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secondary distention of the ventricles. Ventriculomegaly occurs in the absence of altered cilia-generated CSF flow or congenital aqueductal obstruction. Neuroimaging studies in patients with TRIM71 mutations also suggest cortical hypoplasia as the primary physiological factor leading to secondary ventricular dilation and passive pooling of CSF.CONCLUSIONS: We highlight the importance of precisely regulated neural stem cell fate and neurogenesis for maintaining normal brain-CSF biomechanical interactions that limit ventricular expansion. We thus demonstrate that some or potentially many forms of CH are in fact a congenital brain malformation akin to microcephaly with secondarily enlarged ventricles rather than a primary disturbance in CSF circulation.

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