role of flexible bronchoscopy in diagnosis and treatment in children ernst eber, md

Role of flexible bronchoscopy in

diagnosis and treatment in children

Ernst Eber, MD

Respiratory and Allergic Disease Division, Paediatric Department, Medical University of Graz, Austria

Bronchoscopy

• 1897 – open tube, first removal of a foreign body

• Late 1960s – flexible fibreoptic bronchoscope

• 1970s – smaller instruments for paediatric applications

• 1978 – first report on flexible bronchoscopy in infants and children

Rigid vs. flexible bronchoscopy

• Complementary methods

• Each with specific advantages

in different situations

Rigid endoscopy

• Method of choice for

foreign body removal

and other therapeutic

procedures

• Ideal for examination

of posterior aspects of

larynx and trachea

Flexible endoscopy

• Potential preservation of spontaneous ventilation

(vocal cord movements!)

• Entire upper airway visible

• Ideal for assessment of dynamic airway lesions

• Low incidence of complications

Flexible endoscopes considered the instruments

of choice for most diagnostic endoscopies

De Blic J et al. Eur Respir J 2002

“When in doubt as to whether bronchoscopy

should be performed, bronchoscopy should

always be performed.”

Chevalier Jackson, 1915

Flexible bronchoscopy – indications

Wood RE. Pediatr Clin North Am 1984

De Blic J et al. Eur Respir J 2002

ERS Task Force. Eur Respir J 2003;22:698-708.

Wood Pediatr Clin North Am 1984;31:785

Stridor

• Often the most prominent symptom of UAO

• Heard predominantly during inspiration

• Indicative of substantial narrowing or obstruction

of the larynx or extrathoracic trachea

increased velocity and turbulence of airflow

vibration of aryepiglottic folds or vocal cords

• Patients with more than 50% obstruction may be

asymptomatic!

Persistent stridor

n = 124

Results

laryngomalacia (n=95)

membranous subglottic stenosis (n=11)

subglottic haemangioma (n=10)

vocal cord paralysis (n=10; 3 bilateral)

Persistent stridor

Results cont.

cartilaginous subglottic stenosis (n=3)

laryngeal cyst (n=3)

laryngeal web (n=3)

malacia of the extrathoracic trachea (n=1)

laryngeal papillomatosis (n=1)

epiglottis bifida (n=1)

In 14-26% of patients with persistent stridor,

significant additional lower airway

abnormalities, or two or more synchronous

airway lesions may be detected.

Wood Pediatr Clin North Am 1984;31:785

Gonzalez Ann Otol Rhinol Laryngol 1987;96:77

Eber Monatsschr Kinderheilkd 1996;144:43

Persistent stridor

Summary

• Stridor is visible

• Additional pathology in the lower airways is

relatively common complete examination

of the respiratory tract

Persistent stridor

• Most common congenital laryngeal anomaly and

most common cause of persistent stridor in infancy

• Specific disease state with ill-defined pathogenesis

(specific aetiology still obscure)

• Anatomical abnormality or delayed development

in neuromuscular control?

• Worsened by application of lidocaine

Nielson Am J Respir Crit Care Med 2000;161:147

Laryngomalacia

Laryngomalacia

Haemangioma

Eber Paediatr Respir Rev 2004;5:9

Cyst (base of tongue)

Vocal cord paralysis

Laryngeal cyst

Laryngeal web

Atypical croup

Age less than 6 months

Prolonged symptoms

No response to treatment

Vocal cord dysfunction

• Inappropriate vocal cord adduction during inspiration or during both inspiration and expiration

• Often misdiagnosed as asthma

• Often initiated by emotional / physical stress or URTI

• Gastro-oesophageal reflux may play a causative role

Upper airway obstruction

Airway protection may have priority

over diagnostic procedures.

(“Whatever else you do, maintain an

adequate airway”)

For many patients with UAO,

flexible endoscopy is by far the most

important diagnostic tool.

