肝門部が結合織塊を呈したPaucity of Interlobular Bile Ductsの1例

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A one-month-old infant presented prolonged jaundice, hepatomegaly, and systolic murmur. Bilirubin was scarcely detected in his duodenal juice nor feces. HIDA Tc 99m did not appeared in the bowel after 2h. Endoscopic retograde cholangiopancreatography performed at 64 days of age showed the narrow common bile duct, the gall bladder, and proximal portion of the common hepatic duct but not revealed intrahepatic bile ducts. He was dignosed as biliary atresia and operated upon. Hepatic portoenterostomy was performed because repeated intraoperative cholangiograms failed to demonstrate intrahepatic bile ducts and fibrous remnants were detected at the porta hepatis. Liver biopsy revealed no interlobular bile ducts in portal tracts. He was made diagnosis of paucity of interlobular bile ducts postoperatively. After the operation bile juice did not flow out from external fistula and his jaundice had not been disappeared. Our experience suggested that hepatic portoenterostomy was not effective in a patient with paucity of interlobular bile ducts with fibrous remants at the porta hepatis. It was concluded that open liver biopsy specimens taken from both lobes should be immediately examined and diagnosed histopathologically in a patient with fibrous remants whose diagnosis of biliary atresia is doubtful.
特定非営利活動法人日本小児外科学会の論文
1986-05-20