Schizencephaly

Etiology: abnormality of neuronal migration
Imaging: gray matter lined clefts from ependyma of lateral ventricle to cortical surface – a pial-ependymal seam, +/- abnormal adjacent cortex / gyri, usually dimple at ventricular surface, if cleft walls are in apposition = closed lip (Type I), if cleft walls are separated by CSF = open lip (Type II), unilateral or bilateral, often near pre or post central gyri, associated with optic nerve hypoplasia / absent septum pellucidum / septo-optic dysplasia
DDX:
Complications:
Treatment:
Clinical: often have seizures or hemiparesis or developmental delay, open lip – more common than closed lip, open lip more commonly bilateral, closed lip more commonly unilateral

Radiology Cases of Schizencephaly

MRI of perisylvian polymicrogyria and open lip schizencephaly — Coronal and axial T1 (above) and axial T2 (below) MRI without contrast of the brain shows small haphazard appearing gyri and too few sulci present bilaterally, left greater than right, primarily in the sylvian fissures. There is also a cleft of gray matter with cerebrospinal fluid within extending from the cortex to the posterior aspect of the left lateral ventricle.

CT of right open lip schizencephaly — Axial CT without contrast of the brain shows a cerebrospinal fluid tract communicating between the right occipital cortex and the posterior horn of the right lateral ventricle that was felt to be lined by gray matter on both sides.