Ringe B, Lang H, Oldhafer K J, Gebel M, Flemming P, Georgii A, Borst H G, Pichlmayr R
Klinik für Abdominal- und Transplantationschirurgie, Medizinische Hochschule Hannover, Germany.
Hepatology. 1995 May;21(5):1337-44.
The optimal treatment of Budd-Chiari syndrome (BCS) remains an open question. It is still a matter of controversial discussion whether venous decompression or liver transplantation is superior. To elucidate the role and prognosis of both surgical options in our own experience, a consecutive series of 50 patients treated between 1981 and 1993 was retrospectively analyzed. Twelve patients had different types of portosystemic shunts or local decompressive procedures, and transplantation was performed in 43 cases, including five with previous conventional surgery. The overall mortality of 18 of 50 was conventional surgery. The overall mortality of 18 of 50 was concentrated within the early postoperative period, with no patient lost after 1 year. In the venous decompression group, the success rate was only 29%, and treatment failure was closely related to the finding of cirrhosis or technical problems like vascular thrombosis. After transplantation, early complications were rejection, primary nonfunction, or graft necrosis, and contributed significantly to the risk of sepsis. Thirty of 43 liver recipients are currently alive, including four rescued after failed decompressive surgery, with 1- and 10-year survival of 69%, and excellent recurrence-free rehabilitation. These results clearly indicate that patient selection plays a dominant prognostic role in the treatment of BCS. Venous decompression and liver transplantation should both be integrated in a common therapeutic concept, and the individual decision for the preferred approach must be based on the leading clinical symptom: portal hypertension or liver failure, together with the assessment of reversibility of hepatic damage, and the potential of cure of the underlying disease.
布加综合征（BCS）的最佳治疗方案仍是一个悬而未决的问题。静脉减压术和肝移植术哪种更具优势，仍是一个存在争议的讨论话题。为了根据我们自己的经验阐明这两种手术选择的作用和预后，我们对1981年至1993年间连续治疗的50例患者进行了回顾性分析。12例患者接受了不同类型的门体分流术或局部减压手术，43例患者接受了肝移植，其中5例曾接受过传统手术。50例患者中有18例总体死亡率为传统手术。50例患者中有18例总体死亡率集中在术后早期，1年后无患者死亡。在静脉减压组中，成功率仅为29%，治疗失败与肝硬化的发现或血管血栓形成等技术问题密切相关。肝移植术后，早期并发症为排斥反应、原发性无功能或移植物坏死，且显著增加了败血症的风险。43例肝移植受者中有30例目前仍存活，其中4例在减压手术失败后获救，1年和10年生存率为69%，且无复发康复情况良好。这些结果清楚地表明，患者选择在BCS治疗中起着主导性的预后作用。静脉减压和肝移植都应纳入一个共同的治疗理念，对于首选治疗方法的个体决策必须基于主要临床症状：门静脉高压或肝功能衰竭，以及肝损伤可逆性的评估和潜在基础疾病的治愈可能性。
