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Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia

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Abstract

Background

Heterotaxy with polysplenia is associated with many cardiovascular anomalies including the occasional occurrence of congenital extrahepatic portosystemic shunts (CEPS). Missing this anomaly can lead to inappropriate and ineffective therapy.

Objective

To emphasize the importance and associated anatomy of CEPS in conjunction with heterotaxy with polysplenia.

Materials and methods

Review of three young children who presented with cyanosis and pulmonary hypertension without a cardiac etiology. They were known (1) or discovered (2) to have heterotaxy with polysplenia.

Results

There was absence of the intrahepatic inferior vena cava (IVC) with azygos or hemiazygos continuation in all three cases. In spite of normal liver function, they were discovered to have large portosystemic shunts, splenorenal in location, along with diffuse peripheral pulmonary arterial dilatation suggestive of CEPS (Abernethy malformation) with hepatopulmonary or, more accurately, portopulmonary syndrome. All CEPS were ipsilateral to the spleens. Patency of the portal veins in these cases allowed for percutaneous shunt closure with resolution of cyanosis.

Conclusion

CEPS is associated with heterotaxy with polysplenia and can be symptomatic because of pulmonary arteriovenous (AV) shunting. Portal and hepatic vein patency are critical for determining feasibility of CEPS closure.

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Authors and Affiliations

  1. Department of Radiology, Stanford University School of Medicine, Lucile Packard Children’s Hospital, 725 Welch Road, Stanford, CA, 94305, USA

    Beverley Newman

  2. Division of Pediatric Cardiology, Department of Pediatrics, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Stanford, CA, USA

    Jeffrey A. Feinstein

  3. Department of Diagnostic Radiology, Children’s Hospital and Research Center, Oakland, CA, USA

    Ronald A. Cohen & Hitendra Patel

  4. Department of Pediatrics, Division of Pediatric Cardiology, Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA

    Brian Feingold & Jacqueline Kreutzer

  5. Cardiovascular Imaging Section, Department of Radiology, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Stanford, CA, USA

    Fandics P. Chan

Authors
  1. Beverley Newman
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  2. Jeffrey A. Feinstein
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  3. Ronald A. Cohen
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  4. Brian Feingold
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  5. Jacqueline Kreutzer
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  6. Hitendra Patel
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  7. Fandics P. Chan
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Corresponding author

Correspondence to Beverley Newman.

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Newman, B., Feinstein, J.A., Cohen, R.A. et al. Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia. Pediatr Radiol 40, 1222–1230 (2010). https://doi.org/10.1007/s00247-009-1508-y

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  • DOI: https://doi.org/10.1007/s00247-009-1508-y

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