Lang H, Oldhafer K J, Weimann A, Gebel M, Wagner S, Böker K, Prokop M, Reimer P, Ringe B, Pichlmayr R
Klinik für Abdominal- und Transplantationschirurgie, West fälische Wilhelms-Universität Münster.
Bildgebung. 1994 Sep;61(3):173-81.
We report on the clinical presentation and diagnostic findings in 45 patients with Budd-Chiari syndrome. The diagnosis was confirmed by histology at the time of liver transplantation (n = 37) or shunt surgery (n = 8). An underlying disorder could be established only in half of the patients, oral contraceptives as predisposing factor were known in 18 cases. Clinically, abdominal pain and distension as well as hepatomegaly and ascites were most frequent findings, whereas changes in laboratory data were more or less unspecific. By use of repeated ultrasound, a definite diagnosis of a Budd-Chiari syndrome could be confirmed in all cases by obligatory demonstration of obstruction or thrombosis of at least one major liver vein. Hepatic venography revealed only one false-negative result. Celio-mesenteric arteriography plus portography, cavography and preoperative liver biopsy did not present additional diagnostic information. These techniques may contribute to treatment planning of portosystemic shunt surgery or hepatic transplantation. In conclusion, the presence of hepatosplenomegaly, ascites, abdominal pain and distension, especially in combination with a known hypercoagulable state, should alert to the possibility of a Budd-Chiari syndrome. Ultrasound is the diagnostic tool of choice. Hepatic venography should only be performed if even repeated ultrasound is not conclusive.
我们报告了45例布加综合征患者的临床表现及诊断结果。37例患者在肝移植时经组织学确诊，8例患者在分流手术时经组织学确诊。仅半数患者能明确潜在病因，已知18例患者口服避孕药为诱发因素。临床上，腹痛、腹胀以及肝肿大和腹水是最常见的表现，而实验室数据变化或多或少缺乏特异性。通过反复超声检查，所有病例均通过至少一条主要肝静脉阻塞或血栓形成的必要证据确诊为布加综合征。肝静脉造影仅出现1例假阴性结果。肠系膜上动脉造影加门静脉造影、腔静脉造影及术前肝活检未提供额外的诊断信息。这些技术可能有助于门体分流手术或肝移植的治疗规划。总之，肝脾肿大、腹水、腹痛和腹胀的存在，尤其是与已知的高凝状态相结合时，应警惕布加综合征的可能性。超声是首选的诊断工具。仅在反复超声检查仍无法确诊时才应进行肝静脉造影。
