Objective To evaluate therapeutical effect of combined heptic resection and fenestration and liver transplantation for severe adult polycystic liver disease(SAPLD).Methods Preoperative clinical symptoms,postoperative complications and progno-sises from 12 patients with SAPLD treated with combined hepatic resection and fenestration between 2003 and 2023 were recorded.And in same period data of 5 patients with SAPLD who underwent orthotopic liver transplantation was retrospectively analyzed.Demograph-ic,clinical and follow-up data were collected for analysis.Results In combined heptic resection and fenestration group.The mean follow-up time was 105 months.There were four patients with recurrence of symptoms at 102,45,39 and 30 months after operation.One patient died of liver failure due to cystadenoma of liver at 66 months after first operation.And one patient with postoperative hepat-ic in adequacy recieved an orthotopic liver transplantation.The total number of patients with postoperative complications was 6 cases,including one patient with bleeding,one patients with bile leakage,two patients with severe ascites and two patients with pleural effu-sion.and the overall incidence was 50％.In liver transplantation group,over a period of 15 years,three patients received orthopedic liver transplantation for SAPLD.The recipients'median age was 55 years.All patients were classified as Gigot type Ⅲ SAPLD,and all cases were Child-Pugh Class C patients and the model for end-stage liver disease(MELD)score was＞20.The median anhepatic phase and operative blood loss were 40 minutes(range,35-55 minutes)and 1200ml(range,600-12000 ml),respectively.The median hospitalization duration was 21 days(range,0-31 days),and the median length of stay in intensive care unit was 3 days(range,0-7 days).One patients died of severe bleeding during transplantation.The median follow-up time was 63 months(range,0-178 months).One patient survived for more than 15 years,one patients survived for more than 5 years.No acute rejection of grafts was observed at the end of the follow up.Conclusions Combined hepatic resection and fenestration is a safe and acceptable procedure for the treatment of SAPLD.And Liver transplantation is the only curative and safe procedure for SAPLD,and it provides a good long-term prognosis and high quality of life for SAPLD patients.
关键词
成人严重多囊肝病/肝切除/开窗术/肝移植/预后
Key words
severe adult polycystic liver disease/hepatectomy/fenestration/liver transplantation/prognosis
维普
万方数据