Table of Contents (click to jump to sections)

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 reduce intracranial pressure may be placed inside the skull to 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?
Children with Dandy-Walker Syndrome may never have 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

Hydrocephalus Support Group, Inc.
P.O. Box 4236
Chesterfield, MO 63006-4236
hydrodb@earthlink.net
Tel: 636-532-8228

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

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.