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Objective To summarize our experience in the diagnosis and treatment of hepatic epithelioid angiomyolipoma (HEAML),with the aim to reduce the future misdiagnosis rate.Methods The PubMed,Medline,China Science Periodical Database (CSPD),and VIP Databases were searched from January 2000 to March 2018 on all reports on HEAML.Results There were 409 cases of HEAML in 97 reports.The ratio of men to women was 1∶4.84.The age ranged from 12 to 80 years and the median age was 44 years.61.9％ of patients (205/331) were asymptomatic,while 34.7％ (115/331) had upper or right upper quadrant abdominal discomfort.Some patients presented with abdominal mass,gastrointestinal reaction,low grade fever or weight loss.The clinical symptoms in 78 patients were not mentioned in the reports.The misdiagnostic rate of HEAML was as high as 40.3％ (165/409).The imaging findings of HEAML were nonspecific.Ultrasound,CT and MRI scan usually showed contrast enhancement in the arterial phase.Most lesions were accompanied by central vessels with early drainage veins.The enhanced scans showed varied characteristics.The ratios of fast wash-in and fast wash-out,to fast wash-in and slow wash-out,and to delayed enhancement were roughly 4∶ 5∶ 1.A definitive diagnosis of HEAML is based on the pathological findings of epithelioid cells in the lesions and the expressions of HMB45,SMA,Melan-A and Actin on immunohistochemical staining.HEAML had a relatively low malignant rate of 3.9％.Surgical resection was the main treatment for HEAML.Conclusion HEAML was a rare and easily misdiagnosed disease.,which could be diagnosed by taking into account the clinical course,imaging,pathological and immunohistochemical findings.HEAML.
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Objective To investigate some improvements in the surgical techniques of adult-to-adult living donor liver transplantation( A-A LDLT) without the middle hepatic vein(MHV) for hepat-ic vein reconstruction. Methods The retrospective analysis was made on the clinical data of 11 recipi-ents who underwent the operation in A-A LDLT including the hepatic vein reconstructed in right liver lobe without MHV from June 2007 to January 2008. The key techniques included reconstructing out-flow of graft on shaping the tips of vena cava and right hepatic veins, cadaveric vein allografts stored in 4℃ UW solution within 7d being used for significant-sized hepatic vein reconstruction such as tributa-ries of the middle hepatic vein from V5, V8 and right inferior hepatic vein. Results 10 cases success-fully underwent reconstruction of outflow of graft on shaping the tips of vena cava and right hepatic veins and the outflow reconstruction ratio of V5, V8 and right inferior hepatic vein was 81. 8% (9/11), 7 one-vein reconstruction, 1 two-vein reconstruction and 1 three-vein reconstruction. 1 recipient died of renal failure and pulmonary infection 14 days after operation without venous outflow obstruc-tion. Doppler ultrasonography showed no thrombosis and the blood flowed smoothly in the right he-patic vein of other 8 recipients during the 9th to 15th mouth of follow-up. The cumulative patency rates of these 8 survivals for interposition vein grafts were 100% (11/11), 72. 7 %(8/11), 54. 5%(6/11) and 36. 5%(4/11) in 1, 3, 6 and 9 mouths, respectively. The regeneration of paramedian sectors was equivalent. Conclusion Shaping the tips of vena cava and right hepatic veins and using cadaveric vein allografts in adult-to-adult right lobe living donor liver transplantation for hepatic vein reconstruc-tion are both safe,simple and effective methods.This approach can be recommended.
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Objective To investigate the surgical options for the management of portal vein thrombosis (PVT) during liver transplantation and its impact on the outcome of patients. Methods 773 cases of liver transplantation were analyzed retrospectively. PVT occurred in 107 patients, inclu-ding 59 of grade Ⅰ ,33 of grade Ⅱ, 12 of grade Ⅲ and 3 of grade Ⅳ. Simple thrombectomy or thrombus-extraction was performed in grade Ⅰ and Ⅱ. 12 patients with grade Ⅲ received thrombus-extraction or using the donor iliac vein to act as a bridge between the donor portal vein and host superior mesenteric vein. Two cases of grade Ⅳ received a modified cavo-portal hemitransposition and one case received portal-vena coronaria varication anastomosis. Results Liver function had a good recover and the perio-perative mortality is 4. 3% in grade Ⅰ and Ⅱ. In grade Ⅲ , 5 cases received thrombus-extraction had a normal liver function after transplantation and had no died. 2 cases among the other 7 cases using por-tal vein reconstruction had bad liver function and died. The liver function recovered well after trans-plantation and there was no died in grade Ⅳ. Conclusions PVT is not a contraindication for liver transplantation. Good results can be obtained by applying reasonable operative procedures individually.
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Objective To summarize the clinical experience in liver retransplantation. Methods The clinical data of 24 patients receiving liver retransplantation 28 times in this hospital were retrospectively analyzed and discussed with relevant literature. Results Among the 880 consecutive liver transplantations, 28(3.18%) had liver retransplantation. The causes of liver retransplantation were biliary complications ( 16 cases, 57. 1%), carcinoma recurrece (6 cases, 21. 4%), hepatic artery thrombosis (4 cases, 14. 3%), chronic rejection (1 case, 3. 6%), primary nonfunction (1 case, 3.6%). Thirteen patients among the 24 were discharged healthy and were followed up for 51days to 67months. Eleven patients died. Three of them died of hemorrhagic shock, 2 of septic shock, 2 of hepatocellular carcinoma recurrence, 2 of cardiovascular system complication, 1of nervous system complication, and 1 of hepatic artery thrombosis. Conclusion Liver retransplantation can effectively save patients with graft failure. Proper indication, optimal operating time, improvement of operative skills,and appropriate treatment during the perioperative period are very important for promoting the rate of successful liver retransplantation.
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Objective To evaluate the therapeutic effects of liver transplantation (LT) for cholangiocarcinoma(CC)and analyze the prognostic factors.Methods From December 2001 to December 2006,234 patients receiving LT for hepatic carcinoma in our institute were enrolled as a basis of comparative study for 6 CC patients undergoing LT during the same period.Results These 6 patients were followed-up from 1 to 56 months.Five patients died and one recurred.The 0.5-,1-and 2-year patient cumulative survival rates were 4/6,3/6 and 1/6,respectively.The 0.5-,1-and 2-year tumor-free survival rates were 3/6,2/6 and 1/6,respectively.The average patient or tumor-free survival time were both(14±4) months.Conclusion The prognosis of cholangioearcinoma patients after LT iS poor.
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The clinical data of 67 patients underwent orthotopic liver transplantation, including 4 cases of early intra abdominal hemorrhage after orthotopic liver transplantation, were analyzed retrospectively.Anastomotic liver artery hemorrhage was found in 2 cases, hemorrhage of IVC in 1 case and hemorrhage of right adrenal gland in 1 case. All the 4 patients were correctly dignosed and successfully treated by operation in time. It suggests that the early intra abdominal hemorrhage after orthotopic liver transplantation is mostly due to surgical technique. The improvement of surgical technique,correct diagnosis and timely operation are very important for the treatment of intra abdominal hemorrhage.