1. The Child with Stridor 2: Chronic Stridor Chris Kingsnorth

2. Before We Begin

3. Overview Definition of stridor Differential diagnoses: Croup Acute epiglottitis Bacterial tracheitis Foreign body aspiration Laryngomalacia Subglottic stenosis

4. Stridor: Recap “…High-pitched breath sound resulting from turbulent air flow secondary to narrowing in the upper airway…”

5. Stridor: Recap Timing of stridor suggests the level of narrowing: Inspiratory: Laryngeal region Expiratory: Tracheobronchial region Biphasic: Subglottic/glottic region

6. Differential Diagnoses Acute Croup Acute epiglottitis Foreign body Chronic Laryngomalacia Subglottic stenosis

7. History, Examination and Investigations (Overview) See ‘The Child with Stridor 1: Acute Stridor’

8. Chronic Stridor: Laryngomalacia

9. Laryngomalacia: Epidemiology A.k.a ‘floppy larynx’ Most common cause of stridor overall Most common congenital laryngeal abnormality (60% of all cases) M = F

10. Laryngomalacia: Pathophysiology May affect epiglottis, arytenoid cartilages or both Epiglottis: Elongated and ‘omega-shaped’ Ω Arytenoid cartilages: Enlarged In both cases, cartilage floppy and structures prolapse over larynx during inspiration

11. Laryngomalacia: Classification Type 1Aryepiglottic folds are tightened/ foreshortened Type 2Redundant soft tissue in any area of supraglottic region Type 3 Associated with other disorders (e.g. neuromuscular disease, gastro-oesophageal reflux)

12. Laryngomalacia: Clinical Features Noisy respiration and inspiratory stridor develops within weeks of birth (often absent at birth; typically begins at 4-6 wks) Worse when supine, distressed or feeding Cry normal (distinguishes from abnormality around vocal cords) Otherwise happy, healthy babies 10 week old baby with laryngomalacia. Note that stridor is inspiratory only.

13. Laryngomalacia: Investigations Not required if Hx clear and baby well Laryngoscopy and bronchoscopy most useful for Dx Fluoroscopy Laryngomalacia on laryngoscopy. Note curled ‘omega-shaped’ epiglottis

14. Laryngomalacia: Prognosis Increase in severity of Sx during first 8 months Peak at 9-12 months Resolution thereafter 99% of cases resolve spontaneously

15. Laryngomalacia: Management Pulse oximetry Supportive care and regular review Monitor for signs of OSA (cyanosis, apnoeas, respiratory distress during sleep)  sleep study

16. Laryngomalacia: Management Surgical Mx considered in severe cases (e.g. FTT, respiratory failure, OSA, pulmonary HTN, cor pulmonale) Options include: Tracheotomy Supraglottoplasty (tightening of support structures and removal of excess tissue) Laser epiglottopexy

17. Chronic Stridor: Subglottic Stenosis

18. Subglottic Stenosis: Epidemiology Incidence unknown Can be congenital or acquired: CongenitalMalformation of cricoid cartilage Acquired Most commonly secondary to trauma (esp. intubation) or infection

19. Subglottic Stenosis: Clinical Features Biphasic stridor Respiratory distress Recurrent croup Gastro-oesophageal reflux Children with severe subglottic stenosis at birth may require intubation +/- tracheostomy

20. Subglottic Stenosis: Investigation and Grading Direct laryngoscopy and bronchoscopy Fluoroscopy Grade 3 + 4 = severe

21. Subglottic Stenosis: Management Grade 1Monitor; intervention often not required Grade 2Balloon dilatation Grade 3 Tracheostomy, then laryngotracheal reconstruction (LTR) or partial cricotracheal resection (CTR) Grade 4 Rx gastro-oesophageal reflux if present (prior to surgical intervention)

22. Subglottic Stenosis: Prognosis Dependent on grade In severe subglottic stenosis, success rate of surgical intervention 80-90%

23. What Now? Download slides/ notes pages Online MCQ: https://www.goconqr.com/en- GB/p/3962527-The-Child-with-Stridor-2-- Chronic-quizzeshttps://www.goconqr.com/en- GB/p/3962527-The-Child-with-Stridor-2-- Chronic-quizzes Request a Podcast/ ask a question

24. References Stridor sound clip: https://en.wikipedia.org/wiki/File:Stridor_NP_ OGG_2.ogg https://en.wikipedia.org/wiki/File:Stridor_NP_ OGG_2.ogg Laryngoscopy images: https://www.youtube.com/watch?v=6xOHYOu iSH0 https://www.youtube.com/watch?v=6xOHYOu iSH0 Subglottic stenosis grading: http://www.chop.edu/conditions- diseases/subglottic- stenosis/about#.Vjt8WPnhDIU http://www.chop.edu/conditions- diseases/subglottic- stenosis/about#.Vjt8WPnhDIU