Henderson J M, Warren W D, Millikan W J, Galloway J R, Kawasaki S, Stahl R L, Hertzler G
Department of Surgery, Emory University School of Medicine, Atlanta, Georgia.
Am J Surg. 1990 Jan;159(1):41-8; discussion 48-50. doi: 10.1016/s0002-9610(05)80605-1.
This article presents a scheme of management for Budd-Chiari syndrome based on experience with 33 patients. Therapy in acute Budd-Chiari syndrome is dictated by the liver biopsy, with hepatocyte necrosis indicating the need for placement of a decompressive shunt. The type of shunt was determined by intrahepatic vena cava obstruction; a higher morbidity rate was associated with the mesoatrial shunt in 11 patients than with a portacaval shunt in 10 patients. Successful shunt placement allowed stabilization of the liver biopsy and maintenance of good hepatocyte function [galactose elimination capacity (preoperative: 349 +/- 40 mg/minute; 20 months: 344 +/- 60 mg/minute)]. Severe fibrosis and reduced galactose elimination capacity (264 +/- 43 mg/minute) indicated advanced disease--chronic Budd-Chiari syndrome--and were indications for liver transplant. Hematologic evaluation documented a myeloproliferative disorder in 8 of the last 13 patients evaluated; perioperative and late anticoagulation and/or chemotherapy reduced recurrent thrombosis. We conclude that the Budd-Chiari syndrome requires different therapies depending on the stage of disease. If no hepatocyte injury is present on biopsy, therapy may not be needed. Acute, reversible injury can be managed by placement of a decompressive shunt. Irreversible damage requires transplantation. Selection of the right therapy requires a complete evaluation.
本文基于对33例布-加综合征患者的治疗经验，提出了一套治疗方案。急性布-加综合征的治疗方案取决于肝活检结果，若存在肝细胞坏死，则需要进行减压分流术。分流类型由肝内静脉阻塞情况决定；11例行肠系膜-心房分流术患者的发病率高于10例行门腔分流术患者。成功的分流术可使肝活检结果趋于稳定，并维持良好的肝细胞功能[半乳糖清除能力（术前：349±40mg/分钟；20个月：344±60mg/分钟）]。严重纤维化和半乳糖清除能力降低（264±43mg/分钟）提示疾病进展至晚期——慢性布-加综合征，这是肝移植的指征。血液学评估显示，在最后评估的13例患者中有8例存在骨髓增殖性疾病；围手术期及后期抗凝和/或化疗可减少复发性血栓形成。我们得出结论，布-加综合征需要根据疾病阶段采取不同的治疗方法。如果活检未发现肝细胞损伤，则可能无需治疗。急性可逆性损伤可通过减压分流术进行治疗。不可逆性损伤则需要进行肝移植。选择正确的治疗方法需要进行全面评估。
