Esophageal Atresia and Tracheoesophageal
Fistula: Goals

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 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.