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Dandy-Walker malformation, first described by Dr. Walter Dandy and Dr. Arthur Walker, is a congenital brain defect where there’s a cystic malformation in the cerebellum that blocks cerebrospinal fluid from exiting the brain.
The cerebellum, or "little brain", sits just below the main part of the brain and it’s divided at the midline into two hemispheres by a short worm-like structure called the vermis.
The cerebellum is contained within the back of the skull, in an area known as the posterior fossa, and it sits above the foramen magnum, an opening at the base of the skull through which the spinal cord enters.
The cerebellum controls balance and posture, and helps to initiate as well as fine-tune voluntary motor activity - think about the fancy finger work of a piano player or the vocal cords of a singer - that’s the cerebellum in action.
Highest up, are two C-shaped lateral ventricles that lie deep in each cerebral hemisphere.
Dandy-Walker malformation (DWM) is a congenital brain defect in which the 4th ventricle fails to close. It is characterized with a cysts in the 4th ventricle, hypoplasia of the cerebellar vermis, and enlargement of the posterior cranial fossa. This condition results in hydrocephalus, increased intracranial pressure, and a characteristic "dandy walker" appearance on neuroimaging.
Symptoms of DWM vary depending on the degree of cerebellar hypoplasia. Patients may experience delayed milestones, hypotonia, ataxia, vomiting, and seizures. Hydrocephalus can cause enlargement of the head (macrocephaly) and bulging fontanelles.
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