Congenital causes of stridor

1. PEDIATRIC
STRIDOR
DR. SHIVRAM GAUTAAM.M

2. PEDIATRIC AIRWAY ANATOMY

3. AIRWAY ANATOMY
 The newborn larynx is about 1/3 the
size of the adult counterpart.
 The epiglottis is also narrower in
infants.
 The larynx lies at the level of the c3/4 at
birth.
 By 15 years of age it will descend to the level
of the c6

4.  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 sub glottis
 The subglottic region is about 4 to 5
mm in diameter
 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.

6. DIFFERENCES BETWEEN PEDIATRIC AND ADULT AIRWAY
 More rostral larynx
 larger tongue
 Size –relatively smaller
 Mucosa – more reactive and prone to
airway infections
 Angled vocal cords
 Curled epiglottis
 Funneled shaped larynx
 Immature vocal fold structure

7. POISEUILLE’S LAW
 airway resistance is inversely proportional to
the fourth power of its radius
 50% reduction in the radius of the airway
therefore results in a 16-fold increase in
resistance to airflow.
 One millimetre of narrowing in a 4 mm
diameter infantile airway thus results in a
75% change in airflow

8. BERNOULLI’S PRINCIPLE
 Increased airflow velocity results in negative
pressure on the walls of the airway leading to
inward collapse.
 smooth or ‘laminar’ airflow then becomes
more turbulent

9. STRIDOR AND STRETOR
Noise originating in the larynx or
trachea is typically high-pitched and
termed ‘stridor’. It may have a
musicalquality
the low-pitched snoring type of noise
made by naso- and oropharyngeal
obstruction (and rarelyby the
supraglottic larynx) has a rougher
quality and is best described as ‘stertor’

10. HISTORY
 Intubation
 Admission in SCBU
 h/o difficult delivery
 Feeding history
 Stridor if presents at birth is generally
due to congenital fixed narrowing
such as laryngeal web, subglottic or
b/ vc palsy
 Dynamic conditions like
laryngomalacia become evident in
first few weeks of life

11. PATTERN OF STRIDOR
laryngomalacia – stridor is better when the child is at
rest or asleep but made worse by crying, feeding and
excitement.
Upper airway obstruction at the level of the
pharynx stridor is worse when the child is asleep
and is associated with stertor.
• Stridor is greatest in a supine position can occur
with a pedunculated laryngeal mass ,
supralaryngeal obstruction such as micrognathia
and resultant tongue base occlusion.
• Improvement in the airway with crying occurs in
significant nasal obstruction such as bilateral
choanal atresia.

12. SYMPTOMS

13. LOCAL EXAMINATION POINTS
 Mallampati staging
 Loose/missing teeth
 Size and configuration of palate
 Size and configuration of mandible
 Location of larynx
 Cyanosis
 Subcostal, intercostal and suprasternal recession
 Nasal flaring
 head bobbing
 abnormal posturing; the child may adopt
a position of neck extension
 Nasal patency should be assessed with a
metal instrument or mirror, a wisp of
cotton wool or using the bell end of a
stethoscope

14. INVESTIGATIONS
 Chest radiograph-
 CT/MRI
 Awake flexible endoscopy
 Airway fluoroscopy/bronchography
 ECG- r/o vascular compression
 Laryngeal ultrasound- r/o cysts polyps and
papilloma
 Flow volume loops to diff intrathoracic and
extrathoracic obstruction
 Reflux assessment –with radioopaque contrast
study and milk scan, pH study, endoscopy
 Laryngotracheobronchoscopy is the gold standard
in the assessment of the stridulous child

16. CONGENITAL LESIONS

17. SUPRAGLOTTIS- LARYNGOMALCIA
 Most common congenital laryngeal anomaly.
 60 percent of laryngeal problems in the newborn.
 Boys are affected twice as often as girls.
 self-limiting condition, but when severe may produce Fatal outcomes .
 characterized by partial or complete collapse of the supraglottic structures on inspiration
 Severe cases may require intubation or tracheotomy to secure the airway

18.  epiglottis is long and curled (omega-shaped)
 the aryepiglottic folds are short and tightly tethered to
the epiglottis;
 redundant mucosa and submucosa of the aryepiglottic
folds medially
 Inspiratory stridor- tends to increase as the child
becomes more active during the first 9 months of life,
and then gradually diminishes until by the age of 2
years .
 Very rarely, stridor may persist into late childhood.

