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Causes of bronchiectasis in children

Khoulood Fakhoury, MD
Adaobi Kanu, MD
Section Editor
George B Mallory, MD
Deputy Editor
Alison G Hoppin, MD


Bronchiectasis is a structural abnormality characterized by abnormal dilation and distortion of the bronchial tree, resulting in chronic obstructive lung disease. This condition is typically the end result of a variety of pathophysiologic processes that render the bronchial walls weakened, easily collapsible, chronically inflamed, and plugged with mucus secretions.

In resource-rich countries, cystic fibrosis (CF) is the most common cause of bronchiectasis in children. The evaluation and management of CF-related bronchiectasis is discussed in detail in separate topic reviews. (See "Cystic fibrosis: Clinical manifestations and diagnosis" and "Cystic fibrosis: Clinical manifestations of pulmonary disease" and "Cystic fibrosis: Overview of the treatment of lung disease" and "Cystic fibrosis: Antibiotic therapy for chronic pulmonary infection".)

Bronchiectasis can be caused by a variety of disease processes other than CF, most of which include some combination of bronchial obstruction and infection. The types of disorders that cause bronchiectasis vary among populations and age groups. As examples, infections and acquired causes of bronchiectasis predominate in adults and in resource-limited countries, whereas congenital anomalies of the airways or immune system are more prominent in children and resource-rich countries.

This topic review will outline the pathogenesis and main causes of non-CF related bronchiectasis in children. The evaluation and management of non-CF bronchiectasis in children, and the causes of bronchiectasis in adults are discussed in separate topic reviews. (See "Clinical manifestations and evaluation of bronchiectasis in children" and "Management of bronchiectasis in children without cystic fibrosis" and "Clinical manifestations and diagnosis of bronchiectasis in adults".)


Prevalence rates vary substantially depending on the method used for case identification. Use of high-resolution computed tomography (CT) will detect milder disease, and result in higher prevalence estimates than strategies employing less sensitive forms of radiography.

