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13052 2012 Article 261 Fig1 HTML.webp
a) MRI sagittal T1 scan demonstrating a left-sided hemihydranencephaly b) coronal

Hemihydranencephaly is a severe cephalic disorder characterized by complete or almost complete absence of the cerebral cortex with preservation of meninges, basal ganglia, pons, medulla, cerebellum, and falx. It is a special type of hydranencephaly.

It is a very rare disease. As it stands, only 7 cases have been reported.


  • Greco F, Finocchiaro M, Pavone P, Trifiletti RR, Parano E. Hemihydranencephaly: case report and literature review. J Child Neurol. 2001;16 :218–221