Congenital Hepatic Fibrosis in a Patient With Suspected Postcholecystectomy Syndrome: Hepatobiliary Scintigraphy at Diagnosis and During Follow-up

A 43-year-old woman was thought to have a postcholecystectomy syndrome with biliary pain and mild elevation of liver enzymes. Quantitative hepatobiliary scintigraphy demonstrated gross hepatomegaly with a dominant left lobe with radionuclide retention in the intrahepatic biliary tree. Abdominal ultrasound, computed tomography, and endoscopic retrograde cholangiography were unable to establish an exact diagnosis. Liver biopsy revealed advanced fibrotic changes with an abnormal arrangement of dilated bile ducts and fibroblasts, and a diagnosis of congenital hepatic fibrosis was established. Because of the signs of intrahepatic cholestasis, ursodeoxycholic acid treatment using Ursofalk (Falk, Germany) 250 mg thrice daily was initiated. After 6 months of medical therapy, quantitative hepatobiliary scintigraphy was repeated to assess the improvement of hepatic bile secretion.