Hydranencephaly

  • Etiology: in utero bilateral internal carotid artery occlusion due to in-utero infection, vascular accident, hypoxic ischemic encephalopathy, maternal carbon monoxide or butane inhalation
  • Imaging: have cranial vault, complete absence of cerebral cortex + basal ganglia with preservation of falx / thalami / brainstem / cerebellum

Radiology Cases of Hydranencephaly

US of hydranencephaly
Coronal US of the brain (above) shows only the thalamus in the midline surrounded by fluid in the supratentorial region of the brain. No discernable lateral and third ventricles are seen and no rim of cortical parenchyma could be appreciated. Sagittal US of the brain (below) shows the cerebellum to be normal in appearance.