Budd-Chiari综合征的介入治疗

0引言Budd-Chiari综合征(BCS)是由于肝静脉(HV)或肝段下腔静脉(IVC)阻塞引起的一组综合征.其共同的主要病因是血栓形成和膜性狭窄.BCS一旦形成,罕见自行缓解的病例,病情常进行性发展,因此BCS应尽早治疗.由于药物治疗效果欠佳,目前主要采取外科手术和介入治疗.介入治疗自1974年日本学者用于治疗BCS以来,技术渐成熟,是当前BCS治疗的热点,操作简便,安全,创伤性小,并发征少而轻,可重复性强,疗效肯定,已成为BCS治疗的首选方法.1适应征IVC各种膜型和节段型或闭塞及肝静脉入口狭窄或闭塞均可采用球囊扩张术(PTA)及血管内支架置入术(E…     

下腔静脉单向造影对下腔静脉完全闭塞型Budd-Chiari综合征的诊断

77例下腔静脉(IVC)闭塞型Budd-Chiari综合征(BCS)患者术前行彩超、CT/MRI检查,术中经股静脉途径行IVC单向造影.使用钝性破膜导丝经股静脉途径开通IVC闭塞病变,继而完成球囊扩张或内支架置入等介入治疗.结果 77例IVC闭塞远端结构与闭塞段结构显示满意,25例IVC闭塞近心段显示清晰,52例IVC闭塞近心段结构通过降低图像窗宽和调整窗位也得到满意显示.在良好的IVC图像导引下IVC闭塞开通全部成功,未见严重并发症出现.认为经股静脉途径行单向IVC造影对IVC闭塞型BCS的IVC病变与临近结构的显示和介入治疗有重要意义.     

介入治疗下腔静脉节段闭塞型Budd—Chiari综合征的方法学研究

目的探讨介入治疗下腔静脉节段闭塞型Budd-Chiari综合征（BCS）的有效方法。方法对经造影确诊的26例下腔静脉肝后段节段型完全闭塞型BCS患者,采用聚乙烯球囊导管或国产Inoue球囊导管行经皮腔内血管成形术（PTA）及支架置入,其中闭塞段穿刺过程中综合运用直接穿刺技术、导管挤钻技术、逆向穿刺技术、双向穿刺技术、导丝轨道技术。PTA前后行下腔静脉造影及血液动力学检查;观察患者临床表现变化及并发症发生情况。结果23例成功穿通闭塞段并行PTA治疗,均无严重并发症,技术成功率为88．5％;15例置入下腔静脉支架。术后闭塞段下腔静脉内径为（19．20±6．21）cm,下腔静脉压、下腔静脉至右房压力阶差显著降低,右心房压显著升高;随访1～5a复发3例。结论介入治疗下腔静脉节段闭塞型BCS安全、有效,综合应用各种穿刺技术及合理选择球囊导管类型、直径可提高成功率、减少并发症。     

布-加综合征64例临床分析

布-加综合征（Budd—Chiari syndrome，BCS）是由肝静脉（hepatic veins，HV）或下腔静脉（inferior vena cava，IVC）阻塞性病变引起的以IVC、肝后性门静脉高压为特点的临床综合征。BCS的定义可分为广义和狭义两种，狭义的BCS是指单纯HV狭窄，主要表现为门静脉高压；而各种原因导致的HV和（或）HV开口及以上水平各种类型的IVC阻塞称为广义BCS。由于以往对该病的认识不足，其临床表现复杂多变而无特异性，常误诊为其他原因导致的肝硬化。随着临床治疗手段的进步，特别是介入治疗的引入，使本病早期接受治疗患者的预后得到了改善，所以对BCS临床特点的熟悉及早期诊断成为影响本病预后的重要因素。     

彩色多普勒超声在诊断与治疗肝段下腔静脉梗阻中的应用

目的 探讨应用彩色多普勒血流显像结合二维图像对肝段下腔静脉梗阻的诊断及其临床价值.方法 应用彩色多普勒对10例Budd-Chiari综合征患者的肝静脉、下腔静脉(IVC)、门静脉声像图进行分析,明确病变部位、范围及阻塞性质、程度,做出超声分型诊断,并与静脉造影对比后选择不同治疗方案.结果1.IVC节段性狭窄4例;2.隔膜状阻塞2例;3.IVC穿膈处血栓形成闭塞1例;4.IVC长段闭塞2例;5.肝硬化合并肝癌致IVC外压性狭窄1例.本组中除2例IVC长段闭塞者行腔房搭桥术及1例合并肝癌者未做手术外,余7例患者分别采取局麻下IVC内球囊扩张术并Z型支架植入术及局部抗凝溶栓治疗.结论 彩色多普勒能清晰显示血管管腔及血流情况,做出明确的超声分型诊断从而对外科手术方式的选择,判断术后效果有重要意义.其无创的可重复性及诊断价值可取代或部分取代有创的静脉造影.     

