Port-wine nevi-mega cisterna magna-hydrocephalus syndrome

What is Port-wine nevi-mega cisterna magna-hydrocephalus syndrome?

A rare developmental defect during embryogenesis syndrome characterized by a glabellar capillary malformation, congenital communicating hydrocephalus, and posterior fossa brain abnormalities, including Dandy-Walker malformation, cerebellar vermis agenesis, and mega cisterna magna. Seizures are occasionally associated. There have been no further descriptions in the literature since 1979.

Other condition names

  • Nova syndrome


  • Worldwide: <1 in 1,000 000

Age of Onset

  • Antenatal
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