Dandy-Walker syndrome is an inborn abnormality that impacts the cerebellum, a region located at the posterior part of the brain, as well as the fluid-filled cavities surrounding it. A prominent characteristic of this disorder is the presence of a disproportionately enlarged fourth ventricle, which facilitates the flow of cerebrospinal fluid between the spinal cord and the upper and lower regions of the brain. Additionally, certain individuals may experience elevated intracranial pressure.
Abnormalities of the Brain in Dandy-Walker Syndrome
In addition to Dandy-Walker syndrome, there are various other brain abnormalities that can occur. These include the absence or underdevelopment of the tissue that connects the right and left sides of the brain, a small projection of brain tissue through the posterior part of the skull, and a group of nerve cells that fail to migrate to their appropriate position during fetal development.
Frequency
Dandy-Walker malformation is a relatively uncommon condition, with an occurrence rate of only one in every 10,000 to 30,000 births. It tends to affect females more frequently than males. In approximately 10 to 20 percent of cases, symptoms and signs may not manifest until later in childhood or adulthood. Interestingly, individuals who develop symptoms at a later stage in life often display a distinct set of symptoms compared to those affected during infancy.