Long-Term Functional Outcome and Quality of Life After Kasai Operation

The Japanese Biliary Atresia Registry reported that 10-year and 20-year native liver survival rates were 55% and 49%, respectively. An increasing number of patients with biliary atresia have now survived into adulthood with their native livers, achieving normal physical growth, favorable liver function, near-normal neurodevelopment, and social performances. However, more than half of these patients have had liver-related complications such as portal hypertension and cholangitis, which can be a risk factor for requiring liver transplantation (LT) and impairing health-related quality of life (QOL). Therefore, life-long meticulous follow-up is mandatory in order to promote health and QOL, deal with sequelae and/or complications appropriately, as well as to determine the optimal timing of LT.