Clinical manifestations and evaluation of bronchiectasis in children
- Khoulood Fakhoury, MD
Khoulood Fakhoury, MD
- Assistant Professor of Pediatrics
- Baylor College of Medicine
- Adaobi Kanu, MD
Adaobi Kanu, MD
- Associate Professor of Pediatrics
- Texas Tech Health Sciences Center
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. Associated findings include atelectasis, emphysema, fibrosis, and hypertrophy of the bronchial vasculature.
In developed nations, cystic fibrosis (CF) is the most common cause of bronchiectasis in children. The evaluation of CF-related bronchiectasis is presented elsewhere. (See "Cystic fibrosis: Clinical manifestations and diagnosis" and "Cystic fibrosis: Clinical manifestations of pulmonary disease".)
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. Infections and acquired causes of bronchiectasis are more common in adults and in developing nations, whereas congenital anomalies of the bronchi or immune system are more prominent in children and in industrialized nations.
This topic review will outline the clinical manifestations and evaluation of non-CF-related bronchiectasis in children. The causes and management of non-CF-related bronchiectasis in children, and the clinical manifestations and diagnosis of bronchiectasis in adults are discussed separately. (See "Causes of bronchiectasis in children" and "Management of bronchiectasis in children without cystic fibrosis" and "Clinical manifestations and diagnosis of bronchiectasis in adults".)
The most common symptom in children with bronchiectasis is persistent cough, which is present in 80 to 90 percent of pediatric patients with bronchiectasis, and is typically "wet" or productive [1,2]. Fifty-seven to 74 percent of children also produce purulent sputum. The absence of sputum production does not exclude bronchiectasis because young children may not be able to expectorate sputum.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Karakoc GB, Yilmaz M, Altintas DU, Kendirli SG. Bronchiectasis: still a problem. Pediatr Pulmonol 2001; 32:175.
- CLARK NS. Bronchiectasis in childhood. Br Med J 1963; 1:80.
- 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.
- Sethi GR, Batra V. Bronchiectasis: causes and management. Indian J Pediatr 2000; 67:133.
- Kumar NA, Nguyen B, Maki D. Bronchiectasis: current clinical and imaging concepts. Semin Roentgenol 2001; 36:41.
- Marwah OS, Sharma OP. Bronchiectasis. How to identify, treat, and prevent. Postgrad Med 1995; 97:149.
- Cockrill BA, Hales CA. Allergic bronchopulmonary aspergillosis. Annu Rev Med 1999; 50:303.
- Bolman RM 3rd, Wolfe WG. Bronchiectasis and bronchopulmonary sequestration. Surg Clin North Am 1980; 60:867.
- 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.
- Van Schoor J, Joos G, Pauwels R. Tracheobronchomegaly--the Mounier-Kuhn syndrome: report of two cases and review of the literature. Eur Respir J 1991; 4:1303.
- 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.
- Kollée LA, van Heeswijk PJ, Schretlen ED. Unilateral hyperlucent lung with decreased vascular markings (Swyer-James syndrome). Padiatr Padol 1975; 10:10.
- Smith IE, Flower CD. Review article: imaging in bronchiectasis. Br J Radiol 1996; 69:589.
- Rencken I, Patton WL, Brasch RC. Airway obstruction in pediatric patients. From croup to BOOP. Radiol Clin North Am 1998; 36:175.
- Piccione JC, McPhail GL, Fenchel MC, et al. Bronchiectasis in chronic pulmonary aspiration: risk factors and clinical implications. Pediatr Pulmonol 2012; 47:447.
- Pizzutto SJ, Grimwood K, Bauert P, et al. Bronchoscopy contributes to the clinical management of indigenous children newly diagnosed with bronchiectasis. Pediatr Pulmonol 2013; 48:67.