Skip to main content
SpringerLink
Log in

Radiologic Findings in the Fibrocystic Diseases

  • Chapter
  • First Online:
Fibrocystic Diseases of the Liver

Part of the book series: Clinical Gastroenterology ((CG))

  • 817 Accesses

Summary

Noninvasive evaluation of patients with suspected or known fibrocystic liver diseases is widely available using cross-sectional imaging techniques. The various types of fibrocystic diseases, as well as important differential diagnostic entities, can be distinguished from each other confidently using morphologic and functional criteria. Biliary atresia, for example, may not only be suspected by morphologic ultrasound appearance of the liver in combination with the appropriate clinical parameters but also be proven by cholangiogram.

When specific questions, such as communication between the biliary system and the small intrahepatic cysts, need to be answered to find the correct diagnosis, endoscopic retrograde cholangiography (ERC) can provide the best spatial resolution as well as functional information. ERC is considered the gold standard for evaluation of the biliary tree and, in selected cases, may be the only modality to demonstrate key features differentiating biliary hamartomas, polycystic liver disease, and Caroli disease. However, magnetic resonance cholangiography (MRC) has reached a level of spatial resolution that frequently allows noninvasive delineation of these small structures confidently in many patients.

Choledochal cysts are often diagnosed on computed tomography, magnetic resonance tomography, or endoscopic ultrasound incidentally or when evaluating a complication such as cholangitis. ERC may be indicated to clarify the type of choledochal cyst or treat complications such as choledocholithiasis. More invasive methods, such as percutaneous transhepatic cholangiography or postoperative T-tube cholangiography, are predominantly performed as an interventional procedure for the treatment of complications of fibrocystic hepatic diseases.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 299.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Humphrey TM, Stringer MD. Biliary atresia: US diagnosis. Radiology 2007;244, 845–851.

    Article  PubMed  Google Scholar 

  2. Lee HJ, Lee SM, Park WH, Choi SO. Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 2003;229, 395–400.

    Article  PubMed  Google Scholar 

  3. Park WH, Choi SO, Lee HJ. The ultrasonographic 'triangular cord' coupled with gallbladder images in the diagnostic prediction of biliary atresia from infantile intrahepatic cholestasis. J Pediatr Surg 1999;34, 1706–1710.

    Article  CAS  PubMed  Google Scholar 

  4. Choi SO, Park WH, Lee HJ, Woo SK. 'Triangular cord': A sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg 1996;31, 363–366.

    Article  CAS  PubMed  Google Scholar 

  5. Li SX, Zhang Y, Sun M et al. Ultrasonic diagnosis of biliary atresia: A retrospective analysis of 20 patients. World J Gastroenterol 2008;14, 3579–3582.

    Article  PubMed  Google Scholar 

  6. Johnson K, Alton HM, Chapman S. Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice. Pediatr Radiol 1998;28, 937–941.

    Article  CAS  PubMed  Google Scholar 

  7. Gerhold JP, Klingensmith WC, 3rd, Kuni CC et al. Diagnosis of biliary atresia with radionuclide hepatobiliary imaging. Radiology 1983;146, 499–504.

    CAS  PubMed  Google Scholar 

  8. Guibaud L, Lachaud A, Touraine R et al. MR cholangiography in neonates and infants: Feasibility and preliminary applications. AJR Am J Roentgenol 1998;170, 27–31.

    CAS  PubMed  Google Scholar 

  9. Norton KI, Glass RB, Kogan D, Lee JS, Emre S, Shneider BL. MR cholangiography in the evaluation of neonatal cholestasis: Initial results. Radiology 2002;222, 687–691.

    Article  PubMed  Google Scholar 

  10. Han SJ, Kim MJ, Han A et al. Magnetic resonance cholangiography for the diagnosis of biliary atresia. J Pediatr Surg 2002;37, 599–604.

