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.     

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.     

MDCT venography in the evaluation of inferior vena cava in Budd-Chiari syndrome

Objective  To assess the role of multidetector computed tomography (MDCT) venography in the evaluation of the inferior vena cava (IVC) in Budd-Chiari syndrome (BCS), its accuracy as compared to digital subtraction venography (DSV) and the potential of this technique to replace venography for the definitive diagnosis of BCS. Methods  Twenty-five suspected cases of BCS were prospectively enrolled in this study and underwent both MDCT venography and DSV. Two observers independently evaluated and graded both the axial and reformatted MDCT images for the presence, site, degree and length of IVC narrowing. The collateral pathways and the hepatic veins were also assessed in all cases. The degree of correlation between MDCT venography and DSV was expressed using Spearman’s rank correlation coefficient (Rs). Results  There was excellent correlation between MDCT venography and DSV in predicting the presence of stenosis and in grading the degree and length of IVC stenosis (Rs=0.58, p<0.05). Four patients had presence of a web within the IVC and the reconstructed MDCT venography images detected the flap of the membrane in all of them. In three cases of complete obstruction the cranial extent of the obstruction could be determined on the reconstructed MDCT venography images, while double catheter access through the femoral and jugular routes was needed to determine the same on DSV. MDCT venography was significantly more informative in depicting the presence and site of both intrahepatic and extrahepatic collaterals as compared to DSV. Conclusion  MDCT venography, in the present study, accurately provided information of both conventional CT and IVCgraphy, in the evaluation of the IVC in a non-invasive way. It helped overcome the shortcomings of CT in the evaluation of IVC and was better than DSV for the evaluation of collaterals, calcification and complete IVC obstruction. We suggest that CT venography can be used as a frontline investigation for the diagnosis of IVC obstruction and for planning surgery or percutaneous endovascular intervention.     

布-加综合征、肝血窦阻塞综合征与肝硬化的鉴别

布-加综合征是由肝静脉或其开口以上的下腔静脉阻塞性病变引起的以下腔静脉阻塞、门静脉高压为特点的综合征,诊断依靠影像学检查,治疗的关键是解除梗阻。肝血窦阻塞综合征是指肝窦内皮完整性破坏和肝窦内充血阻塞而产生的肝内窦后性门静脉高压症,临床表现类似布-加综合征,诊断依靠肝组织活检。这两种疾病的治疗和预后均与肝实质病变导致的肝硬化不同,所以临床上需要注意这三种疾病的鉴别。     

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.     

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.     

Membranous obstruction of inferior vena cava in Taiwan

Abstract Membranous obstruction of the inferior vena cava (MOVC) is a rare cause of Budd-Chiari syndrome. When compared to the West, the incidence of MOVC was reported to be higher in the Orient, India and South Africa. From 1979 to 1993, 16 consecutive Chinese patients (mean age 50 years) with MOVC were retrospectively evaluated. The diagnosis in these MOVC patients was usually delayed with a meanlag time of lll months. Most of the cases were detected by a delicate ultrasonographic examination of the abdomen. Segmental narrowing of the inferior vena cava in 11 patients was the predominant type of MOVC, while five experienced a membranous obstructive type. Only one of five surgery-treated patients had a postoperation survival > 5 years, while five out of the nine patients who received conservative treatment still survived after a 6-15 year follow up. Two patients received percutaneous angioplasty (PTA). One survived 4 years and the other expired 5 years after the PTA. Two patients (12%) developed a hepatocellular carcinoma in their disease course, and the incidence was lower than in previous reports from Japan and South Africa. The incidence of hepatitis B surface antigen in MOVC did not increase in our patients compared with the general population. In conclusion, the inferior vena cava should be carefully evaluated in an ultrasonographic examination of the abdomen to increase the diagnosis rate of MOVC. Surgical intervention should be carefully justified according to the patient's symptoms and signs, the types of obstructive lesions and the expertise of the surgeons.     

