A 15-year-old female presents with RUQ abdominal pain. Workup reveals a choledochal cyst. Which of the following statements is TRUE?

Correct Answer: Patients with a choledochal cyst have an increased risk of cholangiocarcinoma.
Description: Choledochal cysts can involve the intrahepatic and/or extrahepatic biliary tree (see Figure below for classification). Choledochal cysts present more commonly in infants and children but may present in adults. They are more common in females. The classic triad of jaundice, RUQ mass and abdominal pain is found in less than a third of patients. There is an association between choledochal cysts and hepatobiliary cancers, most commonly cholangiocarcinoma. For most types of choledochal cyst, excision of the cyst with a Roux-en-Y biliary enteric anastomosis is recommended. Laparoscopic cholecystectomy alone is not sufficient.Classification of choledochal cysts. Type I, fusiform or cystic dilations of the extrahepatic biliary tree, are the most common type, making up over 50% of all choledochal cysts. Type II, saccular diverticulum of an extrahepatic bile duct, is rare, comprising <5% of choledochal cysts. Type III, bile duct dilations within the duodenal wall (choledochoceles), make up about 5% of choledochal cysts. Types IV a and IV b, multiple cysts, make up 5-10% of choledochal cysts. Type IV a affects both extrahepatic and intrahepatic bile ducts, while type IV b cysts affect the extrahepatic bile ducts only. Type V, intrahepatic biliary cysts, are very rare and make up only about 1% of choledochal cysts.
Category: Surgery
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