Skip to main content
SpringerLink
Log in

Congenital extrahepatic portosystemic shunts

  • Original Article
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

Background

A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy.

Objective

To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature.

Materials and methods

The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed.

Results

Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4).

Conclusion

MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested.

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

Fig. 1a–e.
Fig. 2a–c.
Fig. 3a–c.

Similar content being viewed by others

References

  1. Marois D, van Heerden JA, Carpenter HA, et al (1979) Congenital absence of the portal vein. Mayo Clin Proc 54:55–59

    CAS  PubMed  Google Scholar 

  2. Laverdiere JT, Laor T, Benacerraf B (1995) Congenital absence of the portal vein: case report and MR demonstration. Pediatr Radiol 25:52–53

    CAS  PubMed  Google Scholar 

  3. Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portosystemic vascular anomalies. J Pediatr Surg 29:1239–1241

    CAS  PubMed  Google Scholar 

  4. Bellah R, Hayek J, Littlewood R (1989) Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr Radiol 20:115–117

    CAS  PubMed  Google Scholar 

  5. Yonemitsu H, Mori H, Kimura T, et al (2000) Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin-Johnson syndrome. Abdom Imaging 25:572–575

    Article  CAS  PubMed  Google Scholar 

  6. Abernethy J (1793) Account of two instances of the uncommon formation of the viscera in the human body. Phil Trans B83:59–66

    Google Scholar 

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

    CAS  PubMed  Google Scholar 

  8. Kitagawa S, Gleason WA, Northrup H, et al (1992) Symptomatic hyperammonemia caused by a congenital portosystemic shunt. J Pediatr 121:917–919

    CAS  PubMed  Google Scholar 

  9. Mizoguchi N, Sakura N, Ono H, et al (2001) Congenital porto-left renal venous shunt as a cause of galactosemia. J Inherit Metab Dis 24:72–78

    Article  CAS  PubMed  Google Scholar 

  10. Woodle ES, Thistlethwaite JR, Emond JC, et al (1990) Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery 107:475–479.

    CAS  PubMed  Google Scholar 

  11. Nakasaki H, Tanaka Y, Ohta M, et al (1989) Congenital absence of the portal vein. Ann Surg 210:190–193

    CAS  PubMed  Google Scholar 

  12. Howard ER, Davenport M (1997) Congenital extrahepatic portocaval shunts-The Abernethy malformation. J Pediatr Surg 32:494–497

    CAS  PubMed  Google Scholar 

  13. Joyce AD, Howard ER (1988) Rare congenital anomaly of the portal vein. Br J Surg 75:1038–1039

    CAS  PubMed  Google Scholar 

  14. Ikeda S, Sera Y, Ohshiro H, et al (1999) Surgical indications for patents with hyperammonemia. J Ped Surg 34:1012–1015

    CAS  Google Scholar 

  15. Olling S, Olsson R (1974) Congenital absence of portal venous system in a 50-year-old woman. Acta Med Scand 196:343–345

    CAS  PubMed  Google Scholar 

  16. Morse SS, Taylor KJW, Strauss EB, et al (1986) Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome). Pediatr Radiol 16:437–439

    CAS  PubMed  Google Scholar 

  17. Okuda Y, Ono M, Kato N, et al (1988) Congenital absence of the portal vein in a 17-year-old woman. Nippon Igaku Hoshasen Gakkai Zasshi 48:86

    Google Scholar 

  18. Guariso G, Fiorio S, Altavilla G, et al (1998) Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney. Eur J Pediatr 157:287–290

    Article  CAS  PubMed  Google Scholar 

  19. Wakomoto H, Kohji M, Hitoshi K, et al (1999) Subclinical portal-systemic encephalopathy en a child with congenital absence of the portal vein. Brain Dev 21:425–428

    Article  PubMed  Google Scholar 

  20. Kinjo T, Aoki H, Sunagawa H, et al (2001) Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst: a case report. J Pediatr Surg 36:622–625

    Article  CAS  PubMed  Google Scholar 

  21. Grazioli L, Alberti D, Olivetti L, et al (2000) Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 10:820–825

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  23. Arana E, Marti-Bonmati L, Martinez V, et al (1997) Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom Imaging 22:506–508

    Article  CAS  PubMed  Google Scholar 

  24. Motoori S, Shinozaki M, Goto N, et al (1997) Case report: congenital absence of the portal vein associated with nodular hyperplasia in the liver. J Gastroenterol Hepatol 12:639–643

    CAS  PubMed  Google Scholar 

  25. Matsuoka Y, Ohtomo K, Okubo T, Nishikawa J, et al (1992) Congenital absence of the portal vein. Gastrointest Radiol 17:31–33

