eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Bronchiectasis

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

Updated: Jan 22, 2007

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, 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.

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.

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. 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.

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 (Field, 1969).
• Twiss et al (2005) reported the incidence of bronchiectasis in children younger than 15 years is 3.7 per 100,000 population. The incidence is highest among children who live in Pacific regions at 17.8 per 100,000 population. The incidence is 4.8 per 100,000 population in Maori and 1.5 per 100,000 in New Zealand compared with 2.4 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 et al (2005) reported the incidence among Alaskan Native children in the Yuskon-Kuskokwim region to be about 140 cases per 10,000 population, compared with 1 per 10,000 in other continental US studies. Redding et al (2004) reported the incidence of bronchiectasis in southwest Alaskan Natives is 16 per 1,000 population.
• In central Australian aborigines, the incidence is 14 per 1,000 population, compared with 0.1 per 1,000 in Scotland and 4.9 per 1,000,000 in Finnish children (Redding, 2004).

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 (9-10 from infection), and 3% of children who were surgically treated died (8-9 immediately following or as a late result of surgery) in the ensuing 2 decades.
• Karadag et al (2005) illustrated that bronchiectasis remains one of the most common causes of childhood morbidity in developing countries.
• Twiss et al recently demonstrated that children with bronchiectasis have significant airway obstruction that deteriorates over time. However, Karadag et al demonstrated that children with CF have a more rapid decline in forced expiratory volume at one second (FEV₁) than children with non-CF bronchiectasis.
• Akalin et al reported decreased left ventricular function and exercise capacity in bronchiectasis. In children with CF, ventilation perfusion mismatch and hypoxemia may result in cor pulmonale.

Race

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

Sex

Morrissey et al found non-CF bronchiectasis to be more common and more virulent in women.

Age

• Karadag et (2005) reported a mean age at presentation of 7.4 ±3.7 years. In a survey of newborns diagnosed with CF, Farrell et al found potentially irreversible abnormalities in one half of the children by age 2 years.
• 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. 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.

Clinical

History

• Cough
  • Non-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.
  • Recurrent cough with fetid sputum, hemoptysis, or recurrent pneumonia are important clues to early diagnosis of this disease.
  • 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.
  • While cough is a cardinal symptom in bronchiectatic disease, Smyrnios et al concluded that cough is much more common in patients with asthma (24%), gastroesophageal reflux disease (GERD) (15%), and bronchitis (11%) than in patients with bronchiectasis (4%).
• Other
  • Tsao et al (2002) reported hemoptysis to be the second most common symptom of bronchiectasis.
  • 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.

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 et al (2004) reported crackles and wheezing as the most frequent findings of the physical examination.
• Digital clubbing is reported in 37-51% of patients with bronchiectasis. Edwards et al (2003) 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. In Field's 1949 series, clubbing was present in 78 cases (43.7%). 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. Field concluded that clubbing in the absence of congenital heart disease signifies irreversible bronchiectasis. This concept is not universally held.

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, GERD, or tracheoesophageal fistula
• Congenital conditions, including CF, 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
• 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

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