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肝門部に嚢胞を形成し,肝内胆管の開存がある胆道閉鎖症は葛西分類ではI cystとして分類される.I cystは肝門部に嚢胞状胆管拡張があり,閉塞部位は必ず総胆管で,十二指腸との間に交通がないと明記されている.今回我々は,最初はI cystの胆道閉鎖症と考えられたが十二指腸への交通も認め,診断・分類に苦慮した胆道奇形の1症例を経験したので報告する.症例は2か月女児.日齢77に黄疸,白色便を主訴に受診し,胆道閉鎖症の疑いで日齢80に手術を施行した.術中胆道造影では肝管は嚢胞状に拡張し,I cystの胆道閉鎖症と思われたが,総胆管は開存し十二指腸内への造影剤の流出も確認された.術中造影や病理所見から我々は本症例を胆道閉鎖症と考えたが,I cyst,III型のどちらにも当てはまらず病型分類不能であった.現行の葛西分類において当てはまらない症例に対して,今後の分類方法について議論していく必要があると思われた.
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.