Clinical spectrum of chronic Budd‐Chiari syndrome and surgical relief for ‘coarctation’ of the inferior vena cava

Abstract

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.

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

“…In the Western world, most patients with BCS have primary hepatic vein thrombosis without MOVC and have an underlying disease that leads to BCS, such as coagulopathy, autoimmune diseases and myeloproliferative diseases 19–21 . In contrast, most patients with BCS in Japan, 2,3,5–7 Nepal, 22 India 23,24 and South Africa 8,9 have MOVC, but the etiology is still unknown. Okuda et al concluded that primary hepatic vein thrombosis (the classical BCS) and IVC obstruction (MOVC) should be considered separately because these two types of BCS are very different in epidemiology, clinical features and natural history 2 .…”

Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment

“…In our series, IVC involvement in the majority of cases was due to IVC thrombosis. Previously, MOVC and IVC thrombosis were considered to be idiopathic in origin by most Indian and Asian workers [5,6,11,[13][14][15]17,35,49,50,53,54] ; but this view has been challenged recently in a few reports [22,29,30,42,[55][56][57][58] . In cases of IVC thrombosis in our study, the etiology was identified in 50%.…”

“…All previous Indian series, except two [29,32] , describe the predominance of IVC up to 79.2% [7,9,[11][12][13]15,30,39,40,42] or IVC-HV obstruction up to 57.7% [14,24,25,35] ; HV involvement was described in 0%-32% of cases in these series [7,9,[11][12][13][14][15]24,25,30,35,39,40,42] . Of the two distinct previous series, one (total 53 cases) showed similar frequency of HV (35.8%), IVC (33.9%) and combined IVC-HV involvement (30.1%) [29] ; whereas the other series involving only chronic BCS showed predominance of HV (45.9%) followed by combined IVC-HV (29.7%) and IVC involvement (24.3%) [32] .…”

“…Previously, MOVC was thought to be the predominant cause of IVC involvement (20.4%-58.6%) and of BCS in India [6,7,[11][12][13][14][15]17,29,35,39] , as well as in Asian countries [49,50,53] . In two recent Indian studies, MOVC was present in 0% and 17.2%, while IVC involvement was seen in 56.2% and 62% cases, respectively [24,40] .…”

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

“…The majority of patients are adults, and this simple fact alone speaks against the congenital theory; clinical presentations of child cases are the same as adults 20,21 . Victor et al 8 and Madanagopalan et al 22 who long maintained that IVC stenosis confined to the hepatic portion is an equivalent of coarctation of the aorta, have recently changed their stance and admit that IVC stenosis could be sequela to thrombosis; they experienced filariasis along IVC that caused thrombosis and subsequent stenosis 23 . Kage et al studied 16 autopsy cases of Budd–Chiari syndrome in Japan, the majority being MOVC, and demonstrated that the membrane was an organized old thrombus often consisting of several portions of varying ages 24 .…”