Imaging of Budd-Chiari syndrome

Budd-Chiari syndrome occurs when venous outflow from the liver is obstructed. The obstruction may occur at any point from the hepatic venules to the left atrium. The syndrome most often occurs in patients with underlying thrombotic disorders such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria and pregnancy. It may also occur secondary to a variety of tumours, chronic inflammatory diseases and infections. Imaging plays an important role both in establishing the diagnosis of Budd-Chiari syndrome as well as evaluating for underlying causes and complications such as portal hypertension. In this review article, we discuss the role of modern imaging in the evaluation of Budd-Chiari syndrome.     

Radiation-induced liver injury showing low intensity on T2-weighted images noted in Budd-Chiari syndrome

PURPOSE: Although it is documented that radiation can cause density or intensity changes on computed tomography or MR imaging in the irradiated hepatic parenchyma, few researchers have reported or understood the MR presentation of changes in hepatic parenchyma following radiotherapy in the patient with Budd-Chiari syndrome. The purpose of this study was to investigate the MR appearance of hepatic radiation injury in Budd-Chiari syndrome and to consider the underlying pathophysiology. MATERIALS AND METHODS: The MR examinations of two patients with Budd-Chiari syndrome were compared with those of 11 patients without Budd-Chiari syndrome. The two groups, both of which suffered from hepatocellular carcinoma, underwent 50-72 Gy of proton-beam irradiation during a period of 14-43 days. Examinations including T1- and T2-weighted imaging, superparamagnetic iron oxide-enhanced imaging, and dynamic study were performed 3-10 weeks after the end of irradiation. RESULTS: Radiation-induced hepatic injury was observed as a low-intensity area on T2-weighted images and on delayed phase images of dynamic study in the Budd-Chiari patients, and as iso- or high-intensity areas on both images in the patients without Budd-Chiari syndrome. US-guided needle biopsy from the irradiated area in one patient with Budd-Chiari syndrome revealed mostly necrotic tissue and fibrous tissue. CONCLUSION: These MR features of hepatic radiation injury in Budd-Chiari syndrome were considered to be due to severe hepatic fibrosis.     

MRI of the Budd-Chiari syndrome

Five of six patients with angiographically proved Budd-Chiari syndrome (hepatic venous outflow obstruction) showed multiple specific MRI abnormalities: striking reduction in caliber or complete absence of the hepatic veins, "comma-shaped" intrahepatic collateral vessels, and/or marked constriction of the intrahepatic inferior vena cava. The sixth patient had angiographically proven sinusoidal hepatic venous obstruction and patent central hepatic veins; MRI showed ascites but revealed no specific features of the Budd-Chiari syndrome. Patients with end-stage cirrhosis also showed compressed, distorted hepatic veins; however, these cirrhotic livers were distinguished by their small size, nodular surface, and extrahepatic collateral varices. In patients without cirrhosis or the Budd-Chiari syndrome, normal hepatic, portal, and inferior caval veins were routinely seen when technically adequate MRI examinations were obtained (94 of 100 cases). Four of the six patients with Budd-Chiari syndrome had been treated surgically. In three, MRI identified patent portocaval shunts. In the fourth, angiographically confirmed shunt stenosis was demonstrated by MRI.     

Budd-Chiari综合征:下腔静脉闭塞钝性开通的技术探讨

目的探讨下腔静脉闭塞型Budd-Chiari综合征钝性开通技术临床应用的安全性。方法96例患者中下腔静脉膜性闭塞63例、下腔静脉节段性闭塞33例,其中15例合并肝静脉狭窄或闭塞。透视下,使用开通导丝开通闭塞段下腔静脉。结果96例技术操作全部成功,下腔静脉开通率100%,未出现心脏压塞、下腔静脉破裂、胸腔出血、腹腔出血等严重并发症。结论钝性破膜开通下腔静脉闭塞段操作简单、安全有效、费用低、无严重并发症,值得大力推广。     

