Esophageal atresia
Esophageal atresia is an inborn malformation found in one of 3000 newborns. Here the esophagus is not a continuous tube going from the mouth to the stomach but is interrupted. Two types can be identified in most of the cases:
- The upper part of the esophagus is ending as a blind tube, but the lower part is connected to the trachea as a fistula. Air passes through the fistula into the intestinal tract and gastric acid juice can reflux into the airways. The distance is often moderate. A small number of cases has fistula from both parts of the esophagus, the management is the same.
- Both parts are blind tubes, there is no fistula. The distance between the ends is often longer. Single cases show a fistula from the proximal segment, the clinical situation is similar to cases without fistula, because the distal part is not “fixed” to the trachea and short.
The suspicion is often given by the impossibility to feed the child and/or to probe the esophagus. An x-ray of the thorax and of the upper abdomen shows a dilated upper esophageal end filled with air. The gastrointestinal tract is even filled with air via the fistula (type 1) or not (type 2). Additional malformations of the heart, the kidneys, the skeleton and the anus are found in 30% and have to be diagnosed or ruled out by clinical evaluation, echocardiography, abdominal ultrasound and x-rays.
Treatment is always surgical and depends on the type. It starts during the first 48 hrs.
- The thorax is opened on the right side. The fistula between the trachea and the lower esophageal segment is cut and closed. The upper and the lower segment are brought together and connected (anastomosis). Sometimes connection is impossible for long distance, bad general condition of the child or technical reasons. Then further steps are as in type 2.
- Through a left cervical incision the upper part of the esophagus is taken to the skin and opened as an esophagostomy for discharge of saliva and prevention of aspiration. Through an abdominal incision the stomach is opened and probed as a feeding gastrostomy. Thoracotomy is normally not necessary during the first operation.
If an anastomosis was possible the connected esophagus is probed. During healing of the anastomosis nutrition can be started through the probe. Disruption or leakage as well as shrinkage and stenosis of the anastomosis are possible complications. Seldom a recurrent fistula may develop. Around the 10th day after operation, an x-ray contrast examination inspects whether the anastomosis is tight before drinking by mouth is started. If drinking is difficult, a stenosis should always be excluded or dilated endoscopically.
If an anastomosis was not possible the child is fed through the gastrostomy. Sham feeding by mouth is important to prevent loss of the swallowing reflex. This should be done during gastrostomy feeding that the child can learn that drinking and swallowing have something to do with being satisfied. The child can gain weight. Around the age of 3 month esophageal reconstruction can be planned. This is possible as a direct anastomosis if the distal segment grew sufficiently or as a gastric interposition. Gastric interposition means that the stomach is mobilized, transferred to the thorax and directly connected to the cervical upper esophageal end. This method is today accepted as the best way of interposition (especially compared to colon) from the functional view.
Today mortality is low and mainly restricted to cases with complex heart failures. Problems may still arrive from stenosis making repeated endoscopic dilatations necessary. Sometimes the mobilization of the lower esophagus disturbs the anti-reflux mechanisms which prevent gastric acid reflux to the esophagus. A pathological gastro-esophageal reflux with vomiting and failure of thrive can make special feeding procedures, medicaments or (seldom) surgery necessary.
If a gastrostomy is still open this probe or button can be removed if oral feeding is completely established and any problems from the anastomosis are excluded.
Children will often have a typical coughing because the tracheal wall is somewhat instable at the point where the fistula ended. This will stabilize over the first year of life in most cases.
