Budd-Chiari syndrome caused by membranous obstruction of the inferior vena cava associated with coeliac disease

Ten cases of Budd-Chiari syndrome associated with coeliac disease have been reported in the literature, most of them in North African subjects. Supporting this association, we report a new case in a young Spanish Caucasian man in whom the cause of the syndrome was the membranous obstruction of the inferior vena cava, an infrequent cause of Budd-Chiari syndrome in Western countries. A percutaneous balloon angioplasty was performed, with satisfactory outcome.     

介入治疗下腔静脉节段闭塞型Budd—Chiari综合征的方法学研究

目的探讨介入治疗下腔静脉节段闭塞型Budd-Chiari综合征（BCS）的有效方法。方法对经造影确诊的26例下腔静脉肝后段节段型完全闭塞型BCS患者,采用聚乙烯球囊导管或国产Inoue球囊导管行经皮腔内血管成形术（PTA）及支架置入,其中闭塞段穿刺过程中综合运用直接穿刺技术、导管挤钻技术、逆向穿刺技术、双向穿刺技术、导丝轨道技术。PTA前后行下腔静脉造影及血液动力学检查;观察患者临床表现变化及并发症发生情况。结果23例成功穿通闭塞段并行PTA治疗,均无严重并发症,技术成功率为88．5％;15例置入下腔静脉支架。术后闭塞段下腔静脉内径为（19．20±6．21）cm,下腔静脉压、下腔静脉至右房压力阶差显著降低,右心房压显著升高;随访1～5a复发3例。结论介入治疗下腔静脉节段闭塞型BCS安全、有效,综合应用各种穿刺技术及合理选择球囊导管类型、直径可提高成功率、减少并发症。     

Balloon dilatation of inferior vena cava obstruction in budd-chiari syndrome

Budd-Chiari syndrome is a well described entity which can easily be confused with congestive hepatomegaly. The syndrome usually is caused by thrombosis of the hepatic veins, however it also can be caused by congenital fibrous webs that occlude hepatic vessels in the inferior vena cava. It leads to an enlarged liver with ascites, peripheral edema, and portal hypertension.     

原发性Budd-Chiari综合征的介入治疗

目的分析经皮球囊扩张治疗原发性Budd-Chiari综合征下腔静脉闭塞病变的疗效。方法采用Seldinger穿刺血管方法,行下腔静脉及右心房造影,确定下腔静脉闭塞段,用Brockenbrough穿刺针开通闭塞段,球囊导管扩张病变治疗下腔静脉闭塞病变42例。结果成功率为100％,病变区血管直径扩至12～21mm,平均(18±3．1)mm,下腔静脉压自(17±3)mmHg,降至(7．5±1．8)mmHg,疗效显著(P＜0．01),症状体征明显减轻或消失。结论临床观察表明,Brockenbrough穿刺针开通病变血管是安全的,球囊扩张对绝大部分原发性Budd-Chiari综合征下腔静脉闭塞病变的疗效满意。     

Redefining Budd-Chiari syndrome:A systematic review

AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.     

Asymptomatic membranous obstruction of the inferior vena cava forming intrahepatic collateral pathways

Intrahepatic and/or extrahepatic collateral pathways result from the membranous obstruction of the inferior vena cava. These collaterals are usually insufficient to prevent Budd-Chiari syndrome. We reprot an unusual case of asymptomatic membranous obstruction of the inferior vena cava in which marked intrahepatic collateral pathways were formed. Although the inferior vena cava terminated above the orifice of the right hepatic vein, the middle and left hepatic veins were patent above the membrane, without narrowing. Blood from the inferior vena cava drained into the right atrium via the intrahepatic collaterals between the right and middle hepatic veins without resistance.     

