Dandy-Walker syndrome is a brain deformity that develops before birth. The deformity is 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 may have an abnormal buildup of fluid.
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.
You will be asked about your child's symptoms and medical history. A physical exam will be done.
Images will be taken of your child's brain. This can be done with:
Dandy-Walker syndrome cannot be cured. Instead, treatment will focus on managing certain brain abnormalities and symptoms.
The buildup of fluid in the ventricles may need treatment. Extra fluid can cause increased pressure and swelling in the brain. Fluid may be drained by:
Some children will require physical therapy and educational help.
Talk with your doctor about the best treatment plan for your child.
Genetics Home Reference
National Institute of Neurological Disorders and Stroke
Canadian Neurological Sciences Federation
Aldinger KA, Lehmann OJ, 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.
Dandy-Walker syndrome information page. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/Disorders/All-Disorders/Dandy-Walker-Syndrome-Information-Page. Accessed February 21, 2018.
Spennato P, Mirone G, et al. Hydrocephalus in Dandy-Walker malformation. Childs Nerv Syst. 2011;27:1665-1681.
Last reviewed February 2018 by EBSCO Medical Review Board 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.