Abstract
Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT). Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs) typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects.
Highlights
Mounier-Kuhn syndrome (MKS) is an infrequent congenital syndrome, whose hallmark is airway enlargement
Its diagnosis is based on chest radiograph and chest computed tomography (CT)
The patient refused to undergo any further examination. This is the oldest female patient at presentation of MKS hitherto described. This condition is typically encountered in middle-aged men [3,4,5, 11,12,13] our patient was an elderly female, which emphasises that MKS needs to be considered in older subjects as well
Summary
Mounier-Kuhn syndrome (MKS) is an infrequent congenital syndrome, whose hallmark is airway enlargement. Absence or atrophy of the elastic fibres within the tracheal wall is typically found [1,2,3,4,5,6,7]. This condition results in airway dilatation in the trachea and bronchi. The typical features of this entity were first described by Mounier-Kuhn in 1932 [9]. This report presents a female patient, the oldest hitherto described, with MKS who had been previously diagnosed with pulmonary fibrosis
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