Mounier-Kuhn syndrome: A variable course disease

Abstract

Mounier-Kuhn syndrome or tracheobronchomegaly is a rare disease characterized by marked dilation of the trachea and proximal bronchi with recurrent lower tract respiratory infections. Computed tomography and bronchoscopy are the key tools to accomplish the diagnosis. This is a condition with a clinical polymorphism, symptoms vary from minor with preserved respiratory function, to very severe with life threatening exacerbations leading to respiratory failure and premature death. The treatment is mainly symptomatic, stenting or surgery are reserved to extreme cases.Herein, we report two cases of the same condition with different clinical signs and diverse outcome.

Tracheobronchomegaly (Mounier-Kuhn Syndrome) with CT and bronchoscopic correlation: A case report

“…Smoking cessation is highly beneficial. Exposure to industrial and occupational irritants and pollutants should be minimized [30] . Bronchial dilation may require endoscopic treatment with a stent or surgical tracheobronchoplasty in severe cases [31] .…”

Unexpected curious cause of serious air leakage after endotracheal intubation: A case report of tracheobronchomegaly and literature review

“…Except for congenital TBM, some other clinical conditions were also associated with secondary airway enlargement; thus, the change of this patient was suspected acquired because of no congenital respiratory disease but ankylosing spondylitis. Although clinical presentations of TBM are not specific, recurrent pulmonary infection is the most common symptom ( 6 , 8 , 9 ). This is the reason why most cases of TBM were reported with the presentation of pulmonary diseases.…”

“…Because of its rare presentation and clinical polymorphism, there is a lack of enough attention to TBM. There were fewer than 400 cases reported outside China and just fewer than 30 cases reported in China ( 6 , 7 ). We presented a scoliosis case with a postoperative diagnosis of TBM who encountered unexpected severe per-cuff leakage after tracheal intubation and mechanical ventilation.…”