Single-center experience with aortopexy in pediatric tracheomalacia: a case series

Tracheomalacia in infants often results from vascular compression and is frequently associated with multiple congenital anomalies. Aortopexy is a well-established surgical treatment for severe cases; however, long-term outcomes vary significantly, especially in patients with syndromic conditions.

This study aims to present the clinical, surgical, and long-term outcomes of aortopexy performed in six infants diagnosed with severe tracheomalacia and multiple congenital anomalies, focusing on determining whether persistent morbidity and mortality are attributable to the airway intervention itself or to underlying comorbidities.

We conducted a single-center case series involving six infants diagnosed with tracheomalacia confirmed by rigid bronchoscopy. All subjects underwent open aortopexy combined with thymectomy (partial, subtotal, or total). Data collected included preoperative respiratory support status, bronchoscopic findings-specifically the degree of airway collapse and posterior wall dynamics-perioperative details, and long-term follow-up outcomes. Due to the small sample size and lack of a control group, no formal analysis was performed to determine the independent effects of thymectomy.

Each patient presented with multiple congenital anomalies, with symptom onset ranging from 6 days to 1 year of age. Surgical approaches included thoracotomy in five cases and cervicotomy in one. Preoperative respiratory support varied: two patients required invasive mechanical ventilation, two received non-invasive support, one was on supplemental oxygen, and one required no respiratory assistance. Thymectomy was successfully performed in all cases (partial in four, subtotal in one, and total in one) without intraoperative complications. No major early postoperative complications were observed; however, two patients required reintervention-one for tracheal stenosis and one for tracheostomy plug removal-and two patients died from comorbidity-related causes (acute respiratory distress syndrome and pulmonary hypertension complicated by COVID-19). Among the four patients with extended follow-up (up to eight years), persistent morbidities such as tracheal stenosis, gastroesophageal reflux disease, and scoliosis were common, with half reporting poor or fair quality of life.

Aortopexy effectively alleviates airway obstruction in infants with tracheomalacia secondary to vascular compression, especially when guided by intraoperative bronchoscopy. However, long-term outcomes are primarily influenced by associated congenital anomalies rather than procedural failure. Although routine thymectomy was feasible, its additional benefit remains unproven. Future multicenter prospective studies and investigations into adjunctive surgical techniques, such as posterior tracheopexy, are warranted to improve prognosis in this complex patient population.