Síndrome de Mounier-Kuhn / Mounier-Kuhn Syndrome

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)

Airway management of a patient incidentally diagnosed with Mounier-Kuhn syndrome during general anesthesia

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.

Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report / Broncoscopia flexível e ventilação mecânica no tratamento da síndrome de Mounier-Kuhn: relato de caso

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.

Traqueobroncomegalia: a propósito de un caso clínico-radiológico / Tracheobronchomegaly: a case report

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.

Mounier Kuhn syndrome in adult: a case report.

Mounier-Kuhn syndrome or tracheobronchomegaly is a rare congenital abnormality of the trachea and main bronchi characterized by marked cystic dilatation of the tracheobronchial tree, associated with tracheal diverticulosis, bronchiectasis and recurrent lower respiratory tract infections. We report a case of 22 years adult male with history of recurrent lower respiratory tract infection.

Infección respiratoria causada por Alcaligenes xylosoxidans en un paciente con síndrome de Mounier-Kuhn / Case report: respiratory infection due to Alcaligenes xylosoxidans in a patient with Mounier-Kuhn syndrome

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.

Tracheobronchomegaly with Bronchomalacia / 대한내과학회지

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.

Síndrome de Mounier-Kuhn: achados radiológicos e apresentação clínica / Mounier-Kuhn syndrome: radiological findings and clinical presentation

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.

Anesthetic management of a patient with Mounier-Kuhn syndrome undergoing off-pump coronary artery bypass graft surgery: A case report / 대한마취과학회지

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.

Anesthetic experience of a patient with tracheomegaly: A case report / 대한마취과학회지

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.

Anesthetic experience of a patient with tracheomegaly: A case report / 대한마취과학회지

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.

Traqueomegalia y artririts reumatoide / Traqueomegalia and rheumatoid arthritis

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.

A Case of Tracheobronchomegaly with Pneumonia / 결핵및호흡기질환

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.