BACKGROUND: Various surgical procedures for treating chronic Budd-Chiari syndrome have been established, but none are adequate because of the variation in underlying pathologic vascular changes. METHODS: This article presents a 32-year-old patient with a 5 cm long segment of obstruction of the retrohepatic inferior vena cava involving the main hepatic veins with severe portal hypertension. RESULTS: After five unsuccessful attempts at percutaneous transluminal angioplasty, simultaneous hepatic venoplasty was conducted with the saphenous vein patch and retrohepatic inferior venacavoplasty by the expanded polytetrafluoroethylene patch with a 3 cm long cuff interposition for suprahepatic reconstruction of the inferior vena cava. Hypothermic preserved liver perfusion after vascular isolation and femoroportoaxillary venovenous bypass with a centrifugal blood pump throughout the anhepatic stage ensured safe operation on the liver and maintained hemodynamics. Early postoperative anticoagulant is recommended. CONCLUSIONS: An uneventful postoperative course and a 10-month follow-up showing excellent condition indicated this one-stage simultaneous patch hepatic venoplasty and cavoplasty to be adequate for appropriate correction very of complex obstructive vascular lesions in Budd-Chiari syndrome.
BACKGROUND: Various surgical procedures for treating chronic Budd-Chiari syndrome have been established, but none are adequate because of the variation in underlying pathologic vascular changes. METHODS: This article presents a 32-year-old patient with a 5 cm long segment of obstruction of the retrohepatic inferior vena cava involving the main hepatic veins with severe portal hypertension. RESULTS: After five unsuccessful attempts at percutaneous transluminal angioplasty, simultaneous hepatic venoplasty was conducted with the saphenous vein patch and retrohepatic inferior venacavoplasty by the expanded polytetrafluoroethylene patch with a 3 cm long cuff interposition for suprahepatic reconstruction of the inferior vena cava. Hypothermic preserved liver perfusion after vascular isolation and femoroportoaxillary venovenous bypass with a centrifugal blood pump throughout the anhepatic stage ensured safe operation on the liver and maintained hemodynamics. Early postoperative anticoagulant is recommended. CONCLUSIONS: An uneventful postoperative course and a 10-month follow-up showing excellent condition indicated this one-stage simultaneous patch hepatic venoplasty and cavoplasty to be adequate for appropriate correction very of complex obstructive vascular lesions in Budd-Chiari syndrome.