Congenital tracheobronchomegaly or Mounier-Kuhn Syndrome is a rare disorder of controversial etiology. It is characterized by an abnormal enlargement of the trachea and primary bronchi, because of atrophy or absence of their elastic fibers and smooth muscles. Such alterations lead to the collapse of the respiratory tract during forced exhalation, making expectoration by coughing of little use. Subjects with this disorder are, therefore, predisposed to the development of phlogistic bronchopulmonary pathologies such as bronchitis, emphysema, bronchiectasis and pulmonary fibrosis. The present work reports the case of a 65-year-old man suffering from asymptomatic congenital tracheobronchomegaly which was unknown until preoperative testing was performed (standard chest x-ray, tracheobronchoscopy) following a diagnosis of squamous cell carcinoma of the larynx. The patient underwent total laryngectomy and bilateral neck dissection without any intra- and postoperative complication. This is the first case reported in the literature of an association between laryngeal carcinoma and Mounier-Kuhn Syndrome, although a cause-effect relationship between the two pathologies cannot be advanced at this time. This paper also reports how the patient was managed in terms of anesthesiology and surgical technique, both conditioned by the marked tendency for anteroposterior tracheal wall collapse and its high reactivity to mechanical insults.

Un caso di carcinoma laringeo associato a tracheobroncomegalia congenita (sindrome di Mounier-Kuhn) / C. Piazza, A. Bolzoni, M. Giudice, G. Peretti. - In: ACTA OTORHINOLARYNGOLOGICA ITALICA. - ISSN 0392-100X. - 22:1(2002 Feb), pp. 34-38.

Un caso di carcinoma laringeo associato a tracheobroncomegalia congenita (sindrome di Mounier-Kuhn)

C. Piazza;
2002-02

Abstract

Congenital tracheobronchomegaly or Mounier-Kuhn Syndrome is a rare disorder of controversial etiology. It is characterized by an abnormal enlargement of the trachea and primary bronchi, because of atrophy or absence of their elastic fibers and smooth muscles. Such alterations lead to the collapse of the respiratory tract during forced exhalation, making expectoration by coughing of little use. Subjects with this disorder are, therefore, predisposed to the development of phlogistic bronchopulmonary pathologies such as bronchitis, emphysema, bronchiectasis and pulmonary fibrosis. The present work reports the case of a 65-year-old man suffering from asymptomatic congenital tracheobronchomegaly which was unknown until preoperative testing was performed (standard chest x-ray, tracheobronchoscopy) following a diagnosis of squamous cell carcinoma of the larynx. The patient underwent total laryngectomy and bilateral neck dissection without any intra- and postoperative complication. This is the first case reported in the literature of an association between laryngeal carcinoma and Mounier-Kuhn Syndrome, although a cause-effect relationship between the two pathologies cannot be advanced at this time. This paper also reports how the patient was managed in terms of anesthesiology and surgical technique, both conditioned by the marked tendency for anteroposterior tracheal wall collapse and its high reactivity to mechanical insults.
Aged; Carcinoma, Squamous Cell; Humans; Laryngeal Neoplasms; Male; Tracheobronchomegaly
Settore MED/31 - Otorinolaringoiatria
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/649004
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