Skip to main content
SpringerLink
Log in

Value of carbon dioxide wedged venography and transvenous liver biopsy in the definitive diagnosis of Abernethy malformation

  • Published:
Abdominal Imaging Aims and scope Submit manuscript

Abstract

We report a 25-year-old man who presented with congenital absence of the portal vein, or Abernethy malformation, a rare congenital disorder in which the mesenteric and splenic venous drainages bypass the liver and directly drain into the inferior vena cava through an extrahepatic portosystemic shunt. Magnetic resonance imaging, which showed multiple nodular lesions in both liver lobes that were associated with an absence of intrahepatic portal venous branches, strongly suggested the diagnosis of the Abernethy malformation. Carbon dioxide wedged venography and transvenous liver biopsy, which were performed in the same session by a right jugular approach, confirmed these findings. This technique can be considered a valuable alternative diagnostic tool to catheter arteriography and percutaneous transhepatic liver biopsy.

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.

Institutional subscriptions

Figure 1
Figure 2
Figure 3
Figure 4

Similar content being viewed by others

References

  1. Badler R, Price AP, Moy L, Katz DS. Congenital portacaval shunt: CT demonstration. Pediatr Radiol 2002;32:28–30

    Article  PubMed  Google Scholar 

  2. Niwa T, Aida N, Tachibana K, et al. Congenital absence of the portal vein: clinical and radiologic findings. J Comput Assist Tomogr 2002;26:681–686

    Article  PubMed  Google Scholar 

  3. Pichon N, Maisonnette F, Pichon-Lefièvre F, et al. Hepatocarcinoma with congenital agenesis of the portal vein. JJCO 2003;33:314–316

    Google Scholar 

  4. Kohda E, Saeki M, Nakano M, et al. Congenital absence of the portal vein in a boy. Pediatr Radiol 1999;29:235–237

    Article  CAS  PubMed  Google Scholar 

  5. Tanaka Y, Takayanagi M, Shiratori Y, et al. Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver. J Gastroenterol 2003;38:288–294

    Article  PubMed  Google Scholar 

  6. Morgan G, Superina R. Congenital absence of the portal vein and a proposed classification for portosystemic vascular anomalies. J Pediatr Surg 1994;29:1239–1241

    Article  CAS  PubMed  Google Scholar 

  7. Abernethy J. Account of two instances of uncommon formation in the viscera of the human body. Phil Trans R Soc Lond B Biol Sci 1793;83:295–299

    Google Scholar 

  8. Murray CP, Yoo S-J, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 2003;33:614–620

    Article  PubMed  Google Scholar 

  9. Marks C. Development of the portal venous system. Am J Surg 1969;117:671–681

    Article  CAS  PubMed  Google Scholar 

  10. Massin M, Verloes A, Jamblin P. Cardiac anomalies associated with congenital absence of the portal vein. Cardiol Young 1999;9:522–525

    CAS  PubMed  Google Scholar 

  11. Kanamori Y, Hashizume K, Kitano Y, et al. Congenital extrahepatic portocaval shunt (Abernethy type 2), huge liver mass, and patent ductus arteriosus—a case report of its rare clinical presentation in a young girl. J Pediatr Surg 2003;38:E15

    Article  PubMed  Google Scholar 

  12. Karrer FM, Hall RJ, Lilly JR. Biliary atresia and the polysplenia syndrome. J Pediatr Surg 1991;26:524–527

    Article  CAS  PubMed  Google Scholar 

  13. Altavilla G, Guariso G. Focal nodular hyperplasia of the liver associated with portal vein agenesis: a morphological and immunohistochemical study of one case and review of the literature. Adv Clin Pathol 1999;3:139–145

    CAS  Google Scholar 

  14. Motoori S, Shinozaki M, Goto N, Kondo F. Congenital absence of the portal vein associated with nodular hyperplasia in the liver. J Gastroenterol Hepatol 1997;12:639–643

    CAS  PubMed  Google Scholar 

  15. Matsuoka Y, Ohtomo K, Okubo T, et al. Congenital absence of the portal vein. Gastrointest Radiol 1992;17:31–33

    Article  CAS  PubMed  Google Scholar 

  16. DeGaetano AM, Gui B, Macis G, et al. Congenital absence of the portal vein associated with focal nodular hyperplasia in the liver in an adult woman: imaging and review of the literature. Abdom Imaging 2004;29:455–459

    CAS  Google Scholar 

  17. Bellah RD, Hayek J, Teele RL (1989) Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr Radiol 20:115–117

    Article  CAS  PubMed  Google Scholar 

  18. Debernardi-Venon W, Bandi J-C, Garcia-Pagan J-C, et al. CO2 wedged hepatic venography in the evaluation of portal hypertension. Gut 2000;46:856–860

    Article  CAS  PubMed  Google Scholar 

  19. Krajina A, Lojik M, Chovanec V, et al. Wedged hepatic venography for targeting the portal vein during TIPS: comparison of carbon dioxide and iodinated contrast agents. Cardiovasc Intervent Radiol 2002;25:171–175

    Article  PubMed  Google Scholar 

  20. Martinez-Cuesta A, Elduayen B, Vivas I, et al. CO2 wedged hepatic venography: technical considerations and comparison with direct and indirect portography with iodinated contrast. Abdom Imaging 2000;25:576–582

    Article  CAS  PubMed  Google Scholar 

  21. Shinkai M, Ohhama Y, Nishi T, et al. Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 2001;36:1026–1031

    Article  CAS  PubMed  Google Scholar 

  22. Ikeda S, Sera Y, Ohshiro H, et al. Surgical indications for patients with hyperammonemia. J Pediatr Surg 1999;34:1012–1015

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, University Hospital Gasthuisberg, Leuven, Belgium

    B. Collard, G. Maleux, S. Heye & D. Bielen

  2. Department of Hepatology, University Hospital Gasthuisberg, Leuven, Belgium

    M. Cool & W. Van Steenbergen

  3. Department of Gastroenterology, Groeninghe Hospital, Kortrijk, Belgium

    C. George

  4. Department of Pathology, University Hospital Gasthuisberg, Leuven, Belgium

    T. Roskams

Authors
  1. B. Collard
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. G. Maleux
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. S. Heye
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M. Cool
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. D. Bielen
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. C. George
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. T. Roskams
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. W. Van Steenbergen
    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

Collard, B., Maleux, G., Heye, S. et al. Value of carbon dioxide wedged venography and transvenous liver biopsy in the definitive diagnosis of Abernethy malformation. Abdom Imaging 31, 315–319 (2006). https://doi.org/10.1007/s00261-005-0151-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00261-005-0151-9

Keywords

Search

Navigation