Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment
Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment
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DOI：
10.3748/wjg.14.278
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发表时间：
2008-01-14
影响因子：
4.3
通讯作者：
Patel, Nikhil D.
中科院分区：
文献类型：
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作者：
Amarapurkar, Deepak N.;Punamiya, Sundeep I.;Patel, Nikhil D.
目的：评价印度Budd-Chiari综合征患者梗阻的类型、病因谱和非手术治疗。方法：对连续49例Budd-Chiari综合征（BCS）患者进行前瞻性评价。所有难治性腹水或肝功能恶化的患者，根据下腔静脉（IVC）和/或肝静脉（HV）阻塞的形态，除抗凝治疗外，还进行放射干预。无症状患者、利尿反应性腹水及肝功能稳定的患者、不愿手术干预的患者对症给予抗凝治疗。结果：平均症状持续时间为41.5 +/- 11.2（范围= 1-240）个月。HVT (HVT) 29例（59.1%），IVC血栓8例（16.3%），IVC膜性梗阻2例（4%），IVC-HV血栓合并10例（20.4%）。35例高凝血检测中，27例（77.1%）高凝血状态阳性。放射干预在技术上是成功的37/38 (97.3%):IVC支架置入7例（18.9%），IVC球囊血管成形术2例（5.4%），IVC-HV联合支架置入2例（5.4%），HV支架置入11例（29.7%），经颈静脉肝内门体分流术（TIPS） 13例（35.1%），TIPS-IVC联合支架置入2例（5.4%）。随访中出现的并发症：5例死亡，5例支架再狭窄（17.1%），2例肝性脑病，1例肝细胞癌。在接受医学治疗的9例患者中，2例HVT完全消退。结论：在我们的研究中，HVT是BCS的主要原因。在过去的五年中，随着高凝性疾病的复杂测试的可用性，85.7%的病例确定了病因。大多数病例非手术治疗成功。(c) 2008 WJG。版权所有。
AIM: To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India.METHODS:, Forty-nine consecutive cases of Budd-Chiari syndrome (BCS) were prospectively evaluated. All patients with refractory ascites or deteriorating liver function were, depending on morphology of inferior vena cava (IVC) and/or hepatic vein (HV) obstruction, triaged for radiological intervention, in addition to anticoagulation therapy. Asymptomatic patients, patients with diuretic-responsive ascites and stable liver function, and patients unwilling for surgical intervention were treated symptomatically with anticoagulation.RESULTS: Mean duration of symptoms was 41.5 +/- 11.2 (range = 1-240) mo. HV thrombosis (HVT) was present in 29 (59.1%), IVC thrombosis in eight (16.3%), membranous obstruction of IVC in two (4%) and both IVC-HV thrombosis in 10 (20.4%) cases. Of 35 cases tested for hypercoagulability, 27 (77.1%) were positive for one or more hypercoagulable states. Radiological intervention was technically successful in 37/38 (97.3%): IVC stenting in seven (18.9%), IVC balloon angioplasty in two (5.4%), combined IVC-HV stenting in two (5.4%), HV stenting in 11 (29.7%), transjugular intrahepatic portosystemic shunt (TIPS) in 13 (35.1%) and combined TIPS-IVC stenting in two (5.4%). Complications encountered in follow-up: death in five, re-stenosis of the stent in five (17.1%), hepatic encephalopathy in two and hepatocellular carcinoma in one patient. Of nine patients treated medically, two showed complete resolution of HVT.CONCLUSION: In our series, HVT was the predominant cause of BCS. In the last five years with the availability of sophisticated tests for hypercoagulability etiologies were defined in 85.7% of cases. Non-surgical management was successful in most cases. (c) 2008 WJG. All rights reserved.