Venoocclusive liver disease after bone marrow transplantation: findings at duplex sonography.

Ascites, thickening of the gallbladder wall, and reversal of portal flow are documented sonographic findings in venoocclusive disease of the liver. The frequency and specificity of these findings and their relationship to the severity of this disease have not been studied. In an attempt to clarify these issues, 65 patients who had bone marrow transplantations were prospectively studied with serial B-scans and duplex color Doppler sonography. For all patients, assessment included liver size and texture, thickening of the gallbladder wall (greater than 10 mm), and presence of ascites. Doppler flow velocity profiles were obtained from the portal vein, hepatic veins, and inferior vena cava. The hepatic artery resistive index (RI) was calculated. Twenty volunteers were also studied to establish normal flow values. Nineteen patients had documented venoocclusive disease, nine had hepatic graft-vs-host disease (GVHD) (five after proved venoocclusive disease), two had hepatitis, and 40 had no clinical or biochemical evidence of liver injury after bone marrow transplantation. Ascites (n = 16), thickening of the gallbladder wall (n = 8), hepatomegaly (n = 8), and altered liver texture (n = 3) were not distinguishing features of venoocclusive disease. Mean hepatic artery RI was as follows (ranges are in parentheses): control group, 0.69 (0.58-0.76); venoocclusive disease patients, 0.81 (0.75-0.87); GVHD patients, 0.69 (0.63-0.71); all other patients after bone marrow transplantation, 0.66 (0.61-0.71). The RI values in venoocclusive disease were significantly elevated, but an incremental rise in RI with increasing severity of the disease was not seen. Abnormalities in portal vein flow were seen in only two patients: in one with fatal venoocclusive disease, reversed portal flow developed, and in one with GVHD, portal vein thrombosis developed. Contrary to previous reports, no correlation between abnormalities in portal flow and venoocclusive disease was seen. Flow velocities in the hepatic veins and the inferior vena cava were not significantly different from values in the volunteer group. These results suggest that a significant elevation of the hepatic artery RI may be a sensitive index of liver damage related to venoocclusive disease after bone marrow transplantation and an important distinguishing sonographic feature.     

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     

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.     

Budd-Chiari syndrome: the results of duplex and color Doppler imaging

This study was designed to evaluate duplex and color-flow Doppler imaging as potential noninvasive methods of diagnosing patients with Budd-Chiari syndrome and following them after surgery. Five patients with confirmed hepatic venoocclusive disease were imaged. All five underwent duplex Doppler examinations; three were also evaluated with color-flow Doppler. The hepatic vasculature was examined in all five patients; decompressive mesoatrial shunts were present and were evaluated in four of the five patients. Color-flow Doppler precisely defined intrahepatic, portal, and inferior vena caval circulatory dynamics. Correlation with angiography was excellent. In the two patients in whom hepatic vasculature was evaluated with duplex Doppler alone, the results were less impressive. Intrahepatic flow abnormalities were identified, but the sites of occlusion were not determined convincingly. Signals transmitted from the heart and the inability to visualize the hepatic veins made duplex Doppler evaluation problematic. Duplex Doppler was able to define patency and the direction of flow in the portal vein and inferior vena cava. Our results suggest that color-flow Doppler is an excellent technique for the initial evaluation of patients suspected of having Budd-Chiari syndrome. In the evaluation of decompressive mesoatrial shunts, color-flow Doppler produced dramatic images. However, both duplex and color-flow Doppler were highly accurate in determining the patency of decompressive shunts. Either duplex or color-flow Doppler may be used as the primary imaging procedure to determine shunt patency.     

