Focal liver hyperplasia in Alagille syndrome: assessment with hepatoreceptor and hepatobiliary imaging

Abstract

A child with Alagille syndrome, characterized by intrahepatic bile duct paucity, developed severe liver cirrhosis and was referred for liver transplantation. In the pre-transplantation evaluation, scintigraphic scans were performed using 99mTc-galactosyl serum albumin (99mTc-GSA) as a hepatoreceptor binding agent and 99mTc-pyridoxyl-5-methyl-tryptophan (99mTc-PMT) as a hepatobiliary agent. These studies demonstrated severe hepatobiliary dysfunction with an area of increased focal uptake in the liver. Histological examination at surgery confirmed that this focal lesion was an area of compensatory hyperplasia in advanced biliary cirrhosis. We present the usefulness of these tracers for detecting the focal hyperplasia of the liver.