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 area of the brain between the two cerebellar
hemispheres (cerebellar vermis), 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 area made up of nerve
fibers connecting the two cerebral hemispheres (corpus callosum) 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 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.
Organizations
Dandy-Walker Alliance, Inc.
4422 Clearbrook Lane
Kensington, MD 20895
comments@dandy-walker.org
www.dandy-walker.org
Tel: 877.Dandy.Walker
Guardians of Hydrocephalus Research Foundation
2640 East 28th Street
Brooklyn, NY 11235
GHRF2618@aol.com
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
www.hydroassoc.org
Tel: 415.732.7040 888.598.3789
Fax: 415.732.7044
March of Dimes Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
askus@marchofdimes.com
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
debbifields@nhfonline.org
nhfonline.org
Tel: 562.924.6666 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
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: 9/16/2008...#6002
