Abstract Introduction Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by marked tracheobronchial dilation and recurrent lower respiratory tract infections. Diagnosis is typically accomplished with the use of computed tomography and bronchoscopy, as well as pulmonary function tests. Patients may be asymptomatic; but they can present from recurrent pneumonia to pulmonary failure. Case We present a case of a 37-year-old male who presented to our institution with a 2-week history of productive cough, fever and hypoxia. He had prior hospitalizations for recurrent pneumonia without positive sputum cultures. Initial workup showed neutrophilia and left lung base airspace consolidation with multiple cysts on initial chest x-ray. Computed tomography of chest showed cystic bronchiectasis involving all lobes to variable degrees with surrounding pneumonia. Cystic Fibrosis was top in the differentials but was ruled out with additional testing. Upon investigation, he was found to have Mounier Kuhn’s Syndrome diagnosed in his teens at Children’s Hospital. He received an extensive course of antibiotics, steroids, and mucolytics. Subsequently, due to the extent of his disease, repeated infections despite treatment, the patient is currently on the transplant list. Discussion Mounier-Kuhn syndrome is characterized by a distinct tracheobronchial dilation that is due to atrophy of the muscular and elastic tissues in the trachea and main bronchial wall.To consider the diagnosis, the diameter of the trachea should be greater than 3 cm: this is usually measured 2 cm above the aortic arch. Other measurements that have been used to make the diagnosis include bronchial diameters of 20 or 24 mm (right), and 15 or 23 mm (left). Though Mounier Kuhn syndrome is rare, with only about 30 case reports in the literature, it should always be kept in the differential in a patient with recurrent pneumonias. The end goal is to prevent respiratory failure and lung transplant if warranted. This abstract is funded by: None
Haider et al. (Fri,) studied this question.