BRONCHIECTASIS

DR.CSBR.PRASAD, M.D.

CSBRP-Dec-2012

CSBRP-Dec-2012

Definition:

Is a chronic necrotising infection of the bronchi and bronchioles leading to or associated with abnormal dilation of these airways.

Prevalence:

* 1.5 per 1000 population

* Has decreased in recent times

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Clinical features

Cough, fever, copious foul smelling sputum

The dilation is permanent.

Reversible dilation occurs in viral and

bacterial pneumonia.

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Causes:

Bronchial obstruction

Congenital / Hereditary conditions

Necrotizing pneumonias

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Causes:

Bronchial obstruction –

Tumor

Foreign body

Mucous impaction [complication of atopic asthma, chronic bronchitis]

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The three

layers are

purulent

sediment,

clear middle

liquid and

top foamy

layer.

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Causes : cont…..

Congenital / Hereditary conditions

• Congenital bronchiectasis (developmental anomaly)

• Cystic fibrosis

• Immunodeficiency states

• Immotile cilia & Kartagener syndromes

Necrotizing pneumonia

[Post infective bronchiectasis - TB, Staph, mixed

infection]

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Ciliary dyskinesia: [Immotile cilia syndrome]

[Kartagener’s syndrome]

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Ciliary dyskinesia:

[Immotile cilia syndrome]

[ Kartagener syndrome ]

Ciliary beating

Mucociliary clearance

Increased susceptibility to infections

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NORMAL CILIARY DYSKINESIA

DYNEIN ARM

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Commonest abnormalities:

Absence of either outer or inner

dynein arms

Defects in radial spokes etc.

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Clinical features of

Ciliary dyskinesia:

Repeated bouts of otitis & sinusitis

Recurrent chest infections

Situs inversus - [ 50% ]

[ Kartagener syndrome - triad ]

Males --- Infertility

CSBRP-Dec-2012

Kartagener’s syndrome

1. Bronchiectasis

2. Situs inversus

3. Sinusitis

4. Infertility in men

Characterised by ultrastructural changes in

microtubules causing immotility of cilia of

respiratory epithelium, sperms.

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Kartagener’s syndrome

Why “Situs inversus” ?

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Cystic fibrosis (CF)

[Mucoviscidosis]

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Cystic fibrosis [Mucoviscidosis]

Systemic disease

Prevalence: 1 in 2500 live births

Common in western countries

Uncommon in Asians & Africans

Pulmonary involvement dominates

Inheritance - AR

95% deaths - pulmonary disease

[ Bronchiectasis ] CSBRP-Dec-2012

Pancreas

Fibrosis Malabsorption

of fat

Gut

Meconium

ileus

Sterility

Lungs

Vas deferens

seminal vesicles

Sweat

glands

Increased

Na+ and Cl-

in sweat

Biliary

system

Jaundice

cirrhosis

Bronchiectasis

CYSTIC

FIBROSIS

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Pathogenesis of CF:

Thick viscous mucus

Impaired mucociliary clearance

Repeated bouts of pulmonary infections

Parenchymal fibrosis

[ Bronchiectasis ] CSBRP-Dec-2012

Molecular defect:

Gene - CFTR

Chromosome - 7q31 - 32

Function - Anion channel

Defect in cystic fibrosis ( 550 mutations )

--- deletion of codon 508 [phenylalanine]

CFTR: Cystic Fibrosis Transmembrane conductance Regulator

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Na+ H20

Normal

Na+ H2O

AIRWAY

Cystic Fibrosis

Cl Cl

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Pathogenesis:

Thick mucus

Obstruction to the bronchus

Infection

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Morphology - Gross

Affects lower lobes

Bilateral

Air passages that are vertical

Tumor/FB aspiration – localized single

segment

Severe involvement - more distal bronchi

Airways are 4x dilated than normal

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Gross contd….

1. Cylindrical – most common, tube like dilation

2. Fusiform – spindle shaped dilation.

3. Saccular – sac like distension.

4. Varicose – irregular bronchial enlargement.

Cut sections shows HONEY COOMBED appearance.

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OBSTRUCTING LESION

BRONCHIECTASIS

POSSIBLE LIPID OR

INFECTIVE PNEUMONIA

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This is a

bronchogram

that

demonstrates

saccular

bronchiectasis

on the right in

the lower lobe.

The contrast

media fills

dilated bronchi,

giving a

saccular, dilated

outline.

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Dilatation of Bronchi

& Bronchioles

2cms

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Bronchiectasis, secondary

to obstruction involving

the right middle lobe.

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A closer view of the lung demonstrates the focal area of

dilated bronchi typical of bronchiectasis. CSBRP-Dec-2012

Fig15-3 Bronchiectasis

in a patient with cystic

fibrosis, who underwent

lung transplantation. Cut

surface of lung shows

markedly distended

peripheral bronchi filled

with mucopurulent

secretions.

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Bronchiectasis is seen here. The repeated episodes of inflammation can result in

scarring, which has resulted in fibrous adhesions between the lobes. Fibrous pleural

adhesions are common in persons who have had past episodes of inflammation of the

lung that involve the pleura. With extensive involvement, the pleural space may be

obliterated. CSBRP-Dec-2012

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Clinical course:

Cough - severe, persistent

Expectoration - copious, foul smelling

Fever

Dyspnoea

Orthopnea

Cyanosis

Severe cases

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Complications of

bronchiectasis: Metastatic abscesses ( brain abscess)

Recurrent pulmonary infection

Right sided cardiac failure

[ chronic cor pulmonale ]

Massive haemoptysis

Reactive systemic amyloidosis

[ Nephrotic syndrome ]

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E N D

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SCHEMATIC

DIAGRAM

OF A

CILIUM

DYNEIN ARM

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