ABSTRACT
Introduction Budd-Chiari syndrome (BCS) is a very rare condition characterized by hepatic venous outflow obstruction at the level of the suprahepatic inferior vena cava (IVC). This obstruction is caused by an IVC lesion/occlusion and/or occlusion of the hepatic veins (HVs) (Figure 83.1).1-3 There are several types of BCS, based on its etiology (e.g. primary and secondary) and underlying pathogenesis (e.g. congenital, inflammatory and/or infectious origin).4-9 The primary/idiopathic BCS has great geographic and racial variance with regard to its underlying etiology: the membranous obstruction of vena cava (MOVC)5,7 or hepatic vein thrombosis due to the hypercoagulable state.6,9 There is a striking difference between Asian/African patients and Caucasian patients,4-9
where MOVC is the most frequent cause of primary BCS among the Asian/African populations and the hypercoagulable state is the most frequent cause of BCS among the Caucasian population. The developmental/congenital anomaly theory is therefore considered the most compatible pathogenic mechanism with Asian/African patients while the thrombosis theory is most compatible with Caucasian patients.
