Role of flexible bronchoscopy in diagnosis and treatment in children Ernst Eber, MDRespiratory and Allergic Disease Division, Paediatric Department, Medical University of Graz, Austria

Bronchoscopy1897 open tube, first removal of a foreign bodyLate 1960s flexible fibreoptic bronchoscope1970s smaller instruments for paediatric applications1978 first report on flexible bronchoscopy in infants and children

Rigid vs. flexible bronchoscopyComplementary methods

Each with specific advantages in different situations

Rigid endoscopyMethod of choice for foreign body removal and other therapeutic proceduresIdeal for examination of posterior aspects of larynx and trachea

Flexible endoscopyPotential preservation of spontaneous ventilation (vocal cord movements!)Entire upper airway visibleIdeal for assessment of dynamic airway lesionsLow 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

StridorOften the most prominent symptom of UAOHeard predominantly during inspirationIndicative of substantial narrowing or obstruction of the larynx or extrathoracic trachea increased velocity and turbulence of airflow vibration of aryepiglottic folds or vocal cordsPatients with more than 50% obstruction may be asymptomatic!

Persistent stridorn = 124Results

laryngomalacia (n=95)membranous subglottic stenosis (n=11)subglottic haemangioma (n=10)vocal cord paralysis (n=10; 3 bilateral)

Persistent stridorResults 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;144:43Persistent stridor

Summary

Stridor is visible

Additional pathology in the lower airways is relatively common complete examination of the respiratory tractPersistent stridor

Most common congenital laryngeal anomaly and most common cause of persistent stridor in infancySpecific 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;161:147Laryngomalacia

Laryngomalacia

HaemangiomaEber Paediatr Respir Rev 2004;5:9

Cyst (base of tongue)

Vocal cord paralysis

Laryngeal cyst

Laryngeal web

Atypical croupAge less than 6 monthsProlonged symptoms

No response to treatment

Vocal cord dysfunctionInappropriate vocal cord adduction during inspiration or during both inspiration and expirationOften misdiagnosed as asthmaOften initiated by emotional / physical stress or URTIGastro-oesophageal reflux may play a causative role

Upper airway obstructionAirway 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 causesInfections Acute laryngotracheobronchitis, bacterial tracheitisAccidents/trauma Foreign body, postintubation injury, airway burn, external traumaTumors Bronchogenic cyst, enlarged lymph node, mediastinal tumorCongenital Fixed stenosis (incl. webs, cysts), dynamic stenosis (malacia)

Tracheo-/bronchomalaciacongenital - acquiredlocalised - generalisedprimary - secondary

Wood RE. Pediatr Clin North Am 198461 childrenn %

Normal813 Abnormalities of lower airways 4879Tracheomalacia1220Tracheal compression711 Compression of left main bronchus915 Bronchial compression23 Foreign body711 Miscellaneous1118 Abnormalities of upper airways58 Subglottic oedema23Laryngomalacia35Persistent wheezing

Schellhase DE et al. J Pediatr 199830 children (0 - 18 months)

FB for recurrent wheezingAbnormalities of the airways 17Segmental tracheomalacia12 with vascular compression10Laryngomalacia6Abnormalities more frequent in children 0 - 6 months oldRecurrent wheezing

F.B., male, 6.8 yrsPrimary tracheomalaciaPersistent wheezing

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

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

always suspect foreign bodyPersistent 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 lobe29 patientsRight upper lobe26 patientsLeft upper lobe 3 patientsRight / left lower lobe11 patientsRight / left lung17 patientsSegmental10 patientsAtelectasis

Flexible bronchoscopy

96 consecutive children (43m, 53f; age 1.7 4.6 years) with recurrent or persistent atelectasis

Bronchial stenosis / bronchomalacia42 patientsInflammation / mucus plugging24 patientsGranulation tissue10 patients Endobronchial tuberculosis5 patientsCarcinoid1 patientNo bronchial pathology6 patientsAtelectasis

Flexible bronchoscopy

Atelectasis

Flexible bronchoscopyBronchial lavage

Tb positive:microscopyPCRcultureMTD

2 weeks prior to admissionat admissionAtelectasis

CT scan

Flexible bronchoscopy

Recurrent/persistent pneumoniaV.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 endoscopyWood Pediatr Pulmonol 1985Bagley Chest 1994Eber Wien Klin Wochenschr 1995 American Thoracic Society Am J Respir Crit Care Med 2000Midulla Eur Respir J 2003

Paediatric tracheostomy

Flexible endoscopyTracheal granulomaSuction trauma

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

Bronchoalveolar lavageIndications

Diagnostic- immunocompetent child- immunocompromised child

Therapeutic

Research

Bronchoalveolar lavageMicrobiological 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 studiesBacterial infection vs. contamination

Quantitative cultures ( 105 CFU/ml)

Bilateral BAL

Protected BAL

BAL microbiological studiesResults 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 studiesPseudoinfections relatively common

Stringent adherence to cleaning and disinfection guidelines

Routine microbiological checks of instruments

Diagnostic BALImmunocompetent child

Pulmonary infection Tuberculosis Cystic fibrosis Non-infectious lung diseases

BAL immunocompetent childPulmonary 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 childTuberculosis

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*: microscopy (ZN), cultures (liquid, solid), PCR, MTDThalhammer GH et al. Eur Respir J 2000

Patient, gender, agegastric aspiratesbronchial lavage fluidMJ, f, 7 monthsculture&PCR 1x positiveall positive*HK, f, 9 monthsnegativeall positive*KJ, f, 9 monthsnegativeall positive*FE, m, 9 monthsculture 1x positiveall positive*MC, f, 11 monthsPCR 1x positiveall positive*MM, f, 15 monthsnegativeall positive*HN, f, 18 monthsculture 1x positiveZN, culture, PCR positive

BAL immunocompetent childCystic 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 fibrosisArmstrong 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 fibrosisRosenfeld 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 alveolitisBAL immunocompetent childNon-infectious lung diseases

ERS Task Force. Eur Respir J 2004Non-infectious lung diseasesBAL immunocompetent child

DiseaseCytologyCD4/CD8 Pulmonary haemorrhageHaemosiderin laden AMPulmonary alveolar proteinosisMilky fluid, foamy AMLangerhans cell histiocytosisLangerhans cells CD1a>5%SarcoidosisLymphocytosis Hypersensivity pneumonitisLymphocytosis or normalCollagen vascular disordersLymphocytosis/Neutrophilia or Bowel diseasesLymphocytosis Pulmonary fibrosisNeutrophilia

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 diseasesBAL immunocompetent child

BAL immunocompromised childHIV 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 childImmunodeficiencies, 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 childBronchoscopy and BAL safe and effective

First line investigation in the exploration of acute pneumonia and acute/chronic interstitial pneumonitis

Diagnostic BALSummary

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 BALAlveolar proteinosis

Cystic fibrosis ?

Flexible bronchoscopyContraindications

Absolute:Investigation will yield no information of valueThe same diagnostic information can be obtained by a less invasive method

Relative:Severe pulmonary hypertensionSevere hypoxaemiaSevere airway stenosis / bronchospasmUncorrected bleeding diathesisMassive haemoptysis

lginiz iin teekkr ederim!

**In children with persistent wheezing, only 27 % of findings were described as normal. The others... *In a group of 30 children with recurrent obstructive bronchitis, Schelhase and colleagues have found airway abnormalities in 57% of children.****