Presentation
Newborn female baby with poor feeding and abnormal facies, cleft lip and hypotelorism.
Patient Data
partial cleavage of the cerebral hemispheres with fusion across the frontal lobes
monoventricle with partial occipital and temporal horns of the lateral ventricles
partially fused thalami which are abnormally rotated and anteriorly situated with consequently small sized 3rd ventricle
absence of septum pellucidum
rudimentary falx cerebri and interhemispheric fissure which is formed caudally with cleavage and separation of the occipital lobes
hypoplasia of the corpus callosum
Case Discussion
Semilobar holoprosencephaly is an intermediate form of holoprosencephaly with partial cleavage of the prosencephalon.
Absent septum pellucidum is present in all types of prosencephaly and the presence of septum pellucidum virtually excludes holoprosencephaly.
Alobar holoprosencephaly is non-cleavage of the cerebral hemispheres with single monoventricle (crescentic-shaped holoventricle), dorsal cyst widely communicating with the single monoventricle, horse-shoe configuration of the cerebral mantle, fused thalami & absence of the corpus callosum, falx cerberi, interhemispheric fissure, optic tracts and olfactory bulbs as well as absent 3rd ventricle, internal cerebral veins, vein of Galen, straight sagittal sinus and Sylvian fissures.
Lobar holoprosencephaly is just rostral or anteroinferior fusion of the cerebral hemispheres & the absence of the septum pellucidum is the clue for diagnosis. The differential diagnosis is septooptic dysplasia or De-Morsie syndrome.
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