Definition

Dandy-Walker syndrome is a brain deformity present at birth. The deformity involves the cerebellum, which is an area in the back of the brain that controls movement. The symptoms of this syndrome may develop suddenly or may go unnoticed.

Cerebellum

© 2008 Nucleus Medical Art, Inc.

Causes

Dandy-Walker syndrome occurs during brain development before birth. The reason the brain does not develop normally is not clearly understood.

Risk Factors

Aside from association with certain inherited genetic conditions, there are no known risk factors. The following factors are associated with Dandy-Walker syndrome, but do not increase the risk of it’s occurrence.

• Absence of the corpus callosum , which connects the brain's hemispheres
• Malformations of the heart, face, limbs, fingers, and toes

Symptoms

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

Diagnosis

Your doctor will ask about symptoms and medical history, and perform a physical exam. He or she will also likely perform a CT or MRI scan to view the inside of the brain.

Treatment

Talk with your 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.

Prevention

There is no known way to prevent Dandy-Walker syndrome.