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Mounier-Kuhn syndrome, also known as tracheobronchomegaly, is a rare clinical and radiological condition characterized by tracheobronchial dilatation and recurrent respiratory infections. Patients may be asymptomatic or present with severe respiratory illnesses. A CT scan is sufficient to confirm the diagnosis. Treatment is symptomatic, and surgery is rarely indicated. We present the case of a 55-year-old patient with a 30-year history of chronic dyspnea, who presented with worsening shortness of breath, a productive cough, and episodes of bronchial superinfection. A chest CT scan, performed before and after contrast injection during both inspiratory and expiratory phases, confirmed the diagnosis of Mounier-Kuhn syndrome. This case aims to raise awareness of this rare condition and highlights a presentation where dyspnea is the primary symptom.

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. [ABSTRACT FROM AUTHOR]

Mounier-Kuhn syndrome (MKS), also known as tracheobronchomegaly (TBM), is a rare disorder characterized by marked dilatation of the trachea and proximal bronchi. We hereby present a case of MKS in a 40-year-old renal transplant recipient. He had multiple admissions in the recent past for lower respiratory tract infections. During recent admission for lobar pneumonia, he continued to exhibit respiratory symptoms including cough and fever, despite culture-sensitive antibiotics. Imaging studies revealed tracheal and bronchial dilatation with right lower lobe consolidation. Bronchoscopy confirmed TBM and severe excessive dynamic airway collapse. Mucolytics, chest physiotherapy, and intermittent continuous positive airway pressure were game changers in this case. This case highlights the importance of recognizing structural lung pathology in solid organ transplant recipients presenting with recurrent respiratory tract infections.

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.

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.

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. Case 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.

Mounier-Kuhn syndrome (MKS), also known as tracheobronchomegaly (TBM), is a rare disorder characterized by marked dilatation of the trachea and proximal bronchi. We hereby present a case of MKS in a 40-year-old renal transplant recipient. He had multiple admissions in the recent past for lower respiratory tract infections. During recent admission for lobar pneumonia, he continued to exhibit respiratory symptoms including cough and fever, despite culture-sensitive antibiotics. Imaging studies revealed tracheal and bronchial dilatation with right lower lobe consolidation. Bronchoscopy confirmed TBM and severe excessive dynamic airway collapse. Mucolytics, chest physiotherapy, and intermittent continuous positive airway pressure were game changers in this case. This case highlights the importance of recognizing structural lung pathology in solid organ transplant recipients presenting with recurrent respiratory tract infections. [ABSTRACT FROM AUTHOR]

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. Case: 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. [ABSTRACT FROM AUTHOR]

Tracheobronchomegaly (TBM) is a rare condition characterized by the dilatation of the trachea and bronchi due to severe atrophy of elastic fibers, accompanied by the thinning of the muscularis mucosae and the development of diverticula between cartilaginous rings. The etiology of this condition remains unclear. Tracheobronchopathia osteochondroplastica (TO) is another uncommon airway disease with an unknown etiology. The co-occurrence of these two diseases has not been reported. In this study, we report and discuss a case involving an elderly man with TBM and TO with a history of recurrent pneumonia over the past 6 years.

Abstract 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.

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. [ABSTRACT FROM AUTHOR]

Deborah Babirye,1 Jonathan Walubembe,1 Juliet Allen Babirye,2 Joseph Baruch Baluku,2,3 Pauline Byakika-Kibwika,4 Eva Nabawanuka1 1Department of Radiology, Makerere University College of Health Sciences, Kampala, Uganda; 2Department of Research, Makerere University Lung Institute, Kampala, Uganda; 3Division of Pulmonology, Kiruddu National Referral Hospital, Kampala, Uganda; 4Department of Medicine, Makerere University College of Health Sciences, Kampala, UgandaCorrespondence: Deborah Babirye, Department of Radiology, Makerere University College of Health Sciences, PO Box 7072, Kampala, Uganda, Email babiryedebbiekiwa@gmail.comAbstract: 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.Keywords: Mounier-Kuhn syndrome, tracheobronchomegaly, MKS

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.

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.

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.

PurposeTracheobronchomegaly (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 featureWe 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.ConclusionAnesthesiologists should keep the possibility of the unpredicted anatomic abnormal respiratory tract in mind, such as TBM.

Tracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn syndrome. In this connection, the need arises for the early detection of this pathology in children and its differentiation from other diseases and malformations of the tracheobronchial tree, which mask a recurrent inflammatory process in the lung and upper respiratory tract, and primarily with those, such as cystic fibrosis and primary ciliary dyskinesia.The paper describes a case of secondary tracheobronchomegaly in a child with obvious clinical manifestations of Mounier-Kuhn syndrome in the presence of bronchiectasis, pulmonary fibrosis, and signs of systemic connective tissue disease. It discusses the possibilities of chest computed tomography in the diagnosis of this defect in children, as well as its causes. The sizes of the trachea and main bronchi in this patient are compared with those in the groups of children of the same age and sex who have cystic fibrosis or diffuse pulmonary fibrosis to identify a more significant role of hereditary connective tissue diseases in the development of secondary tracheobronchomegaly. The authors propose to separate the concepts of tracheobronchomegaly and Mounier-Kuhn syndrome in children and to recognize the threshold for tracheal expansion in these patients having +3SD or more.

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. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

Mounier-Kuhn syndrome is a congenital abnormality characterized by tracheobronchomegaly as a result of atrophy or absence of elastic fibers and thinning of smooth muscle layer in trachea and main bronchi. The usual presentation is one of recurrent respiratory tract infections with a broad spectrum of functional impairment ranging from minimal disease with preservation of lung function to severe disease in the form of bronchiectasis. We describe a case of an elderly man who presented with a recurrent respiratory infection who was subsequently diagnosed as Mounier-Kuhn syndrome.

Tracheobronchomegaly (also called Mounier–Kuhn syndrome) is a rare disease characterized by flaccid and markedly dilated trachea and main bronchi on inspiration with narrowing or collapse on expiration or cough. It is associated with recurrent lower respiratory tract infection. A 75-year-old man with unexpected giant tracheomegaly had a significant peritubal air leak which impeded an operation. Lumbar epidural anesthesia was performed for a subsequent operation without any sequela. Careful evaluation with chest radiography is basic to exclude a large airway. Chest computed tomography and fiber-optic bronchoscopy provided the diagnosis of a large airway. If a large airway is suspected, these examinations help to evaluate and manage the airway.

Abstract: Background and Aim: Mounier‐Kuhn syndrome (MKS) is a congenital disorder characterized by tracheobronchomegaly resulting from the absence of elastic fibers in the trachea and main bronchi or atrophy and thinning of the smooth muscle layer. In this syndrome, dead space associated with tracheobronchomegaly increases and discharge of secretions decreases because of ineffective coughing. The most common complications are recurrent lower respiratory tract infections and bronchiectasis. We examined the clinical characteristics, radiological features, and related complications of patients with MKS. Methods: The cases were obtained between September 2007 and November 2015. Computed tomography scans of the chest were used to diagnose tracheobronchomegaly. Results: All cases (a total of 11) were males with a mean age of 63 ± 13 (range, 38‐80) years. The mean diameter of the trachea was 31.53 ± 2.99 mm; the mean transverse diameter was 31.69 ± 3.10 mm and the mean sagittal diameter was 31.36 ± 3.01 mm. Complaints at the time of presentation included chronic cough, purulent sputum, dyspnea, and hemoptysis. There were recurrent pulmonary infections in seven cases, bronchiectasis in six, and tracheal diverticulum in four at the time of diagnosis. Conclusions: In this article, 11 cases with various rarely seen complications are presented and evaluated in the light of current literature. We recommend that if chronic cough, recurrent pulmonary infections, and bronchiectasis seen in a patient, MKS should be kept in mind. [ABSTRACT FROM AUTHOR]

