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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 ofthese airways.Prevalence:
* 1.5 per 1000 population* Has decreased in recent times
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Clinical features
Cough, fever, copious foul smelling sputumThe dilation is permanent.
Reversible dilation occurs in viral andbacterial pneumonia.
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Causes:
Bronchial obstruction
Congenital / Hereditary conditions Necrotizing pneumonias
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Causes:
Bronchial obstruction TumorForeign body
Mucous impaction [complication of atopicasthma, chronic bronchitis]
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The threelayers are
purulentsediment,clear middle
liquid andtop foamylayer.
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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, mixedinfection]
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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 armsDefects in radial spokes etc.
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Clinical features of
Ciliary dyskinesia:Repeated bouts of otitis & sinusitisRecurrent chest infectionsSitus inversus - [ 50% ]
[ Kartagener syndrome - triad ]
Males --- Infertility
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Kartageners syndrome
1. Bronchiectasis2. Situs inversus3. Sinusitis4. Infertility in menCharacterised by ultrastructural changes in
microtubules causing immotility of cilia ofrespiratory 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 diseasePrevalence: 1 in 2500 live birthsCommon in western countriesUncommon in Asians & AfricansPulmonary involvement dominates
Inheritance - AR95% deaths - pulmonary disease
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Pancreas
FibrosisMalabsorption
of fat
Gut
Meconiumileus
Sterility
Lungs
Vas deferensseminal vesicles
Sweat
glands
IncreasedNa+ and Cl-
in sweat
Biliarysystem
Jaundicecirrhosis
Bronchiectasis
CYSTICFIBROSIS
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Molecular defect:
Gene - CFTRChromosome - 7q31 - 32Function - Anion channelDefect in cystic fibrosis ( 550 mutations )--- deletion of codon 508 [phenylalanine]
CFTR : C ystic F ibrosis T ransmembrane conductance R egulator
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Na + H20
Normal
Na+ H2 O
AIRWAY
Cystic Fibrosis
ClCl
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Pathogenesis:
Thick mucus
Obstruction to the bronchusInfection
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Morphology - Gross
Affects lower lobesBilateralAir passages that are verticalTumor/FB aspiration localized singlesegmentSevere involvement - more distal bronchiAirways are 4x dilated than normal
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Gross contd.
1. Cylindrical most common, tube likedilation
2. Fusiform spindle shaped dilation.3. Saccular sac like distension.4. Varicose irregular bronchial
enlargement.Cut sections shows HONEY COOMBEDappearance.
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OBSTRUCTING LESION
BRONCHIECTASIS
POSSIBLE LIPID ORINFECTIVE PNEUMONIA
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This is a bronchogramthatdemonstratessaccular
bronchiectasis
on the right inthe lower lobe.The contrastmedia fillsdilated bronchi,giving asaccular, dilatedoutline.
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Dilatation of Bronchi& Bronchioles
2cms
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Bronchiectasis, secondary
to obstruction involvingthe 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
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Fig15-3 Bronchiectasisin a patient with cysticfibrosis, who underwentlung transplantation. Cutsurface of lung showsmarkedly distendedperipheral bronchi filledwith mucopurulent
secretions.
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Bronchiectasis is seen here. The repeated episodes of inflammation can result inscarring, which has resulted in fibrous adhesions between the lobes. Fibrous pleuraladhesions 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 beobliterated. CSBRP-Dec-2012
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C l i n i c a l c o u r s e :
Cough - severe, persistentExpectoration - copious, foul smellingFeverDyspnoeaOrthopnea
Cyanosis
Severe cases
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Complications of
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Complications ofbronchiectasis:
Metastatic abscesses ( brain abscess)Recurrent pulmonary infectionRight sided cardiac failure
[ chronic cor pulmonale ]Massive haemoptysisReactive systemic amyloidosis
[ Nephrotic syndrome ]
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E N D
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SCHEMATIC
DIAGRAMOF ACILIUM
DYNEI N ARM