Dandy-Walker Syndrome

By: Morgan Rawls

What is it?

Dandy-Walker Syndrome 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.

Causes

The exact cause of Dandy-Walker syndrome is not known. It is likely a combination of genetic and environmental factors, although familial occurrence has been noted.

Symptoms

-Slow motor development

-Progressive enlargement of the skull

-Increased intracranial pressure such as irritability and vomiting

-Signs of cerebellar dysfunction such as unsteadiness, lack of muscle coordination, or jerky movements of the eyes

-Increased head circumference

-Bulging at the back of the skull

-Problems with the nerves that control the eyes, face and neck

-Abnormal breathing patterns

  • -Nausea and vomiting
  • -Convulsions
  • -Deformities may be present on the patient’s face, limbs, extremities, and heart
  • Treatment

    Treatment for individuals with Dandy-Walker Syndrome generally consists of treating the associated problems, if needed. A surgical procedure called a shunt may be required to drain off excess fluid within the brain. This will reduce intracranial pressure and help control swelling.

    Parents of children with Dandy-Walker Syndrome may benefit from genetic counseling if they intend to have more children.

    Life expectancy

    The effect of Dandy-Walker Syndrome on intellectual development is variable, with some children having normal cognition and others never achieving normal intellectual development even when the excess fluid buildup is treated early and correctly. Longevity depends on the severity of the syndrome and associated malformations.

    The presence of multiple congenital defects may shorten life span.

    Key features

    -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)

    -Acyst formation near the lowest part 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

    Diagnosis

    Dandy Walker Syndrome can be diagnosed through the following examinations:

    MRI – This has been noted as one of the best diagnostic exam for neurological or CNS related conditions.

    CT scan – This procedure assists in the diagnosis of Dandy Walker syndrome. There are 3D studies that are good in the evaluation process of DWS.

    Angiography –This can demonstrate or provide the features of malformations of Dandy Walker syndrome.

    Ultrasonography – The malformation can be identified through the antenatal ultrasound.

    Research

    The NINDS conducts and supports a wide range of studies that explore the complex mechanisms of normal brain development. The knowledge gained from these fundamental studies provides the foundation for understanding abnormal brain development and offers hope for new ways to treat and prevent developmental brain disorders such as Dandy-Walker Syndrome.
    Big image
    Big image
    Big image