[size=20px]A 4-month old girl underwent repair of esophageal atresia with a distal tracheoesophageal fistula at birth.She continues to have stridor, wheezing, and brief resolved unexplained events (BRUE).Her reflux is controlled with proton pump inhibitors.Airway evaluation demonstrates tracheomalacia and she undergoes aortopexy. Which preoperative factor is associated with increased risk of failure of aortopexy?

 

A Compression from innominate artery

 

B Laxity of cartilaginous rings

 

C > 80% narrowing of trachea

 

D Presence of bronchomalacia

 

E Posterior tracheopexy at initial repair[/size]

C

correct answer

D Presence of bronchomalacia

 

Although tracheomalacia is the most common congenital tracheal anomaly, most children are either asymptomatic or minimally symptomatic. Tracheomalacia is due to abnormally compliant trachea that collapses during the respiratory cycle. Coughing and crying will often exacerbate symptoms. Tracheomalacia is associated with all types of esophageal atresia (EA) and tracheoesophageal fistula (TEF). In-utero dilation of the esophageal pouch leads to abnormal development of the cartilage of the trachea resulting in a wide membranous portion rather than the normal C-shape. Symptoms range from minimal (only noted with upper respiratory infections) to severe (cyanotic spells) with clinically significant symptoms seen in 10-30% of patients.

 

Patients with repaired EA/TEF who develop cyanotic spells or brief resolved unexplained events (BRUE) require prompt evaluation with bronchoscopy. Bronchoscopy should be performed while the patient is spontaneously ventilating in order to appreciate the dynamic collapse of the trachea. The percentage of airway collapse should be estimated with > 75% collapse considered as severe. The extent of compression and any involvement of the larynx proximally and bronchial structures distally should also be noted. Anterior extrinsic pulsatile compression will be seen in children with innominate artery or other vascular compression.

 

Aortopexy is effective in correcting anterior tracheal wall collapse. It is very effective when the compression of the anterior trachea is due to the innominate artery. Suturing the adventitia of the aortic arch to the sternum leads to ventral suspension of the trachea. Intraoperative endoscopy is valuable and allows the surgeon to visualize the improvement of the airway collapse. Aortopexy is not effective in correcting bronchomalacia. Previous studies have shown the presence bronchomalacia to be an independent risk factor predicting reintervention following aortopexy.

 

Recently, posterior tracheopexy has been advocated to treat the posterior membrane collapse associated with EA/TEF. Coupled with aortopexy, posterior tracheopexy may improve bronchomalacia involving the left main stem bronchus. Posterior tracheopexy has also been advocated at the time of initial EA/TEF repair in select patients with severe tracheal collapse.