Long-term Outcome of Budd-Chiari Syndrome: A Single Center Experience.

BACKGROUND

Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction(HVOO). BCS is an uncommon, life-threatening liver disorder. This studydescribes the clinical and etiological characteristics in addition to the longtermoutcome of BCS in a single referral center in Tehran, Iran.

METHODS

We reviewed long-term outcome of patients who were diagnosed with BCSbetween 1989 and 2012 at Shariati Hospital, a tertiary hospital affiliated withTehran University of Medical Sciences, Tehran, Iran. The diagnosis was confirmedby at least two imaging techniques. A comprehensive analysis of theclinical and paraclinical manifestations, etiology and long-term outcome of thedisease was conducted.

RESULTS

Seventy one patients (43 female) with a diagnosis of Budd-Chiari syndromewere identified during the 22 year period of study. The age were ranged from17 to 64 years (median: 29 years). We excluded 16 patients because of incompleteinformation or follow up. The remaining 55 cases were the subjects ofthis study. Underlying etiologies consisted of congenital thrombophilia factorsin 50% (28 cases) which was defined as protein C deficiency (12 cases),protein S deficiency (3 cases), antithrombin deficiency (3 cases) and factor VLeiden mutation (10 cases). Etiology was unknown in 18% (10 cases). Acquiredcauses of thrombophilia were observed in 25% (14 cases) that consistedof 9 cases of myeloproliferative disease and 5 cases of autoimmune diseases.In 3 cases pregnancy was the only etiology. The main clinical presentationswere abdominal pain in 33 (60%), abdominal distention in 21 (38.2%), andjaundice in 10 (18%) cases. The main clinical signs were ascites (76.4%), splenomegaly(34%), hepatomegaly (25.5%) and deep vein thrombosis (1.8%). All55 patients were treated with anticoagulants (heparin followed by warfarin)and supportive care. Two cases underwent mesocaval shunt surgery, 2 patientsrequired transjugular portosystemic shunt (TIPS) and 5 were referred for livertransplantation. A total of 17 (30%) patients died during 22 years of follow up.

CONCLUSION

BCS, although uncommon in Iran, is a challenging liver disease with an importantburden. Medical therapy that includes anticoagulation seems to be effectivein most cases although the prognosis is guarded. In long-term followup, 40% of cases will need liver transplant or die from end stage liver disease.