Duodenal atresia with bile-duct anomaly

A full-term female infant is born via vaginal delivery to a 26-year-old primigravida with unexplained polyhydramnios and antenatal diagnosis of duodenal atresia. After birth, bilious fluid was aspirated through the orogastric tube and the initial abdominal radiograph was suggestive of double bubble sign with the absence of air in the distal bowel. Baby passed meconium at 4 h of life and repeat abdominal radiograph showed double bubble sign with the presence of air in distal bowel. Upper gastrointestinal contrast study done revealed distension of the stomach and duodenal bulb with an abrupt narrowing. It also revealed flow of contrast into the jejunal loops on the right side of the spine which was suggestive of intestinal malrotation as the leading diagnosis. However, intra-operative findings confirmed a Type III Duodenal atresia with bile proximal and distal to atretic. Occasionally, distal bowel gases can be seen in radiographs of infants with duodenal atresia due to abnormal development of the biliary system that can allow passage of air from the proximal duodenal segment to the distal. Here, we report a case and discuss the embryology of this rare entity.