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Dandy Walker Syndrome

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Dandy walker syndrome photo The Dandy Walker Syndrome is a disease that is based on defects in some parts of the brain that appear during the intrauterine development of the child. The Dandy Walker syndrome is rare enough, its frequency is 1 case per 25 thousand newborns. But if the causes leading to the emergence of Dandy-Walker syndrome are superimposed on the already existing hydrocephalus, then the risk of developing clinical manifestations of this disease doubles.

It is important to know that Dandy-Walker syndrome is a priori considered a child's disease. In addition, it is usually diagnosed during intrauterine development of the child. Since the discovery of diagnostic signs and the first manifestations that the Dandy-Walker syndrome has on the body in children, such patients become mandatory for dispensary registration with relevant specialists.

Dandy Walker Syndrome Causes of

The causes of Dandy Walker's syndrome in a child are teratogenic factors that have a negative effect on fetal organ development. This and the mothers in the first trimester of pregnancy are life-threatening infectious diseases, such as measles and rubella. It is important to know the effect of measles virus on the fetal tissues. The thing is that the measles virus tropen to the tissues of the embryo - it literally "eats" them. Therefore, as a result of its impact, measles virus can lead to defects in the brain tissue and the development of Dandy Walker syndrome.

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Also, the Dandy Walker syndrome in a woman's pregnancy can occur in her fetus due to exposure to toxic substances. Thus, taking certain medicines( especially certain types of antibiotics) can cause disruption of the laying of the central nervous system structures in the fetus. A certain effect on the fetus can also have intrauterine injuries.

So what happens in the structures of the brain, which leads to the development of the Dandy-Walker syndrome? All events of this syndrome take place in a certain section of the brain - the 4th ventricle. In this ventricle special liquids are produced by special plexuses. As a result of the above-mentioned causes, there is underdevelopment of the holes that allow this fluid to pass into the subsequent sections. Thus, in the 4th ventricle, a large amount of CSF gradually accumulates, which either does not go completely to the lower parts, or remains in the cavity of the 4th ventricle. Such a constantly growing volume causes a lot of pressure, which affects the surrounding structures. In the cavity of the 4th ventricle, the nuclei of the cranial nerves come out, therefore, when the pressure of increased cerebrospinal fluid is exerted on them, excitations are transmitted to these nerves and a definite symptom appears in clinical manifestations.

Constantly accumulating fluid forms a cyst in the posterior fossa of the skull. This cyst can be so large that it can deform the skull and interfere with the development of structures adjacent to the cavity of the 4th ventricle. All the above changes are accompanied by a permanent hydrocephalus.

Dandy Walker Syndrome Symptoms

As a result of impaired passableness for cerebrospinal fluid from the 4th ventricle, the child has a violation of the correct reflex bookmarking, there is a serious delay in the development of motor skills.

With the Dandy Walker syndrome, a small child is extremely irritable. He can often cry, his crying is associated with a significant headache due to the pressure of the cerebrospinal fluid. Also, his parents may notice that a child, when trying to take a toy in his hands, may miss it.

The main symptom of Dandy Walker syndrome is the increasing hydrocephalus: a child can pulsate a large fontanelle and appear a visible enlargement of the skull, due to an increase in its brain portion. In such children, the rate of growth of the skull will significantly exceed the growth rate of the facial part of the skull.

Children with Dandy Walker Syndrome for a long time can not hold their heads and fall behind these indicators from healthy children. Hyperactivity syndromes or hyper-responsiveness of certain nerves may also be detected( due to the effect of liquor pressure on their nuclei).

It is important to know that the Dandy Walker syndrome can have both a fast current and a slower and longer duration. Most often, the second, longer version will appear already in adulthood. His symptoms will be much softer and more sparing than the fast current version. But it is impossible in adults with this syndrome to forget about the possible increase in the effect of hydrocephalus.

Dandy Walker Syndrome treatment of

Treatment of the Dandy Walker syndrome depends directly on its degree and severity of the course. At mild degrees and insignificant manifestation, Dandy-Walker syndrome is treated symptomatically: diuretics are prescribed to reduce the pressure of the CSF, cope with pain syndrome and dizziness.

But if the disease continues to progress despite any treatment, the only sure way to treat it is a neurosurgical operation. In its course, new courses are created for the cerebrospinal fluid: drains can be created in the abdominal cavity or chamber of the heart, or into the external environment.

Dandy-Walker syndrome forecast is favorable if the disease is detected and treated. In severe stages or without treatment, children develop certain defects in their mental and mental development.

It is extremely important to conduct timely diagnosis of this syndrome in the fetus. To do this, a woman is assigned ultrasound examinations: in case of revealing signs of Dandy-Walker syndrome in the fetus, ultrasound examination is prescribed to the woman 1 time every three weeks. It is also possible to administer an MRI study in order to clarify the diagnosis and to determine the precise localization of the lesion.

In addition, it is necessary to diagnose the presence of antibodies to measles and rubella in pregnant women. And if there are such antibodies, then immediately carry out ultrasound examination of the fetus and evaluate the extent of its damage. With significant damage to the fetus, you can raise the issue of immediate artificial termination of pregnancy.

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