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- Title
Biliary atresia and cerebellar hypoplasia in polysplenia syndrome.
- Authors
Vanderdood, Kurt; Op de Beeck, Bart; Desprechins, Brigitte; Osteaux, Michel
- 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.
- Subjects
BILIARY atresia; BILE duct abnormalities; OBSTRUCTIONS of the bile ducts; JUVENILE diseases; VENA cava inferior; VENAE cavae; CEREBELLUM abnormalities; MULTIPLE human abnormalities; COMPUTED tomography; MAGNETIC resonance imaging; SPLEEN; SYNDROMES; DISEASE complications; DIAGNOSIS
- Publication
Pediatric Radiology, 2003, Vol 33, Issue 9, p652
- ISSN
0301-0449
- Publication type
journal article
- DOI
10.1007/s00247-003-0961-2