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Dandy-Walker Syndrome

Dandy-Walker Syndrome belongs to the category of birth defects known as brain/spinal cord defects.

What it is:

Dandy-Walker Syndrome is a congenital malformation of the brain that can develop shortly after birth or progress more slowly and undetectably in early childhood. It is characterized by an abnormally developed cerebellum, which is the area at the back of the brain that controls movement. Typically, the condition includes:

  • Enlargement of the fourth ventricle (a fluid-filled cavity of the brain)
  • A partial or complete absence of brain tissue between the brain’s two hemispheres
  • A cyst located in the back of the brain
  • Presence of hydrocephaly (an excess of cerebrospinal fluid on the brain)

Dandy-Walker Syndrome is found in about one in every 25,000 births and is slightly more common in females than males. It often occurs alongside other congenital defects, such as malformations of the heart, face, fingers, toes or limbs. About half of patients suffering from Dandy-Walker Syndrome possess a below-normal IQ, and roughly 15% experience seizures.

Symptoms:

In babies:

  • Delayed motor development
  • Enlargement of the cranium

In older children:

  • Jerky eye movement
  • Double vision
  • Unsteadiness/ lack of coordination
  • Irritability
  • Vomiting
  • Abnormal breathing
  • Bulge at the back of the skull

Causes:

Clomid: A major study by the Centers for Disease Control and Prevention (CDC) found that mothers who took the fertility drug Clomid shortly before or during pregnancy were more than four times more likely to deliver a baby with Dandy-Walker Syndrome.

Treatment

Treatment of Dandy-Walker Syndrome usually involves managing the symptoms associated with the condition. To treat the hydrocephaly that occurs in 90% of cases, a shunt is usually placed in the baby’s brain to drain excess fluid and relieve pressure on the brain. Patients who experience seizures may be prescribed medication to manage their condition.

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