ABSTRACT
El síndrome de Mounier Kühn es una patología infrecuente de la vía aérea, caracterizada por una dilatación anormal de tráquea y bronquios. Se debe sospechar ante la presencia de infecciones broncopulmonares recurrentes e irritación traqueobronquial. El diagnóstico se lleva a cabo a través de la medición del diámetro traqueal en tres segmentos de su anatomía, a través de tomografía torácica. Se presenta el caso de un paciente masculino escolar de 10 años de edad, con antecedentes de infecciones respiratorias recurrentes, hospitalizado por un cuadro neumónico; los hallazgos reportados en la tomografía de tórax corresponden a traqueobroncomegalia además de incremento del diámetro esofágico. El tratamiento de esta entidad es sintomático con medidas de sostén y fisioterapia pulmonar.
Mounier Kühn syndrome is a rare airway pathology characterized by abnormal dilatation of the trachea and bronchi. It should be suspected in the presence of recurrent bronchopulmonary infections and tracheobronchial irritation. The diagnosis is made by measuring the tracheal diameter in three segments of its anatomy, through thoracic tomography. We present the case of a 10 year-old male school boy with a history of recurrent respiratory infections, hospitalized for a pneumonic condition; the findings reported in the thoracic tomography correspond to a tracheobronchomegaly in addition to an increase of the esophageal diameter. The treatment of this entity is symptomatic with supportive measures and pulmonary physical therapy.
A síndrome de Mounier Kühn é uma patologia incomumdas vias aéreas, caracterizada por dilatação anormal da traqueia e brônquios. Devese suspeitar na presença de infecções broncopulmonares recorrentes e irritação traqueobrônquica. O diagnóstico é realizado através da medida do diâmetro traqueal em três segmentos de sua anatomia, através da tomografia de tórax. Apresentamos o caso de um paciente escolar de 10 anos de idade, do sexo masculino, com história de infecções respiratórias de repetição, internado por sintomas pneumônicos; os achados relatados na tomografia de tórax correspondem a uma traqueobroncomegalia, além de aumento do diâmetro esofágico. O tratamento dessa entidade é sintomático com medidas de suporte e fisioterapia pulmonar.
Subject(s)
Humans , Male , Child , Tracheobronchomegaly/diagnostic imaging , Respiratory Tract Infections/etiology , Tracheobronchomegaly/complications , Tracheobronchomegaly/therapy , Reinfection/etiologyABSTRACT
El síndrome de Mounier-Kuhn o traqueobroncomegalia constituye una rara entidad clínica y radiológica caracterizada por una dilatación de la tráquea y los bronquios principales. Puede asociarse con estructuras saculares en las membranas intercartilaginosas (diverticulosis traqueal), bronquiectasias e infecciones respiratorias a repetición. Las formas de presentación clínica son variables. Tiene una prevalencia entre el 1-4,5 por ciento de la población y se presenta, mayoritariamente, en hombres con un amplio rango etario que oscila entre los 20 y 50 años de edad. Se presenta el caso de un paciente masculino de 77 años con cuadros de infecciones respiratorias bajas a repetición desde la juventud. Luego de realizarle estudios de imagen y broncoscopia, se llegó al diagnóstico de síndrome de Mounier-Kunh. Recibió tratamiento antibiótico inmediatamente y su evolución fue de manera satisfactoria(AU)
Mounier-Kuhn syndrome or tracheobroncomegaly is a rare clinical and radiological entity characterized by dilatation of the trachea and the main bronchi. It can be associated with saccular structures in the intercartilaginous membranes (tracheal diverticulosis), bronchiectasis, and recurrent respiratory infections. The forms of clinical presentation vary. It has a prevalence between 1-4.5 percent of the population and it occurs mainly in men, widely ranging ages between 20 and 50 years of age. We report the case of a 77-year-old male patient with recurrent lower respiratory infections since his youth. After performing imaging studies and bronchoscopy, he could be diagnosed with Mounier-Kunh syndrome. He immediately underwent antibiotic treatment and his evolution was satisfactory(AU)
Subject(s)
Humans , Male , Aged , Cephalexin/therapeutic use , Tracheobronchomegaly/epidemiology , Azithromycin/therapeutic use , Tomography, Spiral Computed/methods , Tracheobronchomegaly/drug therapyABSTRACT
Mounier-Kuhn syndrome (MKS) is a disease characterized by dilation of the trachea and mainstem bronchi. Due to the risk of airway leakage, pulmonary aspiration, and tracheal damage, MKS can be fatal in patients undergoing tracheal intubation. Moreover, MKS may not be diagnosed preoperatively due to its rarity. In this case, a patient undergoing neurosurgery was incidentally diagnosed with MKS during general anesthesia. During anesthesia induction, difficulties in airway management led the anesthesiologist to suspect MKS. Airway leakage was resolved in this case using oropharyngeal gauze packing. Anesthesiologists should be aware of the possibility of MKS and appropriate management of the airways.
Subject(s)
Humans , Airway Management , Anesthesia , Anesthesia, General , Bronchi , Intubation , Intubation, Intratracheal , Neurosurgery , Trachea , TracheobronchomegalyABSTRACT
ABSTRACT CONTEXT: Mounier-Kuhn syndrome is a rare congenital condition with distinct dilatation and diverticulation of the tracheal wall. The symptoms may vary and the treatment usually consists of support. CASE REPORT: The patient was a 60-year-old male with recurrent hospital admission. He was admitted in this case due to dyspnea, cough and sputum production. An arterial blood sample revealed decompensated respiratory acidosis with moderate hypoxemia. A chest computed tomography (CT) scan showed dilatation of the trachea and bronchi, tracheal diverticula and bronchiectasis. Flexible bronchoscopy was performed, which revealed enlarged airways with expiratory collapse. Furthermore, orifices of tracheal diverticulosis were also detected. Non-invasive positive pressure ventilation (NPPV) was added, along with long-term oxygen therapy. At control visits, the patient's clinical and laboratory findings were found to have improved. CONCLUSION: Flexible bronchoscopy can be advocated for establishing the diagnosis and non-invasive mechanical ventilation can be used with a high success rate, for clinical wellbeing in Mounier-Kuhn syndrome.
RESUMO CONTEXTO: A síndrome de Mounier-Kuhn é uma condição congênita rara com dilatação e diverticulação distintas da parede traqueal. Os sintomas podem ser variáveis e o tratamento geralmente é de suporte. RELATO DE CASO: Paciente do sexo masculino, de 60 anos, com internação hospitalar recorrente, foi internado neste caso devido a dispneia, tosse e produção de expectoração. A amostra de sangue arterial revelou acidose respiratória descompensada, com hipoxemia moderada. A tomografia computadorizada de tórax mostrou dilatação da traqueia e brônquios, divertículos traqueais e bronquiectasias. Realizou-se broncoscopia flexível, que revelou aumento das vias aéreas com colapso expiratório. Além disso, também foram detectados orifícios de diverticulose traqueal. Foi adicionada ventilação com pressão positiva não invasiva (NPPV) juntamente com a oxigenoterapia a longo prazo. Foram verificadas melhoras dos resultados clínicos e laboratoriais do doente nas visitas de controle. CONCLUSÃO: A broncoscopia flexível pode ser defendida para estabelecer o diagnóstico, e a ventilação mecânica não invasiva pode ser utilizada com alta taxa de sucesso, para bem-estar clínico, na síndrome de Mounier-Kuhn.
Subject(s)
Humans , Male , Middle Aged , Bronchoscopy/methods , Tracheobronchomegaly/therapy , Positive-Pressure Respiration/methods , Diverticulum/therapy , Noninvasive Ventilation/methods , Oxygen Inhalation Therapy/methods , Tomography, X-Ray Computed , Tracheobronchomegaly/diagnostic imaging , Diverticulum/diagnostic imagingABSTRACT
Resumen Se presenta el caso de una mujer de 78 años de edad con fibrosis pulmonar idiopàtica, quien consultó por exacerbación de sus síntomas respiratorios, a quien se le realizan estudios radiológicos, donde se evidencia dilatación de la vía aérea, previamente no descrita, y se realiza diagnóstico de síndrome de Mounier Kuhn. Hasta donde el conocimiento alcanza, es el primer caso reportado en Colombia y la tercera persona de mayor edad reportada en el mundo. Este es un hallazgo incidental, dado que desde el punto de vista fisiopatológico no hay ninguna relación causal entre la fibrosis pulmonar idiopática y el síndrome de Mounier Kuhn. Sin embargo, su presencia puedo afectar aún más el compromiso funcional, dado el eventual colapso de la vía aérea, lo cual puede llevar a intervenciones terapéuticas adicionales, como colocación de stent. (Acta Med Colomb 2017: 42-198-201).
