Todani et al developed a classification for congenital bile ducts cysts based on the number and involvement of the intrahepatic and extrahepatic bile ducts.
NOTE: Todani et al preferred the use of the term "bile duct cyst" over "choledochal cyst" since the lesions can occur anywhere along the bile duct system, not just in the choledochus (the common bile duct).
Extrahepatic Bile Duct
Intrahepatic Bile Duct
cystic dilation of common bile duct; may involve common hepatic and cystic ducts
single diverticulum, supraduodenal
single diverticulum, intraduodenal (choledochocele)
single or multiple
• Type V with multiple intrahepatic cysts is referred to as Caroli's disease.
• These lesions are more common in Asians, but rare in European descendents.
The current recommendation is that these cysts be resected, since there is an increased risk for cholangiocarcinoma. For Caroli's disease this may involve liver transplant.
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Specialty: Gastroenterology, Genetics