.

A 58-year-old male was admitted to the emergency department due to abdominal pain, vomiting, fever and diarrhea. On admission, the patient was tachycardic, had abdominal distension, no peritoneal irritation signs, and a serum lactate level of 5 mmol/L. The computed tomography (CT) scan showed gastric and intestinal pneumatosis associated with pneumoportia. Conservative treatment with intravenous fluids, antibiotics and low molecular weight heparin, was initiated. The reassessment CT scan showed resolution of the gastric and intestinal pneumatosis and a severe stenosis of the initial portion of the celiac trunk with associated angulation. After recovery from the acute episode, an abdominal duplex ultrasound and an aortoiliac arteriography in forced inspiration and expiration was performed, corroborating the diagnosis of MALS. A laparoscopic decompression of the celiac trunk was performed.

Symptoms of MALS closely mimic other abdominal disorders, and it should be considered in the differential diagnosis. Currently, there are no international guidelines on MALS diagnostic criteria. Treatment is focused on decompression of the median arcuate ligament constriction of the celiac artery, with or without celiac lymph node removal.

MALS diagnostic and therapeutic approach must be patient focused. Laparoscopic decompression is an effective treatment and can provide immediate symptomatic relief, associated with the benefits of the less invasive nature of the procedure.