Abstract The case of a 78-year-old woman with idiopathic pulmonary fibrosis who consulted for exacerbation of her respiratory symptoms is presented. Radiologic studies showed dilation of the airway that was not previously described, and for this reason the diagnosis of Mounier Kuhn Syndrome was made. To our knowledge, this is the first case reported in Colombia and the third oldest person reported in the world. This is an incidental finding, since from the pathophysiological point of view there is no causal relationship between idiopathic pulmonary fibrosis and Mounier Kuhn's syndrome. However, its presence may further affect the functional compromise, given the eventual collapse of the airway, which may lead to additional therapeutic interventions, such as stent placement. (Acta Med Colomb 2017: 42-198-201).
Subject(s)
Humans , Female , Aged , Tracheobronchomegaly , Pulmonary Fibrosis , TracheomalaciaABSTRACT
Tracheobronchomegaly corresponds to the anomalous diffuse dilatation of the trachea and main bronchi, usually accompanied by bronchiectasis, which predisposes to recurrent infections. The imaging study is essential to recognize this entity. A case of a 40-year-old woman, with suspicion of bronchial asthma is presented. Imaging study and bronchofiberscopy confirmed the diagnosis of tracheobronchomegaly in this patient.
La traqueobroncomegalia corresponde a la dilatación anómala y difusa de la tráquea y bronquios principales, acompañado habitualmente de bronquiectasias, lo que predispone a infecciones recurrentes. El estudio radiológico resulta fundamental para reconocer esta entidad. Se presenta un caso de una mujer de 40 años en estudio por sospecha de asma bronquial en que el estudio de imágenes (Rx y TAC) y lafibrobroncoscopia confirmó el diagnóstico de traqueobroncomegalia.
Subject(s)
Humans , Female , Adult , Tracheobronchomegaly/pathology , Tracheobronchomegaly/diagnostic imaging , Biopsy , Bronchiectasis , Tomography, X-Ray Computed/methods , Histological TechniquesABSTRACT
Mounier-Kuhn syndrome is a rare entity characterized by abnormal dilatation of the trachea and main bronchi (tracheobronchomegaly). Alcaligenes xylosoxidans is a non fermenting gram-negative pathogen common in extra-and intra-hospital environment, which may be related to immunosuppression states. We describe the case of a 75 years old male, ex-smoker with moderate functional obstruction, chronic respiratory failure and chronic colonization by Pseudomonas aeuriginosa. He had an infectious exacerbation of his disease, reason that previously required several hospital admissions. The patient was treated with antibiotics and his evolution was favourable with negativization in cultures of the pathogen. This is the first description of the isolation of Alcaligenes xylosoxidans as a cause of respiratory infection in a patient with Mounier-Kuhn syndrome.
Subject(s)
Aged , Humans , Male , Alcaligenes/isolation & purification , Gram-Negative Bacterial Infections/microbiology , Respiratory Tract Infections/microbiology , Tracheobronchomegaly/complications , Gram-Negative Bacterial Infections/complications , Respiratory Tract Infections/complicationsABSTRACT
Tracheobronchomegaly, or Mounier-Kuhn syndrome, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilatation and recurrent lower respiratory tract infections. It is thought to be due to a congenital anomaly. A diagnosis is typically made using computed tomography (CT) and bronchoscopy. Symptoms can range from minimal with preserved lung function to severe respiratory failure. Here, we present the case of a 50-year-old male who was referred to our hospital for chronic cough, sputum, and dyspnea. In addition, the patient showed tracheobronchomegaly with bronchomalacia on CT and bronchoscopy. This report describes a rare case of Mounier-Kuhn syndrome with a literature review.