Budd-Chiari综合征30例临床分析

1　材料与方法　　本文总结自 1995～ 2 0 0 2我院收治的 3 0例Budd Chiari综合征 (BCS)患者。本组 3 0例经血管造影证实的BCS患者 ,男 18例 ,女 12例 ,年龄 2 4～ 63岁 ,平均 3 7 4岁 ,自症状出现到确诊的时间为 1～ 2 7a ,平均 6 8a。术前常规行CT、B超、胃镜检查 ,实验室检测血清白蛋白、总胆红素、血小板。单纯膜性下腔静脉闭塞的患者 ,采用经股静脉下腔静脉开通术和球囊成形术 ,必要时植入下腔静脉支架。对下腔静脉节段性闭塞的患者 ,在开通、扩张闭塞静脉后 ,植入下腔静脉支架。压迫性下腔静脉狭窄的患者均采取球囊扩张术和内支…     

小冠状动脉狭窄性病变的介入治疗

目的 :探讨小冠状动脉 （直径 <3 mm）狭窄性病变实施普通球囊、切割球囊或小支架介入治疗的疗效和并发症。方法 :小冠脉狭窄性病变介入治疗 （PCI）患者 13 6（男 87,女 49）例 ,年龄 3 2～ 85（54± 17）岁。根据手术方法分为普通球囊组、切割球囊组和小支架组。残余狭窄率 <3 0 %且无动脉夹层、撕裂等并发症者为手术成功 ,术后 6个月复查冠脉造影。结果 :普通球囊组 3 2例 ,手术成功 2 6例 （81% ） ,出现动脉夹层或扩张不满意改支架术 6例 （2 4% ）。切割球囊组 48例 ,手术成功 43例 （90 % ） ,出现动脉夹层或扩张不满意改支架术 3例 ,出现造影剂血管外漏 2例。支架组 56例 ,手术成功 53例 （95% ） ,出现造影剂血管外漏 2例 ,出现心包填塞抢救成功 1例。3组均未出现血管急性闭塞。术后 6个月 ,切割球囊组、小支架组、普通球囊组冠脉造影狭窄率分别为 2 3 % （11例 ）、16% （9例 ）、3 8% （12例 ）。结论 :小冠脉狭窄性病变实施介入治疗能取得显著效果 ,小支架术优于普通球囊扩张术 ,切割球囊扩张与普通球囊扩张具有近似疗效     

MSCT和MRI在布加综合征诊疗中的应用

目的:探讨多排螺旋CT(multislice spiral computed tomography,MSCT)及核磁共振成像(magnetic resonance imaging,MRI)在布加综合征(Budd-Chiari syndrome,BCS)诊疗中的应用价值.方法:回顾性分析53例经数字减影血管造影(digital subtraction angiography,DSA)或介入手术证实BCS患者的MSCT、MRI和手术资料.男35例,女18例;原发型49例,继发型4例;将BCS分为3型:下腔静脉型、肝静脉型和混合型.分析其直接征象、侧支血管、尾叶改变、强化特点、门脉高压征象以及合并疾病等,与DSA结果比较.结果:MSCT和MRI对BCS的诊断正确率分别为82.9%、93.8%,差异无统计学意义(?2=1.489,P0.05).49例准确显示B C S病变部位、阻塞性质、阻塞长度及走形方向、与周围组织的关系;准确显示肝内、肝外侧枝血管及其空间位置关系.下腔静脉(inferior vena cava,IVC)膜性阻塞17例,有孔型9例,无孔型8例;IVC节段性闭塞7例(≤2.5 cm),长段闭塞8例(2.5 cm).14例清晰显示"危险"侧支血管,清晰显示需成形的肝静脉(hepatic vein,HV)7例,副肝静脉3例.8例MRI准确显示IVC和/或HV内血栓的位置、血栓分期,12例MSCT发现IVC和/或HV内血栓;18例MSCT显示IVC内钙化.全部手术成功,3 m o后复查,治疗血管血流通畅、支架位置佳.结论:MSCT和MRI为BCS的诊断和介入治疗提供丰富、全面的信息,具有重要的应用价值.     