Budd-Chiari Syndrome Associated with Visceral Leishmaniasis and Factor V Leiden Mutation

We here report a case of subacute Budd-Chiari syndrome (BCS) related to Factor V Leiden (FVL) mutation in the presence of visceral leishmaniasis. A 17-year-old man was admitted to hospital because of abdominal pain, pretibial edema and fever. The clinical picture of BCS had been developed within several months. BCS was diagnosed by radiographic examination. On DNA analysis, a heterozygote Arg506Gln mutation in the factor V gene was found. Histological examination of the bone marrow showed intracellular leishmania amastigotes. Despite appropriate treatment patients clinical condition deteriorated rapidly and died with multiorgan failure. FVL mutation is the most common procoagulant disorder and account for many cases of BCS. This case report demonstrates that in addition to duration and severity of the disease accompanying conditions including infections are prognostically significant for the outcome of this potentially lethal disease.This revised version was published online in May 2005 with a corrected cover date.     

两种球囊导管治疗Budd-Chiari综合征的对比研究

目的:探讨两种球囊导管治疗Budd-Chiari综合征的效果和适应证。方法:分别选用聚乙烯18 mm、20 mm球囊和Inoue 24 mm、26 mm球囊对52例肝段下腔静脉阻塞的病人进行了经皮腔内血管成形术(PTA),12例置放了血管内支架。术前、后行下腔静脉造影并行血液动力学检查。结果:PTA术后下腔静脉内径由1.9±0.4 mm增至19.5士6.5mm。下腔静脉压由3.24±0.88 kPa降至1.48±0.55 kPa。术后残余压力阶差聚乙烯18 mm球囊组、20 mm球囊组分别为0.77±0.32 kPa、0.62±0.53 kPa;Inoue 24 mm球囊组、26 mm球囊组分别为0.34±0.27 kPa、0.32±0.28 kPa。结论:PTA是治疗肝段下腔静脉阻塞型Budd-Chiari综合征的有效方法,应根据病变的类型选择不同的球囊,而大腔球囊、充分扩张是提高疗效、预防再狭窄的关键。     

奇静脉迂曲扩张在布加氏综合征中的诊断价值

目的探讨奇静脉迂曲扩张在布加氏综合征中的诊断价值。方法对28例经血管造影证实的布加氏综合征患者的MRI资料进行回顾性分析,重点观察奇静脉的大小和形态变化。结果肝脏体积增大伴尾叶增大23例,明确显示下腔静脉狭窄或闭塞19例,肝外侧枝血管形成25例,腹水25例。28例患者奇静脉明显扩张迂曲,形成侧支循环。结论在低场强MRI对布加氏综合征患者的诊断中,奇静脉的变化有重要的价值。     

Endemicity and clinical picture of liver disease due to obstruction of the hepatic portion of the inferior vena cava in Nepal

Obstructive lesion of the hepatic portion of the inferior vena cava is common in Nepal. The clinical data on 150 patients who were seen at the Liver Unit, Bir Hospital, Kathmandu, in three years from 1990 to 1992 were analysed. Although the majority of patients were over 20 years of age, 25 patients were below 10 years of age; there were more males than females in this study. This disease accounted for 17% of 866 patients with chronic liver disease and for nearly one quarter of 267 biopsies performed on this patient group during the same period. Obstructive lesions of the inferior vena cava seem to be more common among poor people with malnutrition. Clinically, our patient group could be divided into acute (n= 27), subacute (n= 43) and chronic (n= 80) cases. The important clinical features are hepatomegaly and/or ascites and, in chronic cases, prominent dilated superficial veins over the body trunk with cephalad flow. Ultrasound is the most helpful diagnostic procedure, especially in subacute and chronic cases, as it frequently demonstrates caval obstruction, thrombosis, dilated hepatic veins and intrahepatic collaterals. Diagnosis is confirmed by cavography, which shows a caval obstruction of varying lengths at the cavo-atrial junction or a marked narrowing of the hepatic portion of the vena cava. In subacute and chronic cases cavography also demonstrates collateral veins, such as the ascending lumbar, hemiazygos and azygos that drain into the superior vena cava. Chronic cases had periods of exacerbation often associated with bacterial infection. The aetiology of inferior vena cava obstruction at its hepatic portion is not known, but there seems to be a frequent association of bacterial infection with the disease.     

