Superior mesenteric artery (SMA) syndrome can be a life-threatening gastrointestinal (GI) disorder caused by a compression of the third portion of the duodenum (part of the small intestine) in between the abdominal aorta (AA) and superior mesenteric artery. SMA is also called Wilkie’s syndrome, cast syndrome (in scoliosis patients), mesenteric root syndrome, chronic duodenal ileus or intermittent arterio-mesenteric occlusion.
Superior mesenteric artery syndrome usually occurs in older children and adolescents. In one study, 75% of SMA cases occurred in patients aged 10-30 years. Since females between the ages of 10 and 30 are most frequently afflicted with SMA, some doctors incorrectly assume that the emaciation, or being too thin, is a choice of the teenage girl or woman instead of a result of SMA syndrome.
Symptoms include early satiety or quickly feeling full, nausea, vomiting, extreme “stabbing” abdominal pain after eating, abdominal distention or bloating, eructation (belching or burping), tenderness of the abdominal area or stomach, weight loss and severe malnutrition. Symptoms are often aggravated when leaning to the right or taking a supine or face up position. “Food fear” is a common development among patients with chronic SMA syndrome due to the pain and discomfort that follows after eating.
Conservative treatment is recommended in all patients with superior mesenteric artery syndrome. This includes adequate nutrition, nasogastric decompression, and proper positioning of the patient after eating in the left lateral decubitus, prone, or knee-to-chest position. Enteral (tube feeding) and total parenteral nutritional (TPN) through a central catheter (PICC line) may be needed to provide the much needed calories.
If conservative treatment fails to alleviate the compression, surgery may be needed. The most performed operation for SMA syndrome is called duodenojejunostomy. A duodenojejunostomy involves the creation of an anastomosis or connection between the duodenum and the jejunum, bypassing the compression caused by the AA and the SMA. Other surgical treatments for SMA syndrome include Roux-en-Y duodenojejunostomy, intestinal derotation, anterior transposition of the third portion of the duodenum, gastrojejunostomy, or division of the ligament of Treitz.
Any delay in the diagnosis or treatment of SMA syndrome can cause severe malnutrition, dehydration, oliguria (low urine output), electrolyte abnormalities, hypokalemia (decreased potassium level), acute gastric perforation or rupture, portal venous gas, intestinal perforation, mesenteric ischemia, gastrectasia (dilation of stomach), upper gastrointestinal (GI) bleeding, massive GI hemorrhage, hypovolemic shock, aspiration pneumonia, sudden cardiovascular collapse or death.
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