Budd-Chiari syndrome. Treatment options and the value of liver transplantation.

The Budd-Chiari syndrome encompasses a group of conditions that cause partial or complete obstruction of the hepatic venous outflow tract. This leads to hepatocyte necrosis which can manifest as a devastating fulminant illness, or a more indolent condition that eventually presents with features of portal hypertension. Doppler ultrasonography of the hepatic veins and inferior vena cava has become the initial diagnostic test of choice, but most patients require venography and liver biopsy prior to definitive therapy. Multiple therapeutic modalities have been used for the Budd-Chiari syndrome, including medical, radiological, and surgical approaches. The role, indications, and outcome of liver transplantation for individuals with the Budd-Chiari syndrome are discussed.