中国河南地区Budd-Chiari综合征病变特点的影像分析

目的 探讨中国河南省Budd-Chiari综合征患者肝静脉阻塞和下腔静脉阻塞病变的分布特点.方法 应用3种血管成像技术,彩色多普勒超声、CTA和MRA检查,经DSA证实的Budd-Chiari综合征患者231例,对影像结果进行分析,分析清晰显影的主肝静脉、副肝静脉和下腔静脉走行与阻塞部位分布特点.结果 231例中肝静脉分支都正常的单纯性下腔静脉阻塞5例,占2.2%;下腔静脉正常的单纯性肝静脉阻塞33例,占14.3%;下腔静脉和肝静脉同时阻塞的193例,占83.5%.结论 中国河南地区Budd-Chiari综合征以下腔静脉阻塞合并肝静脉阻塞的复合型病变最多见,而单纯性下腔静脉阻塞最少见.     

应用血管造影鉴别诊断误诊为巴德-吉亚利综合征的隐匿性缩窄性心包炎

目的探讨应用血管造影技术鉴别诊断误诊为巴德-吉亚利综合征的缩窄型心包炎患者。 方法回顾性分析378例以"巴德-吉亚利综合征"为早期诊断的病例,对所有病例均进行常规血管造影检查和术中测压。 结果在所有病例中有5例患者血管造影检查显示下腔静脉和肝静脉管腔通畅,但压力明显高于正常,右心房压力也明显高于正常,定期随访1～24个月,这5例患者均发现不同程度心包钙化,最终进行心包部分或全部剥离术。病理结果:结核性心包炎4例,化脓性心包炎1例,支持缩窄型心包炎诊断。 结论缩窄性心包炎部分临床病例症状不典型易误诊,建议有条件单位可应用血管造影技术进行下腔静脉、肝静脉、心脏各房室造影和测压明确有无上述血管疾患。     

Budd-Chiari综合征的CT与超声、静脉造影对比研究

目的：评价ＣＴ、超声、静脉造影对Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的诊断价值。方法：回顾性分析４４例Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的ＣＴ、超声、静脉造影表现,比较三种检查方法显示肝脏的形态、肝静脉、下腔静脉、肝内外的侧枝血管情况。结果：ＣＴ显示肝尾叶增大４２例（９５％）,肝脏密度不均或呈低密度表现,１９例（４３％）显示有肝内侧枝血管,４２例（９５％）见有肝外侧枝血管,其中奇静脉扩张３５例（８０％）,半奇静脉扩张３９例（８９％）,下腔静脉钙化７例（１６％）。超声显示３８例（８６％）有下腔静脉狭窄或阻塞,１６例（３６％）有肝静脉狭窄或阻塞,３７例（８４％）显示有肝内侧枝血管,１６例（３６％）显示有肝外侧枝血管。静脉造影显示下腔静脉狭窄或阻塞２３例（５３％）,肝静脉狭窄或阻塞５例（１１％）,其余１６例（３６％）同时累及下腔静脉和肝静脉,３８例（８４％）显示有肝内侧枝血管,４４例（１００％）见有肝外侧枝血管。结论：ＣＴ对显示肝脏形态、下腔静脉钙化、肝外侧枝血管尤其是奇静脉和半奇静脉扩张有优势,超声则对显示下腔静脉和肝静脉狭窄或阻塞、肝内侧枝血管有优势,ＣＴ、超声和静脉造影相互补充有助于本病的正确诊断     

Budd-Chiari综合征的磁共振影像分析

目的：探讨MRI对Budd-Chiari综合征的诊断价值.方法：回顾性分析24例经DSA证实的Budd-Chiari综合征MRI表现.所有病例均采用FSPGR T1和FRFSE T2序列扫描.其中12例行FSPGR T1动态增强扫描.结果：肝静脉型6例,下腔静脉型8例,混合型10例,其中合并肝静脉或下腔静脉血栓6例.急性6例,亚急性6例,慢性12例.所有病例均有不同程度的肝肿大.肝脏尾叶增大20例,肝实质信号不均匀12例,肝内侧支血管17例,副肝静脉2例,肝外侧枝血管16例,脾大腹水16例,合并肝癌和脾梗死各1例.结论：MR能直接显示下腔静脉和肝静脉的狭窄,明确诊断并分型.并能根据不同时期信号特点和侧支血管分布的影像特征对其进行分期,判断疾病的病程和预后.MR是一个独立的非侵袭性的多方面评价Budd-Chiari综合征的方法.     

