Bronchiectasis in children is an irreversible dilation of the airways caused by destructive changes to the airway walls. It has many causes including cystic fibrosis, infections, immunodeficiencies, and anatomical defects. The pathology involves a vicious cycle of impaired mucus clearance leading to recurrent infections, inflammation, and further airway damage. Symptoms include cough, sputum production, and breathing difficulties. Diagnosis is made through imaging like HRCT that shows changes to airway contours. Treatment focuses on airway clearance techniques and controlling infections with antibiotics. Management of underlying conditions and lung transplantation may be needed in severe cases.

1. Bronchiectasis in Children
Dr. Virendra Kumar Gupta
Assiociate Professor
Department Of Pediatrics
NIMS Medical College & Hospital , Jaipur

2. Definition…..
Chronic abnormal, permanent and
irreversible dilation of the medium
sized sub segmental bronchus
due to
destructive changes in the elastic
and muscular layers of bronchial
walls.
May be diffuse or localized
resulting in
impairment of the drainage
of bronchial secretions.

5. Etiology
Congenital
• Cystic Fibrosis (Most common cause)
• Primary hypogammaglobinemia leading to recurrent
infection
• Ciliary dysfunction syndrome
Acquired (In children)
• Secondary to pneumonia which occurs often as
complication of whooping cough and measles

6. 1. Childhood
Cystic Fibrosis, Pertussis, measles,
Necrotizing pneumonia
2. Primary infections
Pseudomonas
klebsiella species
staphylococcal species
Mycoplasma pneumoniae
Mycobacterium tuberculosis
Measlesvirus
Influenza virus
Herpes simplexvirus
Adenovirus
Pertusisvirus

7. 3. Obstruction
Foreign body
4. Tumor
Laryngeal papillomatosis,adenoma,
bronchogenic , carcinoma
5. Lymphadenopathy
Tuberculosis, histoplasmosis
6. Mucoid impaction
Allergic bronchopulmonary aspergillosis,
bronchocentric granulomatosis, fibrinous bronchitis

8. 7.Congenital anatomicdefect
• Williams-Campbell syndrome (congenital cartilage
deficiency)
• Mounier-Kuhn syndrome (tracheo-bronchomegaly)
• Swyer-James syndrome(unilateral hyperlucent lung),
pulmonary sequestration, pulmonaryartery aneurysm
• yellow nail syndrome(Hypoplastic lymphatics, pleural
effusion, yellow nails )

9. 8.Immunodeficiency state
IgG subclass deficiency, X-linked,
agammaglobulinemia, selective IgA, IgM, or IgE
deficiency, bare lymphocyte syndrome, chronic
granulomatous disease, Nezelof syndrome (Thymic
dysplasia with normal immunoglobulins)
9.Hereditaryabnormality
Dyskinetic cilia syndrome,
Kartagener’s syndrome (situs inversus,
nasal polyposis and bronchiectasis)
α 1-antitrypsin deficiency
cystic fibrosis, ADPKDK

10. Pathogenesis
The common thread in the pathogenesis of
bronchiectasis
consists of difficulty clearing secretions & recurrent
infections with a
“vicious circle”
of infection and inflammation
resulting in airway injury and remodelling.

11. Pathogenesis of Bronchiectasis: The Vicious Cycle
Destruction
of mucociliary and
cartilagenous supporing
structures
Release
of inflammatory cytokines
peroxidases, proteinases
elastase, etc.
Colonization
and biofilm formation
intermittent dispersals
Infection
with acute inflammation
and recruitment of
inflmmatory cells
Impairment
mucociliary clearance
sputum retention
Loss
of ventilatory
function

13. 3 mechanisms:
1.Obstruction- can occur because of tumour, foreign
body, impacted mucus due to poor muco-ciliary
clearance, external compression, bronchial webs, and
atresia.
2.Infections d/t Bordetella pertusis, measles, rubella,
adenovirus, and mycobacterium tuberculosis induce
chronic inflammation.

14. 3. Chronic inflammation contributes to the mechanism
by which obstruction leads to bronchiectasis.
• Inflammatory mediators such as neutrophil elastase,
interleukin-6, interleukin-8, and Tumor necrosis factor-
α (TNF-α) have been found to be elevated in the
airways of patients with bronchiectasis

15. Pathological forms of Bronchiectasis
• Cylindrical bronchiectasis- bronchial outlines are
regular, but there’s diffuse dilatation of the bronchial
unit. Bronchial lumen ends abruptly because of mucous
plugging.
• Tramline appearance on CT scan.
• Varicose bronchiectasis- degree of dilatation is greater,
local constrictions cause irregularity of outline
resembling that of varicose veins.
• Beaded contour on CT scan.

