Median Arcuate Ligament Syndrome

PEDIATRIC SURGERY UPDATE: VOL 39 NO 03 SEPTEMBER 2012 has a concise review of Median Arcuate Ligament Syndrome (MALS) along with some references -listed with other references below (thanks to Ben Gold for this reference).  MALS occurs when a fibrous portion of the diaphragmatic crura crosses the celiac artery and compression occurs. This disorder is described as a diagnosis of exclusion.  Symptoms may include weight loss, nausea, abdominal pain, vomiting, and diarrhea.  Imaging modalities including CT angiography, MR angiography or arteriography can increase the suspicion for this disorder.

While not alluded to in this reference, one of the problems is knowing whether MALS is a spurious finding.  That is, in young individuals there is typically a robust blood supply to the intestine and it is not clear whether compression of the celiac trunk is responsible for specific symptoms as there is a compensatory blood supply.

Fortunately this disorder is very rare (or at the very least rarely recognized).  One of the most experienced vascular surgeons relayed his experience over more than three decades.  He stated that he had operated on four cases of suspected MALS –two improved with surgery.  A better batting average than Chipper Jones! –but a lot fewer at bats.

References:

  • Median arcuate ligament syndrome – Wikipedia, the free encyclopedia “It is estimated that in 10-24% of normal, asymptomatic individuals the median arcuate ligament crosses in front of (anterior to) the celiac artery, causing some degree of compression.”
  • – Median Arcuate Ligament Syndrome – YouTube
  • -Alehan D, Dogan OF: Pediatric surgical image. A rare case: celiac
    artery compression syndrome in an asymptomatic child.  J Pediatr Surg.
    39(4):645-7, 2004
  • – Gander S, Mulder DJ, Jones S, Ricketts JD, Soboleski DA, Justinich CJ:
    Recurrent abdominal pain and weight loss in an adolescent: celiac artery
    compression syndrome. Can J Gastroenterol. 24(2):91-3, 2010
  • – Said SM, Zarroug AE, Gloviczki P, Shields RC: Pediatric median arcuate
    ligament syndrome: first report of familial pattern and  transperitoneal
    laparoscopic release. J Pediatr Surg. 45(12):e17-20, 2010
  • – Aschenbach R, Basche S, Vogl TJ: Compression of the celiac trunk caused
    by median arcuate ligament in children and adolescent subjects: evaluation
    with contrast-enhanced MR angiography and comparison with Doppler US
    evaluation.  J Vasc Interv Radiol. 22(4):556-61, 2011
  • – Ozel A, Toksoy G, Ozdogan O, et al: Ultrasonographic diagnosis of
    median arcuate ligament syndrome: a report of two cases. Medical
    Ultrasonography 14(2): 154-157, 2012
  • – Wani S, Wakde V, Patel R, et al: Laparoscopic release of median arcuate
    ligament. J Minim Access Surg 8(1): 16-18, 2012
  • – “Median arcuate ligament syndrome”.Curr Treat Options Cardiovasc Med 2008 10 (2):
  • -“Median arcuate ligament syndrome: evaluation with CT angiography”. Radiographics 2005; 25 (5): 1177–82.

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