Domino Liver Transplant in Maple Syrup Urine Disease: Technical Details of Cases in Which the First Surgery Involved a Living Donor
| Autor: | Karina M. O. Roda, Rogerio C. Afonso, Paulo Chapchap, Gilda Porta, Tássia Tonon, Plinio Turine, Eduardo A. Fonseca, Irene K. Miura, Ida Vanessa Doederlein Schwartz, Renata Pugliese, Joao Seda Neto, Rodrigo Vincenzi, Marcel Benavides |
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| Rok vydání: | 2019 |
| Předmět: |
Male
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Tissue and Organ Procurement medicine.medical_treatment Hepatic Veins Liver transplantation Living donor Domino Body Mass Index Hepatic Artery Maple Syrup Urine Disease Living Donors medicine Humans Child Transplantation Portal Vein business.industry Maple syrup urine disease Anastomosis Surgical Graft Survival Infant nutritional and metabolic diseases medicine.disease Tissue Donors Transplant Recipients Liver Transplantation Surgery Liver Child Preschool Female business |
| Zdroj: | Transplantation. 103:536-543 |
| ISSN: | 0041-1337 |
| DOI: | 10.1097/tp.0000000000002300 |
| Popis: | 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.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.(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.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. |
| Databáze: | OpenAIRE |
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