Abstract

INTRODUCTION: Gray matter heterotopia is a cortical malformation and a known risk factor for seizures and epilepsy. Historically, presence of heterotopia in one or both cerebral ventricles was a concern for postnatal neurological status in patients undergoing fetal closure of myelomeningocele/myeloschisis. There is little information known regarding the overall impact of heterotopia in this patient population. METHODS: A retrospective chart review was conducted for all patients evaluated at the Center of Fetal Diagnosis and Treatment with a diagnosis of myelomeningocele or myeloschisis between June 2016 to March 2023. Gray matter heterotopia was determined from prenatal US and confirmed with prenatal and postnatal MRI. The Bayley Scales of Infant and Toddler Development (edition III and IV) were used for neurodevelopmental assessments. RESULTS: Of 497 patients evaluated with a prenatal diagnosis of myelomeningocele or myeloschisis, 99 were found to have gray matter heterotopia on prenatal MRI. In the postnatal period, 347 patients ultimately delivered at our institution, of which 26 had confirmed gray matter heterotopia on postnatal MRI as well as underwent neurodevelopmental testing. The median age at neurodevelopmental assessment was 13.3 months. The median cognitive and language scores (95 and 99, respectively) were average in those patients with gray matter heterotopia. CONCLUSIONS: This study suggests that the presence of gray matter heterotopia in patients with prenatal diagnosis of myelomeningocele or myeloschisis is not associated with increased cognitive or language delay on neurodevelopmental testing at approximately 1 year of age. These findings can aid clinicians during prenatal consultation regarding fetal repair of spina bifida.

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