Long-term outcome of Budd-Chiari syndrome: a single center experience

ABSTRACT

Budd-Chiari syndrome [BCS] is defined as hepatic venous outflow obstruction [HVOO]. BCS is an uncommon, life-threatening liver disorder. This study describes the clinical and etiological characteristics in addition to the long-term outcome of BCS in a single referral center in Tehran, Iran. We reviewed long-term outcome of patients who were diagnosed with BCS between 1989 and 2012 at Shariati Hospital, a tertiary hospital affiliated with Tehran University of Medical Sciences, Tehran, Iran. The diagnosis was confirmed by at least two imaging techniques. A comprehensive analysis of the clinical and paraclinical manifestations, etiology and long-term outcome of the disease was conducted. Seventy one patients [43 female] with a diagnosis of Budd-Chiari syndrome were identified during the 22 year period of study. The age were ranged from 17 to 64 years [median 29 years]. We excluded 16 patients because of incomplete information or follow up. The remaining 55 cases were the subjects of this study. Underlying etiologies consisted of congenital thrombophilia factors in 50% [28 cases] which was defined as protein C deficiency [12 cases], protein S deficiency [3 cases], antithrombin deficiency [3 cases] and factor V Leiden mutation [10 cases]. Etiology was unknown in 18% [10 cases]. Acquired causes of thrombophilia were observed in 25% [14 cases] that consisted of 9 cases of myeloproliferative disease and 5 cases of autoimmune diseases. In 3 cases pregnancy was the only etiology. The main clinical presentations were abdominal pain in 33 [60%], abdominal distention in 21 [38.2%], and jaundice in 10 [18%] cases. The main clinical signs were ascites [76.4%], splenomegaly [34%], hepatomegaly [25.5%] and deep vein thrombosis [1.8%]. All 55 patients were treated with anticoagulants [heparin followed by warfarin] and supportive care. Two cases underwent mesocaval shunt surgery, 2 patients required transjugular portosystemic shunt [TIPS] and 5 were referred for liver transplantation. A total of 17 [30%] patients died during 22 years of follow up. BCS, although uncommon in Iran, is a challenging liver disease with an important burden. Medical therapy that includes anticoagulation seems to be effective in most cases although the prognosis is guarded. In long-term follow up, 40% of cases will need liver transplant or die from end stage liver disease