Summary

A 31-year-old man presented with features of recurrent cholangitis for 7 months. Examination revealed icterus and a palpable 10×8 cm lump in the right hypochondrium extending into the epigastrium. Total and direct bilirubin was raised (8.4, 6.7 mg/dL). Alkaline phosphatase (468 U/L) was raised but other liver enzymes were normal. Ultrasound of the abdomen showed dilated intrahepatic biliary radicals (IHBRs) and common hepatic duct (CHD) with a significant intrahepatic portion. Contrast-enhanced CT scan of the abdomen showed similar findings. MR cholangiopancreatography demonstrated diffusely dilated IHBRs and choledochal cyst involving the CHD. The common bile duct was mildly dilated without any filling defect. Tumour markers (carcinoembryonic antigen and cancer antigen 19-9) were normal. The patient underwent complete excision of the extrahepatic biliary system including choledochal cyst and Roux-en-Y hepaticojejunostomy. Opening up the bile duct showed very thick gelatinous material and multiple pedunculated papillary type structures arising from the wall of the choledochal cyst.