Abstract

A 25-year-old male presented with repeated attacks of cholangitis of six months duration. On examination, the patient was febrile and icteric, abdominal examination revealed a 6 cm firm hepatomegaly. The routine blood investigations were normal except for a raised serum bilirubin (4 mg/dL) and alkaline phosphatase (940 IU/mL). The patient underwent an ultrasound followed by an MRCP and contrast-enhanced CT scan of the abdomen, (Fig. 1). Serum alpha fetoprotein was 3 ng/mL and CA 19-9 was 3656 U/mL. Figure 1 MRCP and contrast-enhanced CT scan of the abdomen. Question What is the diagnosis? Figure 2 MRI showing intraductal calculi. Figure 3 MRCP showing multiple cystic dilations of bilateral intrahepatic ducts with signal voids seen in left duct suggestive of calculi, (white arrows). This is a typical picture of Caroli’s disease with bilateral intrahepatic cystic dilatations with ... Answer Caroli’s disease with hepatolithiasis and cholangiocarcinoma.

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