Recurrence of the Budd-Chiari syndrome after orthotopic liver transplantation.

Obstruction of the hepatic venous outflow with or without involvement of the vena cava results in the Budd-Chiari syndrome (BCS). BCS may be limited to the liver but there is a variety of systemic disorders forming the etiology 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 option 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 complete documentation. The literature is reviewed and the probable underlying causes for recurrent disease after OLTx for BCS are discussed including therapeutic consequences.