Presentation
Developmental delay.
Patient Data
A right parietal external ventricular drainage (EVD) tube is noted.
Absent septum pellucidum and corpus callosum agenesis are noted.
Bilateral asymmetrical dilatation of both occipital horns of lateral ventricles, the right side being larger, suggesting colpocephaly, along with prominent temporal horns.
A right parietal external ventricular drainage (EVD) tube is noted.
Corpus callosum agenesis is noted.
Case Discussion
The Barkovich classification of interhemispheric cysts associated with agenesis of corpus callosum (ACC) includes type 1 cysts, which appear unilocular and communicate with lateral or third ventricles (diverticula), and type 2 cysts, which are often multiloculated and do not appear to communicate with the ventricular system 1.
Type 2 cysts are further divided on the basis of imaging appearance and co-existing morphology. Type 2c cysts are associated with subcortical heterotopia and thus the diagnosis in this instance 1.
Additionally, in the presence of asymmetric ventriculomegaly and dysgenesis of the corpus callosum along with the interhemispheric cyst, features are suggesting the triad of congenital cerebral anomalies: Asymmetric ventriculomegaly, interhemispheric cyst and dysgenesis of the corpus callosum (AVID).