What is Dandy-Walker Syndrome?
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 cerebellar vermis (the area between the two cerebellar hemispheres), and cyst formation near the internal base 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, vomiting, and convulsions, 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 corpus callosum (the connecting area between the two cerebral hemispheres, and malformations of the heart, face, limbs, fingers and toes.
Is there any treatment?
Treatment for individuals with Dandy-Walker Syndrome generally consists of treating the associated problems, if needed. A special tube to drain off excess fluid may be placed inside the skull. 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.
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 hydrocephalus 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.
Organizations
Guardians of Hydrocephalus Research Foundation
2618 Avenue Z
Brooklyn, NY 11235-2023
GHRF2618@aol.com
http://ghrf.homestead.com/ghrf.html
Tel: 718-743-GHRF (4473)
Fax: 718-743-1171
Hydrocephalus Association
870 Market Street Suite 705
San Francisco, CA 94102
info@hydroassoc.org
http://www.hydroassoc.org/
Tel: 415-732-7040 888-598-3789
Fax: 415-732-7044
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
http://www.marchofdimes.com/
Tel: 914-428-7100 888-MODIMES (663-4637)
Fax: 914-428-8203
National Hydrocephalus Foundation
12413 Centralia Road
Lakewood, CA 90715-1623
hydrobrat@earthlink.net
http://nhfonline.org/
Tel: 562-402-3523 888-857-3434
Fax: 562-924-6666
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 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
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 2/12/2007...#6002
