Jhamb, Satvik; Decker, Christopher; Romero, Ricardo; Rivera, Ramon E.; Conway, W. Charles
Background: Congenital or primary intrahepatic bile duct (IHBD) dilatation is a rare disorder with symptoms of abdominal pain and hepatomegaly that usually presents in childhood and adolescence. Recurrent cholangitis, liver abscesses, septicemia, and biliary cirrhosis may result secondary to biliary sludge and hepatolithiasis.; Case Report: We present a case of IHBD dilatation with hepatolithiasis cured with surgical resection and discuss the management of this disease.; Conclusion: IHBD dilatation should be treated, as chronic biliary stasis and hepatolithiasis can lead to infection and recurrent cholangitis that can progress to cholangiocarcinoma. Treatment for IHBD dilatation usually involves multiple modalities including medical therapy, but ultimately resection of the diseased segments or lobes is required given the increased risk of malignancy.