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Definition

Dandy-Walker syndrome is a brain malformation present at birth. The malformation involves the cerebellum, which is an area in the back of the brain that controls movement. The symptoms of this syndrome may develop suddenly or may go unnoticed.

Cerebellum

Causes

Dandy-Walker syndrome occurs during brain development before birth, but the reason the brain does not develop normally is not clearly understood.

Risk Factors

Aside from association with certain inherited genetic conditions, there are no known risk factors. The following factors are associated with Dandy-Walker syndrome, but do not increase the risk of it’s occurrence.

Absence of the corpus callosum , which connects the brain's hemispheres
Malformations of the heart, face, limbs, fingers, and toes

Symptoms

Symptoms of Dandy-Walker syndrome often occur in infancy but can also occur in older children. 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

Diagnosis

Your doctor will ask about symptoms and medical history, and perform a physical exam. He or she will also likely perform a CT or MRI scan to view the inside of the brain.

Treatment

Talk with your doctor about the best treatment plan. Treatment will depend on the problems caused by the syndrome. This may involve placing a special tube called a shunt inside the skull to drain excess fluid to reduce pressure and help control swelling.

Prevention

There is no known way to prevent Dandy-Walker 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:

Boltshauser EJ. Dandy-Walker syndrome. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. Available at: http://www.medlink.com . Accessed August 10, 2007.

Dandy-Walker. Hyman-Newman Institute for Neurology and Neurosurgery website. Available at: http://www.nyneurosurgery.org/dandywalker.htm . Accessed May 22, 2007.

Dandy-Walker syndrome. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm . Accessed May 22, 2007.

Last reviewed August 2007 by J. Thomas Megerian, MD, PhD, FAAP

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

Dandy-Walker Syndrome

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