BRONCHIECTASIS IN CHILDREN

By- Dr. Yusuf Imranj.n medical collegeamu-Aligarh (India)

INTRODUCTION

Bronchiectasis is the irreversible abnormal

dilatation of bronchi with destruction of elastic and

muscular components of their walls.

ETIOPATHOGENESIS

Bronchiectasis generally results from obstruction

and/or inflammation of the airway.

Chronic infection lead 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.

Bronchiectasis associated with bronchial obstruction

may have a focal distribution distal to the site of

obstruction [ Foreign body] .

 Bronchiectasis associated with underlying disease

is likely to be diffuse [ Cystic Fibrosis].

ETIOLOGY

Bronchiectasis may result from infection, congenital

or acquired disorders, or obstruction.

 All causes share the same pathophysiologic pathway:

ineffective pulmonary toilet and chronic or

recurrent infection and inflammation.

Common infectious causes includes:

1. Severe pneumonia, especially viral, [inadequately

treated]

2. Measles, tuberculosis, pertussis, Adenovirus,

Mycobacterium avium, and Aspergillus

fumigatus infections.

3. HIV infection

Congenital disorders associated with bronchiectasis include the following:

1. CYSTIC FIBROSIS

2. Young syndrome

3. CILIARY DYSKINESIA

4. Marfan syndrome

DISORDERS ASSOCIATED WITH DEFECTIVE DEFENCE-

1. Bruton agammaglobulinemia.

2. Congenital absence of bronchial muscle (Mounier-Kuhn

syndrome) or cartilage (Williams-Campbell syndromes).

3. Immunoglobulin A (IgA) and G (IgG) deficiencies and

IgG subclass deficiencies, especially IgG2 deficiency.

Acquired disorders associated with bronchiectasis include the following:

1. Intrinsic airway luminal obstruction by a bronchial foreign

body or extrinsic compression by mass.

2. Chronic aspiration, which is associated with swallowing

dysfunction, gastroesophageal reflux disease, or

tracheoesophageal fistula.

MISCELLANEOUS CAUSES :

1.Connective tissue disorders, including rheumatoid

arthritis and systemic lupus erythematosus.

2. Allergic bronchopulmonary aspergillosis

3. Tracheal stenosis with impaired mucociliary clearance

4. Fibrosing lung diseases associated with sarcoidosis or

idiopathic pulmonary fibrosis

5. Persistent atelectasis

CLINICAL PRESENTATION

Recurrent cough often productive and longer than 6 weeks, related to posture.

Purulent Sputum, Often large in amount.

Recurrent respiratory infections.

Hemoptysis.

EXAMINATION

MALNUTRITION - Child is usually in poor health due to poor appetite and recurrent infections.

Clubbing may be present. CHEST –coarse leathery crepitations

–localized/generalized.

DIFFERENTIAL DIAGNOSIS

Aspiration Pneumonia.

Non/slowly resolving Bacterial Pneumonia.

Chronic Obstructive Pulmonary Disease.

Emphysema.

DIAGNOSTIC WORKUP

CHEST X RAY: Honeycombing.

CT SCAN: gold standard for diagnosis.

SPUTUM CULTURE OR DEEP OROPHARYNGEAL SWAB.

Serum IgG,IgM and IgA.

Sweat chloride

Ciliary biopsy.

Antinuclear antibody and rheumatoid factor.

Flexible fiberoptic bronchoscopy and bronchoalveolar lavage.

HIV and TB should be ruled out.

MEDICAL MANAGEMENT

Acute exacerbation-

1. Antibiotics to cover both Gram+ ,Gram- as well as anaerobes.

eg- Combination of Cephalosporins, aminoglycosides and metronidazole.

2. Good chest physiotherapy- to drain the secretions.

3. Treatment related to underlying disorders

Eg. Mucolytics, ICS in Cystic fibrosis.

Surgical treatment- Only when medical

management fails or there is a sequestrated lung.

Pulmonary segmental resection.

Transplantation.