Background/aims: The authors present the methods and results of treatment of patients with Budd-Chiari syndrome.
Methodology: From a group of 15 patients with Budd-Chiari syndrome, treated in our department from January 1996 to September 2001, nine patients (6 females and 3 males, aged from 23 to 45 years) were qualified for surgical or radiological treatment. In 3 of them the hepatic vein thrombosis resulted from untreated polycythemia. In the remaining 6 cases the etiology of thrombosis remained unknown. In four patients with dominating portal hypertension symptoms, a meso-caval H-type graft was created. Four patients with severe liver insufficiency were qualified for orthotopic liver transplantation; all of them were transplanted. In 1 patient a balloon dilatation of the right and middle hepatic veins orifices was performed during the diagnostic cavography. The follow-up period ranged from 4 months to 5 years.
Results: In 2 patients with Budd-Chiari syndrome of unknown etiology, the "H"-shunt remained patent, and the patients were free of ascites and splenomegaly. During the period of follow-up (more than 5 years) they remained free of symptoms and demonstrated acceptable liver function parameters. Doppler sonography revealed the partial recanalization of the venous system. In 2 polycythemic patients, the shunt thrombosed, which was the indication for orthotopic liver transplantation in one of them. The transplantation was performed, but was unsuccessful because of hepatic graft artery thrombosis. The retransplantation in this case was also unsuccessful. The remaining 3 patients transplanted for cryptogenic Budd-Chiari syndrome are alive and well, 4, 5 and 11 months after orthotopic liver transplantation. In the patient subjected to interventional radiological hepatic veins dilation, the vessels remain patent 26 months post-procedure. The patient is free from ascites.
Conclusions: Shunt surgery and liver transplantation are the valuable methods of treatment in selected cases of Budd-Chiari syndrome. In patients with polycythemia, however, thrombotic complications may occur in spite of anticoagulant and chemotherapeutic treatment. Balloon dilatation may appear an alternative to surgical treatment, but it seems that its indications should be limited to a highly selected group of patients.