Congenital Lesions of Larynx

Presented by : . Anup Bhatta

Yugal Bhattrai

(MBBS , KUSMS)

Development of larynx

• Development of larynx occur during the 4th week of intra uterine life.

• The larynx begins as a slit like diverticulum(laryngotrachialgroove) in the ventral wall of the primitive pharynx.

• The groove gradually deepens and its edges fuse to form a septum.

• This septum separates the laringotrachial tube from the pharynx and oesophagus.

• Between 5th and 6th weeks,

-3 swellings appear at the . laryngeal aditus.

• An anterior swelling, a

derivative of the hypobranchial. eminence from 4th arch- forms . . . . epiglottis

• 2 lateral arytenoid

Swellings appear, derived from the . 6th branchial arch, move medially . . and form a t shaped aperture.

• Laryngeal lumen- temporarily occluded at 8 weeks gestational age as a result of epithelial proliferation.

• By the 10th week of gestation, recanalization occurs and consequently pair of laryngeal ventricles are formed.

• The larynngeal ventricles are bound by mesenchyme tissue that condense and progress into false and true vocal cords.

• Laryngeal cartilaes develop from the mesenchyme of the branchial arches.

• Thyroid cartilage – from the 4th arch mesenchye as two lateral plates meet in the midline.

• Arytenoids, cornuculate, cricoid and tracheal cartilages– 6th brachial arch

• Epiglottis – develops from hypobrachial eminence

• Intrinsic laryngeal muscles develop from the mesodern of the 4th and 6th arches

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

• Congenital laryngeal stridor

Etiology:

• Exact cause is not known

1. Mal-development of cartilaginous structures

2. Gastro-esophageal reflux disease

3. Immaturity of neuromuscular control

• Characterized by excessive flaccidity of supraglottic larynx which is sucked in during inspiration producing stridor and sometimes cyanosis.

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

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

• Dilatation of laryngeal saccule and extends between thyroid

cartilage and ventricle.

• 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.

X-ray neck A.P. view

Congenital laryngeal cyst

• Arises in the aryepiglottic fold and appears as bluish

,fluid filled smooth swelling in the supraglottic larynx.

• 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

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: it is a second most common abnormality

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)

disphonia

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 , most cases resolve

within 6 months.

Vocal cord lateralization (laterofixation/ cordopexy)

Cordectomy

Cordectomy + lateralization

Glottic web

• It occurs due incomplete recanalization of larynx.

• It is mostly seen in between vocal cords .

symptoms

weak cry and aphonia, hoarseness, inspiratory stridor in severe cases, dyspnea on exertion.

Signs

Indirect laryngoscopy white or pink thick or thin membrane, attached anterior to both vocal cords with a sharp and curved posterior border.

Treatment

• Thick web can be cut with knife or co2 laser and subsequent dilation by placing silicon keel.

• Tracheostomy is done when stridor and dyspnea are severe.

Glottic stenosis

It is stenosis of the glottic area which is

presented by the features of airway

obstruction.

Treatment

Endoscopic division

with knife / laser &

insertion of

McNaught laryngeal

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.

TreatmentIt is diagnosed by bronchoscopy.

Most cases resolve spontaneously by 4 years as the subglottic space

widens with age.

Tracheostomy and endotracial intubation 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• Patient is asymtomatic till 3-6 months and symptoms appears with increase of its size.

Symptoms

• appear by age 2-12 months

• Biphasic stridor, barking cough ,dyspnea, cyanosis, retraction of intercostal spaces &

normal cry .

• Agitation of the patient or crying may increase airway obstruction due to venous filling.

• 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.co2 Laser ablation 2. Cryosurgery

3. Sclerosing agent: intra - lesional injection

4. Open surgical excision

Subglottic web

• It is due to incomplete recanalization of larynx.

• Presenting features are airway obstruction , weak cry or aphonia.

Treatment

• It can be cut with a knife or co2 laser.

• Silicon keel can be inserted and subsequent dilations.

THANK YOU !