(Dandy-Walker Malformation; Dandy Walker Syndrome; Familial Dandy Walker; Dandy Walker Malformation)
Dandy-Walker syndrome is a brain deformity present at birth consisting of a deformity of the cerebellum and the presence of cyst in the lower portion of the brain. The deformity involves an area in the back of the brain that controls movement and cognitive learning. In many cases, there is also an abnormal accumulation of cerebrospinal fluid within the ventricles of the vein. The symptoms of this syndrome may develop suddenly or may go unnoticed.
Dandy-Walker syndrome occurs during brain development before birth. The reason the brain does not develop normally is not clearly understood. Recently, a gene has been discovered that appears to be the cause of at least some cases.
Dandy Walker Syndrome may be inherited, therefore having a parent with Dandy Walker Syndrome may increase the risk of occurrence in the children.
Aside from association with certain inherited genetic conditions, there are no known risk factors. The following factors are associated with Dandy-Walker syndrome, but do not increase the risk of it’s occurrence:
- Absence of the corpus callosum, which connects the brain's hemispheres
- Malformations of the heart, face, limbs, fingers, and toes
Symptoms of Dandy-Walker syndrome often occur in infancy, but can also occur in older children. Eighty percent of cases are diagnosed within the first year of life. Symptoms may include:
- Impaired development of normal speech and language
- Slow motor development
- Lack of muscle coordination
- Jerky eye movements
- Increased head circumference
- Bulging of the back of the skull
- Problems with the nerves that control the eyes, face, and neck
- Abnormal breathing
Children with this condition may have problems with other organs, including heart malformations, kidney and urinary tract abnormalities, cleft lip]]> , and extra digits.
Talk with your doctor about the best treatment plan. Treatment will depend on the problems caused by the syndrome. This may involve placing a special tube called a shunt inside the skull to drain excess fluid to reduce pressure and help control swelling.
Children's Craniofacial Association
National Institute of Neurological Disorders and Stroke
Canadian Neurological Sciences Federation
Boltshauser EJ. Dandy-Walker syndrome. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. MedLink website. Available at: http://www.medlink.com . Accessed August 10, 2007.
Dandy-Walker. Hyman-Newman Institute for Neurology and Neurosurgery website. Available at: http://www.nyneurosurgery.org/dandywalker.htm . Accessed May 22, 2007.
Dandy-Walker syndrome. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm . Accessed May 22, 2007.
Sarnat HB. Flores-Sarnat L. Developmental disorders of the nervous system. In: Bradley WG, Daroff RB, Fenichek GM, Jankovic J, eds. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Butterworth Heinemann Elsevier; 2008.
Last reviewed January 2009 by ]]>J. Thomas Megerian, MD, PhD, FAAP]]>
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