Abstract

Patients with esophageal atresia (EA) suffer from abnormal and permanent esophageal intrinsic and extrinsic innervation that affects severely esophageal motility. The repair of EA also results in esophageal shortening that affects distal esophageal sphincter mechanism. Consequently, gastroesophageal reflux (GER) is common in these patients, overall approximately half of them suffer from symptomatic reflux. GER in EA patients often resists medical therapy and anti-reflux surgery in the form of fundoplication is required. In patients with pure and long gap EA, the barrier mechanisms against reflux are even more damaged, therefore, most of these patients undergo fundoplication during first year of life. Other indications for anti-reflux surgery include recalcitrant anastomotic stenoses and apparent life-threatening episodes. In short term, fundoplication alleviates symptoms in most patients but recurrences are common occurring in at least one third of the patients. Patients with fundoplication wrap failure often require redo surgery, which may be complicated and associated with significant morbidity. A safe option in a subset of patients with failed anti-reflux surgery appears to be long-term medical treatment with proton pump inhibitors.

Highlights

  • The esophagus is not normal following repair of an esophageal atresia (EA)

  • The abnormal esophageal anatomy after repair of EA plays a significant role in the etiology of Gastroesophageal Reflux Disease (GERD)

  • Some patients may be treated by fundoplication alone if the etiology of acute life-threatening events (ALTE) is considered to be mainly GERD [21]

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Summary

INTRODUCTION

The esophagus is not normal following repair of an esophageal atresia (EA). The tension and abnormal perfusion at the anastomotic site commonly cause stricture formation that requires anastomotic dilatations. Pathological gastroesophageal reflux (GER) that is caused by shortening of the esophagus and abnormal clearance of esophageal contents due to abnormal motility affects up to two thirds of patients with EA [1, 4]. Some EA patients experience acute life-threatening events (ALTE) that may be associated with proximal extension of GER and with tracheomalacia that commonly accompanies EA. Most pediatric surgeons agree that patients with pure or long-gap EA very often require fundoplication to overcome severe GER and anastomotic strictures associated with the significant shortening of the esophagus. The overall rate of fundoplication in patients with EA ranges between 10 and 45% [1, 4, 5]

INDICATIONS FOR FUNDOPLICATION IN EA
Anastomotic Stricture
Severe Respiratory Disease
OUTCOMES OF FUNDOPLICATION IN EA
WHAT ARE THE OPTIONS WHEN FUNDOPLICATION HAS FAILED?
Findings
CONCLUSION
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