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     

MR evaluation of the portal vein in pediatric liver transplant candidates

Nine pediatric liver transplant candidates underwent preoperative MR evaluation of the portal vein and the inferior vena cava. Sonographic correlation was available in all patients and angiographic correlation was available in five. Pathologic correlation was obtained in seven cases either at liver transplantation or autopsy. MR demonstrated portal vein patency in three cases when it was not seen by angiography and confirmed portal vein patency in one patient when it was questionably identified on sonography. The portal vein was not seen on MR imaging in two cases when it was seen on sonography and angiography: in one case, it was small and to-and-fro flow was demonstrated angiographically; in the second case, the portal vein was occluded by tumor thrombus. Two vessels in two patients were misidentified by sonography and identified correctly by MR. These were an azygous continuation of the inferior vena cava and a large collateral vein in the portal region. Knowledge of the anatomy and documentation of vascular patency are essential in evaluation of patients before liver transplantation. In patients with complex anatomy or hemodynamics, it may be necessary to obtain this information from several imaging techniques (sonography, angiography, and MR).     

Budd-Chiari syndrome: detection with color Doppler sonography.

The value of color Doppler sonography in evaluating newly diagnosed Budd-Chiari syndrome in five patients was studied. Hepatic venous findings included absence of vessels (one patient), flow reversal (two patients), narrowing (four patients), and tortuosity (three patients). Detected collaterals included hepatic venous to hepatic venous (four patients), hepatic venous to subcapsular systemic venous (two patients), and portosystemic (three patients). Hepatic venous spectral Doppler waveforms were flat and essentially aphasic in four patients, indicative of distal hepatic venous compression. The inferior vena cava was markedly compressed in two patients and slightly compressed in one. Color Doppler sonography allowed more reliable and confident identification of irregular, compressed, or otherwise abnormal hepatic veins than did conventional sonography. Color Doppler sonography also showed collateral vessels that were undetected with conventional sonography or other imaging techniques. Our results suggest that color Doppler sonography may be a valuable tool in the initial diagnosis and evaluation of suspected Budd-Chiari syndrome.     

Evaluation of children for liver transplantation: value of MR imaging and sonography

Diagnostic imaging plays a major role in the diagnosis and treatment of chronic liver disease in children. Decisions regarding the need for sclerotherapy, palliative shunts, and anatomic suitability for hepatic transplantation are based on the upper abdominal anatomy. Thirty-six children with various forms of chronic liver disease were referred for diagnostic imaging, including MR and sonography. Each study was evaluated independently without knowledge of the other examination. The first six patients were evaluated retrospectively and the last 30 patients prospectively. The size of the portal vein and inferior vena cava varied in this population, with excellent agreement between sonography and MR. A 2-mm portal vein was shown only on MR imaging in two patients, but MR missed a 3-mm portal vein. Portosystemic collateral vessels also were evaluated and noted to be detected more readily with MR imaging (64%) than with sonography (22%). In particular, paraumbilical veins were detected more often with MR (28% vs 6% by sonography). Associated or coexisting anomalies were relatively common in children with chronic liver disease (14%). These included polysplenia (two cases), preduodenal portal vein (one), unilateral dysplastic kidneys (two), ureteropelvic junction obstruction (one), and splenic (one) and renal (one) cysts. MR imaging was more sensitive than sonography for detection of abnormal anatomy in this group of patients and should be considered the pretransplantation imaging technique of choice in children with end-stage liver disease.     

Angiography of liver transplantation patients

Over 45 months, 119 angiographic examinations were performed in 95 patients prior to liver transplantation, and 53 examinations in 44 patients after transplantation. Transplantation feasibility was influenced by patency of the portal vein and inferior vena cava. Selective arterial portography, wedged hepatic venography, and transhepatic portography were used to assess the portal vein if sonography or computed tomography was inconclusive. Major indications for angiography after transplantation included early liver failure, sepsis, unexplained elevation of liver enzyme levels, and delayed bile leakage, all of which may be due to hepatic artery thrombosis. Other indications included gastrointestinal tract bleeding, hemobilia, and evaluation of portal vein patency in patients with chronic rejection who were being considered for retransplantation. Normal radiographic features of hepatic artery and portal vein reconstruction are demonstrated. Complications diagnosed using results of angiography included hepatic artery or portal vein stenoses and thromboses and pancreaticoduodenal aneurysms. Intrahepatic arterial narrowing, attenuation, slow flow, and poor filling were seen in five patients with rejection.     

