Colpocephaly: Pitfalls in the Diagnosis of a Pathologic Entity Utilizing Neuroimaging Techniques

Abstract

Colpocephaly has been described as the persistence of the fetal configuration of the lateral ventricles. The pathologic picture is characterized by multiple features of disturbed or arrested development of the brain, which results in diminished thickness of the cerebral white matter in the posterior portion of the centrum semiovale, giving rise to large occipital horns of the lateral ventricles. This ventricular configuration allows the clinician to suspect the presence of this developmental disturbance utilizing computed tomographic images. In this paper, we present a case that demonstrates that not every patient with enlargement of the posterior horns of the lateral ventricles has the underlying developmental abnormalities that constitute colpocephaly as described by Yakovlev and Wadsworth. Furthermore, we point out the difficulties that can arise in the attempt to make a diagnosis of a pathologically defined condition on the basis of neuroimaging results alone. (J Child Neurol 1990;5:166-168).