Dandy-Walker syndrome (DWS) is a congenital (present at birth) brain malformation typically involving the fourth ventricle and the cerebellum. The disorder was first described in 1914 by W. Dandy and K. Blackfan and was designated as Dandy-Walker syndrome in 1954 by C. Benda, who also reported familial occurrence. DWS occurs in one out of every 25,000 live births and occurs more frequently in females than males.
Features and Characteristics
The following characteristics have been seen in children with Dandy-Walker syndrome:
- Enlargement of the fourth ventricle
- Absence (partial or complete) of the cerebellar vermis (area between the two cerebral hemispheres)
- Cyst formation in the posterior fossa (internal base of the skull)
- Slow motor development in early infancy
- Progressive macrocrania (abnormally enlarged skull)
- Intracranial pressure in older children, causing irritability, vomiting, and convulsions
- Cerebellar dysfunction causing ataxia and nystagmus
- Bulging occiput (back of head)
- Cranial nerve dysfunction
- Abnormal breathing patterns
- Agenesis of the corpus callosum
- Malformations of the face, limbs, digits, and heart
- Cleft lip and palate
- Urinary structural abnormalities
The majority of individuals with DWS are diagnosed their first year of life as a result of the commonly associated hydrocephalus. However, the definitive diagnosis can be made by ultrasound, CT-scan, or MRI.
Treatment consists of treating the associated symptoms (i.e., anticonvulsants for those with seizures and the insertion of a shunt for those with hydrocephalus). Balance problems and spasticity may occur and warrant physical therapy. Occupational therapy may be helpful for those with poor fine motor control.
What to Expect
The syndrome can appear dramatically, or be totally asymptomatic. Therefore, the prognosis for normal intellectual development varies depending on the severity of the syndrome and associated malformations. Difficulties in learning occur in 35-70% of children with DWS. Statistics in the literature citing a high rate of mental retardation most likely reflect injury due to inadequate management of the associated hydrocephalus, something which is less likely to occur today than in the past. Many children with DWS can be mainstreamed at school. Pediatricians, pediatric neurologists, pediatric neurosurgeons, geneticists, physical therapists, and educational specialists may be required to systematically follow these children and work towards ensuring that the child is given the best opportunities to reach his or her full potential.
Dandy-Walker syndrome/hydrocephalus recovery is so different today than it was even 20 years ago. When we found out Marlei had this condition, we searched the Internet for the latest information. We were frightened with stories by people in their mid to late 30s who were barely able to walk and chew gum at the same time. Most couldn't swim or drive a car. It didn't sound promising. We determined that it must be due to lack of technology in the past and that we were looking at a different situation. Technology today is nothing short of a miracle. Once that shunt is in place and the cerebral fluid begins draining from the brain, the brain can start re-growing those neural synapses that we hear so much about.
At birth, Marlei appeared normal except for a slight breathing disorder called laryngomalasia. She even progressed to rolling over and holding her head up and complaining with "UNGEE" when she wanted to eat. But slowly she began to lose those abilities. It was so gradual, and she was only a couple of months old, that no one really noticed. Since she cried all the time, the pediatrician said she must be hungry, so he had me force feeding her rice cereal at two months old. The one thing I did notice was her eyes. They would literally fight to stay looking at me. The pressure was pushing them down to the "moon rise" position characteristic of hydrocephalus while she was trying to bring them up to look at me. It caused a rapid up and down motion. Within days of noticing this, her head swelled up like a light bulb. The surgery was performed a few days later, when she was 3 and a half months old.
Naturally, I had many questions for the neurosurgeon, but he wouldn't elaborate. I even faxed him a sheet of questions about Dandy-Walker syndrome and he faxed them back with 1 or 2 word answers (yes; no; not exactly). So, we changed doctors. Dandy-Walker syndrome, we were told by the new doctor, has a general 1/3 - 1/3 - 1/3 rule. (I'm quoting from memory here ) Approximately 1/3 of the children will grow up to require assistance with most daily activities, 1/3 will grow up to require some assistance, and 1/3 will grow up to require minimal, if any, assistance.
