Superior mesenteric artery syndrome in intellectually disabled children

Superior mesenteric artery syndrome (SMAS) is a rare cause of small intestinal obstruction in pediatric patients. Children with intellectual disabilities are a challenging patient population in which to make this diagnosis. The goal of this study was to determine the frequency, presenting symptoms, and outcomes of SMAS in intellectually disabled and non-intellectually disabled children.

Retrospective chart review of pediatric patients with SMAS admitted to Penn State Hershey Children's Hospital between January 1999 and July 2010.

Sixteen children with SMAS were identified. The majority were male (n = 9) and more than two thirds had an intellectual disability (n = 11). Presenting symptoms were similar between groups, but 78% (7/9) of intellectually disabled patients who had a gastrostomy tube presented with feeding intolerance. Although intellectually disabled patients had a higher number of comorbidities and the number of health care visits before diagnosis was higher, this did not reach statistical significance. There were also no significant differences in length of symptoms before diagnosis and amount of weight loss. However, the weight-for-age percentiles in intellectual disabled children were significantly lower in those without an intellectual disability (9.09 [20.31] vs 48 [20.19], respectively, P ≤ 0.001). Seventy-five percent of patients responded favorably to conservative treatment, but 36% (4/11) of intellectually disabled patients required multiple treatments before there was an improvement in their condition.

Superior mesenteric artery syndrome was found more often in children with an intellectual disability. These data highlight the need to consider SMAS if there is difficulty tolerating gastrostomy tube feedings in patients with weight-for-age percentiles less than 5%.