Dandy-Walker Malformation

What is Dandy-Walker malformation?

Dandy-Walker malformation is a rare brain malformation that occurs during the development of the cerebellum and fourth ventricle in the brain. These are the areas that help coordinate movement, cognition, and behavior, and that channel fluid from the inside to the outside of the brain, respectively. With Dandy-Walker malformation, the central part of the cerebellum may be very small or even absent or abnormally positions. Fluid-filled cavities in the brain may also be abnormally large. These defects can lead to a series of associated complications, including, but not limited to, hydrocephalus, bad coordination, and neurological problems.

What causes Dandy-Walker malformation?

Dandy-Walker malformation results from defects in early embryonic development of the brain area that includes the cerebellum and its’ surrounding structures. It is not known exactly what causes these defects, but doctors speculate that a mix of chromosome abnormalities and environmental triggers may be to blame.

What are the symptoms of Dandy-Walker malformation?

Due to the nature of Dandy-Walker malformation, the signs and symptoms of the malformation are present at birth or develop within the first year of the patient’s life. These signs and symptoms include the following:

Developmental delay
Low muscle tone or later high muscle ton (spasticity)
Poor coordination and balance
Enlarged head circumference and increased pressure within the skull
Headache
Facial palsy (paralysis)

In addition to these, mental retardation, severe hydrocephalus, respiratory failure, and/or other birth defects have been known to occur, as well.

How is Dandy-Walker malformation diagnosed?

Dandy-Walker malformation is diagnosed after a thorough clinical evaluation and a review of patient history. To confirm a diagnosis, an ultrasound, a CT scan, and/or a MRI scan may be used. Dandy-Walker malformation may also be diagnosed prenatally using an ultrasound or a fetal MRI.

What are the available treatments for Dandy-Walker malformation?

First and foremost, if a patient with Dandy-Walker malformation also has hydrocephalus, he/she should undergo surgery to drain the fluid from the brain. This surgery entails inserting a tube into the head to redirect the fluid to other areas of the body that can absorb it. The other complications associated with Dandy-Walker malformation can be managed using a combination of special education, physical therapy, and other medical or social services.

Where can I find more information on Dandy-Walker malformation?