Domino Liver Transplant in Maple Syrup Urine Disease: Technical Details of Cases in which the First Surgery Involved a Living Donor

Background Data describing the technical aspects of living donor domino liver transplantation (LD-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 living donor, 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 LDLT at our institution, and MSUD livers were used as domino grafts in 11 children. Results:1- MSUD patients Ten patients received a left lateral segment. The donor's left hepatic vein was anastomosed to the confluence of the recipient's 3 hepatic veins (HV). No venous grafts (VG) were required for portal vein (PV) anastomosis. Single arterial anastomosis was performed with microsurgery in 10 out 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. Conclusion LDLT followed by DLT for MSUD is a complex procedure and demands technical refinement. Special attention must be paid to vascular reconstruction. Corresponding author: 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. Phone number: 55-11-32310800 Fax number: 55-11-32310900. E-mail: joaoseda@gmail.com Authorship Statements Concept/design: Roda KMO, Vincenzi R, Seda-Neto J Drafting of the article: Roda KMO, Vincenzi R, Seda-Neto J Critical revision of the article: Seda-Neto J, Vincenzi R, Fonseca EA, Chapchap P Data analysis/Data collection: Pugliese R, Miura IK, Benavides MR, Turine P, Afonso RC Schwartz I, Tonon T, Porta G Final approval: Seda-Neto J Disclosure No conflicts of interest exist. Funding No financial support was received. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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