Case 1
A 45-years-old man was admitted for fever of 3 days duration and was diagnosed with acute cholangitis, biliary cirrhosis. He has congenital cystic dilatation of the intrahepatic bile duct but has not been examined and treated. 30 years ago, he underwent splenectomy and venous devascularization due to upper gastrointestinal bleeding caused by portal hypertension-related liver cirrhosis. In the past 20 years, he had repeated gastrointestinal bleedings and underwent endoscopic varicose ligation combined with embolization for hemostasis. In the past 1 year, the patient has repeatedly had fever with chills, abdominal pain, diarrhea, jaundice, and other symptoms. He denied smoking and alcohol abuse. Physical examination was essentially negative except for body temperature as high as 38.7℃. Lab-examination showed: (white blood cell [WBC] = 15.7*109 /L,NE%= 77.8%༌hemoglobin [HB] = 122g/L, platelet [PLT] = 207*106/L; albumin [ALB] = 27.4g/L, alanine aminotransferase [ALT] = 112U/L, aspartate aminotransferase [AST] = 80U/L, total bilirubin [TB] 30.3umol/L, direct bilirubin [DB] = 10.2umol/L; blood ammonia = 106umol/L; prothrombin time [PT] = 17.4s, prothrombin time activity [PTA] = 76.2%, international normalized ratio [INR] = 1.16, activated partial thromboplastin time [APTT] = 30.0s; CA19-9 150.20U/L, CA12-5 174.50U/L, hepatitis related tests are negative. Abdominal enhanced CT indicated multiple dilation of intrahepatic bile duct, which is consistent with the manifestations of Caroli disease (Fig. 1A, B), and calcifications in the portal and superior mesenteric were detected (Fig. 1C, D), liver cirrhosis with portal hypertension. His liver function was Child-Pugh A grade and MELD 6 points. Based on comprehensive considerations, the patient met the indications for liver transplantation, and was reviewed and approved by the hospital ethics committee to undergo the allogeneic modified piggyback liver transplantation on January 23, 2021. The surgical procedure was complicated and took 25 hours, including 7 hours in the anhepatic phase. The cold ischemia-time of the donor liver was 16.5 hours. After a difficult hepatectomy, the portal vein was found to be calcified and thrombosed. Attempted at direct intraluminal dissection and thrombectomy failed to reestablished patency. Finally, the portal vein stent was placed, and portal flow was reestablished. Intraoperative blood loss is estimated to be 16000ml, 6500ml autologous blood transfusion, 3600ml suspended red blood cells, and 2400ml fresh frozen plasma. Postoperative ultrasound of the transplanted liver vessels showed that the inner diameter of the portal vein was 1.0cm and the flow rate was 18.0cm/s. With long operation time, excessive bleeding, long cold ischemia time of the donor liver, and poor portal vein condition, the patient died of transplanted liver failure, kidney failure, and heart failure 2 days after surgery.
Case 2
A 50-years-old man was diagnosed as having liver cirrhosis twenty years prior to his present admission. In the past 10 years, he had repeated gastrointestinal bleedings and underwent endoscopic varicose ligation. The patient developed refractory ascites 2 years ago and hepatic encephalopathy occurred 1 year ago. On admission, the patient was complained of refractory ascites, and a physical examination revealed remarkable abdominal distension and positive shifting dullness. He had a long-term history of heavy alcohol abuse. Lab-examination showed abnormal liver function and pertinent data were as follows: WBC 3.05*109/L, HB 113g/L(mild anemia), PLT 139*109/L; ALB 28g/L(hypoproteinemia), AST 137U/L, ALT 56U/L, TB 68.8umol/L, DB 43.7umol/L; PT 14.6s, INR 1.26, APTT 25.4s; blood ammonia: 112umol/L (hyperammonemia); Tumor marker: CA19-9 248.78U/L, CA12-5 412.50U/L; Abdominal imaging examination revealed liver cirrhosis with portal hypertension (Fig. 2A), the presence of calcifications of full-length in the portal vein and superior mesenteric vein (Fig. 2B, C, D). His liver function was Child-Pugh C grade and MELD 24 points, was in end-stage liver disease. Based on comprehensive considerations, the patient met the indications for liver transplantation, and was reviewed and approved by the hospital ethics committee to undergo the allogeneic modified piggyback liver transplantation, but the patient and his family refused to be treated by surgery and decided to be discharged.