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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.
- Synonym(s): -
- Prevalence: -
- Inheritance: Multigenic/multifactorial or Not applicable
- Age of onset: Infancy, Neonatal
- ICD-10: Q00.1
- OMIM: -
- UMLS: C0152426
- MeSH: -
- GARD: 10504
- MedDRA: 10011321
The prevalence is not known.
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 is possible by ultrasonographic monitoring.