Liver transplant (txp) is the definitive branched chain keto-acid (BCKA) replacement therapy for patients with Maple Syrup Urine Disease (MSUD). Due to the extra-hepatic distribution of BCKAs (>70%) in non-MSUD patients, the affected explanted liver can be transplanted into non-MSUD recipients (domino liver transplant) without dietary restriction or risk of disorder-associated metabolic crises.

Herein, we describe our domino liver transplant (DLT) experience for MSUD including preoperative patient selection processes, multi-team operative strategy, and postoperative graft performance.

Between 2006 and 2014, 9 children with MSUD underwent brain-deceased liver txp and donated their livers to 9 recipients with ESLD of varying etiology. Patient follow-up post txp was > 6 months (range 6.3-102.8 months). All patients are alive with functioning grafts including one patient who received a left lateral segment. Although DLT recipients were transplanted at a significantly lower MELD/PELD score than their matched MSUD donors, there were no significant differences in mean age/weight at txp, waitlist time, cold-ischemia time, length of stay, or quantitative amino acid levels at discharge.

DLT was successfully performed utilizing surgical techniques to minimize operative and cold ischemia times, and to preserve the optimal vascular and biliary anatomy for donor hepatectomy and transplant. A well-synchronized team approach with communication at key decision points is also mandatory. In our experience, DLT recipients have excellent immediate graft function, and comparable long-term graft and patient survival compared to their MSUD donors. Increased consideration to domino transplantation from patients with select metabolic disease may increase transplantable grafts.

« Back to 2015 American Transplant Congress