    CAS  PubMed  Google Scholar 

  26. Hellweg G (1954) Congenital absence of intrahepatic portal venous system simulating Eck fistula. Report of a case with necropsy findings. AMA Arch Path 57:425–430

    Google Scholar 

  27. Shah R, Ford EG, Woolley MM (1992) Congenital portocaval shunt. Case report. Pediatr Surg Int 7:216–217

    Google Scholar 

  28. Yoshidome H, Edwards MJ (1999) An embryological perspective on congenital portocaval shunt: a rare anomaly in a patient with hepatocellular carcinoma. Am J Gastroenterol 94:2537–2539

    Article  CAS  PubMed  Google Scholar 

  29. Usuki N, Miyamoto T (1998) A case of congenital absence of the intrahepatic portal vein diagnosed by MR angiography. J Comput Assist Tomogr 22:728–729

    Article  CAS  PubMed  Google Scholar 

  30. Ohwada S, Hamada Y, Morishita Y, et al (1994) Hepatic encephalopathy due to congenital splenorenal shunts: report of a case. Surg Today 24:145–149

    CAS  PubMed  Google Scholar 

  31. Kerlan RK, Sollenberger RD, Palubinskas AJ, et al (1982) Portal-systemic encephalopathy due to a congenital portocaval shunt. Am J Roentgenol 139:1013–1015

    Google Scholar 

  32. Tanano H, Hasegawa T, Kawahara H, et al (1999) Biliary atresia associated with congenital structural anomalies. J Pediatr Surg 34:1687–1690

    CAS  PubMed  Google Scholar 

  33. Kalifa G, Brunelle F, Chaumont P (1978) Fistule port-cave congenitale. Ann Radiol 21:183–186

    CAS  PubMed  Google Scholar 

  34. Le Borgne J, Paineau J, Hamy A, et al (2000) Interruption of the inferior vena cava with azygous continuation associated with congenital absence of portal vein. Surg Radiol Anat 22:197–202

    PubMed  Google Scholar 

  35. Ji EK, Yoo SJ, Kim JH, et al (1999) Congenital splenorenal venous shunt detected by prenatal ultrasonography. J Ultrasound Med 18:437–439

    CAS  PubMed  Google Scholar 

  36. Kiernan F (1833) The anatomy and physiology of the liver. Phil Trans R Soc 113:711–760

    Google Scholar 

  37. Komatsu S, Nagino M, Hayakawa N, et al (1995) Congenital absence of portal venous system associated with a large inferior mesenteric-caval shunt: a case report. Hepatogastroenterology 42:286–290

    CAS  PubMed  Google Scholar 

  38. Badler R, Price AP, Moy L, et al (2002) Congenital portocaval shunt: CT demonstration. Pediatr Radiol 32:28–30

    Article  PubMed  Google Scholar 

  39. Kim SZ, Marz PL, Laor T, et al (1998) Elevated galactose in newborn screening due to congenital absence of the portal vein. Eur J Pediatr 157:608–609

    Article  CAS  PubMed  Google Scholar 

  40. Marks C (1969) Development of the portal venous system. Am J Surg 117:671–681

    CAS  PubMed  Google Scholar 

  41. Hunt GB, Bellenger CR, Borg R, et al(1998) Congenital interruption of the portal vein and caudal vena cava in dogs: six case reports and a review of the literature. Vet Surg 27:203–215

    CAS  PubMed  Google Scholar 

  42. Ewing GO, Suter PF, Bailey CS (1984) Hepatic insufficiency associated with congenital anomalies of the portal vein in dogs. J Am Anim Hosp Assoc 10:463–476

    Google Scholar 

  43. International Working Party (1995) Terminology of nodular hepatocellular lesions. Hepatology 22:983–993

    PubMed  Google Scholar 

  44. Siegelman ES, Outwater EK, Furth EE, et al (1995) MR imaging of hepatic nodular regenerative hyperplasia. J Mag Reson Imaging 5:730–732

    CAS  Google Scholar 

  45. Moore KL (1988) The developing human. Saunders, Toronto, pp 221–308

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

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada

    Conor P. Murray, Shi-Joon Yoo & Paul S. Babyn

Authors
  1. Conor P. Murray
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Shi-Joon Yoo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Paul S. Babyn
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Shi-Joon Yoo.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Murray, C.P., Yoo, SJ. & Babyn, P.S. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 33, 614–620 (2003). https://doi.org/10.1007/s00247-003-1002-x

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-003-1002-x

Keywords

Search

Navigation