According to the doctors in one of these studies, the expected outcome for SMA syndrome treatment is generally considered to be excellent. Epidemiology
According to a 1956 study, only 0.3% of patients referred for an upper-gastrointestinal-tract barium studies fit this diagnosis, and is thus a rare disease. Recognition of SMA syndrome as a distinct clinical entity is controversial, due in part to its possible confusion with a number of other conditions, though it is now widely acknowledged. However, unfamiliarity with this condition in the medical community coupled with its intermittent and nonspecific symptomatology probably results in its underdiagnosis.As the syndrome involves a lack of essential fat, more than half of those diagnosed are underweight, sometimes to the point of sickliness and emaciation. Females are impacted more often than males, and while the syndrome can occur at any age, it is most frequently diagnosed in early adulthood. The most common co-morbid conditions include mental and behavioral disorders including eating disorders and depression, infectious diseases including tuberculosis and acute gastroenteritis, and nervous system diseases including muscular dystrophy, Parkinsons disease, and cerebral palsy. History
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. Society and culture
American actor, director, producer, and writer Christopher Reeve had the acute form of SMA syndrome as a result of spinal cord injury. Non-profit
In 2017 the only US non-profit began distributing grants to those with SMAS.
In some cases, emergency surgery is necessary upon presentation. A six-week trial of medical treatment is recommended in pediatric cases. The goal of medical treatment for SMA syndrome is resolution of underlying conditions and weight gain. Medical treatment may involve nasogastric tube placement for duodenal and gastric decompression, mobilization into the prone or left lateral decubitus position, 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). Pro-motility agents such as metoclopramide may also be beneficial. Symptoms may improve after restoration of weight, except when reversed peristalsis persists, or if regained fat refuses to accumulate within the mesenteric angle. Most patients seem to benefit from nutritional support with hyperalimentation irrespective of disease history.If medical treatment fails, or is not feasible due to severe illness, surgical intervention is required. The most common operation for SMA syndrome, duodenojejunostomy, was first proposed in 1907 by Bloodgood. Performed as either an  open surgery or laparoscopically, duodenojejunostomy involves the creation of an anastomosis between the duodenum and the jejunum, bypassing the compression caused by the AA and the SMA. Less common surgical treatments for SMA syndrome include Roux-en-Y duodenojejunostomy, gastrojejunostomy, anterior transposition of the third portion of the duodenum, intestinal derotation, division of the ligament of Treitz (Strongs operation), and transposition of the SMA.