Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis.
Panagiotis BoglouNikoloas PapanasAnastasia OikonomouStamatia BakaliPaschalis SteiropoulosPublished in: Case reports in medicine (2016)
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.
Keyphrases
- pulmonary fibrosis
- middle aged
- respiratory tract
- computed tomography
- case report
- lung function
- respiratory failure
- cystic fibrosis
- dual energy
- image quality
- high resolution
- end stage renal disease
- contrast enhanced
- positron emission tomography
- community dwelling
- ejection fraction
- newly diagnosed
- magnetic resonance imaging
- chronic obstructive pulmonary disease
- chronic kidney disease
- extracorporeal membrane oxygenation
- mass spectrometry
- peritoneal dialysis
- mycobacterium tuberculosis
- mechanical ventilation
- genome wide
- polycystic ovary syndrome
- gene expression
- skeletal muscle
- pulmonary tuberculosis
- single cell
- intensive care unit
- sleep quality
- metabolic syndrome
- depressive symptoms
- dna methylation
- patient reported outcomes
- tandem mass spectrometry
- adipose tissue