Neonatal cholestasis most often presents as prolonged jaundice, beyond 2 weeks of age. Such babies should always be investigated for conjugated hyperbilirubinemia and if this is detected further work-up for cholestasis is mandatory. Simultaneously, prothrombin time must be checked and pathologic levels need to be treated with intravenous vitamin K, to avoid detrimental bleedings. Neonatal cholestasis occurs in approximately 1 in 2500 term infants, the most common underlying diseases being biliary atresia (BA), viral infections, a₁-antitrypsin deficiency and other genetic cholestatic disorders.

Patients with BA most commonly present with jaundice and pale stools. Population based screening of all infants, by the use of stool colour cards sent to the parents can speed up the detection of cases with BA and thereby improve the success rate of the surgical procedure.

Once cholestasis is established, there is a need for a relatively rapid series of investigations. The reason for this “semiacute” management is to define certain situations where early treatment is lifesaving, such as septicemia, galactosemia or hypocortisolism, as well as to ensure timely surgical management in BA patients. A stepwise approach of investigations is suggested, where percutaneous liver biopsy is of value to select patients with suspected biliary atresia for laparotomy.

All patients, regardless of aetiology, should receive fat soluble vitamins as long as they are cholestatic. Although widely used, the effect of ursodeoxycholic acid is less well proven. However, it seems to be beneficiary in subsets of patient groups and the side effects are very minor.