Medicine & USMLE

Dandy-Walker Malformation

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Posterior Fossa Malformations
  1. Chiari I Malformation
  2. Chiari II Malformation
  3. Dandy-Walker Malformation

Dandy-Walker Malformation

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Summary

Dandy-Walker malformations are a congenital brain malformation that affects the posterior fossa. These malformations are marked by an absent or underdeveloped cerebellar vermis, accompanied by cystic enlargement of the 4th ventricle and in some cases, non-communicating hydrocephalus. The malformation is also classically associated with spina bifida. Dandy Walker malformations typically present in newborn infants with progressive skull enlargement and developmental delay.

Key Points

  • Dandy-Walker Malformation
    • Type of posterior fossa malformation
    • Pathophysiology
      • Absence of cerebellar vermis
      • Cystic enlargement of 4th ventricle
      • Causes non-communicating hydrocephalus
      • Associated with spina bifida
    • Presentation
      • Presents in infants
      • Progressive skull enlargement
      • Developmental delay