Definition

What is Dandy-Walker Syndrome?

Dandy-Walker Syndrome is a congenital brain malformation involving the cerebellum (an area of the back of the brain that coordinates movement) and the fluid-filled spaces around it.

The key features of this syndrome are an enlargement of the fourth ventricle (a small channel that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord), a partial or complete absence of the area of the brain between the two cerebellar hemispheres (cerebellar vermis), and cyst formation near the lowest part of the skull. An increase in the size and pressure of the fluid spaces surrounding the brain (hydrocephalus) may also be present.

The syndrome can appear dramatically or develop unnoticed.

How common is Dandy-Walker Syndrome?

Dandy-Walker malformation is estimated to affect 1 in 10,000 to 30,000 newborns. It affects more females than males. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Dandy-Walker Syndrome?

Early symptoms:

  • Slow motor development
  • Progressive enlargement of the skull

Late symptoms:.

  • Irritability and vomiting due to increased intracranial pressure
  • Unsteadiness, lack of muscle coordination, or jerky movements of the eyes due to cerebellar dysfunction

Other symptoms:

  • Head circumference
  • Bulging at the back of the skull
  • Abnormal breathing problems
  • Problems with the nerves that control the eyes, face and neck.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If your children have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Dandy-Walker Syndrome?

Dandy-Walker Syndrome results from defects in early embryonic development of the cerebellum and surrounding structures. It may also occur as part of a genetic syndrome that includes multiple birth defects, such as the PHACES syndrome of facial hemangioma, heart and sternal defects and Dandy-Walker Syndrome.

Risk factors

What increases my risk for Dandy-Walker Syndrome?

Please discuss with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Dandy-Walker Syndrome diagnosed?

Dandy Walker malformation is diagnosed with the use of ultrasound, CT and MRI. Prenatal diagnosis of Dandy-Walker malformation is sometimes made by ultrasound or fetal MRI.

How is Dandy-Walker Syndrome treated?

Hydrocephalus associated with Dandy Walker syndrome is treated with surgery to insert a tube to redirect the fluid that surrounds the brain and to assist fluid drainage into other parts of the body that can absorb the fluid.

A supportive team approach for children with Dandy-Waller malformation is often warranted and may include special education, physical therapy and other medical, social or vocational services.

Genetic counseling is recommended for families that have a child with Dandy Walker malformation.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Dandy-Walker Syndrome?

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Sources

Review Date: May 22, 2018 | Last Modified: May 22, 2018

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