Colpocephaly in adults

Abstract

Colpocephaly is a congenital abnormality in the ventricular system of the brain. The radiological diagnosis is usually made in the perinatal period and later presages intellectual disability. Adult cases of newly diagnosed colpocephaly have only rarely been reported. We have studied an adult with massive colpocephaly who is an otherwise functional woman. The diagnosis should be considered in patients with ventriculomegaly disproportionately affecting the occipital horns and must be differentiated from the more common form of adult ventriculomegaly-idiopathic normal pressure hydrocephalus (NPH).

Figure 1

CT scan demonstrating ventriculomegaly and punctate calcifications within the parenchyma, consistent with the given history of congenital toxoplasmosis.

Figure 2

Non-contrast brain MRI in the (A) axial plane showing massive dilation of the occipital horns and of the third ventricle and (B) sagittal plane, again showing massive ventriculomegaly, as well as agenesis of the corpus callosum.

Figure 3

The posterior to anterior (P/A) ratio is calculated by taking the maximal width of the posterior horn and dividing by the maximal width of the anterior horn of the lateral ventricle. A ratio ≥3 is specific for colpocephaly.