What are the guideline sources, guidelines and references for Tracheoesophageal malformations ?
Guideline 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). 1 2 Calculator Apgar score Guidelines 1. Diagnostic investigations https://web.pathway.md/diseases/recupm1q0hR7kJU6b 1/5 6/24/23, 4:06 PM Tracheoesophageal malformations Pathway Evaluation for tracheobronchomalacia: perform flexible bronchoscopy as the gold standard for the evaluation of tracheobronchomalacia. A Show 6 more Evaluation for anatomic anomalies: As per INEARC-WG 2020 guidelines, perform rigid bronchoscopy for the evaluation of a possible laryngeal cleft. A As per ESPGHAN 2016 guidelines, assess for and rule out anatomic abnormalities (laryngeal cleft, vocal cord paralysis, missed or recurrent fistulae, anastomotic stricture, congenital stenosis, vascular ring) in patients with esophageal atresia with respiratory symptoms. A Show 2 more Evaluation for vascular malformations: As per INEARC-WG 2020 guidelines: Obtain an echocardiogram to rule out anatomical shunts in infants with esophageal atresia- tracheoesophageal fistula developing episodes of severe cyanosis. A Obtain chest CT with contrast when a vascular anomaly is suspected. B As per ESPGHAN 2016 guidelines, obtain chest CT or MRA to exclude congenital VMs as they may be the underlying etiology for dysphagia, dyspnea, or cyanosis by causing external compression on the esophagus and/or trachea. B Evaluation for dysphagia: suspect dysphagia in patients with esophageal atresia presenting with food aversion, food impaction, difficulty swallowing, odynophagia, choking, cough, pneumonia, alteration in eating habits, vomiting, and malnutrition. B Show 4 more Evaluation for acid reflux: consider obtaining pH monitoring to assess the severity and symptom association of acid reflux in patients with esophageal atresia. B Show 4 more Evaluation for eosinophilic esophagitis: Exclude eosinophilic esophagitis in patients of all ages with esophageal atresia and dysphagia, reflux symptoms, coughing, choking, or recurrent strictures refractory to PPIs, before offering anti-reflux surgery. B Perform multiple esophageal biopsies, both proximal and distal to the anastomosis, to diagnose eosinophilic esophagitis. B Evaluation for aspiration: evaluate multiple episodes of lobar pneumonia for aspiration or possible underlying anatomical abnormalities. A Show 7 more Evaluation for bronchitis and pneumonia: obtain further evaluation in the presence of a chronic wet cough. A Show 6 more Evaluation for bronchiectasis: do not rule out bronchiectasis in case of a negative CXR. D Show 3 more https://web.pathway.md/diseases/recupm1q0hR7kJU6b 2/5 6/24/23, 4:06 PM Tracheoesophageal malformations Pathway 2. Respiratory support Airway clearance: institute airway clearance techniques in asymptomatic pediatric patients with positive cultures in BAL. B Noninvasive ventilation: consider initiating noninvasive positive pressure ventilation in pediatric patients with severe tracheobronchomalacia. B Tracheostomy: consider performing tracheostomy in patients with tracheobronchomalacia associated with severe cyanotic episodes and/or respiratory failure. Consider using a custom- made long tracheostomy tube and positive pressure support for severe tracheobronchomalacia. B 3. 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 As per ESPGHAN 2016 guidelines, initiate acid suppression therapy for the treatment of gastroesophageal reflux in all patients with esophageal atresia in the neonatal period. Initiate PPIs as first-line therapy for gastroesophageal reflux/GERD. B Show 3 more Antibiotic therapy: set a lower threshold for initiating antibiotics in pediatric patients with esophageal atresia-tracheoesophageal fistula. A Show 2 more Bronchodilators: do not use bronchodilators in patients with esophageal atresia- tracheoesophageal fistula because they may worsen the airway collapse. D Mucolytics: do not use mucolytics routinely to facilitate airway clearance in patients with esophageal