TRA1 mRNA在肝硬化及肝癌组织中表达的研究

目的探讨肝癌(hepatocellular carcinoma,HCC)组织、肝硬化(liver cirrhosis,LC)组织及正常对照组织中肿瘤排斥抗原1(tumor rejection antigen 1,TRA1)mRNA的表达及关系。方法采用逆转录-聚合酶链反应的方法检测34例肝癌患者肝癌组织、癌旁肝硬化组织、非肝癌的肝硬化患者活检组织、肝血管瘤及肝内胆管结石等对照组织中TRA1 mRNA的表达,用捷达801分析软件对结果进行相对定量分析。结果肝癌组织、肝硬化组织、对照组织TRA1 mRNA表达率分别为95.00%、84.21%、50.00%,肝癌组与对照组间表达率的比较,差异有统计学意义(P0.05),肝硬化组织、对照组织表达率之间差异无明显统计学意义(P0.05);肝癌组织、肝硬化组织、对照组织TRA1 mRNA表达量分别为1.67±0.96、1.49±0.53、0.57±0.27;扩增产物表现出表达量的不同,呈现渐变趋势,肝癌组织、肝硬化组织与对照组之间差异有统计学意义(P0.05),肝癌组织与肝硬化组织比较,差异无统计学意义(P0.05)。结论 TRA1参与了肝硬化、肝癌的发生、发展,TRA1可能是肝硬化和肝癌潜在诊断标志物和治疗靶点。     

Loss of heterozygosity of the retinoblastoma gene in liver cirrhosis accompanying hepatocellular carcinoma

Carcinogenesis is a multistep process. Most hepatocellular carcinoma (HCC) is preceded by liver cirrhosis, but the genetic changes involved in cirrhosis are not known well. The present study was conducted to evaluate aberration of the retinoblastoma (RB) gene in HCC and adjacent non-tumorous liver using 22 patients with chronic liver damage accompanying HCC. The specimens obtained by microdissection from paraffinembedded tissues were analyzed using an assay based on the polymerase chain reaction for highly polymorphic nucleotide sequences of microsatellites in theRB gene. Out of 22 cases, 15 showed constitutional heterozygosity for the microsatellite markers. In 11 (73.3%) of these 15 informative cases, the primary HCC foci showed loss of heterozygosity (LOH). In 8 of these 11 doubly informative (informative and LOH-positive in primary HCC) cases, LOH was found in 20 (64.5%) of 31 microdissected non-tumorous foci. All of the non-tumorous foci showingRB loss were cirrhotic lesions but there were no foci of chronic hepatitis. The remaining 4 cases without LOH in HCC foci showed no LOH in non-tumorous lesions. In our study, LOH of theRB gene was frequently observed in liver cirrhosis surrounding tumor.Abbreviations RB retinoblastoma - HCC hepatocellular carcinoma - LOH loss of heterozygosity - PCR polymerase chain reaction - (CA) _n cytosine-adenine - (AT) _n adenine-thymine - n number of repeats     

Prediction of chronic hepatitis B, liver cirrhosis and hepatocellular carcinoma by SELDI-based serum decision tree classification

PURPOSE: To screen potential serological biomarkers and develop decision tree classifications of chronic hepatitis B, liver cirrhosis (LC) and hepatocellular carcinoma (HCC), respectively, with high prediction score for improving diagnosis of liver diseases. METHODS: The total serum samples were randomly divided into three training sets (41 HBV and 35 health; 36 LC and 35 health; 39 HCC and 35 health) and three testing groups (34 HBV and 38 health; 18 LC and 52 health; 42 HCC and 47 health). Selected WCX2 protein chip capture followed by SELDI-TOF-MS analysis was applied to generate the serum protein profiles. Subsequently serum protein spectra were normalized and aligned by Ciphergen SELDI Software 3.1.1 with Biomarker Wizard including baseline subtraction, mass accuracy calibration, automatic peak detection. Once the intensities of selected significant peaks from the training data set were transferred to further BPS analysis, an optimized classification tree with sequence-decision was established to divide training data set into disease group and control group successfully. A double blind test was employed to determine the clinical sensitivity and clinical specificity of three models. RESULTS: After comparative analysis of SELDI based serum protein profile between the cases of disease and healthy, a HCC decision tree classification with sensitivity of 94.872% and specificity of 94.286%; a LC decision tree classification with sensitivity of 91.667% and specificity of 94.286% and a HBV decision tree classification with sensitivity of 95.122% and specificity of 94.286% were produced by BPS respectively. When three decision tree models were challenged by the double-blind test samples, clinical sensitivity and clinical specificity of these models were predicted in diagnosis of three liver diseases (HCC: 90.48 and 89.36%; cirrhosis: 100 and 86.5%; HBV: 85.29 and 84.21%). CONCLUSION: SELDI-based decision tree classifications showed great advantages over conventional serological biomarkers in the diagnosis of chronic hepatitis B, LC as well as HCC.     

