Liver transplantation for Budd-Chiari syndrome.

BACKGROUND: Budd-Chiari syndrome (BCS) is a clinical condition characterized by hepatic venous outflow obstruction secondary to an underlying systemic predisposition to thrombosis.
METHODS: We reviewed our experience of 19 adult patients who underwent orthotopic liver transplantation for BCS from April 1988 to May 1999 to assess their long-term outcome and specific complications related to this procedure.
RESULTS: Of these patients, 13 presented with chronic and 6 with acute liver failure. At presentation predisposing factors included polycythemia rubra vera in five, an undefined myeloproliferative disorder in four, essential thrombocythemia in two, presence of lupus anticoagulant in one, antiphospholipid antibody positivity in one, post-gestational in one, oral contraceptive pill in one, and idiopathic in four. Five patients had undergone previous porto-systemic shunt. Of the 19 patients, 16 are alive at a median follow-up of 89 months (range 1-119) with 2 patients developing disease recurrence at 4 months and 7 years posttransplant, respectively. Four patients have been retransplanted: one for progressive graft dysfunction due to nodular regenerative hyperplasia secondary to azathioprine toxicity, two for hepatic artery thrombosis (one soon after and the other 47 months posttransplant), and one for recurrent BCS. Three patients have died: one from an intra-abdominal bleed secondary to acute hemorrhagic pancreatitis 8 years posttransplant, another from acute myeloid leukemia at 6 years posttransplant, and the third patient from graft failure secondary to severe rejection 1 month posttransplant.
CONCLUSION: Liver transplantation for BCS provides good long-term survival with acceptable morbidity. Long-term survival may be prejudiced by progression of the underlying hematological disorders.