Recurrence of the Budd-Chiari syndrome after orthotopic liver transplantation

Obstruction of the hepatic venous outflow with or without involvement of th e vena cava results in the Budd-Chiari syndrome (BCS). BCS may be limited t o the liver but there is a variety of systemic disorders forming the etiolo gy of BCS in the majority of cases. Surgery has a major impact on treatment of the BCS within a wide range of therapeutic strategies. The ultimate opt ion of surgical management of the BCS is orthotopic liver transplantation ( OLTx). The case of a patient with recurrent disease more than 5 years after OLTx for BCS due to paroxysmal nocturnal hemoglobinuria is analyzed with c omplete documentation. The literature is reviewed and the probable underlyi ng causes for recurrent disease after OLTx for BCS are discussed including therapeutic consequences.