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 autosoma l recessive polycystic kidney disease were investigated with radionucl ide hepatobiliary scintigraphy. In nine patients (60%), cholestasis an d intrahepatic bile duct dilatation were demonstrated. A 10th child ha d scintigraphic evidence of cholestasis, but the bile ducts were not d ilated. The other five children had normal hepatobiliary scans. The au thors conclude that intrahepatic bile duct dilatation with cholestasis (Caroli's disease) is part of the clinical spectrum of autosomal rece ssive polycystic kidney disease and that hepatobiliary scintigraphy ca n be of value in determining the extent of hepatobiliary disease in th is group of patients.