Idiopathic intestinal perforations in premature infants without evidence of necrotizing enterocolitis.

In the past three years five premature very low birth weight infants (VLBW, birth weight 720-1,300 g) developed spontaneous localized perforations of the small intestine during the second week after birth. There was no evidence of intestinal obstruction or necrotizing enterocolitis (NEC). The clinical presentation and laboratory values as well as the radiologic, intraoperative and histologic findings were different from those of NEC. In four cases the initial symptoms presented as a gray-green discoloration of the flank and inguinal region with an otherwise unremarkable general condition. All patients exhibited a leukocytosis (range 14,700-19,300) and increased neutrophil count (range 9,900-14,800). Additionally, a pronounced increase in the activity of alkaline phosphatase (> 2.000 U/l in 3 cases) and a renewed increase of serum bilirubin was observed. Four of the five infants survived following laparotomy with ileostomy (n = 2) or primary anastomosis (n = 3).