Genetic diseases

Dandy Walker syndrome

Dandy-Walker Syndrome

Walker syndrome dandy photo Dandy-Walker Syndrome - a disease in which lie the basis of certain defects of the brain that appear even during fetal development.Dandy-Walker syndrome is quite rare, its incidence - 1 case on 25,000 newborns.But if the causes leading to the emergence of Dandy-Walker syndrome, superimposed on an existing hydrocephalus, the risk of clinical manifestations of this disease doubles.

important to know that Dandy-Walker syndrome is a priori considered a children's disease.In addition, it is usually diagnosed even during intrauterine development of the child.Since the identification of diagnostic features and the first manifestations of which has on the Dandy-Walker syndrome in children, such patients become mandatory on account of dispensary from relevant specialists.

Dandy-Walker syndrome causes

causes leading to the appearance of Dandy-Walker syndrome, the child are teratogenic factors that have a negative impact on the development of the fetus.This transferred the

mother in the first trimester of pregnancy are dangerous for the life of the child infectious diseases such as measles and rubella.It is important to know the effects of the measles virus in the tissues of the fetus.The fact is that the measles virus trails to the tissues of the embryo - it is literally "eats" them.Therefore, as a result of their exposure to the measles virus can cause defects in the tissues of the brain and the development of Dandy-Walker syndrome.

also Dandy-Walker syndrome in pregnancy a woman may have her fetus due to exposure to toxic substances.For example, taking certain medications (especially certain types of antibiotics) may cause a violation of the tab structure of the central nervous system of the fetus.Certain effects on the fetus and may have fetal injury.

So what happens in the structures of the brain, which leads to the development of Dandy-Walker syndrome?All events of this syndrome are at a certain part of the brain - the fourth ventricle.This special ventricle plexus produces CSF.As a result of the above-described reasons it occurs hypoplasia holes which permit the liquid in the subsequent sections.Thus, in the 4th ventricle gradually accumulate a large amount of liquor which passes either entirely in the lower divisions, or remains in the cavity of the 4th ventricle.This growing volume causes a lot of pressure, which affects the surrounding structures.In the cavity of the 4th ventricle out the nucleus of cranial nerves, so when exposed to high pressure in their cerebrospinal fluid volumes on these nerves and excitement is passed in the clinical manifestations of certain symptoms appear.

constantly accumulating fluid forms a cyst in the posterior fossa of the skull.This cyst can be so large that can deform the skull and hinder 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

a result of impaired patency of cerebrospinal fluid from the 4th ventricle, a child is a violation of the right reflexes favorites, there is a serious delay in motor development.

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

main symptom Dandy-Walker syndrome is increasing pressure hydrocephalus: a child can pulse large fontanelle and a visible enlargement of the skull due to the increase of its part of the brain.These children skull growth rate will be significantly higher than the growth rate of the front of the skull.

children with Dandy-Walker syndrome for a long time can not hold his head and smaller than those of healthy children.Also can be detected hyperactivity or giperreagentnost certain nerves (due to the impact on their core liquor pressure).

important to know that Dandy-Walker syndrome may be as rapid flow, and a slow and long.In most cases a second, longer version of the already manifest in adulthood.Its symptoms are a much more soft and gentle than the current version quickly.But it is impossible in adult patients with this syndrome and forget about the possible build-up effects of hydrocephalus.

Dandy-Walker syndrome treatment

Treatment of Dandy-Walker syndrome is directly dependent on its extent and severity.When lung degrees and a slight manifestation, Dandy-Walker syndrome is treated symptomatically: prescribe diuretics to reduce CSF pressure, cope with pain and dizziness.

But if the disease no matter what treatment continues to progress, the only sure way of treatment - neyrohiorurgicheskaya operation.In the course of its moves to create new liquor: can be created drains into the abdominal cavity or the chambers of the heart, or in the outer environment.

forecast Dandy-Walker syndrome is favorable if the disease is diagnosed and treated.In severe stages, or no treatment in children developing certain defects in the mental and psychological development.

It is important to timely diagnosis of this syndrome in the fetus.For this woman to be appointed by ultrasound: the detection of signs of Dandy-Walker syndrome, fetal ultrasound - Research appointed 1 woman every three weeks.It is also possible to assign the MRI - studies to clarify the diagnosis and find out the exact location of the lesion.

also need to diagnose the presence of pregnant women have antibodies to measles and rubella.And if these antibodies have, immediately conduct ultrasound of the fetus and to evaluate the extent of his injuries.With significant lesions of the fetus, can the question of immediate artificial abortion.

service physician recruitment is relevant only for the citizens of the Russian Federation

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