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Congenital Larynx Lesions & Stridor Evaluation

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Presentation on theme: "Congenital Larynx Lesions & Stridor Evaluation"— Presentation transcript:

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 Cordotomy 3. Cordectomy 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

51 Cordotomy + arytenoidectomy

52 Glottic web Treatment: Endoscopic division with knife / laser &
insertion of McNaught laryngeal keel

53 Glottic stenosis Treatment: Endoscopic division with knife / laser &
insertion of McNaught laryngeal keel

54 McNaught Keel

55 Cri-du-chat syndrome Cri – du – chat means cry of the cat
Partial depletion of short arm of chromosome 5 High pitched mewing stridor Diamond shaped glottic space, narrow vocal cords, curved & elongated supraglottis Treatment: 1. Supportive care 2. Genetic counseling

56 Sub-glottic abnormalities

57 Congenital subglottic stenosis
Definition: diameter of subglottic lumen < 4 mm in term infant & < 3 mm in pre-term infant Etiology: Incomplete recanalization of laryngo tracheal tube during 3rd month of gestation Types: 1. Membranous: more common & mild form 2. Cartilaginous: less common & severe form Clinical presentation: Symptoms appear in first few months of life. Biphasic stridor. Cry is normal.

58 Flexible laryngoscopy

59 Radiology

60 Treatment Most cases resolve spontaneously by 4 years.
Tracheostomy for significant stridor. Tube removed by 4 years when subglottic space widens. Laser ablation for membranous stenosis < 5 mm. Crico-tracheal resection & Laryngo-tracheo-plasty in patients who could not be decannulated.

61 Tracheostomy

62 Laryngo-tracheoplasty

63 Subglottic hemangioma
Capillary hamartomas Symptoms appear by age 2-12 months Biphasic stridor, barking cough & hoarse cry 50% have cutaneous hemangiomas of head & neck Flexible laryngoscopy: unilateral or bilateral lesion Located postero-laterally in subglottis submucosa, pink-blue in color, sessile & easily compressible

64 Flexible laryngoscopy

65 Management Observation: for small lesions without stridor
Tracheostomy: for significant airway obstruction. Tube kept till 5 years. Specific treatment: 1. Laser ablation Cryosurgery 3. Sclerosing agent: intra-lesional injection 4. Open surgical excision

66 Subglottic web Treatment: Endoscopic division with knife
/ laser & insertion of McNaught laryngeal keel

67 Evaluation of Stridor

68 Stridor vs. Stertor Stertor is noisy respiration due to turbulent air flow through partially narrowed air passage above larynx Stridor is noisy respiration due to turbulent air flow through partially narrowed air passage at or below level of larynx

69 Etiology for stertor Nasal: choanal atresia, ethmoid polyps
Mandible: Pierre Robin syndrome Tongue: macroglossia, lingual thyroid Pharynx: adeno-tonsillar hypertrophy, retro- pharyngeal abscess, neoplasm Miscellaneous: Ludwig’s angina, Maxillo-facial #

70 Etiology for stridor

71 Congenital Acquired  Laryngomalacia 1. Inflammatory:  Vocal cord palsy Acute epiglottitis, croup,  Subglottic stenosis laryngeal edema, T.B.  Subglottic hemangioma 2. Trauma: accidental,  Laryngeal web & atresia iatrogenic, heat, chemical  Laryngeal cyst Neoplasm  Vascular compression on 4. Foreign body trachea B/L vocal cord palsy

72 Causes of B/L vocal cord palsy
Thyroid surgery Ca thyroid Cancer cervical esophagus Cervical lymphadenopathy

73 History Taking 1. Congenital or acquired after birth
2. Present only during sleep  stertor 3. Related to feeding  aspiration due to laryngeal paralysis, esophageal obstruction 4. Foreign body, blunt injury, endoscopy, intubation 5. Sudden onset  foreign body, injury, infection 6. Long standing + progressive  Laryngomalacia, laryngeal stenosis, neoplasm

74 Physical Examination 1. Respiratory timing of stridor:
Inspiratory  supraglottis or pharynx Biphasic  glottis, subglottis or cervical trachea Expiratory  lower trachea, bronchi or alveoli 2. Signs of airway resistance: nasal flaring, intercostal / subcostal / supraclavicular recession, cyanosis

75 Physical Examination 3. Associated fever: inflammatory cause
4. Stridor disappears in prone position: laryngomalacia, macroglossia, micrognathia, vascular compression of trachea 5. Resting respiratory rate: look for tachypnoea 6. Resting heart rate: look for tachycardia

76 Investigations Arterial blood gas analysis: for hypoxia
X-Ray soft tissue neck: for epiglottitis, stenosis X-Ray chest: for mediastinal lesion Flexible laryngoscopy & bronchoscopy Direct laryngoscopy & rigid bronchoscopy C.T. scan of neck & chest M.R.I. of neck & chest Barium swallow & esophagoscopy

77 Thank You

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