Purpose or Case Report: To describe ultrasound (US) and magnetic resonance imaging (MRI) findings of giant hepatic regenerative nodules (GHRN) in pediatric patients with Alagille Syndrome (AS).
Methods & Materials: In a retrospective review over a 12 year period of hepatic masses in patients with AS, we found six patients with large hepatic masses, each of whom had US and MRI studies. Imaging and medical records were then analyzed.
Results: Our study revealed 3 males and 3 females, mean age of 8.8 years (range 1.4 - 25.9 years). All patients had imaging findings showing varying degrees of chronic liver disease and portal hypertension. In the setting of cirrhosis, large hepatic masses, which ranged from 6.4 cm to 11.8 cm in size, were also identified. On US, all masses were isoechoic to adjacent liver, and splayed portal vessels; large segments of the portal veins (PV) and hepatic veins (HV) were also noted to course through the center of several masses. On MRI, 5/6 masses were slightly low to isointense in signal intensity (SI) on T1W and low SI on T2W images. One GHRN showed increased SI on T1. Post-contrast with conventional gadolinium chelates, the enhancement pattern of the masses followed that of adjacent liver. In one case where Eovist was given, the mass enhanced similar to the adjacent liver. Similar to US, PV branches were seen centrally. In 4/6 cases with DWI sequences, GHRN did not restrict. Four patients had pathologic correlation demonstrating benign hepatic nodules with preserved ductal architecture.
Conclusions: Patients with AS can develop cirrhosis which puts them at high risk of developing primary hepatic neoplasms. Among our group of AS patients from over a 12-year period, GHRN were the only hepatic masses we identified. Strikingly large in size, with distinct US and MRI features, recognition and discrimination of these lesions from malignant tumors, such as hepatocellular carcinoma, is important to avoid unnecessary intervention.