Recurrence of hepatocellular carcinoma 102 months after successful eradication and removal of membranous obstruction of the inferior vena cava

We report a 54-year-old Japanese woman who developed liver tumors 102 months after hepatic resection for hepatocellular carcinoma (HCC) and percutaneous transluminal angioplasty (PTA) for membranous obstruction of the inferior vena cava (MOVC), which is one form of Budd-Chiari syndrome. In the present admission workup showed no evidence of co-infection with hepatitis B and C viruses. Dynamic computed tomography (CT) and magnetic resonance imaging showed an enhanced lesion, 1.5cm in diameter, in segment 3 of the liver, and no obstruction of the inferior vena cava after PTA. CT during both arterial portography and hepatic arteriography revealed another lesion, showing different hemodynamics, in segment 2. The patient therefore underwent hepatic resection, and the tumors were diagnosed histologically as HCC. The two tumors differed in their morphological features, one containing abundant fibrous stroma, whereas the other did not. The nontumorous liver tissue showed central zonal fibrosis, i.e., reversed lobulation, and partial expansion of nodule-like formations, indicating lack of progression since the situation seen at the initial hepatectomy. The presence of nontumorous liver tissue showing the above features suggests that, even after successful treatment for relief of congestion, patients who have had MOVC should be followed closely for as long as possible because of the risk of HCC recurrence. This is the first reported case of HCC recurrence after successful treatment of MOVC.     

Value of serum C-reactive protein measurement in the detection of hepatocellular carcinoma superimposed on liver cirrhosis

We investigated whether, in Italian patients, C-reactive protein (CRP) determination could be considered a useful adjunct, complementary to ₁-fetoprotein, in the detection of liver cancer. CRP was determined by particle-enhanced nephelometry in 171 subjects (102 male, 69 female). Fifty-five patients had mild chronic liver disease (CLD), 45 cirrhosis (CIR), 38 hepatocellular carcinoma (HCC); 33 subjects were healthy controls. Patients with HCC and CIR had higher CRP levels (P<0.05) than those found in patients with CLD and controls. CRP higher than 5 mg/l was found in 30/38 (78.9%) patients with HCC, 28/45 (62.2%) patients with CIR, 16/55 (29.1%) patients with CLD (² 56.0,P<0.0001). Sensitivity, specificity and diagnostic accuracy of CRP in diagnosing HCC with respect to CLD+CIR were: 78.9%, 56.0% and 34.9%. However, when considered only in the subgroup of patients with ₁-fetoprotein below or equalling 30 ng/ml, they were 50.0%, 54.3% and 4.3% respectively. In conclusion, CRP concentration is frequently elevated in patients with HCC, however, it does not seem to improve the ability of ₁-fetoprotein to discriminate HCC from CIR.     

