Colocación de stent y traqueobroncoplastia en el síndrome de Mounier-Kuhn: reporte de un caso / Stent placement and tracheobronchoplasty in Mounier-Kuhn syndrome: a case report

Se presenta el caso de un paciente diagnosticado a raíz de cuadros de disnea recurrentes de traqueobroncomegalia o Síndrome de Mounier-Kunh, patología congénita, muy infrecuente, clasificada como una clase de traqueomalacia del tipo cartilaginosa, cursando por ello con un colapso dinámico de la vía aérea causante de los episodios de disnea. Otros síntomas frecuentemente observados son la tos no productiva, retención de secreciones o infecciones recurrentes.El paciente es tratado inicialmente de forma conservadora mediante la colocación de una prótesis intratraqueal de silicona (Stent Dumon) en forma de "Y" que permite, de forma provisional, la estabilización de la vía aérea y mejoría de su calidad de vida. Posteriormente, se le practica un tratamiento quirúrgico definitivo o traqueobroncoplastia, que reconfigura la estructura traqueal evitando el colapso espiratorio y permitiendo la retirada del stent intratraqueal

We present the case of a patient diagnosed based on recurrent dyspnea from tracheobronchomegaly or Mounier-Kuhn syndrome, a very rare congenital disease classified as a type of cartilaginous tracheomalacia, leading to a dynamic collapse of the airway causing episodes of dyspnea. Other frequently observed symptoms are nonproductive cough, secretion retention and recurrent infections.The patient is initially treated conservatively by placing a Y-shaped silicone intratracheal prosthesis (Dumon stent) which provisionally allows for airway stabilization and improved quality of life. Later, the patient undergoes a definitive surgical treatment or tracheobronchoplasty, which reconfigures the tracheal structure, avoiding expiratory collapse and allowing the intratracheal stent to be removed

Tracheobronchoplasty after a trial with a silicone Y-stent in a patient with Mounier-Kuhn syndrome.

Tracheobronchomegaly or Mounier-Kuhn syndrome is a rare disorder characterized by the widening of the trachea and the main bronchi. It is a form of tracheomalacia called 'cartilaginous malacic' and is characterized by deformation of the tracheal cartilages and intrusion of the redundant membranous wall into the lumen of the airway. We present a patient with Mounier-Kuhn syndrome managed like patients with tracheomalacia of other aetiologies-a tracheobronchoplasty with a reconstructed D-shaped trachea and stabilization of the posterior membranous wall by attaching a polypropylene mesh to the posterior membranous wall of the trachea and the main bronchi after a trial period with a tracheobronchial Y-shaped silicone stent.

[Tracheobronchoplasty for Severe Diffuse Tracheomalacia]. / Tracheobronchoplastie bei schwerer diffuser Tracheobronchomalazie.

Patients with diffuse airway instability due to tracheobronchomalacia or excessive dynamic airway collapse are typically highly symptomatic, with marked dyspnoea, recurrent bronchopulmonary infections and excruciating intractable cough. Silicone stents achieve immediate symptom control, but are - due to the typical complications associated with stent treatment - usually not an option for long-term treatment. The aim of surgical intervention is definitive stabilisation of the trachea and of both main bronchi by posterior splinting of the Paries membranaceus with a polypropylene mesh. This operation is an appropriate treatment option for patients with documented severe tracheobronchomalacia or excessive dynamic airway collapse and is ultimately the only therapy that can achieve permanent symptom control. The success of the operation, however, depends on many factors and requires close interdisciplinary collaboration.

[Tracheobronchial prosthesis in Mounier-Kuhn syndrome: New perspectives]. / Prothèse trachéobronchique dans le syndrome de Mounier-Kuhn : nouvelles perspectives.

INTRODUCTION: Mounier-Kuhn syndrome or tracheobronchomegaly is a rare congenital condition, the management of which is complex. We report the case of a patient who was treated with interventional endoscopy. OBSERVATION: We describe the case of a 74-year-old man with a diagnosis of tracheobronchomegaly who was admitted in 2003 with a background of deteriorating respiratory status and the occurrence of postural syncope. He initially received a tracheobronchial silicone Y prosthesis, extended with metal prostheses at the tracheal and bronchial level. This arrangement remained stable until 2011. He then began to develop episodes of asphyxia related to posterior dislocation of the tracheobronchial prosthesis, after breakage of the metallic mesh tracheal prosthesis. A new tracheobronchial prosthesis Y was then placed, custom-made from a 3D model of the airways. This was clinically and functionally effective. DISCUSSION: This case describes the management of a patient with Mounier-Kuhn syndrome by interventional bronchoscopy, with the adaptation of prosthetic materials, on an individual basis, to the anatomy of the patient's airway.

Clinical-radiological, histological and genetic analyses in a lung transplant recipient with Mounier-Kuhn syndrome and end-stage chronic obstructive pulmonary disease.

