Introduction: Intestinal transplantation remains the treatment of choice for patients with irreversible intestinal failure (IIF) and severe complications of parenteral nutrition. We describe the first case of a living donor intestinal transplantation (LDIT) performed in Brazil. This strategy was used after the clinical condition of the patient deteriorated while on the waiting list for a deceased donor. Methods: Case report, highlighting the planning and execution of the technique of LDIT. Result: A 3-year-old patient with IIF due to intestinal atresia was referred for intestinal transplantation due to progressive loss of venous access sites, with worsening of the venous thrombosis during the waiting time on the list for a deceased donor. After 8 months on the list, a LDIT was performed: the donor was her mother, and a 160 cm length small bowel graft was harvested. Total ischemia time was 62 minutes, and the graft was transplanted using the recipient’s aorta and inferior vena cava for vascular anastomosis. On postoperative day (POD) 15, the patient presented a severe acute cellular rejection (ACR) resistant to corticosteroids and thymoglobulin, successfully treated with alemtuzumab. She was discharged home on POD 80, totally free of parenteral nutrition or any intravenous fluid/electrolyte supplementation. The patient remained stable with a totally functioning graft during all the follow-up period; 11 months after LDIT, she was diagnosed with a central nervous system lymphoma, and died after treatment failure, 17 months after LDIT. Conclusion: LDIT is a viable and safe strategy for the treatment of children with IIF, especially in the scenario of the shortage of pediatric organ donors. Possible immunological advantages using a graft obtained from a living donor are uncertain.

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.