Fibropolycystic Liver Disease: CT and MR Imaging Findings

Published Online:https://doi.org/10.1148/rg.253045114

Fibropolycystic liver disease encompasses a spectrum of related lesions of the liver and biliary tract that are caused by abnormal embryologic development of the ductal plates. These lesions (congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, choledochal cysts) can be clinically silent or can cause signs and symptoms such as cholangitis, portal hypertension, gastrointestinal bleeding, infections, and space-occupying masses. The different types of fibropolycystic liver disease demonstrate characteristic findings at computed tomography (CT) and magnetic resonance (MR) imaging. Patients with congenital hepatic fibrosis typically have imaging evidence of liver morphologic abnormalities, varices, splenomegaly, renal lesions, and other associated ductal plate abnormalities. Biliary hamartomas usually manifest as multiple cysts that are nearly uniform in size and measure up to 15 mm in diameter. Autosomal dominant polycystic disease typically manifests as an enlarged and diffusely cystic liver. In Caroli disease, cystic or fusiform dilatation of the intrahepatic ducts is seen, as well as the “central dot sign,” which corresponds to a portal vein branch protruding into the lumen of a dilated bile duct. Choledochal cyst manifests as a fusiform or cystic dilatation of the extrahepatic bile duct. Awareness of these CT and MR imaging features is essential in detecting and differentiating between various fibropolycystic liver diseases and can assist in proper management.

