Posterior aortopexy is the option

Tracheomalacia is the abnormal collapse and ﬂ accidity of the supporting tracheal cartilage, leading to a reduction in the caliber of the anterior-posterior airway. Tracheomalacia is common after cardiac compression or tracheoesophageal ﬁ stula. In the severe type, tracheomalacia may lead to signi ﬁ cant morbidity, especially if encountered at a younger age or in a critical area, such as the distal part of the trachea. We report two cases that did not improve after initial cardiac surgery with persistent airway narrowing of more than 80%, requiring prolonged ventilatory support. Both cases were di ﬃ cult to manage but eventually showed a signi ﬁ cant improvement after posterior aortopexy, which helped to wean them off the ventilator and produced marked clinical and radiological improvement. Both cases had residual malacia that was clinically presented as persistent audible wheezing and recurrent chest exacerbation. The diagnostic and therapeutic options for both cases are discussed.


Introduction
Malacia is an abnormal collapse of the airway walls [1]. There are three main types of malacia: laryngomalacia, tracheomalacia and bronchomalacia [2]. Laryngomalacia is a common pediatric problem that often presents with stridor's breath a few weeks after delivery and subsides by the fi rst year in the majority of cases. Laryngoma is often a self-limited condition unless it affects feeding, breathing or growth. Refl ux therapy is the fi rst-line therapy if the condition affects feeding, breathing, or growth, followed by epiglottoplasty or other options, as indicated by the laryngologist. Tracheomalacia often affects the distal third of the trachea, and the lesion typically occurs in infants and young children. There are two main types of tracheomalacia [3].

Primary (rare)
This rare type is characterized by congenital absence or abnormal compliance of the cartilaginous part of the tracheal lumen.

Secondary (common)
This type is due to tracheoesophageal fi stula, cardiac or mediastinal compression, or prolonged intubation. In the majority of patients, the tracheal cartilage normalizes as the airway enlarges, and symptoms may resolve with time and often by late childhood [4]. Clinical symptoms can range from minor expiratory stridor with typical barking cough, recurrent/ persistent wheezy chest, or severe respiratory distress episodes leading to apnea [5,6]. Aortopexy was proven to be helpful in severe types of tracheomalacia [7,8], whereas bronchomalacia is a rare condition that is often segmental and secondary to other conditions, such as cardiac compression.

Case report (1)
A 6-month-old girl, a product of full-term, normal, vertex delivery, presented with poor weight gain and interrupted feeding with signs of respiratory distress. Later, at the age of three months, she was diagnosed with congenital heart disease with a preoperative diagnosis of type 1 truncus arteriosus with severe truncal valve stenosis and regurgitation, and the chest    the fourth year, they had two, very limited, short admissions and did not require PICU admission ( Figure 5).

Discussion
Tracheomalacia is a common reason to consult pediatric pulmonologists, especially at cardiac centers. The presentation of patients with suspected tracheomalacia depends on the severity of malacia, location, length of segment, age and associated anomalies, which could vary from asymptomatic to ventilatordependent or persistent lung collapse [3]. As the common site is the distal third of the trachea in moderately severe cases, children will present with persistent wheezing and a barky cough that does not respond to asthma therapy [4]. In addition, the cough may worsen after the use of a bronchodilator or a viral respiratory illness [5]. Severe cases manifest as episodic paroxysmal cough, cyanotic episodes leading to the loss of consciousness, apnea, and even cardiac arrest, similar to pertussis-like disease, and the initial assessment depends on the severity and potential underlying cause [6]. Wheezing is the usual presentation, especially when excited, such as playing, laughing or due to chest infection. However, some cases could present with stridor if the malacia is long and affects the upper third of the trachea as it is outside the thoracic inlet. Bronchomalacia is commonly observed in the cardiac center, often due to vascular compression from cardiac chamber enlargement or vascular compression, leading to variable abnormalities on the left-sided chest ranging from hyperinfl ation when there is partial compression to near or total collapse of the left-sided lung. The diagnosis is easy and can be confi rmed by fl exible bronchoscopy with minimal sedation, but treatment depends on the severity. Mild cases often resolve spontaneously with no intervention [5,6]. Moderate to severe cases require supportive therapy, suctioning and chest physiotherapy, lower thresholds for starting antibiotics, minimal use of bronchodilators and potential CPAP therapy during exacerbation, and in severe cases, tracheopexy, tracheoplasty and stenting of the airway have been reported in the literature with variable and limited indications [7][8][9][10].

Conclusion
Vascular compression of the left main bronchus is not uncommon in the pediatric age group, especially in those with congenital heart disease. Posterior aortopexy has proven to be a safe, effective, and simple technique for managing a complex pathology and preventing further irreversible damage to the airway. It can help to avoid more complex interventions that could increase both morbidity and mortality. Residual malacia should be considered a common complication, and the management of such cases is a real challenge that requires a multidisciplinary team.