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Classification of agenesis of the corpus callosum with interhemispheric cyst after Barkovich et al (3)
Type 1^*
Subtype Cyst Characteristics Communication Associated Abnormalities
Type 1a: Presumed communicating hydrocephalus Isointense to CSF (MR), unilocular Communication with lateral ventricles only Macrocephaly, hydrocephalus, Dandy-Walker malformation
Type 1b: Hydrocephalus secondary to diencephalic anomaly Isointense to CSF (MR), unilocular Communication with and obstruction of third ventricle Macrocephaly, diencephalic malformation (eg, thalamic fusion without subcortical heterotopia)
Type 1c: Small head size and cerebral hypoplasia Isointense to CSF (MR), unilocular Communication with lateral and third ventricles Microcephaly, cerebral dysplasia or hypoplasia
Type 2^†
Type 2a: No abnormality apart from ACC Isointense to CSF (MR), multilocular No communication with lateral or third ventricles Macrocephaly, hydrocephalus
Type 2b: Aicardi syndrome Hyperattenuation (CT), hyperintense (T1W MR), multilocular No communication with lateral or third ventricles Female predominance, subependymal heterotopia, polymicrogyria, seizures, hypoplastic falx cerebri, uni- or bilateral ventriculomegaly, developmental delay
Type 2c: Subcortical heterotopia Isointense to CSF (MR), multilocular No communication with lateral or third ventricles Subcortical heterotopia, developmental delay
* Extension or diverticulation of third or lateral ventricles.
† Loculated, lack of communication with ventricular system.