Treatment of inferior vena cava obstruction producing Budd-Chiari syndrome

We treated a patient who had an inferior vena cava (IVC) obstruction associated with Budd-Chiari syndrome. All of the right, middle, and left hepatic veins were completely obstructed. The IVC was obstructed by a membranous substance and thrombus at the hepatic portion and was completely occluded by a fibrous septum at the site of a suprahepatic coarctation. A cavotomy was performed transversely at the suprahepatic level and then longitudinally to the level just above the renal veins, and the obstructing tissue was removed. An additional vertical incision was made in the IVC over the coarctation, and an autologous pericardial patch was sutured in place to widen the IVC. The patient was discharged with the patency of the IVC restored.     

Living donor liver transplantation for Budd-Chiari syndrome with inferior vena cava obstruction and associated antiphospholipid antibody syndrome

BACKGROUND: Recently, the antiphospholipid antibody syndrome (APLS) has been recognized as the cause of the Budd-Chiari syndrome (BCS) in adults. However, APLS-induced BCS has been seen rarely in children. The surgical strategy for BCS depends on the patency of the inferior vena cava (IVC) and on the primary disease. METHODS: A 10-year-old boy with a diagnosis of BCS complicated with IVC obstruction caused by APLS received medical treatment and radiologic intervention for 2 years. In spite of these treatments, no relief of the symptoms could be achieved. Finally, the patient underwent living donor liver transplantation (LDLT), which required cavoplasty. He has had an uneventful course since the LDLT. CONCLUSIONS: IVC-obstructed BCS associated with APLS seems to be a good indication for LDLT with cavoplasty. Because liver transplantation (LT) itself is not a cure for APLS, the risk of thrombosis cannot be eliminated entirely by LT. Although immunosuppression may influence antiphospholipid antibody production, long-term observation and life-long anticoagulant treatment is needed even after LT. J Pediatr Surg 36:659-662.     

下腔静脉隔膜阻塞型Budd-Chiari综合征血流数值模拟

目的:应用计算机仿真的方法研究Budd-Chiari综合征(BCS)下腔静脉隔膜生长过程中血管的血流动力学变化。方法:基于MRA图像建立下腔静脉隔膜阻塞型BCS血管模型,利用Ansys Fluent软件进行血流参数仿真。结果:成功建立下腔静脉隔膜阻塞型BCS血管模型。模型实验结果显示,在隔膜生长过程中,在下腔静脉及3支主干肝静脉汇合处与隔膜之间有一低速区,该区域面积随隔膜的增长而逐渐变大;随着隔膜的发展,下腔静脉及肝右静脉低剪切力的区域不断扩大,而切应力变大。结论:下腔静脉隔膜的发生发展是一个缓慢变化的过程,可能与下腔静脉壁面剪切力的变化密切相关。     

Chronic Budd-Chiari syndrome due to congenital membranous obstruction of the inferior vena cava: clinical experience

Budd-Chiari syndrome due to congenital membranous obstruction of inferior vena cava can be cured by appropriate surgical correction. A high index of suspicion is required to identify this condition and investigate it properly. Experience of three such cases is presented; two had a successful surgical correction and one refused any surgical intervention. Both patients who underwent operation are doing well and are symptom-free after 4 and 6 years, respectively. The diagnosis and the presentation of inferior vena caval obstruction and the various surgical procedures that are available for relieving the congenital obstruction of inferior vena cava are reviewed.     

Acute Budd-Chiari syndrome secondary to leiomyosarcoma of the inferior vena cava

The authors report a case of leiomyosarcoma of the inferior vena cava, responsible for an acute Budd-Chiari syndrome. The diagnosis, suggested by lower limb edema, ascites, and renal failure, was confirmed by sonography, CT scan, and pathological examination. A mesoatrial shunt and right atrial thrombectomy were performed under extracorporeal circulation. The inferior vena cava was ligated because the tumor was considered to be unresectable.     

A radical operation for the Budd-Chiari syndrome associated with obstruction of the inferior vena cava. A report of six patients

The Budd-Chiari syndrome is caused by an occlusion of the hepatic veins and is often associated with an obstruction in the hepatic portion of the inferior vena cava (IVC). Therefore, the various shunt operations done in an attempt to relieve the portal hypertension are often not effective. By using a radical surgical technique on six patients with Budd-Chiari syndrome, the liver was freed and a wide longitudinal incision was made in the hepatic portion of the IVC. The obstructed hepatic vein was reopened using a Fogarty catheter, and a pericardial patch reinforced with a Teflon prosthesis was secured over the incision. Two patients were still asymptomatic 3 and 4 years after operation. Two patients died postoperatively of pneumonia and hepatic insufficiency. The two other patients were discharged in fair condition, and one died 6 months postoperatively following hepatic failure.     

