Congenital intrahepatic portosystemic shunts: a potential cause for early-onset neonatal cholestasis

Cholestasis in the first days of life is uncommon in neonates. Neonatal cholestasis is usually associated with shock, sepsis, alloimmunity, metabolic disorders or biliary obstruction. A congenital intrahepatic portosystemic shunt results from failed involution of primordial liver vessels during the first days of life. Resulting shunts can lead to hepatic encephalopathy or liver tumors. A congenital intrahepatic portosystemic shunt should be considered when an alternative explanation cannot be found. In most cases, congenital intrahepatic portosystemic shunts will involute spontaneously by 1-2 years of age; however, surgical or radiologic closure may be needed.