RESUMEN
Mounier-Kuhn Syndrome (MKS) is a rare disorder derived from the muscular and elastic tissue defects of the trachea and the main bronchial walls, characterized by tracheobronchomegaly. Patients may present with complaints of cough, phlegm, dyspnoea and haemoptysis. Haemoptysis may be minor and mixed with phlegm or it may be massive. Establishment of airway patency is a priority in the management of massive haemoptysis. Cold saline solution, diluted adrenaline or tranexamic acid may be administered via the endobronchial route to stop haemorrhage while establishing the airway patency. Ankaferd Blood Stopper (ABS) has a haemostatic property and can be locally administered to the airway. In this report, we aim to highlight the effects of ABS administered via an endobronchial route for emergency palliation of a patient with MKS presenting with massive haemoptysis.
Asunto(s)
Broncoscopía , Hemoptisis/terapia , Hemostasis Endoscópica , Hemostáticos/uso terapéutico , Extractos Vegetales/uso terapéutico , Traqueobroncomegalia/diagnóstico por imagen , Adulto , Antifibrinolíticos/uso terapéutico , Crioterapia , Epinefrina/uso terapéutico , Hemoptisis/etiología , Humanos , Masculino , Tomografía Computarizada por Rayos X , Traqueobroncomegalia/complicaciones , Ácido Tranexámico/uso terapéutico , Insuficiencia del Tratamiento , Resultado del Tratamiento , Vasoconstrictores/uso terapéuticoRESUMEN
Mounier-Kuhn syndrome is a rare condition that combines tracheobronchomegaly (TBM) and severe tracheobronchomalacia. Symptoms can be severe with recurrent bronchopulmonary infections and cough-induced syncope. Therapeutic management is non-specific and limited to chest physiotherapy and antibiotics during infectious exacerbations. We report a case of Mounier-Kuhn syndrome that was successfully managed by treating the posterior collapse of the central airway with yttrium aluminum pevroskyte laser. Endoscopic aspects, respiratory symptoms, and lung function tests all improved and remained stable with a follow-up of 8 years. Laser, at low power settings, could be a new therapeutic option in selected cases of tracheobronchomalacia.
Asunto(s)
Láseres de Estado Sólido/uso terapéutico , Terapia por Luz de Baja Intensidad/métodos , Traqueobroncomalacia/radioterapia , Traqueobroncomegalia/radioterapia , Anciano , Broncoscopía/métodos , Femenino , Estudios de Seguimiento , Humanos , Recurrencia , Infecciones del Sistema Respiratorio/etiología , Traqueobroncomalacia/complicaciones , Traqueobroncomegalia/complicaciones , Resultado del TratamientoRESUMEN
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.
Asunto(s)
Humanos , Masculino , Adulto , Neumonía/etiología , Traqueobroncomegalia/complicaciones , Antibacterianos/uso terapéutico , Ejercicios Respiratorios , Neumonía/tratamiento farmacológico , Tomografía Computarizada por Rayos X , Traqueobroncomegalia/diagnóstico , Traqueobroncomegalia/rehabilitaciónRESUMEN
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.