ConnectABILITY

Dandy-Walker Syndrome

Fact Sheet

What is Dandy Walker Syndrome?

Dandy-Walker Syndrome (DWS) is a congenital brain malformation involving the cerebellum (an area at the back of the brain that controls 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 cerebellar vermis (the area between the two cerebellar hemispheres), and cyst formation near the internal base of the skull. An increase in the size of the fluid spaces surrounding the brain as well as an increase in pressure may also be present.

Dandy-Walker Syndrome is frequently associated with disorders of other areas of the central nervous system including absence of the corpus callosum (the connecting area between the two cerebral hemispheres), and malformations of the heart, face, limbs, fingers and toes.

How is it manifested?

Symptoms of Dandy-Walker Syndrome often occur in infancy but can also occur in older children. Symptoms may include:

  • Impaired development of normal speech and language
  • Slow motor development
  • Irritability
  • Vomiting
  • Convulsions
  • Unsteadiness
  • Lack of muscle coordination
  • Jerky eye movements
  • Increased head circumference
  • Bulging of the back of the skull
  • Problems with the nerves that control the eyes, face, and neck
  • Abnormal breathing
  • head circumference or progressive enlargement of the skull

Who is affected?

  • The incidence is approximately 1 in 2,500 newborns.
  • DWS occurs more frequently in females than males

Diagnosis:

The majority of individuals with DWS are diagnosed in their first year of life as a result of the commonly associated hydrocephalus. However, a definitive diagnosis can be made by ultrasound, CT-scan, or MRI (allows a detailed evaluation of Dandy-Walker malformation lesions and complications. MRI evaluation can then be used antenatally to confirm the diagnosis and gain more detailed information).

Treatment:

Talk with your child’s doctor about the best treatment plan. Treatment will depend on the problems caused by the syndrome. This may involve placing a special tube called a shunt inside the skull to drain excess fluid to reduce pressure and help control swelling.

Additional Resources:

Spina Bifida and Hydrocephalus of Ontariowww.sbhao.on.ca
Spina Bifida and Hydrocephalus (SB&H) has grown to provide a comprehensive and essential range of services for parents, families, youth and adults with SB&H. Their programs are rooted in the principles of self-help and personal support. They also serve the broader community which includes parents who receive a pre-natal diagnosis of SB&H, extended family members, and all women of child-bearing age. The benefits of folic acid in the prevention of neural tube defects are stressed and a wide spectrum of professionals in the social services, medical, health and education fields are identified. SB&H is the only organization in Ontario addressing the particular needs of their members.

Dandy Walker Alliancewww.dandy-walker.org
This website is an effort to pull together a compilation of reliable and medically-accurate resources that have been gathered over the years.

Yahoo! Groupshttp://groups.yahoo.com/group/dandywalker/
An open forum is provided for anyone affected by a diagnosis of DWS. Information is shared in an attempt to inform and educate a person with this diagnosis and to save time on research.

The Official Parent’s Sourcebook on Dandy-Walker Syndrome: Updated Directory for the Internet Age – Authors: Icon Health Publications
This sourcebook is organized into three parts. Part I explores basic techniques to researching Dandy-Walker Syndrome (e.g. finding guidelines on diagnosis, treatments, and prognosis), followed by a number of topics, including information on how to get in touch with organizations, associations, or other patient networks dedicated to DWS.

The content contained in this document is for general information purposes. It is not intended to diagnose or treat a child.