Living donor liver transplantation for adult Budd Chiari syndrome - Resection without replacement of retrohepatic IVC: A case report.

INTRODUCTION: Suprahepatic caval resection and replacement of inferior vena cava (IVC) is standard procedure in deceased donor liver transplantation for patients with Budd-Chiari syndrome (BCS). However, replacement of IVC in living donor liver transplantation (LDLT) is difficult. We report a case of BCS successfully treated by LDLT without replacement of IVC.

PRESENTATION OF CASE: A 52-years-old female with a primary BCS due to IVC thrombosis. A vena cava (VC) stent placed after angioplasty without improvement of the hepatic, portal venous flow and liver functions, Transjugular intrahepatic portosystemic shunt was considered and the patient had a rapid deterioration and increased ascites. The patient was scheduled for living donor liver transplantation (LDLT). Her Child-Paugh and MELD scores were 11, 18, respectively at time of transplantation. Left lobe was obtained from her son. Preservation of the native suprarenal IVC was impossible due to massive fibrosis and thrombosed. The suprahepatic IVC was also fibrotic and unsuitable for anastomosis with hepatic vein. The retrohepatic IVC resected include suprahepatic IVC together with the liver. The supradiaphragmatic IVC was reached and encircled through opening the diaphragm around the IVC and a vascular clamp applied on the right atrium with subsequent anastomosis with hepatic vein of the graft. The hemodynamic stability of the patient was maintained throughout the operation without IVC replacement due to developed collateral vessels.

CONCLUSION: Patients with Budd-Chiari syndrome with obstructive IVC are successfully treated with living donor liver transplantation without replacement of IVC.