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.     

超声对布-加综合征的诊断价值

目的探讨灰阶与彩色多普勒、频谱多普勒及能量多普勒超声对布-加综合征的诊断价值。方法对38例经下腔静脉造影和／或经手术证实的布-加综合征患者的超声影像资料进行分析总结。结果38例布-加综合征患者中，超声确诊36例，漏诊2例，诊断准确率95％，与行下腔静脉造影相比，二者诊断准确率无显著性差异（P〉0．05）。其中下腔静脉型23例，肝静脉型5例，混合型10例。结论灰阶超声结合彩色多普勒、频谱多普勒、能量多普勒超声对布-加综合征的诊断符合率高，对临床治疗方式的选择具有重要意义。     

布加氏综合征的彩色多普勒检查

目的　探讨彩色多普勒超声对布加氏综合征的诊断要点和临床价值,为临床诊治提供可靠依据。方法　用彩色多普勒超声诊断布加氏综合征住院病人33例。结果　肝静脉阻塞5例,肝段下腔静脉阻塞17例,二者同时病变11例,全部经血管造影或MRA、核素扫描及手术确诊。结论　彩色多普勒超声可明确判断血管阻塞部位、程度、范围及侧支循环,对诊断具有重要参考价值,并可指导临床治疗,应作为首选的诊断方法。     

FIESTA序列对Budd-Chiari综合征下腔静脉病变的诊断价值

目的：探讨FIESTA序列对Budd-Chiari综合征(BCS)下腔静脉病变的诊断价值。材料与方法回顾性分析51例临床诊断为BCS的患者的下腔静脉病变情况,以DSA为诊断金标准,评估FIESTA序列对下腔静脉病变的诊断能力,包括检出静脉病变的敏感度、特异度、诊断准确度,显示下腔静脉血栓、下腔静脉瘤的能力。结果 MRI诊断准确有41例,灵敏度为97.61%、特异度为55.56%、准确度为90.20%;发现下腔静脉血栓8例,下腔静脉瘤样扩张4例。结论 FIESTA序列在显示BCS的下腔静脉中具有直观、准确率较高、无需对比剂的优点,可作为诊断BCS下腔静脉病变的常规序列。     

布-加综合征的影像诊断及综合介入治疗

目的回顾性分析31例门诊疑诊为布加综合征患者的诊疗过程,探讨布加综合征的影像学诊断方法及影响介入疗效的因素。方法31例门诊疑诊为布加综合征的患者行DSA,17例证实为布加综合征,其中16例行介入治疗。结果16例行介入治疗的病人技术上均获成功。治疗采用单纯球囊扩张7例,放置下腔静脉支架4例(5枚),放置肝静脉支架1例,下腔静脉支架结合下腔静脉滤器1例,单纯溶栓1例,行TIPSS术2例。术后30天症状明显改善13例,无变化1例,死亡2例。结论超声可作为布加综合征的筛查手段,最终确诊要依靠DSA。介入术后开通的静脉血管再闭塞是影响布加综合征疗效的主要因素。     

Sonography in Budd-Chiari syndrome.

OBJECTIVE: The objective of this presentation is to provide an overview of sonographic manifestations of Budd-Chiari syndrome (BCS). METHODS: Patients were scanned with ultrasound systems using mainly a 2- to 5-MHz curvilinear transducer and in some patients a 5- to 12-MHz linear transducer. The patients were asked to fast from the previous night or for at least 6 hours. Color and spectral Doppler sonography was performed in all patients. RESULTS: Commonly seen findings in BCS include inferior vena cava (IVC) webs and thrombi, IVC narrowing, hepatic venous thrombosis, enlarged caudate lobes, ascites, intrahepatic or extrahepatic collaterals, monophasic to absent flow in the hepatic veins, and high flow velocities in areas of stenosis in the IVC or hepatic veins. Inferior vena cava stents used in the treatment of BCS could also be seen. CONCLUSIONS: Budd-Chiari syndrome is an uncommon disorder; outcome is poor in many cases; and the condition is often misdiagnosed or underdiagnosed. Sonography is a noninvasive and effective modality for diagnosis of BCS.     

