By: Morgan Rawls
What is it?
-Slow motor development
-Progressive enlargement of the skull
-Increased intracranial pressure such as irritability and vomiting
-Signs of cerebellar dysfunction such as unsteadiness, lack of muscle coordination, or jerky movements of the eyes
-Increased head circumference
-Bulging at the back of the skull
-Problems with the nerves that control the eyes, face and neck
-Abnormal breathing patterns
Treatment for individuals with Dandy-Walker Syndrome generally consists of treating the associated problems, if needed. A surgical procedure called a shunt may be required to drain off excess fluid within the brain. This will reduce intracranial pressure and help control swelling.
Parents of children with Dandy-Walker Syndrome may benefit from genetic counseling if they intend to have more children.
The effect of Dandy-Walker Syndrome on intellectual development is variable, with some children having normal cognition and others never achieving normal intellectual development even when the excess fluid buildup is treated early and correctly. Longevity depends on the severity of the syndrome and associated malformations.
The presence of multiple congenital defects may shorten life span.
-An enlargement of the fourth ventricle (a small channel that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord)
-A partial or complete absence of the area of the brain between the two cerebellar hemispheres (cerebellar vermis)
-Acyst formation near the lowest part 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
Dandy Walker Syndrome can be diagnosed through the following examinations:
MRI – This has been noted as one of the best diagnostic exam for neurological or CNS related conditions.
CT scan – This procedure assists in the diagnosis of Dandy Walker syndrome. There are 3D studies that are good in the evaluation process of DWS.
Angiography –This can demonstrate or provide the features of malformations of Dandy Walker syndrome.
Ultrasonography – The malformation can be identified through the antenatal ultrasound.