Welcome. In this talk, we're going to cover
tracheoesophageal fistula and esophageal atresia.
And it may seem like we're talking about two very different
In fact, as you'll see developmentally, they kind of go
So esophageal atresia formally is just where we've lost a
segment of the esophagus.
It doesn't completely develop. So we end up with a
They may even be the two ends of the esophagus may be
connected by a fiber strand but nothing can get through it.
That's esophageal atresia.
On the other hand, a tracheoesophageal fistula is an
between the esophagus and the trachea.
So we're seeing here atresia because the esophagus is
but we're also seeing a fistulous tract where the lower
segment of the esophagus
actually connects to the trachea.
This association is actually the most common of the various
and/or atresias that can occur involving the esophagus and
And then, finally, we have this entity which is a fistula
So a connection between esophagus and trachea
but the esophagus itself is one continuous tube and
everything flows down that way.
With this though, you can probably anticipate that there are
going to be problems
because of the abnormal connections and/or the gaps that can
occur within the esophagus.
So let's talk about how those play out. First, we'll do a
little bit on the epidemiology.
So this is showing the most common of the various anomalies
involving the esophagus and the trachea.
It's a combination of atresia.
So that gap between the top and the bottom of the esophagus
and an associated tracheoesophageal fistula.
Altogether, this particular combination occurs about 85% of
involving the esophagus and the trachea and those anomalies.
When this happens, there is going to be a number of
that are secondary to the fact that the GI tract is no
longer a continuous tube from top to bottom.
In the absence of that continuous tube in the ability of the
to swallow amniotic fluid and move it through the GI tract,
in fact, there will be too much amniotic fluid, so called
There are a number of associated findings that come
along with tracheoesophageal fistula and/or esophageal
The acronym for this is VACTERL, V-A-C-T-E-R-L.
Not a great acronym because it doesn't really mean anything
but it does succinctly describe a lot of the associated
So let's look at those in turn. The V stands for vertebral
And in fact, the number of the defects under VACTERL are
because of some underlying genetic disorder.
So a syndrome and the esophageal atresia or the
tracheoesophageal fistula's just part of that.
Some of the defects that we're going to talk about in
are actually coming from the fact that the baby is not able
to swallow amniotic fluid.
But the vertebral defect by and large is part of a syndromic
something going wrong overall with the development of the
A stands for anal atresia.
This may be primary or it may be related to the fact
that the baby's not able to swallow and passage the fluid
from the amniotic sac.
C are cardiac defects. These are probably part of the
underlying genetic basis of the disorder.
T and E for VACTERL, the T and E are the tracheoesophageal
fistula and/or esophageal atresia.
R stands for renal anomalies.
And again, this may be part of the underlying genetic
or it may be because the baby's not passaging fluid in a
So the fluid is not going across just down the GI tract,
but in fact, is being absorbed and may be part of the
normal, renal development.
And finally, limb abnormalities.
And again, the limb abnormalities may be part of the basic
or it could be because of all the abnormal amount of
that's left behind and there may not be typical development
of arms and legs.
So the pathophysiology, the normal tube that's going to
the bronchial tree and the esophagus is one central midline
tube early on.
There's a ventral aspect that will become the bronchial tree
and there is a dorsal aspect that will become the esophagus.
During normal development, the ventral aspect will branch
and then, branch and branch again to produce the bronchial
The posterior aspect will separate out and become an
esophagus whom we will form a separation
between that with a combination of smooth muscle and
In the setting where we don't have complete development of
that dorsal aspect,
the tube that's going to become the esophagus, we have
So we have a segment that has become stenotic or frankly,
On the other hand, when we form a tracheal esophageal
we have our ventral area that's going to be the bronchial
We have the posterior or dorsal aspect that's going to
become the esophagus.
And then, during development, we end up with either a
complete gap, so we have atresia of the esophagus
and we also have an area where the esophagus is connected
aberrantly to the bronchial tree,
so a tracheoesophageal fistula. And what we're seeing here
is the one that - one entity,
the one malformation that is the most common of all the
of esophageal atresia and/or tracheoesophageal fistula.
The exact etiology remains unknown. There's very likely a
genetic component because half of the cases
have all those associated malformations, remember VACTERL.
The clinical presentation based on what you've already seen
kind of comes from first principles.
There may either be an abnormal connection between the
so when the baby swallows, it goes into the bronchial tree.
Not good. On the other hand, you may have air going in to
the rest of the GI tract
as the baby inhales and we have air going through a
down into the lower end of the esophagus, the stomach, and
So in the infant who has one of these anomalies,
the symptoms usually manifest pretty early, usually, within
the first 24 hours.
With esophageal atresia where there is not a complete
formation of the esophagus
and there's gap, the baby will have drooling, an inability
to feed, and regurgitation.
Now, this is completely an absence of anything connecting
the esophagus to the trachea.
This is just atresia. In tracheoesophageal fistula
however, every time the baby ingests something or even just
that is going to be going into the lungs, not a good
because now, the baby's going to have respiratory distress.
The baby's going to have coughing and choking.
Baby will likely develop an aspiration pneumonia and the
because there is now probably a distal connection from the
trachea into the lower GI tract,
every time the baby inhales, there will be now air going
into the stomach,
so there will be abdominal distention, basically,
hyper-inflating the GI tract.
So the diagnosis, not a hard one based on what you're seeing
but nevertheless, one that has to be recognized very early
otherwise, the baby will get into a lot of trouble.
So on physical exam, a simple thing is that frequently,
if there's drooling and the baby's not apparently able to
move fluid from top to bottom,
we'll pass a nasogastric tube or will attempt to do that.
In a setting of esophageal atresia, you put it in there and
it doesn't go anywhere.
So it will often come back out as you do that.
If there is a tracheoesophageal fistula such as you see here
with the dotted line and the arrow, there will be abdominal
Baby - every time the baby inhales, air is getting out into
the GI tract
and it'll just inflate the stomach. How can we see this on
So a plain radiograph frequently will help you make the
You may see a completely gas filled gastrointestinal tract
that says you probably have a tracheoesophageal fistula.
On the other hand, the nasogastric tube,
you can see it here is that very electron-dense line
that's coming from the head area down,
trying to go into the esophagus, curls up, and comes back
So that's saying that we have esophageal atresia.
You may also, if you have just isolated esophageal atresia,
so no fistula, just atresia, you may have an entirely
And usually, the baby is sucking down some air.
So there should be some air, just shouldn't be
What do we do about this? Okay. We need to recognize it
When we do recognize that this is going on,
we want to keep the baby in the head up position.
We don't want to regurgitate gastric contents through a
into the lungs that will cause very nasty chemical
We definitely want to provide nutrition, so we'll put them
on total parenteral nutrition.
We are going to give them broad spectrum antibiotics just to
against the possibility that they have aspirated.
And we're going to completely suction everything that ever
into the esophagus just to keep that from regurgitating.
And then, we call our friendly surgeons and we say,
"Dudes, you need to come in and you need to now reestablish
across the atretic portion of the esophagus if present
and you need to cut the connection between the esophagus and
The prognosis if we do everything that we're supposed to do
and we recognize the entity early on, survival is quite good
with surgical treatment
and in fact, the early [inaudible 11:11] are usually not
associated with the esophageal or tracheal anomalies
but rather due to the other associated anomalies, the heart
defects, the renal defects, etc.
And with that, we finish tracheoesophageal fistula and