Schizencephaly: Pre- and Postnatal Magnetic Resonance Imaging

Abstract

Schizencephaly is a rare disorder of neuronal migration that is characterized by the presence of clefts that extend from the ependymal surface of the lateral ventricles to the pial lining of the cortex. The authors present the case of a female patient with a prenatal diagnosis made by magnetic resonance imaging (MRI), her clinical course, and neurorradiological evolution following birth. A 6-year-old female, with right open lip schizencephaly, was diagnosed by means of prenatal cerebral magnetic resonance at the gestational age of 25 weeks. The patient does not present intellectual disability, reaching developmental mile-stones at normal time points. The MRI of the brain reveals right, perisylvian, closed lip schizencephaly. Prenatal MRI is remarkably useful in the diagnosis and prognostic approach to the condition. It is less useful in classifying the unilateral forms (open vs closed lips), and hence, its prognostic validity is more limited.

Correlation of Prenatal and Postnatal MRI Findings in Schizencephaly

“…24,26 Our study of 18 clefts demonstrated closure of prenatally diagnosed open clefts in 47% of cases on postnatal imaging. We qualitatively and quantitatively assessed a number of parameters to determine whether we can predict closure of prenatally open clefts.…”