Bronchiectasis Dr.kassim.M.Sultan F.R.C.P

Definition: abnormal and permanent dilatation&destruction of bronchial wall. typically affects older individuals; approximately two-thirds of patients are women. Need 2 factors: 1-infection. 2-impaired drainage of bronchial secretions. three different radiological pattern: 1-cylindrical bronchiectasis, the involved bronchi appear uniformly dilated and end abruptly at the point that smaller airways are obstructed by secretions. 2-varicose bronchiectasis, the affected bronchi have an irregular or beaded pattern of dilatation resembling varicose veins. 3-saccular (cystic) bronchiectasis, the bronchi have a ballooned appearance at the periphery, ending in blind sacs without recognizable bronchial structures distal to the sacs.

CAUSES OF BRONCHIECTASIS: Congenital: 1-Ciliary dysfunction syndromes : A-Kartagener's syndrome (dextrocardia or complete situs inversus, bronchiectasis, recurrent sinusitis,infertility in male patients (secondary to immotile ciliae, small volume complexes in lateral leads) B-Young's syndrome (bronchiectasis, sinusitis, and azoospermia). 2-Primary hypogammaglobulinaemia 3-Cystic fibrosis:increase Nacl in sweat,bronchiectasis involving the upper lobes&superadded pseudomonas infection.

CAUSES OF BRONCHIECTASIS: Acquired 1-Pneumonia (complicating whooping cough or measles),PulmonaryTB (major cause) 2-Inhaled foreign body in childrens,altered state of consciousness(stroke,alcoholics,general anesthesia). 3-bronchial obstruction(partial or complete,intrabronchial or extrabronchial)e.g:lung tumors or enlarged lymph nodes in TB. 4-Allergic bronchopulmonary aspergillosis complicating asthma(resistant to bronchodilators,need high dose oral steroids,usually central). 5-immune-defficiency:HIV, CLL, nephrotic syndrome. 6-toxic insult(aspiration, Inhalation of toxic gases&chemicals). 7-associations:connective tissue diseases,inflammatory bowel diseases,marfan syndrome,yellow nail syndrome.

Pathology: there is destructive and inflammatory changes in the walls of medium-sized airways, often at the level of segmental or subsegmental bronchi. the main pathological features: bronchial and peribronchial inflammation and fibrosis, ulceration of the bronchial wall, squamous metaplasia, and mucous gland hyperplasia. focal or diffuse Bronchial neovascularization with hypertrophy&tortuosity of the bronchial arteries

Clinical features: Chronic productive cough usually worse in mornings and often brought on by changes of posture. Sputum often copious and persistently purulent in advanced disease. Halitosis is a common. Haemoptysis Can be slight or massive and is often recurrent.however,sometimes be the only symptom in so-called 'dry bronchiectasis‘ Fever, malaise,weight loss, anorexia, lassitude,, and failure to thrive in children. Recurrent pleurisy in the same site often occurs in bronchiectasis Physical exam:may be normal 1-Coarse inspiratory and expiratory crackles on auscultation 2-Airflow obstruction with wheeze. 3-digital clubbing is common. 4-signs of collapse(proximal obstruction)&cosolidation(peripheral obstruction).

Investigations: Sputum microbiology, C & S (including for atypical organisms), acid-fast bacilli, and Aspergillus. CXR:classically shows ring shadows and tramlines indicating thickened airways, and the gloved finger appearance,consolidation around thickened and dilated airways. HRCT chest is 97% sensitive in detecting disease. PFTwith reversibility testing is usually obstructive,low FVC in advanced disease. Bronchoscopy to exclude a foreign body if suggested by CT; obtain microbiological samples if unusual clinical presentation, or failure to respond to standard antibiotics. Saccharin test to asses ciliary function CF genotyping(if Predominantly upper lobe disease, Family history of cystic fibrosis)

A posterior-anterior chest radiograph with walls of airways dilated and thickened (arrow) in the right upper lobe as seen in allergic bronchopulmonary aspergillosis. In the left upper lobe are airways filled with mucus and cellular debris.

Kartagener syndrome. Dextrocardia and left lower lobe bronchiectasis.

Management: A-medical treatment: physiotherapy:Patients should adopt a position in which the lobe to be drained is uppermost.Deep breathing followed by forced expiratory manoeuvres.vigorous coughing.5-10 minutes 1-2 daily inhaled B2-agonists,mucolytics,increase fluid intake. Systemic steroid 20-30mg/d for 2days with tapering over days with antibiotics. antibiotics: cornerstone of management.should be guided by sputum c/s especially when staph.,G-ve&pseudomonas are isolated for pseudomonas: oral ciprofloxacin ( mg 12-hourly) or ceftazidime by intravenous injection or or infusion (1-2 g 8-hourly) for 2 weeks. Long term oral antibiotics e.g azithromycin mg 2-3/week. Nebulized tobramycin 300mg 12hourly in alternate months for CF patients colonised with pseudomonas. B-Surgical treatment: 1-lobectomy:young patients in whom the bronchiectasis is unilateral and confined to a single lobe or segment as demonstrated by CT. 2-lung Transplant is most commonly performed for CF bronchiectasis.

Complications of bronchiectasis: Recurrent pneumonia&pleurisy Massive hemoptysis. Brain abscess. Amyloidosis-nephrotic syndrome. Corpulmonale. Empyema&pneumothorax.

Suppurative lung diseases include ( aspiration pneumonia, lung abscess and bronchiectasis ). Predisposing factors ( obstruction and super added infection in bronchiectasis, neurological diseases as ( CVA, fits, …et) in aspiration pneumonia. Diagnosis with sputum exam., chest-X ray, C.T of chest and sometimes bronchoscope.

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