This study evaluates the feasibility and safety of combining living donor liver transplantation (LDLT) with domino liver transplantation (DLT) for maple syrup urine disease (MSUD). MSUD causes neurological impairment due to branched-chain amino acid accumulation (BCAA). Liver transplantation restores metabolic homeostasis via branched-chain ketoacid dehydrogenase (BCKDH) present in the donor graft. MSUD livers can be reused as domino liver grafts (DLG) since enzyme deficiency in DLG is supplied by production in other recipient's tissues. From October 2012 to September 2025 live donors (LD), MSUD patients and DLG recipients were enrolled for DLT and followed for at least 1 year after transplantation. Plasma BCAA levels in MSUD patients at 3 time points (pretransplantation, early [1-90 d], and late [>90-365 d] posttransplantation) were compared with age-matched pediatric controls without metabolic disorders. Surgical and metabolic outcomes were monitored up to 1-year post-transplantation. Among 32 LDLT for MSUD, 27 (84.4%) grafts were reused. Patient/graft survival was 93.8% for MSUD recipients and 96.3%/85.2% for DLG recipients, respectively. Late hepatic artery thrombosis occurred in 3.1% of MSUD patients and 7.4% of DLG recipients. Primary factors for DLG disposal were double arteries and younger MSUD patients, related to small arterial caliber. No increased risk related to DLG donation was demonstrated. Combining LDLT and DLT expands the donor pool for pediatric patients while ensuring safety in MSUD treatment, with microsurgical arterial reconstruction supporting DLG reuse. Higher graft-to-recipient weight ratio (GRWR) was associated with better early metabolic control, but this association was not significant after adjustment for age, suggesting that the apparent contribution of graft size to early metabolic adaptation may be age-related.
Robotic living donor hepatectomy (R-LDH) aims to improve the safety and experience of healthy donors by combining three-dimensional visualization, wristed instrumentation, precision, and stable ergonomics with the benefits of minimal access. This review article summarizes current evidence and offers a pragmatic approach for centers to transition from open to routine robotic donation. Available evidence from retrospective feasibility series, matched cohorts, multicenter analyses, and registry data suggests that R-LDH may reduce donor morbidity compared to open and some evidence of fewer recipient biliary complications, although current evidence remains heterogeneous and largely non-randomized. Available comparative data, including single-center and international registry analyses, suggest lower morbidity with the robotic approach than with laparoscopy, although these comparisons remain limited and would benefit from confirmation in prospective multicenter studies. Learning-curve studies and prospective series suggest that with standardized technique, simulation, and early proctorship, proficiency appears achievable within a limited number of cases, with reduced complications over time. A center-readiness structure focused on “People” (trained team), “Process” (protocolized pathways with explicit conversion criteria), “Platform” (reliable robot access, ICG, ERAS), and “Proof” (prospective audit and external validation) supports phased adoption from standard to complex anatomy. Persistent barriers include limited robotic platform access, scheduling constraints, heterogeneity in reporting, and incomplete cost data, though shorter hospital stay, less pain, and faster return to normal daily activities may partially or potentially offset procedural costs in high-volume programs. In appropriately prepared centers, routine consideration of R-LDH for eligible donors may represent a reasonable donor-centered strategy. Concrete steps are outlined that surgeons and hospital leaders can undertake to implement R-LDH safely, monitor outcomes, and improve equitable access.
