Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy

PURPOSE Whereas the Kasai portoenterostomy (KPE) is an accepted first line of surgery for bile drainage in infants with biliary atresia, its long-term effectiveness is not clear because its etiology and pathogenesis remains unknown. This study was aimed to investigate the late complications occurring in long-term survivors and the current status of living patients who survived over 10 years after KPE. METHODS A retrospective analysis of the medical records of 32 patients who underwent KPE from 1990 to 2000 was done. We analyzed 10-year survival rates with the Kaplan-Meier method and the current status of the long-term survivors. RESULTS The overall 10-year survival rate by Kaplan-Meier method after KPE was 76.2%. Eight (25%) patients had died, including 4 who were transplanted. Nineteen (59.4%) patients survived over 10 years. Among them, 6 (31.6%) patients had portal hypertension, and 5 (26.3%) had episodes of cholangitis. Two had intrahepatic cyst and 2 had intestinal obstruction. Six (31.6%) patients have been well without any complications. CONCLUSION The long-term survival rate of biliary atresia is slightly improving. However, two thirds of patients suffer from various complications. One-third of survivors go on without any complication. As biliary atresia is known as a progressive inflammatory disease, careful life-long follow- up is needed in long-term survivals after KPE.