Suhas H. S1, Ketaki Utpat2
PRESENTATION OF CASE
Mounier-Kuhn Syndrome (MKS) is an unwonted disorder of obscure aetiology signalised by constellation of marked tracheal dilatation, tracheal diverticulum and associated bronchiectasis. Its pathogenesis is postulated to be enrooted in aberrant connective tissue proliferation. Chronic cough with sputum production, recurrent lower respiratory tract infections and spontaneous pneumothoraces trademark the clinical picture. Bronchiectasis is the customary pattern of lung involvement. However, pulmonary fibrosis and emphysema could also could be encountered infrequently. We hereby report a divergent presentation of this syndrome with exhibition of a combination of bronchiectasis and emphysema.
Keywords- Mounier-Kuhn Syndrome, Emphysema, Tracheobronchomegaly.
A 30-year-old man non-addict was symptomatic with complaints of cough with expectoration copious in amount with postural variation and dyspnoea on exertion since childhood with history of infective exacerbations 2 to 3 per year. There was past history of empirical antituberculous therapy intake for 3 months. There were no history of similar complaints in family. There was no history suggestive of malabsorption or history of consanguineous marriage in parents. On general examination, grade 2 clubbing was seen. There were no other features suggestive of connective tissue disorder. On respiratory system examination, there was presence of bilateral coarse crackles. Examination of other systems was within normal limits.
Chest x-ray was suggestive of bilateral cystic opacities with hyperlucent area in left lower zone (Figure 1). On HRCT (high resolution computerised tomography of thorax), thorax examination revealed the presence of bilateral cystic bronchiectasis with air fluid levels within and tracheobronchomegaly with diameter of trachea, right main bronchus and left main bronchus being 32.3 mm, 21.9 mm and 18.1 mm, respectively. There was presence of diffuse centrilobular emphysematous changes in both lungs along with presence of panlobular emphysema seen in left lower lobe. Expiratory scans revealed bilateral patchy air trapping (Figure 2a, 2b, 2c). Bronchoscopy was suggestive of tracheal diverticulum with increased collapsibility of the airway (Figure 3). Spirometry was suggestive of obstructive abnormality with poor bronchodilator reversibility. Alpha-1 antitrypsin was 240 mg/dL (100-300 mg/dL). Patient was managed with adequate chest physiotherapy for postural drainage of secretions and initiated on inhalers for airway involvement.