Dandy-Walker Syndrome

(Dandy-Walker Malformation; Dandy Walker Syndrome; Familial Dandy Walker; Dandy Walker Malformation)

Definition

Dandy-Walker syndrome is a brain deformity that develops before birth. The deformity occurs in an area in the back of the brain. This area controls movement and cognitive learning. This syndrome can also affect fluid-filled chambers of the brain called ventricles. The ventricles may have an abnormal build-up of fluid.
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Causes

It is not clear what causes Dandy-Walker syndrome. There may be a genetic factor.

Risk Factors

Dandy-Walker syndrome may be inherited. If a parent has Dandy-Walker syndrome, the child has a higher risk of the condition. There are no other known risk factors.

Symptoms

Symptoms of Dandy-Walker syndrome often occur in infancy. Some may not appear until childhood. Most are found within the first year of life. Symptoms may include:
  • Increased head size
  • Bulging of the back of the skull
  • Impaired development of normal speech and language
  • Slow motor development
  • Irritability
  • Headache
  • Vomiting
  • Unsteadiness, especially with walking
  • Lack of muscle coordination
  • Jerky eye movements
  • Abnormal breathing
  • Trouble with vision or hearing
  • Jerking movements of the arms and legs or seizures
Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs.

Diagnosis

You will be asked about your child's symptoms and medical history. A physical exam will be done.Images will be taken of your child's brain. This can be done with:

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