Dandy-Walker Syndrome
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What is Dandy-Walker Syndrome?
Is there any treatment?
What is the prognosis?
What research is being done?
Organizations
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?
What research is being done?
Select this link to view a list of studies currently seeking patients.
Dandy-Walker Alliance, Inc. 4422 Clearbrook Lane Kensington, MD 20895 eric.cole@dandy-walker.org http://www.dandy-walker.org Tel: 301-919-2653 |
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 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 debbifields@nhfonline.org http://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 http://www.rarediseases.org Tel: 203-744-0100 Voice Mail 800-999-NORD (6673) Fax: 203-798-2291 |
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Last updated April 02, 2008