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Encephalocele - A Rare Infant Disorder

By Chloe Thorn, published Aug 20, 2007
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Encephalocele is a very uncommon disease where babies are born with a gap or opening in their head sometime allowing their brain to protrude. Or in other words the outer skull or plates that encompass the brain normally are not doing so. They do not connect correctly leaving a large open gash. What starts the opening is that the neural tube of an infant does not seem to develop at the first stages of pregnancy. This usually only affects 1 in 5,000 babies that are born presently. Out of that small percent only 4 babies have surgery a year for this disease.

For some reason where the Encephalocele happens is different for female and male children. For female babies the opening is usually in the front area of their head, and for boys the back. The severity and width of this gap on a babies head can actually be attributed to location it seems. The third worlds countries see this more often, and the poorer the people the worse it is. This hints at poor nutrition playing a part in creating the problem.

Other names you may find this under is Bifid Cranium or Cephalocele. The cause for this terrible infant syndrome is mostly unknown. They believe there could be an issue with low folic acid causing this.

This disease usually does not cause death in babies although it can cause mental health issues down the road. At times if the brain is protruding out of the child's skull it is still encased in a sac that saves it from being exposed and causing lethal damage. Otherwise it is covered with skin or may even be completely open.

The only resolution for this severe infect defect is to have surgery done. If the opening is exposed or only covered in the sac that encircles the brain than the surgery has to be done immediately after birth. However the more common practice is the children whose skin still covers the opening and surgery does not have to be done till the child is a little older.

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While I appreciate that you tried to shed light on this subject, you've done it no justice. First of all, it's a birth defect - not a disease. Also: *The skull plates do not form properly because the neural tube fails to close (it does develop). *Another reason that more severe encephaloceles are seen in third world countries is because there aren't routine ultrasounds in these countries, thus parents do not know and do not abort the fetus. (90-95% of encephalocele pregnancies are terminated). *It is not so much an issue of disease causing fatality, as it is infection from staphylococcus which lives on our skin normally. The membranes of an encephalocele can be extremely thin, or rupture causing the staph to grow at an amazing rate. Any infection that makes it to the bloodstream can cause sepsis (blood poisoning). *Often times encephaloceles that are surgically repaired to continue to have problems including developing hydrocephaly and requiring a shunt to regulate this problem. H

Posted on 05/07/2008 at 3:05:12 AM

 
Thanks for this information. I haven't heard of Encephalocele before this!

Posted on 08/23/2007 at 8:08:00 AM

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