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Literature review current through: Nov 2017. | This topic last updated: Sep 12, 2016.
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  1. Kumar NA, Nguyen B, Maki D. Bronchiectasis: current clinical and imaging concepts. Semin Roentgenol 2001; 36:41.
  2. Marwah OS, Sharma OP. Bronchiectasis. How to identify, treat, and prevent. Postgrad Med 1995; 97:149.
  3. Säynäjäkangas O, Keistinen T, Tuuponen T, Kivelä SL. Evaluation of the incidence and age distribution of bronchiectasis from the Finnish hospital discharge register. Cent Eur J Public Health 1998; 6:235.
  4. Twiss J, Metcalfe R, Edwards E, Byrnes C. New Zealand national incidence of bronchiectasis "too high" for a developed country. Arch Dis Child 2005; 90:737.
  5. Weycker D, Edelsberg J, Oster G, et al. Prevalence and economic costs of bronchiectasis. American Thoracic Society International Conference, May 21–26 2004, Orlando, Florida, USA. Amer J Resp Crit Care Med 2004; 169 (7 supplement):330.
  6. Waite DA, Wakefield SJ, Mackay JB, Ross IT. Mucociliary transport and ultrastructural abnormalities in Polynesian bronchiectasis. Chest 1981; 80:896.
  7. Chang AB, Grimwood K, Mulholland EK, et al. Bronchiectasis in indigenous children in remote Australian communities. Med J Aust 2002; 177:200.
  8. Singleton R, Morris A, Redding G, et al. Bronchiectasis in Alaska Native children: causes and clinical courses. Pediatr Pulmonol 2000; 29:182.
  9. Munro KA, Reed PW, Joyce H, et al. Do New Zealand children with non-cystic fibrosis bronchiectasis show disease progression? Pediatr Pulmonol 2011; 46:131.
  10. Singleton RJ, Valery PC, Morris P, et al. Indigenous children from three countries with non-cystic fibrosis chronic suppurative lung disease/bronchiectasis. Pediatr Pulmonol 2014; 49:189.
  11. Karadag B, Karakoc F, Ersu R, et al. Non-cystic-fibrosis bronchiectasis in children: a persisting problem in developing countries. Respiration 2005; 72:233.
  12. Goyal V, Grimwood K, Marchant J, et al. Pediatric bronchiectasis: No longer an orphan disease. Pediatr Pulmonol 2016; 51:450.
  13. Osika E, Cavaillon JM, Chadelat K, et al. Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease. Eur Respir J 1999; 14:339.
  14. Fiel, SB. Bronchiectasis: the changing clinical scenario. J Respir Dis 2000; 21:666.
  15. Sethi GR, Batra V. Bronchiectasis: causes and management. Indian J Pediatr 2000; 67:133.
  16. REID LM. Reduction in bronchial subdivision in bronchiectasis. Thorax 1950; 5:233.
  17. Mysliwiec V, Pina JS. Bronchiectasis: the 'other' obstructive lung disease. Postgrad Med 1999; 106:123.
  18. Cohen M, Sahn SA. Bronchiectasis in systemic diseases. Chest 1999; 116:1063.
  19. Gaillard EA, Carty H, Heaf D, Smyth RL. Reversible bronchial dilatation in children: comparison of serial high-resolution computer tomography scans of the lungs. Eur J Radiol 2003; 47:215.
  20. Redding GJ. Bronchiectasis in children. Pediatr Clin North Am 2009; 56:157.
  21. Eastham KM, Fall AJ, Mitchell L, Spencer DA. The need to redefine non-cystic fibrosis bronchiectasis in childhood. Thorax 2004; 59:324.
  22. Crowley S, Matthews I. Resolution of extensive severe bronchiectasis in an infant. Pediatr Pulmonol 2010; 45:717.
  23. Javidan-Nejad C, Bhalla S. Bronchiectasis. Radiol Clin North Am 2009; 47:289.
  24. Brower KS, Del Vecchio MT, Aronoff SC. The etiologies of non-CF bronchiectasis in childhood: a systematic review of 989 subjects. BMC Pediatr 2014; 14:4.
  25. Gould CM, Freeman AF, Olivier KN. Genetic causes of bronchiectasis. Clin Chest Med 2012; 33:249.
  26. Jones VF, Eid NS, Franco SM, et al. Familial congenital bronchiectasis: Williams-Campbell syndrome. Pediatr Pulmonol 1993; 16:263.
  27. Rencken I, Patton WL, Brasch RC. Airway obstruction in pediatric patients. From croup to BOOP. Radiol Clin North Am 1998; 36:175.
  28. Cataneo AJ, Reibscheid SM, Ruiz Júnior RL, Ferrari GF. Foreign body in the tracheobronchial tree. Clin Pediatr (Phila) 1997; 36:701.
  29. Karakoc GB, Yilmaz M, Altintas DU, Kendirli SG. Bronchiectasis: still a problem. Pediatr Pulmonol 2001; 32:175.
  30. De Boeck K, Willems T, Van Gysel D, et al. Outcome after right middle lobe syndrome. Chest 1995; 108:150.
  31. Sekerel BE, Nakipoglu F. Middle lobe syndrome in children with asthma: review of 56 cases. J Asthma 2004; 41:411.
  32. Priftis KN, Mermiri D, Papadopoulou A, et al. The role of timely intervention in middle lobe syndrome in children. Chest 2005; 128:2504.
