a. Other birth
The presence of
other birth defects, such as in the heart, chest, abdomen, head or limbs
may be very important to the baby. The tendency of other birth defects
to occur with EA/TEF is well known and termed the VATER or VACTERL associations.
These letters stand for such defects as: missing or deformed Vertebrae,
absence of an Anus, abnormal kidneys (Renal), malformed forearm bones
(Radius or Limb), or blockages in other parts of the intestinal tract
as well as heart (Cardiac) and brain lesions. The TE is from tracheoesophageal
are so many possibilities for birth defects other than EA/TEF, that
a discussion of them will not be presented here. The presence and consequences
of these defects can better be discussed by the baby's doctors.
Some EA/TEF infants
are born very prematurely. If the baby is very small and underdeveloped
or has very immature lungs, the surgical repair of the EA/TEF may be
delayed until the situation is much improved. Treatment by the neonatologists
has greatly improved, nevertheless, prematurity influences the treatment
plan. Among the possibilities, are waiting until the child is larger
and vigorous enough for the operation. In general, a weight of 1,700
grams or about 3¾ pounds would be preferable.
If the wait for
repair will be prolonged, or the lungs are being injured by acid coming
up from the stomach through the TE fistula, then an initial operation
may be recommended. A gastrostomy and/or tying off of the fistula (TEF)
may improve the situation until the primary esophageal repair. Again,
prematurity and its consequences for the EA repair are best left for
the health care providers to discuss.
c. The gap length
- the barrier to true primary esophageal repair.
Once the above
issues have been resolved, the child would be ready for esophageal repair.
Currently, by far the most important issue affecting the repair itself
is the distance or gap between the two esophageal segments. A primary
repair consists of sewing the two esophageal segments together to form
a tube. This is the simplest and best method of repair, but if the ends
are too far apart it may not be possible.
As shown in Figure
1, the gap between the esophageal ends is longest in Types a and b.
This is the most difficult end of the EA spectrum to repair. Usually,
the gaps in Type c and d are short enough to allow a straightforward
primary repair. But even in these groups, some infants may be considered
by the surgeon to have too long a gap. As a result, even some babies
with Type c EA/TEF, are also repaired with a substitution graft.
The distance is
usually measured by an x-ray before the surgical repair. The gap length
is expressed in terms of centimeters (cms) or vertebral bodies (Figures
2,3,4). Vertebral bodies grow with the child, so if a gap is about five
vertebral bodies long, the distance in cms will depend on the size and
age of the child. In a small baby,a vertebral body would be less than
a cm, and a four vertebral body gap might be about 3 cm long. At about
6 months of age, however, the distance would be about one cm per vertebral
body. As the child grows larger a vertebral body would be greater than
one cm. The distance given in vertebral bodies gives an idea of the
relative amount of esophagus missing. For example, in Figure 3, virtually
all of the intrathoracic esophagus is missing: a gap of about eight
vertebral bodies but, the gap is only about 5 cm long. Figure 4, a gapogram
of a larger child, shows a gap of 6.2 cm but a space of only about six
vertebral bodies. The absolute distance (in cm) will also indicate the
difficulty with a primary repair.
An x-ray study which
allows the gap length between the two esophageal segments to
be determined. The upper esophageal pouch is outlined by the
black arrows. There is no TEF and this is an example of pure
EA (type a). A contrast agent (usually barium) has been put
into the stomach through the g-tube just prior to making this
x-ray. The lower end of the esophagus is seen ending just about
the diaphragm which separates the lungs from the abdominal contents
including stomach and intestines. The segments seen between
the two ends of the esophagus are the vertebral bodies which
make up the spinal column. Each vertebral bodies has a pair
of ribs arising from it, one to the right and one to the left
and they can also be seen. The gap length can either be determined
by simple measurement and expressed in centimeters (cms) or
the number of vertebral bodies between the two esophageal segments
can be counted. In this case the gap length is about five vertebral
bodies. If the child weighs about 11 pounds, the distance would
be about 5 cm as each vertebral body would be about 1 cm thick.
If the child weighed about 15 pounds, however, then the gap
length for five vertebral bodies might be about 6 cm. Whichever
method of measurement is used, this would be considered an ultra
which shows a seven vertebral body gap length. This was taken
from another child with pure EA and, because the upper and
lower segments are shorter, the relative gap is somewhat longer.
In this case, there is almost no esophagus present in the
chest. Truly, an example of a very long gap.
with even a shorter esophageal segments. In this case, the
intrathoracic (chest portion) esophagus is entirely missing.
A primary repair
which joins the two ends of the esophagus together and leaves the stomach
below the diaphragm in the abdomen where it belongs, is by far the best
solution. A long gap provides the main obstacle to a satisfactory primary
repair. Virtually everyone agrees that the child's own esophagus is
best and bringing the two ends together is preferable to any substitution
using bowel or stomach. Nevertheless, virtually all pediatric surgeons
will state that some gaps just are too long and the ends cannot be brought
The length of the
gap that is too long for a primary repair varies somewhat from surgeon
to surgeon. As one of the best known writers in this area, Lewis Spitz,
has stated, the difficulty posed by the gap is often in the eye of the
beholder (reference 2). If the
child is small and the tissues seem more fragile, then relatively short
gaps may be judged too long. Certainly, a gap longer than 3 cm has proven
to be a significant obstacle to a primary repair. For
perhaps 10-20% of infants with some form of EA/TEF, the gap between
the two esophageal ends is considered too long to allow them to be brought
together. These babies are considered to have a long or very long gap
EA and will likely not have a true primary repair.
A note of caution
is necessary regarding the gap definitions. We have defined a very long
or even ultra long gap as being over 3.5 cm (reference
3). Although there are no other reports of true primary repairs
of gaps between 3.5 and 6 cm, one writer has stated that a primary repair
is almost always done for them (reference
4). What is meant by a primary repair is that article is that one
or more circular myotomies may be done and the stomach may be pulled
up part way into the chest. That is why we are so careful about the
definition of a true primary repair.
Commonly, a baby
with long gap EA will be allowed to grow for several months with the
hope that the two ends would also grow together. Although this may occur,
typically it does not and the gap grows with the child. Although the
relative gap is not any longer because the size of the child has increased,
the absolute gap is greater. In some instances, however, the gap does
shrink as the two ends grow longer. But setting these fortunate few
aside, the problem of the babies with long gap EA remains.