BRONCHIECTASIS

Bronchiectasis is a chronic suppurative airway infection resulting in irreversible abnormal dilatation of bronchi with production of foul smelling sputum and progressive scarring and damage of the lungs.

Aetiology:

a) Congenital: Due to defective airway ion transport or ciliary functions. Examples:
• Cystic fibrosis
• Ciliary dysfunction syndromes (Immotile ciliary syndrome, Kartagener’s syndrome)
• Primary hypogammaglobulinemia

b) Acquired: Secondary to damage to the airway by infection, inhaled toxins or foreign body. Examples:

• In children:
i. Severe infections in infancy(whooping cough, measles)
ii. Primary tuberculosis
iii. Inhaled foreign body

• In adults:
i. Suppurative pneumonia
ii. Pulmonary tuberculosis(most common)
iii. Allergic bronchopulmonary aspergillosis complicating asthma
iv. Bronchial tumors

Pathology:

Obstructive bronchial lesions caused by tuberculous hilar lymph nodes, bronchial tumors or inhaled foreign body (eg: aspirated peanuts Accumulation of pus beyond the obstructive bronchial lesion which leads to formation of bronchiectatic cavities which are lined by granulation tissues, squamous or normal ciliated epithelium Inflammatory changes in the deeper layers of bronchial walls and hypertrophy of the bronchial arteries Chronic inflammatory and fibrotic changes in surrounding lung tissue Progressive destruction of the normal lung architecture (in advanced cases)

Types:

• Cylindrical or fusiform
• Varicose
• Cystic or saccular
• Follicular

Clinical features:

Symptoms
• Cough (chronic, daily, persistent)
• Sputum(copious, continuously purulent, foul smelling)
• Pleuritic chest pain(when infection involves pleura or when segmental collapse occurs due to retained secretion)
• Hemoptysis(streaks of blood, sometimes massive)
• Infective exacerbation(increased sputum volume with fever, malaise, anorexia)
• Halitosis(accompanying with purulent sputum)
• Others(weight loss, anorexia, exertional breathlessness)

Signs
• May be unilateral or bilateral
• Physical signs may be absent if the bronchiectatic airways lack secretions or if there is no any associated lobar collapse
• In presence of large amount of sputum in bronchiectatic spaces following signs are seen:
1. Coarse crakles over affected lung
2. Diminished breath sound(in collapse with retained secretions blocking a proximal bronchus)
3. Bronchial breath sounds
4. Clubbing

Complications:

• Acute hemoptysis
• Recurrent pneumonia
• Empyema
• Pneumothorax
• Lung abscess
• Brain abscess
• Cor pulmonale

Investigations:

• Sputum culture [ bacterial(Pseudomonas aeruginosa, Staphylococcus aureus), fungal(Aspergillosis), Mycobacteria]

• Radiological :
1. Chest x-ray: not usually apparent unless very gross; in advanced disease-
i. Dilated thickened bronchial walls (tramlines, ring shadows or honeycombing appearance)
ii. Cystic bronchiectatic spaces
iii. Associated areas of consolidation or collapse
2. CT Scan: more sensitive
3. Screening test:
– In patients suspected of having Ciliary dysfunction syndrome
– Saccharin test
4. Assessment of ciliary beat frequency
5. Electron microscopy for detecting structural abnormality of cilia

Management:

In patients with airflow obstruction, inhaled bronchodilators or glucocorticoids to enhance airway patency.
• Physiotherapy:
– For drainage of bronchial secretions
– Active cycle breathing techniques
– Devices to aid sputum clearance(positive expiratory pressure mask or flutter valve)
• Antibiotic therapy
• Surgical treatment: lobectomy(when confined to a lobe) or pneumonectomy(when entire lung is involved)
• Treatment of acute hemoptysis: percutaneous embolisation of bronchial artery)

Contributor- Dr. Bidhata Rayamajhi