Neurological and clinical outcomes in infants and children with a fetal diagnosis of asymmetric ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum

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  • 1 Division of Neurological Surgery, University of Vermont Medical Center, Burlington, Vermont;
  • | 2 Division of Pediatric Neurology, Department of Pediatrics, University of Utah, Primary Children’s Hospital, Salt Lake City;
  • | 3 Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City; and
  • | 4 Division of Pediatric Neurosurgery, Department of Neurosurgery, University of Utah, Primary Children’s Hospital, Salt Lake City, Utah
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OBJECTIVE

Advances in prenatal imaging have facilitated improvements in the fetal diagnosis of congenital anomalies. Asymmetric ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum (AVID) is a constellation of congenital anomalies reported in fetal imaging. However, few data are available regarding postnatal outcomes of infants and children with a fetal diagnosis of AVID. The authors sought to report the neurodevelopmental outcomes of patients diagnosed with AVID before birth at a single institution.

METHODS

An institutional fetal imaging database was queried to identify cases with ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum over a 10-year study period from 2000 to 2019. Overall, 41 maternal-infant dyads who met imaging criteria for AVID were identified; medical records were reviewed for prenatal variables including gestational age at birth, perinatal complications including fetal demise, and postnatal variables including demographics, mortality, hydrocephalus diagnosis and management, epilepsy, and neurodevelopmental outcomes at 2 years or the last follow-up.

RESULTS

Among 41 patients, 25 (61%) were male. A slight majority of patients (55%) were born before 36 weeks of gestational age, and 27 patients (68%) were delivered via cesarean section because their head size precluded vaginal delivery. There were 8 incidences of fetal demise, 1 pregnancy was terminated, and 32 patients were born alive. Neonatal or early infant death occurred in 5 patients. Two children died during follow-up after the neonatal period (ages 7 months and 7 years). Twenty-six children survived to at least the 2-year follow-up, all of whom required treatment for hydrocephalus. Of those 26 children, 12 (46%) had a diagnosis of epilepsy, 14 (54%) could sit independently, 4 (16%) were in mainstream school, 16 (62%) had expressive language, and 7 (28%) had near-normal development without seizures.

CONCLUSIONS

Among 41 maternal-fetal dyads with AVID, a majority of children survived to the 2-year follow-up, although all developed hydrocephalus. Many continued to have seizures, but expressive language use, attendance at mainstream school, and near-normal development without seizures were not infrequent. These data are critical for prenatal counseling and to establish the natural history of a diagnosis with limited outcome data.

ABBREVIATIONS

AVID = asymmetric ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum; BPD = biparietal diameter; ETV = endoscopic third ventriculostomy; GA = gestational age; OFC = occipital frontal circumference.

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