Primary non-neoplastic diseases of the extrahepatic bile ducts

In this article, we describe the spectrum of primary diseases occurring in the extrahepatic bile ducts. Three major entities (primary sclerosing cholangitis, extrahepatic biliary atresia, and choledochal cyst) are discussed. Cholangitis, which is a more common condition, is also discussed, along with several other rare conditions. Tumours of the extrahepatic bile duct are not included in this article as they are addressed elsewhere in this volume.