Posterior Tracheopexy for Severe Tracheomalacia Associated with Esophageal Atresia (EA): Primary Treatment at the Time of Initial EA Repair versus Secondary Treatment.

Hester F. Shieh,C. Jason Smithers,Russell W. Jennings,Gary A. Visner,Thomas E. Hamilton,Christopher W. Baird,David Zurakowski,Michael A. Manfredi

doi:10.3389/fsurg.2017.00080

Hester F. Shieh, C. Jason Smithers + Show 6 more

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https://doi.org/10.3389/fsurg.2017.00080

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Abstract

We review outcomes of posterior tracheopexy for tracheomalacia in esophageal atresia (EA) patients, comparing primary treatment at the time of initial EA repair versus secondary treatment. All EA patients who underwent posterior tracheopexy from October 2012 to September 2016 were retrospectively reviewed. Clinical symptoms, tracheomalacia scores, and persistent airway intrusion were collected. Indication for posterior tracheopexy was the presence of clinical symptoms, in combination with severe tracheomalacia as identified on bronchoscopic evaluation, typically defined as coaptation in one or more regions of the trachea. Secondary cases were usually those with chronic respiratory symptoms who underwent bronchoscopic evaluation, whereas primary cases were those found to have severe tracheomalacia on routine preoperative dynamic tracheobronchoscopy at the time of initial EA repair. A total of 118 patients underwent posterior tracheopexy: 18 (15%) primary versus 100 (85%) secondary cases. Median (interquartile range) age was 2 months (1-4 months) for primary (22% type C) and 18 months (8-40 months) for secondary (87% type C) cases (p < 0.001). There were statistically significant improvements in most clinical symptoms postoperatively for primary and secondary cases, with no significant differences in any postoperative symptoms between the two groups (p > 0.1). Total tracheomalacia scores improved significantly in primary (p = 0.013) and secondary (p < 0.001) cases. Multivariable Cox regression analysis indicated no differences in persistent airway intrusion requiring reoperation between primary and secondary tracheopexy adjusting for imbalances in age and EA type (p = 0.67). Posterior tracheopexy is effective in treating severe tracheomalacia with significant improvements in clinical symptoms and degree of airway collapse on bronchoscopy. With no significant differences in outcomes between primary and secondary treatment, posterior tracheopexy should be selectively considered at the time of initial EA repair.

Highlights

Tracheomalacia is often associated with esophageal atresia (EA), tracheoesophageal fistula (TEF), and cardiac disease [1, 2]
Older studies report a prevalence of 11–33% in this population, likely an underestimate given the wide spectrum of disease and common misdiagnosis in the pediatric population, with a recent study reporting tracheomalacia in 87% of EA patients [2, 7,8,9,10,11,12]
And accurate diagnosis of tracheomalacia is important because excessive airway collapse leads to ineffective ventilation and poor clearance of secretions, resulting in chronic respiratory symptoms that can progress to bronchiectasis in up to 27% of EA patients by 8 years of age, and in the most severe cases, blue spells and brief resolved unexplained events (BRUEs) [1, 6, 7, 12, 13]

Summary

Introduction

Tracheomalacia is often associated with esophageal atresia (EA), tracheoesophageal fistula (TEF), and cardiac disease [1, 2]. Severe tracheomalacia is characterized by dynamic airway collapse in spontaneously breathing patients with anterior vascular compression, posterior membranous tracheal intrusion, or both [3]. Aortopexy addresses anterior vascular compression by indirectly elevating the anterior wall of the trachea but does not directly address posterior membranous tracheal intrusion [4]. We previously reported a series of patients who underwent posterior tracheopexy for severe tracheomalacia with posterior intrusion with promising short-term results, these reports did not distinguish between primary treatment at the time of initial EA repair and secondary treatment [2, 3, 5]. We review outcomes of posterior tracheopexy in EA patients, comparing primary treatment at the time of initial EA repair versus secondary treatment, to determine whether there were resolution of clinical symptoms and bronchoscopic evidence of improvement in airway collapse

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