[Choledochal cysts in adult patients--presentation and clinical course]

A 35-year-old male was admitted with recurrent acute pancreatitis of unknown origin. He was found to have a cystic lesion in the upper abdomen believed to be a pancreatic pseudocyst (patient 1). A 59-year-old female with a choledochal cyst developed acute pancreatitis (patient 2). A 32-year-old male who had been operated upon for a choledochal cyst during childhood was admitted for upper abdominal pain, fever and vomitus (patient 3).

Patient 1 was found to have a choledochal cyst type IV a according to TODANI. Patient 2 was diagnosed to have a choledochal cyst type Ib according to TODANI. The cyst was believed to contain a bile duct carcinoma. In patient 3, sonography showed an advanced Klatskin tumour with infiltration of the portal vein, the hepatic artery and the liver. Bile cytology confirmed the carcinoma. In all patients cholestasis was found. Pancreatic enzymes were elevated in patients 2 and 3.

Patient 1 underwent left hemihepatectomy and received a biliodigestive anastomosis. In patient 2 the choledochal cyst was resected while undergoing pylorus-preserving resection of the pancreatic head. The tumour suspected was confirmed and R0 resected. Patient 3 presented with an incurable bile duct carcinoma. He died 3 months later from multiple lung emboli.

Choledochal cysts are associated with a 20 fold increase in the incidence of bile duct carcinoma.