Dandy-Walker syndrome is a condition with how the brain forms before birth. Children often also have hydrocephalus, when the fluid in the brain can’t drain properly. Symptoms of Dandy-Walker include slow motor development and large head size. Treatment consists of a VP shunt to drain extra fluid and therapies to help children with daily tasks.
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.
Dandy-Walker involves the cerebellum and the spaces around it. The cerebellum is in the back of the brain, near the brainstem. It's part of the central nervous system. The cerebellum coordinates movements. It also helps control:
Dandy-Walker syndrome is named after the two neurosurgeons (brain surgeons) who described it in the 1900s, Walter Dandy, MD, and Arthur Walker, MD.
In Dandy-Walker syndrome, several conditions may occur:
In the United States, Dandy-Walker malformation affects about 1 in 25,000 to 35,000 babies. It affects more female babies than male babies.
Dandy-Walker happens when there’s an issue with the development of the baby’s cerebellum in the uterus. 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:
Sometimes, the symptoms appear suddenly and dramatically. Other times, symptoms may occur without parents noticing anything is wrong.
Symptoms tend to develop in the baby’s first few months of life, but some children don’t receive a diagnosis until age 3 or 4.
Symptoms in infants include:
Symptoms in older children include:
Other signs include:
Dandy-Walker complex is a group of disorders with similar symptoms. Dandy-Walker syndrome is one of the disorders included in Dandy-Walker complex.
Other disorders included in Dandy-Walker complex are:
Children with Dandy-Walker may have disorders of other parts of the central nervous system, including:
Fewer than half of children with Dandy-Walker have an intellectual disability. Intellectual disability most often occurs in children with Dandy-Walker who also have:
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:
Occasionally, providers diagnose this condition before a baby is born during a prenatal ultrasound or fetal MRI.
If you have a child with Dandy-Walker malformation, talk to your healthcare provider about genetic counseling. A small percentage of people with this condition have family members who also have it. Because there may be a genetic component, many providers recommend genetic testing.
Treatment is based on the symptoms resulting from Dandy-Walker syndrome. A careful evaluation by a healthcare provider is essential before initiating treatment. For example, healthcare providers may recommend:
There is no known way to prevent Dandy-Walker syndrome. Consistent prenatal care provides your best likelihood of a healthy pregnancy. Follow your provider’s recommendations for healthy living while you’re pregnant.
Your child’s outlook and life expectancy depend on how severe their condition is. Other congenital conditions also affect their prognosis.
People with the condition have a wide range of experiences. Some have minor symptoms. Others have profound disabilities. Some children can achieve typical cognitive abilities with the right treatment plan. Others do not, even when their care team diagnoses and treats the condition early.
Early intervention can help your child have the best chance at successful treatment. If you notice your child is not reaching their developmental milestones on time, such as not sitting, walking or talking, see your healthcare provider. They can provide an accurate diagnosis so your child can get the most effective treatment plan.
Your child’s Dandy-Walker care team may include:
If your child receives a diagnosis of Dandy-Walker, ask your provider:
A note from Cleveland Clinic
Dandy-Walker syndrome, or Dandy-Walker malformation, is a brain condition that develops before a baby is born. Babies with Dandy-Walker often don’t meet their developmental milestones. Some children need a shunt to drain excess fluid from the brain. Therapies can help children manage daily tasks, succeed in school and live a fulfilling life. If you notice your child has a large head size or does not seem to be sitting, walking or talking as expected, talk to your provider.
Last reviewed by a Cleveland Clinic medical professional on 04/19/2022.
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