Metastatic Cholangiocarcinoma following Choledochal Cyst Excision: An Unusual Cause of Abdominal Pain in a 35-Year-Old Female

Choledochal cysts are anomalous arrangements of the pancreaticobiliary tract and a well-known aetiology for cholangiocarcinoma. The risk of cholangiocarcinoma in patients with an unresected choledochal cyst remains at 20-30%1. Of the five types of choledochal cyst, by far the most common is the extrahepatic fusiform (Type I). Management involves complete excision of the extrahepatic bile duct and hepaticojejunostomy. This reduces the risk of cholangiocarcinoma in this group of patients to as low as 0.7%2. Because of this, it is rare to encounter a patient with cholangiocarcinoma following previous choledochal cyst excision particularly when the incidence of choledochal cyst is approximately 1:100,000 in western countries3.