Colpocephaly: clinical, radiologic, and pathogenetic aspects

Colpocephaly is an anatomic finding in the brain manifested by occipital horns that are disproportionately enlarged in comparison with other parts of the lateral ventricles. It has been considered to result from localized outpocketing of the ventricular cavity due to failure of thickening of overlying brain. Four new cases are presented here, including a child of near-normal development who appears to be the first reported case of colpocephaly with an associated chromosomal anomaly. Our patients support the view that colpocephaly is a disorder of diverse causation that can arise from a variety of degenerative or encephaloclastic insults to the developing brain.