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20 May 2003

Budd-Chiari syndrome – diagnosis and treatment

P. Małkowski, J. Pawlak, B. Michałowicz, R. Pacho, E. Leowska, T. Wróblewski, I. Gorzelak, M. Andrzejewska, J. Szczerbań, B. Górnicka, B. Wróblewska

Med Sci Monit 2003; 9(2): 41-47 :: ID: 15317


The aim of the paper was to present the authors ’own experience in the field of diagnostics and treatment of Budd-Chiari syndrome.It is a retrospective analysis of 37 cases of Budd-Chiari syndrome treated in the Department since1980.Qualification for treatment was preceded with imaging diagnostics of the lifer and hepatic blood vessels,assessment of hepatic function and hematologic condition of the patients.Ten patients with very advanced disease in the initial period of the analysis were treated symptomatically.In five patients,venous shunts were formed to compensate for the inefficient portal system,three were qualified for intrahepatic portosystemic shunts,six for liver transplantations, one underwent recanalization of the impatent hepatic vein with radiological technique.Twelve patients with incomplete impatency of hepatic veins were treated conservatively.In 7 cases,fibrinolytic treatment for secondary portal thrombosis was instituted.The patients ’age and sex,etiology of the syndrome,clinical course,results of imaging studies and treatment were analyzed.The follow-up time ranged from 2 months to 8 years (mean:34 months). Women with hematologic disorders predominated among the patients.In all the cases,the characteristic symptoms included pain, ascites and hepatosplenomegaly, with additional portal hypertension and hepatic failure at the later stages of the disease. Ultrasonography is an essential diagnostic method used in Budd-Chiari syndrome.Fifteen of the observed patients died,including two cases of postoperative mortality. Budd-Chiari syndrome is a rare pathology of various etiology.The first symptoms include pain,ascites and hepatosplenomegaly. Ultrasonography is an essential diagnostic method.Formation of venous shunts and liver transplantation are effective methods of treatment in selected cases.Polycythemia increases the risk of postoperative complications.Early-onset secondary portal thrombosis provides indication for fibrinolytic treatment

Keywords: Budd-Chiari syndrome, portal thrombosis, thrombolytic treatment

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Medical Science Monitor eISSN: 1643-3750
Medical Science Monitor eISSN: 1643-3750