What is Dandy Walker Syndrom ?

Dandy Walker SyndromeDandy-Walker Syndrome is a congenital developmental disorder of the brain that can have various causes.In the disease, the fourth cerebrospinal fluid chamber (ventricle) is enlarged and the chamber extends to the posterior cranial fossa. In addition, the cerebellum is often malformed.

Dandy-Walker syndrome can be highly different, so that the symptoms can manifest itself quite different.Approximately 90 percent of these patients have an increased intracranial pressure (Hydrocephalus or “Water on the brain”). More cerebral malformations can occur.

Possible symptoms of high intracranial pressure are;

  • Blurred vision
  • Nausea
  • Vomit

The Dandy-Walker syndrome occurs alone or as part of complex medical conditions.Doctor can diagnose the disease shortly after birth through an ultrasound (sonography) in the newborn. Further investigations such as computed tomography (CT) or magnetic resonance imaging (MRI) can confirm the diagnosis.

If the disease is detected and treated, the prognosis is good. A causative treatment  is not yet available, the treatment focuses primarily on relieving the symptoms. Physician may insert a small plastic tube into the cerebrospinal fluid chamber so that excess cerebrospinal fluid flows and the intracranial pressure decreases.

It is not always possible to prevent the Dandy-Walker syndrome. Avoidable risks to a malformation of the brain are alcohol consumption and a rubella infection during pregnancy. Against rubella there is an effective vaccination.

Dandy-Walker syndrome  Definition

The Dandy-Walker Syndrome is a congenital developmental disorder of the brain. It is characterized by:

  • A malformation of the cerebellum: The cerebellar vermis (vermis), so the connection between two halves of the cerebellum is missing or underdeveloped.
  • An extension of the fourth cerebral fluid chamber (ventricle): The fourth cerebrospinal fluid chamber expands into the posterior cranial fossa and so there cerebrospinal fluid (CSF) accumulates and hydrocephalus occurs (is also called “water on the brain”.).

Other names for this developmental disorder are:

  • Dandy-Walker syndrome
  • Dandy-Walker malformation
  • Dandy-Walker cyst
  • Dandy-Walker complex

The disease was first described in 1914 by the neurosurgeon Walter Dandy and is defined further by the neurologist Arthur Earl Walker in 1942. Dandy-Walker name is introduced in 1954.

Dandy Walker Syndrome Causes

dandy-walker-syndrome-causesThe Dandy-Walker syndrome is the result of a developmental disorder that occurs during pregnancy. It causes the roof of the fourth cerebral fluid chamber (ventricle) expands the embryo. What causes this developmental disorder is unknown in most cases. However, there are several factors that increase the risk of malformations ;

Genetic factors, such as various rare inherited disorders, e.g .:

  • Joubert syndrome
  • Meckel-Gruber syndrome
  • Ehlers-Danos syndrome

Non-genetic factors, e.g .:

  • Rubella infection during pregnancy
  • Alcohol consumption during pregnancy

Dandy Walker Syndrome Symptoms

The Dandy-Walker syndrome can manifest itself through different symptoms.

How strong the symptoms are pronounced depends on many factors. For example, it plays a role in whether is called “water on the brain” (hydrocephalus) is present. It is also crucial if the Dandy-Walker syndrome is present as a single malformation or whether there are more faults are present.

In early childhood, the Dandy-Walker syndrome is mainly due to a large head circumference.

Symptoms are ;

  • Headache
  • Nausea
  • Vomit
  • Disturbances of consciousness
  • Blurred vision
  • Seizures

The increased intracranial pressure can also affect the function of the cerebellum. Then there is mainly:

  • Movement and balance disorders (ataxia)
  • Nystagmus (nystagmus) and
  • Speech disorders.
  • The mental development is often impaired in Dandy-Walker syndrome. But the malformation causes symptoms by affecting the cerebellum only about 15 percent of those affected.

In addition, abnormalities of the heart may occur as part of the Dandy-Walker syndrome.

Diagnosis of Dandy Walker Syndrome

Doctor can usually recognize the Dandy-Walker syndrome in newborns shortly after birth using an ultrasound (sonography). This reveals whether the cerebellum falseformed or the fourth ventricle is enlarged. A computed tomography (CT) or magnetic resonance imaging (MRI) can confirm the diagnosis.

There is no causative treatment for Dandy-Walker syndrome. Diseased children regularly must be medically examined to detect if there is an increased intracranial pressure or not .

İt is also better to get physiotherapy exercises and a therapy can help with anti-epileptic drugs.

Dandy-Walker Syndrome Prevention

Since the cause of Dandy-Walker syndrome is usually unknown, it is difficult to prevent the disease. However, there are two factors that can increase the risk of developing disorder.It can be actively done someting on these factors.

  • Rubella infection during pregnancy: There is an effective vaccine for Rubella infection. As in pregnancy can not be vaccinated against rubella, women should early take care of to have their vaccination or check the vaccination records. Often the rubella vaccination has also been made in childhood.
  • Alcohol in pregnancy: Pregnant women has to avoid from alcohol in this period.
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