GARD Superior Mesenteric Artery Syndrome

Genetic and Rare Diseases Information Center (GARD)

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

Superior mesenteric artery syndrome (SMAS) is a digestive condition that occurs when the duodenum (the first part of the small intestine) is compressed between two arteries (the aorta and the superior mesenteric artery). This compression causes partial or complete blockage of the duodenum.[1] Signs and symptoms may include abdominal fullness; bloating after meals; nausea and vomiting; and abdominal cramping that may be helped by lying in certain positions. A variety of factors may contribute to SMAS, including prolonged bed rest, weight loss, rapid growth, previous abdominal surgery, lordosis, use of body casts, and loss of tone in abdominal muscles. It may also occur with pancreatitis, peptic ulcers, and other inflammatory abdominal conditions.[2] Treatment may include addressing the underlying cause and/or dietary modifications (small feedings or a liquid diet).[2][1]

Last updated: 12/11/2014

References

  1. Frederick Merrill Karrer, MD. Superior Mesenteric Artery Syndrome Clinical Presentation. Medscape Reference. October 2014; http://emedicine.medscape.com/article/932220-overview.
  2. Superior Mesenteric Artery (SMA) Syndrome. International Foundation for Functional Gastrointestinal Disorders. September 2014; http://www.iffgd.org/site/gi-disorders/other/sma-syndrome.

What are the signs and symptoms of superior mesenteric artery syndrome?

The signs and symptoms of superior mesenteric artery syndrome vary but may include:[1]

  • Abdominal fullness
  • Bloating after meals
  • Nausea and vomiting of partially digested food
  • Mid-abdominal “crampy” pain that may be relieved by the prone or knee-chest position.
Last updated: 12/11/2014
References

  1. Superior Mesenteric Artery (SMA) Syndrome. International Foundation for Functional Gastrointestinal Disorders. September 2014; http://www.iffgd.org/site/gi-disorders/other/sma-syndrome.

What causes superior mesenteric artery syndrome?

A variety of factors can contribute to the development of superior mesenteric artery syndrome (SMAS), including:[1]

  • prolonged bed rest
  • rapid, severe weight loss
  • rapid growth
  • previous abdominal surgery
  • lordosis
  • use of body casts
  • loss of tone in abdominal muscles

SMAS has also been reported to occur in some people with pancreatitis, peptic ulcers, and other inflammatory conditions of the abdomen.[1] Rarely, the condition may be caused by an abnormality in the structure of the body (such as unusual position or location of the artery). Familial SMAS, recurrent SMAS, and idiopathic neonatal SMAS have also been reported.[2]

Last updated: 12/11/2014
References

  1. Superior Mesenteric Artery (SMA) Syndrome. International Foundation for Functional Gastrointestinal Disorders. September 2014; http://www.iffgd.org/site/gi-disorders/other/sma-syndrome.
  2. Frederick Merrill Karrer, MD. Superior Mesenteric Artery Syndrome Clinical Presentation. Medscape Reference. October 2014; http://emedicine.medscape.com/article/932220-overview.

How is superior mesenteric artery syndrome diagnosed?

Superior mesenteric artery syndrome (SMAS) is usually suspected based on signs and symptoms. A diagnosis can be confirmed by imaging studies such as an upper GI series, hypotonic duodenography (an x-ray procedure that produces images of the duodenum), and computed tomography (CT scan).[1]
Last updated: 12/13/2014
References

  1. Frederick Merrill Karrer, MD. Superior Mesenteric Artery Syndrome Clinical Presentation. Medscape Reference. October 2014; http://emedicine.medscape.com/article/932220-overview.

How might superior mesenteric artery syndrome be treated?

Treatment for superior mesenteric artery syndrome typically focuses on addressing the underlying cause of the condition.[1] For example, symptoms often improve after lost weight is restored or a body cast is removed.[2]Nasogastric decompression (a tube passed through the nose into the stomach) and proper positioning after eating (such as lying prone and knee-to-chest position) may be recommended to alleviate symptoms.[1]

In severe cases, intravenous (IV) nutritional support and/or a feeding tube may be needed to provide enough calories. Affected people can usually then be started on oral liquids, followed by slow and gradual introduction of small and frequent soft meals as tolerated. Then, regular solid foods may be introduced. Metoclopramidetreatment may be beneficial for some people.[1]

Surgery may be needed if other treatment strategies do not work. However, other treatment options should usually be tried for at least 4-6 weeks before considering surgery.[1]

Last updated: 12/11/2014
References

  1. Frederick Merrill Karrer, MD. Superior Mesenteric Artery Syndrome Clinical Presentation. Medscape Reference. October 2014; http://emedicine.medscape.com/article/932220-overview.
  2. Superior Mesenteric Artery (SMA) Syndrome. International Foundation for Functional Gastrointestinal Disorders. September 2014; http://www.iffgd.org/site/gi-disorders/other/sma-syndrome.

What is the long-term outlook for people with superior mesenteric artery syndrome?

The long-term outlook (prognosis) for people with superior mesenteric artery syndrome is typically excellent with early diagnosis and appropriate treatment.[1]
Last updated: 12/13/2014
References

  1. Frederick Merrill Karrer, MD. Superior Mesenteric Artery Syndrome Clinical Presentation. Medscape Reference. October 2014; http://emedicine.medscape.com/article/932220-overview.

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