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Congenital Lesions of Larynx
and Evaluation of Stridor
Dr. Krishna Koirala
2019-03-18
Epidemiology
• 80 – 85 % Children of < 3 yrs of age with stridor
have congenital etiology
• 60 % : Anomalies of larynx
• 20-25 % : Anomalies of Trachea and Bronchi
• 45% patients have more than 1 anomalies
• Supraglottis:
– Laryngomalacia, Web, Saccular cyst, Congenital Laryngocele,
Cleft
• Glottis:
– Vocal cord paralysis, Web, Stenosis, Cri-du-chat syndrome
• Subglottis:
– Stenosis, Web, Hemangioma
• Trachea and bronchi: Web, Stenosis, Tracheo -malacia , Vascular
compression, Cysts
Etiology
Common laryngeal causes
• Laryngomalacia (60%)
• Congenital vocal cord paralysis (20%)
• Congenital subglottic stenosis (15%)
• Subglottic hemangioma (1.5%)
Supraglottic abnormalities
• Most common congenital laryngeal anomaly
Etiology:
• Exact cause is not known
–Maldevelopment of cartilaginous structures
–Gastro-esophageal reflux disease
–Immaturity of neuromuscular control
Laryngomalacia
Clinical presentation
• Symptoms begin few weeks after birth, progress over 9-12
months and resolve by 2 years
• Inspiratory stridor:
– Increased in supine position, feeding, respiratory infection
and exertion (crying)
– Relieved in neck extension and prone position
• Phonation and cry are normal
• Feeding difficulties, failure to thrive, dyspnea and cyanosis are
rare
Flexible laryngoscopy
• Epiglottis : elongated and longitudinally folded (omega
shaped, ), falls postero-inferiorly on inspiration
• Arytenoids : redundant and bulky , prolapse anteriorly and
medially on inspiration
• Aryepiglottic folds : Shortening and medial collapse
• Expiration results in expulsion of these structures with free flow
of air
• Rigid bronchoscopy GA : to exclude other anomalies
Omega shaped epiglottis in flexible
Laryngoscopy
Inspiration vs. Expiration
Treatment
• Reassurance
• Sleep in prone position
• Treatment of gastro-esophageal reflux disease
• Surgical management:
– Emergency Tracheostomy: kept till 2 yrs of age
– Epiglottoplasty: cautery or laser assisted
works in 99% cases
Epiglottoplasty
Medial collapse of A.E. fold Removing wedge of A.E. folds
Long tubular epiglottis Trimming the epiglottis
Medial collapse of
corniculate
and cuneiform cartilages
Removing corniculate and
cuneiform cartilages and
redundant arytenoid mucosa
Posterior epiglottis displacement Epiglottopexy
Short A.E. folds Division of A.E. Folds
Pre-operative Post-operative
Laryngeal cyst
• Commonly seen as Saccular cyst
• Usually asymptomatic
• Rapid increase in size leads to hoarseness and airway
obstruction
• Flexible laryngoscopy: Saccular swelling
• Treatment: M.L.S. excision with cold knife, cautery or
Laser, Marsupialisation, Tracheostomy
Saccular cyst
Congenital Laryngocele
• Air filled dilatation of ventricular sinus of Morgagni
• Types: Internal, external, mixed
• C/F
– Hoarseness or respiratory distress
– Neck swelling increases on Valsalva maneuver
• Investigations:
– Plain X-ray Soft Tissue Neck AP view on Valsalva
– Flexible laryngoscopy
• Treatment: Endoscopic / External Excision
Swelling increasing with Valsalva suggestive of
laryngocele
X-ray neck A.P. view with Valsalva showing
air filled space in lateral neck
Flexible laryngoscopy
Supraglottic web
Supraglottic cleft
Glottic abnormalities
Etiology
• Idiopathic : most common
• C.N.S. Lesions
– Arnold- Chiari malformation, cerebral palsy, spina
bifida, hydrocephalus, myelomeningocele
• Birth trauma
– Cervical spine, recurrent laryngeal nerve
• Mediastinum lesions: tumors, vascular malformation
Congenital vocal cord palsy
Clinical Features
