Dandy-Walker Syndrome

Dandy-Walker Syndrome (DWS) is when the brain has been damaged or flawed during the time the foetus is developing in the womb. The main feature of this syndrome is a half or complete lack of the cerebral vermis, the cerebral vermis is a thin worm like part that is between the hemispheres (sides) of the cerebellum (which is at the bottom of the brain, closer to the spine). Also the half or complete lack of the corpus collosum (which joins the left and right sides of your brain, called the cerebrum), that can be linked to DWS. There is a good chance that the damage is caused by a cyst growth. This cyst damages the part of the brain that allows a person to control their movement and cognitive learning abilities. The cyst blocks fluid to freely flow between the upper and lower parts of the brain and to the spinal cord. Since this fluid cannot flow freely, it builds up in the skull causing an increase of pressure on the bones that surround brain and can also create a larger head. The cyst can also cause a lump or growth at the back of the head which is closer to the neck.  The cyst cannot be removed because it has been growing with the child since the womb and has blood vessels that go through it and attaches it to the brain. If the doctor tries to remove the cyst, it can cause the patient/child too much brain damage to live.  

Dandy-Walker Syndrome is rare, it takes place in 1 in ever 25 000 to 30 000 live births. Depending on how severe a person’s DWS is, then that is what will determine their level of intelligence and their life span. The more severe the DWS and the more associated disorders in the heart, brain, nervous system etcetera, will shorten a person’s life span. About 50% of person that are diagnosed with DWS will have normal intelligence but, it is important to remember that all cases are different. Some people that are diagnosed with DWS can have a chance of growing up and having their own family.  Some research says that girls have a higher chance of having DWS, but it looks like males and females both have an equal chance of having DWS.

Dandy-Walker Syndrome can be noticed right away or it can develop unknown to a person. For an infant, symptoms to raise awareness are;

                        
slow motor development like walking
                         hard time moving their head
                         larger head/skull

Some symptoms for children could be;

                        
pressure in the head
                         vomiting
                         growth on the back on the head closer to the neck
                         lack of coordination
                         unbalanced
                         jerky/fast eyed movements
                         again a larger head
                         dizziness
                         seizures (about 20 to 30% of kids with DWS will have this)
                         hard time breathing or sometimes will have periods of not breathing

Most Dandy-Walker Syndrome cases are diagnosed within the first year the child is born. Often children with DWS will have a hard time walking and about 30% may have an abnormal growth on or in their body. Please remember that not all cases of Dandy-Walker Syndrome are the same, every case is different.

When the fluid builds up in the brain doctor may have to put a special tube called a shunt, to drain the extra fluid. By draining the fluid it will help get rid of the pressure in the head and prevent the head from getting much larger. But the treatment that the patient/child can get is to treat the associated disorders that DWS causes mentioned above.  

There are no known reasons why the brain doesn’t completely develop in the womb and no known risk factors that cause DWS. Scientists are still researching Dandy-Walker Syndrome; where some seem to think it could be linked to a gene or genetics. If parents had a child with DWS and want to have more children, they could benefit from meeting with a genetic counsellor for support.

If your child has Dandy-Walker Syndrome, then some suggested professional they should have are;

                        
• paediatricians
                        • neurosurgeons
                        • occupational therapist
                        • physical therapist
                        • speech therapist
                        • psychoeducator

 

Please be sure to go to the clinic or hospital if you have any concerns or worried about your child.



Website/Bibliography

“Dandy-Walker Syndrome”. Mama’s Health.com
http://www.mamashealth.com/syndrome/dandy.asp                 (26 Oct, 2010)

“Dandy-Walker Syndrome”. ThirdAge.com
http://thirdage.com/hc/c/dandy-walker-syndrome      (25 Oct, 2010)

“Dandy-Walker Syndrome”. Wikipedia
http://en.wikipedia.org/wiki/Dandy-Walker_syndrome#Treament          (25 Oct, 2010)

“NINDS Dandy-Walker Syndrome Information Page”. National Institute of Neurological Disorder and Stok
http://www.ninda.nih.gov/disorders/dandywalker/dandywalker.htm      (22 Oct, 2010)

“What is Dandy-Walker?”. Dandy-Walker Alliance
http://www.dandy-walker.org/blog/dandy-walker-alliance-brochure/      (22 Oct, 2010)

“What is DWS?”. Dandy Walker Homepage
http://www.dandywalker.net.index.php?option=com_content&task=view&id=2&Itemid=1            (22 Oct, 2010)

“What is Dandy-Walker Syndrome?”. MedFriendly
http://www.medfriendly.com/dandywalkersyndrome.php5      (26 Oct, 2010)

 

 

 

 

 

 

 

 

 

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