Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery: A hereditary compound structure in duodenal atresia?

Duodenal atresia predominantly represents an isolated entity, but could also be part of a complexly structured intestinal malformation. We report four children, including two from one family, with duodeno-jejunal atresia associated with malrotation, volvulus, and absent parietal attachment of the mesentery. Gross absence of the mesentery and absence of distal parts of the superior mesenteric artery were the most remarkable findings. The small intestine was supplied retrogradely from the right colic artery. Consideration of embryological theories of this malformation takes into account the important role of the duodeno-jejunal flexure during the process of entry of the small bowel loops into the abdomen. Familial occurrence suggests autosomal recessive inheritance. We propose differentiating the pathoanatomical findings in our patients from classical apple peel small bowel syndrome (APSB). © 2002 Wiley-Liss, Inc.