Schizencephaly is defined as a congenital brain malformation characterized by clefts extending from pial surface of cerebral mantle to ventricle and are lined by polymicrogyric cortex
CASE 1 :
In this case we can see a hypodense area showing CSF density in right occipital region communicating with ipsilateral lateral ventricle limited by falx cerebri and lined by grey matter suggestive of open lip schizencephaly.
If you carefully see further mild outpouching of CSF noted from posterior end of occipital horn of left lateral ventriclewhich is suggestive of - closed lip schizencephaly…..though we’ve to do MRI evaluation to confirm it.
CASE 2 :
In this second case we can see a large CSF filled cleft is noted extending from the pial surface of the frontotemporoparietal lobes to the ependymal surface of frontal and temporal horns of bilateral lateral ventricles suggestive of bilateral open lip schizencephaly
Septum pellucidum appears absent.
Hypogenesis of bilateral frontal and temporal lobes with decreased sulcation.
TYPES :
Type I (closed-lip): Fused pial-ependymal seam lined by gray matter forms "furrow" in cortex
Type II (open-lip): Large, gray matter lined, fluid-filled cerebrospinal fluid clefts
Clinical Issues
Type I: May be near normal or have congenital hemiparesis and seizures
Type II: Mental retardation, spasticity, seizures, severely compromised
Location : Usually involving insula adjoining pre/postcentral gyri - Bilateral or unilateral full surface clefts of cortical mantle. Gyri and sulci radiate into cleft or furrow, aiding differentiation from late intra-uterine or early postnatal MCA infarction. Deformity of ventricle at site of closed-lip Schizencephaly "points" to cleft
CT Findings
NECT : Relatively dense gray matter lines CSF clefts
CECT : Large, primitive appearing veins near cleft +/- Hydrocephalus (especially open-lip Schizencephaly with ruptured membrane)
Natural History & Prognosis
Malformation is stable, development of epilepsy is common.
Size and magnitude of clefts, as well as number of associated malformative lesions, govern severity of motor deficits, developmental delays, and epilepsies.
Single closed-lip defects may be asymptomatic or diagnosed in adult
Top Differential Diagnoses
"Clastic schizencephaly"
Porencephaly
Hydranencephaly
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