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.

Síndrome de Mounier Kuhn en una paciente de 78 años con fibrosis pulmonar / Mounier Kuhn syndrome in a 78-year-old patient with pulmonary fibrosis

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).

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.

A 54-Year-Old Man with Tracheomegaly, Tracheal Diverticulas and Bronchiectasis — Mounier-Kuhn Syndrome.

Mounier-Kuhn syndrome is a rare idiopathic clinical, radiological and bronchoscopic disorder characterised by abnormal dilatation of the tracheo-bronchial tree. The usual presentation is with recurrent lower respiratory tract infections. Herein, we report the case of an adult male who was diagnosed to have Mounier-Kuhn syndrome based on radiographic finding of a tracheal diameter of 45.5 mm on computed tomography and dynamic complete collapse of the tracheo-bronchial tree on forced expiration, observed during ronchoscopy.

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.

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.

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.