A caval homograft for Budd-Chiari syndrome due to inferior vena cava obstruction

Transjugular intrahepatic portosystemic shunt (TIPS) is the standard treatment of Budd-Chiari syndrome (BCS) non responsive to medical therapy. However, patients with inferior vena cava (IVC) obstruction proximal to the atrium do not benefit from TIPS and a surgical approach is mandatory. We report the case of BCS due to intrapericardial IVC obstruction. We describe a novel surgical approach using a fresh caval homograft. An attempt to balloon dilatation of the IVC obstruction was complicated by right atrial disruption with tamponade and ventricular fibrillation. Lately, the patient successfully underwent a reconstruction of the cavo-atrial continuity by the interposition of a fresh caval homograft, a novel surgical approach never described before for BCS. Further follow-up revealed progressive reduction and resolution of ascites, and overall clinical improvement. IVC obstruction near to the atrium can be surgically approached with a new technique consisting in inferior vena cava resection and replacement with a caval homograft.     

Occurrence of hepatopulmonary syndrome in Budd-Chiari syndrome and the role of venous decompression

BACKGROUND & AIMS: Hepatopulmonary syndrome (HPS) has been predominantly detected in cirrhotic patients and rarely in patients with noncirrhotic portal hypertension. The aim of this study was to determine the occurrence of HPS in patients with Budd-Chiari syndrome (only anecdotal reports available) and evaluate the role of venous decompression in its reversal. METHODS: Twenty-nine consecutive cases of Budd-Chiari syndrome without primary cardiopulmonary disease were investigated by air contrast echocardiography and arterial blood gas analysis. Venous decompression (e.g., by balloon cavoplasty) was attempted when feasible. RESULTS: Eight cases (27.6%) of HPS and 9 cases (31.0%) with positive contrast echocardiography but unimpaired oxygenation were detected. Duration of disease was longer (P = 0.026) among those with positive contrast echocardiography. Cavoplasty reversed 4 of 5 cases of HPS and 2 of 2 cases with positive contrast echocardiography alone. Venous decompression by drainage of amebic liver abscess (which was compressing hepatic venous outflow) also reversed 1 case of HPS. HPS was relieved by venous decompression in 5 of 6 cases. CONCLUSIONS: HPS developed in a substantial fraction of our patients with Budd-Chiari syndrome, with positive contrast echocardiography occurring mainly in the benign, slowly progressing variety. Venous decompression showed promise in reversing such cases.     

Acute iatrogenic Budd-Chiari syndrome following hepatectomy for hepatolithiasis: A report of two cases

Budd-Chiari syndrome(BCS)is defined as hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava(IVC)and the right atrium,regardless of the cause of obstruction.We present two cases of acute iatrogenic BCS and our clinical management of these cases.The first case was a 43-year-old woman who developed acute BCS following the implantation of an IVC stent for the correction of stenosis in the IVC after hepatectomy for hepatolithiasis.The second case was a61-year-old woman with complete obstruction of the outflow of hepatic veins during bilateral hepatectomy for hepatolithiasis.Acute iatrogenic BCS should be con-sidered a rare complication following hepatectomy for hepatolithiasis.Awareness of potential hepatic outflow obstructions and timely management are critical to avoid poor outcomes when performing hepatectomy for hepatolithiasis.     

