Primary lactic acidosis.
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The brain MRI demonstrates normal ventricular system and midline structures. A large posterior fossa is noted with cerebellar hypoplasia notably in the left cerebellar hemisphere with enlargement of the retrocerebellar subarachnoid space that communicates with the 4th ventricle. There is no evidence of pontine or brainstem hypoplasia.
The described findings are most likely of Dandy-Walker variant.