Congenital anomalies of larynx

1. CONGENITAL ANOMALIES
OF LARYNX
Dr. SANJAY MAHARJAN
Resident, ENT-HNS
Manipal teaching hospital,
Pokhara.

2. EMBRYOLOGY:
• Organogenesis:
Develops from
endodermal lining &
adjacent mesenchyme
of foregut betn 4th & 6th
branchial arches
2

3. • Days of gestation;
• 20 – Foregut 1st identifiable
with ventral laryngotracheal
groove.
• 22 – Groove differentiates
into primitive laryngeal sulcus
and respi primordium
• 24 – Right & left lung bud
appears
Groove continues to deepen
until its laryngeal edges fuses
3

4. • 26 – Tube descends caudally,
trachea becomes separated
from esophagus by
tracheoesophageal septum
• Fusion occurs in caudal to
cranial direction
• Respi & GIT develops
separately from this point
• 32 – appearance of
mesenchymal arytenoid
swellings from 6th br arches,
just adjacent to cranial end of
LT tube
4

5. Swellings approximate each other in
midline & abut caudal end of hypobranchial
eminence forming T shaped aditus
Compression of tube by these swellings
results in fused epithelial lamina
Swelling continues to grow cranially,
differentiates into arytenoid, corniculate
cartilages and primitive aryepiglottic fold
Hypobranchial eminence gives rise to
epiglottic & cuneiform cartilage
Thyroid cartilage from 4th & cricoid+
tracheal cartilage from 6th branchial arch
5

6. • By end of 8 weeks larynx is
clearly identifiable, Epithelial
lamina dissolves, resulting in
patent opening into trachea
• 3rd month – vocal process develops
from arytenoids & thyroid cartilage
fuses in midline
• 4th month – goblet cells &
submucosal glands develops
• 5th - 7th – epiglottic cartilage
mature to fibrocartilagenous.
Corniculate & cuneiform cartilage
becomes evident
6

7. • Postnatal development:
Larynx grows rapidly during
1st 3 yrs of life, while
arytenoids remain same.
At birth betn C1 and C4
Age 2, descents inferiorly,
axis changes
Age 6, reaches betn C4 and
C7 (greater range of
phonation, because of wider
supraglottic pharynx)
7

8. CONGENITAL ANOMALIES
8

9. • Congenital anomalies, product of errors in embryogenesis or result of
intrauterine events that affect embryonic and fetal growth
• Defects in formation and growth of larynx lead to a variety of
malformations
• Clinical manifestations may be:
Respiratory obstruction
Stridor
Weak cry
Dyspnoea
Tachypnea
Aspiration
Cyanosis
Sudden death etc.
9

10. SUPRAGLOTTIC ABNORMALITIES
10

11. LARYNGOMALACIA:
• Malacia: morbid softening of a part
• Introduced by Jackson & Jackson in 1942
• Features:
Softness, flabbiness or lack of consistency of laryngeal
tissues
Thinning & hypo cellularity of its cartilages
Wrinkled, loose or redundant mucosa, esp. over arytenoid
cartilages
• m/c cause of congenital stridor (60-70%)
11

12. • Clinical feature:
Only inspiratory stridor in majority
High pitched, crowing & fluttering
1st noticed few days of birth
Tends to increase in severity during 1st 8 mnths, maxm
betn 9 to 12 months then decreases
Often intermittent (feeding and crying, sleeping in back)
May be feeding difficulties l/t failure to thrive
Rarely respi distress
12

13. • Diagnosis:
• Endoscopy
Tall , tubular, in-rolled
epiglottis with tendency to
prolapse backwards. (Ω
shaped)
Short, flaccid, medially
prolapsing aryepiglottic folds
Prominent, elongated
arytenoid cartilages
Whole supraglottis is
deepened & narrowed
13

14. • Management:
Reassuring the parents that
prognosis for the child is
favorable.
Position changes
Tracheotomy in severe cases
Supraglottoplasty is now
surgical procedure of choice.
CO2 lasers, microlaryngeal
scissors or microdebrider
used to trim redundant
tissues.
14

15. LARYNGEAL OR SACCULAR CYST:
• Saccule - At its ant end, laryngeal
ventricle has small out pouching called
saccule or laryngeal appendix.
• Blind sac extends upward betn false
vocal cord & thyroid cartilage
• Mucus filled dilatation of it
• May distort aryepiglottic fold, false
cord (lat cyst) or ventricle (ant cyst)
• No communication with airway so only
mucus
15

