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.

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.

An atypical encounter: Mounir-Kuhn syndrome and aspergilloma coexistence: A case report.

We report a coexistence of Mounir-Kuhn syndrome and aspergilloma in a 69-year-old male presented with hemoptysis, cough, shortness of breath and fever. This patient has a history of recurrent hospital admissions for infective exacerbations of chronic obstructive pulmonary disease. Diagnostic imaging revealed the presence of aspergilloma and marked dilatation of the trachea and main bronchi, leading to a diagnosis of Mounier-Kuhn syndrome. Given the frequent association of Mounier-Kuhn syndrome with recurrent respiratory infections, we recommend that clinicians consider this syndrome when evaluating patients with recurrent pneumonia.

A case report of unusual recurrent bronchopneumonia infections in Mounier-Kuhn syndrome.

Mounier-Kuhn syndrome is a rare airway disease characterized by tracheal and bronchial dilatation, primarily affecting middle-aged men. We present a case of Mounier-Kuhn syndrome in a 40-year-old man with a history of recurrent respiratory infections since adolescence. The diagnostic journey involved a multidisciplinary approach incorporating clinical evaluation, radiological imaging, and bronchoscopy. Computed tomography findings, including maximum intensity projection reconstructions and 3D rendering, facilitated the diagnosis by revealing significant airway dilation and associated abnormalities. Treatment primarily focused on supportive measures, including antibiotic therapy and respiratory physiotherapy. This case underscores the importance of considering Mounier-Kuhn syndrome in patients with recurrent respiratory infections and highlights the role of advanced imaging techniques in diagnosis.

Airway management of a patient with Mounier-Kuhn syndrome during general anesthesia - A case report.

BACKGROUND: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by abnormal dilation of the trachea and main bronchi. MKS can be easily missed on chest X-rays, making diagnosis difficult. Under general anesthesia, challenges such as airway leakage or collapse during mechanical ventilation may complicate the achievement of adequate tidal volumes. CASES: A 94-year-old woman requiring emergency hemiarthroplasty of the hip under general anesthesia was admitted. Preoperative chest X-rays revealed dilation of the trachea and main bronchi, but the patient exhibited no respiratory symptoms. We diagnosed her with MKS and opted for an 8.0-mm-inner-diameter reinforced tracheal tube. We positioned the cuff in the subglottic area, inflating it while monitoring for air leakage. Throughout the surgery, adequate tidal volume was maintained. CONCLUSIONS: Anesthesiologists must conduct a comprehensive evaluation of patients with MKS, including a review of chest radiographs, and establish a meticulous anesthesia plan prior to surgery.

Tracheobronchomegaly (Mounier-Kuhn syndrome) and Bronchiectasis as rare manifestations of Homocystinuria.

Homocystinuria (HCU) is a rare autosomal recessive inherited disorder usually diagnosed in childhood. It is characterized by a deficiency of the enzyme that converts homocysteine to cystathionine. The accumulation of homocysteine leads to abnormalities in the ocular, skeletal, cardiovascular, and central nervous systems. HCU shares several clinical features with Marfan syndrome; however, respiratory system involvement in HCU is uncommon and rarely reported. Bronchiectasis has been previously reported in a few cases of HCU, and it was attributed mainly to fibrillin deficiency. This case describes a young girl diagnosed with classical HCU since childhood who presented with a chronic productive cough and was initially misdiagnosed as bronchial Asthma. However, upon further evaluation, she was eventually diagnosed with tracheobronchomegaly (TBM), or Mounier-Kuhn Syndrome, and bronchiectasis based on the computed tomography (CT) scan of chest findings. To our knowledge, this is the first reported case of TBM and bronchiectasis in HCU. We believe that fibrillin degeneration may be the key to understanding this unusual association in HCU.

Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a 43-Year-Old Male: A Case Report.