布-加综合征的CT诊断

目的探讨布-加综合征的CT影像特征及CT诊断价值。方法对11例经DSA证实的布-加综合征及2例误诊为布-加综合征的病例的CT图像进行回顾性分析。结果肝脏体积增大伴尾叶增大9例,下腔静脉病变11例,肝静脉病变3例。结论CT对下腔静脉病变导致的布-加综合征有较高的诊断价值,但对肝静脉病变的诊断价值有限。     

CT diagnosis of primary Budd-Chiari syndrome--membranous obstruction of the inferior vena cava

Five cases with primary Budd-Chiari syndrome due to membranous obstruction of the hepatic segment of the inferior vena cava were examined by CT. In all cases, CT demonstrated caudate lobe enlargement, reticular low density within the liver parenchyma, splenomegaly, and collaterals via the ascending lumbar veins and azygous system. Pathological study revealed liver cirrhosis or fibrosis in all cases. In two cases, calcification was shown in the region of the hepatic segment of the inferior vena cava. Our results suggested that the CT appearance of primary Budd-Chiari syndrome was rather characteristic and useful in diagnosis, although membranous obliteration could not be shown directly on CT.     

Budd-Chiari syndrome: a review of imaging findings

Budd-Chiari syndrome is an uncommon, often fatal disorder resulting from an obstructed hepatic venous outflow tract. The obstructive lesion is situated in the main hepatic veins, in the inferior vena cava or in both. The nature, location and extension of the obstruction can be displayed on diagnostic imaging techniques. In addition to this direct evidence, the indirect findings of venous obstruction such as the presence of intra- and extrahepatic collateral veins, when combined with the altered morphology and enhancement pattern of the liver enables one to arrive at a confident diagnosis. In patients with suspected Budd-Chiari syndrome, gray-scale sonography with complementary support of color and pulsed Doppler examinations is the first step in approaching the diagnosis. It is followed by a contrast-enhanced cross-sectional technique, preferrentially by MR angiography. The patients with a high clinical suspicion of Budd-Chiari syndrome may undergo hepatic venography or venacavography directly so that a potential of recanalization (e.g. percutaneous transluminal angioplasty with or without stent placement or TIPS) of the obstructed segment under the guidance of these techniques would not be delayed.     

Budd-Chiari syndrome: CT observations

The authors describe four patients with Budd-Chiari syndrome in whom contrast material-enhanced computed tomographic (CT) scans demonstrated low-density venous thrombosis in three sites not, to our knowledge, previously described with this modality. Thrombosis was seen in the portal circulation, the hepatic veins, and the intrahepatic inferior vena cava. It is known that concomitant portal vein thrombosis may be seen in 20% of patients with Budd-Chiari syndrome. Three of the four patients in the current study had this finding, one with extensive thrombosis of portal, mesenteric, and splenic veins and the other two with portal vein branch involvement. In one patient hepatic vein thrombosis was demonstrated with CT, and in three inferior vena cava clot was demonstrated. All four patients had the distinctive hepatic parenchymal contrast enhancement pattern seen in this condition, which the authors think may be at least partially caused by associated portal thrombosis. The presence of portal venous thrombosis should prompt the observer to consider the diagnosis of Budd-Chiari syndrome. Detection of hepatic vein clot confirms the diagnosis and may be seen in this condition in association with inferior vena cava thrombus.     

