Presentation
Cerebral palsy.
Patient Data
The posterior horns of both lateral ventricles are disproportionately enlarged compared to the anterior horns in keeping with colpocephaly. There is fusion and squaring of the anterior horns. The septum pellucidum is absent.
There is prominent polymicrogyria in both cerebral hemispheres.
Corpus callosal dysgenesis with deficient posterior body and splenium is also noted.
Case Discussion
Holoprosencephaly is a rare congenital brain malformation resulting from incomplete cleavage of the two cerebral hemispheres. There are three classical subtypes; alobar, semilobar and lobar holoprosencephaly.
Lobar holoprosencephaly is the less severe form. Findings may include fusion of the anterior horns, absent septum pellucidum and corpus callosal dysgenesis which were demonstrated in this case. Polymicrogyria is also demonstrated which has been associated with absent septum pellucidum1.