An 8-year-old girl has recurrent episodes of jaundice with abdominal pain. Examination reveals a smooth cystic mass in the right upper quadrant. Ultrasound shows fusiform cystic dilatation of both intrahepatic and extrahepatic bile ducts without an obstructing cause. The most appropriate definitive treatment is:

• A Cystoduodenostomy for internal drainage
• B Percutaneous catheter drainage followed by annual surveillance
• C Complete excision of the cyst with Roux-en-Y hepaticojejunostomy ✓
• D Liver transplantation

Explanation

This is a Todani type IVA choledochal cyst involving both intrahepatic and extrahepatic ducts. Definitive treatment is complete cyst excision because retained cyst mucosa carries a lifetime risk of cholangiocarcinoma, which drainage procedures such as cystoduodenostomy do not prevent; they leave dysplastic epithelium in situ and are associated with recurrent cholangitis and malignancy. Transplantation is reserved for diffuse Caroli disease with cirrhosis.

Reference: Sabiston Textbook of Surgery, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.