下腔静脉塌陷指数筛查肺栓塞的价值

目的　探讨超声测量下腔静脉塌陷指数对肺栓塞筛查的价值。方法　利用二维超声、彩色多普勒超声对 3 1例急性、慢性肺栓塞患者和 3 1例常规体检者进行下腔静脉塌陷指数测量。选择空腹检查条件 ,患者取仰卧位 ,充分显示距下腔静脉入右心房 2cm以内纵断面的管腔结构 ,以彩色多普勒超声证实此段血流通畅 ,而后测量吸气、呼气时下腔静脉的内径。结果　 3 1例急性、慢性肺栓塞患者下腔静脉塌陷指数下降占 19例 (5 9.4% )。大块肺栓塞组、非大块 /次大块肺栓塞组和正常对照组的呼气末下腔静脉内径、吸气末下腔静脉内径和下腔静脉塌陷指数分别为 (1.78± 0 .70 )cm ,(1.49± 0 .61)cm ,0 .14 8± 0 .0 974;(1.0 6± 0 .3 8)cm ,(0 .5 0± 0 .2 1)cm ,0 .5 17± 0 .0 75 ;(2 .3 7± 0 .3 4)cm ,(0 .46± 0 .2 2 )cm ,0 .80 5± 0 .115。结论　无创伤性超声技术对急性、慢性肺栓塞筛查具有较高特异性 ,单独根据下腔静脉塌陷指数判断肺栓塞可能会出现较高的假阴性。     

Numerical simulation of hemodynamics in membranous obstruction of the suprahepatic inferior vena cava based on a subject-specific Budd-Chiari syndrome model

BackgroundThis study was performed to determine the hemodynamic changes of Budd-Chiari syndrome when the inferior vena vein membrane is developing.MethodsA patient-specific Budd-Chiari syndrome vascular model was reconstructed based on magnetic resonance images using Mimics software and different degrees (16%, 37%, and 54%) of idealized membrane were built based on the Budd-Chiari syndrome vascular model using Geomagic software. Three membrane obstruction Budd-Chiari syndrome vascular models were established successfully and fluent software was used to simulate hemodynamic parameters, including blood velocity and wall shear stress.FindingsThe simulation results showed that there is low velocity and a low wall shear stress region at the junction of the inferior vena cava and the branches of the hepatic veins, and swirl may occur in this area. As the membrane develops, the size of the low velocity and low wall shear stress regions enlarged and the wall shear stress was increased at the membrane region. There was a significant difference in the mean values of wall shear stress between the different obstruction membrane models (P < 0.05).InterpretationHemodynamic parameters play an important role in vascular disease and there may be a correlation between inferior vena cava wall shear force changes and the slow development process of the inferior vena cava membrane.     

布-加综合征合并上腔静脉阻塞诊治分析

目的探讨布一加综合征合并上腔静脉阻塞的诊断与治疗方法。方法本组3例布一加综合征术前均经腹部彩超及磁共振静脉成像检查确诊，上腔静脉阻塞经上腔静脉造影证实。3例下腔静脉均狭窄闭塞行球囊扩张成形术；例1上腔静脉入右房口处狭窄采用球囊单纯扩张，例2、3因无明显上腔静脉阻塞症状且上腔静脉完全闭塞导丝无法通过而未行介入处理。结果3例介入手术后下腔静脉压力分别由术前的23．33、25．88和17．55mmHg降至9．60、9．60和7．20mmHg。例1上腔静脉压力由术前16．58mmHg降至术后6．98mmHg。3例术后皆恢复顺利，出院。随访症状完全消失、肝肾功能恢复正常。结论对布一加综合征患者术前应充分了解上腔静脉通畅情况，避免漏诊上腔静脉阻塞。对上腔静脉阻塞症状较轻或无症状者可不予处理，症状较重者应根据病因进行治疗。     

Recurrent acute Budd–Chiari syndrome after right hepatectomy: US color-Doppler vascular pattern and left hepatic vein stenting for treatment

After extended right hepatectomy remnant liver can be affected by outflow obstruction due to torsion of the inferior vena cava or kinking of the left hepatic vein. Remnant liver fixation is therefore suggested to avoid postoperative acute Budd–Chiari syndrome. Despite remnant liver reposition during surgery, a 76-years-old woman developed complete outflow obstruction. This clinical situation, due to left hepatic vein kinking, was suspected by US examination and confirmed by CT scan that showed a pathological intrahepatic vascular pattern. Patient required urgent relaparotomy and the liver was replaced in normal position. However, recurrence of outflow obstruction occurred and it was ultimately treated by inferior vena cava angiogram with left hepatic vein stenting.     

Relief of Budd-Chiari syndrome by primary placement of a metallic stent in the inferior vena cava: Case report

Summary. A case of Budd-Chiari syndrome with associated lower limb oedema due to concomitant inferior vena caval and hepatic venous thrombosis is presented. Percutaneous placement of a Wallstent through the occluded vena cava resulted in resolution of both the lower limb oedema and the hepatic vein thrombosis. In this instance recannalization of the inferior vena cava alone resulted in improvement of his liver function.     

