Holoprosencephaly
Background
Holoprosencephaly: HPE
Holoprosencephaly is a series of brain malformations in which there is incomplete development of the cerebrum. Its effects on the child range from severe and lethal to mild and almost undetectable.
In the most severe form, alobar HPE, the cerebrum is a single U-shaped mass rather than being divided into right and left hemispheres. Death most often occurs before, during or soon after birth. Among liveborn children with alobar HPE, half will have died by four months of age, but survival for several years has been noted in a number of children.