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

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

2: PPIs should be the first-line therapy for GER/GERD.

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.

4a: pH monitoring is useful in evaluating the severity and symptom association of acid reflux in patients with EA.

5: Endoscopy with biopsies is mandatory for routine monitoring of GERD in patients with EA.

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.

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).

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.

9: Barium-contrast study, endoscopy with biopsies and pH-metry should at least be performed before fundoplication.

10: Symptoms of aspiration during swallowing may be identical to GER symptoms in young children.

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

12: Acid suppression should be used with caution in patients with extra-esophageal manifestations of reflux.

13a: The etiology of life-threatening events is multifactorial and merits a multidisciplinary diagnostic evaluation before surgical intervention.

14: Dysphagia should be suspected in patients with EA who present with food aversion, food impaction, difficulty in swallowing, odynophagia, choking, cough, pneumonia, alteration in eating habits, vomiting, and malnutrition.

15: We recommend that all EA patients with dysphagia undergo evaluation with upper GI contrast study and esophagoscopy with biopsies.

16: Esophageal manometry is useful to characterize esophageal motility patterns in EA patients with dysphagia. However, the impact on clinical outcome has yet to be determined.

17: We recommend tailoring management of dysphagia to the underlying mechanisms.

18: In EA patients with post-fundoplication dysphagia, we recommend a contrast study to rule out mechanical complications, EGD with biopsy and, if inconclusive, high-resolution manometry ± impedance.

19: We recommend tailoring management of post-fundoplication dysphagia to the underlying mechanism(s).

20: Even though congenital vascular malformations are usually asymptomatic, they may be the underlying etiology for dysphagia, dyspnea, or cyanosis, by causing external compression on the esophagus and/or trachea. We recommend that congenital vascular malformations be excluded in these situations by chest CT or MR angiography.

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.

22: Intensive early enteral and oral nutrition intervention and advances in neonatal care and surgery have reduced the risk of long term malnutrition in children with EA. However, other associated comorbidities may increase this risk.

23: In addition to relative esophageal narrowing at the level of the anastomosis (by contrast and/or endoscopy), significant functional impairment and associated symptoms need to be present for anastamotic strictures to be considered clinically significant.

24: There is no evidence that routine screening and dilation is superior to evaluation and treatment in symptomatic patients. We recommend that AS be excluded in symptomatic children, and those children who are unable to achieve feeding milestones.

25: Diagnosis of anastomotic stricture can be done by either contrast study and/or endoscopically.

26a: We recommend that dilation be performed in children with EA under general anesthesia and tracheal intubation.

27: No evidence exists on the definition of recurrent anastomotic stricture in EA patients. Based on expert opinion we believe 3 or more clinically relevant stricture relapses constitutes recurrent stricture.

28: Potential adjuvant treatments for the management of recurrent strictures in EA patients may include intralesional and/or systemic steroids, topical application of mitomycin C, stents and an endoscopic knife.

29: In EA patients we recommend esophagram as the first step in suspected CES, and endoscopy to confirm the diagnosis and exclude other pathology.

30: We suggest endoscopic dilation as the first line of treatment in CES.

Statement 31: EoE needs to be excluded in EA patients of all ages with dysphagia, reflux symptoms, coughing, choking, or recurrent strictures that are refractory to PPI, before proceeding to anti-reflux surgery.

32: We recommend multiple esophageal biopsies, both proximal and distal to the anastomosis for the diagnosis of EoE. Management of EA patients with EoE should follow consensus recommendations for treatment of EoE in the general population.

33a: Dysphagia and symptoms of GER continue into adulthood in EA patients, and are more frequent in EA survivors than in the general population.

34: We recommend transition of young adults from pediatric care to an adult physician with expertise in EA (general practitioner, surgeon, gastroenterologist, pulmonologist, or any informed specialist aware of the specificities of the care of adults operated for EA).

35: We recommend regular clinical followup in every adult patient with EA, with special reference to presence of dysphagia, GER, respiratory symptoms and anemia with:

36: Although GI and respiratory symptoms and associated comorbidities (esophageal replacement and congenital anomalies) may negatively impact HRQoL, no evidence currently shows that the overall HRQoL is impaired in children and adults with EA compared with the general population. We recommend long-term medical and psychosocial support for these patients and families.