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Background: Disease of hepatic portion of the inferior vena cava (IVC) now renamed hepatic vena cava syndrome (HVCS) is an insidious onset chronic disease characterized by recurrent acute exacerbations and development of cavo-caval collaterals. In adults it is complicated by high incidence of liver cirrhosis. Aim of this study was to assess the incidence of liver cirrhosis in children with HVCS and discuss its pathogenesis., Method: One hundred and seventy eight children with HVCS were followed up with ultrasonography (USG), routine hematology and liver tests. During acute exacerbations, cultures of blood and ascitic fluid for aerobic microorganisms were also done. Diagnosis of liver cirrhosis was based on transformation of the liver parenchymal echo-texture from normal to uniformly coarse with rounded edges in follow-up USG It was confirmed by direct inspection of the liver or by biopsy in six. The duration from disease onset to detection of liver cirrhosis was also assessed., Results: HVCS was seen in children from poor socio-economic background. Forty nine patients (27.5%) developed liver cirrhosis within a few months to a few years of onset of the disease. Development of cirrhosis was related to frequency or severity of acute exacerbations and not to duration of illness or severity of the caval lesion., Conclusion: The cause of cirrhosis in HVCS was the ischemic injury of the hepatocytes and sinusoids following thrombosis of the IVC and/or intra-hepatic veins during severe acute episodes or exacerbations precipitated by bacterial infection. Since such acute exacerbations are amenable to medical treatment, HVCS may be a cause of preventable liver cirrhosis in developing countries.

Background: Little is known about the prevalence of hepatitis B virus (HBV) DNA and the genotype distribution among patients with liver diseases in Nepal, where obstruction of the hepatic portion of the inferior vena cava (IVCO) is common. The aim of the present paper was to assess the roles of HBV infection and IVCO in liver cirrhosis (LC) and hepatocellular carcinoma (HCC) in Nepal., Methods: Serum samples from 121 patients (89 male, 32 female; age, 55.0 +/- 13.6 years) with or without IVCO consisting of 70 LC patients and 51 HCC patients in Nepal, were tested for HBV-DNA., Results: The HBV-DNA was detected in 68 patients (56%) including 20 hepatitis B surface antigen (HBsAg)-negative patients: 33 LC patients (47%) and 35 HCC patients (69%) had detectable HBV-DNA (P = 0.0303). Among the 89 patients with IVCO, HBV-DNA was detected in HCC patients significantly more frequently than in LC patients (80%vs 43%, P = 0.0005). The frequency of HBV viremia was significantly higher among HCC patients with IVCO than those without (80%vs 44%, P = 0.0236), and that of HBV viremia with IVCO was significantly higher among HCC patients than among LC patients (55%vs 27%, P = 0.0153). The HBV genotypes A and D were predominant, and genotype A was significantly more frequent among HCC patients than among LC patients (22%vs 6%, P = 0.0090). Among HCC patients, those with genotype A HBV were significantly younger than those with genotype D (43 +/- 13 vs 57 +/- 12 years, P = 0.0252)., Conclusion: Hepatitis B virus alone (especially genotype A) or in concert with IVCO may be responsible for development of HCC in Nepal.

Pleural effusion is not uncommon in developing countries. It is usually considered to be due to tuberculosis and treated with anti-tubercular chemotherapy without much diagnostic workup. Hepatic vena cava disease (HVD), a disease caused by obliterative lesion of the hepatic portion of inferior vena cava induced by bacterial infection is common in developing countries. We report here the occurrence of pleural effusion in 10% of the cases of HVD. Four patients, one with acute and three with chronic HVD that presented with pleural effusion are described. Pleural effusion in HVD responded to treatment with antibiotic and diuretic. In developing countries HVD should be considered in the differential diagnosis of pleural effusion. It is postulated that bacterial infection and sodium retention resulting from acute caval obstruction are important in the pathogenesis of pleural effusion in HVD.

