Diagnosis, treatment and evolution of the Budd-Chiari syndrome: a single center experience

SOLARI J; BANDI JC; GALDAME O.; SEBASTIAN MARCIANO; MULLEN E; GARCIA MR; NUCIFORA E; DE SANTIBAÑES E; ADRIAN GADANO

ACTA GASTROENTEROLOGICA LATINOAMERICANA.

SOCIEDAD ARGENTINA DE GASTROENTEROLOGÍA

Año: 2010 p. 225 - 235

0300-9033

INTRODUCTION:The Budd-Chiari syndrome is a low-prevalence disease due to an hepatic outflow obstruction. It is associated with procoagulant status and liver transplantation is one of the therapeutic tools for the treatment.OBJECTIVE:To evaluate the etiology, presenting form, treatment and evolution of patients with Budd-Chiari syndrome.PATIENTS AND METHOD:Ten consecutive adult patients with Budd-Chiari syndrome evaluated from January 1998 to June 2009 were prospectively included. The median follow up was 32.4 months (4-108 months).RESULTS:The mean age of patients was 34 +/- 12 years old. Presentation was acute in 1 patient, chronic in 2 and subacute in 7. The mean time from consultation to diagnosis was 4 +/- 2 days. Clinical manifestations were splenomegaly in 8 patients, malnutrition in 7, ascites in 6 and encephalopathy in 4. Diagnosis was confirmed by angiography in all cases. Initial prothrombin concentration was < 30% in 3 patients, 31% to 50% in 5, and > 50% in 2; hematocrit was > 45% in 5 patients and platelet count was > 400.000/mm3 in 6. MELD distribution at diagnosis was < or = 13 points in 4 patients, between 14 and 16 points in 5 and > or = 17 points in 1. Policytemia vera was detected in 7 patients, essential thrombocythemia in 1 and positive lupus inhibitor in 4. Nine patients were anticoagulated after diagnosis. Angioplasthy was required in 1 patient and 6 were treated with a transjugular intrahepatic portosystemic shunt. Death occurred in 1 patient due to gastrointestinal bleeding. Two patients were transplanted.CONCLUSION:In our experience all patients with Budd-Chiari syndrome have a procoagulant status. The transjugular intrahepatic portosystemic shunt is effective in treating this syndrome and liver transplantation should be reserved for patients who are refractory to other therapeutics.