布加综合征的CT和MRI诊断

布加综合征(BCS)是一种因肝静脉流出道阻塞而导致的少见病变,本文对BCSCT和MRI表现进行了综述,CT和MRI检查可以显示直接征象即静脉阻塞的部位和范围,对于肝内外的侧支血管、肝尾叶增大、肝脏不均匀强化、再生结节等静脉阻塞间接表现也能较好显示,认识这些表现有助于病变的早期诊断和选择合适的治疗方法。     

64层螺旋CT门腔静脉同步成像技术对布-加综合征的诊断价值

目的:探讨64层螺旋CT双通路注射法行门腔静脉同步成像技术在布-加综合征(BCS)诊断中的应用价值。方法:选择行64层螺旋CT双通路注射法造影并经数字减影血管造影(DSA)证实的20例BCS患者,对其影像资料进行回顾性分析。结果:①血管重建显示:单纯下腔静脉受累7例;单纯肝静脉受累5例;下腔静脉伴肝静脉受累4例;下腔静脉血栓、外压性狭窄病变4例。与DSA进行比较,符合率达95%,二者具有较好的相关性,r值=0.966。②肝实质动态增强扫描显示:典型斑片样强化12例;3例不典型强化;5例肝实质未见明显异常强化。结论:64层螺旋CT双通路注射法行门腔静脉同步成像技术在诊断BCS中有较高的临床应用价值,同时能更好地显示门静脉与下腔静脉,准确、完整地显示BCS的梗阻部位和性质。     

Budd-Chiari syndrome: spectrum of imaging findings

OBJECTIVE: The objective of our study was to illustrate the imaging findings of Budd-Chiari syndrome, including CT, MRI, sonographic, and angiographic findings. CONCLUSION: The key imaging findings in Budd-Chiari syndrome are occlusion of the hepatic veins, inferior vena cava, or both; caudate lobe enlargement; inhomogeneous liver enhancement; and the presence of intrahepatic collateral vessels and hypervascular nodules. Awareness of these findings is important for early diagnosis and appropriate treatment.     

Budd-Chiari syndrome: value of duplex sonography and colour Doppler imaging for diagnosis and follow-up

This retrospective study was designed to evaluate duplex sonography in the diagnosis and follow-up of patients with Budd-Chiari syndrome. Thirteen patients with clotting disease and histologically proven Budd-Chiari syndrome (3 acute and 10 chronic cases) were examined, using conventional duplex sonography (n=5) or colour-coded duplex sonography (n=8). Results were compared with CT in 6 cases, MRI in 11, coeliac and mesentric angiography in 5, and hepatic wedge venography and cavography in 6. Of 39 hepatic veins examined, 33 had an abnormal appearance on ultrasound studies: endoluminal thrombus, stenosis, dilatation, thick wall echoes. Doppler findings included total obstruction of 8 hepatic veins, reversed flow in 22 hepatic veins and intrahepatic collaterals with continous non-phasic flow in all cases. Duplex sonography showed portal thrombosis (n=1), evidence of portal hypertension (n=8) and inferior vena cava thrombosis (n=2). Nine patients were treated surgically. Patency of portocaval shunts was correctly assessed with colour coded duplex sonography in 8 of 9 patients. We believe that colour Doppler imaging is the procedure of choice for initial diagnosis of Budd-Chiari syndrome. Correspondence to: M. F. Bellin     

Radionuclide venography as a clue to the diagnosis of Budd-Chiari syndrome

A patient was examined with radionuclide venography (RVG) to investigate unilateral leg oedema which might be due to deep vein thrombosis. RVG with Tc-99m MAA demonstrated no findings to suggest deep vein thrombosis of the right leg. However, collateral flow derived from the left common iliac vein and truncated inferior vena cava (IVC) were revealed. Contrast venography confirmed the obstruction of IVC and collateral flow from the left common iliac vein to the left ascending lumbar vein. It also showed the obstruction of hepatic veins and the patient was finally diagnosed as Budd-Chiari syndrome. Although unilateral leg oedema is an atypical symptom in Budd-Chiari syndrome, the findings on RVG led us to conduct further imaging studies to reach the diagnosis.     