One problem that continues to deter Marlei's advancement is her sensory integration deficiency (a.k.a., sensory defensive syndrome). This syndrome causes her to have similar symptoms to autism, like flapping her arms or rubbing her chin raw on a pillow or repeating sounds to no one. We actively treat this every couple of hours with joint compression and skin brushing to combat her becoming intolerable to stimuli. This syndrome affects her eating foods with too much texture on 'bad' days.
Well, it is 3 years later and Marlei is a happy, healthy, red-blooded human girl. She loves her big sister and follows her around the house wondering why they aren't the same height, since they are obviously the same age (in Marlei's eyes). She loves her cousins - boys, both of them, and they tackle each other as if they had full football pads on. She loves her bun-bun when she goes to sleep. And she adores her Daddy, as do most daughters... won't let him out of her site.
The latest evaluation from the speech therapist placed her around 23 months behind, from the occupational therapist around 18 months behind, and her fine motor skills are only 8 months behind.
I hear parents of special needs children say this all the time, but I have to agree: each and every little thing she accomplishes is like a Nobel Prize to us. We are so encouraged by her progress. - Leila Parker
My 2 ½ year old daughter, Cheyanne, has Dandy-Walker syndrome along with multiple other problems such as agenesis of the corpus callosum and CHARGE syndrome. Cheyanne was diagnosed when she was just a few weeks old and spent the first 3 ½ months in Riley Hospital for Children. She was born on March 13, 1997, and on July 30, 1997 she had a VP shunt placed, and has had two shunt replacements thus far. She has had several operations and continues to go back for appointments and has also had a long list of hospital stays for numerous reasons. She was put on a ventilator and had a tracheostomy placed on November 25, 1997 and continues to be on the ventilator. She is the love of my life, she is very active and alert, and she is so used to the vent tubes, that she seems to think that they are like a blanket for her; she is constantly tangling and twisting in them. She has become very good at breathing on her own, and with a little more growing and being the fighter that she is, we believe that she will be free from the tracheostomy and ventilator very soon.
Cheyanne has red hair and blue eyes and with that combination, you know she has the attitude that goes along with it. That is partly the reason, I believe, that she is such a fighter. Cheyanne is not sitting, standing or walking on her own, but she has therapy that comes to our home once a week to work with her: she has physical, occupational, developmental, and speech. I have to give praise to all of her therapists because with their help, along with our home-care nursing and her father and I, she would not be progressing as well as she is. She is trying really hard to sit up and stand right now. She does not speak, but she has said "Mom" a few times with her speaking valve on. Cheyanne has her own language and you can usually tell what she wants or is trying to say. I think she and I have our own kind of language; she seems to understand what I am saying, but I don't have a clue (laugh out loud).
The advice I would tell other parents is to not always take what doctors say as "gospel" because Cheyanne has defied them several times and made it through life-threatening situations and laughed them off. She has amazed doctors and nurses, and you just have to do what you think is best in the interest of your own child. Sometimes our children have no way of telling us what is hurting or what is wrong, and when the time comes for you to decide what to do, you will do what you think is right for your child and not what everyone else thinks you should do. I have tried to do the best for Cheyanne, and I hope I have, but she will always know that her mother was there by her side through every step of every day. Let your children know that mommy and daddy are right there no matter what. - Jeni Bray
If you are interested in meeting other parents and individuals who are involved in raising a child with Dandy-Walker syndrome, the following listserv is available:
Dandy-Walker email discussion group - To subscribe, visit: http://www.egroups.com/group/dandywalker
For more information on Dandy-Walker syndrome, please see the following helpful references:
- National Institute of Health http://www.ninds.nih.gov/patients/Disorder/DANDYWAK/dandy-walker.HTM
- Baylor College of Medicine http://www.bcm.tmc.edu/pednsurg/disorder/wpdandy.htm
- University of California, Los Angeles http://neurosun.medsch.ucla.edu/Pages/Dandy.html
- Beth Israel Medical Center http://www.nyneurosurgery.org
The Disorder Zone has been created for educational purposes only and is not intended to serve as medical advice. The information provided in The Zone should not be used for diagnosing or treating a health problem or disease. It is not a substitute for professional care. If your child has any health concerns, please consult your health care provider.
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