A rare congenital condition – Mounier-Kuhn syndrome – was identified as the cause of bronchiectasis in a man in India. The authors of the report suggest the condition might be a more common source of bronchiectasis than currently believed.
The study, “Tracheobronchomegaly as a Cause of Bronchiectasis in an Adult,“ was published by the journal Case Reports in Pulmonology.
Mounier-Kuhn syndrome, also known as tracheobronchomegaly, is a condition characterized by a dilated windpipe and main bronchi associated with frequent infections of the lower respiratory tract. The condition arises due to the absence of elastic fibers and smooth muscle cells in these parts of the airways. Clinicians may find it difficult to diagnose the condition because many other diseases cause similar symptoms. The syndrome can be brought on by an initial triggering event, such as infection or fibrosis development, and some groups of children and infants who were exposed to prolonged intubation or certain surgical procedures develop Mounier-Kuhn syndrome as a result.
But there’s a group of patients with no apparent cause underlying the condition, who often start to produce symptoms once they reach middle age. This was the case with the man described in the report, who sought care after 10 years of respiratory symptoms. He described having exacerbations of the respiratory symptoms several times a year.
His long history of symptoms alerted the doctors at the Jawaharlal Institute of Postgraduate Medical Education and Research, who performed a chest X-ray. Noting signs of what looked like bronchiectasis, the clinicians performed a high-resolution chest computed tomography (CT), which revealed a severely dilated windpipe and main bronchi. The windpipe also had an irregular inner wall, as well as bronchiectasis in one of the lungs. The features made the team suspect Mounier-Kuhn syndrome.
The patient was subjected to one further analysis – bronchoscopy – that, together with chest CT, is the standard method to confirm a diagnosis of Mounier-Kuhn syndrome. The bronchoscopy showed that in addition to the dilated airways, they also expanded when the man inhaled and collapsed almost completely when he coughed.
The symptoms of Mounier-Kuhn syndrome can range from mild nonspecific symptoms to respiratory failure. Typical symptoms are recurrent pneumonia, chronic productive cough that is occasionally bloody, and an increasing difficulty to breathe.
While there is no specific treatment for the syndrome, symptoms are usually managed with bronchodilators and antibiotics. The man in the case report is now treated with postural drainage and chest physiotherapy – techniques for removing excess mucus in the airways that are also frequently used to manage the syndrome.
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