【Abstract】ObjectiveTo report the author’s experience with the first case of an adult-to-adult living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) using cryopreserved vena cava graft in postheptic vena cava reconstruction. MethodsA 35-year-old male patient with a diagnosis of BCS complicated with inferior vena cava (IVC) obstruction received medical treatment and radiologic intervention for nine months, no relief of the symptoms could be achieved. Finally, the patient underwent LDLT, which required posthepatic vena cava reconstructed using cryopreserved vena cava graft. ResultsThe patient has had an uneventful course since the LDLT. ConclusionWe believe that LDLT combined with posthepatic IVC reconstruction using cryopreserved vena cava graft is considered to be a sound modality for IVC obstructed BCS.
ObjectiveTo investigate the basic operation and treatment experiences of the surgical treatment of Budd-Chiari syndrome (BCS). MethodsClinical data of 1 024 cases of BCS who received surgical treatment in our hospital from April 1994 to December 2013 were collected and analyzed. ResultsThere were 1 024 cases in our study, 116 cases of them underwent surgery, 908 cases of them underwent interventional surgery; 265 cases underwent inferior vena cava (IVC) balloon dilatation, 464 cases underwent IVC balloon dilatation and stenting, 97 cases underwent open surgery of hepatic vein (HV), 52 cases underwent right atrium femoral vein combined membrane rupture balloon dilation stent, 7 cases underwent caval shunt, 20 cases underwent radical surgery, 45 cases underwent IVC-right atrium bypass, 6 cases underwent intestinal cavity-real shunt, 9 cases underwent intestinal cavity-neck combined shunt, 30 cases underwent transjugular intrahepatic portosystemic shunt, 29 cases underwent intestinal-line real shunt. There were 902 cases were followed-up for 1 day-19 years (13 years on average), and the application of many kinds of operation strictly and flexibly brought satisfactory results for cases of BCS. ConclusionsThe diagnosis and classification of BCS will help us to make safe, effective, and appropriate treatment plan. In addition, we must use color Doppler ultrasound to observe the pathological changes of the situation, in this way we can have a clear goal in the treatment process.
ObjectiveTo summarize clinical applications of inferior right hepatic vein (IRHV) in liver surgery and to provide a basis for clinical applications of IRHV.MethodThe relevant literatures about clinical applications of IRHV in liver surgery in recent years were reviewed.ResultsAs a kind of short hepatic veins, the IRHV directly flowed into the inferior vena cava, often accompanied by the portal vein of the segment Ⅵ. The occurrence rate of IRHV was 80%–90% by the autopsy examination, while which was 10%–30% by the imaging examination. The caliber of IRHV was 0.22–0.95 cm, and its caliber was negatively correlated with the caliber of right hepatic vein. The IRHV played a great role in the classification and treatment of the Budd-Chiari syndrome. According to the Couinaud liver classification method, the IRHV mainly drained the blood of segment Ⅵ. The existence of IRHV expanded the indications of hepatectomy. The reconstruction of IRHV in the liver transplantation could not only reserve the function of donor liver, but could compensatively drain the corresponding liver areas if the acute occlusion of other major hepatic veins happened.ConclusionsIRHV has some important clinical significances in liver surgery. Fully studying course characters and adjacent relationship of IRHV can not only avoid injury during surgery, but also provide a new treatment idea for related liver diseases.
