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Superior Mesenteric Artery Syndrome, Pediatric

Keisha R. Barton, MD, Kristin L. Van Buren, MD, MEd and Eric H. Chiou, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Superior mesenteric artery (SMA) syndrome is the extrinsic compression of the third portion of the duodenum between the SMA and aorta.

  • It is also called Wilkie syndrome, Cast syndrome...

DIAGNOSIS

HISTORY

  • Clinical presentation can be acute or chronic with gradual, progressive symptoms.

  • Symptoms are generally consistent with proximal small bowel obstruction, including the following:

    • Nause...

TREATMENT

GENERAL MEASURES

  • Correct fluid and electrolyte imbalances.

  • Decompress obstruction.

    • Insert nasogastric tube to decompress stomach and proximal duodenum.

  • Definitive treatment is aimed at correctin...

ONGOING CARE

PROGNOSIS

  • Delay in diagnosis of SMA syndrome can result in the following:

    • Electrolyte disturbances

    • Dehydration and malnutrition

    • In severe cases, possible gastric pneumatosis, perforation, for...

ADDITIONAL READING

  • Agrawal GA, Johnson PT, Fishman EK. Multidetector row CT of superior mesenteric artery syndrome. J Clin Gastroenterol.  2007;41(1):62–65. [View Abstract on OvidInsights]

  • Altiok ...

CODES

ICD9

557.1 Chronic vascular insufficiency of intestine 

ICD10

K55.1 Chronic vascular disorders of intestine 

SNOMED

197006009 superior mesenteric artery syndrome (disorder) 

FAQ

  • Q: When the diagnosis of SMA syndrome is suspected, what are the next steps in management?

  • A: The general sequence is to confirm the diagnosis with an imaging study such as an upper GI contrast stu...

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