Klein A S, Sitzmann J V, Coleman J, Herlong F H, Cameron J L
Department of Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Ann Surg. 1990 Aug;212(2):144-9. doi: 10.1097/00000658-199008000-00005.
Twenty-six patients with the Budd-Chiari syndrome were treated surgically at the Johns Hopkins Hospital. Twenty-one of the patients were female and five were male, with a median age at diagnosis of 37 years. Nine patients had polycythemia vera, 6 were receiving estrogen therapy, 5 had a previous hepatitis A or B infection, and 4 had cirrhosis. There was one case each of hepatic malignancy, paroxysmal nocturnal hemoglobinuria, and idiopathic thrombocytopenic purpura. In five cases no etiologic factors or associated disorders were identified. Ascites was the most common presenting feature in this group of patients. Hepatic function at the time of diagnosis, as measured by standard serum chemistries, was only minimally abnormal. The diagnosis of the Budd-Chiari syndrome was confirmed in all 26 patients by hepatic vein catheterization. Inferior vena cavography was also performed and revealed caval occlusion in 4 patients, significant caval obstruction in 13 patients, and a normal vena cava in 9 patients. Interpretation of the vena cavogram was helpful in selecting the appropriate surgical procedure for each patient. Twenty-three of the twenty-six patients underwent percutaneous liver biopsy before operation, with no morbidity or mortality. Four patients had well-established cirrhosis noted on biopsy. Thirty mesenteric-systemic venous shunts were performed on the 26 patients. In 11 patients a mesocaval shunt was performed and in one instance conversion to a mesoatrial shunt was required as a second procedure. In 15 patients a mesoatrial shunt was performed as the initial procedure. Graft thrombosis occurring in 2 of these 15 patients prompted one revision in 1 patient and 2 revisions in the second patient. After mesenteric-systemic venous shunt, eight of the patients (31%) died before discharge from the hospital. The remaining 18 patients in this series were discharged from the hospital alive and well with patent shunts. Patients were followed for a median of 43 months (range, 9 months to 13 years). Five late deaths occurred between 5 and 84 months after the operation. Three- and five-year actuarial survival rates were 65% and 59%, respectively.
26例布加综合征患者在约翰霍普金斯医院接受了手术治疗。其中21例为女性，5例为男性，诊断时的中位年龄为37岁。9例患者患有真性红细胞增多症，6例正在接受雌激素治疗，5例既往有甲型或乙型肝炎感染史，4例患有肝硬化。各有1例肝恶性肿瘤、阵发性夜间血红蛋白尿和特发性血小板减少性紫癜。5例未发现病因或相关疾病。腹水是该组患者最常见的临床表现。根据标准血清化学指标测定，诊断时的肝功能仅轻度异常。所有26例患者均经肝静脉插管确诊为布加综合征。还进行了下腔静脉造影，结果显示4例患者存在腔静脉闭塞，13例患者存在明显的腔静脉梗阻，9例患者腔静脉正常。腔静脉造影的结果有助于为每位患者选择合适的手术方式。26例患者中有23例在术前接受了经皮肝活检，无并发症或死亡发生。4例患者活检显示有明确的肝硬化。对26例患者进行了30次肠系膜-体静脉分流术。11例患者进行了肠系膜-腔静脉分流术，其中1例在第二次手术时需要转换为肠系膜-心房分流术。15例患者首次手术时进行了肠系膜-心房分流术。这15例患者中有2例发生移植血管血栓形成，其中1例进行了1次修复，另1例进行了2次修复。肠系膜-体静脉分流术后，8例患者（31%）在出院前死亡。该系列中其余18例患者出院时存活且分流血管通畅。对患者进行了中位时间为43个月（范围为9个月至13年）的随访。术后5至84个月发生了5例晚期死亡。3年和5年的精算生存率分别为65%和59%。
