A Rare Anomaly of the Biliary Tree in an Infant : Biliary Atresia or Congenital Biliary Dilatation?

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  • 先天性胆道拡張症と胆道閉鎖症(I cyst)との鑑別が困難な胆道奇形の1例
  • 症例報告 先天性胆道拡張症と胆道閉鎖症(Ⅰ cyst)との鑑別が困難な胆道奇形の1例
  • ショウレイ ホウコク センテンセイタンドウ カクチョウショウ ト タンドウ ヘイサショウ(Ⅰ cyst)ト ノ カンベツ ガ コンナン ナ タンドウ キケイ ノ 1レイ

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According to the Kasai classification, I cyst type biliary atresia (BA) is defined as that with hepatic hilum cyst and patent hepatic duct. Herein, we report an infant case of anomaly of the biliary tree which resembled I cyst type BA but with a patent common bile duct. A 2-month-old girl with jaundice and acholic stool was found to have a hepatic hilum cyst by ultrasound. She underwent an operation at 80 days of age with suspicion of BA. The first intraoperative cholangiography showed a hepatic hilum cyst with fine intrahepatic ducts which suggested I cyst type BA. However, the patent common bile duct from cyst to duodenum was demonstrated by operative finding and a second cholangiography. As a correctable hepatic duct did not exist, hepatic portoenterostomy was performed. Her jaundice was cured within 1 month after the operation and her postoperative course was uneventful without jaundice as of 13 months after operation. According to operative findings, cholangiography, and liver biopsy, we diagnosed this case as BA rather than congenital biliary dilatation. As this case did not fit with the definition of either I cyst type nor type III BA, we considered the case as unclassified type. Further study and an understanding on the classification of BA is necessary.

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