Bronchiectasis

Bronchiectasis

PKGhatak,MD

Bronchiectasis is a permanent dilatation of a small segment of the bronchial tube that results from the destruction of walls of the bronchus from repeated inflammation or infection.

Bronchiectasis due to cystic fibrosis accounts for 50% of all cases of bronchiectasis. Congeal or acquired deficient mucociliary defense mechanism, ciliary dyskinesia, alpha 1 antitrypsin deficiency, congenital mitochondrial diseases, congenital or acquired Immunoglobulin diseases, immunocyte depression from immunotherapy, etc. favor repeated bacterial, fungal, and mycobacterial infections. HIV infection and account for a few cases. Chronic lung diseases like pulmonary emphysema, silicosis, allergic aspergillosis, pulmonary tuberculous and foreign body bronchus, pulmonary fibrosis secondary to rheumatoid arthritis, Sjorgren syndrome, SLE, Crohn's disease, etc. accounts for the rest of cases of bronchiectasis.

Childhood infection and Bronchiectasis.

Measles and whooping cough are dominant causes of bronchiectasis in poor countries, equally important, post bacterial pneumonia, specially due to Staphylococcus aureus, Hemophilus influenza and pulmonary tuberculosis.

Bronchiectasis is a small pouch, as the mucus accumulates in the pouch, the mucus remains at the site. It is a favorable medium for bacteria, fungus, or mycobacteria growth. Repeated and/persistent infections damage the structure of the bronchus, erode blood vessels causing hemoptysis, or ruptures into the pleural space and develop an open path to the pleural space and produce a bronchopleural fistula and which can easily lead to a purulent pleural effusion and pleural fibrosis.

Descriptive types.

About 50% of bronchiectasis are cylindrical types, 40% are cystic, 10% are beaded - (narrow segments of structurally normal bronchi are interspersed between rows of the dilated portions). 25% are of mixed types.

Presenting symptoms.

Cough productive of a large volume of purulent sputum; when allowed settling, separates in three distinct layers -  the bottom consists of necrotic cells and tissues, the middle layer contains clear fluid and the top layer contains foaming surface. The sputum has a sweet offensive odor. Fever, loss of weight, malnutrition, etc. are due to chronic wasting conditions, but hemoptysis occurs frequently, the volume of blood brought up is generally moderate quantity, and rarely massive hemoptysis can exsanguinate the patient.

Kartagener Syndrome.

Kartagener syndrome is an autosomal recessive inherited disease. A patient presents with repeated sinusitis, bronchitis/pneumonia, hemoptysis, clubbing of fingers and toes and sterility in males. The defect is due to ciliary dyskinesia. Associated conditions are situs inversus of various degrees – from dextrocardia (heart and great vessels are on the right side) to a complete reversed position of all internal organs and the presence of bronchiectasis.

Diagnosis of Bronchiectasis.

A chest x-ray may or may not show any abnormality that is the specific radiological sign for bronchiectasis. CT of the chest, specially HTCT, shows the following signs – tram-lines (parallel lines with a clear central area), a bunch of grapes sign, signet ring sign, a string of pearl sign (narrow but intact bronchial segments in between small dilated bronchiectasis segments appear in a row). Location of positive radiological findings are in the - basal segments, right apical segments, left lingular segment and posterior upper middle segment.

Bronchoscopy.

All cases, at least initially, require bronchoscopic examination and are valuable to diagnose the presence of a foreign body bronchus, adenoma, carcinoma, or aspirated gastric contents and also to determine the degree of bronchiectasis involvement. Appropriate cultures, smears and cytology studies are routinely ordered.

Nowadays bronchograms are not performed. CT/ MRI eliminated that practice.

Pulmonary function impairment evaluations

Pulmonary function evaluations are carried out by PFT, oximetry, 6 mins walk tests and other appropriate tests to detect any and all underlying conditions that may lead to bronchiectasis.

Goal of treatment.

Depending on the underlying condition, the treatment and prognosis vary from one case to the other. A prompt and aggressive treatment protocol consists of proper antibiotic therapy, postural drainage of the retained bronchial secretion, chest percussion to help loosen thick sticky mucus, proper hydration of patients and the humidification of inhaled air.

Complications are minimized by antibiotic prophylaxis, bronchodilator drugs, mucolytic agents, appropriately administered pneumonia, influenza vaccines and surgical intervention when hemoptysis continues.

Surgery.

If the bronchiectasis is localized to one segment only, the lobectomy is only curative therapy, if the patient still has an adequate pulmonary reserve.

Bronchiectasis numbers are decreasing in developing countries because of good public health measures, childhood vaccination like MMR and adult vaccination programs and prompt medical attention to the needs of patients.

But the same cannot be said about poor countries and where people are forced to live in refugee camps.

Attempts should be made to limit the damage to the lungs by implementing good public health measures.

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