Biliary Atresia (BA) is the most common liver disease leading to liver transplantation (LT) during childhood. The optimal timing of listing and LT for children with failed porto-enterostomy (PE) is not clear.
Objective: to determine the optimal timing of LT for children with BA and failed PE by using a Markov model simulation analysis.
Methods: A Markov model was constructed presenting the progression of the severity of liver status for patients with BA who had PE before 60 days old. Three treatment strategies were compared: liver transplant for moderate liver disease (MLD), liver transplant for severe liver disease (SLD) and no LT and 10,000 patients were simulated in each strategy. Health states were defined as: LT, early Re-LT (<=30 days after LT), late Re-LT (>30 days after LT), status post LT (period after first liver transplantation) and death.
Results: For cases with an available liver for transplantation (living donors) LT at MLD was associated with an increase of 17.4% additional expected life years (LY) as compared with LT at SLD. Patient survival rates after 10 years were 84.7% and 75.5% in the MLD and SLD strategies respectively. For the patients with no-LT the survival rate after 10 years was 48.1%. When the probability of deceased donor LT was lower than 50% from time of listing at 3 months, there was no increase in expected LY of MLD strategy. "No LT" resulted in about a 60% reduction of expected LY compared to LT for patients with SLD.
Conclusions: Our model suggests that early listing and transplantation is beneficial in cases with an available liver for transplantation. For cases where the probability for LT is low there appears to be no advantage to early listing. A validation of this model in a "real" cohort of patients with BA is needed.
(C) 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,