Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatr
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ORIGINAL ARTICLE
Successful living donor liver transplantation plus domino‑auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders Chong Dong1,2 · Zhuolun Song1,2 · Xingchu Meng1,2 · Chao Sun1,2 · Kai Wang1,2 · Yang Yang1,2 · Hong Qin1,2 · Chao Han1,2 · Fubo Zhang1,2 · Weiping Zheng1,2 · Wei Gao1,2 Accepted: 29 September 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose To investigate the efficacy of living donor liver transplantation (LDLT) plus domino-auxiliary partial orthotopic liver transplantation (D-APOLT) in pediatric patients with metabolic disorders. Methods From May 2017 to October 2018, two patients with ornithine aminotransferase deficiency (OTCD) and one patient with type I Crigler-Najjar syndrome (CNS1) received LDLT, their livers were prepared as donors for D-APOLT. Two patients with CNS1 received domino liver grafts from OTCD patients; one OTCD patient received a domino liver graft from a CNS1 patient. Results The mean follow-up was 26.6 months. The liver function and ammonia remained in the normal range at the end of the follow-up in all recipients. One D-APOLT patient experienced portal vein thrombosis 2 days after transplantation and required reoperation, this patient presented an imbalance of portal blood perfusion between the native and the domino liver at 8 months after liver transplant. The imbalance was improved by interventional radiology treatment. Two LDLT patients experienced early mild acute rejection. Conclusions The non-cirrhotic livers from pediatric patients with metabolic liver disease can be used as domino donor grafts for selected pediatric patients with different metabolic liver disease. D-APOLT achieves ideal recipient outcomes and provides a strategy to expand donor source for children. Keywords Auxiliary partial orthotopic liver transplantation · Domino donor · Living donor · Metabolic liver disease · Pediatric liver transplantation Abbreviations ALT Alanine transaminase AST Aspartate transaminase APOLT Auxiliary partial orthotopic liver transplantation CNS1 Crigler-Najjar syndrome CT Computed tomography D-APOLT Domino-auxiliary partial orthotopic liver transplantation HV Hepatic vein * Wei Gao [email protected] 1
Organ Transplantation Center, Tianjin First Central Hospital, No.24 Fukang Road, Nankai District, 300192 Tianjin, China
Tianjin Key Laboratory for Organ Transplantation, Tianjin, China
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LDLT Living donor liver transplantation LLS Left lateral segment LT Liver transplantation MHV Middle hepatic vein MLD Metabolic liver diseases MRCP Magnetic resonance cholangiopancreatography NCMLD Non-cirrhotic metabolic liver diseases OTCD Ornithine aminotransferase deficiency PLT Partial liver transplantation PV Portal vein RL Right lobe TB Total bilirubin
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Introduction Metabolic liver diseases (MLD) are inherited disorders of metabolic system and are usually caused by single enzyme defects. Although MLD are generally considered as rare dis
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