Role of flexible bronchoscopy in diagnosis and treatment in children Ernst Eber, MD

1. Role of flexible bronchoscopy in diagnosis and treatment in childrenErnst Eber, MD Respiratory and Allergic Disease Division, Paediatric Department, Medical University of Graz, Austria

2. 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

3. Rigid vs. flexible bronchoscopy • Complementary methods • Each with specific advantages in different situations

4. Rigid endoscopy • Method of choice for foreign body removal and other therapeutic procedures • Ideal for examination of posterior aspects of larynx and trachea

5. 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

6. De Blic J et al. Eur Respir J 2002

7. Flexible bronchoscopy – indications “When in doubt as to whether bronchoscopy should be performed, bronchoscopy should always be performed.” Chevalier Jackson, 1915

8. Wood RE. Pediatr Clin North Am 1984

9. De Blic J et al. Eur Respir J 2002

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

11. Wood Pediatr Clin North Am 1984;31:785

12. 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!

13. Persistent stridor n = 124 Results laryngomalacia (n=95) membranous subglottic stenosis (n=11) subglottic haemangioma (n=10) vocal cord paralysis (n=10; 3 bilateral)

14. 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)

15. Persistent stridor 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

16. Persistent stridor Summary • Stridor is visible • Additional pathology in the lower airways is relatively common  complete examination of the respiratory tract

17. 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

18. Laryngomalacia

19. Haemangioma Eber Paediatr Respir Rev 2004;5:9

20. Cyst (base of tongue)

21. Vocal cord paralysis

22. Laryngeal cyst

23. Laryngeal web

24. Atypical croup Age less than 6 months Prolonged symptoms No response to treatment

25. 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

26. Upper airway obstruction For many patients with UAO, flexible endoscopy is by far the most important diagnostic tool. Airway protection may have priority over diagnostic procedures. (“Whatever else you do, maintain an adequate airway”)

27. Persistent wheezingTracheobronchial 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)

28. Tracheo-/bronchomalacia • congenital - acquired • localised - generalised • primary - secondary

29. Persistent wheezing 61 children n % • Normal 8 13 • Abnormalities of lower airways 48 79 • Tracheomalacia 12 20 • Tracheal compression 7 11 • Compression of left mainbronchus 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 Wood RE. Pediatr Clin North Am 1984

30. Recurrent wheezing 30 children (0 - 18 months) • FB for recurrent wheezing • Abnormalities of the airways 17 • Segmental tracheomalacia 12 with vascular compression10 • Laryngomalacia 6 • Abnormalities more frequent in children 0 - 6 months old Schellhase DE et al. J Pediatr 1998

31. Persistent wheezing F.B., male, 6.8 yrs Primary tracheomalacia

32. Persistent wheezing M.A., male, 7.8 yrs Secondary tracheomalacia due to double aortic arch

33. Persistent wheezing L.K., female, 15.1 yrs Secondary tracheomalacia due to pulmonary vascular sling

34. Persistent wheezing „always suspect foreign body“

35. Wood Pediatr Clin North Am 1984;31:785

36. De Blic J et al. Eur Respir J 2002

37. 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 AtelectasisFlexible bronchoscopy

38. 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 AtelectasisFlexible bronchoscopy

39. Atelectasis

40. Flexible bronchoscopy Bronchial lavage Tb – positive: • microscopy • PCR • culture • MTD

41. Atelectasis 2 weeks prior to admission at admission

42. CT scan

43. Flexible bronchoscopy

44. Recurrent/persistent pneumonia V.M., female, 2 months H-type tracheo-oesophageal fistula

45. Wood Pediatr Clin North Am 1984;31:785

46. 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 tracheostomyFlexible endoscopy Wood Pediatr Pulmonol 1985 Bagley Chest 1994 Eber Wien Klin Wochenschr 1995 American Thoracic Society Am J Respir Crit Care Med 2000 Midulla Eur Respir J 2003

47. Paediatric tracheostomyFlexible endoscopy Tracheal granuloma Suction trauma

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

49. Bronchoalveolar lavage • Indications • Diagnostic • - immunocompetent child • - immunocompromised child • Therapeutic • Research

50. Bronchoalveolar lavage • Microbiological studies • Cellular components • - Total & differential cell counts • - Lymphocyte subsets • - Specific inclusions • Noncellular components