Dandy Walker Syndrome is a congenital birth defect that involves the failure of the brain, particularly the cerebellum, to form properly. Those with Dandy Walker Syndrome can have a varying degree of intellectual capacity and may even live a shortened lifespan due to the disease.
The cerebellum is an area in the rear of the brain that coordinates and controls movement. In a healthy person, the cerebellum is protected and cushioned by fluid that rests between the brain and skull. In Dandy Walker Syndrome, a channel that allows this protective fluid to flow from the upper and lower areas of the brain is enlarged. In most cases, the area between the two areas is also missing, and there may be a cyst at the base of the skull.
All of this conspires to increase the amount of fluid and the amount of fluid-filled space around the brain and spinal cord. In some cases of Dandy Walker Syndrome, the increase of cerebral fluid is minimal and can be drained periodically relieving most symptoms. In other cases, the amount of fluid is excessive and results in neurological impairment.
The Symptoms Of Dandy Walker Syndrome
The first signs of Dandy Walker Syndrome can appear gradually and most often appear in infancy. Sometimes, the symptoms of this disease are so mild that they at first go unnoticed. Symptoms include slow motor development and a progressive increase in the size of the skull. As the disorder progresses, the increased pressure can result in irritability, vomiting, convulsions, and other signs of motor impairment. Problems with facial nerves are also very common with Dandy Walker Syndrome. Patients with a severe case of the syndrome may also have other birth defects involving the heart, face, limbs, and digits.
Dandy Walker Syndrome Treatment
Although the prognosis for Dandy Walker Syndrome is grim, there are some treatment options that can alleviate, not cure, the condition. In some cases, a tube or catheter is placed inside the head to drain the excess fluid and relieve pressure. This is also very helpful in controlling the brain swelling that occurs due to excess pressure and may prevent brain damage.
The effects of Dandy Walker Syndrome are far reaching and can be debilitating. Some children with the disorder are able to develop quite well cognitively speaking whereas others fail to develop a normal intellectual capacity. Even in cases where the condition is caught early and the fluid drained faithfully, severe brain malformations can be present. The lifespan for those with Dandy Walker Syndrome is dependent on how severe the condition is and how early treatment is sought.
The Causes Of Dandy Walker Syndrome
While it is unclear what causes the syndrome, Dandy Walker is believed to be a birth defect. Studies continue to be performed on this syndrome and others like it so that doctors and patients can have a better understanding of what causes the brain not to form properly. It is hoped that through understanding more treatments courses and maybe even a cure can be found.
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