Duodenojejunal atresia with apple peel configuration of the ileum and absent superior mesenteric artery: observations on pathogenesis.

A child with loss of the third and fourth part of the duodenum and of the proximal jejunum was found to have an apple peel configuration of the remaining small bowel. The complete absence of branches from the superior mesenteric artery impaired the blood supply of the distal duodenum. An annular pancreas was found in this patient with Down's syndrome. This anomaly may have impaired the flow through the pancreaticoduodenal arcade, which would normally compensate for the distal vascular occlusion. According to current understanding, duodenal atresia is a primary malformation. The current case suggests, however, that in rare circumstances vascular accidents may be the underlying cause for duodenal atresia.