华法令抗凝对于合并下腔静脉血栓的Budd-Chiari综合征患者的疗效评价

目的探讨口服华法令维持抗凝在合并下腔静脉（IVC）血栓的Budd-Chiari综合征（Budd-Chiarisyndrome，BCS）治疗中的临床应用价值。方法选择合并IVC血栓的BCS患者16例，其中肝功A级7例，B级9例；所有患者均无消化道出血病史和出血倾向。给予患者口服华法令2．5mg／日，定期复查凝血指标并通过彩超或DSA了解IVC血栓情况。结果14例IVC血栓在服药6个月后完全消失，2例IVC血栓部分消失；随访时间（1年）内所有患者均无严重并发症发生。结论对于无出血倾向的合并IVC血栓的BCS患者，维持口服华法令的抗凝抗栓效果安全可靠，服药期限以6个月为宜。     

布加综合征继发下腔静脉血栓的诊治

目的：探讨布加综合征继发下腔静脉（inferior vena cava,IVC)血栓形成的诊断方法，方法：本组31例，均做术前造影，彩超检查28例，28例行根治术并取栓。结果：造影发现血栓24例，彩超发现25例，手术无死亡及急性肺动脉栓塞，随访6个月－80个月，复发3例。结果：造影对附着于侧壁或大块的血栓诊断确切，对前后壁或漂浮的血栓易漏诊。在治疗中，新鲜或漂浮的血栓在切开IVC后多被血流冲出，附壁血栓可采用球囊导管拖栓或手法取出。     

CT指导介入治疗下腔静脉Budd-Chiari综合征

目的评估CT对下腔静脉型Budd-Chiari综合征(BCS)介入治疗的临床指导价值。方法收集于我院接受介入治疗的329例下腔静脉型BCS患者,所有患者术前均接受CT检查,并根据CT诊断结果制定介入治疗方案。以DSA治疗结果为金标准,评估CT诊断的准确率、敏感度、特异度、阳性预测值和阴性预测值。结果 DSA示下腔静脉不完全闭塞型BCS 108例,下腔静脉完全闭塞型221例;CT诊断下腔静脉不完全闭塞型99例,下腔静脉完全闭塞型230例。与DSA结果相比,CT诊断15例假阴性,6例假阳性。CT术前制定是否破膜的准确率、敏感度、特异度、阳性预测值及阴性预测值分别为94.19%、97.29%、86.11%、93.49%及93.94%,CT术前制定介入治疗方法的准确率、敏感度、特异度、阳性预测值及阴性预测值分别为97.55%、100%、92.52%、96.49%和100%。结论 CT诊断下腔静脉型BCS准确率较高,且有助于制定介入治疗方案。     

Surgical treatment of Budd-Chiari syndrome associated with superior vena cava obstruction; report of a case

We report a case of a 37-year-old woman with Budd-Chiari syndrome who underwent a radical treatment. She had had ascites, general edema, and liver dysfunction for 5 years. Preoperative cineangiogram showed a membranous stenosis at the retrohepatic inferior vena cava and the catheter could not be advanced into superior vena cava. In the operation, obstructed superior vena cava was identified. Membranous tissue at retrohepatic inferior vena cava was removed, and patch cavoplasty was performed under circulatory arrest through Senning procedure. Postoperative cavogram revealed good patency of inferior vena cava and her symptoms were disappeared.     

