Distinct Hepatic Retention of Tc-99m IDA in Arteriohepatic Dysplasia (Alagille Syndrome)

Abstract

An unusual pattern of Tc-99m IDA scintigraphy was observed in a 20-year-old woman with arteriohepatic dysplasia (Alagille syndrome). Although neither hepatic imaging with Tc-99m tin colloid nor radiographic CT revealed any morphologic abnormality in the liver, sequential hepatobiliary imaging demonstrated abnormal radiotracer behavior—specifically, differences in tracer clearance between the peripheral and central portions of the liver. Radioactivity was persistently retained in the periphery of the liver, but the central portion showed normal tracer clearance, resulting in a photopenic center surrounded by a hot peripheral rim on the later images. The gallbladder and small intestine appeared promptly. This image pattern may be characteristic for intrahepatic bile duct hypoplasia.