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Dandy-Walker Syndrome by Krisha McCoy, MS

Definition

Dandy-Walker syndrome is a brain deformity. It 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 have an abnormal build-up of fluid with Dandy-Walker.

The syndrome can cause a variety of problems. These problems may develop suddenly.

Cerebellum

Causes

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

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:

Impaired development of normal speech and language
Slow motor development
Irritability
Vomiting
Convulsions
Unsteadiness
Lack of muscle coordination
Jerky eye movements
Increased head circumference
Bulging of the back of the skull
Problems with the nerves that control the eyes, face, and neck
Abnormal breathing

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

Diagnosis

Your doctor will ask about your child's symptoms and medical history. A physical exam will be done.

Your doctor may also order test to take details pictures of the brain. This will help show changes to the ventricles. Pictures may be made with:

Treatment

Dandy-Walker syndrome itself cannot be treated. Instead, treatment will focus on managing your child's 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 in the brain to allow the extra fluid to drain out of the brain.
Ventriculostomy—a tube from one ventricle to another. It allows extra fluid to drain out of the affected ventricle.

Talk with your doctor about the best treatment plan.

Prevention

There is no known way to prevent this syndrome.

RESOURCES:

Children's Craniofacial Association http://www.ccakids.com/
National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/

CANADIAN RESOURCES:

Canadian Neurological Sciences Federation http://www.ccns.org/
Health Canada http://www.hc-sc.gc.ca/index_e.html/

References:

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.
Boltshauser EJ. Dandy-Walker syndrome. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. MedLink website. Available at: http://www.medlink.com . Accessed September 20, 2012.
Dandy-Walker. Hyman-Newman Institute for Neurology and Neurosurgery website. Available at: http://www.nyneurosurgery.org/dandywalker.htm . Accessed September 20, 2012.
Dandy-Walker syndrome. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm . Accessed September 20, 2012.
Sarnat HB. Flores-Sarnat L. Developmental disorders of the nervous system. In: Bradley WG, Daroff RB, Fenichek GM, Jankovic J, eds. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Butterworth Heinemann Elsevier; 2008.
Spennato P, et al. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst. 2011;27:1665-81.

Last reviewed September 2012 by Rimas Lukas, MD

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