Frequent loss in chromosome 8p loci in liver cirrhosis accompanying hepatocellular carcinoma

Most hepatocellular carcinoma (HCC) is preceded by liver cirrhosis, but the genetic changes involved in cirrhosis are not well understood. We therefore studied loss of heterozygosity (LOH) in cirrhotic and neoplastic foci in livers of 14 patients with HCC. The samples, microdissected from paraffin-embedded tissues, were analyzed using a polymerase-chain-reaction-based assay for dinucleotide repeat polymorphisms on 8p. Of the 14 cases, 13 showed constitutional heterozygosity for the microsatellite markers. In 7 (54%) of these 13 informative cases, LOH was detected in the primary HCC and, in these 7 doubly informative (informative and LOH-positive in primary HCC) cases, LOH was found in 16 (70%) of 23 liver cirrhotic foci. The pattern of 8p allelic loss was identical in each doubly informative tumor; however, some of the liver cirrhotic foci harbored an 8p loss identical to that seen in the primary HCC, some harbored a different 8p loss, and some did not harbor any 8p loss. The remaining 6 cases without LOH on 8p in HCC showed no 8p loss in any cirrhotic foci. Presumably HCC could develop from cirrhotic cells harboring 8p loss.Abbreviations HCC hepatocellular carcinoma - LOH loss of heterozygosity - PCR polymerase chain reaction - AH adenomatous hyperplasia - AAH atypical adenomatous hyperplasia     

Membranous obstruction of the inferior vena cava and its causal relation to hepatocellular carcinoma.

Although rare in most countries, membranous obstruction of the inferior vena cava (MOIVC) occurs more frequently in Nepal, South Africa, Japan, India, China, and Korea. The occlusive lesion always occurs at approximately the level of the diaphragm. It commonly takes the form of a membrane, but may be a fibrotic occlusion of variable length. Controversy exists as to whether MOIVC is a developmental abnormality or a result of organization of a thrombus in the hepatic portion of the inferior vena cava. The outstanding physical sign associated with MOIVC are large truncal collateral vessels with a cephalad flow. A dilated vena azygous is seen on chest radiography. Definitive diagnosis is made by contrast inferior vena cavography. The long-standing obstruction to hepatic venous flow causes severe centrolobular fibrosis and predisposes to the development of hepatocellular carcinoma (HCC). Percutaneous balloon angioplasty, transatrial membranotomy, or more complex vena caval and portal decompression surgery should be performed to prevent these complications. HCC occurs in more than 40% of South African Black and Japanese patients with MOIVC, but less often in other populations. It is thought to result from the tumour-promoting effect of continuous hepatocyte necrosis, although the associated environmental risk factors have not been identified.     

Nerve growth factor involvement in liver cirrhosis and hepatocellular carcinoma

AIM To define NGF (nerve growth factor) and its highaffinity receptor trkANGF presence and distribution in fibrotic liver and in HCC, and to verify if NGF might have a role in fibrosis and HCC.METHODS Intracellular distribution of NGF and trkANGF were assessed by immunohistochemistry and immunoelectron microscopy in liver specimens from HCC,cirrhosis or both. ELISA was used to measure circulating NGF levels.RESULTS NGF and trkANGF were highly expressed in HCC tissue, mainly localized in hepatocytes, endothelial and some Kupffer cells. In the cirrhotic part of the liver they were also markedly expressed in bile ducts epithelial and spindle-shaped cells. Surprisingly, in cirrhotic tissue from patients without HCC, both NGF and trkANGF were negative. NGF serum levels in cirrhotic and/or HCC patient were up to 25-fold higher than in controls.CONCLUSION NGF was only detected in liver tissue with HCC present. Intracellular distribution suggests paracrine and autocrine mechanisms of action. Better definition of mechanisms may allow for therapeutic and diagnostic/prognostic use of NGF.     

Development from simple steatosis to liver cirrhosis and hepatocellular carcinoma: a 27-year follow-up case

