Living donor liver transplantation for Budd-Chiari syndrome with inferior vena cava obstruction and associated antiphospholipid antibody syndrome

BACKGROUND: Recently, the antiphospholipid antibody syndrome (APLS) has been recognized as the cause of the Budd-Chiari syndrome (BCS) in adults. However, APLS-induced BCS has been seen rarely in children. The surgical strategy for BCS depends on the patency of the inferior vena cava (IVC) and on the primary disease. METHODS: A 10-year-old boy with a diagnosis of BCS complicated with IVC obstruction caused by APLS received medical treatment and radiologic intervention for 2 years. In spite of these treatments, no relief of the symptoms could be achieved. Finally, the patient underwent living donor liver transplantation (LDLT), which required cavoplasty. He has had an uneventful course since the LDLT. CONCLUSIONS: IVC-obstructed BCS associated with APLS seems to be a good indication for LDLT with cavoplasty. Because liver transplantation (LT) itself is not a cure for APLS, the risk of thrombosis cannot be eliminated entirely by LT. Although immunosuppression may influence antiphospholipid antibody production, long-term observation and life-long anticoagulant treatment is needed even after LT. J Pediatr Surg 36:659-662.