Introduction: The segmentation and skeletonisation of images via computed tomography (CT) airway lumen volumetry provide a new perspective regarding the incorporation of this technique in medical practice. Our aim was to quantify morphological changes in the large airways of patients with acromegaly through CT and, secondarily, to correlate these findings with hormone levels and pulmonary function testing (PFT) parameters.Methods: This was a cross-sectional study in which 28 non-smoker patients with acromegaly and 15 control subjects underwent CT analysis of airway lumen volumetry with subsequent image segmentation and skeletonisation. Moreover, all participants were subjected to PFT.Results: Compared with the controls, patients with acromegaly presented higher diameters in the trachea, right main bronchus and left main bronchus. The patients with acromegaly also showed a higher tracheal sinuosity index (the deviation of a line from the shortest path, calculated by dividing total length by shortest possible path) than the controls [1.06 (1.02-1.09) vs. 1.03 (1.02-1.04), P = 0.04], and tracheal stenosis was observed in 25% of these individuals. The tracheal area was correlated with the levels of growth hormone (rs  = 0.45, P = 0.02) and insulin-like growth factor type I (rs  = 0.38, P = 0.04). The ratio between the forced expiratory flow and forced inspiratory flow at 50% of the forced vital capacity was correlated with the tracheal area (rs  = 0.36, P = 0.02) and Δ tracheal diameters (rs  = 0.58, P < 0.0001).Conclusion: Patients with acromegaly exhibit tracheobronchomegaly and tracheal sinuosity/stenosis. Moreover, there are associations between the results of CT airway lumen volumetry, hormone levels and functional parameters of large airway obstruction. [ABSTRACT FROM AUTHOR]

Objectives: To describe the imaging features of the central airway pathology, correlating the findings with those in pathology and virtual endoscopy. To propose a schematic and practical approach to reach diagnoses, placing strong emphasis on multidetector computed tomography (MDCT) findings. Methods: We reviewed our thoracic pathology database and the central airway pathology-related literature. Best cases were selected to illustrate the main features of each disease. MDCT was performed in all cases. Multiplanar and volume-rendering reconstructions were obtained when necessary. Virtual endoscopy was obtained from the CT with dedicated software. Results: Pathological conditions affecting the central airways are a heterogeneous group of diseases. Focal alterations include benign neoplasms, malignant neoplasms, and non-neoplastic conditions. Diffuse abnormalities are divided into those that produce dilation and those that produce stenosis and tracheobronchomalacia. Direct bronchoscopy (DB) visualises the mucosal layer and is an important diagnostic and therapeutic weapon. However, assessing the deep layers or the adjacent tissue is not possible. MDCT and post-processing techniques such as virtual bronchoscopy (VB) provide an excellent evaluation of the airway wall. Conclusion: This review presents the complete spectrum of the central airway pathology with its clinical, pathological and radiological features. Teaching points: • Dividing diseases into diffuse and focal lesions helps narrow the differential diagnosis. • Focal lesions with nodularity are more likely to correspond to tumours. • Focal lesions with stenosis are more likely to correspond to inflammatory disease. • Posterior wall involvement is the main feature in diffuse lesions with stenosis. [ABSTRACT FROM AUTHOR]

Mounier-Kuhn-syndrome patients have markedly dilated trachea and main bronchi due to an atrophy or absence of elastic fibers and thinning of smooth muscle layers in the tracheobronchial tree. Although this syndrome is rare, airway management is challenging and general anesthesia may produce fatal results. However, only a few cases have been reported and this condition is not widely known among anesthesiologists. We present the case of a tracheobronchomegaly patient undergoing an emergency off-pump coronary artery bypass. Although the trachea was markedly dilated with numerous tracheal diverticuli, there was an undilated 2 cm portion below the vocal cords found on the preoperative CT. Under a preparation of extracorporeal membrane oxygenation, we intubated and placed the balloon of an endotracheal tube (I.D. 9 mm) at this portion, and maintained ventilation during the operation. This case showed that a precise preoperative evaluation and anesthetic plan is essential for successful anesthetic management.

Tracheomegaly or tracheobronchomegaly is a rare syndrome that consists of marked dilatation of the trachea and the major bronchi, and this is usually due to a congenital defect of the elastic and muscle fibers of the tracheobroncheal tree. Physicians have had only limited experience with performing anesthesia in patients with this type of syndrome. This syndorme is diagnosed by roentenological investigation and this condition is frequently associated with chronic respiratory infection and partial airway obstruction. In this report, we present a case of performing tracheostomy for a patient with tracheomegaly, and this was probably secondary to mechanical ventilator therapy. The regular tracheostomy tube did not provided sufficient length to allow the cuff to lie properly in the trachea in this patient. Because of the peri-cuff air leakgae and hypercapnea after tracheostomy, we needed a longer tracheostomy tube. But we didn't have such a tube and we didn't know any other method, so we couldn't perform tracheostomy. Therefore, we introduced a method of reducing the length of the endotracheal tube to a suitable size until a longer tracheostomy tube can be obtained for those patients having tracheomegaly.

