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Published on: March 7, 2013
Primary Liver Transplantation for Biliary Atresia: Another Piece of the Puzzle
Jean de Ville de Goyet1,2, Giovanni Boroni3, Daniela Liccardo4
1Paediatric Liver Unit, ISMETT, Palermo, Italy.
Background:
Primary liver transplantation (PLT) remains a controversial alternative strategy for children with biliary atresia (BA). This national study of BA management in Italy over a 10-year period aimed to better define the indications, limitations, and outcomes of PLT.
Methods:
This was a retrospective, multicenter, observational study based on the Italian BA Registry, including 312 infants with BA managed in Italy between January 2012 and December 2021. All children who underwent PLT were selected for analysis. Children who underwent liver transplantation (LT) after Kasai portoenterostomy (KPE) failure were used as a comparison group.
Results:
Primary liver transplantation was performed in 37 children with BA and established advanced liver disease; 181 other BA patients who underwent LT were selected for comparison. Minimum follow-up was 3 years for all patients. PLT infants were significantly older at diagnosis (13 were diagnosed after 120 days of life). They had a significantly worse clinical condition both at diagnosis and at listing for transplantation. No child died before LT in the PLT group, whereas 5 patients died before LT in the early rescue-LT group. Post-transplant outcomes were comparable across groups.
Conclusions:
Kasai portoenterostomy should remain the recommended first-line treatment for most infants with BA, but its benefit is limited to only a proportion of patients. Some patients may be processed rapidly to LT because they present too late or with advanced disease: these patients have excellent outcomes as well.
