Congenital Disorders of Larynx

Congenital Anomalies of Larynx
and Stridor
Dr. Kartikeya Mishra
Assistant Professor
Department of ENT and Head Neck Surgery
• Laryngomalacia
• Congenital Vocal Fold Paralysis
• Congenital Subglottic Stenosis
• Congenital Laryngeal Webs
• Subglottic Hemangioma
• Congenital Laryngeal Atresia, Cysts, and
Lymphangioma
• Genetic and Central Congenital Neuromuscular
Anomalies
Laryngomalacia
• Most frequent(most common) congenital
laryngeal anomaly.
• Produces partial obstruction of the supraglottic

airway.
• Cause is unclear and can be immaturity and

alterations of the cartilaginous and muscular
components.
• Excessive flaccidity of supraglottic larynx which
collapses in during forceful inspiration(crying)
• Manifests at birth or soon after
• Producing stridor and sometimes cyanosis
• Direct laryngoscopy shows:

1. Elongated epiglottis, curled upon itself (omega-
shaped Ω),
2. Floppy aryepiglottic folds
3. Prominent arytenoids.
• Treatment
1. Conservative – usually disappears by 2 years of age
2. Pronate the child
3. Tracheotomy – if severe respiratory obstruction
4. Supraglottoplasty – rarely needed
Congenital vocal cord paralysis
• Second most common anomaly.
• It results from birth trauma(when recurrent

laryngeal nerve is stretched during breech or
forceps delivery) or
• Can result from anomalies of the central nervous

system
Congenital Subglottic Stenosis
• Third most common
• Incomplete recanalization of the laryngotracheal tube during

the third month of gestation
• 2 types- Membranous (mc), Cartilaginous
• Biphasic stridor+/-respiratory distress, barking cough
• Ddx-Croup(1-3 days)
• Dx-lumen diameter <4 mm in a term infant or <3 mm in a
preterm infant
• Mx-tracheotomy, resolve spontaneously,Laser ablation,
Laryngotracheoplasty.
Laryngeal clefts
• May extend to the trachea and even to the carina, RARE.
1. Interarytenoid clefts develop as a consequence of lack of

development of the inter-arytenoid and ary-epiglottic
muscles.
2. Cricoid clefts, which may be partial or total, may arise from

incomplete dorsal fusion of the limbs of the single ventral
chondriﬁc center.
3. Complete clefts, those that involve the trachea, probably

develop during separation of the trachea and esophagus at 4
to 5 weeks, as suggested by the frequent association with
tracheoesophageal ﬁstulae.
Congenital laryngeal atresia
• Non canalisation of lumen.
• Probably arises between 6 and 10 postovulatory weeks,
• If arise in: Embryonic stages-extensive Fetal-less

severe.
• They are supraglottic rather than infraglottic.

• Complete atresia, which is probably the least frequent
of congenital laryngeal anomalies,is associated with
polyhydramnios.
• Incomplete atresia arises later; hence, the vestibule

has already developed to a variable degree.
• Glottic atresia, the most frequent type, probably arises

early during the fetal period, when the epithelial
lamina has failed to undergo further delamination.
Congenital laryngeal webs
• Situated ventrally and extending dorsally to a variable
degree,
• thought to result probably from a localized failure of

splitting of the epithelial lamina into two walls,most
likely early in the fetal period.
• A special instance is a web at the vocal folds,namely at

the lower border of the epithelial lamina.
• It presents, early in the fetal period,as a perforated

membrane that obstructs the glottic opening.
Congenital laryngeal cysts
• Uncommon -- May be ventricular or infraglottic.
• They are usually in the region of the saccule but do

not communicate with the laryngeal lumen.
compare/from laryngocele
• Obstruction of the laryngeal saccule orifice in the

ventricleretention of mucusLaryngeal Cysts
• Smairway obstruction, an inaudible or muffled cry, or

dysphagia
• Dx endoscopy
• MxForceps or laser excision
Laryngocele
• It is dilatation of laryngeal saccule and extends
between thyroid cartilage and the ventricle.
• It may be internal, external or combined.
• Treatment is endoscopic or external excision.

Nonrecurrent laryngeal nerve
• Is associated with an abnormal origin of the
right subclavian artery, usually from the dorsal
aspect of the arch of the aorta.
• The condition is believed to arise from an

embryonic failure of the right aortic
arch,allowing the nerve to proceed directly to the
larynx.
Laryngeal Lymphangioma
• Rare
• Lymphatic vessel malformations
• Asymptomatic or may present with significant airway

obstruction
• DxEndoscopy
• MxTracheotomy, excision, Laser ablation,
Sclerosing agents
Genetic and Central Congenital
Neuromuscular Anomalies
Cri du chat syndrome
• deletion of chromosome arm 5p
• mewing cry (cat cry) interarytenoid muscle paralysis in an
elongated larynx with a floppy epiglottis
Plott syndrome
• x-linked disorder.
• Associated with laryngeal adductor paralysis.
Arthrogryposis multiplex congenita

• B/L vocal cord paralysis
• hypertrophy of the cricopharyngeus,
• supraglottic redundancy similar to laryngomalacia.
STRIDOR
• Stridor is noisy respiration produced by turbulent
airflow through the narrowed air passages.
• Inspiratory stridor is often produced in obstructive

lesions of supraglottis or pharynx.
• Expiratory stridor is produced in lesions of thoracic

trachea, primary and secondary bronchi.
• Biphasic stridor is seen in lesions of glottis, subglottis and

cervical trachea.
References
• Ballenger's Otorhinolaryngology 16th Ed
• Bailey's Otorhinolaryngology 4th Ed
• Scott Brown’s Otorhinolaryngology, Head and
Neck Surgery
• Moore and Persaud, The Developing Human-
Clinically Oriented Embryology, 8th Ed
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Congenital Disorders of Larynx

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Congenital Anomalies of Larynx

• Produces partial obstruction of the supraglottic

• Cause is unclear and can be immaturity and

• Manifests at birth or soon after

• Producing stridor and sometimes cyanosis

• Direct laryngoscopy shows:

• It results from birth trauma(when recurrent

• Can result from anomalies of the central nervous

• Incomplete recanalization of the laryngotracheal tube during

• 2 types- Membranous (mc), Cartilaginous

• Biphasic stridor+/-respiratory distress, barking cough

1. Interarytenoid clefts develop as a consequence of lack of

2. Cricoid clefts, which may be partial or total, may arise from

3. Complete clefts, those that involve the trachea, probably

• Probably arises between 6 and 10 postovulatory weeks,

• If arise in: Embryonic stages-extensive Fetal-less

• They are supraglottic rather than infraglottic.

• Incomplete atresia arises later; hence, the vestibule

• Glottic atresia, the most frequent type, probably arises

• thought to result probably from a localized failure of

• A special instance is a web at the vocal folds,namely at

• It presents, early in the fetal period,as a perforated

• They are usually in the region of the saccule but do

• Obstruction of the laryngeal saccule orifice in the

• Smairway obstruction, an inaudible or muffled cry, or

• It may be internal, external or combined.

• Treatment is endoscopic or external excision.

• The condition is believed to arise from an

• Lymphatic vessel malformations

• Asymptomatic or may present with significant airway

Arthrogryposis multiplex congenita

• Inspiratory stridor is often produced in obstructive

• Expiratory stridor is produced in lesions of thoracic

• Biphasic stridor is seen in lesions of glottis, subglottis and

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