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Liver Involvement in Hereditary Hemorrhagic Telangiectasia: CT and Clinical Findings Do Not Correlate in Symptomatic Patients

Jim S. Wu1,2, Sanjay Saluja1, Guadalupe Garcia-Tsao3, Alice Chong1, Katherine J. Henderson1 and Robert I. White, Jr.1

1 Department of Diagnostic Radiology, Yale University School of Medicine and Yale-New Haven Hospital, New Haven, CT.
2 Present address: Department of Radiology, Beth Israel Deaconness Medical Center, 330 Brookline Ave., Landry Bldg., Rm. 357, Boston, MA 02215.
3 Department of Gastroenterology, Section of Digestive Diseases, Yale University School of Medicine and Yale-New Haven Hospital, New Haven, CT.


Figure 1
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  		Fig. 1A —Axial CT angiography arterial phase images in 46-year-old woman with hereditary hemorrhagic telangiectasia liver disease. CT angiogram shows diffuse parenchymal heterogeneity and numerous telangiectases. All patients in this study showed this heterogenous parenchymal enhancement pattern.

 

Figure 2
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  		Fig. 1B —Axial CT angiography arterial phase images in 46-year-old woman with hereditary hemorrhagic telangiectasia liver disease. Dilation and early filling of main portal vein (arrow) during arterial phase are consistent with arterioportal shunt.

 

Figure 3
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  		Fig. 2 —Axial CT angiography arterial phase image in 57-year-old woman with symptomatic hereditary hemorrhagic telangiectasia liver disease shows markedly dilated common hepatic artery that measures 18 mm in diameter (arrow). Dilated common hepatic artery (> 4.5 mm) was seen in all of our patients.

 

Figure 4
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  		Fig. 3A —Axial CT angiography arterial phase images in 64-year-old woman with high-output cardiac failure subtype of hereditary hemorrhagic telangiectasia liver disease. Marked dilation of common hepatic artery (arrow) and extensive parenchymal heterogeneity and vascularity are seen.

 

Figure 5
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  		Fig. 3B —Axial CT angiography arterial phase images in 64-year-old woman with high-output cardiac failure subtype of hereditary hemorrhagic telangiectasia liver disease. Early filling of right hepatic vein (arrow) is consistent with arteriovenous shunt.

 

Figure 6
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  		Fig. 4 —Axial CT angiography image in 48-year-old woman with symptomatic hereditary hemorrhagic telangiectasia liver disease. Early filling of right, middle, and left hepatic veins (arrows) during arterial phase of study is consistent with arteriovenous shunt.

 

Figure 7
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  		Fig. 5A —Axial CT angiography images of 62-year-old man with symptomatic hereditary hemorrhagic telangiectasia liver disease. Opacification of main portal vein (arrow) is seen during arterial phase, which is consistent with arterioportal shunt. Splenomegaly is also present.

 

Figure 8
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  		Fig. 5B —Axial CT angiography images of 62-year-old man with symptomatic hereditary hemorrhagic telangiectasia liver disease. Contour nodularity and atrophy of right hepatic lobe are consistent with cirrhosislike changes.

 

Figure 9
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  		Fig. 6 —Axial portovenous phase CT angiography image in 48-year-old woman with biliary subtype of hereditary hemorrhagic telangiectasia liver disease. Dilation of peripheral biliary branch in right hepatic lobe (large arrow) is seen. Smaller areas of biliary dilation are also present (small arrows).

 

Figure 10
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  		Fig. 7A —Multiple axial CT angiography images in 70-year-old woman with hereditary hemorrhagic telangiectasia and liver disease show progression of biliary disease. Baseline study shows characteristic heterogeneous hepatic parenchyma and small biliary cyst in right hepatic lobe (arrow).

 

Figure 11
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  		Fig. 7B —Multiple axial CT angiography images in 70-year-old woman with hereditary hemorrhagic telangiectasia and liver disease show progression of biliary disease. One month after A, large biliary cyst lake (arrow) has developed.

 

Figure 12
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  		Fig. 7C —Multiple axial CT angiography images in 70-year-old woman with hereditary hemorrhagic telangiectasia and liver disease show progression of biliary disease. One month after B, biliary collection has become infected despite biliary drainage and antimicrobial therapy.

 

Figure 13
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  		Fig. 7D —Multiple axial CT angiography images in 70-year-old woman with hereditary hemorrhagic telangiectasia and liver disease show progression of biliary disease. Four days after C, biliary collection has markedly increased. Patient died shortly after last study.

 

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