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Gastroesophageal reflux (GER) often develops in children who have undergone prior repair of esophageal atresia/tracheoesophageal fistula (EA/TEF). Fundoplication is necessary in many of these children. The complete wrap (Nissen) fundoplication is often used in this setting. However, poor results have been noted, with a mean failure rate of 30% reported in four recent studies. A partial wrap fundoplication for GER associated with EA/TEF is theoretically attractive, because the poor esophageal motility and diminished acid clearance (already physiologically present) is exacerbated by a complete wrap fundoplication. The authors reviewed their extensive experience with partial wrap (Thal) fundoplication in EA/TEF to determine if the failure rate was better than that reported for the Nissen fundoplication. In the past 18 years, the authors performed 1,467 fundoplication procedures. During the same period, 143 children underwent repair of EA/TEF. Fifty-nine children underwent fundoplication after a previous EA/TEF repair. Most of the fundoplications (58 of 59, 98%) were Thal procedures. Defining failure strictly as a need for reoperation, the failure rate in our series was 15% (9 of 59 children). Compared with the failure rate in the 1,408 non-EA/TEF patients (61 of 1408, 4.3%), results were significantly worse for the EA/TEF group (P > .001). The failure rate of Thal fundoplication performed for GER in the EA/TEF population is substantially higher than the non-EA/TEF patients. The same factors responsible for the development of reflux in these children (poor acid clearance, altered motility, esophageal shortening) may contribute to the higher failure rate. Although partial wrap fundoplication frequently failed (15%), the results were still substantially better than those reported for Nissen fundoplication in these children (30% failure rate).

Ninety cases of esophageal atresia (EA) with double tracheoesophageal fistula (TEF) from the literature and 4 new cases are examined. The incidence may be more common than is generally recognized with incidences up to 5.3% reported. The pre-, intra-, and postoperative diagnosis may be difficult, and half have been missed initially with almost half of these being first recognized at autopsy. During repair of EA and TEF the proximal esophagus should be mobilized looking for a proximal fistula. Many proximal TEF are missed at the initial operation and discovered in the postoperative period. The symptoms, diagnosis and treatment of the unrecognized proximal TEF are similar to that for isolated TEF. Results should be good since this problem occurs in large babies with fewer and less complex associated conditions.

Twelve patients with gastroesophageal reflux following repair of esophageal atresia are presented. Reflux produced recurrent stricture, failure to thrive, repeated pneumonitis, and in one patient, respiratory arrest and nearly death. Treatment consists of positional therapy or fundoplication operation both of which seem less successful in this combination of lesions than with GER and a normal esophagus. There was one death as a late postoperative complication of fundoplication.

One hundred consecutive patients with esophageal atresia or tracheoesophageal fistula, or both, were treated at The Children's Mercy Hospital during the past 14 years. Each patient was evaluated and a plan for therapy was formulated depending on the type and urgency of concomitant disease. Healthy patients were treated by primary repair. Those with respiratory disease were treated before repair by gastrostomy, upper pouch suction, and antibiotics. In the small premature infant and the patient with major associated anomalies, repair of the esophageal atresia and tracheoesophageal fistula was postponed by gastrostomy, upper pouch suction, and parenteral nutrition while the concomitant disease was treated. Fifteen patients with life-threatening associated anomalies required operation before esophageal repair and 13 of them survived. During the initial hospitalization two deaths related to esophageal atresia occurred, whereas three prerepair and two late (2 1/2 and 3 months) postrepair deaths related to associated anomalies occurred. Thus, 93 of the 100 patients left the hospital alive. The increased survival is due not only to improved care of esophageal atresia and tracheoesophageal fistula, but also to a more aggressive approach to the treatment of the serious associated anomalies.