19. PATHOLOGY OF LARYNGOMALACIA
• Due to continued immaturity
of the larynx, as if the fetal
stage of laryngeal
development has persisted.
 Flaccidity or in-
coordination of the supra-
laryngeal cartilages,
especially the arytenoids

20. TYPES
 bulky arytenoids
 Short AE folds
 Floppy epiglottis

21. MANAGEMENT
• Confirming the diagnosis
by flexible laryngoscopy &
reassuring the parents that
the prognosis for the child
is favorable.
• Position changes of the infant may help
alleviate the stridor as it typically
worsens in the supine position.
• In the past, tracheotomy was the
surgical procedure of choice for severe
cases.
• Supraglottoplasty/aryepiglottoplasty
has proven successful for the correction
of supraglottic obstruction and is now the
surgical procedure of choice.
• Less severe cases- division of
aryepiglottic folds is only done

22. LARYNGEAL CYSTS-SACCULAR CYSTS AND LARYNGOCOELES
 The ventricle is the fossa bounded by the
false vocal fold and the vocal cord. The
anterior portion of the ventricle leads
superiorly to the saccule
 A saccular cyst also represents an abnormal
dilatation or herniation of the saccule of the
ventricle of the larynx;
 however, it differs from a laryngocoele in that
there is no opening into the larynx and it is
filled with mucus instead of air.
 laryngocoele is an air-filled dilatation of the
laryngeal ventricle which communicates with
the laryngeal lumen.
 It is an uncommon lesion which usually
occurs in middle age but may rarely be seen
in infancy
 When filled with mucus and infected it is
called laryngopyocoele

23.  Ant. Saccular cysts protrudes medially and
posteriorly from the saccule and so protrudes
into the laryngeal airway between the true
and false vocal cords.
 The lateral saccular cyst is most common in
infants and expands posterosuperiorly into
the false cord and aryepiglotticfold
 internal or type 1 if contained
entirely within the laryngeal
framework, and
 external or type 2 if it pierces the
thyrohyoid membrane

24. DIAGNOSIS AND MANAGEMENT
 Endoscopy
 Imaging for saccular cyts
 Saccular cysts are best treated at the initial
endoscopy by wide endoscopic
marsupialization
If the cyst recurs, then the procedure
of,choice is a lateral cervical approach

25. BIFID EPIGLOTTIS
 Bifid epiglottis is a rare laryngeal anomaly
in which the epiglottis fails to fuse in the
midline and thus has a cleftextending
down to its tubercle.
 Pallister–Hall syndrome, the cardinal
elements of which are hypothalamic
hamartoblastoma, hypopituitarism,
imperforate anus and postaxial
polydactyly
 feeding difficulties due to aspiration.
 Endoscopy establishes the diagnosis,
and
 treatment options include amputation of
the epiglottis and tracheostomy

26. GLOTTIS- LARYNGEAL WEBS
 Failure of recanalization of larynx
 75% at glottic level
 Most anterior with rarely supraglottic
involvement
 May present at birth
 Diagnosis: flexible laryngoscopy
 Airway films for extension to subglottis
 inspiratory stridor and a rather weak, high-
pitched, squeaky voice.
 associated with 22q11 deletions, other
chromosomal anomalies and cardiac
abnormalities.
 all anterior glottic webs are fairly thin posteriorly,close to
their free border, but become progressively thicker
anteriorly with increasing subglottic extension

27. PATHOLOGY
• Type I laryngeal webs involve
35% or less of the glottis.
– The true vocal cords are visible
through the web and there is little or
no subglottic extension.
– Symptoms include a mildly abnormal
cry with some hoarseness.
– Respiratory distress is usually not
a feature.
• Type II webs are anterior webs
involving 35-50% of the glottis.
– Subglottis involvement .
– Airway symptoms are uncommon
except during infection or after
intubation trauma

28. • Type III webs involve 50-75%
of the glottis.
– The web is thick anteriorly and the
true vocal cords may not be
visualized.
– There may be associated cricoid
anomalies.
– Airway symptoms are often
severe and marked vocal
dysfunction may occur
• Type IV webs occlude 75-90%
or more of the glottis.
– It is uniformly thick and the true
vocal cord is not identifiable.
– The patient is aphonic and
immediate airway management
is required at birth.

