DOI: 10.1148/rg.253045114
Fibropolycystic Liver Disease: CT and MR Imaging Findings1
Giuseppe Brancatelli, MD2,
Michael P. Federle, MD,
Valérie Vilgrain, MD,
Marie-Pierre Vullierme, MD,
Daniele Marin, MD and
Roberto Lagalla, MD
1 From the Department of Radiology, University of Palermo, Italy (G.B., R.L.); the Department of Radiology, University of Pittsburgh Medical Center, Rm 4660, CHP, MT, 200 Lothrop St, Pittsburgh, PA 15213 (M.P.F.); the Department of Radiology, Beaujon Hospital, Clichy, France (V.V., M.P.V.); and the Department of Radiology, University of Rome "La Sapienza," Italy (D.M.). Recipient of a Cum Laude award for an education exhibit at the 2003 RSNA Scientific Assembly. Received May 26, 2004; revision requested June 30 and received August 12; accepted August 18. All authors have no financial relationships to disclose.
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Figure 1. Drawings illustrate ductal plate malformation in normal embryologic development. During the early developmental stage, there is a single-layer ductal plate surrounding the portal vein (a), followed by the formation of double-layered plates with a slitlike primitive bile duct lumen (b). Extensive resorption of the primitive bile ducts leads to the final stage, in which a network of fine bile ducts surrounds the portal vein (c). Insufficient resorption of ductal plates can lead to large dilated segments of the primitive bile duct surrounding the central portal vein ("central dot sign") (d). (Reprinted, with permission, from reference 6.)
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Figure 2a. Congenital hepatic fibrosis. (a) Axial T2-weighted MR image shows enlargement of the lateral segment (LS) and medial segment (MS), severe atrophy of the posterior segment (arrowheads), and renal cysts. The gall-bladder (g) separates the right lobe from the medial segment. Splenomegaly (not shown) was better visualized on a different section. (b) Axial T2-weighted MR image obtained at a different level more clearly depicts the coexistence of numerous biliary hamartomas (arrows). Two hypointense regenerative nodules are also seen (arrowheads).
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Figure 2b. Congenital hepatic fibrosis. (a) Axial T2-weighted MR image shows enlargement of the lateral segment (LS) and medial segment (MS), severe atrophy of the posterior segment (arrowheads), and renal cysts. The gall-bladder (g) separates the right lobe from the medial segment. Splenomegaly (not shown) was better visualized on a different section. (b) Axial T2-weighted MR image obtained at a different level more clearly depicts the coexistence of numerous biliary hamartomas (arrows). Two hypointense regenerative nodules are also seen (arrowheads).
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Figure 3. Congenital hepatic fibrosis and Caroli syndrome in a 24-year-old man. Coronal T2-weighted MR image shows splenomegaly (S), multiple renal cysts (arrows), and saccular dilatation of the intrahepatic biliary tree (arrowhead), findings that are typically seen in association with Caroli disease.
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Figure 4a. Congenital hepatic fibrosis. (a) Transverse contrast material–enhanced arterial phase helical CT scan shows a tangled cluster of abnormally enlarged hepatic arteries at the hilum (arrow). (b) Transverse contrast-enhanced portal venous phase helical CT scan shows a dysmorphic liver, with enlargement of the medial segment (arrowheads) and lateral segment and an atrophic right lobe. Had arterial phase images not been obtained, the hepatic arteries at the hilum could have been mistaken for portal vein cavernomatosis. In this case, there was an association of congenital hepatic fibrosis with biliary hamartomas and Caroli disease, although these findings were better seen on other images.
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Figure 4b. Congenital hepatic fibrosis. (a) Transverse contrast material–enhanced arterial phase helical CT scan shows a tangled cluster of abnormally enlarged hepatic arteries at the hilum (arrow). (b) Transverse contrast-enhanced portal venous phase helical CT scan shows a dysmorphic liver, with enlargement of the medial segment (arrowheads) and lateral segment and an atrophic right lobe. Had arterial phase images not been obtained, the hepatic arteries at the hilum could have been mistaken for portal vein cavernomatosis. In this case, there was an association of congenital hepatic fibrosis with biliary hamartomas and Caroli disease, although these findings were better seen on other images.
