Hemming A W, Langer B, Greig P, Taylor B R, Adams R, Heathcote E J
Department of Surgery, Toronto Hospital, University of Toronto, Ontario, Canada.
Am J Surg. 1996 Jan;171(1):176-80; discussion 180-1. doi: 10.1016/S0002-9610(99)80095-6.
Budd-Chiari syndrome is an uncommon disorder caused by obstruction to hepatic venous outflow, causing varying degrees of hepatic injury depending on the extent, severity, and acuity of the obstruction.
We reviewed the indications for operative intervention and the results of treating 32 patients with Budd-Chiari syndrome seen at Toronto Hospital between 1968 and 1995.
Twenty-one patients underwent portosystemic shunt (PSS) and 7 patients underwent liver transplantation (LT) as their initial operative management. Three patients who initially had PSS subsequently required LT. Patients with cirrhosis found on biopsy and preservation of hepatocellular function were treated with PSS and showed no difference in outcome when compared with patients without cirrhosis (P = 0.35). Patients who were treated by PSS with retrohepatic vena caval compression, as shown by high caval gradients had outcomes similar to those for patients with low gradients (P = 0.31). Using the Kaplan-Meier method, 5-year survival of PSS patients was 57%. Liver transplantation was used to manage patients with hepatic decompensation, as well as patients with vena caval occlusion or failed PSS. The 5-year Kaplan-Meier survival for LT was 67%.
Both PSS and LT are effective options in the management of Budd-Chiari syndrome. Portosystemic shunt is the preferred initial approach even with cirrhosis or retrohepatic caval compression as long as there is preservation of liver function and a patent vena cava. Liver transplantation should be used as primary therapy for patients with irreversible hepatic decompensation or vena caval occlusion, and it can be an effective salvage procedure following failed PSS.
布加综合征是一种由肝静脉流出道梗阻引起的罕见疾病，根据梗阻的程度、严重性和急性程度，会导致不同程度的肝损伤。
我们回顾了1968年至1995年间在多伦多医院就诊的32例布加综合征患者的手术干预指征及治疗结果。
21例患者接受了门体分流术（PSS），7例患者接受了肝移植（LT）作为初始手术治疗。3例最初接受PSS的患者随后需要进行LT。活检发现有肝硬化且保留肝细胞功能的患者接受了PSS治疗，与无肝硬化的患者相比，其预后无差异（P = 0.35）。经PSS治疗且肝后腔静脉受压（表现为高腔静脉梯度）的患者，其预后与低梯度患者相似（P = 0.31）。采用Kaplan-Meier法，PSS患者的5年生存率为57%。肝移植用于治疗肝失代偿患者以及腔静脉闭塞或PSS失败的患者。LT的5年Kaplan-Meier生存率为67%。
PSS和LT都是治疗布加综合征的有效选择。只要肝功能保留且腔静脉通畅，即使存在肝硬化或肝后腔静脉受压，门体分流术仍是首选的初始治疗方法。肝移植应作为不可逆肝失代偿或腔静脉闭塞患者的主要治疗方法，并且在PSS失败后可作为一种有效的挽救措施。
