Transcript Congenital lesions of larynx

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%)
Supra-glottic
abnormalities
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
Omega-shaped epiglottis
Flexible laryngoscopy
Inspiration vs. Expiration
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
Epiglottoplasty for
laryngomalacia
Problem: tubular epiglottis
Rx: trimming of epiglottis
Problem: medial collapse of
corniculate cartilages
Rx: removing cartilage +
redundant mucosa
Problem: posterior
displacement of epiglottis
Rx: epiglottopexy
Epiglottopexy
Problem: short ary-epiglottic
folds
Rx: division of ary-epiglottic
folds
Pre-op vs. Post-op
Problem: medial collapse of
ary-epiglottic fold
Rx: removing wedge of aryepiglottic folds
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
Swelling es with Valsalva
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
X-ray neck A.P. view
Flexible laryngoscopy
CT scan: mixed
laryngocoele
Endoscopic
marsupialization
External approach
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
Glottic
abnormalities
Congenital vocal
cord palsy
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) + nearnormal 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
3. Cordectomy
2. Cordotomy
4.Subtotal arytenoidectomy
5. Tracheostomy
Unilateral paralysis: Observation
Fibre-optic laryngoscopy
paralyzed vocal fold foreshortened, lateralized & flaccid
B/L abductor palsy
Inspiration
Expiration
Vocal cord lateralization
(laterofixation / cordopexy)
Cordectomy
Cordectomy + lateralization
Posterior cordotomy
Arytenoidectomy
Cordotomy + arytenoidectomy
Glottic web
Treatment:
Endoscopic division
with knife / laser &
insertion of
McNaught laryngeal
keel
Glottic stenosis
Treatment:
Endoscopic division
with knife / laser &
insertion of
McNaught laryngeal
keel
McNaught Keel
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
Sub-glottic
abnormalities
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.
Flexible laryngoscopy
Radiology
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.
Tracheostomy
Laryngo-tracheoplasty
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
Flexible laryngoscopy
Management
Observation: for small lesions without stridor
Tracheostomy: for significant airway obstruction.
Tube kept till 5 years.
Specific treatment:
1. Laser ablation
2. Cryosurgery
3. Sclerosing agent: intra-lesional injection
4. Open surgical excision
Subglottic web
Treatment:
Endoscopic
division with knife
/ laser & insertion
of McNaught
laryngeal keel
Evaluation of
Stridor
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
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 #
Etiology for stridor
Congenital
 Laryngomalacia
Acquired
1. Inflammatory:
 Vocal cord palsy
Acute epiglottitis, croup,
 Subglottic stenosis
laryngeal edema, T.B.
 Subglottic hemangioma
 Laryngeal web & atresia
 Laryngeal cyst
2. Trauma: accidental,
iatrogenic, heat, chemical
3. Neoplasm
 Vascular compression on 4. Foreign body
trachea
5. B/L vocal cord palsy
Causes of B/L vocal cord
palsy
• Thyroid surgery
• Ca thyroid
• Cancer cervical esophagus
• Cervical lymphadenopathy
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
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
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
Investigations
1. Arterial blood gas analysis: for hypoxia
2. X-Ray soft tissue neck: for epiglottitis, stenosis
3. X-Ray chest: for mediastinal lesion
4. Flexible laryngoscopy & bronchoscopy
5. Direct laryngoscopy & rigid bronchoscopy
6. C.T. scan of neck & chest
7. M.R.I. of neck & chest
8. Barium swallow & esophagoscopy
Thank You