Skip to main content

Advertisement

Log in

Clinical features of congenital portosystemic shunt in children

  • Original Paper
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Clinical features, images, complications, treatments, and prognosis of 10 children with congenital portosystemic shunt (CPSS) were reviewed. Nine children were diagnosed with intrahepatic shunts while one presented with extrahepatic shunt. CPSS was detected by prenatal ultrasonography in four infants. Three infants presented with galactosemia without an enzyme deficiency. Two children presented with mental retardation and attention deficit hyperactivity disorder. Pulmonary hypertension developed in two patients. Spontaneous closure occurred in four infants with intrahepatic shunts including patent ductus venosus. The shunts were closed using transcatheter embolizations in four patients with intrahepatic shunts. Conclusion: Intrahepatic shunts may close spontaneously. Transcatheter embolization is effective for the treatment of symptomatic intrahepatic shunts.

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

Access this article

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Delle Chiaie L, Neuberger P, Von Kalle T (2008) Congenital intrahepatic portosystemic shunt: prenatal diagnosis and possible influence on fetal growth. Ultrasound Obstet Gynecol 32:233–235

    Article  PubMed  CAS  Google Scholar 

  2. Evans WN, Galindo A, Acherman RJ, Rothman A, Berthoty DP (2009) Congenital portosystemic shunts and AMPLATZER vascular plug occlusion in newborns. Pediatr Cardiol 30:1083–1088

    Article  PubMed  Google Scholar 

  3. Ferrero GB, Porta F, Biamino E, Mussa A, Garelli E, Chiappe F, Veltri A, Silengo MC, Gennari F (2010) Remittent hyperammonemia in congenital portosystemic shunt. Eur J Pediatr 169:369–372

    Article  PubMed  Google Scholar 

  4. Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, Riou JY, Pariente D, Gauthier F, Jacquemin E, Bernard O (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–330

    PubMed  Google Scholar 

  5. Gitzelmann R, Forster I, Willi UV (1997) Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt. Eur J Pediatr 156:719–722

    Article  PubMed  CAS  Google Scholar 

  6. Kamata S, Kitayama Y, Usui N, Kuroda S, Nose K, Sawai T, Okada A (2000) Patent ductus venosus with a hypoplastic intrahepatic portal system presenting intrapulmonary shunt: a case treated with banding of the ductus venosus. J Pediatr Surg 35:655–657

    Article  PubMed  CAS  Google Scholar 

  7. Kono T, Hiki T, Kuwashima S, Hashimoto T, Kaji Y (2009) Hypergalactosemia in early infancy: diagnostic strategy with an emphasis on imaging. Pediatr Int 51:276–282

    Article  PubMed  Google Scholar 

  8. Konstas AA, Digumarthy SR, Avery LL, Wallace KL, Lisovsky M, Misdraji J, Hahn PF (2010) Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients. Eur J Radiol [Epub ahead of print]

  9. Lautz TB, Tantemsapya N, Rowell E, Superina RA (2011) Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 46:308–314

    Article  PubMed  Google Scholar 

  10. Nishimura Y, Tajima G, Dwi Bahagia A, Sakamoto A, Ono H, Sakura N, Naito K, Hamakawa M, Yoshii C, Kubota M, Kobayashi K, Saheki T (2004) Differential diagnosis of neonatal mild hypergalactosaemia detected by mass screening: clinical significance of portal vein imaging. J Inherit Metab Dis 27:11–18

    Article  PubMed  CAS  Google Scholar 

  11. Oguz B, Akata D, Balkanci F, Akhan O (2003) Intrahepatic portosystemic venous shunt: diagnosis by colour/power Doppler imaging and three-dimensional ultrasound. Br J Radiol 76:487–490

    Article  PubMed  CAS  Google Scholar 

  12. Ohno T, Muneuchi J, Ihara K, Yuge T, Kanaya Y, Yamaki S, Hara T (2008) Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 121:e892–899

    Article  PubMed  Google Scholar 

  13. Ono H, Mawatari H, Mizoguchi N, Eguchi T, Sakura N (1998) Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia. Acta Paediatr 87:631–634

    Article  PubMed  CAS  Google Scholar 

  14. Park JH, Cha SH, Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 155:527–528

    PubMed  CAS  Google Scholar 

  15. Sakura N, Mizoguchi N, Eguchi T, Ono H, Mawatari H, Naitou K, Ito K (1997) Elevated plasma bile acids in hypergalactosaemic neonates: a diagnostic clue to portosystemic shunts. Eur J Pediatr 156:716–718

    Article  PubMed  CAS  Google Scholar 

  16. Stringer MD (2008) The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 21:147–157

    Article  PubMed  Google Scholar 

  17. Takehara Y, Mori K, Edagawa T, Sugimoto M, Takehara H, Ito M, Kuroda Y (2004) Presumed hypoplastic intrahepatic portal system due to patent ductus venosus: importance of direct occlusion test of ductus venosus under open laparotomy. Pediatr Int 46:484–486

    Article  PubMed  Google Scholar 

  18. Tanoue S, Kiyosue H, Komatsu E, Hori Y, Maeda T, Mori H (2003) Symptomatic intrahepatic portosystemic venous shunt: embolization with an alternative approach. AJR Am J Roentgenol 181:71–78

    PubMed  Google Scholar 

  19. Uchino T, Matsuda I, Endo F (1999) The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 135:254–256

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This study was supported by grant no. 04–20071070 from the Seoul National University Hospital Research Fund.

Conflict of interest

There was no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jae Sung Ko.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Kim, M.J., Ko, J.S., Seo, J.K. et al. Clinical features of congenital portosystemic shunt in children. Eur J Pediatr 171, 395–400 (2012). https://doi.org/10.1007/s00431-011-1564-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-011-1564-9

Keywords

Navigation