Skip to main content

Advertisement

SpringerLink
Log in

Fibropolycystic liver disease in children

  • Review
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Fibropolycystic liver diseases are a group of associated congenital disorders that present most often in childhood. These disorders include congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic liver disease, choledochal cysts and Caroli disease. We present a discussion and illustrations of the embryology, genetics, anatomy, pathology, imaging approach and key imaging features that distinguish fibropolycystic liver disease in children. The pathogenesis of these disorders is believed to be abnormal development of the embryonic ductal plates, which ultimately form the liver and biliary systems. An understanding of the abnormal embryogenesis helps to explain the characteristic imaging features of these disorders.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12
Fig. 13
Fig. 14
Fig. 15
Fig. 16
Fig. 17
Fig. 18
Fig. 19
Fig. 20

Similar content being viewed by others

Article 21 February 2019

Pooja Abbey, Devasenathipathy Kandasamy & Priyanka Naranje

References

  1. Desmet VJ (1992) Congenital diseases of intrahepatic bile ducts: variations on the theme “ductal plate malformation”. Hepatology 16:1069–1083

    Article  PubMed  CAS  Google Scholar 

  2. Diehl-Jones WL, Askin DF (2002) The neonatal liver, Part 1: embryology, anatomy, and physiology. Neonatal Netw 21:5–12

    PubMed  Google Scholar 

  3. Lonergan GJ, Rice RR, Suarez ES (2000) Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation. Radiographics 20:837–855

    PubMed  CAS  Google Scholar 

  4. Jorgensen MJ (1977) The ductal plate malformation. Acta Pathol Microbiol Scand Suppl 257:1–87

    PubMed  Google Scholar 

  5. Krause D, Cercueil JP, Dranssart M et al (2002) MRI for evaluating congenital bile duct abnormalities. J Comput Assist Tomogr 26:541–552

    Article  PubMed  Google Scholar 

  6. Marchal GJ, Desmet VJ, Proesmans WC et al (1986) Caroli disease: high-frequency US and pathologic findings. Radiology 158:507–511

    PubMed  CAS  Google Scholar 

  7. Akhan O, Karaosmanoglu AD, Ergen B (2007) Imaging findings in congenital hepatic fibrosis. Eur J Radiol 61:18–24

    Article  PubMed  Google Scholar 

  8. Desmet VJ (2004) Pathogenesis of ductal plate malformation. J Gastroenterol Hepatol 19:S356–S360

    Article  Google Scholar 

  9. Brancatelli G, Federle MP, Vilgrain V et al (2005) Fibropolycystic liver disease: CT and MR imaging findings. Radiographics 25:659–670

    Article  PubMed  Google Scholar 

  10. Ernst O, Gottrand F, Calvo M et al (1998) Congenital hepatic fibrosis: findings at MR cholangiopancreatography. AJR 170:409–412

    PubMed  CAS  Google Scholar 

  11. Lowe LH, Schlesinger AE (2007) Hepatobiliary system, congenital abnormalities. In: Slovis TL (ed) Caffey’s pediatric diagnostic imaging, 11th edn. Mosby Elsevier, Philadelphia, pp 1861–1869

    Google Scholar 

  12. Nazer H, Nazer D (2006) Congenital hepatic fibrosis. eMedicine. Available via DIALOG. http://www.emedicine.com/ped/topic459.htm. Accessed 27 Nov 2008

  13. Zeitoun D, Brancatelli G, Colombat M et al (2004) Congenital hepatic fibrosis: CT findings in 18 adults. Radiology 231:109–116

    Article  PubMed  Google Scholar 

  14. Yonem O, Ozkayar N, Balkanci F et al (2006) Is congenital hepatic fibrosis a pure liver disease? Am J Gastroenterol 101:1253–1259

    Article  PubMed  Google Scholar 

  15. Karahan OI, Kahriman G, Soyuer I et al (2007) Hepatic von Meyenburg complex simulating biliary cystadenocarcinoma. Clin Imaging 31:50–53

    Article  PubMed  Google Scholar 

  16. Zheng RQ, Zhang B, Kudo M et al (2005) Imaging findings of biliary hamartomas. World J Gastroenterol 11:6354–6359

    PubMed  Google Scholar 

  17. Bistritz L, Tamboli C, Bigam D et al (2005) Polycystic liver disease: experience at a teaching hospital. Am J Gastroenterol 100:2212–2217

    Article  PubMed  Google Scholar 

  18. Jackson HH, Mulvihill SJ (2006) Hepatic cysts. eMedicine. Available via DIALOG. http://www.emedicine.com/med/topic2716.htm. Accessed 26 Nov 2008

  19. Morgan DE, Lockhart ME, Canon CL et al (2006) Polycystic liver disease: multimodality imaging for complications and transplant evaluation. Radiographics 26:1655–1668

    Article  PubMed  Google Scholar 

  20. Todani T, Watanabe Y, Narusue M et al (1977) Congenital bile duct cysts: classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg 134:263–269

    Article  PubMed  CAS  Google Scholar 

  21. Kim OH, Chung HJ, Choi BG (1995) Imaging of the choledochal cyst. Radiographics 15:69–88

    PubMed  CAS  Google Scholar 

  22. Wiedmeyer DA, Stewart ET, Dodds WJ et al (1989) Choledochal cyst: findings on cholangiopancreatography with emphasis on ectasia of the common channel. AJR 153:969–972

