Skip to main content
SpringerLink
Log in

Portal cavernoma in congenital hepatic fibrosis

Angiographic reports of 10 pediatric cases

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

Abstract

A retrospective pediatric study of 30 angiograms in congenital hepatic fibrosis (CHF) was undertaken to analyze the different aspects of the portal venous system in this disease. Besides the classical angiographic signs (hepatofugal veins and intrahepatic duplications), a hepatopetal venous network was present in ten children, contrasting with the patency of the portal venous system. Analysis of the medical charts for these ten patients showed that the clinical signs and outcome were similar to classical CHF. Liver biopsies were available in six cases and, in addition to the diagnostic features of CHF, demonstrated paucity or absence of portal venules. This unusual association of hepatopetal and hepatofugal collateral veins in an intrahepatic block could be explained by hypoplasia or thrombosis of portal venules. Thus, the association between hepatomegaly and hepatopetal collateral veins without portal obstruction should be suggestive of CHF.

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.

Similar content being viewed by others

Article 21 February 2019

Pooja Abbey, Devasenathipathy Kandasamy & Priyanka Naranje

References

  1. Alvarez F, Bernard O, Brunelle F, Hadchouel M, Leblanc A, Odievre M, Alagille D (1981) Congenital hepatic fibrosis in children. J Pediatr 99: 370–375

    PubMed  Google Scholar 

  2. Daroca P, Tuthill R (1975) Cholangiocarcinoma arising in congenital hepatic fibrosis. Arch Pathol 99: 592–595

    PubMed  Google Scholar 

  3. Alvarez F, Bernard O, Brunelle F, Hadchouel P, Alagille D (1985) Portal hypertension in children. Clin Gastroenterol 14: 33–55

    PubMed  Google Scholar 

  4. Cardella JF, Amplatz K (1987) Preoperative angiographic evaluation of prospective liver recipients. Radiol Clin North Am 25: 299–308

    PubMed  Google Scholar 

  5. Odièvre M, Chaumont P, Montagne JP, Alagille D (1977) Anomalies of the intrahepatic portal venous system in congenital hepatic fibrosis. Radiology 122: 427–430

    PubMed  Google Scholar 

  6. Markov BA, Taichibakov Sh M (1985) Radiodiagnosis of congenital hepatic fibrosis in childhood (in Russian) Vestn Roentgenol Radiol 4: 17–21

    Google Scholar 

  7. Tewarson IP, Crowe CP, Verhagen AD (1964) Congenital hepatic fibrosis: a case with portal hypertension and gastroesophageal bleeding requiring an unusual surgical treatment. Clin Pediatr 3: 679–683

    Google Scholar 

  8. Laing IA, Buist TAS, Fraser MS (1981) Percutaneous transhepatic occlusion for bleeding oesophageal varices in polycystic disease. Arch Dis Child 56: 954–963

    PubMed  Google Scholar 

  9. Thaler MM, Ogata ES, Goodman JR, Piel CF, Korobkin MT (1973) Congenital fibrosis and polycystic disease of liver and kidneys. Am J Dis Child 126: 374–380

    PubMed  Google Scholar 

  10. Albukerk J, Duffy JL (1971) Fibrogenesis in congenital hepatic fibrosis: an electron and light microscopic study. Arch Pathol 92: 126–135

    PubMed  Google Scholar 

  11. Kerr DNS, Harrison CV, Sherlock S, et al (1961) Congenital hepatic fibrosis. Q J Med 117: 91–117

    Google Scholar 

  12. Grumbach R, Bourillon J, Auvert J (1954) Maladie fibrocystique du foie avec hypertension portale chez l'enfant: deux observations. Semin Hop Paris 20: 1–9

    Google Scholar 

  13. Cho KJ, Lunderquist A (1983) The peribiliary vascular plexus: the microvascular architecture of the bile duct in the rabbit and in clinical cases. Radiology 147: 357–364

    PubMed  Google Scholar 

  14. Alvarez F, Bernard O, Hadchouel M, et al (1983) Portal obstruction in children: clinical investigations and hemorrhage risk. J Pediatr 103: 696–702

    PubMed  Google Scholar 

  15. Terada T, Ishida F, Nakanuma Y (1989) Vascular plexus around intra-hepatic bile ducts in normal livers and portal hypertension. J Hepatol 8: 139–149

    PubMed  Google Scholar 

  16. Andrade ZA, Cheever AW (1971) Alterations of the intrahepatic vasculature in hepatosplenicSchistosomas mansoni. Am J Trop Med Hyg 20: 425–432

    PubMed  Google Scholar 

  17. Mikkelsen WP, Edmonson HA, Peters RI, Redeker AG, Reynolds TB (1965) Extra-and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg 162: 602–620

    PubMed  Google Scholar 

  18. Carson JA, Tunnel WP, Barnes P, Altshuler G (1981) hepatoportal sclerosis in childhood: a mimic of extrahepatic portal vein obstruction. J Pediatr Surg 16: 291–296

    PubMed  Google Scholar 

  19. Sommerschild HC (1973) Congenital hepatic fibrosis: reports of two new cases and review of the literature. Surgery 1: 53–58

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Service de Radiopédiatrie, Hôpital de Bicêtre, 78, rue du Général Leclerc, F-94270, Kremlin-Bicêtre, France

    M. Besnard, D. Pariente & P. Chaumont

  2. INSERM U347, Bicêtre, France

    M. Hadchouel

  3. Service d'Hepatologie Pédiatrique, Kremlin-Bicêtre, France

    O. Bernard

Authors
  1. M. Besnard
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. D. Pariente
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Hadchouel
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. O. Bernard
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. P. Chaumont
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Besnard, M., Pariente, D., Hadchouel, M. et al. Portal cavernoma in congenital hepatic fibrosis. Pediatr Radiol 24, 61–65 (1994). https://doi.org/10.1007/BF02017666

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02017666

Keywords

Search

Navigation