布加综合征6例治疗分析

对我们1998～2004年收治的布加综合征（BCS）6例分析如下。 1临床资料 1．1一般资料本组男1例，女5例，年龄14～25岁。单纯腔静脉隔膜状阻塞2例，肝静脉狭窄并腔静脉阻塞1例，单纯肝静脉狭窄阻塞3例。     

布加综合征的超声诊断

目的：总结布加综合征与肝硬化的彩色多普勒(CDFI)鉴别诊断价值及临床意义。方法：8例布加综合征的患均行二维与CDFI超声检查。观察声像图特征，肝内、外血管病变及一些侧支血管异常显示与血流动力学改变，找出病因，后经血管造影验证。结果：8例中4例为下腔静脉阻塞型，1例为下腔静脉外压性狭窄，1例为肝静脉阻塞型。2例为混合性阻塞。声像图特征；肝脏及尾状叶增大，脾肿大，顽固性大量腹水、肝段下腔静脉和(或)肝静脉狭窄、不完全或完全性阻塞。副肝静脉与尾状叶静脉扩张，内脏与下肢静脉淤血扩张。结论：布加综合征是门脉高压的原因之一，用CDFI能提高鉴别诊断的准确率，为临床制定治疗方案和评估预后具有重耍意义，并提供可靠依据。     

布加综合征的3D-CEMRA表现及诊断价值

目的：分析布加综合征（BCS）的三维对比剂增强MR血管成像（3D-CE-MRA）表现特征,探讨MRI对BCS的诊断价值.材料与方法：对39例BCS采用3.0T MR扫描仪进行常规MRI平扫及3D-CEMRA检查.观察肝静脉（HV）、下腔静脉（IVC）和门静脉（PV）的开放性,观察有无肝内外侧支循环、门静脉-体静脉间曲张静脉和肝实质病变.其中,17例行超声检查,10例行DSA检查,12例行CT检查.结果：39例BCS中,IVC阻塞型（Ⅰ型）6例,HV阻塞型（Ⅱ型）5例,混合性阻塞型（Ⅲ型）28例;39支IVC中,MRI清晰显示34支IVC阻塞（膜性阻塞15例,节段性阻塞19例）;117支HV中,MRI显示62支HV阻塞（肝右静脉24支,肝中静脉17支,肝左静脉21支）.MRI直接征象表现为HV和/或IVC狭窄、闭塞;间接征象表现为HV和/或IVC阻塞远端血管扩张,肝大、尾叶肿大、脾大,肝内侧枝血管,副HV,肝外侧枝血管,肝硬化,肝癌和肝脏其他占位特别是第二肝门周围占位,腹水,IVC和HV血栓形成.结论：MRI能直接显示HV和/或IVC阻塞的部位、程度及侧枝循环情况,MRI可作为诊断BCS的一种优选的非侵袭性的影像学检查方法.     

三维超声彩色能量多普勒血管成像对Budd-Chiari综合征第三肝门引流状况的评价

目的：评价三维超声彩色能量多普勒血管成像（3D—CPA）在Budd-Chiari综合征（BCS）患者第三肝门血管引流状况中的临床应用价值。方法：对60例BCS患者主肝静脉和第三肝门引流血管（包括右后下肝静脉及尾叶静脉）进行彩色能量多普勒血管三维重建，评价主肝静脉阻塞后肝内交通静脉及第三肝门血管的引流状况。结果：本组60例BCS患者共计180支主肝静脉中，病变主肝静脉139支，正常通畅主肝静脉41支。除43支完全闭塞的主肝静脉无血流信号外，3D—CPA清晰显示了，其余96支未完全闭塞的主肝静脉，通过数量不等、内径不同的肝内交通支汇入右后下肝静脉和／或尾叶静脉，然后经第三肝门引流入下腔静脉。交通支内径3-10mm。梗阻主肝静脉通过交通支经43支右后下肝静脉和71支尾叶静脉引流入下腔静脉。第二、三肝门的立体空间解剖关系显示明确。对肝静脉的闭塞部位显示血流信号消失，肝内交通支桥接于阻塞肝静脉与右后下肝静脉和／或尾叶静脉之间。结论：3D—CPA在BCS第三肝门的应用更直观显示了阻塞后肝静脉的血液引流状况，对临床制定治疗方案提供了更为丰富的信息。     

