Dandy Walker's syndrome
Contents:
Description:
The Dandy's syndrome — Walker — anomaly of development of a cerebellum and the likvorny spaces surrounding it; genetically caused disease with a frequency of occurrence of 1:25000, is preferential at women.
Reasons of a syndrome of the Dandy Walker:
The etiological reason of this syndrome is unknown. Earlier was considered that this exclusively hereditary, genetically caused disease. However this inborn pathology of a fruit is diagnosed for the drinking mothers very often, even without the accompanying genetic anomalies.
As a rule, women of similar social groups irresponsibly treat pregnancy incubation, pre-natal diagnosis is not carried out, and at them children with typical signs of a syndrome of the Dandy Walker are born.
The Cytomegaloviral ifektion, rubella of the pregnant woman belong to risk factors too. Sometimes children with this syndrome are born at mothers with the hereditary caused family diabetes mellitus.
Symptoms of a syndrome of the Dandy Walker:
Signs of a syndrome come to light at the first screening of pregnancy — in 19-22 weeks. On ultrasonography of a fruit typical, pathognomonic signs are visible: expansion of the fourth ventricle of a brain, a hypoplasia or an aplasia of a worm of a cerebellum, a cyst of a back cranial pole in the field of the big tank.
On volume ultrasonography on later terms it is possible to see an union of fingers of extremities (syndactylia), anomalies of development of kidneys of a fruit, "labium leporium" and "a wolf mouth".
After the birth at the child the heavy neurologic symptomatology which expressiveness is variable — from moderated to bright quickly accrues what the pathology form influences. Quickly enough there are symptoms of intracranial hypertensia, and then hydrocephaly.
The child is uneasy, lags behind in development — as physical, so psychoneurological. Cerebellar symptoms — an ataxy, spastic conditions of muscles, lacks of coordination develop.
The accruing brain decompensation at heavy a form of a syndrome the Dandy Walker leads to death within the first half a year of life, especially in the absence of full clinical observation in the conditions of department of pathology of children of early age.
Diagnosis:
Makes the antenatal diagnosis of a syndrome of the Dandy Walker before the doctor and parents the most serious problem of the choice between preservation of pregnancy with almost absolute probability of the birth of the intellectual and defective child and abortion.
Numerous ultrasonography of a fruit for the purpose of dynamic observation of a condition of a brain, heart, kidneys is carried out. After the birth the diagnosis is confirmed by MRT (carrying out MRT during pregnancy is inexpedient because of impossibility of fixing of a fruit in a cavity of the uterus).
Treatment of a syndrome of the Dandy Walker:
At an incomplete form of a syndrome, hydrocephaly perhaps surgical treatment — shunting of a cyst and a side ventricle.
However this palliative treatment — damage of a brain cannot be eliminated, the child remains the heavy physical and psychoneurological disabled person with the accompanying heart pathologies, anomalies of kidneys, eyes, intellectual inferiority.
Sometimes, in exceptional cases the syndrome of the Dandy Walker proceeds without the accompanying pathologies of heart and other bodies, but the level of intellectual development is always extremely low.
