Dandy-Walker Syndrome

1174747864brain.gifDandy Walker is known by many names: Dandy Walker Syndrome, Dandy Walker Malformation, Dandy Walker Cyst, Dandy Walker Variant.


By definition,Dandy Walker is a congenital brain malformation typically characterized by incomplete formation of the cerebella vermis, dilation of the fourth ventricle, and enlargement of the posterior fossil. In layman's terms, Dandy Walker is a cyst in the cerebellum (typically symmetrical) that is involved with the fourth ventricle. This may interfere with the body's ability to drain cerebrospinal fluid from the brain, resulting in hydrocephalus.

Dandy Walker cysts are formed during early embryonic development, while the brain forms. The cyst in the cerebellum typically has several blood vessels running through it connecting to the brain, thereby prohibiting surgical removal. In most cases the cause of Dandy Walker is not known, though there are a few known cases resulting from autosomal recessive genes.

Dandy Walker is generally discovered before birth, by ultrasound. Symptoms in the newborn may include a bulge or lump on the back of the head ("bulging occiput"); a "hair collar", or patch of long hair just above this lump; red pigmented skin in the area of the lump (similar to a "stork bite" birthmark); increased head circumference (if hydrocephalus is present); a downward gaze (if hydrocephalus is present). Other symptoms of the syndrome can also include congenital heart problems; malformations of the face, fingers, or toes; other central nervous system structural anomalies (often agenesis of the corpus callosum); abnormal breathing patterns; motor delays; hypotonia; learning problems; mental retardation; high pain tolerance; seizures; reflux; nystagmus (jerky eyes); ataxia; and other complications.

There is an extreme range of severity with Dandy Walker. Some people have it their whole lives without any symptoms ever presenting; some infants may have it in association with other syndromes resulting in severe complications or death.

Ivan Jr's. case,  we were not to know for 22 months as he sat up at 3 months, walked at about 12 1/2 months. There were no signs whatsoever to indicate anything wrong. However, after his CT scan it was revealed that his fourth ventricle was enlarged, this caused a build up of hydrocephalus at the back of his head. It was also noticed that part of his brain was missing. He has no physical malformation or growth delays physically.

Ivan is still at a very early developmental stage mentally.

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