Paediatric liver transplantation: Life after portoenterostomy in biliary atresia

Abstract

Seventy-five percent to 80% of patients with biliary atresia (BA) will be candidates for paediatric liver transplantation (PLTx) throughout the first 14 years of life. They form the main group of recipients in our Paediatric Liver Transplant Unit. Of 48 children transplanted, 21 (44%) had BA. These patients present particular features, average weight of 12 kg, mean age of 3 years, and severe malnutrition prior to PLTx, which distinguish them from other paediatric candidates. With the advent of PLTx, portoenterostomy (PE) has ceased to be the only recourse for treating the majority of patients with BA. Different factors converge in these patients: some, including haemorrhage and adhesions, may present technical difficulties, and others, such as infections (19% in this series) due to severe malnutrition and malabsorption and consequent chronic rejection (14% in this series), often lead to death in the postoperative period (33% in this series). BA is treated by all paediatric surgeons, but the overall success rate now depends not only on PE but also on PLTx. In an attempt to facilitate possible later PLTx in pts with BA, the authors as paediatric surgeons performing PE or PLTx present surgical modifications and emphasize the most important medical aspects conductive to the improved general status of these pts. Our aim was to establish guidelines for taking full advantage of PE while preventing posterior problems and facilitating future transplant surgery.