Dandy Walker Syndrome: A Developmental Brain Anomaly
DOI:
https://doi.org/10.9734/bpi/mono/978-81-19315-51-2/CH4Keywords:
Congenital, cerebellar, vermis, ventricle, hydrocephalusAbstract
Dandy-Walker syndrome is a rare condition that is characterized by certain key features, including the complete or partial absence of the vermis, expansion of the posterior fossa, and cystic dilation of 4th ventricle. Although some studies have suggested that the syndrome may be linked to serious consequences like maternal viral infections (such as rubella, toxoplasma, and cytomegalovirus) and alcohol use, but the exact cause of the condition remains unknown. One per 2500 births to one per 100,000 births have been recorded as the occurrence. Treatment for Dandy-Walker syndrome involves finding ways to manage hydrocephalus, however this is still subject to debate. The related abnormalities play a significant role in the prognosis. The death rate is between 27% to 50%, and poor intellectual development is linked to hearing and/or vision issues.