三维薄层动态增强MRI在原发性Budd-Chiari综合征诊断及其分型中的价值

目的：探讨三维薄层动态增强MRI检查对原发性Budd-Chiari综合征的显示和分型价值。方法：14例原发性Budd-Chiari综合征患者均行MRI检查,采用肝脏加速容积采集序列（LAVA）,分析图像质量及其对下腔静脉、肝静脉和侧支循环的显示情况。结果：14例中下腔静脉阻塞型4例,其中膜型1例,节段性3例;肝静脉阻塞型5例;混合型阻塞5例。尾状叶增大9例,肝实质强化不均匀8例,5例显示有副肝静脉,7例可见肝内侧支血管,9例可见肝外侧支血管。结论：三维薄层动态增强MRI技术能清楚显示肝静脉和下腔静脉的正常解剖和各种病变,对诊断原发性Budd-Chiari综合征的病变部位、程度、侧支分布及分型均具有较高价值。     

Budd-Chiari综合征的MRI诊断

目的 探讨ＭＲＩ对Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的诊断价值。材料与方法 分析１９例Ｂｕｄｄ－Ｃｈｉａｒｉ综合征的ＭＲＩ表现,并与超声和静脉造影进行对比。结果 肝脏增大１８例（９５％）,肝尾叶增大１４例（７４％）,肝脏信号不均匀１４例（７４％）,７例（３７％）显示下腔静脉阻塞,４例（２１％）下腔静脉内见有血栓,肝静脉狭窄或阻塞１９例（１００％）,１７例（８９％）显示有肝内侧支血管,１７例（８９％）显示     

Membranous obstruction of the inferior vena cava with Budd-Chiari syndrome: MR imaging findings.

Membranous obstruction of the inferior vena cava (IVC) is a curable cause of a primary type of Budd-Chiari syndrome. Magnetic resonance (MR) imaging and vena cavography were performed on nine patients with membranous obstruction of the IVC. The MR findings were retrospectively analyzed and compared with computed tomographic findings in seven patients. The morphologic features of membranous obstruction of the IVC on spin-echo MR images were a curvilinear soft-tissue membrane (five cases) or an obliterated lumen of a hepatic segment of the IVC (four cases) in transverse or sagittal views. The lumen below the obstruction revealed flow-related signal (seven cases), intraluminal thrombus (one case), and thrombotic occlusion (one case). The hepatic veins were narrow and disoriented without connection to the hepatic segment of the IVC just below the diaphragm. On T2-weighted images, inhomogeneity with high signal intensity was shown more prominently in the hepatic parenchyma in Simson type II or III membranous obstruction. Other findings were hepatosplenomegaly, enlarged caudate lobe, cirrhotic liver, associated hepatoma, and presence of various collaterals.     

土三七致肝窦阻塞综合征的CT及MRI诊断

目的探讨土三七致肝窦阻塞综合征(HSOS)的CT及MRI的表现与特征。 方法收集经临床或肝穿刺活检病理证实的服用土三七致HSOS的初诊患者20例。其中男性19例,女性1例;其中12例经肝组织活检证实,8例经临床表现、土三七药用史及辅助检查综合诊断。20例患者均有外伤、脑卒中后服用土三七病史,服药后10天~6个月出现临床症状。回顾性分析患者的临床资料、腹部CT及MR主要表现。 结果20例患者中有14例患者行CT检查,11例患者行MRI检查。CT平扫显示肝实质密度不均,增强扫描门脉期、延迟期肝实质灌注不均,呈地图样、花斑样强化;MRI平扫较CT平扫可以更清楚显示肝实质密度情况,沿肝静脉三大分支走行处的肝实质信号较其余部分肝实质信号稍高并更均匀,三支肝静脉纤细或显示不清,肝段下腔静脉静脉纤细或扁平,但下腔静脉及肝静脉仍然通畅;此外,CT与MRI均能显示门脉周围水肿,门脉高压症表现。 结论土三七致HSOS的CT及MRI表现具有一定的特征;CT、MRI检查在复查及评价临床疗效中具有重要作用;MRI检查可较好评价肝实质损伤程度及肝内静脉通畅情况,CT图像重建可清楚显示下腔静脉及肝内主要血管,二者在诊断及鉴别诊断中具有重要作用,结合患者土三七药用史及病理检查,有助于HSOS诊断。     

Membranous obstruction of the inferior vena cava: comparison of findings at sonography, CT, and venography.

