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Superior mesenteric artery syndrome (SMAS), Wilkie syndrome or cast syndrome is a rare condition that usually presents with symptoms of mid to upper gastrointestinal obstruction due to the compression of the duodenum between the abdominal aorta, posteriorly, and the superior mesenteric artery, anteriorly. The aim of this study was to analyze the clinical characteristics, means of diagnosis and management of SMAS in a pediatric population.Retrospective chart review of all patients at the Children's Hospital of Wisconsin with SMAS from 1985 to 2005.Twenty-two cases of SMAS where diagnosed at Children's Hospital of Wisconsin between 1985 and 2005 [14, (64%) female]. Symptoms developed 1 to 393 days (median 5 days) before diagnosis. Presenting symptoms included abdominal pain (59%), vomiting (50%), nausea (40%), early satiety (32%) and anorexia (18%). Diagnosis was made by upper-gastrointestinal radiography in 18 (82%), by computed tomography in 2 (9%) and at laparotomy in 2 (9%). One patient was treated surgically after medical management failed. Mean length of treatment was 65 days (range 13-169), with a mean length of hospitalization of 21 days (range 0-68 days).SMAS usually presents more acutely than chronically with symptoms of small bowel obstruction. Weight loss is not necessary for SMAS development. Prior neurological injury may be a risk factor for development of SMAS. Upper gastrointestinal radiography remains the primary means of diagnosis. SMAS is typically successfully managed medically. Surgical intervention should be reserved for patients' refractory to medical therapy. The expected outcome of SMAS is excellent.