Dandy Walker Malformation, Defect of the Brain

What is Dandy-Walker Syndrome?

Dandy-Walker Syndrome or Dandy-Walker complex is a congenital brain malformation involving the area at the back of the brain that controls movement and the fluid-filled spaces around it.

A key feature of DWS is the partial or even complete absence of the part of the brain located between the two cerebellar hemispheres. An increase in the size of the fluid spaces surrounding the brain as well as an increase in pressure may also be present.

The Dandy-Walker complex is a very rare congenital brain disorder, genetically sporadic occurring one in every 25,000 live births, mostly girls. Seventy to 90 percent of babies with DWS will also have hydrocephalus.

Surprisingly, Dandy-Walker Syndrome can dramatically appear or develop unnoticed. Early infancy symptoms include slow motor development and progressive enlargement of the skull. In older children, symptoms of increased intracranial pressure such as irritability, vomiting, and convulsions, and unsteadiness, lack of muscle coordination, or jerky movements of the eyes may occur. Other symptoms include increased head circumference, bulging at the back of the skull, nerve problems, and abnormal breathing patterns.

Dandy-Walker Syndrome is associated with central nervous system disorders and malformations of the heart, face, limbs, fingers, and toes.

What causes Dandy-Walker Syndrome?

The cause for DWS is unknown but the causes of hydrocephalus are:

• Toxoplasmosis - an infection from eating undercooked meat, or by coming in contact with infected soil or an infected animal
• Cytomegalovirus or CMV - an infection from a herpes virus
• Rubella or German measles

As of March 2011 a new report from the Center for Disease Control said that pregnant or women thinking about becoming pregnant, should know there are risks associated with using prescription painkillers. One of these risks is hydrocephaly and spina bifida.

What is the treatment for Dandy-Walker Syndrome?

Treatment for Dandy-Walker Syndrome consists of treating associated problems. A special tube or shunt to drain excess fluid may be placed inside the skull to reduce intracranial pressure and control swelling. Parents of children with Dandy-Walker Syndrome should seek genetic counseling if they plan to have more children.

Treatment may also consist of occupational therapy, physical therapy, speech therapy, and specialized education.

Outcomes vary from worst case scenario to mental retardation to normal intellectual development.

Here's a resource devoted to Dandy-Walker Syndrome.

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