Definition

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.

Cerebellum

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Causes

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.

Risk Factors

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

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
• 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 digits.

Diagnosis

Your doctor will ask about symptoms and medical history, and perform a physical exam. He will also likely do a CT]]> or ]]>MRI scan]]> to view the inside of the brain.

Treatment

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.

Prevention

There is no known way to prevent Dandy-Walker syndrome.