Background. The Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly (TBM) is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT). Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients) also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive.

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. [ABSTRACT FROM AUTHOR]

A síndroma de Mounier-Kuhn é uma doença rara, de etiologia desconhecida e que se presume subdiagnosticada. Cursa frequentemente com infecções respiratórias de repetição e bronquiectasias. A clínica inespecífica, semelhante a outras patologias supurativas brônquicas crónicas, dificulta o diagnóstico, muitas vezes efectuado vários anos após o início dos sintomas. Os autores apresentam uma revisão da literatura a propósito de dois casos clínicos que ilustram a heterogeneidade e as dificuldades diagnósticas desta entidade nosológica. Um dos doentes encontrava-se em idade pediátrica na altura do diagnóstico e outro na quarta década de vida.The incidence of the syndrome may be greater than is suspected. Patients present with recurrent respiratory infections and bronchiectasis. The symptoms are non-specific and indistinguishable from those of other chronic respiratory diseases. The diagnosis is often made several years after the first clinical complaints. The authors perform a brief review of the literature and report two cases of Mounier-Kuhn syndrome. One of the patients was diagnosed in childhood and the other in the fourth decade of life.

Mounier-Kuhn syndrome, also called tracheobronchomegaly, is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications. Here we report a rare case of 55 year old nonsmoker male who presented with recurrent lower respiratory tract infection and bronchiectasis on chest xray, ultimately diagnosed to have bilateral bronchiectasis due to tracheobronchomegaly.

Tracheobronchomegaly (TBM) is a rare enlargement of the tracheal cartilage, also known as Mounier-Kuhn syndrome (MKS). Here, we describe an unusual case of acquired TBM in an adult, caused by amyloid regeneration and associated tracheal weakening, rather than by MKS. CT scan and fiberscopic examination of a 55-year-old woman suffering from severe dyspnea revealed TBM and subglottic stenosis, which was caused by deposition of amyloid tissue. We performed a tracheostomy and vaporized the subglottic stenosis with a CO2 laser, after which we installed a silicone T-tube. After the first operation, re-stenosis occurred, and the procedure was repeated; stenosis was subsequently cured and the dyspnea disappeared, after which the tracheostomy could be closed. This is the first report of adult TBM associated with amyloid deposition in the subglottis and trachea. This diagnosis is very difficult, as amyloid deposition in the trachea can have various clinical presentations. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A síndrome de Mounier-Kühn, ou traqueobroncomegalia congênita, é uma entidade clínica rara caracterizada pela dilatação anormal de traquéia e brônquios principais. O diagnóstico geralmente pode ser realizado através da mensuração do diâmetro traqueal. Os autores apresentam o caso de um homem de 40 anos com pneumonia refratária ao tratamento, no qual a traqueobroncomegalia foi confirmada através de tomografia computadorizada.Mounier-Kuhn syndrome, or tracheobronchomegaly, is a rare clinical entity characterized by abnormal dilation of the trachea and main bronchi. The diagnosis can usually be made by measuring the tracheal diameter. We report the case of a 40-year-old black man with refractory lower respiratory tract infection. Tracheobronchomegaly was confirmed through computed tomography.

Mounier-Kuhn syndrome, also referred to as tracheobronchomegaly, is a rare idiopathic clinical and radiologic disorder characterized by significant tracheobronchial dilation. It results in recurrent lower respiratory tract infections and bronchiectasis. In severe cases, patients may present with acute respiratory distress requiring hospital admission and ventilatory support. Clinical examination and chest radiography may be misleading in these patients as tracheobronchomegaly is easily overlooked on radiographs. We present an interesting report of our patient who presented with acute shortness of breath. A diagnosis of acute pulmonary thromboembolism was suggested by initial evaluation. Computed tomography pulmonary angiography was negative for thromboembolism; however, it revealed diagnosis of this rare disorder.

