Dandy Walker Syndrome, Causes, Diagnosis, Treatment, Prevention, Complications

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Dandy Walker Syndrome, Causes, Diagnosis, Treatment, Prevention, Complications

Dandy-Walker syndrome is a congenital disorder due to disturbances in the process of forming the cerebellum and the channels or cavities in the brain that contain fluid (ventricles). This disorder can make the sufferer experience an enlarged head size and slow growth and development.

The brain is divided into several parts, including the cerebellum and the fluid channels around the brain (ventricles). The cerebellum functions to maintain balance and coordinate body movements.

In Dandy-Walker syndrome, the formation of the cerebellum and ventricles of the brain is impaired. Given that the cerebellum has various functions in body movement, people with Dandy-Walker syndrome will experience disturbances in these functions.

Dandy-Walker syndrome or Dandy-Walker syndrome is very rare, ie 1 in 25,000 births each year. Generally, this disorder can be seen when the baby is born, but can also be seen in late childhood.

Causes of Dandy-Walker Syndrome

As already mentioned, Dandy Walker syndrome occurs due to disturbances in the formation of the cerebellum and ventricles of the brain. This condition occurs when the baby is still in the womb.

Some forms of formation disorders that can occur in Dandy-Walker syndrome are:

  • There is no space that separates the right brain and left brain
  • Enlargement of the 4th ventricle
  • Cyst formation in the cerebrospinal fluid
  • Cyst formation in the space near the cerebellum and brainstem

The exact cause of Dandy-Walker syndrome is not known. However, it is suspected that this disorder occurs due to genetic mutations or certain chromosomal abnormalities.

Dandy-Walker Syndrome risk factors

Although the exact cause is not known, there are several factors that are thought to increase the risk of Dandy-Walker syndrome, namely:

  • Certain viral infections when the mother is pregnant so that the fetus is infected
  • Exposure to chemicals and toxins that can cause defects in the fetus when the mother is pregnant
  • Certain diseases, such as diabetes, during pregnancy

Symptoms of Dandy-Walker Syndrome

Symptoms of Dandy-Walker syndrome can vary from person to person. Symptoms can appear at birth or in late childhood.

Some of the common symptoms of Dandy-Walker syndrome that can appear are:

  • Accumulation of excess fluid in the brain (hydrocephalus) so that the baby's head enlarges
  • Child development disorders
  • Impaired contraction of limb muscles
  • Balance disorders
  • Difficulty doing light activities, such as writing and holding objects
  • Repetitive and sudden eye movements (nystagmus)
  • Seizures due to increased pressure in the head cavity (intracranial)

In infants, Dandy-Walker syndrome can also appear accompanied by disorders of the formation of other body organs, such as the heart, face, limbs, and digestive tract. Patients with this syndrome can also have nervous system disorders, such as the absence of the neural network that connects the left brain and right brain.

When to go to the doctor

Symptoms of Dandy-Walker syndrome are generally seen since the baby is born. Immediately see a doctor if your child has any of the above-mentioned complaints. Early examination needs to be done to prevent complications.

If you are pregnant, have regular prenatal checkups, ultrasounds, and genetic tests. The goal is to help doctors detect Dandy-Walker syndrome early. If your child is diagnosed with this disease, the doctor will immediately get treatment after birth.

If your child is diagnosed with Dandy-Walker syndrome, have regular check-ups and follow treatment as recommended by the doctor so that the child's condition is monitored.

Diagnosis of Dandy-Walker Syndrome

To diagnose Dandy-Walker syndrome, the doctor will ask questions about the symptoms experienced, family and child health history, and maternal health history during pregnancy.

The doctor will also perform a thorough physical examination, especially on the head circumference and the child's growth and development.

To confirm the diagnosis, the doctor will perform several follow-up examinations, such as:

  • Ultrasound of the head, to detect abnormalities in the formation of the child's brain
  • CT scan or MRI of the head, to see the condition of the brain more clearly

If Dandy-Walker syndrome has been detected during pregnancy, some tests that can be done are:

  • Ultrasound of the womb, to see the condition of the fetus, including by seeing if there are abnormalities in the fetus
  • Fetal MRI, to detect organ abnormalities in the fetus

Dandy-Walker Syndrome Treatment

Treatment of Dandy-Walker syndrome aims to control symptoms and prevent complications. Treatment that can be done depends on the severity of the disorder experienced by the child. The sooner the child's condition is treated, the better the child's quality of life will be.

Some of the treatment methods that can be done are:

  • Installation of a ventriculoperitoneal (VP) shunt
This procedure is performed on children who have increased intracranial pressure. The doctor will insert a small device called a VP shunt to drain the excess fluid so the pressure will be reduced.
  • Therapy
To help the child in his activities, the doctor will recommend several types of therapy based on the disorder suffered. Some of the therapies that can be done are physical therapy (physiotherapy), occupational therapy, and speech therapy.
  • Special education
To get the right education and social activities, doctors will recommend parents to send their children to special schools (SLB). This school will provide teaching according to the condition of the child.
  • Drugs
Doctors can prescribe anti-seizure medications to control seizure symptoms in children.

Complications of Dandy-Walker Syndrome

If not treated immediately, Dandy-Walker syndrome can cause several complications, namely:

  • Developmental disorders
  • Impaired cognitive function so that the learning process is not optimal
  • Mental retardation
  • Disability

Increased intracranial pressure can also cause continuous seizures (status epilepticus) to brain damage.

Dandy-Walker Syndrome Prevention

There is no way to prevent Dandy-Walker syndrome. However, ask your doctor about the need for genetic testing if the mother has given birth to a child with Dandy-Walker syndrome or has a family history of this syndrome. Genetic testing can provide information on how much risk your child has Dandy-Walker syndrome.

Other efforts that expectant mothers and pregnant women can do to avoid risk factors for Dandy-Walker syndrome are:

  • Vaccination before pregnancy to prevent transmission of certain diseases to the fetus
  • Undergo a TORCH check before getting pregnant
  • Do not take drugs without a doctor's advice, especially drugs that are dangerous for pregnant women

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