布加综合征合并肝细胞癌的介入治疗

目的 探讨布加综合征合并肝细胞癌介入治疗的效果.方法 回顾性分析17例布加综合征合并肝细胞癌患者的临床资料.布加综合征经彩色多普勒超声及血管造影确诊;肝细胞癌5例由肝穿刺细胞学检查确诊,12例由彩色多普勒超声、CT或MRI及甲胎蛋白(AFP)进行综合诊断.所有患者均施行下腔静脉成形术及动脉化疗栓塞术(TACE).术后随访采用肝脏超声、CT及实验室检查,观察肿瘤大小、肝功能、AFP及下腔静脉通畅情况.采用t检验比较介入治疗前后压力变化.结果 17例患者共进行了39次介入治疗,均成功,无并发症.下腔静脉成形术后:患者随访2～90个月,1例下腔静脉再闭塞,经第2次血管成形术开通.TACE术后:2个月时随访,所有患者均生存;15例随访6个月,13例生存;12例随访12个月,10例生存;8例随访24个月,5例生存.介入治疗后,下腔静脉-右心房压力差由术学的(20.5±2.1)cm H2O(1cm H2O=0.098 kPa)降至(3.6±1.0)cm H2O,差异有统计学意义(t=30.32,P＜0.05).结论 布加综合征合并肝细胞癌的介入治疗可取得较好临床效果.     

Benign hepatic nodules in Budd-Chiari syndrome: radiologic-pathologic correlation with emphasis on the central scar

OBJECTIVE: The purpose of this study was to determine the imaging features of benign hepatic nodules in patients with Budd-Chiari syndrome and to correlate them with pathologic findings, with special attention placed on the presence of a central scar. MATERIALS AND METHODS: Imaging findings of 59 benign hepatic nodules in four patients with chronic Budd-Chiari syndrome were analyzed retrospectively, and radiologic- pathologic correlation was performed in three patients with 50 hepatic nodules who underwent liver transplantation. All patients underwent multiphasic helical CT. In three patients with 29 lesions, MR imaging, including a multiphasic dynamic study, was performed. The CT and MR imaging findings in these patients were compared with those of 103 small hepatocellular carcinomas in 56 other patients (54 of them displayed chronic hepatitis or liver cirrhosis associated with viral hepatitis but none had Budd-Chiari syndrome). Image analysis was performed by two radiologists with no knowledge of the diagnosis. RESULTS: All patients with Budd-Chiari syndrome exhibited multiple benign nodules up to 3 cm in diameter, and 42 of 59 lesions were hypervascular. Microscopically, 15 of 32 nodules demonstrated a central scar; moreover, some nodules closely resembled focal nodular hyperplasia. Frequencies of hyperintensity on T1-weighted images (14/29 vs 25/103), hypointensity on T2-weighted images (7/29 vs 1/103), and the presence of a central scar (6/59 vs 1/103) were significantly higher in benign nodules than in hepatocellular carcinomas (p < 0.05; Fisher's exact test). Moreover, for lesions larger than 1 cm, imaging studies revealed a central scar in six of 15 benign lesions. CONCLUSION: Benign hepatic nodules in patients with in Budd-Chiari syndrome are usually small, multiple, and hypervascular. The presence of a central scar is a characteristic feature in those larger than 1 cm in diameter.     

Budd-Chiari syndrome: spectrum of appearances of acute, subacute, and chronic disease with magnetic resonance imaging