Persistent wheezing

Tracheobronchial stenosis – causes

• Infections Acute laryngotracheobronchitis, bacterial tracheitis

• Accidents/trauma Foreign body, postintubation injury, airway burn, external trauma

• Tumors Bronchogenic cyst, enlarged lymph node, mediastinal tumor

• Congenital Fixed stenosis (incl. webs, cysts), dynamic stenosis (malacia)

Tracheo-/bronchomalacia

• congenital - acquired

• localised - generalised

• primary - secondary

Wood RE. Pediatr Clin North Am 1984

61 children n %

• Normal 8 13 • Abnormalities of lower airways 48 79

– Tracheomalacia 12 20– Tracheal compression 7 11 – Compression of left main bronchus 9 15 – Bronchial compression 2 3 – Foreign body 7 11 – Miscellaneous 11 18

• Abnormalities of upper airways 5 8 – Subglottic oedema 2 3– Laryngomalacia 3 5

Persistent wheezing

Schellhase DE et al. J Pediatr 1998

30 children (0 - 18 months)

• FB for recurrent wheezing

• Abnormalities of the airways 17– Segmental tracheomalacia 12

with vascular compression 10

– Laryngomalacia 6

• Abnormalities more frequent in children 0 - 6 months old

Recurrent wheezing

F.B., male, 6.8 yrsPrimary tracheomalacia

Persistent wheezing

M.A., male, 7.8 yrsSecondary tracheomalacia due to double aortic arch

Persistent wheezing

L.K., female, 15.1 yrsSecondary tracheomalacia due to pulmonary vascular sling

Persistent wheezing

„always suspect foreign body“

Persistent wheezing

Wood Pediatr Clin North Am 1984;31:785

De Blic J et al. Eur Respir J 2002

96 consecutive children (43m, 53f; age 1.7 ± 4.6 years)

with recurrent or persistent atelectasis

• Middle lobe 29 patients

• Right upper lobe 26 patients

• Left upper lobe 3 patients

• Right / left lower lobe 11 patients

• Right / left lung 17 patients

• Segmental 10 patients

Atelectasis

Flexible bronchoscopy

96 consecutive children (43m, 53f; age 1.7 ± 4.6 years)

with recurrent or persistent atelectasis

• Bronchial stenosis / bronchomalacia 42 patients

• Inflammation / mucus plugging 24 patients

• Granulation tissue 10 patients

Endobronchial tuberculosis 5 patients

• Carcinoid 1 patient

• No bronchial pathology 6 patients

Atelectasis

Flexible bronchoscopy

Atelectasis

Flexible bronchoscopyFlexible bronchoscopy Bronchial lavageBronchial lavage

Tb – positive:Tb – positive:

- microscopymicroscopy

- PCRPCR

- cultureculture

- MTDMTD

2 weeks prior to admission at admission

Atelectasis

CT scan

Flexible bronchoscopy

Recurrent/persistent pneumonia

V.M., female, 2 monthsH-type tracheo-oesophageal

fistula

Wood Pediatr Clin North Am 1984;31:785

• Endoscopic evaluation every 6 – 12 months (more

frequently in infants, patients with cerebral palsy or spinal

deformity, patients with unstable/rapidly changing medical

condition or severe complications)

• In children with acute complications (bleeding, UAO)

• Prior to decannulation (removal of the tube during

endoscopy)

Paediatric tracheostomy

Flexible endoscopy

Wood Pediatr Pulmonol 1985Bagley Chest 1994

Eber Wien Klin Wochenschr 1995 American Thoracic Society Am J Respir Crit Care Med 2000

Midulla Eur Respir J 2003

Paediatric tracheostomy

Flexible endoscopy

Tracheal granuloma Suction trauma

ERS Task Force. Eur Respir J 2000;15:217-231.

Bronchoalveolar lavage

Indications

• Diagnostic

- immunocompetent child

- immunocompromised child

• Therapeutic

• Research

Bronchoalveolar lavage

• Microbiological studies

• Cellular components- - Total & differential cell

counts

- Lymphocyte subsets

- Specific inclusions

• Noncellular components

Eber E. Journal of Bronchology 1998

Different disease processes and pretreatment with

antibiotics and antifungals affect the yield from BAL

BAL early in the course of the disease, ideally

before starting treatment (may also help to decrease

morbidity from therapy)

BAL – microbiological studies

BAL – microbiological studies

Bacterial infection vs. contamination

• Quantitative cultures (≥ 105 CFU/ml)

• Bilateral BAL

• Protected BAL

BAL – microbiological studies

Results must be interpreted with care, with regard to the underlying disease, the history, and the whole clinical picture

Diagnostic: M. tuberculosis, L. pneumophila, M. pneumoniae, P. carinii, Nocardia, Histoplasma, Blastomyces, influenza virus, RSV

Not diagnostic: atypical mycobacteria, bacteria, Aspergillus, Candida, CMV, HSV

BAL – microbiological studies

Pseudoinfections relatively common

Stringent adherence to cleaning and disinfection guidelines

Routine microbiological checks of instruments

Diagnostic BAL

Immunocompetent child

• Pulmonary infection

• Tuberculosis

• Cystic fibrosis

• Non-infectious lung diseases

BAL – immunocompetent child

Pulmonary infection

• Less clear role than in the immunocompromised

child (retrospective study: diagnostic yield 30%)

