Liver transplantation in children: Case

This case traces the arc of a paediatric liver transplant from listing through immunosuppression to the late complication that defines the Epstein-Barr-virus-seronegative recipient. The girl's pretransplant course, her graft type, and her serostatus together explain why she developed post-transplant lymphoproliferative disorder, and the case turns on recognising it early on surveillance and managing it by the disciplined reduction of immunosuppression. ^[11]

Pretransplant course and listing

This girl had biliary atresia, the single most common indication for paediatric liver transplantation, accounting for up to half of all cases. Despite a Kasai portoenterostomy in infancy, she developed growth failure, intractable pruritus, and recurrent cholangitis, the accepted indications for transplantation in a child with a failing Kasai. She was listed for deceased-donor transplantation and prioritised by the Paediatric End-stage Liver Disease score, calculated from her bilirubin, international normalised ratio, albumin, growth failure, and age at listing, with growth failure weighted because malnutrition reflects disease severity in children. ^[3]

Because the smallest infants have the highest waiting-list mortality as size-matched deceased donors are scarce, her father was evaluated as a living donor. He donated a left lateral segment graft, the graft type that fits an infant and shortens waiting time. The donor evaluation was conducted to the same safety standards as a deceased-donor pathway, and the donor hepatectomy carried a small but real morbidity that was disclosed to the family. ^[1]