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Congenital Larynx Lesions & Stridor Evaluation
Dr. Vishal Sharma
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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
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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
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Common congenital lesions
Laryngomalacia (60%) Congenital vocal cord paralysis (20%) Congenital subglottic stenosis (15%) Subglottic hemangioma (1.5%)
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Supra-glottic abnormalities
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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
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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.
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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
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Omega-shaped epiglottis
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Flexible laryngoscopy
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Inspiration vs. Expiration
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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
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Epiglottoplasty for laryngomalacia
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Problem: tubular epiglottis
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Rx: trimming of epiglottis
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Problem: medial collapse of corniculate cartilages
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Rx: removing cartilage + redundant mucosa
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Problem: posterior displacement of epiglottis
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Rx: epiglottopexy
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Epiglottopexy
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Problem: short ary-epiglottic folds
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Rx: division of ary-epiglottic folds
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Pre-op vs. Post-op
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Problem: medial collapse of ary-epiglottic fold
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Rx: removing wedge of ary-epiglottic folds
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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
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Swelling es with Valsalva
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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.
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Types of laryngocoele Internal External Combined
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X-ray neck A.P. view
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Flexible laryngoscopy
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CT scan: mixed laryngocoele
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Endoscopic marsupialization
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External approach
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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
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Anterior saccular cyst
Smaller in size, project into laryngeal lumen in anterior ventricular region
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Lateral saccular cyst Larger, present as bulge in false vocal fold or ary-epiglottic fold, extend into neck
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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
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Glottic abnormalities
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Congenital vocal cord palsy
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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
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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
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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
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Fibre-optic laryngoscopy
paralyzed vocal fold foreshortened, lateralized & flaccid
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B/L abductor palsy Inspiration Expiration
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Vocal cord lateralization (laterofixation / cordopexy)
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Cordectomy
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Cordectomy + lateralization
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Posterior cordotomy
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Arytenoidectomy
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Cordotomy + arytenoidectomy
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Glottic web Treatment: Endoscopic division with knife / laser &
insertion of McNaught laryngeal keel
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Glottic stenosis Treatment: Endoscopic division with knife / laser &
insertion of McNaught laryngeal keel
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McNaught Keel
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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
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Sub-glottic abnormalities
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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.
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Flexible laryngoscopy
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Radiology
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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.
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Tracheostomy
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Laryngo-tracheoplasty
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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
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Flexible laryngoscopy
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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
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Subglottic web Treatment: Endoscopic division with knife
/ laser & insertion of McNaught laryngeal keel
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Evaluation of Stridor
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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
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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 #
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Etiology for stridor
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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
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Causes of B/L vocal cord palsy
Thyroid surgery Ca thyroid Cancer cervical esophagus Cervical lymphadenopathy
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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
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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
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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
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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
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Thank You
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