Diseases of bile duct in children

Figure 15  A 2-month-old male infant presented with jaundice and pale stools. Exploratory laparotomy revealed a collapsed gallbladder, yellow bile, and patent right hepatic bile duct. The liver histopathology at the age of 2 months revealed cirrhosis with cholestasis jaundice. At the age of 7 months, repeated ultrasonography showed liver cirrhosis with evidence of portal hypertension, multiple cysts along the periportal distribution with a hyperechogenic area at the periportal region, probably a periportal type of Caroli disease, enlarged and diffusely increased parenchymal echogenicity of both kidneys, splenomegaly, and a moderate amount of ascites. The MRCP findings were diffuse cystic dilatation of bilateral intrahepatic bile ducts with the presence of a central dot sign at the peripheral zone and evidence of liver cirrhosis with portal hypertension. The patient underwent liver transplantation with a favorable outcome and a follow-up period of 10 years. A: Ultrasonography shows liver cirrhosis with multiple cysts along the periportal distribution, probably a periportal type of Caroli disease; B: Magnetic resonance cholangiopancreatography shows diffuse cystic dilatation of bilateral intrahepatic bile ducts; C: A 1.1 cm probable hepatic cyst at segment VI with a central dot sign.