Yoon Jung Oh,M.D.

Departments of Pulmonary and Critical Care Medicine

Ajou University School of Medicine

BronchiectasisBronchiectasisBronchiectasisBronchiectasis

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 bronchiectasis⑵ Cystic bronchiectasis⑶ Varicose bronchiectasis

Involved site⑴ Lt.lower lobe post.basal segment (common) ⑵ Rt.middle lobe⑶ bilateral (30%)

Definition

Case M/30

상기 30 세 남환은 수일 전 감기를 앓은 후 내원 당일 고열과

화농성 객담을 동반한 기침을 주소로 내원하였다 .

환자는 부비동염으로 이비인후과에서 치료를 자주 받았다하

며 , 1 년전에도 폐렴으로 입원한적이 있었다 한다 . 평소

간헐적인 객혈이 있었으나 결핵의 기왕력은 없었다 .

P/E moist crackles on left lower lung field

Bronchiectasis

Cystic Bronchiectasis

Cystic Bronchiectasis

Bronchogram HRCT

Bronchiectasis

Cylindrical Bronchiectasis

Varicose Bronchiectasis

• Bronchopulmonary infections

• Hereditary abnormalities

• Immunodeficiency states

• Bronchial obstruction

• Congenital anatomic defects

• Miscellaneous disorders

Pathogenesis and Predisposing Factors

Post pneumonia (Measle, Pertussis, Tbc)1. Mechanical obstruction(foreign body)2. Cystic fibrosis3. -globulin deficiency(cong./aquired)4. Immotile cilia syndrome

1. Post pneumonia(viral, aspiration)2. Pulmonary tuberculosis3. Asthma, ABPA(allergic bronchopulmonary aspergillosis)4. Toxic fume or congenital5. Neoplasm, Chagas’ disease

연령에 따른 원인

Childhood

Adulthood

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

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

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)

Kartagener’s syndrome

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 mucocili

ary transport

3. Total or near-total absence of dynein arms of the cilia in nasal

or bronchial mucosa

4. Sterility in males

MT : microtubulesC : central sheathR : radial spokesD : dysnein armsN : nexin

Normal Cilia

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

Tuberculosis and Bronchiectasis

1. 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

Clinical Features of Bronchiectasis

Symtoms

sputumchronic coughhemoptysisdyspneafeverchest pain

Signs

cracklesfinger clubbingcyanosiscor pulmonale

• History / Physical examination

• Plain chest X-ray : normal finding in 20%

• HRCT(high resolution CT)

• Bronchogram

Diagnosis

Indications① Preoperative evaluation of unilateral or segmental disease previously identified on CT② Postoperativ evaluation of surgical airway complications such as dehiscence or fistula formation

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

Aim of Treatment

1. Elimination of an identifiable underlying problem

2. Improved clearance of tracheobronchial secretions

3. Control of infection

4. Reversal of airflow obstruction

1. Treatment of predisposing factors hypogammaglobulinemia administration immunogl

obulin tuberculosis anti-tbc medication ABPA steroid2. Bronchial drainage : most important PDPV(postural drainage, percussion, vibration) PEEP deep brathing, continued coughing3. Antibiotics

Treatment : Medical

Treatment : Surgical

Indication

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)

Contraindication

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)

• 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

Cystic Fibrosis

Cystic fibrosis

1. Sweat chloride test > 60 mEq : confirmative 50 – 60 mEq : highly probable < 50 mEq : normal2. Chronic pulmonary disease (99%)3. Pancreatic insufficiency(80-90%)4. CXR increased interstitial marking (98%) cystic bronchiectasis (64%) hyperinflation(58%)

Diagnosis of cystic fibrosis

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