• Budd-Chiari syndrome;
  • cavoplasty;
  • hepatic vein reconstruction;
  • liver transplantation;
  • living donor

We have developed the surgical techniques of living donor liver transplantation (LDLT) for Budd-Chiari syndrome (BCS) and evaluated long-term outcomes including specific complications. BCS is characterized by hepatic outflow obstruction. Liver transplantation from living donors poses a unique challenge as liver replacement therapy does not replace the retrohepatic segment of inferior vena cava (IVC). We have performed 1105 LDLTs in 1055 patients from January 1990 to March 2005. Of these, nine patients (eight males and one female) underwent LDLT for BCS. Five out of nine patients underwent LDLT as a primary procedure and four patients had received other treatments before transplantation. Eight patients presented with chronic and one with fulminant liver failure. Predisposing factors were identified in three patients. IVC reconstruction without patch plasty was performed on four patients. Five patients needed cavoplasty using a replacement vein graft. Of the nine patients, seven are alive at a median follow-up of 58 months (range 1 month to 15.2 years) with two patients developing recurrent hepatic vein stenosis which were treated successfully with metallic stent placement. Two patients died: one from multiorgan failure and the other from pulmonary embolism secondary to disease recurrence.

LDLT for BCS is highly effective by using modified cavoplasty and provides good long-term survival which may be obtained by life-long anticoagulant treatment and nonsurgical interventions.