Traqueomalacia: diagnóstico y alternativas terapéuticas / Tracheomalacia: diagnosis and treatment options

La traqueomalacia (TM) consiste en una excesiva colapsabilidad traqueal debida a una anomalía estructural del cartílago y/o de la pared membranosa posterior. Cuando se extiende a uno o ambos bronquios principales se denomina traqueobroncomalacia (TBM). Considerando diferentes clasificaciones, la mayoría de las TM son adquiridas, localizadas e intratorácicas. El diagnóstico clínico es difícil porque los síntomas son inespecíficos y se superponen con los de otras enfermedades respiratorias crónicas. Los síntomas más frecuentes incluyen estridor espiratorio, tos perruna e infecciones respiratorias recurrentes, en los casos más graves se presentan episodios de dificultad respiratoria severa, cianosis e incluso muerte súbita. La fibrobroncoscopía sigue siendo el método diagnóstico estándar de oro, complementándose con la tomografía computarizada que es esencial en la visualización de las estructuras adyacentes a la vía aérea. En los casos leves el tratamiento es conservador, considerando la resolución espontánea de la mayoría de los casos hacia los 2 años de edad. En los pacientes más sintomáticos la estrategia terapéutica se debe evaluar caso a caso, siendo la presión positiva contínua en vía aérea (no invasiva o invasiva por traqueostomía) el tratamiento más utilizado. En las TM-TBM más severas, entre posibles tratamientos que incluyen cirugía traqueal y prótesis en la vía aérea, lo más usado es aortoarteriopexia y traqueopexia, a la espera de resultados promisorios de mallas endoluminales biodegradables y prótesis reabsorbibles personalizadas impresas en 3D.

Tracheomalacia (TM) is defined as an increased collapsibility of the trachea due to structural anomalies of the cartilaginous rings and/or the posterior membrane. When the main bronchi is also affected this condition is termed as tracheobronchomalacia (TBM). According classifications TM is mostly acquired, localized and intrathoracic. Diagnosing TM is challenging because symptoms are nonspecific and overlap with those of other chronic respiratory disorders. The most common symptoms include expiratory stridor, barking cough and recurrent respiratory tract infections, in severe cases, severe respiratory distress episodes and acute life threatening events can occur. While flexible bronchoscopy is still considered as the gold standard diagnostic method, computed tomography is essential in assessing the surrounding structures. Conservative therapy is preferred in milder cases since the outcome is usually favorable within the first 2 years of life. Treatment of more symptomatic children should be discussed on an individual basis, continuous positive airway pressure (non invasive o invasive via tracheostomy) being the most widely used therapy. For more severe TM-TBM, amongst possible treatments including tracheal surgery and airway stenting, aortoarteriopexy and tracheopexy are mostly used, nevertheless absorbable stent and 3D printed customed prosthesis are being developed with promising results.

Traqueomalacia pediátrica / Pediatric tracheomalacia

RESUMEN La malacia de la vía aérea central puede afectar la tráquea y/o los bronquios, haciéndola susceptible al colapso de sus paredes durante el ciclo respiratorio. Puede clasificarse como primaria o secundaria y clínicamente se manifiesta por síntomas respiratorios recurrentes o persistentes (especialmente en espiración), infecciones recurrentes y en casos severos, episodios de hipoventilación con cianosis. El diagnóstico se establece mediante broncoscopía flexible o rígida. Los estudios de imágenes se consideran complementarios, siendo especialmente útiles en casos de duda diagnóstica, estudio de causas secundarias o para la planificación preoperatoria. Su tratamiento depende de distintos factores como la severidad de los síntomas, su etiología, ubicación y extensión. En casos leves, una observación activa y tratamiento médico en espera de la resolución espontánea en los primeros 2 años, suelen ser suficientes. En casos moderados, la ventilación a presión positiva podría ser de utilidad. En casos severos está indicado el tratamiento quirúrgico, mediante distintas técnicas que buscan dar soporte a la estructura traqueobronquial debilitada, incluyendo traqueostomía, suspensiones directas (traqueopexias) o indirectas (aortopexia), tutores externos o stents intraluminales. En este articulo se revisarán las principales causas y tratamientos disponibles para la traqueomalacia pediátrica. Ya que su diagnóstico y manejo son complejos, es fundamental el trabajo de equipos médicos multidisciplinarios familiarizados con esta patología.

