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Mounier-Kühn Syndrome

SCD


Presentation

  • The clinical presentation is variable and it can occur in several different age groups. We present the case of a young man who was admitted to the hospital for an asthma exacerbation and was found to have tracheobronchomegaly on his CT scan.[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]
  • Here, we described a patient who presented with hoarseness and pneumonia. Further studies confirmed the diagnosis of Mounier-Kuhn syndrome with bilateral vocal cord paralysis.[experts.umn.edu]
  • This case is being presented due to its rarity and unusual presentation as a single mass lesion arising from the posterior wall of the larynx causing near total obliteration of the tracheal lumen. Financial support and sponsorship Nil.[ijpmonline.org]
  • In mild cases, patients are asymptomatic or present only with chronic cough and have normal pulmonary function.[orpha.net]
Pallor
  • Physical examination revealed pallor. Antero-posterior diameter of his chest had increased. Auscultation disclosed inspiratory crepitations in both lower lung fields. There was no cyanosis or clubbing.[jemds.com]
  • On general physical examination, the patient had pallor. However, there was no cyanosis, clubbing or pedal edema. Cardiorespiratory examination was significant for bilateral crepitations and occasional bronchial breath sounds.[lungindia.com]
Malaise
  • 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]
Splenomegaly
  • There was no lymphadenopathy or splenomegaly. Routine laboratory findings revealed low hemoglobin level with elevated serum transaminases and alkaline phosphatase levels.[academic.oup.com]
Purulent Sputum
  • 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.[en.wikipedia.org]
  • sputum production, dyspnea (occasionally on exertion) and hemoptysis.[orpha.net]
  • For three years she had had a chronic cough, productive of several mouthfuls of purulent sputum each day. The preceding year she had noticed progressive exertional dyspnea.[jamanetwork.com]
  • sputum consistent with bronchiectasis Signs and symptom range from minimal disease with preservation of pulmonary function to progressive disease leading to respiratory failure and death Imaging Findings Tracheobronchomegaly (see below) Tracheal size[learningradiology.com]
Dry Cough
  • A 52-year-old woman was referred for evaluation of a persistent dry cough and episodic hemoptysis of about 20 years’ duration.[cmaj.ca]
  • FOB was performed to investigate the underlying causes of chronic dry cough. FOB showed that submucosal nodules protruded into the airway lumen.[jtd.amegroups.com]
  • Clinical case A 46-year-old, no smoker woman, with a 5 years medical history of episodic dry cough, consult for a chronic abdominal pain with weight loss during 5 months. The physical examination was unremarkable, except hepatomegaly.[academic.oup.com]
  • He had a past history of pulmonary tuberculosis (25 years ago; unavailable medical records) and mentioned occasional episodes of dry cough every year for the last 3 years. His family and occupational history were not significant.[karger.com]
  • Case presentation A 69-year-old male presented to our pulmonary and critical care center suffering from chronic dry cough since several months and haemoptysis since about 4 weeks.[coughjournal.biomedcentral.com]
Persistent Cough
  • Coughing frequency in patients with persistent cough: assessment using a 24 hour ambulatory recorder. Eur Respir J 1994;7:1246-53. [ PubMed ] Meyer CN, Døssing M, Broholm H. Tracheobronchopathia osteochondroplastica.[jtd.amegroups.com]
  • On computed tomography (CT), these appear as calcified nodules 1–3 mm in diameter lining the anterolateral tracheobronchial walls. [1] These conditions may be cause of persistent cough, dyspnoea, recurrent infections, hoarseness or recurrent haemoptysis[aihbonline.com]
  • View Article PubMed Google Scholar Gillissen A, Richter A, Oster H: Clinical efficacy of short-term treatment with extra-fine HFA beclomethasone dipropionate in patients with post-infectious persistent cough. J Physiol Pharmacol. 2007, 58: 223-232.[coughjournal.biomedcentral.com]
Rales
  • Examination of Chest revealed the presence of bilateral rales, more on basal regions. The chest radiograph showed ill-defined shadows bilaterally on lower zones. A computed tomography (CT) scan of thorax was performed.[elsevier.es]
Orthopnea
  • Breathlessness was of Grade 1 severity on MRC grading and there was no orthopnea or paroxysmal nocturnal dyspnea. He gave a history of recurrent respiratory infection from his adolescence.[jacpjournal.org]
Chronic Abdominal Pain
  • In our case, despite presence of chronic cough, the diseases was incidentally discovered at imaging during exploration of a chronic abdominal pain.[academic.oup.com]
Abdominal Pain
  • In our case, despite presence of chronic cough, the diseases was incidentally discovered at imaging during exploration of a chronic abdominal pain.[academic.oup.com]
Regurgitation
  • Associated findings included decreased contractility of RV and moderate tricuspid regurgitation. A diagnosis of acute pulmonary thromboembolism (PTE) was suggested.[lungindia.com]
Tachycardia
  • On examination, there was tachycardia, tachypnoea and reduced oxygen saturation (SpO 2 84%). Physical examination revealed pallor. Antero-posterior diameter of his chest had increased.[jemds.com]
  • On examination, there was tachycardia (heart rate of 100 bpm), tachypnea (respiratory rate was 25/min) with SpO 2 85% and FiO 2 50%. Patient was afebrile and blood pressure was within normal range (measured on at least three occasions).[lungindia.com]
  • Tachycardia, bronchial breath sounds, diffuse expiratory wheezing, and rhonchi were noted on physical examination.[cureus.com]
Cyanosis
  • There was no cyanosis or clubbing. His blood work showed mild anemia (Hb- 11.1 gm %) and leucocytosis (TLC-12,500 mm 3 ), RBC-5.9 million cells/mm 3. MCV, MCH and MCHC were within normal limits.[jemds.com]
  • However, there was no cyanosis, clubbing or pedal edema. Cardiorespiratory examination was significant for bilateral crepitations and occasional bronchial breath sounds. The heart sounds were normal.[lungindia.com]

