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Hepatic Nodular Lesions Associated with Abnormal Development of the Portal Vein

Tonsok Kim1, Takamichi Murakami1, Eiji Sugihara1, Masatoshi Hori1, Kenji Wakasa2 and Hironobu Nakamura1

1 Department of Radiology, Osaka University Graduate School of Medicine, D1, 2-2 Yamadaoka, Suita City, Osaka 565-0871, Japan.
2 Department of Pathology, Osaka City University Medical School, 1-4-3 Asahimachi, Abenoku, Osaka City 545-8585, Japan.



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Fig. 1A. 18-year-old man (patient 1) with hepatic masses associated with patent ductus venosus. Early phase contrast-enhanced helical CT image shows heterogeneous enhancement of right lobe of liver.

 


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Fig. 1B. 18-year-old man (patient 1) with hepatic masses associated with patent ductus venosus. Late phase CT image shows mass (thick arrows) with hypoattenuation. Enlarged abnormal shunt vein (arrowhead) connecting main portal trunk to inferior vena cava is seen, but no intrahepatic portal branches are observed. Left and middle hepatic veins (thin arrows) are visible.

 


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Fig. 1C. 18-year-old man (patient 1) with hepatic masses associated with patent ductus venosus. T2-weighted spin-echo MR image (TR/TE, 2,000/80) more conspicuously shows greater number of hepatic masses (arrows) with high signal intensity than do CT images. Linear high-signal-intensity area (arrowhead) indicating central scar within mass is seen.

 


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Fig. 1D. 18-year-old man (patient 1) with hepatic masses associated with patent ductus venosus. Arterial portogram shows venous shunt flow (arrow) from main portal trunk to right atrium, but no visible intrahepatic portal branches.

 


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Fig. 1E. 18-year-old man (patient 1) with hepatic masses associated with patent ductus venosus. Microscopic image of hepatic mass obtained by percutaneous needle biopsy shows hyperplasia of hepatocytes and led to diagnosis of focal nodular hyperplasia. (H and E, x50)

 


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Fig. 2A. 6-year-old boy (patient 2) with hepatic mass associated with patent ductus venosus. Maximum-intensity-projection image from 2D time-of-flight MR angiography shows abnormal shunt vein (arrow) running from main portal trunk toward right atrium, but no intrahepatic branches. Inferior vena cava is also shown.

 


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Fig. 2B. 6-year-old boy (patient 2) with hepatic mass associated with patent ductus venosus. Retrograde venogram of abnormal shunt vein (arrow) through catheter inserted via right atrium reveals hypoplastic intrahepatic portal branch (arrowhead).

 


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Fig. 2C. 6-year-old boy (patient 2) with hepatic mass associated with patent ductus venosus. T2-weighted spin-echo MR image (TR/TE, 1,800/80) shows large hepatic mass (arrow) of mild high signal intensity in right lobe of liver.

 


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Fig. 3A. 8-year-old boy (patient 3) with hepatic masses associated with congenital absence of portal vein. Early phase contrast-enhanced helical CT image obtained at level of hepatic hilum shows hepatic artery (arrow).

 


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Fig. 3B. 8-year-old boy (patient 3) with hepatic masses associated with congenital absence of portal vein. Early phase CT image obtained at more caudal level than A shows superior mesenteric vein (arrow) connected with left renal vein (arrowhead).

 


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Fig. 3C. 8-year-old boy (patient 3) with hepatic masses associated with congenital absence of portal vein. Late phase CT image shows dilated hepatic artery (arrow) and also shows portal vein is absent. Hypoattenuated mass (arrowheads) is seen in left lobe of liver.

 


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Fig. 3D. 8-year-old boy (patient 3) with hepatic masses associated with congenital absence of portal vein. T1-weighted spin-echo MR image (TR/TE, 600/10) more conspicuously shows greater number of hepatic masses (arrows) of increased signal intensity than do CT images.

 


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Fig. 3E. 8-year-old boy (patient 3) with hepatic masses associated with congenital absence of portal vein. Arterial portogram depicts venous flow from superior mesenteric vein (arrow) to inferior vena cava (arrowhead).

 

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