Congenital Larynx Lesions & Stridor Evaluation

1. Congenital Larynx Lesions & Stridor Evaluation Dr. Vishal Sharma

2. Epidemiology • 80 – 85 % children < 3 yrs with stridor have congenital etiology for stridor • 60 % of these anomalies are in larynx • 20-25 % are anomalies of trachea + bronchi • 45% patients have more than 1 anomalies

3. Etiology Supraglottis: Laryngomalacia, Supraglottic web, Saccular cyst, Congenital laryngocoele, Supraglottic cleft Glottis:Vocal cord paralysis, Glottic web, Glottic stenosis, Cri-du-chat syndrome Subglottis: Subglottic stenosis, Subglottic web, Subglottic hemangioma

4. Common congenital lesions • Laryngomalacia (60%) • Congenital vocal cord paralysis (20%) • Congenital subglottic stenosis (15%) • Subglottic hemangioma (1.5%)

5. Supra-glottic abnormalities

6. Laryngomalacia • Most common congenital laryngeal anomaly Etiology: • Exact cause is not known 1. Mal-development of cartilaginous structures 2. Gastro-esophageal reflux disease 3. Immaturity of neuromuscular control

7. Clinical presentation • Symptoms begin few weeks after birth, progress over 9-12 months & resolve by 2 years • Inspiratory stridor:1. increased by:supine position, feeding, resp. infection & exertion (crying).2. relieved by:neck extension & prone position. • Phonation & cry are normal. Feeding difficulties, failure to thrive, dyspnoea & cyanosis are rare.

8. Flexible laryngoscopy • Elongation + longitudinal folding of epiglottis (omega shaped, ), falls postero-inferiorly on inspiration • Redundant bulky arytenoids prolapse anteriorly & medially on inspiration. Shortening + medial collapse of aryepiglottic folds. Expiration results in expulsion of these structures with free flow of air • Rigid bronchoscopy GA: exclude other anomaly

9. Omega-shaped epiglottis

10. Flexible laryngoscopy

11. Inspiration vs. Expiration

12. Treatment 1. 99% cases: reassurance, sleep in prone position 2. Treatment of gastro-esophageal reflux disease 3. Surgical management (for 1% cases): a. Emergency Tracheostomy: kept till 2 yrs age b. Epiglottoplasty: cautery or laser assisted

13. Epiglottoplasty for laryngomalacia

14. Problem: tubular epiglottis

15. Rx: trimming of epiglottis

16. Problem: medial collapse of corniculate cartilages

17. Rx: removing cartilage + redundant mucosa

18. Problem: posterior displacement of epiglottis

19. Rx: epiglottopexy

20. Epiglottopexy

21. Problem: short ary-epiglottic folds

22. Rx: division of ary-epiglottic folds

23. Pre-op vs. Post-op

24. Problem: medial collapse of ary-epiglottic fold

25. Rx: removing wedge of ary-epiglottic folds

26. Congenital laryngocoele Air filled dilatation of ventricular sinus of Morgagni C/F:1. Hoarseness or respiratory distress 2. Neck swelling es on Valsalva maneuver Investigation: 1. Plain X-ray soft tissue neck 2. Flexible laryngoscopy Treatment:1. Endoscopic marsupialization 2. External excision by thyrotomy

27. Swelling es with Valsalva

28. Types of laryngocoele Internal (20%):contained entirely within endolarynx with bulge in false vocal fold & aryepiglottic fold External (30%): only neck swelling without visible endolaryngeal swelling Combined (50%):Also extends into anterior triangle of neck through foramen for superior laryngeal nerve & vessels in thyrohyoid membrane. Dumbbell shaped.

29. Types of laryngocoele Internal External Combined

30. X-ray neck A.P. view

31. Flexible laryngoscopy

32. CT scan: mixed laryngocoele

33. Endoscopic marsupialization

34. External approach

35. Congenital saccular cyst Due to obstruction of orifice of saccule in laryngeal ventricle 40% congenital cysts found within hours of birth 95% of infants have symptoms within 6 months C/F:Inspiratory stridor improves on extension of head, cyanosis, feeding problem & failure to thrive

36. Anterior saccular cyst Smaller in size, project into laryngeal lumen in anterior ventricular region

37. Lateral saccular cyst Larger, present as bulge in false vocal fold or ary-epiglottic fold, extend into neck

38. Treatment 1. Emergency tracheostomy for acute stridor 2. Endoscopic de-roofing or marsupialization:  cold knife  Laser-assisted 3. Endoscopic incision & drainage 4. Total excision:  endoscopic  laryngofissure approach

39. Glottic abnormalities

40. Congenital vocal cord palsy

41. Etiology 1. Idiopathic: most common 2. C.N.S. Lesions:Arnold-Chiari malformation, cerebral palsy, hydrocephalus, myelo- meningocele, spina bifida, hypoxia 3. Birth trauma: a. cervical spine b. recurrent laryngeal nerve 4. Mediastinum lesions:a. tumors b. vascular malformation

42. Clinical Features Unilateral paralysis:4 times common  Hoarse, breathy cry aggravated by agitation  Feeding difficulty  Aspiration Bilateral paralysis:  Biphasic stridor(worsens on agitation)+ near-normal phonation: abductor paralysis  Lung aspiration + aphonia: adductor paralysis

43. Diagnosis: 1. Flexible laryngoscopy shows vocal fold palsy 2. Rigid bronchoscopy  GA: other anomaly Treatment: Bilateral paralysis: 1. Vocal cord lateralization 2. Cordotomy 3. Cordectomy 4.Subtotal arytenoidectomy 5. Tracheostomy Unilateral paralysis: Observation

44. Fibre-optic laryngoscopy paralyzed vocal fold foreshortened, lateralized & flaccid

45. B/L abductor palsy Inspiration Expiration

46. Vocal cord lateralization (laterofixation / cordopexy)

47. Cordectomy

48. Cordectomy + lateralization

49. Posterior cordotomy

50. Arytenoidectomy