Mini-symposium: Esophageal Atresia and Tracheoesophageal Fistula
Motility, digestive and nutritional problems in Esophageal Atresia

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Summary

Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is a rare congenital malformation. Digestive and nutritional problems remain frequent in children with EA both in early infancy and at long-term follow-up. These patients are at major risk of presenting with gastroesophageal reflux and its complications, such as anastomotic strictures. Esophageal dysmotility is constant, and can have important consequences on feeding and nutritional status. Patients with EA need a systematic follow-up with a multidisciplinary team.

Section snippets

Gastro-esophageal reflux (GER)

Patients with EA are at major risk of having GER [5]. Several factors contribute to the physiopathology of GER in EA (Table 1).

Anastomotic stricture

An anastomic stricture [AS] may arise because of important anastomotic tension related to a long-gap between the two esophageal pouches, occurrence of a post-operative anastomotic leak and GER [18].

Dysmotility

Dysmotility is not only a consequence of surgical repair as abnormal innervation is present at birth. It involves the Auerbach plexus, ganglion cells, interstitial cells of

GER

GER is frequent in patients with EA, especially in isolated forms, where it is reported in almost all the patients and often requires fundoplication [30]. Depending on age, patient selection and diagnostic methods, the prevalence of GER in patients with EA varies from 20% to 63% [4], [6], [10], [17], [31], [32], [33], [34], [35], [36], [37], [38], [39], [40], [41], [42], [43], [44], [45]. GER can persist lifelong. Complications such as late or recurrent anastomotic stenosis, esophagitis and

Gastro-esophageal Reflux

GER is frequent during infancy and complications due to GER are observed mostly during the first 2 years of life [42]. Most of the complications due to GER in EA are linked to acid reflux (ie esophagitis, refusal to eat, anastomotic stenosis) rather than non-acidic reflux. Medical management of GER by whatever the agent used (mainly proton pump inhibitor (PPI) and H2 receptor antagonists) is successful by reducing gastro-intestinal symptoms, respiratory symptoms or showing an improved weight

Special focus on long gap EA

Long-gap (LG) EA is a high-risk group for digestive and nutritional problems. LG represents 10% to 26% of the total esophageal atresia (EA) patients –according to its definition- mainly observed in type A (pure EA >50%) but also in other forms (type B, C and D). It is a major surgical challenge. Options for esophageal reconstruction include the use of native esophagus –which is the preferred strategy- or esophageal replacement with stomach, colon, or small intestine. Besides surgical

Conflicts of interest

None

Future directions for clinical research

  • To assess natural history of gastro-esophageal reflux with pH-impedancemetry.

  • To assess the incidence of Barrett esophagus and esophageal carcinoma in children and adults with esophageal atresia.

  • To assess the prognostic value of high definition manometry in esophageal atresia.

  • To test new prokinetics in the management of dysphagia.

Educational Aims

The reader will come to appreciate that:

  • Digestive and nutritional problems are frequent and interlinked in esophageal atresia.

  • A multidisciplinary approach is needed in esophageal atresia.

  • Esophageal atresia is not only a surgical neonatal problem but has lifelong consequences for digestive and nutritional morbidity.

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