1. Congenital Lesions of Larynx
Presented by :
. Anup Bhatta
Yugal Bhattrai
(MBBS , KUSMS)
2. Development of larynx
• Development of larynx occur
during the 4th week of intra
uterine life.
• The larynx begins as a slit like
diverticulum(laryngotrachial
groove) in the ventral wall of the primitive
pharynx.
3. • The groove gradually deepens and
its edges fuse to form a septum.
• This septum separates the laringo
trachial tube from the pharynx
and oesophagus.
4. • 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.
5. • 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.
6. • 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
7. 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
11. 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
12. • Characterized by excessive flaccidity of
supraglottic larynx which is sucked in during
inspiration producing stridor and sometimes
cyanosis.
13. 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.
14. 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
17. 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
18.
19.
20. 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
22. 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.
24. 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
29. Etiology
1. 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
30. Clinical Features
Unilateral paralysis : 4 times common
Hoarse, breathy cry aggravated by agitation
Feeding difficulty Aspiration
Bilateral paralysis:
Biphasic stridor (worsens on agitation)
disphonia
35. 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.
36. 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.
37.
38. 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
39. 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
41. 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.
42. Treatment
It 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.
46. 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
49. 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
50. 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.
