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Dandy-Walker Syndrome - Causes, Symptoms, Diagnosis, and Treatment

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Dandy-walker syndrome primarily affects the cerebellum of the brain that helps coordinate bodily movements. Read the article below to know more in detail.

Written byDr. Anuthanyaa. R

Medically reviewed byDr. Abhishek Juneja

Published At July 28, 2022
Reviewed AtNovember 29, 2022

What Is Dandy-Walker Syndrome?

Dandy-walker syndrome, also known as dandy-walker malformation, is a rare type of congenital brain malformation. It mainly involves the cerebellum and the space that surrounds it. The incidence of dandy-walker malformation is infrequent and occurs only in 1 out of 25,000 to 35,000 births. This condition leads to abnormal development of the cerebellum, which differs from one person to another. It appears suddenly, and in the majority of the cases, we would not be able to notice that the child has the dandy-walker syndrome.

Since the brain is the most crucial part of the human body, any malformation in the brain leads to troubled growth and impaired motor and sensory development. There have been some common malformations that have been associated with the dandy-walker syndrome.

They are:

  1. The enlargement of the space in the brain prevents the flow of brain fluid from the upper and lower part of the brain to the spinal cord.

  2. Shunted development or absence of the central part of the cerebellum, called the vermis.

  3. A cyst-like formation near the base of the skull is very large, to be exact, the place where the cerebellum and the brainstem are located.

  4. Very little or no development of the left and right sides of the cerebellum.

  5. An enlarged fluid space might also lead to increased pressure around the brain.

The cerebellum of our brain is responsible for many bodily functions, including vision, balance, posture, mobility, etc. They also collect and send information from the brain to other body parts, such as the spinal cord, cerebrum, and brain stem. Hence, people affected by dandy-walker syndrome might find it very hard to walk and have proper balance. They also have involuntary muscle movement and experience trouble coordinating their feet and hand movement.

What Are the Causes of Dandy-Walker Syndrome?

Dandy-walker syndrome occurs very early during the pregnancy when the cerebellum begins to form and develop. This condition starts to initiate when the structures around the cerebellum or the cerebellum itself fail to develop adequately. It has been observed that most people affected by dandy-walker syndrome have defects or abnormalities in their chromosomes that are directly linked to the condition. However, upon full investigation and proper in-depth research, it has been found that dandy-walker syndrome is caused by genetic components that are highly complex and also due to long exposure to environmental factors that cause other congenital disabilities.

What Are the Symptoms of Dandy-Walker Syndrome?

In most cases, the dandy-walker syndrome will be identified at the time of birth or probably within a year. However, in some rare cases, symptoms might not be exhibited until early adulthood or late childhood, and this accounts for about ten to twelve percent of the total cases. Though symptoms of dandy-walker syndrome differ from one person to another, some of the key symptoms of the condition are:

  1. Increased head size (in terms of circumference) as a result of hydrocephalus.

  2. Developmental troubles during infancy.

In addition, some common symptoms associated with the dandy-walker syndrome are as follows.

  1. Delayed or Slowed Motor Development: Babies with the dandy-walker syndrome have trouble coordinating muscle movements, leading to delay in motor skills such as crawling, walking, and balancing themselves.

  2. Increased Intracranial Pressure: The buildup of fluid in the spaces between the brain and skull might increase the intracranial pressure. Though this is hard to detect during early childhood, certain factors such as double vision, irritability, and bad mood might help to detect this symptom later in life.

  3. Enlargement of the Skull: Due to the buildup of fluid, the circumference of the skull can increase over a period of time. There is also a possibility of causing a bulge at the base of the skull.

  4. Seizures: It has been observed that almost 25 % - 30 % of people with dandy-walker syndrome experience seizures.

symptoms

What Are the Risk Factors of Dandy-Walker Syndrome?

Dandy-walker malformation develops early at the time of pregnancy, so the state of the mother's health plays a major role in the occurrence of this syndrome. Research has shown that the risk of the dandy-walker syndrome is high for the child if the mother has diabetes. In addition, family members who are an immediate circle of the affected person have a high chance of developing the dandy-walker syndrome. It does not mean that it is highly inheritable, and in fact, the rate of occurrence for a sibling of the affected person is only five percent.

How Is Dandy-Walker Syndrome Diagnosed?

Dandy-walker syndrome can be visually diagnosed during the time of birth or within a year and is marked by enlarged head size. If the doctor suspects dandy-walker syndrome, then imaging tests might help in confirming the diagnosis by identifying any abnormalities or malformation of the brain. These imaging tests include:

  1. Computed tomography (CT) scan.

  2. Ultrasound of the head.

  3. Magnetic resonance imaging (MRI).

How Is Dandy-Walker Syndrome Treated?

