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.     

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.     

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.     

Membranous obstruction of the inferior vena cava (obliterative hepatocavopathy, Okuda)

Confusion prevails throughout the world regarding the definition and classification of the Budd-Chiari syndrome. The original patients (Budd and Chiari) described had hepatic vein thrombosis, but this syndrome now encompasses various hepatic venous outflow blocks, of which membranous obstruction of the inferior vena cava (IVC) is the most common. This author has been suggesting that the classical Budd-Chiari syndrome or hepatic vein thrombosis and membranous obstruction of IVC or primary thrombosis of IVC at its hepatic portion are epidemiologically, pathologically and clinically different, and that they should be treated as two clinical entities that are not to be mixed. The two diseases have a different onset, different clinical manifestations and a different natural history. Whereas hepatic vein thrombosis is a severe disease with an acute onset, IVC thrombosis presents mildly at onset, but it recurs and eventually turns into a fibrous occlusion of IVC of varying thickness or stenosis of a various degree. The fibrous IVC occlusion is found as a mysterious thin membrane, but is more often much thicker than a membrane, and therefore 'membrane' is a misnomer. Although the genesis is not established, formation of a thin membrane may be an outcome of recurrent thrombosis. The past congenital vascular malformation theory no longer holds, because the disease occurs mostly in adulthood, and transformation of thrombosis into a membrane has now been well documented pathologically as well as clinically. This author suggests that a term 'obliterative hepatocavopathy' replace membranous obstruction of IVC, the term 'Budd-Chiari syndrome' be abandoned, and that primary hepatic venous outflow block be divided into primary hepatic vein thrombosis and primary IVC thrombosis (obliterative hepatocavopathy).     

Complete membranous obstruction of the inferior vena cava: clinical characteristics of Chinese patients

Abstract. Objectives. To analyse the clinical presentations of membranous-type Budd-Chiari syndrome and the long-term outcome of balloon angioplasty. Design. A case-control study. Patients who received abdominal sonography and were suspected of having hepatic venous outflow obstruction were advised to receive cardiac catheterization and angiography. Setting. Patients were collected from Chung Hisao Hospital and Chang Gung Medical Centre. Angioplasty was done at Chang Gung Medical Centre. Subjects. Seventeen patients who had the symptoms and signs of portal hypertension were suspected of having hepatic venous outflow obstruction after abdominal sonography and a radionuclide liver scan and eight of them were confirmed as having membranous-type obstruction. Interventions. Simultaneous catherization and contrast angiography was performed via the right brachial and femoral veins. Angioplasty was performed afterwards. Main outcome measures. The remission of clinical symptoms and signs and a decrease of the pressure gradient between the right atrium and inferior vena cava were used as indicators of successful intervention. Results. All patients had remarkable remission of symptoms and signs. The average pressure gradient between right atrium and inferior vena cava dropped from 16.5 ± 1.6 to 6.5 ± 1 mmHg (mean ± su). Conclusion. The clinical presentations of membranous-type Budd-Chiari syndrome are somewhat different from hepatic vein occlusion. Balloon angioplasty may be a modality for curing this disease. A larger patient population is necessary to confirm this study.     

Multiple venous thromboses and membranous obstruction of inferior vena cava in association with hereditary protein C deficiency: A case report

A forty year old male presented with multiple dilated venous channels over the whole body involving both inferior and superior vena caval territories, along with features of chronic liver disease and portal hypertension. On investigation, he was found to have membranous obstruction of the inferior vena cava (MOIVC) as well as obstruction of both brachiocephalic and right subclavian veins and 'hereditary protein C deficiency'. He was managed successfully by percutaneous transluminal balloon angioplasty for the inferior vena cava (IVC) obstruction and was doing well on follow-up.     

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.     

Cavographic study of an early stage of obstruction of the hepatic portion of the inferior vena cava

BACKGROUND: Liver disease caused by a chronic lesion of the hepatic portion of the inferior vena cava (IVC) is clinically characterized by dilated superficial veins in the body trunk with cephalad flow, hepatomegaly and splenomegaly. Cavography shows stenosis or complete obstruction near the cava-atrial junction. METHODS: Early (acute and subacute) forms of the disease were recognized. The early stage of the disease manifested as jaundice, hepatomegaly or ascites and fever. Patients with acute and subacute onset of the illness with no past history of liver disease were studied with inferior vena cavography. Some of the patients had repeat cavography at 6 months and at 1 year after the initial investigations. RESULTS: Three types of cavographic lesions were observed in the early stages of the disease: type 1, linear lucent area in the IVC close to cava-atrial junction; type 2, a smooth or irregular narrowing of almost the whole segment of the hepatic portion of the IVC; and type 3, a constriction or narrowing of a segment of the IVC near the cava-atrial junction. The first two types were associated with the acute stage of the disease and type 3 with the subacute stage. Type 2 and 3 lesions were associated with post-stenotic dilatation (PSD) close to the atrium. Lucent areas resulting from thrombosis are common in PSD. The acute and subacute hepatic IVC diseases in Nepal are commonly associated with bacterial infection. CONCLUSIONS: It is postulated that the early cavographic lesions are consistent with thrombosis and thrombophlebitis of the hepatic portion of the IVC, and that resolution of the lesions leads to the development of stenosis and to complete obstruction.     

