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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. Overview. Discuss Pathogenesis Clinical presentation Laryngomalacia and GERD Diagnosis

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Current Concepts in Diagnosis and anagement of Laryngomalacia


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current concepts in diagnosis and anagement of laryngomalacia

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

overview
Overview

Discuss Pathogenesis

Clinical presentation

Laryngomalacia and GERD

Diagnosis

Medical and surgical management

Parental counseling

what is laryngomalacia
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; 1942. p.63–9

etiopathogenesis
Etiopathogenesis

Cartilage immaturity

Anatomic abnormality

Neuromuscular immaturity

cartilage immaturity
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

anatomic abnormality
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. 1984 Sep-Oct;93(5 Pt 1):430-7.

neuromuscular immaturity
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

laryngomalacia and gerd
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

proposed pathogenesis of gerd in lm
Proposed pathogenesis of GERD in LM

Respiration against a

fixed obstruction

Increasedobstruction

Large –ve intrathoracic

pressure

Increasedprolapse

Reflux into esophagus and

LPR

Laryngeal edema

proposed pathogenesis of gerd in lm10
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

conditions that worsen lm
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.

clinical presentation
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
complications of lm
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
classification schemes
Classification schemes

Based on symptomatology/flexible laryngoscopy

Mild

Moderate

Severe

Based on mechanism of collapse

Anterior: epiglottis

Posterior: large arytenoids

Laterally: AE folds

diagnosis
Diagnosis
  • History
  • Physical examination
  • Flexible laryngoscopy
direct laryngoscopy video
Direct laryngoscopy video

You may have to click or double-click to see the movie

complementary studies
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
management
Management
  • Medical

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.

empiric reflux acid suppression
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.

feeding modifications
Feeding modifications
  • Pacing
  • Thickening formula feeds
  • Upright feeding position
  • Small, frequent feeds
evolving concepts in surgical management of lm
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

indications for surgery
Indications for surgery

Richter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am. 2008 Oct;41(5):837-64, vii.

contraindications
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
pre operative counseling
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
anesthetic considerations
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
surgical set up
Surgical Set-up
  • Rigid bronchoscopy
    • Visualize subglottis, trachea and bronchi
    • R/O synchronous airway lesion
  • Assess VC mobility if not assessed previously
what is supraglottoplasty
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
surgical steps of supraglottoplasty
Surgical Steps of Supraglottoplasty

1

Pharyngoepiglottic fold

2

Arytenoids

AE folds

Extent of AE fold dissection

removal of redundant arytenoid mucosa
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

how much supra arytenoid mucosa is to be removed
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. 2005 Mar;69(3):305-9. Epub 2004 Dec 8.

instrumentation
Instrumentation

Cold instruments

CO2 laser

Microdebrider

post operative care
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
complications after surgery
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
epiglottopexy
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
epiglottopexy surgical technique
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.

indications for tracheotomy
Indications for tracheotomy
  • Presence of > 3 comorbidities
  • Severe sleep apnea
  • Worsening symptoms after revision supraglottoplasty
proposed algorithm for the treatment of mild and moderate laryngomalacia
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

FU @3m till resolution

Complications

proposed algorithm for treatment of severe lm
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

so what did we learn
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 80-100% of patients with LM
  • History, PE and Flexible laryngoscopy aid diagnosis
learning pearls contd
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