Hydranencephaly

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[edit] Discussion of Hydranencephaly

  • Hydranencephaly is a rare, isolated abnormality occurring in less than 1 per 10,000 births.
  • It is the most severe form of bilateral cerebral cortical destruction.
  • The differential diagnosis for hydranencephaly includes:


  • Hydranencephaly occurs after the brain and ventricles have fully formed, usually in the second trimester.
  • The brain destruction is complete or almost complete in a bilateral internal carotid artery distribution, with the cerebral hemispheres replaced by fluid covered with leptomeninges and dura.
  • Because the ventricles have already been formed, the falx cerebri is present.
  • The cerebellum, midbrain, thalami, basal ganglia, choroid plexus, and portions of the occipital lobes, all fed by the posterior circulation, are typically preserved.
  • Pathogenesis of hydranencephaly is thought to be a vascular accident involving the internal carotid arteries

[edit] Imaging Findings for Hydranencephaly

  • US scans demonstrate no identifiable cortical mantel.
  • The cerebellum, midbrain, thalami, basal ganglia, choroid plexus, and portions of the occipital lobes are typically present.
  • Falx cerebri is present (falx is absent in holoprosencephaly)


[edit] Images

Patient #1

[edit] See Also

[edit] External Links

[edit] References for Hydranencephaly