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Congenital Larynx Lesions & Stridor Evaluation. Dr. Vishal Sharma. 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. Etiology.
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Congenital Larynx Lesions & Stridor Evaluation Dr. Vishal Sharma
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
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
Common congenital lesions • Laryngomalacia (60%) • Congenital vocal cord paralysis (20%) • Congenital subglottic stenosis (15%) • Subglottic hemangioma (1.5%)
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
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.
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
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
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
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.
Types of laryngocoele Internal External Combined
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
Anterior saccular cyst Smaller in size, project into laryngeal lumen in anterior ventricular region
Lateral saccular cyst Larger, present as bulge in false vocal fold or ary-epiglottic fold, extend into neck
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
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
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
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
Fibre-optic laryngoscopy paralyzed vocal fold foreshortened, lateralized & flaccid
B/L abductor palsy Inspiration Expiration