Definition

Dandy-Walker syndrome is a rare brain deformity that happens before birth. It affects an area in the back of the brain that controls movement, thinking, and behavior. It also causes a buildup of fluid in ventricles (chambers) around it.

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Causes

The exact cause is not known. Genes may play a role.

Risk Factors

This problem may be more common in people who have a parent with it.

Symptoms

Problems may be:

• Increased head size
• Muscles that are very floppy or stiff
• Delayed development doing things like sitting up and walking
• Irritability
• Vomiting
• Jerky eye movements
• Problems with coordination
• Bulging of the back of the skull
• Breathing problems
• Seizures
• Problems controlling facial movements
• Learning problems

Children with this syndrome may also have other birth deformities of the brain, heart, face, or limbs.

Diagnosis

The doctor will ask about your child's symptoms and health history. A physical exam will be done.

Images may be taken of the brain. This can be done with:

• Ultrasound
• CT scan
• MRI scan

Treatment

There is no cure. The goal is to manage symptoms. This can be done with physical therapy and education services.

The buildup of fluid in the ventricles may need to be treated. Extra fluid can cause pressure and swelling in the brain. Fluid may be drained by:

• Shunt—A tube is placed from the brain to the abdomen to allow the extra fluid to drain out of the brain.
• Ventriculostomy—A connection is made from one ventricle to other areas that contain fluid. It allows extra fluid to drain out of the affected ventricle.

Prevention

There is no known way to prevent this syndrome.