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Published in: Pediatric Radiology 9/2003

01-09-2003 | Case Report

Biliary atresia and cerebellar hypoplasia in polysplenia syndrome

Authors: Kurt Vanderdood, Bart Op de Beeck, Brigitte Desprechins, Michel Osteaux

Published in: Pediatric Radiology | Issue 9/2003

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Abstract

We report a 3.5-month-old boy with polysplenia syndrome who demonstrated hemiazygos continuation of the inferior vena cava, extrahepatic biliary atresia, multiple splenunculi, bowel malrotation, and the rare finding of brainstem and cerebellar hypoplasia. A possible pathogenesis for cerebellar hypoplasia in this syndrome is suggested after review of the literature. The importance of seeking associated anomalies in biliary atresia, which may be possible indicators of polysplenia syndrome, is stressed since these patients need appropriate management when surgery is considered.
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Metadata
Title
Biliary atresia and cerebellar hypoplasia in polysplenia syndrome
Authors
Kurt Vanderdood
Bart Op de Beeck
Brigitte Desprechins
Michel Osteaux
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/s00247-003-0961-2

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