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Extrahepatic bile ducts

Congenital anomalies

Choledochal cyst

Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 15 February 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.


● Associated with other hepatobiliary tract abnormalities
● May rupture spontaneously, be associated with reflux of pancreatic enzymes into bile duct
● Associated with stones in 1-30% of cases
● Not actually a cyst, but a dilation of common bile duct which may secondarily obstruct other biliary ducts or the duodenum
● 2-8% develop biliary tract carcinoma (20x normal risk) at mean age 34 years, lower risk if surgery earlier in life (age 10 years or less), carcinoma may develop within wall of cyst, within gallbladder or bile ducts
● In Korea, usually type I or IVa (Arch Surg 2011;146:1178)

Type 1: segmental or diffuse fusiform dilation of common bile duct (50-90%)
Type 2: diverticulum of common bile duct
Type 3: dilation of intraduodenal common bile duct (choledochocele)
Type 4: multiple cysts of extrahepatic bile ducts with (4A) or without (4B) cysts of intrahepatic ducts
Type 5: one or more cysts of intrahepatic ducts (Caroliís disease)


● 1 per 13,000 live births in US vs. 1 per 1000 in Japan
● 75% girls
● Most common cause of obstructive jaundice in infants beyond infancy, but may be found at any age

Case reports

● 11 year old boy (Hum Pathol 2003;34:99)
● 34 year old man with adenosquamous carcinoma in congenital choledochal cyst associated with pancreatico-biliary maljunction (Pathol Int 2009;59:482)
● 41 year old woman with squamous cell carcinoma arising within choledochal cyst (Dig Dis Sci 2008;53:2822)
● Type IV A choledochal cyst with cystic duct cyst (HPB (Oxford) 2010;12:285)


● Complete cyst removal with biliary reconstruction, usually with Roux-en-Y hepaticojejunostomy

Gross description

● Contain 1-2 liters of bile, up to 15 cm in diameter
● Wall is fibrous, variable calcification, 2-10 mm thick

Micro description

● Focal columnar epithelium (more intact in infants)
● Walls composed of dense fibrous tissue, scattered smooth muscle and elastic fibers
● Variable chronic inflammatory infiltrate (increases with age)
● Variable hyperplasia, metaplasia, dysplasia

Micro images

Giant choledochal cyst

End of Extrahepatic bile ducts > Congenital anomalies > Choledochal cyst

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