ABSTRACT Central airway malacia can affect the trachea and/or the main bronchi, making their walls susceptible to collapse during the respiratory cycle. It can be classified as primary or secondary, and clinically presents with recurrent or persistent respiratory symptoms (especially on expiration), recurrent infections and in severe cases, episodes of hypoventilation with cyanosis. The diagnosis is established by flexible or rigid bronchoscopy; imaging studies are considered as complementary, especially in cases of unclear diagnosis, secondary causes or for preoperative planning. Treatment depends on different factors such as the severity of the symptoms, their etiology, location and extension. In mild cases, active observation and medical treatment waiting for spontaneous resolution may be enough, which usually occurs in the first 2 years of age. In moderate cases, positive pressure ventilation could be useful. In severe cases, surgical treatment is indicated. Different techniques aiming to provide support to the weakened tracheobronchial structures are available, including tracheostomy, direct (tracheopexies) or indirect suspensions (aortopexy), external splints and intraluminal stents. In this article we present the main etiologies and available treatments for pediatric tracheomalacia. Given that diagnosis and management of these patients is complex, the work of multidisciplinary teams familiar with this pathology is of paramount importance.

Pediatric tracheomalacia and the perioperative anesthetic management of thoracoscopic posterior tracheopexy.

Tracheomalacia is a broad term used to describe an abnormally compliant trachea that can lead to exaggerated collapse and obstruction with expiration. We describe the perioperative management of a complex pediatric patient undergoing a posterior tracheopexy which is a relatively new surgical treatment, with a novel surgical approach-thoracoscopy. This procedure has competing surgical and anesthetic needs and presents unique challenges to the physicians involved in caring for these patients. We also review the current literature on pediatric tracheomalacia and examine the newest treatment options to highlight the potential anesthetic challenges and pitfalls associated with management.

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.

Tracheomalacia.

Tracheomalacia is excessive collapsibility of the trachea, typically during expiration. Congenital forms are associated with severe symptoms. Milder forms often present after the neonatal period. Adult malacia is mostly associated with chronic obstructive pulmonary disease. Functional bronchoscopy is still not standardized. Dynamic airway CT is a promising tool for noninvasive diagnosis. Bronchoscopy and stent insertion lead to significant improvement, but with a high complication rate. Surgical lateropexia, tracheal resection, and surgical external stabilization are options. Tracheoplasty seems to be the best choice for selected cases of adult malacia. The most commonly performed surgery in children is aortopexy.

[Tracheobronchomalacia]. / Traqueobroncomalacia.

Tracheobronchomalacia is a central airway disease characterised by weakness of the wall and dynamic decrease in the tracheal lumen and the large bronchi, particularly while exhaling. It is more common in middle age and the elderly with previous exposure to cigarettes. It causes chronic symptoms such as cough, dyspnea, increase in recurrent infections, and poor secretion management, but it can also progress to chronic respiratory failure and death. It is usually confused with other common diseases like chronic obstructive pulmonary disease (COPD) or asthma. Its causes can be congenital or acquired and its diagnosis involves the dynamic assessment of the airway with tomography and fibrobronchoscopy. It is classified as mild, moderate or severe depending on the degree of collapse of the airway when exhaling. Management consists of a primary phase, in which concomitant diseases must be controlled, such as COPD, asthma or gastro-oesophageal reflux. In diffuse moderate to severe symptomatic tracheobronchomalacia tracheobronchoplasty must be considered with strengthening of the posterior wall. Silicone and "Y" stents can be used to identify patients who could potentially benefit from surgical treatment as well as being used for the definitive symptomatic treatment with high surgical risk. More prospective studies need to be done in order to standardise certain common criteria for the management of this usually under-diagnosed disease.