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Synonym(s) -
Prevalence -
Inheritance Multigenic/multifactorial
or Not applicable
Age of onset Infancy
  • Q00.1
  • C0152426
MeSH -
  • 10011321


Disease definition

Craniorachischisis is the most severe form of neural tube defect in which both the brain and spinal cord remain open to varying degrees. It is a very rare congenital malformation of the central nervous system.


The prevalence is not known.

Clinical description

Craniorachischisis totalis, the most complete form of craniorachischisis, presents anencephaly and total spina bifida together and is lethal.


As with other neural tube defects, craniorachischisis is thought to be of multifactorial origin.

Antenatal diagnosis

Antenatal diagnosis is possible by ultrasonographic monitoring.

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