Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Tracheomalacia Tracheomalacia is a condition of the neonatal and infant airway characterized by weakness of the supporting tracheal cartilage and widening of the posterior membranous wall. Together, these factors cause tracheal collapse especially during times of increased airflow, such as coughing, crying, or feeding. Tracheomalacia most commonly affects the distal one-third of the trachea and can be associated with a variety of congenital anomalies including cardiovascular defects, developmental delay, gastroesophageal reflux, and tracheoesophageal fistula. It is, however, rarely found in combination with laryngomalacia because of the separate developmental pathways for both the trachea and the larynx. Although the accepted term remains "tracheomalacia," it has been redefined by Mair and Parson as "major airway collapse." There are three categories based on the histological, endoscopic, and clinical presentation. Type one includes congenital or intrinsic tracheal abnormalities which can be associated with a tracheoesophageal fistula. Type two involves extrinsic defects or anomalies such as a vascular ring or a vascular sling causing abnormal development or undue pressure on the trachea. Type three or acquired malacia occurs in children with prolonged intubation or chronic tracheal infections and may be a progressive process. Regardless of the underlying defect or anomaly, the signs and symptoms remain similar: chronic or recurrent pulmonary infections; prolonged expiratory phase; expiratory stridor and coughing which often increases with feedings; and reflex apnea or "dying spells." The latter are episodes of progressive hypoxia, apnea, cyanosis, bradycardia and even cardiac arrest which occur during feedings. The diagnosis of major airway collapse depends on an accurate history combined with proper endoscopic evaluation. The airway must be observed during spontaneous respiration which is accomplished using ventilating laryngoscopy and telescopic bronchoscopy. The classic triad consists of 1) loss of the normal semicircular shape of the tracheal lumen; 2) forward ballooning of the posterior membranous wall; and 3) anteroposterior narrowing of the tracheal lumen. The majority of these infants will respond to conservative management of humidified air, chest physical therapy, or Continued Positive Airway Pressure (CPAP). The symptoms often resolve by the age of 18 months to 24 months. When conservative measures are not adequate or when reflex apnea is present, surgical intervention must be considered. This includes correction of the underlying cause, such as a vascular ring when present, tracheostomy and aortopexy. Although effective in some cases, a tracheostomy can cause airway damage and may not support the distal trachea. Aortopexy is considered the treatment of choice because it relieves external pressure on the flaccid trachea. It is successful in 75% of the cases of tracheomalacia. Case Presentations Case 1 A 3-month-old former 33-week premature infant underwent repair of a esophageal atresia with distal tracheoesophageal fistula, and placement of a feeding gastrostomy tube on day two of life. Postoperatively he was noted to have a barky cough, prolonged expiratory phase, expiratory stridor and episodes of cyanosis with bradycardia (reflex apnea) during feeding. A barium swallow revealed prominent gastroesophageal reflux without recurrence of the fistula. A Nissen fundoplication was performed which initially appeared to relieve the severity of the apnea. The family elected further conservative home management. However, the reflex apnea episodes increased. On readmission he was evaluated by the Otolaryngology service. Direct laryngoscopy and telescopic bronchoscopy were performed which revealed findings consistent with tracheomalacia. The Pediatric surgery service then performed an aortopexy via a left anterolateral thoracotomy approach. After the aortopexy was completed, bronchoscopy revealed a much improved tracheal lumen. Postoperatively, he had resolution of the reflex apnea. Case 2 A 3-month-old former full-term infant was noted to have noisy breathing at age 2 weeks of life. While at home this continued, consisting of sternal retraction, a barky cough and prolonged expiratory phase. Over the past month the patient had three episodes of apnea and cyanosis while feeding. A barium swallow revealed no gastroesophageal reflux. The Otolaryngology service then performed direct laryngoscopy and telescopic bronchoscopy. This revealed findings consistent with severe tracheomalacia. The patient then underwent aortopexy via a left anterolateral thoracotomy approach. Upon completion, bronchoscopy revealed improved tracheal lumen diameter. The patient had complete resolution of apnic spells. Bibliography Anedee RG, Mann WJ, Lyons GD. Tracheomalacia repair using ceramic rings. Otolaryngol Head Neck 1992;106:270-274. Applebaum H, Woolley MM. Pedicardial flap aortopexy for tracheomalacia. J Pediatr Surg 1990;25:30-32. Azizkhan RG, Lacey SR, Wood RE. 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