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European Journal of Pediatrics

, Volume 175, Issue 2, pp 195–201 | Cite as

Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome

  • Lijun Fu
  • Qian Wang
  • Jinjin Wu
  • Ying Guo
  • Meirong Huang
  • Tingliang Liu
  • Qimin ChenEmail author
  • Fen LiEmail author
Original Article

Abstract

Congenital extrahepatic portosystemic shunt (CEPS) is a rare malformation of the mesenteric vasculature, which may lead to severe complications. In this report, we describe a case series of three children with type II CEPS (presenting as hypoxemia) and hepatopulmonary syndrome (HPS). The first patient was a 4-year-old male who did not receive any specific treatment and subsequently died of brain abscess 5 years after the diagnosis. The second patient was a 10-year-old female with a 5-year history of cyanosis and dyspnea on exertion. She had partial regression of hypoxemia and improved exercise tolerance at 8 months after a surgical shunt closure. The third patient was a 4-year-old male with a 3-year history of cyanosis and decreased exercise tolerance. He had full regression of hypoxemia at 3 months after a transcatheter shunt closure.

Conclusion: These results indicate that CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences. Closure of portosystemic shunts may result in resolution of HPS in type II CEPS and the length of period for resolution varies depending on the severity of HPS.

What is Known:

Congenital extrahepatic portosystemic shunt (CEPS) is a rare cause of hepatopulmonary syndrome (HPS).

There have been few reports in the literature about the management and outcome of HPS in children with CEPS.

What is New:

CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences.

Closure of portosystemic shunts may result in resolution of HPS in type II CEPS.

Keywords

Abernethy malformation Congenital extrahepatic portosystemic shunt (CEPS) hepatopulmonary syndrome (HPS) Portosystemic shunt 

Abbreviations

APTT

Activated partial thromboplastin time

CEPS

Congenital extrahepatic portosystemic shunt

CT

Computed tomography

HPS

Hepatopulmonary syndrome

INR

International normalized ratio

IVC

Inferior vena cava

MPV

Main portal vein

Notes

Acknowledgments

The research was supported by the Medical Guidance Project of Shanghai Science and Technology Commission (No. 14411965300).

Compliance with ethical standards

Funding

The research was supported by Medical Guidance Project of Shanghai Science and Technology Commission (No. 14411965300).

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

Contribution of each co-authors

Conceived and designed the experiments: Q.C., F.L. Performed the experiments: L.F., Q.W., J.W. Analyzed the data: Y.G., M.H. Contributed reagents/materials/analysis tools: T.L. Wrote the paper: L.F.

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of Cardiology, Shanghai Children’s Medical CenterShanghai Jiao Tong University School of MedicineShanghaiChina
  2. 2.Department of Radiology, Shanghai Children’s Medical CenterShanghai Jiao Tong University School of MedicineShanghaiChina
  3. 3.Department of Surgery, Shanghai Children’s Medical CenterShanghai Jiao Tong University School of MedicineShanghaiChina

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