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What is the shape of the fourth ventricle likened to?
Bat wing (similar to the Batman symbol).
Can you name some of the more common associations?
Ocular anomalies, multicystic dysplastic kidney (MCDK), polydactyly. Many other less common ones are also described.
Name two other conditions with hypoplastic / aplastic cerebellar vermis.
Dandy-Walker malformation, rhomboencephalosynapsis.
MRI of the brain demonstrates absence of the cerebellar vermis, with apposition (but no fusion) of the cerebellar hemispheres. The fourth ventricle has a bat-wing configuration, and elongated superior cerebellar peduncles giving the midbrain a characteristic molar-tooth shape.