    Article  PubMed  Google Scholar 

  11. Ryeom HK, Choe BH, Kim JY et al. Biliary atresia: Feasibility of mangafodipir trisodium-enhanced MR cholangiography for evaluation. Radiology 2005;235, 250–258.

    Article  PubMed  Google Scholar 

  12. Vogl TJ, Kummel S, Hammerstingl R et al. Liver tumors: Comparison of MR imaging with Gd-EOB-DTPA and Gd-DTPA. Radiology 1996;200, 59–67.

    CAS  PubMed  Google Scholar 

  13. Halavaara J, Breuer J, Ayuso C et al. Liver tumor characterization: Comparison between liver-specific gadoxetic acid disodium-enhanced MRI and biphasic CT–a multicenter trial. J Comput Assist Tomogr 2006;30, 345–354.

    Article  PubMed  Google Scholar 

  14. Zech CJ, Herrmann KA, Reiser MF, Schoenberg SO. MR imaging in patients with suspected liver metastases: Value of liver-specific contrast agent Gd-EOB-DTPA. Magn Reson Med Sci 2007;6, 43–52.

    Article  PubMed  Google Scholar 

  15. Lonergan GJ, Rice RR, Suarez ES. Autosomal recessive polycystic kidney disease: Radiologic-pathologic correlation. Radiographics 2000;20, 837–855.

    CAS  PubMed  Google Scholar 

  16. Zeitoun D, Brancatelli G, Colombat M, Federle MP. Congenital hepatic fibrosis: CT findings in 18 adults. Radiology 2004;231, 109–116.

    Article  PubMed  Google Scholar 

  17. Jung G, Benz-Bohm G, Kugel H, Keller K-M, Querfeld U. MR cholangiography in children with autosomal recessive polycystic kidney disease. Pediatr Radiol 1999;29, 463–466.

    Article  CAS  PubMed  Google Scholar 

  18. Ernst O, Gottrand F, Calvo M et al. Congenital hepatic fibrosis: Findings at MR cholangiopancreatography. AJR Am J Roentgenol 1998;170, 409–412.

    CAS  PubMed  Google Scholar 

  19. Mathieu D, Vilgrain V, Mahfouz AE, Anglade MC, Vullierme MP, Denys A. Benign liver tumors. Magn Reson Imaging Clin N Am 1997;5, 255–288.

    CAS  PubMed  Google Scholar 

  20. Gaines PA, Sampson MA. The prevalence and characterization of simple hepatic cysts by ultrasound examination. Br J Radiol 1989;62, 335–337.

    Article  CAS  PubMed  Google Scholar 

  21. Caremani M, Vincenti A, Benci A, Sassoli S, Tacconi D. Ecographic epidemiology of non-parasitic hepatic cysts. J Clin Ultrasound 1993;21, 115–118.

    Article  CAS  PubMed  Google Scholar 

  22. Larssen TB, Rorvik J, Hoff SR, Horn A, Rosendahl K. The occurrence of asymptomatic and symptomatic simple hepatic cysts. A prospective, hospital-based study. Clin Radiol 2005;60, 1026–1029.

    Article  CAS  PubMed  Google Scholar 

  23. Gunay-Aygun M, Avner ED, Bacallao RL, Choyke PL, Flynn JT. Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: Summary statement of a first national institutes of health/office of rare diseases conference. J Pediatr 2006;149, 159–164.

    Article  PubMed  Google Scholar 

  24. Liang P, Cao B, Wang Y, Yu X, Yu D, Dong B. Differential diagnosis of hepatic cystic lesions with gray-scale and color Doppler sonography. J Clin Ultrasound 2005;33, 100–105.

    Article  PubMed  Google Scholar 

  25. Mortele B, 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 Tomogr 2002;26, 438–443.

    Article  PubMed  Google Scholar 

  26. Brancatelli G, Federle MP, Vilgrain V, Vullierme M-P, Marin D, Lagalla R. Fibropolycystic liver disease: CT and MR imaging findings. Radiographics 2005;25, 659–670.

