Dandy-Walker Syndrome

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(Dandy-Walker Malformation; Dandy Walker Syndrome; Familial Dandy Walker; Dandy Walker Malformation)

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.

Cerebellum

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:

Impaired development of normal speech and language
Slow motor development
Irritability
Vomiting
Unsteadiness
Lack of muscle coordination
Jerky eye movements
Increased head size
Bulging of the back of the skull
Abnormal breathing
Trouble with vision or hearing
Seizures

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:

CT scan
MRI scan
Ultrasound

Treatment

Dandy-Walker syndrome cannot be treated. Instead, treatment will focus on managing 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.cnsfederation.org

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

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.

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 May 21, 2013.

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.

Revision Information

Reviewer: Rimas Lukas, MD; Michael Woods, MD
Review Date: 05/2013 -
Update Date: 05/21/2013 -

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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