2. Introduction
Bronchion -- wind pipe / air ways
Ektasis -- streching out
Term coined by Laennec.
Abnormal irreversible dilatation and destruction of
cartilage containg airways/bronchi. ( i.e medium
sized sub segmental bronchi from 4 -9 th generations
)
3. Mechanism Disease ex
Congenital defects Mounier-Kuhn syndrome
William-Campbell syndrome
Alpha 1 anti-trypsin def
Infection Tuberculosis,
Pnemonias
Pertussis
Abnormal mucociliary clearance Cystic fibrosis
Primary ciliary dyskinesia
Decreased systemic immunity Hypo-gammaglobulinemia
Lung and bone transplantation
Exaggerated immune response ABPA
Inflammatory bowel disease
Rheumatoid arthritis
Inhalational injury Smoke and gaseous toxins
Chronic GERD and aspiration
Infection secondary to bronchial
obstruction
Slow-growing tumour,
aspirated foreign body
Lymphadenopathy
4. Tracheo-Bronchomegaly (Mounier-kuhn
dis )
Pathological thinning of the elastic and muscular
layers of the airway walls.
Involves the entire trachea and bronchi of first to
fourth order
Tracheal diameter exceeds 3 cm in both coronal
and sagittal planes.
Intrathoracic trachea and main bronchi dilate during
inspiration and collapse on expiration.
Associated with Ehlers-Danlos syndrome, Marfan
syndrome, generalized elastosis (cutis laxa).
5.
6. Bronchomalacia (Williams–Campbell
syndrome)
Defective / completely absent bronchial wall cartilage.
Cartilage of the fourth-, fifth-, and sixth-generation
bronchi is defective
Associated with congenital heart disease, poly-spleenia,
bronchial isomerism, and situs inversus.
Dynamic CT imaging reveals ballooning on inspiration
and collapse on exhalation.
Tracheo bronchomalacia- EDAC >50 %
7. Primariy ciliary dyskinesia
Immotile cilia syndrome
Absence of one or both rows of dynein arms
Prevent normal mucocilary escalator and result in
impairment of the lungs defence systems.
Kartagener syndrome or triad is present in half of the
PCD patients. This triad consists of situs inversus,
bronchiectasis, and sinusitis .
8.
9.
10. Pathogenesis :
Atelectatic theory:
Atelectasis gives loss of lung volume ,increases intra
pulmonary negative pressure; it dilates bronchi
proximal to the obstruction.
Pressure secretion theory:
Following plugging of bronchus with mucus,
secretions distal to the obstruction accumulate and
mechanically distended bronchi beyond the block.
Traction theory : bronchial dilatation occurs
secondary to fibrosis of lung parenchyma.
15. Clinical features :
Symptoms :
Cough - productive( wet )
- dry - upper lobes –TB
Haemoptysis – mild/ mod/massive
Dyspnoea – late symptom/advanced stages
Signs :
Coarse crackles -persistent early and
mid-inspiratory crackles
not altered with coughing
Clubbing : fingers and/or toes
16. Signs of collapse/ fibrosis.
Advanced cases : Cyanosis,
Signs of pulmonary hypertension
Right heart failure
PFT : Obstructive defect
Decreased DLCO
17. Complictions :
Infective exacerbations
Suppurative pneumonia and lung abscess
Extensive lung fibrosis may progress to respiratory
failure and cor pulmonale.
Haemoptysis- BAE
Sinusitis - rx steroids ,FESS
Amyloidosis- proteinuria, hepato spleenomegaly
18. Diagnosis :
Radiological :
Chest X ray
Bronchogram
HRCT
Other supportives :
Sputum/LRT aspirates for C/S
PFT
2D echo
19. Chest X ray : unreliable
Dilated bronchi appear as
Ring shadows : seen on end-on view
Parallel lines : seen on side-on view
Solid tubular opacities :bronchi contain mucopus
gloved finger shadows (ABPA)
PAH -prominent proximal pulmonary artery
cardiac enlargement
Volume loss apperance
20.
21.
22. Bronchogram :
• A Liquid contrast medium is instilled into the
tracheobronchial tree and X ray is taken.
• Most commonly used is Propyl-iodone
• Out dated procedure now.
23. HRCT : gold standard
Major signs :
1.Bronchial dilatation –
* horizontal bronchi- signet ring appearance
bronchus/artery ratio > 1.5
* vertical bronchi – tram lines
2. Presence of bronchi in the outer lung fields
Minor signs :
1. Bronchial wall thickening.
2. Mucus impaction.
3. Crowding of bronchi.
4. Areas of low attenuation/mosaic pattern
24.
25. SPUTUM C/S :
usually yields H. influenzae
Strep. pneumoniae
Staph. aureus
Ps.aeruginosa & Klebsiella spp – chronic
and severe cases.
antibiotics directed against these microbes.
26. Treatment :
Medical - to relieve and control the symptoms
Surgical - to eliminate the disease itself.
Physiotherapy – to aid in the drainage of secretions
29. Postural drainage therapy
usual sputum clearance mechanisms are disrupted by
(i) swamping of the normal blanket of mucus via
excessive secretion
(ii) the replacement of ciliated epithelium with
squamous cells so that the mucociliary escalator
cannot operate well
PDT helps in gravity aided drainage of sump
Basal bronchi- leaning forwards over the edge of a low
bed using the arms as a support against the floor.
Middle or lingular bronchi-supine posture with the
foot of the bed elevated and the affected side lifted off
the bed by pillows.
30. Other techniques like
Huffing- traditional postural drainage with the forced
expiration technique.
reduce the bronchial collapse induced by coughing.
High-frequency chest wall compression.
Active Cycle of Breathing Technique( ACBT )
Autogenic drainage ( AD )
31. Role of lung transplantation ? ? ?
Severe bronchiectasis in CF patients :
FEV1 <30% of predicted, or rapidly declining lung
function if FEV1 >30% (females and patients <18 years
of age have a poorer prognosis) and/or any of the
following:
Increasing oxygen requirements
Hypercapnia
Pulmonary hypertension
usually Double lung transplantation done.