(Dandy-Walker Malformation; Dandy Walker Syndrome; Familial Dandy Walker; Dandy Walker Malformation)
Dandy-Walker syndrome is a rare brain deformity that happens before birth. It affects an area in the back of the brain that controls movement, thinking, and behavior. It also causes a buildup of fluid in ventricles (chambers) around it.
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The exact cause is not known. Genes may play a role.
This problem may be more common in people who have a parent with it.
A person with Dandy-Walker syndrome may have:
- Increased head size
- Muscles that are very floppy or stiff
- Delays in doing things like sitting up and walking
- Jerky eye movements or problems controlling facial movements
- Problems with coordination
- Bulging of the back of the skull
- Breathing problems
- Learning problems
Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs.
The doctor will ask about the child's symptoms and health history. A physical exam will be done.
Images may be taken of the brain. This can be done with:
There is no cure. The goal of treatment is to manage symptoms. This can be done with physical therapy and education services.
The buildup of fluid in the ventricles may need to be treated. Extra fluid can cause pressure and swelling in the brain. Fluid may be drained by:
- Shunt—A tube is placed from the brain to the belly to let the extra fluid drain out of the brain.
- Ventriculostomy—A connection is made from one ventricle to other areas that have fluid. This lets extra fluid drain out of the affected ventricle.
There is no known way to prevent this syndrome.
- Dandy-Walker malformation. Genetics Home Reference—National Institutes of Health website. Available at: https://ghr.nlm.nih.gov/condition/dandy-walker-malformation.
- Dandy-Walker malformation. National Organization for Rare Disorders website. Available at: https://rarediseases.org/rare-diseases/dandy-walker-malformation/.
- Dandy Walker syndrome. BrainFacts.org—Society for Neuroscience website. Available at: https://www.brainfacts.org/diseases-and-disorders/neurological-disorders-az/diseases-a-to-z-from-ninds/dandy-walker-syndrome.
- 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.
- Hydrocephalus in children. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/hydrocephalus-in-children.
- Kahle, K.T., Kulkarni, A.V., et al. Hydrocephalus in children. Lancet, 2016; 387 (10020): 788-799.
- Rimas Lukas, MD
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