https://pubmed.ncbi.nlm.nih.gov/33040172/ Metabolic liver disease

Pediatr Surg Int. 2020 Oct 10.
doi: 10.1007/s00383-020-04756-3. Online ahead of print.

Successful living donor liver transplantation plus domino-auxiliary partial orthotopic liver transplantation for pediatric patients with metabolic disorders

Chong Dong 1 2, Zhuolun Song 1 2, Xingchu Meng 1 2, Chao Sun 1 2, Kai Wang 1 2, Yang Yang 1 2, Hong Qin 1 2, Chao Han 1 2, Fubo Zhang 1 2, Weiping Zheng 1 2, Wei Gao 3

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.