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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