Again, the goals are quite simple. The child should be able to eat
normally and as they wish. There should be no need for a g-tube for
supplemental feedings or for medications.
So far, we have met our goals. All of the children (about 90 cases) have
had a true primary repair. No interposition grafts have been done since
1983. Only the most recently repaired infants have not converted
completely to normal diets and still have their g-tubes. They are on track,
however, and should accomplish this soon.
We believe the benefits of a true primary repair will only increase with
time.
a. The treatment plan
We believe that a true primary repair can be reliably achieved no matter
the length of the initial gap between esophageal segments. Although the
end result will be the same, the operations themselves may vary
depending on how long a gap is present. As detailed in reference 3,
gaps (up to 4-5 cm) that would usually preclude a true primary repair
elsewhere, have been successfully joined together without operative
complications. As the gaps increase in length, however, internal or
external traction sutures may be necessary to stimulate growth before
the ends can be joined together. Which operative approach is used
depends on the child's situation and the initial esophageal gap length. To
date, a true primary repair has been achieved in all.
Once continuity is established, and healed for about three weeks, we
determine if significant gastroesophageal (GE) reflux is present. Usually,
this is done by an X-ray study. If a lot of reflux is present, we will
recommend a fundoplication (stomach wrap). Prevention of reflux from
the stomach will decrease stricture formation and, over a longer period,
inflammation of the esophagus (esophagitis). Most of the infants with
very long gap repairs have significant reflux and require a fundoplication.
Most of the infants with a short gap repair do not need a fundoplication.
Once the GE reflux is controlled, the learning to feed can begin in
earnest. Depending on how old the child is, this may or may not be
difficult. The instinct to feed lasts for only a short period of time and,
once it passes, learning to eat must be learned.
Another issue, is whether or not an anastomotic narrowing (stricture) has
developed. After three-four weeks time, sufficient healing has take place
so that any narrowing can be dilated. If there was not significant GE
reflux and no fundoplication has been done, then the dilations are simply
done in an outpatient clinic. After a fundoplication, however, we use
balloon dilation of the narrowing which is guided by X-ray to assure
exact placement. In this way, the fundoplication is not also dilated. The
balloon dilations are done under a light anesthetic. Although done on an
outpatient basis, they do take more time than the simple dilations which
can be done if no fundoplication is present.
b. Post operative problems
There are several problems which occur among all EA/TEF patients and
some are more frequent after a long gap EA repair. The problems are
important but each can be effectively treated.
Two of the most common problems are significant GE reflux and
persistent strictures (narrowings) at the esophageal repair site. We
believe that actively treating these two problems in the first year after the
EA repair not only improves the initial results, but, also, will help prevent
severe difficulties from developing over the years.
i. GE reflux
We believe that significant GE reflux should be effectively
controlled, not just treated with medications. Reflux produces
inflammation of the esophagus (esophagitis). The long-term
consequences of esophagitis may develop despite control of
stomach acid levels (reference 6). The symptoms of reflux
(heartburn) are lessened by reducing acid production but the
long term consequences may not be. For this reason, we
recommend a fundoplication (upper stomach wrap) to prevent
reflux when a lot is present. An additional benefit of preventing
reflux is that medications which only work by controlling acid
production by blocking H2 receptors (e.g. Zantac) or by
inhibiting the proton pump (e.g. Prilosec) may not be
necessary. The cost of these medications and their side
effects may be avoided.
ii. strictures
Healing, even for an esophageal anastomosis, involves some
degree of fibrosis and a stricture (narrowing) is common after an EA repair. The nature of scars in general is to contract. The
anastomotic line of healing is circular when the two
esophageal ends are sewn together and there is a tendency to
develop a stricture. Strictures are more common when there is
a lot of GE reflux because acid irritates the EA repair site. In
addition, when the anastomosis is created under a lot of
tension as with long gap repairs, the chance for a significant
stricture also increases.
Luckily, the tendency to contract and narrow does not persist
indefinitely. Over the first few weeks-months, however, it does
exist. We believe that repeated dilations relatively early after
an EA repair will improve the likelihood the stricturing will
relent. Consequently, if there is a narrowing present we
recommend dilations every 2-3 weeks until the stricture does
not recur. In this way, the child will be able to eat whatever
they wish and not be concerned about eating solids or foods
that may stick.
When the strictures do not relent despite repeated dilations,
we usually recommend an operation to remove them. The
stricture resection operation consists simply of removing the
area of scar and rejoining the two ends of the esophagus
together. This operation has proven to be very effective and
the hospital stay is usually very short (three-four days).
There are probably two main reasons why the stricture
resection works so well and the excessive scarring does not
return. Both ends of the esophagus will have grown
substantially by this time and, therefore, there will not be as
much tension on the anastomosis. Furthermore, because the
ends have grown, the diameter of the anastomosis will be
much larger (often at the time of the original primary repair, the
lower end of the esophagus is relatively narrow and makes the
anastomosis of a small caliber). Following the stricture
resection, the opening is large and little if any narrowing
results.
iii. oral aversion
Oral aversion or refusal to eat may be a significant problem
despite a good esophageal repair. The instinct to eat lasts for
only a short time after birth. If the period is missed, the children
must later learn to eat. They must make the connection between being hungry and eating. The older the
child at the time of EA repair, the greater the oral aversion is
likely to be. If the child has a cervical esophagostomy (spit
fistula), then sham feeding may lessen the oral aversion while
the child waits for an esophageal substitute. Of course, a spit
fistula makes a true primary repair more difficult, so we do not
recommend them.
For children learning to eat, it is important that the G-tube
feedings are spaced to allow the child to become hungry. The
recognition of hunger and the connection between being
hungry and eating must be made and established. The
feedings usually have to be significantly reduced so the
connection can be made. Once established, the problem
disappears.
d. tracheomalacia
Usually, infants with EA/TEF have a softer-than-normal
trachea (windpipe) which is called tracheomalasia. The
trachea branches into the right and left bronchi, which connect
to the respective lungs. Together this is referred to as the
tracheo-bronchial tree. For unknown reasons, the tracheobronchial
cartilages may be soft allowing the airway to partially
collapse under exertion. This partial collapse also accounts for
the barky cough associated with EA/TEF infants.
When the tracheomalacia is very severe, however, noisy
breathing and even difficulty getting air in can result. With
time, the tracheo-bronchial cartilages will firm up. But, for a
small percentage of patients, very severe breathing problems
may occur. For these children, we may recommend an
aortopexy which rounds up the airway and prevents these
spells.
The aortopexy operation consists of pulling forward the large
vessels, including the aorta, which overlie the tracheobronchial
tree. The airway is held it open which prevents
collapse with exertion. We have found this to be a very
effective operation. With the airway in the normal rounded
configuration, the cartilages will firm up with time and resist
collapse.
e. The final step
With these potential issues resolved, the child should be well
on the way to eating normally. The question then becomes
when should the G-tube be removed? When it is no longer
used.