Wednesday, November 13, 2013

Dandy Walker Malformation - A Brief Overview

By Jack Morgan


Dandy Walker Malformation is a congenital syndrome of the brain that involves the area at the back of the brain which is referred to as cerebellum. It also affects the area that is filled with fluid around the cerebellum. The key characteristics of this condition are that there is an increase in the fluid space size that is surrounding the brain. There is also an increase of pressure in this fluid.

One thing about this syndrome is that it does not come the same way in different people. While it chooses to come dramatically in some people, it can also develop slowly in other people that they may not even notice until its symptoms are evident. Symptoms like slow motor growth and the skull enlarging progressively are mostly evident during infancy.

The other features of Dandy Walker Malformation are the absence of the area the brain area which should be between the cerebellar hemispheres. There is formation of cyst near the lowest part of the skull. There is also an enlargement of the small channel that allows fluid to flow freely between the lower and upper of the spinal cord and the brain.

This malformation can develop unnoticed or in a dramatic way. The early stages symptoms of this syndrome in infants include progressive enlargement of the skull plus slow motor development. There is increased intracranial pressure in older children. The other symptoms in older children include signs of cerebellar dysfunction, vomiting, poor muscle coordination, increased head circumference, jerky eye movements, bulging of the back of the skull and abnormal breathing patterns.

Treatment for people with dandy walker malformation normally involves treating the problems that are associated with the disease when it is needed. There is also a surgical procedure referred to as a shunt that is necessary to drain the excess liquid found in the brain off. The draining off reduces the intracranial pressure which reduces the swelling. It is wise for parents of such children to go for genetic counseling just in case they may have any plans for getting more children in future.

One key feature with dandy walker syndrome is that the cerebellar vermis could be partially or completely absent in an individual. Cerebellar vermis is a brain part that is found between the two cerebellar hemispheres. Medics say that it is genetic and that unlike other conditions, it is very sporadic. Statistics show that it occurs in every one of thirty thousand live births. It is for this reason that genetic counseling is preferred for parents.

The effect of Dandy Walker Malformation on intellectual development is variable on different children. This is determined by the fact that some children have got normal cognition but there are others that don't achieve the normal intellectual development even when there is early treatment of excess fluid buildup in the brain. The severalty of the malformation will determine its longevity. Lifespan of an individual can in some way be affected by the presence of multiple Dandy Walker Malformations.

The phrase Dandy walker malformation is a combination of several abnormalities in the central nervous system. The term came from Walter Dandy and Arthur Earl Walker.




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