• Unilateral paralysis (4 times more common than B/L
– Hoarse, breathy cry aggravated by agitation
– Feeding difficulty
– Aspiration
• Bilateral paralysis
– Inspiratory stridor : worsens upon agitation
– Near normal phonation
– Aspiration with recurrent chest infection
– Feeding difficulty
Management
• Diagnosis:
– Flexible laryngoscopy : vocal fold paralysis
– Rigid bronchoscopy  GA to r/o other anomalies
• Treatment
• Bilateral paralysis
– Vocal cord lateralization
– Subtotal Arytenoidectomy
– Tracheostomy
• Unilateral paralysis: Observation
Flexible laryngoscopy
Vocal cord lateralization
Laser-assisted Subtotal
Arytenoidectomy
Posterior Cordotomy + Submucosal
dissection
Thyroarytenoid muscle vaporization
and subtotal arytenoidectomy
Pre-op vs. post-op
Glottic web
• Treatment:
– Endoscopic division
with knife / laser
and insertion of Mc
Naught laryngeal
keel
Glottic stenosis
Treatment:
Endoscopic division
with knife / laser and
insertion of
Mc Naught laryngeal
keel
McNaught Keel
Cri-du-chat syndrome
• Cry of the cat
• Partial depletion of short arm of chromosome 5
• High pitched mewing stridor
• Diamond shaped glottic space, narrow vocal cords,
curved and elongated supraglottis
• Treatment
– Supportive care
– Genetic counselling
Subglottic abnormalities
Congenital subglottic stenosis
• Definition: diameter of subglottic lumen < 4 mm in
term infant and < 3 mm in pre-term infant
• Etiology: Incomplete recanalization of laryngotracheal
tube during 3rd month of gestation
• Types:
– Membranous: more common , mild form
– Cartilaginous: less common , severe form
• Clinical presentation
– Symptoms appear in first few months of life
– Biphasic stridor
– Normal cry
Flexible laryngoscopy
Radiology
Treatment
• Most cases resolve spontaneously by 4 years
• Tracheostomy for significant stridor
• Tube removed by 4 years when subglottic space
widens
• Laser ablation for membranous stenosis < 5 mm
• Crico -tracheal resection & Laryngo-tracheo- plasty in
patients who could not be decannulated
Tracheostomy
Laryngo- tracheoplasty
Subglottic web
Treatment:
Endoscopic
division with knife
/ laser & insertion
of McNaught
laryngeal keel
Subglottic hemangioma
• Capillary Hamartoma
• Symptoms appear by 2-12 months of age
• Biphasic stridor, barking cough & hoarse cry
• 50% have cutaneous hemangiomas of head and neck
• Flexible laryngoscopy
– Unilateral or bilateral lesion located postero-
laterally in subglottic submucosa, pink-blue in color,
sessile and easily compressible
Flexible laryngoscopy
Management
• Observation: small lesions without stridor
• Tracheostomy for significant airway obstruction
• Tube kept till 5 years
• Specific treatment
– Laser ablation
– Cryosurgery
– Sclerosing agent: intra-lesional injection
– Open surgical excision
Tracheo-bronchial
abnormalities
Tracheomalacia
Tracheal stenosis
Evaluation of Stridor
Stertor vs. Stridor
• Stertor
– Noisy breathing due to turbulent air flow through
partially narrowed air passage above the larynx
• Stridor
– Noisy breathing due to turbulent air flow through
partially narrowed air passage at or below the
level of larynx
Etiology
• Nasal
– Choanal atresia, Ethmoidal polyps
• Mandibular
– Pierre Robin Syndrome
• Tongue
– Macroglossia, Lingual thyroid
• Pharynx
– Adeno-tonsillar hypertrophy, Retropharyngeal
abscess, Neoplasm
• Miscellaneous
– Ludwig’s angina, Maxillofacial fracture, G.E.R.D.,
Mediastinal mass
Larynx and Trachea
Congenital
 Laryngomalacia
 Vocal cord palsy
 Subglottic stenosis
 Subglottic hemangioma
 Laryngeal web & atresia
 Laryngeal cyst
 Vascular compression
Acquired
• Inflammatory
– Acute Epiglottitis, Croup,
Laryngeal edema, T.B.
