Betti A, Vittori O, Vezzoli G
Servizio di Radiologia I, Spedali Civili, Brescia.
Radiol Med. 1990 Jan-Feb;79(1-2):70-6.
Budd-Chiari syndrome is caused by the obstruction of the hepatic veins or of the inferior vena cava. It is characterized by the classic symptomatological triad: ascites, hepatomegaly, and abdominal pain. In 2/3 cases its etiology remains unknown. Budd-Chiari syndrome may be associated with polycythemia vera, neoplasms, chronic leukemia, congenital abnormalities, hypercoagulation conditions, pregnancy, oral contraceptives, and constrictive pericarditis. Even though its clinical diagnosis is difficult, radiology plays a decisive role with US, CT, MR imaging and, above all, angiography; the latter, together with liver biopsy, generally provides with an unquestionable diagnosis. Through the definition of stage of the disease, of level (intrahepatic, venous, caval, cardiac), of type (intrinsic or extrinsic), and degree of both obstruction and consequent development of collateral channels, radiology determines which patients should undergo a medical or a surgical treatment. In some case, percutaneous angioplasty can be performed. Four cases of Budd-Chiari syndrome, including two children, were investigated with US, CT, angiography, and liver biopsy; MR imaging was also employed in one case. The underlying cause was identified in 3 patients: constrictive pericarditis of probable congenital origin and web occlusion of the inferior vena cava near the right atrium in the 2 children; hepatic vein thrombosis due to essential thrombocythemia in the third case. In the fourth patient, thrombosis of the inferior vena cava and hepatic veins was unexplained. The diagnosis was established by means of liver biopsy and phlebography of the hepatic veins. Good diagnostic information was also supplied by non-invasive techniques, such as US, CT, and MR imaging.(ABSTRACT TRUNCATED AT 250 WORDS)
布加综合征是由肝静脉或下腔静脉阻塞引起的。其特征为典型的症状三联征:腹水、肝肿大和腹痛。三分之二的病例病因不明。布加综合征可能与真性红细胞增多症、肿瘤、慢性白血病、先天性异常、高凝状态、妊娠、口服避孕药及缩窄性心包炎有关。尽管其临床诊断困难,但放射学检查在超声、CT、磁共振成像尤其是血管造影中起决定性作用;血管造影与肝活检一起通常能提供明确的诊断。通过确定疾病的阶段、水平(肝内、静脉、腔静脉、心脏)、类型(内在或外在)以及阻塞程度和随之而来的侧支循环发展情况,放射学检查可确定哪些患者应接受药物治疗或手术治疗。在某些情况下,可进行经皮血管成形术。对4例布加综合征患者进行了研究,其中包括2名儿童,采用了超声、CT、血管造影和肝活检;1例还进行了磁共振成像检查。3例患者明确了潜在病因:2名儿童可能为先天性缩窄性心包炎以及右心房附近下腔静脉蹼状闭塞;第3例为原发性血小板增多症导致的肝静脉血栓形成。第4例患者下腔静脉和肝静脉血栓形成原因不明。通过肝活检和肝静脉静脉造影确诊。超声、CT和磁共振成像等非侵入性技术也提供了良好的诊断信息。(摘要截取自250字)