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Pediatric Dandy-Walker Malformation

The Dandy-Walker malformation is a congenital (present at birth) defect affecting the cerebellum, the back part of the brain that controls movement, behavior and cognitive ability.

Dandy-Walker can cause obstruction of the normal drainage of cerebrospinal fluid (CSF), resulting in a build-up of CSF and a condition called hydrocephalus.

The Dandy-Walker malformation is part of a spectrum of conditions that include the classic form and other mild variants. It is important to make a correct diagnosis because the different conditions may have very different outcomes. The classic form of Dandy-Walker malformation has certain key features:

  • Enlarged back compartment (posterior fossa) of the brain
  • Malformed or missing cerebellar vermis (part of the cerebellum)
  • Large cyst in the fourth ventricle of the brain

In addition, most children will develop hydrocephalus (CSF buildup in the brain) before their first birthday. 

Learn more about our Prenatal Pediatrics Institute.