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
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
We believe the benefits
of a true primary repair will only increase with time.
a. The treatment
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
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
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.
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
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.
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 tracheo-bronchial
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 tracheo-bronchial 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.