) is a condition that affects the brain
and the mid-line organs
such as the heart
, and kidneys
. It results from failure of the prosencephalon
into the cerebral
and lateral hemispheres
. It is found in 1:16000 live-born
There are three types of HPE:
- where there are two lobes
in the brain.
- where there are two lobes to the rear but not the front of the brain.
- where there are no lobes at all.
HPE can be:
- it can be caused by a genetic
problem in one or both sides of the parental families. This is what your geneticist
is for. If you don't have one, then ask your own doctor to get you in touch with one.
- "they" just don't know. Loads of causes have been sited from dry cleaning fluid to alcohol
. The best thing here is not to blame yourself, and not to worry too much about cause in the early days.
HPE is related to:
- a general term given when the head size of the child is said to be below the 5th centile
of what is seen to be normal growth. These "centiles" are different depending on where you live and who calibrated the ultra-sound
scanner. So don't go getting worried too early. However, if you are scanned and the head of your baby is found to be significantly smaller than expected, it is a good idea to have a further set of tests to assess the liklihood of HPE.
- is usually defined as the presence of excessive cerebral spinal fluid
) within the cranium
. However, this situation is sometimes referred to as dilated ventricles
, and hydrocephalus
is restricted for use only when intracranial pressure
is increased. Congenital hydrocephalus
occurs in .3 to .8 per 1,000 births.
variable degrees of mental retardation
abnormalities of other organ systems such as cardiac
, and gastrointestinal
Mild forms of facial abnormalities
may include a flat single-nostril
), close set eyes (hypotelorism
), cleft lip
, or just one upper middle tooth (single maxillary
More severe facial deformities
may include a single central eye (cyclopia
), a nose located on the forehead (proboscis
), or missing facial features
More Information: www.hopkins-id.edu/