• Traumatic
– Accidental, iatrogenic, heat,
chemical
• Neoplastic
• Foreign body
• B/L vocal cord palsy
History Taking
• Congenital or acquired after birth
• Present only during sleep  stertor
• Related to feeding  aspiration due to laryngeal
paralysis, esophageal obstruction
• H/O Foreign body aspiration , blunt injury, endoscopy,
intubation
• Sudden onset  foreign body, injury, infection
• Long standing and progressive  Laryngomalacia,
laryngeal stenosis, neoplasm
• Respiratory timing of stridor
– Inspiratory  supraglottis
– Biphasic  glottis, subglottis or cervical trachea
– Expiratory  lower trachea, bronchi or alveoli
• Airway resistance
– Nasal flaring
– Intercostal, subcostal , supraclavicular recession
– Cyanosis
Physical Examination (1)
Physical Examination (2)
• Fever: inflammatory cause
• Stridor disappears in prone position
– Laryngomalacia, macroglossia, micrognathia,
vascular compression
• Resting respiratory rate ( tachypnea)
• Resting heart rate (tachycardia)
Investigations
• Arterial blood gas analysis
• X-Ray soft tissue Neck AP and Lateral view
• X-Ray Chest
• Flexible laryngoscopy & bronchoscopy
• Direct laryngoscopy and Rigid bronchoscopy
• C.T. scan of Neck & chest
• M.R.I. of Neck and chest
• Barium Swallow and Esophagoscopy

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Congenital lesions of larynx and evaluation of stridor

  • 1. Congenital Lesions of Larynx and Evaluation of Stridor Dr. Krishna Koirala 2019-03-18
  • 2. Epidemiology • 80 – 85 % Children of < 3 yrs of age with stridor have congenital etiology • 60 % : Anomalies of larynx • 20-25 % : Anomalies of Trachea and Bronchi • 45% patients have more than 1 anomalies
  • 3. • Supraglottis: – Laryngomalacia, Web, Saccular cyst, Congenital Laryngocele, Cleft • Glottis: – Vocal cord paralysis, Web, Stenosis, Cri-du-chat syndrome • Subglottis: – Stenosis, Web, Hemangioma • Trachea and bronchi: Web, Stenosis, Tracheo -malacia , Vascular compression, Cysts Etiology
  • 4. Common laryngeal causes • Laryngomalacia (60%) • Congenital vocal cord paralysis (20%) • Congenital subglottic stenosis (15%) • Subglottic hemangioma (1.5%)
  • 6. • Most common congenital laryngeal anomaly Etiology: • Exact cause is not known –Maldevelopment of cartilaginous structures –Gastro-esophageal reflux disease –Immaturity of neuromuscular control Laryngomalacia
  • 7. Clinical presentation • Symptoms begin few weeks after birth, progress over 9-12 months and resolve by 2 years • Inspiratory stridor: – Increased in supine position, feeding, respiratory infection and exertion (crying) – Relieved in neck extension and prone position • Phonation and cry are normal • Feeding difficulties, failure to thrive, dyspnea and cyanosis are rare
  • 8. Flexible laryngoscopy • Epiglottis : elongated and longitudinally folded (omega shaped, ), falls postero-inferiorly on inspiration • Arytenoids : redundant and bulky , prolapse anteriorly and medially on inspiration • Aryepiglottic folds : Shortening and medial collapse • Expiration results in expulsion of these structures with free flow of air • Rigid bronchoscopy GA : to exclude other anomalies
  • 9. Omega shaped epiglottis in flexible Laryngoscopy
  • 11. Treatment • Reassurance • Sleep in prone position • Treatment of gastro-esophageal reflux disease • Surgical management: – Emergency Tracheostomy: kept till 2 yrs of age – Epiglottoplasty: cautery or laser assisted works in 99% cases
  • 13. Medial collapse of A.E. fold Removing wedge of A.E. folds
  • 14. Long tubular epiglottis Trimming the epiglottis
  • 15. Medial collapse of corniculate and cuneiform cartilages Removing corniculate and cuneiform cartilages and redundant arytenoid mucosa
  • 17. Short A.E. folds Division of A.E. Folds
  • 19. Laryngeal cyst • Commonly seen as Saccular cyst • Usually asymptomatic • Rapid increase in size leads to hoarseness and airway obstruction • Flexible laryngoscopy: Saccular swelling • Treatment: M.L.S. excision with cold knife, cautery or Laser, Marsupialisation, Tracheostomy
  • 21. Congenital Laryngocele • Air filled dilatation of ventricular sinus of Morgagni • Types: Internal, external, mixed • C/F – Hoarseness or respiratory distress – Neck swelling increases on Valsalva maneuver • Investigations: – Plain X-ray Soft Tissue Neck AP view on Valsalva – Flexible laryngoscopy • Treatment: Endoscopic / External Excision
  • 22. Swelling increasing with Valsalva suggestive of laryngocele
  • 23. X-ray neck A.P. view with Valsalva showing air filled space in lateral neck
  • 28. Etiology • Idiopathic : most common • C.N.S. Lesions – Arnold- Chiari malformation, cerebral palsy, spina bifida, hydrocephalus, myelomeningocele • Birth trauma – Cervical spine, recurrent laryngeal nerve • Mediastinum lesions: tumors, vascular malformation Congenital vocal cord palsy
