ENT

Lecture 21

Congenital disorders of the larynx

 The larynx develops from the
fourth and fifth branchial
arches. At the third week of
gestation, the respiratory
primordium is derived from the
primitive foregut to later form
the lung bud and later the
bronchial bud which will
eventually develop into the
tracheobronchial tree.
 At the fourth and fifth week of
gestation the tracheo-
oesophageal folds fuse to
form the tracheo-oesophageal
septum leading to the
separation of the tracheal
airway lumen from the
esophageal digestive tract.
The pediatric Larynx

 The newborn larynx is about one third the size of the

adult counterpart.
 The diameter of the subglottic and glottis are
narrower which leads to an increase propensity for
airway obstruction and compromise.
 The subglottic region is about 4 to 5 mm in diameter.
 The epiglottis is also narrower in infants.
 The larynx lies at the level of the third/fourth cervical
vertebrae at birth.
 By fifteen years of age it has descend to the level of
the sixth vertebrae.


 These dimensions leave little margin for obstruction

in the infant, unlike the adult.
 The narrowest portion of the airway in the older
child and adult is the glottic aperture, while the
narrowest part of the airway in the infant is the
subglottis. Of sub glottic

 A diameter of 4.0 mm is considered the lower limit

of normal in a full term infant and 3.5 mm in a
premature infant.
 Indeed, an infant with one millimeter of glottic
edema will experience a 35% obstruction of the
airway.
 In the subglottis, one millimeter of circumferential
edema leads to over 60% narrowing.
Clinical Manifestations of congenital
anomalies
 Respiratory obstruction
 Stridor
 Weak cry
 Dyspnoea
 Tachypnea
 Aspiration
 Cyanosis
 Sudden death
 Laryngomalacia
 Subglottic stenosis
 Subglottic hemangioma
 Laryngotracheal clefts
 Laryngocele
 Laryngeal web/ atresia
 Vocal cord palsy
Laryngomalacia Malacia > soft

The most common type

The larynx is of an exaggerated infantile type. The
epiglottis is long, narrow and folded backwards(omega
shaped). The laryngeal inlet is therefore deep and
narrow.
Stridor is the main symptom and appears within the first
2 weeks of life, it is intermittent and inspiratory,
diminished by rest and sleeping and on lying on prone
position, and increase in severity on exertion and
crying.


The condition will disappear between the 2nd and 5th

years of life. Infrequently it is sever causing feeding
Rare
difficulties, failure to thrive, apnea and cyanosis.
On examination the laryngeal inlet is deep and narrow
and there is collapse of the supraglottic structures on
inspiration Stridor Due to thining of these structures

Treatment: reassurance is usually all that is necessary.

Surgery is rarely needed.
 Normal
Larynx

Collapse of The
arytenoid supraglottic
mucosa; structures
shortened are pulled
aryepiglottic into the
folds; lumen
tubular around a
epiglottis vertical axis
with with
posterior inspiration
collapse

Laryngomalacia
 Subglottic stenosis less common but more Serious

Defined as a cricoid diameter of less than 3.5 mm in full

term infant. measuredeitherby Radiology or throughoperation whichsite f Else
can insert

Clinical features: stridor either inspiratory or biphasic

which is partially relieved by rest and worsened by
exertion but not affected by posture. Feeding difficulty
and failure to thrive Common 1 5intermittentfeeling
Treatment: conservative in mild cases. Tracheostomy in
sever cases, growth of the subglottis allow
e a
decannulation after some years or laryngotracheal
I reconstruction to expand the cricoid. Usually,
means removal f
tracheostomy or
laryngotracheal
reconstruction is needed.
Tracheostomy
Subglottic hemangioma
The difference is that with stenosis, there is fibrosis while in hemangioma it is caused by vascular tumor.

 Clinically is indistinguishable from subglottic stenosis

and the treatment is the same. Laser is particularly
effective. Laser is useful in vascular tumors
 Might be associated with external hemangiomas.
On cheek, lips
Laryngotracheal clefts
 Due to incomplete development of the tracheo-
esophageal septum. At 4th - 5 th week of gestation
 Clinical features: stridor, aspiration and recurrent
pneumonia.
 Diagnosis: Ba swallow and endoscopic examination
 Treatment: clefts at or above the cords treated
conservatively. While clefts below the cords cause
aspiration and require open surgical repair.
Laryngocele
 Originate from the laryngeal ventricle
 Dilated sac filled with air
 Either internal or external. Small cyst present as
hoarseness or muffled cry. Larger ones cause
inspiratory stridor.
 Treatment: endoscopy is required to evacuate and
uncap the cyst. Treatment may need to be required.
Laryngeal web/atresia
 Web formed due to arrest in development or failure of
recanalization of the larynx and consists of a fibrous
stroma covered by epithelium. The most sever form of
laryngeal web is total atresia which is incompatible
with life.
 Clinical features: hoarseness and inspiratory stridor.
 Treatment: mild cases treated conservatively while
sever cases require tracheostomy and surgical excision.
Laryngeal webs
Vocal cord palsy
 Due to birth trauma or idiopathic
 Unilateral or bilateral.
 Clinical features: inspiratory stridor and hoarseness.
 Treatment:
 Mild cases treated conservatively Unilateral

 Sever cases require tracheostomy and surgical

intervention.
 Stridor and stertor
They are not a disease !

Auditory manifestations of disordered respiratory function

Stertor:
Low pitched snoring or snuffly sound caused by obstruction of the airway above the
larynx; in the nose, nasopharynx and oropharynx.

Stridor:
Noisy breathing due to obstruction at the level of the larynx, trachea or bronchi.

Both due to turbulence of airflow within a partially obstructed respiratory tract.

While stertor is always inspiratory, stridor could be inspiratory, expiratory or

both(biphasic) Like in asthma,
and it is below
At the level of cricoid as it is the only complete ring the cricoid.
 Choanal atresia

Septal hematoma or deviation

Causes of stertor: pathology above the larynx

 1- congenital
 2- traumatic
 3- inflammatory
 4- neoplastic
 5- allergic rhinitis and nasal polyp
 6- hypertrophied tonsils and adenoid

Causes of stridor: pathology at or below the larynx

 1- congenital
 2- traumatic
 3- foreing body
 4- inflammatory
 5- neoplastic
 6- allergy
 7- pressure on the larynx, trachea or bronchi from without. Anaphylactic
reaction type I
(due to aspirin)
  Management of stertor and stridor
1- History
 a- age of onset
 b- character
 c- onset
 d- associated features
 e- review of other systems
 f- smoking and alcohol dinking
 g- pastmedical and surgical history

2- Examination
 a- general exam.
 b- vital signs
 c- nasal exam.
 d- exam. Of the oral cavity and oropharynx
 e- exam. Of the larynx
 f-exam. Of related systems : lungs,heart and CNS.
 PaO2 --> saturation of the blood.
PaCO2 --> alveolar function.

3- Investigations:
 a. Pulmonary functon test and arterial blood gases
 b. Laboratory
 c. Radiological:
 1- plain x-ray Vascular tumors
 2- CTS and MRI
 3- Ba swallow, angiography and thyroid scan
 d- endoscopy : panendoscopy

4- Treatment:
 a- hospital admission
 b- careful follow up
 c- conservative measures
 d- tracheostomy or endotracheal intubation in sever cases
 e- traetment of underlying cause