Lower respiratory tract infection is one of the common causes of morbidity in India which is occasionally undiagnosed. In this regard tracheobronchomegaly is one of those conditions which masquerade as chronic bronchitis and bronchiectasis and are usually undiagnosed. It is a well-defined clinical and radiologic entity characterized by marked dilatation of the trachea and the central bronchi and is frequently associated with recurrent lower respiratory tract infection. Tracheobronchomegaly has been described by a variety of names, including Mounier-Kuhn syndrome, tracheal diverticulosis, tracheobronchiectasis, tracheocele, tracheomalacia, and tracheobronchopathia malacia.

Background. Mounier-Kuhn syndrome or tracheobronchomegaly is a rare disorder characterized by marked dilatation of the trachea and main bronchi, bronchiectasis, and recurrent respiratory tract infections. Its clinical presentation may vary and mimick a variety of disorders. Case report. A 43-year-old female patient, non smoker, complained of intermittent mild dyspnea. Lung function tests and cardiologic findings were within normal limits. The diagnosis was established by computed tomography, which was undertaken due to recurrent lower respiratory tract infections suggestive of bronchiectasis. The transversal tracheal diameter was 2.8 cm that was the criteria for making the diagnosis. In this sporadic case, no association with other disease or condition known to cause secondary tracheobronchomegaly was established. Conclusion. Although rare in clinical practice, Mounier-Kuhn syndrome is an important differential diagnosis in cardio-pulmonary medicine due to a variety of its clinical manifestations. Nowadays, it is easy to diagnose it owing to advanced imaging techniques.

Amyloid is a heterogeneous family of extracellular proteinaceous deposits characterized by apple-green birefringence on polarized light microscopy. There are rare case reports of these extracellular deposits accumulating in the upper and central airways. Progressive infiltration may impair glottic and airway function with some cases requiring intervention to improve flow. Bronchoscopy and lung function testing provide dynamic information to monitor for disease progression; however, the recent development of 320 multislice computed tomography (320 CT) enables dynamic, four-dimensional (4-D) evaluation of laryngeal and tracheal structure and function and presents as a noninvasive, low-radiation dose surveillance tool. We reviewed a 43-year-old man with primary amyloidosis of the larynx and central airways who presented with an 18-year history of progressive dysphonia without breathlessness and preserved lung function. 4-D CT demonstrated marked thickening of supraglottic folds and trachea with marked tracheal dilatation. Despite gross structural abnormalities, dynamic function assessed throughout inspiration and expiration was normal, demonstrating neither rigidity nor dynamic collapse. This combination of structural and functional assessment of the proximal airway by 4-D CT is a novel application to surveillance for laryngeal and tracheal amyloid. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Tracheobronchomegaly or Mounier-Kuhn syndrome 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 on the basis of the characteristic CT scan findings. We report a case in a 21-year-old man presenting with recurrent lower respiratory tract infections.

Tracheobronchomegaly (Mounier-Kuhn syndrome) is defined as marked cystic tracheobronchial dilatation. Tracheobronchomegaly (TBM) is a rare condition primarily effecting men between the ages of 40 to 50 years. The etiology of TBM is unknown. Pathologically, there is atrophy of the muscular and elastic elements of the trachea and central bronchi. The diagnosis of TBM and the associated pathology of the respiratory system are primarily based on radiographic examinations such as plain chest radiographs and CT scans. We report two cases of TBM with a history of recurrent lower respiratory tract infections. [ABSTRACT FROM AUTHOR]

Mounier-Kuhn syndrome is a rare syndrome characterized by tracheobronchomegaly resulting from the loss or atrophy of musculo-elastic fibers within the trachea and main bronchi wall. This syndrome is more common in the third or fourth decades of life. The diagnosis can usually be made by measuring the diameters of trachea and main bronchi radiologically. A 52-year-old male patient was referred to our outpatient clinic with chronic productive cough and recurrent respiratory tract infections. We detected tracheobronchomegaly, tracheal diverticula, and bronchiectasis in the chest CT scans. This rare case is presented due to later onset than expected and very demonstrative radiological findings. [ABSTRACT FROM AUTHOR]