Median arcuate ligament syndrome
Median arcuate ligament syndrome (MALS) can cause a range of symptoms, including abdominal pain, nausea, vomiting, and weight loss. Because all patients have some degree of celiac artery compression by the median arcuate ligament (MAL), it may be difficult to discern which patients have a pathologic compression. Based on the multiple theories of MALS etiology, it is unlikely that we know the true cause of this syndrome. In fact, there are many physicians who question the validity of the diagnosis of MALS. Before offering intervention for MALS, a thorough gastrointestinal evaluation should be performed, including consideration of diagnostic temporary percutaneous celiac ganglion block. Patients who are on chronic narcotics preoperatively have a lower likelihood of postoperative symptom relief and therefore should be evaluated by a pain specialist preoperatively. The most reliable treatment comprises open surgical treatment with division of the MAL, removal of surrounding celiac ganglion, evaluation of the celiac artery with pressure measurements or ultrasound, and celiac artery reconstruction if indicated. Laparoscopic and endovascular interventions are novel treatments and may be considered in select patients who cannot undergo an open surgical procedure.
Available from: Colleen Stiles-Shields
- "Although MALS has been advocated as an unusual cause of abdominal pain, the evidence has been based principally on anecdotal or small single-center retrospective analysis rather than level 1 or level 2 evidence   . In anatomical terms, MALS is felt to be caused by a compressive anatomic relationship of the diaphragmatic crura to the celiac vessels leading to decreased flow, a steal phenomenon and resultant postprandial abdominal pain    . Similarly it has been suggested that neurogenic compression may lead to the clinical symptoms . "
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ABSTRACT: Median arcuate ligament syndrome (MALS) is a vascular compression syndrome with symptoms that overlap chronic functional abdominal pain (CFAP). We report our experience treating MALS in a pediatric cohort previously diagnosed with CFAP.
We prospectively evaluated 46 pediatric (<21years of age) patients diagnosed with MALS at a tertiary care referral center from 2008 to 2012. All patients had previously been diagnosed with CFAP. Patients were evaluated for celiac artery compression by duplex ultrasound and diagnosis was confirmed by computed tomography. Quality of life (QOL) was determined by pre- and postsurgical administration of PedsQL™ questionnaire. The patients underwent laparoscopic release of the median arcuate ligament overlying the celiac artery which included surgical neurolysis. We examined the hemodynamic changes in parameters of the celiac artery and perioperative QOL outcomes to determine correlation.
All patients had studies suggestive of MALS on duplex and computed tomography; 91% (n=42) positive for MALS were females. All patients underwent a technically satisfactory laparoscopic surgical release resulting in a significant improvement in blood flow through the celiac artery. There were no deaths and a total of 9 complications, 8 requiring a secondary procedure; 33 patients were administered QOL surveys. 18 patients completed the survey with 15 (83%) patients reporting overall improvement in the QOL. Overall, 31/46 patients (67%) reported improvement of symptoms since the time of surgery.
MALS was found to be more common in pediatric females than males. Laparoscopic release of the celiac artery can be performed safely in the pediatric population. Surgical release of the artery and resultant neurolysis resulted in significant improvement in the blood flow, symptoms, and overall QOL in this cohort. The overall improvement in QOL outcome measures after surgery leads us to conclude that MALS might be earlier diagnosed and possibly treated in patients with CFAP. We recommend a multidisciplinary team approach to care for these complex patients.
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