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Published in: Pediatric Radiology 7/2010

01-07-2010 | Original Article

Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia

Authors: Beverley Newman, Jeffrey A. Feinstein, Ronald A. Cohen, Brian Feingold, Jacqueline Kreutzer, Hitendra Patel, Fandics P. Chan

Published in: Pediatric Radiology | Issue 7/2010

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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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Metadata
Title
Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia
Authors
Beverley Newman
Jeffrey A. Feinstein
Ronald A. Cohen
Brian Feingold
Jacqueline Kreutzer
Hitendra Patel
Fandics P. Chan
Publication date
01-07-2010
Publisher
Springer-Verlag
Published in
Pediatric Radiology / Issue 7/2010
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-009-1508-y

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