Abstract
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.
