Ataxia and developmental delay.
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4th ventricle has "bat wing" configuration (umbrella shaped) Thickened superior cerebellar peduncles around the elongated 4th ventricle forms the classic "molar tooth". The vermis is small and dysplastic. Cleft cerebellar vermis is suggested. Large cistern magna is also seen.
- "molar tooth" appearance of midbrain on axial images
- midline vermian clefting
- thick, horizontal (perpendicular to brainstem), superior cerebellar peduncle
- HASTE allows clear identification of "molar tooth" sign in fetuse
Top differential diagnoses:
- Dandy-walker continuum
- vermian and pontocerebellar hypoplasia
- cerebellar vermian atrophy