Mounier-Kuhn syndrome (MKS) or congenital tracheobronchomegaly is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. The etiology of this disorder is uncertain and the clinical presentation is variable. The diagnosis is usually made based on the characteristic computed tomography (CT) scan findings. This report describes a case of a 43-year-old man presenting with persistent cough and recurrent lower respiratory tract infections since childhood associated with copious amounts of purulent sputum, difficulty in breathing, and weight loss. In addition, he reported palpitations, dyspnea, orthopnea, abdominal and lower limb swelling. The chest X-ray showed a dilated trachea (35mm) and bronchi (26mm (right) and 27mm (left)) with cystic bronchiectasis and reticulolinear opacities predominantly involving the middle and lower lung zones. Chest CT scan confirmed the diagnosis of MKS as evidenced by dilated trachea and bronchi complicated by diverticula formation. Electrocardiogram, echocardiography and abdominal ultrasound scan showed features of right-sided heart failure secondary to pulmonary hypertension. MKS, although rare, should be considered as a possible diagnosis in patients presenting with productive chronic cough, recurrent pneumonia, or incomplete response to appropriate antibiotic therapy for pneumonia.

Tracheobronchomegaly (Mounier-Kuhn Syndrome) with CT and bronchoscopic correlation: A case report.

Tracheobronchomegaly, or Mounier-Kuhn syndrome, is a clinical and radiological entity characterized by marked dilatation of the trachea and bronchi as a result of severe atrophy of the elastic fibers, with thinning of the muscularis, and the formation of diverticula between the cartilaginous rings. The etiopathogenesis is uncertain and may be congenital or acquired. The clinical signs are not specific and are frequently revealed by recurrent respiratory infections and chronic cough. The diagnosis of Mounier-Kuhn syndrome is based on well-documented measurements of the trachea and main bronchi performed on a chest computed tomography scan. The management of patients is based on symptomatic treatment and may require, in severe cases, the use of endoscopic treatment by stent placement or surgical tracheobronchoplasty. We present a case of a 59yearold patient with recurrent respiratory infections that required several hospitalizations. Diagnosed with Mounier Kuhn syndrome, the thoracic computed tomography scan demonstrated a dilated trachea until the bifurcation and focal points of bronchial dilatation. Bronchoscopic examination showed a dilated and deformed trachea with the presence of diverticula on the tracheal anterior wall. The diameter of the trachea was reduced by more than 50% during expiration and coughing. For this reason, Mounier-Kuhn syndrome should be considered in cases of recurrent respiratory infection or persistent respiratory symptoms.

Unexpected curious cause of serious air leakage after endotracheal intubation: A case report of tracheobronchomegaly and literature review.

Purpose: Tracheobronchomegaly (TBM) is a rare disease with enlarged trachea and mainstem bronchi, which might not be diagnosed preoperatively because of patient's nonsymptoms or clinicians' overlook. These patients would be at fatal risk after general anesthesia endotracheal intubation due to severe peritubal leakage. This case may provide a helpful and informative resource for anesthesiologists and other clinicians, especially those managing patients' airways. Clinical feature: We presented a patient undergoing elective scoliosis orthopedics who was postoperatively diagnosed with TBM. After general anesthesia endotracheal intubation, difficulty in maintaining ventilation with obvious peri-cuff air leakage made this rare disease to be suspected. The peritubal leakage was resolved by relocating the endotracheal tube to the subglottic area. Fortunately, there were no air leakage and postoperative complications. Conclusion: Anesthesiologists should keep the possibility of the unpredicted anatomic abnormal respiratory tract in mind, such as TBM.

Recurrent Lower Respiratory Tract Infections Due to Mounier-Kuhn Syndrome.

Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by recurrent lower respiratory tract infections and bronchiectasis due to dilation of the trachea and bronchi. Diagnosis is made based on clinical suspicion along with radiographic evidence of tracheobronchomegaly. Mucolytic agents and chest physiotherapy have been shown to offer symptomatic improvement, and definitive surgical treatment is reserved for those with persistent symptoms. Herein, we report a case of MKS in a 72-year-old woman with bronchiectasis and recurrent multidrug-resistant lower respiratory tract infections.