Workup

  • The clinical presentation of TO is variable and ranges from incidental diagnosis in asymptomatic patients during workup or management for unrelated medical problems, to devastating disease with central airway obstruction.[utsouthwestern.influuent.utsystem.edu]
  • For further diagnostic workup bronchoscopy was performed afterwards. How to Cite: Schiettecatte E, Van Hedent E, Van Herreweghe R, Verstraete K. Mounier-Kuhn Syndrome. Journal of the Belgian Society of Radiology. 2015;98(3):139–40.[jbsr.be]
  • Although her HIV has been well-managed, clinical management of future exacerbations could lead to complicated and extensive infectious disease workups.[cureus.com]
Air Bronchogram
  • The study revealed an air space consolidation involving right upper lobe with air bronchograms appearing as a soft tissue density on soft tissue window [Figure 1] a and [Figure 1] b.[aihbonline.com]
Staphylococcus Aureus
  • She underwent bronchoscopy for further work up and was found to have methicillin-resistant Staphylococcus aureus (MRSA) pneumonia. Incidental nodules were found in her trachea during the bronchoscopy (Figure 1).[swjpcc.com]
Streptococcus Pneumoniae
  • Bronchoalveolar lavage fluid cultures grew Serratia marcescens at Month 1, Moraxella catarrhalis at Month 3, Streptococcus pneumoniae at Month 6, and pan-sensitive P. aeruginosa at Month 12.[atsjournals.org]
Pseudomonas in the Sputum
  • We describe a case of an older lady labelled for many years as COAD but who was a lifelong non-smoker and had Pseudomonas in her sputum. A CT of thorax for suspected bronchiectasis revealed tracheobroncomegaly (Mounier-Kuhn syndrome).[ageing.oxfordjournals.org]

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]
  • The treatment of TO is usually symptomatic. with emphasis on the management and prevention of recurrent respiratory infections.[utsouthwestern.influuent.utsystem.edu]
  • We report on a group of patients with TO and discuss their presentation and their treatment modalities.[link.springer.com]
  • Treatment is mainly supportive. Symptomatic patients may require endobronchial stenting if airway collapse is encountered. Here, we described a patient who presented with hoarseness and pneumonia.[experts.umn.edu]

Prognosis

  • Overall prognosis is generally good and is dependent upon airway stenosis caused by nodules.[radiopaedia.org]
  • Prognosis The impact of this condition on life expectancy has not been evaluated.[orpha.net]

Etiology

  • Etiology The etiology of tracheobronchomegaly is unknown. Some authors have postulated a congenital etiology Only gold members can continue reading.[radiologykey.com]
  • The etiologic mechanism remains unknown. Irritants like cigarette smoke and air pollution could act as irritating factors.[orpha.net]
  • This is the first report which strongly supports granulomatous etiology such as TB as possible etiology for TPO.[lungindia.com]
  • HRCT not only allows the identification of bronchiectasis, determining the extent and type of the disease, but also allows in some cases the establishment of the etiology of bronchiectasis. 1. Habesoglu MA, Ugurlu AO, Eyuboglu FO.[thoracicmedicine.org]

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]
  • Epidemiology Mounier-Kuhn syndrome is most frequently seen in middle age men before the age of 50 1,6. Clinical pr...[wcsjrawq.ga]
Sex distribution
Age distribution

Pathophysiology

  • This is an incidental finding, since from the pathophysiological point of view there is no causal relationship between idiopathic pulmonary fibrosis and Mounier Kuhn's syndrome.[scielo.org.co]
  • The etiology and pathophysiology of TO are still needed to be found. An association between atrophic rhinitis and TO was reported ( 6 ), but the directly relationship still lacks enough evidence.[jtd.amegroups.com]
  • View Article PubMed Google Scholar McCool FD: Global physiology and pathophysiology of cough: ACCP evidence-based clinical practice guidelines. Chest. 2006, 129: 48S-53S. 10.1378/chest.129.1_suppl.48S.[coughjournal.biomedcentral.com]

Prevention

  • The treatment of TO is usually symptomatic. with emphasis on the management and prevention of recurrent respiratory infections.[utsouthwestern.influuent.utsystem.edu]
  • Postoperative continuous positive airway pressure to prevent airway collapse followed awake extubation.[dailyrounds.org]
  • It is important to identify MKS, as early recognition can lead to better management and prevention of complications. Very few cases of MKS with Bronchial Asthma have been reported so far.[ijmrhs.com]
  • 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]
  • Management: There is no permanent and definitive treatment to prevent the formation of the respiratory nodules. In advanced cases, surgical resection or laser therapy of the nodules may be required.[xpertdox.com]

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