Abstract

Holotelencephaly is a congenital malformation of the telencephalon which characterized by developmental failure of the cerebral hemispheres. It is frequently associated with midline facial defect. There are rare cases of holotelencephaly not associated with midline facial defect. We have experienced an autopsy case of atypical form of holotelencephaly characterized by partial fusion of cerebral hemispheres at the parietal region with developmental failure of the corresponding lateral ventricle. On the other hand, interhemispheric fissure of the frontal and occipital regions was well developed as well as lateral ventricle of the corresponding regions. No facial anomaly was present in this case. The above findings do not seem to coincide with reported cases of holotelencephaly. It is postulated that this is an unusual variant of holotelencephaly because of the developmental correlation of the cerebral hemisphere and lateral ventricle.