Sep 30, 2008

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Esophageal atresia, with or without tracheoesophageal fistula

Esophageal atresia, with or without tracheoesophageal fistula, occurs in 1 out of 4500 live births in the United States. The most common anatomic variant of esophageal atresia is the presence of a tracheal fistula to the distant remnant of the esophagus. This type of tracheoesophageal fistula occurs in 85% of all infants born with esophageal atresia. Other organ systems can also be affected in up to 20% of infants born with esophageal atresia. The spectrum of associated anomalies is frequently referred to by the acronym VACTERL, which includes vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb abnormalities.

Diagnosis of esophageal atresia, with or without tracheoesophageal fistula, is usually made after birth with symptoms of respiratory distress, excess oral secretions, choking on attempted feeds and an inability to pass an oro- or naso-gastric tube beyond 12 cm. The ultimate goal of the surgical correction of esophageal atresia with distal tracheoesophageal fistula is to restore esophageal continuity and ligate the tracheal fistula. The traditional operation occurs via a right thoracotomy (large rib- and muscle-splitting incision of the posterior and lateral chest wall) with subsequent division/ligation of the tracheoesophageal fistula and anastomosis of the proximal and distal esophageal segments. The thoracoscopic repair is different from the traditional operation only in that the chest is accessed through 3 incisions, 5 mm each, in the right chest.[5, 6] Early complications include esophageal anastomotic leak, recurrent tracheoesophageal fistula, gastroesophageal reflux, tracheomalacia and esophageal anastomotic stricture formation. Late complications include progressive gastroesophageal reflux, recurrent esophageal stricture formation and recurrent tracheoesophageal fistula.

Holcomb et al.[7] reviewed the combined results of 104 neonates undergoing primary thoracoscopic repair of esophageal atresia with tracheoesophageal fistula at 6 different institutions worldwide. The mean weight at operation was 2.6 kg (+/-0.5) with a mean operative time of 129.9 minutes (+/-55.5). Outcomes were compared to historical controls in infants undergoing traditional thoracotomy repair. The esophageal anastomotic leak rate was 7.6% (compared to 10%-21% historical) with a stricture rate of 3.8% (compared to 18% historical). Recurrent tracheoesophageal fistulization rate is widely reported to be between 2.2% to 12% in various historical reports. In this series, the recurrent fistulization rate was 1.9%. Therefore, thoracoscopic repair of esophageal atresia with distal tracheoesophageal fistula is feasible and can be done with equivalent surgical outcomes to historical controls of the traditional thoracotomy repair. However, the retrospective nature of this large series does not allow for direct comparison of results between thoracoscopic and traditional thoracotomy approaches. Without these direct data, comparative outcomes for length of stay and postoperative pain are not yet available.

, occurs in 1 out of 4500 live births in the United States. The most common anatomic variant of esophageal atresia is the presence of a tracheal fistula to the distant remnant of the esophagus. This type of tracheoesophageal fistula occurs in 85% of all infants born with esophageal atresia. Other organ systems can also be affected in up to 20% of infants born with esophageal atresia. The spectrum of associated anomalies is frequently referred to by the acronym VACTERL, which includes vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb abnormalities.

Diagnosis of esophageal atresia, with or without tracheoesophageal fistula, is usually made after birth with symptoms of respiratory distress, excess oral secretions, choking on attempted feeds and an inability to pass an oro- or naso-gastric tube beyond 12 cm. The ultimate goal of the surgical correction of esophageal atresia with distal tracheoesophageal fistula is to restore esophageal continuity and ligate the tracheal fistula. The traditional operation occurs via a right thoracotomy (large rib- and muscle-splitting incision of the posterior and lateral chest wall) with subsequent division/ligation of the tracheoesophageal fistula and anastomosis of the proximal and distal esophageal segments. The thoracoscopic repair is different from the traditional operation only in that the chest is accessed through 3 incisions, 5 mm each, in the right chest.[5, 6] Early complications include esophageal anastomotic leak, recurrent tracheoesophageal fistula, gastroesophageal reflux, tracheomalacia and esophageal anastomotic stricture formation. Late complications include progressive gastroesophageal reflux, recurrent esophageal stricture formation and recurrent tracheoesophageal fistula.

Holcomb et al.[7] reviewed the combined results of 104 neonates undergoing primary thoracoscopic repair of esophageal atresia with tracheoesophageal fistula at 6 different institutions worldwide. The mean weight at operation was 2.6 kg (+/-0.5) with a mean operative time of 129.9 minutes (+/-55.5). Outcomes were compared to historical controls in infants undergoing traditional thoracotomy repair. The esophageal anastomotic leak rate was 7.6% (compared to 10%-21% historical) with a stricture rate of 3.8% (compared to 18% historical). Recurrent tracheoesophageal fistulization rate is widely reported to be between 2.2% to 12% in various historical reports. In this series, the recurrent fistulization rate was 1.9%. Therefore, thoracoscopic repair of esophageal atresia with distal tracheoesophageal fistula is feasible and can be done with equivalent surgical outcomes to historical controls of the traditional thoracotomy repair. However, the retrospective nature of this large series does not allow for direct comparison of results between thoracoscopic and traditional thoracotomy approaches. Without these direct data, comparative outcomes for length of stay and postoperative pain are not yet available.

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