1. Current Concepts in Diagnosis and anagement of Laryngomalacia
Shraddha Mukerji, MD
Harold Pine, MD
Department of Otolaryngology
University of Texas Medical Branch, Galveston
March 31, 2009

2. Overview Discuss Pathogenesis Clinical presentation
Laryngomalacia and GERD
Diagnosis
Medical and surgical management
Parental counseling

3. What is laryngomalacia?
Laryngomalacia (LM) is the commonest congenital laryngeal anomaly of the newborn characterized by flaccid laryngeal tissue and inward collapse of the supraglottic structures leading to upper airway obstruction
Jackson C, Jackson C. Diseases and injuries of the larynx. New York: MacMillan; p.63–9

4. Etiopathogenesis Cartilage immaturity Anatomic abnormality
Neuromuscular immaturity

5. Cartilage immaturity
First proposed by Sutherland and Lack in the late 19th century
Delayed development of the cartilageneous support of the larynx
Theory has been disproved
No histological evidence of chondropathy
Incidence not different in premature infants

6. Anatomic abnormality
LM is a result of the exaggeration of an infantile larynx (Iglauer1922)
May or may not be an important factor since stridor is not seen in all infants with ‘omega epiglottis’
Congenital laryngeal stridor (laryngomalacia): etiologic factors and associated disorders. Belmont JR, Grundfast K. Ann Otol Rhinol Laryngol Sep-Oct;93(5 Pt 1):430-7.

7. Neuromuscular immaturity
There is a high prevalance of neurologic disorders with LM
Some believe that neuromuscular immaturity leads to laryngeal hypotonia and LM
May be one of the several components of LM

8. Laryngomalacia and GERD
80-100% of infants with LM have GERD
It is not clear whether GERD is a cause or an effect of LM
EMPIRIC REFLUX THERAPY
choking,
frequent emesis,
regurgitation
or feeding difficulty

9. Proposed pathogenesis of GERD in LM
Respiration against a
fixed obstruction
Increased obstruction
Large –ve intrathoracic
pressure
Increased prolapse
Reflux into esophagus and
LPR
Laryngeal edema

10. Proposed pathogenesis of GERD in LM
Disruption of effective vagal tone to LES
Relative decreased LES
GERD
This pathogenesis leads credence to “NEUROLOGICAL IMMATURITY” theory of LM

11. Conditions that worsen LM
Prematurity
Neuromuscular disorders: higher incidence, increased severity
Synchronous airway lesion
20% incidence
Tracheomalacia, sublglottic stenosis, bronchomalacia, pharyngomalacia, vallecular cyst
Potentiates GERD
Surgical failures
Toynton SC, SaundersMW, Bailey CM. Aryepiglottoplasty for laryngomalacia: 100 consecutive cases. J Laryngol Otol 2001;115:35–8.

12. Clinical presentation
Stridor is the hallmark of congenital LM
High pitched, inspiratory, worsens with agitation, crying, feeding or in the supine position
Feeding symptoms
Choking, coughing, prolonged feeding time, recurrent emesis, dysphagia, weight loss
GERD symptoms
Complications

13. Complications of LM 10-20% of patients present with complications
Life threatening airway obstruction
Failure to thrive
Cyanosis
Sleep apnea
Pulmonary hypertension, developmental delay and cardiac failure

14. Classification schemes
Based on symptomatology/flexible laryngoscopy
Mild
Moderate
Severe
Based on mechanism of collapse
Anterior: epiglottis
Posterior: large arytenoids
Laterally: AE folds

15. Classification scheme based on symptoms, flexible laryngoscopy
MILD
SEVERE

16. Classification scheme based on mechanism of LM
ANTERIOR
LATERAL
POSTERIOR

17. Diagnosis
History
Physical examination
Flexible laryngoscopy

18. Direct laryngoscopy video
You may have to click or double-click to see the movie

19. Complementary studies
Chest X-ray to r/o aspiration
Esophagram
Extent and degree of reflux
r/o concomitant GI disorder
pH study if Nissen’s surgery is necessary
Sleep Study to document severity of apnea in severe LM and in surgical failures

20. Management Medical Surgical Empiric reflux acid suppression
Feeding modifications
Posture repositioning
Surgical
Supraglottoplasty
Epiglottopexy
Tracheostomy
Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Laryngoscope 2007;117:1–33.
Giannoni C, Sulek M, Friedman EM, et al. Gastroesophageal reflux association with laryngomalacia:a prospective study. Int J Pediatr Otorhinolaryngol 1998;43:11–20.

21. Empiric reflux acid suppression
80-100% of patients with LM have GERD
H2 receptor antagonist (RA) or Proton pump inhibitor (PPI)
H2RA: ranitidine 3mg/kg three times daily
PPI: 1mg/kg daily
If symptoms worsen6mg/kg of ranitidine at night + 1mg/kg of PPI daily
Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Laryngoscope 2007;117:1–33.