29. MANAGEMENT
Long anterior web-divided endoscopically along the margin of one
vocal cord with a knife or CO2 laser
Thin web-endoscopic dilatation may be sufficient
Subglottis extension-A keel placed endoscopically following
division can prevent recurrence
With subglottis stenosis- LTR + CCG

30. CONGENITAL VOCAL FOLD PARALYSIS
• a significant cause of stridor and
hoarseness in infants and
children.
• It is the second most common
cause of stridor in the newborn
behind laryngomalacia.

31. • Laryngeal paralysis may be
present at birth or may manifest
itself in the first month or two of
life.
• The neurologic impairment
reflects an injury to the vagus
nerve
 Unilateral vocal cord paralysis is
usually not congenital,most cases
being acquired as a result of
surgical injury to the left recurrent
laryngeal nerve, often following
correction of a congenital cardiac
anomaly

32. • Many paralyses are idiopathic in up to 47% of
cases, -delayed maturation of vagal nuclei
• The most common causative factors include
entities such as:
– Arnold Chiari malformations,
– Hydrocephalus, neonatal hypotonia,
– Multiple peripheral paralysis (myasthenia
gravis).
– Other causes include birth trauma and
cardiac anomalies.
• Associated laryngeal lesions such as clefts and
stenosis are also commonly often found
 flexible fibre-optic laryngoscopy
a formal MLB under general anaesthesia is
essential

33. SYMPTOMS
• Stridor
• Ineffective cough, aspiration,
recurrent pneumonia, and feeding
difficulties
• Consistent stridor, cyanosis, and
apnea
• Voice and cry, however, may be
normal particularly in cases of
bilateral vocal cord paralysis.
• Hoarseness and dysphonia are
common in cases of unilateral
vocal fold paralysis

34. MANAGEMENT
tracheostomy
• Endoscopic CO2 laser
cordotomy- as early as 2 yrs
• open arytenoidectomy at 4-5 yrs
• artenoidpexy,
• arytenoid separation with
cartilage grafting or laser
arytenoidectomy and
• cordectomy

35. SUBGLOTTIS- CONGENITAL SUBGLOTTIC STENOSIS
• acquired or congenital.
• third most common congenital
airway problem (after
laryngomalacia and vocal cord
paralysis).
• failure of the laryngeal lumen to
recanalize properly during
embryogenesis.
• SGS is considered congenital if there is no history
of endotracheal intubation or other forms of
laryngeal trauma.
• Subglottic stenosis is defined as
a subglottic lumen 4.0 mm in
diameter or less at the level of
the cricoid in a full term infant.
• The normal newborn subglottic
diameter is 4.5 – 5.5 mm and in
premature neonates around 3.5
mm.

36. • Membranous and cartilaginous
types.
• Membranous SGS is usually
circumferential and consists of
fibrous soft-tissue thickening.
• The cartilaginous type usually
results from a thickened or
deformed cricoid cartilage

37. • The severity of congenital
subglottic stenosis depends on
the degree of SG narrowing.
• Children with subglottic stenosis
usually present with stridor
and/or respiratory distress.
 MYER COTTON GRADING
 • Grade I : 0–50% obstruction
 • Grade II: 51–70% obstruction
 • Grade III: 71–99% obstruction
 • Grade IV: 100% obstruction

38. • The McCaffrey system
classifies laryngotracheal
stenosis based on the
subsites involved and the
length of the stenosis.
• Four stages are described
• Stage I lesions are confined to the subglottis
or trachea and are less than 1cm long
– Stage II lesions are isolated to the
subglottis and are greater then 1 cm
long
– Stage III are subglottic/tracheal
lesions not involving the glottis
– Stage IV lesions involve the glottis