Subject(s)
Humans , Male , Middle Aged , Bronchomalacia , Bronchoscopy , Cough , Dilatation , Dyspnea , Lung , Respiratory Insufficiency , Respiratory Tract Infections , Sputum , TracheobronchomegalyABSTRACT
A síndrome de Mounier-Kuhn é uma doença rara, caracterizada clinicamente por infecções respiratórias de repetição. Apresentamos um relato de caso desta doença com análise da radiografia e da tomografia computadorizada de alta resolução do tórax que mostram como principais alterações aumento do calibre da traqueia, brônquios principais e bronquiectasias centrais. Estas alterações, associadas às informações clínicas, sugerem o diagnóstico.
Mounier-Kuhn syndrome is a rare disease clinically characterized by recurrent respiratory infections. The present report describes a case of this disease with analysis of chest radiography and high resolution computed tomography showing increased caliber of the trachea, main bronchi and central bronchiectasis. Such changes, in association with clinical data, suggest the diagnosis.
Subject(s)
Humans , Male , Adult , Lung Diseases, Interstitial , Lung, Hyperlucent , Tracheal Diseases , Tracheobronchomegaly , Atrophy , Radiography, Thoracic , Respiratory Tract Infections , Tomography, X-Ray ComputedABSTRACT
Mounier-Kuhn-syndrome patients have markedly dilated trachea and main bronchi due to an atrophy or absence of elastic fibers and thinning of smooth muscle layers in the tracheobronchial tree. Although this syndrome is rare, airway management is challenging and general anesthesia may produce fatal results. However, only a few cases have been reported and this condition is not widely known among anesthesiologists. We present the case of a tracheobronchomegaly patient undergoing an emergency off-pump coronary artery bypass. Although the trachea was markedly dilated with numerous tracheal diverticuli, there was an undilated 2 cm portion below the vocal cords found on the preoperative CT. Under a preparation of extracorporeal membrane oxygenation, we intubated and placed the balloon of an endotracheal tube (I.D. 9 mm) at this portion, and maintained ventilation during the operation. This case showed that a precise preoperative evaluation and anesthetic plan is essential for successful anesthetic management.
Subject(s)
Humans , Airway Management , Anesthesia, General , Atrophy , Bronchi , Coronary Artery Bypass, Off-Pump , Elastic Tissue , Emergencies , Extracorporeal Membrane Oxygenation , Muscle, Smooth , Trachea , Tracheobronchomegaly , Transplants , Ventilation , Vocal CordsABSTRACT
Tracheomegaly or tracheobronchomegaly is a rare syndrome that consists of marked dilatation of the trachea and the major bronchi, and this is usually due to a congenital defect of the elastic and muscle fibers of the tracheobroncheal tree. Physicians have had only limited experience with performing anesthesia in patients with this type of syndrome. This syndorme is diagnosed by roentenological investigation and this condition is frequently associated with chronic respiratory infection and partial airway obstruction. In this report, we present a case of performing tracheostomy for a patient with tracheomegaly, and this was probably secondary to mechanical ventilator therapy. The regular tracheostomy tube did not provided sufficient length to allow the cuff to lie properly in the trachea in this patient. Because of the peri-cuff air leakgae and hypercapnea after tracheostomy, we needed a longer tracheostomy tube. But we didn't have such a tube and we didn't know any other method, so we couldn't perform tracheostomy. Therefore, we introduced a method of reducing the length of the endotracheal tube to a suitable size until a longer tracheostomy tube can be obtained for those patients having tracheomegaly.
Subject(s)
Humans , Airway Obstruction , Anesthesia , Bronchi , Congenital Abnormalities , Dilatation , Muscles , Trachea , Tracheobronchomegaly , Tracheostomy , Ventilators, MechanicalABSTRACT
Tracheomegaly or tracheobronchomegaly is a rare syndrome that consists of marked dilatation of the trachea and the major bronchi, and this is usually due to a congenital defect of the elastic and muscle fibers of the tracheobroncheal tree. Physicians have had only limited experience with performing anesthesia in patients with this type of syndrome. This syndorme is diagnosed by roentenological investigation and this condition is frequently associated with chronic respiratory infection and partial airway obstruction. In this report, we present a case of performing tracheostomy for a patient with tracheomegaly, and this was probably secondary to mechanical ventilator therapy. The regular tracheostomy tube did not provided sufficient length to allow the cuff to lie properly in the trachea in this patient. Because of the peri-cuff air leakgae and hypercapnea after tracheostomy, we needed a longer tracheostomy tube. But we didn't have such a tube and we didn't know any other method, so we couldn't perform tracheostomy. Therefore, we introduced a method of reducing the length of the endotracheal tube to a suitable size until a longer tracheostomy tube can be obtained for those patients having tracheomegaly.