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Figure 5a. Biliary microhamartomas. (a) Photograph of a hepatectomy specimen shows multiple 5-mm cystic lesions in the hepatic parenchyma (arrows). (b) Photomicrograph (original magnification, x50; hematoxylin-eosin stain) shows a lesion (arrowheads) containing several cystic spaces, which are interspersed with fibrous stroma and lined by a layer of biliary epithelium. Normal hepatic parenchyma surrounds the hamartoma. Note the inspissated bile plugs within the dilated bile ducts (arrow).
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Figure 5b. Biliary microhamartomas. (a) Photograph of a hepatectomy specimen shows multiple 5-mm cystic lesions in the hepatic parenchyma (arrows). (b) Photomicrograph (original magnification, x50; hematoxylin-eosin stain) shows a lesion (arrowheads) containing several cystic spaces, which are interspersed with fibrous stroma and lined by a layer of biliary epithelium. Normal hepatic parenchyma surrounds the hamartoma. Note the inspissated bile plugs within the dilated bile ducts (arrow).
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Figure 6a. Biliary microhamartomas. (a) Axial portal venous phase CT scan shows multiple hypoattenuating cystic lesions in both lobes that measure up to 10 mm in diameter. (b) Axial portal venous phase T1-weighted MR image shows multiple nonenhancing hypointense lesions measuring up to 10 mm scattered throughout the liver. (c) Axial T2-weighted MR image shows innumerable high-signal-intensity liver lesions (arrowheads). (d) Coronal MR cholangiogram shows no communication between the cystic lesions and the normal-sized intra- and extrahepatic biliary system. Note also the normal-sized main pancreatic duct (arrowheads).
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Figure 6b. Biliary microhamartomas. (a) Axial portal venous phase CT scan shows multiple hypoattenuating cystic lesions in both lobes that measure up to 10 mm in diameter. (b) Axial portal venous phase T1-weighted MR image shows multiple nonenhancing hypointense lesions measuring up to 10 mm scattered throughout the liver. (c) Axial T2-weighted MR image shows innumerable high-signal-intensity liver lesions (arrowheads). (d) Coronal MR cholangiogram shows no communication between the cystic lesions and the normal-sized intra- and extrahepatic biliary system. Note also the normal-sized main pancreatic duct (arrowheads).
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Figure 6c. Biliary microhamartomas. (a) Axial portal venous phase CT scan shows multiple hypoattenuating cystic lesions in both lobes that measure up to 10 mm in diameter. (b) Axial portal venous phase T1-weighted MR image shows multiple nonenhancing hypointense lesions measuring up to 10 mm scattered throughout the liver. (c) Axial T2-weighted MR image shows innumerable high-signal-intensity liver lesions (arrowheads). (d) Coronal MR cholangiogram shows no communication between the cystic lesions and the normal-sized intra- and extrahepatic biliary system. Note also the normal-sized main pancreatic duct (arrowheads).
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Figure 6d. Biliary microhamartomas. (a) Axial portal venous phase CT scan shows multiple hypoattenuating cystic lesions in both lobes that measure up to 10 mm in diameter. (b) Axial portal venous phase T1-weighted MR image shows multiple nonenhancing hypointense lesions measuring up to 10 mm scattered throughout the liver. (c) Axial T2-weighted MR image shows innumerable high-signal-intensity liver lesions (arrowheads). (d) Coronal MR cholangiogram shows no communication between the cystic lesions and the normal-sized intra- and extrahepatic biliary system. Note also the normal-sized main pancreatic duct (arrowheads).
