Semilobar holoprosencephaly

Semilobar holoprosencephaly is a subtype of holoprosencephaly (HPE) characterised by an incomplete forebrain division. It is intermediate in severity, being worse than lobar holoprosencephaly and better than alobar holoprosencephaly.

Please refer on the general article of holoprosencephaly (HPE).

Contrasting from alobar HPE, facial malformations are usually mild or absent in semilobar HPE 5.

As with all types of holoprosencephaly, 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.

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

The basic structure of the cerebral lobes are present, but are fused most commonly anteriorly and at the thalami and there is partial diverticulation of brain (dorsal cyst). Features include 1-4:

An easier approach might be to assess fusion of more than 50% of the frontal lobes which might be suggestive of semilobar HPE 5.

In addition, this condition is associated with mild degree of facial abnormality such as hypotelorism and cleft lip.

General imaging differential considerations include:

Malformations of the central nervous system
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Article information

rID: 7000
Section: Pathology
Synonyms or Alternate Spellings:
  • Semilobar holoprosencephaly
  • Semi-lobar holoprosencephaly
  • Semilobar HPE
  • Semi-lobar HPE

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