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.     

Surgical anatomy of the inferior vena cava ligament

BACKGROUND/AIMS: The inferior vena cava ligament is a fibrous membrane located around the inferior vena cava. Few reports exist on the ligament's location, attachment to the liver, or the inferior vena cava. METHODOLOGY: We obtained 16 specimens of human liver and inferior vena cava from cadavers. The inferior vena cava ligament was photographed and then dissected for histological examination. Relationships among the ligament, inferior vena cava, and liver were examined microscopically. The numbers and diameters of veins, arteries, and lymph vessels at least 1 mm in diameter were recorded. RESULTS: The cranial margin of the inferior vena cava ligament was ended in a blind loop. The cranial portion above the mid-portion of the Spiegel lobe was thicker than the caudal portion. The ligament was attached to the right and left hepatic veins. The mean length of the right side of the inferior vena cava ligament was 37.0 mm and the mean width 15.6 mm. The inferior vena cava ligament had a mean thickness of 0.8 mm (thin end) and 2.5 mm (thick end). Although the inferior vena cava ligament was usually tightly continuous with the liver capsule, microscopically the attachment between the ligament and the inferior vena cava was loose. The mean number and diameter of veins in the inferior vena cava ligament was 1.0 and 1.4 mm, respectively. The mean number and diameter of arteries was 0.2 and 2.4 mm, respectively. The mean number and diameter of lymphatic vessels was 2.8 and 1.7 mm, respectively. CONCLUSIONS: After dissection of the inferior vena cava ligament, major hepatic veins can be dissected extrahepatically. Because the ligament is wider caudally, the forceps should be inserted caudocranially during separation. Since both the number and diameters of lymphatic vessels in the ligament are large, the ligament should be ligated and cut.     

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.     

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 and systemic lupus erythematosus

A 37-year-old woman who presented with systemic lupus erythematosus was found to have a membranous obstruction of the inferior vena cava above the hepatic veins, with an extensive collateral circulation. The obstruction was relieved by a renal vein-right atrial interposition graft, and this was followed by disappearance of the anti-nuclear and anti-DNA antibodies. We postulate that the systemic lupus erythematosus might have been provoked by life-long immune stimulation due to bowel-derived antigens bypassing the hepatic reticuloendothelial system and reaching the systemic circulation, or by immune hyperreactivity associated with the abnormal abdominal venous circulation.     

Anomalies of the inferior vena cava in patients with iliac venous thrombosis

BACKGROUND: Cases of deep venous thrombosis in the lower extremities triggered by abnormalities of the vena cava have been reported. OBJECTIVE: To describe anomalies of the inferior vena cava in patients with deep venous thrombosis. DESIGN: Prospective, consecutive case series. SETTING: University Hospital, Graz, Austria. PATIENTS: 97 patients with deep venous thrombosis. INTERVENTION: Sonography, venography, or both to diagnose deep venous thrombosis; magnetic resonance angiography to image the inferior vena cava. MEASUREMENTS: Anomalies of the inferior vena cava imaged by magnetic resonance angiography. RESULTS: 31 of 97 patients showed thrombotic occlusion of iliac veins (common and external iliac vein [ n = 29] or external iliac vein [ n = 2]). Five of 31 patients (3 men, 2 women) had an anomaly of the inferior vena cava. Anomalies were missing inferior vena cava, hypoplastic hepatic segment, and missing renal or postrenal segments. Patients with anomalies were significantly younger than the 92 patients without (mean age+/-SD, 25+/-6 years vs. 53+/-19 years; P = 0.002). In 2 patients with anomalies, the thrombotic occlusion was recurrent. CONCLUSIONS: An anomaly of the inferior vena cava should be suspected if thrombosis involving the iliac veins is seen in patients 30 years of age or younger. Patients with both an anomaly and thrombosis may be at higher risk for thrombotic recurrence.     

Acute thrombosis of pelvic and leg veins in agenesis of the renal segment of the inferior vena cava

A 19-year-old, otherwise asymptomatic man presented to the hospital of orthopaedic surgery with acute severe pain like lumbago. Symptomatic treatment was performed after extensive orthopaedic diagnostic procedures. On the third day after admission he showed clinical signs of deep vein thrombosis with painful swelling and livid discoloration of both legs. Colour duplex ultrasound revealed complete thrombosis of the leg and pelvic veins bilaterally, but the cranial extent was not clear. Contrast-enhanced helical computer tomography of the abdomen and the pelvis confirmed deep pelvic vein thrombosis and showed extension into the inferior vena cava. Moreover, the study revealed the agenesis of the renal segment of the inferior vena cava with collateral flow through dilated lumbar veins to enlarged azygous and hemiazygous, through vertebral and paravertebral venous plexus. The renals were drained via dilated capsular veins. The agenesis of renal vena cava is a very rare anomaly causing acute thrombosis of the deep leg and pelvic veins. Other risk factors of thromboembolic disease were not found. The patient was treated successfully with systemic thrombolysis. Therefore we used ultra-high streptokinase infusion (9 million units over 6 hours). Colour duplex ultrasound revealed good flow into deep leg and pelvic veins after three cycle of lysis. Magnetic resonance angiography of the abdomen and pelvis was performed to evaluate the successful fibrinolysis with complete recanalisation of the pelvic veins and to demonstrate the venous anatomy. Permanent oral anticoagulation with phenprocoumon is indicated to decrease the high rate of recurrent thrombosis. Compression stockings were prescribed. To prevent thrombosis, additional risk factors like smoking, immobilization and unusual physical activity should be strictly avoided.     

