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Pediatric Radiology

Published in:

01-09-2003 | Original Article

Congenital extrahepatic portosystemic shunts

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

Published in: Pediatric Radiology | Issue 9/2003

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.

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Title: Congenital extrahepatic portosystemic shunts
Authors: Conor P. Murray
Shi-Joon Yoo
Paul S. Babyn
Publication date: 01-09-2003
Publisher: Springer-Verlag
Published in: Pediatric Radiology / Issue 9/2003
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-003-1002-x

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Congenital extrahepatic portosystemic shunts

Abstract

Background

Objective

Materials and methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Objective

Materials and methods

Results

Conclusion

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