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Biliary cysts

Mark Topazian, MD
Section Editors
Sanjiv Chopra, MD, MACP
Elizabeth B Rand, MD
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF


Biliary cysts are cystic dilations that may occur singly or in multiples throughout the biliary tree. They were originally termed choledochal cysts due to their involvement of the extrahepatic bile duct. However, the original clinical classification [1] was revised in 1977 to include intrahepatic cysts [2]. Biliary cysts are associated with significant complications such as ductal strictures, stone formation, cholangitis, rupture, and secondary biliary cirrhosis. In addition, certain types of biliary cysts have a high risk of malignancy.

This topic will review the pathogenesis, clinical manifestations, diagnosis, and management of biliary cysts. Cholangiocarcinoma, gallbladder cancer, and a detailed discussion of type V biliary cysts (Caroli disease) are discussed elsewhere. (See "Epidemiology, pathogenesis, and classification of cholangiocarcinoma" and "Pathology of malignant liver tumors" and "Clinical manifestations and diagnosis of cholangiocarcinoma" and "Treatment of localized cholangiocarcinoma: Adjuvant and neoadjuvant therapy and prognosis" and "Gallbladder cancer: Epidemiology, risk factors, clinical features, and diagnosis" and "Caroli disease".)


A classification scheme for cysts of the extrahepatic bile ducts (choledochal cysts) was proposed initially in 1959 [1]. It was expanded in 1977 to include intrahepatic cysts [2] and further refined in 2003 to incorporate the presence of an abnormal pancreaticobiliary junction (APBJ) [3]. (See 'Abnormal pancreatobiliary junction' below.)

The classification scheme defines five types of biliary cyst (figure 1) [3-5]:

Type I cysts (50 to 85 percent of cysts) – Type I cysts are characterized by cystic or fusiform dilation of the common bile duct (image 1) [2,6]. Type I cysts do not involve the intrahepatic bile ducts. Type I cysts are further subcategorized as [3]:


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Literature review current through: Sep 2016. | This topic last updated: Feb 6, 2015.
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