Dandy-Walker Syndrome

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Definition

Dandy-Walker syndrome is a brain deformity that develops before birth. The deformity occurs in an area in the back of the brain. This area controls movement and cognitive learning. This syndrome can also affect fluid-filled chambers of the brain called ventricles. The ventricles may have an abnormal build-up of fluid.

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Causes

It is not clear what causes Dandy-Walker syndrome. There may be a genetic factor.

Risk Factors

Dandy-Walker syndrome may be inherited. If a parent has Dandy-Walker syndrome, the child has a higher risk of the condition. There are no other known risk factors.

Symptoms

Symptoms of Dandy-Walker syndrome often occur in infancy. Some may not appear until childhood. Most are found within the first year of life. Symptoms may include:

  • Increased head size
  • Bulging of the back of the skull
  • Impaired development of normal speech and language
  • Slow motor development
  • Irritability
  • Headache
  • Vomiting
  • Unsteadiness, especially with walking
  • Lack of muscle coordination
  • Jerky eye movements
  • Abnormal breathing
  • Trouble with vision or hearing
  • Jerking movements of the arms and legs or seizures

Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs.

Diagnosis

You will be asked about your child's symptoms and medical history. A physical exam will be done.

Images will be taken of your child's brain. This can be done with:

Treatment

Dandy-Walker syndrome cannot be treated. Instead, treatment will focus on managing certain brain abnormalities and symptoms.

The build-up of fluid in the ventricles may need treatment. Extra fluid can cause increased pressure and swelling in the brain. Fluid may be drained by:

  • Shunt—a tube is placed from the brain to the abdomen to allow the extra fluid to drain out of the brain.
  • Ventriculostomy—a connection is made from one ventricle to other areas that contain fluid. It allows extra fluid to drain out of the affected ventricle.

Some children with require physical therapy and educational help.

Talk with your doctor about the best treatment plan for you.

Prevention

There is no known way to prevent this syndrome.

Revision Information

  • Reviewer: Rimas Lukas, MD
  • Review Date: 02/2014 -
  • Update Date: 02/13/2014 -
  • Children's Craniofacial Association

    http://www.ccakids.com

  • National Institute of Neurological Disorders and Stroke

    http://www.ninds.nih.gov

  • Canadian Neurological Sciences Federation

    http://www.cnsfederation.org

  • Health Canada

    http://www.hc-sc.gc.ca

  • Aldinger KA, Lehmann OJ, Hudgins L, et al. FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nature Genetics. 2009;41(9):1037-1042.

  • Dandy-Walker syndrome. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm. Updated December 16, 2011. Accessed February 13, 2014.

  • Spennato P, et al. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst. 2011;27:1665-81.