布加综合征的超声诊断

目的探讨布加综合征的声像图表现及超声对布加综合征的诊断价值。方法分析31例经下腔静脉造影确诊的布加综合征病例的超声图像。结果肝静脉阻塞或闭塞（型）6例,下腔静脉阻塞或闭塞（型）22例,肝静脉和下腔静脉混合病变（型）3例。本组中超声诊断准确率93.55%。结论超声可以明确布加综合征血管病变的部位、范围及类型,准确反映下腔静脉、肝静脉的血流颜色、方向及流速曲线的变化,对诊断具有重要参考价值,可为治疗提供可靠信息。     

磁共振多期动态增强扫描与血管重建对Budd-Chiari综合征的评价

目的:分析Budd-Chiari综合征(BCS)磁共振多期动态增强扫描的影像学征象,评价磁共振三维动态增强血管成像(3D DCE MRA)对BCS的诊断价值。材料与方法:33例BCS患者进行了磁共振多期动态增强扫描,对其相关血管分别以最大强度投影法(MIP)和多平面重建法(MPR)进行3D DCE MRA。以手术或DSA结果作对照,评价磁共振多期动态增强扫描与血管重建对判断BCS血管梗阻部位、程度以及肝内外侧支循环建立的价值。结果:磁共振多期动态增强扫描显示:下腔静脉狭窄21例,下腔静脉闭塞12例。肝静脉狭窄或闭塞15例,副肝静脉增粗10例,肝内或肝外侧支循环形成23例。MIP血管重建可显示血管和侧支循环总体情况,而MPR血管重建可多角度直接显示血管。肝实质异常包括:肝脏各叶比例失调、尾状叶增大11例,肝实质信号不均匀并斑片状强化25例,显示良性结节7例。结论:磁共振多期动态增强扫描能够准确地反映BCS肝脏血流动力学变化,结合3D DCE MRA,能够更直观、准确地显示血管梗阻平面及肝内、外侧支循环,是一种有效的术前全面评价BCS的无创性检查技术。     

Budd-Chiari综合征的彩色多普勒超声诊断与分型

目的 探讨Budd-Chiari综合征(BCS)的彩色多普勒超声图像特点和超声诊断分型,以利于选择治疗方式.方法 回顾性分析126例BCS患者的临床资料,全部患者经血管造影、介入或手术治疗证实.根据彩色多普勒超声图像特征进行分型.结果 根据彩色多普勒超声图像特点,将BCS分为8型:Ⅰ型,不完全性下腔静脉隔膜,30例;Ⅱ型,完全性下腔静脉隔膜,3例;Ⅲ型,下腔静脉狭窄,8例;Ⅳ型,下腔静脉阻塞,3例;V型,大肝静脉狭窄,20例;Ⅵ型,大肝静脉阻塞,15例;Ⅶ型,小肝静脉广泛性阻塞,9例;Ⅷ型,混合型病变,38例.结论 彩色多普勒超声检查能良好显示下腔静脉、肝静脉狭窄或阻塞的性质、部位、程度及血流动力学变化,对BCS的诊断分型准确可靠.     

布加综合征的螺旋CT诊断研究

目的:研究螺旋CT在布加综合征疾病诊断中的临床价值.方法:选取2018年1月—2019年10月期间,我院接收经手术证实为布加综合征的患者30例,对所有患者开展螺旋CT检查,比较CT检查结果与手术病理结果的符合率.结果:30例患者经手术病理诊断共13例为中下腔静脉全部或部分阻塞,10例为肝静脉阻塞,7例为混合型,30例患者均存在肝内或者肝外侧支静脉循环;螺旋CT诊断结果为12例中下腔静脉全部或部分阻塞,9例肝静脉阻塞,9例混合型,2例误诊,诊断结果符合率为93.33%,与手术诊断结果对比,P>0.05.结论:螺旋CT是临床诊断布加综合征疾病的一种有效方法,具备较高的临床推广运用价值.     

