Superior Mesenteric Artery Syndrome
Superior mesenteric artery (SMA) syndrome is a very rare, life-threatening gastro-vascular disorder characterized by a compression of the third portion of the duodenum by the abdominal aorta (AA) and the overlying superior mesenteric artery. The syndrome is typically caused by an angle of 6°-25° between the AA and the SMA, in comparison to the normal range of 38°-56°, due to a lack of retroperitoneal and visceral fat. In addition, the aortomesenteric distance is 2-8 millimeters, as opposed to the typical 10-20.
SMA syndrome was first described in 1861 by Carl Freiherr von Rokitansky in victims at autopsy, but remained pathologically undefined until 1927 when Wilkie published the first comprehensive series of 75 patients. Only 0.013 - 0.3% of upper-gastrointestinal-tract barium studies support a diagnosis, making it one of the rarest gastrointestinal disorders known to medical science. With only about 500 cases reported in the history of English-language medical literature, recognition of SMA syndrome as a distinct clinical entity is controversial, though it is now widely acknowledged. SMA syndrome is estimated to have a mortality rate of 1 in 3.
SMA syndrome is also known as Wilkie's syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arterio-mesenteric occlusion. It is distinct from Nutcracker syndrome, which is the entrapment of the left renal vein between the AA and the SMA.
Read more about Superior Mesenteric Artery Syndrome: Symptoms, Causes, Demographics, Mortality, Diagnosis, Treatment, See Also
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