Esophageal Atresia & Tracheoeophageal Fistula

Our purpose in creating this EA/TEF website is to provide information to the families of these babies as well as to health care providers who would like to review their treatment. Our extensive experience with the treatment of these infants has led to important information about the available options. We believe the advantages and disadvantages of the various treatments can be understood and will be useful in making decisions. We have also found that the parents and primary care providers are the best and most effective advocates for these children.

The acknowledged advantages of using the child's own esophagus and our success with true primary esophageal repairs for even the longest gaps, are the reason for presenting this option. We have observed that the primary repair option is not always presented to parents and for the more difficult cases, only less satisfactory esophageal substitutes may be offered.

Our overall goal is quite simple. The child should be able to eat normally as they wish. There should be no need for a stomach (gastric or g-) tube for supplemental feedings or for medications.

What is EATF?

Visitors at this website will likely have some knowledge of EA/TEF, nevertheless an introduction may prove useful. There is a lot of background information available (reference 1).

Infants born with esophageal atresia (EA) have a portion missing (a gap) in their esophagus, the tube that goes from the back of the mouth to the stomach. With part of the esophagus missing, the baby cannot swallow food or even its own saliva. These defects are discovered either before birth by ultrasound examination or very shortly after birth in the delivery room. The reason these birth defects occur is unknown but, luckily, it rarely recurs in the immediate family.

EA takes many forms and there is a lot of variation in this group of defects. The spectrum of the defects including the five main types, are shown in Figure 1. The approximate percentages of each type are shown. Many additional variations, however, can occur. Because of the many children who have come here from outside our area, we treat a much higher percentage of long gap EA (type a). Overall, our series has had 20% pure EA (type a) and more recently it has been even higher.

(Figure 1)

Most of these babies (about 85%) will have an upper esophageal pouch that ends blindly and a lower esophageal segment that connects into the trachea (Figure 1c). For 8-10%, however, there will not be a connection between esophagus and trachea (no TEF). This is considered to be pure esophageal atresia (EA) with two blind esophageal ends (Figure 1a). For these babies, the distance between the two esophageal ends is usually longer (a long gap EA). Similarly, when the TEF is only between the upper pouch and the trachea, the lower end of the esophagus tends to be short leaving a long gap between the ends (Figure 1b).

For all the babies with EA, with or without a TEF, the missing segment between the two ends of the esophagus must be connected for normal eating. If the EA repair cannot be carried out within a matter of days, a gastric tube is usually placed directly into the stomach. This allows the child to be fed until the ends of the esophagus are put together and the baby is able to take food by mouth.

Issues

The baby's condition before these large operations is important. But in addition to the baby's general condition, there are at least three important issues which will affect the treatment plan. These are whether or not the baby: (1) is very premature, (2) or has other serious birth defects in the heart, kidney or elsewhere or (3) has a type of esophageal atresia that will be difficult to repair. These are potentially complicating issues that may significantly affect the proposed operation in terms of either timing or the final result.

a. Other birth defects

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 anomalies.

There 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.

b. Prematurity

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 3> 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.

(images)

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 together.

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.

Treatment

The basic operation

The basic operation consists of joining (sewing) together the two ends of the esophagus (an anastomosis) using fine sutures. If a TEF is present, this must be repaired first and the hole in the trachea closed. For about 80% of the children born with EA and TEF the operation is straight forward and completed satisfactorily. The basic operation for EA/TEF is diagrammed in figure 5. With minor variations this is the usual technique used.

The long gap situation

For infants with long gap EA, however, the operation may be much more difficult and the results not always good. The two ends of the esophagus may be thought to be too far apart, or the tissues too thin, raising concern that the repair would be under too much tension and not hold up. Whether the gap is merely long or too long depends on these factors and the viewpoint of the surgeon. For gaps 2-3 cm long or 2-4 vertebral bodies apart, a primary repair will usually be carried out. Surgeons are realizing that some tension will be tolerated by a well constructed anastomosis. As the distance increases, however, so do the likelihood of complications with an attempted primary repair. To avoid this possibility, many surgeons will use an esophageal substitute such as stomach or colon.

a. The true primary repair

Despite the 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 1).

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 sewn together.

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. 

c. Esophageal 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).

The interposition 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.

Long Term

a.  Overall goals

Our late results, so far, have been excellent. All children, even those who began with the longest gaps or had failed previous operations, have had a successful true primary repair. Those who are far enough out from the repair (usually a year), to allow complete resolution of anastomotic strictures and to have overcome oral aversion, are eating normally. One child came to us with a tracheostomy in place and although he now has a very satisfactory esophagus, he will not learn to eat until the tracheostomy can be removed. We believe this will be accomplished this summer. Oral aversion seems to be the most lingering barrier to eating a normal diet. Although oral aversion may require a great deal of effort by the parents, we believe that in all cases it will be solved.

As the testimony to how well they eat, we published our long term follow-up studies and found the weight of our patients was within the normal range. In fact, their weights slightly exceeded their range of heights (reference 5). The size distributions were almost precisely normal indicating good nutrition and growth and development.

