BronchiectasisPresented by

Dr. Mohamed Abdelaziz OmerConsultant chest physician

The respiratory system Distributes air to the lungs Exchange gases ( primary function of the lungs )

Nostrils , mouth , pharynx , larynx , bronchial tree

Wind pipePassage way that supplies air to the lungs

4.5 inches long ……. 1 inch in diameter

smooth muscle & several C shaped rings of cartilage

which provides stability & help prevent collapse

Start in the neck , divided into two main branches

trachea

BronchiStructurally similar to the trachea

Two primary bronchi inside the lungs

Rt. Is slightly larger ..minimal angulations…

continuation

foreign bodies

BranchingEach bronchi divided into 5 smaller secondary

bronchi

2ndry bronchi branched to form tertiary bronchi

Tertiary bronchi divided into terminal bronchioles (cartilage less)

30 000 bronchioles in each lung

Alveolar ducts, sacs

Alveoli …. Very thin wall 2cells thick

PurifierMucus blanket covers large proportion of the

membrane lining the bronchial tree

125 ml daily

Cilia

Hair like moves the mucus up to the pharynx

BroncheictasisUncommon disease 20-50% cause not found ( B L F )

Most often secondary to an infectious process

Could be congenital

described by Laennec 1819

detailed by sir Wilham Osler in late 1800

Reid characterized it as cylindrical ,cystic ,varicose in 1950

DefinitionAbnormal permanent distortion of one or > conducting

bronchi

Abnormal dilatation of the proximal & medium sized >2mm

Cylindrical diffuse mucosal edema & dilatation but straight end

abruptly

Varicose has a bulbous appearance with a dilated bronchus &

interspersed sites of relative constriction & obstructive scarring

picture

Caused by weakness or destruction of the muscular elastic

component of the bronchial wall

Transmural inflammation , edema , scarring , ulceration

causesimpaired drainage

aspiration

Obstruction (middle lobe syndrome )

Defect of defense mechanism or host response

neutrophilic proteases , cytokines , NO , O2 radicals

. CF , William-Capell syndrome

Mounier-Kuhn (tracheobronchomegally), Swyer-James

Macleod syndrome (unilateral hyper lucent lung) ,

yellow nail syndrome young syndrome , Iry ciliary

dyskinasia , AAT deficiency , AD PKD , Toxic gas exposure

CTD , autoimmune diseases , idiopathic inflammatory

disease , immune deficiency

Bronchiectasis most commonly present as a focal process

involving a lobe , segment or sub-segment of the lung

Far less commonly it may be a diffuse process involving

entire lung or both lungs these cases most often occur

in association with systemic illness such as CF ,

sinopulmonary disease or both

Majority of this article will address non-CF related

Typical offending organismsNecrotizing infection either inadequately or non treated at all

klebsiella sp. , staph.aureus , MTB , NMTB , Mycoplasma

pneumonia measles , pertussis , HSV , RSV in childhood ,

MAC and certain types of adenoviruses

. Widened airways with extra mucus are prone to infection

. Haemophilus species 50% , pseudomonas 20%

Cystic fibrosis The most common cause in developed countries

Multisystem disorder , chloride transport system in

exocrine

2ry to a defect in CFTR protein

Autosomal recessive

Gregor Mendel

Symptoms No to few symptoms morphological diagnosis

Dry variant post TB upper lobes

Weakness , weight loss

Cough , sputum ,blood streaks , dyspnea , pleuritic chest pain

wheezing , fever

complicationsRecurrent pneumonia , Chronic bronchial infection

Empyema , abscess

Core pulmonale

Pneumothorax

Life threatening hemoptysis ?

Respiratory failure

Diagnosis

Compatible clinical Hx. Of chronic respiratory symptoms

Daily cough & viscid sputum production

Characteristic radiographic finding on CT scan ( bronchial

wall thickening & luminal dilatation )

prognosis

Pre-antibiotic era die within 5 years

1940 the mortality was 30% mostly die within 2 years

1990 in Finland compared the mortality rate :-

20% BA , 28% bronchiectasis , 38% COPD

Bronchiectasis with CF is of worst prognosis

Prevention

Immunization

Flu vaccination

Smoking

counseling

managementControl infection antibiotics

Bronchodilators

Control secretions steroids Postural drainage

Lobectomy , artery embolization

oxygen

Dietary supplementation

creosote

Thank you