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.     

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。     

Diagnostic imaging of Budd-Chiari syndrome in adults and children

Budd-Chiari syndrome is caused by the obstruction of the hepatic veins or of the inferior vena cava. It is characterized by the classic symptomatological triad: ascites, hepatomegaly, and abdominal pain. In 2/3 cases its etiology remains unknown. Budd-Chiari syndrome may be associated with polycythemia vera, neoplasms, chronic leukemia, congenital abnormalities, hypercoagulation conditions, pregnancy, oral contraceptives, and constrictive pericarditis. Even though its clinical diagnosis is difficult, radiology plays a decisive role with US, CT, MR imaging and, above all, angiography; the latter, together with liver biopsy, generally provides with an unquestionable diagnosis. Through the definition of stage of the disease, of level (intrahepatic, venous, caval, cardiac), of type (intrinsic or extrinsic), and degree of both obstruction and consequent development of collateral channels, radiology determines which patients should undergo a medical or a surgical treatment. In some case, percutaneous angioplasty can be performed. Four cases of Budd-Chiari syndrome, including two children, were investigated with US, CT, angiography, and liver biopsy; MR imaging was also employed in one case. The underlying cause was identified in 3 patients: constrictive pericarditis of probable congenital origin and web occlusion of the inferior vena cava near the right atrium in the 2 children; hepatic vein thrombosis due to essential thrombocythemia in the third case. In the fourth patient, thrombosis of the inferior vena cava and hepatic veins was unexplained. The diagnosis was established by means of liver biopsy and phlebography of the hepatic veins. Good diagnostic information was also supplied by non-invasive techniques, such as US, CT, and MR imaging.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

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.     

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.     

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     

Imaging and interventions in Budd-Chiari syndrome

Budd-Chiari syndrome(BCS) consists of a group of disorders with obstruction of hepatic venous outflow leading to increased hepatic sinusoidal pressure and portal hypertension.Clinically,two forms of disease(acute and chronic) are recognized.Mostly the patients present with ascites,hepatomegaly,and portal hypertension.In acute disease the liver is enlarged with thrombosed hepatic veins(HV) and ascites,whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava(IVC),or there may be short or long segment fibrotic constriction of HV or the suprahepatic IVC.Due to advances in radiological interventional techniques and hardware,there have been changes in the management protocol of BCS with surgery being offered to patients not suitable for radiological interventions or having acute liver failure requiring liver transplantation.The present article gives an insight into various imaging findings and interventional techniques employed in the management of BCS.     

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.     

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 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     

布-加氏综合征的腔静脉造影与B超诊断对照分析

目的　探讨B超及下腔静脉造影检查在布—加氏综合征临床诊断中的准确性和实用价值。方法　 42例病人均于血管造影前行下腔静脉或肝部的B超扫描。血管造影采用Seldinger法行单向 ( 14例 )或双向 ( 2 8例 )下腔静脉插管法 ,并快速摄片观察。结果　B超发现下腔静脉节段性或膜性阻塞 18例 ,下腔静脉入口处狭窄 2 4例。其中伴有单纯性肝右静脉狭窄或 (和 )阻塞 2 0例 ,肝左静脉狭窄 8例 ,肝中静脉狭窄 12例。肝左—中、中—右肝静脉之间形成交通支血管各 8例 ,左—中—右之间形成网状交通血管 8例。 5例下腔静脉轻度狭窄 ,血流通畅。下腔静脉造影示 12例下腔静脉近端狭窄 ,30例呈节段性或膜性完全阻塞。肝中静脉和肝右静脉显影各 8例 ,余 2 6例肝静脉均未显影。有 2 6例见明显的侧枝循环形成 ,且可见有侧枝循环血管呈瘤样扩张。结论　B超作为一种简便易行的无创伤性检查是筛选病人的首选方法。下腔静脉造影是布—加氏综合征的确诊手段 ,对完全性节段或膜性阻塞者行双向下腔静脉造影是十分必要的 ,尤其是欲行介入治疗者更是如此。     

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综合征:肝静脉病变的多排螺旋CT诊断

目的探讨多排螺旋CT(MSCT)对Budd-Chiari综合征(BCS)肝静脉阻塞病变的诊断价值。方法对比分析26例BCS的肝静脉血管造影和术前1周内的MSCT平扫与增强扫描表现。结果26例MSCT横断面图像和重建图像显示肝静脉共计70条,其中56条肝静脉闭塞,包括13条肝右静脉闭塞,21条肝中静脉闭塞,22条肝左静脉闭塞;另显示21条副肝静脉代偿性增粗;其中1例肝右静脉伴有血栓形成。与下腔静脉或经皮肝穿刺肝静脉造影相比,肝静脉的符合率为92.11%。结论MSCT能准确显示BCS肝静脉的血流动力学变化、阻塞部位,性质以及肝内交通支情况,诊断价值高,能可靠的指导制订治疗方案。     

肝静脉阻塞型布-加氏综合征的超声诊断价值

目的:探索彩色多普勒超声对肝静脉阻塞型布-加氏综合征的诊断价值.材料和方法:应用彩色多普勒超声观察138例肝静脉阻塞型布-加氏综合征的彩色多普勒超声的声像图特点, 并与血管造影对比分析. 结果:肝静脉隔膜型阻塞83 例(60.1 %),节段性闭塞45 例(32.6 %),单纯狭窄10例(7.3 %).表现为肝静脉流出道有狭窄、闭锁、栓子或隔膜梗阻图像,肝静脉间可见交通支形成,肝短静脉代偿性扩张,第三肝门开放等.同时存在肝静脉和下腔静脉阻塞者35例,合并下腔静脉血栓者11例,肝静脉血栓7例.超声诊断假阳性2例,假阴性3例.结论:彩色多普勒超声可较准确地判定肝静脉血管狭窄及闭塞的部位和类型,并明确血管梗阻的程度及侧支血管形成情况,有助于手术方式的选择以及术后疗效的判断.     

