Scintigraphic progress of the liver in a patient with Alagille syndrome (arteriohepatic dysplasia).

We encountered a 9-year-old Japanese girl with Alagille syndrome. Her scintigraphic examinations of the liver were performed at the ages of 16 months and 9 years. 99mTc-PMT, a hepatobiliary imaging agent, was distributed homogeneously in the liver at the younger age, but unevenly produced an area of focally increased uptake in the medial segment of the liver surrounded by peripheral atrophy at the older age. 99mTc-GSA, a hepatoreceptor binding agent, was highly accumulated in the area, corresponding to the focally increased uptake of 99mTc-PMT. These imaging findings suggest that the pathophysiological and morphological changes of the liver occurred in our patient during the clinical course.