Dandy-Walker Syndrome
En Español (Spanish Version)Dandy-Walker syndrome is a brain deformity present at birth. With this syndrome, there is 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.
Cerebellum
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Dandy-Walker syndrome occurs during brain development before birth. It is unclear why the brain does not develop normally. Recently, genes have been discovered that appear to contribute to some cases of Dandy-Walker syndrome.
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.
These factors are associated with Dandy-Walker syndrome, but they do not increase the risk of being born with the syndrome:
- 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. Most cases are diagnosed within the first year of life. Symptoms may include:
- Impaired development of normal speech and language
- Slow motor development
- Irritability
- Vomiting
- Convulsions
- Unsteadiness
- 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 fingers or toes.
The doctor will:
- Ask about your child's symptoms and medical history
- Do a physical exam
-
Order tests (eg,
CT scan
,
MRI scan
)
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. The shunt drains excess fluid to reduce pressure and help control swelling.
There is no known way to prevent this syndrome.
Children's Craniofacial Association
National Institute of Neurological Disorders and Stroke
Canadian Neurological Sciences Federation
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 August 10, 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 September 2011