Computational fluid dynamics simulation of time-resolved blood flow in Budd-Chiari syndrome with inferior vena cava stenosis and its implication for postoperative efficacy assessment

BackgroundThis study aimed to adopt computational fluid dynamics to simulate the blood flow dynamics in inferior vena cava stenosis based on time-dependent patient-specific models of Budd-Chiari syndrome as well as a normal model. It could offer valuable references for a retrospective insight into the underlying mechanisms of Budd-Chiari syndrome pathogenesis as well as more accurate evaluation of postoperative efficacy.MethodsThree-dimensional inferior vena cava models of Budd-Chiari syndrome patient-specific (preoperative and postoperative) and normal morphology model were reconstructed as per magnetic resonance images using Simpleware. Moreover, computational fluid dynamics of time-resolved inferior vena cava blood flow were simulated using actual patient-specific measurements to reflect time-dependent flow rates.FindingsThe assessment of the preoperative model revealed the dramatic variations of hemodynamic parameters of the stenotic inferior vena cava. Moreover, the comparison of the preoperative and postoperative models with the normal model as benchmark showed that postoperative hemodynamic parameters were markedly ameliorated via stenting, with the attenuation of overall velocity and wall shear stress, and the increase of pressure. However, the comparative analysis of the patient-specific simulations revealed that some postoperative hemodynamic profiles still bore some resemblance to the preoperative ones, indicating potential risks of restenosis.InterpretationComputational fluid dynamics simulation of time-resolved blood flow could reveal the tight correlation between the hemodynamic characteristics and the pathological mechanisms of inferior vena cava stenosis. Furthermore, such time-resolved hemodynamic profiles could provide a quantitative approach to diagnosis, operative regimen and postoperative evaluation of Budd-Chiari syndrome with inferior vena cava stenosis.     

布-加综合征肝脏病理学与肝静脉和下腔静脉阻塞类型的相关性研究

目的:探讨布-加综合征肝脏病理学改变与肝静脉、下腔静脉病变的关系。方法:27例布-加综合征患者术前行彩超检查,术中常规行下腔静脉造影和选择性肝静脉造影或经皮肝穿刺肝静脉造影了解肝静脉、下腔静脉阻塞情况;影像引导下经皮肝穿刺组织活检了解肝脏病理学变化。根据病变累及血管分为肝静脉型、下腔静脉型、肝静脉和下腔静脉混合型;根据血管阻塞程度将其分为狭窄型和完全闭塞型。将肝脏病理学改变与肝静脉、下腔静脉阻塞情况进行相关性分析。结果:25例穿刺组织达满意组织学诊断要求,其中肝静脉阻塞型11例,下腔静脉阻塞型4例,肝静脉和下腔静脉混合阻塞型10例;狭窄型7例,完全闭塞型18例。肝纤维化程度与血管阻塞程度密切相关;肝血窦扩张和肝细胞变性与血管阻塞程度无关,肝脏病变与阻塞部位无相关性。结论:肝静脉和下腔静脉阻塞对肝脏的损害程度是一致的,血管阻塞程度较阻塞部位对肝脏损害的影响更大。     

定量型声辐射力脉冲弹性成像评价慢性心力衰竭患者肝脏弹性

目的 探讨定量型声辐射力脉冲弹性成像（ARFI）评估慢性心力衰竭（简称心衰）患者肝脏弹性的应用价值。方法 对30例左心衰患者（左心衰组）、30例右心衰患者（右心衰组）及30名健康志愿者（对照组）行常规超声检查及血液生化指标检测,并以定量型ARFI检测肝脏杨氏模量值。分析慢性心衰患者肝脏杨氏模量值与生化指标、下腔静脉内径、肝静脉内径及肝右叶最大斜径的相关性。结果 左心衰组、右心衰组肝脏杨氏模量值均高于对照组（P均<0.05）,且右心衰组高于左心衰组（P<0.05）。心衰患者肝脏杨氏模量值与脑钠肽、总胆红素、γ-谷氨酰基转移酶、碱性磷酸酶、下腔静脉内径、肝左静脉内径、肝中静脉内径及肝右静脉内径均呈正相关（r=0.325、0.382、0.355、0.379、0.451、0.445、0.395、0.645,P均<0.05）,与左心室射血分数、肝右叶最大斜径无明显相关（P均>0.05）。结论 定量型ARFI可用于评估慢性心衰患者肝脏弹性,且肝脏弹性与心衰严重程度相关。     

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.     

