SESSION TYPE: Airway Student/Resident Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Mounier-Kuhn’s syndrome is a rare congenital abnormality characterized by recurrent infections and productive cough. Often times it presents similarly to more common pulmonary disorders such as chronic obstructive pulmonary disease (COPD) and asthma, and the diagnosis may be missed. We report a case of Mounier-Kuhn syndrome or Tracheobronchomegaly in an ex-smoker thought to have COPD, but further work-up revealed otherwise.
CASE PRESENTATION: This is a case report of 60 years-old male who presented to the pulmonary clinic for difficult to treat COPD. He had been seen by his primary care physician for COPD and stated that he had minimal improvement in symptoms with conventional COPD therapy. He had been hospitalized several times for COPD exacerbations without significant findings on plain film chest x-ray. Patient has a 30 pack year smoking history and had quit 20 years ago. He is not actively smoking. Physical exam showed mild digital clubbing, but otherwise unremarkable. Spirometry showed and FEV1/FVC ratio of 80% and an FEV1 of 2.57 Liters (97%) and computed tomography (CT) showed tracheobronchomegaly with the trachea measuring 49.2 mm in largest diameter, collapsed middle lobe and right middle lobe bronchus.
DISCUSSION: Mounier-Kuhn’s syndrome is an uncommon pulmonary disorder caused of Tracheobrochomegaly and dilation of the tracheobronchial tree. The larger trachea can lead to mucus collection in the airways resulting airways collapse and chronic infection. The etiology of Mouner-Kuhn’s disease is unknown and dilation is due to atrophy of the muscular and elastic tissues and it is more common in men in the 3rd and 4th decades of life. It is unclear at this time is smoking exacerbates the disease. Diagnosis is made by CT and treatment is mainly supportive. Clinically, patients may present with cough and recurrent lung infections similarly to COPD.
CONCLUSIONS: This patient was diagnosed with COPD secondary to his clinical history of smoking, and not necessarily his spirometry or CT findings. A strong index of suspicion may have prompted a CT scan earlier in his clinical presentation and an earlier diagnosis.
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DISCLOSURE: The following authors have nothing to disclose: Nayle Araguez Ancares, Noeen Ahmad, Pablo Loarte, Say Salomon, Yanely Pineiro-Puebla
No Product/Research Disclosure InformationWoodhull Medical Center, Brooklyn, NY