Ultrasonic assessment of abdominal venous return. I. Effect of cardiac action and respiration on mean velocity pattern, cross-sectional area and flow in the inferior vena cava and portal vein

Duplex scanning, i.e. combined real time ultrasonography and pulsed Doppler velocity measurement, of the inferior vena cava and portal vein was performed in 85 patients subjected to routine left and right heart catheterization. Mean blood velocity and volume blood flow in the inferior vena cava were found to be pulsatile, reflecting both cardiac action and respiration. Different flow patterns could be related to various heart conditions. The cross-sectional area of the inferior vena cava was also pulsatile, the normal variation with respiration being partial collapse during inspiration and maximum distension at end expiration. In the majority of patients, portal vein flow showed variation with respiration only, maximum flow occurring during expiration. The flow patterns found in the two veins were well in accordance with previous invasive findings in animals. It is concluded that duplex scanning is a useful tool in the assessment of abdominal venous return.     

彩超在减体积肝移植术后流出道梗阻的诊断及介入治疗中的应用

 目的探讨彩色多普勒超声在儿童减体积肝移植术后流出道梗阻的诊断及介入治疗中的应用价值.方法 2例儿童减体积肝移植术后流出道梗阻并发症病例,分析流出道梗阻的彩色多普勒超声表现及介入治疗前后的血流动力学改变.结果例1为肝静脉狭窄,超声表现为管腔直径不足2.5 mm,狭窄长度达15 mm,血流速度115.0 cm/s,同时伴大量腹水、脾脏增大.球囊括张术后血流速度轻度下降,1周后又重新恢复高速血流.支架植入术后血流速度降至正常范围,腹水消失.例2为下腔静脉狭窄,超声表现为下腔静脉肝后段内径明显变细、其直径不足0.3 mm,血流速度增快达200 cm/s以上.结论彩色多普勒超声对减体积肝移植术后流出道梗阻的诊断及监测介入术后血流动力学改变方面具有重要作用.     

Doppler waveform of hepatic veins in healthy children

OBJECTIVE: This study intends to document the presence or absence of triphasic waveforms in hepatic veins in healthy children. Does absence of triphasic hepatic vein flow indicate hepatic abnormality? SUBJECTS AND METHODS: One hundred children without a known hepatic or intrathoracic abnormality underwent Doppler sonography of their hepatic veins. Fifty girls and 50 boys were divided into five age groups. RESULTS: Forty-two children had triphasic flow in all three hepatic veins. Veins approaching an angle of 90 degrees with the inferior vena cava could not be assessed or had the least flow modulations despite angle correction. Neonates had the highest percentage of monophasic flow (seven of 21) in all three hepatic veins and none had triphasic flow in all three veins. CONCLUSION: Not all healthy children have a triphasic flow pattern in all hepatic veins. Before suspecting hepatic abnormality with abnormal parenchymal compliance (cirrhosis, graft rejection) by virtue of lack of triphasic hepatic vein flow, a normal variant of the flow should be considered. Only the change of a previously documented triphasic flow to monophasic flow in a given vein should be assessed as a sign of possible abnormality.     

Venous variants and anomalies on routine abdominal multi-detector row CT

OBJECTIVE: This study aims to determine the types and prevalence rates of anatomic variations of the hepatic veins, portal vein, inferior vena cava and renal veins, and to establish statistical correlations between various anomalies and frequency differences between male and female using multi-detector row computed tomography (CT). MATERIALS AND METHODS: One thousand one hundred and twenty patients (588 men, 532 women) were evaluated with routine abdominal CT. Frequencies of different variants were noted and compared, and correlations between three categories of variation were tested. RESULTS: In total, 1261 abdominal vein variants and anomalies were identified in 756 (67.5%) of 1120 patients. Six hundred and forty-two hepatic vein variants were detected in 468 (41.8%) patients. One or more inferior right hepatic veins were identified in 356 (31.8%) individuals, and tributary hepatic veins were detected in 147 (13.1%) patients. Portal vein variations and anomalies were observed in 307 (27.4%) cases. The most frequent of these was trifurcation (139 patients, 12.4%). A total of 311 inferior vena cava and renal vein variants were identified in 258 (23%) cases. Six patients (0.5%) exhibited inferior vena cava anomalies, 62 (5.5%) had circumaortic renal veins, 53 (4.7%) had retroaortic renal veins, and 210 (18.8%) had multiple renal veins. CONCLUSION: The prevalence of abdominal vein variations is high, and routine abdominal CT demonstrates these abnormalities very well. The data suggest that hepatic vein variants and multiple right renal veins are more frequent in women than in men, and that hepatic vein variation is correlated with portal vein variation.     

