Mounier-Kuhn syndrome: a rare cause of severe bronchial dilatation with normal pulmonary function test: a case report

Airway stenting and tracheobronchoplasty improve respiratory symptoms in Mounier-Kuhn syndrome

Mounier-Kuhn Syndrome (MKS) is a rare congenital disease that presents with abnormal enlargement in the central airways. In MKS, tracheomegaly is accompanied by difficulty in expelling recurrent lung infections and bronchiectasia. We presented a patient with MKS where commercially made stents were inadequate for stabilization and a custom-made, self-expandable metallic stent with a diameter of 28 mm and length of 100 mm was used. Chest pain that was thought to develop due to the stent and that disappeared after stent removal may be considered the main complication leading to stent removal. Continuous positive airway pressure therapy (CPAP) therapy was planned for the control of symptoms, which re-emerged after stent removal. This case is presented as an example that complications developing due to the stent as well as patient noncompliance may lead to stent removal, even when useful results are obtained from treatment of MKS.

/pdf/tracheomalacia-treatment-using-a-large-diameter-custom-made-2swj8bgkkj.pdf

Tracheomalacia treatment using a large-diameter, custom-made airway stent in a case with mounier-kuhn syndrome.

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.

/pdf/mounier-kuhn-syndrome-in-an-elderly-female-with-pulmonary-46rwv3nox1.pdf

Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis.

Resume La tracheobronchomegalie est une pathologie rare caracterisee par une dilatation de la trachee et des gros troncs bronchiques. Sa presentation clinique est non specifique. Sa confirmation diagnostique est radiologique, se basant surtout sur la tomodensitometrie thoracique. L’exploration fonctionnelle respiratoire au cours de cette pathologie est souvent anormale montrant un trouble ventilatoire obstructif avec augmentation du volume residuel. Nous rapportons le cas rare d’un patient âge de 31 ans ayant une tracheobronchomegalie et dont l’exploration fonctionnelle respiratoire etait normale.

Trachéobronchomégalie avec exploration fonctionnelle respiratoire normale : une association rare

Tracheobronchomegaly, also called Mounier-Kuhn syndrome, consists in dilatation of the trachea and major bronchi because of atrophy or absence of their elastic fibers and smooth muscle cells. Standard chest radiography often does not permit diagnosis because only lateral imaging, obtained with X-rays or chest CT scan, shows the true degree of tracheal dilatation. Surgery has no role in tracheomegaly, except for the complications of tracheal stenosis or pneumothorax. The present work reports cadaveric renal transplantation in a 43-year-old woman affected by end-stage renal disease and suffering from congenital tracheobronchomegaly diagnosed during the first decade of life. No surgical or anesthetic problems were encountered during the immediate perioperative period. The patient did not require pulmonary physiotherapy. Antibiotic prophylaxis was given for 10 days. No pulmonary infection developed, and the patient was discharged from the hospital asymptomatic with normal renal function at 25 days after the transplant. Four months later, the patient experienced bronchitis with cough and fever. Antibiotic therapy was performed with totally resolution of symptoms. At 8 months of follow-up after kidney transplantation, the patient is asymptomatic with normal renal function.

Renal Transplantation in a Patient Affected by Mounier-Kuhn Syndrome

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Renal Transplantation in a Patient Affected by Mounier-Kuhn Syndrome