FRCR CNS / Paediatrics : Holoprosencephaly

Holoprosencephaly

A spectrum of congenital brain malformations resulting from incomplete cleavage into the two hemispheres.

Types: Lobar, Semi lobar and Alobar

Lobar Holoprosencephaly

1. Focal areas of incomplete fusion anteriorly (usually at the fornix)

2. Mild fusion of the frontal horns of the lateral ventricles

3. Normal thalamus

4. Absent septum pellucidum and corpus callosum

Alobar Holoprosencephaly

1. No midline cleavage

2. Cerebral hemispheres are fused with a single midline ventricle

3. Fused thalamus

4. Absent Septum Pellucidum, corpus callosum, interhemispheric fissure, falx cerebri

Associated with facial abnormalities such as cleft lip/palate

References

1. Agrawal R, Yap J, Sharma R, et al. Holoprosencephaly. Reference article, Radiopaedia.org (Accesse...