Congenital malformations of the
larynx and trachea
Michaela Máchalová
KDORL LF MU a FN Brno
Embryological introduction
• Primitive gut – at the end of 1st month –
laryngotracheal diverticulum + lung bud
• Tracheoesophageal septum separates them from
primitive gut between 4th and 5th week
• Superior end of respiratory diverticulum forms
larynx, luminisation in 10th week
• Inferior end forms trachea and 2 lung buds
• Oesophagus originates from foregut, grows
longer
• temporary obturation followed by recanalization
in 2nd month
Anathomy
• larynx: in newborns at the
level of C1-C4, glottis is 7
mm long, 4 mm wide,
subglottic region 4-5 mm
• Then it moves downward,
becomes larger and wider
• trachea:
• newborn: C4-Th3,
• 5 years: C5-Th4, 5,5 cm
long, 7 mm wide
• adult: trachea - 10-12 cm
long, 15-22 mm wide
• bifurcation: right main
bronchus 25°, left 45°
• Right bronchus: divides to
three lobar bronchi
(superior, medium, inferior)
• left bronchus: divides to
two lobar bronchi (superior,
inferior)
• Size of the main bronchi
expands with age
Congenital malformations of the larynx
• Laryngeal atresia - lethal
• diaphragma laryngis – membrane between vocal
cord, usually not complete, can be also above or
below vocal cord (symptoms vary by size and
localization: dyspnoea, voice disorders)
• dg.: direct laryngoscopy
• treatment: tracheostomy, dilatation, discision
(scar!) per laryngoscopiam, ev. Externa surgical
approach
Congenital malformations of the larynx
• laryngomalacia – temporary disorder causing
stridor in infants, caused by immaturity of
laryngeal tissue, resolves spontaneously (2
years)
• dg.: laryngoscopy – drawing of epiglottis and
other soft tissues into the larynx – narrowing
of air ways – inspiratory stridor
• treatment: conservative, rarely tracheostomy
Congenital malformations of the larynx
• Congenital cyst of the larynx: endolaryngeal
paralaryngeal, contains mesenchyme and
ectoderm, acquierd: post-inflammatory or
retention
• symptoms: depend on localization (breathing
or swallowing difficulties)
• dg: direct laryngoscopy
• treatment: excision, marsupialization
Congenital malformations of the larynx
• Internal laryngocele: herniation of laryngeal
ventricle (various contents - mucus, air),
protrudes, when the intralaryngeal pressure is
increased, symtpoms: breathing, speaking
difficulties
• dg.: direct laryngoscopy, (X-ray)
• treatment: incision, cauterization
• External laryngocele: protrudes over thyrohyoid
membrane to the neck
• treatment: surgery
Congenital malformations of the larynx
• Neurogenic laryngeal lesions: uni- or bilateral
paresis of vocal cords – often related to
congenital malformations of heart, big vessels,
CNS, oesophagus or intrathoracal organs
• dg: direct laryngoscopy – change of vocal
cords posititon in breathing and phonation,
unilateral lesions – phonation difficulties,
bilateral – phonation and breathing difficulties
• treatment: bilateral lesion - tracheostomy
Congenital malformations of the larynx
• Congenital subglottic stenosis: rare (usually
acquired), breathing problems exacerbated by
respiratory diseases and physical exercise
treatment: tracheostomy, endoscopic dilatation,
external approach surgeries
• Laryngeal, laryngotracheoesophagal cleft: rare,
defect of tracheoesophagal septum, respiratory
and swallowing difficulties, asphyxiation by food,
treatment: surgery, external approach
Congenital malformations of the larynx
• hemangioma, lymfangioma of the larynx:
congenital benign tumor formed by ectatic blood
or lymphatic vessels, more common in subglottic
region, often also in other localizations
• symptoms: dyspnoea, stridor of various severity
• dg: direct laryngoscopy, (X-ray)
• treatment: tracheostomy, conservative –
administration of propranolol (infantile
hemangioma)
Congenital malformations of the
trachea
• Tracheal agenesis/atresia: rare, lethal
• Congenital stenoses: membranous, fibrous
strictures, cartilaginous deformities (t:
dilatation, ev. end to end anastomosis,
tracheostomy – special tubes)
• tracheomalacia: most common, inmature
cartilages cause collapse of trachea in
inspiration, stridor of various severity, dg.:
tracheoscopy, t.: conservative, ev.
tracheostomy
Congenital malformations of the
trachea
• Tracheal ectasia: rare
• Tracheal diverticula: cysts, various content
• tracheoesophageal fistulas: often connected with
esophageal atresia, 3 kinds
• Symptoms of esophageal atresia: accumulation of
pharyngeal secretion – aspiration, no gas in the
intestine, TEF: cyanosis, dyspnoea of food
aspiration
• dg.: isolated fistula is difficult to diagnose –
endoscopy, esophagography
• treatment: surgical
Breathing and swallowing difficulties
caused by outer pressure – large vessels
anomalies
• Compression of trachea and esophagus: arcus aortae
duplex – complete vascular ring – dyspnoea, dysphagia
• Arcus aortae dexter – incomplete vascular ring ,
compression of esophagus
• aberant a. subclavia dx. – aortic arch branch,
„dysphagia lusoria“, pulsations at the espohagus
• arteria innominata – distal branch, compression of
trachea
• a. carotis communis sin. anomaly – compression of
trachea
• arteria pulmonalis anomaly – compression of trachea
and right main bronchus
Breathing and swallowing difficulties
caused by outer pressure – large vessels
anomalies
• symptoms: stridor, dyspnoea, swallowing
difficulties
• diagnosis: endoscopy, X-ray, esophagography,
cardiology – heart ultrasound, angiography
• treatment: depends on severity, cardiosurgery
– in cases of severe tracheal compression
(irreversible deformities of tracheal cartilages)
Outer compression of the airways
• Large congenital lymphangiomas of head and
neck – hygroma colli cysticum
• stridor, dyspnoea of various degree depending
on size and localization of the tumor
• dg.: clinical examination, ultrasound, MRI –
typical multicystic formations
• treatment: small tumors - observation, large
tumors – surgery, ev. punction
Thank you for your attention