OBJECTIVE. Membranous or segmental obstruction of the inferior vena cava is one of the common causes of chronic Budd-Chiari syndrome. In this study, the venographic findings are compared with the results of sonography and CT in order to ascertain their role in the management of these cases. MATERIALS AND METHODS. Fifteen patients with membranous (n = 8) or segmental (n = 7) obstruction of the inferior vena cava who had been examined with sonography and CT were studied retrospectively. Diagnosis was made at surgery (n = 3) or by venacavography (n = 12). Sonographic findings were analyzed on the basis of the initial report, and CT findings were reviewed retrospectively with knowledge of the sonographic findings. RESULTS. Sonography showed membranous obstruction (n = 5), segmental cordlike obstruction (n = 3), and unspecified obstruction (n = 5) of the inferior vena cava, while CT showed a flap of the membrane (n = 1) and segmental narrowing or obstruction of the inferior vena cava (n = 7). In the remaining cases, the inferior vena cava either appeared normal (n = 6) or was not visualized (n = 1) on CT or was not described in the sonographic report (n = 2). In nine cases, CT showed one or several tiny calcific foci in the inferior vena cava. Sonography showed obliteration of at least one hepatic vein (n = 8) and of intrahepatic collateral vessels (n = 12), whereas CT was less sensitive in evaluating obliteration of intrahepatic veins (n = 4) and collateral vessels (n = 7). Sonography and CT both showed hepatic masses (n = 6), evidence of liver cirrhosis and portal hypertension (n = 14), hepatomegaly (n = 14), enlargement of the caudate lobe (n = 9), and intraabdominal (n = 11) and abdominal wall (n = 15) collateral vessels. CONCLUSION. Sonography was superior to CT in delineating pathologic venous anatomy of the inferior vena cava and hepatic veins whereas CT was better in evaluating hepatic cirrhosis and tumor. We believe that these techniques are useful complements to venography in the diagnosis and management of these cases.     

The Budd-Chiari syndrome: a review

Ascites, hepatomegaly, and abdominal pain constitute the classic triad of the Budd-Chiari syndrome of hepatic-vein or inferior-vena-cava obstruction. This condition was first mentioned by Budd in the mid 1800s and additional information was provided by Chiari in the 1890s. In nearly two-thirds of patients the exact etiology cannot be determined. The syndrome has, however, been associated with hypercoagulable states, neoplasms, trauma, medications, and congenital abnormalities. The diagnosis is difficult to make clinically; therefore, radiology plays a critical role in the workup of these patients. Nuclear medicine, sonography, CT, angiography, and MRI all provide valuable diagnostic information. These data combined with hepatic biopsy determine which patients should be treated by percutaneous angioplasty or surgery, and also determine the type of shunt to be performed (such as the mesoatrial shunt when the inferior vena cava is occluded or severely compressed). Noninvasive imaging is also useful in the follow-up of patients after both percutaneous angioplasty and surgery.     

Budd-Chiari综合征多层CT动态增强扫描及CT血管成像分析

目的探讨Budd-Chiari综合征(BCS)肝脏多层CT动态强化规律及形成机制，分析CT血管成像(CTA)在BCS诊断中的应用价值。方法经DSA证实的28例BCS患者，在DSA检查前1周内行多层CT动态增强扫描，并对其相关血管分别以最大密度投影法(MIP)、容积成像法(VR)和多平面重建法(MPR)进行血管重建。分析BCS患者的肝脏多层CT动态强化规律，并以DSA结果为对照，评价多层CT动态增强扫描及CTA技术在判断血管梗阻平面及显示肝内、外侧支循环中的价值。结果与DSA对照，28例BCS中多层CT扫描准确判断梗阻平面者26例，未能诊断者2例，为膈肌水平以上的下腔静脉膜性阻塞。22例肝静脉梗阻的患者中，CT动态增强扫描表现为肝脏典型斑片状强化者19例，不典型强化者3例，8例出现肝内良性结节，同时还显示门脉分支狭窄、僵直20例，肝动脉增粗14例，肝内侧支静脉开放20例。6例单纯性下腔静脉梗阻患者未出现肝脏异常表现。结论肝内、外侧支静脉的出现与梗阻部位有关。多层CT动态增强扫描能够准确地反映BCS患者肝脏血流动力学变化，结合CTA技术，能够更直观、准确地显示血管梗阻平面及肝内、外侧支循环。