ABSTRACT
RESUMEN La traqueobroncomalacia es una enfermedad de la vía aérea central caracterizada por una debilidad de la pared, con disminución dinámica de la luz de la tráquea y grandes bronquios principalmente durante la espiración. Genera síntomas crónicos que pueden evolucionar hasta la falla respiratoria grave, frecuentemente diagnosticados de forma errónea como asma o enfermedad pulmonar obstructiva crónica (EPOC). Presentamos el caso de una paciente femenina de 70 años, con antecedente de artritis reumatoide y múltiples internaciones por cuadros respiratorios infecciosos en los 3 años previos.
ABSTRACT Tracheobroncomalacia is a disease of the central airway due to weakness of the wall with dynamic narrowing of the lumen of the trachea and mainstem bronchi during exhalation. It produces chronic symptoms that can progress to severe respiratory failure, often misdiagnosed as asthma or chronic obstructive pulmonary disease (COPD). We report the case of a 70-year-old female patient with a history of rheumatoid arthritis and multiple hospitalizations for recurrent respiratory infections over the past 3 years.
Subject(s)
Humans , Female , Aged , Tracheobronchomalacia , Tracheomalacia , Respiratory Insufficiency , Asthma , Signs and Symptoms , Trachea , Bronchi , Exhalation , Tracheobronchomalacia/complications , FrailtyABSTRACT
Tracheobronchomalacia (TBM) is the most common tracheobronchial obstruction. Most cases are mild to moderate; therefore, they do not need surgical treatment. Severe tracheomalacia, however, represents a diagnostic and therapeutic challenge since they are very heterogeneous. In the armamentarium of resources for the treatment of dynamic airway collapse, splints and stents are two underused strategies and yet, they may represent the best alternative in selected cases. Lately, computed tomography 3D reconstruction of the airway has been used for the design of virtual models that can be 3D-printed for the creation of novel devices to address training, simulation, and biotechnological implants for refractory and severe airway malformations. This manuscript examines the role of resorbable stents, splints, and the 3D reconstruction and printing of the pediatric airway in tracheobronchomalacia.
Subject(s)
Airway Obstruction , Tracheobronchomalacia , Airway Obstruction/diagnostic imaging , Airway Obstruction/surgery , Child , Humans , Imaging, Three-Dimensional , Printing, Three-Dimensional , Splints , Stents , Tracheobronchomalacia/surgeryABSTRACT
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.
Subject(s)
Humans , Child , Tracheomalacia/diagnosis , Tracheomalacia/therapy , Signs and Symptoms , Bronchoscopy , Tracheomalacia/classificationABSTRACT
BACKGROUND: Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare disease characterized by dilation of the trachea and the main bronchi within the thoracic cavity. The predominant signs and symptoms of the disease include coughing, purulent and abundant expectoration, dyspnea, snoring, wheezing, and recurrent respiratory infection. Symptoms of the disease in some patients are believed to be pathological manifestations arising due to resident tracheobronchomalacia. Although treatment options used for the management of this disease include inhaled bronchodilators, corticosteroids, and hypertonic solution, there is no consensus on the treatment. The use of continuous positive airway pressure (CPAP) has been reported as a potential therapeutic option for tracheobronchomalacia, but no prospective studies have demonstrated its efficacy in this condition. OBJECTIVE: The purpose of this is to identify the presence of tracheobronchomalacia and an optimal CPAP pressure that reduces the tracheobronchial collapse in patients with Mounier-Kuhn syndrome and to analyze the repercussion in pulmonary ventilation. In parallel, we aim to evaluate the prevalence of obstructive sleep apnea/hypopnea syndrome. METHODS: This interventional, open-label, single-arm clinical trial will enroll patients who are diagnosed Mounier-Kuhn syndrome. Patient evaluation will be conducted in an outpatient clinic and involve 3 visits. Visit 1 will involve the collection and registration of social demographic, clinical, and functional data. Visit 2 will entail polysomnography, bronchoscopy for the evaluation of tracheobronchomalacia, titration of the optimal pressure that reduces the degree of collapse of the airway, and electrical impedance tomography. In visit 3, patients exhibiting a reduction in collapse areas will be requested to undergo chest computed tomography during inspiration and forced expiration with and without positive pressure (titrated to determine optimal CPAP pressure). RESULTS: This protocol is a doctorate project. The project was submitted to the institutional review board on January 24, 2017, and approval was granted on February 2, 2017 (Brazilian Research database number CAAE 64001317.4.000.0068). Patient evaluations started in April 2018. Planned recruitment is based on volunteers' availability and clinical stability, and interventions will be conducted at least once a month to finish the project at the end of 2020. A preliminary analysis of each case will be performed after each intervention, but detailed results are expected to be reported in the first quarter of 2021. CONCLUSIONS: There is no consensus on the best treatment options for managing Mounier-Kuhn syndrome. The use of positive pressure could maintain patency of the collapsed airways, functioning as a "pneumatic stent" to reduce the degree of airflow obstruction. This, in turn, could promote mobilization of thoracic secretion and improve pulmonary ventilation. TRIAL REGISTRATION: ClinicalTrails.gov NCT03101059; https://clinicaltrials.gov/ct2/show/NCT03101059. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/14786.
