What is Dandy-Walker Syndrome?

Dandy-Walker Syndrome is a rare congenital malformation that involves the cerebellum and fourth ventricle. The cerebellum is the portion of the brain controlling balance, posture and the voluntary movement of muscles. While many additional problems may be present, Dandy Walker is recognized by three main features: the agenesis (non-development) or hypoplasia (under-development) of the cerebellar vermis; cystic dilation of the fourth ventricle; and enlargement of the posterior fossa.

Medscape.com lists 41 other serious medical conditions commonly associated with a diagnosis of Dandy-Walker Syndrome. These include Hydrocephalus (in 70% -90% of patients, usually developing after birth), Agenesis of the Corpus Callosum (20%-25% of patients), numerous other brain abnormalities, cardiac anomalies, Spina Bifida, increased pressure in the brain, Meckel-Gruber syndrome, microcephaly (abnormally small head), brainstem compression, polycystic kidneys, and malformations involving the face, limbs, fingers and toes.

The prognosis and life-expectancy of Dandy-Walker Syndrome patients depends largely on how severely they’re affected by the syndrome, and which associated medical conditions they have.

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A Heartbreaking Choice parental stories involving Dandy-Walker Syndrome