Identification of latent myeloproliferative disease in patients with Budd-Chiari syndrome using X-chromosome inactivation patterns and in vitro erythroid colony formation

Abstract: Some patients with an early or latent myeloproliferative disorder (MPD) present with Budd-Chiari syndrome (BCS, hepatic vein thrombosis). Cell culture analysis of erythroid progenitors (BFU-E) can be used to discriminate primary from secondary MPD and examination of X-chromosome inactivation (in females) can be used to demonstrate clonality in neoplastic tissues. The present study used these techniques to examine whether a group of 7 female patients who presented with BCS had evidence to support a diagnosis of MPD. Unilateral X-inactivation and therefore clonality can be studied in females heterozygous for X-linked restriction fragment length polymorphisms (RFLP) by differences in methylation between active and inactive chromosomes. Probes for two polymorphic loci, phosphoglycerate kinase (PGK, at Xq13.3 [BstX1 RFLP]) and M27β (an anonymous locus DXS255 at Xp11.22 [Pst1 RFLP]) were used to study methylation patterns. All 7 patients were heterozygous using M27β and 2/7 were also heterozygous using the PGK probe. Polyclonal patterns of X-inactivation in granulocytes were demonstrated in 3/7, a skewed/monoclonal pattern in 1/7 and aberrant patterns in 3/7 using M27β. Two patients who had aberrant patterns of X inactivation with M27β demonstrated a skewed/monoclonal pattern with PGK. The results of BFU-E growth patterns and clonality were entirely concordant in 5/6 patients. Thus X-chromosome inactivation patterns, in conjunction with erythroid colony studies, can be used to assist in the diagnosis of an underlying MPD in BCS.     

Liver cirrhosis in hepatic vena cava syndrome (or membranous obstruction of inferior vena cava)

Hepatic vena cava syndrome (HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction (HVOO) that is complicated by high incidence of liver cirrhosis (LC) and moderate to high incidence of hepatocellular carcinoma (HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome (BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction, and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations (AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, porto-systemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.     

Budd-Chiari syndrome with complete occlusion of the inferior vena cava: percutaneous recanalization by angioplasty and stenting

A 47-year-old man was diagnosed with primary antiphospholipid syndrome and Budd-Chiari syndrome (membranous complete obstruction of the intrahepatic inferior vena cava), with edema and ascites refractory to medical treatment. The inferior vena cava membrane was punctured with a Brockenbrough needle under multidirectional fluoroscopic guidance via a transfemoral approach. The occlusion was dilated with balloons of increasing size and was subsequently stented successfully. At 1-year follow-up venography showed patency of the stent, and the patient remains asymptomatic 2 years after the procedure.     

Budd-Chiari syndrome: a short radiological review

A short review of Budd–Chiari syndrome (BCS) is given with detailed radiological findings from ultrasound, computed tomography (CT) and angiography and illustrated with two case reports. The cases had different clinical presentations: one antedated by Behçet’s syndrome and complicated with aortic aneurysm, the other with a provisional diagnosis of a tuberculous abdomen. The radiological features of BCS, especially the changing appearance under dual-phase CT, are discussed. The respective diagnostic sensitivity and efficacy of other imaging modalities are mentioned. © 1999 Blackwell Science Asia Pty Ltd     

Budd-Chiari syndrome： A case with a combination of hepatic vein and superior vena cava occlusion

We here report a recent, rare case of Budd-Chiari syndrome, associated with a combination of hepatic vein and superior vena cava occlusion. A young female, who had been in good health, was admitted to our hospital because of massive ascites. The patient had used no oral contraceptives. Tests for coagulation disorders, hematological disorders, and antiphospholipid syndrome were all negative. Budd-Chiari syndrome was diagnosed by radiographic examination. The patient was suffering from a combination of hepatic vein and superior vena cava occlusion. In particular, the venous flow returned from the liver mainly through a right accessory hepatic vein, and stenosis was recognized at the orifice of this collateral vein into the vena cava. Subsequently, the patient underwent percutaneous balloon dilatation therapy for this stenosis. After this treatment, the massive ascites was gradually reduced, and she was discharged from our hospital. It has now been one year since discharge, and the patient has been doing well. If deteriorating liver function or intractable ascites occur again, a liver transplantation may be anticipated. This is the first case report of Budd-Chiari syndrome associated with a superior vena cava occlusion.     

Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of Budd-Chiari syndrome (BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.