The purpose of this study was to describe our collective experience in the magnetic resonance (MR) investigation of patients with proven acute, subacute, and chronic Budd-Chiari syndrome and to demonstrate the spectrum of appearances on T1- and T2-weighted as well as dynamic post-gadolinium spoiled gradient-echo imaging. All patients with proven Budd-Chiari syndrome who underwent MR examinations between June, 1992 and October, 1998 were included in the study. Fourteen patients were included in the study: four with acute, three with subacute, three with chronic, and four with acute superimposed on either subacute (two) or chronic (two) Budd-Chiari syndrome. MR imaging features were retrospectively evaluated to determine: a) liver morphology, b) pattern of signal intensity (SI) on T1-weighted images, c) pattern of SI on T2 weighted images, d) dynamic enhancement characteristics, e) presence or absence of visible venous thrombosis, and f) presence or absence of venous macroscopic collaterals. The MR findings were correlated with surgical, histopathological, and laboratory data to determine imaging characteristics related to the chronicity of the disease process. Hepatic venous thrombosis or absence of hepatic venous flow was demonstrated in all patients in the study. In the four patients with acute Budd-Chiari syndrome, the liver periphery was moderately low signal on T1 and moderately high signal on T2-weighted images relative to the central liver; both early and late gadolinium-enhanced images revealed diminished peripheral enhancement. In the three patients with subacute Budd-Chiari syndrome, the liver periphery was moderately low signal on T1, and moderately high signal on T2-weighted images, while early and late gadolinium-enhanced images revealed heterogenously increased enhancement within the liver periphery. In the three patients with chronic Budd-Chiari syndrome, the SI differences between peripheral and central liver were minimal on T1- and T2-weighted images, and enhancement differences were also minimal. Extensive bridging intrahepatic and capsular venous collaterals were visualized in chronic cases. In the four patients with acute Budd-Chiari syndrome superimposed on more chronic disease, a combination of gadolinium enhancement patterns was observed on MR images. Enhancement patterns between central and peripheral liver were different for acute, subacute, and chronic Budd-Chiari syndromes, suggesting differentiation between these phases of the disease process. Application of this pattern approach permitted recognition of acute changes superimposed on more chronic disease.     

Mesoatrial shunts for Budd-Chiari syndrome and inferior vena cava thrombosis: angiographic and hemodynamic evaluations

When inferior vena caval obstruction complicates the Budd-Chiari syndrome, conventional portosystemic shunts are not possible. The mesoatrial shunt has been devised to enable portal and sinusoidal decompression in these patients. Findings in 12 patients with Budd-Chiari syndrome and inferior vena caval obstruction in whom a mesoatrial shunt was performed are reported. Preoperative inferior vena cavography with pressure measurements is essential to determine the appropriate shunt procedure. Postoperatively, shunt patency is assessed with superior mesenteric arterial portography. Where possible, transvenous catheterization of the shunt is performed to confirm patency and assess hemodynamic function.     

腔内治疗幼儿Budd-Chiari综合征1例并文献分析

目的总结血管腔内治疗I型幼儿布一加综合征（Budd—Chiari syndrome,BCS）的经验,并回顾相关文献。方法对1例BCS患儿行下腔静脉（IVC）造影,根据造影结果选择应用介入方法经皮腔内血管成形术治疗,即用超滑导丝硬头穿过下腔静脉阻塞狭窄部位,进行球囊扩张。结果①球囊扩张操作成功,无并发症发生。术后造影见残余狭窄≤30％,远期疗效有待进一步随访。②近年有关幼儿BCS的报道极少,静脉血管造影为诊断此病的金标准。结论介入治疗具有微创、安全、有效,术后并发症少,恢复快的优点,为治疗Ⅰ型幼儿BCS的首选方法。     

Sonography of the caudate vein: value in diagnosing Budd-Chiari syndrome

OBJECTIVE: The aim of this study was to evaluate the usefulness of sonography in measuring the caudate vein and lobe when diagnosing Budd-Chiari syndrome. MATERIALS AND METHODS: Our study included a group of 16 patients with Budd-Chiari syndrome. We compared this patient group with a control group of 40 patients with cirrhosis and 66 healthy subjects without liver disease. On sonography, we measured the diameter of the caudate lobe and the presence and diameter of the caudate vein. These measurements were then compared for both groups. RESULTS: In half of the patients with Budd-Chiari syndrome, sonography showed a visible caudate vein (3-21 mm; mean, 7.3 mm; median, 4.6 mm). However, in the control group, spontaneous visualization was seen in only eight (7.5%) of 106 subjects, none of whom had cirrhosis. The caudate lobe was larger in patients with cirrhosis than in healthy subjects. Likewise, in patients with Budd-Chiari syndrome, the caudate lobe was larger than that in patients with cirrhosis. CONCLUSION: When sonography reveals a caudate vein equal to or larger than 3 mm in diameter in the appropriate clinical setting, one should strongly suspect Budd-Chiari syndrome.     