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.     

The role of membranous obstruction of the inferior vena cava in the etiology of hepatocellular carcinoma in Southern African blacks

Membranous obstruction of the inferior vena cava has been incriminated as a risk factor for hepatocellular carcinoma in South African Blacks and in Japanese. However, the frequency with which this anomaly is found in patients with hepatocellular carcinoma, and hence its numerical importance as an etiological association of the tumor, has not been ascertained. Using radionuclide and contrast venography as well as necropsy and laparotomy examination, we investigated 162 unselected southern African Blacks with hepatocellular carcinoma together with appropriate controls for the presence of membranous obstruction of the inferior vena cava. Membranous obstruction of the inferior vena cava was detected in six of 162 (3.7%) hepatocellular carcinoma patients, compared with one of 279 subjects (0.36% p = 0.011) dying a violent death, none of 55 patients (p = 0.169) with malignant disease other than hepatocellular carcinoma and eight of 150 patients (5.3%; p = 0.336) being investigated for conditions which might have been associated with membranous obstruction of the inferior vena cava. Six of the 15 individuals (40%) found to have membranous obstruction of the inferior vena cava had concomitant hepatocellular carcinoma, confirming that membranous obstruction of the inferior vena cava constitutes a risk factor for the development of the tumor. However, only a very small proportion of hepatocellular carcinoma patients have this abnormality, so that it is a minor causal association of the tumor only.(ABSTRACT TRUNCATED AT 250 WORDS)     

The portal vein as collateral in inferior vena cava obstruction.

In five patients with obstruction of the inferior vena cava, collateral flow to the portal vein was demonstrated. Although there are many reports on caval occlusion, only 21 cases with this collateral circulation pattern have been reported. The reasons for this are most likely technical: To demonstrate this circulation, a large volume of contrast medium must be used and, because of the low flow velocity in the numerous wide collaterals, delayed films are also necessary.     

Intrapericardial control of the inferior vena cava from the abdominal cavity

Vascular isolation of the liver is a useful technique in major hepatic surgery that involves hepatic veins and the inferior vena cava. In some patients, exposure of the suprahepatic inferior vena cava is suboptimal from the abdominal cavity, and extension into the chest is required. This report details technical considerations of the control of the inferior vena cava within the pericardium from the abdominal cavity, through a vertical incision in the diaphragm, and without the need for a thoracic incision. We review the clinical situations when a transdiaphragmatic, intrapericardial access of the inferior vena cava should be considered.     

Membranous obstruction of the inferior vena cava associated with a myeloproliferative disorder: a clue to membrane formation?

Although membranous obstruction of the inferior vena cava is frequently assumed to be a congenital defect, it has recently been hypothesized that it could be acquired and secondary to thrombosis at the ostium of hepatic veins. We report the case of a membranous obstruction of the inferior vena cava responsible for isolated hepatomegaly in a young Frenchman. No known cause of thrombosis was found but spontaneous formation of erythroid colonies occurred on bone marrow cultures. This indicated the existence of a latent myeloproliferative disorder, a frequent cause of hepatic vein thrombosis. We suggest that this association of a membranous obstruction of the inferior vena cava with a known, albeit often overlooked, cause of hepatic vein thrombosis provides strong support to the thrombosis theory of membrane formation.     

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.     

Histopathology of membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome.

It is generally believed that membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome is caused by congenital malformation. However, it does not explain the late onset of the disease. In the current study, hepatic portion of the inferior vena cava and hepatic veins were studied in 17 autopsy cases of the Budd-Chiari syndrome, 16 of which had no demonstrable cause (idiopathic). A sufficient amount of vena cava tissue was available for evaluation in 15 cases. Nine had membranous obstruction, with thickness varying from 3 to 8 mm. Thrombus formation was recognized in 7 of 9 cases. Occlusion of hepatic vein orifices of varying degree was present in 8 cases. In these occluding lesions, the basic structure of the venous wall was maintained. The intima was transformed into a fibrous laminar structure, and organized thrombi of varying ages were recognized; they were a mixture of fresh thrombi, organized thrombi, fibrous tissues, recanalizations, and calcifications. It is concluded that in these cases of the Budd-Chiari syndrome, occluding and stenosing lesions in the inferior vena cava and hepatic veins were thrombosis and its sequelae. There was no indication of congenital malformation.     

Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa

This study was undertaken to establish the frequency of membranous obstruction of the inferior vena cava in South Africa, to characterize the pathology of the lesion and to define its relationship to hepatocellular carcinoma. Over a 9-yr period 101 cases of membranous obstruction of the inferior vena cava into the right atrium was occluded in all cases in which it was examined, and two basic patterns of the abnormality in the hepatic portion of the inferior vena cava have been described. The histologic picture in the liver was a chronic congestive fibrosis. In 44 cases of congestive fibrosis, diagnosed from liver biopsy specimens from black patients over a 5-yr period, vena caval membranes were demonstrated on cavography in 38 (86.4%). Hepatocellular carcinoma developed in 48 of the 101 cases (47.5%), indicating the importance of this defect in the pathogenesis of hepatocellular carcinoma in South Africa.     

Percutaneous balloon angioplasty of membranous obstruction of the inferior vena cava

BACKGROUND: Membranous obstruction of the inferior vena cava is common in African and Asian countries. METHODS AND RESULTS: Between January 1999 and January 2002, 19 patients were prospectively studied. The mean age of the patients was 38+/-6.9 years. All of them had swelling of the abdomen and ankle edema. Five patients (26.3%) had jaundice, 9 (47.3%) had hepatomegaly, and 5 (26.3%) splenomegaly. Ultrasonography could detect the site of obstruction in 18 patients (94.7%). Vena cavography demonstrated obstruction of the inferior vena cava at the level of the diaphragm, with 2 patients (10.5%) having additional intrahepatic obstruction. The mean pressure gradient was 22+/-3.5 mmHg. Seventeen patients underwent balloon angioplasty using a Joseph balloon. The procedure was successful in 15 patients (88.2%). The post-angioplasty mean pressure gradient was 5+/-1.4 mmHg. On follow-up, 3 patients (20%) developed features of restenosis: out of them, 2 underwent successful redilatation. CONCLUSIONS: Balloon angioplasty of membranous obstruction of the inferior vena cava is feasible with a high success rate, without any rupture of the inferior vena cava.     

Surgical repair for entire inferior vena cava occlusion in Budd-Chiari syndrome

Successful repair of thrombotic occlusion of the entire inferior vena cava, including the iliac and hepatic veins, was performed on a 51-year-old man with Budd-Chiari syndrome associated with severe liver dysfunction.     

A case of Kartagener's syndrome with bilateral superior vena cava and absent inferior vena cava

A 39-year-old man with a history of repeated attacks of acute bronchitis and pneumonia since childhood was admitted complaining of cough, sputum, and a high fever. A diagnosis of Kartagener's syndrome was made because of the presence of mirror-image dextrocardia with complete situs inversus, bronchiectasis in right B6 and B10, and chronic sinusitis. Examination of the ciliary ultrastructure of the bronchial epithelium showed defective inner dynein arms and impaired nasal clearance was suggested by the saccharin particle method. Contrast venography of great veins revealed a McCotter type II bilateral superior vena cava and an absent inferior vena cava with (hemi-) azygos continuation. A search to find a similar case in the literature failed. Abnormal ciliary movement from the embryonic period was implicated as a causative factor in Kartagener's triad and the morphological abnormalities of the great veins.     

Membranous obstruction of the inferior vena cava in the United States

The pathogenesis of membranous obstruction of the inferior vena cava (MOVC) is unclear. Although the lesion is rare in the United States compared to Japan, India, and black South Africa, it has been responsible for 23% of cases of hepatic outflow obstruction we have encountered in the ethnically heterogeneous indigent population of Los Angeles. Most patients with MOVC are male. In contrast, recent series of patients with Budd-Chiari Syndrome (BCS) have demonstrated a female predominance. Compared to BCS without involvement of the inferior vena cava (IVC), patients with MOVC have more chronic symptoms. Large truncal collaterals, particularly on the back, strongly suggest MOVC. In patients without this sign, a high index of diagnostic suspicion is required. Chronic hepatitis B infection occurs with increased frequency in these patients. Chest radiograph may show an enlarged azygous shadow. Liver-spleen scan is not helpful, and the liver biopsy is frequently nondiagnostic. A useful screening procedure for hepatic outflow block is transhepatic portal pressure measurement demonstrating aberrant hepatic veins with pressures higher than in the portal vein and, occasionally, hepatofugal portal flow. Transcardiac membranotomy appears to be symptomatically effective in patients with MOVC and at least one patent hepatic vein. It is not known whether this operation will prolong life and prevent the development of hepatocellular cancer, which may occur in up to 48% of these patients. The correct therapeutic approach has not been established for those patients whose lesion is not amenable to surgery because of extensive IVC occlusion or absence of patent hepatic veins.