  • 29. Clinical Features • Unilateral paralysis (4 times more common than B/L – Hoarse, breathy cry aggravated by agitation – Feeding difficulty – Aspiration • Bilateral paralysis – Inspiratory stridor : worsens upon agitation – Near normal phonation – Aspiration with recurrent chest infection – Feeding difficulty
  • 30. Management • Diagnosis: – Flexible laryngoscopy : vocal fold paralysis – Rigid bronchoscopy  GA to r/o other anomalies • Treatment • Bilateral paralysis – Vocal cord lateralization – Subtotal Arytenoidectomy – Tracheostomy • Unilateral paralysis: Observation
  • 34. Posterior Cordotomy + Submucosal dissection
  • 35. Thyroarytenoid muscle vaporization and subtotal arytenoidectomy
  • 37. Glottic web • Treatment: – Endoscopic division with knife / laser and insertion of Mc Naught laryngeal keel
  • 38. Glottic stenosis Treatment: Endoscopic division with knife / laser and insertion of Mc Naught laryngeal keel
  • 40. Cri-du-chat syndrome • Cry of the cat • Partial depletion of short arm of chromosome 5 • High pitched mewing stridor • Diamond shaped glottic space, narrow vocal cords, curved and elongated supraglottis • Treatment – Supportive care – Genetic counselling
  • 42. Congenital subglottic stenosis • Definition: diameter of subglottic lumen < 4 mm in term infant and < 3 mm in pre-term infant • Etiology: Incomplete recanalization of laryngotracheal tube during 3rd month of gestation • Types: – Membranous: more common , mild form – Cartilaginous: less common , severe form • Clinical presentation – Symptoms appear in first few months of life – Biphasic stridor – Normal cry
  • 45. Treatment • Most cases resolve spontaneously by 4 years • Tracheostomy for significant stridor • Tube removed by 4 years when subglottic space widens • Laser ablation for membranous stenosis < 5 mm • Crico -tracheal resection & Laryngo-tracheo- plasty in patients who could not be decannulated
  • 48. Subglottic web Treatment: Endoscopic division with knife / laser & insertion of McNaught laryngeal keel
  • 49. Subglottic hemangioma • Capillary Hamartoma • Symptoms appear by 2-12 months of age • Biphasic stridor, barking cough & hoarse cry • 50% have cutaneous hemangiomas of head and neck • Flexible laryngoscopy – Unilateral or bilateral lesion located postero- laterally in subglottic submucosa, pink-blue in color, sessile and easily compressible
  • 51. Management • Observation: small lesions without stridor • Tracheostomy for significant airway obstruction • Tube kept till 5 years • Specific treatment – Laser ablation – Cryosurgery – Sclerosing agent: intra-lesional injection – Open surgical excision
  • 56. Stertor vs. Stridor • Stertor – Noisy breathing due to turbulent air flow through partially narrowed air passage above the larynx • Stridor – Noisy breathing due to turbulent air flow through partially narrowed air passage at or below the level of larynx
  • 57. Etiology • Nasal – Choanal atresia, Ethmoidal polyps • Mandibular – Pierre Robin Syndrome • Tongue – Macroglossia, Lingual thyroid • Pharynx – Adeno-tonsillar hypertrophy, Retropharyngeal abscess, Neoplasm • Miscellaneous – Ludwig’s angina, Maxillofacial fracture, G.E.R.D., Mediastinal mass
  • 58. Larynx and Trachea Congenital  Laryngomalacia  Vocal cord palsy  Subglottic stenosis  Subglottic hemangioma  Laryngeal web & atresia  Laryngeal cyst  Vascular compression Acquired • Inflammatory – Acute Epiglottitis, Croup, Laryngeal edema, T.B. • Traumatic – Accidental, iatrogenic, heat, chemical • Neoplastic • Foreign body • B/L vocal cord palsy
  • 59. History Taking • Congenital or acquired after birth • Present only during sleep  stertor • Related to feeding  aspiration due to laryngeal paralysis, esophageal obstruction • H/O Foreign body aspiration , blunt injury, endoscopy, intubation • Sudden onset  foreign body, injury, infection • Long standing and progressive  Laryngomalacia, laryngeal stenosis, neoplasm
  • 60. • Respiratory timing of stridor – Inspiratory  supraglottis – Biphasic  glottis, subglottis or cervical trachea – Expiratory  lower trachea, bronchi or alveoli • Airway resistance – Nasal flaring – Intercostal, subcostal , supraclavicular recession – Cyanosis Physical Examination (1)
  • 61. Physical Examination (2) • Fever: inflammatory cause • Stridor disappears in prone position – Laryngomalacia, macroglossia, micrognathia, vascular compression • Resting respiratory rate ( tachypnea) • Resting heart rate (tachycardia)
  • 62. Investigations • Arterial blood gas analysis • X-Ray soft tissue Neck AP and Lateral view • X-Ray Chest • Flexible laryngoscopy & bronchoscopy • Direct laryngoscopy and Rigid bronchoscopy • C.T. scan of Neck & chest • M.R.I. of Neck and chest • Barium Swallow and Esophagoscopy