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 p roximal jejunum was found to have an apple peel configuration of the remain ing small bowel. The complete absence of branches from the superior mesente ric artery impaired the blood supply of the distal duodenum. An annular pan creas was found in this patient with Down's syndrome. This anomaly may have impaired the flow through the pancreaticoduodenal arcade, which would norm ally compensate for the distal vascular occlusion. According to cur rent un derstanding, duodenal atresia is a primary malformation. The current case s uggests, however, that in rare circumstances vascular accidents may be the underlying cause for duodenal atresia. J Pediatr Surg 34:1427-1429. Copyrig ht (C) 1999 by W.B. Saunders Company.