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Tracheoesophageal malformations

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of tracheoesophageal malformations are prepared by our editorial team based on guidelines from the International Network of Esophageal Atresia, Respiratory Complications Working Group (INEARC-WG 2020) and the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN/NASPGHAN 2016). ...
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Diagnostic investigations

Evaluation for tracheobronchomalacia: as per INEARC-WG 2020 guidelines, perform flexible bronchoscopy as the gold standard for the evaluation of tracheobronchomalacia.
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  • Evaluation for anatomic anomalies

  • Evaluation for VMs

  • Evaluation for dysphagia

  • Evaluation for acid reflux

  • Evaluation for eosinophilic esophagitis

  • Evaluation for aspiration

  • Evaluation for bronchitis and pneumonia

  • Evaluation for bronchiectasis

Respiratory support

Airway clearance: as per INEARC-WG 2020 guidelines, institute airway clearance techniques in asymptomatic pediatric patients with positive cultures in bronchoalveolar lavage.
B

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  • Noninvasive ventilation

  • Tracheostomy

Medical management

Acid suppression therapy: as per INEARC-WG 2020 guidelines, consider initiating a trial of anti-gastroesophageal reflux therapy in pediatric patients with persistent respiratory symptoms.
C

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  • Antibiotic therapy

  • Bronchodilators

  • Mucolytics

  • Corticosteroids

  • Bethanechol

Nonpharmacologic interventions

Nutrition: as per ESPGHAN/NASPGHAN 2016 guidelines, ensure a multidisciplinary approach to prevent and treat feeding difficulties in patients with esophageal atresia.
B

Therapeutic procedures

Endoscopic dilation: as per ESPGHAN/NASPGHAN 2016 guidelines, consider performing endoscopic dilation as first-line therapy in patients with congenital esophageal stenosis.
C

Surgical interventions

Fundoplication: as per INEARC-WG 2020 guidelines, consider performing fundoplication for chronic respiratory symptoms and poorly controlled gastroesophageal reflux.
C

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  • Aortopexy

Preventative measures

Influenza vaccination: as per INEARC-WG 2020 guidelines, offer annual influenza vaccination in pediatric patients with esophageal atresia-tracheoesophageal fistula.
A

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  • RSV prophylaxis

Follow-up and surveillance

Follow-up: as per INEARC-WG 2020 guidelines, follow up pediatric patients with esophageal atresia-tracheoesophageal fistula by a specialized interdisciplinary team.
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  • Evaluation of recurrent tracheoesophageal fistula

  • Management of postoperative anastomotic strictures

  • Management of postoperative dysphagia

  • Transition to adult care