• Empiric antibiotic therapy still the standard

treatment for pneumonia

• BAL should not be performed routinely, but in

patients unresponsive to empiric therapy or in a

severe clinical condition

BAL – immunocompetent child

Tuberculosis

• Chest radiographs frequently underestimate bronchial involvement in children

• Bronchoscopy valuable in management (assessment of bronchial involvement, need for steroids)

• Role in diagnosis of pulmonary tuberculosis not clear (BAL vs. gastric aspirate)

Gastric aspirates vs. BAL fluid in infants with endobronchial tuberculosis

Patient, gender, agePatient, gender, age gastric aspiratesgastric aspirates bronchial lavage fluidbronchial lavage fluid

MJ, f, 7 months culture&PCR 1x positive all positive*

HK, f, 9 months negative all positive*

KJ, f, 9 months negative all positive*

FE, m, 9 months culture 1x positive all positive*

MC, f, 11 months PCR 1x positive all positive*

MM, f, 15 months negative all positive*

HN, f, 18 months culture 1x positive ZN, culture, PCR positive

*: microscopy (ZN), cultures (liquid, solid), PCR, MTD

Thalhammer GH et al. Eur Respir J 2000

BAL – immunocompetent child

Cystic fibrosis

• Valuable in identifying the need for antibiotics in young children (before such a need is clinically apparent)

• Research tool

• Therapeutic role?

Bronchoalveolar lavage or oropharyngeal cultures to identify lower respiratory

pathogens in infants with cystic fibrosis

Armstrong DS et al. Pediatr Pulmonol 1996

Oropharyngeal cultures:

• Sensitivity 82%• Specificity 83%• Positive predictive value 41%• Negative predictive value 97%

Diagnostic accuracy of oropharyngeal cultures in infants and young children with

cystic fibrosis

Rosenfeld M et al. Pediatr Pulmonol 1999

Oropharyngeal cultures(P. aeruginosa, children 18

months):

• Sensitivity 44%• Specificity 95%• Positive predictive value 44%• Negative predictive value 95%

Foamy macrophagesNeutrophilic alveolitis

BAL – immunocompetent child

Non-infectious lung diseases

Disease Cytology CD4/CD8

Pulmonary haemorrhage Haemosiderin laden AM

Pulmonary alveolar proteinosis

Milky fluid, foamy AM

Langerhans cell histiocytosis

Langerhans cells CD1a>5%

Sarcoidosis Lymphocytosis

Hypersensivity pneumonitis

Lymphocytosis or normal

Collagen vascular disorders

Lymphocytosis/Neutrophilia

or

Bowel diseases Lymphocytosis

Pulmonary fibrosis Neutrophilia

ERS Task Force. Eur Respir J ERS Task Force. Eur Respir J 20042004

Non-infectious lung diseases

BAL – immunocompetent child

• In a few diseases diagnostic

• In several diseases useful (at least in eliminating a number of causes of ILD)

• Research tool (e.g. evaluation of disease activity)

Non-infectious lung diseases

BAL – immunocompetent child

BAL – immunocompromised child

HIV infection

• Microbiologic yield 55-84% (P. carinii and

other fungi, viruses, and bacteria)

• Sensitivity of BAL for P. carinii greater than

90% (superior to TBB)

BAL – immunocompromised child

Immunodeficiencies, haematologic diseases,

post bone marrow / solid organ transplantation

• Diagnostic yield 27-86% (P. carinii, CMV, other microorganisms)

• Differences in the diagnostic yield due to differences in the underlying disease, pretreatment, techniques for detection of organisms etc.

BAL – immunocompromised child

Bronchoscopy and BAL safe and effective

First line investigation in the exploration

of acute pneumonia and acute/chronic

interstitial pneumonitis

Diagnostic BAL

Summary

• Well-established role in the diagnosis of pulmonary infections, especially in immunocompromised children. Molecular methods allow identification of pathogens from small samples.

• Role in children with non-infectious interstitial lung diseases still to be defined.

Therapeutic BAL

• Alveolar proteinosis

• Cystic fibrosis ?

Flexible bronchoscopy

Contraindications

• Absolute: Investigation will yield no information

of value

The same diagnostic information can

be obtained by a less invasive method

• Relative: Severe pulmonary hypertension

Severe hypoxaemia

Severe airway stenosis / bronchospasm

Uncorrected bleeding diathesis

Massive haemoptysis

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