16. • Clinical features:
Usu asymptomatic
Occasionally enlarges &
become infected l/t hoarse
stridor & rapidly increasing
airway obstruction
• Endoscopy:
Large bluish lesion in
reason of aryepiglottic fold
16

17. LARYNGOCOELE:
• Air filled dilatation of
ventricular sinus of Morgagni
• External type:
Sac extends beyond limits of
thyroid cartilage
• Internal type:
Sac lies deep to laryngeal
cartilage
• Mixed type:
17

18. • Clinical features:
• Intermittent hoarseness or
respi distress on crying or
straining
• Rarely Soft, fluctuant swelling
in neck above thyroid
cartilage (external type)
• Plain X-rays of neck :
• Air filled sac
18

19. • Management:
Endolaryngeal excision of cyst
If not possible, Wide marsupialization
If cyst recurs, lateral cervical approach
extending via thyrohyoid membrane at
superior margin of ala of thyroid
cartilage, with subperichondrial
resection of a portion of upper part of
ala
Rarely tracheostomy is required if
severe respi distress
19

20. LYMPHANGIOMA:
• Cystic malformations (sometimes termed cystic hygromas) that
result from abnormal development of the lymphatic vessels
• Macrocystic (usually infrahyoid)
• Microcystic (usually suprahyoid)
• Combination of both
• Management:
Debulking lesion by endoscopic vaporization using a CO2 laser
Tracheostomy if extensive involvement
20

21. BIFID EPIGLOTTIS:
• Epiglottis fails to fuse in the
midline thus has a cleft
extending down to its tubercle
• Pallister-hall syndrome
• Feeding difficulties d/t
aspiration
• Stridor d/t collapse &
enfolding of two halves of
epiglottis
21

22. • Diagnosis by endoscopy
• Management:
• Amputation of epiglottis
and tracheostomy
22

23. GLOTTIC ANOMALIES
23

24. LARYNGEAL WEBS:
• Failure of complete
canalization of the larynx
• Glottic or rarely
supraglottic
• Majority involve anterior
glottis
• Occasionally, congenital
posterior, interarytenoid
web occurs
24

25. • Clinical features:
• variable degree of
respiratory obstruction and
dysphonia
• inspiratory stridor
• weak, high-pitched,
squeaky voice
• weak cry from birth +
recurrent croup in infancy,
raises suspicion
25

26. • Management:
• Small web – leave alone
• Longer web – divided endoscopically with a knife or CO2
laser & if thin subsequent endoscopic dilatation
• Thick web – endoscopic keel to prevent recurrence
• Longer, thicker webs with inadequate airway – 1st
tracheostomy then corrected at 3-4yrs via laryngofissure
26

27. LARYNGEAL ATRESIA:
• Incompatible with life
• Unless there is associated tracheo-oesophageal fistula or
emergency tracheostomy
27

28. VOCAL CORD PARALYSIS:
• Second most common congenital anomaly of larynx
• Unilateral vocal cord paralysis –
Usu acquired as a result of surgical injury to left RLN
Vocal cord is in intermediate position,
Weak cry, Mild stridor, dysphonia & sometimes aspiration.
Surgical intervention is not usually necessary
Either recovery occurs or other vocal cord compensates
28

29. • Bilateral vocal cord palsy
Usu congenital abductor paralysis
Vocal cords lie in paramedian
position with consequent inspiratory
stridor, Normal cry
Tracheostomy is necessary in 50%
29

30. • Causes:
Most cases are idiopathic
Hydrocephalus, meningocele, arnold-chiari malformation
Congenital myasthania gravis
Cardiomegaly or abnormalities of great vessels
Benign congenital hypotonia
30

31. • Management:
58% will eventually recover
Infant with an inadequate
airway and failure to thrive
will require a tracheostomy
Endoscopic laser cordotomy
or arytenoidectomy should
be considered at age of
eleven or more
31

32. SUBGLOTTIC ANOMALIES
32

33. CONGENITAL SUBGLOTTIC STENOSIS:
• Due to defective canalization of cricoid
cartilage and/or conus elasticus, resulting
in a small, elliptical, thickened cricoid
and/or excessive submucosal soft tissue.
• Third most common congenital anomaly
of larynx
• Milder degrees of stenosis present as
inspiratory or biphasic stridor as child
becomes older and more active, or as
recurrent ‘croup’
33

34. • Diagnosis requires a
microlaryngoscopy and
bronchoscopy
• MYER-COTTON GRADING
SYSTEM:
• Grade I 0–50% obstruction
• Grade II 51–70% obstruction
• Grade III 71–99% obstruction
• Grade IV 100% obstruction
34

