Dandy-Walker syndrome is a congenital brain malformation. It involves the cerebellum (an area at the back of the brain that controls movement) and the fluid-filled spaces around it. The key characteristics of this syndrome are an enlargement of the fourth ventricle, a partial or complete absence of the area of the brain between the two cerebellar hemispheres (cerebellar vermis) and cyst formation near the internal base of the skull. An increase in the size of the fluid spaces surrounding the brain as well as an increase in pressure may also be present.
The symptoms of Dandy-Walker syndrome often occur in early infancy. The symptoms are slow motor development and progressive enlargement of the skull. In older children, the symptoms of Dandy-Walker syndrome are increased intracranial pressure such as irritability, vomiting, and convulsions, and signs of cerebellar dysfunction such as unsteadiness, lack of muscle coordination, or jerky movements of the eyes may occur.
Among other symptoms of the condition are increased head circumference, bulging at the back of the skull, problems with the nerves that control the eyes, face and neck, and abnormal breathing patterns.
The treatment for Dandy-Walker syndrome usually consists of treating the problem associated with the syndrome, if required. A surgical procedure called a shunt may be needed to drain off the extra fluid from the brain. This will help is lessening the intracranial pressure and prevent swelling. The parents of children suffering from Dandy-Walker syndrome can also benefit from the genetic counselling if they are aiming at having more children.
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