Mounier-Kuhn syndrome (Tracheobronchomegaly): Radiological diagnosis.

Mounier Kuhn Syndrome or tracheobronchomegaly is a rare disease, characterized by dilatation of the trachea and the main bronchi. Our study concerns a case of 67-years old male patient, smoker, with a chronic cough. Chest scan was requested; it shows an enlargement of the tracheal clarity and the two main bronchi. Meanwhile, the Functional respiratory exploration was normal. The treatment includes mucolytics and pulmonary physiotherapy. Mounier-Kuhn syndrome is rare and the clinical signs are not specific, the positive diagnosis is purely radiological. The treatment is about to free the airways to prevent infection.

Congenital tracheobronchomegaly (Mounier-Kuhn syndrome) in a 28-year-old Zambian male: a case report.

Congenital tracheobronchomegaly, also known as Mounier Kuhn Syndrome (MKS) is a rare respiratory disorder characterized by dilatation of the trachea and bronchi. We report a case of a 28-year-old male of African descent in Zambia, who presented with a history of chronic productive cough and repeated chest infections since childhood. He had been treated numerous times for lower respiratory tract infections, and had received empiric tuberculosis (TB) treatment based on chest radiograph findings, despite negative sputum microscopy and molecular tests for TB. Investigations revealed normal baseline blood results and sputum results. He however, had markedly increased levels of serum immunoglobulin E, and spirometry showed an obstructive pattern with significant post bronchodilator improvement. High-resolution computed tomography scan revealed tracheal dilatation, extensive bilateral bronchiectasis and tracheal and bronchial diverticula. The latter were also seen on bronchoscopy, confirming the diagnosis of Mounier-Kuhn syndrome. The patient was treated with combined inhaled corticosteroids and bronchodilators, as well as chest physiotherapy for mucus clearance, which led to improvement in his symptoms. Our case highlights how in low-resource settings, chronic lung diseases, particularly bronchiectasis, are often clinically and radiologically mistaken for and presumptively treated as TB (or its sequelae). Mounier-Kuhn syndrome, albeit rare, should be considered in the differential diagnosis of patients with recurrent lower respiratory tract infections or bronchiectasis. Multidisciplinary team meetings can help in the diagnosis of rare lung diseases.

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.

Improving Airways Patency and Ventilation Through Optimal Positive Pressure Identified by Noninvasive Mechanical Ventilation Titration in Mounier-Kuhn Syndrome: Protocol for an Interventional, Open-Label, Single-Arm Clinical Trial.

BACKGROUND: Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare disease characterized by dilation of the trachea and the main bronchi within the thoracic cavity. The predominant signs and symptoms of the disease include coughing, purulent and abundant expectoration, dyspnea, snoring, wheezing, and recurrent respiratory infection. Symptoms of the disease in some patients are believed to be pathological manifestations arising due to resident tracheobronchomalacia. Although treatment options used for the management of this disease include inhaled bronchodilators, corticosteroids, and hypertonic solution, there is no consensus on the treatment. The use of continuous positive airway pressure (CPAP) has been reported as a potential therapeutic option for tracheobronchomalacia, but no prospective studies have demonstrated its efficacy in this condition. OBJECTIVE: The purpose of this is to identify the presence of tracheobronchomalacia and an optimal CPAP pressure that reduces the tracheobronchial collapse in patients with Mounier-Kuhn syndrome and to analyze the repercussion in pulmonary ventilation. In parallel, we aim to evaluate the prevalence of obstructive sleep apnea/hypopnea syndrome. METHODS: This interventional, open-label, single-arm clinical trial will enroll patients who are diagnosed Mounier-Kuhn syndrome. Patient evaluation will be conducted in an outpatient clinic and involve 3 visits. Visit 1 will involve the collection and registration of social demographic, clinical, and functional data. Visit 2 will entail polysomnography, bronchoscopy for the evaluation of tracheobronchomalacia, titration of the optimal pressure that reduces the degree of collapse of the airway, and electrical impedance tomography. In visit 3, patients exhibiting a reduction in collapse areas will be requested to undergo chest computed tomography during inspiration and forced expiration with and without positive pressure (titrated to determine optimal CPAP pressure). RESULTS: This protocol is a doctorate project. The project was submitted to the institutional review board on January 24, 2017, and approval was granted on February 2, 2017 (Brazilian Research database number CAAE 64001317.4.000.0068). Patient evaluations started in April 2018. Planned recruitment is based on volunteers' availability and clinical stability, and interventions will be conducted at least once a month to finish the project at the end of 2020. A preliminary analysis of each case will be performed after each intervention, but detailed results are expected to be reported in the first quarter of 2021. CONCLUSIONS: There is no consensus on the best treatment options for managing Mounier-Kuhn syndrome. The use of positive pressure could maintain patency of the collapsed airways, functioning as a "pneumatic stent" to reduce the degree of airflow obstruction. This, in turn, could promote mobilization of thoracic secretion and improve pulmonary ventilation. TRIAL REGISTRATION: ClinicalTrails.gov NCT03101059; https://clinicaltrials.gov/ct2/show/NCT03101059. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/14786.

