A review of long-term outcome and quality of life of patients after Kasai operation surviving with native livers

Abstract

Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy. These morbidities impose a huge impact on the quality of life of the patients and their families. Herein, we performed a comprehensive review on the clinical status and quality of life of long-term survivors of biliary atresia with their native livers, to facilitate meticulous longitudinal follow-up of these patients, and alert caregivers the probable complications to be aware of.