BRONCHIECTASIS

DR.CSBR.PRASAD, M.D.
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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]
[Kartageners 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
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Kartageners 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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Kartageners 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 ]
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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 ]
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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.
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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.
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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
DYNEI N ARM
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