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Benign conditions of the intra- or extrahepatic bile ducts can range from focal or diffuse dilations (choledochal cyst) to obstructive strictures of the biliary tree. Historically, choledochal cyst disease was considered a disease of childhood but is increasingly being recognized in adults. In the United States, benign biliary strictures most commonly occur as a result of injury after cholecystectomy but also occur in a number of diverse inflammatory conditions affecting the biliary tree. Both conditions represent significant clinical challenges where proper evaluation and management are paramount to prevent serious clinical sequelae.

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Choledochal cysts are focal or diffuse dilations of the biliary tree and, aside from biliary atresia, are the most common congenital abnormality of the biliary tree. Choledochal cysts can occur as single or multiple cysts throughout the extra- or intrahepatic bile ducts. The cysts can predispose patients to recurrent cholangitis or pancreatitis, choledocholithiasis, secondary biliary cirrhosis, biliary stricture, and malignancy.

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The incidence of choledochal cysts varies significantly throughout the world. Choledochal cysts appear to be most common in Asian countries with an estimated incidence of 1 in 13,000 and have been reported to be as high as 1 in 1000, in reports from Japan. In Western countries, choledochal cysts occur much less frequently, with reported rates that vary from 1 in 150,000 to 1 in 2 million live births.1 Biliary cysts are four times more common in women compared with men. Approximately 60% of patients with choledochal cysts present during their first decade of life and 25% present in adults.2 There are a few case reports of choledochal cysts occurring within families but generally do not have a recognized hereditary pattern.

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Classification

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The anatomy of choledochal cyst disease was first described by Vater in 1723 and in 1959 Alonso-Lej categorized three types of choledochal cysts.3 The classification system was revised by Todani and colleagues in 1977 to the five cyst categories4 that are in use today (Table 50-1). A similar classification has been proposed based on bile duct cholangiographic appearance.5 A further Todani classification reflects the presence or absence of pancreaticobiliary maljunction; however, this revision has not widely been accepted.6

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Table Graphic Jump Location
Table 50-1: Alonso-Lej/Todani Modification of the Classification of Choledochal Cysts

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