SEQUENTIAL TREATMENT OF BILIARY ATRESIA WITH KASAI PORTOENTEROSTOMY AND LIVER-TRANSPLANTATION - A REVIEW

Biliary atresia is the most frequent cause of chronic cholestasis in i nfants. When left untreated, this condition leads to death from liver insufficiency within the first 2 yr of life. The modern therapeutic ap proach consists of a sequential strategy with Kasai portoenterostomy a s a first step and, in case of failure, liver transplantation. After p ortoenterostomy, no more than 20% to 30% of patients will live jaundic e-free into adulthood. Illness in another third will be palliated, and these patients have extended survival, delaying liver transplantation to later childhood (2 to 15 yr). The remaining 30% to 40% will not be nefit from the Kasai operation and will die of liver failure in infanc y. The annual need of liver transplantation for biliary atresia is one case per million people. This indication represents 35% to 67% of the reported series of pediatric liver transplantation and between 5% and 10% of the indications for liver transplantation, all ages included. Approximately four of five children transplanted for biliary atresia w ill become long-term survivors with good physical and mental developme nt; recurrence of the disease after transplantation has not been obser ved. Because most candidates are young children (<3 yr) of small size (<10 kg), there is a shortage of size-matched donors (which has been a lleviated by the use of innovative techniques such as reduced and spli t livers). The resulting redistribution of the adult donor liver pool is ethically justified by: the equal quality of the results after tran splantation of a full-size or partial graft.