Thoracic Computed Tomography Scan and Bronchoscopy Appearance of Mounier-Kuhn Syndrome: A Case Report.

Mounier-Kuhn syndrome (MKS) is a rare congenital disease with an autosomal recessive inheritance pattern, characterized by an enlargement of the trachea and bronchi. MKS is secondary to a thinning of the muscular mucosa and atrophy of the longitudinal muscle and elastic fibers of the tracheobronchial tree. As a consequence, tracheal diverticulosis and dilatations in the posterior membranous wall appear, along with bronchiectasis that tend to be cystic in appearance. Overall, there is an impairment of mucocilliary clearance, with an ineffective cough, which predisposes the patient to recurrent lower respiratory tract infections. Clinical manifestations vary from asymptomatic to respiratory failure and death, most patients being diagnosed between the third and fourth decades of life. It is an often undiagnosed disease, with a diagnostic algorithm that includes the use of radiological techniques, alone or in combination with bronchoscopy. Specific diagnostic criteria have been developed, based on patients' tracheal and main bronchi diameter on chest X-ray and thoracic computed tomography scan. We present the case of a 45-year-old African American man who presented with a history of multiples episodes of pneumonia that required management in the intensive care unit, on whom MKS was diagnosed.

A curious case of cough: Mounier-Kuhn syndrome in a Namibian female patient.

We report a case of a 43-year-old female who presented with a history of recurrent productive cough, since her teenage years. Her associated symptoms included dyspnoea, occasional pleuritic chest pain and rarely, constitutional symptoms. Treated numerous times for lower respiratory tract infections, her symptoms would improve after antimicrobial therapy, but always recurred. She had a background of HIV infection and was virologically suppressed on antiretroviral therapy for nine years. Investigations revealed an active pseudomonas infection and high-resolution computed tomography scan (HRCT) and bronchoscopy confirmed features of Mounier-Kuhn syndrome. The patient was treated accordingly with positive airway pressure, mucolytic agents and chest physiotherapy aimed at aiding mucus clearance and received pneumococcal and influenza vaccines. Mounier-Kuhn syndrome, though rare, should be considered in the differential diagnosis of patients with recurrent lower respiratory tract infections. In Africa, more cases may be identified and treated appropriately with timely investigation and treatment.

Mounier-Kuhn syndrome: A variable course disease.

Mounier-Kuhn syndrome or tracheobronchomegaly is a rare disease characterized by marked dilation of the trachea and proximal bronchi with recurrent lower tract respiratory infections. Computed tomography and bronchoscopy are the key tools to accomplish the diagnosis. This is a condition with a clinical polymorphism, symptoms vary from minor with preserved respiratory function, to very severe with life threatening exacerbations leading to respiratory failure and premature death. The treatment is mainly symptomatic, stenting or surgery are reserved to extreme cases.Herein, we report two cases of the same condition with different clinical signs and diverse outcome.