Dandy-Walker Syndrome

Dandy-Walker syndrome is a condition that is present at birth (congenital). Dandy-Walker syndrome is when the brain is not formed correctly. This problem occurs at the back of the brain that controls movement (cerebellum). The key problems are:

  • An enlargement of a small channel that allows fluid to flow through the upper and lower areas of the brain and spinal cord (the fourth ventricle).

  • A partial or complete absence of the cerebellum.

  • Fluid-filled spaces around the cerebellum.

There are many different types of Dandy-Walker syndrome.

SYMPTOMS

The syndrome can appear dramatically or develop unnoticed. Symptoms, which often occur in early infancy, include slow development of movement skills (motor), poor coordination, loss of balance and jerky movements of the eyes. Other symptoms, which require an immediate evaluation by your health care provider, include rapidly increasing head size, a bulging soft spot on top of the head (fontanelle), decreased alertness, and vomiting. Dandy-Walker syndrome is frequently associated with other birth defects.

DIAGNOSIS

A computerized magnetic scan (magnetic resonance imaging, MRI) is a machine that uses a magnet and computer to get detailed images of the brain. Your caregiver may use an MRI to find Dandy-Walker syndrome. Based on the type of Dandy-Walker syndrome and other abnormalities in the body, further chemical or genetic testing may be done.

TREATMENT

Treatment for individuals with Dandy-Walker syndrome is symptomatic. This means that medication or therapy is given for symptoms that are causing problems. Common treatments include:

  • Physical therapy for problems with large muscle groups like the legs (gross motor).

  • Occupational therapy for problems with small muscle groups like the hands (fine motor).

  • Speech therapy for language problems.

  • Anticonvulsant medications to treat seizures.

PROGNOSIS

Children with Dandy-Walker syndrome may never have normal intellectual development, even when the excessive fluid in the brain is treated early. Longevity depends on the severity of the syndrome and other birth defects in the body.