acrania

semstr · Oct 29, 2001

Hi,

looks like anencephaly to me too,

had two births like (I mean witnessed them) both babies were 32 weeks of gestation, one of them was dead at birth and the other survived for a week!

We didn't prolonged life though, but this baby breathed on its own and even drank a little bit. There was quite a bit of prolonged braintrunk , but after a week she died.

It was very sad and tragic, also she didn't look very nice, her head was very strange of course, hardly a skull, just tissue.

For her parents it was a very important week, they could say goodbye to their child, hold her sing to her kiss her and she even opened her eyes sometimes.

It was a cery touching experience for all of us working NICU.

So, yes everything is possbile, but the tip with the ethical commission is very important. Ours' had meetings almost every day at that time, becuase it was the first time a child like that survived that long. Nobody really knew what to do.

We still have contact with these parents, they had a healthy girl three years after this sad experience!!

Take care, Renee

jama · Oct 30, 2001

Thanks for your replies. I have seen a few anencephalic babies after delivered . I don't think any were born alive. i have not cared for the moms while in labor. this baby is supposed to have a brain but missing skull. I also had not heard of this and was really clueless of how bad it could be. i didn't know if maybe grafts or something could be done. Also i was more concerned about being told 'do nothing" at birth and not knowing is baby going to die from defect or because she's32wks and i didn't do anything. also there was a case few years ago here where an anecephalic went home and lived several weeks. also a friend of mine cared for a 5 yr. old anecephalic in a nursing home. i quess i was hoping to hear something could be done for this baby. jama

**canoehead, BSN, RN** · Oct 31, 2001

A friend in high school had a child with anencephaly, diagnosed inutero. She chose to have the baby, and he lived 6 years I believe but with constant health problems and severe developmental delay (of course).

Jolie, BSN · Nov 1, 2001

Anencephaly is a type of neural tube defect which occurs when the cephalic (head) end of the neural tube (fore-runner of the brain and spine) fails to close in the 3rd-4th week of pregnancy. These babies are born without a forebrain or cerebrum, and are not believed to be capable of conscious activities or thoughts. The bony structure of the skull may be missing, and brain tissue may be exposed, or may be covered by skin. Since they may have a functioning brain stem which controls heart beat, respirations and some reflexes, it is possible for these infants to live for a period of time, although it is not likely that they will achieve any significant developmental milestones. Most of these infants die within the first few hours or days of life.

Some parents opt to take their children home with the support of home care or Hospice. With the availability of testing and ultrasound, the diagnosis of anencephaly is usually made prenatally, allowing the parents time to plan and prepare for their baby's birth and care. I can't imagine the agony of carrying a baby to term knowing that it has a fatal defect. These parents need all the support we can offer.

Research has shown dietary supplementation with folic acid to be effective in reducing cases of neural tube defects in general. It is not certain whether folic acid supplements can reduce the number of cases of this specific type of defect.

KRVRN, BSN, RN · Nov 1, 2001

I would think that even if the brain wasn't damaged by trauma or something funky in the amniotic fluid, it would still be damaged somehow and you just wouldn't be able to protect the integrity of the brain tissue.

I'm saying this because we received a baby with a myelomenigocele in the NICU. It was prenatally diagnosed so she was delivered c-section. No trauma to the spinal cord. The sac covering the lesion was ruptured and had been for awhile (occurred in utero) and the area was swollen and macerated-looking. She had no movement or apparent feeling below the lesion. It was assumed that the spinal cord had been damaged from contact with the amniotic fluid for however long (although I'm suppose it could have received some mechanical damage in utero from movement).

As I recall, gastroschisis babies DO have some swelling and/or damage to the intestines from the contact with the amniotic fluid. It's just that the intestines heal without problems. Brain tissue isn't as forgiving.

Am I way off in thinking that just being in contact with the amniotic fluid would probably damage the brain tissue?

Jolie, BSN · Nov 1, 2001

Dear KRVRN,

You are most certainly right in your thinking that exposure to amniotic fluid is damaging to tissues. That is the rationale behind the in-utero repair of spina bifida. Research has shown that babies who have pre-natal repair of their spinal defects have better motor and sensory outcomes than those who don't, apparently because the delicate nerve tissues of their spinal cords have less exposure to the caustic effects of amniotic fluid. It is somewhat hard to understand how amniotic fluid could be so damaging, but I guess it was simply never intended to come in contact with nerve tissue.

Not all babies are candidates for in-utero repair, and this surgery carries many serious risks of its own, prematurity chief among them. But it may offer hope to parents facing this life-long challenge.

Jolie, BSN · Nov 1, 2001

Kday,

If an anencephalic baby has skin covering his scalp, it will act to protect the brain tissue from the damaging effects of the amniotic fluid. But caustic amniotic fluid is not the only problem these babies face. They are born without vital parts of their brains. The forebrain and cerebrum are missing, the areas which control conscious thought, activity, sensations, etc. They have only various levels of brain stem functioning. Since the brain stem controls heart rate, respirations, and some reflexes, these babies may live for some time, but will not develop the skills, abilities, or awareness we normally expect of growing newborns.

The baby you describe apparently had a defect of the bony structure of the scalp, but was spared any neurological problems. Did he require reconstructive surgery or protective headgear? It seems that he would be at very high risk for neurological damage if he were to suffer even a minor injury involving that portion of his head.

Jolie, BSN · Nov 2, 2001

OK, I finally found some useful information. Go to Google.com and type in acraina. The first listing is a study done at Vanderbilt University of several babies diagnosed with this condition. The second listing shows ultrasounds and post-delivery photos of fetuses with acraina. How sad, they are perfectly formed otherwise.

It gives the following definition: a developmental anomoly resulting in the partial or complete absence of the neurocranium. Brain development is complete but abnormal. It is a uniformly fatal condition.

So, whatever your little patient had, it must not have been a variation of this.

Never thought I'd come across something worse than anencephaly.

jama · Nov 29, 2001

thanks for all the replies. This pt ended up delivering a week later at a small community hosp er (no ob dept). She arrived and was complete (and breech) they said the baby lived about 3 hours. jama

betts · Nov 29, 2001

FYI:

http://www.beebware.com/directory/Health/Conditions_and_Diseases/Neurological_Disorders/Congenital_Anomalies/Anencephaly/

rph3664 · Sep 24, 2006

A friend in high school had a child with anencephaly, diagnosed inutero. She chose to have the baby, and he lived 6 years I believe but with constant health problems and severe developmental delay (of course).

There was a story in my local paper a while back about a family who had a 6-year-old daughter with hydranencephaly, where the brain does not develop but the head looks normal because the skull is filled with fluid.

I work with a woman whose son is severely brain-damaged due to meningitis, and they go to the same school. It's one of those schools that basically exists to provide therapy to children whose IQs are essentially zero, and that's the case here. The little girl is on a G-tube and they initially thought she was blind and deaf but they have since learned she isn't. My co-worker of course knows the girl and her family, and she says the girl has the sweetest laugh. The parents aren't married and are quite scruffy looking so some people might get the wrong idea, but she says they are actually very nice people and they take good care of her AND also manage to care for their 3 teenagers by prior relationships.