39. MANAGEMENT
Grade I subglottic stenosis usually requires no surgical
Intervention
. Grade II stenosis - LTR with anterior cartilage grafting +/−posteriorcricoid split.
Mild grade III stenosis anterior graft with posterior cricoid split +/− posterior cartilage grafting.
Severe grade III stenosis(a pinhole airway) requires both anterior and posterior grafts.
Grade IV stenosis demands anterior and posteriorgrafts with prolonged stenting.
For severe grade III andfor grade IV stenosis, PCTR is an alternative technique

40. SUBGLOTTIC HEMANGIOMA
 Infantile subglottic haemangioma is a
well-recognized cause of gradually
worsening inspiratory or biphasic
stridor presenting in the first few
weeks of life
 with 85% presenting within the first 6
months.
 The natural history
 proliferative phase lasting 6–
12 months
 complete involution over 1–5
years

41.  ENDOSCOPY-compressible, pear-shaped red
swelling in the subglottis on oneside, left more
commonly than right
 Tracheostomy will maintain the airway until
involution occurs
 In past radiotherapy, CO2 laser ablation,
systemic steroids, intralesional steroid
injection followed by intubation,and interferon
alpha-2a. However, all of these are associated
with unwanted side effects
 PROPRANALOL-Treatment is initiated at 1
mg/kg/day for 1 week, then
 advancing to 2–3 mg/kg/day as tolerated.33
Propranolol has proven to be effective at
reducing/resolving stridor in as little as 24
hours.
 Treatment is continued till the proliferative
phase

42. LARYNGEAL CLEFTS
 result from failure of the posterior
cricoid lamina to fuse
 laryngotracheo-oesophageal clefts
there is also incomplete
development of the tracheo-
oesophageal septum.
 Benjamin and Inglis classification
 A type I cleft extends down to the level of
the vocal cords;
 a type II cleft extends below the vocal
cords into the cricoid;
 a type III cleft extends down into the
cervical trachea; and the, fortunately rare,
 type IV cleft extends into the thoracic
trachea and mayeven reach the carina.

43.  Opitz-Frias syndrome (G syndrome)
comprising hypertelorism, cleft lip and palate.
laryngeal cleft and hypospadias;
 Pallister–Hall syndrome , consisting of
congenital hypothalamic hamartoblastoma,
hypopituitarism, imperforate anus and
postaxial polydactyly, and sometimes
including a laryngeal cleft.
 Symptoms
 Type1-cyanotic attacks on feeding and
recurrent chest infections.
 Type 2 and 3-clefts produce dramatic
aspiration with recurrent pneumonia,
sometimes with stridor and an abnormal cry
 Type-4-aspiration, cyanosis and incipient
cardiorespiratory failure.

44. MANAGEMENT
 A short type 1 cleft-no aspiration requires no
treatment. Thick feeds
 Short type 2 – repaired endoscopically
 Long type 2 and 3- needs to be approached
anteriorly through an extended
laryngofissure with a low tracheostomy
 Surgical repair of the cleft is undertake in
three layers in an effort to optimize healing:
the two mucosal layers are reinforced by an
interposition graft oftibial periosteum or
temporalis facia
 Type 4 cleft-will require a lateral
cervical approach in combination with
a thoracotomy, or preferably an
anterior cervicothoracic approach via
a median sternotomy with repair on
extracorporeal membrane
oxygenation (ECMO) or
cardiopulmonary bypass

45. TRACHEA AND BRONCHI- STENOSIS
 The most common finding in congenital
tracheal stenosis is a segment of complete
tracheal cartilaginous rings with an airway
lumen as narrow as 2 mm
 A long-segment congenital tracheal
stenosis (LSCTS) is one that is over 1 cm in
neonates and 1.5 cm in infants or greater than
50% of the length in older children
 Sixty per cent of patients with LSCTS will
have associated malformations such as
pulmonary artery sling, right-sided aortic arch,
subglottic stenosis and tracheoesophageal
fistula
 biphasic stridor, respiratory distress, tracheal
tug and episodes of cyanosis