Budd-Chiari syndrome: typical and atypical scintigraphic aspects

Budd-Chiari syndrome, a well known entity, is often difficult to diagnose, mostly due to the nonspecificity of its symptomatology. Radiocolloid liver scans were evaluated in eight cases of this disease, proven by surgical biopsy. Five cases showed the "classic" scintigraphic pattern of caudate lobe hypertrophy (62.5%), and other abnormalities observed included segmental hepatic insufficiency, diffuse hepatic insufficiency, and relative hypertrophy of both the caudate lobe and a portion of the parenchyma of segment VI (one case each). An experimental study of hepatic venous drainage performed on livers at autopsy revealed four groups of accessory hepatic veins in addition to the main hepatic veins. The occlusion of various parts of this drainage appears to relate to the various scintigraphic patterns that were encountered in patients with Budd-Chiari syndrome. A review of the literature revealed three additional patterns previously reported in association with Budd-Chiari syndrome (normal scan, diffuse hepatomegaly, and multiple filling defects). If all these variations are appreciated, liver scanning can be a valuable screening tool for Budd-Chiari syndrome and may also serve as a noninvasive means of follow-up.     

Segmental obstruction of the hepatic inferior vena cava treated by transluminal angioplasty

Five patients with segmental obstruction of the hepatic inferior vena cava associated with Budd-Chiari syndrome were successfully treated by percutaneous transluminal angioplasty using Grüntzig balloon catheters. Three or four balloons were used simultaneously to achieve sufficient dilatation. Long-term patency was obtained in all 5 cases without serious complications. This procedure may be applicable to other portions of the venous system as well.     

Hepatic caudate vein in Budd-Chiari syndrome: Depiction by using magnetic resonance imaging

Objective

To study the visibility of the caudate vein and its diameter on MR imaging in healthy people and in patients with Budd-Chiari syndrome.

Materials and methods

In this study there were 14 patients with Budd-Chiari syndrome and 54 healthy subjects without hepatic lesion or liver disease, all of whom had upper abdominal enhanced MRI. The visibility of the caudate vein and its diameter on MR images was compared between Budd-Chiari patients and healthy subjects, and among Budd-Chiari patients, the correlation between the visibility of caudate vein and extrahepatic collaterals were compared.

Results

Caudate vein was noted in 64% of patients with Budd-Chiari syndrome and in 7% of healthy subjects (P = 0.000). The diameter of the caudate vein visualized on MR imaging in Budd-Chiari syndrome was significantly larger than that in healthy group (7.3 ± 3.9 mm vs 2.6 ± 0.6 mm, P = 0.037). Among Budd-Chiari patients, both caudate vein and extrahepatic collateral veins were noted in 9 patients, only extrahepatic collateral veins were noted in 4 patients and neither caudate vein nor extrahepatic collateral veins were noted in 1 patient. No correlation was found between the visibility of caudate vein and that of extrahepatic collateral vein in patients with Budd-Chiari (P = 0.375).

Conclusion

Gadolinium enhanced dynamic MR imaging can visualize hepatic caudate vein frequently. The visibility and dilation of hepatic caudate veins on MR imaging in Budd-Chiari syndrome were more frequent than in control subjects. MR depiction of a caudate vein may help differentiate Budd-Chiari from cirrhosis.     

Hepatic inferior vena cava obstructions: clinical results of treatment with percutaneous transluminal laser-assisted angioplasty

Three cases of hepatic inferior vena cava (IVC) obstruction (two segmental and one membranous) associated with Budd-Chiari syndrome were successfully treated with percutaneous transluminal angioplasty (PTA) with use of an Nd-YAG (neodymium-yttrium, aluminum, garnet) laser. The occluded portions were canalized by advancing a ceramic-capped delivery system and delivering intermittent laser emissions. The canal was widened by simultaneous inflation of three or four Gruentzig balloon catheters. In two of the three cases, this procedure was done after unsuccessful canalization of the occluded portions by conventional means. Postoperatively, all patients showed disappearance of Budd-Chiari syndrome. One patient also showed marked regression of a huge intraluminal thrombus. There were no serious complications during and after the procedures. Use of the Nd-YAG laser seems to be of value in PTA for the treatment of hepatic IVC obstructions as well as in treatment of arteriosclerotic lesions.     

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.     

