Priya Ramachandran1, Mohamed Safwan, Mettu Srinivas Reddy, Mohamed Rela. 1. Institute of Liver Disease and Transplantation, Global Health Chennai; and Childs Trust Medical Research Foundation, National Foundation for Liver Research; Chennai, India. Correspondence to: Prof Mohamed Rela, Director, Institute of Liver Disease and Transplantation, Global Health City, #439, Cheran Nagar, Perumbakkam, Chennai 600 100, India. mohamedrela@gmail.com.
Abstract
NEED AND PURPOSE OF REVIEW: Biliary atresia is a progressive obstructive cholangiopathy and is fatal if left untreated within 2 years of life. Delay in referral is because of difficulties in differentiating it from physiologic jaundice and identifying an abnormal stool color. This paper presents an overview on the diagnosis and discusses the current strategies in the management of this disease in developing countries. METHODS: Articles were retrieved from the PubMed database using the terms biliary atresia, Kasai portoenterostomy and pediatric liver transplantation. Contents of the article are also based on personal experience of the authors. CONCLUSION: A national screening program using stool color cards as part of standard care in the neonatal period will greatly improve early detection of biliary atresia. Outcomes will improve if it is diagnosed at the earliest after birth, the child is referred to an experienced pediatric hepatobiliary unit for evaluation, and undergoes an early Kasai procedure. If an early Kasai portoenterostomy is performed, nearly half of all children survive into adolescence, and about one-third are likely to have a long-term, symptom-free life with normal liver biochemistry. Sequential treatment combining Kasai as first line and liver transplantation as second line results in 90% survival for children with biliary atresia.
NEED AND PURPOSE OF REVIEW: Biliary atresia is a progressive obstructive cholangiopathy and is fatal if left untreated within 2 years of life. Delay in referral is because of difficulties in differentiating it from physiologic jaundice and identifying an abnormal stool color. This paper presents an overview on the diagnosis and discusses the current strategies in the management of this disease in developing countries. METHODS: Articles were retrieved from the PubMed database using the terms biliary atresia, Kasai portoenterostomy and pediatric liver transplantation. Contents of the article are also based on personal experience of the authors. CONCLUSION: A national screening program using stool color cards as part of standard care in the neonatal period will greatly improve early detection of biliary atresia. Outcomes will improve if it is diagnosed at the earliest after birth, the child is referred to an experienced pediatric hepatobiliary unit for evaluation, and undergoes an early Kasai procedure. If an early Kasai portoenterostomy is performed, nearly half of all children survive into adolescence, and about one-third are likely to have a long-term, symptom-free life with normal liver biochemistry. Sequential treatment combining Kasai as first line and liver transplantation as second line results in 90% survival for children with biliary atresia.