46. MANAGEMENT
 Suspected LSCTS should be assessed
and managed by a multidisciplinary team
including cardiothoracics, ENT surgeons,
upper GI surgeons, respiratory and
cardiac paediatricians, interventional
radiologists and allied healthcare
professionals
 Endoscopy ‘gold standard’ investigation.
 MLB demonstrates complete tracheal rings
without the posterior bulge of trachealis
 unsheathed 1.9 mm telescope to survey the length
of the narrow segment and assess the state of the
distal trachea, carina and bronchi.
 Bronchoscopy and bronchography (B&B)
demonstrate the the size of the tracheal lumen and
outline the trachea and bronchi distal to the stenosis
 Optical coherence tomography (OCT) can be
combined with B&B to confirm the presence of
complete rings if.
 Contrast CT and an echocardiogram are essential in
view of the high incidence ofassociated anomalies of
the heart and great vessels

47.  MILD CASES- NO INTERVENTION
 VERY NARROW SHORT SEGMENTS-Augmentation
tracheoplasty with costal cartilage grafting or
a pericardial flap or free patch
 balloon dilatation is used as an adjunctive
treatment modality for granulations and
fibrous stenoses such as may develop post-
operatively at the site of an anastomosis

48.  For LSCTS, the gold standard treatment of
choice is slide tracheoplasty with the patient
on cardiopulmonary bypass with concurrent
repair of coexisting cardiovascular anomalies.
 The tracheoplasty is performed by dividing the
stenosis at its midpoint, incising the proximal
and distal narrowed segments vertically on
opposite anterior and posterior surfaces and
sliding these together.
 The stenotic segment is thus shortened
by half, the circumference is doubled, and
the luminal cross section quadrupled

49. TRACHEOMALACIA AND BRONCHOMALACIA
 Tracheomalacia is a condition in which there is
reduced stiffness of the tracheal wall, resulting in
abnormal collapse of the trachea during
expiration
 a widening of the trachealis relative to the
cartilage rings, which become C-shaped instead
of horseshoe-shaped.
 Normally, the ratio is 1 : 4 or 1 : 5, but in
tracheomalacia it may be closer to 1 : 2
 Tracheomalacia is traditionally classified as
primary (idiopathic), due to an intrinsic
abnormality in the wall of the airway
 secondary, due to another associated anomaly
or to external compression.
 The primary form is less common and tends to
affect a longer segment of the airway.
 Secondary tracheomalacia is usually more
localized and may be associated with TOF or
laryngeal cleft or with extrinsic compression by
an anomaly of the great vessels or a mediastinal
mass.

50.  A localized secondary tracheomalacia is the
suprastomal collapse which arises above most
long-standing pediatric tracheostomies,
produced by pressure from the convexity of the
tracheostomy tube.
 In first few weeks of life The stridor of
tracheomalacia becomes apparent and consists
of a very variablehigh-pitched prolonged
expiratory noise.
 This may be accompanied by a harsh, barking
cough, especially in the localized form of the
condition. And may be associated with cyanotic
attacks termed dying spells
 Investigations
 To r/o other anomalies, CT angiogram
 Barium swallow
 Contrast bronchography
 MLB

51. MANAGEMENT
 Mild tracheobronchomalacia (less
than 75% collapse) requires no
intervention, and the stridor can be
expected to resolve spontaneously by
around the age of 2 years.
 Severe tracheobronchomalacia
(more than 75% collapse) may
require treatment, especially if
associated with failure to thrive.
 The severe localized tracheomalacia often
associated with a TOF usually responds
well to an aortopexy
 extended tracheostomy tube will
effectively support a midtracheal malacic
segment, but this is not a satisfactory
solution for lower-end tracheal or for
bronchial collapse
 Long term CPAP
 internal or external stenting of the trachea,
 segmental resection and cartilage grafting

52. TRACHEOOESOPHAGEAL FISTULA
• Feeding difficulties (coughing, choking and cyanosis) and
breathing problems
• Associated with congenital heart (VSA, PDA, TOF),
VACTERL, GI, musculoskeletal and urinary tract defects
• Occurs in 1/ 3000-5000 births
• Most common type is the blind esophageal pouch with a
fistula between the trachea and the distal esophagus (87%)

54.  Radiograph of a neonate
with suspected esophageal
atresia.
 Note the nasogastric tube
coiled in the proximal
esophageal pouch The
prominent gastric bubble
indicates a concurrent
tracheoesphageal fistula
(open arrow)

55. MANAGEMENT
 Management of OA and TOF is
ligation of the fistula and end-to-
end anastomosis of the
oesophagus before that a
bronchoscopy is done to locate
the exact site of fistula.