三维DSA在腔静脉闭塞型布加综合征诊断和介入治疗中的价值

目的 评价三维DSA(3D DSA)在腔静脉闭塞型布加综合征诊断和介入治疗中的价值.方法21例下腔静脉(IVC)闭塞情况复杂的布加综合征患者经二维DSA(2D DSA)后前位检查确诊后,加做3D DSA检查.由2名介入放射专业主任医师采用双盲法分别阅读2D DSA和3D DSA图像以评价IVC解剖结构,并采用x2检验比较两者对血管的显示情况.根据2D和3D DSA检查结果施行IVC球囊扩张术或支架置入术.结果 所有患者3D DSA均能准确显示IVC闭塞端位置、形态、侧支血管开口及其空间位置关系,检出侧支血管起源于闭塞端9例;2D DSA能显示闭塞端位置、形态、侧支血管开口及其空间关系7例,检出侧支血管起源于闭塞端2例,两者比较差异均有统计学意义(x2值分别为12.07和5.14,P＜0.05).仿真血管内镜成像显示IVC内游离血栓3例、附壁血栓1例.全部患者均治疗成功,1例并发IVC破裂出血,无其他并发症.结论3D DSA在IVC闭塞的诊断中能提供有价值信息,对腔静脉闭塞型布加综合征介入治疗有指导意义.     

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。     

Percutaneous Sclerotherapy for Budd-Chiari Syndrome Secondary to Giant Hepatic Venous Malformations (Hemangiomas)

This prospective study evaluated the safety and effectiveness of percutaneous sclerotherapy in the treatment of secondary Budd-Chiari syndrome due to hepatic venous malformations (HVMs). Four patients (mean age, 40 years; 3 women) with 5 HVMs underwent 7 sessions of percutaneous sclerotherapy with a mixture of bleomycin and lipiodol. All patients had chronic Budd-Chiari syndrome, determined based on imaging findings, with the main symptom being abdominal discomfort and distention. On physical examination, 2 patients had ascites and the other 2 had an epigastric mass. The indication for treatment was intractable abdominal symptoms due to hepatic and/or inferior vena cava (IVC) outflow compression. All procedures were technically successful, with no major complications. Three patients underwent a second session because of incomplete IVC decompression. The patients’ symptoms completely resolved at 6 and 12 months of follow-up. There was a significant reduction in lesion volume (P = .007) and an increase in IVC luminal area (P = .018) at 12 months of follow-up.     

Hepatic nodules in Budd-Chiari syndrome: imaging features

PURPOSE: To analyze the imaging features of nodules associated with Budd-Chiari syndrome. MATERIALS AND METHODS: The authors retrospectively studied images obtained in 23 patients with liver nodules who were being followed up for Budd-Chiari syndrome. Doppler ultrasonography was performed in all patients, computed tomography in 16, and magnetic resonance (MR) imaging in 20. The following lesion features were evaluated: location, number, size, vascularization, qualitative signal intensity at MR imaging, and homogeneity. Nodules were diagnosed on the basis of histopathologic findings or clinical and biologic data with no change at imaging during 2-year follow-up. RESULTS: All patients had histopathologic features of chronic Budd-Chiari syndrome. Four patients had hepatocellular carcinoma (HCC), with one to three lesions. The mean diameter of the largest HCC lesion in each patient was 7.3 cm. All HCC lesions were heterogeneous and had high signal intensity on T2-weighted MR images. Nineteen patients had multiple benign regenerative nodules, most of which were smaller than 4 cm. Most nodules were homogeneous and hyperintense on T1- and T2-weighted images. In 15 patients, nodules were hypervascular in the arterial phase. CONCLUSION: In patients with chronic Budd-Chiari syndrome, multiple (> 10) small (< 4-cm) lesions are suggestive of benignity.     

布-加综合征支架植入术后血栓形成的监测和溶栓治疗

目的:探讨布-加综合征支架植入术后彩超监测血栓形成并行导管溶栓治疗的应用价值.方法:回顾性分析2000年1月至2007年1月期间180例布-加综合征患者支架植入术后的病历以及随访资料.结果:180例布-加综合征支架植入术后患者中,有血栓形成者21例(11.67%),其中彩超监测发现20例(95.24%),经及时导管溶栓治疗后效果良好,漏诊1例.结论:布-加综合征支架植入术后应该常规彩超监测以期及时发现血栓并行导管溶栓治疗,提高疗效.