Dandy Walker Syndrome is a disorder of the brain which involves the cerebrum and the fluid-filled spaces that surround it. The syndrome is characterized by an expansion of the fourth ventricle in the brain. The ventricle allows blood to flow freely around the upper and lower hemispheres of the brain.
A person with the syndrome can also have a cyst forming at the lower region of their skull. The syndrome causes an expansion in the fluids spaces that surround the brain. A victim experiences a build up in pressure in their head.
The syndrome is known to affect one child in every twenty five thousand new born. This means it is a rare condition. The syndrome was first described by Drs. Dandy and Blackfan in 1914. There are no known preventive measures against Dandy Walker Syndrome
The syndrome can be inherited. Having parents with Dandy Walker Syndrome increases the chances of the syndrome in their children. Other factors that increase its occurrence are malformations of face, fingers, toes, heart, and limbs. A person who has an absence of the corpus callosum is also prone to the syndrome.
Dandy Walker Syndrome can occur dramatically or even unnoticed. There are two symptoms of the syndrome that occur during infancy. First, a child may have a slow motor development. A child can also have a progressive expansion of the skull.
In slightly older kids, symptoms of an elevated intra-cranial pressure may be seen. Such pressure causes frequent vomiting and irritability to children. They may also have cerebellar dysfunction. Cerebral dysfunction causes spasmodic movement of the eyes and an irregular muscle coordination.
Other visible symptoms are enlargement in the circumference of the head and bulging of the skull. Victims manifest laboured breathing and may have problems with nerve endings that control the eye. Most kids with this syndrome suffer from urinary tract and kidney abnormalities. They may also have heart malfunctions or Oral Facial Clefts.
Effects of Dandy Walker Syndrome vary from one victim to another. If the build up in fluids is treated early, some kids can have a normal cognition. Others never achieve a normal intellectual development. Longevity in victims is dependent on the severity of the Dandy Walker Syndrome on a victim. Multiple congenital defects on a person can reduce their life span.
Treating Dandy Walker Syndrome entails treating its associated problems. At times, a surgical procedure on a victim may be required. Such a procedure is known as a shunt. Surgery helps to drain excess fluids contained in the brain. It also minimizes the intra-cranial pressure and swelling. Parents who live with children with this syndrome can attend genetic counselling sessions. This is in the event the parents desire is to have more children.
There are numerous experiments conducted every day on Dandy Walker Syndrome. NINDS supports and performs a broad range of studies on the syndrome. They also study the different mechanisms involved in brain development. The knowledge attained from such studies lays the foundations for understanding anomalous brain development.