Cite

Copy

Tap on and choose 'Add to Home Screen' to create a shortcut app

Tap on and choose 'Install/Install App' to create a shortcut app

Epidemiology of Dandy-Walker Syndrome in Children

This page was last updated on May 9th, 2017

Incidence and Prevalence

  • 1:30,000 births: Dandy-Walker syndrome occurs in approximately 1 in every 30,000 births.

Age Distribution

  • Perinatal as opposed to trapped fourth ventricle that occurs later: As a congenital abnormality, Dandy-Walker syndrome presents at birth. The ability to have a fetal diagnosis is one determinant of whether the condition is Dandy-Walker syndrome or is simply hydrocephalus due to a trapped fourth ventricle.

Sex Predilection

  • Female preponderance: The condition is up to three times more common in females.

Geographic Distribution

  • None

Risk Factors

  • Maternal use of warfarin: Some studies have suggested a link between maternal use of warfarin during pregnancy and Dandy-Walker malformations (7).

Relationships to Other Disease States and Syndromes

Hydrocephalus

  • Present in nearly all: 80–90% of patients with a Dandy-Walker malformation have hydrocephalus. Up to 4% of cases of congenital hydrocephalus are Dandy-Walker syndrome associated.

Other CNS anomalies

  • Posterior fossa anomalies: Other developmental abnormalities of the posterior fossa besides those seen in the cerebellum’s vermis are caused by the enlarged fourth ventricle.
  • Cephaloceles and agenesis of corpus callosum: Dandy-Walker syndrome is associated with other CNS abnormalities, such as agenesis of corpus callosum and cephaloceles, 20–70% of the time.
  • Dandy-Walker variant: The Dandy-Walker variant connotes a version of Dandy-Walker syndrome with a lesser degree of vermian abnormalities and less fourth ventricular enlargement.
  • PHACES syndrome: Dandy-Walker syndrome often occurs in patients with PHACES syndrome.

Your donations keep us going

The ISPN Guide is free to use, but we rely on donations to fund our ongoing work and to maintain more than a thousand pages of information created to disseminate the most up-to-date knowledge in the field of paediatric neurosurgery.

By making a donation to The ISPN Guide you are also indirectly helping the many thousands of children around the world whose treatment depends on well-informed surgeons.

Please consider making a donation today.

Use the app

The ISPN Guide can be used as a standalone app, both on mobile devices and desktop computers. It’s quick and easy to use.

Fully featured

Free registration grants you full access to The Guide and host of featured designed to help further your own education.

Stay updated

The ISPN Guide continues to expand both in breadth and depth. Join our mailing list to stay up-to-date with our progress.