Dandy-Walker Syndrome: Definition, Causes, Symptoms, Diagnosis and Treatment

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Dandy-Walker syndrome is a medical condition that involves the cerebellum and the fluid-filled spaces that surround it. This syndrome is defined as a congenital brain malformation. This syndrome is characterized by an enlargement of the fourth ventricle, a cyst that forms close the the internal skull base and a complete or partial absence of the cerebellar vermis. Those with this syndrome may also experience an increase in pressure and a size increase in the fluid-filled spaces that surround the brain.


This syndrome can develop unnoticed or it can appear dramatically. The symptoms of this syndrome occur most often during early infancy. These symptoms include progressive skull enlargement and slow motor development. When the symptoms begin to occur in older childhood they include increased intracranial pressure. This can cause several symptoms including irritability, jerky eye movements, vomiting, lack of muscle coordination, unsteadiness (a sign of cerebellar dysfunction) and convulsions. Other symptoms that may occur with this syndrome include abnormal breathing patterns, an increase in head circumference, trouble with the nerves that control eye movement and a bulge at the back of the skull.


Diagnosing Dandy-Walker syndrome involve the use of imaging. The imaging technology used to diagnose this syndrome include magnetic resonance imaging (MRI), ultrasound and computed tomography (CT scan). Those who are diagnosed with this syndrome are most often diagnosed during their first year of life.


This syndrome is incurable so those with this syndrome will receive treatment that helps them to manage their symptoms and associated problems. Those who experience intracranial pressure can have a special tube placed in their skull to help reduce this pressure by draining excess fluid. Medications can be prescribed to help alleviate some problems and sometimes physical therapy can be beneficial in helping those diagnosed walk better.


The prognosis for those with Dandy-Walker syndrome depends on how severe the syndrome is and how severe the associated malformations are. Those with multiple congenital defects will have a shorter life span. Some children with this syndrome experience normal cognition and others never achieve normal intellectual development.


NINDS. (2008). Dandy-Walker Syndrome Information. Retrieved on July 17, 2009 from Website: https://www.ninds.nih.gov/disorders/dandywalker/dandywalker.htm