Pezzuoli G, Spina G P, Opocher E, Galeotti F, Zannini P
Surgery. 1985 Aug;98(2):319-23.
We have performed a side-to-side portacaval shunt as the treatment of choice in six cases of primary Budd-Chiari syndrome. All the patients (three men and three women, mean age 33 years) had marked hepatomegaly and massive ascites. Four patients had endoscopically proved esophageal varices with no episodes of bleeding. Preoperative angiographic studies, caval pressure measurement, laparoscopy, liver biopsy specimens, liver scanning, and liver function tests confirmed the diagnosis. A possible etiologic factor was evident in only two cases. A plain side-to-side portacaval shunt was performed in four patients, while two required the interposition of a graft. One patient died after surgery of hepatorenal syndrome. Four of the surviving patients are free of ascites and doing well at 29, 27, 25, and 6 months. The remaining patient subsequently developed cirrhosis and died 76 months after surgery. None of the patients who survived developed encephalopathy. Shunt patency was confirmed endoscopically by variceal decompression in the four patients with esophageal varices. We believe the side-to-side portacaval shunt is a reliable and effective procedure for the definitive management of primary budd-Chiari syndrome.
我们已对6例原发性布加综合征患者实施了端侧门腔分流术，并将其作为首选治疗方法。所有患者（3男3女，平均年龄33岁）均有明显肝肿大和大量腹水。4例患者经内镜证实有食管静脉曲张，但无出血发作。术前血管造影研究、腔静脉压力测量、腹腔镜检查、肝活检标本、肝脏扫描和肝功能检查均确诊了该疾病。仅2例患者存在可能的病因。4例患者实施了单纯的端侧门腔分流术，而2例患者需要植入移植物。1例患者术后死于肝肾综合征。4例存活患者无腹水，分别在术后29、27、25和6个月时情况良好。其余1例患者随后发展为肝硬化，并在术后76个月死亡。存活患者均未发生脑病。4例有食管静脉曲张的患者经内镜检查通过静脉曲张减压证实分流通畅。我们认为端侧门腔分流术是确定性治疗原发性布加综合征的一种可靠且有效的方法。