138例肝静脉阻塞型Budd-Chiari综合征介入治疗的研究

背景肝静脉阻塞型Budd-Chiari综合征(BCS)的病因尚未完全明了,其传统治疗方法为门体或肠腔分流术,近年介入放射学技术已成为治疗BCS的常用方法.目的总结11年期间以介入技术治疗肝静脉阻塞型BCS的经验,评价其疗效.方法以介入技术治疗138例肝静脉阻塞型BCS患者.介入治疗方法有经皮腔内血管成形术(PTA)、置入支架和经导管清除血栓.以血管造影表现和压力变化评价即刻疗效,以症状显著改善或消除评价临床疗效,随访期间行超声检查,部分复查CT和CT血管造影(CTA).结果肝静脉阻塞多为膜性阻塞(60.1%).同时存在肝静脉和下腔静脉阻塞者占25.4%,合并下腔静脉血栓占8.0%,肝静脉血栓形成占5.1%.介入治疗成功率为97.1%.单纯行肝静脉球囊扩张成形术28例(20.9%),球囊扩张后向狭窄段置入支架106例(79.1%).介入开通阻塞后,肝静脉压力从(36±9)cm H2O(1 cm H2O=0.098 kPa)降至(18±7)cm H2O.术中未出现并发症.治疗成功者术后相关症状明显改善或完全消失;32例术前以门静脉高压症为主要表现者术后未发生静脉曲张破裂出血,复查内镜示静脉曲张程度减轻.术后随访118例,均生存,其中9例(7.6%)临床症状复发,经血管造影证实支架区狭窄,开通再狭窄成功6例.结论介入放射学技术,如PTA、置入支架和经导管清除血栓是治疗肝静脉阻塞型BCS安全和有效的方法,远期疗效优良.     

布加氏综合征患者下腔静脉病变的超声诊断误诊分析

目的本文主要是为了分析布加氏综合征(BCS)患者下腔静脉(IVC)病变运用超声诊断出现误诊的原因。方法选择2014年2月~2015年1月来我院接受诊断的布加氏综合征下腔静脉病变患者100例的超声图像和IVC造影结果进行分析和比较。结果 BCS患者100例中IVC病变出现误诊的患者23例,误诊率为23%,其中下腔静脉节段性和膜性闭塞误诊为狭窄的16例、节段性及膜性狭窄误诊为闭塞的3例、外压性假性狭窄误诊为真心狭窄的5例。结论受BCS患者血流动力学特征因素以及IVC解剖特点的影响,超声对于BCS患者的IVC病变诊断容易出现误诊,ICV病变远段管腔扩张实际程度并不是该种病变狭窄程度的一个可靠指征。     

布-加综合征疑难病例的介入治疗

介入治疗已成为布一加综合征首选治疗方法。回顾了布-加综合征的影像学诊断以及6种疑难类型,即肝静脉阻塞、下腔静脉长段闭塞、下腔静脉闭塞合并血栓形成、下腔静脉闭塞近心端盲端缺如或很短、广泛肝静脉闭塞和下腔静脉支架放置后阻塞肝静脉的诊治经验。介绍了此6种类型的介入治疗技术要点和注意事项。分析表明,只要充分评估病情,选择合理的治疗方案,将有助于提高疑难布一加综合征病例的介入治疗成功率,进一步提高我国布一加综合征的整体诊疗水平。     

Fulminate intracardiac thrombosis associated with Budd-Chiari-syndrome and inferior vena cava thrombosis

