Neonatal Cholestasis and Biliary Atresia

Abstract

Neonatal cholestasis is defined as an impairment of bile formation or bile flow in the first 3months of life and is associated with a conjugated or direct bilirubin fraction of ≥1mgdL−1 (17.1μmolL−1) and ≥20% of total bilirubin. There are numerous causes of neonatal cholestasis, of which the majority fall into a few discrete yet overlapping categories. The importance of prompt recognition and definitive diagnosis of neonatal cholestasis cannot be overemphasized. Many causes of cholestasis, such as sepsis, endocrinopathies, inborn errors of metabolism, biliary atresia, and choledochal cyst, are treatable. Medical management includes monitoring for growth failure, fat soluble vitamin deficiencies and end stage liver disease.