Two patients with TBM are presented. In one case, a marked decrease of elastic and smooth muscle tissue was present in the bronchial biopsy specimens, obtained by rigid bronchoscopy. [ncbi.nlm.nih.gov]

A case is described which presented as a severe life threatening pneumonia in which the bronchoscopic, radiographic, and computed tomographic findings are given. [doi.org]

We describe a case of an elderly male who presented with a recurrent respiratory infection who was subsequently diagnosed as Mounier-Kuhn syndrome. [jacpjournal.org]

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. [lungindia.com]

• Recurrent Respiratory Infections

  Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly includes clinical and radiographic findings of tracheobronchial dilatation and recurrent respiratory infections. [ncbi.nlm.nih.gov]

  We describe a case of an elderly male who presented with a recurrent respiratory infection who was subsequently diagnosed as Mounier-Kuhn syndrome. [jacpjournal.org]

  He had a history of chronic bronchitis and recurrent respiratory infections. After endotracheal intubation and tube-cuff inflation, a severe peritubal air leak was identified. [nejm.org]

  Mounier-Kuhn syndrome, also known as tracheobronchomegaly, is a rare congenital abnormality of the trachea and main bronchi characterized by cystic dilatation of the tracheobronchial tree and recurrent respiratory infections. [roentgenrayreader.blogspot.com]

• Hypercapnic Respiratory Failure

  In this report we describe a Marfanoid patient who developed TBM in his adulthood with rapid deterioration, leading to hypercapnic respiratory failure. [ncbi.nlm.nih.gov]

• Cutis Laxa

  Rocha MPD, Neugebauer S, Wolter M, Rocha NM (2007) Acquired cephalic cutis laxa. Dermatology Online J 13(3) Google Scholar 35. Krajnc I, Rems D, Vizjak A, Hödl S (1996) Acquired generalized cutis laxa with paraproteinemia (IgG lambda). [link.springer.com]

  If the latter definition is used, associations include connective tissue disorders such as 2,6 : Ehlers-Danlos syndrome cutis laxa The underlying abnormality is an absence or marked atrophy of the elastic fibers and smooth muscle within the wall of the [radiopaedia.org]

  Secondary causes of tracheobronchial enlargement are Marfan syndrome, Ehlers–Danlos syndrome, ataxia-telangiectasia, connective tissue diseases, ankylosing spondylitis, cutis laxa, and light chain deposition diseases. [elsevier.es]

  The disease is sometimes associated with connective tissue diseases such as Ehlers-Danlos syndrome, Marfan syndrome, and cutis laxa (see these terms) and might have a genetic origin; however no gene mutation has been identified to date. [orpha.net]

• Fracture

  For the same photo without the arrows, click here eMedicine Carol A Boles, MD Wrist, Scaphoid Fractures and Complications [learningradiology.com]

  […] loss Limb-girdle muscular dystrophy Muscular dystrophy Witkop syndrome Brugada syndrome Ruzicka Goerz Anton syndrome Sacral hemangiomas multiple congenital abnormalities Trisomy 2 mosaicism Epidermolysis bullosa Spondylometaphyseal dysplasia corner fracture [checkrare.com]

• Ankylosis

  […] albinism type 2 Male pseudohermaphroditism intellectual disability syndrome, Verloes type Spinocerebellar ataxia 4 Ataxia with vitamin E deficiency Intrinsic factor deficiency ALG8-CDG (CDG-Ih) Fibro-adipose vascular anomaly Charcot-Marie-Tooth disease Ankylosis [checkrare.com]

Although her HIV has been well-managed, clinical management of future exacerbations could lead to complicated and extensive infectious disease workups. [cureus.com]

Management and treatment Treatment is symptomatic. Chest physiotherapy can be proposed to improve mucociliary clearance and antibiotics are administered for treatment of pulmonary infections. [orpha.net]

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. [ncbi.nlm.nih.gov]

The possible relationship between the two entities is discussed and the etiology, pathogenesis, clinical manifestations, prognosis and treatment of TBM are reviewed. [ncbi.nlm.nih.gov]

Cystic or cylindrical bronchiectasis affecting particularly first- to fourth-order branches may also be present Prognosis - Tracheobronchomegaly Not supplied. Treatment - Tracheobronchomegaly Not supplied. [checkorphan.org]

Treatment and prognosis Treatment is usually conservative with physiotherapy and postural drainage. Acute exacerbations are treated with antibiotics. In rare instances, tracheal stenting has been used 4. [radiopaedia.org]

Prognosis The impact of this condition on life expectancy has not been evaluated. [orpha.net]

We also hypothesize that TBM and generalized elastolysis may have etiological commonalities, suggesting a need for further study. [ncbi.nlm.nih.gov]

The etiologic mechanism remains unknown. Irritants like cigarette smoke and air pollution could act as irritating factors. [orpha.net]

Summary Epidemiology It is a rare underdiagnosed condition of unknown prevalence. About 300 cases have been reported to date. The condition is more frequent in men. [orpha.net]

While no epidemiologic study has been published, MKS has been found to disproportionally affect men with an 8:1 male to female ratio [4-5]. [cureus.com]

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. [ncbi.nlm.nih.gov]

Prevention - Tracheobronchomegaly Not supplied. Diagnosis - Tracheobronchomegaly The diagnosis is radiological. The dilatation of the trachea and the main bronchi is visible on the chest radiograph. [checkorphan.org]

Control and prevention of recurrent infections will prevent progression to irreversible pulmonary fibrosis. Several trials have also shown benefit of continuous positive airway pressure, airway stenting, and tracheobronchoplasty [9]. [cureus.com]

Since 1981, we have specialized in treating dysfunction related to the spine and offer on and offsite spine school for prevention. [backclinicinc.com]

Success has been achieved in few cases with tracheal stenting to prevent expiratory collapse(6). [rb.org.br]