B-TFE与3D DCE-MRA联合应用在布加综合征诊断中的价值

目的探讨B-TFE与3D DCE-MRA联合应用在布加综合征（BCS）诊断中的价值。材料与方法搜集经DSA证实的布加综合征患者32例,其中8例为单纯下腔静脉阻塞,2例为单纯肝静脉阻塞,22例为下腔静脉阻塞伴肝静脉阻塞。32例患者均行B-TFE和3D DCE-MRA检查,分析两种方法及联合应用时对BCS及其侧支循环的显示情况。结果 B-TFE正确诊断8例下腔静脉阻塞、18例下腔静脉阻塞伴肝静脉阻塞和2例肝静脉阻塞,总检出率为87.5%（28/32）;3D DCE-MRA正确诊断8例下腔静脉阻塞、19例下腔静脉阻塞伴肝静脉阻塞和1例肝静脉阻塞,总检出率为93.8%（30/32）。B-TFE共显示个193侧支循环,3DDCE-MRA则显示248个侧支循环。两者结合准确诊断全部BCS及其肝内外侧支循环。结论联合应用B-TFE和3DDCE-MRA是诊断BCS的理想方法。I     

胎儿下腔静脉离断的超声诊断分析

[目的]探讨产前超声诊断胎儿下腔静脉离断的价值。[方法]对2007年3月至2012年12月在本院产前超声系统筛查201069例孕妇,诊断胎儿下腔静脉离断34例的声像图特征及漏诊原因进行分析。[结果]胎儿下腔静脉离断诊断率为0．17‰,34例中内脏异位综合征11例（其中左侧异构9例,右侧异构2例）,完全性内脏反位2例;合并心脏畸形18例（房室间隔缺损4例,法洛四联症3例,永存动脉干3例,左上腔静脉3例,右室双出口1例,房室间隔缺损＋肺静脉异位引流1例,法洛四联症＋左上腔静脉1例,永存动脉干＋肺静脉异位引流1例,右室双出口＋肺静脉异位引流1例）;漏诊3例,2例在孕23、24周筛查时漏诊,孕32周复查时检出;1例出生后诊断,随访至今5年余,患儿生长发育正常,无异常临床表现。[结论]上下腔静脉长轴切面、胸腹部斜冠状切面、四腔心切面及上腹部横切面是筛查胎儿下腔静脉离断的重要切面,对产前超声诊断胎儿下腔静脉离断具有重要的临床价值。     

下腔静脉离断产前超声诊断

目的 探讨胎儿下腔静脉离断产前超声声像图特征和有效诊断切面,以提高其产前超声诊断准确率.方法 回顾性分析我院2006年1月至2010年5月诊断的10例下腔静脉离断胎儿(其中7例经引产后尸体解剖证实)产前超声声像图,与正常超声声像图对比,总结其产前超声声像图特征及有效切面.结果 10例下腔静脉离断胎儿,9例合并严重心内结构畸形,7例合并心外结构畸形(其中5例合并内脏反位).本组10例下腔静脉离断胎儿产前超声声像图均表现为四腔心切面异常(奇静脉扩张)、上腹部横切面异常(正常下腔静脉不能显示)、胸腹部斜冠状切面异常[离断的下腔静脉异位连接于奇(半奇)静脉并穿过膈肌连于腔静脉]及右心房纵切面异常(肝上段下腔静脉入右心房,肾上段肝段间的下腔静脉缺失并离断).结论 下腔静脉离断常合并心内、外结构畸形.下腔静脉离断胎儿在四腔心切面、上腹部横切面、胸腹部斜冠状切面及右心房纵切面上均有特征性超声声像图表现.识别下腔静脉离断特征性超声声像图表现可明显提高其产前超声检出率和诊断准确率.     

CT及MRI诊断子宫平滑肌瘤病累及下腔静脉及右心腔

目的 观察子宫平滑肌瘤病累及下腔静脉及右侧心腔的CT及MRI影像学表现。方法 收集8例病理诊断为子宫平滑肌瘤病累及下腔静脉及右侧心腔患者,回顾性分析术前CT及MRI表现。结果 子宫平滑肌瘤病累及下腔静脉及右侧心腔CT及MRI影像学特点:病灶大部分游离于静脉或心腔内,中等不均匀强化,病变与子宫静脉平滑肌瘤原发灶或复发灶相延续;累及右心腔时可见似"拐杖头"或"蛇头"状改变。子宫平滑肌瘤病的特异性表现为子宫或宫旁血管增粗、纡曲成团。结论 CT及MRI不仅可显示子宫平滑肌瘤病累及下腔静脉及右心受累范围,同时可以检出盆腔原发或复发病变,对指导治疗及评价预后有重要意义。     

布-加综合征的诊断与治疗(附46例临床分析)

目的：探讨减少布－加综合征的误诊误治措施。方法：分析布－加综合征（Budd-Chiari syndrome)46例的临床资料,结果①33例有误诊经过,平均误诊时间5．3年;②下肢病变为本病的主要表现,占84．8％;③彩色超声多普勒探查肝静脉及下腔静脉,100％均有阳性征象发现,而误诊的病例均未做该项检查;④仅4．3％患者的病毒标记为阳性,不超过一般人群阳性率。结论：布－加综合征症状多变,超声多普勒肝静脉及下腔静脉是筛查该病的重要手段。