A 28-year-old woman with recurrent attacks of cholangitis has MRCP showing cystic dilatation of both intrahepatic ducts and the extrahepatic common bile duct. According to the Todani classification, this is which type of choledochal cyst, and what is the definitive treatment?

• A Type II, cystoduodenostomy
• B Type III, transduodenal sphincterotomy
• C Type V, left lateral segmentectomy
• D Type IVa, complete excision with Roux-en-Y hepaticojejunostomy ✓

Explanation

Todani type IVa denotes cystic dilatation involving both intrahepatic and extrahepatic bile ducts. Definitive treatment is complete excision of the extrahepatic cyst with mucosa-to-mucosa Roux-en-Y hepaticojejunostomy, because retained cyst mucosa carries a high lifetime risk of cholangiocarcinoma, recurrent cholangitis and anastomotic stricture. Internal drainage procedures such as cystoduodenostomy are abandoned for exactly this reason. Type V disease is confined to the intrahepatic ducts and corresponds to Caroli disease.

Reference: Sabiston Textbook of Surgery, 21st ed.

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