Dandy-Walker syndrome – causes, symptoms & therapy

The congenital developmental disorder Dandy-Walker syndrome is characterized as follows: First and foremost, it is a malformation of the cerebellum. This means that the connection between the hemispheres of the cerebellum is either completely absent or partially underdeveloped. Furthermore, there is an expansion of the 4th cerebrospinal fluid chamber. This means that the accumulation of excessive liquor – cerebrospinal fluid – results in hydrocephalus.

causes

In Dandy-Walker syndrome, the developing brain of the embryo is damaged. There is no evidence or evidence as to why Dandy-Walker syndrome actually occurs. In most cases, doctors are at a loss as to why the child has Dandy-Walker syndrome. The causes are individual. A genetic disease can also be a trigger. However, there are indications that favor Dandy-Walker syndrome. This includes, for example, the infection of rubella during pregnancy.Excessive alcohol consumption or abuse during pregnancy can also be a cause of Dandy-Walker syndrome and promote it accordingly. There are also various hereditary diseases that also cause Dandy-Walker syndrome. These include Meckel-Gruber syndrome, Ehlers-Danlos syndrome and Joubert syndrome. However, there is no explicit evidence that Dandy-Walker syndrome is favored.

symptoms and course

Typical symptoms of Dandy-Walker syndrome:

• headache

• Vomit

• nausea

• visual disturbances

• seizure

• disturbances of consciousness

The symptoms are relatively different in Dandy-Walker syndrome. Even the course of the disease is individual. There are illnesses that, in the worst case, can even be fatal. On the other hand, there is also a form of Dandy-Walker syndrome that has gone undetected for a long time. Symptoms are factor dependent. So it plays an important role whether the baby suffers from hydrocephalus or not.

Furthermore, other malformations or disorders also play an important role in the symptoms of how severe the Dandy-Walker syndrome is. The most common symptom of Dandy-Walker syndrome is excessive head circumference. Other symptoms include headaches, vomiting, nausea, visual disturbances such as seizures and also disturbances of consciousness.

Increased intracranial pressure can also be a symptom. Above all, the increased intracranial pressure causes impairment of the cerebellum. Thus, the person suffers from balance and movement disorders and from eye tremors. Many patients also suffer from speech disorders. Many patients with Dandy-Walker syndrome also have intellectual disabilities in their development.

Other symptoms include cardiac malformations. The course is different. The smaller the defect, the better the mental abilities of the patient. Doctors, however, only ever make cautious predictions about the course of the disease. This is primarily due to the fact that the course also depends on the “water head”.

The prognosis and course also depend on how quickly Dandy-Walker syndrome is diagnosed. The earlier treatment begins, the higher the chances that Dandy-Walker syndrome will have an easier course. In the case of complex malformations, however, the prognosis is negative. Mainly because the course of the Dandy-Walker syndrome also affects the other malformations.

Diagnose

The doctor made the diagnosis of Dandy-Walker syndrome shortly after delivery. The doctor uses an ultrasound examination to determine whether the newborn is suffering from Dandy-Walker syndrome. During the ultrasound examination, the doctor detects a malformation of the cerebellum and also a malformation of the fourth ventricle. The diagnosis of Dandy-Walker syndrome is often confirmed by computed tomography – CT – or magnetic resonance imaging – MRI.

complications

Dandy-Walker syndrome severely disrupts the development of children. This disorder can have a negative effect on the further life of the affected person and also lead to consequential damage in adulthood. In most cases, the patients suffer from severe headaches, which are triggered by the increased pressure in the brain. Nausea and vomiting also occur and the patient’s everyday life is made considerably more difficult by the Dandy-Walker syndrome. In severe cases, the patient may also experience impaired consciousness or visual disturbances.

Complete blindness of the patient can also occur. The head circumference of those affected is usually increased by the Dandy-Walker syndrome, so that bullying or teasing can occur, especially in children. Treatment of Dandy-Walker syndrome can be carried out with the help of surgical intervention. Under certain circumstances, however, not all complaints can be limited.

Epileptic seizures can also be limited with the help of medication. There are no complications. However, the life expectancy of the patient can be limited by the Dandy-Walker syndrome. It is not uncommon for psychological treatment to be necessary to prevent complications in adulthood.

treatment and therapy

The problem with Dandy-Walker syndrome is that there is no primary treatment and therapy. The doctors regularly examine the affected children. This is also because any increase in intracranial pressure is recognized and treated in good time. Doctors insert a tube when the intracranial pressure increases and the intracranial pressure builds up. The doctor inserts this and ensures that the water is drained away. The shunt diverts the liquor into the peritoneum or into the right atrium.

If the patient suffers from balance disorders, physiotherapeutic treatment and exercise usually helps. If the patient reports seizures caused by Dandy-Walker syndrome, therapies using antiepileptic drugs can help . There are no tablets or therapies that treat Dandy-Walker syndrome – only symptoms are combated and the course is reduced.

prevention

Due to the fact that the cause of Dandy Walker Syndrome is still unknown, it is difficult to take preventive measures. However, the expectant mother should stop the possible causes – rubella and excessive alcohol consumption – in advance or take countermeasures. As a preventative measure, the expectant mother can be vaccinated against rubella in advance. Avoiding alcohol is also a preventive measure against Dandy-Walker syndrome.

If the Dandy-Walker syndrome is due to a genetic disease or disorder, since the parents already have a chromosomal disorder, targeted diagnostics will help in advance. However, this does not mean that the child does not develop Dandy-Walker syndrome.