What is DandyWalker Syndrome?

Written by Medicover Team and Medically Reviewed by Dr Rohit Ramesh Wadikhaye , Neurosurgeon


Dandy-Walker Syndrome is a brain problem that affects a part called the cerebellum. This area helps with movement and coordination. In this condition, the middle part of the cerebellum called the vermis, doesn't form properly. There's also a space in the brain that becomes enlarged, leading to pressure due to fluid buildup (hydrocephalus). These changes affect how a child moves, grows, and learns.

How Common Is It?

Dandy-Walker Syndrome is very rare. It affects about 1 in every 25,000 to 35,000 newborns. It can happen in both girls and boys and may appear with other brain or genetic problems. Often, families don't know about it until symptoms start to show or doctors find it during a scan.


What are the Types of Dandy-Walker Syndrome?

Dandy-Walker Syndrome can appear in different forms, depending on how the brain structures are affected. Each type varies in severity and symptoms. Understanding the type helps doctors choose the right care and predict possible challenges in growth and development.

  • Classic Dandy-Walker Malformation
  • Full or partial absence of the cerebellar vermis
  • Enlarged fourth ventricle
  • May have hydrocephalus
  • Dandy-Walker Variant
  • Milder changes in the cerebellum
  • Mega Cisterna Magna
  • A larger fluid space in the back of the brain
  • Normal cerebellum and fourth ventricle

Doctors will decide which type is present based on brain scans and developmental signs.


What are the Symptoms of Dandy-Walker Syndrome?

The symptoms can vary from one child to another. Some babies are born with a large head or soft spots that bulge. Others may be slow to sit, crawl, or walk. There can also be balance problems, weak muscles, seizures, eye movement issues, or speech delays. The severity depends on how much the brain is affected.

Common symptoms include

  • A large head or a head that grows too fast
  • A bulging soft spot (fontanelle) on the top of the baby's head
  • Vomiting or fussiness due to pressure in the brain
  • Poor muscle tone or stiffness
  • Trouble with balance and coordination
  • Delayed milestones, like sitting, crawling, or walking
  • Problems with eye movement
  • Seizures (in some cases)
  • Slow development of speech and learning skills

In mild cases, children may grow and develop normally for a while before symptoms appear.


What are the Causes and Risk Factors of Dandy-Walker Syndrome?

This condition starts early in pregnancy when the brain is forming. The exact cause is often unknown. Some cases are linked to changes in genes, especially if the child has a condition like Trisomy 13, 18, or Down syndrome.

Genetic Causes

Some cases are linked to changes or mutations in genes. It may also be part of a larger syndrome, like:

  • Trisomy 13
  • Trisomy 18
  • Trisomy 21 (Down syndrome)

Pregnancy Complications

  • Infections during pregnancy, like rubella or cytomegalovirus, may increase the risk.

Environmental Factors

  • Exposure to harmful substances, like alcohol, certain drugs, or radiation, might affect brain development.

Other causes may include infections during pregnancy or exposure to harmful substances like alcohol or certain medicines. In many cases, there is no clear reason.


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How is Dandy-Walker Syndrome Diagnosed?

Sometimes, signs of Dandy-Walker Syndrome are seen during a pregnancy ultrasound. After birth, doctors use tests like MRI or CT scans to see if the cerebellum and brain spaces are normal. They may also do genetic tests or check how the child is growing and developing through simple physical exams.

After birth, the diagnosis may involve

  • MRI or CT scan: These imaging tests show the brain's structure and check for enlarged fluid spaces.
  • Ultrasound of the brain: Often used in newborns.
  • Genetic testing: To check for related syndromes.
  • Neurological exams: To check reflexes, movement, and development.

Doctors can sometimes see signs of Dandy-Walker Syndrome before birth during a prenatal ultrasound. If brain structures look unusual, more tests may be done.


What are the Treatment options for Dandy-Walker Syndrome?

Treatment focuses on managing symptoms and helping a child grow and learn as best as possible. It often includes a mix of surgery, therapy, and regular medical care. Early treatment can greatly improve a child's quality of life.

Managing Brain Pressure

If there is too much fluid in the brain (hydrocephalus), surgery may be needed to place a shunt. This is a thin tube that drains excess fluid from the brain to another part of the body.

Therapies and Support

  • Physical therapy: To improve balance, strength, and movement
  • Occupational therapy: To help with daily activities
  • Speech therapy: If speech is delayed or unclear
  • Special education support: For learning and development

If the child has seizures or other conditions, medications may be prescribed.


When to See a Doctor?

Parents should see a doctor if their baby has a very large head, slow development, trouble moving, or unusual eye movements. If the child seems very sleepy, fussy, or vomits often, this could mean too much brain pressure.

You should speak with a doctor if:

  • Your baby has an unusually large head
  • Developmental milestones are delayed
  • Your child seems very fussy, vomits often, or has trouble balancing
  • There's a family history of brain malformations
  • Early diagnosis and support can make a big difference in how well a child does.

Early medical care can prevent complications and improve outcomes.


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Can Dandy-Walker Syndrome Be Prevented?

Since it begins in early pregnancy, prevention can be hard. But good prenatal care can lower the chances of brain problems. This includes taking vitamins like folic acid, avoiding alcohol or drugs, treating infections, and talking to a doctor if there's a family history of birth defects or genetic conditions.

  • Getting regular prenatal care
  • Avoiding alcohol, drugs, and smoking
  • Protecting against infections like rubella (get vaccinated before pregnancy if needed)
  • Taking prenatal vitamins with folic acid
  • Discussing family history with a doctor or genetic counselor.

Our Experience in Treating Walker Syndrome

Our Experience in Treating Walker Syndrome at Medicover. At Medicover, we offer expert care for children with Walker Syndrome through a team-based, compassionate approach.

  • We use advanced MRI and neurological exams to confirm the condition early.
  • Pediatric neurologists, neurosurgeons, therapists, and psychologists work together to provide complete care.
  • Children receive personalized physiotherapy, speech therapy, and occupational therapy to support development.
  • When needed, our skilled neurosurgeons perform shunt surgery for hydrocephalus with precision and safety.
  • We offer regular follow-ups, developmental tracking, and family counseling for ongoing care.

With Medicover, you're never alone. We're here to support your child's health, growth, and future.


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Frequently Asked Questions

It varies some children have normal intelligence while others experience developmental and cognitive delays.

The brain's fluid buildup causes pressure that may affect brain function and requires shunt surgery.

Yes, prenatal ultrasound or MRI can identify the condition as early as the second trimester.

Yes, it can be associated with genetic disorders like trisomy 13, 18, or other malformations.

The condition is congenital, but symptoms and complications may evolve as the child grows.

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