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Figure 7a. Autosomal dominant polycystic kidney and liver disease. (a) Contrast-enhanced CT scan shows multiple renal (arrowheads) and hepatic (arrows) cysts. The cysts are thin walled with regular margins. (b) Photograph of the hepatectomy specimen shows numerous cysts that extensively replace the hepatic parenchyma. The cysts range in size from microscopic to 5 cm in diameter and contained clear fluid. At microscopic analysis, the cysts were lined by a single layer of flattened to cuboidal epithelial cells.
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Figure 7b. Autosomal dominant polycystic kidney and liver disease. (a) Contrast-enhanced CT scan shows multiple renal (arrowheads) and hepatic (arrows) cysts. The cysts are thin walled with regular margins. (b) Photograph of the hepatectomy specimen shows numerous cysts that extensively replace the hepatic parenchyma. The cysts range in size from microscopic to 5 cm in diameter and contained clear fluid. At microscopic analysis, the cysts were lined by a single layer of flattened to cuboidal epithelial cells.
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Figure 8a. Autosomal dominant polycystic liver disease. (a) Unenhanced CT scan shows multiple hepatic cysts (C). Note the calcifications in the cyst wall (arrowhead). (b) On an axial T1-weighted MR image, some of the cysts (C) are hyperintense due to the presence of blood. (c) Axial T2-weighted MR image shows hemorrhagic cysts and noncomplicated cysts, with the former being less hyperintense than the latter, as is typically the case. The largest hemorrhagic cyst (C) is surrounded by a hypointense rim of hemosiderin. Note also the presence of ascites (a).
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Figure 8b. Autosomal dominant polycystic liver disease. (a) Unenhanced CT scan shows multiple hepatic cysts (C). Note the calcifications in the cyst wall (arrowhead). (b) On an axial T1-weighted MR image, some of the cysts (C) are hyperintense due to the presence of blood. (c) Axial T2-weighted MR image shows hemorrhagic cysts and noncomplicated cysts, with the former being less hyperintense than the latter, as is typically the case. The largest hemorrhagic cyst (C) is surrounded by a hypointense rim of hemosiderin. Note also the presence of ascites (a).
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Figure 8c. Autosomal dominant polycystic liver disease. (a) Unenhanced CT scan shows multiple hepatic cysts (C). Note the calcifications in the cyst wall (arrowhead). (b) On an axial T1-weighted MR image, some of the cysts (C) are hyperintense due to the presence of blood. (c) Axial T2-weighted MR image shows hemorrhagic cysts and noncomplicated cysts, with the former being less hyperintense than the latter, as is typically the case. The largest hemorrhagic cyst (C) is surrounded by a hypointense rim of hemosiderin. Note also the presence of ascites (a).
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Figure 9a. Caroli disease. (a) Unenhanced CT scan shows multiple stones (arrowheads) within dilated bile ducts (arrows). (b) Axial T2-weighted MR image shows multiple hyperintense cystic ectasias (arrows) and calculi (arrowheads). (c) Coronal MR cholangiogram shows multiple dilated bile ducts with calculi. Multiple stones are seen within the dilated common bile duct (arrow). The cystic dilatations communicate with the major biliary tree.
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Figure 9b. Caroli disease. (a) Unenhanced CT scan shows multiple stones (arrowheads) within dilated bile ducts (arrows). (b) Axial T2-weighted MR image shows multiple hyperintense cystic ectasias (arrows) and calculi (arrowheads). (c) Coronal MR cholangiogram shows multiple dilated bile ducts with calculi. Multiple stones are seen within the dilated common bile duct (arrow). The cystic dilatations communicate with the major biliary tree.
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Figure 9c. Caroli disease. (a) Unenhanced CT scan shows multiple stones (arrowheads) within dilated bile ducts (arrows). (b) Axial T2-weighted MR image shows multiple hyperintense cystic ectasias (arrows) and calculi (arrowheads). (c) Coronal MR cholangiogram shows multiple dilated bile ducts with calculi. Multiple stones are seen within the dilated common bile duct (arrow). The cystic dilatations communicate with the major biliary tree.
