Congenital Larynx Lesions & Stridor Evaluation Dr. Vishal Sharma

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

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

Etiology

Common congenital lesions

• Laryngomalacia (60%)

• Congenital vocal cord paralysis (20%)

• Congenital subglottic stenosis (15%)

• Subglottic hemangioma (1.5%)

Supra-glottic

abnormalities

• 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

Laryngomalacia

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 ary-epiglottic folds

Congenital laryngocoeleAir 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 cystSmaller in size, project into laryngeal lumen in

anterior ventricular region

Lateral saccular cystLarger, 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

Etiology1. 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 FeaturesUnilateral 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

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

TreatmentMost 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 Taking1. 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

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

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

Investigations1. 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