TOPIC: Imaging TYPE: Medical Student/Resident Case Reports INTRODUCTION: Mounier-Kuhn syndrome (MKS), also known as tracheobronchomegaly is a rare cause of proximal bronchiectasis which is characterized by deficient smooth muscle and elastic fibers and absence of myenteric plexus in the proximal airway wall. Less than 400 cases of MKS have been reported (1) (2). We present a patient with numerous hospitalizations due to recurrent dyspnea and pneumonia spanning twenty years who met chest CT criteria for MKS. CASE PRESENTATION: 44-year-old African American male, a former smoker, presented with worsening dyspnea and productive cough for 2 days. He has had similar symptoms for 20 years. Examination revealed clubbing of extremities and coarse auscultation crackles bilaterally. Chest x-ray revealed right upper lobe emphysematous changes and bilateral diffuse central opacities with cystic changes. CT chest revealed para-septal emphysema and extensive proximal cystic bronchiectasis bilaterally. Blood tests were negative for HIV, connective tissue disorders, autoimmune disorders and immunodeficiency. The absence of asthma, patient's ethnicity, having biological children and absence of other organ damage, made cystic fibrosis (CF) and allergic bronchopulmonary aspergillosis (ABPA) unlikely. The trachea, left and right mainstem bronchus measured at 30mm, 25mm, 27mm on chest CT with outpouching noted on proximal trachea, which led to the diagnosis of MKS. DISCUSSION: Proximal bronchiectasis is rare. Acquired causes are ABPA, CF, and congenital causes are MKS and William Campbell Syndrome. MKS is a rare congenital abnormality characterized by tracheobronchial dilation due to atrophy of elastic fibers and thinning of smooth muscle layer in the trachea and main bronchi (2) up to 1st to 4th bronchial generations. Clinical manifestations include that of chronic bronchitis and bronchiectasis with recurrent lower respiratory infection. Subtypes include 1) tracheobronchomegaly alone, 2) tracheobronchomegaly with diverticula in the proximal trachea as seen in our patient, 3) tracheobronchomegtly with diverticula extending to lower bronchi. The gold standard diagnostic test for MKS is CT chest. Diagnostic criteria are trachea > 30mm, right main bronchus > 20-24mm, and left main bronchus >18-23mm (3). Airway wall weakness can lead to extensive dynamic airway occlusion further accentuating symptoms. Treatments rely on daily frequent chest physiotherapy with postural drainage, antibiotics during infections, and immunization to help prevent infections and further airway damage. CONCLUSIONS: In patients with bronchiectasis, early diagnosis of tracheobronchomegaly using chest CT criteria will allow for implementation of treatment strategies that will hopefully prevent recurrent infection and further airway damage. REFERENCE #1: Mounier-Kuhn syndrome in a Namibian female patient. Mkandawire MJ, Muramira NM, Mraba N. s.l. : Pan Afr Med J, 2020. REFERENCE #2: Tracheobronchomegaly. The Mounier-Kuhn Syndrome. Katz I, Levine M, Herman P. s.l. : Am Journal Roentgenol Radium Ther Nucl Med. 88:1084-1094. REFERENCE #3: Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications. Balakrishnan, Menon, et al. 1, s.l. : Southern Medical Journal, 2008, Vol. 101. pp.83-87. DISCLOSURES: No relevant relationships by Thomas George, source=Web Response No relevant relationships by Padmanabhan Krishnan, source=Web Response No relevant relationships by Nikisha Pandya, source=Web Response No relevant relationships by Gurjit Inder Sidhu, source=Web Response No relevant relationships by Sarita Singh, source=Admin input No relevant relationships by Fardad Zaghi, source=Web Response
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