Klein A S, Cameron J L
Department of Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Am J Surg. 1990 Jul;160(1):128-33. doi: 10.1016/s0002-9610(05)80882-7.
Occlusion or obstruction of hepatic venous outflow results in the Budd-Chiari syndrome. The disorder should be suspected in any patient who suddenly develops massive ascites, and the diagnosis can be confirmed quickly and accurately by hepatic venography. In the absence of surgical intervention, survival is rare. Inferior venacavography and percutaneous liver biopsy can be performed safely in these patients, and both procedures provide useful information for the selection of appropriate surgical therapy. Most cases of the Budd-Chiari syndrome are amenable to mesocaval or mesoatrial shunting. Those patients with documented cirrhosis or fulminant hepatic failure are best managed by orthotopic liver transplantation.
肝静脉流出道的闭塞或阻塞会导致布加综合征。任何突然出现大量腹水的患者都应怀疑患有这种疾病,通过肝静脉造影可以快速准确地确诊。若不进行手术干预,存活几率很小。在这些患者中可以安全地进行下腔静脉造影和经皮肝活检,这两种检查都能为选择合适的手术治疗提供有用信息。大多数布加综合征病例适合进行肠系膜上静脉-下腔静脉或肠系膜上静脉-心房分流术。那些有肝硬化或暴发性肝衰竭记录的患者最好通过原位肝移植进行治疗。