Dandy-Walker syndrome can cause balance issues and difficulty controlling movements. It’s characterized by a small cerebellum and excess fluid in the space near the cerebellum.

Dandy-Walker syndrome (DWS) is a rare condition present at birth in which the cerebellum in the brain does not develop as expected. This can cause issues with balance and controlling movements.

For people with DWS, portions of the cerebellum may never grow or will be much smaller than typical. Extra fluid may also fill up in the spaces in the brain. This can lead to an increased head size and extra pressure on the brain, which is life threatening.

Sometimes, prenatal testing can detect DWS. Between 1 in 25,000 and 1 in 35,000 babies are born with DWS.

DWS is considered the same as Dandy-Walker complex, which can include several variants. Dandy-Walker malformation is one of these variants.

Illustration showing healthy brain vs. brain with enlarged ventricles (Dandy-Walker syndrome)Share on Pinterest
Healthy brain vs. brain with enlarged ventricles (Dandy-Walker syndrome). Illustration by Paul Lawrence

Symptoms of DWS in babies may include:

  • slow development of motor skills
  • difficulty controlling movements
  • large head size for their age

In older children, symptoms of DWS may include:

  • headaches and dizziness
  • vomiting
  • irritability
  • irregular eye movements
  • difficulty controlling movements and balancing

The exact cause of DWS is not fully understood. Sometimes, doctors can see certain anatomical changes during ultrasound in the second trimester.

Many genetic abnormalities have been linked to DWS.

In addition to genetics, other factors may increase the risk of DWS.

Large population-based studies found a slight increase in DWS associated with the following maternal factors:

  • diabetes developed during pregnancy
  • history of infertility
  • certain viral infections contracted during pregnancy
  • environmental exposure to toxins during pregnancy
  • being of non-Hispanic, African descent

These maternal factors are only associated with a slight increased risk of DWS. The chance of having a baby with DWS is still very low.

Although there’s no way to prevent DWS, you can talk with your doctor about anything to avoid during pregnancy and what you can do to promote healthy fetal growth.

What’s the difference between Dandy-Walker complex and DWS?

“Dandy-Walker complex” is an umbrella term for several types of central nervous malformations. This includes Dandy-Walker malformation, Dandy-Walker variant, mega cysterna magna, and posterior fossa arachnoid cyst.

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Doctors may identify a heightened chance for DWS if genetic testing identifies abnormalities.

DWS can sometimes be identified while a baby is still growing in the womb through ultrasounds or a fetal MRI.

Treatment for DWS focuses on managing structural changes in the brain and any symptoms. Early treatment and intervention are crucial to improve health outcomes and a better quality of life.

Doctors may recommend surgery to remove some of the extra fluid and relieve pressure on the brain. Special tubes or shunts are typically placed as part of this process.

Physical and occupational therapy can help with some of the physical challenges. Speech and language therapy can also be helpful in some cases.

Medications can help manage conditions such as seizures or ADHD as a result of DWS.

While some people with DWS experience physical and intellectual disabilities, others have no or mild symptoms.

The quality of life with DWS largely depends on:

  • the size of the cerebellum
  • how much the cerebellum has developed
  • whether any extra fluid is present in and around the brain
  • whether additional pressure is being placed on the brain

Some possible complications related to DWS include:

  • seizures
  • difficulties learning to walk
  • impaired balance
  • spinal changes
  • cognitive and learning difficulties

In addition to affecting quality of life, DWS may also shorten life expectancy. About 10–66% of people are reported to die from DWS, and 50% of those with untreated fluid in the brain die before age 3 years.

However, a 2015 analysis found fluid drainage surgery may reduce the risk of death from DWS by 44%. Researchers found the risk of death also decreases as a child ages.

Dandy-Walker syndrome (DWS) is a congenital condition in which the cerebellum in the brain does not develop as expected. This can cause issues with balance and controlling movements.

Surgery to reduce the amount of fluid in the brain and physical therapy may help manage some symptoms.

Detecting DWS can begin during early pregnancy. Sometimes, doctors can identify the condition in ultrasounds before a baby is born.

While there is no cure, doctors can treat some of its symptoms and help answer any questions about what life with this condition may look like.