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。     

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.     

Budd–Chiari syndrome due to membranous obstruction of inferior vena cava in a patient with Behcet's disease

Budd–chiari syndrome is a well known complication of Behcet's disease. All cases of Behcet's disease with Budd–Chiari syndrome that have been reported in the literature had thrombosis of hepatic veins or inferior vena cava. We describe a 33‐year‐old woman with Behcet's disease who developed Budd–Chiari syndrome due to a membranous web of the inferior vena cava.     

Balloon angioplasty for complete obstruction of inferior vena cava: Needle puncture followed by balloon dilatation

We report herein 2 cases with complete obstruction of the suprahepatic portion of the inferior vena cava successfully treated by balloon angioplasty after crossing the obstructing segment with the stiff end of the guidewire in 1 case and the Brockenbrough's trans-septal needle in the other. The gradients across the obstruction fell from 13 and 20 mm Hg to 3 and 4 mm Hg, respectively. Angioplasty resulted in dramatic clinical hemo-dynamic and angiographic improvement.     

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.     

Aneurysmal dilatation of the inferior vena cava

Aim: Inferior vena cava aneurysms (IVCA) are rare, unlike aortic aneurysms. The diagnosis and treatment is challenging. This study defines clinical and echocardiographic findings in a prospective cohort of sixteen patients with fusiform IVCA. Methods and Results: All patients referred to the Mayo Clinic between January 2006 and July 2009 for a clinically indicated echocardiogram (36,128 patients) were screened for a dilated IVC. Sixteen cases of fusiform IVCA were identified. Eleven cases (68.8%) were female. Mean age at presentation was 76 years (range 51–89). Eleven (68.8%) had structural heart disease: with right ventricular (RV) dysfunction in 45.5% (n = 5), moderate or greater tricuspid regurgitation (TR) was seen in 36.4% (n = 4) and RV enlargement was seen in 18.2% (n = 2). The most common clinical indication for echocardiography was dyspnea (25%; n = 4) and heart failure (18.8%; n = 3). The mean IVCA diameter was 4.1 cm (range 3.8–5 cm) and the mean length of the aneurysms was 6.2 cm (range 3.5–8.7 cm), with mean right ventricular systolic pressure of 55 mmHg (range 31–105 mmHg). Five (31.3%) had at least a moderate reduction in right ventricular ejection fraction and five (31.3%) had significant TR. Among these five patients with significant TR, severe TR was present in 80%; (n = 4) and moderate to severe TR was present in 20%; (n = 1). Conclusions: IVC aneurysms are more common in the elderly, and is associated with an increase in right sided heart pressures, significant TR, and RV dysfunction. (Echocardiography 2011;28:833‐842)     

电视纵隔镜检查在上腔静脉综合征病因诊断中的应用

目的探讨电视纵隔镜检查在上腔静脉综合征病因诊断中的应用价值。方法对13例上腔静脉综合征患者,经颈部行电视纵隔镜检查,其中4例联合应用超声检查。结果13例均成功取到活检组织,病理检查证实纵隔小细胞未分化癌或神经内分泌癌8例,淋巴瘤3例,胸腺瘤2例。平均检查时间为35min,无手术并发症及死亡者。结论通过电视纵隔镜检查可对上腔静脉综合征的病因作出准确诊断．该检查安全可行。     

Clinical features and etiology of hepatocellular carcinoma arising in patients with membranous obstruction of the inferior vena cava: in reference to hepatitis viral infection

BACKGROUND AND AIMS: Budd-Chiari syndrome (BCS) comprises hepatic vein thrombosis and inferior vena cava (IVC) obstruction known as membranous obstruction of the IVC (MOVC). The latter is frequently complicated by hepatocellular carcinoma (HCC).The etiology of MOVC-associated HCC in relation to hepatitis viral infection is not known. In this study, we investigated the clinical features and etiology of HCC in MOVC. METHODS: Membranous obstruction of IVC and HCC were diagnosed and studied by using imaging techniques. Sera from patients with MOVC, complicated by HCC, were examined for hepatitis viral antigens and antibodies (hepatitis B surface antigen (HBsAg), antibody to HBsAg (anti-HBs), antibody to hepatitis B core antigen (anti-HBc) and third generation antibody to hepatitis C virus (anti-HCV)) and for hepatitis viral nucleic acids (hepatitis B virus (HBV)-DNA, hepatitis C virus (HCV)-RNA, hepatitis G virus (HGV)-RNA and TT virus DNA). RESULTS: We studied 12 patients with BCS who were seen between April 1968 and February 1999. All of them had MOVC. Hepatocellular carcinoma developed in three (25%) of them. There were no obvious differences in the clinical features and imaging findings concerning MOVC between patients with and without HCC. Hepatocellular carcinoma in these three patients showed no clear trend in clinical features and imaging findings. Of the hepatitis viral markers examined, HBsAg, anti-HBc and HBV-DNA were positive in only one of three patients with HCC and all of the viral markers were negative in the other two patients. CONCLUSIONS: Chronic congestion in the liver, caused by an outflow block of hepatic veins and subsequent histopathologic change, must have led to HCC in two patients without any hepatitis viral markers. Patients with MOVC should be followed closely as a high-risk group for HCC.     

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.