Biliary atresia and cerebellar hypoplasia in polysplenia syndrome.
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.
Vanderdood
Kurt
K
Department of Radiology, Free University Brussels, AZ-VUB, Laarbeeklaan 101, 1090 Brussels, Belgium. kvanderd@yahoo.com
Op de Beeck
Bart
B
Desprechins
Brigitte
B
Osteaux
Michel
M
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001656:Biliary Atresia / Q000150:complications*; D002531:Cerebellum / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome; D014057:Tomography, X-Ray Computed