All children were eating whatever they wished and, at the time the study was completed, no child two years after repair, still had a gastrostomy tube (reference 5). With the referral here of increasingly complicated cases of EA in children who arrive at a year of age or older, not all g-tubes have been out by the age of two.

b. Esophageal Function

The normal esophagus empties in to the stomach by a combination of muscular contraction (peristalsis) and gravity. A rhythmic wave of peristalsis which is propagated within the muscle itself, begins in the throat and passes down to the stomach. It has been believed that the vagus nerve provides the stimulus for the contraction wave. But, both older experimental studies as well as our clinical observations indicate that the contraction stimulus passes within the muscle itself. Unfortunately, the contraction impulse does not pass across a repair site. Consequently, wherever a primary repair has been done or a segment of esophagus removed and the ends joined together, the contraction wave will not pass smoothly to the lower portion of the esophagus.

The esophageal muscle below the repair site does have an intrinsic firing mechanism, however, and contractions will occur. The contractions they are not smoothly rhythmic and progressive. As a result, any child who has had an EA repair, are considered to have disordered function of the lower esophagus. The practical consequences, however, do not appear to be great. The contractions will empty the esophagus satisfactorily into the stomach and the children are able to eat whatever they wish. A normal diet can be enjoyed.

On the other hand, interpositions of colon and stomach have no peristalsis and can empty only by gravity. Therefore, these children may have significant eating difficulties and may not be able to eat whatever they wish or enjoy a normal diet.

c. GE reflux

In our opinion, follow-up evaluations should be done for GE reflux. Reflux is common, particularly after very long gap repairs. In our patients, fundoplications have been done in about 30% of the children and more often in the long gap patients. We recommend a fundoplication for significant GE reflux, because of the uncertainties of treatment by medications alone (reference 6). Whether or not fundoplication will eliminate the need for long term antacid medication remains to be seen. We still recommend, therefore, esophagoscopy and biopsies every 1-3 years to make sure significant inflammation of the esophagus has not developed. Because our final goal, which has been realized in a large majority of our patients, is to be medication free, this follow-up is necessary. More time will be needed to accurately answer how often the potential problems of GE reflux occur, particularly in the children who have had very long gap repairs.

d. The very long term

For the very long term, we do not foresee any problems with the esophagus after a true primary repair. There are patients who had a relatively straight forward repair of EA/TEF forty years ago. The function of their esophagus appears to be very satisfactory. This should be true even for the longest gap repairs, because of the great growth potential inherent in the esophagus of all babies. It should not matter that originally the esophageal pouches were relatively short. The growth potential should more than compensate for this beginning.

In summary, we have shown that true primary repair is possible, apparently in all EA patients. This esophageal repair will reliably allow the children to eat normally and not be dependent on a g-tube. We believe the benefits of this approach will only increase with time. There seems to be no evidence for late problems arising with the esophagus, however, the potential for difficulties from untreated GE reflux remain. Clearly, however, after a few years, even the very long gap repairs cannot be distinguished from normal by what they eat.

Contact Us

Contacts:

Daniel A. Saltzman, M.D., Ph.D.
Professor of Surgery
Chief, Division of Pediatric Surgery

Robert D. Acton, M.D.
Associate Professor of Surgery
Division of Pediatric Surgery

Donavon J. Hess, M.D., Ph.D., M.B.A.
Assistant Professor of Surgery
Division of Pediatric Surgery

Bradley J. Segura, M.D., Ph.D.
Assistant Professor of Surgery
Division of Pediatric Surgery

Mailing Address:

Pediatric Surgery
2450 Riverside Ave S
East Bldg, MB505
Minneapolis, MN 55454

Phone: (612) 626-4214
Fax: (612) 624-6969
Email: hessx006@umn.edu

Other Resources:

References

  1. Boyle, E.M., Irwin, E.D., Foker, J.E.: Primary repair of ultra long gap esophageal atresia: results without a lengthening procedure. Ann. Thorac. Surg. 57:576-579, 1994.
  2. Coran, Arnold G.: Ultra-Long-Gap Esophageal Atresia: How Long Is Long?. Ann Thorac Surg. 57:528-529, 1994.
  3. Foker, J.E. and Boyle, E.M.: Esophageal atresia and tracheoesophageal fistula. In: Pearson, F.G., Deslauriers, J., Ginsberg, R.J., Hiebert, C.A., McKneally, M.F. and Urschel, Jr., H.C. (eds), Esophageal Surgery. New York, NY, Churchill Linvingstone Inc., 1995, pp. 151-183.
  4. Spitz, Lewis: Esophageal Atresia: Past, Present, and Future. Journal of Pediatric Surgery. 1:2:19-25, 1996.
  5. Foker, J.E., Linden, B.C., Boyle, E.M., Marquardt, C.: Development of a true primary repair for the full spectrum of esophageal atresia. Annals of Surgery. 226:4:533-543, 1997.
  6. DeMeester, T.R., Peters, J.H., Bremner, C.G., Chandrasoma, P.: Biology of gastroesophageal reflux disease: Pathophysiology relating to medical and surgical treatment. Annu. Rev. Med. 1999. 50:469-506, 1999.
  7. Foker JE, Kendall TC, Catton K, Khan KM. A flexible approach to achieve a true primary repair for all infants with esophageal atresia. Semin Pediatr Surg. 2005 Feb;14(1):8-15.
  8. Khan KM, Foker JE. Use of high-resolution endoscopic ultrasonography to examine the effect of tension on the esophagus during primary repair of long-gap esophageal atresia. Pediatr Radiol. 2007 Jan;37(1):41-5. Epub 2006 Oct 17.
  9. Khan KM, Sabati AA, Kendall T, Foker JE. The effect of traction on esophageal structure in children with long-gap esophageal atresia. Dig Dis Sci. 2006 Nov;51(11):1917-21. Epub 2006 Sep 15.