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Congenital Hepatic Fibrosis: CT Findings in 18 Adults¹

¹ From the Departments of Radiology (D.Z., G.B., V.V.), Pathology (M.C., C.D.), and Hepatology (D.V.), Hopital Beaujon, Clichy, France; and Departments of Radiology (M.P.F.) and Pathology (T.W.), University of Pittsburgh Medical Center, Pa. Received January 22, 2003; revision requested April 11; revision received June 24; accepted August 8. Address correspondence to G.B., Department of Radiology, University of Palermo, Via Villaermosa 29, 90139 Palermo, Italy (e-mail: gbranca@yahoo.com).

View larger version (132K): [in a new window]   Figure 1a. Patient 16. A 26-year-old woman with congenital hepatic fibrosis. (a) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase shows an enlarged liver and cystic and fusiform dilatation (arrows) of the intrahepatic bile ducts, a finding that is consistent with Caroli disease. (b) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase at a higher level than a better shows the hypertrophic medial (MS) and lateral (LS) segments. Note splenomegaly (S).

View larger version (135K): [in a new window]   Figure 1b. Patient 16. A 26-year-old woman with congenital hepatic fibrosis. (a) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase shows an enlarged liver and cystic and fusiform dilatation (arrows) of the intrahepatic bile ducts, a finding that is consistent with Caroli disease. (b) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase at a higher level than a better shows the hypertrophic medial (MS) and lateral (LS) segments. Note splenomegaly (S).

View larger version (158K): [in a new window]   Figure 2a. Patient 10. A 47-year-old woman with congenital hepatic fibrosis. (a) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase shows a dysmorphic liver with a hypertrophic lateral segment. The multiple low-attenuation subcentimeter round lesions (black arrows) are biliary hamartomas and are located mostly in a periportal location. Note large renal cysts (white arrows). (b) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase at a lower level than a shows an atrophic right lobe (arrowheads).

View larger version (169K): [in a new window]   Figure 2b. Patient 10. A 47-year-old woman with congenital hepatic fibrosis. (a) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase shows a dysmorphic liver with a hypertrophic lateral segment. The multiple low-attenuation subcentimeter round lesions (black arrows) are biliary hamartomas and are located mostly in a periportal location. Note large renal cysts (white arrows). (b) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase at a lower level than a shows an atrophic right lobe (arrowheads).

View larger version (115K): [in a new window]   Figure 3a. Patient 1. A 33-year-old woman with congenital hepatic fibrosis. (a) Transverse unenhanced helical CT scan shows an enlarged left lateral segment (LS). Note bilateral renal calculi (arrows). (b) Transverse contrast-enhanced helical CT scan obtained during the hepatic arterial phase shows a hyperattenuating lesion (arrow) with marked homogeneous enhancement. These were large, multiacinar regenerative nodules at pathologic examination. (c) Transverse contrast-enhanced helical CT scan obtained in portal venous phase shows that the lesion is still hyperattenuating in comparison with the surrounding parenchyma. Note the paraumbilical vein (arrow).

View larger version (133K): [in a new window]   Figure 3b. Patient 1. A 33-year-old woman with congenital hepatic fibrosis. (a) Transverse unenhanced helical CT scan shows an enlarged left lateral segment (LS). Note bilateral renal calculi (arrows). (b) Transverse contrast-enhanced helical CT scan obtained during the hepatic arterial phase shows a hyperattenuating lesion (arrow) with marked homogeneous enhancement. These were large, multiacinar regenerative nodules at pathologic examination. (c) Transverse contrast-enhanced helical CT scan obtained in portal venous phase shows that the lesion is still hyperattenuating in comparison with the surrounding parenchyma. Note the paraumbilical vein (arrow).

View larger version (123K): [in a new window]   Figure 3c. Patient 1. A 33-year-old woman with congenital hepatic fibrosis. (a) Transverse unenhanced helical CT scan shows an enlarged left lateral segment (LS). Note bilateral renal calculi (arrows). (b) Transverse contrast-enhanced helical CT scan obtained during the hepatic arterial phase shows a hyperattenuating lesion (arrow) with marked homogeneous enhancement. These were large, multiacinar regenerative nodules at pathologic examination. (c) Transverse contrast-enhanced helical CT scan obtained in portal venous phase shows that the lesion is still hyperattenuating in comparison with the surrounding parenchyma. Note the paraumbilical vein (arrow).

View larger version (138K): [in a new window]   Figure 4a. Patient 17. A 22-year-old man with congenital hepatic fibrosis. (a) Transverse contrast-enhanced helical CT scan obtained during the hepatic arterial phase shows a tangled cluster of abnormally enlarged hepatic arterial vessels (arrow) at the hilum. (b) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase shows a dysmorphic liver with enlarged medial (arrowheads) and lateral segments and an atrophic right lobe. Had arterial phase images not been obtained, the hepatic arterial vessels at the hilum could have been mistaken for cavernous transformation of the portal vein. In this patient, there was association of congenital hepatic fibrosis with biliary hamartomas and Caroli disease, although this was better seen on other images.

View larger version (141K): [in a new window]   Figure 4b. Patient 17. A 22-year-old man with congenital hepatic fibrosis. (a) Transverse contrast-enhanced helical CT scan obtained during the hepatic arterial phase shows a tangled cluster of abnormally enlarged hepatic arterial vessels (arrow) at the hilum. (b) Transverse contrast-enhanced helical CT scan obtained during the portal venous phase shows a dysmorphic liver with enlarged medial (arrowheads) and lateral segments and an atrophic right lobe. Had arterial phase images not been obtained, the hepatic arterial vessels at the hilum could have been mistaken for cavernous transformation of the portal vein. In this patient, there was association of congenital hepatic fibrosis with biliary hamartomas and Caroli disease, although this was better seen on other images.

View larger version (147K): [in a new window]   Figure 5. Patient 13. A 39-year-old woman with congenital hepatic fibrosis. Transverse contrast-enhanced helical CT scan obtained during the hepatic late arterial phase shows hyperattenuating lesions (arrows). Although biopsy of these nodules was not performed, they were stable at 1-year follow-up and were likely multiacinar and regenerative. Enlarged inferior vena cava (IVC) is due to a spontaneous splenorenal shunt (SS).