1. Bronchiectasis Yoon Jung Oh,M.D. Departments of Pulmonary and
Critical Care Medicine
Ajou University School of Medicine

2. Definition
Abnormal and permanent dilatation of medium sized( ≥ 2mm) bronchus by destruction of muscular, cartilage and elastic tissue component
Three patterns of bronchiectasis (by Reid in 1950)
⑴ Cylindrical bronchiectasis
⑵ Cystic bronchiectasis
⑶ Varicose bronchiectasis
Involved site
⑴ Lt.lower lobe post.basal segment (common)
⑵ Rt.middle lobe
⑶ bilateral (30%)

3. Case M/30
상기 30세 남환은 수일 전 감기를 앓은 후 내원 당일 고열과 화농성 객담을 동반한 기침을 주소로 내원하였다.
환자는 부비동염으로 이비인후과에서 치료를 자주 받았다하며, 1년전에도 폐렴으로 입원한적이 있었다 한다. 평소 간헐적인 객혈이 있었으나 결핵의 기왕력은 없었다.
P/E moist crackles on left lower lung field

4. Bronchiectasis

5. Cystic Bronchiectasis

6. Cystic Bronchiectasis
Bronchogram
HRCT

7. Bronchiectasis

8. Cylindrical Bronchiectasis

9. Varicose Bronchiectasis

10. Pathogenesis and Predisposing Factors
Bronchopulmonary infections
Hereditary abnormalities
Immunodeficiency states
Bronchial obstruction
Congenital anatomic defects
Miscellaneous disorders

11. 연령에 따른 원인 Childhood Adulthood Post pneumonia (Measle, Pertussis, Tbc)
Mechanical obstruction(foreign body)
Cystic fibrosis
-globulin deficiency(cong./aquired)
Immotile cilia syndrome
Adulthood
1. Post pneumonia(viral, aspiration)
2. Pulmonary tuberculosis
3. Asthma, ABPA(allergic bronchopulmonary aspergillosis)
4. Toxic fume or congenital
5. Neoplasm, Chagas’ disease

12. Bronchopulmonary infections
Childhood diseases
Other bacterial infections
Other viral infections
Miscellaneous infections
pertussis; measles
S.aureus, Klebsiella, M.tuberculosis, H.influenaze
adenovirus, influenza, H.simplex, HIV
Mycotic infections
Mycolplasmal infections
Nontuberculous mycobacteria

13. Hereditary Abnormalities
Ciliary defects of respiratory mucosa
Kartagener’s syndrome
: bronchiectasis, situs inversus, sinusitis
Immotile cilia syndrome
: male infertility, defect in dynein arm or radical spoke
1-Antitrypsin deficiency
: panacinar emphysema, bronchiectasis
Cystic fibrosis

14. Kartagener’s Syndrome
Triad
bronchiectasis, sinusitis, situs inversus
Incidence : 1/68,000
Inheritance : Autosomal recessive trait
Pathogenesis
* absent or defective dysnein arms
* functional abnormality
(e.g., perhaps dysnein ATPase)

15. Kartagener’s syndrome

16. Immotile Cilia Sydrome
Diagnostic Criteria
1. Recurrent & Chronic URI and LRI
: rhinitis, sinusitis, otitis, bronchitis, bronchiectasis
2. Absence or near absence of tracheobronchial or nasal mucociliary transport
3. Total or near-total absence of dynein arms of the cilia in nasal or bronchial mucosa
4. Sterility in males

17. Normal Cilia MT : microtubules C : central sheath R : radial spokes
D : dysnein arms
N : nexin

18. Schematic cross section views of cilia
A : Normal cilium
Nine outer pairs of MT
around a central pair
B – D : congenital ciliary defects
B : lacking dysnein arms
C : missing radial spokes
D : abnormal transposition

19. Tuberculosis and Bronchiectasis
Marked degree of caseation necrosis of bronchial walls, particularly when upper lobes are invaded
2. Scarring of larger bronchi can produce bronchial stenosis
3. Extraluminal obstruction of larger bronchi by tuberculous hilar lymphadenopathy
4. Penetration by a calcified tuberculous node into the airway and broncholith formation
5. Healed tuberculous cavities

20. Clinical Features of Bronchiectasis
Symtoms
sputum
chronic cough
hemoptysis
dyspnea
fever
chest pain
Signs
crackles
finger clubbing
cyanosis
cor pulmonale

21. Diagnosis History / Physical examination
Plain chest X-ray : normal finding in 20%
HRCT(high resolution CT)
Bronchogram
Indications
① Preoperative evaluation of unilateral or segmental disease previously identified on CT
② Postoperativ evaluation of surgical airway complications such as dehiscence or fistula formation

22. Differential Diagnosis
Chronic bronchitis
Endobronchial tuberculosis
Endobronchial adenoma
Right middle lobe syndrome
Cystic fibrosis
Allergic bronchopulmonary aspergillosis
Pulmonary sequestration
Congenital abnormalities of the trachiobronchial tree

23. Aim of Treatment Elimination of an identifiable underlying problem
Improved clearance of tracheobronchial secretions
Control of infection
Reversal of airflow obstruction

24. Treatment : Medical 1. Treatment of predisposing factors
hypogammaglobulinemia  administration immunoglobulin
tuberculosis  anti-tbc medication
ABPA  steroid
2. Bronchial drainage : most important
PDPV(postural drainage, percussion, vibration)
PEEP
deep brathing, continued coughing
3. Antibiotics

25. Treatment : Surgical Indication Contraindication
Localized bronchiectasis
Massive/frequent hemoptysis
Intractable infection
Long standing collapse
Adequate residual lung function
Diffuse fibrosis or COPD
Bilateral bronchiectasis
Bronchiectasis complicating asthma
Low FEV1/FVC
Persisting predisposing factors
(e.g. immunodeficiency)

26. Complications Recurrent pneumonia, sinusitis Lung abscess
Pleuritis, empyema
Emphysema
Brain abscess(rare, but 10% of mortality)
Amyloidosis
Cor pulmonale(common in Korea)
Massive hemoptysis(rare)

27. Cystic Fibrosis Incidence : Caucasian 1/1,500 – 2,000 Asian 1/90,000
Inheritance : Autosomal recessive trait
Pathogenesis
Single gene disorder
defective c-AMP mediated regulation of chloride channels
-> failure to secete chloride toward the lumen
-> unusually viscid mucous secretions
-> chronic pulmonary disease and pancreatic insufficiency

28. Cystic fibrosis

29. Diagnosis of cystic fibrosis
1. Sweat chloride test
> 60 mEq : confirmative
50 – 60 mEq : highly probable
< 50 mEq : normal
2. Chronic pulmonary disease (99%)
3. Pancreatic insufficiency(80-90%)
4. CXR
increased interstitial marking (98%)
cystic bronchiectasis (64%)
hyperinflation(58%)

30. Treatment of cystic fibrosis
Infection control
pseudomonas(80%) S.aureus, H.influenza
Bronchial drainage
postural drainage, percussion and vibration
forced exhalation technique
positive expiratory pressure