磁共振血管成像诊断布加综合征6例分析

布加综合征（Budd-Chiari syndrome,BCS）是肝段下腔静脉和（或）肝静脉狭窄或阻塞所致肝静脉回流障碍引起的一种肝后性门静脉高压症[1]。本院应用磁共振血管成像（magnetic resonanceangiography,MRA）确诊6例,现就BCS的表现及MRA对BCS的诊断价值探讨如下。     

64层螺旋CT血管成像技术对Budd-Chiari综合征侧支循环的评价

目的：探讨64层螺旋CT血管成像（CTA）技术对Budd-Chiari综合征（BCS）侧支循环的显示价值。方法：对74例BCS病人术前行CTA检查,观察肝静脉、下腔静脉病变类型及侧支循环情况。结果：74例均诊断为混合型BCS,下腔静脉和肝静脉存在不同程度的梗阻,每一例病人均有侧支循环形成。侧支循环分为肝内侧支、肝外侧支及门静脉-体静脉交通。肝内侧支循环分为6种类型：①肝静脉-副肝静脉引流型（n=47,63.5%）;②肝静脉-肝静脉引流型（n=5,6.7%）;③肝静脉-副肝静脉加肝静脉引流型（n=6,8.1%）;④腔-肝-房引流型（n=4,5.4%）;⑤肝静脉-脐静脉引流型（n=4,5.4%）;⑥肝静脉-肝被膜下静脉引流型（n=8,10.8%）。上述6型肝内侧支均通过数量不等、内径不同的交通支经引流静脉引流入肝段下腔静脉、右心房、脐静脉或被膜下肝静脉与体循环相通。肝外侧支循环分为4种类型：①腰静脉-腰升静脉-奇静脉及半奇静脉侧支型（n=74,100%）;②左肾静脉-腰升静脉-半奇静脉侧支（n=74,100%）和左肾静脉-左膈下静脉侧支型（n=48,64.8%）;③肾静脉-肾包膜下静脉-腹壁浅静脉侧支型（n=26,35.1%）;④腹壁浅静脉侧支型（n=10,13.5%）。结论：64层螺旋CTA可无创性清晰显示BCS患者各种类型的侧支循环途径,对指导临床选择正确的治疗方案具有重要价值。     

Budd-Chiari综合征64层CT动态增强扫描强化特征及CT血管成像分析

目的：探讨布-加综合征（Budd-Chiari syndrome,BCS）肝脏64层CT动态强化规律及成像基础,分析CT血管成像技术（CTA）在Budd-Chiari综合征中的应用价值.材料与方法：对1 1例Budd-Chiari综合征病人进行三期增强检查,并对相关血管分别行最大密度投影（MIP）、容积成像（VR）和多平面重建（MPR）,分析Budd-Chiari综合征患者肝脏64层CT动态强化的规律,并观察肝静脉、下腔静脉病变类型及肝内外侧枝循环情况.结果：11例螺旋CT动态增强的BCS患者3例为急性期,8例为慢性期.7例为肝静脉梗阻,4例为下腔静脉梗阻.门脉期肝脏周边强化型4例,中心强化型2例,斑片强化型1例,均匀强化4例.肝内侧支静脉开放6例,其它征象如肝肿大、肝脏尾叶增大、脾大、腹水、肝内外侧枝血管形成等表现也可见到.结论：64层螺旋CT动态增强扫描可无创性显示肝脏血流动力学变化,结合CTA技术,能直观、准确地显示血管梗阻平面及肝内、外侧支循环情况.     

彩色多普勒超声在不同类型布-加综合征诊断与治疗中的价值

目的探讨彩色多普勒超声在不同类型布-加综合征(BCS)诊断与治疗中的价值。方法应用彩色多普勒超声对临床拟诊的不同类型BCS患者106例及30例健康对照者进行检测,观察其特异性血管形态及血流动力学变化,与DSA对比,并治疗后随访。结果(1)超声诊断BCS90例,DSA确诊89例,超声诊断的敏感性97.8%,特异性82.4%,准确性95.3%,假阳性率17.7%,假阴性率2.3%。(2)血流动力学变化:与对照组相比,下腔静脉(IVC)狭窄者可见局部高速血流,阻塞者局部无血流信号;肝静脉(HV)扩张,血流减慢,并可见肝内侧枝血管;门静脉(PV)入肝血流速度减慢.(3)介入治疗及手术后随访:①介入治疗后,IVC病变处增宽,血流恢复,并可见支架回声,HV及PV血流速度加快;②手术治疗组,根治者可见IVC及HV血流恢复正常,人工血管转流者,可见人工血管呈螺旋管状,内血流清晰。结论彩色多普勒超声对BCS的诊断与血管造影具有良好的相关性,可明确BCS特异性异常血管形态及血流动力学变化,是BCS诊断及术后随访的可靠方法。     

