How to Deal with Intrahepatic Bile Duct Stenosis in Congenital Biliary Dilatation and Pancreaticobiliary Maljunction

Abstract

Congenital stenosis of the bile duct in congenital biliary dilatation is not rare and is present in almost all cases of type IV-A, and it plays a major role in the development of postoperative cholangitis, intrahepatic calculi, and/or cancer. There are two different types of stenosis: membranous stenosis and septal stenosis. When extrahepatic bile duct resection is performed without appropriate treatment of the stenosis, non-smooth bile flow affects bacterial growth through the hepaticoenteric anastomosis and leads to recurrent ascending cholangitis and intrahepatic calculus formation. Meticulous probing and excision of intrahepatic bile duct stenosis from the cut end of the common hepatic duct is effective to prevent intrahepatic calculus formation after surgery. Wide hilar hepaticoenterostomy may be a safe and durable reconstructive technique that can be performed at any age and may help to minimize the long-term risk of complications. Hepatectomy may be the treatment of choice when the stenosis is distant from the hepatic hilum or when it is impossible to reach the hepatic hilum due to severe adhesion. In any case, it is important to ensure that the stenosis is resected or reconstructed during the initial operation.