In patients with EA, the key role of the abnormal development of esophageal innervation and musculature in esophageal dysmotility is supported by several lines of evidence. Romeo et al. have reported an esophageal manometry study in 20 newborns with EA and have demonstrated motor abnormalities in the proximal (pouch) and distal esophagus prior to surgery [32]. Similarly, abnormal esophageal motility patterns have been described in children and adults with isolated TEF without atresia before surgical repair [40].

Pathological data support also the role of abnormal intrinsic and vagal innervation of the esophagus. Detailed analysis of esophageal intrinsic innervation in deceased newborns with EA reported abnormalities in the Auerbach plexus (plexus hypoplasia, abnormal interganglionic network) [41]. Other studies found hypoplasia of esophageal innervation or smooth muscle [42] in the proximal pouch [43], in the distal esophagus [22, 23], or in the fistula [42, 44]. Findings on adriamycin-induced EA fetal rat model have similarly shown an abnormal distribution of nerve tissue in the esophagus [45] and inherent abnormalities in the branching pattern of the vagus nerves [46].

On the other hand, the dysmotility may also be secondary to the dissection during surgery damaging vagal nerve and its esophageal branches [22]. However, unilateral vagotomy has no effect on peristalsis, presumably because of extensive crossover of vagal innervation within the esophageal wall [47]. Surgery may also result in an extensive mobilization and denervation of the esophagus. Shono et al. demonstrated in two patients with pure EA studied before surgery a coordinated peristalsis between the proximal and the distal esophagus as well as a normal LES reflex relaxation suggesting that surgery may alter the esophageal motility [48]. However, this is not supported by experimental animal studies where transection and anastomosis of the esophagus did not cause severe esophageal dysmotility [49].

Nissen fundoplication may worsen the symptoms of esophageal dysmotility, and careful attention must be considered when such procedure is indicated [3]. The wrap creates a mechanical obstruction for those patients with an abnormal esophageal motility leading to a potential exacerbation of the dysphagia secondary to the combination of reduced esophageal motility and a tight wrap and outflow obstruction at the wrap level. Current recommendations suggest that antireflux procedure may be considered if, despite maximal medical therapy, there is life-threatening or life-limiting symptoms (recurrent esophageal strictures, poorly-controlled GERD despite maximal PPI therapy, long-term dependence on transpyloric feeding, dying spells) [3].

Romeo et al. reported that 36 % of patients with EA have delayed gastric emptying on scintigraphy and 45 % abnormal gastric peristalsis activity on manometry [50]. Using ¹³C-octanoate gastric emptying breath test, Van Wijk et al. reported 57 % of a small cohort of children having a gastric emptying time >90th % [10].

Dumping syndrome is often unrecognized and its diagnosis delayed. In children with EA, it is most often encountered after a fundoplication or in patients with microgastria [51]. It has also been reported in patients with EA with no other precipitating factors [52]. Whether gastric motility disorder may be primitive or secondary to vagus nerve injury is unknown.