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Isolated Dandy-Walker malformation

Synonym(s) -
Prevalence 1-9 / 100 000
Inheritance Multigenic/multifactorial
Age of onset Neonatal
  • Q03.1
MeSH -
MedDRA -


Disease definition

Dandy-Walker malformation (DWM) is the association of three signs: hydrocephalus, partial or complete absence of the cerebellar vermis, and posterior fossa cyst contiguous with the fourth ventricle, presenting early in life with hydrocephalus, bulging occiput and posterior fossa signs such as cranial nerve palsies, nystagmus and ataxia.

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