eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Bronchiectasis

Author: Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Coauthor(s): Pauline Fani, MD, Fellow, Department of Pediatrics, Division of Pulmonary Medicine, Columbia University Children's Hospital; Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center
Contributor Information and Disclosures

Updated: Sep 8, 2009

Introduction

Background

René Laennec, inventor of the stethoscope, first described bronchiectasis in 1819 while observing patients with tuberculosis and the sequelae of pneumonia in the preantibiotic era. The term bronchiectasis is derived from the Greek bronchion, meaning windpipe, and ektasis, meaning stretched. Bronchiectasis is characterized by the dilatation of bronchi with destruction of elastic and muscular components of their walls. Bronchiectasis can be focal or diffuse. It is usually due to acute or chronic infection or inflammation, anatomic airway obstruction, or underlying congenital disease that predisposes to chronic infection.

The presentation includes recurrent respiratory infections, productive cough, shortness of breath, and occasional hemoptysis. In the developed world, immunizations and antibiotics have led to a declining incidence. In these countries, diffuse bronchiectasis is more often found in association with underlying disorders such as cystic fibrosis (CF), immune deficiencies (including human immunodeficiency virus [HIV]), primary ciliary dyskinesia, and recurrent aspiration syndromes. Focal bronchiectasis is usually associated with bronchial obstruction (ie, foreign body) that leads to infection. In developing countries, bronchiectasis is still frequently encountered as one of the sequelae of acute infection.

This article focuses on children with non-CF bronchiectasis. See Cystic Fibrosis for a more in-depth discussion.

Pathophysiology

Bronchiectasis generally results from obstruction and inflammation of the airway. The obstruction and inflammation may result from any of the underlying disorders listed above or from infection with acute tuberculosis, adenovirus, measles, Mycobacterium avium, or Aspergillus fumigatus.

The damage may result from chronic infection that leads to recruitment of neutrophils, T lymphocytes, and monocyte-derived cytokines. The release of inflammatory mediators, elastases, and collagenases leads to inflammation and destruction of elastic and muscular components of bronchial walls. In addition, the outward elastic recoil forces of surrounding lung parenchyma exert traction, which causes expansion of airway diameter. These changes may be accompanied by bronchial arterial proliferation, which predisposes to hemoptysis.

Bronchiectasis associated with bronchial obstruction may have a focal distribution distal to the site of obstruction. Bronchiectasis associated with underlying disease is likely to be diffuse.

Two different types of bronchiectasis are noted: cylindrical, which is presumably more readily reversible if the underlying disorder can be controlled, and saccular, which is less readily reversible even if the underlying disorder is controlled.

Frequency

United States

Current population-based estimates of occurrence are not available. In 1963, Clark estimated an incidence of 1.06 cases per 10,000 population.¹ The incidence of bronchiectasis associated with underlying systemic disease reflects the incidence of the particular disease. The most common congenital disease associated with bronchiectasis is CF. One study estimates that 110,000 people in the United States have bronchiectasis, including adults.²

International

In developed countries, the frequency is similar to that in the United States. The frequency is higher in the developing world, where measles, pneumonia, tuberculosis, and HIV infection are all on the rise and are associated with bronchiectasis. Childhood national rates have been reported out of London, Finland, New Zealand, and Alaska.

Starting in 1949, Field studied children with bronchiectasis in London for almost 2 decades and documented a fall in the annual hospitalization rate for bronchiectasis in 5 British hospitals, from approximately 48 cases per 10,000 population to 10 cases per 10,000 population.³

Twiss and colleagues reported the incidence of bronchiectasis in children younger than 15 years is 3.7 cases per 100,000 population.⁴ The incidence is highest among children who live in Pacific regions at 17.8 cases per 100,000 population. The incidence is 4.8 cases per 100,000 population in Maori and 1.5 cases per 100,000 in New Zealand compared with 2.4 cases per 100,000 in other Pacific regions. The incidence of bronchiectasis in New Zealand children is nearly twice the rate of CF and 7 times that of Finland. They found variations in incidence among ethnic groups. Most New Zealand children with bronchiectasis developed disease in early childhood and had a delayed diagnosis.

Callahan and associates reported the incidence among Alaskan Native children in the Yuskon-Kuskokwim region to be about 140 cases per 10,000 population, compared with 1 case per 10,000 in other continental US studies.⁵ Redding and colleagues reported the incidence of bronchiectasis in southwest Alaskan Natives is 16 cases per 1,000 population.⁶ In central Australian aborigines, the incidence is 14 cases per 1,000 population, compared with 0.1 cases per 1,000 in Scotland and 4.9 cases per 1,000,000 in Finnish children.

