Application of ex vivo liver resection and autotransplantation in treating Budd-Chiari syndrome secondary to end-stage hepatic alveolar echinococcosis: A case series

BACKGROUND: Secondary Budd-Chiari syndrome (BCS) occurs due to a blockage in the liver caused by invasion or compression by a large lesion. Conventional treatments for BCS do not solve practical problems, wherease liver transplantation has been only applied as a last-resort therapy and as the only opportunity for a radical cure. We explored the feasibility of applying ex vivo liver resection and autotransplantation (ELRA) for the new indications of treating patients with end-stage hepatic alveolar echinococcosis (HAE). Our center has firstly proposed the idea and successfully treated the 49 patients with HAE. This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE. METHODS: According to the degree of lesion invasion and surgical options, 11 patients were divided into 4 types. These 11 patients had large lesions that invaded the second and third hilum of the caudate lobe and involved the confluence of the hepatic vein and the inferior vena cava, suprahepatic vena cava, or at least 2 hepatic veins and led to secondary BCS. The aim of the present work was to report 11 patients with life-threatening diseases who underwent ELRA (ex vivo liver resection and autotransplantation) for secondary BCS, to propose a classification system for secondary BCS, and to suggest that secondary BCS is an indication for ELRA. RESULTS: Eleven patients successfully underwent ELRA without intraoperative mortality. The median autograft weight was 690 g (440–950 g); operative time was 12.5 hours (9.4–16.5 hours); Postoperative hospital stay was 15 days (7–21 days). Clavien-Dindo grade IIIa or higher postoperative complications occurred in only 5 patients. CONCLUSIONS: This article for the first time reports the application of ELRA in treating patients with BCS secondary to HAE, not only provides new ideas for alternative treatments of secondary BCS, but also provides a classification system for secondary BCS. This article describes the technical process of outflow tract reconstruction and the experience for expanding the indications for ELRA. Our study demonstrated that ELRA is well feasible for treatment of BCS secondary to advanced HAE.