The most common cause of edema of the legs and dyspnea is congestive heart failure. Further differential diagnosis such as renal or hepatic failure have to be considered. We report the case of a previous healthy 65-year-old woman who developed dyspnea and massive edema of the legs followed by acute hepatic and renal failure. Imaging studies showed a thrombosis of the inferior vena cava (IVC) caused by a tumor between the right kidney and the IVC. Histological examination revealed a leiomyosarcoma of the IVC. Hepatic failure due to venous outflow obstruction (Budd-Chiari syndrome, BCS) was diagnosed. Coagulation profile showed a complex disorder due to acute hepatic failure. Factor V Leiden and prothrombin gene mutation G20210A could be excluded. The thrombosis extended from the femoral veins up to the right atrium. After 11 days of anticoagulation with heparin platelet counts decreased by more than 50%. Suspecting a heparin-induced thrombocytopenia the patient was placed on recombinant hirudin (lepirudin) for anticoagulation. Hepatic venogram showed a thrombosis of the hepatic vein orifices but not of the hepatic veins. The tumor and the thrombi were removed surgically. When the cardiopulmonary bypass was terminated new intracardiac thrombi occurred. Despite immediate surgical intervention the patient finally died due to right ventricular failure caused by the fulminate intracardiac thrombosis. In conclusion, thrombosis of the IVC may mimic congestive heart failure and may cause BCS. Neoplasms and coagulation disorders may cause thrombosis of the IVC.     

Percutaneous balloon angioplasty of inferior vena cava in Budd-Chiari syndrome-R1

This study was to evaluate the clinical effects of percutaneous balloon angioplasty of Budd-Chiari syndrome (BCS) caused by inferior vena cava (IVC) obstruction. Between 1993 and 1999, 28 men and 14 women with mean age of 44+/-12 years underwent percutaneous balloon angioplasty for primary BCS. Color Doppler ultrasound and venography showed membranous and segmental obstruction of IVC in 29 and 13 patients, respectively. Fourteen patients also had left- and/or mid-hepatic vein obstruction. Angioplasty of IVC was successful in 41 patients (97.6%), resulting in a reduction of pressure gradient between IVC and the right atrium from 15.0+/-2.5 to 5.5+/-0.8 mmHg (P<0.01). A stent was placed in the site of obstruction in the patient with unsuccessful balloon angioplasty. Patients with successful angioplasty or stent placement had significant improvement in clinical symptoms indicated by a reduction in hepatomegaly and the degree of ascites. No specific attempt was made to treat the occluded left- and/or mid-hepatic vein due to the presence of potent right hepatic vein. Over the follow-up period of 32+/-12 months, restenosis of IVC occurred in only one patient (2.4%), which was redilated successfully. Percutaneous balloon angioplasty is a safe and effective therapy for Budd-Chiari syndrome caused by IVC obstruction, therefore should be the first choice of treatment for this condition.     

Acute iatrogenic Budd-Chiari syndrome following hepatectomy for hepatolithiasis: A report of two cases

Budd-Chiari syndrome(BCS)is defined as hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava(IVC)and the right atrium,regardless of the cause of obstruction.We present two cases of acute iatrogenic BCS and our clinical management of these cases.The first case was a 43-year-old woman who developed acute BCS following the implantation of an IVC stent for the correction of stenosis in the IVC after hepatectomy for hepatolithiasis.The second case was a61-year-old woman with complete obstruction of the outflow of hepatic veins during bilateral hepatectomy for hepatolithiasis.Acute iatrogenic BCS should be con-sidered a rare complication following hepatectomy for hepatolithiasis.Awareness of potential hepatic outflow obstructions and timely management are critical to avoid poor outcomes when performing hepatectomy for hepatolithiasis.     

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

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） too. 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.     

血管内介入技术治疗Budd—Chiari综合征患者

目的评价血管内介入技术治疗Budd-Chiari综合征的长期效果。方法应用经股静脉、经颈静脉或经皮经肝途径施行下腔静脉或肝静脉的球囊扩张和（或）内支架置入术,治疗97例Budd—Chiari综合征患者。结果96例患者治疗成功,1例失败。彩色多普勒超声或血管造影随访89例患者,随访时间6～20年,平均（14）年;其中2例患者下腔静脉内径小于12mm,但无临床症状,其余87例患者未见异常,7例失访。结论应用血管内介入技术治疗Budd-Chiari综合征临床疗效显著,支架可保持长期畅通。     

Ex Vivo Liver Resection Followed by Autotransplantation to a Patient With Advanced Alveolar Echinococcosis With a Replacement of the Retrohepatic Inferior Vena Cava Using Autogenous Vein Grafting: A Case Report and Literature Review

