Bronchiectasis

· Pathophysiology

· Chronic dilation and distortion of bronchial airways

· Excessive production of foul-smelling sputum

· Smooth muscle constriction of bronchial airways

· Hyperinflated alveoli

· Atelectasis

· Consolidation

· Parenchymal fibrosis

· Anatomic Alterations

· Hyperinflation of the distal alveoli

· Atelectasis

· Consolidation

· parenchymal fibrosis

· Forms of Bronchiectasis

· Varicose

· Bronchi are dilated and constricted in an irregular fashion

· Cylindrical

· Bronchi are dilated and have regular outlines

· Like a tube. The dilated bronchi fail to taper for six to ten generations

· They will appear to end squarely in a bronchogram because of mucous obstruction

· Saccular

· Bronchi progressively increase in diameter and end in large, cyst-like sacs in the lung parenchyma

· This form causes the most damage to the tracheobronchial tree

· The bronchial walls become fibrous. Cartilage, elastic tissue, and smooth muscle are absent

· Etiology: Acquired or Congenital

· Acquired

· Pulmonary Infection

· Repeated and prolonged resp tract infections. Children who have frequent bouts of broncho-pneumonia-because of resp complications of measles, chickenpox, pertussis, or influenza, - may acquire bronchiectasis later in life.

· Bronchial Obstruction

· caused by tumor masses, enlarged hilar lymph nodes, or aspirated foreign bodies may result in bronchiectasis distal to the obstruction.

· These conditions impair the mucociliary clearance mechanism, and this impairment, in turn, favor, the development of necrotizing bacterial infections.

· Pulmonary TB

· Because of the inflammatory process and the bronchial wall destruction associated with pulmonary TB, bronchiectasis is a common secondary complication

· Congenital

· Kartagener’s Syndrome

· A triad consisting of bronchiectasis, dextrocardia (having the heart on the right side of the body), and paranasal (alongside the nose) sinusitis

· Accounts for about 20% of all congenital bronchiectasis.

· Hypogammaglobulinemia

· individuals who have inadequate regional or systemic defense mechanisms because of inherited or acquired immune deficiency disorders. These individuals have a high risk for recurrent episodes of respiratory infections.

· Cystic Fibrosis

· Because of impairment of the mucociliary clearance mechanism and the abundance of stagnant, thick mucus associated with CF, bronchial obstruction from mucus plugging and bronchial infections frequently result.

· The necrotizing inflammation that develops under these conditions often leads to secondary bronchiectasis

· Physical Examination

· Increased RR

· Several pathophysiologic mechanisms operating simultaneously may lead to an increased RR:

· Stimulation of Peripheral chemoreceptors

· Decreased lung compliance/increased ventilatory rate relationship

· Anxiety

· Increased HR, CO, and BP

· Accessory Muscle Use

· On inspiration and expiration

· Pursed-lip breathing

· Increased AP diameter

· Barrel Chest

· Digital Clubbing

· Cause unknown (often seen with chronic hypoxemia)

· Peripheral edema and distention

· Polycythemia and cor pulmonale are associated with severe emphysema: You may see:

· Distended neck veins

· Pitting edema

· Enlarged and tender liver

· Cyanosis

· Cough and Sputum Production

· Chronic productive cough of large quantities of foul-smelling sputum

· Settles into different layers

· Hemoptysis

· Streaks of blood are seen frequently, presumably originating from necrosis of the bronchial walls and erosion of bronchial blood vessels

· Frank hemoptysis may also occur from time to time but is rarely life threatening

· Bacterial colonization

· Streptococcus pneumoniae

· Hemophilus influenzae

· Pseudomonas aeruginosa

· Due to the excessive bronchial secretions

· Secondary bacterial infections are frequent

· Chest Assessment

· Obstructive Bronchiectasis

· Air trapping

· Hyperresonant percussion note

· Air trapping

· Decreased tactile and vocal fremitus

· Air trapping

· Diminished breath sounds

· Bronchospasm

· Wheezing, Crackles, and Rhonchi

· when accompanied by acute or chronic bronchitis

· Restrictive Bronchiectasis

· Dull percussion note

· Over areas of atelectasis and consolidation

· Increased tactile and vocal fremitus

· Bronchial breath sounds

· Whispered Pectoriloquy

· When patient whispers “1, 2, 3” the sounds are much louder and more intelligible over the affected area

· Laboratory Evaluation

· Increased H&H

· Hemoglobin (Hb: each RBC contains app. 280 million Hb molecules, grams /100ml of blood. Normal healthy adult male = 14-16 g% normal healthy adult female = 12-15 g%)

· Hematocrit (Hct: volume of RBC in 100 ml of blood. Normal healthy adult male = 45% normal healthy adult female = 42%

· Hypochloremia

· With chronic ventilatory failure

· Haemophilus influenzae

· Pseudomonas aeruginosa

· Chest Radiograph

· Signs of hyperinflation in Obstructive disorder

· Translucent lung fields (dark)

· Flattened diaphragms (depressed)

· Long narrow heart (pulled down by diaphragm)

· Enlarged Heart (lateral x-ray)

· Restrictive disorder

· Increased opacity (white)

· Atelectasis and consolidation

· When atelectasis and consolidation develop as a result of bronchiectasis, an increased opacity is seen

· Bronchogram

· Cylindrical bronchiectasis

· Dilated, cylinder-shaped bronchioles

· Increased bronchial markings and adjacent emphysema

· Saccular bronchiectasis

· Large sac-like structures, fibrotic markings, associated atelectasis, and adjacent emphysema

· Varicose bronchiectasis

· Bronchi are dilated and constricted in an irregular fashion

· Bronchi terminate in a distorted, bulbous shape

· Computed Tomography (CT) of the chest has largely replaced this technique

· CT Scan

· Bronchial wall thickness and opacity is often seen

· Dilated

· Characterized by ring lines or clusters

· Signet ring-shaped

· Produced by the ring shadow of a dilated airway with its accompanying artery

· Flame-shaped

· Airways are filled with secretions

· PFT

· Obstructive bronchiectasis

· Decreased expiratory flows

· Decreased: FVC, FEV1, PEFR

· Increased lung volumes

· Increased VT, RV, FRC

· Restrictive bronchiectasis

· Decreased lung volumes and capacities

· Decreased: VT, RV, FRC, TLC, VC, IC, ERV

· ABG

· Mild to Moderate bronchiectasis

· Early hypoxemia with respiratory alkalosis

· Acute alveolar hyperventilation with hypoxemia

· pH increased

· PaCO2 decreased

· HCO3 slightly decreased

· PaO2 decreased

· Severe bronchiectasis

· Late respiratory acidosis with hypoxemia

· Chronic ventilatory failure with hypoxemia

· pH normal

· PaCO2 increased

· HCO3 increased significantly

· PaO2 decreased

· General Management

· Patient education

· patient and family should be instructed on disease and the effects on the body, Instructed on home care therapy

· Use of pulmonary rehab if needed.

· Behavioral management

· Stop smoking

· Avoid inhaled irritants such as

· Dust

· Fumes

· Mist

· toxic gases

· Mobilization of secretions

· Bronchial hygiene therapy

· Medications

· Sympathomimetic and parasympatholytic agents for bronchospasms

· Xanthines to enhance bronchial smooth muscle relaxation

· Expectorants and Antibiotics to treat infections

· Supplemental oxygen

· Treat hypoxemia

· Decrease myocardial work

· Decrease WOB

· (in late stages be careful not to over oxygenate)

· Mechanical ventilation

· provide and support alveolar gas exchange

· eventually return the patient to spontaneous breathing