EN
TÜRKÇE
Oral Presentation - 1
Domino liver transplantation with MSUD allografts for end stage liver disease: Technique and Outcomes
N Celik*, A Ganoza**, A Khanna**, G Bond**, K Soltys**, J Squires***, P McKiernan***, G Vockley****, R Sindhi**, G Mazariegos**
*Thomas E. Starzl Transplantation Institute, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
**Department of Surgery, Hillman Center for Pediatric Transplantation, UPMC Pittsburgh Children's Hospital, Pittsburgh, PA, USA
***Department of Pediatrics, Pediatric Hepatology, UPMC Pittsburgh Children's Hospital, Pittsburgh, PA, USA
****Department of Pediatrics, Center for Rare Disease Therapy, UPMC Pittsburgh Children's Hospital, Pittsburgh, PA, USA
Aim:Domino liver transplantation (DomLT) using allografts with metabolic disorders enhances organ utilization but it is not well described in children. Since the documentation of short and long-term course of these patients are critical to decision making about the safety of this procedure, we reviewed the outcomes of DomLT at our pediatric and adult center.
Methods:DomLT patients were analyzed retrospectively for patient and donor characteristics, postoperative complications and patient and graft survival with their MSUD (Maple Syrup Urine Disease).
Results:Between 2006 and 2019, 21 patients underwent DomLT from MSUD patients.The indications were progressive familial intrahepatic cholestasis (PFIC), cystic fibrosis, congenital hepatic fibrosis (n=2), neonatal hepatitis, embryonal sarcoma, hepatocellular carcinoma, primary biliary cirrhosis, primary sclerosing cholangitis (PSC, n=4), alpha-1 antitrypsin deficiency (n=2), Caroli disease, and chronic rejection after LT for PSC and PFIC. Four children with alternate metabolic diseases also received DomLT including propionic acidemia, Crigler-Najjar syndrome type-1 (n=2) and carbamoyl phosphate synthetase deficiency. All patients and grafts survived at mean follow-up of 5.5 years (range 1.4-12.5 years). Intraoperative HAT occurred in propionic acidemia patient and needed reconstruction and, one patient had PVT 3 years after transplantation and meso-Rex bypass was performed. Small for size syndrome occurred in reduced left lobe DomLT recipient and was managed successfully. The comparison between DomLT and MSUD recipients’ peak ALT results showed lower levels in DomLT group (p-value<0.05).
Conclusions:Patient and graft survival in DomLT from MSUD donors including recipients with selected metabolic diseases has been excellent at short and long-term follow-up. Metabolic function has been normal in all recipients on normal unrestricted protein intake. Ischemia preservation injury based on peak ALT has been significantly decreased in DomLT recipients. Domino transplantation from pediatric and adult recipients with selected metabolic diseases should be increasingly considered.