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.

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Objective To assess the incidence of hemophagocytic lymphohistiocytosis (HLH) in a well defined population of children with inflammatory bowel disease (IBD) and evaluate the common clinical and laboratory characteristics of individuals with IBD who developed HLH. Study design We conducted a retrospective study of all children who developed HLH over an 8 year period. The incidence of HLH in patients with IBD was calculated using US census data and a statewide project examining the epidemiology of pediatric IBD. RESULTS 20 cases of HLH occurred among children in Wisconsin during the study period. Five cases occurred in children with IBD. Common characteristics include: Crohn disease (CD), thiopurine administration, fever >5 days, lymphadenopathy, splenomegaly, anemia, lymphopenia and elevated serum triglycerides and ferritin. Four patients had primary Epstein Bar Virus (EBV) infections. The incidence of HLH among all children in Wisconsin was 1.5/100,000 per year. The risk was more than 100 fold greater for children with CD (p< 0.00001). CONCLUSIONS Pediatric patients with CD are at increased risk for developing HLH; primary EBV infection and thiopurine administration may be risk factors.