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Bronchiectasis
Dr Murali Krishna MD
Senior resident
Dept of Pulmonary medicine.
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
)
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
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).
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 %
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 .
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.
Role of obstruction in Pathogenesis
Types :
 Tubular / cylendrical
 Varicose- beaded apperance
 Cystic / sacular – grape like clusters
- severe form
Tubular Cystic
Varicose
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
 Signs of collapse/ fibrosis.
 Advanced cases : Cyanosis,
Signs of pulmonary hypertension
Right heart failure
PFT : Obstructive defect
Decreased DLCO
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
Diagnosis :
 Radiological :
Chest X ray
Bronchogram
HRCT
 Other supportives :
Sputum/LRT aspirates for C/S
PFT
2D echo
 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
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.
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
 SPUTUM C/S :
usually yields H. influenzae
Strep. pneumoniae
Staph. aureus
Ps.aeruginosa & Klebsiella spp – chronic
and severe cases.
antibiotics directed against these microbes.
Treatment :
 Medical - to relieve and control the symptoms
 Surgical - to eliminate the disease itself.
 Physiotherapy – to aid in the drainage of secretions
Antimicrobial therapy
 Guided against sputum/LRT secretions culture &
sensitivity – best
 Empirical Rx –
* Pencillins + B-lactams
( Amoxycillin- clavulonic acid 625 mg tid )
* 3 rd gen Cephalosporins
( Ceftriaxone 1 gm , i.v, bid )
*Macrolides
( Azithromycin 500 mg p.o )
 Pseudomonas infections – chronic / severe cases,
- cystic fibrosis pt
* ceftazidim 1-2 gm i.v bid
* piperacillin +tazobzctam 4.5 gm i.v tid
 Nebulised antibiotics - CF patients
* colistin 1-2 m.u bid
* tobramycin
 Mucolytics - rhDNAase in CF.
Preventive/ supressive therapy :
 To prevent relapses and acute excerabations.
Azithromycin 500 mg p.o weekly three times.
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.
 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 )
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.
Murali bronchiectasis.pptx

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Murali bronchiectasis.pptx

  • 1. Bronchiectasis Dr Murali Krishna MD Senior resident Dept of Pulmonary medicine.
  • 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).
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  • 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 .
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  • 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.
  • 11. Role of obstruction in Pathogenesis
  • 12. Types :  Tubular / cylendrical  Varicose- beaded apperance  Cystic / sacular – grape like clusters - severe form
  • 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
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  • 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
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  • 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
  • 27. Antimicrobial therapy  Guided against sputum/LRT secretions culture & sensitivity – best  Empirical Rx – * Pencillins + B-lactams ( Amoxycillin- clavulonic acid 625 mg tid ) * 3 rd gen Cephalosporins ( Ceftriaxone 1 gm , i.v, bid ) *Macrolides ( Azithromycin 500 mg p.o )
  • 28.  Pseudomonas infections – chronic / severe cases, - cystic fibrosis pt * ceftazidim 1-2 gm i.v bid * piperacillin +tazobzctam 4.5 gm i.v tid  Nebulised antibiotics - CF patients * colistin 1-2 m.u bid * tobramycin  Mucolytics - rhDNAase in CF. Preventive/ supressive therapy :  To prevent relapses and acute excerabations. Azithromycin 500 mg p.o weekly three times.
  • 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.