Cavernous transformation of the portal vein: three-dimensional dynamic contrast-enhanced MR angiography

BACKGROUND: To investigate characteristic features of three-dimensional dynamic contrast-enhanced MR angiography (3D DCE-MRA) and validate its clinical significance for the diagnosis of cavernous transformation of the portal vein (CTPV). METHODS: 3D DCE-MRA, along with subsequent data processing using three-dimensional reconstruction, was performed in 33 CTPV patients. We observed 33 emboli in the portal vein, 29 in the left and/or right portal branches, 18 in the superior mesenteric vein, and nine emboli in the splenic vein. RESULTS: The main presentation of CTPV on 3D DCE-MRA was the replacement of the normal configuration of the portal vein by numerous distorted hepatopetal collateral vessels, the presence of arterio-portal venous shunts, and the appearance of hepatofugal collateral vessels in the abdominal cavity and wall. CONCLUSION: 3D DCE-MRA can be used to simultaneously characterize the portal system and collateral vessels, and can improve the diagnosis and clinical treatment of CTPV.     

The application and efficacy of stent place for Budd-Chiari syndrome

Objective To evaluate the application value and efficacy on stent place for Budd-Chiari syndrome (BCS).Methods From January 1990 to May 2017, 2228 patients with BCS were admitted to our institution. The mean age was 43.3 years. Stents were placed in inferior vena cava (IVC), hepatic vein (HV), or both after balloon dilation. During follow-up period, the patency of stent was evaluated by ultrasound regularly and the clinic sign was surveyed by letter, telephone or clinic visit. The restenosis of stent were treated with balloon dilatation and thrombolysis to restore the its function.Results IVC type was diagnosed in 1492 cases, HV type in 510 cases, and mixed type in 226 cases. Eighteen patients aborted treatment because of economic reasons, advanced liver cancer, severe scoliosis, or both bilateral iliac veins and total IVC occlusion. Among the other 2210 cases who underwent endovascular therapy, stents were implanted into IVC in 339 cases, HV in 97 cases, mixed type in 64 cases. The rate of restenosis in IVC stent was 11.50% (39/339). After repeat angioplasty, the long-term patency rate reached to 98.12%. The incidence of HV occlusion caused by IVC stent was 12.09% (n = 41). Restenosis occurred in 47 cases (48.45%) after HV stent placement. However, the 5-year patency rate was 91.75% (89/97) after repeat dilatation and stent re-implantation. The incidence of IVC obstruction caused by HV was 3.33% (3 cases).Conclusion IVC stent placement appears to be an effective treatment for the cases of IVC segmental occlusion, and at the same time, the stent has the dual role of compression and fixation of thrombus and support of lumen. The HV and accessory hepatic vein obstruction could happen when the IVC stent crossed these veins ostium. The incidence of the stent restenosis in the HV was higher than that in the IVC.     

中国河南地区布加氏综合征患者肝内静脉及侧支循环的超声表现分析

目的：探讨中国河南地区布加氏综合征（Budd-Chiari syndrome, BCS）患者肝内静脉及侧支循环的超声影像表现特点。方法：分析567例BCS患者肝内静脉及侧支循环的超声图像资料。结果：①肝左静脉（LHV）病变507例,肝中静脉（MHV）病变517例,肝右静脉（RHV）病变418例。LHV、MHV以膜性闭塞性病变最多,RHV以条索状闭塞最多。②567例BCS患者副肝静脉（AHVs）扩张403例,病变27例,AHVs有病变与无病变在下腔静脉（IVC）是否病变方面差异有统计学意义（P=0.000,连续校正法）。③肝内侧支循环类型8种。④567例BCS患者中395例肝内形成侧支循环,尾叶静脉与肝右后静脉在与主肝静脉（MHVs）形成侧支方面有统计学差异（P=0.000）。⑤病变的LHV、MHV、RHV形成侧支循环例数分别为331、319、104例,三者在形成侧支循环方面有统计学差异（P=0.000）,两两比较：LHV与MHV在形成侧支循环方面无统计学差异（P=0.234）,LHV与RHV及MHV与RHV在形成侧支循环方面有统计学差异（P=0.000）。结论：①中国河南地区BCS患者MHVs病变发生率高,多伴有副肝静脉代偿扩张及侧支循环形成。②BCS患者肝内侧支循环类型丰富,尾叶静脉与MHVs形成侧支循环数量明显多于肝右后静脉;LHV、MHV侧支循环形成数量多于RHV,MHVs病变主要类型与侧支循环形成多少有关。③AHVs有一定的病变率,IVC病变不是AHVs病变的主要诱因。     

