What is it?

Dandy-Walker Syndrome (DWS), or Dandy Walker malformation is a rare congenital malformation which occurs during the embryonic development. In this condition, the cerebellar vermis- which connects both cerebellar hemispheres is not fully formed, causing enlargement of the base of the skull, the posterior fossa. Sometimes, it can cause hydrocephalus because of the blockage of cerebrospinal fluid drainage . DWS is sometimes in comorbidity with other genetic conditions, therefore it is important to look for heart defects, eye abnormalities, other brain defects such as agenesis of corpus callosum and malformation in bones, kidneys and genitalia. DWS is caused by a impatired cell migration during embryonic period, and in some cases a chromosomal genetic condition is found.

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Additional names

This group contains additional names:
- Aqueduct of Sylvius anomaly
- Hydranencephaly
- Dandy-Walker Syndrome (DWS)
- hydrocephalus

Signs & symptoms

Dandy-Walker Syndrome (DWS) symptoms may include developmental delay, hypotonia, spasticity, poor coordination, ataxia, enlarged head circumference, vomiting, sleepiness, irritability, downward deviation of the eyes, seizures.

Diagnosis

Dandy-Walker Syndrome (DWS) is diagnosed in a brain US, CT or MRI. Prenatally, it can be diagnosed in fetal US or MRI.

Treatment

Dandy-Walker Syndrome (DWS) has no cure. Hydrocephalus can be treated with surgery during which a tube is inserted to help fluid drainage to other parts of the body- such as a VP shunt. Other supportive treatment may include special education, physical therapy and other medical, social or vocational services.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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