Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome

a case report

Authors

  • Aslihan Gürün Kaya Ankara University School of Medicine
  • Aydin Çiledağ Ankara University School of Medicine
  • Çetin Atasoy Ankara University School of Medicine
  • Demet Karnak Ankara University School of Medicine

Keywords:

Bronchoscopy, Respiration, artificial, Optical fibers, Tracheobronchomegaly, Positive-pressure respiration

Abstract

CONTEXT: Mounier-Kuhn syndrome is a rare congenital condition with distinct dilatation and diverticulation of the tracheal wall. The symptoms may vary and the treatment usually consists of support. CASE REPORT: The patient was a 60-year-old male with recurrent hospital admission. He was admitted in this case due to dyspnea, cough and sputum production. An arterial blood sample revealed decompensated respiratory acidosis with moderate hypoxemia. A chest computed tomography (CT) scan showed dilatation of the trachea and bronchi, tracheal diverticula and bronchiectasis. Flexible bronchoscopy was performed, which revealed enlarged airways with expiratory collapse. Furthermore, orifices of tracheal diverticulosis were also detected. Non-invasive positive pressure ventilation (NPPV) was added, along with long-term oxygen therapy. At control visits, the patient’s clinical and laboratory findings were found to have improved. CONCLUSION: Flexible bronchoscopy can be advocated for establishing the diagnosis and non-invasive mechanical ventilation can be used with a high success rate, for clinical wellbeing in Mounier-Kuhn syndrome.

Downloads

Download data is not yet available.

Author Biographies

Aslihan Gürün Kaya, Ankara University School of Medicine

MD. Professor, Department of Chest Diseases, Ankara University School of Medicine, Ankara, Turkey.

Aydin Çiledağ, Ankara University School of Medicine

MD. Professor, Department of Chest Diseases, Ankara University School of Medicine, Ankara, Turkey.

Çetin Atasoy, Ankara University School of Medicine

MD. Professor, Department of Radiology, Ankara University School of Medicine, Ankara, Turkey.

Demet Karnak, Ankara University School of Medicine

MD. Professor, Department of Chest Diseases, Ankara University School of Medicine, Ankara, Turkey.

References

Shah SS, Karnak D, Mason D, et al. Pulmonary transplantation in Mounier-Kuhn syndrome: a case report. J Thorac Cardiovasc Surg. 2006;131(3):757-8.

Krustins E, Kravale Z, Buls A. Mounier-Kuhn syndrome or congenital tracheobronchomegaly: a literature review. Respir Med. 2013;107(12):1822-8.

Celik B, Bilgin S, Yuksel C. Mounier-Kuhn syndrome: a rare cause of bronchial dilation. Tex Heart Inst J. 2011;38(2):194-6.

Abdelghani A, Bouazra H, Hayouni A, et al. Mounier-Kuhn syndrome: A rare cause of bronchial dilatation: A case report. Respiratory Medicine CME. 2009;2(4):164-6. Available from: http://www.sciencedirect.com/science/article/pii/S175500170900013X Accessed in 2017 (Feb 20).

El-Kersh K, Yasin M, Cavallazzi R, Perez RL. Mounier-Kuhn syndrome. Imaging and bronchoscopic findings. Am J Respir Crit Care Med. 2014;190(1):e2-3.

Al-Qadi MO, Artenstein AW, Braman SS. The "forgotten zone": acquired disorders of the trachea in adults. Respir Med. 2013;107(9):1301-13.

Ozdemir C, Sökücü SN, Karasulu L, Altin S, Dalar L. Tracheomalacia treatment using a large-diameter, custom-made airway stent in a case with mounier-kuhn syndrome. Case Rep Pulmonol. 2014;2014:910135.

Downloads

Published

2018-06-07

How to Cite

1.
Kaya AG, Çiledağ A, Atasoy Çetin, Karnak D. Flexible bronchoscopy and mechanical ventilation in managing Mounier-Kuhn syndrome: a case report. Sao Paulo Med J [Internet]. 2018 Jun. 7 [cited 2025 Mar. 9];136(3):266-9. Available from: https://periodicosapm.emnuvens.com.br/spmj/article/view/1273

Issue

Section

Case Report