Budd-Chiari syndrome: a rare manifestation of hereditary thrombophilia

Budd-Chiari syndrome is a rare manifestation of hereditary or acquired thro mbophilia. We saw a case of Budd-Chiari syndrome in a 30-year-old woman lea ding to initial diagnostic difficulties. She underwent surgical side-to-sid e shunt and 9 weeks later an almost normal liver could be demonstrated on c omputerized tomography. Budd-Chiari syndrome should be considered if the Ch iari triad with abdominal pain, hepatomegaly and ascites occurs in a patien t. If necessary, invasive diagnostic procedures (e.g. angiography) must be performed. Therapeutic options are anticoagulative therapy and porto-system ic shunt, either as a TIPS or a surgical shunt. If severe liver failure occ urs or Liver cirrhosis is present, orthotopic liver transplantation is an a dditional option which also cures hereditary thrombophilia.