    Article  PubMed  Google Scholar 

  27. Murphy BJ, Casillas J, Ros PR, Morillo G, Albores-Saavedra J, Rolfes DB. The CT appearance of cystic masses of the liver. Radiographics 1989;9, 307–322.

    CAS  PubMed  Google Scholar 

  28. Semelka RC, Hussain SM, Marcos HB, Woosley JT. Biliary hamartomas: Solitary and multiple lesions shown on current MR techniques including gadolinium enhancement. J Magn Reson Imaging 1999;10, 196–201.

    Article  CAS  PubMed  Google Scholar 

  29. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 1977;134, 263–269.

    Article  CAS  PubMed  Google Scholar 

  30. Krause D, Cercueil JP, Dranssart M, Cognet F, Piard F, Hillon P. MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr 2002;26, 541–552.

    Article  PubMed  Google Scholar 

  31. Orii T, Ohkohchi N, Sasaki K, Satomi S, Watanabe M, Moriya T. Cholangiocarcinoma arising from preexisting biliary hamartoma of liver – report of a case. Hepatogastroenterology 2003;50, 333–336.

    Google Scholar 

  32. Irie H, Honda H, Jimi M et al. Value of MR cholangiopancreatography in evaluating choledochal cysts. Am J Roentgenol 1998;171, 1381–1385.

    CAS  Google Scholar 

  33. Sherlock DS. Diseases of the liver and biliary system, 6th edn. Oxford, London: Blackwell Scientific Publications, 1981.

    Google Scholar 

  34. Lao OB, Stein S, Ely KA, Lovvorn HN, 3rd. Synchronous Todani types I and III choledochal cysts in a 10-month-old-infant: Type IVb. Pediatr Surg Int 2008;24, 859–862.

    Article  PubMed  Google Scholar 

  35. Desmet VJ. Ludwig symposium on biliary disorders–part I. Pathogenesis of ductal plate abnormalities. Mayo Clin Proc 1998;73, 80–89.

    Article  CAS  PubMed  Google Scholar 

  36. Levy AD, Rohrmann CA, Jr., Murakata LA, Lonergan GJ. Caroli’s disease: Radiologic spectrum with pathologic correlation. AJR Am J Roentgenol 2002;179, 1053–1057.

    PubMed  Google Scholar 

  37. Choi BI, Yeon KM, Kim SH, Han MC. Caroli disease: Central dot sign in CT. Radiology 1990;174, 161–163.

    CAS  PubMed  Google Scholar 

  38. Miller WJ, Sechtin AG, Campbell WL, Pieters PC. Imaging findings in Caroli’s disease. Am J Roentgenol 1995;165, 333–337.

    CAS  Google Scholar 

  39. Bloustein PA. Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 1977;67, 40–46.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, University of Washington, Seattle, WA, USA

    Orpheus Kolokythas MD

  2. Department of Radiology, Seattle Children’s Hospital, Seattle, WA, USA

    Grace Phillips MD

Authors
  1. Orpheus Kolokythas MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Grace Phillips MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. School of Medicine, Children's Hospital & Regional, University of Washington, Sand Point Way NE. 4800, Seattle, 98105, USA

    Karen F. Murray

  2. St. Paul University Hospital, Harry Hines Blvd. 5939, Dallas, 75390-9258, USA

    Anne M. Larson

Rights and permissions

Reprints and permissions

Copyright information

© 2010 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Kolokythas, O., Phillips, G. (2010). Radiologic Findings in the Fibrocystic Diseases. In: Murray, K., Larson, A. (eds) Fibrocystic Diseases of the Liver. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-60327-524-8_5

Download citation

  • DOI: https://doi.org/10.1007/978-1-60327-524-8_5

  • Published:

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-60327-523-1

  • Online ISBN: 978-1-60327-524-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation