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>> Fetal gastroschisis is a anomaly
that we observe in fetuses
that has to do with the abdominal wall.
What we find, is that in certain fetuses,
the abdominal wall doesn't form completely
and there ends up being a defect, or hole,
right next to the umbilicus,
usually on the right side of the umbilicus,
the belly button.
And that allows intestine, or other viscera,
to actually herniate outside of the abdominal cavity
into the amniotic space.
Usually it's only intestine,
but sometimes they'll be other organs there.
And the cause
is not yet really known,
but we do have some epidemiologic understanding
of what seems to be associated with it.
For example, young maternal age, as well as smoking,
are heavily associated with this defect.
And we believe that there is likely
a toxin in the environment that contributes to it.
There may be more than one toxin.
We have noticed an increase
in this disease around pesticides
and also around the use of vasoconstrictive drugs.
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Gastroschisis generally,
is a very well-tolerated defect
that has a overall survival around 98%.
The issues we run into during pregnancy
generally occur later on, at around the 36 to 38-week point.
We do find, that on average, these babies tend to deliver
a little bit prematurely
and some of them will be a bit small,
compared to normal babies that are developing.
We tend to err on
the side of watching these babies more carefully,
than we would babies without such an anomaly
in order to make sure
that all of their biophysical parameters
are optimally maintained
and that we can intervene and deliver earlier, if need be.
In terms of the mode of delivery,
we haven't found any evidence to suggest
that from the perspective of the gastroschisis,
a cesarean section is indicated for this condition.
And that's very important,
because a lot of the people that have gastroschisis,
are young and it's their first pregnancy,
and we'd like to have
as little morbidity for the mom, as possible.
And so, that's quite important.
Obviously, a C-section could be indicated for other reasons
that have to do with obstetrics, or any other indication.
But just from the perspective of gastroschisis
a C-section is not mandated.
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Gastroschisis is treated after the baby is born
by having a extremely coordinated,
and multi-disciplinary team approach.
Here at the Children's Center,
the neonatologists attend the birth.
And the first step, once the baby is delivered,
is to make sure that the baby's breathing appropriately
and that the heart is maintaining
its circulation appropriately.
But then we move on to
the gastroschisis specific evaluation.
And the primary goal there,
and this is all sort of happening at the same time,
is to make sure that the bowel is well-protected.
And the way that we do that is by placing the bowel,
and actually the lower extremities
of the baby, and lower abdomen, in a plastic bag,
that both allows the bowel to have a barrier
between it and the outside world,
and also, preserves heat and fluid.
So that the baby doesn't get dehydrated,
it doesn't get cold.
And then, our pediatric surgery team comes in,
and makes sure that the bowel is oriented correctly
and is ready for a primary closure.
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Gastroschisis in general,
as compared to another abdominal wall defect, omphalocele,
is much more isolated.
Generally, in about 85 to 90% of the cases,
it's the only defect that the baby has.
We are able to treat that defect
in almost all cases
and have the baby go on to live a pretty normal life
without any consequences.
However, in a small percentage, about 10 to 15%,
there are other anomalies.
The most frequent other anomaly,
is something called an atresia.
That's when there's discontinuity in the bowel.
And that may be because of an improper blood supply
to the intestine, as it's floating in the amniotic cavity,
or because the ring around which the,
or inside of which the bowel actually is herniating,
gets too tight and cuts off the blood supply,
or the blood that's returning to the baby.
In those cases, what we do,
is we wait until we think the baby is stable,
and then we perform abdominal surgery
here at the Children's Center,
to reconnect a bowel that's interrupted,
or fix what other defect may be present.
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So, the good news about gastroschisis,
is that overall survival is excellent,
here in the United States,
and also here at the Children's Center.
The survival rates that we have seen,
are basically around 98 to 99%.
So mortality is really not an issue.
In terms of the way the belly button looks
with our new closure technique, using the sutureless repair,
we have found that the belly button looks excellent.
In the past, whereas the belly buttons
didn't look that good, now they look great.
What we do find, in general,
is that these babies do have some degree
of what we call, bowel dysmotility.
They will have either a little more constipation
or a little more diarrhea
than you would expect in a normal child.
We think that has to do with some kind of injury sustained
from the intestine being on
the outside of the body during pregnancy.
We have seen no evidence of any neurocognitive defects.
And basically, these kids are able to thrive
and grow as completely normal kids.
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What we do, is we give the baby some pain medicine
and make sure that the baby's comfortable.
And then we very carefully, under sterile conditions,
put all of that intestine that's on the outside
into the baby's abdomen, very carefully,
with the goal of not creating any damage
and not providing too much stretch on the abdominal wall.
We do generally, get all of it back in on that first go.
And then once we've done that and put it back in,
in an orderly fashion, we put over a special bandage
that allows the abdominal wall to essentially heal
over the viscera that we've put back in.
And that works great,
and it ends up with an excellent cosmetic result.
Sometimes we'll have to delay that procedure, a little bit,
if the abdominal contents won't fit,
if the intestine won't fit into the abdominal cavity.
'Cause we don't wanna push it
and possibly compromise the bowel in any way.
And so in that situation, we'll use a spring-loaded silo,
that we can put the intestine into,
and actually we'll stretch the abdominal wall.
And over a period of days,
allows the intestine to descend into the abdominal cavity.
And that works quite well,
and still allows us to perform the sutureless repair.
In some cases, where it's more complicated
and the intestine's either stuck to the abdominal wall
or there is a large amount of intestine that's compromised,
we'll actually do an operation right away
in the operating room where we will address
what other defects there are in the intestine,
and also close the abdominal wall with sutures.
That leads to a slightly less optimal cosmetic result,
but we pride ourselves on making it look
as good as possible.
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(children talking and giggling)