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.
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.
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.
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.
Dandy-Walker Alliance, Inc. 10325 Kensington Pkwy. Suite 384 Kensington, MD 20895 comments@dandy-walker.org http://www.dandy-walker.org Tel: 877-Dandy-Walker (326-3992) |
Hydrocephalus Association 4340 East West Highway Suite 905 Bethesda, MD 20814 info@hydroassoc.org http://www.hydroassoc.org Tel: 301-202-3811 888-598-3789 Fax: 301-202-3813 |
March of Dimes 1275 Mamaroneck Avenue White Plains, NY 10605 askus@marchofdimes.com http://www.marchofdimes.com Tel: 914-997-4488 888-MODIMES (663-4637) Fax: 914-428-8203 |
National Hydrocephalus Foundation 12413 Centralia Road Lakewood, CA 90715-1653 debbifields@nhfonline.org http://nhfonline.org Tel: 562-924-6666 888-857-3434 |
National Organization for Rare Disorders (NORD) 55 Kenosia Avenue Danbury, CT 06810 orphan@rarediseases.org http://www.rarediseases.org Tel: 203-744-0100 Voice Mail 800-999-NORD (6673) Fax: 203-798-2291 |
Pediatric Hydrocephalus Foundation 2004 Green Hollow Drive Iselin, NJ 08830 info@hydrocephaluskids.org http://www.HydrocephalusKids.org Tel: 732-634-1283 |
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National Institute of Neurological Disorders and Stroke
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Last updated December 16, 2011