atresia-tracheoesophageal fistula. D Corticosteroids: Administer ICSs in patients with chronic airway inflammation or suspected pneumonia. B Administer systemic corticosteroids in patients with suspected pneumonia. B Bethanechol: consider administering bethanechol in patients with severe tracheobronchomalacia. C 4. Nonpharmacologic interventions Nutrition: ensure a multidisciplinary approach to prevent and treat feeding difficulties in patients with esophageal atresia. B https://web.pathway.md/diseases/recupm1q0hR7kJU6b 3/5 6/24/23, 4:06 PM Tracheoesophageal malformations Pathway 5. Therapeutic procedures Endoscopic dilation: consider performing endoscopic dilation as first-line therapy in patients with congenital esophageal stenosis. C 6. Surgical interventions Fundoplication: As per INEARC-WG 2020 guidelines, consider performing fundoplication for chronic respiratory symptoms and poorly controlled gastroesophageal reflux. C As per ESPGHAN 2016 guidelines: Consider performing fundoplication in patients with esophageal atresia and any of the following: recurrent anastomotic strictures, especially in long-gap esophageal atresia B poorly controlled GERD despite maximal PPI therapy B long-term dependency on trans-pyloric feeding C cyanotic spells. C Obtain barium-contrast study, endoscopy with biopsies, and pH-metry before fundoplication. A Aortopexy: perform aortopexy as a safe and effective mean to treat severe tracheobronchomalacia. Prefer posterior tracheopexy over aortopexy for severe tracheobronchomalacia. B 7. Preventative measures Influenza vaccination: offer annual influenza vaccination in pediatric patients with esophageal atresia-tracheoesophageal fistula. A RSV prophylaxis: administer anti-RSV prophylaxis in < 12 months old patients with esophageal atresia-tracheoesophageal fistula. B 8. 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. A Show 2 more As per ESPGHAN 2016 guidelines, evaluate patients with esophageal atresia regularly by a multidisciplinary team, including pulmonology and otolaryngology, even in the absence of symptoms. B https://web.pathway.md/diseases/recupm1q0hR7kJU6b 4/5 6/24/23, 4:06 PM Tracheoesophageal malformations Pathway Show 3 more Evaluation of recurrent tracheoesophageal fistula: Perform combined endoscopy and bronchoscopy as the gold standard for diagnosing recurrent tracheoesophageal fistula. A Obtain barium swallow in prone/semi-prone position as the preferred radiographic method to detect recurrent tracheoesophageal fistulas. Prefer pressure esophagogram to detect a small tracheoesophageal fistula. B Management of postoperative anastomotic strictures: view anastomotic strictures as clinically significant in the presence of significant functional impairment and associated symptoms, in addition to a relative esophageal narrowing at the level of the anastomosis (by contrast and/or endoscopy). Show 3 more Management of postoperative dysphagia: Obtain a contrast study to rule out mechanical complications, perform upper gastrointestinal endoscopy with biopsy, and if inconclusive, obtain high-resolution manometry with impedance in patients with post-fundoplication dysphagia. B Tailor management of post-fundoplication dysphagia to the underlying mechanisms. B Transition from pediatric to adult care: recognize that dysphagia and symptoms of gastroesophageal reflux continue into adulthood in patients with esophageal atresia and are more frequent in esophageal atresia survivors than in the general population. A Show 2 more References 1. Usha Krishnan, Hayat Mousa, Luigi Dall'Oglio et al. ESPGHAN NASPGHAN Guidelines for the Evaluation and Treatment of Gastrointestinal and Nutritional Complications in Children With Esophageal Atresia- Tracheoesophageal Fistula. J Pediatr Gastroenterol Nutr. 2016 Nov;63 5 550 570. Open 2. Anastassios C Koumbourlis, Yvonne Belessis, Mary Cataletto et al. Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula. Pediatr Pulmonol. 2020 Oct;55 10 2713 2729. Open https://web.pathway.md/diseases/recupm1q0hR7kJU6b 5/5