Hiromi ISHIBASHI, Yoshihiro TSUCHIYA, Kazufumi DOHMEN, Ryuji SHIMAMURA, Hiroko KONDO, Yasuhiko HIRATA, Jiro KUDO

The First Department of Internal Medicine, Faculty of Medicine, Kyushu University

キーワード : Wilson's disease, Copper deposition, Cirrhosis, Nodular lesion, Fulminant hepatitis

The hepatic ultrasonographic and CT findings in two patients with Wilson's disease which was manifested as fulminant hepatitis are here presented and the literature describing this subject is reviewed. The sonography of one patient in this study revealed an enlarged liver with a smooth surface and a sharp edge, which had, however, a finely irregular parenchymal echo pattern. The postmortem examination revealed that the surface of the liver was smooth and there were no findings of cirrhosis. The histology of the liver showed massive necrosis without any nodular formations. The liver of another patient, however, presented a cirrhotic sonographic pattern. Multiple indistinctly demarcated hyperechoic nodular lesions could be observed diffusely in the coarse reticulated hepatic parenchyma of the liver. CT also showed multiple high density areas in the liver. A pathological examination revealed cirrhotic liver with pseudolobules, the majority of which had fallen into massive necrosis.
The ultrasonographic appearance of the liver with Wilson's disease or with abdominal Wilson is not specific and depends on the hepatic pathology. Notwithstanding, the feature of multiple hyperechoic nodular pattern seems to be a characteristic finding for a cirrhotic liver with Wilson's disease.