Aim/ Background:

Domino liver transplantation (DomLT) using allografts with metabolic disorders enhances organ utilization but is not well described in children^[1][2]. 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.

Methods

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.

Results

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:

Conclusion

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.