Angiographic abnormalities in partial Budd-Chiari syndrome.

Partial Budd-Chiari syndrome was produced in 8 monkeys by obstructing 2 or 3 lobar hepatic veins with a rapidly polymerizing plastic. Hepatic angiography done 1-3 months later revealed crowded tortuous arteries and a dense hepatogram in the lobe with venous occlusion. Unobstructed lobes were hypertrophied. Retrograde injection of persistently patent hepatic veins demonstrated a "spiderweb" collateral system. Retrograde venography to demonstrate this pathognomonic plexus is the procedure of choice when partial Budd-Chiari syndrome is suspected.     

Liver scan in Budd-Chiari syndrome.

Seventeen liver scans were performed in seven patients with occulsion of hepatic veins: the Budd-Chiari syndrome. When some, but not all liver veins were occluded, markedly diminished uptake over the affected segments was usually seen (absence of uptake may occur in acute infarction and in chronic cases). When all major liver veins are occluded, markedly diminished uptake is seen over the peripheral parts of the right and left lobes with a triangular midline area of normal or excessive activity. The latter effect is probably caused by uptake in segments surrounding the inferior vena cava (frequently the caudate lobe) that have direct venous drainage. This liver-scan appearance is characteristic enough to warrant consideration of the Budd-Chiari syndrome as the first diagnosis. Good correlation existed between selective venography and liver-scan findings. The usefulness of liver scans in the followup after portocaval shunting is illustrated in one patient.     

Ultrasonically guided percutaneous transhepatic hepatic vein stent placement for Budd-Chiari syndrome.

PURPOSE: To evaluate the utility of ultrasonically guided hepatic vein stent placement in the treatment of Budd-Chiari syndrome (BCS) in patients with short hepatic vein obstruction. MATERIALS AND METHODS: Twenty-five patients with BCS, each with three obstructed hepatic veins diagnosed with ultrasound (US), color Doppler, probing with guide wire, and echo contrast, underwent hepatic vein stent placement under US guidance. Nine patients had hepatic vein obstruction alone, and 16 had hepatic vein obstruction along with primary inferior vena cava (IVC) obstruction. In each patient, only one of the hepatic veins was selected for recanalization and stent placement. In patients with primary IVC lesions, a stent was placed in the IVC first. Clinical and US examinations were performed at 3-6-month intervals on every patient during follow-up. RESULTS: Hepatic vein stents were successfully placed in 23 of the 25 patients, a success rate of 92%. The mean +/- SD hepatic vein pressure decreased from 25.57 mm Hg +/- 9.46 to 9.67 mm Hg +/- 2.31 (P < .01), and the flow direction in the hepatic vein became centripetal and its spectral analysis showed a normal phasic flow. Twenty-two patients experienced a significant improvement in hepatic outflow, as evidenced by disappearance of ascites, remission of hepatosplenomegaly, improvement in liver function, and alleviation of esophageal varices. Severe intraperitoneal hemorrhage occurred in one patient. No other serious procedure-related complications were observed. During follow-up of 1-43 months (mean, 23 months), stent reocclusion occurred in one patient. The other stents remained patent, and clinical features of BCS did not recur. CONCLUSION: Percutaneous transhepatic hepatic vein stent placement is a reasonable treatment for BCS in patients with hepatic vein obstruction, and the procedures can be performed safely and accurately with US.     

三维对比剂增强MR血管成像诊断布加综合征的价值

目的观察三维对比剂增强MR血管成像（3DCEMRA）上布加综合征（BCS）的各种表现,并初步评价该项新技术的价值。方法33例BCS患者行3DCEMRA检查。23例为继发性BCS,分别继发于肝细胞癌（21例）、右肾上腺癌（1例）或血栓性静脉炎（1例）。10例为原发性BCS。观察肝静脉、下腔静脉（IVC）和门静脉的开放性,观察有无肝内外侧支、肝实质病变和门静脉一体静脉间曲张静脉。10例患者行下腔静脉造影术,2例行肝右静脉穿刺造影术,把3DCEMRA所获的诊断结果与造影相对照。结果3DCEMRA可显示BCS的各种表现。肝静脉表现包括：癌栓形成（19例）、肿瘤压迫（2例）、肝静脉未显示（4例）和局限性狭窄（4例）。IVC表现为严重狭窄或闭塞（10例）、肿瘤直接侵犯（2例）、癌栓形成（3例）、血栓性静脉炎（1例）和隔膜形成（3例）。9例显示肝内侧支形成,其中2例显示“蜘蛛网”征象。所见的肝外侧支包括扩张的奇静脉和半奇静脉（13例）,以及左肾一膈下一心包膈静脉侧支（2例）形成。2例患者发现门静脉左支闭塞,10例患者发生门静脉．体静脉间静脉曲张。3DCEMRA发现的肝实质病变有：尾叶增大（7例）、不均匀强化（18例）和并发肿瘤（18例）。12例3DCEMRA诊断结果均与造影结果一致。结论3DCEMRA能显示BCS的各种征象,并能帮助提供正确诊断。