Source:Nasr A, Ein SH, Gerstle JT. Infants with repaired esophageal atresia and distal tracheoesophageal fistula with severe respiratory distress: is it tracheomalacia, reflux, or both?
J Pediatr Surg

Infants with repaired esophageal atresia and distal tracheoesophageal fistula are at risk of severe respiratory distress related to tracheomalacia, gastroesophogeal reflux (GER), or both. Investigators from the Hospital for Sick Children in Toronto performed a retrospective study of 288 patients who had surgery to repair proximal esophageal atresia or distal tracheoesophageal atresia between 1977 and 2003. Following surgery, 29 (10%) of these patients developed severe respiratory compromise characterized as pneumonia, cyanotic episodes, or true respiratory arrest. These events occurred in the absence of cardiac anomalies or evidence of recurrent fistula. Diagnostic studies included upper gastro-intestinal swallow and direct endoscopy, which confirmed the presence of both significant tracheomalacia and GER in 22 of these 29 patients. In the remaining 7 patients, 1 of these 2 conditions was confirmed.

The decision as to whether a patient should undergo surgery for tracheomalacia or GE reflux was left to the patient’s surgeon’s judgment. Thirteen of the 22 (59%) patients underwent an initial procedure for tracheomalacia. Six of these 13 patients (46%) had ongoing respiratory symptoms and required an anti-reflux procedure within 1 to 3 months of the first operation. All subsequently improved following the second operation.

Nine of the 22 (41%) infants had an anti-reflux procedure as their initial operation with symptomatic relief in 6. Three (33%) had ongoing symptoms and required a second operation to correct the tracheomalacia, after which all 3 improved. The authors conclude that regardless of whether tracheomalacia or GER is considered to be the major cause of respiratory distress following repair of esophageal atresia and distal tracheoesophageal fistula, many infants will ultimately require a second operation.

Dr. Cavett has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of a commercial product/device. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

That the initial surgical procedure was left to the “individual surgeon’s discretion and judgment” might be considered a weakness of this study. Unfortunately, the study was unable to answer the question of how to determine which operation to perform first in these infants. What it does tell us is that some patients will need both fundoplication and tracheomalacia repair, although this group may not be identified until after initial corrective surgery has proved only partially successful.

The etiology of respiratory distress in these infants remains poorly understood. It may be related to obstructed and weakened tracheal cartilage at the old fistula site or from chronic compression from the previously dilated proximal esophageal pouch – the weakened tracheal cartilage then collapses during respiration. On the other hand, reflex apnea from GE reflux into the pharynx and trachea has also been suggested as a cause.1 Nevertheless, some degree of both tracheal collapse and GE reflux are common in children who have...

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