Surgical aspects of liver transplantation and domino liver transplantation in maple syrup urine disease: analysis of 15 donor / recipient pairs.

Liver transplantation (LT) has been shown to be a feasible treatment in patients with severe forms of maple syrup urine disease (MSUD). Due to a sufficient extrahepatic enzyme activity in non-MSUD individuals, the organ of MSUD patients can be used as domino graft. We performed a retrospective data collection of all LTs for MSUD carried out at the University Medical Center Hamburg-Eppendorf (2016-2018). Moreover, data from all consecutive domino LT of the MSUD grafts either transplanted at our institution or allocated to other transplant centers were analyzed. Fifteen LTs in MSUD patients were performed during the study period (12 children, 3 adults; median age 10.9 (range 0.3 - 26.1) years. Biliary complications occurred in 20%, 13.3% suffered from bleeding complications; no further surgical problems occurred. At present, all MSUD patients are alive with well-functioning liver graft and on an unrestricted diet. Fourteen consecutive domino LT were performed. No surgical complications requiring intervention occurred. One patient died due to HCC relapse, all other patients are alive with good liver graft function. CONCLUSION: The use of MSUD livers as domino grafts is safe and allows application of LT in MSUD patients without net extraction of a liver graft from of the limited donor pool. This article is protected by copyright. All rights reserved.