Paeds · gastroenterology-hepatology-and-nutrition
Liver transplantation in children
Also known as Paediatric liver transplant · Pediatric liver transplantation · Living donor liver transplantation in children · PELD score and transplant listing · Tacrolimus immunosuppression in children
Fellowship guide to liver transplantation in children: the indications with biliary atresia dominant, graft types including living-donor and split grafts, the Paediatric End-stage Liver Disease score for deceased-donor allocation, tacrolimus-anchored maintenance immunosuppression, acute cellular rejection diagnosed on biopsy and treated with high-dose corticosteroid, and the long-term complications of hepatic artery thrombosis, post-transplant lymphoproliferative disorder and non-adherence.
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Target exams
Red flags
- A rising transaminase in the first weeks after transplant demands immediate Doppler ultrasound to exclude hepatic artery thrombosis
- Persistent fever with a rising Epstein-Barr viral load in a seronegative recipient signals post-transplant lymphoproliferative disorder
- An immediately failing graft that produces no bile in the first hours is primary non-function and an indication for urgent retransplantation
- Non-adherence in adolescence is a leading preventable cause of graft loss
- Fever with raised liver enzymes in the first month is acute cellular rejection until proven otherwise
- Portopulmonary hypertension is a contraindication to transplantation and must be excluded before listing
Life stages
Care settings
Clinical exam formats
Board mappings
- Identifies biliary atresia as the most common indication for paediatric liver transplantation
- Recognises the indications for transplant referral including synthetic failure, intractable pruritus, growth failure and recurrent cholangitis
- Describes the basic graft types including living-donor and split grafts
- Calculates and interprets the Paediatric End-stage Liver Disease score and its role in deceased-donor allocation
- Constructs a maintenance immunosuppression regimen anchored on tacrolimus with therapeutic drug monitoring
- Distinguishes acute cellular rejection from hepatic artery thrombosis and post-transplant lymphoproliferative disorder
- Indications for and contraindications to liver transplantation in children
- The PELD score and its components
- Acute cellular rejection: diagnosis by biopsy and treatment with high-dose corticosteroid
- Takes a focused history and examines a child after liver transplantation
- Communicates the immunosuppression regimen, its side effects and adherence to a family
- Counsels on long-term outlook and the transition to adult care
- Level 1: Recognition of the indications for liver transplant referral in a child with chronic liver disease
- Level 2: Interpretation of post-transplant complications including rejection, vascular thrombosis and infection
- Level 3: Coordination of multidisciplinary post-transplant care and transition planning
- Components and use of the Paediatric End-stage Liver Disease score
- Maintenance immunosuppression with tacrolimus and therapeutic drug monitoring
- Post-transplant lymphoproliferative disorder and its relationship to Epstein-Barr virus
- Assessment of a child with a rising transaminase after liver transplant
- Interpretation of post-transplant investigations including Doppler ultrasound and biopsy
- Structured explanation of the immunosuppression regimen and long-term surveillance
- NASPGHAN approach to paediatric liver transplant indications and pretransplant assessment
- PELD score for deceased-donor allocation and standardised exception cases
- Tacrolimus-based immunosuppression and the management of acute cellular rejection
- Recognition of the indications for and contraindications to paediatric liver transplantation
- Post-transplant complication recognition including hepatic artery thrombosis and rejection
- Adherence and transition planning in the adolescent transplant recipient
- Canadian approach to paediatric liver transplant allocation and living-donor programmes
- Multidisciplinary long-term follow-up after paediatric liver transplantation
- Structured transition from paediatric to adult transplant services
Overview & Definition
A child who reaches the point of liver transplantation has travelled one of three roads: chronic decompensated liver disease, most often biliary atresia that has exhausted the Kasai operation; an acute catastrophe such as fulminant hepatic failure; or a metabolic disease in which the native liver, though structurally intact, is biochemically dangerous. The task of the paediatric team is to recognise the destination early, to prioritise the child correctly on the waiting list, and to support a graft and a growing child through decades of immunosuppression. Few areas of paediatrics demand a longer view, and few reward early, structured referral more handsomely. [1]
Liver transplantation is the replacement of a failing native liver with a vascularised graft that restores synthetic, metabolic and excretory function. In children this is rarely a straightforward whole-organ deceased-donor transplant, because the small size of the recipient and the scarcity of size-matched paediatric deceased donors mean that most children receive a segmental graft, whether a reduced-size, split, or living-donor graft. The single most common indication is biliary atresia, which accounts for up to half of all paediatric transplants, followed by metabolic and genetic disease and then acute liver failure. [10]
The discipline rests on three pillars that the candidate must hold together. The first is allocation: the Paediatric End-stage Liver Disease score ranks children on the deceased-donor waiting list and is the numerical backbone of who is transplanted and when. The second is immunosuppression: a tacrolimus-anchored regimen walks the narrow line between rejection and the infections, malignancy and organ toxicity of over-treatment. The third is the long arc of follow-up, in which hepatic artery thrombosis, post-transplant lymphoproliferative disorder, and adolescent non-adherence decide whether a technically perfect transplant succeeds over a lifetime. [8]
References12ShowHide
- [1]Smith SK, Miloh T Pediatric Liver Transplantation. Clin Liver Dis, 2022.PMID 35868688
- [2]Pham YH, Miloh T Liver Transplantation in Children. Clin Liver Dis, 2018.PMID 30266163
- [3]McDiarmid SV, Merion RM, Dykstra DM, et al Use of a pediatric end-stage liver disease score for deceased donor allocation: the United States experience. Indian J Pediatr, 2007.PMID 17476086
- [4]Chang CH, Bryce CL, Shneider BL, et al Accuracy of the Pediatric End-stage Liver Disease Score in Estimating Pretransplant Mortality Among Pediatric Liver Transplant Candidates. JAMA Pediatr, 2018.PMID 30242345
- [5]Swenson SM, Roberts JP, Rhee S, et al Impact of the Pediatric End-Stage Liver Disease (PELD) growth failure thresholds on mortality among pediatric liver transplant candidates. Am J Transplant, 2019.PMID 31370108
- [6]Reding R Tacrolimus in pediatric liver transplantation. Pediatr Transplant, 2002.PMID 12453195
- [7]Trezeguet Renatti G, Riva N, Minetto J, et al Feasibility of steroid-free tacrolimus-basiliximab immunosuppression in pediatric liver transplantation and predictors for steroid requirement. Liver Transpl, 2024.PMID 37439661
- [8]Antala S, DiNorcia J, Bucuvalas J Balancing immunosuppression in pediatric liver transplantation: Playing the long game. Pediatr Transplant, 2023.PMID 37439035
- [9]Wozniak LJ, Venick RS, Naini BV, et al Operational tolerance is not always permanent: A 10-year prospective study in pediatric liver transplantation recipients. Liver Transpl, 2022.PMID 35395132
- [10]Anouti A, Patel MS, VanWagner LB, et al Biliary atresia and liver transplantation in the United States: A contemporary analysis. Liver Int, 2023.PMID 37548078
- [11]Okamoto T, Okajima H, Uebayashi EY, et al Management of Epstein-Barr Virus Infection and Post-Transplant Lymphoproliferative Disorder in Pediatric Liver Transplantation. J Clin Med, 2022.PMID 35456259
- [12]Baumann U, Karam V, Adam R, et al Prognosis of Children Undergoing Liver Transplantation: A 30-Year European Study. Pediatrics, 2022.PMID 36111446