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.
Figure 5
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.
Figure 6,7
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.