Abstract
A series of 20 cases from 2 academic institutions is presented with a
characteristic imaging triad of asymmetric ventriculomegaly, a large
interhemispheric cyst, and partial or complete agenesis of the corpus callosum.
Most cases were initially referred as aqueduct stenosis and hydrocephalus or
focal porencephaly. We describe the imaging findings that identify an abnormal
or absent corpus callosum associated with a type 1 interhemispheric cyst in
fetuses initially thought to have hydrocephalus attributable to aqueductal
stenosis. We suggest that the acronym AVID (asymmetric ventriculomegaly,
interhemispheric cyst, and dysgenesis of the corpus callosum) may be useful in
recognition of these cases. All cases presented with markedly asymmetric
ventriculomegaly on initial sonography, with progressive hydrocephalus
throughout gestation. Fetal magnetic resonance imaging was performed in 15 of
20 cases. Thirteen of 20 cases were identified in male fetuses. Associated
fetal and postnatal abnormalities are also reported. Technological improvements
in sonography and fetal magnetic resonance imaging allow improved
characterization of associated intracranial anomalies in the setting of
hydrocephalus. Accurate diagnosis can aid parental counseling, especially
because isolated aqueductal stenosis suggests a better prognosis than
hydrocephalus with anomalies. Markedly asymmetric ventriculomegaly in this
series was the key to excluding isolated aqueductal stenosis and was associated
with callosal malformation with a type 1a interhemispheric cyst.
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