Erschienen in:
01.09.2003 | Case Report
Biliary atresia and cerebellar hypoplasia in polysplenia syndrome
verfasst von:
Kurt Vanderdood, Bart Op de Beeck, Brigitte Desprechins, Michel Osteaux
Erschienen in:
Pediatric Radiology
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Ausgabe 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.