Intrahepatic bile duct dilatation and cholestasis in autosomal recessive polycystic kidney disease. Demonstration with hepatobiliary scintigraphy.

Fifteen patients with the clinical and radiologic features of autosomal recessive polycystic kidney disease were investigated with radionuclide hepatobiliary scintigraphy. In nine patients (60%), cholestasis and intrahepatic bile duct dilatation were demonstrated. A 10th child had scintigraphic evidence of cholestasis, but the bile ducts were not dilated. The other five children has normal h hepatobiliary scans. The authors conclude that intrahepatic bile duct dilatation with cholestasis (Caroli's disease) is part of the clinical spectrum of autosomal recessive polycystic kidney disease and that hepatobiliary scintigraphy can be of value in determining the extent of hepatobiliary disease in this group of patients.