Duodenojejunal Atresia With Volvulus, Absent Dorsal Mesentery, And Absent Superior Mesenteric Artery

Clinical Features

Duodenal atresia (223400) predominantly represents an isolated entity, but also can be part of a complexly structured intestinal malformation such as apple peel small bowel syndrome (243600) or familial intestinal polyatresia syndrome (FIPA; 243150). Pumberger et al. (2002) described 4 children, including 2 from 1 family, with duodenojejunal 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 embryologic theories of this malformation took into account the important role of the duodenojejunal flexure during the process of entry of the small bowel loops into the abdomen. Familial occurrence suggested autosomal recessive inheritance. Because of the constant association with malrotation and the normal length of the small bowel, Pumberger et al. (2002) proposed differentiating the pathoanatomic findings in these patients from those of classic apple peel small bowel syndrome.