Subject(s)
Humans , Airway Obstruction , Anesthesia , Bronchi , Congenital Abnormalities , Dilatation , Muscles , Trachea , Tracheobronchomegaly , Tracheostomy , Ventilators, MechanicalABSTRACT
La traqueo-broncomegalia es una rara condición de etiología desconocida que ha sido descrita en asociación con enfermedades del tejido conectivo ocasionalmente. Presentamos un caso de traqueomegalia en una paciente con artritis reumatoide de larga evolución. Este es el segundo caso reportado en la literatura médica hasta ahora. La asociación entre estas patologías es incierta y no se puede establecer una clara relación fisiopatológica debido a la rareza de su ocurrencia y el inicio tardío de los síntomas.
Tracheobronchomegaly is a rare condition of unknown etiology that has been described in association with connective tissue diseases. We present a case of tracheomegaly in a patient with a long evolution rheumatoid arthritis. This is the second case reported in the medical literature until now. Association between these pathologies is uncertain and we can not establish a clear pathophysiological link due to the rarity of its occurrence and the late onset of symptoms.
Subject(s)
Humans , Female , Aged , Arthritis, Rheumatoid , Association , Tracheobronchomegaly , Pathology , Signs and Symptoms , DiseaseABSTRACT
A síndrome de Mounier-Kühn, ou traqueobroncomegalia congênita, é uma entidade clínica rara caracterizada pela dilatação anormal de traquéia e brônquios principais. O diagnóstico geralmente pode ser realizado através da mensuração do diâmetro traqueal. Os autores apresentam o caso de um homem de 40 anos com pneumonia refratária ao tratamento, no qual a traqueobroncomegalia foi confirmada através de tomografia computadorizada.
Subject(s)
Humans , Male , Adult , Pneumonia/etiology , Tracheobronchomegaly/complications , Anti-Bacterial Agents/therapeutic use , Breathing Exercises , Pneumonia/drug therapy , Tomography, X-Ray Computed , Tracheobronchomegaly/diagnosis , Tracheobronchomegaly/rehabilitationABSTRACT
A 66-years-old man was refered to our hospital because of cough, sputum, chill and fever. Enlargement of the trachea and main bronchi on radiography and bronchoscopy is compatible with Mounier-Kuhn syndrome. Mounier-Kuhn syndrome or tracheobronchomegaly is a rare disorder of uncertain etiology, characterized by marked dilatation of the trachea and major bronchi. This syndrome is associated with tracheal diverticulosis, bronchiectasis and recurrent respiratory tract infection. We report a rare case of Mounier-Kuhn syndrome with pneumonia and literature reviews.
Subject(s)
Bronchi , Bronchiectasis , Bronchoscopy , Cough , Dilatation , Diverticulum , Fever , Pneumonia , Radiography , Respiratory Tract Infections , Sputum , Trachea , TracheobronchomegalyABSTRACT
Tracheobronchomegaly (TBM) occasionally may progress to extensive tracheomalacia which leads to respiratory failure. Spirometry, dynamic expiratory multidetector computed tomography (CT), bronchoscopy are used to diagnose patients of suspected tracheobronchomalacia. We used the technique of night-time monitoring of respiratory variables to show the presence of respiratory abnormalities during sleep and which was corrected by applying nasal continuous positive airway pressure (CPAP). The study showed the presence of both apnoea and hypopnoeas, which were obstructive in nature with an apnoea-hypopnoea index (AHI) of 11, no snoring and associated oxygen desaturation of 75 per cent. A second overnight study with nasal continuous positive airway pressure at a critical pressure of 8 cm, the AHI decreased to 3 along with no drop in oxygen saturation. This non-invasive technique should be considered as a diagnostic tool in tracheobronchomalacia and to know the outcome of CPAP, surgical or stent therapy in this condition.