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

Abstract

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).

The serosal mesothelium is a major source of smooth muscle cells of the gut vasculature

“…Thus, it is possible that the serosa may provide a natural source of divergent cells to be used in the repair of damaged adult structures. Finally, a relatively uncommon developmental syndrome, called 'Apple Peel Bowel', producing intestinal atresia has been reported (Federici et al, 2003;Pumberger et al, 2002;Waldhausen and Sawin, 1997). Interestingly, this atresia or intestinal wasting is associated with regional loss of the serosa and its associated blood vessels, and is suggestive of a mechanistic relationship between the generation of coelomic mesothelia and vascular development.…”

Double bubble, double trouble

“…1 Upper gastrointestinal contrast study of case 1 revealing dilated stomach and duodenum with failure of the contrast to progress beyond the third part of the duodenum, consistent with a congenital duodenal obstruction [8]. Another report described four cases of duodenal atresia in which two siblings from one family had duodenojejunal atresia associated with malrotation, volvulus, and absent parietal attachment of mesentery, suggesting a familial occurrence as an autosomal recessive inheritance condition [12].…”

Duodenojejunal atresia with absent dorsal mesentery, choledochal cyst, and malrotation in a premature newborn—a case report

“…The association of duodenal atresia and a choledochal cyst is very rare, with only sporadic cases reported [2,4,5]. Very few reports have described extensive continuous atresias from the duodenum to the mid small bowel [6][7][8]. In the cases described, the duodenal atresia was from the third or fourth part of the duodenum.…”