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

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 ary-
epiglottic 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) + 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
 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
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 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 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 /

 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