56. VASCULAR COMPRESSION- VASCULAR RING
 The commonest vascular ring is a double
aortic arch.
 In this abnormality the ascending aorta
divides into two arches, one of which passes
to the right of the trachea and the other to
the left, reuniting posterior to the
oesophagus to form the descending aorta on
the left.
 A less common and less constricting ring is
produced when there is a right-sided aortic
arch and descending aorta associated with
an aberrant left subclavian artery.
 In this situation the ring is completed by the
ligamentum arteriosum which passes to the
left of the trachea, con- necting the
descending aorta to the pulmonary trunk.

57. MANAGEMENT
 A barium swallow is diagnostic,
showing a characteristic double
impression upon the column of con-
trast, and an echocardiogram will
confirm the anomaly.
 Surgery

58. VASCULAR SLING
 The commonest vascular sling is an
aberrant innominate artery. The artery
arises further to the left and more pos-
teriorly than usual, and crosses the
anterior surface of the trachea obliquely
just above the carina from the left inferi-
orly to the right superiorly
 expiratory stridor,v cough, recurrent chest
infection and sometimes reflex apnoea.
 bronchoscopic appearances are diagnostic,
with a charac- teristic sloping, pulsatile
compression of the trachea 1–2 cm above
the carina which is most marked on its
anterolateral aspect
 Upward pressure with the tip of the
bronchoscope compresses the artery against
the ster- num and obliterates the right radial
pulse.

59.  A ‘pulmonary artery sling’ is produced
by an anomalous left pulmonary artery,
which arises on the right and passes
between the trachea and oesophagus,
compressing both
 Associated with lower tracheal stenosis
 Surgical reanastomosis is needed

60. CHOANAL ATRESIA
 Complete nasal obstruction of the newborn
 Occurs in 0.82/10 000 births
 During inspiration, tongue pulled to palate, obstructs oral airway
 Unilateral nare (right>left)
 Bilateral choanal atresia is airway emergency
 Death by asphyxia
 Associated with other congenital defects

61. • Immediate diagnosis is by
passage of infant rubber
catheter
• Presence of nasal secretions
without airbubbles
• Feeding difficulties.
• CHARGE
• Bilateral choanal atresia to be
managed with early surgical
intervention
• The choanae are made patent by
dilators of various sizes.
• Drill for bony atresia, lasers are
also used
• Post op period, stent is places for
minimum of 6 weeks to prevent
restenosis

62. PIERRE ROBIN SYNDROME
 Occurs in 1/8500 births
 Autosomal recessive
 Mandibular hypoplasia, micrognathia, cleft
palate, retraction of inferior dental arch,
glossptosis
 Severe respiratory and feeding difficulties
 Associated with OSA, otitis media, hearing
loss, speech defect, ocular anomalies, cardiac
defects, musculoskeletal (syndactyly, club
feet), CNS delay, GU defects)

63. MANAGEMENT
OF ACUTE
AIRWAY
OBSTRUCTION
 Child arriving in a emergency room with stridor
>>>pediatrician assess the degree of stridor/ history in
parallel>>>nurse will be checking the oxygen saturation and
call the anaesthetist and the ent surgeon and alert the
operating room
 Medical management- o2 through face mask
 Heliox-70% helium and 30% oxygen inhalation
 Steroids-nebulized budesonide (2 mg) or oral or
intramuscular dexamethasone (the optimal dose needs
to be defined; 0.15–0.6 mg/kg
 Nebulized adrenaline (400 μg/kg or 0.4 mL/kg of 1 :
1000 to a maximum of 5 mL) may result in a transient
improvement within 10-30 minutes, lasting up to 2
hours.

64. SURGICAL
 Emergency tracheostomy
 right child should be taken at the right time, by the right people,
to the right place, by the right form of transport, and receive the
right care throughout- APLS

65. ANY PEDIATRIC CASE IS AN EMERGENCY
THANK YOU