Objective To analyze the prognosis and indications of transjugular intrahepatic portosystemic shunt (TIPS) in patients with Budd-Chiari syndrome (BCS). MethodsPatients with primary BCS who received TIPS in the Department of Gastroenterology, West China Hospital of Sichuan University between February 2009 and February 2020 were retrospectively reviewed. The medical history, preoperative imaging, surgical records, and postoperative outpatient follow-up medical records were recorded. The laboratory indexes before and after operation were compared, and the cumulative free from hepatic encephalopathy rate, stent patency rate, and cumulative survive rate were calculated. Cox proportional hazards model was used to analyze the independent risk factors of hepatic encephalopathy, shunt dysfunction and death. Results A total of 48 patients were included. The main indications for TIPS included variceal bleeding (16 cases), refractory ascites (24 cases), and diffuse obstruction of hepatic vein with acute liver function impairment (8 cases). The cumulative 1 year, 2 years and 3 years of free from hepatic encephalopathy rates were 92.3%, 89.2% and 85.3%, respectively. The stent patency rates were 89.7%, 72.2% and 54.8% at postoperative 1 year, 3 years and 5 years, respectively. The cumulative survival rates were 86.0%, 79.5% and 71.4% at postoperative 1 year, 3 years and 5 years, respectively. Conclusion TIPS can achieve good efficacy in patients with BCS, and most patients receive TIPS for portal hypertension complications rather than acute liver function impairment.
ObjectiveTo evaluate the applicability and the long-term outcomes of percutaneous transluminal balloon angioplasty (PTBA) in the management of Budd-Chiari syndrome (BCS) secondary to hepatic venous obstruction. MethodsClinical data of 94 patients with BCS secondary to hepatic venous obstruction who underwent PTBA of the hepatic vein from Jan. 2005 to Dec. 2013 in The First Affiliated Hospital of Zhengzhou University were analyzed retrospectively. ResultsPTBA were technically successful in 93 of the 94 patients (98.94%). Ninety-one of the 93 patients (97.85%) were treated with PTBA alone and 2 patients (2.15%) were treated with PTBA and stent. One patient with primary hepatocellular carcinoma (HCC) underwent resection of liver cancer after interventional therapy. Hepatic venous pressure value of 93 patients was significantly decreased after balloon interventional procedures. Symptoms were significantly improved in the 93 patients who had successful PTBA. Procedure-related complications occurred in 6 of the 93 patients (6.38%), and 1 patient (1.06%) died in 2 months after operation because of intra-abdominal bleeding. Two patients lost during follow-up with a follow-up loss rate of 2.15% (2/93), and the 91 patients were followed-up for 1-96 months [(49.72±28.60) months]. HCC occurred in 3 patients during follow-up period. Restenosis of targeted hepatic vein developed in 8 patients (11 times), and the overall recurrence rate was 11.83% (11/93). One patient of them underwent surgical operation, the remaining 7 patients underwent PTBA successfully. The 1-, 2-, 3-, and 5-year primary patency rates were 97.47% (77/79), 94.20% (65/69), 91.67% (55/60), and 91.67% (33/36), respectively. The 1-, 2-, 3-, and 5-year secondary patency rates were 98.73% (78/79), 98.55% (68/69), 98.33% (59/60), and 97.22% (35/36), respectively. ConclusionsPTBA is a safe and effective treatment for BCS with the hepatic vein obstruction and had good mild-term outcomes. The liver function of the patients improved after treatment, with few patients died from HCC caused by hepatic cirrhosis, so we must pay attention on it, as well as the targeted hepatic vein.
目的 探讨布加综合征肠房转流术后顽固性腹水的治疗。方法 对2008年收治的1例经多次治疗(包括肠房转流术)后均于短期内复发的布加综合征患者进行回顾性分析。结果 临床表现为重度腹水致呼吸困难，CT静脉造影检查示肠房人工血管通畅但血流量低，考虑吻合口狭窄所致。术中探查发现吻合口极度狭窄，用带外支撑环的补片重建吻合口，疗效满意。结论 复杂或经多次手术或介入治疗的布加综合征患者，要遵循个体化治疗原则，强调术前明确诊断及选择正确治疗方案和手术方式。
Hepatic sinusoidal obstruction syndrome (HSOS) can be easily missed or misdiagnosed as Budd-Chiari syndrome in clinical practice. The authors displayed the imaging pictures of one patient with HSOS and made a brief description of typical imaging features, underlying pathophysiological mechanisms, and differential diagnosis of HSOS, with the hope of improving the understanding of HSOS and reducing the rates of leak diagnosis or misdiagnosis.