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Diseases

Genetic and Rare Diseases Information Center (GARD)

Superior mesenteric artery syndrome


Other Names for this Disease
  • Vascular compression of the duodenum
  • Wilkie syndrome
  • Cast syndrome
  • Arteriomesenteric duodenal compression syndrome
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Cause

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What causes superior mesenteric artery syndrome?

The superior mesenteric artery arises from the aorta and is enveloped in fatty and lymphatic tissue (mesenteric pad). It forms an angle of about 38 º and 65º with the abdominal aorta (due in part, to the mesenteric fat pad). Because the third part of the duodenum courses between the angle formed by the superior mesenteric artery and aorta, any factor that sharply narrows the angle between the aorta and superior mesenteric artery can cause entrapment and compression of the third part of the duodenum resulting in superior mesenteric artery syndrome. This angle correlates with body mass index.[1][2]

A variety of factors can contribute to the narrowing of the aorto-mesenteric angle:[2]

  • Significant weight loss: Is the most common factor because it leads to loss of the mesenteric fat pad. Weight loss may occur with severe, debilitating illnesses, trauma, surgery, prolonged bed rest and anorexia nervosa
  • Corrective spinal surgery for scoliosis: This procedure lengthens the spine displacing the superior mesenteric artery origin and reduces mobility of the mesenteric artery because of the cast
  • Congenital defects: Including a short ligament of Treitz (the tissue that suspends the duodenum in the normal position) or an abnormally low origin of the superior mesenteric artery
  • Peritoneal adherences: Caused by inflammatory diseases in the abdomen (pancreatitis, peptic ulcers and others) and by previous abdominal surgeries.

There are some reports about more than one case in the same family and one report about affected identical twins, which suggests a genetic predisposition in some patients.[2]

There are also several reported cases of superior mesenteric artery syndrome associated with celiac axis compression syndrome.[2]

Last updated: 12/18/2015

References
  1. Karrer FM. Superior Mesenteric Artery Syndrome. Medscape Reference. July 2015; http://emedicine.medscape.com/article/932220-overview.
  2. Scovell S & Hamdan A. Superior Mesenteric Artery Syndrome. UpToDate. Waltham, MA: UpToDate; September 19, 2015; http://www.uptodate.com/contents/superior-mesenteric-artery-syndrome. Accessed 12/18/2015.
  3. Superior Mesenteric Artery (SMA) Syndrome. International Foundation for Functional Gastrointestinal Disorders. September 2014; http://www.iffgd.org/site/gi-disorders/other/sma-syndrome.


Other Names for this Disease
  • Vascular compression of the duodenum
  • Wilkie syndrome
  • Cast syndrome
  • Arteriomesenteric duodenal compression syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.