Dandy Walker Syndrome

Dandy-Walker syndrome is sometimes called Dandy-Walker malformation or just Dandy-Walker. It’s a congenital brain malformation that causes an issue with how the brain forms. It is congenital meaning a baby is born with the condition, and it occurs as the baby develops during pregnancy.

Causes of Dandy Walker Syndrome #

• Dandy-Walker happens when there’s an issue with the development of the baby’s cerebellum in the womb.

• In some cases, a genetic mutation (change) may cause the condition.

• Some people with Dandy-Walker have chromosomal conditions, such as extra or missing pieces of chromosomes.

• Chromosomes are packages of DNA that carry genes.

• Dandy-Walker syndrome may also happen as part of a genetic disorder that includes several birth defects (congenital conditions).

Other potential causes of Dandy-Walker may include:

• Certain viruses that pass from the birthing parent to baby during pregnancy.

• Exposure to certain toxins or medications during pregnancy.

• The birthing parent had diabetes.

Symptoms of Dandy Walker Syndrome #

Symptoms in infants include:

• Meeting motor milestones later than expected (developmental delays).

• Disproportionately large skull.

• Low muscle tone (hypotonia).

• Stiff muscles (spasticity).

Symptoms in older children include:

• Vomiting, convulsions and irritability signs of increased pressure on the brain.

• Uncoordinated movements, unsteadiness or jerky eye movements signs of conditions with the cerebellum.

Other signs include:

• Bulge or swelling at the back of the skull.

• Conditions with the nerves that control the neck, face and eyes.

• Unusual breathing rhythm.

• Seizures.

• Intellectual disabilities.

• Symptoms of hydrocephalus.

Diagnosis of Dandy Walker Syndrome #

Healthcare providers or parents may notice that a child has increased head size. A child may also not meet developmental milestones. Providers order brain imaging to diagnose Dandy-Walker. Tests may include:

• Ultrasound.

• CT.

• MRI.

Treatment of Dandy Walker Syndrome #

Ventriculoperitoneal (VP) shunt #

Surgeons place a VP shunt, a small device, to drain excess fluid from the brain. The shunt can reduce pressure on the brain and improve symptoms.

Medications #

Your child’s provider may prescribe medications to control seizures.

Therapy #

Physical and occupational therapy can help children maintain muscle strength. Therapists can also teach children new methods of doing their regular activities. Speech therapy can help with language and speech development.

Special education #

The right learning environment can help children reach educational and social goals.