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Figure 10a. Caroli disease. (a) Unenhanced CT scan shows a dysmorphic liver. Multiple stones (arrows) are seen within the bile ducts. (b) Photograph of the cut surface of the explanted liver shows multiple black bilirubin casts within the intrahepatic bile ducts (arrow). Scale is in centimeters.
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Figure 10b. Caroli disease. (a) Unenhanced CT scan shows a dysmorphic liver. Multiple stones (arrows) are seen within the bile ducts. (b) Photograph of the cut surface of the explanted liver shows multiple black bilirubin casts within the intrahepatic bile ducts (arrow). Scale is in centimeters.
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Figure 11a. Caroli disease. Axial portal venous phase T1-weighted (a) and T2-weighted (b) MR images show multiple cystic dilatations of the intrahepatic bile ducts (arrows in a). The central fibrovascular bundle (central dot sign) is also seen (arrowhead).
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Figure 11b. Caroli disease. Axial portal venous phase T1-weighted (a) and T2-weighted (b) MR images show multiple cystic dilatations of the intrahepatic bile ducts (arrows in a). The central fibrovascular bundle (central dot sign) is also seen (arrowhead).
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Figure 12. Caroli disease. Percutaneous transhepatic cholangiogram shows multiple saccular dilatations of the intrahepatic bile ducts (arrows), mostly at the periphery of the liver, and fusiform dilatation of the common bile duct.
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Figure 13a. Primary sclerosing cholangitis. (a) Unenhanced CT scan shows hypertrophy of the caudate lobe (arrowheads) and decreased attenuation of the surrounding right lobe. The relative hyperattenuation of the caudate lobe gives the appearance of pseudotumor. (b) Contrast-enhanced CT scan better shows dilatation of the intrahepatic biliary tree (arrow). The posterior segment of the right hepatic lobe is severely atrophic (arrowhead). The elongated appearance of intrahepatic biliary dilatation in primary sclerosing cholangitis allows differentiation from the typical cystlike dilatation observed in Caroli disease.
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Figure 13b. Primary sclerosing cholangitis. (a) Unenhanced CT scan shows hypertrophy of the caudate lobe (arrowheads) and decreased attenuation of the surrounding right lobe. The relative hyperattenuation of the caudate lobe gives the appearance of pseudotumor. (b) Contrast-enhanced CT scan better shows dilatation of the intrahepatic biliary tree (arrow). The posterior segment of the right hepatic lobe is severely atrophic (arrowhead). The elongated appearance of intrahepatic biliary dilatation in primary sclerosing cholangitis allows differentiation from the typical cystlike dilatation observed in Caroli disease.
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Figure 14a. Choledochal cyst (type 1). (a) Contrast-enhanced CT scan shows a cystic mass (C) well demarcated due to dilatation of the distal portion of the common bile duct. (b) Coronal oblique multiplanar reformatted image shows fusiform dilatation of the common bile duct (arrow). Note also the dilatation of the intrahepatic biliary tree (arrowhead).
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Figure 14b. Choledochal cyst (type 1). (a) Contrast-enhanced CT scan shows a cystic mass (C) well demarcated due to dilatation of the distal portion of the common bile duct. (b) Coronal oblique multiplanar reformatted image shows fusiform dilatation of the common bile duct (arrow). Note also the dilatation of the intrahepatic biliary tree (arrowhead).
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Figure 15. Choledochal cyst (type 1). Percutaneous transhepatic cholangiogram shows a large choledochal cyst (C) at the level of the extrahepatic bile duct. Note the aberrant entry of the common bile duct at the side of the pancreatic duct (arrowhead).
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Figure 16. Photograph shows an excised type 1 choledochal cyst of the common bile duct (arrow) in continuity with the cystic duct and the gallbladder (arrowhead). Scale is in centimeters.
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Copyright © 2005 by the Radiological Society of North America.
