Successful treatment of chronic Budd-Chiari syndrome with a transjugular intrahepatic portosystemic shunt

Budd-Chiari syndrome is characterized by obstruction of the hepatic venous outflow tract. Therapeutic options for chronic Budd-Chiari syndrome are lim ited. We report the case of a 28-year-old woman who presented with recurren ce of chronic Budd-Chiari syndrome with total obstruction of all major hepa tic veins. Due to worsening liver function over the course of 1 year, she h ad to be listed for liver transplantation. Because of therapy-refractory as cites, declining renal function and severe esophageal varices, a transjugul ar intrahepatic portosystemic shunt (TIPS) was placed, planned as a bridge to transplantation. Following TIPS, a marked recovery of liver function cou ld be observed, accompanied by disappearance of ascites, esophageal varices , and normalization of kidney function. Therefore, the patient could be rem oved from the waiting list for liver transplantation. This case demonstrate s for the first time that the use of TIPS in chronic Budd-Chiari syndrome m ay result in marked recovery of liver function.