3D CE-MRA在评价腹部静脉系统中的应用

目的探讨三维增强磁共振血管成像（3DCE—MRA）技术在腹部静脉系统中的应用价值。方法收集具有完整资料的16例病例，先行常规MRI检查，随后经静脉注射Gd—DTPA20—30mL后连续三次行3DCE—MRA采集数据，对静脉系统原始图像进行MIP重建。主要观察指标为下腔静脉、脾静脉、肝静脉、肠系膜上静脉、门静脉形态变化。结果16例患者中，门腔静脉正常者9例，3DCE—MRA清晰显示下腔静脉、肝静脉、脾静脉、肠系膜上静脉、门静脉结构；门静脉高压3例，示脾静脉增宽迂曲，门静脉主干增宽及明显侧支循环形成；2例布加氏综合征；1例下腔静脉血栓形成；1例门静脉海绵样改变。结论3DCE—MRA是一种安全、敏感性高的血管造影技术，可提高腹部静脉系统疾病的术前诊断。     

Ultrasonic assessment of abdominal venous return. II. Volume blood flow in the inferior vena cava and portal vein

Eighty-five patients subjected to routine heart catheterization, were examined with duplex scanning of the inferior vena cava and portal vein. Volume blood flow in the two veins was estimated and compared with Fick measured cardiac output and duplex measured flow in the lower thoracic aorta. Inferior vena caval and portal vein flow were found to comprise approximately 30 to 40 per cent and 20 per cent, respectively, of cardiac output, but with a large dispersion of individual percentages. A high correlation (r = 0.88) was found between the sum of inferior vena caval and portal vein flow and duplex measured flow in the lower thoracic aorta, the sum of flow in the two veins comprising approximately 90 per cent of lower thoracic aortic flow. It is concluded that duplex scanning seems to be a useful method in estimating volume blood flow in both the inferior vena cava and portal vein.     

Duplex Doppler sonography of the hepatic vein in tricuspid regurgitation

Patients with tricuspid regurgitation may present initially with vague abdominal symptoms and elevated liver enzymes. In the absence of diagnostic sonographic findings, patients may be subjected to an unnecessary invasive liver biopsy for an accurate diagnosis. We recently described the association of the pulsatile portal venous waveform on duplex Doppler sonography with tricuspid regurgitation in 15 patients. In this study I describe the changes in the hepatic venous waveform in these patients and compare the findings with the final diagnosis as determined by Doppler echocardiography (n = 14) or ultrafast CT (n = 1). All patients had clinical findings consistent with liver dysfunction and were referred for sonography to rule out diseases of the liver, biliary tree, or hepatic or portal veins. All patients had persistently dilated hepatic veins and inferior venae cavae. Twenty-four volunteers, 11 of whom had simultaneous ECG tracings, served as a control group. The main findings on the hepatic duplex sonogram in the disease group were a decrease in the size of the antegrade systolic wave with a systolic/diastolic flow velocity ratio of less than 0.6 (n = 4) or reversal of the systolic wave (n = 10). In all volunteers, systolic flow was antegrade and the ratio was more than 0.6. Two diagnoses were false positive and one was false negative. In some patients with sonographic signs of congestive heart failure, duplex Doppler sonography of the hepatic vein may be helpful in the diagnosis of one of the causes of liver dysfunction, tricuspid regurgitation.     

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.     

