Congenital short small bowel with malrotation in a neonate.

A 1-day-old male infant with congenital short small bowel was transferred to our hospital because of bilious vomiting and abdominal distension. An exploratory laparotomy performed at the age of 8 days revealed malrotation without any volvulus or stenosis of the small intestine, which was 45 cm long. There were no other anomalies except absence of the appendix. Postoperative nutritional support was by intravenous hyperalimentation and administration of elemental diet. However, parenteral nutrition was discontinued because of hyperbilirubinaemia. The patient died from marked emaciation at 2 months of age despite the administration of elemental diet. Therefore, careful total parenteral nutrition is the only management for successful therapy until the child's intestinal absorptive function develops.