Sonography and color Doppler imaging of Budd-Chiari syndrome of membranous obstruction of the inferior vena cava.

We examined 18 patients with Budd-Chiari syndrome (membranous obstruction of the hepatic IVC). IVC obstruction was detected by sonography in 17 cases (94%). The causes were a web in 10 cases and a fibrous cord in four cases. In three cases, the cause was unspecified. Obliteration of the hepatic veins or intrahepatic collateral vessels or both was delineated in 16 cases (89%). With seven cases of color Doppler imaging, there was no detectable color or Doppler signal within the IVC, and the direction of hepatic venous blood flow through intravenous communications was fairly well demonstrated in all cases. In four cases of follow-up color Doppler imaging, the patency of the IVC was well demonstrated. Sonography is useful in detecting obstruction of the hepatic IVC and in demonstrating the hepatic venous obstruction and intrahepatic collateral vessels. Color Doppler sonography is useful in demonstrating the direction of blood flow through interconnecting vessels and in evaluating the patency of the IVC after treatment.     

Diagnosis of Budd-Chiari syndrome: three-dimensional dynamic contrast enhanced magnetic resonance angiography

Objective

To investigate the characteristics and diagnostic value of three-dimensional contrast-enhanced magnetic resonance angiography (3D CE-MRA) in the diagnosis of Budd?CChiari syndrome (BCS).

Methods

One hundred thirty-three BCS patients underwent 3D CE-MRA, 64 patients had primary BCS, and 69 had secondary BCS.

Results

Fifty five cases (41.4%) showed a segmental stenosis of the inferior vena cava, 9 cases (6.8%) a membranous obstruction of the inferior vena cava, 5 cases (3.8%) an unobstructed inferior vena cava and hepatic veno-occlusive condition, 16 cases (12.0%) an inferior vena cava stenosis and hepatic veno-occlusive disease, and 48 cases (36.1%) an intraluminal filling defect in the inferior vena cava. In 52 cases (39.1%), collateral blood vessels were formed, with deep, medium, and shallow portal veins and intrahepatic collateral veins in 88 groups. Among these, 41 (46.6%) had deep venous collateral channels, 24 (27.3%) had medium venous collateral channels, 9 (10.2%) had superficial venous collateral channels, 5 (5.7%) had portal vein collateral channels, and 9 (10.2%) had intrahepatic venous collateral channels.

Conclusion

3D CE-MRA is important in the clinical diagnosis and treatment planning of BCS and displays hepatic veins, the inferior vena cava system, and collateral vessels.     

电子束CT增强双期扫描和血管重建对Budd-Chiari综合征的评价

目的 分析Budd-Chiari综合征的CT增强双期扫描影像学征象，评价血管三维重建的临床意义。方法 32名患者CT扫描增强动脉期延迟时间为30s，门脉期延迟时间为70s。血管三维重建方式为表面阴影遮盖法(SSD)和多平面重建法(MPR)。所有患者均进行了下腔静脉数字减影血管造影。结果 下腔静脉梗阻：隔膜性5例，闭塞性15例，狭窄性12例。肝静脉完全梗阻10例，不完全梗阻14例。可发现有两种肝外侧支循环形成，其中10例有肝内侧支循环形成。SSD法血管重建可显示血管和侧支循环总体情况，而MPR法可多角度直接显示血管。结论 电子束CT增强双期扫描和血管三维重建可全面评价Budd—Chiari综合征的血管和器官病变状况。