Mortality/Morbidity

Limited morbidity and mortality data are available. In Field's original group, who were studied at the beginning of the antibiotic era, 4% of children with medically treated bronchiectasis died (mostly from infection), and 3% of children who were surgically treated died (many immediately following or as a late result of surgery) in the ensuing 2 decades.⁷ Karadag and associates illustrated that bronchiectasis remains one of the most common causes of childhood morbidity in developing countries.⁸ Twiss and colleagues recently demonstrated that children with bronchiectasis have significant airway obstruction that deteriorates over time.⁹ However, Karadag and associates demonstrated that children with non-CF bronchiectasis have a much slower decline in lung function than children with CF.³ Akalin and colleagues reported decreased left ventricular function and exercise capacity in bronchiectasis.¹⁰

Race

Bronchiectasis is more common in patients of Polynesian and Alaskan Native ancestry. Among 26,000 Samoans in Western Samoa, the prevalence of bronchiectasis was 0.6%, probably the highest of any population in the world. Karadag and associates' study in Turkey suggests possible genetic predisposition in some populations and found that 43% of children with bronchiectasis had parents who were first-degree or second-degree relatives.⁸

Sex

Morrissey and colleagues found non-CF bronchiectasis to be more common and more virulent in women.¹¹

Age

Karadag and colleagues reported a mean age at presentation of 7.4 ± 3.7 years.⁸ In Field's 1949 survey, 15% of patients presented when younger than 2 years, 43% when aged approximately 2 years, and 92% when younger than 10 years.⁷ These data predate most current immunizations and antibiotics. In Clark's 1963 series, one half of the children developed symptoms when younger than 3 years.¹

Clinical

History

• Cough
  • Non–cystic fibrosis (CF) bronchiectasis in children presents with a wide spectrum of disease severity. Some children have intermittent symptoms of cough and occasional lower respiratory tract infections. Others experience daily cough and produce purulent fetid sputum, requiring frequent hospitalizations for respiratory exacerbations.
  • The diagnosis should be considered in children with a daily productive cough for longer than 6 weeks. Bronchiectasis should also be considered in children with chronic or persistent cough in whom another diagnosis has been made but who are not responding to therapy. If children with cough respond to antibiotics on a recurrent basis, bronchiectasis should be considered.
  • Recurrent cough with fetid sputum, hemoptysis, or recurrent pneumonia are important clues to early diagnosis of this disease. Ensure that pneumonia clears on radiography findings; follow-up radiography is indicated 6-8 weeks after the acute infection occurs.
  • Cough is an almost universal symptom and is frequently described as productive in older children or loose in toddlers and infants. Because small children rarely expectorate, the clinician may observe the child with a loose-sounding cough who swallows after coughing.
  • Although cough is a cardinal symptom in bronchiectasis, Smyrnios and colleagues concluded that cough is much more common in patients with asthma (24%), gastroesophageal reflux disease (15%), and bronchitis (11%) than in patients with bronchiectasis (4%). However, if children with GERD or asthma do not respond to therapy, bronchiectasis should be considered. Furthermore, recurrent aspiration can lead to bronchiectasis, and allergic bronchopulmonary aspergillosis (a complication of asthma) includes bronchiectasis.
• Other
  • Tsao and associates reported that hemoptysis is the second most common symptom of bronchiectasis.¹²
  • The diagnosis of asthma or reflux-associated lung disease does not preclude bronchiectasis. In fact, bronchiectasis may be a complication of these diseases.
  • An underlying systemic disease or congenital anatomical malformations such as tracheoesophageal fistula or history of foreign body aspiration in conjunction with respiratory symptoms should increase suspicion for bronchiectasis.
  • Bronchiectasis can be a complication of recurrent respiratory infections associated with the entities listed below. 

Physical

• Physical examination findings in patients with bronchiectasis may include variable degrees of crackles or coarse rhonchi and clubbing. However, the examination findings may be normal. Lai and colleagues reported crackles and wheezing as the most frequent findings of the physical examination.¹³ An inspiratory "honk" has been described in some children with bronchiectasis, the etiology of which is unclear.
• Digital clubbing is reported in 37-51% of patients with bronchiectasis. Edwards and associates found that children with digital clubbing and chest deformity showed significantly higher scores for extent of bronchiectasis, bronchial wall dilatation and thickness, and overall changes based on CT score.¹⁴ In Field's 1949 series, clubbing was present in 78 cases (43.7%).⁷ In many of her cases, the clubbing cleared after the affected section of the lung was surgically removed. In medically treated cases, clubbing often improved and, in some cases, disappeared despite persistent bronchographic evidence of bronchiectasis. Although Field concluded that clubbing in the absence of congenital heart disease signifies irreversible bronchiectasis, a myriad of entities are now known to cause clubbing.

Causes

All causes share the same pathophysiologic pathway: ineffective pulmonary toilet and chronic or recurrent infection and inflammation. Common causes include the following:

• Severe pneumonia
• Measles, tuberculosis, pertussis, M avium, and A fumigatus infections
• Intrinsic airway luminal obstruction by bronchial foreign body or extrinsic compression by mass
• Chronic aspiration, which is associated with swallowing dysfunction, gastroesophageal reflux disease, or tracheoesophageal fistula
• Congenital conditions, including CF, Young syndrome, ciliary dyskinesia, Marfan syndrome, Bruton agammaglobulinemia, and congenital absence of bronchial muscle (Mounier-Kuhn syndrome) or cartilage (Williams-Campbell syndromes)
• Immunoglobulin A (IgA) and G (IgG) deficiencies and IgG subclass deficiencies, especially IgG2 deficiency
• Connective tissue disorders, including rheumatoid arthritis and systemic lupus erythematosus
• HIV infection: Children who develop lymphocytic interstitial pneumonitis seem at increased risk of subsequent bronchiectasis.
• Allergic bronchopulmonary aspergillosis
• Tracheal stenosis with impaired mucociliary clearance
• Severe tracheomalacia with impairment of mucociliary clearance
• Fibrosing lung diseases associated with sarcoidosis or idiopathic pulmonary fibrosis
• Persistent atelectasis
• Retained airway foreign body

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