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Results of liver transplantation in the treatment of Budd-Chiari syndrome.

BACKGROUND
Budd-Chiari syndrome (BCS) is a rare disorder which can lead to liver failure. It arises from an occlusion of small vessels and/or thrombosis of the major hepatic veins. Polycythemia vera is believed to be one of the most common factors causing BCS. In cases not responding to other measures, liver transplantation may be a successful method of radical treatment. The purpose of this study was to assess the value and results of liver transplantation in the treatment of Budd-Chiari syndrome.


MATERIAL/METHODS
This retrospective study included all the cases of LTx (liver transplantation) performed because of BCS in the Department of General, Transplant and Liver Surgery, Medical University of Warsaw and it covered the period from 2000 to 2009. Twenty-four patients (8 men, 16 women) with the average age of 33 ± 11 presented with BCS, 6 patients (25%) underwent previous surgical therapy, 2 patients were treated by mesocaval "H" anastomosis (portosystemic shunts) and 4 patients received TIPSS (transjugular intrahepatic portosystemic shunt) prior to LTx. Polycythemia vera was an underlying risk factor in 11 cases. Two patients needed early retransplantation (re-LTx). In immunosuppressive therapy, schema with glucocorticosteroids and FK-506 predominated.


RESULTS
Four cases of bleeding to peritoneal cavity requiring surgical intervention were observed; 3 patients died in the early post-transplant period due to multiorgan failure and 1 late death occurred because of internal causes. Actual survival rate was 83% at 5 years.


CONCLUSIONS
In the late stage, Budd-Chiari syndrome should be considered as an indication for liver transplantation, especially when other measures such as porto-systemic shunts are scarce and ineffective.