Department of Gastroenterology, University Hospital Heidelberg, Heidelberg, Germany.
BMC Gastroenterol. 2010 Mar 1;10:25. doi: 10.1186/1471-230X-10-25.
Budd-Chiari syndrome (BCS) generally implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. Treatment depends on the underlying cause, the anatomic location, the extent of the thrombotic process and the functional capacity of the liver. It can be divided into medical treatment including anticoagulation and thrombolysis, radiological procedures such as angioplasty and transjugular intrahepatic porto-systemic shunt (TIPS) and surgical interventions including orthotopic liver transplantation (OLT). Controlled trials or reports on larger cohorts are limited due to rare disease frequency. The aim of this study was to report our single centre long term results of patients with BCS receiving one of three treatment options i.e. medication only, TIPS or OLT on an individually based decision of our local expert group.
20 patients with acute, subacute or chronic BCS were treated between 1988 and 2008. Clinical records were analysed with respect to underlying disease, therapeutic interventions, complications and overall outcome.
16 women and 4 men with a mean age of 34 +/- 12 years (range: 14-60 years) at time of diagnosis were included. Myeloproliferative disorders or a plasmatic coagulopathy were identified as underlying disease in 13 patients, in the other patients the cause of BCS remained unclear. 12 patients presented with an acute BCS, 8 with a subacute or chronic disease. 13 patients underwent TIPS, 4 patients OLT as initial therapy, 2 patients required only symptomatic therapy, and one patient died from liver failure before any specific treatment could be initiated. Eleven of 13 TIPS patients required 2.5 +/- 2.4 revisions (range: 0-8). One patient died from his underlying hematologic disease. The residual 12 patients still have stable liver function not requiring OLT. All 4 patients who underwent OLT as initial treatment, required re-OLT due to thrombembolic complications of the graft. Survival in the TIPS group was 92.3% and in the OLT group 75% during a median follow-up of 4 and 11.5 years, respectively.
Our results confirm the role of TIPS in the management of patients with acute, subacute and chronic BCS. The limited number of patients with OLT does not allow to draw a meaningful conclusion. However, the underlying disease may generate major complications, a reason why OLT should be limited to patients who cannot be managed by TIPS.
布加氏综合征（BCS）通常意味着肝静脉和/或肝内或肝上腔静脉血栓形成。治疗取决于潜在病因、解剖位置、血栓形成过程的程度和肝脏的功能能力。它可以分为包括抗凝和溶栓在内的药物治疗、血管成形术和经颈静脉肝内门体分流术（TIPS）等放射学程序以及包括原位肝移植（OLT）在内的外科干预。由于疾病频率罕见，因此受控试验或较大队列的报告受到限制。本研究的目的是报告我们的单中心结果，即根据我们当地专家组的个人决定，对接受三种治疗方案之一的 BCS 患者进行治疗，即仅药物治疗、TIPS 或 OLT。
1988 年至 2008 年间，20 名急性、亚急性或慢性 BCS 患者接受了治疗。对临床记录进行了分析，以了解潜在疾病、治疗干预、并发症和总体结果。
16 名女性和 4 名男性，诊断时的平均年龄为 34 +/- 12 岁（范围：14-60 岁）。13 名患者的潜在疾病为骨髓增生性疾病或血浆凝血功能障碍，其他患者的 BCS 病因仍不清楚。12 名患者出现急性 BCS，8 名患者出现亚急性或慢性疾病。13 名患者接受了 TIPS 治疗，4 名患者接受了 OLT 作为初始治疗，2 名患者仅需要对症治疗，1 名患者因肝功能衰竭在开始任何特定治疗前死亡。13 名 TIPS 患者中有 11 名需要 2.5 +/- 2.4 次修订（范围：0-8 次）。1 名患者死于其潜在的血液疾病。其余 12 名患者的肝功能仍然稳定，不需要进行 OLT。作为初始治疗接受 OLT 的 4 名患者均因移植物的血栓栓塞并发症而需要再次进行 OLT。TIPS 组的中位随访 4 年和 11.5 年时的生存率分别为 92.3%和 75%，OLT 组的生存率分别为 75%和 75%。
我们的结果证实了 TIPS 在管理急性、亚急性和慢性 BCS 患者中的作用。接受 OLT 的患者数量有限，无法得出有意义的结论。然而，潜在疾病可能会产生重大并发症，这就是为什么 OLT 应该仅限于不能通过 TIPS 治疗的患者。
