Congenital tracheal stenosis is a rare and potentially life-threatening anomaly that often necessitates invasive mechanical ventilation and definitive tracheal reconstruction. Patients typically present with inspiratory stridor, expiratory wheezing, apnea, feeding difficulties, recurrent pneumonia, upper respiratory tract infections, and episodes of cyanosis [2, 5]. Up to 60% of cases are reported to have associated congenital malformations, including vascular rings, congenital heart diseases, tracheoesophageal fistula, and hemivertebrae [6].

Diagnosis is sometimes complicated; it can be achieved through chest radiographs, bronchoscopy, CT scans, and MRI [1]. CT is reported to be superior to MRI in providing higher spatial resolution with more apparent tracheal contours and shorter scanning times. Congenital tracheal stenosis consists of complete cartilaginous rings. It can be classified according to Grillo classification: type I—generalized tracheal hypoplasia, where nearly the entire trachea is stenotic, with only average cranial rings 1–3; type II—funnel-shaped tracheal narrowing, where the standard segment varies in location and length but always assumes a funnel shape from cranial to caudal; type III—segmental tracheal stenosis, characterized by short-segment stenosis at various levels of the trachea, sometimes below an anomalous right upper lobe bronchus; and type IV—bridge bronchus stenosis is a variant of type III, in which the anomalous right upper lobe bronchus lies near the carina via horizontally branching bronchi, and the stenotic bridge bronchus connects the proximal trachea to the rest of the lungs [7, 8].

Various approaches exist for managing congenital tracheal stenosis, from observation to various surgical treatments [8]. The patient’s age and symptoms during initial evaluation are crucial in determining the treatment course. Patients are generally categorized into three groups.

Patients with mild stenosis (narrow posterior membranous trachea, asymptomatic or with occasional symptoms) allow for observation over a variable period, monitoring of airway growth, and accompanying symptoms. Patients with moderate stenosis (absence of posterior membranous trachea, complete tracheal rings, symptomatic but without critical airway compromise) require short-term surgical intervention. Patients with severe stenosis (complete tracheal rings, highly symptomatic, and critically compromised airway) can be further subdivided into type A, without associated anomalies, and type B, with associated anomalies, necessitating immediate intervention [9].

Traditional surgical treatment options include endoscopic dilation with or without stent placement, tracheal resection with end-to-end anastomosis for short-segment stenosis, and sliding tracheoplasty, as well as its modified version for long-segment stenosis, along with the placement of biological grafts, with varied success rates [10]. Surgical intervention depends on the surgeon’s preference; however, sliding tracheoplasty is currently the treatment for severe congenital stenosis, as it allows for a loss of only 50% of the stenotic trachea’s length. This procedure is typically performed via median sternotomy with ECMO or extracorporeal circulation [5, 11]. The original sliding tracheoplasty technique described by Tsang and colleagues in 1989 [2] does not include carinal repair. It is generally not considered an option for infants with involvement of both proximal bronchi, for whom the modified technique is recommended [12, 13].

Concomitant anomalies, such as atrial and ventricular septal defects, can be corrected simultaneously. Complex anomalies, such as Tetralogy of Fallot, pulmonary atresia with ventricular septal defect, and complete atrioventricular septal defects, are recommended for staged repair [7]. However, the decision between simultaneous or staged repair is controversial; simultaneous repair is believed to be more physiological, but surgeries requiring prolonged cardiopulmonary bypass are generally associated with a poor prognosis [4, 14].

A retrospective observational study conducted from 1998 to 2018 described that staged repair will be offered in patients with complex abnormalities, especially if they are newborns, premature, or weigh less than 5 kg. They can be operated on during the same hospitalization, following the correction of the most limiting pathology first, followed by the next anomaly. After a cardiovascular surgeon establishes ECMO or extracorporeal circulation, any cardiovascular anomalies are corrected, and a pediatric surgeon performs tracheoplasty. The reported criteria for surgical intervention in congenital tracheal stenosis include symptoms like persistent wheezing, recurrent respiratory infections, respiratory failure requiring endotracheal intubation, an inability to wean off mechanical ventilation, a degree of stenosis like long-segment stenosis and a segmental diameter of less than 50% of the average value, a potentially life-threatening dyspnea, and the need for endotracheal intubation [2, 4, 15].

Urgent surgery is suggested in cases with severe symptoms or repeated respiratory infections after controlling inflammation. Elective surgery is recommended for mild symptoms, managed conservatively at first, with surgical correction delayed until 6 months of age [2].

Factors associated with prolonged intubation have been reported to include low body weight and concurrent cardiovascular abnormalities. Factors associated with a higher likelihood of death include low body weight, preoperative advanced ventilation, prolonged extracorporeal circulation time, and the presence of granulation tissue [3, 16].

Heterogeneous factors can influence the outcomes, like postoperative tracheomalacia and restenosis. Associated factors of adverse outcomes include additional cardiovascular anomaly, carinal stenosis, and bronchial stenosis with concomitant carinal stenosis and compression. Preoperative morphological evaluation is of surgical importance, and with adequate preoperative recognition and close surveillance postoperatively, the intermediate outcomes in patients with the ring-sling complex are satisfactory [17, 18].

In the previously reported case, a patient with complex type 3 long-segment congenital tracheal stenosis, concurrent congenital heart disease with VSD and ASD, was surgically intervened in a single surgical session by the pediatric cardiothoracic service and pediatric thoracic surgery due to respiratory urgency with correction of the cardiac anomaly and the pulmonary artery sling.