Overview

Dandy-Walker Syndrome is a congenital brain malformation involving the cerebellum (an area at the back of the brain that controls movement) and the fluid-filled spaces around it. The key features of this syndrome are an enlargement of the fourth ventricle (a small channel that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord), a partial or complete absence of the area of the brain between the two cerebellar hemispheres (cerebellar vermis), and cyst formation near the lowest part of the skull. An increase in the size of the fluid spaces surrounding the brain as well as an increase in pressure may also be present.

The syndrome can appear dramatically or develop unnoticed. Symptoms, which often occur in early infancy, include slow motor development and progressive enlargement of the skull. In older children, symptoms of increased intracranial pressure such as irritability and vomiting, and signs of cerebellar dysfunction such as unsteadiness, lack of muscle coordination, or jerky movements of the eyes may occur. Other symptoms include increased head circumference, bulging at the back of the skull, problems with the nerves that control the eyes, face and neck, and abnormal breathing patterns.

Dandy-Walker Syndrome is frequently associated with disorders of other areas of the central nervous system, including absence of the area made up of nerve fibers connecting the two cerebral hemispheres (corpus callosum) and malformations of the heart, face, limbs, fingers and toes.

Management and Treatment

Is there any treatment?

Treatment for individuals with Dandy-Walker Syndrome generally consists of treating the associated problems, if needed. A surgical procedure called a shunt may be required to drain off excess fluid within the brain. This will reduce intracranial pressure and help control swelling. Parents of children with Dandy-Walker Syndrome may benefit from genetic counseling if they intend to have more children.

Outlook / Prognosis

What is the prognosis?

The effect of Dandy-Walker Syndrome on intellectual development is variable, with some children having normal cognition and others never achieving normal intellectual development even when the excess fluid buildup is treated early and correctly. Longevity depends on the severity of the syndrome and associated malformations. The presence of multiple congenital defects may shorten life span.

What research is being done?

The NINDS conducts and supports a wide range of studies that explore the complex mechanisms of normal brain development. The knowledge gained from these fundamental studies provides the foundation for understanding abnormal brain development and offers hope for new ways to treat and prevent developmental brain disorders such as Dandy-Walker Syndrome.

Resources

Organizations

Dandy-Walker Alliance, Inc.

10325 Kensington Pkwy., Suite 384
Kensington, MD   20895
comments@dandy-walker.org
www.dandy-walker.org
Phone: 877.326.3992

Hydrocephalus Association

4340 East West Highway, Suite 905
Bethesda, MD   20814
info@hydroassoc.org
www.hydroassoc.org
Phone: 301.202.3811
Toll-free: 888.598.3789
Fax: 301.202.3813

March of Dimes Foundation

1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
www.marchofdimes.com
Phone: 914.428.7100
Toll-free: 888.MODIMES (663.4637)
Fax: 914.428.8203

National Hydrocephalus Foundation

12413 Centralia Road
Lakewood, CA 90715-1623
debbifields@nhfonline.org
nhfonline.org
Phone: 562.924.6666
Toll-free: 888.857.3434

National Organization for Rare Disorders (NORD)

55 Kenosia Avenue
Danbury, CT 06813-1968
orphan@rarediseases.org
www.rarediseases.org
Phone: 203.744.0100
Voice Mail: 800.999.NORD (6673)
Fax: 203.798.2291

*Source: National Institutes of Health; National Institute of Neurological Disorders and Stroke *

Reviewed by a Cleveland Clinic medical professional.

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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy