Bronchiectasis is a chronic lung condition characterized by abnormal dilation of the bronchi and bronchioles. It can be caused by bronchial obstruction, congenital defects, or necrotizing pneumonia. Symptoms include cough, copious foul-smelling sputum, and recurrent lung infections. Long-term complications can include lung abscesses, lung damage, and heart failure. Treatment involves airway clearance and antibiotics to prevent infections.

1. BRONCHIECTASIS
DR.CSBR.PRASAD, M.D.

2. CSBRP-Dec-2012

3. CSBRP-Dec-2012

4. 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
CSBRP-Dec-2012

5. Clinical features
 Cough, fever, copious foul smelling sputum
 The dilation is permanent.
 Reversible dilation occurs in viral and
bacterial pneumonia.
CSBRP-Dec-2012

6. Causes:
 Bronchial obstruction
 Congenital / Hereditary conditions
 Necrotizing pneumonias
CSBRP-Dec-2012

7. Causes:
Bronchial obstruction –
 Tumor
 Foreign body
 Mucous impaction [complication of atopic
asthma, chronic bronchitis]
CSBRP-Dec-2012

8. CSBRP-Dec-2012

9. CSBRP-Dec-2012

10. CSBRP-Dec-2012

11.  The three
layers are
purulent
sediment,
clear middle
liquid and
top foamy
layer.
CSBRP-Dec-2012

12. 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]
CSBRP-Dec-2012

13. Ciliary dyskinesia:
[Immotile cilia syndrome]
[Kartagener’s syndrome]
CSBRP-Dec-2012

14. Ciliary dyskinesia:
[Immotile cilia syndrome]
[ Kartagener syndrome ]
Ciliary beating
Mucociliary clearance
Increased susceptibility to infections
CSBRP-Dec-2012

15. CSBRP-Dec-2012

16. CSBRP-Dec-2012

17. CSBRP-Dec-2012

18. CSBRP-Dec-2012

19. CSBRP-Dec-2012

20. NORMAL CILIARY DYSKINESIA
DYNEIN ARM
CSBRP-Dec-2012

21. Commonest abnormalities:
 Absence of either outer or inner
dynein arms
 Defects in radial spokes etc.
CSBRP-Dec-2012

22. Clinical features of
Ciliary dyskinesia:
 Repeated bouts of otitis & sinusitis
 Recurrent chest infections
 Situs inversus - [ 50% ]
[ Kartagener syndrome - triad ]
 Males --- Infertility
CSBRP-Dec-2012

23. Kartagener’s 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.
CSBRP-Dec-2012

24. CSBRP-Dec-2012

25. CSBRP-Dec-2012

26. Kartagener’s syndrome
Why “Situs inversus” ?
CSBRP-Dec-2012

27. Cystic fibrosis (CF)
[Mucoviscidosis]
CSBRP-Dec-2012

28. 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 ]
CSBRP-Dec-2012

29. Sterility
Increased
Na+ and Cl-
Bronchiectasis
Vas deferens in sweat
seminal vesicles
Sweat
Lungs glands
CYSTIC
FIBROSIS
Biliary
Gut
system
Meconium Pancreas Jaundice
ileus
cirrhosis
Malabsorption
of fat Fibrosis
CSBRP-Dec-2012

30. Pathogenesis of CF:
Thick viscous mucus
Impaired mucociliary clearance
Repeated bouts of pulmonary infections
Parenchymal fibrosis
[ Bronchiectasis ]
CSBRP-Dec-2012

31. 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
CSBRP-Dec-2012

32. CSBRP-Dec-2012

33. Normal Cystic Fibrosis
AIRWAY
Na+ H20 Na+ H2O
Cl
Cl
CSBRP-Dec-2012

34. CSBRP-Dec-2012

35. CSBRP-Dec-2012

36. Pathogenesis:
 Thick mucus
 Obstruction to the bronchus
 Infection
CSBRP-Dec-2012

37. 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
CSBRP-Dec-2012

38. 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.
CSBRP-Dec-2012

39. CSBRP-Dec-2012

40. CSBRP-Dec-2012

41. OBSTRUCTING LESION
BRONCHIECTASIS
POSSIBLE LIPID OR
INFECTIVE PNEUMONIA
CSBRP-Dec-2012

42. 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.
CSBRP-Dec-2012

43. Dilatation of Bronchi
& Bronchioles
2cms
CSBRP-Dec-2012

44. Bronchiectasis, secondary
to obstruction involving
the right middle lobe.
CSBRP-Dec-2012

45. A closer view of the lung demonstrates the focal area of
CSBRP-Dec-2012
dilated bronchi typical of bronchiectasis.

46. 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.
CSBRP-Dec-2012

47. CSBRP-Dec-2012

48. 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 extensiveCSBRP-Dec-2012 the pleural space may be
involvement,
obliterated.

49. CSBRP-Dec-2012

50. CSBRP-Dec-2012

51. CSBRP-Dec-2012

52. Clinical course:
 Cough - severe, persistent
 Expectoration - copious, foul smelling
 Fever
 Dyspnoea
 Orthopnea Severe cases
 Cyanosis
CSBRP-Dec-2012

53. Complications of
bronchiectasis:
 Metastatic abscesses ( brain abscess)
 Recurrent pulmonary infection
 Right sided cardiac failure
[ chronic cor pulmonale ]
 Massive haemoptysis
 Reactive systemic amyloidosis
[ Nephrotic syndrome ]
CSBRP-Dec-2012

54. E N D
CSBRP-Dec-2012

55. DYNEIN ARM
SCHEMATIC
DIAGRAM
OF A
CILIUM
CSBRP-Dec-2012