Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children With Esophageal Atresia-Tracheoesophageal Fistula

Publication Date: November 1, 2016
Last Updated: March 14, 2022

Statements

Gastroesophageal reflux (GER)

1: It is recommended that GER be treated with acid suppression in all EA patients in the neonatal period.

(Expert Opinion, Low)
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2: PPIs should be the first-line therapy for GER/GERD.

(Expert Opinion, Low)
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3. It is recommended that GER be systematically treated for prevention of peptic complications and anastomotic stricture up to the first year of life or longer, depending on persistence of GERD.

(Expert Opinion, Low)
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4a: pH monitoring is useful in evaluating the severity and symptom association of acid reflux in patients with EA.

(Expert Opinion, High)
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4b: pH-impedance monitoring is useful to evaluate and correlate non-acid reflux with symptoms in selected patients (symptomatic on PPI, on continuous feeding, with extra-digestive symptoms, ALTE, GER symptoms with normal pH-probe and endoscopy).

(Expert Opinion, Low)
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5: Endoscopy with biopsies is mandatory for routine monitoring of GERD in patients with EA.

(Expert Opinion, High)
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6: All EA patients (including asymptomatic patients) should undergo monitoring of GER (impedance/ pH-metry and/or endoscopy) at time of discontinuation of anti-acid treatment and during long-term follow-up.

(Expert Opinion, High)
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7: Routine endoscopy in asymptomatic EA patients is recommended. The expert panel recommends 3 endoscopies throughout childhood (1 after stopping PPI therapy, 1 before the age of 10 years, and 1 at transition to adulthood).

(Expert Opinion, Low)
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8: Severe esophageal dysmotility predisposes EA patients to post-fundoplication complications. However, EA patients may benefit from fundoplication in: 8a: Recurrent anastomotic strictures, especially in long-gap EA.

(Expert Opinion, High)
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8b: Poorly controlled GERD despite maximal PPI therapy.

(Expert Opinion, High)
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8c: Long-term dependency on trans-pyloric feeding.

(Expert Opinion, Very low)
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8d: Cyanotic spells.

(Expert Opinion, Very low)
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9: Barium-contrast study, endoscopy with biopsies and pH-metry should at least be performed before fundoplication.

(Expert Opinion, High)
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10: Symptoms of aspiration during swallowing may be identical to GER symptoms in young children.

(Expert Opinion, Low)
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11a. Patients with EA should be evaluated regularly by a multidisciplinary team including pulmonology and otolaryngology, even in the absence of symptoms.

(Expert Opinion, Low)
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11b. Anatomic abnormalities (laryngeal cleft, vocal cord paralysis, missed or recurrent fistulae, anastomotic stricture, congenital stenosis, vascular ring) should be ruled out in EA patients with respiratory symptoms.

(Expert Opinion, High)
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11c. If pH-metry or pH-MII is performed, symptom correlation during reflux testing, rather than total reflux burden is the most important indicator of reflux-associated symptoms.

(Expert Opinion, Very low)
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12: Acid suppression should be used with caution in patients with extra-esophageal manifestations of reflux.

(Expert Opinion, Low)
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13a: The etiology of life-threatening events is multifactorial and merits a multidisciplinary diagnostic evaluation before surgical intervention.

(Expert Opinion, Very low)
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13b: Anatomic issues (strictures, recurrent or missed fistulae, congenital esophageal stenosis, vascular rings, laryngeal clefts) and aspiration need to be excluded in children with ALTE.

(Expert Opinion, Low)
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Overview

Title

Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children With Esophageal Atresia-Tracheoesophageal Fistula

Authoring Organization

Consensus and Physician Experts