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Case Reports
. 2021 Nov 12:40:153.
doi: 10.11604/pamj.2021.40.153.31703. eCollection 2021.

Congenital tracheobronchomegaly (Mounier-Kuhn syndrome) in a 28-year-old Zambian male: a case report

Saifurrahman Shahin  1 , Thijs Hoffman  2 , Wouter van Es  3 , Jan Grutters  2   4 , Kondwelani Mateyo  1
Affiliations
Case Reports

Congenital tracheobronchomegaly (Mounier-Kuhn syndrome) in a 28-year-old Zambian male: a case report

Saifurrahman Shahin et al. Pan Afr Med J. .

Abstract

Congenital tracheobronchomegaly, also known as Mounier Kuhn Syndrome (MKS) is a rare respiratory disorder characterized by dilatation of the trachea and bronchi. We report a case of a 28-year-old male of African descent in Zambia, who presented with a history of chronic productive cough and repeated chest infections since childhood. He had been treated numerous times for lower respiratory tract infections, and had received empiric tuberculosis (TB) treatment based on chest radiograph findings, despite negative sputum microscopy and molecular tests for TB. Investigations revealed normal baseline blood results and sputum results. He however, had markedly increased levels of serum immunoglobulin E, and spirometry showed an obstructive pattern with significant post bronchodilator improvement. High-resolution computed tomography scan revealed tracheal dilatation, extensive bilateral bronchiectasis and tracheal and bronchial diverticula. The latter were also seen on bronchoscopy, confirming the diagnosis of Mounier-Kuhn syndrome. The patient was treated with combined inhaled corticosteroids and bronchodilators, as well as chest physiotherapy for mucus clearance, which led to improvement in his symptoms. Our case highlights how in low-resource settings, chronic lung diseases, particularly bronchiectasis, are often clinically and radiologically mistaken for and presumptively treated as TB (or its sequelae). Mounier-Kuhn syndrome, albeit rare, should be considered in the differential diagnosis of patients with recurrent lower respiratory tract infections or bronchiectasis. Multidisciplinary team meetings can help in the diagnosis of rare lung diseases.

Keywords: Case report; Mounier-Kuhn syndrome; Tracheobronchomegaly; Zambia; bronchiectasis; recurrent respiratory tract infections.

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Conflict of interest statement

The authors declare no competing interest.

Figures

Figure 1
Figure 1
(A,B) chest CT showing a dilated trachea (>3cm), dilated main bronchi, and cystic bronchiectasis, mainly in the lower lobes; C) bronchoscopy image showing a significantly dilated trachea and bronchi; D) tracheal diverticula
See this image and copyright information in PMC

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