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Domino Liver Transplant in Maple Syrup Urine Disease

Technical Details of Cases in Which the First Surgery Involved a Living Donor

Roda, Karina M. O., MD1; Vincenzi, Rodrigo, MD, PhD1; Fonseca, Eduardo A., MD, PhD1; Benavides, Marcel, MD1; Turine, Plínio, MD1; Afonso, Rogerio C., MD1; Tonon, Tassia, MD2,3; Schwartz, Ida, MD, PhD2,4,5; Miura, Irene K., MD, PhD1; Pugliese, Renata, MD, PhD1; Porta, Gilda, MD, PhD1; Chapchap, Paulo, MD, PhD1; Seda Neto, Joao, MD, PhD1

doi: 10.1097/TP.0000000000002300
Original Clinical Science—Liver

Background. Data describing the technical aspects of living donor (LD) domino liver transplantation (DLT) in maple syrup urine disease (MSUD) are limited. The largest published series includes only 3 cases. One great challenge of this procedure is to ensure adequate vascular stumps for the LD, the MSUD patient, and the recipient of the domino graft. Here, we describe our experience in 11 cases of LD-DLT in MSUD, highlighting the technical aspects of LD-DLT.

Methods. From September 2012 to September 2017, 11 patients with MSUD underwent LD liver transplantation at our institution, and MSUD livers were used as domino grafts in 11 children.

Results. (1) MSUD patients: 10 patients received a left lateral segment. The donor’s left hepatic vein (HV) was anastomosed to the confluence of the recipient’s 3 HVs. No vascular grafts (VG) were required for portal vein (PV) anastomosis. Single arterial anastomosis was performed with microsurgery in 10 of 11 patients. (2) MSUD graft recipients: In 8 cases, HV reconstruction was performed between the graft’s HV confluence and the recipient’s HV confluence, and in 3 cases, a vena cava triangulation was necessary; 6 MSUD grafts required HV venoplasty. No VG were needed for HV reconstruction. VG were used for PV reconstruction in 3 cases due to sclerotic PV. In 2 cases, double arterial anastomoses were performed in the MSUD liver. All patients remain alive and well.

Conclusions. Living donor liver transplantation followed by DLT for MSUD is a complex procedure and demands technical refinement. Special attention must be paid to vascular reconstruction.

1Department of Hepatology and Liver Transplantation, Hospital Sirio-Libanes, Sao Paulo, SP, Brazil.

2BRAIN Laboratory (Basic Research and Advanced Investigations in Neurosciences), Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil.

3Post Graduation Program in Medicine, Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.

4Department of Genetics, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, Brazil.

5Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.

Received 17 December 2017. Revision received 25 April 2018.

Accepted 15 May 2018.

The authors declare no funding or conflicts of interest.

K.M.O.R., RV., J.S.-N. participated in the concept/design. K.M.O.R., R.V., J.S.-N. participated in the drafting of the article. J.S.-N., R.V., E.A.F., P.C. participated in the critical revision of the article. R.P., I.K.M., M.R.B., P.T., R.C.A., I.S., T.T., G.P. participated in the data analysis/data collection. J.S.-N. gave the final approval.

Correspondence: Joao Seda Neto, MD, PhD, Hospital Sirio-Libanes/Hospital A. C. Camargo, Rua Barata Ribeiro, 414, cj 65, Bela Vista, 01308-000, Sao Paulo, Brazil. (

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