三维动态增强磁共振血管成像在肝移植术前的应用

 目的探讨三维动态增强磁共振血管成像(3D DCE MRA)三期扫描技术,评价其在肝移植术前的诊断价值.方法对拟进行肝移植的183例患者进行术前3D DCE MRA成像扫描,采用Siemens Symphony 1.5T超导MRI扫描机,圆形极化相控阵体线圈,3D DCE MRA三期(动脉期、门脉期和静脉期)扫描技术,即对所得图像进行综合评价.结果全部病例均获得了满意的血管成像图像,肝动脉可显示2～3级分支,门静脉可显示2～5级分支,肝静脉可显示1～2级分支.183例肝移植患者,5例显示肝动脉变异,其中2例起自肠系膜上动脉,2例直接起自腹腔干,1例起自胃左动脉.单纯肝硬化门脉高压103例,其中,冠状静脉和食道胃底静脉曲张23例、脐周静脉曲张5例、肠系膜静脉曲张2例、脾门周围静脉曲张30例,门静脉玻璃样变性1例;原发性肝癌79例,5例肝动脉包埋、僵直、推移,2例肝内动-静脉瘘,门静脉右支癌栓23例,门静脉左支癌栓7例,门静脉主干癌栓3例,同时发生门静脉左右支癌栓的2例,MRA表现为门静脉呈半月形或杯口形缺损或不显影.7例肝静脉出现栓塞,5例下腔静脉受压推移,1例下腔静脉瘤栓.结论 3D DCE MRA三期扫描能很好的显示肝动脉、门静脉、肝静脉及下腔静脉系统病变,肝移植术前应用3D DCE MRA,基本达到临床要求,是术前血管评估的有效方法.     

Portal hypertension: angiographic and hemodynamic evaluation

There has been a correlation of three hemodynamic parameters with the etiology of portal hypertension and one of the major determinants of therapeutic success, namely, the direction of portal flow. The presence of a 4 mm Hg or greater gradient between the right atrium and the intrahepatic inferior vena cava associated with a "lumpy" pull-back tracing between the wedged and free positions has been associated with alcoholic liver disease. Such patients have antegrade portal flow when their AoD/HWP ratio is in the range of 2.6 to 2.0, and flow becomes stagnant or reversed below this range. Nonalcoholic liver disease is characterized by the absence of a gradient between the right atrium and the inferior vena cava and by a pull-back tracing that falls smoothly and rapidly to the free hepatic vein value. These patients have antegrade portal flow with an AoD/HWP ratio in the range of 1.7 to 1.5. The correct characterization of the cause for diffuse liver disease and direction of portal flow applies to the selection process for patients being considered for the selective distal splenorenal shunt as well as for the newer procedure of orthotopic liver transplantation. It is hoped that the wider application of these physiologic parameters, in the context of an increasing array of imaging tools for the portal system, including high-resolution ultrasound, computed tomography, and magnetic resonance imaging (MRI), will continue to offer all clinicians interested in the problem of portal hypertension a reliable guide to prognosis and the success of the particular treatment provided.     

彩色多普勒超声对肝小静脉闭塞症的诊断价值

目的:探讨肝小静脉闭塞症(hepatic veno-occlusive disease,HVOD)的超声影像特点及其诊断价值.材料和方法:回顾性分析6例HVOD患者11次灰阶及彩色多普勒超声图像,观察肝、肝静脉、门静脉、肝动脉、下腔静脉、胆囊、脾和腹水量.结果:6例HVOD治疗前均有不同程度的门静脉高压表现:灰阶超声示6例肝大,5例胆囊壁增厚呈双边影及腹水,4例脾大.彩色多普勒示门静脉血流速度减慢,1例出现反流,1例附脐静脉开放;4例患者肝静脉内径变窄,频谱呈门脉样;2例患者肝动脉阻力指数增高(>0.65).经过系统的抗HVOD治疗后4例患者上述超声表现得到了不同程度的改善.结论:灰阶及彩色多普勒超声对HVOD具有重要的辅助诊断及鉴别诊断价值.