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.

Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report.

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.