DOI: 10.14260/jemds/2015/71 CASE REPORT J of Evolution of Med and Dent Sci/ eISSN- 2278-4802, pISSN- 2278-4748/ Vol.4/ Issue 03/Jan 08, 2015 Page 485 MOUNIER KUHN SYNDROME: A RARE CAUSE OF BRONCHIECTASIS Sachin Khanduri 1 , Deepak Agrawal 2 , Bhumika Singh 3 , Garima Varshney 4 , Fahimul Huda 5 HOW TO CITE THIS ARTICLE: Sachin Khanduri, Deepak Agrawal, Bhumika Singh, GarimaVarshney, Fahimul Huda. “Mounier Kuhn Syndrome: A Rare Cause of Bronchiectasis”.Journal of Evolution of Medical and Dental Sciences 2015; Vol. 4, Issue 03 January 08; Page: 485-488, DOI:10.14260/jemds/2015/71 ABSTRACT: Mounier Kuhn syndrome, also known as tracheobronchomegaly is a rare idiopathic 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. The diagnosis is easily missed as tracheobronchomegaly is overlooked on plain chest x-ray. Here we present an interesting report of our patient who presented with recurrent cough and shortness of breath. A diagnosis of bronchiectasis was first thought of. Computed tomography scan revealed a final diagnosis of this rare disorder. Management of the disease is only supportive and depends on the severity of the condition. KEYWORDS: Mounier Kuhn, Tracheobronchomegaly, Bronchiectasis, Computed tomography. INTRODUCTION: Mounier Kuhn syndrome or tracheobronchiomegaly also known as tracheocele, tracheobronchiectasis and tracheomalacia is a very rare entity presenting as recurrent lower respiratory tract infection. (1) Due to this nonspecific presentation it is a possible differential for various diseases. The focal findings of the condition being dilatation of trachea along with mainstem bronchi, down till the 4 th bronchi level. Bronchiectasis is the usual diagnosis in these cases. It is diagnosed on x-ray, bronchoscopy but decisively on computed tomography scan. CASE REPORT: A 28 year old male came to our outpatient department with cough, fever, dyspnoea and chest pain since one month. There had been episodic exacerbations of these complaints since three years. 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. On examination, there was tachycardia, tachypnoea and reduced oxygen saturation (SpO2 84%). 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. 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. Renal and liver function tests were also within normal range. On radiological examination, chest x-ray showed prominent trachea with emphysematous lung fields. The hilum and pleural spaces were normal. Areas of honeycomb shadowing in bilateral lower zones were also noted. Patchy areas of fibrotic opacities were noted on the left lower zones. Computed tomography scans of the chest (Plain) revealed dilated trachea measuring approximately 3 cm with dilated mainstem bronchi (Figure 1). Multiple sacculations were noted in the proximal part of thoracic part of trachea involving posterolateral walls (Figure 2& 3). Bilateral lung fields were hyperinflated with retrosternal and prevertebral herniations of lung fields with areas of air trapping in both lung fields with centiacinar emphysematous changes with few paraseptal changes. Patchy