35. • Management:
Grade I : usu. Requires no surgical intervention
Grade II: LTR with anterior cartilage grafting, generally
without stent
Mild grade III: anterior graft with posterior cricoid split
supported by an endoluminal stent
Severe grade III: anterior and posterior grafts with stenting.
Grade IV: anterior and posterior grafts with prolonged
stenting
Partial cricotracheal resection (PCTR) is an alternative
technique
35

36. 36

37. • LTR:
Involves augmentation of laryngotracheal complex by anterior
and/or posterior midline incision of cricoid with insertion of costal
cartilage grafts to expand airway
Decannulation rate:
90% for stage I & II
80% for stage III
<50% for stage IV
• PCTR:
Complete resection of stenotic segment with end-to-end
anastomosis of tracheal stump to thyroid cartilage
98% for primary and 92% for salvage surgery
37

38. SUBGLOTTIC HEMANGIOMA:
• Capillary hamartoma which enlarges
rapidly up to age of about one year and
then involutes slowly
• Gradually increasing stridor, peak at 6
wks
• Life threatening as it lies in narrrowest
part of airway
• 50% mortality if untreated
• Endoscopy:
Compressible, pear-shaped red
swelling in the subglottis on one side
38

39. • Management:
Very small: No treatment or CO2 laser vaporization
Medium-sized: Intralesional steroids and intubation
Large ones: Primary submucous resection.
Very large (circumferential or extension to trachea
and/or through tracheal wall to surrounding tissues):
tracheostomy and awaiting spontaneous involution
39

40. LARYNGEAL &
LARYGOTRACHEOESOPHAGEAL CLEFT
• Laryngeal clefts:
failure of posterior
cricoid lamina to fuse
• Laryngotracheo-
esophageal clefts:
incomplete
development of
tracheoesophageal
septum
40

41. • Classification:
• Type I: extend down to
vocal cords
• Type II: below vocal
cords into cricoid
• Type III: extends down
into cervical trachea; and
• Type IV: extends into
thoracic trachea and may
even reach carina.
41

42. • Most have Associated Congenital Anomalies like:
Tracheobronchomalacia,
Congenital heart disease
Dextrocardia and situs inversus.
Severe gastro-oesophageal reflux.
Opitz-frias syndrome (g syndrome)
Pallister-hall syndrome
42

43. • Clinical feature:
• Type I:
Cyanotic attacks on feeding & recurrent chest infections.
Stridor
• Types II and III:
Dramatic aspiration with recurrent pneumonia, sometimes
with stridor and abnormal cry.
• Type IV:
Severe aspiration, cyanosis and incipient cardiorespiratory
failure
43

44. • Management:
Depends entirely upon length of cleft.
• Short type I cleft
With no aspiration - requires no treatment
Minimal aspiration - thickening the feeds
Significant aspiration or very short type II - endoscopic repair of
cleft in two layers, nasogastric feeding tube until suture line has
healed.
• Long type II or a type III - approached anteriorly via extended
laryngofissure with low tracheostomy to cover the procedure
After successful cleft repair long-term gastrostomy feeding
44

45. • Type IV cleft –
Tracheostomy is unhelpful in stabilizing airway,
Short type – anterior approach via cervical incision
Long type – lateral cervical approach in combination with
thoracotomy or preferably anterior cervicothoracic
approach via median sternotomy
• Mortality 14% overall and 66% type IV
45

46. GENETIC AND CENTRAL CONGENITAL
NEUROMUSCULAR ANOMALIES
46

47. CRI DU CHAT SYNDROME:
• Partial deletion of the short arm
of chromosome 5
• Cat-like mewing cry in infancy,
microcephaly, downward-
slanting palpebral fissures,
mental retardation and
hypotonia
• No respiratory embarrassment
• Cry becomes less abnormal with
age
47

48. • Endoscopy
During phonation posterior part of glottis remains open,
giving it a diamond-shaped appearance
48

49. PLOTT SYNDROME:
• X-linked disorder
• Associated with laryngeal adductor paralysis.
49

50. ARTHROGRYPOSIS MULTIPLEX
CONGENITA
• Associated with multiple joint
disorders and central nervous
system abnormalities
• Bilateral vocal cord paralysis,
hypertrophy of cricopharyngeus,
and supraglottic redundancy
• Almost always require
tracheostomy
• Prognosis poor
50

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