Tracheobronchomalacia, Tracheobronchial Compression, and Tracheobronchial Malformations: Diagnostic and Treatment Strategies.

Tracheobronchomalacia (TBM) is an excessive dynamic narrowing of the airway that is greatest with increased mediastinal pressure such as coughing, Valsalva, and forced expiration. Airway compression and/or cartilage malformation is a fixed or static narrowing of the airway typically caused by great vessel malposition and/or abnormalities and may also contribute to airway narrowing. Although imprecise and misleading, the term TBM is often used to represent both problems, static and dynamic airway narrowing, which only serves to confuse and may mislead the treatment team into ineffective therapies. The consequences of airway narrowing caused by dynamic TBM and/or static compression includes a range of clinical signs and symptoms, depending on the location, extent, and severity of the airway collapse. All patients with mild to severe TBM benefit from medical management to optimize airway clearance of mucus. The milder cases of TBM may become asymptomatic with this therapy, allowing time for the child to grow and the airway to enlarge without the consequences of recurrent infections. In cases of more severe TBM with clinical sequelae, more aggressive management may be warranted. Multiple options for surgical intervention are available. This article discusses the details of clinical presentation, evaluation, diagnosis, and a variety of treatments.

Circumflex aorta: An uncharted territory.

BACKGROUND: Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to tracheo-esophageal compression. Circumflex retro-esophageal aortic arch is an extreme form of true vascular ring. It remains an uncharted territory to many surgeons. METHODS: A comprehensive search of peer reviewed journals was completed based on the key words, "Circumflex aorta," "Circumflex retro-esophageal aorta" and "circumflex arch" using Google scholar, Scholars Portal Journals and PubMed. The reference section for each article found was searched to obtain additional articles. Literature on the circumflex aorta was reviewed starting from the embryogenesis to the latest management strategies. RESULTS: Right circumflex aorta is more prevalent compared to left circumflex aorta. It can occur in isolation or in association with other intracardiac lesions. Mainly presents in children, however reported in adults too. The presentation may vary from asymptomatic lesion to acute respiratory distress secondary to airway compression. Computerized tomography (CT) and magnetic resonance imaging (MRI) are important tools in delineating the vascular anatomy. Aortic uncrossing is the definitive procedure. However, the role of concomitant tracheobronchopexy is emerging. Native tissue-to-tissue anastomosis is commonly preferred, but cases of extra-anatomic grafts are reported. CONCLUSION: Circumflex aorta is amenable to complete repair. Preoperative delineation of anatomy is important for successful surgical outcome. Division of the retro-esophageal segment is crucial in relieving the compressive symptoms. In addition, tracheobronchopexy is helpful in addressing residual tracheomalacia but this accounts for a high-risk surgery.

Aortic uncrossing and tracheobronchopexy corrects tracheal compression and tracheobronchomalacia associated with circumflex aortic arch.

OBJECTIVE: Aortic uncrossing is an effective procedure for relieving the external airway compression from a circumflex aortic arch by transferring the aortic arch to the same side as the descending aorta. However, patients frequently have residual tracheobronchomalacia (TBM), which may result in persistent postoperative symptoms. We review a series of patients who underwent an aortic uncrossing and concomitant tracheobronchopexy to correct the airway compression and residual TBM. METHODS: Retrospective review of all patients who underwent aortic uncrossing and concomitant tracheobronchopexy at a single institution between September 2016 and March 2019. Preoperative evaluation included computed tomography angiography and rigid 3-phase dynamic bronchoscopy. RESULTS: Eight patients who ranged in age from 4 months to 15 years with significant respiratory symptoms underwent an aortic uncrossing procedure with concomitant tracheobronchial procedures. Mild hypothermic cardiopulmonary bypass (mean time, 105.6 ± 39.4 min) and regional perfusion (mean time, 44 ± 10 min) were used without circulatory arrest. Intraoperative bronchoscopy demonstrated no patients had residual TBM. There were no postoperative mortalities, neurologic complications, chylothoraces, coarctations, or obstructed aortic arches. Two patients required tracheostomy and gastrostomy for bilateral recurrent laryngeal nerve paresis (patients 2 and 3). One patient with bronchial stenosis after concomitant slide bronchoplasty required stenting. At a median follow-up of 22 months (range, 5-34 months), all patients were alive without evidence of significant respiratory symptoms. CONCLUSIONS: The aortic uncrossing procedure can be performed safely in pediatric patients of all ages without circulatory arrest. Concomitant procedures addressing associated TBM can significantly improve respiratory symptoms.

