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8/12/2019 Bronchiectasis Csbrp 130111111739 Phpapp01

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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

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