Imaging findings of liver involvement of Wilson's disease

The major purpose of this paper is to outline and correlate US, CT and MR imaging findings of liver in patients with Wilson's disease.

Twenty-eight patients (10 male, 18 female, median age 16) with Wilson's disease were examined with US, CT and MRI. Liver echogenicity, echo pattern, contour irregularity, periportal thickness, perihepatic fat layer thickness, the presence of focal parenchymal lesion, and other associated findings were recorded by US in every patient. CT and MRI were done in 20 and 12 patients, respectively.

Contour irregularity was observed in 24 patients and heterogeneous parenchymal echo pattern was seen in 26 patients with US. The presence of increased perihepatic fat layer was observed in eight patients. Severe parenchymal atrophy was seen in all lobes of the liver including caudate in contrast to post-viral chronic liver disease. Nineteen patients had periportal thickening. US showed multiple hyper and/or hypoechoic nodules smaller than 1cm in 13 patients. MRI demonstrated small nodular hypointense areas on T2-weighted images in five patients. In only one patient these nodules showed contrast enhancement in CT and MR and pathologically proved to be dysplastic nodule.

Wilson's disease involving the liver has several unique radiological findings in comparison to other types of cirrhosis. These specific features include multiple nodular lesions in the liver, presence of perihepatic fat layer and normal caudate lobe which is contrary to other types cirrhosis. Liver injury in the Wilson's disease can be defined by imaging modalities, especially by US which we think is the most precise imaging modality to detect early parenchymal changes in the progress of the disease.