Mounier-Kuhn syndrome in poorly controlled asthma.

INTRODUCTION: Mounier-Kuhn syndrome or tracheobronchomegaly, is a rare condition that consists of abnormal dilation of the trachea and main bronchi due to a pathological arrangement of smooth muscle fibers in this area. CASE REPORT: We present the case of a 46-year-old woman with poorly controlled asthma and recurrent infections, who was diagnosed with Mounier-Kuhn syndrome through a computed tomography scan revealing an unusual enlargement of the trachea with associated bronchiectasis. RESULTS: The diagnosis of Mounier-Kuhn syndrome is radiological, involving measurement of the trachea where a diameter >25 mm in men and >21 mm in women is observed. While diagnosis is sometimes incidental, there is an association with respiratory diseases such as asthma or COPD, hence clinical suspicion is important in patients with poorly controlled underlying conditions who present with recurrent infections, inadequate secretion management, or even hemoptysis. CONCLUSIONS: Despite its rarity, this syndrome significantly impacts patients' quality of life. Diagnosis and management involve comprehensive evaluations including computed tomography, with a multidisciplinary approach including pulmonologists and radiologists. Exploring its clinical features, associations with other respiratory diseases and treatment options is crucial in managing this rare respiratory condition.

Airway management for a patient with tracheobronchomegaly undergoing lobectomy: a case report.

BACKGROUND: Tracheobronchomegaly (TBM) is a rare disorder mainly characterized by dilatation and malacia of the trachea and major bronchi with diverticularization. This will be a great challenge for airway management, especially in thoracic surgery requiring one-lung ventilation. Using a laryngeal mask airway and a modified double-lumen Foley catheter (DFC) as a "blocker" may achieve one-lung ventilation. This is the first report introducing this method in a patient with TBM. CASE PRESENTATION: We present a 64-year-old man with TBM receiving left lower lobectomy. Preoperative chest computed tomography demonstrated a prominent tracheobronchial dilation and deformation with multiple diverticularization. The most commonly used double-lumen tube or bronchial blocker could not match the distorted airways. After general anesthesia induction, a 4# laryngeal mask was inserted, through which the modified DFC was positioned in the left main bronchus with the guidance of a fiberoptic bronchoscope. The DFC balloon was inflated with 10 ml air and lung isolation was achieved without any significant air leak during one-lung or two-lung ventilation. However, the collapse of the non-dependent lung was delayed and finally achieved by low-pressure artificial pneumothorax. The surgery was successful and the patient was extubated soon after the surgery. CONCLUSIONS: Using a laryngeal mask airway with a modified double-lumen Foley catheter acted as a bronchial blocker could be an alternative method to achieve lung isolation.

The effect of ankaferd blood stopper used for massive haemoptysis in a patient with mounier-kuhn syndrome: A case report.

Mounier-Kuhn Syndrome (MKS) is a rare disorder derived from the muscular and elastic tissue defects of the trachea and the main bronchial walls, characterized by tracheobronchomegaly. Patients may present with complaints of cough, phlegm, dyspnoea and haemoptysis. Haemoptysis may be minor and mixed with phlegm or it may be massive. Establishment of airway patency is a priority in the management of massive haemoptysis. Cold saline solution, diluted adrenaline or tranexamic acid may be administered via the endobronchial route to stop haemorrhage while establishing the airway patency. Ankaferd Blood Stopper (ABS) has a haemostatic property and can be locally administered to the airway. In this report, we aim to highlight the effects of ABS administered via an endobronchial route for emergency palliation of a patient with MKS presenting with massive haemoptysis.

Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases.

BACKGROUND: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections. OBJECTIVE: This multicenter, retrospective study was carried out to describe respiratory conditions associated with tracheobronchomegaly. METHODS: Nine institutions involved in the 'Groupe d'Endoscopie de Langue Française' (GELF) participated in this study. A standard form was used to record patient characteristics, treatments and follow-up from medical charts. RESULTS: Seventeen patients, 53% male, aged 58 ± 18 years at diagnosis were included. Recurrent infections revealed MKS in 88% of cases. Main comorbid conditions were diffuse bronchiectasis in 88% of patients and tracheobronchomalacia in 67% of cases. The exacerbation rate was 1.5 exacerbations/patient/year. The main non-respiratory morbid condition was gastroesophageal reflux disease in 29% of cases. Interventional bronchoscopy was performed in seven patients (41%), consisting of laser (n = 2) and tracheal stenting (n = 5). Complications related to stents were observed in 80% of cases with a mean stent duration of 8 months. Four deaths, including three due to respiratory causes, occurred during follow-up. CONCLUSIONS: This is the largest series of MKS reported in the literature, showing that bronchiectasis and tracheobronchomalacia are the main associated morbid conditions that constitute a challenge for treatment.

An unusual cause of recurrent spontaneous pneumothorax: the Mounier-Kuhn syndrome.

We present a case of 63-year-old man who was referred to the emergency department with a right-sided pneumothorax. He had a history of spontaneous pneumothorax for 2 times. The chest computed tomographic scan showed tracheobronchomegaly with an increase in the diameter of the trachea and right and left main bronchus. Fiberoptic bronchoscopy revealed enlarged trachea and both main bronchus with diverticulas. These findings are consistent with a diagnosis of Mounier-Kuhn syndrome. Mounier-Kuhn syndrome is a rare clinical and radiologic condition. It is characterized by a tracheal and bronchial dilation. Diagnosis is made by computed tomography and bronchoscopy. Mounier-Kuhn syndrome should be kept in mind in the differential diagnosis of recurrent spontaneous pneumothorax.

