Schizencephaly in neonates and clinical importance. Cases report.

Abstract

Background: Schizencephaly is a congenital alteration of the architecture of the cerebral cortex, and its diagnosis in the neonatal period will be determined by prenatal diagnosis or severity of the cases.

Clinical cases: We present three neonatal cases with variable clinical expression and electroencephalographic findings during clinical course. Case 1: term neonate, born by cesarean section due to pre-eclampsia and presenting early respiratory distress. On clinical examination, macrocranial was clear, so a cranial tomography (CT) scan was performed showing bilateral open-lip schizencephaly. He early presented recurrent seizure, associated with hypotonia, and swallowing disorder with little improvement despite interventions, and palliative management was decided. Case 2: 36-week-old neonate with a prenatal diagnosis of schizencephaly and delivery by cesarean section due to altered fetal monitoring. Low birth weight and requiring positive pressure ventilation. On day 2, CT scan confirmed the prenatal diagnosis and the video electroencephalogram was normal. Progression of enteral feeding was achieved, without neurological deterioration, and the patient was discharged with recommendations for follow-up. An outpatient magnetic resonance imaging confirms the diagnosis. Case 3: preterm twin of 34 weeks, with prenatal diagnosis of ventriculomegaly. At birth without achieving early saturation goals and on clinical examination with large macrocrania and CT scan showing an image suggestive of open-lipped schizencephaly, associated with hydrocephalus, dysgenesis/agenesis of the corpus callosum. Seizure of difficult control, and required ventriculoperitoneal shunt.

Conclusions: Neonatal clinical course is variable but seizures are relevant. Other assessments may be needed early, including genetic concepts.