Once in a lifetime

Today I saw something that most people only see once in a lifetime of working in medicine in the United States. Anencephaly. Anencephaly is defined as "congenital absence of the brain and cranial vault, with the cerebral hemispheres m issing or reduced to small masses. This condition is incompatible with life." The way this happens is early in pregnancy, a complication of development of neural tube closure, often due to a lack of folic acid intake before the mother even knows she is pregnant (hint - those of you women of childbearing age - take folic acid). You very rarely see a full term baby born with this condition mostly due to the fact that it can be detected early enough in a pregnancy that the pregnancy is terminated. The mother was pregnant with twins; one perfectly healthy and one that she knew would be born with anencephaly. Most babies born with anencephaly will survive a few hours a most. The reason they even survive those hours is that sometimes they are born with an intact brainstem, the part that sustains the necesities of life such as a heartbeat and breathing (the reason you can be "braindead" but still be breathing). Interestingly enough, at PCOM one of our "babies in a jar" (babies that were stillborn or pregnancies that were terminated) - was an anencephaly baby, but only about 28 weeks gestation when it was terminated (full term is 38-40 weeks). But the baby I saw today was still premature, but was about 33 weeks I think. Amazingly, she has survived about 24 hours breathing on her own, nothing keeping her alive. In the rare cases this happens, the baby will likely die of meningitis due to the fact that the little brain she has is exposed to open air, nothing to protect it from bacteria. In looking for a picture to show you of an anencephaly baby born at nearly full term I am having little luck. Many ultrasound pictures. Many pictures of 20 week terminated fetuses.

Those of you that don't want to see, don't scroll down any further. The first picture isn't bad, but the next 2 are if this might gross you out.

This is a set of twins, not unlike the ones I saw today. As you can see, if you put a hat on the anencephaly baby it dosen't look too bad. This is what they often do for the family.


This is what a baby with anencephaly would look like from the front.


And... from the back