The duodenal atresia describes a congenital developmental disorder in which, due to a lack of recanalization of the duodenal canal in the embryonic period, the lumen of the duodenum is not continuous. Either a short or a longer part of the intestine is missing, a membrane lies in the intestinal lumen or the pancreas lies in the wrong place.
The consequence of the lack of patency is a greatly enlarged stomach and intestinal portion before atresia, the remaining intestine remains small, because the swallowed amniotic fluid can not reach the intestinal component. Furthermore, a polyhydramnione is present.
Depending on the developmental disorder, the duodenal atresia is divided into three forms:
Type I - Duodenal Diaphragm: a diapragmatic membrane formed from the mucosa and intact muscle wall.
Type II - complete duodenal atresia: Connective tissue tract connecting the two ends of the atretic duodenum.
Type III - complete duodenal atresia: complete separation of the two ends of the duodenum, sometimes together with the anus pancreas.
Duodenal atresia is an isolated anomaly in 30-52% of children, all others suffer from other congenital malformations. The disease is in about 20-30% of cases associated with a trisomy 21 and in 20-25% with a heart defect. Annular pancreas, underdeveloped duodenum and biliary and pancreatic ducts or choledochal cysts are also often associated with anomalies of duodenal atresia.
The symptoms vary depending on the severity of the disease. If the defect is cranial of the Papilla Vateri manifested in the first days of life a food intolerance. In the case of a localization caudal to the papilla, the children begin to vomit with difficulty for several hours after birth. caudal to the papilla. Other related symptoms include dehydration, weight loss and hypochloremic metabolic alkalosis.
If there is a severe bruising, the only sign is a failure to thrive. After several months or years after birth, the children vomit bile without the abdomen bothering.
The cause is unknown so far, most cases occur sporadically. However, autosomal recessive inheritance is suspected for some familial cases. Vascular anomalies, lack of recanalization of the duodenal lumen and abnormal immigration of neutral cells may play a causative role.
The prognosis is excellent for early surgical interventions.