  33. Eaton T, Garrett J, Milne D, et al. Allergic bronchopulmonary aspergillosis in the asthma clinic. A prospective evaluation of CT in the diagnostic algorithm. Chest 2000; 118:66.
  34. Wark PA, Gibson PG. Allergic bronchopulmonary aspergillosis: new concepts of pathogenesis and treatment. Respirology 2001; 6:1.
  35. Sheikh S, Madiraju K, Steiner P, Rao M. Bronchiectasis in pediatric AIDS. Chest 1997; 112:1202.
  36. Pursner M, Haller JO, Berdon WE. Imaging features of Mycobacterium avium-intracellulare complex (MAC) in children with AIDS. Pediatr Radiol 2000; 30:426.
  37. Gadola SD, Moins-Teisserenc HT, Trowsdale J, et al. TAP deficiency syndrome. Clin Exp Immunol 2000; 121:173.
  38. Donato L, de la Salle H, Hanau D, et al. Association of HLA class I antigen deficiency related to a TAP2 gene mutation with familial bronchiectasis. J Pediatr 1995; 127:895.
  39. Doğru D, Ozbaş Gerçeker F, Yalçin E, et al. The role of TAP1 and TAP2 gene polymorphism in idiopathic bronchiectasis in children. Pediatr Pulmonol 2007; 42:237.
  40. Buckley RH. Immunodeficiency diseases. JAMA 1992; 268:2797.
  41. Cowan MJ, Gladwin MT, Shelhamer JH. Disorders of ciliary motility. Am J Med Sci 2001; 321:3.
  42. de Iongh R, Ing A, Rutland J. Mucociliary function, ciliary ultrastructure, and ciliary orientation in Young's syndrome. Thorax 1992; 47:184.
  43. Neville E, Brewis R, Yeates WK, Burridge A. Respiratory tract disease and obstructive azoospermia. Thorax 1983; 38:929.
  44. Hendry WF, A'Hern RP, Cole PJ. Was Young's syndrome caused by exposure to mercury in childhood? BMJ 1993; 307:1579.
  45. Arya AK, Beer HL, Benton J, et al. Does Young's syndrome exist? J Laryngol Otol 2009; 123:477.
  46. Ichioka K, Kohei N, Okubo K, et al. Obstructive azoospermia associated with chronic sinopulmonary infection and situs inversus totalis. Urology 2006; 68:204.e5.
  47. Kim CK, Chung CY, Kim JS, et al. Late abnormal findings on high-resolution computed tomography after Mycoplasma pneumonia. Pediatrics 2000; 105:372.
  48. CLARK NS. Bronchiectasis in childhood. Br Med J 1963; 1:80.
  49. Pasteur MC, Helliwell SM, Houghton SJ, et al. An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000; 162:1277.
  50. Similä S, Linna O, Lanning P, et al. Chronic lung damage caused by adenovirus type 7: a ten-year follow-up study. Chest 1981; 80:127.
  51. Sly PD, Soto-Quiros ME, Landau LI, et al. Factors predisposing to abnormal pulmonary function after adenovirus type 7 pneumonia. Arch Dis Child 1984; 59:935.
  52. Daniel TL, Woodring JH, Vandiviere HM, Wilson HD. Swyer-James syndrome--unilateral hyperlucent lung syndrome. A case report and review. Clin Pediatr (Phila) 1984; 23:393.
  53. Kollée LA, van Heeswijk PJ, Schretlen ED. Unilateral hyperlucent lung with decreased vascular markings (Swyer-James syndrome). Padiatr Padol 1975; 10:10.
  54. Kim CK, Koh JY, Han YS, et al. Swyer-James Syndrome with finger clubbing after severe measles infection. Pediatr Int 2008; 50:413.
  55. El-Serag HB, Gilger M, Kuebeler M, Rabeneck L. Extraesophageal associations of gastroesophageal reflux disease in children without neurologic defects. Gastroenterology 2001; 121:1294.
  56. Pitney AC, Callahan CW, Ruess L. Reversal of bronchiectasis caused by chronic aspiration in cri du chat syndrome. Arch Dis Child 2001; 85:413.
  57. Piccione JC, McPhail GL, Fenchel MC, et al. Bronchiectasis in chronic pulmonary aspiration: risk factors and clinical implications. Pediatr Pulmonol 2012; 47:447.
  58. Kim JS, Lee KS, Koh EM, et al. Thoracic involvement of systemic lupus erythematosus: clinical, pathologic, and radiologic findings. J Comput Assist Tomogr 2000; 24:9.
  59. Fenlon HM, Doran M, Sant SM, Breatnach E. High-resolution chest CT in systemic lupus erythematosus. AJR Am J Roentgenol 1996; 166:301.
  60. Moffat RE, Sobonya RE, Chang CH. Childhood sarcoidosis with fatal cor pulmonale. Pediatr Radiol 1978; 7:180.
  61. Udwadia ZF, Pilling JR, Jenkins PF, Harrison BD. Bronchoscopic and bronchographic findings in 12 patients with sarcoidosis and severe or progressive airways obstruction. Thorax 1990; 45:272.
  62. Wood JR, Bellamy D, Child AH, Citron KM. Pulmonary disease in patients with Marfan syndrome. Thorax 1984; 39:780.
  63. Greenstein AJ, Janowitz HD, Sachar DB. The extra-intestinal complications of Crohn's disease and ulcerative colitis: a study of 700 patients. Medicine (Baltimore) 1976; 55:401.
  64. Camus P, Piard F, Ashcroft T, et al. The lung in inflammatory bowel disease. Medicine (Baltimore) 1993; 72:151.
  65. Türktaş I, Bostanci I, Altuntaş B. Rapidly progressive bronchiectasis complicating ulcerative colitis in a child. Turk J Pediatr 2001; 43:151.