Duodenal atresia type III showing distal bowel gas and atypical malrotation in association with gastric and bile duct duplications

Association of duodenal atresia (DA) with gastric duplication cyst is an extremely rare entity. Furthermore, DA type III with the presence of distal bowel gas is very rare. We report the case of a 13-day-old preterm male with neonatal intestinal obstruction. Radiological evaluation revealed duodenal obstruction at the second part with the presence of few distal bowel gas shadows. Laparotomy revealed dilated gastroduodenum, atypical malrotation, and a cystic structure (duplication cyst) identical to the stomach attached to its greater curvature along with type III atresia of the second part of the duodenum. Duodenotomies revealed the presence of bile along with air at either blind ends of the duodenum which could be explained by the presence of bile duct duplication at the distal end. We propose that this association is due to “defective mesenchyme formation during organogenesis,” which may explain multiple duplication abnormalities and DA.