(Dandy-Walker Malformation; Dandy Walker Syndrome; Familial Dandy Walker; Dandy Walker Malformation)
by Krisha McCoy, MS
Dandy-Walker syndrome is a brain deformity that develops before birth. The deformity is 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 buildup of fluid.
It is not clear what causes Dandy-Walker syndrome. There may be a genetic factor.
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 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:
Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs.
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:
Dandy-Walker syndrome cannot be cured. Instead, treatment will focus on managing certain brain abnormalities and symptoms.
The buildup of fluid in the ventricles may need treatment. Extra fluid can cause increased pressure and swelling in the brain. Fluid may be drained by:
Some children will require physical therapy and educational help.
Talk with your doctor about the best treatment plan for your child.
There is no known way to prevent this syndrome.
Genetics Home Reference
National Institute of Neurological Disorders and Stroke
Canadian Neurological Sciences Federation
Aldinger KA, Lehmann OJ, 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 information page. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/Disorders/All-Disorders/Dandy-Walker-Syndrome-Information-Page. Accessed February 21, 2018.
Spennato P, Mirone G, et al. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst. 2011;27:1665-1681.
Last reviewed March 2018 by EBSCO Medical Review Board Rimas Lukas, MD
Last Updated: 2/13/2014
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