Surgical Aspects of Liver Transplantation and Domino Liver Transplantation in Maple Syrup Urine Disease: Analysis of 15 Donor‐Recipient Pairs

Abstract

Liver transplantation (LT) has been shown to be a feasible treatment in patients with severe forms of maple syrup urine disease (MSUD). Because of a sufficient extrahepatic enzyme activity in non‐MSUD individuals, the organ of MSUD patients can be used as a 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 LTs of the MSUD grafts either transplanted at our institution or allocated to other transplant centers were analyzed. During the study period, 15 LTs in MSUD patients were performed (12 children, 3 adults; median age, 10.9 years; range, 0.3‐26.1 years). Biliary complications occurred in 20%, and 13.3% suffered from bleeding complications. No further surgical problems occurred. At present, all MSUD patients are alive with a well‐functioning liver graft and on an unrestricted diet. In total, 14 consecutive domino LTs were performed. No surgical complications requiring intervention occurred. One patient died because of HCC relapse, and all other patients are alive with good liver graft function. In 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 the limited donor pool.

Maple Syrup Urine Disease and Domino Liver Transplantation: When and How?

“…In this issue of Liver Transplantation , Herden et al describe the outcomes of 15 LTs in MSUD patients (12 children and 3 adults) from whom 14 grafts were used for domino LT in the Eurotransplant area. After 23 months of follow‐up, the patient survival rates were 100% for MSUD recipients (all on a free diet) and 93% for domino LT recipients (1 MSUD liver recipient died from tumor recurrence).…”

“…Although there are not yet data comparing LT outcomes in MSUD domino donors versus nondonors, the European data showed no differences in morbidity and survival rates in the FAP patients. (4) In this issue of Liver Transplantation, Herden et al (5) describe the outcomes of 15 LTs in MSUD patients (12 children and 3 adults) from whom 14 grafts were used for domino LT in the Eurotransplant area. After 23 months of follow-up, the patient survival rates were 100% for MSUD recipients (all on a free diet) and 93% for domino LT recipients (1 MSUD liver recipient died from tumor recurrence).…”

“…Despite the technically challenging implantation procedure, (5) 2 domino liver grafts of the Eurotransplant series underwent an ex situ split procedure, and the derived 4 partial grafts were successfully transplanted, reflecting excellent MSUD graft quality. Moreover, the good outcomes of domino liver grafts shipped to other centers of the Eurotransplant area support that intercenter domino organ sharing does not affect domino LT outcomes despite prolonging ischemia times.In the Eurotransplant network, (5) the presence of an intercenter allocation system facilitated allocation of Abbreviations: FAP, familial amyloidotic polyneuropathy; LT, liver transplantation; MSUD, maple syrup urine disease.…”

“…In the Eurotransplant network, (5) the presence of an intercenter allocation system facilitated allocation of Abbreviations: FAP, familial amyloidotic polyneuropathy; LT, liver transplantation; MSUD, maple syrup urine disease.…”

“…As for MSUD LTs, small-sized domino liver grafts will come also from children with other metabolic diseases, for which early LT is recommended. (7) As documented by Herden et al, (5) half of MSUD domino liver grafts did not follow the regular allocation system but were allocated on the basis of size matching, preferentially choosing patients belonging to disadvantaged weight (neonates or adolescents) and low Pediatric End-Stage Liver Disease score. Hence, with domino LT, the transplant community will have to review and update allocation rules for domino liver grafts in order to maximize the use of this resource.…”