Bile duct cysts also called is choledochal cysts. This a congenital abnormality of the bile duct that causes abnormal enlargement of the bile duct. Bile duct cysts are rare and of uncertain origin. Most have been found in young females of Asian descent, but an increasing number have occurred in Western adults. The majority of Bile duct cysts present in childhood. The patient presents with jaundice due a decrease in drainage of bile from the abnormal bile duct. The poor drainage cause infections in the bile duct in many patient. Some patients develop repeated attacks of pancreatitis since the pancreatic duct may enter into the abnormal bile duct.

Congenital bile duct cysts are related to anomalous arrangements of the pancreaticobiliary duct system which seems to play a crucial role in the development of cystic bile ducts and biliary carcinogenesis. Bile stasis together with reflux of pancreatic juice causing longstanding inflammation and activation of bile acids might be the factors in carcinogenesis of the exposed bile duct epithelium in the cystic wall. In the case of primary or secondary extrahepatic bile duct cysts, primary excision is mandatory because of the high risk to develop biliary cancer with even nowadays poor prognosis despite advantages in biliary surgery during the last years.

The treatment of choice for choledochal cysts is dismissal of the cyst by surgery. In this procedure the abnormal segment of the bile duct is completely removed by surgery and the small intestine is then brought up to the bile duct and sutured to the normal bile duct so that bile secretions are routed back into the intestine. Antibiotic therapy and supportive care should be given to patients presenting with cholangitis. In selected patients with a favorable anatomy we will consider a laparoscopic approach for removal of the cyst. In this procedure the cyst is completely excised and a segment of small intestine is brought up.