    PubMed  CAS  Google Scholar 

  23. Sawyer MAJ, Patel TH (2007) Choledochal cysts. eMedicine. Available via DIALOG. http://www.emedicine.com/med/topic349.htm. Accessed 26 Nov 2008

  24. Bloustein PA (1977) Association of carcinoma with congenital cystic conditions of the liver and bile ducts. Am J Gastroenterol 67:40–46

    PubMed  CAS  Google Scholar 

  25. Arcement CM, Towbin RB, Meza MP et al (2000) Intrahepatic chemoembolization in unresectable pediatric liver malignancies. Pediatr Radiol 30:779–785

    Article  PubMed  CAS  Google Scholar 

  26. Irie H, Honda H, Jimi M et al (1998) Value of MR cholangiopancreatography in evaluating choledochal cysts. AJR 171:1381–1385

    PubMed  CAS  Google Scholar 

  27. Vitellas KM, Keogan MT, Freed KS et al (2000) Radiologic manifestations of sclerosing cholangitis with emphasis on MR cholangiopancreatography. Radiographics 20:959–975

    PubMed  CAS  Google Scholar 

  28. Han BK, Babcock DS, Gelfand MH (1981) Choledochal cyst with bile duct dilatation: sonography and 99mTc IDA cholescintigraphy. AJR 136:1075–1079

    PubMed  CAS  Google Scholar 

  29. Toshihisa I, Shin’ichi S, Shigehisa F et al (2006) Ductal plate malformation of the liver in biliary atresia. Jpn J Pediatr Surg 38:1007–1010

    Google Scholar 

  30. Dillon P, Belchis D, Tracy T (1994) Increased expression of intercellular adhesion molecules in biliary atresia. Am J Pathol 145:263–267

    PubMed  CAS  Google Scholar 

  31. Arikan C, Berdeli A, Kilic M (2008) Polymorphisms of the ICAM-1 gene are associated with biliary atresia. Dig Dis Sci 53:2000–2004

    Article  PubMed  CAS  Google Scholar 

  32. Vejchapipat P, Jirapanakorn N, Thawornsuk N (2005) There is no association between K469E ICAM-1 gene polymorphism and biliary atresia. World J Gastroenterol 11:4886–4890

    PubMed  Google Scholar 

  33. Kirks DR, Coleman RE, Filston HC et al (1984) An imaging approach to persistent neonatal jaundice. AJR 142:461–465

    PubMed  CAS  Google Scholar 

  34. O’Neill JA Jr, Templeton JM Jr, Schnaufer L et al (1987) Recent experience with choledochal cyst. Ann Surg 205:533–540

    Article  PubMed  Google Scholar 

  35. Barlow B, Tabor E, Blanc WA et al (1976) Choledochal cyst: a review of 19 cases. J Pediatr 89:934–940

    Article  PubMed  CAS  Google Scholar 

  36. Babbitt DP, Starshak RJ, Clemet AR (1973) Choledochal cyst: a concept of etiology. AJR 119:57–63

    CAS  Google Scholar 

  37. Humphrey TM, Stringer MD (2007) Biliary atresia: US diagnosis. Radiology 244:845–851

    Article  PubMed  Google Scholar 

  38. Lee HJ, Lee SM, Park WH et al (2003) Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 229:395–400

    Article  Google Scholar 

  39. Papanicolaou N, Abramson SJ, Teele AL et al (1985) Specific preoperative diagnosis of choledochal cysts by combined sonography and hepatobiliary scintigraphy. Ann Radiol 28:276–282

    PubMed  CAS  Google Scholar 

  40. Freidman JR, Piccoli D, Baldassano R (2007) Caroli disease. eMedicine. Available via DIALOG. http://www.emedicine.com/ped/topic325.htm. Accessed 26 Nov 2008

  41. Toma P, Lucigrai G, Pelizza A (1991) Sonographic patterns of Caroli’s disease: report of 5 new cases. J Clin Ultrasound 19:155–161

    Article  PubMed  CAS  Google Scholar 

  42. Choi BI, Yeon KM, Kim SH et al (1990) Caroli disease: central dot sign in CT. Radiology 174:161–163

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Kansas City University of Medicine & Biosciences, Kansas City, MO, USA

    Myka Call Veigel

  2. Department of Radiology, University of Missouri-Kansas City, St. Luke’s Hospital, 4401 Wornall Road, Kansas City, MO, 64111, USA

    Myka Call Veigel, Julia Prescott-Focht & Reza Zinati

  3. University of Missouri-Kansas City School of Medicine, Kansas City, MO, USA

    Michael G. Rodriguez

  4. Department of Pathology, Children’s Mercy Hospitals and Clinics, Kansas City, MO, USA

    Lei Shao

  5. Department of Radiology, University of Missouri-Kansas City, 2401 Gillham Road, Kansas City, MO, 64108, USA

    Charlotte A. W. Moore & Lisa H. Lowe

  6. Department of Radiology, Children’s Mercy Hospitals and Clinics, Kansas City, MO, USA

    Charlotte A. W. Moore & Lisa H. Lowe

Authors
  1. Myka Call Veigel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Julia Prescott-Focht
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Michael G. Rodriguez
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Reza Zinati
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Lei Shao
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Charlotte A. W. Moore
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Lisa H. Lowe
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Myka Call Veigel.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Veigel, M.C., Prescott-Focht, J., Rodriguez, M.G. et al. Fibropolycystic liver disease in children. Pediatr Radiol 39, 317–327 (2009). https://doi.org/10.1007/s00247-008-1070-z

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-008-1070-z

Keywords

Search

Navigation