Korean J Radiol.  2010 Dec;11(6):665-672. 10.3348/kjr.2010.11.6.665.

Clinical Application of Liver MR Imaging in Wilson's Disease

• Cheon JE
• Kim IO
• Seo JK
• Ko JS
• Lee JM
• Shin CI
• Kim WS
• Yeon KM

• KMID: 1119230
• DOI: http://doi.org/10.3348/kjr.2010.11.6.665

Abstract

OBJECTIVE
To determine whether there is a correlation between liver MR findings and the clinical manifestations and severity of liver dysfunction in patients with Wilson's disease.
MATERIALS AND METHODS
Two radiologists retrospectively evaluated MR images of the liver in 50 patients with Wilson's disease. The Institutional Review Board approved this retrospective study and informed consent was waived. MR images were evaluated with a focus on hepatic contour abnormalities and the presence of intrahepatic nodules. By using Fisher's exact test, MR findings were compared with clinical presentations (neurological and non-neurological) and hepatic dysfunction, which was categorized by the Child-Pugh classification system (A, B and C). Follow-up MR images were available for 17 patients.
RESULTS
Contour abnormalities of the liver and intrahepatic nodules were observed in 31 patients (62%) and 25 patients (50%), respectively. Each MR finding showed a statistically significant difference (p < 0.05) among the three groups of Child-Pugh classifications (A, n = 36; B, n = 5; C, n = 9), except for splenomegaly (p = 0.243). The mean age of the patients with positive MR findings was higher than that of patients with negative MR findings. For patients with Child-Pugh class A (n = 36) with neurological presentation, intrahepatic nodules, surface nodularity, and gallbladder fossa widening were more common. Intrahepatic nodules were improved (n = 8, 47%), stationary (n = 5, 29%), or aggravated (n = 4, 24%) on follow-up MR images.
CONCLUSION
MR imaging demonstrates the contour abnormalities and parenchymal nodules of the liver in more than half of the patients with Wilson's disease, which correlates with the severity of hepatic dysfunction and clinical manifestations.

Keyword

Hepatolenticular degeneration; Liver; Magnetic resonance (MR)

MeSH Terms

Adolescent
Adult
Chi-Square Distribution
Child
Child, Preschool
Female
Hepatolenticular Degeneration/*diagnosis/pathology
Humans
Image Interpretation, Computer-Assisted
Magnetic Resonance Imaging/*methods
Male
Retrospective Studies
Statistics, Nonparametric

Figure

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  Fig. 1 Interpretation of intrahepatic nodules in Wilson's disease on T2-weighted images (TR/TE = 3500/100) A. 9-year-old girl with Wilson's disease and elevated liver enzymes (Child-Pugh class A) shows innumerable tiny, hypointense nodules (≤ 3 mm in diameter, grade 1) in liver on T2-weighted image. B. 24-year-old woman with Wilson's disease who presented with jaundice (Child-Pugh class B) shows innumerable hypointense nodules (> 3 mm in diameter, grade 2) on T2-weighted image.

• 

  Fig. 2 11-year-old girl diagnosed with Wilson's disease (Child-Pugh class A). A. T2-weighted axial image shows multiple small hypointense nodules (≤ 3 mm in diameter, grade 1) in liver. B. Follow-up T2-weighted axial image obtained 18 months after D-penicillamine treatment shows near disappearance of hypointense nodules in liver.

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  Fig. 3 11-year-old boy with Wilson's disease (Child-Pugh class B) A. T2-weighted image shows mild irregularity of liver surface and multiple, hypointense nodules in liver. B. These macronodular lesions show hyperintensities on T1-weighted images.

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  Fig. 4 24-year-old man with Wilson's disease and advanced cirrhosis (Child-Pugh class C). A. Disseminated hyperintense nodules greater than 1 cm in size relative to liver parenchyma are seen on axial T2-weighted MR image. Note surface irregularity and parenchymal contraction of liver. B. T1-weighted axial image of liver shows multiple hyperintense nodules. Gallbladder stone (arrow) is seen as nodular hyperintensity near porta hepatis. C. Harvested liver during liver transplantation shows macronodular cirrhosis. There was no evidence of hepatocellular carcinoma.

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