Guidelines for Esophageal Atresia-Tracheoesophageal Fistula

From a NASPGHAN-ESPGHAN working group of surgeons and pediatric gastroenterologists. a recent guideline (U Krishan et al JPGN 2016; 63: 550-70) regarding esophageal atresia (EA) and tracheoesophageal fistula (TEF) has made 36 specific statements and graded the evidence for these recommendations.

On GERD:

  • Low level evidence: The authors recommended starting PPIs in the neonatal period and continuing them “up to the first year of life or longer, depending on persistence of GERD.”
  • High level evidence: pH monitoring is useful in evaluating the severity of acid reflux in patients with EA.  Low level evidence: pH-impedance monitoring is useful to evaluate and correlate non-acid reflux in selected patients.
  • High level evidence: “Endoscopy with biopsies is mandatory for routine monitoring of GERD in patients with EA.” The authors recommend evaluation (pH, impedance, and/or endoscopy) at time of stopping anti-acid treatment and during long-term follow-up. Specifically,  with low level of evidence, the authors advocate for one endoscopy after stopping PPI therapy, one before age 10 years, and one at transition to adulthood.”
  • High level evidence: Fundoplication has accepted role, despite dysmotility, in patients with recurrent anastomotic strictures and poorly controlled GERD despite maximal PPI therapy. Full evaluation prior to fundoplication is recommended.

Other Statements:

  • Low level evidence: the authors recommend regular multidisciplinary evaluation, the authors note that symptoms of aspiration during swallowing may be identical to GERD symptoms, and evaluation of dysphagia with EGD/UGI.
  • High level evidence: ENT evaluation may disclose other anatomic abnormalities
  • Low level evidence: The authors recommend that anastomotic strictures be excluded in symptomatic children and that there is no “evidence that routine screening and dilatation” is necessary in asymptomatic patients.
  • Low level evidence: Management of EA patients with eosinophilic esophagitis (EoE) should follow the treatment of EoE in non-EA patients.
  • High level evidence: Incidence of Barrett’s esophagus is increased in adults with EA.
  • No level evidence: Potential treatments for recurrent strictures: steroids, mitomycin C, stents, and endoscopic knife.

While some these recommendations noted above are based on low level evidence, it is worthwhile for experts to provide their opinions.  At the same time, there are some completely useless statements included in the guidelines, like those that suggest tailoring the treatment to the underlying problem.  For example: “Statement 19: We recommend tailoring management of post-fundoplication dysphagia to the underlying mechanism(s).”  Another useless statement: “Statement 21: No data are available on the most efficacious methods of avoiding feeding disorders in children with EA. However, the committee recommends a multidisciplinary approach to prevent and treat feeding difficulties.” (very low level evidence).  In my view, shortening the number of recommendations, mainly by eliminating the useless ones, would have been helpful.

My take: Despite my view that about 20% of the recommendations are useless (Statements 17, 19, 21, 25, 26A & 26B, 31, and 34), the overall guideline is helpful and it is still worthwhile for experts to provide their recommendations.

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