Ayşe MURAT AYDIN, Ahmet Kürşad POYRAZ, HAKAN ARTAŞ, Teyfik TURGUT

Tracheobronchomegaly (Mounier-Kuhn Syndrome)

belirgin kistik trakeobronşiyal dilatasyon olarak tanımlanır. Trakeobronşiomegali (TBM) öncelikle 40 ila 50 yaş arasındaki erkekleri etkileyen nadir görülen bir durumdur. TBM etyolojisi bilinmemektedir. Patolojik olarak trakea ve santral bronşların kas ve elastik elemanlarının atrofisi vardır. TBM ve solunum sisteminin ilgili patolojisinin tanısı esas olarak düz akciğer grafileri ve BT gibi radyolojik incelemelere dayanmaktadır. Tekrarlayan alt solunum yolu enfeksiyonları öyküsü olan TBMli iki olgu sunulmuştur.

Trakeobronşiomegali (Mounier-Kuhn sendromu)

ABSTRACT Tracheobronchomegaly (Mounier-Kuhn syndrome) is defined as marked cystic tracheobronchial dilatation. Tracheobronchomegaly (TBM) is a rare condition primarily effecting men between the ages of 40 to 50 years. The etiology of TBM is unknown. Pathologically, there is atrophy of the muscular and elastic elements of the trachea and central bronchi. The diagnosis of TBM and the associated pathology of the respiratory system are primarily based on radiographic examinations such as plain chest radiographs and CT scans. We report two cases of TBM with a history of recurrent lower respiratory tract infections.

PDF

___

Benesch M, Eber E, Pfleger A, Zach MS. Recurrent lower respiratory tract infections in a 14-year-old boy with tracheobronchomegaly (Mounier-Kuhn syndrome). Pediatr Pulmonol 2000; 29: 476-9.
Krustins E, Kravale Z, Buls A. Mounier-Kuhn syndrome or congenital tracheobronchomegaly: A literature review. Respir Med 2013; 107: 1822-8.
Katz I, LeVine M, Herman P. Tracheobronchiomegaly. The Mounier-Kuhn syndrome. Am J Roentgenol Radium Ther Nucl Med 1962; 88: 1084-94.
Marom EM, Goodman PC, McAdams HP. Diffuse abnormalities of the trachea and main bronchi. AJR Am J Roentgenol 2001; 176: 713-7.
Hubbard M, Masters B, Chang AB. Rapidly progressing Case of Mounier-Kuhn syndrome in early childhood. Pediatr Pulmonol 2003; 36: 353-6.
Woodring JH, Howard RS 2nd, Rehm SR. Congenital tracheobronchomegaly (Mounier-Kuhn syndrome): a report of 10 cases and review of the literature. J Thorac Imaging 1991; 6: 1-10.
Hunter TB, Kuhns LR, Roloff MA, Holt JF. Tracheobronchomegaly in an 18 month old child. AJR Am J Roentgenol 1975; 123: 687-90. Shivaram U, Shivaram I, Cash M. Acquired tracheobronchomegaly resulting in respiratory failure. Chest 1990; 98: 491-2.
Grünebaum M, Kornreich L, Horev G, Ziv N. Tracheobronchomegaly in Brachmann-de Lange Syndrome. Pediatr Radiol 1996; 26: 184-7.
Ayres JG, Pope FM, Reidy JF, Clarck TJH. Abnormalities of the lungs and thoracic cage in the Ehlers-Danlos syndrome. Thorax 1985; 40: 300-5.
Lazzarini-de-Oliveira LC, Costa de Barros Franco CA, Gomes de Salles CL, de Oliveira AC Jr. A 38-year-old man with tracheomegaly, tracheal diverticulosis, and bronchiectasis. Chest 2001; 120: 1018-20.
Noori F, Abduljawad S, Suffin DM, et al. Mounier-Kuhn syndrome: a case report. Lung 2010; 188: 353-4. Gurney JW. The pathophysiology of airways disease. J Thorac Imaging 1995; 10: 227-35. 14. 15. 16. Celik B, Bilgin S, Yuksel C. Mounier-Kuhn syndrome: a rare cause of bronchial dilation. Tex Heart Inst J 2011; 38: 194-6. Schwartz M, Rossoff L. Tracheobronchomegaly. Chest 1994; 106: 1589-90.
Subramani S, Freeman B, Rajagopal S. Anesthetic Considerations for Bilateral Lung Transplantation in Mounier- Kuhn Syndrome. J Cardiothorac Vasc Anesth 2014; 1053- 0770: 605-8.