Lobar holoprosencephaly

Lobar holoprosencephaly is the least severe of the classical subtypes of holoprosencephaly (HPE), characterised by the presence of the inter-hemispheric fissure along almost the entire midline, and with the thalami being completely or nearly completely separated 5.

As with all types of HPE, it is a rare congenital brain malformation in which there is failure of complete separation of the two hemispheres and failure of transverse cleavage into diencephalon and telencephalon.

For a general discussion of epidemiology, clinical presentation and pathology, please refer to the article on holoprosencephaly (HPE)

Radiographic features

As will most cerebral structural congenital abnormalities, lobar HPE is visible on all modalities, but in general is identified on antenatal ultrasound, and best characterised by MRI. 

Unlike the more severe forms, in lobar HPE the cerebral hemispheres are present. Features include 1-4:

Unlike semilobar holoprosencephaly, the falx is present, the interhemispheric fissure is fully formed and the thalami are not fused. 

An easier way to assess may be looking for the presence of a fused fornix which could be seen as an anteroposteriorly aligned echogenic linear structure within the third ventricle.

Differential diagnosis

  • semilobar holoprosencephaly
    • fusion of the anterior aspects of the hemispheres
    • falx and interhemispheric fissure incomplete
    • partial fusion of the thalami
  • septooptic dysplasia
    • can appear very similar
    • fusion of the frontal lobes and of the fornices is not a feature

Malformations of the central nervous system
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Article Information

rID: 10508
Tag: cases
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