Dandy Walker Syndrome

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Dandy Walker Syndrome
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Specialty scores for Dandy Walker Syndrome

Overview

Source: In partnership with Genetic and Rare Diseases Information Center, funded by the National Center for Advancing Translational Sciences, and the National Human Genome Research Institute https://rarediseases.info.nih.gov/gard.

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Dandy-Walker complex

overview Question

What is Dandy-Walker complex?

Dandy-Walker complex is a congenital brain malformation involving the cerebellum (an area at the back of the brain that controls movement) and the fluid-filled spaces around it. This condition is characterized by an enlargement of the fourth ventricle (a small channel that allows fluid to flow freely between the upper and lower areas of the brain and spinal cord), a partial or complete absence of the cerebellar vermis (the area between the two cerebellar hemispheres), and cyst formation near the internal base of the skull. This condition may present with an increase in fluid and pressure around the skull (hydrocephalus). It may also be associated with developmental delay, or malformations of the heart, face, limbs, fingers, or toes. The term "Dandy-Walker complex" represents a spectrum of abnormalities that includes Dandy-Walker variant, Dandy-Walker malformation, and mega cisterna magna.

Date Modified: 2013-01-07T16:54:00

basic Questions

Is Dandy-Walker complex inherited?

Most cases of isolated Dandy-Walker complex are sporadic (they occur in people with no history of the condition in their family) and the cause frequently remains unknown. A small percentage of cases have been reported to "run in families," but no clear pattern of inheritance exists. When the condition is not thought to be associated with a single-gene disorder, the recurrence risk is estimated to be on the order of 1 to 5%.

It is thought that multiple genetic and environmental factors interact to predispose a person to having Dandy-Walker complex. Research suggests that it could be caused by environmental factors that affect early development before birth. For example, exposure of the fetus to infections (such as rubella or toxoplasmosis) or substances that cause birth defects (teratogens) may be involved. In addition, a mother with diabetes is more likely than a non-diabetic mother to have a child with Dandy-Walker complex.

Dandy-Walker complex has been associated with multiple chromosomal and other genetic abnormalities. When a person has Dandy-Walker complex as a feature of a genetic condition, it is inherited in the same pattern as the underlying condition.

Date Modified: 2016-06-17T00:00:00

What is the long-term outlook for people with Dandy-Walker complex or Dandy-Walker variant?

The long-term outlook (prognosis) for people with Dandy-Walker complex varies, because the "complex" represents abnormalities ranging from mild to severe. The prognosis also depends on whether it occurs alone (isolated) or in association with other malformations or conditions. Life expectancy for affected people depends on the nature and severity of each person's condition. Generally, as the number of additional abnormalities increases, the life expectancy decreases. When the condition is associated with an underlying genetic condition, the prognosis depends on that of the specific condition.

Prognosis for people with Dandy-Walker variant can also vary and may also depend on whether a person has extra-cranial abnormalities. Studies have reported normal outcomes, chromosome disorders such as Down syndrome, physical disabilities, and intellectual disabilities. Isolated Dandy-Walker variant reportedly has the highest likelihood of normal neurologic and physical outcome in the newborn period. The functional abilities for survivors vary depending on the degree of the cerebellar vermis abnormality. Even with normal development, affected children may have overall developmental, functional, and/or behavioral profiles below average.

Date Modified: 2015-06-16T00:00:00

Presentation(s)

Abnormality of the vertebrae

Dandy-Walker malformation

Prominent occiput

Frontal bossing

Abnormality of the urinary system

Aplasia/Hypoplasia of the corpus callosum

Encephalocele

Tetralogy of Fallot

Agenesis of cerebellar vermis

Cranial nerve paralysis

Dilated fourth ventricle

Elevated imprint of the transverse sinuses

Heterogeneous

Hydrocephalus

Nystagmus

Partial absence of cerebellar vermis

Posterior fossa cyst at the fourth ventricle

Thinning and bulging of the posterior fossa bones

Truncal ataxia