Nonalcoholic fatty liver disease (NAFLD) is classified as nonalcoholic steatohepatitis (NASH) or simple steatosis (SS) according to histological findings. It is well recognized that NASH may develop into cirrhosis and hepatocellular carcinoma (HCC), both with unfavorable prognoses. Although the outlook of SS is reported to be better than that of NASH, the long-term prognosis of SS remains unclear. Here, we report the case of a patient who was diagnosed as having SS by a first liver biopsy, and later developed into cirrhosis and HCC over a period of 27 years. In 1980, a 42-year-old Japanese man was admitted because of abnormal liver function tests. He had no history of alcohol intake and was negative for hepatitis virus markers and autoantibodies. A liver biopsy specimen showed macrovesicular steatosis without ballooned hepatocytes, Mallory hyaline, lobular inflammation, or perisinusoidal/perivenular fibrosis, confirming the diagnosis of SS. The patient’s serum aminotransferase levels did not normalize despite repeated dietary instruction, and in 2001, liver histology demonstrated cirrhosis with mild steatosis and hepatocyte ballooning, leading to the diagnosis of NASH-related cirrhosis. HCC appeared in 2007. Overall, this patient progressed to cirrhosis and HCC in 20 and 27 years, respectively, following initial diagnosis. Platelet counts and degree of steatosis, as assessed by periodic ultrasonography, were seen to gradually reduce with progression of fibrosis. This case demonstrates that even a diagnosis of SS does not guarantee non-progression to cirrhosis and HCC, and careful follow-up is needed not only in patients with NASH, but also in those with SS.     

Complete membranous obstruction of inferior vena cava: Case treated by balloon dilatation

A 30 year-old male with the Budd-Chiari syndrome due to complete membranous obstruction to the inferior vena cava was successfully treated by Brockenbrough needle puncture followed by balloon dilatation of the membrane. The procedure produced marked symptomatic relief and excellent haemodynamic and angiographic results. The case is reported to highlight the application of balloon angioplasty in this rare condition.     

Budd-Chiari like syndrome in decompensated alcoholic steatohepatitis and liver cirrhosis

INTRODUCTIONBudd-Chiari Syndrome(BCS)is a rare,heterogeneous and potentially lethal condition caused by obstruction of the hepatic venous outflow tract[1],situated anywhere between the small hepatic venules until the right atrium[2].In Western countries,t…     

Membranous obstruction of the inferior vena cava and invasion of the inferior vena cava by tumour in hepatocellular carcinoma

A 28 yr old Zulu presented with a painful swelling in the right hypochondrium and severe swelling of the legs of short duration. The serum alpha-fetoprotein concentration was over 2 X 10(5) ng/ml and imaging showed a large hepatic mass-lesion. Radionuclide venography revealed no flow through the inferior vena cava but flow through a large collateral vessel. Contrast venography showed the upper portion of the inferior vena cava to be occluded: large collateral vessels arose from the lower vena cava and the iliac veins. The histological features were those of longstanding hepatic venous outflow obstruction with irregular centrizonal and portal fibrosis: severe acute centrizonal congestion was not seen. This combination of findings indicates the presence of both membranous obstruction of the inferior vena cava, a rare developmental abnormality which predisposes to hepatocellular carcinoma formation, and invasion by the tumour of the inferior vena cava via the hepatic veins, an uncommon complication of hepatocellular carcinoma.     

Clinical spectrum of chronic Budd-Chiari syndrome and surgical relief for 'coarctation' of the inferior vena cava

This report presents the clinical features of 78 cases of the chronic Budd-Chiari syndrome encountered over a period of 13 years. The diagnosis of hepatic venous outflow obstruction was confirmed by venographic studies in all cases. In 20 patients there was hepatic vein occlusion without inferior vena caval (IVC) obstruction (Group A). In 17 patients there was constriction of the IVC above the drainage site of the right hepatic vein which was patent (Group B). In 13 patients there was short segmental obstruction of the hepatic segment of the IVC along with blockage of the hepatic venous orifices (Group C). In 28 patients there was hepatic venous obstruction with long segment involvement of the IVC extending to varying lengths of the infrahepatic segment (Group D). Of particular interest are the operative findings in 12 of 17 patients of Group B of hour glass constriction of the IVC, which can be labelled as ‘coarctation of the IVC'. Dorsal cavoatrial bypass using a polytetra fluoroethylene graft has proved useful in Group B. Interesting histopathological findings of the liver in some of the cases are also described. The possible aetiology of the Group B cases is discussed.