Laryngotracheal Abnormalities in Esophageal Atresia Patients: A Hidden Entity

Abstract

Importance: Presence of laryngotracheal abnormalities is associated with increased morbidity and higher mortality rate in esophageal atresia patients.Objective: Determine the prevalence of laryngotracheal abnormalities (LTA) in a prospectively collected cohort of patients treated for esophageal atresia and/or tracheoesophageal fistula (EA/TEF). Analysis of the impact of those airway anomalies in early post-operative outcomes was performed.Patients and Methods: This was a review of a prospectively collected database, including patients from January 2008 to December 2017. Patients enrolled in the present study were treated in a high-volume referral center. Present study included all newborn-infants consecutively treated for EA/TEF. All patients were evaluated by flexible laryngotracheoscopy performed under local anesthesia in spontaneous breathing. In case of airway malformation suspected during flexible endoscopy, a rigid endoscopy was performed to complete airway assessment. If post-operative respiratory symptoms (noisy breathing, respiratory difficulty, failure to extubate, or difficulty feeding) were noted, a second laryngotracheoscopy was performed. Primary study outcome was to evaluate the prevalence of LTA in EA/TEF infants, characterizing of LTA, and their impact on early post-operative outcomes. Those primary study outcomes were planned before data collection began.Results: During the study period 207 patients with EA/TEF were treated. LTA had a period prevalence of 40.1% (83/207). Although no differences were recorded in terms of demographics and clinical presentation, LTA+ infants more frequently required tracheostomy (12/52, 23% vs. 0/124, 0%; p 0.0001) and were at increased risk of death (12/83, 14% vs. 5/124, 4%; p 0.009) in comparison with EA/TEF without LTA.Conclusions: Present data suggest a high prevalence of congenital LTA in patients affected by EA. Most of the abnormalities are congenital and a high proportion of patients with LTA require a tracheostomy. Mortality significantly correlates with the presence of LTA. Systematic airway endoscopic preoperative evaluation has to be pushed forward to minimize LTA-related morbidity and mortality.

Patterns of malformation associated with esophageal atresia/tracheoesophageal fistula: A retrospective single center study

“…We agree with considering alternative diagnosis when there are atypical features, however after reviewing in detail the individual records, we decided to separate the patients under the unknown subcategory only when the overall pattern was clearly atypical and not previously described. One study reporting a 40% frequency of laryngotracheal anomalies in TEF patients (Conforti et al, 2018; Hseu, Recko, Jennings, & Nuss, 2015), considered tracheomalacia a laryngeal defect which we did not, as recommended by Rasmussen et al (2003). We did find a similar rate of laryngeal cleft malformation (Table 3).…”

Care Recommendations for the Respiratory Complications of Esophageal Atresia-Tracheoesophageal Fistula: The International Network of Esophageal Atresia, Respiratory Complications Working Group

“…Of special interest are associated abnormalities of the upper airways [74][75][76][77] as well as various cardiovascular anomalies [78][79][80][81][82][83][84] . All of these conditions increase the morbidity and, in the case of congenital heart disease, the mortality as well 85 .…”

Tracheostomy in Postoperative Pediatric Cardiac Surgical Patients—The Earlier, the Better

“…Conforti et al in their study found that infants with congenital airway anomalies more frequently required ET (12/52, 23% vs. 0/124, 0%; p = 0.0001). 24 We did not make any such observation in our study cohort.…”