History of Budd–Chiari Syndrome

Abstract

A clinical syndrome caused by obstruction of hepatic veins was described for the first time by George Budd in 1846. Fifty-three years later, Hans Chiari enriched the first description with clinical-pathological elements. Pathophysiological background of Budd–Chiari syndrome (BCS) was not known and several authors proposed different hypotheses such as syphilitic disease, endophlebitis, and trauma. The importance of an underlying condition of thrombophilia was recognized through the work of Parker in 1959, who reviewed the literature and found an association between BCS and thrombophilic conditions such as polycythemia vera, pregnancy, and estroprogestinic therapy. In the following years, the use of anticoagulants was proposed but only in the mid-1980s such therapy became generalized, with a consequent improvement of the survival. However, the initial fear of hemorrhagic complications discouraged this therapeutic approach, therefore different types of portosystemic shunts were conceived, but were associated with high morbidity and mortality. Two milestones in the treatment of BCS were represented by liver transplantation and trans-jugular intrahepatic portosystemic shunt that were first performed in 1976 and 1993, respectively. Such progress allowed modifying the treatment of BCS until the modern concept of stepwise therapy. The present chapter thoroughly reviews the major landmarks in the discovery and management of BCS.