ABSTRACT
Resumen Las alteraciones dinámicas del sistema respiratorio que condicionan colapso del lumen traqueobronquial durante la espiración favorecen síntomas inespecíficos o no atribuibles a una enfermedad respiratoria en particular. El diagnóstico preciso de la entidad clínica tiene implicaciones terapéuticas y pronosticas. La traqueobroncomalacia se diagnostica cuando existe una disminución en el lumen traqueobronquial mayor al 50% visible mediante exploración con broncoscopía. La etiología más frecuente en adultos es la forma adquirida y es rara la asociación con atelectasia.
Abstract Respiratory dynamic abnormalities that contributes with collapse of the airway luminal during expiration can cause nonspecific symptoms or not related to a respiratory disease. Accurate diagnosis of the clinical entity has therapeutic implications and is important for the prognosis. Tracheobronchomalacia is diagnosed when there is a decrease in the lumen tracheobronchial greater than 50% visible through exploration with Bronchoscopy. The main etiology in adults was the acquired form and association with lung atelectasis was rare.
ABSTRACT
Tracheobronchomalacia refers to the presence of trachea and bronchi with soft, collapsible walls. Its incidence has been reported from 1:1500 to 1:2500. It can be congenital or acquired. This disease ranges from mild to life threatening. A high clinical suspicion is required for diagnosis, which needs to be confirmed endoscopically. Depending on severity, treatment can include from physical therapy and antibiotics to mechanical ventilation, surgery and tracheostomy, and is based on expert opinion and case series. Prognosis is usually good, with tendency to spontaneous resolution near the 2d year of life.
Traquebroncomalacia se refiere a la presencia de tráquea y/o bronquios cuya pared es blanda y tiene tendencia al colapso. Se ha reportado una incidencia entre 1:1.500 a 1:2.500. Puede ser congénita o adquirida. La severidad de los síntomas varía desde leve a incluso riesgo vital y muerte. El diagnóstico requiere una alta sospecha clínica y confirmación endoscópica. El tratamiento está basado en opiniones de expertos y series clínicas y varía según la severidad de los síntomas desde kinesioterapia y antibióticos en los casos más leves hasta ventilación mecánica, traqueostomía y cirugía en los más severos. El pronóstico en general es bueno con tendencia a la resolución espontánea aproximadamente a los 2 años de vida.
Subject(s)
Humans , Child , Tracheobronchomalacia/diagnosis , Tracheobronchomalacia/therapy , Evidence-Based Medicine , Prognosis , Signs and Symptoms , Tracheobronchomalacia/classification , Tracheobronchomalacia/epidemiologyABSTRACT
CONTEXT: Tracheobronchomalacia (TBM) results from structural and functional abnormalities of the respiratory system. It is characterized by excessive collapse: at least 50 percent of the cross-sectional area of the trachea and main bronchi. In this paper, we present a rare case of a patient with TBM who first presented with stridor and respiratory failure due to exacerbation of chronic bronchitis. CASE REPORT: An 81-year-old Caucasian man was admitted presenting coughing, purulent sputum, stridor and respiratory failure. He had a medical history of chronic obstructive pulmonary disease (COPD) and silicosis and was a former smoker. Axial computed tomography on the chest revealed marked collapse of the trachea in its middle third. Bronchoscopy showed characteristics compatible with TBM. He was treated with noninvasive ventilation, without any good response. Subsequently, a Dumon Y stent was placed by means of rigid bronchoscopy. After the procedure, he was discharged with a clinical improvement. CONCLUSION: TBM is fatal and often underdiagnosed. In COPD patients, stridor and respiratory failure may be helpful signs that should alert physicians to consider TBM as an early diagnosis. Thus, these signs may be important for optimizing the treatment and evolution of such patients.
CONTEXTO: Traqueobroncomalácia (TBM) é resultado de alterações funcionais e estruturais do aparelho respiratório. É caracterizada pelo colapso excessivo de pelo menos 50 por cento da área de secção transversal da traqueia e dos brônquios principais. Neste trabalho, descrevemos um raro caso de paciente com TBM que primeiro apresentou estridor e insuficiência respiratória devido à exacerbação da bronquite crônica. RELATO DE CASO: Homem de 81 anos de idade, caucasiano, foi admitido apresentando tosse, expectoração purulenta, estridor e falência respiratória. Ele apresentava história médica prévia de doença pulmonar obstrutiva crônica (DPOC), silicose e era ex-tabagista. A tomografia axial computadorizada de tórax revelou marcado colapso da traqueia em seu terço médio. A broncoscopia mostrou aspectos compatíveis com TBM. Foi submetido a ventilação não invasiva, sem boa resposta. Na sequência, foi colocado stent Dumon em Y por broncoscopia rígida. Após o procedimento, o paciente teve alta com melhora clínica. CONCLUSÃO: TBM é uma entidade fatal e muitas vezes subdiagnosticada. Em pacientes com DPOC, o estridor e a insuficiência respiratória podem ser sinais úteis que devem alertar os médicos a considerar TBM como diagnóstico precoce. Assim, pode ser importante para otimizar o tratamento e a evolução dos pacientes.