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.

Hepatic IVC obstruction though common is usually misdiagnosed because of lack of appreciation of the disease. Patient with chronic disease may develop acute exacerbation, which may be precipitated by surgery or endoscopic procedures. It is a report of a case of chronic IVC disease with acute development of ascites following gallbladder surgery.

Budd-Chiari syndrome (BCS) was initially defined as a symptomatic occlusion of the hepatic veins, but subsequent reports on various obliterative changes that occur in the hepatic portion of the inferior vena cava (IVC) and hepatic vein orifices have resulted in a broadened and ambiguous definition. Membranous obstruction of the inferior vena cava has been regarded by many as a congenital vascular malformation, but its relation to the classical BCS has remained obscure. With modern imaging and recent histological study of new cases, membranous obstruction of the IVC is now considered to be a sequela to thrombosis. How to classify various forms of occlusion and stenosis of the IVC and hepatic vein ostia is a major challenge. In this review, we emphasize that primary hepatic vein thrombosis (classical Budd-Chiari) and an obliterative disease predominantly affecting the hepatic portion of the IVC, both of which account for most patients with venous outflow block, are clinically quite different. In the West, the former is more common than the latter, which constitutes the vast majority of cases of outflow block in developing countries such as Nepal, South Africa, China, and India. The latter is frequently complicated by hepatocellular carcinoma (HCC), and primary hepatic vein thrombosis is not. The major cause of thrombosis is a hypercoagulable state in hepatic vein thrombosis, but more of the latter cases are idiopathic. The clinical presentation of the latter is milder, and onset is frequently inapparent, whereas the former is more severe, sometimes causing acute hepatic failure. Markedly enlarged subcutaneous veins over the body trunk characterize the latter. We propose that these two disorders be clinically distinguished with a suggested term "obliterative hepato-cavopathy" for the latter against classical BCS.

Intra hepatic inferior venacaval (IVC) obstruction at the site or just above hepatic vein opening in the IVC, is a common hepatic problem in Nepal. These patients either present as a chronic IVC obstruction with insidious onset or may present with rapid onset disease (acute/subacute disease). The former type may be asymptomatic (20%) or may have features of chronic liver dysfunction. The latter presents with rapid onset ascites. Presence of hepatomegaly with flank/back veins are characteristic clinical features of the disease. The disease is invariably encountered amongst rural poor, alcoholic and in the peripartum period. Ultrasonography, IVC graphy hepatogram and liver biopsies are the usual diagnostic modalities. Twenty four percent of the 126 chronic hepatic IVC disease had associated liver cell cancer at our centre. The aetiology of the disease is unclear. Presently it is believed that local thrombophlebitis in IVC causes such obstruction. Despite, surgical and radiological intervention, the ideal management in such patients is yet to be established. We believe conservative therapy, particularly prevention and early therapy of sepsis in such patients prolongs survival.

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.

Chronic liver disease due to obstructive lesion of the hepatic inferior vena cava (IVC) is common in Nepal. This study presents 10 patients with cavographically documented membranous obstruction of the hepatic IVC with minimal symptoms and a benign course. One patient with an incomplete membrane had balloon dilation of the lesion. The duration of the disease among these patients varied from 5 months to 23 yr; eight patients have been followed for periods varying from 1 to 15 yr, with an average of 6 yr. In developing countries, the possibility of hepatic IVC obstruction should be considered in the differential diagnosis of hepatomegaly or hepatosplenomegaly, even in the absence of ascites and external cava-caval collateral. Routine liver and hematology tests in these patients usually are normal, and liver biopsy findings may not always be specific. Ultrasound, however, has proved to be the best diagnostic procedure for the condition. The lesion can be demonstrated easily by cavography. The disease runs a chronic course, and, in the absence of resistant ascites or repeated variceal bleeding, surgical procedures such as cavoatrial bypass, which are a significant cause of death in developing countries, may be contra-indicated.