22. Feeding modifications
Pacing
Thickening formula feeds
Upright feeding position
Small, frequent feeds

23. Evolving concepts in surgical management of LM
Variot was the first to suggest removal of excess of AE tissue as treatment of LM
1920
Re-introduction of concept of removal of SG tissue for treatment of LM
Sporadic reports of endoscopic trimming, partial epiglottopexy, wedge resection but no definite technique
1980
Endoscopic techniques revisited and defined
Endoscopic supraglottoplasty
Epiglottopexy
Current

24. Indications for surgery
Absolute indications
Relative indications
Cor pulmonale
Aspiration
Pulmonary hypertension
Difficult-to-feed child who has failed medical intervention
Hypoxia
Weight loss with feeding difficulty
Apnea
Recurrent cyanosis
Failure to thrive
Pectus excavatum
Stridor with respiratory compromise
Stridor with significant retractions
Richter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am Oct;41(5):837-64, vii.

25. Contraindications Relatively uncommon Proceed with caution
Patients with comorbidities
Patients with multiple levels of airway obstruction
Postpone surgery till resolution of URI
WEIGHT AND AGE ARE NOT CI TO SURGERY

26. Pre-operative counseling
Overnight hospitalization in the ICU
The stridor will improve, but NOT DISAPPEAR
Expect feeding improvement
Reflux precautions and medications to be continued
Risk of revision surgery

27. Anesthetic considerations
Spontaneous breathing analgesia
ETT in the nasopharynx, mouth
Spray (1% lidocaine <2 years, 2% ≥ 2yrs)
0.5 mg/kg of decadron
Intubation only for unstable patients or patients with poor pulmonary reserve
GOOD COMMUNICATION WITH THE ANESTHESIOLOGISTS

28. Surgical Set-up Rigid bronchoscopy
Visualize subglottis, trachea and bronchi
R/O synchronous airway lesion
Assess VC mobility if not assessed previously

29. What is Supraglottoplasty
It is a surgery designed to treat LM that aims to trim the aryepiglottic folds and remove soft tissue, overriding the arytenoids

30. Surgical Steps of Supraglottoplasty1
Pharyngoepiglottic fold 2
Arytenoids
AE folds
Extent of AE fold dissection

31. AE fold trimming with forceps and scissors

32. Surgical steps, contd…
Pre-op
Post-op

33. Removal of redundant arytenoid mucosa
Achieve hemostasis using Afrin pledgets
Laser precautions
CO2 laser to remove redundant soft tissue over both arytenoids
Preserve inter-arytenoid mucosa

34. How much supra-arytenoid mucosa is to be removed?
Suction test
Polonovski JM, Contencin P, Francois M, et al. Aryepiglottic fold excision for the treatment of severe laryngomalacia. Ann Otol Rhinol Laryngol 1990;99:625–7.
Zalzal GH, Collins WO. Microdebrider-assisted supraglottoplasty Int J Pediatr Otorhinolaryngol Mar;69(3): Epub 2004 Dec 8.

35. Pre and Post op results
Pre-op
Post-op

36. Instrumentation
Cold instruments
CO2 laser
Microdebrider

37. Post-operative care Intubation versus immediate extubation
Feeding may be started when infant is awake
One or two doses of post-operative steroids
Aggressive empiric reflux therapy
Follow-up in 2-4 weeks
Monitor airway symptoms, apneic spells and feeding adequacy

38. Complications after surgery
8%, relatively uncommon
Increases with multiple comorbidites
Site-specific complications include bleeding, infection, web formation, granulation tissue
Technical complications include supraglottic stenosis – difficult to treat, so best is prevention

39. Epiglottopexy
Indicated if the primary level of obstruction is a retroflexed epiglottis
Commonly seen in infants with global delay, hypotonia & neurological disorders
Tell parents that tracheostomy may be necessary
Main risks are aspiration, supraglottic stenosis

40. Epiglottopexy: Surgical technique
Suspension of the patient
Mucosa of the epiglottis is denuded with CO2 laser (1-10W) under microscopic guidance
Additionally the epiglottis can be secured to the tongue base with 4.0 vicryl
Whymark AD, Clement WA, Kubba H, et al. Laser epiglottopexy for laryngomalacia:10 years’ experience in the west of Scotland. Arch Otolaryngol Head Neck Surg 2006;132:978–82.

41. Indications for tracheotomy
Presence of > 3 comorbidities
Severe sleep apnea
Worsening symptoms after revision supraglottoplasty

42. Proposed algorithm for the treatment of mild and moderate laryngomalacia
Moderate LM
Mild LM
1m FU + FL
2m FU + FL
Acid suppression +
Feeding modification
3m FU + FL
Symp worsen, persist
SURGERY
till resolution
Complications

43. Proposed algorithm for treatment of severe LM
Consider tracheotomy
Severe LM
Consider PSG
Maximum acid suppression and SGP
Symptoms worsen
Symptoms worsen
Revision SGP
FU 2-4 weeks post op
pH study and Nissen’s fundocplication
FU as recommended for mild/moderate LM

44. So what did we learn?
LM is the commonest congenital anomaly of the newborn larynx.
80-90% of patients have a benign course
High pitched inspiratory stridor is the hallmark clinical presentation
Feeding difficulties and GERD are seen in % of patients with LM
History, PE and Flexible laryngoscopy aid diagnosis

45. Learning pearls contd…
Identifying patients who will benefit most from surgery is of paramount importance
“Less is More” when performing surgery on the infant larynx
Strict FU and reflux therapy

46. QUESTIONS?