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.

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