Dandy-Walker malformation, an uncommon brain abnormality that may be seen in isolation or in association with other congenital abnormalities of brain development, is characterized by an enlarged posterior fossa, agenesis or hypoplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. In this case of a term infant with multiple congenital abnormalities, the superior most aspect of the cerebellar vermis is present whereas the middle and inferior portions are absent. Note the marked volume loss, as well as the lateral and superior deviation, of the cerebellar hemisphere.
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