Alveolar echinococcosis (AE) of the liver is a rare disease. In advanced cases of this parasitic disease, the inferior vena cava (IVC) can be invaded; in these cases, the optimal treatment is liver transplantation and replacement of the IVC. Considering the donor shortage and the drawbacks of immunosuppressive therapy, ex vivo liver resection followed by autotransplantation may be the first choice for these patients.We report the first case of advanced AE successfully treated by an ex vivo liver resection, followed by autotransplantation with a replacement of the retrohepatic IVC using autogenous vein grafting. This graft included the following regions: the bilateral great saphenous vein, part of the retrohepatic inferior vena and the middle hepatic vein with no invasion, the inferior mesenteric vein, and part of the side wall of the infrahepatic vena cava. This patient had an uneventful postoperative recovery; currently, she has been enjoying a normal life and is 12 months postoperative with no immunosuppressive therapy or AE recurrence.In conclusion, ex vivo liver resection followed by autotransplantation with a replacement of the retrohepatic IVC using autogenous vein grafting might be a useful surgical practice for advanced AE.     

重症布-加综合征的诊断与治疗

目的：探讨重症布－加综合征的诊断标准与治疗方案。方法：对我院治疗的126例重症布－加综合征病人的临床资料进行回顾性分析。介入治疗10例，均行经皮肝穿肝静脉球囊导管扩张成形和内支架置入；手术治疗102例，其中行肠系膜上静脉－下腔静脉人工血管C型架桥术68例，脾静脉－颈内静脉人工血管架桥术33例，肠系膜上静脉－颈内静脉人工血管架桥术1例；介入加常规手术治疗14例，采用Seldinger技术行下腔静脉球囊导管扩张成形和内支架置入后行肠系膜上静脉－下腔静脉人工血管C型超桥术或改良脾－肺固定术。结果：围手术期死亡6例，随访120例，随访时间6个月－7年，效果优者89例（74．2％），良者31例（25．8％）。结论：提出了重症布－加综合征的诊断标准，不同的病变类型应采取不同的治疗方法。     

Combination of thrombolytic therapy and angioplastic stent insertion in a patient with Budd-Chiari syndrome

A 31-year-old female who had well-established polycythemia vera one year before, presented with the sudden onset. She had severe ascites and hepatic encephalopathy 12 d prior to admission. Real-time ultrasonography revealed a supra hepatic thrombosis extending toward the inferior vena cava （IVC）. Thrombolytic therapy with systemic streptokinase （250000 IU loading ＋ 100000 IU/h infusion） was started. At the end of 72 h infusion, the patient＇s general condition improved. A color Doppler ultrasonography then showed complete and partial resolution of the thrombosis in the supra hepatic vein and IVC, respectively. Despite this good response, 12 d later, the symptoms recurred. Venography detected complete obstruction of the IVC. Percutanous balloon angioplasty with stent insertion was performed successfully and the patient was discharged without any evidence of liver disease. A combination of systemic streptokinase and radiological intervention was effective in our patient.     

Inferior vena cava thrombosis at its hepatic portion (obliterative hepatocavopathy)

The Budd-Chiari syndrome was primarily described as hepatic vein thrombosis within the liver, but it now includes inferior vena cava (IVC) thrombosis and other conditions that cause hepatic vein outflow obstruction. This author and several others maintain that primary hepatic vein thrombosis and primary IVC thrombosis represent two different clinical disorders. Primary thrombosis of the IVC most commonly occurs in its hepatic portion, which seems to be predisposed to thrombosis and has been called membranous obstruction of IVC, because the thrombus organizes into a fibrous and frequently membranous occlusion of the IVC. The hepatic vein orifices are affected to varying degrees, resulting in congestive liver damage. The cause of IVC thrombosis may be a hypercoagulable state such as coagulation factor deficiency and myeloproliferative disorders, but is more often idiopathic. In Nepal, it is endemic with a suspected association with infections. To consider IVC thrombosis and the congestive liver damage as a disease entity, this author proposes the term obliterative hepatocavopathy, separate from hepatic vein thrombosis. Clinically obliterative hepatocavopathy is less severe in its acute phase compared with hepatic vein thrombosis, but it aggravates occlusion of hepatic vein orifices with recurrent thrombosis. Primary hepatic vein thrombosis and obliterative hepatocavopathy display different hemodynamics of the hepatic veins, IVC, and portal vein; dilatation of the subcutaneous veins in the body trunk is more pronounced in obliterative hepatocavopathy because the ascending lumbar vein becomes the major collateral route. Congestive liver cirrhosis develops after a long clinical course that may be complicated by hepatocellular carcinoma.