a. The true primary
difficulties imposed when a long gap is present, we believe a true
primary repair using the child's own esophagus will be best for the long
term. A true primary repair can be defined simply as joining the two
esophageal ends together and leaving the stomach entirely below the
diaphragm. The stomach must remain in the abdomen where it belongs.
Furthermore, no circular incision is made through the esophageal
muscles. A circular cut through the muscle wall will allow the remaining
tissue to stretch; a circular myotomy. Circular myotomies are not used
because of the potential for complications from the weakened esophageal
wall. The area of myotomy is unsupported by muscle and may balloon up to
a serious degree (reference
With the esophageal
ends joined together and the stomach below the diaphragm, the child has
by far the best chance of eating normally. Later problems are also much
less likely to occur.
The result of a
true primary repair is always the same, the esophageal ends are joined
together and the stomach kept below the diaphragm. For most of these
infants it can be done at one operation. This has proven true, even if
there is a long gap between the esophageal ends (reference 3). The
two esophageal ends can be brought together, even sometimes under a
great deal of tension and the repair will still hold together.
Therefore, even babies whose gaps are rather long can have an initial
true primary repair.
b. Stimulating the
esophagus to grow
It is not always
possible, however, to do a true primary repair initially. If the child
has been born with most of the esophagus missing or the first operation
has failed, or the upper pouch has been brought out the neck (a spit
fistula), the gap will be too great for an immediate (one step) true
primary repair. For these children, the esophagus must be made to
quickly grow so the repair can be accomplished. We have found that the
growth will be rapid and may take only a few days or, at most, 12-14
days. Over this relatively short period of time, the ends of the
esophagus will grow significantly and allow a true primary repair to be
carried out. The rapid growth of the esophagus is the most important
discovery we have made and allows these operations to be carried out (reference 5).
At the first
operation, the two esophageal ends are put on traction towards each
other. Occasional, when the gap is not overly long, the traction sutures
will rapidly stimulate enough esophageal growth relatively rapidly. When
this appears to be the case, the traction sutures are placed internally.
After 2-3 days time, the incision is reopened and the esophageal ends
For the very
longest gap infants, however, more time will be needed. The traction
sutures are placed in the esophageal ends and brought through the skin
to the outside of the chest wall. This allows the traction to be
increased daily and maximizes the growth stimulus. These children are
kept on the ventilator and heavily sedated so they do not tear the
traction sutures loose. Even the very longest gaps rapidly respond to
this growth stimulus. When the ends are virtually together, the infant
is returned to the operating room and the esophagus joined.
A chest x-ray of an
infant who is undergoing an esophageal growth procedure by traction
sutures. The small markers seen on the x-ray indicate that the
two ends of the esophagus are close together. There has been good
growth of the two ends and the baby is now ready for a true primary
An esophagram taken
after a true primary repair of an ultra long gap EA. The
esophageal repair is intact and shows a virtually normal appearing
esophagus. This patient was born with an ultra long gap pure EA. A
period of external traction was needed until the ends grew enough
so that they could be brought together. After several dilations,
the esophagus now looks relatively normal and occupies its normal
position within the chest. At the bottom of the x-ray, the top of
the stomach can be seen, again in its normal position within the
abdomen and below the diaphragm.
substitutions (interposition grafts)
Usually at other
hospitals, if the gap is very long a true primary repair is not
recommended or attempted. If the esophageal ends can not be brought
together then another tubular organ must be used to bridge the gap and
provide continuity. The most commonly used esophageal substitutions,
include colon interpositions, the creation of a stomach tube or a
pull-up of the stomach (gastric transposition).
grafts, with the exception of the jejunum, cause increasing problems and
severe consequences with time. Pulling part of the stomach up into the
chest so that the two esophageal ends can be joined together is not a
true primary repair. Any partial division or elongation or an upward
pull-up of the stomach will lead to significant long term consequences
and would not meet the definition of a true primary repair.
The most commonly
used esophageal substitutions include colon interpositions, the creation
of a stomach tube or a pull-up of the stomach (gastric transposition).
The consequences of these will be discussed under early and long-term
results, but suffice it to say, the likelihood of a difficult early
course is high.