Congenital short small intestine: report of three cases

Three cases are presented in which the recognized syndrome of functional small bowel obstruction with malrotation and short small bowel was diagnosed. Each patient underwent an exploratory laparotomy for intestinal obstruction, which revealed short small intestines with malrotation, measuring 30 cm to 39 cm. Ladd's procedure was performed in each case and gastrointestinal patency was assured during the operations. Two patients received long-term parenteral nutrition and one, in addition, received bowel lengthening using Bianchi's method. However, the symptoms persisted. The third patient received a prokinetic agent (cisapride) but with no effect; the parents withdrew further management. All three patients subsequently died. Although autopsies were performed in two cases, the exact deficits of intestinal motility are unknown.