A Hayes maneuver, which corresponds to applying pressure below the umbilicus in cephalad and dorsal direction, elevates the root of the SMA, also slightly easing the constriction.
[citation needed] Patients with the chronic, congenital form of SMA syndrome predominantly have a lengthy or even lifelong history of abdominal complaints with intermittent exacerbations depending on the degree of duodenal compression.
[9] Predisposition is easily aggravated by any of the following: poor motility of the digestive tract,[3] retroperitional tumors, loss of appetite, malabsorption, cachexia, exaggerated lumbar lordosis, visceroptosis, abdominal wall laxity, peritoneal adhesions, abdominal trauma,[10] rapid linear adolescent growth spurt, weight loss, starvation, catabolic states (as with cancer and burns), and history of neurological injury.
Standard diagnostic exams include abdominal and pelvic computed tomography (CT) scan with oral and IV contrast, upper gastrointestinal series (UGI), and, for equivocal cases, hypotonic duodenography.
[14][15] Despite multiple case reports, there has been controversy surrounding the diagnosis and even the existence of SMA syndrome since symptoms do not always correlate well with radiologic findings, and may not always improve following surgical correction.
[17] Since females between the ages of 10 and 30 are most frequently affected, it is not uncommon for physicians to initially and incorrectly assume that emaciation is a choice of the patient instead of a consequence of SMA syndrome.
Patients in the earlier stages of SMA syndrome often remain unaware that they are ill until substantial damage to their health is done, since they may attempt to adapt to the condition by gradually decreasing their food intake or naturally gravitating toward a lighter and more digestible diet.
Medical treatment may involve nasogastric tube placement for duodenal and gastric decompression, mobilization into the prone or left lateral decubitus position,[20] the reversal or removal of the precipitating factor with proper nutrition and replacement of fluid and electrolytes, either by surgically inserted jejunal feeding tube, nasogastric intubation, or peripherally inserted central catheter (PICC line) administering total parenteral nutrition (TPN).
[7] Performed as either an open surgery or laparoscopically, duodenojejunostomy involves the creation of an anastomosis between the duodenum and the jejunum,[23] bypassing the compression caused by the AA and the SMA.
Reversed peristalsis has been shown to respond to duodenal circular drainage—a complex and invasive open surgical procedure originally implemented and performed in China.
[29] Recognition of SMA syndrome as a distinct clinical entity is controversial, due in part to its possible confusion with a number of other conditions,[30] though it is now widely acknowledged.
[19] 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.
[31] American actor, director, producer, and writer Christopher Reeve had the acute form of SMA syndrome as a result of spinal cord injury.
SMAS Patient assistance works to disprove inaccurate information, educate on better diagnostic testing, and more successful surgeries such as Duodenal Deroation with LADDS.