The treatment of dandy-walker syndrome is based on the patient's severity. It also depends on the level of incoordination and the complexity of the developmental issues that the patient is experiencing. Though it might interfere with regular everyday life, treatment is not required in case of a mild condition. The treatment options for dandy-walker syndrome include:

  1. Surgery: In case of increased intracranial pressure, the doctors recommend surgical placement of a shunt in the skull to avoid the fluid buildup and help re-route the fluid to other parts of the body. This allows the fluid to get easily absorbed and decreases the pressure within the skull.

  2. Therapies: A wide range of therapies are used to help the patient deal with the symptoms associated with a dandy-walker syndrome, such as coordination of hands and feet, body balance, controlling the involuntary muscle responses, etc.

The rate of survival and the prognosis of the condition is purely dependent on the severity and the complexity of the condition, which varies from one person to another.

Conclusion:

Similar to other brain-related conditions, the dandy-walker syndrome is also quite scary. However, with the help of various educational and occupational therapies, people suffering from dandy-walker syndrome can lead happy and healthy lives. If you are someone suffering from the dandy-walker syndrome, it is important to be hopeful and confident as it can be managed.

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Frequently Asked Questions

The life expectancy of Dandy-Walker syndrome varies according to the severity of the condition and the presence or absence of hydrocephalus. The symptoms may range from mild in some to severe in others. Mostly the affected make it to adulthood. Studies show a chance of ten to 66 percent mortality associated with the condition, but drainage surgery reduces mortality by 44 percent.
The malformation complex triad of Dandy-Walker syndrome consists of the following:
- Partial or complete vermis agenesis (vermis is the space between the cerebellar hemispheres).
- Enlarged posterior fossa (skull base) with upwardly displaced tentorium, lateral sinuses, and torcular Herophili.
- Cystic dilatation of the fourth ventricle.
- Mostly the malformation triad is found along with supratentorial hydrocephalus, which is considered a complication of the condition.
Dandy-Walker syndrome is a birth defect (congenital defect) resulting in malformation of the cerebellum. Studies show that 71 percent of those with Dandy-Walker syndrome have an intelligence quotient (IQ) less than 83 (subnormal mental development).
The treatment for Dandy-Walker syndrome involves a multidisciplinary approach and includes physical therapy, occupational therapy, medication for seizures, speech therapy, special education, and vocational services. If hydrocephalus is present, drainage surgery is done to redirect the fluid surrounding the brain to other body parts that absorb the fluid. In families who have a child with  Dandy-Walker syndrome, genetic counseling is conducted.
Neural tube defects are a group of neurological disorders that affect the brain and spinal cord resulting in its malformation. Dandy-Walker syndrome may occur alone or with other congenital defects, including neural tube defects.
Dandy-Walker syndrome is a birth defect (congenital defect) resulting in malformation of the cerebellum. In some of the affected, chromosomal abnormalities have been reported and are mostly seen as deletion of chromosome 3q24.3, 13q32.2-q33.2, or 6p25, or duplication of chromosome 9p.
Ultrasound imaging is one of the diagnostic tests for Dandy-Walker syndrome. Ultrasound imaging shows the following findings:
- Complete or partial absence of cerebellar vermis.
- Enlargement (greater than or equal to 10mm) of cisterna magna.
- Trapezoid-shaped space between cerebellar hemispheres.
However, an antenatal ultrasound may give a false diagnosis if performed before eighteen weeks, as the vermis is not fully developed in this stage.
While there is a potential overlap in symptoms between Dandy-Walker and Cerebral Palsy, Dandy-Walker Syndrome (DWS) itself does not typically cause Cerebral Palsy (CP). However, children with DWS may be at a higher risk of developing CP if they experience brain damage or other complications during birth or infancy.
There is no permanent cure or treatment for Dandy-Walker syndrome. Treatment options vary according to the need, symptoms, and difficulties of the affected. The symptoms vary, and the severity also varies among the affected. Some of the affected may achieve the required cognitive development with proper treatment, but some do not, even after early diagnosis and proper treatment.
Dandy-Walker syndrome can result in delays in the development of motor and language skills, balance and coordination issues, poor muscle tone, vision impairment, hearing impairment, and problems with eye movement. Treatment varies from one individual to another depending on the nature of speech and language disorders and mainly focuses on the development of communication skills.
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Dr. Abhishek Juneja from iCliniq
Dr. Abhishek Juneja

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