BACKGROUND AND AIMS: The Mounier-Kuhn syndrome (MKS) is a rare disease characterized by a pathological dilation of the trachea and the bronchial system. The etiology of the disorder remains elusive, but genetic alterations and degradation of elastic fibers are thought to be involved in the pathogenesis. No causative treatment is available although transplantation is an option for end-stage disease. Here, we describe a patient suffering from MKS who received a double lung transplant at our department. METHODS: Since a familial clustering of MKS is discussed in the literature, we performed a chromosomal analysis and an array-comparative genomic hybridization (CGH) to search for genetic abnormalities. At the time of transplantation, we collected samples from the bronchi and performed hematoxylin and eosin (HE), Elastic von-Gieson (EVG) and immunohistochemical stains of the explanted MKS bronchus, a control bronchus and of the inflammatory infiltrates. Specimens of main bronchi from the donor lung harvested for transplant served as control. Bronchial smears were taken from both main bronchi of the recipient for microbiological cultures. RESULTS: No genetic alterations could be found in chromosomal analysis and in array-CGH. Histological analysis revealed a strong reduction of elastic fibers in the submucosal connective tissue and a diffuse inflammatory infiltrate, mainly comprised of CD4+ cells. In addition, immunohistochemistry showed increased matrix metalloproteinases (MMPs) protein expression of MMP-1, 2, 3 and 9. CONCLUSIONS: Based on our findings, we hypothesize that MKS is a chronic inflammatory disease characterized by an MMP-mediated degradation of submucosal elastic fibers.

Central airway obstruction due to malignant fibrous histiocytoma metastasis in a case with Mounier-Kuhn syndrome.

Malign fibrous histiocytoma is one of the most observed soft tissue sarcomas seen in the adults. The most common metastasis region is the lung and metastasis. Mounier-Kuhn syndrome is characterized by the highly dilatation of the trachea and bronchi. We may encounter with the major airway obstruction in the endoluminal or extraluminal lung and mediastinal masses or those with both components together. In this article, we would like to highlight the occurrence of a rare seen clinical situation secondary to the giant mediastinal malign fibrous histiocytoma metastasis and the clinical difficulties experienced in resolving of the main airway obstruction caused by the mass. Since the lack of the similar studies conducted previously, we found the case worth presenting.

[Dyspnea and chronic recurrent respiratory tract infections]. / Dyspnoe und rezidivierende Atemwegsinfekte.

A 48-year-old man presented with recurrent bronchopulmonary infections of many years' standing. The diagnosis of tracheobronchomegaly (Mounier-Kuhn syndrome) was based on CT examination and subsequent bronchoscopy. Since lung transplantation was not considered advisable, a biluminal stent was inserted to prevent bronchial collapse.

Double lung transplantation in a patient with tracheobronchomegaly (Mounier-Kuhn syndrome).

Mounier-Kuhn syndrome is a rare condition characterized by marked dilation of the trachea and main bronchi resulting in bronchiectasis and emphysema. We report a case in which a patient underwent successful double lung transplantation for COPD that was found on pathologic examination of the explanted lungs to be Mounier Kuhn syndrome. To our knowledge this is the first case reporting lung transplantation in this syndrome.

Renal transplantation in a patient affected by Mounier-Kuhn syndrome.

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.

Tracheomalacia associated with Mounier-Kuhn syndrome in the Intensive Care Unit: treatment with Freitag stent. A case report.

Tracheomalacia is a process characterized by softness of the supporting tracheal cartilages, by the extension of the posterior membranous wall and by reduction of the tracheal antero-posterior diameter. Exceptionally, tracheomalacia can be associated with tracheobronchomegaly or Mounier-Kuhn syndrome. Fibro-bronchoscopy represents the ''gold standard'' for diagnosis. The case of a 79-year-old male observed after hospitalization in a medical ward for chronic pulmonary obstructive disease (COPD) decompensation, and with basal left bronchopulmonary focus, is described. During this period, a progressive worsening of clinical conditions occurred, despite cortisone and antibiotic therapy, and the patient was transferred to the ICU for dyspnea, hypoxia, hypocapnia and with a diagnosis of pulmonary fibrosis. Bronchoscopy, performed during spontaneous breathing, revealed tracheomalacia which was responsible for tracheal dynamic complete stenosis during expiration and dynamic subtotal stenosis of the left primary bronchus in the first tract, together with sputum retention. Moreover, this investigation confirmed the diagnosis of tracheobronchomegaly already seen on CT. It was suggested to place a Freitag stent, since the insertion of another model would not have had enough chance of stability, due to the enormous extension of the tracheal lumen and could not have guaranteed good clearance of the secretions. Seven days after this intervention, performed in an outpatients' setting, the patient was dismissed from the ICU, without the help of O2, with good ventilation, saturation in line with his age and good expectoration.

[Laryngeal carcinoma associated with congenital tracheobronchomegaly (Mounier-Kuhn syndrome): a case report]. / Un caso di carcinoma laringeo associato a tracheobroncomegalia congenita (sindrome di Mounier-Kuhn).

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.

[Tracheobronchomegaly: an exceptional predisposing factor for pulmonary aspergillomas and massive hemoptysis]. / Traqueobroncomegalia: un factor predisponente excepcional de aspergilomas pulmonares y hemoptisis masiva.

Mounier-Kuhn syndrome--or tracheobronchomegaly--is a rare congenital disorder characterized by significant dilation of the trachea and main bronchi. It is accompanied by ineffective cough and is often complicated by recurrent lung infections and bronchiectasis. Clinical presentation varies widely, ranging from forms with scarce involvement of functional capacity to others that progress to respiratory failure that can prove fatal. We report an exceptional case of massive hemoptysis secondary to the presence of pulmonary aspergillomas and bilateral bronchiectasis in which bronchial arteriography with embolization and surgical resection were insufficient for resolving the complications.