Infecciones respiratorias recurrentes en paciente escolar, asociadas a Síndrome de Mounier-Khün: reporte de caso / Recurrent respiratory infections associated with Mounier-Khün Syndrome in a school patient: clinical case report / Infecções respiratórias recorrentes associadas à Síndrome de Mounier-Khün num paciente escolar: relato do caso

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.

Thoracic Computed Tomography Scan and Bronchoscopy Appearance of Mounier-Kuhn Syndrome: A Case Report.

Mounier-Kuhn syndrome (MKS) is a rare congenital disease with an autosomal recessive inheritance pattern, characterized by an enlargement of the trachea and bronchi. MKS is secondary to a thinning of the muscular mucosa and atrophy of the longitudinal muscle and elastic fibers of the tracheobronchial tree. As a consequence, tracheal diverticulosis and dilatations in the posterior membranous wall appear, along with bronchiectasis that tend to be cystic in appearance. Overall, there is an impairment of mucocilliary clearance, with an ineffective cough, which predisposes the patient to recurrent lower respiratory tract infections. Clinical manifestations vary from asymptomatic to respiratory failure and death, most patients being diagnosed between the third and fourth decades of life. It is an often undiagnosed disease, with a diagnostic algorithm that includes the use of radiological techniques, alone or in combination with bronchoscopy. Specific diagnostic criteria have been developed, based on patients' tracheal and main bronchi diameter on chest X-ray and thoracic computed tomography scan. We present the case of a 45-year-old African American man who presented with a history of multiples episodes of pneumonia that required management in the intensive care unit, on whom MKS was diagnosed.

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.

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

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.

Síndrome de Mounier-Kühn / Mounier-Kuhn syndrome

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.

Mounier-Kuhn syndrome.

Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical entity characterized by abnormal dilation of the trachea and main bronchi. The diagnosis can usually be made by measuring the tracheal diameter. We report the case of a 40-year-old black man with refractory lower respiratory tract infection. Tracheobronchomegaly was confirmed through computed tomography.