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Tracheobronchomegaly

Mounier Kuhn Syndrome


Presentation

  • 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]
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]
Constitutional Symptom
  • There were generalized constitutional symptoms of fatigue, malaise and weight loss. The patient had no family history of chronic obstructive pulmonary diseases. He had always been a non- smoker and worked as manual labourer.[jemds.com]
Regurgitation
  • Associated findings included decreased contractility of RV and moderate tricuspid regurgitation. A diagnosis of acute pulmonary thromboembolism (PTE) was suggested.[lungindia.com]
  • […] nerve Glutamine deficiency, congenital Renier Gabreels Jasper syndrome Malignant hyperthermia arthrogryposis torticollis Townes-Brocks syndrome TMEM165-CDG (CDG-IIk) Nail-patella syndrome Schneckenbecken dysplasia Ataxia-oculomotor apraxia type 4 Mitral regurgitation[checkrare.com]
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]
  • 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]
  • 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]
Erythema
  • Pierre Robin sequence with pectus excavatum and rib and scapular anomalies Insulin-resistance type B Minicore myopathy with external ophthalmoplegia Beta ketothiolase deficiency Chromosome 17p duplication Mungan syndrome 3MC syndrome Keratolytic winter erythema[checkrare.com]
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]

Workup

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

Treatment

  • 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]

Prognosis

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

Etiology

  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • Barotrauma is speculated to be the primary pathophysiologic factor in these cases.[ncbi.nlm.nih.gov]

Prevention

  • 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]
  • Basic aim in all patients is to prevent further damage to the airway tract. This is achieved by cessation of smoking, minimizing exposure to the industrial and occupational irritants and pollutants.[lungindia.com]
  • Since 1981, we have specialized in treating dysfunction related to the spine and offer on and offsite spine school for prevention.[backclinicinc.com]

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