16. Cont..
• Saccular (Cystic) bronchiectasis- bronchial dilatation
progresses and results in ballooning of bronchi that
end in fluid or mucous filled sacs.
• Most severe form of Bronchiectasis.
• Prebronchiectasis- chronic or recurrent
endobronchial infection with non specific HRCT
changes – may be reversible.

18. Reid’s subtypes
CysticCylindrical Varicose

19. • Cough with mucopurulent expectoration(copious amount,
foul smelling)
• Haemoptysis
• Breathlessness
• Fever
• Postural variation of symptoms
• Chest pain

20. • Poor general condition
• Tachypnea
• Dyspnea - Use of accesory muscle of respiration
• Clubbing
• BS – harsh with prolonged expiration
• Coarse leathery crackles
• wheeze

21. Diagnosis
• Thin-section HRCT scanning- Gold standard
excellent sensitivity and specificity
• CT - Disease location, presence of mediastinal lesions, and
the extent of segmental involvement.
• Chest X-ray- increase in size and loss of bronchovascular
markings, crowding of bronchi, and loss of lung volume.
Severe case: Honeycombing
• Sputum culture
• Bronchoscopy

27. • Local
• Haemoptysis
• Secondary bacterial infections
• Fungal infections
• Tuberculosis
• Lung abscess
• Frequent exaceberations
• Systemic
• Respiratory failure
• Cor pulmonale
• Pulmonary artery hypertension
• Sinusitis
• Allergic broncho pulmonary
aspergillosis
• (ABPA)
• Aspergilloma
• Brain abscess
• Secondary amyloidosis

28. Treatment
• Aims at decreasing airway obstruction and controlling infection.
• Postural drainage and control Infection.
• 2 to 4 wk of parenteral antibiotics is often necessary to manage acute
exacerbations adequately.
• Amoxicillin/ Clavulanic acid (22.5mg/kg/dose twice daily) has been
successful at treating the exacerbations.
• Long-term prophylactic oral (macrolide) or nebulized antibiotics (e.g.,
tobramycin, colistin, aztreonam) may be beneficial.
• Airway hydration (inhaled hypertonic saline or mannitol) also improves
quality of life in adults with bronchiectasis.
• Any underlying disorder (immunodeficiency, aspiration) that may be
contributing must be addressed.

29. Supportive Treatment
Cessation of smoking
Avoidance of second-handsmoking
Adequate nutritional intake
Immunizations for influenza andpneumococcal
pneumonia
Conformation of immunizations for measles,rubella
and pertusis
Oxygen therapy is reserved for patients with
hypoxemiaand end stagecomplicationssuch as cor-
pulmonale

30. Tapping of the chest wallto
dislodge thesecretions

31. Positional drainageand
physiotherapy

32. Positive expiratory thera- PEP

33. Use of oscillator

34. Use of Flutter

35. AcapellaDevice

36. Use of avest

37. • EQUIPMENT:
• 1) Oxygen mask
• 2 ) Emesis basin or sputum cup .
• 3 ) Pulse oximeter .
• 4 ) Tissues
• 5) Pillows

51. • CONTRA INDICATIONS TO CHEST PT:
• Unstabilized head and/or neck injury
• Active hemorrhage with hemodynamic instability or
significant possibility of occurrence.
• Osteogenesis imperfecta or other bone disease associated
with brittle or extremely fragile bones/ Fracture of ribs
• OTHER CONTRA INDICATIONS:
• Intracranial pressure > 20 mm Hg
• Active hemoptysis
• Acute spinal injury/ Spine surgery
• Pulmonary embolism
• Worsening bronchospasm etc

52. Helpful in advanced or complicateddisease.
Indications :
1. Patientswho have focal disease that is poorly
controlled by anti-biotics.
2.Reduction of acute infectiveepisodes
3. Massive haemoptysis(Alternatively bronchial artery
embolization may beattempted)
4. Foreign body or tumorremoval
5. Consideration in the treatment of MACor
Aspergillus specific infections
Surgical resection

53. Lung transplantation
Single or double lung transplantation for severe
bronchiectasis, predominantly related to CF. FEV1 < 30
and in youngerpatients it may beconsidered.

54. Prognosis
• Children with bronchiectasis often suffer from
recurrent pulmonary illnesses.

55. Bronchiectasis: Summary
Abnormal irreversibly dilated and often thick-walled bronchi
Pathogenesis related to one or more defects of mucociliary
clearance, cellular and immunity defense mechanism or
presence of associated conditions
“The vicious cycle” and P aeruginosa contributes progression
and severity of disease
Imaging greatly helps in diagnosis: Tram line, honeycombing,
cystic, signet ring sign
Additional test may be required in specific clinical settings
Microbiology of the diseased airway may aid proper
antimicrobial therapy

56. THANK YOU
Dr. Virendra Kumar Gupta
Assiociate Professor
Department Of Pediatrics
NIMS Medical College & Hospital , Jaipur