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Presenter: Neslihan, Celik, Pittsburgh , United States
Authors: Neslihan Celik, F Vyas, GJ Bond, KA Soltys, R Sindhi, GV Mazariegos
Domino liver transplantation: Technique and outcome
Neslihan Celik1, F Vyas1, G J Bond1, K A Soltys1, R Sindhi1, G V Mazariegos1.
1Hillman Center for Pediatric Transplantation, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, United States
Domino liver transplantation (DomLT) using allografts with metabolic disorders enhances organ utilization but 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 a single center.
All patients receiving DomLT were analyzed retrospectively with minimum one year follow-up period for patient and donor characteristics, early and late postoperative complications and patient and graft survivals. We also reviewed the MSUD (Maple Syrup Urine Disease) donors of these patients who received deceased donor liver transplantation at the same time in terms of age, weight, cold ischemia time, postoperative leucine levels and peak ALT (Alanine Aminotransferase) levels during postoperative 48 hours.
Between 2007 and 2014, 10 patients underwent liver transplantation with domino live-donor allografts from MSUD patients. The indications were progressive familial intrahepatic cholestasis (PFIC, n=1), cystic fibrosis (n=1), congenital hepatic fibrosis (n=2), embryonal sarcoma (n=1), primary sclerosing cholangitis (PSC, n=2), alpha-1 antitrypsin deficiency (n=1), chronic rejection after liver transplantation for PSC (n=1) and PFIC (n=1). All patients and grafts survived. Median recipient age was 19.4 years (range 6.9-64.6 years). The median donor age was 16.6 years (range 4.8-32.1 years). There was no vascular complication at early postoperative period, one patient had portal vein thrombosis 3 year after transplantation and Meso-Rex shunt was performed. Small for size syndrome occurred in reduced left lobe DomLT recipient and managed succesfully. Biliary stricture occurred in 2 patients and solved by stenting.
The data and comparison between DomLT and MSUD recipients were listed in the Table below:
|Recipient Demographics and Functional Metrics||DomLT Recipient||MSUD Recipient||p-value|
|Mean age at transplantation (years)||22.4||15.9||0.28|
|Mean weight (kg)||56.1||55.1||0.93|
|Posttransplant mean leucine level (umol/dL)||12.3||18.1||0.000|
|Posttransplant mean cold ischemia time (minute)||302.4||349.0||0.26|
|Posttransplant peak ALT in 48 hours (IU/L)||288.1||984.8||0.007|
Patient and graft survival in DomLT has been excellent at current follow-up. Metabolic function has been normal in all recipients on normal protein intake. Ischemia preservation injury based on peak ALT has been significantly decreased in DomLT recipients. Decremental pattern in leucine levels and cold ischemis times in DomLT patients also give courage for the selection of this procedure. Domino transplant should be increased in pediatric and adult transplantation.
 Mazariegos GV, Morton DH, Sindhi R, Soltys K, Nayyar N, Bond G, Shellmer D, Shneider B, Vockley J, Strauss KA. Liver transplantation for classical maple syrup urine disease: long-term follow-up in 37 patients and comparative United Network for Organ Sharing experience. J Pediatr. 2012 Jan;160(1):116-121
 Schielke A, Conti F, Goumard C, Perdigao F, Calmus Y, Scatton O. Liver transplantation using grafts with rare metabolic disorders. Dig Liver Dis. 2015 Apr;47(4):261-270