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Laryngomalacia
PAUZIN MUPIDAH
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
Cartilage immaturity
Anatomic abnormality
Neuromuscular immaturity
Cartilage immaturity
First proposed by Sutherland and Lack in the late 19th centuryDelayed development of the cartilageneous support of the larynxTheory has been disproved No histological evidence of chondropathy Incidence not different in premature infants
Anatomic abnormalityLM 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
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
Conditions that worsen LM
PrematurityNeuromuscular disorders: higher incidence, increased severitySynchronous 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 presentationStridor 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
Complications
Complications of LM10-20% of patients present with complications
Life threatening airway obstruction
Failure to thrive
Cyanosis
Sleep apnea
Pulmonary hypertension, developmental delay and cardiac failure
Classification schemes
Based on symptomatology/flexible laryngoscopy Mild Moderate Severe
Based on mechanism of collapse Anterior: epiglottis Posterior: large arytenoids Laterally: AE folds
Classification scheme based on symptoms, flexible laryngoscopy
MILD SEVERE
Classification scheme based on mechanism of LM
ANTERIOR
POSTERIOR
LATERAL
Laryngomalacia Classification
Type I: inward collapse of the aryepiglottic folds
Laryngomalacia Classification
Type II: long tubular epiglottis which curls on itself Often occurs with type I laryngomalacia
Laryngomalacia Classification
Type III: anterior, medial collapse of corniculate and cuneiform cartilages
Laryngomalacia Classification
Type IV: posterior inspiratory displacement of the epiglottis against the posterior pharyngeal wall or inferior collapse to the vocal folds
Diagnosis
History
Physical examination
Flexible laryngoscopy
Management
Medical Empiric reflux acid
suppressionFeeding modificationsPosture repositioning
SurgicalSupraglottoplastyEpiglottopexyTracheostomyThompson 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
80-100% of patients with LM have GERDH2 receptor antagonist (RA) or Proton pump inhibitor (PPI)H2RA: ranitidine 3mg/kg three times dailyPPI: 1mg/kg dailyIf symptoms worsen6mg/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.
Indications for surgeryAbsolute 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 compromiseStridor with significant retractions
Richter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am. 2008 Oct;41(5):837-64, vii.
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
AE folds
Pharyngoepiglottic fold
Arytenoids
Extent of AE fold dissection
1
2
AE fold trimming with forceps and scissors
Surgical steps, contd…
Pre-op Post-op
Removal of redundant arytenoid mucosa
Achieve hemostasis using Afrin pledgetsLaser precautions
CO2 laser to remove redundant soft tissue over both arytenoidsPreserve inter-arytenoid mucosa
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.
Pre and Post op results
Pre-op
Post-op
Instrumentation
Microdebrider
CO2 laserCold instruments
Post-operative care
Intubation versus immediate extubationFeeding may be started when infant is awakeOne or two doses of post-operative steroidsAggressive empiric reflux therapyFollow-up in 2-4 weeksMonitor airway symptoms, apneic spells and feeding adequacy
Complications after surgery
8%, relatively uncommonIncreases with multiple comorbiditesSite-specific complications include bleeding, infection, web formation, granulation tissueTechnical complications include supraglottic stenosis – difficult to treat, so best is prevention
Epiglottopexy
Indicated if the primary level of obstruction is a retroflexed epiglottisCommonly seen in infants with global delay, hypotonia & neurological disordersTell parents that tracheostomy may be necessaryMain risks are aspiration, supraglottic stenosis
Epiglottopexy: Surgical technique
Suspension of the patientMucosa of the epiglottis is denuded with CO2 laser (1-10W) under microscopic guidanceAdditionally 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
Presence of > 3 comorbidities
Severe sleep apnea
Worsening symptoms after revision supraglottoplasty
Proposed algorithm for the treatment of mild and moderate laryngomalacia
Mild LM Moderate LM
Acid suppression
FU @3m till resolution
3m FU + FL
2m FU + FL
1m FU + FL
+
Symp worsen, persist
ComplicationsSURGERY
Feeding modification
Proposed algorithm for treatment of severe LM
Severe LM
Maximum acid suppression and SGP
FU 2-4 weeks post op
FU as recommended for mild/moderate LM
Symptoms worsen
Revision SGP Symptoms worsen
pH study and Nissen’s fundocplication
Consider PSG
Consider tracheotomy
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…
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