Holoprosencephaly

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Holoprosencephaly

Last updated on Tuesday, September 9 2008 by jdmiles

Related images:

Cyclopia in holoprosencephaly
In general, severity of facial malformations parallels those within the brain. Although patients with severe brain anomalies may have relatively normal-appearing facies, the converse does not appear to occur.

Alobar holoprosencephaly, Coronal section through brain
In addition to the single large hemispheric ventricle, the deep gray matter structure is also demonstrate fusion.

Holoprosencephaly, dorsal view of autopsy brain
Failure of ventral induction results in a continuous cerebral holosphere. The posterior roof of the single ventricle may be seen bulging dorsally between the holosphere and the thalamus.

Holoprosencephaly, ventral surface of brain.
Holoprosencephaly has been associated with a wide variety of cytogenetic abnormalities. This patient demonstrated trisomy 13.

Holoprosencephaly, in situ photograph, coronal section through skull
This in situ section demonstrates the morphology and continuity of the single dilated ventricle.

Isolated arhinencephaly, ventral surface of brain
Although the olfactory bulbs are commonly disrupted during brain removal, the absence of olfactory grooves and this brain confirms olfactory hypoplasia. Isolated absence of the olfactory bulbs may occur as an incidental finding at autopsy, but may also be...