Dandy-Walker malformation (DWM) is a congenital brain defect in which the 4th ventricle fails to close. It is characterized with a cysts in the 4th ventricle, hypoplasia of the cerebellar vermis, and enlargement of the posterior cranial fossa. This condition results in hydrocephalus, increased intracranial pressure, and a characteristic "dandy walker" appearance on neuroimaging.
Symptoms of DWM vary depending on the degree of cerebellar hypoplasia. Patients may experience delayed milestones, hypotonia, ataxia, vomiting, and seizures. Hydrocephalus can cause enlargement of the head (macrocephaly) and bulging fontanelles.
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