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Dandy-Walker syndrome
Background
This is a condition whereby there is probably abnormal development of the foraminae (holes) through which the cerebrospinal fluid (CSF) exits from inside the brain to the outside surface. The foraminae, not having properly opened during fetal development cause a blockage of the flow of the CSF and so produce an abnormal enlargement of the fourth ventricle (fluid cavity between the brainstem and the cerebellum). This results in failure of parts of the cerebellum to develop correctly. A large cyst is then visible on scans of the back of the brain. These are severe abnormalities with hydrocephalus being present in ninety per cent of cases. Dandy-Walker causes two to four per cent of all hydrocephalus cases. Conditions with similar appearances are called Dandy-Walker variant. Most commonly, however, there is no obvious explanation for it's presence.
There are often many associated brain abnormalities including agenesis of the corpus callosum in seventeen per cent of cases, occipital encephalocoele (failure of fusion of the bones at the back of the head) in seven per cent, spina bifida, syringomyelia (cysts in the spinal cord), or small heads and bone abnormalities of the neck (Klippel-Feil syndrome). Other associated abnormalities include facial, ocular (eye) and cardiac.
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