National Institutes of Health, Bethesda,
Maryland 20892
May 1996
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DESCRIPTION: Dandy-Walker syndrome is a congenital brain malformation involving the fourth ventricle and cerebellum. It is defined as an enlargement of the fourth ventricle, an absence (partial or complete) of the cerebellar vermis (the narrow middle area between the two cerebral hemispheres), and cyst formation in the posterior fossa (the internal base of the skull). Hydrocephalus (increased intracranial pressure) may also be present. Symptoms which often occur in early infancy include slow motor development and progressive macrocrania (an abnormally enlarged skull). In older children symptoms of increased intracranial pressure such as irritability, vomiting and convulsions, and/or signs of cerebellar dysfunction such as ataxia and nystagmus (jerky eyes) may occur. The syndrome can appear dramatically or be totally asymptomatic. Other symptoms include increased head circumference, bulging occiput (the back of the head), cranial nerve dysfunction, and abnormal breathing patterns. Of importance is the high association of Dandy-Walker syndrome with other central nervous system structural anomalies including agenesis of the corpus callosum and mal-formations of the face, limbs, digits and heart.
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TREATMENT: Treatment for Dandy-Walker syndrome consists of surgery to remove the cyst from the cervical spinal canal and treatment of the associated anomalies, if needed. A ventriculoperitoneal shunt may also be inserted to control the hydrocephalus. Genetic counselling may also be needed.
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PROGNOSIS: The prognosis for patients with Dandy-Walker syndrome is only moderately favorable, even when the hydrocephalus is treated early and correctly. The presence of multiple congenital defects may adversely affect survival. Prognosis for normal intellectual development is variable depending on the severity of the syndrome and associated malformations.
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RESEARCH: 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 how this process can go awry and, thus, offers hope for new means to treat and prevent developmental brain disorders, including Dandy-Walker syndrome.
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These articles, available from a medical library, may provide more in-depth information on Dandy-Walker syndrome:
Bordarier, C, and Aicardi, J. "Dandy-Walker Syndrome and Agenesis of the Cerebellar Vermis: Diagnostic Problems and Genetic Counselling." Developmental Medicine and Clinical Neurology, 32; 285-294 (1990).
Castroviejo, I, Velez, A, Pascual-Pascual, S, Roche, M, and Villarejo, F. "Dandy- Walker Malformation: Analysis of 38 Cases." Child's Nervous System, 7:2; 88-97 (1991).
James, H. "Hydrocephalus in Infancy and Childhood." American Family Physician, 45:2; 733-742 (February 1992).
Russ, P, Pretorius, D, and Johnson, M. "Dandy-Walker Syndrome: A Review of Fifteen Cases Evaluated by Prenatal Sonography." American Journal of Obstetrics and Gynecology, 161:2; 401-406 (1989).
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Information may also be available from the following organizations:
Hydrocephalus Association
870 Market Street
Suite 955
San Francisco, CA 94102
(415) 776-4713
Guardians of Hydrocephalus Research Foundation
2618 Avenue Z
Brooklyn, NY 11235
(800) 458-8655
(718) 743-4473
National Hydrocephalus Foundation
22427 South River Road
Joliet, IL 60436
(815) 467-6548
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-8923
(800) 999-6673 or (203) 746-6518
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or NINDS Publications Page
Last Updated: May 13, 1997
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
