Distinct and reproducible esophageal motility patterns in children with esophageal atresia

Objectives: Esophageal atresia (EA) is a significant congenital anomaly, with most survivors experiencing esophageal dysmotility. Currently, there is no reliable way to predict which patients will develop significant, life-threatening dysmotility. Using high-resolution impedance manometry (HRIM), this study aimed to characterize the common pressure topography patterns in children with repaired EA. Methods: This prospective longitudinal cohort study focused on children (<18 years) with repaired EA. Utilizing HRIM, esophageal motility patterns were studied. Repeat manometric assessments were performed in a selected group. Results: Seventy-five patients with EA (M:F = 43:32, median age 15 months [3 months to 17 years]) completed 133 HRIM studies. The majority (54 out of 75, 85.3%) had EA with distal tracheo-esophageal fistula. Thirty-five out of 75 (46.7%) underwent one study, 24 out of 75 (32.0%) two studies, 14 out of 75 (18.7%) three studies, and 2 out of 75 (2.7%) four studies. Seventy-two patients had analyzable studies. Three common motility patterns were demonstrated: (1) aperistalsis (26 out of 72, 36.1%); (2) distal esophageal contraction (25 out of 72, 34.7%); and (3) pressurization (6 out of 72, 8.3%). A minority demonstrated combination patterns, including aperistalsis with weak distal contraction (10 out of 72, 13.9%) and aperistalsis with pressurization (2 out of 72, 2.8%). Contraction was normal in 3 out of 72 (4.2%). At repeat assessment, the dominant motility pattern persisted in 26 out of 38 (68.4%) of the second studies and 9 out of 15 (60.0%) of the third studies. Conclusion: Utilizing HRIM in children with repaired EA, we have demonstrated objective, distinct, and reproducible motility patterns. In this cohort, the majority of motility patterns were maintained longitudinally, and dysphagia scores remained unchanged, even after dilatation.