Congenital hepatic shunt

A 9-day-old boy presented to our emergency department with jaundice since birth. Laboratory tests showed marked unconjugated hyperbilirubinemia. Liver US showed an abnormal intrahepatic connection (curved arrows) between the right portal vein (red arrows) and right hepatic vein (white arrows). A turbulent Doppler pattern was noted in the portal vein (Fig 1). No other imaging was performed. At 8 weeks of age, unconjugated bilirubin levels were lower but still abnormal. At the time of this report, the patient was being monitored and had no clinical manifestations of the shunt. Congenital intrahepatic portosystemic shunts are rare and can be found incidentally in asymptomatic patients [1, 2]. When present, symptoms can include hepatomegaly, metabolic disorders, encephalopathy, and jaundice [2]. CT and MRI can provide additional information, but color Doppler US is often the first diagnostic imaging test and may be