A Case of Intrahepatic Cholangiocarcinoma 35 Years After Operation for Biliary Atresia

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  • Endo Kosuke
    Department of General Surgery, Kyoto City Hospital
  • Sato Masahito
    Department of Pediatric Surgery, Kitano Hospital, The Tazuke Kofukai Medical Research Institute
  • Higashio Atsushi
    Department of Pediatric Surgery, Kitano Hospital, The Tazuke Kofukai Medical Research Institute
  • Iimori Kei
    Department of Gastroenterology and Hepatology, Kyoto-Katsura Hospital
  • Sawada Kenji
    Department of Gastroenterology and Hepatology, Japanese Red Cross Osaka Hospital
  • Azuma Shunjiro
    Department of Gastroenterology and Hepatology, Kitano Hospital, The Tazuke Kofukai Medical Research Institute
  • Kurita Akira
    Department of Gastroenterology and Hepatology, Rakuwakai Otowa Hospital
  • Morotomi Yoshiki
    Department of Pediatric Surgery, Kitano Hospital, The Tazuke Kofukai Medical Research Institute

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Other Title
  • 胆道閉鎖症術後35年目に胆管癌が判明した1例
  • タンドウ ヘイサショウ ジュツゴ 35ネンメ ニ タンカン ガン ガ ハンメイ シタ 1レイ

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<p>The patient was a 35-year-old male who underwent Kasai operation (reconstructed by hepatic portoduodenostomy interposed with 20 cm of backflow prevention valve-added jejunum) for biliary atresia (BA) 69 days after birth. His postoperative course was fairly good, but he self-interrupted his outpatient visits at the age of 22. When he was 34 years old, he was referred to the Department of Gastroenterology at our hospital because of jaundice. Abdominal CT scans showed intrahepatic bile duct dilatation, and ERC was attempted with the diagnosis of anastomotic stenosis, but the anastomotic site could not be identified. Owing to the worsening of his jaundice, he was referred to another hospital for liver transplantation. PTBD was successfully carried out at the referral hospital and his jaundice improved. His PTBD tube was managed at our hospital, but he repeatedly had fever and liver dysfunction. He later complained of vomiting, so we performed an upper gastrointestinal endoscopy, which revealed a tumor in the duodenum. CT scans showed multiple liver tumors and pleural/ascitic effusions. He was diagnosed as having intrahepatic cholangiocarcinoma by cytological analysis of bile, pleural fluid and ascites and by EUS-guided needle biopsy. He received chemotherapy, but his response was poor. He passed away 10 months after his second visit at the age of 34. The prognosis of intrahepatic cholangiocarcinoma after surgery for BA is poor. In the follow up of long-term survivors with autologous liver, the possibility of carcinogenesis should be considered.</p>

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