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SCHIZENCEPHALY

Imaging

Imaging for optimized detection of schizencephaly:

While schizencephaly may be detected on USS (fetal and postnatal) and CT, especially open-lipped schizencephaly, MRI is the imaging of choice for assessing the detail and associated structural abnormalities. MRI should include thin slice volumetric T1-weighted images, axial and coronal T2-weighted and FLAIR images.

Imaging characteristics of open-lip schizencephaly:
  • a cleft is seen extending from the ventricle to the cortical surface, lined by polymicrogyria
  • the cleft results in CSF communication between the subarachnoid space and the lateral ventricle; the "pia ependymal seam"
  • the cleft walls are separated by CSF filled space
  • the open-lip form is common in bilateral schizencephaly
Imaging of a schizencephalic cleft

The image below shows a right sided open-lipped schizencephalic cleft. The walls of the cleft are lined by grey matter and are separated by CSF filled space.

schizencephaly 1

Imaging characteristics of closed-lip schizencephaly:
  • a cleft is seen extending from the ventricle to the cortical surface, composed of heterotopic grey matter
  • a 'dimple' in the ventricle is seen, at the point where the cleft commences
  • the cleft walls are in apposition, without CSF filled space separating them
  • the closed-lip form is common in unilateral schizencephaly
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