Bronchiectasis

           Bronchiectasis

Bronchiectasis the term used to describe abnormal dilation of the bronchi. It is usually acquired but may result from an underlying genetic or congenital defect of airway defences.

Or
Abnormal dilatation of the bronchi, usually accompanied by recurrent bronchial suppuration.

Or
Bronchiectasis is an abnormal dilatation of the bronchi associated with obstruction and infection.

Or,
Bronchiectasis is a disease state defined by localized, irreversible dilation of part of the bronchial tree caused by destruction of the muscle and elastic tissue. It is classified as an obstructive lung disease, along with emphysema, bronchitis, asthma, and cystic fibrosis.
Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airway obstruction and impaired clearance of secretions. Bronchiectasis may result from a variety of infective and acquired causes, including severe and recurrent pneumonia, tuberculosis, and cystic fibrosis.
Causes:

[A]Congenital:
1.Cystic fibrosis.
2. Ciliary dysfunction syndromes:
• Immotile cilia syndrome.
• Kartagener’s syndrome.
3. Primary hypo-gamma-globulinaemia.

[B]Acquired:
1. In children:
• Pneumonia (post measles, whooping cough)
• Primary tuberculosis.
• Inhaled foreign body.
2. In adults:
• Suppurative pneumonia.
• Pulmonary tuberculosis.
• Pulmonary oesinophilia.
• Bronchial tumours.

Pathogenesis: Bronchiectasis is usually caused by chronic inflammation, infection and obstruction of bronchi and bronchioles,
1. Excessive accumulation of secretion e.g.
a) In ciliary dysfunctions syndrome.
b) Cystic fibrosis.
2. Recurrent infection & inflammation of bronchi & bronchioles.
3. Obstruction of bronchi causing accumulation of secretion distal to obstruction followed by infection & inflammation e.g.
a) Obstruction by enlarged tuberculous hilar nodes.
b) Bronchial tumours.
c) Inhaled foreign body.

Pathology:

• The bronchiectatic cavities may be lined by granulation tissue, squamous or normal ciliated epithelium.
• Inflammatory changes in the deeper layers of bronchial wall and hypertrophy of the bronchial arteries.
Clinical features:

Symptoms:

1. Due to accumulation of pus in dilated bronchi:
• Chronic productive cough worse in mornings and often brought on by changes of posture.
• Sputum is copious & persistently purulent, foul smelling.

2. Due to inflammatory changes:
• Fever, malaise: Fever with increased cough & sputum develops when spread of infection causes pneumonia.
• Recurrent pleurisy in the same site (often occurs)
3. Haemoptysis:
• Slight/massive and is often recurrent.
• Usually associated with purulent sputum or be the only symptoms in so called ‘dry bronchiectsis’.
4. General health: (in extensive case)
• Weight loss.
• Anorexia.
• Lassitude.
• Low-grade fever.
• Digital clubbing (common).

Signs: 

1. Anaemia.
2. Clubbing.
3. Cyanosis (in sever case)
4. O/E of chest:
• If cavity is dry-No abnormal sign.
• If cavity contains fluid-Coarse crepitation.
• If fibrosis/collapse: Signs of fibrosis & collapse present.
5. Signs of complication: Consolidation, collapse, pleurisy.
Investigations:

1. Blood: TC-Leucocytosis, DC-Neutrophilia, ESR-Raised, Hb%-Usually low.
2. Sputum for AFB, Gram staining and C/S.
3. X-ray chest: Normal or honeycomb may contain fluid.
4. High resolution CT scanning: (investigation of choice) shows bronchial dilatation and wall thickening.
5. Bronchography (confirmatory): Dilated bronchi are visualized. It is performed during fibroptic bronchoscopy.
6. Sinus X-rays: 30% have concomitant purulent rhinosinusitis.
7. Screening test: To exclude ciliary dysfunction syndrome.
8. ECG and echocardiography to exclude corpulmonale.
9. Serum immune globulins: 10% adults have immune deficiency.
10. Respiratory function tests e.g. spirometry to assess respiratory status.
Diagnosis: Bronchiectasis may be diagnosed clinically or on review of imaging. The British Thoracic Society recommends all non cystic-fibrosis-related bronchiectasis be confirmed by CT. CT may reveal tree-in-bud abnormalities, dilated bronchi, and cysts with defined borders. 
Other investigations typically performed at diagnosis include blood tests, sputum cultures, and sometimes tests for specific genetic disorders.
Prevention: In order to prevent bronchiectasis, children should be immunized against measles, pertussis and other acute respiratory infections of childhood. While smoking has not been found to be a direct cause of bronchiectasis, it is certainly an irritant that all patients should avoid in order to prevent the development of infections (such as bronchitis) and further complications. 
Treatments to slow down the progression of this chronic disease include keeping bronchial airways clear and secretions weakened through various forms of pneumotherapy. Aggressively treating bronchial infections with antibiotics to prevent the destructive cycle of infection, damage to bronchial tubes and more infection is also standard treatment. Regular vaccination against pneumonia, influenza and pertussis are generally advised. A healthy body mass index and regular doctor visits may have beneficial effects on the prevention of progressing bronchiectasis. The presence of hypoxemia, hypercapnia, dyspnea level and radiographic extent can greatly affect the mortality rate from this disease.

Management:

[A]Postural drainage: Two times daily for 5-10 (first drainage in the morning and second at bedtime) is a minimum for most patients.

[B] Medical treatment:
a) Inhalation of menthol vapour.
b) Antibiotics (after C/S)
• Cefaclor 500mg three times daily. Or
• Ciprofloxacin 500 mg twice daily.
• Flucloxacillin 500 mg 6-hourly is best (St aurius)
c) Bronchodilators: Useful in patients with demonstrable airflow limitation.
d) Inhaled/Oral corticosteroid: Decrease rate of progression.

[C] Surgical treatment: After exactly demonstrating the extent of bronchiectasis by CT scans or bronchography.
a) Segmectomy or
b) Lobectomy could be done.
Aim of physiotherapy treatment: 

1. To remove secretions and clear lung fields.
2. To teach good coughing technique.
3. To gain patient’s confidence.
4. To maintain mobility of the thorax and good posture.
5. To promote good general health.
6. To teach the patient how to fit in home treatment within the life style.
Complication:

1. Recurrent pneumonia.
2. Lung abscess.
3. Haemoptysis.
4. Pleurisy.
5. Pleural effusion.
6. Empyema.
7. Cor pulmonale.
8. Collapse.

Reference:

1. Davidson’s Principles and Practice of Medicine, 21st edition.
2. Kumar and clark, clinical Medicine, 7th edition.
3. Wikipedia the free encyclopedia.
4. Tidy’s physiotherapy, 12th edition.