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Dandy-Walker syndrome (DWS), or Dandy-Walker complex, is a congenital brain malformation involving the cerebellum and the fluid filled spaces around it. The Dandy-Walker complex is a genetically sporadic disorder that occurs one in every 25,000 live births, mostly in females.
The key features of this syndrome are an enlargement of the fourth ventricle, the space containing cerebrospinal fluid between the medulla and the cerebellum, a partial or complete absence of the cerebellar vermis, the posterior midline area of cerebellar cortex responsible for coordination of the axial musculature, 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 syndrome can appear dramatically or develop unnoticed.
Symptoms, which often occur in early infancy, include slow motor development and progressive enlargement of the skull. In older children, symptoms of 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. 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.
Dandy-Walker syndrome is frequently associated with disorders of other areas of the central nervous system including absence of the corpus callosum, the bundle of axons connecting the two cerebral hemispheres, and malformations of the heart, face, limbs, fingers and toes.
The term Dandy-Walker represents not a single entity, but several abnormalities of brain development which coexist. There are, at present, three types of Dandy-Walker complexes.
They are divided into three closely associated forms: DWS malformation, DWS mega cisterna magna and DWS variant.
The DWS malformation is the most severe presentation of the syndrome. The posterior fossa is enlarged and the tentorium is in high position. There is partial or complete agenesis of the cerebellar vermis. There is also cystic dilation of the fourth ventricle, which fills the posterior fossa. This often involves hydrocephaly and complications due to associated genetic conditions, such as Spina Bifida.
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