Imaging findings of liver involvement of Wilson's disease.

PURPOSE 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. MATERIALS AND METHODS 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. RESULTS 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. CONCLUSION 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.

[1]  A. Erden The Prognostic value of a rare sonographic pattern in Wilson's Disease , 1998, Acta radiologica.

[2]  M. Karçaaltıncaba,et al.  Multidetector CT and MRI findings in periportal space pathologies. , 2007, European journal of radiology.

[3]  U. Ozcelik,et al.  Hepatobiliary manifestations of cystic fibrosis in children: correlation of CT and US findings. , 2002, European journal of radiology.

[4]  O. Akhan Imaging of diffuse liver diseases. , 2007, European journal of radiology.

[5]  D. Conte,et al.  Severe liver fibrosis or cirrhosis: accuracy of US for detection--analysis of 300 cases. , 2003, Radiology.

[6]  J. Hauß,et al.  Liver transplantation for hepatic and neurological Wilson's disease. , 2003, Transplantation proceedings.

[7]  G. Erbaş,et al.  Normal liver, spleen, and kidney dimensions in neonates, infants, and children: evaluation with sonography. , 1998, AJR. American journal of roentgenology.

[8]  N. Robert,et al.  Diagnosis of cirrhosis based on regional changes in hepatic morphology: a radiological and pathological analysis. , 1980, Radiology.

[9]  M. Siegel,et al.  Periportal low attenuation at CT in childhood. , 1992, Radiology.

[10]  R. Felix,et al.  MRT der Leber bei Morbus Wilson , 1994 .

[11]  D G Mitchell,et al.  Enlargement of hilar periportal space: A sign of early cirrhosis at MR imaging , 2000, Journal of magnetic resonance imaging : JMRI.

[12]  R. Zachoval,et al.  IMAGES IN HEPATOLOGY , 1998 .

[13]  N. Heaton,et al.  Liver transplantation for Wilson’s disease: long-term results and quality-of-life assessment , 2003, Transplantation.

[14]  W. Vogel,et al.  Ultrasonography in Wilson disease. , 1988, Annals of internal medicine.

[15]  M. Giagheddu,et al.  Importance and significance of liver echotomography in Wilson's disease during childhood. , 1987, The Journal of nuclear medicine and allied sciences.

[16]  E. Barbosa,et al.  Abdominal ultrasonography in hepatolenticular degeneration. A study of 33 patients. , 1987, Arquivos de neuro-psiquiatria.

[17]  S. Ng,et al.  Unusual liver MR findings of Wilson's disease in an asymptomatic 2-year-old girl , 1998, Abdominal Imaging.

[18]  P. Mergo,et al.  Diffuse disease of the liver: radiologic-pathologic correlation. , 1994, Radiographics : a review publication of the Radiological Society of North America, Inc.

[19]  G. Judmaier,et al.  Zur Sonographie der Leber bei Morbus Wilson , 2008 .

[20]  Caballero Et,et al.  [Wilson's disease]. , 1965 .

[21]  A. Giorgio,et al.  Cirrhosis: value of caudate to right lobe ratio in diagnosis with US. , 1986, Radiology.

[22]  V. Kostic,et al.  Regression of nodular liver lesions in wilson's disease , 2006, Acta radiologica.

[23]  R. Semelka,et al.  MR imaging of diffuse liver disease. , 2003, Radiologic clinics of North America.

[24]  A. Oto,et al.  Unusual imaging findings in Wilson's disease , 2002, European Radiology.

[25]  G. Holcomb Cholelithiasis and Wilson's Disease , 1978 .

[26]  J. Watkins,et al.  Cholelithiasis and Wilson disease. , 1978, The Journal of pediatrics.

[27]  E. Akpınar,et al.  Liver imaging findings of Wilson's disease. , 2007, European journal of radiology.

[28]  T. Williams BASIC PATHOLOGY, 5TH ED , 1992 .

[29]  I. Wanless,et al.  Hepatic subcapsular steatosis in response to intraperitoneal insulin delivery: CT findings and prevalence. , 2003, AJR. American journal of roentgenology.