Dandy-Walker syndrome is a brain deformity. It develops before birth. The deformity occurs in an area in the back of the brain. This area controls movement and cognitive learning. This syndrome can also affect fluid-filled chambers of the brain called ventricles. The ventricles have an abnormal build-up of fluid with Dandy-Walker.
The syndrome can cause a variety of problems. These problems may develop suddenly.
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Dandy-Walker syndrome may be inherited. If a parent has Dandy-Walker syndrome, the child has a higher risk of the condition. There are no other known risk factors.
Symptoms of Dandy-Walker syndrome often occur in infancy. Some may not appear until childhood. Most are found within the first year of life. Symptoms may include:
Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs.
Your doctor will ask about your child's symptoms and medical history. A physical exam will be done.
Your doctor may also order test to take details pictures of the brain. This will help show changes to the ventricles. Pictures may be made with:
Dandy-Walker syndrome itself cannot be treated. Instead, treatment will focus on managing your child's symptoms.
The build-up of fluid in the ventricles may need treatment. Extra fluid can cause increased pressure and swelling in the brain. Fluid may be drained by:
Talk with your doctor about the best treatment plan.
RESOURCES:
Children's Craniofacial Association
http://www.ccakids.com/
National Institute of Neurological Disorders and Stroke
http://www.ninds.nih.gov/
CANADIAN RESOURCES:
Canadian Neurological Sciences Federation
http://www.ccns.org/
Health Canada
http://www.hc-sc.gc.ca/index_e.html/
References:
Aldinger KA, Lehmann OJ, Hudgins L, et al. FOXC1 is required for normal cerebellar development and is a major contributor to chromosome 6p25.3 Dandy-Walker malformation. Nature Genetics. 2009;41(9):1037-1042.
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 September 20, 2012.
Dandy-Walker. Hyman-Newman Institute for Neurology and Neurosurgery website. Available at: http://www.nyneurosurgery.org/dandywalker.htm . Accessed September 20, 2012.
Dandy-Walker syndrome. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm . Accessed September 20, 2012.
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.
Spennato P, et al. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst. 2011;27:1665-81.
Last reviewed September 2012 by Rimas Lukas, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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