Living donor domino liver transplantation using a maple syrup urine disease donor: A case series of three children – The first report from Japan

Abstract

As the priority of LD-Domino LT is the safety of the first recipient, limitations and technical difficulties in the second recipient often occur. The most technically challenging part of LD-Domino LT is the reconstruction of the vessels. For the reconstruction of HVs, the native HVs were exteriorized as far as possible using a CUSA because longer extensive HVs are essential for facilitating the reconstruction. At the back table, the HVs of the domino graft were sutured together, and the single cuff of the HVs was anastomosed to the IVC by joining the orifices. The HAs, the presence of insufficient length, and multiple vessels in the whole liver rendered the reconstruction more difficult. We determined the dividing sites of the vessels according to the preoperative 3D-CT findings obtained in two institutions. This is the first case series using grafts in DLT obtained from LDLT for patients with MSUD between two institutions. In conclusion, LD-Domino LT is a safe and feasible therapeutic option to expand the donor pool by technical refinement in the reconstruction of the second recipient. Further studies with a greater accumulation of patients and a longer follow-up will be necessary to establish LD-Domino LT using an MSUD donor.

Domino liver transplantation for select metabolic disorders: Expanding the living donor pool

“…We have previously reported a series with 100% patient and graft survival and no metabolic derangement following DLT using MSUD donors with short‐term follow‐up . The literature and this series report recipients with normal plasma amino acid profiles and no metabolic decompensations following transplantation, confirming that sufficient extrahepatic BCKDH activity enables unrestricted protein intake with no clinical signs of MSUD …”

“…A 20-year-old female with CN-1 who received 8 to 10 hours/day of phototherapy presented with a total pretransplant bilirubin range 17 a whole-organ domino allograft from an MSUD donor in August 2016. Her total bilirubin normalized in 12 days.…”

“…DLT with MSUD livers has been performed successfully in a hemophiliac with HCV/HIV related cirrhosis and for pyruvate carboxylase deficiency . The latter is the only reported case of successful DLT into a patient with monogenic disease and normal hepatic parenchyma.…”

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

“…Therefore, these organs are suitable without recipient restrictions and, especially in the case of a pediatric donor, as size‐matched organs for children. First case reports and first small series (up to 5 patients) of domino LT with MSUD liver grafts have shown a good outcome accompanied by an unremarkable or near‐normal BCAA metabolism in the recipients …”

Current status of pediatric transplantation in Japan

“…Domino transplants initially involved patients with familial amyloid polyneuropathy as secondary donors but have recently come to involve children with maple syrup urine disease (MSUD), an inborn error of metabolism (IEM), as primary recipients and as secondary donors who, in turn, allow their livers to be removed and transplanted in non-MSUD patients [22]. While MSUD patients are associated with a deficiency in branched-chain alpha keto acid dehydrogenase resulting in impaired catabolism of the branched-chain amino acids (BCCAs), their livers may be made available for use in non-MSUD patients, in that BCCAs become catabolized by other organs than the liver in these patients.…”