Tracheomalacia and Tracheobronchomalacia in Pediatrics: An Overview of Evaluation, Medical Management, and Surgical Treatment.

Tracheobronchomalacia (TBM) refers to airway collapse due to typically excessive posterior membrane intrusion and often associated with anterior cartilage compression. TBM occurs either in isolation or in association with other congenital or acquired conditions. Patients with TM typically present non-specific respiratory symptoms, ranging from noisy breathing with a typical barking cough to respiratory distress episodes to acute life-threatening events and recurrent and/or prolonged respiratory infections. There are no definitive standardized guidelines for the evaluation, diagnosis, and treatment of TBM; therefore, patients may be initially misdiagnosed and incorrectly treated. Although milder cases of TBM may become asymptomatic as the diameter of the airway enlarges with the child, in cases of severe TBM, more aggressive management is warranted. This article is an overview of the clinical presentation, evaluation, diagnosis, medical management, and surgical treatment options in pediatric tracheomalacia.

Direct tracheobronchopexy via left lateral thoracotomy for severe tracheobronchomalacia.

An infant with pulmonary atresia/ventricular septal defect/major aortopulmonary collateral arteries underwent unifocalization, ventriculoseptal defect closure and placement of a right ventricle to pulmonary artery conduit via median sternotomy. Aortopexy and pulmonary arteriopexy via redo sternotomy were insufficient to allow weaning of continuous positive airway pressure and he required direct tracheobronchopexy via left lateral thoracotomy to alleviate posterior trachealis intrusion along the length of the trachea and left main bronchus. He also underwent laryngotracheoplasty with placement of a posterior costal cartilage graft for bilateral vocal cord paralysis. The patient was weaned from positive pressure and discharged in stable condition.

Pediatric tracheomalacia.

Tracheomalacia (TM) is defined as an increased collapsibility of the trachea due to structural anomalies of the tracheal cartilage and/or posterior membrane. Tracheomalacia has a wide range of etiologies but is most commonly present in children born with esophageal atresia and tracheal esophageal fistula. Clinical symptoms can range from minor expiratory stridor with typical barking cough to severe respiratory distress episodes to acute life-threatening events (ALTE). Although the majority of children have mild-to-moderate symptoms and will not need surgical intervention, some will need life-changing surgical treatment. This article examines the published pediatric literature on TM, discusses the details of clinical presentation, evaluation, diagnosis, and a variety of treatments.

Direct tracheobronchopexy to correct airway collapse due to severe tracheobronchomalacia: Short-term outcomes in a series of 20 patients.

PURPOSE: Tracheobronchomalacia (TBM) is associated with esophageal atresia, tracheoesophageal fistulas, and congenital heart disease. TBM results in chronic cough, poor mucous clearance, and recurrent pneumonias. Apparent life-threatening events or recurrent pneumonias may require surgery. TBM is commonly treated with an aortopexy, which indirectly elevates trachea's anterior wall. However, malformed tracheal cartilage and posterior tracheal membrane intrusion may limit its effectiveness. This study describes patient outcomes undergoing direct tracheobronchopexy for TBM. METHODS: The records of patients that underwent direct tracheobronchopexy at our institution from January 2011 to April 2014 were retrospectively reviewed. Primary outcomes included TBM recurrence and resolution of the primary symptoms. Data were analyzed by McNemar's test for matched binary pairs and logistic regression modeling to account for the endoscopic presence of luminal narrowing over multiple time points per patient. RESULTS: Twenty patients were identified. Preoperative evaluation guided the type of tracheobronchopexy. 30% had isolated anterior and 50% isolated posterior tracheobronchopexies, while 20% had both. Follow-up was 5 months (range, 0.5-38). No patients had postoperative ALTEs, and pneumonias were significantly decreased (p=0.0005). Fewer patients had tracheobronchial collapse at postoperative endoscopic exam in these anatomical regions: middle trachea (p=0.01), lower trachea (p<0.001), and right bronchus (p=0.04). CONCLUSION: The use of direct tracheobronchopexy resulted in ALTE resolution and reduction of recurrent pneumonias in our patients. TBM was also reduced in the middle and lower trachea and right mainstem bronchus. Given the heterogeneity of our population, further studies are needed to ascertain longer-term outcomes and a grading scale for TBM severity.