© RSNA, 2005

References

  • 1 SherlockS. Cystic diseases of the liver. In: Schiff L, Schiff E, eds. Diseases of the liver. 7th ed. Philadelphia, Pa: Lippincott, 1999; 1083–1090. Google Scholar
  • 2 IshakKG, Sharp HL. Developmental abnormalities and liver disease in childhood. In: MacSween RN, Burt AD, Portmann BC, Ishak KG, Scheuer PJ, Anthony PP, eds. Pathology of the liver. 3rd ed. Edinburgh, Scotland: Churchill Livingstone, 2001; 107–154. Google Scholar
  • 3 SummerfieldJA, Nagafuchi Y, Sherlock S, Cadafalch J, Scheuer PJ. Hepatobiliary fibropolycystic diseases: a clinical and histological review of 51 patients. J Hepatol1986; 2: 141–156. Crossref, MedlineGoogle Scholar
  • 4 DesmetVJ. Congenital diseases of intrahepatic bile ducts: variations on the theme “ductal plate malformation.” Hepatology1992; 16: 1069–1083. Crossref, MedlineGoogle Scholar
  • 5 KrauseD, Cercueil JP, Dranssart M, Cognet F, Piard F, Hillon P. MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr2002; 26: 541–552. Crossref, MedlineGoogle Scholar
  • 6 MarchalGJ, Desmet VJ, Proesmans WC, et al. Caroli disease: high-frequency US and pathologic findings. Radiology1986; 158: 507–511. LinkGoogle Scholar
  • 7 DesmetVJ. What is congenital hepatic fibrosis? Histopathology1992; 20: 465–477. Crossref, MedlineGoogle Scholar
  • 8 BenhamouJ. Congenital hepatic fibrosis and Caroli’s disease. In: Schiff L, Schiff E, eds. Diseases of the liver. 7th ed. Philadelphia, Pa: Lippincott, 1999; 1204–1209. Google Scholar
  • 9 BernsteinJ, Stickler GB, Neel IV. Congenital hepatic fibrosis: evolving morphology. APMIS Suppl1988; 4: 17–26. MedlineGoogle Scholar
  • 10 BayraktarY, Balkanci F, Kayhan B, et al. Congenital hepatic fibrosis associated with cavernous transformation of the portal vein. Hepatogastroenterology1997; 44: 1588–1594. MedlineGoogle Scholar
  • 11 OdievreM, Chaumont P, Montagne JP, Alagille D. Anomalies of the intrahepatic portal venous system in congenital hepatic fibrosis. Radiology1977; 122: 427–430. LinkGoogle Scholar
  • 12 AverbackP. Congenital hepatic fibrosis: asymptomatic adults without renal anomaly. Arch Pathol Lab Med1977; 101: 260–261. MedlineGoogle Scholar
  • 13 De LedinghenV, Le Bail B, Trillaud H, et al. Case report: secondary biliary cirrhosis possibly related to congenital hepatic fibrosis—evidence of decreased number of portal branch veins and hypertrophic peribiliary vascular plexus. J Gastroenterol Hepatol1998; 13: 720–724. Crossref, MedlineGoogle Scholar
  • 14 De VosM, Barbier F, Cuvelier C. Congenital hepatic fibrosis. J Hepatol1988; 6: 222–228. Crossref, MedlineGoogle Scholar
  • 15 ZeitounD, Brancatelli G, Colombat M, et al. Congenital hepatic fibrosis: CT findings in 18 adults. Radiology2004; 231: 109–116. LinkGoogle Scholar
  • 16 CavallariA. Bile duct hamartomas: diagnostic problems and treatment. Hepatogastroenterology1997; 44: 994–997. MedlineGoogle Scholar
  • 17 Lev-ToaffAS, Bach AM, Wechsler RJ, Hilpert PL, Gatalica Z, Rubin R. The radiologic and pathologic spectrum of biliary hamartomas. AJR Am J Roentgenol1995; 165: 309–313. Crossref, MedlineGoogle Scholar
  • 18 CheungYC, Tan CF, Wan YL, Lui KW, Tsai CC. MRI of multiple biliary hamartomas. Br J Radiol1997; 70: 527–529. Crossref, MedlineGoogle Scholar
  • 19 MorteleB, Mortele K, Seynaeve P, Vandevelde D, Kunnen M, Ros PR. Hepatic bile duct hamartomas (von Meyenburg complexes): MR and MR cholangiography findings. J Comput Assist Tomogr2002; 26: 438–443. Crossref, MedlineGoogle Scholar
  • 20 SloneHW, Bennett WF, Bova JG. MR findings of multiple biliary hamartomas. AJR Am J Roentgenol1993; 161: 581–583. Crossref, MedlineGoogle Scholar
  • 21 YazijiN, Martin L, Hillon P, Favre JP, Henninger JF, Piard F. Cholangiocarcinoma arising from biliary microhamartomas in a man suffering from hemochromatosis. Ann Pathol1997; 17: 346–349. MedlineGoogle Scholar
  • 22 SemelkaR, Hussain SM, Marcos HB, Woosley JT. Biliary hamartomas: solitary and multiple lesions shown on current MR techniques including gadolinium enhancement. J Magn Reson Imaging1999; 10: 196–201. Crossref, MedlineGoogle Scholar
  • 23 MorteleKJ, Ros PR. Cystic focal liver lesions in the adult: differential CT and MR imaging features. RadioGraphics2001; 21: 895–910. LinkGoogle Scholar
  • 24 GuyF, Cognet F, Dranssart M, Cercueil JP, Conciatori L, Krause D. Caroli’s disease: magnetic resonance imaging features. Eur Radiol2002; 12: 2730–2736. Crossref, MedlineGoogle Scholar
  • 25 LevyAD, Rohrmann CA Jr, Murakata LA, Lonergan GJ. Caroli’s disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol2002; 179: 1053–1057. Crossref, MedlineGoogle Scholar
  • 26 MillerWJ, Sechtin AG, Campbell WL, Pieters PC. Imaging findings in Caroli’s disease. AJR Am J Roentgenol1995; 165: 333–337. Crossref, MedlineGoogle Scholar
  • 27 FulcherAS, Turner MA, Sanyal AJ. Case 38: Caroli disease and renal tubular ectasia. Radiology2001; 220: 720–723. LinkGoogle Scholar
  • 28 ChoiBI, Yeon KM, Kim SH, Han MC. Caroli disease: central dot sign in CT. Radiology1990; 174: 161–163. LinkGoogle Scholar
  • 29 BlousteinPA. Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol1977; 67: 40–46. MedlineGoogle Scholar
  • 30 DoddGD III, Baron RL, Oliver JH III, Federle MP. End-stage primary sclerosing cholangitis: CT findings of hepatic morphology in 36 patients. Radiology1999; 211: 357–362. LinkGoogle Scholar
  • 31 LimJH. Oriental cholangiohepatitis: pathologic, clinical and radiologic features. AJR Am J Roentgenol1991; 157: 1–8. Crossref, MedlineGoogle Scholar
  • 32 FederleMP, Cello JP, Laing FC, Jeffrey RB. Recurrent pyogenic cholangitis in Asian immigrants. Radiology1982; 143: 151–156. LinkGoogle Scholar
  • 33 KimMJ, Han SJ, Yoon CS, et al. Using MR cholangiopancreatography to reveal anomalous pancreaticobiliary ductal union in infants and children with choledochal cysts. AJR Am J Roentgenol2002; 179: 209–214. Crossref, MedlineGoogle Scholar
  • 34 KimOH, Chung HJ, Choi BG. Imaging of the choledochal cyst. RadioGraphics1995; 15: 69–88. LinkGoogle Scholar
  • 35 IwaiN, Yanagihara J, Tokiwa K, Shimotake T, Nakamura K. Congenital choledochal dilatation with emphasis on pathophysiology of the biliary tract. Ann Surg1992; 215: 27–30. Crossref, MedlineGoogle Scholar
  • 36 BabbittDP, Starshak RJ, Clemett AR. Choledochal cyst: a concept of etiology. Am J Roentgenol Radium Ther Nucl Med1973; 119: 57–62. Crossref, MedlineGoogle Scholar
  • 37 TodaniT, Watanabe Y, Fujii T, Toki A, Uemura S, Koike Y. Congenital choledochal cyst with intrahepatic involvement. Arch Surg1984; 119: 1038–1043. Crossref, MedlineGoogle Scholar
  • 38 SavaderSJ, Benenati JF, Venbrux AC, et al. Choledochal cysts: classification and cholangiographic appearance. AJR Am J Roentgenol1991; 156: 327–331. Crossref, MedlineGoogle Scholar

Article History

Published in print: May 2005