序贯介入治疗Budd-Chiari综合征合并下腔静脉新鲜血栓

目的观察序贯介入治疗Budd-Chiari综合征合并下腔静脉(IVC)新鲜血栓的有效性及安全性。方法对27例Budd-Chiari综合征合并IVC新鲜血栓患者行小球囊预扩张、置管溶栓及大球囊扩张等序贯介入治疗;记录不良反应;术后随访复查超声,评估治疗效果。结果对27例均以直径10～14 mm小球囊预扩张IVC闭塞段,扩张后原闭塞段血流均部分恢复;经留置溶栓导管泵入尿激酶40～60×10~4U/d、平均(44.10±8.40)×10~(4 )U/d,持续4～15天、平均(6.72±5.21)天后,超声显示20例IVC内血栓完全消失,7例仍残留少量陈旧血栓;以大球囊(直径25～30 mm)扩张IVC原狭窄段,之后IVC血流恢复,侧支血管明显减少或消失。围手术期未见肺栓塞、大出血等严重并发症。术后平均随访(20.22±18.43)个月,27例患者均存活,相关临床症状及体征消失或不同程度缓解。结论序贯介入治疗Budd-Chiari综合征合并IVC新鲜血栓安全、有效。     

布-加综合征下腔静脉病变隔膜形态学特征研究

目的 观察分析布-加综合征下腔静脉(IVC)病变隔膜的形态学特征,探讨病变隔膜的形成机制.方法 大体观察2007年10 月至2010年l2月根治性切除手术切除的10例布-加综合征下控静脉病变隔膜形态特征,并利用光镜、电镜对比观察病变隔膜与 7例正常肝后段下腔静脉壁的组织结构差异.结果 术中见病变隔膜位于下腔静脉入右心房2.0 cm范围内.隔膜边缘较厚,中央偏薄,表面与IVC内膜相连.9例隔膜下方IVC有附壁血栓,5例病变隔膜局部有钙化.光镜下病变隔膜组织由粗大的均质胶原纤维及成纤维细胞构成,胶原排列紊乱,部分胶原纤维呈透明变性.隔膜边缘为不等程度的纤维结缔组织增生,少量肉芽组织和新生血管等病理改变;正常下腔静脉壁内膜、中膜和外膜3层结构完整.电镜下病变隔膜组织中可见较多的纤维细胞,表面有少量内皮细胞.内皮细胞内线粒体大部分嵴和膜脱落融合、模糊不清或缺失,粗面内质网轻度扩张.偶见凋亡细胞,但体积缩小;正常下腔静脉壁大内皮细胞排列规则,呈条带状分布,内皮细胞内可见线粒体、粗面内质网及吞饮小泡,内皮细胞下为少量排列较稀疏的胶原纤维及弹性纤维.结论 布-加综合征下腔静脉病变隔膜形成以纤维细胞增生为主,肝后段下腔静脉的损伤和炎症反应可能参与了隔膜的形成过程.     

Budd-Chiari syndrome and inferior vena cava thrombosis in a nephrotic child

We observed Budd-Chiari syndrome in a boy aged 2 years 6 months with nephrotic syndrome due to hepatic vein and inferior vena cava thrombosis, confirmed by Doppler imaging. Normal values of the routine hemostatic parameters proved that they are of little predictive value for the thrombotic state. Immediate heparin infusion was initiated. High doses of heparin up to 59 IU/kg per hour were required for efficient anticoagulation. A remission of the nephrotic syndrome was achieved with vincristine. Oral anticoagulation with a vitamin K antagonist was continued for 6 months. Doppler imaging then indicated full re-establishment of the blood flow through the affected vessels. The clinical and Doppler data in this case are compatible with acute Budd-Chiari syndrome due to incomplete outflow obstruction of the hepatic veins and inferior vena cava. The favorable outcome was due to the immediate heparin infusion and prompt remission of the nephrotic syndrome. Doppler imaging was an important tool for non-invasive diagnosis and follow-up. Received: 31 May 1999 / Revised: 30 August 1999 / Accepted: 3 September 1999     

下腔静脉二次球囊扩张配合华法林治疗合并下腔静脉血栓形成的布加综合征

目的:探讨下腔静脉二次球囊扩张配合口服华法林治疗布加综合征合并下腔静脉血栓形成的临床疗效。方法:回顾分析2008年2月—2014年1月收治的布加综合征合并下腔静脉血栓形成患者54例。先行下腔静脉小球囊扩张术,同时给予达标量口服华法林抗凝治疗3~6个月,影像学观察患者血栓溶解情况。治疗效果明显者给予下腔静脉大球囊扩张成形术。结果:54例患者一期手术成功率100%,2例手术后口服华法令出现出血,改行外科手术治疗;4例改行其他介入治疗方式;余48例患者术后口服华法林抗凝溶栓治疗3~6个月,治疗效果明显,行二次行大球囊扩张治疗。结论:腔静脉二次球囊扩张同时口服华法林治疗布加综合征合并下腔静脉血栓形成有良好的效果。