What is MALS (Median Arcuate Ligament Syndrome)?

From the Guidelines

Surgical release of the median arcuate ligament is the most effective treatment for MALS syndrome, with symptomatic relief achieved in 84.6% of patients, and subsequent reconstruction of the celiac artery may be necessary for complete relief. MALS syndrome, or Median Arcuate Ligament Syndrome, is a rare condition where the median arcuate ligament compresses the celiac artery, causing chronic abdominal pain. Diagnosis typically requires imaging studies like CT angiography or ultrasound to visualize the compression, as noted in the 2022 update of the ACR Appropriateness Criteria for radiologic management of mesenteric ischemia 1. Treatment options include laparoscopic release of the ligament for severe cases, which aims to decompress the celiac artery.

According to the study published in the Journal of the American College of Radiology, surgical release of the MAL has been associated with symptomatic relief in 84.6% of patients in a small study of 39 patients 1. Furthermore, subsequent reconstruction of the celiac artery, with either endovascular stent placement or surgical bypass creation, may be necessary to provide complete symptomatic relief, restore normal hemodynamics, and prevent the development of splanchnic artery aneurysms in the setting of recurrent symptoms or persistent celiac stenosis.

Key points to consider in the management of MALS syndrome include:

• Symptomatic relief can be achieved in 84.6% of patients with surgical release of the MAL 1
• Subsequent reconstruction of the celiac artery may be necessary for complete relief
• The decision to reconstruct the celiac artery remains debatable, with some studies showing no significant difference in symptom relief or reintervention rates between patients who underwent vascular reconstruction and those who did not 1
• A study evaluating long-term outcomes in 44 patients who received operative management for MAL syndrome reported persistent resolution of clinical symptoms in 76% of patients who underwent some form of revascularization, compared with 53% of patients who received decompression alone 1

From the Research

Definition and Symptoms of MALS

• Median arcuate ligament syndrome (MALS) is an uncommon condition caused by the extrinsic compression of the celiac trunk and celiac ganglion, secondary to an anatomical abnormality of the median arcuate ligament fibers 2.
• It is characterized by postprandial epigastric pain, chronic abdominal pain, weight loss, nausea, and vomiting 2, 3, 4.
• The symptoms of MALS can be mistaken for other gastrointestinal or vascular conditions, making diagnosis challenging 5.

Diagnosis of MALS

• A variety of imaging and diagnostic modalities can suggest findings consistent with MALS, including Duplex ultrasonography, computed tomography angiography, magnetic resonance angiography, gastric tonometry, and angiography 2, 3.
• There is no group consensus agreement on the diagnostic criteria for MALS, and different combinations of diagnostic tests are used with varying diagnostic values throughout the literature 3.
• A thorough gastrointestinal evaluation, including consideration of diagnostic temporary percutaneous celiac ganglion block, should be performed before offering intervention for MALS 4.

Treatment of MALS

• Laparoscopic approach has been proposed as the option of choice for the treatment of MALS, with advantages including short operative time, shorter hospital stay, and low rate of conversion to open surgery 2.
• Open surgical treatment with division of the median arcuate ligament, removal of surrounding celiac ganglion, evaluation of the celiac artery with pressure measurements or ultrasound, and celiac artery reconstruction if indicated, is also a reliable treatment option 4.
• Robotic, laparoscopic, endoscopic retroperitoneal, and open surgical intervention can provide effective symptom relief, but long-term follow-up data (>5 years) are lacking 3.
• Operative management of MALS can be performed with a low rate of complications, and approximately two-thirds of respondents were free of symptoms 5 years after the procedure 6.

Outcomes and Quality of Life

• Treatment success in symptom-free patients is associated with an improved health-related quality of life (HRQOL) on par with the population average compared with symptomatic patients 6.
• The vast majority of respondents would opt to have the operation again if given a choice, despite the possibility of failure to relieve symptoms and symptom recurrence 6.
• Early detection and surgical treatment can increase favorable outcomes for patients with MALS, and patients should be well-informed about the possibility of failure to relieve symptoms and symptom recurrence 6, 5.