[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.

A Clinical Classification Scheme for Tracheobronchomegaly (Mounier-Kuhn Syndrome).

INTRODUCTION: Tracheobronchomegaly (Mounier-Kuhn Syndrome) is a rare disease characterized by tracheal enlargement and associated loss of elastic fibers in the trachea and main bronchi. MATERIALS: MEDLINE, Index Medicus, and other databases were searched with pre-defined criteria to identify cases of tracheobronchomegaly (TBM). Two new cases of TBM were also identified from the Provincial Medical Genetics Program of British Columbia. RESULTS: We identified 166 publications describing 365 occurrences of TBM. We observed that affected individuals could be grouped into subgroups according to clinical features. Type 1A (105 individuals) consists of infants who developed TBM after having undergone fetoscopic tracheal occlusion, and Type 1B patients (24 individuals) are infants and children who developed TBM after prolonged intubation. Type 2 individuals developed TBM following recurrent pulmonary infections (2A) (14 individuals) or pulmonary fibrosis (2B) (10 individuals). Type 3 represents TBM with evidence of extra-pulmonary elastolysis (18 individuals), and Type 4 denotes the development of TBM with no clear predisposing factors (196 individuals). Both of our patients had TBM and evidence of extra-pulmonary elastolysis. As well, one patient had a mildly dilated aortic root, which is a previously unreported co-occurrence. CONCLUSION: We introduce a novel classification scheme, which may sort patients into etiologically distinct groups, furthering our understanding of its pathogenesis and potentially, prevention or therapy. We also hypothesize that TBM and generalized elastolysis may have etiological commonalities, suggesting a need for further study.

Coincidence of Tracheobronchomegaly (Mounier-Kuhn Syndrome) and Juvenile Idiopathic Arthritis.

Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly includes clinical and radiographic findings of tracheobronchial dilatation and recurrent respiratory infections. MKS is a very rare pathology, especially in the paediatric age group which makes it a diagnostic challenge. A 4-year-old girl suffered from dyspnea, recurrent respiratory infections and joint pain. Chest radiography detected peribronchial reinforcement and CT-scan revealed extended tracheal dilatation and bronchiectasis. In addition to MKS our patient was diagnosed with juvenile idiopathic arthritis (JIA) and scleroderma. MKS can be caused by congenital disorder or acquired aetiology. Several connective tissue diseases have been associated with MKS but no cases of JIA or scleroderma are described previously. Our case illustrates that patients who suffer from recurrent respiratory infections with unsatisfactory evolution and unspecific chest X-ray alteration, MKS always has to be considered in the differential diagnosis particularly in patients who suffer from connective tissue diseases.

A 54-Year-Old Man with Tracheomegaly, Tracheal Diverticulas and Bronchiectasis--Mounier-Kuhn Syndrome.

Mounier-Kuhn syndrome is a rare idiopathic clinical, radiological and bronchoscopic disorder characterised by abnormal dilatation of the tracheo-bronchial tree. The usual presentation is with recurrent lower respiratory tract infections. Herein, we report the case of an adult male who was diagnosed to have Mounier-Kuhn syndrome based on radiographic finding of a tracheal diameter of 45.5 mm on computed tomography and dynamic complete collapse of the tracheo-bronchial tree on forced expiration, observed during ronchoscopy.

The wonderful world of the windpipe: a review of central airway anatomy and pathology.

A variety of pathologic processes can involve the central airways. Abnormalities may either diffusely or focally involve the tracheal or mainstem bronchial walls. Diseases that diffusely involve the tracheal wall can be subclassified as sparing the membranous trachea or circumferentially involving the tracheal wall. Focal diseases of the trachea and mainstem bronchi include benign and malignant causes. Additionally, congenital and acquired morphologic abnormalities of the trachea will be reviewed.

A grossly abnormal trachea- severe tracheal diverticulosis and Mounier-Kuhn syndrome.

A 53-year-old smoker presented with a history of recurrent lower respiratory tract infections. A diagnosis of Tracheal Diverticulosis due to Tracheobronchomegaly (Mounier-Kuhn Syndrome) was made. The clinical history, diagnosis and treatment options are discussed.

Tracheobronchomegaly following intrauterine tracheal occlusion for congenital diaphragmatic hernia.

BACKGROUND: Fetuses with severe congenital diaphragmatic hernia (CDH) and pulmonary hypoplasia may benefit from fetal endoluminal tracheal occlusion (FETO). Enlargement of the main bronchi and trachea appears to be a common complication of FETO. OBJECTIVE: To retrospectively evaluate the trachea and main bronchi of infants who underwent FETO for CDH and compare diameters with age-matched references. MATERIAL AND METHODS: Postnatal and follow-up chest radiographs were performed in seven children with unilateral CDH treated by FETO. Additional CT was performed in six of these (one neonate died before CT could be performed). Images were acquired from 3 days to 23 months of age. For each child, radiographs and CT images with optimal visualisation of the airways were selected for retrospective analysis. Tracheal and bronchial morphology was assessed by two experienced paediatric radiologists, and the diameters of these structures measured and compared with age-matched references. RESULTS: Mean diameters of the trachea and main bronchi were above the age-matched normal range in all patients, regardless of the side of the hernia or the degree of lung hypoplasia. CONCLUSION: Enlargement of the trachea and main bronchi appears following FETO and persists at least to the age of 5 years.

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.