Dandy-Walker Syndrome

What is Dandy-Walker Syndrome?
Dandy-Walker syndrome is a congenital brain malformation involving the cerebellum (an area in the back of the brain that controls movement) and the fluid filled spaces around it. The key features of this syndrome include an enlargement of the fourth ventricle (a small channel that allows fluid to flow freely between the upper and lower area of the brain and spinal cord), a partial or complete absence of the area of the brain between two cerebral hemispheres, 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.

This syndrome can usually develop in two ways; it can develop unnoticed or appear dramatically. Symptoms, which often occur in early infancy, include slow motor development and progressive enlargement of the skull. In older children, some symptoms of increased intracranial pressure such as irritability, vomiting, and convulsion and signs of cerebral dysfunction such as unsteadiness, lack of muscle coordination, or jerky movements of the eye may occur. Other symptoms include 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.