Orphanet: Congenital hydrocephalus

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Congenital hydrocephalus

Disease definition

A rare central nervous system malformation characterized by abnormally enlarged cerebral ventricles due to impaired cerebrospinal fluid circulation. It arises in utero and can be either acquired or inherited. The severity of the resulting brain damage depends on the duration and extent of ventriculomegaly.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Antenatal
  • ICD-10: Q03.0  Q03.1  Q03.8  Q03.9
  • OMIM: 236600  615219
  • UMLS: C0020256
  • MeSH: -
  • GARD: -
  • MedDRA: 10010506
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